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Applying Compassionate Allowances to Multiple Sclerosis Presenter John Booss, M.D., F.A.A.N.

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Presentation on theme: "Applying Compassionate Allowances to Multiple Sclerosis Presenter John Booss, M.D., F.A.A.N."— Presentation transcript:

1 Applying Compassionate Allowances to Multiple Sclerosis Presenter John Booss, M.D., F.A.A.N

2 Outline Basic MS background Causes Symptoms Diagnosis Types of MS Treatment New research and potential advances on the horizon Impact on activities of daily living Progression and impact on employment How we think MS fits with the CA process Challenges that we recognize Suggested way to approach MS in terms of Compassionate Allowances

3 What is MS? A chronic, inflammatory, degenerative, and generally disabling condition affecting the brain, optic nerves, and spinal cord Peak age of onset in the early 30’s; 70% have onset between 20 and 50; onset can occur at any age No pathognomonic sign, symptom, or test and so diagnosis is a result of accumulation of clinical and paraclinical evidence Cause is not known but thought to be autoimmune resulting from genetic predisposition and environmental trigger(s) More common in females, Caucasians, northern Europeans, and those living farther from equator but also involves other ethnic groups No cure or prevention at present but many therapies to alter disease course and treat symptoms

4 $721 million cumulative research investment (estimated through 2010) myelinated nerve fiber Myelin Nerve Fiber (Quarles RH et al, 2006)

5 Environmental Trigger(s) Autoimmunity Genetic Predisposition What causes MS? Loss of myelin & nerve fibers

6 $721 million cumulative research investment (estimated through 2010) MS interrupts the flow of information between the brain and the body Functional nerve cell Nerve cell with MS damage – may include transection of axon itself

7 MS histopathology – perivascular inflammation (Berger JR, 2008)

8 Axonal transection in the lesions of MS (Trapp, BD et al, 1998) Transected axon

9 Self-reported symptoms P<.01 (Minden, SL et al, 2004)

10 Diagnostic techniques Clinical signs and symptoms suggestive of CNS demyelination Magnetic resonance imaging of the brain (more often) and/or spinal cord (less often) Oligonclonal bands Evoked potentials (generally visual) Rule out other possibilities (Polman c, et al, in press)

11 $721 million cumulative research investment (estimated through 2010) 85% at onset10-15% 5-10% ~50% of RRMS develop SPMS ~19 yrs

12 Prognostic indicators Better Prognosis Few attacks early in course Long intervals between attacks Complete recovery from attacks Attacks that are mainly sensory Nearly normal neurological exam after 5 years Young when diagnosed Female Caucasian Worse Prognosis Frequent attacks early in course Incomplete recovery from attacks Early cerebellar and/or brainstem symptoms Gait impairment More lesions on MRI early in course Early development of abnormal neurological exam

13 The Expanded Disability Status Scale Illustrated (adapted from Kurtzke, J. 1983) Zone where disability is very likely

14 Therapies used in MS Corticosteroids Interferon-beta Copolymer Chemotherapeutic agents Monoclonal antibodies Symptomatic therapies Rehabilitation

15 Research and advances on the horizon Stop the Progression of MS Development of effective treatments for progressive forms of MS Development of biomarkers to track progression Restore Function Homing in on the most effective rehabilitation strategies Development of viable neural protection/neural repair therapies End MS Forever Enhanced understanding of genetic/environmental contributions to risk Development of measures to prevent the development of MS in the first place

16 Needs help with daily activities (Minden, SL et al, 2004)

17 Need for assistance with ADL’s for RRMS vs PPMS People with PPMS are more than twice as likely to need assistance with ADL’s (Sonya Slifka Longitudinal Study, 2006)

18 Labor force participation Approximately 90% of people with MS have a work history At the time of diagnosis, the labor force participation of people with MS is the same as the general population Labor force participation declines over time and at present only around 40% of people with MS are employed The decline in labor force participation soon after diagnosis can be as high as three percentage points per year (Minden, SL et al, 2006) (Sonya Slifka Study, 2006)

