Because children with chronic conditions, such as spina bifida, have grown up into adults in increasing numbers, they and their families have increasingly questioned whether they have reached their full potential and maximized their participation in adult activities. Lack of knowledgeable adult medical providers and longitudinal data about natural history places more responsibility on individuals and their family for self-care of the impairment. This article describes the need for the life course model, which merges several concepts and principles related to children with disabilities and provides a framework for services and research to achieve the desired adult outcomes.
In previous generations, people with conditions such as spina bifida (SB), sickle cell disease, cystic fibrosis, and muscular dystrophy were not expected to live very far, if at all, into adulthood. If these people did live, health care professionals and other providers were pleased that they had even lived that long. Because children with chronic conditions have grown up into adults in increasing numbers, they and their families have increasingly questioned whether they have reached their full potential and maximized their participation in adult activities. When standards of care and relevant outcomes are expressed in traditional medical parameters (mortality, morbidity, occurrence of secondary health conditions involving other organ systems), scant attention was paid to the functional outcomes that are the norm for typical young adults, including whether the young adult had a job or sufficient self-directed income, had friendships and relationships, lived independently, and engaged in recreation and other health-promoting activities. Expectations were often low to nonexistent about this group’s potential to function well in these domains of adulthood.
Pediatric chronic disease specialists are increasingly recognizing the shortcomings in the quality of life of their patients as they reach adulthood. As patients leave pediatric care, their doctors are moving beyond the issue of a simple transfer of health care to adult doctors to feeling a responsibility and an interest in functional outcomes, in terms of full participation in adult living.
The disability movement of the 1990s, reinforced by the Americans with Disabilities Act and other legislations and policies, has empowered people with disabilities to view themselves differently. These people now aspire to the full quality of life as demonstrated by their typical peers and portrayed in the media. With aspirations, comes an expectation that the systems of services and support will prepare them for these enhanced adult roles.
Transfer of health services
Because of the lack of adult health care providers with expertise in SB, many young people struggle to find health services. Many patients stay with their pediatric provider well into their adulthood, reflecting the trend seen in other childhood-onset lifelong conditions. Indeed, Boston Children’s Hospital now allows patients up to 35 years of age to attend certain clinics run by their pediatric staff. Still, as children with SB reach adulthood, most of them lose access to health care providers who have knowledge about SB. This situation places an increasing responsibility on the patients to understand their own condition, the pattern of symptoms unique to them, the self-management of symptoms that works, and the threshold for seeking medical attention. Shunt function typifies this situation. Young adult patients have a long experience with possibly shunt-related symptoms, such as headache and fever. Ideally, these patients would be able to make measured decisions on what intensity and duration of symptoms can be self-managed and when to seek care for possible shunt malfunction. Management of urinary symptoms is a similar situation. This enhanced self-management would be desirable if the patient has access to experienced adult health care providers but is even more critical when the access is limited. Covering these eventualities requires preparation of the young patient with SB, starting in early childhood.
The Life Course Model merges several concepts and principles related to children with disabilities and provides a framework for services and research to achieve the desired adult outcomes ( Fig. 1 ).
Normal child development
Children are dynamic in their progress from infancy to adulthood. Their development follows a series of predictable stages or milestones in each of their domains of development. An important principle is that stages in development generally have to follow a sequence, that is, important stages cannot be skipped to move on to future stages. One cannot walk until one learns to stand upright. This principle applies to motor milestones as well as language, cognitive, emotional, and behavioral milestones. The sequence of development may be tighter and more predictable in the preschool years but still needs to be followed in school years and adolescence.
If a critical milestone is missed, it may be difficult to go back and relearn that stage. For instance, children who fail to resolve their struggle for autonomy at ages 2 to 3 years may struggle with autonomy in their teen years and may possibly have trouble with authority and self-control throughout their lives.
International classification of functioning, disability and health
The International Classification of Functioning, Disability and Health (ICF) was developed as the latest addition to the family of international classification systems. Thinking that the previous International Classification of Diseases codes were heavily medical and deficit-oriented, experts proposed a new system of classification oriented to function and participation rather than disease and limitations. The ICF, proposed in 1992 and finalized in 2001, is based on the principles that (1) activities and participation are the desired outcomes that should be monitored and measured and (2) these outcomes are determined by an interaction between impairments (of a body part or system) and other factors (environmental and personal). These principles suggest that a responsive accommodative environment will lead to a higher degree of function and participation than a less accommodating environment. Environment includes family, community, school, and service systems and encompasses attitudes as well as direct services. Although the details used in the ICF to measure environmental factors and participation outcomes can be cumbersome, the ICF is especially useful in its broad categorization of outcomes, specifically its participation domains or categories. The ICF is a world-recognized classification system that now shapes almost all current discussions of disability-related services and science.
What is most appealing about the ICF in relation to SB is the recognition that a biosocial model is needed to explain the differences in outcomes for persons with SB. A biologic model would suggest that the initial lesion and birth anatomy would greatly determine the outcomes in children with SB. This suggestion may hold true for some of the biologic outcomes (motor function, cognition) that are heavily influenced by lesion level, anatomic brain differences, and shunt malfunctions, which are always followed in a medical setting.
But the differences in functional outcomes of employment, relationships, and emotional well-being are not explained by these biologic inputs. Other factors must be identified to explain the variation in functional outcomes. So the ICF offers 2 concepts:
- •
Participation is the desired outcome.
- •
Participation results from an interaction between the impairment and environmental response.
There are 8 areas of participation defined by the ICF:
- 1.
Interpersonal interactions and relationships
- 2.
Major life areas
- 3.
Self-care (management)
- 4.
Learning and applying knowledge
- 5.
Communication
- 6.
Domestic life
- 7.
Community, social, and civic life
- 8.
General tasks and demands.
The authors’ Transition Working Group focused on adaptations of the first 3 areas of participation in creating the Life Course Model. These areas were thought to be most compatible with a participation theme and a developmental model that would track progress from early childhood to adulthood.
The authors have operationalized the ICF to define optimal adult function as successful participation in the first 3 areas. Certain elements of the unchosen 5 areas were incorporated into the 3 themes chosen for this project.
| ICF | System for Classifying Impairments, Activities, and Participation |
|---|---|
| Impairment | Affected body structure or function (eg, SB, paralysis, cognitive limitation, primary emotional disturbance, sickle cell disease) |
| Activity | Execution of a task or action by an individual (often in a controlled situation, such as home) |
| Participation | Involvement in a life situation (or performing activities in the real world) |
Definitions
Interpersonal interactions and relationships :
Informal social relationships
Entering into relationships with others, such as casual relationships with people living in the same community or residence; with coworkers, students, playmates; or with people from similar backgrounds or professions.
Formal relationships
Creating and maintaining specific relationships in formal settings, such as with employers, professionals, or service providers.
Intimate relationships
Creating and maintaining close or romantic relationships between individuals, such as husband and wife, lovers, or sexual partners.
Major life areas
Education
Work
Economic life
Self-care
Looking after one’s health
Ensuring physical comfort, health, and physical and mental well-being by maintaining a balanced diet and an appropriate level of physical activity, keeping warm or cool, avoiding harms to health, following safe sex practices, including using condoms, and getting immunizations and regular physical examinations.
Toileting
Planning and performing the elimination of human waste (menstruation, urination, and defecation), and cleaning oneself afterwards.
Impairments are the underlying conditions that are inputs into the Life Course Model. People with impairments are not unhealthy or limited in participation. The goal of persons with impairments should be assumed to be the same as for those who are nonimpaired, namely full participation.
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