Simanski v. Hhs

REDACTED OPINION

In the United States Court of Federal Claims

No. 03-103V

Filed: February 18, 2014

Redacted Version Issued for Publication: April 2, 2014 1

* * * * * * * * * * * * * * * * * *

TODD SIMANSKI and JULIA *

SIMANSKI, as Parents and Next * Motion for Review of Special Master’s

Friends of [O.A.S.], a minor, * Decision; National Vaccine Injury Act,

* 42 U.S.C. 300aa-1 et seq.; Standard of

Petitioners, * Review; Spinal Muscular Atrophy with

* Respiratory Distress (SMARD);

v. * Guillain-Barré Syndrome (GBS);

* Chronic Inflammatory Demyelinating

SECRETARY OF HEALTH AND * Polyneuropathy (CIDP).

HUMAN SERVICES, *

*

Respondent. *

* * * * * * * * * * * * * * * * * *

Ronald C. Homer, Conway, Homer & Chin-Caplan, P.C., Boston, MA, for

Petitioners. With him was Sylvia Chin-Caplan, Conway, Homer & Chin-Caplan, P.C.,

Boston, MA.

Traci R. Patton, Trial Attorney, Torts Branch, Civil Division, United States

Department of Justice, Washington, D.C., for Respondent. With her were Debra F.

Begley, Trial Attorney, Lisa Watts, Trial Attorney, Rupa Bhattacharyya, Director, Torts

Branch, Civil Division, and Stuart F. Delery, Assistant Attorney General, Civil Division,

Washington, D.C.

OPINION

HORN, J.

On January 17, 2003, Petitioners Todd and Julia Simanski filed a timely Petition

for compensation with the National Vaccine Injury Compensation Program, pursuant to

1

This opinion was issued under seal on February 18, 2014. Although the parties had

not filed a motion for redaction, on March 28, 2014 the court contacted the parties to

determine whether redactions would be appropriate. Thereafter, Petitioners filed a

proposed redacted opinion, asking to redact the minor child’s name to initials only, and

to omit the minor’s birthdate.

the National Childhood Vaccine Injury Act of 1986, Pub. L. 99-660, Title III, 100 Stat.

3755 (codified as amended at 42 U.S.C. §§ 300aa-1 et seq. (2006)) (Vaccine Act), on

behalf of [O.A.S.], a minor, as her parents and next friends. After several years of

delay, 2 the Petitioners perfected their Petition pursuant to 42 U.S.C. § 300aa-11(c), by

filing medical records, an affidavit of Petitioner, Ms. Julia Simanski, and expert reports

by Dr. Paul Maertens and Dr. Yehuda Shoenfeld in support of their claim. Special

Master Christian Moran, 3 however, deemed the Petition still insufficient for the

Petitioners to pursue their case for compensation, and, on May 13, 2010, dismissed

their case. See Simanski v. Sec’y of Health & Human Servs., No. 03-103V, 2010 WL

2292200 (Fed. Cl. Spec. Mstr. May 13, 2010), aff’d, 96 Fed. Cl. 588 (2010), rev’d and

remanded, 671 F.3d 1368 (Fed. Cir. 2012). Subsequently, on June 14, 2010, the

Petitioners moved this court to review the Special Master’s decision to dismiss their

case, pursuant to Rule 23 of the Rules of the United States Court of Federal Claims

(RCFC) Appendix B (2013). On December 15, 2010, Judge Christine O.C. Miller, now

retired, 4 of the United States Court of Federal Claims affirmed the Special Master’s

dismissal and denied Petitioners’ Motion for Review. See Simanski v. Sec’y of Health &

Human Servs., 96 Fed. Cl. 588 (2010), rev’d and remanded, 671 F.3d 1368 (Fed. Cir.

2012). The Simanskis appealed Judge Christine O.C. Miller’s decision to the United

States Court of Appeals for the Federal Circuit. On March 6, 2012, the United States

Court of Appeals for the Federal Circuit reversed and remanded the case to this court,

“with instructions for the special master to address the merits of the Simanskis’ claim,

either by applying appropriate summary judgment standards or by conducting a hearing

and resolving the compensation claim on the merits.” Simanski v. Sec’y of Health &

Human Servs., 671 F.3d 1368, 1385 (Fed. Cir. 2012).

On August 20, 2013, Special Master Moran issued a decision on remand. This

time, after receiving evidence and holding hearings, the Special Master denied

Petitioners’ request for compensation for injury suffered by [O.A.S.], which Petitioners

claimed was the result of the vaccinations received on January 26, 2001, when she was

2

The original Petition, filed in 2003, did not specify [O.A.S.]’s injury, but stated that “[a]

fact-specific description of . . . the nature and extent of the injuries caused by the

inoculation . . . will be set forth in affidavits which will be filed and is set forth in the

medical records which, when filed, will be incorporated by reference herein.” It appears

that Petitioners first identified [O.A.S.]’s injury as Guillain-Barré Syndrome (GBS) in May

2005. Petitioners did not complete the filing requirements pursuant to the Vaccine Act,

including not filing [O.A.S.]’s medical records and the expert report prepared by Dr. Paul

Maertens, until 2008.

3

Initially, the above-captioned case was assigned to Chief Special Master Gary

Golkiewicz. On August 7, 2007, the case was reassigned to Special Master John

Edwards, and, subsequently, on August 1, 2008, was reassigned to Special Master

Christian Moran.

4

On September 25, 2013, following the retirement of Judge Christine O.C. Miller, the

above-captioned case was reassigned to the undersigned Judge.

2

two months old. See Simanski v. Sec’y of Health & Human Servs., No. 03-103V, 2013

WL 7017568 (Fed. Cl. Spec. Mstr. Aug. 20, 2013). Petitioners again filed a timely

Motion for Review to this court pursuant to RCFC 23, Appendix B. Respondent filed a

response to the Motion for Review, and the court held oral argument on November 18,

2013. At the oral argument, the Petitioners’ counsel agreed with respect to the

proceedings before the undersigned that “we’re talking only about, for the purposes of

this review, whether or not the SMARD conclusion by Special Master Moran is the

correct one.” Counsel for Petitioners also agreed at the oral argument and asserted in

the Motion for Review that “there was no need to explore in detail . . . whether the

vaccines could have adversely affected [O.A.S.]’s SMARD via the Althen test.” 5

FINDINGS OF FACT

[O.A.S.] was born in [2000] and weighed four pounds, twelve ounces. [O.A.S.]

was diagnosed with intrauterine growth retardation (IUGR), and her Labor and Delivery

Summary noted that she had a decreased muscle tone as a newborn. Otherwise,

however, she seemed healthy. On January 26, 2001, when she was two months old,

[O.A.S.] received a set of five vaccines. 6 Four days later, on January 30, 2001, she

suffered an episode of respiratory arrest and was hospitalized at Mercy Medical Center

in Des Moines, Iowa, for nearly one month, where she was intubated and placed on a

ventilator. While at Mercy Medical Center, doctors detected a respiratory syncytial virus

(RSV). 7 [O.A.S.] was diagnosed with bronchiolitis, and an x-ray indicated that [O.A.S.]

had intermittent atelectasis 8 and lung collapse. [O.A.S.] was sedated because she was

“fighting the ventilators.”

5

The test established by the United States Court of Appeals for the Federal Circuit in

Althen v. Secretary of Health and Human Services, 418 F.3d 1274 (Fed. Cir. 2005)

requires a Petitioner to show, by preponderance of the evidence, that the vaccination

brought about his or her injury by providing: “(1) a medical theory causally connecting

the vaccination and the injury; (2) a logical sequence of cause and effect showing that

the vaccination was the reason for the injury; and (3) a showing of a proximate temporal

relationship between vaccination and injury.” Id. at 1278.

6

Specifically, [O.A.S.] received Diphtheria-Tetanus-acellular-Pertussis, Hepatitis B,

Haemophilus influenza type B, inactivated polio, and pneumococcal conjugate

vaccinations.

7

The Respondent’s filings include an excerpt from the website of the United States

Centers for Disease Control and Prevention, which provides a definition for RSV as “a

respiratory virus that infects the lungs and breathing passages . . . . RSV is the most

common cause of bronchiolitis (inflammation of the small airways in the lung) and

pneumonia in children under 1 year of age in the United States.”

8

According to Dorland’s Illustrated Medical Dictionary, atelectasis is defined as

“incomplete expansion of a lung or a portion of a lung.” Dorland’s Illustrated Medical

Dictionary 171 (32nd ed. 2012).

3

On February 23, 2001, [O.A.S.] was transferred to the Mayo Clinic in Rochester,

Minnesota. While at the Mayo Clinic, she underwent further testing, including a muscle

biopsy, two electromyographies (EMGs), 9 on February 26, 2001 and March 6, 2001,

respectively, including a phrenic 10 nerve study, and a cerebrospinal fluid (CSF) protein

testing. Interpreting [O.A.S.]’s March 6, 2001 left phrenic nerve conduction study, Dr.

Suresh Kotagal concluded that [O.A.S.] suffered from a demyelinating 11 process in her

peripheral nerves. 12 From March 7, 2001 to March 10, 2001, [O.A.S.] underwent a

treatment with intravenous immunoglobulin (IVIG), after which her health improved, so

that on March 14, 2001, she was taken off the ventilator.

On March 16, 2001, [O.A.S.] was transferred from the Mayo Clinic back to Mercy

Medical Center. Her discharge diagnosis from the Mayo Clinic was “[p]robable post

infectious demyelinating neuropathy.” Upon her admission to Mercy Medical Center the

record before the court indicates that [O.A.S.] “looked fairly improved compared to

several weeks ago.” A medical record dated March 21, 2001, stated that [O.A.S.] had

“prob[able] G-B Synd [Guillain-Barré Syndrome or GBS].” 13 On March 28, 2001,

9

Dorland’s Illustrated Medical Dictionary defines electromyography (EMG) as “a

electrodiagnostic technique for recording the extracellular activity (action potentials and

evoked potentials) of skeletal muscles at rest, during voluntary contractions, and during

electrical stimulation.” Dorland’s Illustrated Medical Dictionary 602.

10

Dorland’s Illustrated Medical Dictionary defines phrenic as “pertaining to the

diaphragm of the body.” Dorland’s Illustrated Medical Dictionary 1442.

11

Dorland’s Illustrated Medical Dictionary defines demyelination as “destruction,

removal, or loss of the myelin sheath of a nerve or nerves.” Dorland’s Illustrated

Medical Dictionary 486.

12

Dr. Kotagal also noted: “My colleague, Dr. [Nancy] Kuntz, informs me that the normal

latency should be <2msec.” Dr. Kotagal and Dr. Kuntz concluded that [O.A.S.] suffered

from peripheral neuropathy, which could be characteristic of both GBS and spinal

muscular atrophy with respiratory distress (SMARD). As discussed below, in 2003,

when [O.A.S.] returned to the Mayo Clinic, Dr. Kuntz indicated the diagnosis was

SMARD.

13

Dorland’s Illustrated Medical Dictionary defines GBS as

rapidly progressive ascending motor neuron paralysis of unknown

etiology, frequently seen after an enteric or respiratory infection. An

autoimmune mechanism following viral infection has been postulated. It

begins with paresthesias of the feet, followed by flaccid paralysis of the

entire lower limbs, ascending to the trunk, upper limbs, and face.

Dorland’s Illustrated Medical Dictionary 1832.

4

[O.A.S.] was discharged from Mercy Medical Center in improved condition. At home,

she remained extubated 14 and was bottle fed.

On March 30, 2001, [O.A.S.]’s pediatrician, Dr. Emily Gavin, examined [O.A.S.]

and noted that she “was doing very well on room air without O2 [oxygen]

supplementation until just 4-5 days ago when it was noted that she would drop her

saturations when she got upset and a little bit at night when she was sleeping.”

Subsequently, however, on April 13, 2001, [O.A.S.] was readmitted to Mercy Medical

Center for respiratory failure. She was placed on a ventilator and remained on one at

the time of the Special Master’s decision. On April 17, 2001, another EMG test was

performed, which revealed deterioration from the previous EMG performed at Mayo

Clinic. On April 24, 2001, [O.A.S.] was transferred to Johns Hopkins Hospital in

Baltimore, Maryland. Her discharge report from Mercy Medical Center noted that the

“lack of a definitive diagnosis has been a problem in addressing the extent of supporting

the child.” [O.A.S.]’s April 25, 2001 progress note from Johns Hopkins Hospital stated

that [O.A.S.]’s diagnoses include “post-infectious demyelinating neuropathy vs. spinal

muscular atrophy vs. degenerative vs. other NOS [not otherwise specified]” disorders.

The April 26, 2001 Consultation Request and Report Form from Johns Hopkins Hospital

indicated that [O.A.S.]’s “diagnosis (preliminary) includes a post-infectious GBS-like

process, CIDP [chronic inflammatory demyelinating polyneuropathy 15 ], and infantile

spinal muscular atrophy (Werdnig-Hoffman).”16

Dr. Thomas Crawford at Johns Hopkins Hospital indicated in a Clinical Summary

that [O.A.S.] previously had a “provisional diagnosis of infantile GBS,” and concluded,

after performing another EMG on April 26, 2001, that [O.A.S.]’s condition is “consistent

with either a motor neuropathy or a sensorimotor axonal neuropathy.” On May 3, 2001,

14

Dorland’s Illustrated Medical Dictionary defines extubation as “the removal of a

previously inserted tube.” Dorland’s Illustrated Medical Dictionary 665.

15

According to Dorland’s Illustrated Medical Dictionary, chronic inflammatory

demyelinating polyneuropathy (CIDP) is “slowly progressive, autoimmune type of

demyelinating polyneuropathy characterized by progressive weakness and impaired

sensory function in the limbs and enlargement of the peripheral nerves, usually with

elevated protein in the cerebrospinal fluid. It occurs most commonly in young adults,

and is related to Guillain-Barré syndrome.” Dorland’s Illustrated Medical Dictionary

1491.

16

The Merck Manual of Diagnosis and Therapy indicates that there are four different

types of spinal muscular atrophies. See The Merck Manual of Diagnosis and Therapy

1907 (Mark H. Beers et al. eds., 18th ed. 2006). Type 1, Werdnig-Hoffman disease,

manifests itself in infants, by about six months of age; in type 2, intermediate, symptoms

usually manifest between three months and fifteen months; type 3, Wohlfart-Kugelberg-

Welander disease, usually manifests between age fifteen months and nineteen years;

and, type 4, has an adult onset and manifests between ages thirty to sixty. Id.

[O.A.S.]’s treating physicians only raised type 1 as a possible diagnosis for [O.A.S.].

5

[O.A.S.] was transferred from Johns Hopkins Hospital to the University of Iowa

Hospitals and Clinics. A May 3, 2001 brief discharge summary sheet from Johns

Hopkins Hospital identified [O.A.S.]’s principal diagnosis as “inflammatory and toxic

neuropathies.” At the University of Iowa Hospitals, [O.A.S.] had another EMG. Dr.

Katherine Mathews, a neurologist, wrote a May 8, 2001 report about [O.A.S.]’s history,

which indicated that [O.A.S.]’s “exam and findings are most suggestive of a peripheral

neuropathy. . . . Her clinical picture is not compatible with spinal muscle atrophy (and

DNA testing has been negative[ 17 ]).” Dr. Mathews noted on June 30, 2001 that

“[O.A.S.] is clearly getting stronger.” Dr. Mathews also indicated that she consulted Dr.

Sladky from Atlanta, Georgia, who “favors a diagnosis of an acute axonal neuropathy.”

On August 20, 2001, [O.A.S.] was transferred back to Mercy Medical Center and

on her admission, her diagnosis was indicated as “Flaccid Axonal Neuropathy.” 18 On

September 11, 2001, [O.A.S.] was discharged from Mercy Medical Center, and her

discharge report indicated that an additional IVIG therapy for [O.A.S.] was “discussed

but felt to be not useful at this time.” The discharge report also stated that [O.A.S.]

suffers from “[s]evere generalized muscular weakness secondary to peripheral

neuropathy.” On September 15, 2003, more than two years after her discharge from

Mercy Medical Center, at the recommendation of her pediatrician, Dr. Gavin, [O.A.S.]

returned to the Mayo Clinic. Her medical diagnosis on admission at the Mayo Clinic

stated: “SMA [w] resp distress (SMARD).” 19 (brackets in original). On September 17,

2003, [O.A.S.] had another EMG, which was interpreted as showing “a severe, diffuse

sensorimotor peripheral neuropathy characterized primarily by axonal loss. There has

been significant progression of findings since the prior examination dated February 26,

2001.” Dr. Kuntz at the Mayo Clinic subsequently concluded that:

All of this suggests progressive motor and sensory neuronopathy or

axonopathy. I believe that this is compatible with a recently described

17

The comment about DNA testing is incorrect. [O.A.S.]’s mother, Julia Simanski,

stated in her September 14, 2012 affidavit, submitted to the Special Master that the

DNA “testing was never done nor was blood ever sent to Germany.” The parent

Petitioners ultimately decided not to pursue any genetic testing, claiming that genetic

testing “was not going to alter [O.A.S.]'s treatment nor improve her condition,” and,

therefore, “[s]ince there is no benefit for [O.A.S.], we have decided not to seek genetic

testing.”

18

The court notes that the diagnosis of “Flaccid Axonal Neuropathy” is inconsistent with

the diagnosis of an “acute axonal neuropathy,” discussed above, favored by Dr. Sladky.

19

Respondent suggests that “[w]hile [O.A.S.] does not suffer SMA, a condition separate

and distinct from SMARD, it is clear from these records that she carried a presumed

diagnosis of a genetic neuromuscular condition, rather than GBS or CIDP.”

6

entity[ 20 ] called spinal muscular atrophy with respiratory distress or

SMARD.[ 21]

20

Dr. Richard Finkel testified for the Respondent at the February 2013 hearing that in

2003, an article on SMARD, Infantile Spinal Muscular Atrophy with Respiratory Distress

Type 1 (SMARD1), 54 Ann. Neurol. 719 (2003), authored by Dr. Katja Grohmann, was

published, which brought SMARD to the attention of pediatric neurologists. According

to his testimony, Dr. Finkel assumed that Dr. Kuntz of the Mayo Clinic, after becoming

aware of the article and seeing [O.A.S.] again, changed her diagnosis to SMARD. The

court notes, however, that the article was published in the December 2003 issue of the

Annals of Neurology (made available online in October 2003), after Dr. Kuntz examined

[O.A.S.] in September 2003. It is possible Dr. Kuntz already may have been aware of

SMARD, as the lead author of Infantile Spinal Muscular Atrophy with Respiratory

Distress Type 1 (SMARD1), 54 Ann. Neurol. 719 (2003), Grohmann, et al., had

previously published an article on SMARD in 2001. See Grohmann, et al., Mutations in

the Gene Encoding Immunoglobulin µ-binding Protein 2 Cause Spinal Muscular Atrophy

with Respiratory Distress Type 1, 29 Nat. Genet. 75, 76 (2001). Both the Special Master

in his decision, and Dr. Finkel in his testimony cited to the 2001 article. Although both

Dr. Finkel and the Special Master mark the line of demarcation for when SMARD was

widely known with the publication of the 2003 Grohmann article, it is possible they are

overstating its effect. The 2003 article has only been cited 75 times while the 2001

article has been cited over 200 times. The court also suggests that the 2003 article

likely was not the first time SMARD had become more widely known as the 2001 article

examined 29 infants with SMARD1.