19 Employment status of persons with PPMS vs RRMS drop (Sonya Slifka Study, 2006)

20 Decrease in percent employed with increasing duration of MS (Sonya Slifka Study, 2006)

21 (Rao et al., 1991) Impact of Cognitive Impairment on Daily Functioning (Environmental Status Scale) P<0.01 P<0.05 P<0.01 Mean scale score Worsening Cognitively intact (n=52) Cognitively impaired (n=48) P<0.01 P<0.05 P<0.01 Work status Social activity Personal assistance Community services Financial status Transportation Personal residence

22 How MS fits in with CAL and challenges “The rules for determining disability can be very complicated, but some individuals have such serious medical conditions that their conditions obviously meet our disability standards. To address these individuals’ needs, we strive to provide not only responsive, but also compassionate, public service that ensures the most severely disabled in our society who meet the Act’s requirements are awarded benefits quickly.” Social Security Administration. Compassionate Allowances: Advance notice of proposed rulemaking. Federal Register, Vol 72, No. 146, Tuesday, July 31, (p )

23 How we think MS fits in with CAL and challenges - continued The fit – A substantial proportion of applicants with MS “obviously meet our disability standards.” The challenge – Owing to the complexity of MS, there is no single symptom or sign that one can utilize to determine who “obviously meets the standards.” Instead there are several.

24 $721 million cumulative research investment (estimated through 2010) Examples of handling MS for CAL DeterminationListingRemarks Meets Listing11.09A MS with severe mobility impairment, disorganization of motor function as described in 11.04B (Equivalent to an EDSS score of 5.0 or greater) Meets Listing11.09A MS with severe ataxia, disorganization of motor functioning as described in 11.04B Meets Listing11.09B MS and legally blind as described in 2.02, 2.03A and 2.03B, 2.03C, 2.04 with duration requirement Meets Listing11.09BMS with dementia as described in A & B Meets Listing11.09A MS with titubation, disorganization of motor functioning as described in 11.04B Meets Listing11.09MS and ventilator dependent as described in 3.02

25 Jacqueline du Pré “ …she had little control of her fingering and bowing…” “…there were times when friends attempted to carry her, in the chair, up or down a flight of stairs, and she unceremoniously ended up on the floor…” “…she again had pneumonia, this time there were no options…”

26 Supplementary slides Female to male ratio in the MS population 2010 revision of MS diagnostic criteria Use of paraclinical evidence in MS diagnosis Common differential diagnostic conditions FDA approved drugs for the treatment of MS FDA approved drugs used to treat the symptoms of MS Condensed version of the Expanded Disability Status Scale References

27 MS is more common among women and the gap seems to be growing Females Males Total (Noonan, CW, et al, 2002)

28 Diagnostic criteria – revised 2010 CLINICAL (ATTACKS) LESIONSADDITIONAL CRITERIA TO MAKE DX 2 or moreObjective clinical evidence  2 lesions or objective clinical evidence of 1 lesion with reasonable historical evidence of a prior attack None. Clinical evidence alone will suffice; additional evidence desirable but must be consistent with MS 2 or moreObjective clinical evidence of 1 lesion DIS*; OR await further clinical attack implicating a different CNS site 1Objective clinical evidence of  2 lesions DIT*; OR await a second clinical attack 1Objective clinical evidence of 1 lesion DIS* OR await further clinical attack implicating a different CNS site AND DIT*; OR await a second clinical attack 0 (progression from onset) One year of disease progression (retrospective or prospective) AND at least two of: DIS in the brain based on  1 T2 lesion in periventricular, juxtacortical or infratentorial regions; DIS in the spinal cord based on  2 T2 lesions; or positive CSF (Adapted from Polman, C et al in press)

29 Paraclinical evidence in MS diagnosis - revised 2010 (Adapted from Polman, C et al in press) Evidence for Disssemination of Lesions in Space (DIS) 2 Evidence for Dissemination of Lesions in Time (DIT) 3 ≥ 1 T2 lesion + in at least two out of four areas of the CNS: periventricular, juxtacortical, infratentorial, or spinal cord  Gadolinium enhancement of lesions is not required for DIS  If a subject has a brainstem or spinal cord syndrome, the symptomatic lesions are excluded and do not contribute to lesion count  A new T2 and/or gadolinium-enhancing lesion(s) on follow-up MRI, with reference to a baseline scan, irrespective of the timing of the baseline MRI or  Simultaneous presence of asymptomatic gadolinium-enhancing and non-enhancing lesions at any time Evidence for Positive CSF Oligoclonal IgG bands in CSF (and not serum) or elevated IgG index 2 Swanton KL et al. Lancet Neurology 2007;6: / Swanton KL et al. H Neurol Neurosurg Psychiatry 2006;77: Montalban X, et al. Neurology 2010;74:

30 Common differential diagnostic conditions Variant Neuromyelitis optica (NMO)/Devic’s – recurrent optic neuritis and transverse myelitis extending over 3 or more vertebral segments More Common Mimics Acute disseminated encephalomyelitis (pediatric cases) Human T-cell lymphotrophic virus-1 (HTLV-1) Mild stroke Neuro Lyme disease Less common mimics Lupus Sjogren’s Myasthenia gravis Sarcoidosis Binswanger's Dural Arteriovenous Fistulas

31 FDA approved disease modifying therapies Avonex – weekly IM Betaseron – every other day SC Copaxone – daily SC Extavia – every other day SC Gilenya – daily oral Novantrone – every three months IV Rebif – three times per week SC Tysabri – every four weeks IV

32 Examples of FDA approved drugs used to treat the symptoms of MS Ampyra – approved to treat speed of walking in MS Baclofen – approved for the treatment of spasticity Botox – approved to treat upper limb spasticity Nuedexta – approved to treat pseudobulbar affect Tizanidine – approved for the treatment of spasticity Other classes – antibiotics, bladder control medications, antidepressants, pain killers, stool softeners

33 Expanded Disability Status Scale (abbreviated and paraphrased) 0.0 Normal neurological exam 1.0 No disability, minimal signs in one FS 2.0 Minimal disability in one FS 3.0 Moderate disability in one FS or mild disability in three or four FS 4.0 Fully ambulatory without aid but severe disability in one FS or combination 5.0 Ambulatory without aid for 200 meters; disability impairs full daily activities 6.0 Intermittent or unilateral constant assistance required to walk 100 meters 7.0 Unable to walk more than 5 meters; wheels self in wheelchair 8.0 Restricted to wheelchair or bed; retains some self-care functions 9.0 Confined to bed; needs assistance with all activities; able to communicate and eat 10.0 Death due to MS

34 References Berger JR. Multiple sclerosis. PowerPoint presentation Accessed 2/25/2011: Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983; 33: Minden SL, Frankel D, Hadden LS, Srinath KP, Perloff JN. Disability in elderly people with multiple sclerosis: An analysis of baseline data from the Sonya Slifka Longidtudinal Multiple Sclerosis Study. Neurorehabilitation 2004; 19: Minden SL, Frankel D, Hadden L, Perloff J, Srinath KP, Hoaglin DC. The Sonya Slifka Longitudinal Multiple Sclerosis Study: methods and sample characteristics. Multiple Sclerosis 2006; 12: Noonan CW, Kathman SJ, White MC. Prevalence estimates for MS in the US and evidence of an increasing trend for women. Neurology 2002; 58:136–138. Polman CH, et al. Diagnostic criteria for multiple sclerosis: 2010 revisions to the “McDonald Criteria.” annals of Neurology. (in press).* Quarles RH, Macklin WB, Morrell P. Myelin formation, structure, and biochemistry. In Siegel GJ, Albers RW. (eds.) Basic neurochemistry, Volume I: molecular, cellular, and medical aspects, Seventh Edition. Burlington, MA, 2006, Elsevier Academic Press. (Chapter 4, pp ). Rao SM, Leo GJ, Ellington L, Nauertz T, Bernardin L, Unverzagt F. Cognitive dysfunction in multiple sclerosis. II. Impact on employment and social functioning. Neurology 1991; 41: Social Security Administration. Compassionate Allowances: Advance notice of proposed rulemaking. Federal Register, Vol 72, No. 146, Tuesday, July 31, (p ). Sonya Slifka Longitudinal Multiple Sclerosis Study – findings from the baseline and follow-up interviews, NMSS research contract #HC 0032: Trapp BD, Peterson J, Ransohoff RM, Rudick R, Mork S, Bo L. Axonal transection in the lesions of multiple sclerosis. New England Journal of Medicine 1998; 338: *A pocket card summarizing the 2010 revision of the McDonald Criteria is in preparation by the National MS Society.


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