Sickle Cell Disease
Recent years have seen a number of different investigations of the issues involved in the transition of care — from pediatric-oriented to adult-oriented services — for those who suffer from sickle cell disease. Although different researchers have taken a number of different approaches to the question, which I hope to survey in order to provide some report on the current state of opinion regarding transition of care, all are agreed that the current flurry of investigative interest stems ultimately from a piece of very good news: the vertiginous decline in mortality rates for children suffering from sickle cell disease. The historic response to a diagnosis of pediatric sickle cell disease was to minimize patient and parental expectations for prognosis, for the prospect of reaching adulthood would be slight indeed. Telfair Loosier (2004) note that survival rates for pediatric sickle cell disease have improved so vastly that the vast majority of cases are now likely to live past the age of eighteen: they assess survival rates past eighteen at 94% for sickle cell disease itself, and 98% for the milder forms of the disease. Six years later, Quinn Rogers et al. (2010) note that the statistics have changed drastically even with the current cohort of pediatric patients: the ultimate cause of death for young adult sickle cell disease sufferers has itself shifted, and in the 2010 study population all of the deaths they recorded occurred after the age of eighteen. But the largest demographic cluster of deaths occurs shortly after the transition to adulthood at eighteen, now raising the question of what providers can do differently, or concentrate on, in this critical period in the management of the illness. Hence the recent increase in research interest, as researchers hope to determine how health care providers may best respond to the changing face of this disease, especially in light of Quinn Rogers et al.’s implication that the medical transition process may now hold more culpability for the majority of deaths than the disease. I hope that a survey of the relevant literature will show the broad consensus that exists regarding the crucial issue of transition of care from pediatric to adult.
The basic mechanism of sickle cell disease is well understood and Davies and Oni (1997) provide a good summary — sickle cell disease is a disorder of hemoglobin, the blood’s own mechanism for effecting oxygen transfer from the lungs to be transported elsewhere in the body, causing the distinctive sickle shape of the red blood cells. The most common clinical problems compained of are vaso-occlusion, with attendant risk of vaso-occlusive crisis, usually expressed by the patient as extreme pain. Severity of the disease is measured by the incidence of these severe pain episodes, and the disease can lead to death by pulmonary complication, cerebrovacular accidents, or infections (to which sickle cell sufferers are particularly prone, due to an immunocompromised state caused by the spleen’s inability to process fully the sickle cells themselves). Sickle cell disease is genetically transmitted, and relate to the presence of a single gene (the “sickle Beta globin gene”). It is thought that this gene achieved particular prevalence among the African genetic populations because it also carries with it a phenotypical increase in level of protection against malarial infection, and thus the evolutionary mechanism for its survival (despite its role in the aetiology of sickle cell disease, which easily proves fatal) is explained in these terms. But the genetic prevalence is remarkable: Davies and Oni (1997) estimate it as “up to 1 in 4 West Africans and 1 in 10 Afro-Caribbeans” is a carrier of the gene.
Obviously any assessment of sickle cell disease must take into account the fact that the preponderance of those afflicted by it are African-American. Numerous studies have been done to investigate this specifically, with the sociologic and economic difficulties that are potentially entailed by such a racially-defined patient population, but the most significant conclusions come in the assessment specifically of pediatric care for sickle cell disease. Slaughter and Dilworth-Anderson (1988) specifically surveyed the African-American population to assess standards of family-provided pediatric care, in the presence of and absence of a strong traditional family structure (with two married parents). They concluded that the majority of supportive care came from extended kin networks but with an attendant problem of decline in such support between diagnosis and later childhood. Meanwhile Barakat Lutz et al. (2005) offer an additional complication to the question of pediatric care: adherence to treatment protocols leads to lower overall quality of life for affected children. They specifically note that this leads to particular difficulties within minority communities with members that may have an uneasy relationship already with the health care infrastructure. Overall, then, these sufficient worries about pediatric diagnosis seem to lead to a greater level of attention from health care professionals themselves, in terms of specific supervision and support. How is the transition to be handled if there is a decline in such attentions?