21

Although Dorland’s Illustrated Medical Dictionary does not define SMARD, the

National Institutes of Health’s website defines SMARD as

an inherited condition that causes muscle weakness and respiratory

failure typically beginning in infancy. Early features of this condition are

difficult and noisy breathing, especially when inhaling; a weak cry;

problems feeding; and recurrent episodes of pneumonia. Typically

between the ages of 6 weeks and 6 months, infants with this condition will

experience a sudden inability to breathe due to paralysis of the muscle

that separates the abdomen from the chest cavity (the diaphragm).

Genetics Home Reference, Spinal muscular atrophy with respiratory distress type 1,

Nat’l Insts. of Health (Feb. 18, 2014), http://ghr.nlm.nih.gov/condition/spinal-muscular-

atrophy-with-respiratory-distress-type-1. The National Institutes of Health’s website also

indicates that “SMARD1 appears to be a rare condition, but its prevalence is unknown.

More than 60 cases have been reported in the scientific literature.” Id. As noted in an

exhibit offered by Respondent, SMARD’s “clinical picture is characterized by initial

respiratory insufficiency due to diaphragmatic palsy and often followed by distally

pronounced weakness and wasting.” See Rudnik-Schöneborn, et al., Long-Term

Observations of Patients with Infantile Spinal Muscular Atrophy with Respiratory Distress

Type 1 (SMARD1), 35 Neuropediatrics 174 (2004).

7

Dr. Kuntz recommended genetic testing, and continued: “I answered mother’s questions

regarding 4-amino pyridine treatment trials in adults with Guillain-Barre and

diaphragmatic nerve pacing. Neither of those are applicable to [O.A.S.] at this time.”

In January 2007, [O.A.S.] was a patient in the pediatric neurology clinic at Blank

Children’s Hospital in Des Moines, Iowa, where she was evaluated by Dr. Haidar

Kabbani, a pediatric neurologist. Dr. Kabbani concluded that [O.A.S.] has “a clinical

diagnosis of sensorimotor axonal neuropathy that also can be called spinomuscular

atrophy with respiratory distress.” He noted that “[t]here is very little known about this

entity and it is believed to be progressive. The mechanical ventilation prolongs the life

of these patients to anything between the first to the beginning of the second decade of

life.” Dr. Kabbani also stated: “Unfortunately, there is no well-defined treatment for

those patients that I am aware of. Also, there is no further investigation that I have at

this point to recommend.”

Since September 2003, [O.A.S.]’s treating physicians have primarily referenced

SMARD as [O.A.S.]’s diagnosis. For example, in a November 11, 2003 letter, Dr.

Gavin, [O.A.S.]’s pediatrician, stated that [O.A.S.] has “a current working diagnosis of a

recently described entity called spinal muscular atrophy with respiratory distress or

SMARD.” In February 2004, after [O.A.S.] returned to Mercy Medical Center, she was

seen by Dr. Bala Napa, a pediatric intensivist, who indicated in his discharge report that

[O.A.S.] has a “[k]nown neuromuscular disorder – SMA-RD type.” In a letter to an

insurance company, dated October 25, 2004, Dr. Gavin requested additional services

for [O.A.S.] and stated: “One medical consultant has suggested she may have Spinal

Muscle Atrophy with Respiratory Distress but this diagnosis has yet to be confirmed.”

Additionally, during an August 3, 2006 evaluation by Dr. Stuart Weinstein of the

Department of Orthopedics at the University of Iowa Hospitals, Dr. Weinstein remarked

that [O.A.S.] “has some form of spinal muscle atrophy.” In October 2008, [O.A.S.] was

evaluated by Dr. Ricardo Flores, a pediatric pulmonologist at the Blank Children’s

Hospital, who also concluded that [O.A.S.] “has Spinal Muscular Atrophy with

Respiratory Distress.” Moreover, an August 11, 2011 note written by Dr. Judy Walker of

Blank Children’s Hospital describes [O.A.S.] as a 10 year old girl with a history of spinal

muscular atrophy. According to the record at the time of the Special Master’s decision,

[O.A.S.] still requires the use of a ventilator and a wheelchair, and is paralyzed from the

neck down. She needs an indwelling catheter for continuous urinary drainage. She

needs a feeding tube for proper nutrition and requires 24-hour attendant care.

Nevertheless, [O.A.S.] attends school, and has an individualized education plan.

As noted above, on January 17, 2003, Petitioners filed a timely Petition for

Compensation under the Vaccine Act, although the Petition was filed without the

supporting medical records required by the Vaccine Act. See 42 U.S.C. § 300aa-11(c).

After considerable delay, the Petitioners finally filed expert reports in 2008, which were

deemed insufficient by Special Master Moran to meet the Petitioners’ burden of proof.

Therefore, on November 20, 2009, Special Master Moran issued an order to show

cause, pursuant to 42 U.S.C. § 300aa-12(d)(B), requiring the Petitioners to demonstrate

why Petitioners’ case should not be dismissed, for failure to comply with his previous

8

orders of April 13, 2009 and June 26, 2009, which had required Petitioners to “obtain a

supplemental report from Dr. Shoenfeld.” Special Master Moran concluded that two

expert reports prepared previously by Dr. Shoenfeld were inadequate “to meet the

petitioners’ burden of producing persuasive evidence” under the Althen test. See Althen

v. Sec’y of Health & Human Servs., 418 F.3d at 1278. In his order to show cause,

Special Master Moran stated:

A supplemental report from Dr. Shoenfeld is more likely to advance the

litigation than not obtaining one. The undersigned reaches this conclusion

based upon his experience, including adjudicating several cases in which

Dr. Shoenfeld testified. In the undersigned’s experience, Dr. Shoenfeld

often introduces ideas, which have not been disclosed in his expert

reports, while he is testifying. The response to this new idea theoretically

can take one of two forms: either (a) Dr. Shoenfeld’s new opinion is

excluded on the ground that he failed to disclose the opinion, or (b)

respondent is extended additional time to respond to Dr. Shoenfeld’s new

opinion and a second hearing is required. Because special masters are

inclined to allow petitioners an opportunity to present their case, special

masters rarely exclude an opinion on the ground that it was not disclosed

previously. Thus, by process of elimination, special masters permit

hearings to be continued to allow time to respond to a previously

undisclosed opinion. A second hearing increases the work for everyone

involved - petitioner’s attorney, petitioner’s expert, respondent’s attorney,

respondent’s expert and the undersigned. Thus a second hearing should

be avoided if possible.

If Dr. Shoenfeld disclosed all his opinions completely before trial, then the

hearing would proceed more expeditiously. The Simanskis’ delay in

producing more information about Dr. Shoenfeld’s opinion does not

advance the litigation.

On May 13, 2010, after receiving Petitioners’ response to the order to show

cause, Special Master Moran concluded that “[t]he Simanskis have not presented

evidence to fulfill their burden of proof and have declined the opportunity to present

additional evidence,” for which reason he dismissed Petitioners’ case. See Simanski v.

Sec’y of Health & Human Servs., 2010 WL 2292200, at *1.

As discussed above, the Simanskis subsequently filed a Motion for Review of the

Special Master’s dismissal in this court, claiming that Dr. Shoenfeld’s expert reports

were sufficient to establish causation and that the Special Master’s dismissal of their

case, before requiring the Respondent to submit an expert report to counter the

Simanskis’ evidence, was arbitrary, capricious, an abuse of discretion and not in

accordance with the law. On December 15, 2010, Judge Christine O.C. Miller of the

United States Court of Federal Claims affirmed the Special Master’s dismissal and

denied Petitioners’ Motion for Review. See, generally, Simanski v. Sec’y of Health &

9

Human Servs., 96 Fed. Cl. 588. 22 The Simanskis then appealed to the United States

Court of Appeals for the Federal Circuit on February 14, 2011. On March 6, 2012, the

United States Court of Appeals for the Federal Circuit reversed and remanded the

above-captioned case, “with instructions for the special master to address the merits of

the Simanskis’ claim, either by applying appropriate summary judgment standards[ 23] or

by conducting a hearing and resolving the compensation claim on the merits.” Simanski

v. Sec’y of Health & Human Servs., 671 F.3d at 1385. 24

On May 9, 2012, the Federal Circuit mandate was issued. Thereafter, on June

20, 2012, Petitioners filed [O.A.S.]’s updated medical records with the Special Master,

as well as medical literature. In response to the immunological causation theories

presented by Petitioners’ immunologist expert, Dr. Shoenfeld, on December 14, 2012,

Respondent filed an expert report prepared by Dr. Christine McCusker, a pediatric

22

Judge Christine O.C. Miller concluded that “the special master did not abuse his

discretion by denying compensation when respondent had not submitted any rebuttal

evidence.” Simanski v. Sec’y of Health & Human Servs., 96 Fed. Cl. at 611. Moreover,

noting that the Petitioners failed to “list[] their specific objections to the special master's

findings,” Judge Christine O.C. Miller sustained the Special Master’s ruling. Id.

23

Special Master Moran did not hold a hearing before his May 13, 2010 dismissal of the

above-captioned case, but had conducted several informal telephonic status

conferences. During an April 13, 2009 status conference, the Respondent indicated

that “it intended to file a motion for summary judgment, arguing that petitioners had not

met their burden as established by Althen v. Secretary of Health and Human Services,

418 F.3d 1274, 1278 (Fed. Cir. 2005).” Although the Respondent did not file such a

motion, the case was dismissed “for failing to comply with the show cause order, which

required the Simanskis to produce sufficient evidence to meet the Althen prongs.” See

Simanski v. Sec’y of Health & Human Servs., 2010 WL 2292200, at *5.

24

The Federal Circuit concluded: “[T]he special master should not have dismissed the

petition as a sanction for the Simanskis' failure to comply with the orders to supplement

Dr. Shoenfeld's report.” Simanski v. Sec’y of Health & Human Servs., 671 F.3d at 1382.

The Federal Circuit continued:

if a Vaccine Act petitioner has produced what the petitioner believes is

enough evidence to prevail, or at least to proceed to a hearing, the

petitioner is normally entitled to a ruling on that question. If the petitioner

cannot produce additional evidence in response to a special master's

order—or chooses not to do so—the petitioner may be at risk of an

adverse ruling on the merits, but that ruling should be based on the merits

and not on the petitioner's failure to come forward with additional

evidence.

Id.

10

immunologist. After the Petitioners filed a supplemental expert report by Dr. Shoenfeld,

Dr. McCusker filed a second report.

On August 24, 2012, Special Master Moran requested that Petitioners file an

updated, supplemental affidavit indicating Ms. Simanski’s “understanding of the

condition with which [O.A.S.]’s doctors have diagnosed her,”25 in particular,

Ms. Simanski shall state whether doctors have told her that [O.A.S.]

suffers from Guillain-Barré syndrome. If so, Ms. Simanski shall state

when the Guillain-Barré syndrome diagnosis was most recently

communicated to her. Ms. Simanski shall state whether doctors have told

her that [O.A.S.] suffers from SMARD. If so, Ms. Simanski shall state

when the SMARD diagnosis was most recently communicated to her.

In addition, Ms. Simanski shall state how her family responded to Dr.

Kuntz’s suggestion that there be genetic testing on [O.A.S.]. If the

Simanskis decided not to have genetic testing, Ms. Simanski shall explain

why the family reached that decision. If any member of the Simanski

family had genetic testing, Ms. Simanski shall explain who was tested,

when the test was conducted, and which doctor / facility conducted the

test.

Petitioners filed Ms. Simanski’s affidavit on September 18, 2012, in which Ms. Simanski

indicated that genetic testing “was not going to alter [O.A.S.]'s treatment nor improve

her condition,” and, therefore, “[s]ince there is no benefit for [O.A.S.], we have decided

not to seek genetic testing.” Ms. Simanski also indicated that “virtually all the doctors”

who evaluated [O.A.S.] in 2001 “supported a diagnosis” of GBS. Ms. Simanski’s

affidavit appears not to include a more recent diagnosis of GBS than from 2001.

Petitioners also filed a supplemental expert report on December 5, 2012, 26

prepared by Dr. Maertens, a pediatric neurologist, who stated that [O.A.S.] suffered

from either GBS or CIDP. Respondent filed an expert report prepared by a pediatric

neurologist, Dr. Richard Finkel, who indicated that [O.A.S.] suffered from SMARD, not

GBS or CIDP. In response, Petitioners filed another supplemental report from Dr.

Maertens on December 28, 2012, in which he challenged the diagnosis of SMARD, and

discussed with more specificity why GBS and/or CIDP represents the correct diagnosis

for [O.A.S.]. A final, supplemental expert report by Dr. Finkel was filed by Respondent

on January 18, 2013.

25

Special Master Moran explained that “[i]n an affidavit dated May 20, 2004, Ms.

Simanski stated that [O.A.S.]’s treating doctors had not yet offered a diagnosis for

[O.A.S.]’s condition. Thereafter, Petitioners filed additional records indicating a possible

diagnosis of spinal muscle atrophy with respiratory distress (‘SMARD’) and indicating

requests for genetic testing.”

26

Dr. Maertens first submitted an unsigned version of his expert report and then, a day

later, submitted a signed version, which appears otherwise to be identical.

11

Pursuant to the Federal Circuit’s instructions on remand, Special Master Moran

conducted evidentiary hearings between February 4 and 7, 2013, with a fifth day of

testimony completed via videoconference on February 20, 2013. Petitioners called their

two experts, Dr. Shoenfeld and Dr. Maertens, at the evidentiary hearing. Respondent

also called their two experts, Dr. McCusker and Dr. Finkel, at the hearing.

In their post-hearing briefs submitted to the Special Master, the parties reiterated

their differing positions with respect to [O.A.S.]’s condition. While Petitioners

maintained that [O.A.S.] suffers from GBS/CIDP, Respondent challenged that position,

and argued that [O.A.S.] suffers from a different neurologic problem, SMARD, which is

caused by a genetic mutation. Respondent also asserted that even assuming that

[O.A.S.] suffered from GBS or CIDP, Petitioners have not met their burden of

establishing that the vaccines caused [O.A.S.]’s neurologic problem.

On August 20, 2013, Special Master Moran issued a 72-page decision, denying

the Petitioners’ request for compensation. Special Master Moran concluded that “[a]

preponderance of the evidence establishes that [O.A.S.] suffers from a spinal muscular

atrophy with respiratory distress, not Guillain-Barré syndrome or chronic inflammatory

demyelinating polyneuropathy.” Simanski v. Sec’y of Health & Human Servs., 2013 WL

7017568, at *42. Special Master Moran indicated that the parties’ experts came to very

different conclusions regarding [O.A.S.]’s case, but he determined that the evidence

“overwhelmingly favors a finding that [O.A.S.] suffers from SMARD.” Id. at *1. Because

Special Master Moran concluded that [O.A.S.] appears to suffer from SMARD, the

Special Master did not reach the issue of whether the vaccines [O.A.S.] received at two

months could have caused her condition, making her eligible for compensation under

the Vaccine Compensation Program, pursuant to the Althen test. See Althen v. Sec’y of

Health & Human Servs., 418 F.3d at 1278.

Special Master Moran began his analysis by explaining his understanding of the

etiology of GBS, CIDP, and SMARD, the diseases at issue in Petitioners’ case. He

dedicated multiple pages of his decision to a description of the “Structure of the Nervous

System” and how each of the diseases implicated in this case, GBS, CIDP, and

SMARD, impacts the nervous system. Id. at *4-8. Turning to GBS and CIDP, Special

Master Moran wrote:

Both GBS and CIDP are neuromuscular diseases involving sensory and

motor nerves of the peripheral nervous system. The basic definitions of

these conditions point to similarities and differences between them. GBS

is a “rapidly progressive ascending motor neuron paralysis of unknown

etiology, frequently seen after an enteric or respiratory infection.” CIDP is

a “slowly progressive, autoimmune type of demyelinating polyneuropathy

characterized by progressive weakness and impaired sensory function in

the limbs . . . usually with elevated protein in the cerebrospinal fluid. It . . .

is related to Guillain-Barré syndrome.”

12

Id. at *4.

The Special Master noted that both GBS and CIDP “seem to share a common

pathway,” as both are “demyelinating condition[s].” Id. at *5. He explained that

“[d]octors generally believe that the substance that attacks the myelin is part of the

person’s immune system.” Id. Special Master Moran stated: “Whether the immune

system of newborns is sufficiently strong to damage myelin was a disputed point

between the two immunologists, Dr. Shoenfeld and Dr. McCusker. E.g. compare Tr.

269-78 (Dr. Shoenfeld) with Tr. 345-53, 383-85 (Dr. McCusker).” Id. The Special

Master added: “Dr. McCusker’s view that a newborn’s immune system is not robust

enough to cause autoimmunity is in accord with the general incidence of autoimmune

diseases, including GBS.” Id. Special Master Moran also noted that according to the

testimony of Petitioners’ expert, Dr. Maertens, reports of GBS in infants less than three

months old are very rare, and that “[t]here is some question whether GBS can occur in a

newborn at all.” Id.

Special Master Moran also discussed the symptoms, the diagnostic criteria and

the types of tests used to identify GBS. Id. at *6. He noted that “GBS begins with

‘paresthesias of the feet,’” and thereafter, “usually progresses quickly.” Id. at *6. In

contrast, he pointed out that CIDP is “usually ‘slowly progressive.’” Id. at *7. Special

Master Moran further stated that “[t]he Asbury criteria[ 27 ] establish the symptoms

frequently used to diagnose GBS,” including “elevated protein in the spinal fluid and

response to IVIG treatment,” and “‘reduced or absent reflexes.’” Id. at *6. The Special

Master also quoted from the testimony of Respondent’s expert, Dr. Finkel, who

explained that the “‘reflexes are typically lost early in GBS/CIDP. That’s a hallmark.

