Transition to Adulthood for Persons with Childhood Onset Disabilities

Author(s): Sarah Korth, MD, Christina Kokorelis, MD

Originally published:11/10/2011

Last updated:08/16/2016

1. DISEASE/DISORDER:

Definition

A unique element of childhood is the process of growth and development into independent adults. Children with disabilities face additional challenges related to their specific condition. There is currently no universally accepted age cut-off for transitioning to adult services, but the importance of this transition is increasingly being recognized.4

Etiology

With advances in medical and surgical care, more children with disabilities are now surviving into adulthood; consequently, there is an emerging need to manage these disabilities and their associated challenges into adulthood.13

Epidemiology including risk factors and primary prevention

Ninety percent of children with chronic disability will now survive into adulthood.3 Nevertheless, more than half of patients with chronic health conditions report inadequate support and services during their transition to adult healthcare.1

Patho-anatomy/physiology

Adolescence presents with spurts in linear growth, hormonal changes, and sexual maturation. With these changes arise increased expectations of independence, development of individual identity, and greater emphasis on education, employment, and sexuality. As a result, there are also alterations in family and social dynamics. This transition will ultimately be influenced by the type and scope of disability, degree of impairment, family dynamics, and social milieu.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

Adolescence has 3 stages: Early, Middle and Late

Table 1. Normal Developmental Progress in Adolescence

Sphere of Development Early Adolescence Middle Adolescence Late Adolescence
Social Close friendships gain importance.

Peer group influences interests and clothing styles.

Effort to make new friends.

Strong emphasis on the new peer group with the group identity of selectivity, superiority, and competitiveness.

Ability to compromise. Self-reliance.

Greater concern for others.

Emotional Moodiness.

Less attention shown to parents, with occasional rudeness. Rule and limit testing. Occasional experimentation with cigarettes, marijuana, and alcohol.

Self-involvement, alternating between unrealistically high expectation and poor self-concept. Complaints that parents interfere with independence. Extremely concerned with appearance and with one’s own body. Feelings of strangeness about one’s self and body. Lowered opinion of parents, withdrawal of emotions from them. Periods of sadness as the psychological loss of the parents takes place. Firmer identity. Ability to delay gratification. More developed sense of humor. Greater emotional stability. Stable interests. Pride in one’s work.
Intellectual Struggle with sense of identity. Capacity for abstract thought. Improved abilities to use speech to express oneself, but more likely to express feelings by action than by words. Close to full achievement of adult verbal skills; still developing written skills. Examination of inner experiences, which may include writing a diary. Ability to think ideas through. Ability to make independent decisions. Ability to express ideas in words; full achievement of adult verbal skills; close to full achievement of adult written skills.
Physical Further increases in body strength with beginnings of adult muscle development. Close to full achievement of their adult strength and skills. Full achievement of their adult strength and skills.
Sexual Pubic hair develops. Scrotum begins to enlarge in boys. Breast development begins in girls. Most girls reach menarche by age 14. Pubic hair fills in. Penis enlarges and further scrotal enlargement occurs in boys. Breast enlargement occurs in girls. Adult sexual characteristics (although some boys may not complete maturation until age 21 or 22 years).

Specific secondary or associated conditions and complications

The adolescent’s family structure, its socioeconomic status and geographic location, the availability and type of school services, and the availability and depth of vocational rehabilitation resources will influence the process of transition.

Barriers to a smooth transition include:

  • Inadequate preparation of the patient and/or family prior to transition
  • Inadequate communication between the pediatric and adult providers
  • Inability to access and use adult care services
  • Excessive parental involvement in the care of patients
  • Reluctance of some pediatric providers to relinquish care of the patient

2. ESSENTIALS OF ASSESSMENT

History

Successful transitions begin with addressing the issue during preadolescence. In all but the most disabled children, there is a possibility of promoting some independence and autonomy. Parents/caregivers should be asked about how they are planning for the child’s transition to adulthood. Children should be asked (as they age, possibly without their parents’ presence) about long-term goals, hopes, and relationships with family and peers.

Physical Examination

  • Height and weight, observing particularly for weight gain;
  • Physical development using the Tanner scale;
  • Joint range of motion, especially in conditions where patients are at risk for contractures; rapid growth can exacerbate contractures.
  • Balance, as changes in center of gravity occur with growth.

Functional assessment

  • Functional history from both the parent/caregiver and the adolescent to check for differences in perception. Parents may tend to shelter a child with a disability and may be providing unnecessary assistance in areas where the adolescent has the potential to develop independence.
  • It is important to assess behavioral interactions between the patient and caregiver/parents and, if possible, between the examiner and patient without the parent/caregiver present.
  • It is valuable to establish an accurate GMFCS level prior to transition. Gait function may be further classified using the Functional Mobility Scale (FMS), which is sensitive to change following intervention and natural history. These measurements may increase consistency between providers by offering a basis for comparison.

Laboratory studies

If a disorder of growth or maturation is suspected, endocrine studies including growth hormone, testosterone, and estrogen levels may be considered and/or consult a pediatric endocrinologist.

