BACKGROUND: Youth with Neuromuscular Disorders (NMD) who are wheelchair users can now survive well into adulthood if their multisystem comorbidities are prudently managed. Uptake of health behaviors may optimize their health outcomes.
OBJECTIVE: To explore youths’ perceptions of health, health behaviors and healthcare engagement.
METHODS: This qualitative study purposefully recruited 11 youth with NMD from a concurrent, population-based study for variability of age, gender, type of NMD and their ratings of motivation and engagement. Interview data were analyzed and synthesized by thematic content.
RESULTS: Participants perceived healthcare engagement as being given tools (knowledge and responsibility) and using them to maintain their finely balanced health. Nested in adequate social, emotional and physical support, they took responsibility for creatively integrating health behaviors they felt were informed by credible knowledge, gained primarily through personal experience.
CONCLUSION: Cognizant of their compromised health, youth with NMD in this study were motivated to maintain their physical health. Limited NMD condition specific knowledge challenged youths’ uptake of health behaviors. They valued a learning partnership with their healthcare professionals. By embracing the youth’s experience based knowledge and through facilitating supportive relationships, healthcare professionals co-construct youth’s healthcare engagement that may optimize health behaviors and outcomes.
BachJ. R., & MartinezD. (2011). Duchenne muscular dystrophy: Continuous noninvasive ventilatory support prolongs survival. Respiratory Care, 56(6), 744–750. doi: 10.4187/respcare.00831
3.
BjörquistE., NordmarkE., & HallströmI. (2015). Living in transition – experiences of health and well-being and the needs of adolescents with cerebral palsy. Child: Care, Health And Development, 41(2), 258–265. doi: 10.1111/cch.12151
4.
BladenC. L., RaffertyK., StraubV., MongesS., MorescoA., DawkinsH., et al. (2013). The TREAT-NMD Duchenne muscular dystrophy registries: Conception, design and utilisation by industry and academia. Human Mutation, 34, 1449–1457. doi: 10.1002/humu.22390
BrightF. A., KayesN. M., WorrallL., & McPhersonK. M. (2015). A conceptual review of engagement in healthcare and rehabilitation. Disability and Rehabilitation, 37(8), 643–654. doi: 10.3109/09638288.2014.933899
7.
BucksR. S., HawkinsK., SkinnerT. C., HornS., SeddonP., & HorneR. (2009). Adherence to treatment in adolescents with cystic fibrosis: The role of illness perceptions and treatment beliefs. Journal of Pediatric Psychology, 34(8), 893–902. doi: 10.1093/jpepsy/jsn135
8.
BudychK., HelmsT. M., & SchultzC. (2012). How do patients with rare diseases experience the medical encounter? Exploring role behavior and its impact on patient-physician interaction. Health Policy, 105(2/3), 154–164111p.
9.
BushbyK.,
FinkelR.,
BirnkrantD. J.,
CaseL. E.,
ClemensP. R.,
CripeL., et al. (2010a). Diagnosis and management of Duchenne muscular dystrophy, part 1: Diagnosis, and pharmacological and psychosocial management. The Lancet Neurology, 9(1), 77–93. doi: 10.1016/s1474-4422(09)70271-6
10.
BushbyK.,
FinkelR.,
BirnkrantD. J.,
CaseL. E.,
ClemensP. R.,
CripeL., et al. (2010b). Diagnosis and management of Duchenne muscular dystrophy, part 2: Implementation of multidisciplinary care. The Lancet Neurology, 9(2), 177–189. doi: 10.1016/s1474-4422(09)70271-6
11.
CartwrightT., FraserE., EdmundsS., WilkinsonN., & JacobsK. (2015). Journeys of adjustment: The experiences of adolescents living with juvenile idiopathic arthritis. Child: Care, Health And Development, 41(5), 734–743. doi: 10.1111/cch.12206
12.
Daker-WhiteG., KingstonH., PayneK., GreenfieldJ., EalingJ., & SandersC. (2015). You don’t get told anything, they don’t do anything and nothing changes’. Medicine as a resource and constraint in progressive ataxia. Health Expectations, 18(2), 177–187111p. doi: 10.1111/hex.12016
13.