That’s one of the two main criteria of GBS.’” Id. The Special Master observed that

Petitioners’ expert, Dr. Maertens, agreed with Dr. Finkel’s explanations, and stated, “‘[i]n

most cases, the reflex[es] are decreased or lost.’” Id. The Special Master further noted

that “[t]he duration of GBS is a primary way of distinguishing it from chronic

inflammatory demyelinating polyneuropathy.” Id. The Special Master continued: “As

the name implies, CIDP is a chronic condition, meaning it ‘persists over a long period of

time.’ Unlike GBS, which resolves quickly, CIDP can be diagnosed only if the patient

suffers symptoms for eight or more weeks.” Id. Moreover, the Special Master observed

that “[a]nother difference between GBS and the common presentation of CIDP concerns

how the disease appears initially. People with GBS decline rapidly. In contrast, CIDP is

usually ‘slowly progressive.’” Id. at *7. The presenting symptoms for CIDP “‘often

27

The Asbury criteria reference is to an article originally published in 1990 in Annals of

Neurology by Asbury, et al., Assessment of Current Diagnostic Criteria for Guillain-

Barré Syndrome, 27 Ann Neurol 21 (1990), which established the diagnosis criteria for

GBS. Dr. Maertens testified that the Asbury criteria is a generally accepted assessment

guide in the medical community criteria, which describes the symptoms frequently used

to diagnose GBS, including response to IVIG treatment. Dr. Finkel testified that the

“Asbury Cornblath criteria . . . is still largely accepted as the general clinical means of

making that [GBS] diagnosis.”

13

include tingling or numbness of the digits, weakness of the limbs, hyporeflexia or

areflexia, fatigue, and abnormal sensations.’” Id.

In his decision, Special Master Moran also discussed SMARD, which, he noted,

is “‘characterized by a sudden onset of respiratory distress within the first 13 months of

life and initially distal and later generalized muscular weakness.’” Id. The Special

Master indicated: “For SMARD, ‘[t]he clinical picture is characterized by initial

respiratory insufficiency due to diaphragmatic palsy and often followed by distally

pronounced weakness and wasting.’” Id. Special Master Moran added: “Most pediatric

neurologists became aware of the distinction between SMA and SMARD in 2003, when

the Annals of Neurology published an article on SMARD. Dr. Maertens testified that he

first learned about SMARD in 2005 or 2006.” Id. at *8. Special Master Moran explained

that SMARD and SMA are different because they have different origins, as “[t]he basis

for most cases of SMA is a genetic mutation, located on chromosome 5q, which was

identified in 1995. In contrast, SMARD involves a different gene, known as IGHMBP2.”

Id.

The Special Master explained that “one way to distinguish the diseases

implicated in this case (GBS, CIDP, and SMARD) is to determine what part of the

peripheral nerve is damaged.” Id. at *4. The Special Master indicated that while the

two most common “demyelinating” forms of GBS, “Acute Motor Axonal Neuropathy” and

“Acute Motor-Sensory Axonal Neuropathy” involve “the axon of the nerve,” both SMA,

spinal muscular atrophy, and SMARD, spinal muscular atrophy with respiratory distress,

“are considered diseases of the anterior horn cell.” 28 Id. at *8 n.13.

In his decision, the Special Master also provided a detailed chronology of the

tests and treatments [O.A.S.] had received at various medical institutions, including

references to medical opinions and [O.A.S.]’s test results. See, generally, id. at *8-25.

Special Master Moran concentrated on the testimony of two of the experts, Dr. Maertens

for the Petitioners and Dr. Finkel for the Respondent, and their respective interpretations

of [O.A.S.]’s test results and opinions as to whether [O.A.S.]’ conditions and symptoms,

based on her test results, were more consistent with GBS/CIDP or with SMARD. See,

generally, id. at *9-25.

28

Special Master Moran cited to Kelley v. Secretary of Health and Human Services, 68

Fed. Cl. 84 (2005), Tompkins v. Secretary of Health and Human Services, No.10-

621V, 2013 WL 3498652, at *27 (Fed. Cl. Spec. Mstr. June 21, 2013), and Torday v.

Secretary of Health and Human Services, No. 07-372V, 2009 WL 5196163 (Fed. Cl.

Spec. Mstr. Dec. 10, 2009), for the proposition that the issue “[w]hether GBS and CIDP

are separate and distinct clinical entities or they belong on a spectrum of similar

diseases is a difficult question that has appeared periodically in cases in the Vaccine

Program.” Id. at *7. The Special Master determined that “[a] resolution of that question,

however, is not required in this case because, for the reasons explained below, [O.A.S.]

has not suffered from either GBS or CIDP. Rather, she suffers from an entirely different

disease, SMARD.” Id.

14

Concluding that “the dispute over the correct diagnosis is a critical issue in this

case,” and that “[t]he parties agree that determining [O.A.S.]’s injury is the first step in

determining whether the vaccinations harmed her,” 29 the Special Master proceeded to

analyze “whether the evidence preponderates in favor of either GBS/CIDP or SMARD,”

citing Moberly ex rel. Moberly v. Secretary of Health and Human Services, 592 F.3d

1315, 1322 (Fed. Cir.), reh’g en banc denied (Fed. Cir. 2010), Hodges v. Secretary of

the Department of Health and Human Services, 9 F.3d 958, 962-63 (Fed. Cir. 1993),

and Bunting v. Secretary of the Department of Health and Human Services, 931 F.2d

867, 873 (Fed. Cir. 1991). Id. at *3, *25 (capitalization removed).

Indicating that “[O.A.S.] presented normal and abnormal behaviors,” consistent

and inconsistent, with both SMARD and GBS/CIDP, and that the parties’ experts came

to very different conclusions regarding [O.A.S.]’s case, Special Master Moran focused

on twelve data points in his discussion, representing symptoms that he found were

pertinent to the determination of etiology and nature of [O.A.S.]’s injury. 30 Id. at *25. At

the February 2013 hearing conducted by the Special Master, he asked both Dr. Finkel

and Dr. Maertens “to analogize [O.A.S.]’s signs and symptoms to pebbles[ 31] and then

29

The Petitioners “acknowledge that this is such a case,” in other words, a situation in

which the Special Master must first determine the type of disease or injury at issue.

Petitioners, however, “assert the evidence contained in the medical records and

literature filed in this case unequivocally demonstrate that [O.A.S.] does not have

SMARD, but suffered GBS as a result of her January 26, 2001 vaccinations.”

30

It appears the Special Master’s analysis that [O.A.S.]’s condition was consistent with

SMARD closely resembles the analysis conducted by the Respondent’s expert, Dr.

Finkel. In his September 28, 2012 expert report, Dr. Finkel provided a chart, which

summarized his interpretations of the data included in [O.A.S.]’s medical records. Dr.

Finkel’s “[d]ata items” included: “Intrauterine Growth Retardation (IUGR)”; “[r]espiratory

failure – acute onset, as the presenting symptom, with early right hemi-diaphragm

eventration”; “[r]espiratory failure presenting at age 2 months, 3 weeks”; “[p]rogression

to permanent ventilation support within one month of onset of respiratory failure”;

“[w]eakness in limbs, trunk, neck muscles – onset within days to few weeks of

respiratory failure (RF)”; “[w]eakness: distribution of lower>upper limbs, distal>proximal,

symmetric”; “[n]erve conduction and EMG findings – length-dependent axonal

sensorimotor polyneuropathy, without focal or segmental changes. (May have slowing

of conduction initially that raises the consideration of GBS or CIDP)”; “[s]ural nerve

biopsy”; “[c]erebrospinal fluid (CSF) profile”; “[l]ow serum creatinine (0.1) upon

admission 1/20/00, suggestive of chronic muscle wasting (non-specific as to etiology)”;

“[s]low decline in motor function over years, following the initial acute deterioration”;

“[e]xcessive sweating (diaphoresis)”; “[n]o apparent therapeutic benefit from IVIg or

prednisolone Rx.”

31

A search of published decisions by this court and the Office of Special Masters

indicates the unusual “pebble” analogy approach has not been used before to evaluate

the strength of evidence in a vaccine case. The pebble analogy, however, was not

used consistently by the experts who testified in [O.A.S.]’s case, nor did the Special

15

place each pebble on a scale,” one side of the scale representing SMARD, and the

other side representing GBS/CIDP. Id. The Special Master noted that Dr. Finkel

ranked his pebbles by size 32 (one to five with five being the most weighty) according to

the amount of weight he thought should be attributed to each symptom. The Special

Master explained that this “process allowed the undersigned [Special Master Moran] to

weigh the evidence supporting and opposing a particular diagnosis.” Id.

Special Master Moran’s analysis included evaluation of the following data points

in the discussion section of his decision:

1. Intrauterine Growth Retardation (IUGR)

2. Acute Respiratory Failure as a Presenting Sign

3. Onset of Respiratory Failure at 12 Weeks

4. Weakness Pattern and Reflexes

5. Progression to Permanent Ventilator Support

6. Sural Nerve Biopsy

7. Cerebrospinal 33 Fluid (CSF) Protein

8. Creatinine Levels

9. EMG Tests and Nerve Conduction Studies

10. Response to IVIG Treatment

11. Treating Doctors and

12. Comparison of Experts: Dr. Maertens and Dr. Finkel 34

Master continue to push for the experts to use the pebble scale throughout the case or

the hearing. Moreover, the pebble analogy also was abandoned by the Special Master

in his decision after his discussion on the first three data points.

32

Only Dr. Finkel ranked his pebbles by size. Dr. Maertens did not engage in pebble

ranking, but described three items as “toss” (on neither side of the scale).

33

Dorland’s Illustrated Medical Dictionary defines cerebrospinal as “pertaining to the

brain and spinal cord.” Dorland’s Illustrated Medical Dictionary 333.

34

While Petitioners’ expert, Dr. Maertens, and Respondent’s expert, Dr. Finkel,

concentrated on [O.A.S.]’s diagnosis, Petitioners’ expert, Dr. Shoenfeld, an

immunologist, and Respondent’s expert, Dr. McCusker, a pediatric immunologist,

offered opinions mainly on the issue of causation. Special Master Moran indicated in

his decision that the Petitioners “elicited no testimony on direct examination from either

Dr. Shoenfeld or Dr. Maertens [Petitioners’ experts] about [O.A.S.]’s vaccinations having

played a causal (or aggravating) role under the assumption that [O.A.S.] has SMARD,”

and, therefore, there was no need to discuss the issue of causation in his decision.

Simanski v. Sec’y of Health & Human Servs., 2013 WL 7017568, at *41. For that

reason, while evaluating the expert testimonies, Special Master Moran considered

mainly the testimony of Dr. Maertens and Dr. Finkel, the pediatric neurologists, who

testified about [O.A.S.]’s diagnosis. The Special Master’s focus on Dr. Maertens and

Dr. Finkel is discussed further below.

16

A chart summarizing key findings by the Special Master regarding his

conclusions as to whether the various symptoms and tests results for [O.A.S.] were

consistent or inconsistent with each disease at issue was included on page 41 of the

Special Master’s decision. Simanski v. Sec’y of Health & Human Servs., 2013 WL

7017568, at *41. The chart included by the Special Master, however, is not entirely

consistent with the data points addressed by the Special Master in his discussion

section. The chart includes only ten of the twelve data points addressed at greater

length in the discussion section of the Special Master’s decision. Moreover, although

included on the chart, [O.A.S.]’s health before her respiratory arrest is not listed as a

separate data point in the Special Master’s discussion section of his decision. He

addressed [O.A.S.]’s health before the respiratory arrest in the discussion of his second

data point (“Acute Respiratory Failure as a Presenting Sign”). The Special Master’s

chart also does not reflect the treating doctors’ opinions or the comparisons of experts

included in the Special Master’s discussion. For reference, the Special Master’s chart is

included below. The dots on the chart represent the Special Master’s conclusion

whether the particular data point was consistent, neutral or inconsistent with each of the

three diseases implicated in the case, GBS, CIDP, or SMARD.

GBS/CIDP Data Point SMARD

Consistent Neutral Inconsistent Inconsistent Neutral Consistent

• IUGR •

• Health before Respiratory Arrest •

(some dispute whether [O.A.S.] had a weak (weakly)

cry)

• Age of Onset •

(extremely (strongly)

rare)

• Reflex and Weakness Pattern •

(strongly –

absent

reflexes are

diagnostic

criteria)

• Permanent Ventilator Support •

(but not (strongly)

impossible)

• Sural Nerve Biopsy Showing No •

(strongly) Demyelination

• CSF Protein – Normal •

(but not

impossible)

• Creatinine Levels •

(results varied; a question about Mayo (weakly)

results)

• Response to IVIG •

(weakly –

better

response

to 2d and 3d

dose is

expected)

• EMG and Nerve Conduction Studies •

(strongly) (showing axonal damage, not demyelination) (strongly)

17

Simanski v. Sec’y of Health & Human Servs., 2013 WL 7017568, at *41. (emphasis in

original).

Turning to the first data point in the discussion section of Special Master Moran’s

decision, he noted that [O.A.S.], who weighed only four pounds, twelve ounces at birth,

was diagnosed with IUGR. The Special Master, in reviewing the trial testimony of

Respondent’s expert, Dr. Finkel, and Petitioners’ expert, Dr. Maertens, and their expert

reports and accompanying literature, concluded that “[O.A.S.]’s IUGR supports a

diagnosis of SMARD.” Id. at *26. Specifically, Special Master Moran cited to Dr.

Finkel’s supplemental expert report, which relied on a study “showing that

approximately three-quarters of patients with SMARD have intrauterine growth

retardation.” Id. During his testimony, Dr. Finkel also discussed a study which found

that IUGR was 48% sensitive 35 and 77% specific for SMARD. When asked by the

Special Master to “place the IUGR pebble on the scale,” Dr. Finkel stated that IUGR

was a medium sized pebble for SMARD, a size three.

Dr. Maertens, on the other hand, wrote in his expert report that IUGR is

associated with conditions other than SMARD1. Therefore, according to him, “‘at best,

IUGR is neither supportive nor inconsistent with a diagnosis of SMARD1.’” Id. Special

Master Moran did not give much weight to Dr. Maertens’ opinion, however, noting that,

“at trial, it became apparent that Dr. Maertens was using ‘consistent’ unusually,” and

that, although during his testimony Dr. Maertens agreed that IUGR was “‘consistent

with’” SMARD, Dr. Maertens maintained that IUGR is not a prerequisite for SMARD.

When asked by the Special Master to decide where IUGR should be placed on the

hypothetical scale, Dr. Maertens stated that [O.A.S.]’s IUGR did not go to either side of

the scale. Instead of placing it on one of the scales, Dr. Maertens stated that it would

be a “toss.” The Special Master’s analysis of his first data point in his discussion

section included a single footnote reference to GBS, explaining that “Dr. Maertens did

not provide any testimony regarding any connection between IUGR and GBS/CIDP.” Id.

at *26 n.35. Based on Dr. Finkel’s testimony and the literature introduced into the

record, after considering both experts and the information in the record, the Special

Master found Dr. Finkel’s opinion persuasive.

As a second data point, Special Master Moran examined whether [O.A.S.]’s

January 30, 2001 acute respiratory failure was “consistent with a diagnosis of SMARD.”

Id. at *27. He noted that there was “consensus that respiratory failure is typically not a

presenting symptom for GBS/CIDP,” however, both parties “strenuously disputed

whether [O.A.S.]’s presentation at Mercy [Medical Center] for respiratory failure is

consistent with a diagnosis of SMARD.” 36 Id. Special Master Moran also indicated that

35

Dr. Finkel explained during his testimony that “sensitive” means “what percentage of

patients have that feature.”

36

The Special Master discussed separately, in a section identified as “Respiratory

Arrest and SMARD,” whether respiratory failure is consistent with a diagnosis of

18

Petitioners’ expert, “Dr. Maertens opined that the acute onset of respiratory arrest was

consistent with SMARD,” and that Respondent’s expert, Dr. Finkel also “linked an acute

presentation of respiratory problems with SMARD.” Id. at *27-28. The Special Master

noted that despite this seeming consensus of the experts, the Petitioners “challenged

Dr. Finkel on cross-examination,” and stated in their post-hearing brief that based on

the literature, ‘“every child who was diagnosed with SMARD with a known gene

mutation had the onset of symptoms prior to presentation of respiratory arrest,’” while

[O.A.S.] was basically healthy before her respiratory arrest. Id. at *27. The Special

Master rejected the Petitioners’ arguments, pointing out “two flaws” in their position:

“The first problem stems from the Simanskis’ assertion of what happens in all cases of

SMARD. The second problem concerns what happened to [O.A.S.].” Id. With regards

to the first issue, the Special Master noted that “[i]t is difficult to accept the proposition

that absolutely every child with SMARD has been noted to suffer some relatively benign

symptoms before presenting to a doctor with respiratory arrest.” Id. Special Master

Moran noted that Dr. Finkel refuted a similar argument made by the Petitioners’ counsel

at the hearing, although stating, “I don’t think we went through every case that was in

every report. We went through certain cases that you selected and you wished to

address, but let’s be clear here. We didn’t go through every case in every report.” Id.

Special Master Moran also noted that the medical literature “seems to indicate more

variability in presentation” of SMARD, and quoted an article in the record, Yiu, et al.,

Genetic Axonal Neuropathies and Neuronopathies of Pre-Natal and Infantile Onset, 17

J. Periphe. Nerv. Sys. 285, 289 (2012), which stated that “[e]arly-onset respiratory

distress is the cardinal feature, presenting between 1 and 6 months of age, although a

weak cry, inspiratory stridor,[ 37 ] or foot deformities may have been noted earlier.”

Simanski v. Sec’y of Health & Human Servs., 2013 WL 7017568, at *27. (emphasis in

original). Special Master Moran also noted that despite the Petitioners’ assertions that

[O.A.S.] was healthy before her respiratory arrest, there was contrary evidence in the

record, including Dr. Finkel’s interpretation of the December 28, 2000 video of [O.A.S.]

that she had a weak cry. Id. at *28.

