People with chronic illness face the same transitions. Additional issues arise that most healthy people never encounter, however. Children with chronic illness that stretches into adulthood face the same challenges of adolescence as their healthy counterparts. The challenges are more complex and intertwined with other formidable tasks, such as finding new care providers and institutions. People with sickle cell disease often need help and guidance as they cross life's stream that separates childhood and adulthood.
Patients with sickle cell disease require coordinated care from subspecialists such as cardiologists, endocrinologists, ophthalmologists, gastroenterologists, surgeons, and pulmonologists. Transfer of information is a major problem for anyone with a chronic disease requiring care from multiple providers. This problem is compounded when care is transferred to a different setting as occurs in the shift from pediatric to adult clinics. Some of the subspecialty relationships play out over prolonged periods of time. For instance, endocrinologists treating delayed pubescence must coordinate activities closely with the hematology staff. Problems can develop if a patient's hematology care moves to a different setting while treatment is ongoing with key subspecialty providers.
The consequences of sickle cell disease vary tremendously between patients. Some 18 year-olds lead relatively normal lives, while others are virtually incapacitated by the disease. Complicated patients are most in need of smooth transition. Too often, however, precisely these patients face the greatest challenges with respect to transition. Platt et. al. (Platt, Thorington et al. 1991) showed in a prospective study of over 3,500 patients with sickle cell disease that frequency of pain episodes is a good clinical indicator of disease severity. For patients over age 20, higher frequency of pain episodes tended also to correlate with earlier mortality. Efficient transfer of these patients' care is critical to prevent undue morbidity and mortality.
An extensive study from the Duke Comprehensive Sickle Cell Center showed that after controlling for age and number of painful episodes, the patient's coping style significantly predicts health care contacts, activity, and psychological distress (Gil, Williams et al. 1991). The number of painful episodes correlated positively with age as well as with emergency room and physician office visits. Patients with Active Coping styles (use of multiple cognitive and behavioral strategies) had fewer emergency room visits. Those using Passive Adherence coping styles (reliance on concrete, passive approaches to pain, such as resting, with little flexibility when initial efforts fail to diminish pain) had more emergency room visits, and participated in fewer activities at home and in school.
Negative Thinking (self-statements of fear and anger) was positively correlated with psychological distress (Gil, Thompson, Keith, et. al., 1993). Parentsı coping styles also correlate with childrenıs activity level, distress and coping strategies. By following up on subjects 9 months after the initial assessment, these studies showed that while the coping strategies of younger children and adults are relatively stable, those of adolescents are in flux. For individuals who rely on less adaptive strategies (Passive Adherence or Negative Thinking), adolescence may be a period when these maladaptive styles become entrenched. The authors suggest (Gil, Thompson, Keith, et. al., 1993) "longer-term follow-up studies to trace the changes in coping over time and through transition periods from childhood to adolescence and from adolescence to adulthood". These stresses and changes complicate transition.
The unpredictability and stress of sickle cell crises affects all members of the family. The extent of the psychological impact reported varies between studies. Some show little psychological variation between adjustment in siblings, patients, and little difference from population norms (Gold, Treadwell et al. 1995) while others show significant stress on siblings (Treiber, Mabe et al. 1987). Kramer (Kramer and Nash 1995) found 49% of the parents of sickle cell patients in their sample to be clinically depressed according to the CES-D. Other programs have aimed at improving the education and coping of family members, reducing daily strain, and teaching stress management techniques to parents (Kaslow, Rowland et al. 1995), (Shearer, 1995). Maternal coping and family functioning correlate with patient adjustment and health care utilization (Shearer, 1995). Patients' self-esteem correlates with parental knowledge of sickle cell disease, more available social support for parents, and more adaptive parental coping strategies.
The simmering revolution in health care and health coverage has largely overlooked the needs of patients with chronic illnesses. Few HMOs voluntarily roll patients with sickle cell disease beneath their umbrella. The challenges are even greater with for-profit enterprises. These organizations seek a patient portfolio weighted toward the young and healthy. Patients with chronic illnesses such as sickle cell disease frequently loose medical coverage when they become (legally) independent of their parents.
Integrated transition programs are critically needed to provide age-appropriate treatment plans, so that 30 year-old patients are not treated in pediatric clinics along side six-year olds. The psychosocial challenges of living with a chronic illness must be addressed if each person is to achieve his or her maximum potential irrespective of yardstick. Fear, depression and hopelessness can lead young people to maladaptive options. Equally importantly, society loses the latent talent in these young adults, which is not fully tapped.
Parallel but unconnected health care systems for children and adults with sickle cell disease can lose patients during the transfer process. A number of factors conspire to this end. Perhaps most important is the attachment to a familiar institution and providers. Young people have eidetic memories of pediatric providers to whom they may literally owe their lives. They do not want to go to an adult care facility that they perceive as being cold, uncaring and unfamiliar.
On the adult side of the divide, patients face for the first time a plethora of new responsibilities, including keeping track of appointments and medications. Most young adults need keep track of nothing more serious than appointments for dental hygiene. Compliance failure can lead to caries. Young people with sickle cell disease must remember ophthalmology exams, with the penalty for non-compliance being possible blindness. In the absence of a transition bridge, young people with sickle cell disease often have difficulty crossing the divide.
