Supporting people with cerebral palsy and like physical disabilities.
Ageing and the late effects of disability.

Background

It has become clear to medical practitioners and allied health professionals working with people with cerebral palsy (CP) and like physical disabilities (from this point forwards to be referred to as “CP Plus”) that there is a greater need to maintain the engagement of people with CP Plus in health and therapy services across their lifespan to reduce and prevent the secondary conditions that occur with ageing and the late effects of disability. As stated by Rimmer(1999), it remains very relevant today that “In the long run, preventing secondary health conditions by empowering people with disabilities to take control of their own health will be more cost-effective, and certainly more humane, than watching people with disabilities decline in function from a lack of good health maintenance.”

People with CP Plus have often been heavily engaged in health and therapy throughout their childhood with a very co-ordinated support structure in place through the paediatric health and community settings.

Health professionals in all settings and across the world who are seeing people with CP Plus later in life after they have not engaged in health and therapy services for an extended period of time, are finding that the health and function outcomes are much poorer compared to those who have engaged either consistently or intermittently.

There are a variety of reasons for this.

One is that after they leave school and the paediatric health system, there has historically been limited funding and services available to support adults with CP Plus. This is no longer the case, however adults with CP Plus who have not already engaged in support possibly do not know this as yet.

Another reason is that after intensive therapy as a child, many adults with CP Plus choose to exit services and stay away from anything that is remotely related to “disability”. They have simply had enough.

The final reason that adults with CP Plus have reported here in Western Australia, across Australia and worldwide as reported in the literature is that they just didn’t know that what is happening to them would happen. Indeed, health professionals are only in the past few decades starting to appreciate what happens as part of ageing with a physical disability and the late effects being experienced. People with CP Plus also state that once they do realise that something is happening to their bodies, they do not know what to do.

General Practitioners play a very important role in supporting people with physical disabilities

As supported by Field et al.,(2010), Rapp & Torres, (2004) and Ng et al., (2003), the importance of a GP being involved in the care of an adult with a disability cannot be over stated.

The GP is the connection. It is recognised by specialists in the tertiary setting and allied health professionals working in the community that there is a need to support GPs to support people with CP Plus.

If an adult with CP Plus is not engaged with a comprehensive disability specific service, the GP is likely to be the first health professional the person with CP Plus visits seeking answers to what is happening to them. If the GP has had the opportunity to get to know the adult with CP Plus, they are likely to be the health professional who knows them best and can best assess for changes in their abilities.

For more information:
Online: futurethinking.abilitycentre.com.au 
 

What can the GP working with people with CP Plus do now?

Access:

Consider the access to your rooms:

• Can people with complex physical needs and large mobility devices access your room?
• Do you have larger plinths and hoists to transfer them out of their chair?
• Would you consider home visits if access to your rooms is difficult or hoist access isn’t possible?

Encourage double appointments: people with complex communication needs require extra time to explain their needs. They also need to be encouraged to bring family or support workers who understand their needs.

Understanding what is normal and not normal for any diagnosis:

Familiarise yourself with the clinical presentation of CP and other physical disabilities, the clinical manifestations and secondary conditions and common comorbidities. Understanding these will assist in differentiating between what is normal for CP and what is unlikely to be related to the primary diagnosis.

Encourage people with physical disabilities to have regular reviews, at least yearly. This will assist them and you to determine what their normal is.

Preventative health:

Recommend and perform all preventative health measures that are offered and recommended to any adult across their lifespan. If access either environmentally or physically for the person with CP Plus are a barrier, explore alternatives.

Recommend and perform annual medical reviews. This provides an opportunity to monitor overall health.

Monitoring:

Be proactive when seeing adults and teenagers with CP Plus by asking disability specific questions. It was interesting to note from feedback that people with CP Plus often reported complex problems and issues however never discussed them with the health professionals they were seeing.

Ask people with physical disabilities direct questions about pain, fatigue and mental wellbeing. These are very common issues that people may not discuss for a variety of reasons.

Encourage everyone with CP Plus to have at least yearly check-ups with allied health professionals regardless of ability and current functional status. Assist those with CP Plus to engage in services. Regular reviews with therapists can pick up small problems before they become bigger ones.

Literature Review Summary

The following articles are recommended reading for health professionals working with people with physical disabilities.

Comprehensive Overview of the Adult with Cerebral Palsy

Murphy (2010), Murphy (2009) and Murphy, Sobus and Bliss (2009) are all articles any health professional working with adults with CP are encouraged to read for a comprehensive overview of the adult with CP detailing the common issues experienced by adults with CP and the specific problems that need to be monitored and addressed. These include spasticity, spine, hip, knee and ankle and foot management. Murphy (2010) discusses the two age ranges that appear to be common periods of functional decline, they being 20-25 years of age and 40-45 years of age. These articles are regularly referenced by other authors confirming their findings are similar and is confirmed clinically by health professionals working with people with CP Plus in Western Australia. The author discusses the importance of health professionals understanding the secondary conditions of CP and screening appropriately other health issues ensuring that the primary diagnosis of CP is not attributed to problems that are unrelated to CP.