Indicating that “although Dr. Finkel assigned [O.A.S.]’s respiratory arrest as a

strong pebble on the side of SMARD, a size four; the Simanskis have effectively cast

some doubt on this point,” Special Master Moran determined that “whether these pieces

of information favor SMARD or GBS/CIDP depends upon the other side of the

hypothetical scale,” whereupon he proceeded to the analysis of “Respiratory Arrest and

GBS/CIDP.” Id. The Special Master examined Dr. Maertens’ expert report, and his

determination that “[a]cute respiratory failure is a frequent complication in patients with

severe neuromuscular disease.” Id. The Special Master concluded that Ito, et al.,

Phrenic Nerve Conduction in the Early Stage of Guillain-Barre Syndrome Might Predict

the Respiratory Failure, 116 Acta Neurol. Scand. 255 (2007), and Stojkovic, et al.,

SMARD, and, in a section identified as “Respiratory Arrest and GBS/CIDP,” whether

respiratory failure is consistent with a diagnosis of GBS/CIDP. Id. at *27-29.

37

Dorland’s Illustrated Medical Dictionary defines stridor as “a harsh, high-pitched

breath sound.” Dorland’s Illustrated Medical Dictionary 1785.

19

Phrenic Nerve Palsy as a Feature of Chronic Inflammatory Demyelinating

Polyradiculoneuropathy, 27 Muscle & Nerve 497 (2003), two studies referenced in Dr.

Maertens’ expert report, “provide at least some support for Dr. Maertens’s statement

that patients with severe neuromuscular diseases can develop respiratory problems as

a ‘complication’ to their disease.” Simanski v. Sec’y of Health & Human Servs., 2013

WL 7017568, at *28. The Special Master indicated, however, that based on the

medical records, [O.A.S.]’s presentation was different than the presentation described

in the Stojkovic article, because [O.A.S.]’s respiratory arrest was not “progression” of a

disease, but rather her “first significant problem.” Id. The Special Master also noted Dr.

Maertens’ opinion that “[O.A.S.]’s January 30, 2001 respiratory arrest did not mark the

beginning of her neurological problem,” but rather that [O.A.S.]’s “Guillain-Barré

syndrome probably came on” after [O.A.S.] started to recover from her RSV infection.

Id. The Special Master, therefore, concluded: “Given that Dr. Maertens did not argue

that [O.A.S.]’s presentation for acute respiratory failure was part of her demyelinating

neuropathy, this particular piece of information does not support the diagnosis of

GBS/CIDP.” Id. at *29. Therefore, because “Dr. Finkel believed that [O.A.S.]’s

presentation with acute respiratory failure strongly favored SMARD . . . [and] Dr.

Maertens stated that it was a ‘toss up finding’ that should not be placed on either side of

the scale,” Special Master Moran found that the “more persuasive evidence suggests

that the presentation with respiratory failure is more common in SMARD, than in

GBS/CIDP.” Id. The Special Master, therefore, concluded that [O.A.S.]’s acute

respiratory failure “slightly favors” the SMARD diagnosis. Id. On his chart, however,

the Special Master indicated that [O.A.S.]’s health before respiratory arrest, which the

Special Master discussed under his second data point in the discussion section, was

consistent with GBS/CIDP, but only weakly consistent with SMARD.

As a third data point in his discussion section, Special Master Moran considered

whether [O.A.S.]’s acute presentation of respiratory arrest at twelve-and-one-half weeks

after [O.A.S.]’s birth was more consistent with SMARD or GBS/CIDP. Special Master

Moran referenced the testimony of Dr. Finkel, who stated that [O.A.S.]’s respiratory

failure at her age was consistent with SMARD, but inconsistent with GBS and CIDP,

and the articles cited by Dr. Finkel: Grohmann, et al., Mutations in the Gene Encoding

Immunoglobulin µ-binding Protein 2 Cause Spinal Muscular Atrophy with Respiratory

Distress Type 1, 29 Nat. Genet. 75, 76 (2001), and Pierson, et al., Infantile-Onset Spinal

Muscular Atrophy with Respiratory Disress-1 Diagnosed in a 20-Year-Old Man, 21

Neuromuscular Disord. 353 (2011) in support. Dr. Finkel also testified that “[O.A.S.]’s

manifestation at six weeks to six months would be a size five pebble for SMARD.”

Simanski v. Sec’y of Health & Human Servs., 2013 WL 7017568, at *29. Dr. Finkel

further indicated that “‘GBS can occur at all ages but is decidedly uncommon in

infants.’” Id.

Special Master Moran noted that “Dr. Maertens’s opinion regarding [O.A.S.]’s

age of onset [of her disease] varied.” Id. In his December 5, 2012 supplemental expert

report, Dr. Maertens disagreed that GBS is highly unlikely to occur at two months of age

and noted that “GBS occurs at all ages,” citing a case report of a one-month-old girl the

authors described as having GBS. Id.; see also Gilmartin, et al., Guillain-Barré

20

Syndrome with Hydrocephalus in Early Infancy, 34 Arch. Neurol. 567 (1977).

Subsequently, however, at the hearing, Dr. Maertens testified that it is “‘extremely rare’”

to have the age of onset be two months in GBS, but that the onset of respiratory failure

at two months could occur with SMARD. See Simanski v. Sec’y of Health & Human

Servs., 2013 WL 7017568, at *30. Based on the evidence presented, Special Master

Moran concluded that the onset of SMARD could be consistent for a child of [O.A.S.]’s

age, and determined that “on the whole, [O.A.S.]’s age of onset tends to favor SMARD,

not GBS/CIDP.” Id.

As a fourth data point in his discussion section, Special Master Moran analyzed

whether [O.A.S.]’s weakness pattern, including weakness in her diaphragm and in her

distal 38 extremities, as well as whether her “absent” reflexes, were more consistent with

SMARD or GBS. Special Master Moran cited Dr. Finkel’s testimony that, “[t]he clinical

diagnostic criteria for GBS are weakness in one or more limbs and lack of reflexes.”

The Special Master also noted similar testimony by Dr. Maertens, who indicated that “in

most cases [of GBS], the reflex[es] are decreased or lost.” Additionally, Special Master

Moran quoted Dr. Finkel’s testimony, who stated that “‘absent reflexes [are] not a

necessary feature for SMARD.’” Id. Dr. Maertens was not asked to comment on

reflexes in SMARD cases and did not offer testimony in this regard.

Special Master Moran noted that, according to [O.A.S.]’s discharge report

prepared by Dr. Napa from Mercy Medical Center on March 28, 2001, [O.A.S.]

“‘continue[d] to have normal reflexes.’” Id. Subsequently, however, when [O.A.S.] was

at Johns Hopkins Hospital, on April 26, 2001, “Dr. Crawford tested her tendon reflexes”

and he “found that they were absent.” Id. Finally, when [O.A.S.] was at the University

of Iowa Hospitals, the medical report on May 16, 2001 indicated that her reflexes had

improved. Id. Special Master Moran observed that when:

Dr. Maertens was asked to address [O.A.S.]’s reflexes during her first

hospitalization at Mercy, his response was an indication of 2+ reflexes,

which is a normal result, ‘makes no sense.’ Dr. Maertens ‘just [could not]

believe that [Dr. Narawong was] right’ about [O.A.S.]’s reflexes. In

reference to Dr. Napa’s March 28, 2001 discharge report about [O.A.S.]

maintaining her reflexes, Dr. Maertens said that this data point does not

favor either SMARD or GBS/CIDP.

Id. Special Master Moran agreed with Dr. Finkel that “the preservation of [O.A.S.]’s

reflexes . . . makes the diagnosis of GBS/CIDP less likely.” Id. He also noted that

[O.A.S.]’s weakness in her diaphragm is “helpful in distinguishing SMARD from

38

According to Dorland’s Illustrated Medical Dictionary, distal means “remote; farther

from any point of reference; opposed to proximal.” Dorland’s Illustrated Medical

Dictionary 555.

21

GBS/CIDP” because “[d]iaphragmatic eventration[ 39] is consistent with SMARD.” Id. at

*31.

As a fifth data point in his discussion section, Special Master Moran considered

whether [O.A.S.]’s permanent need for assistance with her breathing is more consistent

with SMARD or GBS/CIDP. Considering whether progress to permanent ventilator

support within one month of respiratory failure is consistent with SMARD, the Special

Master noted Dr. Finkel’s opinion that all “SMARD patients require ventilator support.”

Id. The Special Master cited a 2011 study, in which “researchers reported the results of

a long-term study of 11 children with SMARD who survived their first year of life. The

authors state that by nine months, all children ‘were mechanically ventilated.’” Id.

Special Master Moran also noted that “Dr. Maertens agreed that [O.A.S.]’s progression

to permanent ventilator support ‘would probably go more towards SMARD.’” Id. The

Special Master acknowledged, however, Petitioners’ argument that “[O.A.S.] did not

require ventilator support until more than 30 days after her initial episode of respiratory

arrest on January 30, 2001,” and that [O.A.S.] “could breathe on her own” for one

month, which, “makes [O.A.S.]’s case inconsistent with SMARD and consistent with a

relapsing case of GBS/CIDP.” Id. Special Master Moran noted that “Dr. Finkel

acknowledged the reasonableness of the Simanskis’ point.” Id.

Turning to the discussion on GBS/CIDP, Special Master Moran noted that “Dr.

Finkel stated that permanent ventilator support was inconsistent with GBS and highly

inconsistent with CIDP.” Id. Dr. Maertens’ clinical experience was consistent with Dr.

Finkel’s opinion, as Dr. Maertens testified that, “[a]mong his pediatric patients with

GBS,” probably “one” remained permanently ventilated. Id. Nevertheless, the Special

Master noted, “Dr. Maertens maintained that progression to permanent ventilator

support ‘occurs with GBS and persists in CIDP if the patient is unresponsive to IVIG

and/or steroids.’” Id. Special Master Moran also noted that “Dr. Finkel, too, recognized

that GBS can lead to long-lasting respiratory problems,” and, in two articles cited by Dr.

Finkel in support, “approximately 20 percent of people with GBS have respiratory

problems.” Id. at *31 (citing Halawa, et al., Guillain-Barré Syndrome as a Prominent

Cause of Childhood Acute Flaccid Paralysis in Post Polio Eradication Era in Egypt, 15

Eur. J. Paediatric Neurol. 241, 242 (2011) (stating “[r]espiratory failure is the most life

threatening complication and mechanical ventilation has been reported to be needed in

about 20-30% of patients”), and DiMario Jr., et al., Autonomic Dysfunction in Childhood

Guillain-Barré Syndrome, 27 J. Child Neurol. 581, 585 (2012) (noting respiratory failure

in 15-24% of pediatric cases of GBS)). The Special Master concluded that “[a] fraction

of GBS-afflicted people, perhaps one in five, requires ventilator assistance. But, all

patients with SMARD are aided in their breathing. [O.A.S.]’s progression to permanent

ventilator support weighs in favor of SMARD.” Id. at *32. (emphasis in original).

39

Dorland’s Illustrated Medical Dictionary defines diaphragmatic eventration as “a

congenital anomaly characterized by failure of muscular development of part or all of

one (or occasionally both) hemi diaphragms, resulting in superior displacement of

abdominal viscera and altered lung development.” Dorland’s Illustrated Medical

Dictionary 655.

22

As a sixth data point in his discussion section, Special Master Moran evaluated

the biopsy results of [O.A.S.]’s sural nerve, which showed normal myelination. Id.

Special Master Moran observed that “[t]here is no question that the majority of biopsies

from patients suffering either GBS or CIDP return abnormal results.” Id. He also cited

the testimony of Dr. Finkel, who “estimated that in cases of GBS, the biopsy is abnormal

more than 90 per cent of the time,” and noted that “Dr. Maertens agreed.” Id. Dr.

Maertens stated, however, that since the biopsy showed only “mild inflammation” and

not any demyelination, the results did not go “in any specific direction.” Id. The Special

Master determined that “Dr. Maertens’s opinion overlooks how frequently biopsies are

abnormal in GBS/CIDP. It is also inconsistent with the literature that he provided.” The

Special Master also indicated that Dr. Finkel’s statement that “[O.A.S.]’s normal biopsy

was inconsistent with, but not impossible for, GBS/CIDP, is more persuasive.” Id.

Special Master Moran concluded, with regards to his sixth data point, that “[w]hile a

normal biopsy is not dispositive of a diagnosis, [O.A.S.]’s normal biopsy would place her

in the minority for GBS patients (ten percent).” Id. Therefore, the Special Master found

that “[O.A.S.]’s normal biopsy supports a diagnosis of SMARD.” Id.

As a seventh data point in his discussion section, Special Master Moran

analyzed the results of [O.A.S.]’s CSF level testing. [O.A.S.]’s CSF level was tested on

March 3, 2001, and the result was normal. Special Master Moran noted that “[i]n only

approximately ten percent of GBS/CIDP cases is the protein level normal.” Id. The

Special Master also observed, relying on Dr. Finkel’s testimony, that while “a patient

does not have to have elevated protein levels for a diagnosis of GBS,” “a normal protein

level was ‘certainly not consistent with general concepts about GBS’ and that it would be

‘unusual.’” Id. Dr. Maertens asserted, however, that a “normal [protein level] doesn’t

rule out GBS,” and also testified that “he is quick to conduct a spinal tap in his patients

because ‘spinal fluid is all you need to make a diagnosis of GBS.’” Id. at *33. Dr.

Maertens testified that the timing of the test is critical, and that the level of protein in the

CSF depends “‘on how active the demyelination is at the time of the lumbar puncture

. . . if you do it too early, you might miss it, if you do it too late, you might miss it.’” Id.

Dr. Finkel disagreed, however, with Dr. Maertens’ opinion, and explained that

“the protein level ‘can be normal in the first few days, and it does tend to go up in the first

week to ten days . . . it will build up, but it doesn’t just come back down to normal. So it’s

going to be elevated for several weeks.’” Id. Dr. Finkel maintained that “if [O.A.S.]

suffered from a demyelinating disease (either GBS or CIDP) beginning ‘a few weeks’

before the lumbar puncture, as Dr. Maertens proffered, then she would still be

undergoing demyelination on March 3, 2001 [the date of her CSF test].” Id. Special

Master Moran, therefore, concluded:

Although a normal CSF protein might occur in a patient with GBS/CIDP, a

normal CSF protein is typical for a SMARD patient. Dr. Finkel stated that

[O.A.S.]’s normal protein level was highly consistent with SMARD. Dr.

Maertens, too, testified that a normal CSF protein level is consistent with

23

SMARD. Therefore, [O.A.S.]’s normal CSF protein level tends to favor of

[sic] a diagnosis of SMARD.

Id.

As an eighth data point in his discussion section, Special Master Moran

considered [O.A.S.]’s creatinine levels. The Special Master cited to thirteen examples

of [O.A.S.]’s creatinine level tests, which, with an exception of the results obtained at the

Mayo Clinic, and one result obtained at the University of Iowa Hospitals, were

consistently low. Id. The experts differed in their views as to whether [O.A.S.]’s

creatinine levels results were consistent with SMARD. Dr. Maertens stated that “an

abnormally low creatinine level is consistent with a diagnosis of SMARD,” but, “in

[O.A.S.]’s case her creatinine level did not support a diagnosis of SMARD or GBS/CIDP

because her creatinine levels differed throughout her illness.” Id. at *34. Instead, Dr.

Maertens argued that “he would ‘toss’ the creatinine data point.” Id. Dr. Finkel, on the

other hand, maintained that [O.A.S.]’s creatinine test results were “consistent with a

diagnosis of SMARD” even after the rise of creatinine level because “‘children with

SMARD can actually improve . . . they can increase their muscle strength.’” Id. Special

Master Moran noted that “[n]either Dr. Finkel nor Dr. Maertens associated low levels of

creatinine with GBS/CIDP,” which was reflected in his chart as neutral for GBS/CIDP.

Id. Special Master Moran concluded that “[O.A.S.]’s low creatinine level provides

modest support for SMARD.” Id.

The results of EMG tests and nerve conduction studies were Special Master

Moran’s ninth data point in his discussion section. The Special Master explained that

“[t]he nerve conduction study can measure the latency, velocity, and amplitude of an

electric signal. A low result for amplitude means that the axon, the part of the nerve that

carries the electrical signal, is damaged. When the velocity is slow, the myelin is

damaged.” Id. The Special Master explained that in the background section of his

decision, he had already summarized all of [O.A.S.]’s EMG test results, and provided

the interpretations offered by both Dr. Maertens and Dr. Finkel. Therefore, he indicated

that “[r]ather than repeat these summaries, this portion of the decision highlights the

results of two tests – the first one, which was conducted on February 26, 2001, at the

Mayo Clinic, and the fourth one, which was conducted on April 24, 2001,[ 40] at Johns

Hopkins.” Id. After analyzing the experts’ evaluations of [O.A.S.]’s first EMG from the

Mayo Clinic, the Special Master noted that “both Dr. Maertens and Dr. Finkel interpreted

the first EMG as showing some axonal damage. But, there was some disagreement as

to whether this EMG showed demyelination.” Id. at *35. Specifically, “Dr. Finkel stated

that he did not see any evidence of demyelination and Dr. Maertens said the results

were ‘not totally inconsistent with demyelination.’” Id. The EMG conducted at John

Hopkins Hospital on April 26, 2001, showed, according to Dr. Maertens, some

“‘[d]emyelination,’” as well as indicated a clear decline of [O.A.S.]’s condition, so “that’s

more an axonal injury now.” Id. at *36. Dr. Finkel also “concluded that the process

40

As noted above, the correct date of the EMG study conducted at Johns Hopkins

Hospital is April 26, 2001.

24

affecting [O.A.S.]’s nerves was axonal,” but he determined that the test results showed

“no segmental slowing,” which is a hallmark for GBS/CIDP. Id. Therefore, “Dr. Finkel

found this nerve conduction study as supporting SMARD, and not supporting

GBS/CIDP.” Id. After examining the evidence, the Special Master determined that the

results of the EMG studies supported a “logical deduction” that [O.A.S.] did not have

GBS/CIDP. See id.

As a tenth data point in his discussion section, Special Master Moran analyzed

whether [O.A.S.]’s responses to the IVIG treatment were more consistent with SMARD

or with GBS/CIDP. Special Master Moran noted the testimony of Dr. Maertens, who

stated that [O.A.S.]’s improvement after the IVIG treatments strongly supports a

diagnosis of GBS. Dr. Maertens explained that [O.A.S.] “responded to the first

treatment, and she responded again a little bit to the second treatment, but definitely to

the first treatment she responded to the point of coming off the vent. She really did

improve remarkably. And you would not expect any response to treatment with SMARD,

with IVIG.” Respondent’s expert, Dr. Finkel, also testified that after the IVIG treatments,

[O.A.S.] showed “some marginal signs that hinted at some improvement.” Id. at *37.

Dr. Finkel argued, however, with respect to [O.A.S.]’s first IVIG treatment at the Mayo

Clinic, after which her condition significantly improved, that “the improvement ‘was

largely due to the improvement in the RSV, not the underlying peripheral neuropathy.’”