Imaging

Imaging will be dependent on the specific diagnosis, disability, and other clinical findings.

Supplemental assessment tools

The American Medical Association’s Department of Adolescent Health has developed Guidelines for Adolescent Preventive Services (GAPS). GAPS has developed questionnaires, which address many of the areas relevant to adolescent transition.

Early predictions of outcomes

Table 2: Factors that positively influence the success of transition from child to adult-oriented healthcare

The Patient The Family Pediatric Provider Adult Provider
Independent Behavior.

Higher education.

Greater cognition.

Increased mobility and activities of daily living.

 

Devotion to caring for the child.

Good understanding of disease process.

Financial resources.

Invested in transitioning care.

Familiarity with disease process.

Able to recognize limits of own scope of practice.

 

Able to recognize limits of own scope of practice.

Emphasis placed on receiving thorough communication from pediatric provider.

 

Table 3: Factors that negatively influence the success of transition from child to adult-oriented healthcare

The Patient The Family Pediatric Caregivers Adult Caregivers
Immaturity.

Severe Disability.

Psychopathology.

Poor support system.

Decreased trust in caregivers.

Poor adherence to treatment.

Excessive need for control.

Extreme dependence on provider.

Psychopathology.

Parenting style leading to overprotection.

Decreased trust in caregivers.

 

Reluctance to give up caring for patient.

Perception of short survival of patient.

Ambivalence towards transition and transfer of care.

Lack of familiarity managing rare, congenital diseases. Lack of familiarity managing childhood conditions.

 

Environmental

Some studies show that low socioeconomic and/or minority status does not influence the ability to achieve adult roles. However, these issues should still be considered when planning and obtaining services for adolescents with disabilities. Use of Internet resources (e.g., support groups, informational sites) should be encouraged, particularly for persons living in geographically isolated areas.

Social role and social support system

Support for both the adolescent and his/her caregivers should include peer support (e.g., local or national patient advocacy groups), rehabilitation social workers and, as indicated, rehabilitation psychologists.

Professional Issues

  • Guardianship for adolescents with disabilities who lack decision-making capacity and who are older than 18 can become complicated, especially in situations in which the adolescent’s parents are older and becoming dependent themselves. Consultation with an eldercare/disability lawyer may be beneficial.
  • Many adolescents with disabilities may qualify for programs such as vocational rehabilitation and social security disability, and thus should be encouraged to apply. Resources vary by state.
  • For non-ambulatory children with severe physical and cognitive impairments, reduction in size can be accomplished through growth attenuation therapy (GAT). Although somewhat controversial, this therapy is no longer rare. However, more data is needed to assess its long-term side effects and overall improvement in quality of life for both the patient and caregiver.8

3. REHABILITATION MANAGEMENT AND TREATMENTS

Available or current treatment guidelines

Clinicians are encouraged to check disease-specific guidelines.

At different disease stages:

Early adolescence

  • Caregivers should begin to think about the process of transition through adolescence and to prepare a plan for addressing issues of sexuality (both physical and emotional), increasing independence, and determining academic goals. They need to consider various training options including mainstream post-secondary education, vocational training, supported employment, and sheltered workshops.
  • Physiatrists need to monitor for potential secondary impairments cause by growth spurts (e.g., worsening contractures).
  • Adolescents who are able should be encouraged to learn more about their condition and seek support from others with the same/similar disabilities.

Middle adolescence

  • Adolescents who are able should be encouraged to take increasing responsibility for self-care needs and begin to learn the homemaking skills necessary for independent living, possibly necessitating a course of occupational therapy. Adaptive equipment should also be prescribed as needed.
  • Driving assessment and referral to adaptive driving school should be considered.
  • Entry into the state vocational rehabilitation system should be made.
  • In cases where the adolescent is likely to continue to be dependent for care as an adult, the possibility of group home care should be introduced to caregivers. If that option is chosen, the parents should be encouraged to research a suitable organization and have their child placed on the waiting list as soon as possible. Social work referral may help to identify resources.
  • Physiatrists must monitor closely for excessive weight gain and identify appropriate exercise/fitness strategies to prevent obesity. Programs such as I Can Do It, You Can Do It may be useful.

Late adolescence

  • Resources for post-educational plans and social/employment programs should be explored.
  • The resources of organizations such as independent living centers and statewide independent living councils should be investigated.
  • Adolescents should be made aware of their rights under the Americans with Disabilities Act as they seek jobs and housing.
  • The physiatrist should help the family identify a primary care physician who is comfortable treating adults with disabilities. If the physiatrist is unable to see adults, they need to identify a colleague who can assume the person’s rehabilitation management.

Coordination of care

The appropriate setting for provision of care will depend on the specific adolescent. As the severity of cognitive or physical impairment increases, greater coordination of care should be considered. Ideally, the setting should include both pediatric and adult experts to facilitate a smoother transition.

Patient & family education

Patients often feel anxious and concerned at the thought of transitioning to adult care services; in fact, only 21% of parents reported that their child had discussions with an adult health care provider prior to transitioning. Similarly, a parent’s feelings toward the transition is linked to his or her awareness of the transition plan. Thus, it should be a priority of the provider to discuss the transition plan with both the patient and the family prior to transitioning.14

As noted in the sections above, education regarding resources for education, jobs, housing, social programs, and advocacy should also be undertaken at each visit.