EpsteinA., LeonardH., DavisE., WilliamsK., ReddihoughD., MurphyN., et al. (2016). Conceptualizing a quality of life framework for girls with Rett syndrome using qualitative methods. American Journal of Medical Genetics Part A, 170(3), 645–653. doi: 10.1002/ajmg.a.37500
14.
FreemanM., StewartD., ShimmellL., MissiunaC., Burke-GaffneyJ., JafferS., et al. (2015). Development and evaluation of The KIT: Keeping It Together™ for Youth (the ‘Youth KIT’) to assist youth with disabilities in managing information. Child: Care, Health And Development, 41(2), 222–229. doi: 10.1111/cch.12199
15.
GibsonB. E., ZitzelsbergerH., & McKeeverP. (2009). Futureless persons’: Shifting life expectancies and the vicissitudes of progressive illness. Sociology of Health and Illness, 31(4), 554–568. doi: 10.1111/j.1467-9566.2008.01151.x
16.
GrumanJ., RovnerM. H., FrenchM. E., JeffressD., SofaerS., ShallerD., et al. (2010). From patient education to patient engagement: Implications for the field of patient education. Patient Education and Counselling, 78(3), 350–356. doi: 10.1016/j.pec.2010.02.002
17.
HullJ., AniapravanR., ChanE., ChatwinM., FortonJ., GallagherJ., et al. (2012). British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax, 67(Suppl 1), i1–i40. doi: 10.1136/thoraxjnl-2012-201964
18.
JavalkarK., JohnsonM., KshirsagarA. V., OceguedaS., DetwilerR. K., & FerrisM. (2016). Ecological factors predict transition readiness/self-management in youth with chronic conditions. Journal of Adolescent Health, 58(1), 40–46. doi: http://dx.doi.org/10.1016/j.jadohealth.2015.09.013
19.
JohnsonK. R., FuchsE., HorvathK. J., & ScalP. (2015). Distressed and looking for help: Internet intervention support for Arthritis self-management. Journal of Adolescent Health, 56(6), 666–671. doi: http://dx.doi.org/10.1016/j.jadohealth.2015.02.019
20.
JohnsonK. R., McMorrisB. J., MapelLentzS., & ScalP. (2015). Improving self-management skills through patient-centered csommunication. Journal of Adolescent Health, 57(6), 666–672. doi: http://dx.doi.org/10.1016/j.jadohealth.2015.08.009
21.
KatirjiB., KaminskiH. J., & RuffR. L. (2013). Neuromuscular Disorders in Clinical Practice (2 ed.). New York: Springer New York.
22.
KatzS. L., GabouryI., KeiltyK., BanwellB., VajsarJ., AndersonP., et al. (2010). Nocturnal hypoventilation: Predictors and outcomes in childhood progressive neuromuscular disease. Archives of Disease in Childhood, 95(12), 998–1003.
23.
KauwD., Repping-WutsH., NoordzijA., StikkelbroeckN., HermusA., & FaberM. (2015). The contribution of online peer-to-peer communication among patients with Adrenal Disease to patient-centered care. Journal of Medical Internet Research, 17(3), e54–e5451p. doi: 10.2196/jmir.3869
24.
Keim-MalpassJ., LetzkusL. C., & KennedyC. (2015). Parent/caregiver health literacy among children with special health care needs: A systematic review of the literature. BMC Pediatrics, 15, 92. doi: 10.1186/s12887-015-0412-x
25.
LambC., & PedenA. (2008). Understanding the experience of living with spinal muscular atrophy: A qualitative description. The Journal of Neuroscience Nursing, 40(4), 250–256.
26.
LandfeldtE., LindgrenP., BellC. F., SchmittC., GuglieriM., StraubV., et al. (2015). Compliance to care guidelines for Duchenne Muscular Dystrophy. Journal of Neuromuscular Diseases, 2(1), 63–72. doi: 10.3233/JND-140053
27.