Id. Special Master Moran observed that after her second and third round of IVIG

treatment, [O.A.S.] improved only slightly, if at all. Therefore, although admitting that

“[t]his data point presents a closer call,” the Special Master stated: “[i]f [O.A.S.] truly

suffered from an immune-mediated neurological disease like GBS or CIDP, then she

probably would have made a more significant improvement. Thus, on the whole,

[O.A.S.]’s lack of response to IVIG tends to favor SMARD, but only slightly.” Id.

As the eleventh data point in the discussion section of the Special Master’s

decision, although not included as a separate issue on his chart, Special Master Moran

considered the diagnoses by [O.A.S.]’s treating doctors. At first, he noted that the

“nomenclature” used by “some doctors” to “label [O.A.S.]’s condition,” “limit[s] the value”

of some of the treating physicians’ opinions. Id. The Special Master noted that the

phrase “‘peripheral neuropathy,’” which some of the doctors used to describe [O.A.S.]’s

condition, “without any modification – does not distinguish SMARD from GBS/CIDP.

SMARD also affects the peripheral nerves.” Id. The Special Master indicated that “the

Simanskis appear to interpret this phrase as supporting their claim that [O.A.S.] suffered

GBS/CIDP.” Id. The Special Master also commented that “[i]n contrast, the phrase

‘post-infectious demyelinating neuropathy’ . . . points to GBS/CIDP because those

conditions are demyelinating conditions and SMARD is not.” Id. As a second issue, the

Special Master mentioned the fact that “most pediatric neurologists were not aware of

SMARD until 2003, when an article was published in Annals of Neurology,” alerting

most pediatric neurologists to the existence of the SMARD disease. 41 Id. at *38. He

41

As noted above and as Dr. Finkel testified, an article on SMARD, authored by Dr.

Grohmann, was published in the journal Annals of Neurology in 2003. See Grohmann,

et al., Infantile Spinal Muscular Atrophy with Respiratory Distress Type 1 (SMARD1), 54

25

emphasized that “[t]his lack of awareness needs to be taken into account when

evaluating statements” from physicians diagnosing [O.A.S.] before 2003. Id. Special

Master Moran noted that “[O.A.S.]’s treating physicians have consistently referenced

SMARD as the proper diagnosis since 2003.” Id. at *39. The Special Master stated that

regarding a SMARD diagnosis for [O.A.S.], “[t]he Simanskis have done very little to

refute these conclusions,” including allowing genetic testing. Id. The Special Master

also noted that, “in their reply brief, the Simanskis identified a single post-2003 medical

record in which Dr. Gavin described [O.A.S.] as ‘a four-year-old female with Peripheral

Neuropathy of unknown etiology . . . . One medical consultant has suggested she may

have Spinal Muscular Atrophy with Respiratory Distress but this diagnosis has yet to be

confirmed.’” Id.

As his twelfth and final data point in the discussion section of his decision,

although, as with the eleventh data point, not addressed as a separate consideration on

the chart included in his decision, Special Master Moran compared two of the parties’

experts: Petitioners’ expert, Dr. Maertens, and Respondent’s expert, Dr. Finkel. Noting

that both experts are board-certified in pediatrics and neurology with a special

competence in child neurology, Special Master Moran wrote that, “[u]nlike Dr. Maertens,

Dr. Finkel also possesses separate board certification in electrodiagnostic medicine and

a separate subspecialty certification in neuromuscular medicine,” which “enable him to

perform EMGs and nerve conduction studies.” Id. at *40. Moreover, Special Master

Moran also indicated that Dr. Finkel “also typically reviews the data of tests performed

by other people and offers his interpretation of the study.” Id. Special Master Moran

further noted that Dr. Finkel “has written more than 60 articles published in peer-

reviewed journals,” almost all of which “discuss pediatric neuromuscular issues,” and

also has “served on the editorial board of the journal, Neuromuscular Disorders.” Id.

Dr. Finkel testified regarding SMARD that “the World Muscle Society, [] is where I first

heard about it [SMARD], in about 2000-2001,” while, in contrast, “Dr. Maertens testified

that he first learned about SMARD in 2005 or 2006.” Id. at *38. Special Master Moran

further noted that “[i]n rejecting SMARD as a diagnosis, Dr. Maertens referred to the

lack of a ‘definitive test,’ apparently meaning a genetic test.” Id. at *40. Overall, the

Special Master found Dr. Finkel “consistent and persuasive” while Dr. Maertens,

according to the Special Master, “lack[ed] consistency and persuasiveness.” Id. The

Special Master did not address in any detail the other two experts, Petitioners’ Dr.

Shoenfeld and Respondent’s Dr. McCusker, and gave Dr. Finkel’s expert opinion more

weight than Dr. Maertens’ expert opinion.

Special Master Moran summarized his findings as follows:

[O.A.S.]’s course is very much consistent with SMARD. In contrast, as Dr.

Finkel noted, [O.A.S.] would be an exception in almost every diagnostic

Ann. Neurol. 719 (2003). As also noted above, the same lead author earlier had

published a journal article on SMARD in 2001. See Grohmann, et al., Mutations in the

Gene Encoding Immunoglobulin µ-binding Protein 2 Cause Spinal Muscular Atrophy with

Respiratory Distress Type 1, 29 Nat. Genet. 75, 76 (2001).

26

criteria for GBS. Tr. 1220. Dr. Maertens’s opinion that [O.A.S.] suffered

from GBS, which relapsed and turned into CIDP, largely ignores or

minimizes the significance of most of [O.A.S.]’s symptoms. See Tr. 811-

15 (Dr. Maertens repeatedly stating that various data points do not support

either diagnosis). Under these circumstances, the preponderance of

evidence supports the finding that [O.A.S.] suffers from SMARD.

Id. at *41 (footnote omitted). The Special Master continued: “A preponderance of the

evidence establishes that [O.A.S.] suffers from spinal muscular atrophy with respiratory

distress, not Guillain-Barré syndrome or chronic inflammatory demyelinating

polyneuropathy. The Simanskis have offered no evidence to establish that [O.A.S.]’s

vaccinations caused her to suffer SMARD. Therefore, the Simanskis are not entitled to

compensation.” Id. at *42.

After the Special Master issued his decision, the Petitioners filed a timely Motion

for Review in this court on September 19, 2013. In their Motion for Review, Petitioners

claim that the Special Master “ignored highly relevant evidence on each and every ‘data

point’ he used to make this determination,” and that “[t]he cumulative effect of failing to

consider highly relevant evidence on each ‘data point’ resulted in a blatant error of

law.” 42 While the Petitioners agree that, “in certain cases, ‘[i]dentifying the injury is a

prerequisite to the [Althen] analysis,’” 43 quoting Broekelschen v. Sec'y of Health &

Human Servs., 618 F.3d 1339, 1346 (Fed. Cir.), reh’g en banc denied (Fed. Cir. 2010)),

Petitioners reject the Special Master’s “evaluation of the evidence with respect to each

‘data point’ [which] concluded that every single data point was ‘consistent’ with a

diagnosis of SMARD.” 44

42

The court notes that Petitioners’ Motion for Review is replete with bold, italicized or

underlined phrases. In order to make this opinion more readable, the various forms of

emphasis included by Petitioners in their Motion for Review have been removed and are

not reflected as emphasis in each of Petitioners’ quotes included in this opinion.

43

As noted above, at oral argument on November 18, 2013, the Petitioners’ counsel

agreed with respect to the proceedings before the undersigned that “we’re talking only

about, for the purposes of this review, whether or not the SMARD conclusion by Special

Master Moran is the correct one.” Counsel also agreed at the hearing, and the

Petitioners asserted in their Motion for Review, that “there was no need to explore in

detail . . . whether the vaccines could have adversely affected [O.A.S.]’s SMARD via the

Althen test.”

44

Petitioners also note that the Special Master arrived at this conclusion despite his

initial statement that “‘[s]ome signs and symptoms are consistent with SMARD and

some are inconsistent, or not associated with SMARD.’” As is discussed below,

although the Special Master appears to have made this statement, and although his

chart, included above, indicates “Response to IVIG” as neutral for SMARD, the

discussion section of the Special Master’s decision does not indicate that any of the

other of [O.A.S.]’s presentations for the numerous symptoms and tests are inconsistent

with SMARD.

27

The Petitioners claim that “[t]he cumulative effect of failing to consider highly

relevant evidence on each ‘data point’ . . . grossly elevated the petitioners’ proof

requirement and deprived them of the statutory preponderant requirement.”

Consequently, according to the Petitioners, “in dismissing [O.A.S.]’s claims, the special

master abused his discretion and made arbitrary and capricious findings.”

While the Petitioners contend that each and every determination made by the

Special Master was arbitrary, capricious, and an abuse of discretion, Respondent more

broadly addresses the Petitioners’ allegations, stating generally that the Special

Master’s factual findings “were based on a full and amply reasoned consideration of the

record evidence,” and that Special Master Moran correctly determined that the majority

of [O.A.S.]’s symptoms support a diagnosis of SMARD. According to the Respondent,

based on Munn v. Secretary of the Department of Health and Human Services, 970

F.2d 863, 871 n.10 (Fed. Cir. 1992), the Vaccine Act imposes a highly deferential

standard of review of a Special Master’s factual findings, which can be set aside only

when “arbitrary and capricious,” and that the Special Master’s decision was not arbitrary

or capricious and should be sustained.

Respondent asserts that Special Master Moran correctly determined that the

majority of [O.A.S.]’s signs, symptoms, and test results, included in his data points,

support a diagnosis of SMARD. Respondent also argues that the Special Master

“applied the correct evidentiary standard and stated a rational basis for his conclusion

that ‘the weight of the entire record indicates that [O.A.S.] suffers from SMARD.’”

Respondent adds:

While petitioners argue that the Special Master erred in making such a

finding . . . . He described the diagnoses alleged by petitioners (GBS and

CIDP) and respondent (SMARD), meticulously analyzed the relevant

medical records and literature, and carefully evaluated the opinions of both

petitioners’ and respondent’s medical experts.

Discussing Special Master Moran’s finding that [O.A.S.]’s respiratory failure at

twelve and a half weeks is consistent with the diagnosis of SMARD, Respondent notes

that “[w]hile Dr. Maertens’s testimony on this issue varied at differing times in his

testimony, he conceded that onset of GBS in a two-month-old infant is ‘extremely rare.’”

Respondent also maintains, citing Gilmartin, et al., Guillain-Barré Syndrome with

Hydrocephalus in Early Infancy, 34 Arch. Neurol. 567 (2007), that “literature provided by

petitioners supports the Special Master’s finding that [O.A.S.]’s age at onset argues

against a diagnosis of GBS,” because GBS “has not, to our knowledge, been reported

in early infancy.” According to the Respondent, the Special Master’s other

determinations also support the Special Master’s finding of SMARD and are

inconsistent with diagnosis of either GBS or CIDP, including [O.A.S.]’s need for

permanent ventilator support, her EMGs and nerve conductions studies, her IUGR, her

patterns of weakness and initial preservation of reflexes, as well as the results of her

sural nerve biopsy and the CSF protein level testing. Moreover, Respondent points out

28

that medical records, as well as the statements from [O.A.S.]’s treating physicians,

support the Special Master’s conclusion that [O.A.S.] appears to suffer from SMARD.

Finally, Respondent claims that “in analyzing the various symptoms and test results, the

Special Master necessarily had to weigh the opinions offered by both pediatric

neurologists, Drs. Finkel and Maertens,” and that, based on their respective

qualifications, and testimonies, Special Master Moran’s decision to find Dr. Finkel’s

testimony more persuasive is “well-supported, without legal error, and is ‘virtually

unchallengeable on appeal,’” quoting Porter v. Sec’y of Health & Human Servs., 663

F.3d 1242, 1251 (Fed. Cir. 2011), reh’g en banc denied (Fed. Cir. 2012).

DISCUSSION

Although presented in an order different from the one the Special Master utilized,

the Petitioners focus their objections on seven of the Special Master’s determinations

and dispute the Special Master’s final conclusion. First, Petitioners contest the Special

Master’s tenth data point conclusion in the discussion section of his decision, that

[O.A.S.]’s response to IVIG treatment was consistent with a diagnosis of SMARD, and

that according to the Special Master:

After [O.A.S.] received the first course of IVIG, she did not need

assistance from a ventilator. However, it is less clear that she improved

because of the IVIG . . . . Moreover, after the second and third doses of

IVIG, [O.A.S.] improved only slightly, if at all. If [O.A.S.] truly suffered from

an immune-mediated neurological disease like GBS or CIDP, then she

probably would have made a more significant improvement. Thus, on the

whole, [O.A.S.]’s lack of response to IVIG tends to favor SMARD.

Petitioners claim that the Special Master ignored relevant evidence when he

concluded [O.A.S.]’s response to IVIG treatment was consistent with a diagnosis of

SMARD. Petitioners note that the “medical literature does indicate that,

‘Responsiveness to immune modulating therapy is an important feature of CIDP and

intravenous immunoglobulin (IVIG) is effective.’” Therefore, according to Petitioners, it

is significant that [O.A.S.]’s health improved “dramatically” after the IVIG treatment, and,

four days after the completion of treatment with IVIG, “[O.A.S.] was weaned off positive

pressure to room air.” Petitioners also point out that soon thereafter, on March 28,

2001, [O.A.S.] was discharged from the hospital in improved condition: “She remained

extubated, her reflexes returned, and movement of her arms and legs were noted. By

the time of discharge, she had been weaned off tube feedings and was bottle fed.”

Therefore, Petitioners argue that the IVIG treatment was “an effective treatment for

[O.A.S.]’s GBS/CIDP.”

Petitioners dispute Dr. Finkel’s expert report, stating that [O.A.S.] showed no

apparent therapeutic benefit from IVIG, and contrast the report with Dr. Finkel’s

testimony, during which, according to Petitioners, “he conceded that [O.A.S.] showed

some marginal improvement after both courses of IVIG.” Petitioners further argue that

“SMARD does not have a relapsing remitting course and, IVIG is not effective in

29

treatment of SMARDs.” Further, Petitioners contend that the Special Master ignored

the testimony offered by the Petitioners’ expert, Dr. Maertens, who testified that, based

on his experience, the effectiveness of IVIG wears off 2-3 weeks after treatment.

Petitioners note that after [O.A.S.]’s March 28, 2001 discharge from Mercy Medical

Center, in an improved condition after an IVIG treatment, “she required the reinstitution

of oxygen at low levels at about 20 days after her last treatment with IVIG.” They

allege, therefore, that “[O.A.S.]’s deterioration about 20 days after her last treatment

with IVIG is entirely consistent with the time frame Dr. Maertens’ noted deterioration in

his patients who receive IVIG.” Thus, Petitioners assert, “[O.A.S.]’s improvement after

her IVIG treatments clearly supports a diagnosis of GBS/CIDP, and is highly

inconsistent with a SMARD diagnosis.”

Additionally, Petitioners claim that [O.A.S.]’s response to the second course of

IVIG therapy, which showed less improvement in her health than the previous one,

easily can be explained by the testimony of Dr. Maertens, who stated that, since

[O.A.S.]’s EMG exam showed axonal damage, “he would not expect as much

improvement because it takes time and persistence with injury at that level.” Dr.

Maertens also added that “[a]xonal injuries are more irreversible,” and, although the

potential for recovery exists, “you never get back to normal. At some point you cannot

recover at all.” Petitioners note that “[O.A.S.]’s treating physician at Iowa understood

this,” 45 and stated, on May 7, 2001, although before the 2003 apparently more widely

recognition of SMARD in the medical community, “Acute motor predominantly axonal

neuropathy - not unlike axonal GBS. Pt has been w 4 courses of IVIG + 1 of steroids.

Pt now more recently shows some improvement in legs w reapp. of reflexes. Would

expect gradual incomplete[ 46] recovery slowly.”

Second, Petitioners disagree with Special Master Moran’s determination that

[O.A.S.]’s EMG studies were consistent with a diagnosis of SMARD, the Special

Master’s ninth data point in his discussion section. Petitioners note that although

“[O.A.S.] underwent five (5) EMG studies,” the Special Master concentrated on

[O.A.S.]’s April 26, 2001 EMG study, and “ignored the several studies that supported a

diagnosis of GBS.” Petitioners argue that all five of [O.A.S.]’s EMG studies, conducted

on March 7, 2001, 47 April 17, 2001, April 26, 2001, May 8, 2001 and September 17,

2003, support a diagnosis of a peripheral neuropathy, which they allege is symptomatic

45

The court notes that the May 7, 2001 note was not issued by Dr. Mathews, who

prepared the May 8, 2001 report about history of [O.A.S.]’s disease.

46

The court notes that Petitioners appear to quote the last sentence incorrectly,

indicating that the University of Iowa Hospitals note stated: “Would expect gradual

incomplete recovery slowly.” In the original, there is a question mark after the word

“incomplete.”

47

The court notes that there was no EMG performed on that day. The date listed in

[O.A.S.]’s medical records for an EMG at the Mayo Clinic is March 6, 2001. Also, as

noted above, [O.A.S.] underwent six, not five, EMG studies.

30

for GBS/CIDP. Petitioners conclude that “[f]or the special master to have made a

determination that [O.A.S.]’s EMG studies do not support a GBS/CIDP diagnosis is

another blatant abuse of discretion.”

Third, Petitioners challenge Special Master Moran’s statement regarding his fifth

data point in the discussion section of his decision that:

“The probabilities make [O.A.S.]’s unfortunate need for ventilator support

more consistent with SMARD, not GBS. The need for ventilator

assistance, however, is not unheard of in GBS cases. A fraction of GBS-

afflicted people, perhaps one in five, requires ventilator assistance. But, all

patients with SMARD are aided in their breathing. [O.A.S.]’s progression

to permanent ventilator support weighs in favor of SMARD.”

(emphasis in original).

Petitioners contend that while Special Master Moran and Dr. Finkel agree that

progression to permanent ventilation support within one month of the onset of

respiratory failure is “highly consistent” with SMARD, “[O.A.S.] did not progress to

permanent ventilation support after her respiratory failure.” They claim that during his

testimony, Dr. Finkel “agreed that [O.A.S.] was extubated for approximately one month

after her arrival in respiratory failure.” Moreover, Petitioners claim that “[f]ollowing

[O.A.S.]’s improvement, she was readmitted to the hospital on April 13, 2001 in

respiratory distress. This relapsing and remitting course, however, is inconsistent with a

diagnosis of SMARD, but is characteristic for relapsing GBS and CIDP.”

