Download - Transitional Care for Pediatric Patients with Neuromuscular Diseases: A Health Technology Assessment
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Transitional Care for PediatricPatients with Neuromuscular
Diseases: A Health Technology Assessment
Jackie Tran, MDUniversity of Medicine and Dentistry of New
Jersey, USA
HTAi 9th Annual Meeting, BilbaoIntegrated Care for a Patient Centered System
25 June, 2012
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Outline
Background Transitional Care Overview Neuromuscular Diseases Overview
Objectives Methods Results
Barriers Recommendations
Conclusions
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Transitional Care OverviewBackground
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Unique Adult Needs
Physical maturation Size Endocrine management Deformities
Sexual maturation Menstruation Fertility Pregnancy
Psychosocial maturation Identity Experimentation Independence Family/Friends
Intellectual maturation Education Vocation
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Reality: Unmet Needs
Increase hospitalization, particularly of ages 18-21
© Pediatrics. 2011 Jul;128(1):5-13. Epub 2011 Jun 27.Adults with chronic health conditions originating in childhood: inpatient experience in children's hospitals.
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Reality: Unmet Needs
Non-adherence to medications over a lifetime in renal transplant patients
Decrease clinic attendance and an average of 1.47 health provider changes in diabetes type 1 patients
Decrease mobility in spina bifida patients during transitional years
Increase long-term morbidity and mortality in cystic fibrosis patients
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Neuromuscular Diseases Overview
Background
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Selected Neuromuscular Diseases
of ChildhoodCongenital
myopathies dystrophic (e.g., Duchenne) myotonic congenital
spinal muscle atrophy congenital myasthenia hereditary motor
sensory neuopathy Friedreich ataxia
Acquired
Guillain-Barré syndrome
myasthenia gravis polymyositis toxic myopathy
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Epidemiology
Worldwide prevalence of disabling, inherited neuromuscular diseases: 1 in 3,500
Increased survival rate to adulthoodSome Survival Statistics in the Literature
60% of males born with Duchenne between 1983 – 1987 survived past age 24
More than 33% of Charcot-Marie-Tooth Type 2 patients live past the third decade of life
A patient with Type 2 SMA have survival rate of 68.5% at age 25
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Unique Characteristics
Low recognition Apparent functional deficits Infamous progressive/regressive
courses Media portrayal Multi-organ systems involvement Large team investment
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Equally Poor Transitional Care
A glaring 60% of 850 people living with muscle disease rate transition from child to adult services as “poor” or
“very poor”
Muscular Dystrophy Campaign 2008 survey
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Finding Transitional Solutions
Objectives & Methods
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Objectives
Identify systematic and unique barriers to a successful transition of care for adolescents with neuromuscular diseases (NMDs).
Recommend comprehensive strategies to improve the experience for patients, family members, and health providers alike.
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Methods
Systematic literature review of publications between January 2000 and December 2011
Informal survey of local clinicians’ opinions
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Only the beginning…Results
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General Observations
Notable publication increase on transitional care over the past 5 years
Emphasis on the following diagnoses: diabetes, cancer, renal transplant, inflammatory bowel
dz cerebral palsy and spina bifida
Most prolific countries: USA, Canada and UK Honorable mentions: Australia, France, Japan,
Switzerland
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All Transitional Care Publications
NMD-Relevant Publications
Number of
Publications
60+ 14
Population description
• generic: “chronic illnesses” & “special health care needs” & “congenital disabilities” & “developmental
disabilities”
• multiple diagnoses-specific publications
• inter-mix with spina bifida & cerebral palsy &
“chronic neurological disorders”
• Duchenne dystrophy
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All Transitional Care Publications
NMD-Relevant Publications
Publication
Content
• highlight need for transitional care• clinician & patient survey/commentaries
• anecdote / case reports
• single-institution experience
• general critiques of current transition