LangA. R., MartinJ. L., SharplesS., & CroweJ. A. (2013). The effect of design on the usability and real world effectiveness of medical devices: A case study with adolescent users. Applied Ergonomics, 44(5), 799–810. doi: http://dx.doi.org/10.1016/j.apergo.2013.02.001
28.
LiamputtongP. (2013). Qualitative Research Methods (Fourth Edition ed.). Australia: Oxford University Press.
29.
LiemG. A. D., & MartinA. J. (2012). The motivation and engagement scale: Theoretical framework, psychometric properties, and applied yields. Australian Psychologist, 47(1), 3–13.
30.
MahJ. K., ThannhauserJ. E., McNeilD. A., & DeweyD. (2008). Being the lifeline: The parent experience of caring for a child with neuromuscular disease on home mechanical ventilation. Neuromuscular Disorders, 18(12), 983–988. doi: 10.1016/j.nmd.2008.09.001
31.
MartinA. J. (2007). Examining a multidimensional model of student motivation and engagement using a construct validation approach. British Journal of Educational Psychology, 77(Pt 2), 413–440.
32.
MaslowG. R., & ChungR. J. (2013). Systematic review of positive youth development programs for adolescents with chronic illness. Pediatrics, 131(5), e1605–e1618. doi: 10.1542/peds.2012-1615
33.
McCarthyA. (2015). Summer camp for children and adolescents with chronic conditions. Pediatric Nursing, 41(5), 245–250. doi: 10.1016/j.chc.2007.05.002
34.
MolchoM., GabhainnS. N., & KelleherC. C. (2007). Interpersonal relationships as predictors of positive health among Irish youth: The more the merrier?Irish Medical Journal, 100(8), 33–36.
35.
NierseC. J., AbmaT. A., HoremansA. M. C., & Van EngelenB. G. M. (2013). Research priorities of patients with neuromuscular disease. Disability and Rehabilitation, 35(5), 405–412. doi: 10.3109/09638288.2012.694964
36.
Orphanet. (2014). Prevalence of rare diseases: Bibliographic data. Rare Disease Collection. Retrieved from http://www.orpha.net/consor/cgi-bin/Education_Home.php.
37.
PeterN. G., ForkeC. M., GinsburgK. R., & SchwarzD. F. (2009). Transition from pediatric to adult care: Internists’ perspectives. Pediatrics, 123(2), 417–423. doi: 10.1542/peds.2008-0740
38.
RousseauM. C., PietraS., BlayaJ., & CatalaA. (2011). Quality of life of ALS and LIS patients with and without invasive mechanical ventilation. Journal of Neurology, 258(10), 1801–1804.
39.
SamsonA., TomiakE., DimilloJ., LavigneR., MilesS., ChoquetteM., et al. (2009). The lived experience of hope among parents of a child with Duchenne muscular dystrophy: Perceiving the human being beyond the illness. Chronic Illness, 5(2), 103–114. doi: 10.1177/1742395309104343
40.
SawickiG. S., HellerK. S., DemarsN., & RobinsonW. M. (2014). Motivating adherence among adolescents with cystic fibrosis: Youth and parent perspectives. Pediatric Pulmonology, n/a-n/a. doi: 10.1002/ppul.23017
41.
SawyerS. M., & AmbresinA.-E. (2014). Successful transitions: Beyond disease control to better life chances. Journal of Adolescent Health, 54(4), 365–366. doi: http://dx.doi.org/10.1016/j.jadohealth.2014.01.012
42.
SawyerS. M., DrewS., YeoM. S., & BrittoM. T. (2007). Adolescents with a chronic condition: Challenges living, challenges treating. The Lancet, 369(9571), 1481–1489. doi: 10.1016/s0140-6736(07)60370-5
43.
SchwartzL. A., TuchmanL. K., HobbieW. L., & GinsbergJ. P. (2011). A social-ecological model of readiness for transition to adult-oriented care for adolescents and young adults with chronic health conditions. Child: Care, Health And Development, 37(6), 883–895. doi: 10.1111/j.1365-2214.2011.01282.x
44.