Ideally there would be no such decline; Davies and Oni (1997) are firm in their insistence that “all patients with sickle cell disease should be registered with and monitored by a specialist clinic” so that the close supervision, especially during the pain attacks associated with vaso-occlusion, will always be available. But there is a sense in the literature overall that perhaps the psychological transition between different types of care is not being addressed, certainly not with the level of attention that is required for what may ultimately prove to be a psychologically difficult (as well as physically agonizing) diagnosis for young persons. Jenerette and Brewer (2010) address some of the broader psychological issues present in their assessment of the larger issue of “stigma” associated with the disease. Again, the racial or economic situation of the patient plays a large role in the handling of these social difficulties: Jenerette and Brewer note that “the increased use of the health care system only negatively impacts the sickle cell disease-stigmatization cycle that continues for the young adult with SCD,” potentially leading to avoidance. They also note the rise in “issues related to school absenteeism, increased use of the health care system, decreased school and social activities, as well as poor adaptation” which are only worsened by “variables such as race, gender, age, [and] socioeconomic status” that further negatively impact the condition of the diagnosed child. From the specific racial standpoint of African-Americans, though, Jenerette and Brewer indicate a larger sense of conflict with the health care system in general, noting that “the credibility or trustworthiness of young African-American adults with SCD is often questioned by health care providers, who label patients as malingerers or manipulators, or even drug seekers.” Although Jenerette and Brewer do not specifically address the question of transition, their study has clear implications for the subject: if we consider a patient with sickle cell disease who, complaining of an attack at age ten, is handled well by the hospital system only to be treated with disdain or doubt at age sixteen, then we might understand the disjunction not only between patient care before and after young adulthood, but also the broader disjunction between patient population and heatlh care system. Jenerette and Brewer’s conclusions offer a sobering challenge to health care providers.
But if the current state of the transition process seems to be failing the patient population, is this evident to providers? Telfair Loosier et al.’s 2004 study approaches the question of transition from the angle of provider assessments. They find that health care providers were in broad agreement that greater structure was needed for the transition than was currently in place — surveying the status quo, Telfair Loosier et al. find that only a very small number of health care providers would actively involve themselves in the transition, suggesting the need for some kind of mandate for an official oversight process. The consensus is fairly broad that larger structural support is required across the board to integrate providers’ own involvement with the transition process alongside other, ideally more structured, approaches among the family and extended kin networks, as well as broader community-based awareness and support.
From the standpoint of the patient, however, there may be additional psychological factors involved in whether or not the transition may be a success. McPherson and Thaniel offer an important caveat with their emphasis on “assessing patient readiness” for such transition. Their 2009 study offers some sobering conclusions: they found that, while adolescents were on the whole receiving adequate medical care, it did not include an adequate amount of preparation for the ultimate transition out of pediatric care and into adult care of the condition. The adolescents in their study provided a sharp contrast to the young adults and older adults surveyed, who expressed more comprehensive knowledge about their own condition, and more interest in learning about the eventual transition as part of their own health care. McPherson and Thaniel define the “readiness” for transition to adult care as being a joint measurement of patient knowledge and patient interest. Levels of interest and overall patient “readiness” were found broadly to increase when measured with the patient’s age but had an inversely proportional relationship to self-assessed disease severity (including pain and symptoms). As the pain or severity worsens, McPherson and Thaniel concluded, so does the patient’s psychological readiness for transition decrease.
Treadwell Telfair et al. (2011) represent the most recent comprehensive survey of the transition issues involved in sickle cell disease care, and they hope to offer new directions for both practice and for research. They acknowledge a number of “systemic” difficulties at the outset which have little to do with patient motivation, such as “limited access to adult providers.” Moreover other patient issues they identify are not specifically psychological, such as “absence of financial independence” or “increasing morbidity with age,” both of which seem like specific consequences of chronic illness of this magnitude. For Treadwell Telfair et al. one of the greatest limitations with the present system of transition is the way in which it is observed bureaucratically, in the form of an “exit meeting” almost — instead they emphasize that the transition should be considered “a process occurring over time and not a cross-sectional event.” This allows for the flexibility towards patient readiness that McPherson and Taniel had laid such emphasis on. Studies overall emphasize the need for greater institutional methods of patient support — Wojciechowski Hurtig and Dorn (2002) assess the various complicating factors that have made sickle cell disease so difficult in this regard, and make a strong case that what is required is the institutional adoption of “case management services” so that a full team (including social worker and psychologist) can assist in the handling of the patient. But it is worth noting that other authors have already recorded the first steps taken by many health care providers toward building this new infrastructure from the ground up. The issue, though, is aptly identified by Wojciechowski Hurtig and Dorn: there are so many factors involved in the transition, it may be difficult for just two physicians to handle it together.