Fourth, Petitioners contest Special Master Moran’s determination on his second

data point in his discussion section, that [O.A.S.]’s “presentation with respiratory failure”

is consistent with SMARD. Petitioners dispute his finding that [O.A.S.]’s “‘health

preceding her respiratory arrest and her respiratory arrest are generally consistent with

SMARD,’” and that “‘the presentation with respiratory failure is more common in

SMARD, than in GBS/CIDP.’” Petitioners argue:

The medical literature filed in [O.A.S.]’s case indicates that every child

who was diagnosed with SMARD with a known gene mutation had the

onset of symptoms prior to the presentation of respiratory arrest. In other

words, there was a history of abnormality noted by either the parents or

the pediatricians prior to the respiratory arrest. This is unlike [O.A.S.]’s

history, one that is clearly documented in the records of her multiple

hospitalizations. Every hospitalization notes that [O.A.S.] was doing well,

gaining weight, and without developmental issues prior to her respiratory

arrest.

Petitioners highlight that “even the special master stated, ‘Before the January 26, 2001

vaccinations, [O.A.S.] was basically healthy.’”

31

Petitioners also cite the Grohmann article in the record, Infantile Spinal Muscular

Atrophy with Respiratory Distress Type 1 (SMARD1), 54 Ann. Neurol. 719 (2003),

stating that:

prior to the onset of respiratory failure at a median age of 3.5 months,

100% of patients had a weak cry (median age 1 month) and respiratory

distress (median age 3 months), 50% of patients had inspiratory stridor

(median age 0.5 months), while 58% of patients exhibited poor feeding

prior to the respiratory failure.

Petitioners assert that “[O.A.S.] experienced none of these symptoms prior to her

respiratory arrest.” Petitioners conclude that the Special Master failed to consider the

evidence that “all patients with genetically confirmed SMARD were symptomatic prior to

presenting with respiratory failure at a median age of 3.5 months,” while “[O.A.S.] was

completely asymptomatic prior to presenting with respiratory arrest.”

Fifth, Petitioners contest the Special Master’s determination that “[O.A.S.]’s

treating physicians have consistently referenced SMARD as the proper diagnosis since

2003,” his eleventh data point in the discussion section, is also arbitrary, capricious and

an abuse of discretion. Petitioners cite to ten examples of [O.A.S.]’s doctors’ diagnoses

of peripheral neuropathy, which Petitioners assume is the same as a diagnosis of GBS,

eight diagnoses in 2001, a diagnosis to that effect in 2003 and one in 2004, as follows:

-Results of a February 26, 2001 EMG indicated, “The findings are those of

a length-dependent sensorimotor peripheral neuropathy, such as could be

seen in inherited or metabolic neuropathies. There is no evidence of a

diffuse disorder of anterior horn cells or a myopathy.”

-On March 7, 2001, Dr. Kotagal stated, “The prolonged distal latency in

the [left] peroneal is suggestive of a demyelinating process, an acute or

subacute inflammatory demyelinating neuropathy.”

-On March 8, 2001, a progress noted indicated, “Given the [increased]

phrenic nerve latency[,] considering Guillain Barre syndrome [with]

primarily axial and phrenic nerve involvement.”

-A March 8, 2001 record also stated, “[Question of] AIDP variant.”

her discharge diagnosis was “probable post-infectious demyelinating

neuropathy.”

-On April 26, 2001, [O.A.S.] had a motor nerve conduction and needle

electromyography. The results indicated, “This study demonstrates the

presence of widespread neuropathic weakness. The pattern is consistent

with either a motor neuropathy or a sensorimotor axonal neuropathy.”

-[O.A.S.]’s May 3, 2001 admission note to the University of Iowa Hospitals

stated, “6 month old [with] likely progressive sensorimotor neuropathy.”

32

-On May 7, 2001, a neurological consult indicated that [O.A.S.] suffered an

“acute motor predominant axonal neuropathy – not unlike axonal GBS.”

-The results of an EMG/Nerve Conduction study from May 8, 2001

indicated, “These findings favor the diagnosis of peripheral neuropathy

over motor neuron disease.”

-On May 10, 2001, Dr. Katherine Mathews, a neurologist, stated,

“[O.A.S.]’s exam and findings are most suggestive of a peripheral

neuropathy . . . . Her clinical picture is not compatible with spinal muscular

atrophy (and DNA testing has been negative).”[ 48]

-On September 17, 2003, [O.A.S.] underwent an EMG study, which

indicated, “INTERPRETATION: There is neurophysiologic evidence for a

severe, diffuse sensorimotor peripheral neuropathy characterized primarily

by axonal loss.”

Petitioners also state that even “after the 2003 benchmark that the special mater [sic]

uses as support of a SMARD diagnosis, [O.A.S.]’s treating physician stated, [O.A.S.]

showed “Peripheral Neuropathy of unknown etiology . . . . One medical consultant has

suggested she may have Spinal Muscular Atrophy with Respiratory Distress but this

diagnosis has yet to be confirmed.” Petitioners also assert that the Special Master did

not indicate that a SMARD diagnosis was never confirmed, which also was noted by Dr.

Finkel in his February 20, 2013 testimony.

Sixth, Petitioners also argue that “the Special Master’s determination that clinical

findings alone can accurately diagnose SMARD is arbitrary, capricious, and an abuse of

discretion.” Petitioners assert, “[t]he respondent’s literature shows the poor predictive

value of using the clinical findings alone to predict whether a child has the genetic

mutation that confirms the diagnosis of SMARD.” Petitioners cite to the Grohmann

article, Infantile Spinal Muscular Atrophy with Respiratory Distress Type 1 (SMARD1),

54 Ann. Neurol. 719 (2003), which “explores 65 patients with the SMARD phenotype

(clinical signs and symptoms) who actually were tested for the genetic mutation. Of

these 65, more than half, thirty-six (36), were negative for the genetic mutation

associated with SMARD (IGHMBP2).” Finally, Petitioners argue that [O.A.S.] “did not

have the clinical signs and symptoms prior to her respiratory failure that were noted in

patients who were eventually diagnosed with SMARD,” but even if she did, “the medical

literature supports the poor predictive value of clinical findings alone to confirm the

diagnosis of SMARD.”

Seventh, Petitioners claim that Special Master Moran’s failure to consider

relevant evidence on other “data points,” including [O.A.S.]’s IUGR, the Special Master’s

first data point in the discussion section, the normal sural nerve biopsy, the Special

Master’s sixth data point in the discussion section, her CSF protein level, the Special

48

As explained above, no genetic testing of [O.A.S.] has been conducted and the

Petitioner parents have declined to conduct such testing.

33

Master’s seventh data point in the discussion section and her creatinine level, the

Special Master’s eighth data point in the discussion section, were arbitrary, capricious,

and an abuse of discretion. Regarding [O.A.S.]’s IUGR, Petitioners claim that Special

Master Moran concluded that [O.A.S.]’s IUGR “supports a diagnosis of SMARD,” but

that he failed to consider Dr. Finkel’s concession that while IUGR places an infant at

higher risk for developing problems, “‘[i]t doesn’t imply that they will have problems, of

course.’” Moreover, Petitioners claim Special Master Moran failed to mention Dr.

Finkel’s testimony in which he indicated that “his review of [O.A.S.]’s early pediatric

records did not reveal that [O.A.S.] suffered any ill consequences from her IUGR.”

Regarding the normal sural biopsy, Petitioners state that Special Master Moran

“ignored undisputed evidence that a sampling error could result in obtaining a ‘normal’

tissue during biopsy.” They also allege that the Special Master ignored expert

testimony that [O.A.S.]’s sural nerve biopsy was supportive of an inflammatory

neuropathy. Finally, Petitioners claim that the Special Master ignored the Grohmann

article, Infantile Spinal Muscular Atrophy with Respiratory Distress Type 1 (SMARD1),

54 Ann. Neurol. 719 (2003), which indicated that “sural nerve biopsy was positive in 10

of 15 patients with the confirmed genetic mutation in SMARD, and showed axonal

degeneration.” Petitioners claim that [O.A.S.]’s nerve biopsy did not show axonal

degeneration, but that the Special Master failed to acknowledge this in his decision.

Turning to [O.A.S.]’s “normal” CSF protein level, Petitioners note that Special

Master Moran cited both experts, Dr. Finkel and Dr. Maertens, who each indicated that

[O.A.S.]’s normal protein level was either highly consistent with SMARD (Dr. Finkel) or

consistent with SMARD (Dr. Maertens). Petitioners, however, claim that the Special

Master ignored “highly relevant” evidence from both [O.A.S.]’s medical records and from

the medical literature in the record, which explain that the reason for a normal CSF

protein level “may be that CSF was sampled relatively late in the illness as CSF protein

alteration is maximal in the first 2 weeks of illness.” Moreover, Petitioners claim that

several of the articles in the record demonstrate that normal protein levels are present in

certain GBS cases. The first article Petitioners cite, Eckert, et al., A Case of Influenza

Vaccination Induced Guillain Barré Syndrome with Normal Cerebrospinal Fluid Protein

and Improvement on Treatment with Corticosteroids, 37 Scand. J. Infect. Dis. 621

(2005), indicates that approximately 10% of GBS patients show “no characteristic

protein elevation in the CSF.” Another article cited by the Petitioners, Deceunick RMB,

et al., Epidemiology of Guillain-Barré Syndrome in the Province of Quebec, 35 Can. J.

Neurol. Sci. 472 (2008), references a study in which lumbar punctures were performed

in thirty two of thirty three patients, and the elevated protein was only found in twenty

patients. Finally, the third article referenced in Petitioners’ Motion for Review, Gai-Fen,

et al., A Case-Control Study on Children with Guillain-Barre Syndrome in North China,

16 Biomed. Environ. Sci. 105 (2003), discusses a case-control study on children with

GBS in Northern China, in which thirty five of fifty one patients “‘had elevated protein

level - about 1/3 had normal levels.’” Regarding [O.A.S.]’s creatinine levels, Petitioners

claim that the Special Master “made no mention of the concession by Respondent’s

expert, Dr. Finkel that ‘no other physician considered her creatinine level to be indicative

or supportive of a diagnosis of SMARD.’”

34

When reviewing a Special Master's decision, the assigned Judge of the United

States Court of Federal Claims shall:

(A) uphold the findings of fact and conclusions of law of the special

master and sustain the special master's decision,

(B) set aside any findings of fact or conclusions of law of the special

master found to be arbitrary, capricious, an abuse of discretion, or

otherwise not in accordance with law and issue its own findings of fact and

conclusions of law, or

(C) remand the petition to the special master for further action in

accordance with the court's direction.

42 U.S.C. § 300aa-12(e)(2). The legislative history of the Vaccine Act states: “The

conferees have provided for a limited standard for appeal from the [special] master's

decision and do not intend that this procedure be used frequently, but rather in those

cases in which a truly arbitrary decision has been made." H.R. Rep. No. 101-386, at

517 (1989) (Conf. Rep.), reprinted in 1989 U.S.C.C.A.N. 3018, 3120.

In order to recover under the Vaccine Act, Petitioners must prove that the

vaccine caused the purported injury. See W.C. v. Sec’y of Health & Human Servs., 704

F.3d 1352, 1355-56 (Fed. Cir. 2013) (“The Vaccine Act created the National Vaccine

Injury Compensation Program, which allows certain petitioners to be compensated upon

showing, among other things, that a person ‘sustained, or had significantly aggravated’

a vaccine-related ‘illness, disability, injury, or condition.’” (quoting 42 U.S.C. § 300aa–

11(c)(1)(C))); Lombardi v. Sec’y of Health & Human Servs., 656 F.3d 1343, 1350 (Fed.

Cir.), reh’g en banc denied (Fed. Cir. 2011) (“A petitioner seeking compensation under

the Vaccine Act must prove by a preponderance of the evidence that the injury or death

at issue was caused by a vaccine.”); see also Shapiro v. Sec’y of Health & Human

Servs., 105 Fed. Cl. 353, 358 (2012), aff’d, 503 F. App’x 952 (Fed. Cir. 2013); Jarvis v.

Sec’y of Health & Human Servs., 99 Fed. Cl. 47, 54 (2011). Regarding the standard of

review, articulated in Markovich v. Secretary of Health and Human Services, the United

States Court of Appeals for the Federal Circuit wrote, “[u]nder the Vaccine Act, the

Court of Federal Claims reviews the Chief Special Master's decision to determine if it is

‘arbitrary, capricious, an abuse of discretion, or otherwise not in accordance with the

law.’ 42 U.S.C. § 300aa-12(e)(2)(B).” Markovich v. Sec’y of Health & Human Servs.,

477 F.3d 1353, 1355-56 (Fed. Cir.), cert. denied, 552 U.S. 816 (2007); see also

Deribeaux ex rel. Deribeaux v. Sec’y of Health & Human Servs., 717 F.3d 1363, 1366

(Fed. Cir.) (The United States Court of Appeals for the Federal Circuit stated that “we

‘perform[ ] the same task as the Court of Federal Claims and determine[ ] anew whether

the special master's findings were arbitrary or capricious.’” (quoting Lampe v. Sec’y of

Health & Human Servs., 219 F.3d 1357, 1360 (Fed. Cir. 2000))) (brackets in original),

reh’g and reh’g en banc denied (Fed. Cir. 2013); W.C. v. Sec’y of Health & Human

Servs., 704 F.3d at 1355; Hibbard v. Sec’y of Health & Human Servs., 698 F.3d 1355,

35

1363 (Fed. Cir. 2012); Avera v. Sec’y of Health & Human Servs., 515 F.3d 1343, 1347

(Fed. Cir.) (“Under the Vaccine Act, we review a decision of the special master under

the same standard as the Court of Federal Claims and determine if it is ‘arbitrary,

capricious, an abuse of discretion, or otherwise not in accordance with law.’” (quoting

42 U.S.C. § 300aa-12(e)(2)(B))), reh’g and reh’g en banc denied (Fed. Cir. 2008); de

Bazan v. Sec’y of Health & Human Servs., 539 F.3d 1347, 1350 (Fed. Cir.), reh’g and

reh’g en banc denied (Fed. Cir. 2008); Althen v. Sec’y of Health & Human Servs., 418

F.3d at 1277; Dodd v. Sec’y of Health & Human Servs., 114 Fed. Cl. 43, 47 (2013);

Taylor v. Sec’y of Health & Human Servs., 108 Fed. Cl. 807, 817 (2013). The arbitrary

and capricious standard is “well understood to be the most deferential possible.” Munn

v. Sec’y of Dep’t of Health & Human Servs., 970 F.2d at 870.

Therefore, this court may set aside a Special Master’s decision only if the court

determines that the “findings of fact or conclusion of law of the special master . . . [are]

arbitrary, capricious, an abuse of discretion, or otherwise not in accordance with

law . . . .” 42 U.S.C. § 300aa-12(e)(2)(B); see also Lombardi v. Sec’y of Health &

Human Servs., 656 F.3d at 1350 (“We uphold the special master's findings of fact

unless they are arbitrary or capricious.”) (internal citations omitted); Moberly ex rel.

Moberly v. Sec’y of Health & Human Servs., 592 F.3d at 1321; Markovich v. Sec’y of

Health & Human Servs., 477 F.3d at 1356-57; Lampe v. Sec’y of Health & Human

Servs., 219 F.3d at 1360. The United States Court of Appeals for the Federal Circuit

has indicated that:

These standards vary in application as well as degree of deference. Each

standard applies to a different aspect of the judgment. Fact findings are

reviewed by us, as by the Claims Court judge, under the arbitrary and

capricious standard; legal questions under the “not in accordance with

law” standard . . . ; and discretionary rulings under the abuse of discretion

standard. The latter will rarely come into play except where the special

master excludes evidence.

Munn v. Sec’y of Dep’t of Health & Human Servs., 970 F.2d at 871 n.10; see also

Deribeaux ex rel. Deribeaux v. Sec’y of Health & Human Servs., 717 F.3d at 1366; W.C.

v. Sec’y of Health & Human Servs., 704 F.3d at 1355; Griglock v. Sec’y of Health &

Human Servs., 687 F.3d 1371, 1374 (Fed. Cir. 2012); Porter v. Sec'y of Health &

Human Servs., 663 F.3d at 1249 (citing Broekelschen v. Sec'y of Health & Human

Servs., 618 F.3d at 1345) (explaining that the reviewing court does “reweigh the factual

evidence, assess whether the special master correctly evaluated the evidence, or

examine the probative value of the evidence or the credibility of the witnesses—these

are all matters within the purview of the fact finder”); Dodd v. Sec’y of Health & Human

Servs., 114 Fed. Cl. at 47.

“With regard to both fact-findings and fact-based conclusions, the key decision

maker in the first instance is the special master. The Claims Court owes these findings

and conclusions by the special master great deference – no change may be made

absent first a determination that the special master was ‘arbitrary and capricious.’”

36

Munn v. Sec’y of Dep’t of Health & Human Servs., 970 F.2d at 870; see also 42 U.S.C.

§ 300aa-12(e)(2)(B). Generally, “if the special master ‘has considered the relevant

evidence of record, drawn plausible inferences and articulated a rational basis for the

decision, reversible error will be extremely difficult to demonstrate.’” Hibbard v. Sec'y of

Health & Human Servs., 698 F.3d at 1363 (quoting Hines on Behalf of Sevier v. Sec’y of

Dep’t of Health & Human Servs., 940 F.2d 1518, 1528 (Fed. Cir. 1991)); see also Porter

v. Sec'y of Health & Human Servs., 663 F.3d at 1253-54; Lampe v. Sec’y of Health &

Human Servs., 219 F.3d at 1360; Avila ex rel. Avila v. Sec’y of Health & Human Servs.,

90 Fed. Cl. 590, 594 (2009); Dixon v. Sec’y of Dep’t of Health & Human Servs., 61 Fed.

Cl. 1, 8 (2004) (“The court’s inquiry in this regard must therefore focus on whether the

Special Master examined the ‘relevant data’ and articulated a ‘satisfactory explanation

for its action including a “rational connection between the facts found and the choice

made.”’” (quoting Motor Vehicle Mfrs. Ass’n v. State Farm Mut. Auto. Ins. Co., 463 U.S.

29, 43 (1983) (quoting Burlington Truck Lines, Inc. v. United States, 371 U.S. 156, 168

(1962)))).