SeburgE. M., McMorrisB. J., GarwickA. W., & ScalP. B. (2015). Disability and discussions of health-related behaviors between youth and health care providers. Journal of Adolescent Health. doi: 10.1016/j.jadohealth.2015.03.004
45.
ShakkottaiA., KidwellK. M., TownsendM., & NasrS. Z. (2015). A five-year retrospective analysis of adherence in cystic fibrosis. Pediatric Pulmonology, n/a-n/a. doi: 10.1002/ppul.23307
46.
SilvermanA. M., VerrallA. M., AlschulerK. N., SmithA. E., & EhdeD. M. (2016). Bouncing back again, and again: A qualitative study of resilience in people with multiple sclerosis. Disability and Rehabilitation, 1–9. doi: 10.3109/09638288.2016.1138556
47.
SmithJ. A., & OsbornM. (2008). Chapter 4: Interpretative Phenomenological Analysis. In
SmithJ. A. (Ed.), Qualitative Psychology: A Practical Guide to Research Methods. Second Edition. London: Sage Publications.
48.
SonneveldH. M., StratingM. M., van StaaA. L., & NieboerA. P. (2013). Gaps in transitional care: What are the perceptions of adolescents, parents and providers?Child: Care, Health And Development, 39(1), 69–80. doi: 10.1111/j.1365-2214.2011.01354.x
49.
StrehleE.-M. (2009). Long-term management of children with neuromuscular disorders. Jornal De Pediatria, 85(5), 379–384. doi: 10.2223/jped.1929
50.
SurisJ.-C., & AkreC. (2015). Key elements for, and indicators of, a successful transition: An international Delphi study. Journal of Adolescent Health, 56(6), 612–618. doi: http://dx.doi.org/10.1016/j.jadohealth.2015.02.007
51.
van HuijzenS., & van StaaA. (2013). Chronic ventilation and social participation: Experiences of men with neuromuscular disorders. Scandinavian Journal of Occupational Therapy, 20(3), 209–216. doi: 10.3109/11038128.2013.765033
52.
van StaaA., JedelooS., & van der StegeH. (2011). What we want”: Chronically ill adolescents’ preferences and priorities for improving health care. Patient Preference And Adherence, 5, 291–305. doi: 10.2147/PPA.S17184
53.
van StaaA., & SattoeJ. N. T. (2014). Young adults’ experiences and satisfaction with the transfer of care. Journal of Adolescent Health, 55(6), 796–803. doi: http://dx.doi.org/10.1016/j.jadohealth.2014.06.008
54.
VennikF. D., AdamsS. A., FaberM. J., & PuttersK. (2014). Expert and experiential knowledge in the same place: Patients’ experiences with online communities connecting patients and health professionals. Patient Education and Counselling, 95(2), 265–270. doi: 10.1016/j.pec.2014.02.003
55.
VuillerotC., HodgkinsonI., BisseryA., Schott-PethelazA.-M., IwazJ., EcochardR., et al. (2010). Self-perception of quality of life by adolescents with neuromuscular diseases. Journal of Adolescent Health, 46(1), 70–76. doi: 10.1016/j.jadohealth.2009.05.005
56.
WangC. H., DowlingJ. J., NorthK., SchrothM. K., SejersenT., ShapiroF., et al. (2012). Consensus statement on standard of care for congenital myopathies. Journal of Child Neurology, 27(3), 363–382. doi: 10.1177/0883073812436605
57.
WangC. H., FinkelR. S., BertiniE. S., SchrothM., SimondsA., WongB., et al. (2007). Consensus statement for standard of care in Spinal Muscular Atrophy. Journal of Child Neurology, 22(8), 1027–1049. doi: 10.1177/0883073807305788
58.
World Health Organisation. (2007). International Classification of Functioning, Disability and Health - Children and Youth Version. Switzerland, Geneva: WHO.
59.
YoungH. K., LoweA., FitzgeraldD. A., SetonC., WatersK. A., KennyE., et al. (2007). Outcome of noninvasive ventilation in children with neuromuscular disease. Neurology, 68(3), 198–201.