processes & models
• review of medical topics on transitional ages
Outcome measure
s
• subjective reports of wellness & satisfaction
• individual programs: admissions & biomarkers
(retrospective & limited to 1 year after transition)
• health expenditure at transitional age as a secondary outcome
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Models of Transitional Care
Diagnosis-focused (most common)
pediatric and adult provider collaboration
Medical home-based (least common)
primary care practitioner as coordinator
Adolescent-focused physical, physiological, social issues focused
Transition-based outreach representatives from one facility
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General Barriers
Afterthought process Provider availability (absolute & time)
Coordinator responsibility Decision-making capacity Advisors resourcefulness Insurance coverage Transportation / Architectural Medical records Ventilator, dialysis
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Emotional Barriers
Patient & Family neglect
abandonment
Adult team burden
unfamiliarity
Peds team attachment
over-confidence
FearAnxietyDistrust
Uncertainty
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Unique Barriers &
Opportunities
Assistive technology Malpractice insurance
Insurance / resources for the very ill Association with spina bifida and/or cerebral
palsy
Even more limited provider availability Institutionalized patients Aging care providers Uncertain future
Barriers
Opportunities
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Recommendations
Early notification Individualized plan
flexible but definitive date of transition assess cognitive/emotional and functional
status gradual promote of independence
Comprehensive approach patient’s and family members’ input interdisciplinary team
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Recommendations
Coordinator designation Patient education
self care and disease process healthcare system navigation
Long-term planning vocation and education living situation
Resources availability
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Advanced Topics
Program sustainability Provider training Transition to palliative care Socioeconomic status effects
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Conclusions
All children with neuromuscular diseases need a transition plan before entering adulthood
Successful transition requires coordination of services across all providers
Especially for individuals affected by NMDs, the transition plan should incorporate patient’s and family’s long-term goals
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Primary References
Abbott, D. Transition to adulthood for young men with Duchenne Muscular Dystrophy. International Journal of Integrated Care, 31 December 2009 - ISSN 1568-415
Centers for Disease Control and Prevention (CDC). Prevalence of Duchenne/Becker muscular dystrophy among males aged 5-24 years - four states, 2007. MMWR Morb Mortal Wkly Rep. 2009 Oct 16;58(40):1119-22.
Strehle EM. Long-term management of children with neuromuscular disorders. J Pediatr (Rio J). 2009 Sep-Oct;85(5):379-84. Tiffreau V, et al. Ann Readapt Med Phys. 2006 Dec;49(9):652-8. Epub 2006 Jun 27. [Transition in health care from youth to
adulthood for disabled people]. Ouyang L, et al. Health care utilization and expenditures for childrenand young adults with muscular dystrophy in a privately insured
population. J Child Neurol. 2008 Aug;23(8):883-8. Epub 2008 Apr 10. Manzur AY, Muntoni F. Diagnosis and new treatments in musculardystrophies. J Neurol Neurosurg Psychiatry. 2009 Jul;80(7):706-
14. Hill ME, Phillips MF. Service provision for adults with long-term disability: a review of services for adults with chronic
neuromuscular conditions in the United Kingdom. Neuromuscul Disord. 2006 Feb;16(2):107-12. Epub 2006 Jan 19. Nomura Y. [Care continuity for patients with myasthenia gravis during transition from childhood to adulthood]. Nihon Rinsho. 2010
Jan;68(1):39-44. Yoshioka M, et al. [Care continuity for patients with myopathy during transition of childhood to adulthood]. Nihon Rinsho. 2010
Jan;68(1):53-6. Minicozzi A. Transition to adult care: another view. Pediatr Nurs. 2000 Jul-Aug;26(4):411-2. Katz JD, et al. Parents' perception of self-advocacy of children with myositis: an anonymous online survey. Pediatr Rheumatol Online
J. 2011 Jun 7;9(1):10. Goodman DM, et al. Adults with chronic health conditions originating in childhood: inpatient experience in children's hospitals.
Pediatrics. 2011 Jul;128(1):5-13. Epub 2011 Jun 27. Rearick E. Enhancing success in transition service coordinators: use of transformational leadership. Prof Case Manag. 2007 Sep-
Oct;12(5):283-7. Parker AE, et al. Analysis of an adult Duchenne muscular dystrophy population. QJM. 2005 Oct;98(10):729-36. Epub 2005 Aug 31.