CHALLENGING ISSUES IN CARE FOR ADOLESCENTS AND ADULTS LIVING WITH SPINA BIFIDA

1. CHALLENGING ISSUES IN CARE FOR ADOLESCENTS AND ADULTS LIVING WITH SPINA BIFIDA Mark J. Merkens, M.D. Director, Spina Bifida Program Doernbecher Children’s Hospital Associate Professor of Pediatrics OREGON HEALTH & SCIENCE UNIVERSITY Portland, Oregon

2. My Philosophy • Realistic Optimism • I am a Pediatrician talking about the care of adults living with spina bifida. These are some of my observations, some from literature, some from recent research.

3. Some Issues for Adolescents

4. ADOLESCENTSNormal Questions • How am I different? • Why am I different? • What am I going to do? • What is my identity?

5. WITH SPINA BIFIDA:Learning necessary • Learn all about your condition. • Learn all that mother knows & does. • Learn all the care needs. • Learn all the procedures. • Learn how to purchase. • Learn how to advocate for self.

6. ADOLESCENTSLearning issues • “Learned helplessness” • Nonverbal Learning Disability (NLD) • Self-starting (“Executive function”) • Organization • Math • Body language and Social Situations • Distractibility

7. ADOLESCENTSSocialization • Friendship vs. Isolation • Social boundaries • Intimacy • Sexuality • Sex education • Risk of sexual abuse

8. ADOLESCENTSTransitioning • New PCP • Ongoing SB care • Self-care • Self-advocacy • Independent living • Vocation/job/career

9. VOCATIONAL REHABILITATION TRANSITION FROM SECONDARY SCHOOL TO WORK OR POSTSECONDARY EDUCATION FOR YOUTH WITH DISABILITIES: • Few vocational programs in H.S., and inadequate time spent on vocational and life skills.

10. Some Issues for Adults

11. ADULTSEntitlement • ADA, SSDI • Loss of: • IFSP/IEP/Transition plan • Teachers & Mentors • Classroom Aide

12. ADULTSJob Challenges • You are on your own. • The boss is not your teacher. • Continence is critical. • Social boundaries are critical. But: • It is much more satisfying than watching TV by yourself. • At least go volunteer.

13. NORMAL AGING • Muscular • Decreased strength, endurance, flexibility. • Nervous system • Deceased sensation, balance, cognitive • Skeletal • Arthritis, osteoporosis, contractures.

14. NORMAL AGING • Cardiovascular • Dec. cardiac output • Vascular changes. • Skin • Thinning, less elastic. • Metabolism • Slows, tendency to gain weight. • Vascular changes of aging.

15. NORMAL AGING • Continence • Pain • Musculoskeletal, nerve compression or damage. • Mental Health • Limited horizons, future

16. Aging in Spina Bifida • Decreased skin tone. • Increased risk of decubitus ulcers, abrasions. • Osteoporosis in higher level Spina Bifida from lack of bone stimulation from walking. • Risks of recurrent fractures. • Obesity • From decreased mobility • Causes decreased mobility

17. Aging in Spina Bifida • Uneven pressure on joints. • Earlier arthritis and pain. • Diminished muscle strength & endurance. • Decreased ambulation, mobility, transfers. • Diminished nerve function. • Decreased sensation. • Decline in circulation in legs & feet.

18. AGINGAccelerants • Dangerous drugs • Smoking • Alcoholism • Other Dependencies or Addictions

19. ADULTSMedical issues • “Arrested” Hydrocephalus • VP shunt failure • Spina Cord Tethering • Syrinx • Chiari problems: apnea • Kidney failure • Pressure sores • Equipment needs

20. ADULTSSocialization • Everything Adolescents experience. • Isolation. • Insufficient activities. • Insufficient social groups. • Insufficient successful role models.

21. RESEARCHBowman, MacLone, et al • 80% have social bladder continence. • 85% graduated high school.

22. RESEARCHOther Studies • > 50% have ill fitting braces. • Independence is closely related to the level of the lesion. • Unemployment very high.

23. RESEARCHSawin, Brei, et al. • Adolescent-parent agreement in areas of observable behavior. • Differences in Beliefs, like future expectations, and Competencies, such as school performance. • Providers must question both the family and the teen to understand the real picture.

24. RESEARCHMary Kalfoss, RN, PhD in NorwayQuality of Life Bad News: • 41% report Depression • 19% report Anxiety • 69% report cognitive problems affect ADLs. Good News: • 60% report life is meaningful. • 63% report Quality of Life is complete or high.

25. RESEARCHKalfoss & Merkens: Oregon • 47% report symptoms of Depression • 23% report Anxiety • 69% report Avoidance Thinking. • 65% report cognitive problems affect ADLs. • 33% report declining cognitive status.

26. RESEARCHKalfoss & Merkens • Quality of Life (QoL) was the strongest predictor of satisfaction with health status. • Country (Norway vs. Oregon) was NOT a significant predictor.

27. RESEARCHBier, et al. • Lower Quality of Life (QOL) related to: • more functional challenges in self-care. • the need for more caregiver assistance and supervision. • Age, level of lesion, and social supports were not related to quality of life. • Maximizing functional independence should be a priority for improving HRQOL.

28. ADDRESS THESE ISSUES: Adolescents • Adolescents living with spina bifida “need to achieve developmental, self-management and independence skills to survive in the world of adults.”(Buran) • Acquiring medical supplies • Providing treatments • Money management • Home making skills • Vocational and Job training • Families, institutions, and care providers need to assure these are taught.

29. ADDRESS THESE ISSUES: Adults need… • Interdisciplinary Clinics and networks of care for adults living with spina bifida. • To address pain care. • More Vocational Rehabilitation services & resources. • More support and advice on self-care, social skills, intimacy and sexuality. • More descriptive studies and outcomes research.

30. THE CHALLENGE FOR ALL HUMANS & VULCANS “Live long and prosper”