Pain and Fatigue

Benner et al., (2017) completed a follow up of adults with CP in the Netherlands to assess the perceived health, self-reported health issues and functional level of adults with CP. Their results confirmed previous thoughts that pain and fatigue were the greatest issues for adults with CP and that these influenced other health outcomes. Increased support is required by others and that timely and appropriate input from health professionals with a lifespan perspective is very important for those living with CP.

Cerebral Palsy Clinical Presentations and Management Strategies

Aisen et al., (2011) discusses cerebral palsy clinical care and neurological rehabilitation detailing the clinical presentation and causes, neurological complications and management strategies. The authors recommend that health professionals working with children and adults with CP be familiar with the clinical manifestations, common co-morbidities and optimum treatments for people with CP.

What consumers, parents, doctors and health professionals want and need to know

Rosenbaum (2003) details information about CP and what parents and doctors want to know. While there have been articles providing updated information since this was published, it provides a good summary of CP, clinical presentations, topography, classifications and the role of the team in achieving goals.

Kemp (2005) writes “What the rehabilitation professional and the consumer need to know”. The author details issues with and recent developments in ageing with a disability, discusses some theories that could explain the loss of capacity in people with a disability, ageing related physical changes, functional issues and psychosocial considerations. Useful reading for any health professional working with people with CP Plus.

Life Expectancy

Strauss et al., (2008) is a paper on the life expectancy in CP: an update is useful reading for the health professional working with people with CP when considering the overall health and wellbeing.

Health issues related to Cerebral Palsy

Hahn (2012), Pruitt & Tsai (2009), Svien et al.,(2008) and Rapp & Torres (2004) all detail health issues related to CP and ageing with CP and the need for service planning for ongoing health care. They provide a comprehensive overview of the body’s systems and what may occur or are the consequences of age-related changes including hearing and vision problems, the cardiovascular system, respiratory problems, endocrine problems, the gastrointestinal system, the musculoskeletal system, sexuality and the pelvic and breast exam, nutritional problems, sleep problems, pain, dental problems and urology problems.

Turk (2009) discusses the health, mortality and wellness issues in adults with CP. They also report that it is clear that many adults with CP do not have routine health checks and other preventative health measures.

Havercamp et al., (2004) studied the difference in health and wellbeing between adults with developmental disabilities, other disabilities and those without disabilities. The authors found that those with disabilities were more likely to have fair to poor health compared to those without disabilities. They suggested that health promotion needed to be directed to people with disabilities in a manner that they would be receptive to and that disability support providers and advocates needed to work with health educators to determine the best way to educate and provide services to adults with disabilities.

Secondary conditions of the musculoskeletal system in adults with Cerebral Palsy

Gajdosik & Cicirello(2001) discusses the secondary conditions of the musculoskeletal system in adolescents and adults with cerebral palsy. The authors also discuss health care. They discuss the importance of managing overall health and divide the components in to acute health problems, lifestyle health risks and secondary conditions related to CP and add that the primary condition of CP should not be the focus of health care intervention. It is discussed that most adults with CP see a physician at least once per year however often do not have all their needs met. Adults with CP attend the doctor’s office when their condition worsens and this is often due to a lack of monitoring. For a variety of reasons, primary health conditions and prevention strategies recommended for everyone are not addressed such as heart disease, diabetes or cancer or having breast, pelvic or rectal exams. It is recommended that the barriers to these primary healthcare interventions be addressed. Finally they discuss the role of therapists and the importance that those involved in supporting adults with CP have a role in educating themselves and the adult with CP and then providing and advertising the appropriate services.

Transition

Field et al., (2010) discusses a program that was set up to assist the transition of young adults out of the paediatric system and into adult health in New South Wales, Australia. They comment on the importance of the GP in managing overall care and that further work is required to develop the role with support from specialists.

Ng et al., (2003) studied a group of young adults with CP in Singapore looking at their health care and social isolation comparing those still at school with those who had graduated. This study found that those who had left school had a decline in health care access. One of the recommendations includes providing training to GPs to enable them to co-ordinate health care for children with support from specialists to continue care into adulthood.

Health, Wellbeing and the Lived Experience

Furukawa et al.,(2001) evaluated the subjective well-being of adults with CP in Japan. They found that adults had reduced morale. The study recommended adults with CP receive medical treatment and rehabilitation therapy to prevent or improve secondary conditions. They discussed the reasons adults with CP do not access services after exiting the school environment and recommended that providing information to adults with CP on the secondary disabilities was the first step in preventing problems from not being resolved.