As noted by the United States Court of Appeals for the Federal Circuit:

“Congress assigned to a group of specialists, the Special Masters within

the Court of Federal Claims, the unenviable job of sorting through these

painful cases and, based upon their accumulated expertise in the field,

judging the merits of the individual claims. The statute makes clear that,

on review, the Court of Federal Claims is not to second guess the Special

Masters [sic] fact-intensive conclusions; the standard of review is uniquely

deferential for what is essentially a judicial process. Our cases make clear

that, on our review . . . we remain equally deferential. That level of

deference is especially apt in a case in which the medical evidence of

causation is in dispute.”

Deribeaux ex rel. Deribeaux v. Sec'y of Health & Human Servs., 717 F.3d at 1366

(quoting Hodges v. Sec'y of Dept. of Health & Human Servs., 9 F.3d at 961)

(modification in original); Hibbard v. Sec'y of Health & Human Servs., 698 F.3d at 1363;

Locane v. Sec'y of Health & Human Servs., 685 F.3d 1375, 1380 (Fed. Cir. 2012). The

United States Court of Appeals for the Federal Circuit has further explained that the

reviewing courts “‘do not sit to reweigh the evidence. [If] the special master's conclusion

[is] based on evidence in the record that [is] not wholly implausible, we are compelled to

uphold that finding as not being arbitrary and capricious.’” See Deribeaux ex rel.

Deribeaux v. Sec'y of Health & Human Servs., 717 F.3d at 1367 (quoting Lampe v.

Sec'y of Health & Human Servs., 219 F.3d at 1363) (modification in original); see also

Hibbard v. Sec'y of Health & Human Servs., 698 F.3d at 1363 (citing Cedillo v. Sec’y of

Health & Human Servs., 617 F.3d 1328, 1338 (Fed. Cir. 2010). “‘Clearly it is not then

the role of this court to reweigh the factual evidence, or to assess whether the special

master correctly evaluated the evidence. And of course we do not examine the

probative value of the evidence or the credibility of the witnesses. These are all matters

within the purview of the fact finder.’” Dodd v. Sec’y of Health & Human Servs., 114

Fed. Cl. at 56 (quoting Munn v. Sec'y of Dept. of Health & Human Servs., 970 F.2d at

37

870 n.10); see also Paluck v. Sec'y of Health & Human Servs., 113 Fed. Cl. 210, 224

(2013) (“A special master's findings regarding the probative value of the evidence and

the credibility of witnesses will not be disturbed so long as they are ‘supported by

substantial evidence.’” (quoting Doe v. Sec'y of Health & Human Servs., 601 F.3d

1349, 1355 (Fed. Cir.), cert. denied, 131 S. Ct. 573 (2010))). Additionally, as instructed

by the United States Court of Appeals for the Federal Circuit, “[u]nder the Vaccine Act,

Special Masters are accorded great deference in determining the credibility and

reliability of expert witnesses. Indeed, we have held that a Special Master's ‘credibility

determinations are virtually unreviewable.’” Cedillo v. Sec’y of Health & Human Servs.,

617 F.3d at 1347 (quoting Hanlon v. Sec’y of Health & Human Servs., 191 F.3d 1344,

1349 (Fed. Cir. 2010) (quotation omitted)).

Additionally, a Special Master is “not required to discuss every piece of evidence

or testimony in [his or] her decision.” Snyder ex rel. Snyder v. Sec’y of Health & Human

Servs., 88 Fed. Cl. 706, 728 (2009); see also Paluck ex rel. Paluck v. Sec’y of Health &

Human Servs., 104 Fed. Cl. 457, 467 (2012) (“[W]hile the special master need not

address every snippet of evidence adduced in the case, see id. [Doe v. Sec’y of Health

& Human Servs., 601 F.3d at 1355], he cannot dismiss so much contrary evidence that

it appears that he ‘simply failed to consider genuinely the evidentiary record before

him.’” (quoting Campbell v. Sec’y of Health & Human Servs., 97 Fed. Cl. 650, 668

(2011))).

Regarding the causation analysis, as indicated by the United States Court of

Appeals for the Federal Circuit in Althen v. Secretary of Health and Human Services:

The [Vaccine] Act provides for the establishment of causation in one of

two ways: through a statutorily-prescribed presumption of causation upon

a showing that the injury falls under the Vaccine Injury Table (“Table

injury”), see 42 U.S.C. § 300aa-14(a); or where the complained-of injury is

not listed in the Vaccine Injury Table (“off-Table injury”), by proving

causation in fact, see 42 U.S.C. §§ 300aa-13(a)(1), -11(c)(1)(C)(ii)(I).

Althen v. Sec’y of Health & Human Servs., 418 F.3d at 1278; W.C. v. Sec'y of Health &

Human Servs., 704 F.3d at 1356; Broekelschen v. Sec'y of Health & Human Servs., 618

F.3d at 1346; Pafford v. Sec’y of Health & Human Servs., 451 F.3d 1352, 1356 (Fed.

Cir.), reh’g and reh’g en banc denied (Fed. Cir. 2006), cert. denied, 551 U.S. 1102

(2007); Dodd v. Sec’y of Health & Human Servs., 114 Fed. Cl. at 50; Paluck v. Sec'y of

Health & Human Servs., 113 Fed. Cl. at 212; Fesanco v. Sec’y of Health & Human

Servs., 99 Fed. Cl. 28, 31 (2011). The United States Supreme Court has explained

that:

Claimants who show that a listed injury first manifested itself at the

appropriate time are prima facie entitled to compensation. No showing of

causation is necessary; the Secretary bears the burden of disproving

causation. A claimant may also recover for unlisted side effects, and for

38

listed side effects that occur at times other than those specified in the

Table, but for those the claimant must prove causation.

Bruesewitz v. Wyeth LLC, 131 S. Ct. 1068, 1073-74 (2011) (footnotes omitted);

Kennedy v. Sec’y of Health & Human Servs., 99 Fed. Cl. 535, 539 (2011), aff’d, 485 F.

App’x. 435 (Fed. Cir. 2012).

Petitioners state that, as a result of the vaccinations [O.A.S.] received on January

26, 2001, “[O.A.S.] suffered both from GBS and CIDP. In this regard, as the special

master is well aware, GBS and CIDP are the acute and chronic forms of the same

injury.” GBS and CIDP are not listed as injuries on the Vaccine Table. See 42 U.S.C. §

300aa-14. Under the off-Table theory of recovery, a Petitioner is entitled to

compensation if he or she can demonstrate, by a preponderance of the evidence, see

42 U.S.C. § 300aa-13(a)(1)(A), that the recipient of the vaccine sustained, or had

significantly aggravated, an illness, disability, injury, or condition not set forth in the

Vaccine Injury Table, but which was caused by a vaccine that is listed on the Vaccine

Injury Table. See 42 U.S.C. § 300aa-11(c)(1)(C)(ii)(I); see also W.C. v. Sec’y of Health

& Human Servs., 704 F.3d at 1356 (“Nonetheless, the petitioner must do more than

demonstrate a ‘plausible’ or ‘possible’ causal link between the vaccination and the

injury; he must prove his case by a preponderance of the evidence.” (quoting Moberly

ex rel. Moberly v. Sec’y of Health & Human Servs., 592 F.3d at 1322)); Althen v. Sec’y

of Health & Human Servs., 418 F.3d at 1278; Hines on Behalf of Sevier v. Sec’y of

Dep’t of Health & Human Servs., 940 F.2d at 1525.

Since [O.A.S.]’s conditions do not meet the requirements of a presumptively on-

Table, vaccine-related condition, to prove entitlement for an off-Table injury, Petitioners

must

prove causation-in-fact. Grant [v. Sec'y of Health & Human Servs.], 956

F.2d [1144,] 1147-48 [(Fed. Cir. 1992)]. [The United States Court of

Appeals for the Federal Circuit has] held that causation-in-fact in the

Vaccine Act context is the same as the “legal cause” in the general torts

context. Shyface v. Sec’y of Health and Human Servs., 165 F.3d 1344,

1352 (Fed. Cir. 1999). Therefore, drawing from the Restatement (Second)

of Torts, the vaccine is a cause-in-fact when it is “a substantial factor in

bringing about the harm.”

de Bazan v. Sec’y of Health & Human Servs., 539 F.3d at 1351 (quoting the

Restatement (Second) of Torts § 431(a)); see also Deribeaux ex rel. Deribeaux v. Sec’y

of Health & Human Servs., 717 F.3d at 1367 (“To prove causation, a petitioner must

show that the vaccine was ‘not only a but-for cause of the injury but also a substantial

factor in bringing about the injury.’” (quoting Shyface v. Sec’y of Health & Human

Servs., 165 F.3d at 1352–53)). A “‘substantial factor’ standard requires a greater

showing than ‘but for’ causation.” de Bazan v. Sec’y of Health & Human Servs., 539

F.3d at 1351 (quoting Shyface v. Sec’y of Health & Human Servs., 165 F.3d at 1352).

“However, the petitioner need not show that the vaccine was the sole or predominant

39

cause of her injury, just that it was a substantial factor.” Id. (citing Walther v. Sec'y of

Health & Human Servs., 485 F.3d 1146, 1150 (Fed. Cir. 2007)). A judge of this court

has explained the relationship between “but-for” causation and “substantial factor”

causation in our court’s decision in Deribeaux ex rel. Deribeaux v. Secretary of Health

and Human Services:

The de Bazan court defined but-for causation as requiring that “the harm

be attributable to the vaccine to some nonnegligible degree,” and noted

that, although substantial is somewhere beyond the low threshold of but-

for causation, it does not mean that a certain factor must be found to have

definitively caused the injury. Id. [de Bazan v. Sec’y of Health & Human

Servs., 539 F.3d at 1351] Accordingly, a factor deemed to be substantial

is one that falls somewhere between causing the injury to a non-negligible

degree and being the “sole or predominant cause.” Id.

This definition of substantial—somewhere between non-negligible and

predominant—is applicable to respondent's burden to prove a sole

substantial factor unrelated to the vaccine. Accordingly, a respondent's

burden is to prove that a certain factor is the only substantial factor—one

somewhere between non-negligible and predominant—that caused the

injury.

Deribeaux ex rel. Deribeaux v. Sec’y of Health & Human Servs., 105 Fed. Cl. 583, 595

(2012), aff’d, 717 F.3d 1363 (Fed. Cir.), reh’g and reh’g en banc denied (Fed. Cir. 2013)

(emphasis in original).

The Petitioners must prove their case by a preponderance of the evidence. See

42 U.S.C. § 300aa-13(a)(1)(A). According to the United States Court of Appeals for the

Federal Circuit, the preponderance of evidence standard is “one of proof by a simple

preponderance, of ‘more probable than not causation.’” Althen v. Sec’y of Health &

Human Servs., 418 F.3d at 1279-80 (citing concurrence in Hellebrand v. Sec’y of Dep’t

of Health & Human Servs., 999 F.2d 1565, 1572-73 (Fed. Cir. 1993)); see also W.C. v.

Sec’y of Health & Human Servs., 704 F.3d at 1356 (“In this off-table case, the petitioner

must show that it is ‘more probable than not’ that the vaccine caused the injury.”

(quoting Althen v. Sec’y of Health & Human Servs., 418 F.3d at 1279-80)). Decisions of

the Federal Circuit permit the use of circumstantial evidence, which the court described

as “envisioned by the preponderance standard” and by the vaccine system created by

Congress, in which “close calls regarding causation are resolved in favor of injured

claimants” without the need for medical certainty. See Althen v. Sec’y of Health &

Human Servs., 418 F.3d at 1280; see also Cloer v. Sec’y of Health & Human Servs.,

654 F.3d 1322, 1332 n.4 (Fed. Cir. 2011), cert. denied, 132 S. Ct. 1908 (2012); Andreu

ex rel. Andreu v. Sec’y of Dept. of Health & Human Servs., 569 F.3d 1367, 1379 (Fed.

Cir. 2009) (“In Althen, however, we expressly rejected the Stevens test, concluding that

requiring ‘objective confirmation’ in the medical literature prevents ‘the use of

circumstantial evidence . . . and negates the system created by Congress’ through the

Vaccine Act.”) (modification in original); La Londe v. Sec’y of Health & Human Servs.,

40

110 Fed. Cl. 184, 198 (2013) (“Causation-in-fact can be established with circumstantial

evidence, i.e., medical records or medical opinion.”). The Althen court further noted that

“the purpose of the Vaccine Act’s preponderance standard is to allow the finding of

causation in a field bereft of complete and direct proof of how vaccines affect the human

body.” Althen v. Sec’y of Health & Human Servs., 418 F.3d at 1280 (citing Knudsen by

Knudsen v. Sec’y of Dep’t of Health & Human Servs., 35 F.3d 543, 549 (Fed. Cir.

1994)); see also W.C. v. Sec’y of Health & Human Servs., 704 F.3d at 1356. When

proving eligibility for compensation for an off-Table injury under the Vaccine Act,

however, Petitioner may not rely on her testimony alone. According to the Vaccine Act,

“[t]he special master or court may not make such a finding based on the claims of a

petitioner alone, unsubstantiated by medical records or by medical opinion.” See 42

U.S.C. § 300aa-13(a)(1).

The Federal Circuit in Althen defined a three-prong test which a Petitioner must

meet to establish causation in an off-Table injury case:

To meet the preponderance standard, [Petitioner] must “show a medical

theory causally connecting the vaccination and the injury.” Grant v. Sec'y

of Health & Humans Servs., 956 F.2d 1144, 1148 (Fed. Cir. 1992)

(citations omitted). A persuasive medical theory is demonstrated by “proof

of a logical sequence of cause and effect showing that the vaccination

was the reason for the injury[,]” the logical sequence being supported by

“reputable medical or scientific explanation[,]” i.e., “evidence in the form of

scientific studies or expert medical testimony[.]” Grant, 956 F.2d at 1148.

[Petitioner] may recover if she shows “that the vaccine was not only a but-

for cause of the injury but also a substantial factor in bringing about the

injury.” Shyface [v. Sec'y of the Dep't of Health & Human Servs.], 165 F.3d

at 1352-53. Although probative, neither a mere showing of a proximate

temporal relationship between vaccination and injury, nor a simplistic

elimination of other potential causes of the injury suffices, without more, to

meet the burden of showing actual causation. See Grant, 956 F.2d at

1149. Concisely stated, [Petitioner’s] burden is to show by preponderant

evidence that the vaccination brought about [the] injury by providing: (1) a

medical theory causally connecting the vaccination and the injury; (2) a

logical sequence of cause and effect showing that the vaccination was the

reason for the injury; and (3) a showing of a proximate temporal

relationship between vaccination and injury.

Althen v. Sec’y of Health & Human Servs., 418 F.3d at 1278 (brackets in original); see

also Deribeaux ex rel. Deribeaux v. Sec’y of Health & Human Servs., 717 F.3d at 1367;

Porter v. Sec’y of Health & Human Servs., 663 F.3d at 1249; Moberly ex rel. Moberly v.

Sec’y of Health & Human Servs., 592 F.3d at 1322; Pafford v. Sec’y of Health & Human

Servs., 451 F.3d at 1355; Capizzano v. Sec’y of Health & Human Servs., 440 F.3d

1317, 1324 (Fed. Cir. 2006); C.K. v. Sec’y of Health & Human Servs., 113 Fed. Cl. 757,

766 (2013); Contreras v. Sec’y of Health & Human Servs., 107 Fed. Cl. 280, 291

(2012).

41

With regard to the first Althen prong, “a medical theory causally connecting the

vaccination and the injury,” Althen v. Sec’y of Health & Human Servs., 418 F.3d at

1278, the Althen court analyzed the preponderance of evidence requirement as allowing

medical opinion as proof, even without scientific studies in medical literature that

provide “objective confirmation” of medical plausibility. Id. at 1278-80; see also Shapiro

v. Sec’y of Health & Human Servs., 105 Fed. Cl. at 358. In rejecting a requirement that

a claimant under the Vaccine Act prove confirmation of medical plausibility from the

medical community and medical literature, the Althen court turned to the analysis

undertaken in Knudsen by Knudsen v. Secretary of the Department of Health and

Human Services, 35 F.3d at 549. See Althen v. Sec’y of Health & Human Servs., 418

F.3d at 1279-80. In Knudsen by Knudsen v. Secretary of the Department of Health and

Human Services, the United States Court of Appeals for the Federal Circuit wrote, “to

require identification and proof of specific biological mechanisms would be inconsistent

with the purpose and nature of the vaccine compensation program. The Vaccine Act

does not contemplate full blown tort litigation in the Court of Federal Claims.” Knudsen

by Knudsen v. Sec’y of Dep’t of Health & Human Servs., 35 F.3d at 549. Further,

[t]he Court of Federal Claims is therefore not to be seen as a vehicle for

ascertaining precisely how and why DTP and other vaccines sometimes

destroy the health and lives of certain children while safely immunizing

most others. This research is for scientists, engineers, and doctors

working in hospitals, laboratories, medical institutes, pharmaceutical

companies, and government agencies. The special masters are not

“diagnosing” vaccine-related injuries. The sole issues for the special

master are, based on the record evidence as a whole and the totality of

the case, whether it has been shown by a preponderance of the evidence

that a vaccine caused the [Petitioner’s] injury or that the [Petitioner’s]

injury is a table injury, and whether it has not been shown by a

preponderance of the evidence that a factor unrelated to the vaccine

caused the child's injury. See 42 U.S.C. § 300aa-13(a)(1), (b)(1).

Id. (brackets added). The Federal Circuit recently indicated in this very case on appeal

form the Special Master’s earlier dismissal of the case:

Although a finding of causation “must be supported by a sound and

reliable medical or scientific explanation,” causation “can be found in

vaccine cases . . . without detailed medical and scientific exposition on the

biological mechanisms.” Knudsen v. Sec'y of the Dep't of Health &

Human Servs., 35 F.3d 543, 548–49 (Fed. Cir. 1994). It is not necessary

for a petitioner to point to conclusive evidence in the medical literature

linking a vaccine to the petitioner's injury, as long as the petitioner can

show by a preponderance of the evidence that there is a causal

relationship between the vaccine and the injury, whatever the details of

the mechanism may be.

42

Simanski v. Sec'y of Health & Human Servs., 671 F.3d at 1384 (omission in original).

The second prong of the Althen test requires the Petitioner to demonstrate “a

logical sequence of cause and effect, showing that the vaccination was the reason for

the injury” by a preponderance of the evidence. Althen v. Sec'y of Health & Human

Servs., 418 F.3d at 1278; see also Deribeaux ex rel. Deribeaux v. Sec’y of Health &

Human Servs., 717 F.3d at 1367; Pafford v. Sec’y of Health & Human Servs., 451 F.3d

at 1355. In order to prevail, the Petitioner must show “that the vaccine was not only a

but-for cause of the injury but also a substantial factor in bringing about the injury.”

