Living with people with childhood disabilities: making the world more accessible, inclusive, and sustainable

Abstract

Introduction

When a person with a disability participates in an academic meeting of multidisciplinary professionals in the field of childhood disability, he/she can stimulate these experts’ debates and develop accessible social attitudes toward persons with disabilities.

Disabled initiative

Dr. Lauro S. Halstead and Dr. Nancy Frick, both polio survivor physicians, identified the Post-polio syndrome (PPS) and its connection to a specific personal behavioral type contributing to finding better solutions for the vicious circle of these conditions. Similar to PPS, one of the authors, Kiyoharu Shiraishi, a Japanese activist with cerebral palsy (CP) and his colleagues conducted a large survey on adults with CP.

Findings

From our research results, the people with CP who got the competitive job position in younger days might be at the high risk of functional deterioration such as need for neck surgery. They also tend to delay the use of a power mobility than any other people with CP in different lifestyle. Power mobility appeared to be the optimal choice of mobility for individuals living with CP in a lifelong perspective.

Conclusion

This is where researchers with childhood disabilities, such as CP, can impact our society as ‘experts’ at being lifelong disabled, inspiring professionals without disabilities.

Categories and Subject Descriptors

A.0 GENERAL. [General literary works]: Description of social context on disability and aging society.

General Terms

The theory for management of aging with disability.

• disability
• lifelong perspective
• inclusion
• overuse
• cerebral palsy

In the field of childhood disability, most professionals have focused on childhood intervention. In December 2006, Masayo's letter of complaint was published in the European Academy of Childhood Disability (EACD) newsletter together with a reply from the chair, Martin Bax. Several reader comments were also posted in the following issue. Masayo's complaint was basically regarding an accessibility issue during the meeting.

She wrote ‘I know many professionals think that “independent walking” is one of the most important goals of children with childhood disability. I could walk independently in general community in my younger days. I have walked outside of home every day until age of 37 (from 10). Now I could not walk at all due to the non-existence of preventing deterioration and over use of my body including by walking’.

She stated “This is reality of disabled today.”

She ended the letter as follows “We could focus more on the long term effect of habilitation the children receives such as preventing deteriorations and studying adults with CP. Let's get together to find out the next option toward positive future of today's children!!” (1)

The letter was basically about accessibility; however, in the following year's meeting, there were more research papers regarding adult outcomes or aging issues. During the 18th EACD annual meeting at Barcelona, only three papers focused on adults with CP out of a total of 205 papers (2) The next 19th EACD annual meeting at Groningen, the Netherlands, 18 papers regarding the issues of adulthood were accepted and presented (3) EACD made a difference not only in accessibility of the conference venue and social activities but also with a lifespan approach and inclusive discussions. Participation of researchers with childhood disability can inspire multidisciplinary professionals, or provide them with a closer view of disabilities.

Post-polio syndrome

Post-polio syndrome (PPS) is one of the examples where persons with childhood disabilities themselves played an important role in identifying the condition, including Dr. Lauro S. Halstead at the National Rehabilitation Hospital. The results of the 1985 National Survey of 676 polio survivors indicated that 86% of all survivors stated unexpected secondary conditions, whereas 72% of all survivors did not think the symptoms were related to their polio (4).

The poliomyelitis virus damages motor neurons, and the surviving motor neurons work very hard to cover the muscles’ disrupted neurons control. That was ‘Recovery from Polio’ or ‘Overcome Polio’. With the aging of these motor units, the overworked neurons fail to maintain axonal sprouting and are disrupted, resulting in fatigue and new muscle weakness.

Polio survivors work more hours of overtime and have more Type-A behavior patterns. It was originally described by Friedman and Rosenman who related it to coronary heart disease (5). A person with Type-A behavior who behaves in this pattern is said to be hard-driven, pressured, time-conscious, perfectionistic, and more overachieving than any other group with or with out disabilities. Polio survivors, regardless of the severity of their disability, are often the leaders, movers, and shakers of our communities. What's wrong with being a hard-driving, Type-A community leader in a wheelchair?