Morgan, Soh and McGinley (2014) investigated health-related quality of life of adults with CP in Victoria, Australia and the association with mobility decline. They found health and wellbeing was lower in adults with CP compared to their peers and that physical health and personal wellbeing is lower with physical decline. The authors suggested addressing the mobility decline may assist in addressing the lower health-related quality of life.

Horsman et al., (2010) investigated the unique, lived experience of growing older with CP. From discussions with adults with CP in British Columbia, Canada, they found common themes that each person went through “Awareness, Acceptance and Action”. The awareness referred to adults with CP becoming aware of the difficulties they were experiencing. Acceptance and action referred to them accepting themselves and then making changes. This approach is discussed in various other studies and is noted clinically by allied health professionals working with adults with CP Plus in Western Australia. There is a growing awareness that health professionals need to consider CP as a lifespan condition. The authors discuss the importance of health professionals understanding the needs of the adult with CP and informing them what they can do to assist them. They reiterated in this study points discussed by previous studies that health professionals need to promote to their young clients the need to take responsibility for their health, to conserve energy and maintain fitness to minimise secondary impairments later in life.

Sandstrom (2006) investigated similar to Horsman et al.,(2010) in a population of adults with CP in Sweden. The themes were very similar, “perceptions of: a dys-appearing body, a not-appearing body, being different, being in-between, being normal, restricted autonomy, autonomy” with strategies they employ being “to fight one’s way, to plan, to get used to it, to hide, to give one’s all”.

Morgan, Pogrebnoy and McDonald (2014) interviewed adults with CP in Victoria, Australia, discussing their health service experiences. Many discussed that health service access had been unsatisfactory due to the perceived inadequate expertise and that what was offered in the available services was not suitable for them. They discussed that they often did not feel heard by those supporting them and the specialists. Of the services that did exist, if they were given useful information, they had better outcomes.

Mudge et al., (2016) interviewed adults with CP living in New Zealand discussing ageing and health care experiences. Similar to previous studies, they found some common themes 1. Acceptance of change, 2. Exploring identity; cerebral palsy as only one part of self, 3. Taking charge of help, 4. Rethinking the future and 5. Interacting with health professionals. These themes are similar to those discussed by Horsman et al., (2010), Morgan, Soh and McGinley (2014) and Morgan, Pogrebnoy and McDonald (2014) in that the adults with CP discussed needing to accept that ageing and changes to their body were happening, that they needed to rethink their identity, then make changes and seek help while resetting goals for the future. The participants had mixed feedback on their interaction with health professionals. It appeared if they were given time in appointments, felt that they were heard as well as if the health professional did not define them by their diagnosis, was experienced, was willing to seek out information, kept them informed and respected their knowledge about them, that their experiences were more positive. The authors emphasised the importance of health professionals understanding the ageing needs of adults with CP and respecting the knowledge produced by the lived experience.

References

Aisen, M. L., Kerkovich, D., Mast, J., Mulroy, S., Wren, T. AL, Kay, R. M., & Rethlefsen, S. A. (2011). Cerebral palsy: clinical care and neurological rehabilitation. The Lancet Neurology, 10(9), 844–852. https://doi.org/10.1016/S1474-4422(11)70176-4

Benner, J. L., Hilberink, S. R., Veenis, T., Stam, H. J., van der Slot, W. M., & Roebroeck, M. E. (2017). Long-Term Deterioration of Perceived Health and Functioning in Adults With Cerebral Palsy. Archives of Physical Medicine and Rehabilitation, 98(11), 2196–2205.e1. https://doi.org/10.1016/j.apmr.2017.03.013

Field, B., Scheinberg, A., & Cruickshank, A. (2010). Health care services for adults with cerebral palsy. Australian Family Physician, 39(3), 165–167. Retrieved from https://search.proquest.com/openview/45d09d1d3a44e844781467a46c8b38f0/1?pq-origsite=gscholar&cbl=33668

Furukawa, A., Iwatsuki, H., Nishiyama, M., Nii, E., & Uchida, A. (2001). A Study on the Subjective Well-Being of Adult Patients with Cerebral Palsy. Journal of Physical Therapy Science, 13(1), 31–35. https://doi.org/10.1589/jpts.13.31

Gajdosik, C. G., & Cicirello, N. (2002). Secondary Conditions of the Musculoskeletal System in Adolescents and Adults with Cerebral Palsy. Physical & Occupational Therapy In Pediatrics, 21(4), 49–68. https://doi.org/10.1080/J006v21n04_04