Patient age should not be an automatic trigger to transition. Some patients will be ready for transition from pediatric to adult care by 18 years of age, while others will not. A patient's developmental age is a more appropriate guide than chronological age. Delay in neurocognitive development due to cerebrovascular injury, for instance, can mean that a patient may not be up to the challenges of an adult care setting until age 20 or 21. Similarly, a patient who has recently recovered from a serious complication of sickle cell disease, such as acute chest syndrome, is unlikely to be psychologically prepared for transition to new providers. Individual assessment is paramount.
Parents invest heavily in the care of their children. Sickle cell disease multiplies that investment and builds a tremendously strong bond between parents and pediatric providers. For parents, transition to adult care entails loss both of responsibility for and control over the young person's medical care. They often have difficulty relinquishing the central role in their child's care. The needs and concerns of parents must be assessed and addressed. The pediatric providers with whom they have a close relationship are positioned to do this best.
Patients and parents should view transition as a positive milestone. Together, they have achieved a significant step and should be congratulated. The pediatric providers should provide coaching and support for as long as possible after transition. The adult providers then become positive additions to an already winning team.
Adult and pediatric providers must meet each other. This simple but crucial step is often difficult with the rising demands of clinical practice. Without this first step, however, attempts at transition are doomed. Pediatric providers are reluctant to send their patients into an unknown environment. This is true even if the recipient institution and providers have excellent reputations. Providers who have worked hard to bring children into adolescence and young adulthood are understandably cautious about delivering them to an ill-defined entity.
Regular meetings between adult and pediatric providers allow the groups to discuss the issues unique to each patient considered for transition. Medical records often do not contain important elements of a patient's history. Verbal communication and "group" knowledge of the patient often contain information that cannot be easily, if at all, converted to written format. Meetings assure pediatric providers that patient needs will be adequately addressed. These meetings also give adult providers a chance to ask for information they feel is important.
Successful transitions cannot be rushed. Both pediatric and adult providers must agree on the steps in the process. Furthermore, patients must be alerted to the impending changes as early as possible. The seeds of transition ideally are planted one or more years before the event. The steps should be delineated clearly both for patients and providers. The providers should assess success at each point along the path. Patients should have opportunities to ask questions and voice concerns.
The latter is particularly important. Patients sometimes feel as if their pediatric providers are abandoning them. This can produce both hurt feelings and resistance to the whole transition effort. Reassurance that the pediatric and adult providers will remain in contact is important to alleviating this concern. Patients who have information on transition months or years beforehand are less likely to resent the process.
When possible, a member of the adult team should escort the patient and parents to the new facility to familiarize them with the environment and staff. Finding a clinic a few blocks or even a few corridors away from one's home ground can be challenging. The issue is magnified for people with sickle cell disease, since they must often navigate their way while experiencing the agony of a vaso-occlusive pain crisis. A pain crisis is not an optimal setting in which to meet new providers.
The first clinic appointment is a crucial test of transition. Providers should not simply give patients the phone number and instruct them to make the call. At the last pediatric visit, the pediatric providers should schedule the next appointment, just as they normally do. The appointment simply will be with the adult providers. If the patient misses the first appointment, a member of the pediatric team should phone to assess the situation. Often, patients remain reluctant about transition. They do not attend the adult clinic that they perceive as threatening. They do not return to the pediatric clinic, which they see as having abandoned them. The result can be loss of all medical follow-up. Patients often return in the throes of a medical catastrophe. Early intervention can avert disaster.
Following the introduction of transition as a goal by the health providers, the social service members of the team should begin to lay the groundwork. This includes discussions initially with the parents, and later with the adolescent. Reading material is an excellent way to reinforce the concept and serves as reference and reminder between clinic visits.
As the time for transition approaches, social service providers play a larger role. They often are best positioned to facilitate the introduction of the new adult care team. Patients and parents will have questions and concerns that can then be discussed and resolved. One of the most important tasks for the social service providers is to insure that the patient makes the first visit to the adult care clinic. If the appointment is missed, the social service provider often is the most appropriate person to phone the family and determine the nature of the problem. A "no show" at the adult facility may be an indirect way of asking for more help with the transition process.
Primary pediatricians often are the axis of care for children with sickle cell disease. Hematologists generally are consultants. This structure arises from the fact that all children need pediatricians. The healthiest child makes several obligatory visits to the pediatrician for routine immunizations. Most preschools and day-care facilities require certification that children have received basic vaccinations, such as those for mumps, measles and pertussis. When added to common childhood illnesses such as chicken pox and Fifth Disease, pediatric primary care is the natural result. Since many children with sickle cell disease have relatively few additional problems, general pediatricians remain their focal contacts. Pediatric hematologists provide advice about specific interventions such as pneumoncoccal vaccination and penicillin prophylaxis.