Dr. Nancy Frick, polio survivor and executive director of Harvest Center in Hackensack, New Jersey, stated that Type-A behavior is literally burning polio survivors out. ‘Our surveys show that the more Type-A polio survivors are, the more likely it is they will have PPS—late-onset fatigue, weakness, muscle and joint pain and the more severe their new symptoms will be’. What's worse, being Type-A prevents polio survivors from getting treatment for their PPS. Even though polio survivors realize that burning the candle at both ends is causing pain and loss of function, they just can't slow down. Polio survivors will ignore even excruciating pain for years to avoid ‘wasting time’ by going to the doctor. So for many polio survivors, pain doesn't matter. All that matters is what they ‘do’ (6). Dr. Frick and her team have developed the scientific approach for these behaviors.

Another infantile paralysis

Poliomyelitis is another name for spinal infantile paralysis, or just infantile paralysis, and another infantile paralysis is cerebral palsy (CP). Regarding how childhood disabilities impact on individual personality, we will expand this argument to children with CP. Most parents would never expect their babies to have a disability, specifically CP. Many members of the Green Grass, a Japanese CP activist group (7), had parents confessing to them that more than once they had considered killing their child with CP, as well as themselves, after the child was diagnosed with CP. Parents strongly wanted their baby to become able-bodied. Parents dreamt of their children ‘recovering’ to the point of having almost no disability or limitations such as polio survivors. Parents searched for cures so that their children could make perfect recoveries. Parents believed medical, therapeutic ‘special’ treatment must be crucial for the child with CP, instead of a warm family relationship in the early years of life which is a source of emotional development, self-confidence, and positive attitudes. Some health professionals had promised (or pretend to be able) recovery to able-body and failed. ‘Special place for becoming able-body’ was a goal of the children's hospital in those days. As a result, ambulatory people developed their Type-A personality with a hard-drive attitude for ‘physical independence’, which was a goal of the children's hospital in those days. After all, some people with CP straggled with serious consequences after prolonged physical overuse. That is the reason why Japanese CP activists were so devoted to their health issues for such a long time (over 20 years). By 1985, a total of 148 adults with CP participated in a survey of/by adults with CP (8, 9) They found that walking is lost to 30%; one group stopped in their 40s because of pain; those who continued to walk, distance was compromised (60%), walk speed decreased (70%), became unstable (56%). Seventy-five percent maintained feeding capability. Forty percent needed more time at meals. Thirty-two percent felt fatigue during meals. They also found that musculoskeletal pain was common (85%) in the 30s and the 40s age group. Men reported upper body pain more than women. One third of the population experienced predominant pain from age 20. Most of people who had pain were ambulatory or used to be ambulatory.

Is CP a stable condition?

Robert Palisano and Can-Child people developed the Gross Motor Function Classification System (GMFCS). GMFCS is a five-level, ordinal rating scale, ranging from I (community ambulator), II (limited outdoor ambulator), III (walking with assistive devices) IV (self-mobility with limitations, use power mobility outdoors) to V (self-mobility is severely limited). Targeted at children with CP by age 12 (10). We presented the GMFCS as a useful tool to detect adult deterioration at the Annual Congress of American Academy for Cerebral Palsy and Developmental Medicine (AAPDM) 2005 (11). In 2006, Reiden Jahnsen, a Norwegian researcher proved that a self-reported level of the GMFCS in the adults with CP is reliable, comparing professional's evaluation (12). Her works enable us to investigate GMFCS retrospectively in this lifelong research.

We conducted a survey using peer interviews, people with CP gathered information and life histories from consenting participants. Individuals with CP over the age of 36 living in the community were recruited. Seventy-five participants (mean age 50.1 [SD 7.5], male 58.7%) were recruited through support organizations for people with disabilities in Japan. Regardless of their GMFCS level in their younger days, 71% of them used a power chair as their mode of personal mobility at the time of the investigation (13).

Variables included in the data collected included: age at time of cervical surgery, occupational status at age 19, functional variables (Barthel index), and Craig Handicap Assessment and Reporting Technique (CHART) social integration subscale (14).