Althen v. Sec'y of Health & Human Servs., 418 F.3d at 1278 (quoting Shyface v. Sec’y

of Health & Human Servs., 165 F.3d at 1352). In Capizzano v. Secretary of Health and

Human Services, 440 F.3d at 1326, the Federal Circuit stated, “‘[a] logical sequence of

cause and effect’ means what it sounds like – the claimant's theory of cause and effect

must be logical. Congress required that, to recover under the Vaccine Act, a claimant

must prove by a preponderance of the evidence that the vaccine caused his or her

injury.” Capizzano v. Sec'y of Health & Human Servs., 440 F.3d at 1326 (quoting 42

U.S.C. §§ 300aa-11(c)(1)–13(a)(1)).

The third prong of the Althen test requires the Petitioner to demonstrate, by a

preponderance of evidence, “a proximate temporal relationship between vaccination

and injury.” Althen v. Sec'y of Health & Human Servs., 418 F.3d at 1278; Deribeaux ex

rel. Deribeaux v. Sec’y of Health & Human Servs., 717 F.3d at 1367. The United States

Court of Appeals for the Federal Circuit emphasized the importance of a temporal

relationship in Pafford v. Secretary of Health and Human Services, when it noted that,

“without some evidence of temporal linkage, the vaccination might receive blame for

events that occur weeks, months, or years outside of the time in which scientific or

epidemiological evidence would expect an onset of harm.” Pafford v. Sec'y of Health &

Human Servs., 451 F.3d at 1358; see also de Bazan v. Sec'y of Health & Human

Servs., 539 F.3d at 1352 (“Thus, the proximate temporal relationship prong requires

preponderant proof that the onset of symptoms occurred within a timeframe for which,

given the medical understanding of the disorder's etiology, it is medically acceptable to

infer causation-in-fact.” (citing Pafford v. Sec'y of Health & Human Servs., 451 F.3d at

1358)). Requiring evidence of strong temporal linkage is consistent with the third

requirement articulated in Althen because “[e]vidence demonstrating petitioner’s injury

occurred within a medically acceptable time frame bolsters a link between the injury

alleged and the vaccination at issue under the ‘but-for’ prong of the causation analysis.”

Pafford v. Sec'y of Health & Human Servs., 451 F.3d at 1358 (citing Capizzano v. Sec’y

of Health & Human Servs., 440 F.3d at 1326). The Pafford court further explained:

If, for example, symptoms normally first occur ten days after inoculation

but petitioner's symptoms first occur several weeks after inoculation, then

it is doubtful the vaccination is to blame. In contrast, if symptoms normally

first occur ten days after inoculation and petitioner's symptoms do, in fact,

occur within this period, then the likelihood increases that the vaccination

is at least a factor. Strong temporal evidence is even more important in

cases involving contemporaneous events other than the vaccination,

43

because the presence of multiple potential causative agents makes it

difficult to attribute "but-for" causation to the vaccination. After all, credible

medical expertise may postulate that any of the other contemporaneous

events may have been the sole cause of the injury.

Id.

According to the court in Capizzano v. Secretary of Health and Human Services,

evidence used to satisfy one of the Althen prongs may overlap with and be used to

satisfy another prong. See Capizzano v. Sec’y of Health & Human Servs., 440 F.3d at

1326 (“We see no reason why evidence used to satisfy one of the Althen III prongs

cannot overlap to satisfy another prong.”). If a Petitioner satisfies the Althen burden and

meets all three prongs of the test, the Petitioner prevails, “unless the [government]

shows, also by a preponderance of the evidence, that the injury was in fact caused by

factors unrelated to the vaccine.” Knudsen by Knudsen v. Sec'y of Dep’t of Health &

Human Servs., 35 F.3d at 547 (brackets in original; citation omitted).

When the nature of a Petitioner’s injury is in dispute, however, the Special Master

must first identify the injury before conducting an Althen causation analysis. See

Lombardi v. Sec’y of Health & Human Servs., 656 F.3d at 1353. In Lombardi, the

petitioner's experts offered different theories as to her injury. One of Petitioner’s experts

asserted that Petitioner suffered from transverse myelitis, the other testified that she

suffered from either chronic fatigue syndrome or systemic lupus erythematosus. The

government's experts disputed those diagnoses, and proposed five other possible

diagnoses. See id. at 1352. The United States Court of Appeals for the Federal Circuit

stated that:

In the face of such extreme disagreement among well-qualified medical

experts, each of whom had evaluated the petitioner, it was appropriate for

the special master to first determine what injury, if any, was supported by

the evidence presented in the record before applying the Althen test to

determine causation.

Lombardi v. Sec’y of Health & Human Servs., 656 F.3d at 1352–53 (citing Broekelschen

v. Sec’y of Health & Human Servs., 618 F.3d at 1346). The Lombardi court continued:

“In the absence of a showing of the very existence of any specific injury of which the

petitioner complains, the question of causation is not reached.” Id. at 1353. “If a special

master can determine that a petitioner did not suffer the injury that she claims was

caused by the vaccine, there is no reason why the special master should be required to

undertake and answer the separate (and frequently more difficult) question whether

there is a medical theory, supported by ‘reputable medical or scientific explanation,’ by

which a vaccine can cause the kind of injury that the petitioner claims to have suffered.”

Hibbard v. Sec’y of Health & Human Servs., 698 F.3d at 1365 (quoting Althen v. Sec’y

of Health & Human Servs., 418 F.3d at 1278); see also Locane v. Sec’y of Health &

Human Servs., 685 F.3d at 1381 (“Given the Special Master's finding that the illness

was present before the vaccine was administered, logically, the vaccine could not have

44

caused the illness. The Althen inquiry is inapplicable.”) (footnote omitted); Vinconti v.

Sec’y of Health & Human Servs., 103 Fed. Cl. 600, 612 (2012); Carrino v. Sec’y of

Health & Human Servs., No. 08-0266V, 2013 WL 3328903, at *11 (Fed. Cl. Spec. Mstr.

June 6, 2013); cf. Contreras v. Sec’y of Health & Human Servs., 107 Fed. Cl. at 293

(Stating that “in an atypical case, where ‘the question of causation turns on which injury

[the Petitioner] suffered,’ the special master is permitted to choose between two

competing diagnoses of dissimilar diseases as a first step in the causation analysis.”

The United States Court of Federal Claims noted that this is an “exception” to “the

general rule, first stated in Knudsen [by Knudsen v. Sec’y of Dep’t of Health & Human

Servs., 35 F.3d at 549], that special masters should not diagnose alleged vaccine

injuries.”). In the above-captioned case, during the November 18, 2013 oral argument

held in in this court, the Petitioners’ counsel agreed that, “there was no need to explore

in detail . . . whether the vaccines could have adversely affected [O.A.S.]’s SMARD via

the Althen test.” Instead, Petitioners’ counsel agreed that “we’re talking only about, for

the purposes of this review, whether or not the SMARD conclusion by Special Master

Moran is the correct one.”

A Petitioner’s burden of proof regarding the sustained injury is a preponderance

of the evidence. See 42 U.S.C. § 300aa-13(a)(1); see also Lombardi v. Sec’y of Health

& Human Servs., 656 F.3d at 1353 (noting that the Vaccine statute “places the burden

on the petitioner to make a showing of at least one defined and recognized injury”);

Broekelschen v. Sec’y of Health & Human Servs., 618 F.3d at 1350. If a Petitioner can

show by a preponderance that he or she, in fact, suffered the injury claimed, he or she

can continue to the question of causation, unless the government can demonstrate, also

by preponderance of the evidence, an “alternative evidence on injury.” See

Broekelschen v. Sec’y of Health & Human Servs., 618 F.3d at 1350. The record in the

current case includes differing evidence, as presented by the respective experts and

[O.A.S.]’s medical history, with regards to the nature of [O.A.S.]'s illness. The parties

presented alternative diagnoses as to the Petitioners’ disease based on the record, and

their respective experts also disagreed as to the many of [O.A.S.]’s symptoms and the

disease she presented. See Lombardi v. Sec’y of Health & Human Servs., 656 F.3d at

1352–53. Therefore, the Special Master had to first determine what diagnosis applied

to [O.A.S.]’s illness, before conducting a causation analysis under the Althen test. See

Althen v. Sec’y of Health & Human Servs., 418 F.3d at 1278.

As discussed below, the Special Master rejected Petitioners’ allegation in their

Petition that [O.A.S.] suffered from GBS/CIDP, and concluded that each of the twelve

data points he considered in the discussion section of his decision supported a

diagnosis of SMARD. 49 In addition, on the Special Master’s chart, summarizing the ten

data points he included in his decision, the Special Master indicated that six data points

are “inconsistent” with GBS/CIDP and “consistent” with SMARD,” namely, [O.A.S.]’s age

at onset of her disease, her reflex and her weakness pattern, the permanent ventilator

support, the sural nerve biopsy showing no demyelination, her normal CSF protein

49

The Special Master’s conclusion on each data point in his discussion section ranged

from “consistent,” “slightly favors,” “tends to favor,” to “modest support for SMARD.”

45

levels and the EMG and nerve conduction studies. [O.A.S.]’s response to the IVIG

treatment was considered “inconsistent” with GBS/CIDP and “neutral” for SMARD. The

Special Master stated that [O.A.S.]’s age of onset was “extremely rare” for GBS/CIDP,

and that sural nerve biopsy showing no demyelination, and the EMG and nerve

conduction studies showing axonal damage, not demyelination, were “strongly”

inconsistent with GBS/CIDP. Regarding two other data points reflected on his summary

chart included in his decision, review of [O.A.S.]’s IUGR record and her creatinine

levels, the Special Master used the term “neutral” for GBS/CIDP. Only one data point

included on his summary chart, although not considered in the body of his decision as a

separate data point, 50 [O.A.S.]’s health before her respiratory arrest, was considered by

the Special Master as potentially consistent with GBS/CIDP.

In his decision, the Special Master described the etiology of the GBS and CIDP.

He noted that the Asbury criteria, as described in Asbury, et al., Assessment of Current

Diagnostic Criteria for Guillain-Barré Syndrome, 27 Ann Neurol 21 (1990), “establish the

symptoms frequently used to diagnose GBS,” and indicated that they include “elevated

protein in the spinal fluid, response to IVIG treatment,” and “‘reduced or absent

reflexes.” 51 The Special Master did not discuss other diagnostic criteria for GBS. 52

50

As discussed above, the court notes a discrepancy between the Special Master’s

discussion section of his decision and the data point summary chart he included in his

decision. See, generally, Simanski v. Sec’y of Health & Human Servs., 2013 WL

7017568, at *41. The Special Master did not discuss [O.A.S.]’s health before her

respiratory arrest as one of his discussion data points, although he included it in the

summary chart. He also indicated acute respiratory failure was a presenting sign as a

second discussion data point, but that also was not reflected on his chart. The opinions

of the treating physicians, his eleventh discussion data point, and the comparison of the

experts, his twelfth discussion data point, also were not reflected in the summary chart.

51

The Special Master noted how the Respondent’s expert, Dr. Finkel, had testified that

the “‘reflexes are typically lost early in GBS/CIDP. That’s a hallmark. That’s one of the

two main criteria of GBS.’” Id. at *6. The Special Master further quoted the testimony of

the Petitioners’ expert, Dr. Maertens, who stated, “‘[i]n most cases, the reflex[es] are

decreased or lost.’” Id.

52

In a recent decision, Carrino v. Secretary of Health and Human Services, 2013 WL

3328903, a Special Master noted that a “diagnostic guidance” offered by Francine

Vriesendorp, Clinical Features and Diagnosis of Guillain–Barré Syndrome in Adults,

“was available and relevant in October 2006.” Id. at *14 n.29. Moreover, Dr. Maertens

attached to his December 28, 2012 supplemental expert report an article listing the

current diagnostic criteria for CIDP. See European Federation of Neurological

Societies/Peripheral Nerve Society Guideline on Management of Chronic

Inflammatory Demyelinating Polyradiculoneuropathy. Report of a Joint Task Force of

the European Federation of Neurological Societies and the Peripheral Nerve Society,

10 J. Peripher Nerv Syst 220 (2005). Special Master Moran also cites the same article

when discussing whether a sural nerve biopsy had any role in the diagnosis of GBS,

CIDP or SMARD. See Simanski v. Sec’y of Health & Human Servs., 2013 WL 7017568,

46

The court notes that the Special Master’s decision quoted the definition of GBS

from Dorland’s Illustrated Medical Dictionary at 1832: “GBS begins with ‘paresthesias of

the feet,’” but the Special Master did not discuss how [O.A.S.] presented on the issue.

The Special Master, however, quoted Dr. Maertens, who testified that it is “‘extremely

difficult to make a diagnosis of GBS in a very young infant . . . because the child doesn’t

walk, doesn’t sit up, and it’s a lot easier to miss. It’s going to be the hardest thing to

diagnose.’” Simanski v. Sec’y of Health & Human Servs., 2013 WL 7017568, at *6. A

recent decision by a Special Master which considered the diagnostic criteria for GBS,

Carrino v. Secretary of Health and Human Services, 2013 WL 3328903, described the

presentation of GBS as also including “Lost or significantly diminished deep tendon

reflexes;” “Progressive weakness;” “Paresthesia;” “Symmetric symptom presentation;”

“Elevated protein levels in the cerebrospinal fluid;” and “EMG abnormalities.” Id. at *14-

17. In his decision, Special Master Moran discussed [O.A.S.]’s reflex patterns,

[O.A.S.]’s tests indicating low protein level in the cerebrospinal fluid, and her EMG test

results, finding that [O.A.S.]’s condition was inconsistent with GBS. The Special Master,

however, did not discuss whether [O.A.S.] had paresthesia, whether there was a noted

progression of [O.A.S.]’s weakness, and whether there was a symmetric presentation of

her symptoms. The Special Master mentioned, in passing, that [O.A.S.]’s subsequent

EMG tests, conducted after her initial EMG on February 26, 2001, indicated progress in

her neuropathy, but he did not consider the progression in [O.A.S.]’s condition as one of

the hallmarks for GBS. Finally, the Special Master did not include in his decision any

discussion with respect to a symmetric presentation of [O.A.S.]’s symptoms.

SMARD is a rare and somewhat recently identified disease. As discussed

above, Dr. Finkel and Special Master Moran indicated that neurologists became aware

of SMARD in 2003, after the publication of an article in the Annals of Neurology. See

Grohmann, et al., Infantile Spinal Muscular Atrophy with Respiratory Distress Type 1

(SMARD1), 54 Ann. Neurol. 719 (2003). Moreover, there are no cases identified in the

opinions issued by this court or in any of the decisions by the Office of the Special

Masters, which specifically consider a SMARD diagnosis. 53 It appears that when

at *16. The clinical diagnostic criteria for typical CIDP included as an exhibit by the

Petitioners were described as similar to those for GBS: “Chronically progressive,

stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction

of all extremities, developing over at least 2 months; cranial nerves may be affected;

and [a]bsent or reduced tendon reflexes in all extremities.”

53

The court notes that in a published order to show cause issued by another Special

Master in Sharkey v. Secretary of Health and Human Services, No. 99-669V, 2007 WL

5185477 (Fed. Cl. Spec. Mstr. Aug. 23, 2007), the Special Master mentioned that the

Sharkey child had undergone genetic testing for spinal muscular atrophy (SMA), which

made a diagnosis of SMA less likely. Id. at *5. (noting that since the “test found Ryan

that has at least one intact SMN gene,” he “could be among the 2% of children with

SMA that do not have deletions of both SMN genes”). The child in Sharkey also had

suffered a respiratory failure, but the Special Master did not indicate in the order to

show cause a possible diagnosis of spinal muscular atrophy with respiratory distress

47

Special Master Moran concluded that [O.A.S.] suffers from SMARD, he relied heavily on

the testimony and expert reports provided by Dr. Finkel. In his September 28, 2012

expert report, Dr. Finkel had provided a chart, which summarized his interpretation of

the data points, which the Special Master ultimately included in the discussion section of

his decision. Dr. Finkel’s “[d]ata items” included: “Intrauterine Growth Retardation

(IUGR)”; “[r]espiratory failure – acute onset, as the presenting symptom, with early right

hemi-diaphragm eventration”; “[r]espiratory failure presenting at age 2 months, 3

weeks”; “[p]rogression to permanent ventilation support within one month of onset of

respiratory failure”; “[w]eakness in limbs, trunk, neck muscles – onset within days to few

weeks of respiratory failure (RF)”; “[w]eakness: distribution of lower>upper limbs,

distal>proximal, symmetric”; “[n]erve conduction and EMG findings – length-dependent

axonal sensorimotor polyneuropathy, without focal or segmental changes. (May have

slowing of conduction initially that raises the consideration of GBS or CIDP)”; “[s]ural

nerve biopsy”; “[c]erebrospinal fluid (CSF) profile”; “[l]ow serum creatinine (0.1) upon

admission 1/20/00, suggestive of chronic muscle wasting (non-specific as to etiology)”;

“[s]low decline in motor function over years, following the initial acute deterioration”;

“[e]xcessive sweating (diaphoresis)”; “[n]o apparent therapeutic benefit from IVIg or

prednisolone Rx.” In his chart, Dr. Finkel interpreted [O.A.S.]’s medical records as

either highly consistent (HC), consistent (C), inconsistent (I), highly inconsistent (HI), or

neutral (N) with the three diseases at issue: SMARD, GBS and CIDP.

In Dr. Finkel’s chart, the following data items were ranked “HC,” i.e., highly

consistent with SMARD: IUGR; respiratory failure – acute onset, as the presenting

symptom; progression to permanent ventilator support within one month of onset of

respiratory failure; weakness in limbs, trunk, neck muscles; weakness: distribution of

lower>upper limbs, distal>proximal, symmetric; nerve conduction and EMG findings –

length-dependent axonal sensorimotor polyneuropathy, without focal or segmental

changes; cerebrospinal fluid (CSF) profile; slow decline in motor function over years;

and excessive sweating. In Dr. Finkel’s chart, the following data items were ranked “C,”

i.e., consistent with SMARD, “[s]ural nerve biopsy” and “[l]ow serum creatinine (0.1)

upon admission 1/20/00, suggestive of chronic muscle wasting (non-specific as to

etiology).” Additionally, in Dr. Finkel’s chart, the following data item was ranked “N,” i.e.,

neither support or inconsistent with SMARD, “[n]o apparent therapeutic benefit from IVIg

or prednisolone Rx.” The first four data items, and nerve conduction

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