By contrast, adolescents and young adults generally comprise the healthiest segment of the population. With the exception of required physical examinations for college or the military, few people between the ages of 18 and 30 years have a standing relationship with health care providers. Visits to the doctor trail off with age, until only people with chronic ailments remain.
The result is that young people see doctors for specific problems, rather than disease prevention or health maintenance. The asthma specialist is the only medical contact for most young people with significant bronchospastic disease. The gastroenterologist is the primary provider for people with Crohn's disease. The hematologist is the primary provider for young people with sickle cell disease.
Young adults with mild asthma tend not to follow-up with pulmonologists. Only occasionally does this produce a serious outcome. Young adults with mild sickle cell disease tend not to follow-up with hematologists. Disasters can occur due to otherwise preventable complications of sickle cell disease. Retinal hemorrhage can produce severe and sometimes complete visual loss. The proliferation of retinal microvessels that precedes the catastrophe produces no symptoms. An ophthalmologist can detect and eliminate these vessels by laser surgery before complications develop. Preventative care is a key component in the management of patients with sickle cell disease. Young people who slip between the cracks during transition from pediatric to adult care often suffer unnecessarily.
Young people with sickle cell disease who remain in contact with pediatric providers can be transitioned to adult care more easily. These are often people who have suffered the greatest complications of sickle cell disease and are medically most complex. Adult providers sometimes feel they are given only the problem patients, while the pediatricians keep those who are easiest to treat. An organized transition program where the adult and pediatric providers meet to discuss the patients can dissipate ill feelings before they reify.
Patient support groups often meet in community settings. Patients and parents sometimes find frank discussion easier in the absence of health providers. Gatherings arranged around specific events, such as picnics or holiday celebrations, often work better than meetings focused solely on sickle cell disease. Patients often grow tired of reminders of disability. At a picnic, however, patients can discuss their concerns about sickle cell disease and transition. On the other hand, they can simply enjoy themselves in the company of other young people. Events are supportive. Meetings can carry an unfortunate sense of coercion.
Transition programs require personnel. Administrators usually determine the personnel mix of the institution. A single adult provider working without nursing or social service support cannot engage in transition activities. Even if the pediatric providers do the preparation, the effort will fail since a single overworked physician cannot adequately care for new patients. Transition is a program that exists between institutions. The world's most devoted providers cannot transition patients without support from those institutions.
In contrast, adults with sickle cell disease frequently have difficulty maintaining steady employment in the absence of flexible jobs that take their disability into account. The consequence is that a disproportionate number of adults rely on assistance from the government, including mandated health programs. In the United States, these programs often fall short of comprehensive coverage. Adults with sickle cell disease, like other people with chronic illness, lack the breadth of services that would support activities such as transition programs.
Chronic end-organ damage begins to manifest in adulthood. Problems such as osteonecrosis, renal dysfunction, and cardiac dysfunction are more frequent in adults than in children. The combination of chronic, intractable problems, such as osteonecrosis of the femur, and poor financial compensation deters some adult providers from the care of patients with sickle cell disease. The advent of managed health care has exacerbated the problem, since these patients are least attractive to people who focus on profit/loss margins.
The result is that many pediatric programs lack an adult counterpart to which their patients can be transitioned. Pediatricians heroically hold on as their patients age and sometimes develop problems outside their area of expertise. A pediatrician learning to use nitroglycerin for patients with ischemic heart disease is an unfortunate testimonial to the often-dire nature of the situation.
The institutional commitment, particularly on the adult side, comes to the fore in this circumstance. Current data indicate that a well-organized adult care program for patients with sickle cell disease will be a financial plus for the institution (Benjamin, et al., 2000). There must be a cadre of patient advocates, both professional and lay, who hammer this point home. Patients with sickle cell disease often slip between the cracks when large institutions balance the competing demands for care presented by its diverse patient population.
Without a structured adult care program, pediatric providers can only equip their patients to find their way through the maze on the other side of the divide. Patients should learn to keep copies of their basic records, such as discharge summaries. They should also keep a list of current medications, including the medications that work for them during an acute vaso-occlusive pain crisis. Patients should keep a diary of important events such as immunizations and complications of their sickle cell disease. Finally, the pediatric providers should dictate a brief overview of the patient's medical course during childhood. This means distilling the data in volumes of charts to a couple of pages. The familiarity that the pediatrician has with the patient over the years makes tractable an otherwise formidable task.
Transition programs to not evolve naturally. Someone perceives the need and acts to fill the void. Most often, the impetus comes from pediatricians and parents. Adult providers sometimes are reluctant to engage in a transition effort, for the reasons outlined earlier. The initial phase that follows recognition of need is crucial to the ultimate outcome. Misstep at the outset can result in the erection of an iron curtain, instead of a transition program.
The components needed to establish a transition program vary tremendously. Attempts to establish programs must be individualized and account for differences between institutions, providers and patients. An assessment of needs is a key first step. Before building a house, the planner assesses the needs of the people who will occupy the structure. She also surveys the ground and terrain to determine the type of structure the environment would best support. Only then does the planner move forward with design and construction. The same is true for transition programs. An ounce of preparation is worth more than a pound of repair.