Hahn, J. E. (2012). Minimizing Health Risks Among Older Adults With Intellectual and/or Developmental Disabilities: Clinical Considerations to Promote Quality of Life. Journal of Gerontological Nursing, 38(6), 11–17. https://doi.org/10.3928/00989134-20120510-01

Havercamp, S. M., Scandlin, D., & Roth, M. (2004). Health Disparities among Adults with Developmental Disabilities, Adults with other Disabilities, and Adults Not Reporting Disability in North Carolina. Public Health Reports, 119(4), 418–426. https://doi.org/10.1016/j.phr.2004.05.006

Horsman, M., Suto, M., Dudgeon, B., & Harris, S. R. (2010). Ageing with cerebral palsy: psychosocial issues. Age and Ageing, 39(3), 294–299. https://doi.org/10.1093/ageing/afq018

Kemp, B. J. (2005). What the rehabilitation professional and the consumer need to know. Physical Medicine and Rehabilitation Clinics of North America, 16(1), 1–18, vii. https://doi.org/10.1016/j.pmr.2004.06.009

Morgan, P., Pogrebnoy, D., & McDonald, R. (2014). Health service experiences to address mobility decline in ambulant adults ageing with cerebral palsy. Journal of Intellectual & Developmental Disability, 39(3), 282–289. https://doi.org/10.3109/13668250.2014.927841

Morgan, P. E., Soh, S.-E., & McGinley, J. L. (2014). Health-related quality of life of ambulant adults with cerebral palsy and its association with falls and mobility decline: a preliminary cross sectional study. Health and Quality of Life Outcomes, 12(1), 132. https://doi.org/10.1186/s12955-014-0132-1

Mudge, S., Rosie, J., Stott, S., Taylor, D., Signal, N., McPherson, K. (2016). Ageing wtih cerebral palsy, what are the health experiences of adults with cerebral palsy? A qualitative study. BMJ Open, 6 (10). https://doi:10.1136/bmjopen-2016-012551

Murphy, K. P. (2010). The adult with cerebral palsy. The Orthopedic Clinics of North America, 41(4), 595–605. https://doi.org/10.1016/j.ocl.2010.06.007

MURPHY, K. P. (2009). Cerebral palsy lifetime care - four musculoskeletal conditions. Developmental Medicine & Child Neurology, 51, 30–37. https://doi.org/10.1111/j.1469-8749.2009.03431.x

Murphy, K. P., Sobus, K., & Michael Bliss, P. (2009). The adult with cerebral palsy: a provider-consumer perspective. Physical Medicine and Rehabilitation Clinics of North America, 20(3), 509–522. https://doi.org/10.1016/j.pmr.2009.06.009

Ng, S., Dinesh, S., Tay, S., & Lee, E. (2003). Decreased Access to Health Care and Social Isolation among Young Adults with Cerebral Palsy after Leaving School. Journal of Orthopaedic Surgery, 11(1), 80–89. https://doi.org/10.1177/230949900301100116

Pruitt, D. W., & Tsai, T. (2009). Common medical comorbidities associated with cerebral palsy. Physical Medicine and Rehabilitation Clinics of North America, 20(3), 453–467. https://doi.org/10.1016/j.pmr.2009.06.002

Rapp, C. E., & Torres, M. M. (2000). The adult with cerebral palsy. Archives of Family Medicine, 9(5), 466–472. https://doi.org/10.1001/ARCHFAMI.9.5.466

Rimmer, J. H. (1999). Health Promotion for People With Disabilities: The Emerging Paradigm Shift From Disability Prevention to Prevention of Secondary Conditions. Physical Therapy, 79(5), 495–502. https://doi.org/10.1093/ptj/79.5.495

Rosenbaum, P. (2003). Cerebral palsy: what parents and doctors want to know. BMJ (Clinical Research Ed.), 326(7396), 970–974. https://doi.org/10.1136/bmj.326.7396.970

Sandström, K. (2007). The lived body — experiences from adults with cerebral palsy. Clinical Rehabilitation, 21(5), 432–441. https://doi.org/10.1177/0269215507073489

Strauss, D., Brooks, J., Rosenbloom, L., & Shavelle, R. (2008). Life expectancy in cerebral palsy: an update. Developmental Medicine & Child Neurology, 50(7), 487–493. https://doi.org/10.1111/j.1469-8749.2008.03000.x

Svien, L. R., Berg, P., & Stephenson, C. (2008). Issues in Aging With Cerebral Palsy. Topics in Geriatric Rehabilitation, 24(1), 26–40. https://doi.org/10.1097/01.TGR.0000311404.24426.45

TURK, M. A. (2009). Health, mortality, and wellness issues in adults with cerebral palsy. Developmental Medicine & Child Neurology, 51, 24–29. https://doi.org/10.1111/j.1469-8749.2009.03429.x