Children with complex medical and genetic conditions are living longer than was predicted in the past due to better treatments and overall care (1). According to 2000 nationwide census data, approximately 6% or 2.6 million children and youth aged 5 to 15 years have a disability. Currently, 90% of children born with special health care needs reach adulthood (2). Often, the success of excellent pediatric medicine results in young adults who require ongoing, sometimes intensive, medical supervision throughout their lives.

In 2002, the American Academy of Pediatrics, The American Academy of Family Medicine, and the American College of Physicians—American Society of Internal Medicine collaborated on a policy statement to ensure that young people with special health care needs were equipped to move from child-centered to adult-centered health delivery systems (3). In a report from the Maternal and Child Health Bureau (MCHB), published in 2001, three general barriers were identified to health care transitions for young adults: training of qualified providers, funding for health delivery, and bureaucratic obstacles.

In reality, the process of transition should be viewed as a continuum. There are many barriers that challenge families, patients, and physicians alike that greatly complicate the process. The physician receiving transitioning youth must have an understanding of what some of these barriers entail in order to deliver compassionate care to young adults attempting to make the leap into the adult health care system.

The Medical Home model has been viewed as having primary responsibility for taking charge of transition activities to ensure that young adults with chronic, childhood-onset illness continue to receive the quality of health care that characterized their pediatric years. Thus, the role and responsibility of a medical home has continued to expand with ideals that are difficult to attain without a thorough understanding of the team approach implied in the concept.

The MCHB defines children and youth with special health care needs (CYSHCN) as “those who have, or are at increased risk for having, a chronic physical, developmental, behavioral or emotional condition and who also require health and related services of a type or amount beyond that required by children generally” (4). In 1989, The Omnibus Budget Reconciliation Act (OBRA), Public Law 101-239, amended Title V of the Social Security Act to include the authority and responsibility of MCHB to fully address the needs of all children. In addition, the focus under MCHB for CYSHCN was to provide leadership in promoting a community-based system of services that is family centered, comprehensive, coordinated, and culturally competent, thus building services around a medical home approach. The goals for the Title V CSHCN Program include:

While not all these goals will be the direct responsibility of one clinician, the overall success of medical care delivered in a
chronic care model is hinged on the management of chronic conditions that encompass each of the six goals above.

The Medical Home model addresses barriers to health care access and provides a framework to operationalize efforts to improve health care (see below). The patient and the family are at the center of the defining statement rather than a specific clinician’s office. Each of the seven components of a medical home becomes linked to some extent to every medical provider of care for the patient with special health care needs as they manage chronic diseases. Familiarity with these components is the first step toward increasing the medical home approach in specialty care.

The components of the Medical Home Initiative (5) include:

Access to care refers to whether the patient can receive the health care services they need when they need it, even outside regular business hours and includes having a way to pay for needed services. Family-centered care is health care that takes into consideration the requests of the family and respects them as partners in the health care decision making of the medical home concept. Cultural responsiveness considers the values and beliefs that the family and the patient bring with them into the medical encounter. Continuity of care implies communication among all health care providers, especially primary care physicians and specialists. Comprehensive care is medical care that includes primary medical and developmentally appropriate care, dental care, mental health services, as well as crisis and chronic illness management. Application of routine primary care guidelines is also a component of comprehensive care. Compassionate care considers the concerns of the family and how the diagnosis of the patient may affect others. Coordination of care embodies the day-to-day development of a health care plan, including the transition plan, for a patient and ensures its implementation.

With the medical home concept at the hub of chronic illness care, rehabilitation professionals will be challenged to provide leadership in long-term follow-up of our patients. Preserving wellness and independence is the ultimate goal, not necessarily episodic/acute care management. Preservation of quality-of-life indicators, as individually applied to each patient, while postponing functional decline is an important role.

As young adults with disabilities transition to more independence, physicians need to educate patients and their families about potential age-related changes and medical issues. There is a growing interest in the literature about aging issues and secondary conditions among persons with congenital and childhood-onset disabling conditions. Differentiating between primary, associated, and secondary conditions may help the clinician categorically organize a patient’s care goals.

The primary condition is self-explanatory. It is the diagnosis that initiated entering into medical care from the point of evaluation during gestation, infancy, or childhood. Secondary conditions are impairments, functional limitations, disabilities, diseases, injuries, or other conditions that occur during the life of a person with a disability, in which the primary disabling condition is a risk factor for that secondary condition or may alter the standard intervention for prevention of treatment of any health condition (Syracuse Conference, 1994). A disabling condition, such as a spinal cord injury (SCI) or cerebral palsy (CP), is not synonymous with illness or disease. People with disabilities are generally healthy, and one should not expect a decline in health and function as they mature. Therefore, physicians must have an index of suspicion for such conditions that may have onset in late adolescence or adulthood or be insidiously progressive. They may include progression of pathology or impairment, either through complications or through the aging process. Commonly reported secondary conditions include pain, contractures, recurrent urinary tract infections, pressure sores, and osteoporosis. Associated conditions or residual impairments are conditions that result from the defect, injury, or disease and often may be considered primary impairments depending on their severity. An example is neurogenic bowel/bladder in patients with spina bifida. Comorbidities are medical conditions unrelated to the primary disabling conditions such as adult-onset diabetes or hypertension in a patient with CP.

Health perception is an individual determination and is affected by personal expectations, experiences, sense of vulnerability, support, and locale. The World Health Organization (WHO) defines quality of life (QoL) as “an individual’s perception of their position in life in the context of the culture and value systems in which they live, in relation to their goals, expectations, standards, and concerns” (6). Health-related QoL is a broad concept affected in a complex way by an individual’s physical health, psychological state, personal beliefs, social relationships, and their relationship to salient features of their environment. Although it is widely acknowledged that this is an area of concern for adults with chronic disabilities, there are limited studies that have systematically assessed QoL in these individuals, particularly those with neuromuscular disorders (7). This may be due in part to the fact that QoL is perceived as a somewhat vague entity that many people are concerned about, but something that nobody clearly knows what to do about. These issues are important in examining disability in adults who are survivors of disabling childhood disorders.

What follows is an overview of common chronic conditions and specific management recommendations for care.