Emerging/Unique Interventions

Transition of care should be a process that takes place over time rather than a single event. It is unacceptable to inform a family that they will need to transition to an adult provider without making formal transition arrangements. A structured transition program, involving the child, parents, and both providers, should start years before the formal transfer. The transition process may be improved with visits to the transition clinic before the actual transfer and include a consult with both the pediatric and adult providers. Such arrangements may even include a “graduation visit”, which could include a symbolic token, such as a diploma, to signify the child’s progress and initiation to a new provider. To further facilitate the transition process, it may be beneficial to employ a designated transition coordinator, who would be responsible for organizing the preparations for patients and families prior to and during the transition period, ultimately serving as a conduit between old and new care teams.

4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Cutting edge concepts and practice

Small numbers of physiatrists and primary care physicians (usually trained in family practice but sometimes with dual training and certification in pediatrics and internal medicine) are beginning to take a special interest in treating adults with childhood onset disabilities.

Electronic medical record systems with longitudinal collection of demographics, interventions, and outcomes data may help facilitate the transition from pediatric to adult providers.6 Other options to facilitate this transition include direct 1:1 sign-out between providers and/or obtaining an assistant for the purpose of long-term documentation of the disease process.

Pediatric patients with chronic diseases may benefit from a more staged approach where there is a phased transfer between pediatric subspecialists and primary care providers in a disease-specific manner.1 More specifically, many youth with medically complex diseases have several specialist providers; thus, rather than transition all providers simultaneously, it may be more effective and safer for the patient to transition one provider at a time.

5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE

Gaps in the evidence-based knowledge

There is currently very limited empirical evidence to support a specific approach to the successful transition to adult providers for children with disabilities.

Further research and policy should focus on developing and evaluating coordinated transition interventions involving primary care to better integrate primary and specialized care, rather than focusing solely on specialist-to-specialist provider transition (1).  In this way, the fragmented nature of the primary to specialty care interface could improve.

REFERENCES

  1. Bhawra J et al. Primary Care interventions to improve transition of youth with chronic health conditions from paediatric to adult healthcare: a systemic review. BMJ Open 2016;6:e011871.
  2. Binks J, Barden W, Burke T, Young N. What do we really know about the transition to adult-centered health care? A focus on cerebral palsy and spina bifida. Arch Phys Med Rehabil. 2007;88(8):1064-1073.
  3. Blum, RW. Chronic illness and disability in adolescence. J Adolesc Health. 1992;13(5): 354-368.
  4. Burns, F. et al. The cerebral palsy transition clinic: administrative chore, clinical responsibility, or opportunity for audit and clinical research? J Child Orthop 2014;8:203-213.
  5. Chamberlain M, Kent R. The needs of young people with disabilities in transition from paediatric to adult services. Europa Medicophysica. 2005;41:111-123.
  6. Fairman AD, et al. Implementing a Specialty Electronic Medical Record to Document a Life-Course Developmental Model and Facilitate Clinical Interventions in Spina Bifida Clinics. Pediatr Clin N AM 2010;57:959-971.
  7. Geenen S, Powers L, Sells W. Understanding the role of health care providers during the transition of adolescents with disabilities and special health care needs. J Adolesc Health. 2003;32:225-233.
  8. Pollock AJ, et al. Growth attenuation therapy: practice and perspectives of paediatric endocrinologists. Arch Dis Child. 2015 Dec;100(12): 1185.
  9. Roebroeck M, Jahnsen R, Carona C, Kent R, Chamberlain M. Adult outcomes and lifepan issues for people with childhood-onset physical disability. Dev Med Child Neurol. 2009;51:670-678.
  10. Rosenbaum P, Stewart D. Perspecitives on transitions: rethinking services for children and youth with developmental disabilities. Arch Phys Med Rehabil. 2007;88(8):1090-1082.
  11. Stewart D. Transition to adult services for young people with disabilities: current evidence to guide future research. Dev Med Child Neurol. 2009;51(suppl 4):169-173.
  12. Van Naarden Braun K, Yeargin-Allsopp M, Lollar D. A multi-dimensional approach to the transition of children with developemental disabilities into young adulthood: the acquisition of adult social roles. Disability and Rehabilitation. 2006;28(15):915-928.
  13. Westwood A, et al. Transition from child to adult-oriented care for children with long-term health conditions: A process, not an event. SAMJ April 2014, Vol. 104, No. 4.
  14. Zhou H, et al. Transition adolescent and young adults with chronic disease and/or disabilities from paediatric to adult care services-an integrative review. Journal of Clinical Nursing 2016:10.1111.

Original Version of the Topic:

Scott Paul, MD. Transition to Adulthood for Persons with Childhood Onset Disabilities. Publication Date:2011/11/10.

Author Disclosure

Sarah Korth, MD
Nothing to Disclose

Christina Kokorelis, MD
Nothing to Disclose

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