Based on occupational status at 19 years, we categorized participants as follows: competitive worker group was 24% (n=18); students, sheltered workshop workers, or homemakers group was 26.7% (n=20); and no occupation group was 49.3% (n=37).

Risk for cervical surgery

Adverse event of cervical surgery for secondary problems were compared between groups. After evaluation of cumulative incidence by the Kaplan-Meier survival curve, Log rank test were conducted to compare risk-adjusted event rates. Increased risk ratio and its 95% confidential intervals were calculated by Cox relative hazard model. Among groups, CHART scores and age when power mobility was first used were analyzed by ANOVA and Sheffe's post-hoc test.

Fifteen participants (20%) had histories of cervical surgery; ‘competitive job participants’ received this surgery at a younger age than the ‘no job participants’ (p<0.05); Risk ratio of surgery for the ‘competitive job participants’ was 4.648 (95% CI; 1.229–17.570) when compared to the ‘no job group’. People working in a competitive position were in a stressful situation. Most of them had worked in physically demanding, heavy labor jobs and were more hard-driven, pressured, time-conscious, perfectionistic, and overachieving than any other peers with or without disabilities. The competitive group had not required any assistance at all in activities of daily living in those aged 19 years. People who once had a competitive job did not feel comfortable to ‘depend’ on power mobility until the later stage of developing a secondary condition. In addition, government supports for assistive technology were still a medical model, strictly limited to compensating for lost limbs or perfect loss of functions. If you can use your hand for wheelchair propulsion, you will not be eligible to get a funding for power chair but only for a manual chair (usually ‘DEPO chair’).

Vocational rehabilitation needs a lifelong perspective

Due to their acquired Type-A behaviors, people who got a competitive job at 19 years delayed mobility transition to power mobility. The competitive job group showed the lowest mean score of CHART social score at the time of investigation as a consequence of their restricted personal life. Due to our recruitment, selective bias may have been introduced. Due to our retrospective design, recall bias may also have been introduced. Without being experimental in design, our results cannot directly confirm the causality between developing functional limitation and power mobility.

Coexisting factors such as manual dexterity and secondary problems can correlate the development of functional limitation. Other potential confounders may also exist. Evidence of assistive technology such as secondary condition prevention, changes in people's attitude toward power mobility, environmental barriers, government support and policies should all be addressed to improve the social outcome of adults with CP facing secondary conditions. The cervical spinal complications developing in the long-term might disrupt functional performance and social participation over time.

Surprisingly, the people once got the competitive position experienced the most restricted participation, and delayed the use of power mobility more than any others. Power mobility appeared to be the optimal choice in daily mobility for individuals living with CP from a lifelong perspective.

Discussion

Too much stress on ‘physical independence’ and ‘competitive jobs position’ without reasonable accommodation would lead people to secondary conditions or other health problems by developing Type-A behavior sooner or later. Alternative strategies to conserve their attained functions are crucial from a lifelong perspective. For example, from our recent results, ambulatory people with CP can be candidates for power mobility use, because they might depend on power mobility for daily outdoor mobility sooner or later. Familiar with assistive technology in the early stages of developing functional deterioration will help them to make the transition more easily, when the time comes. Also, those our findings and previously mentioned works of polio surviving researchers both point out the importance of lifelong perspective when facing childhood disabilities such as CP and polio.

The United Nation's Convention of the Rights for Persons with Disabilities marks a paradigm shift in attitudes and approaches to persons with disabilities. The convention gives universal recognition to the dignity of persons with disabilities. Persons with disabilities are not viewed as ‘objects’ of charity, medical treatment, and social protection, rather as ‘subjects’ with rights, who are capable of claiming those rights and making decisions for their lives based on their free and informed consent as well as being active members of society (15).

Conclusion

In the field of disability research and development, researchers with childhood disabilities have been playing a crucial role as pioneers, which has deeply stimulated professionals and experts, for making world more accessible, inclusive, and sustainable.

Conflicts of interest and funding

The authors have not received any funding or benefits from industry or elsewhere to conduct this study.