In response to this accurate assessment of complexity, Treadwell Telfair et al. (2011) propose a “bio-ecological systems” approach which emphasizes five separate environmental systems which may act upon the patient, ranging from the biological (i.e., the effects of sickle cell disease upon the patient’s actual body and physical abilities or wellness) to the social microsystem (the immediate environment of family, school, peer group and neighborhood). The following three emphasize larger factors such as culture (in this case, urban America), patterning of events and transitions over the entire life course (which includes an awareness of the increasingly dim prognosis of sickle cell disease with increasing age), and sociohistorical circumstances (such as the fact of disproportionate representation of African-Americans among the overall patient population for sickle cell disease, a fact readily explained but still deeply felt). Their conclusion is that health care providers have to maximize the ability of the transitioning patient to take control of his or her own health care management, which includes a psychological assessment of the level of willingness or ability on the part of the patient to be receptive to new information and an increased role in self-assessed management of the condition. But Treadwell Telfair et al. suggest a more holistic approach to care management than has previously been recommended, in which they help the transitioning patient assume not only the “adult medical model,” but also are assisted in assuming “responsibility for their physical, psychological, social, educational and vocational well-being.” And providers’ awareness of the transition must be built in with the recognition of the shifting statistics of life expectancy for those with sickle cell disease: Treadwell Telfair et al. state bluntly that “it is now recognized that transition should begin at birth” and the family encouraged “to envision a future for the child with special health care needs.”
Treadwell Telfair et al. offer their thoughts on a future “research agenda” for the issues relate to transition from pediatric care to adult care in the management of sickle cell disease. They note that the increased calls for greater structural support have led to the establishment of transition programs by many health care providers, but they stress that “there is a need to apply consistent measurement across programs.” Stressing the complexity of factors involved in sickle cell disease and its demographics, they recognize the need for a multipronged and flexible approach, stating that “interventions should be multifactorial” and that future research is required on “developmental, medical and life skills” of the pediatric sickle cell patient, as well as for pediatric diagnoses of chronic conditions more generally (a subject whose comparative neglect has compounded the difficulties of treating sickle cell disease specifically, with its host of attendant sociological, economic, and stigma-related complications).
Doulton (2010) describes the operation of one program which has already been imposed to help deal with the issues of transition. It is the program assessed by Doulton, in place at the Children’s Regional Sickle Cell Program, jointly administered by the Santa Rosa Children’s Hospital and the University of Texas Health Science Center in San Antonio, which offers a “comprehensive program” for children and adolescents. It is from Doulton’s account that Treadwell Telfair et al. take their emphasis on the beginning of preparation for transition at birth: Doulton describes how the “newborn’s initial visit” to the clinic will be when the transition program is explained to the family, and at age thirteen all patients are given a “Preparation for Transition Binder” which will provide them with information and which is reviewed with the patient and with guardians on a regular basis by health care providers. Doulton states the goal as providing a “continuum of care” but also establishes that the structure of the program is calculated to ready the patient for transition at eighteen, to coincide with a general sense of life events occurring at the same age. But at the same time, the services Doulton describes are already straining towards the increase in kinds of attention suggested by Treadwell Telfair et al.; social work intervention is required, Doulton notes, to prevent an “emergency ‘transfer of care’ to an adult provider” due to “pregnancy or incarceration,” which obviously requires a community-specific or population-specific approach to the additional services and counseling provided to sickle cell disease patients. Doulton is firmly moralistic in her approach to these sorts of issues, noting of the clinic in general that “one major lesson that has been learned is that you cannot change anyone’s behavior if they do not see their behavior as a problem (i.e., incarceration, not finishing high school, no job potential, pregnancy or not having health insurance).” In other words, the overall boon of increased life expectancy which has arisen quite recently over the past two decades — and somewhat unexpectedly from the standpoint of institutional health care providers, who suddenly look like they are not doing their job — is a double-edged sword, in that it now forces the patient population to address various forms of psychological difficulty or social pathology that may come from the sense that one is destined to die young. From Doulton’s standpoint, this sort of social work is a necessity. It is handled largely in a series of age-structured stages, so that at age thirteen the patient is given an initial binder of information related to the transition, with instructions on everything from how to track medical appointments to pain management plans to educational CD-ROMs and web site addresses. But from fourteen to sixteen there is a more practical hands-on approach to this support, as the patients are coached to “develop the skills necessary to communicate and advocate for themselves from triage through to the discharge” from an emergency room to deal with the vaso-occlusive pain of an attack of sickle cell disease.
But even Doulton’s model has its problems, ones which may perhaps be addressed by Treadwell Telfair et al.’s suggestions for future research on these transition issues. For Doulton’s part she notes certain things which the program surveyed has not yet managed to deal with adequately: for example, in the specific attempt to bolster peer support for the patient population as they transition, Doulton notes that “we have attempted to bring our teenagers together for peer support groups without success.” She identifies this as a goal for the future, and hopes that a solution will present itself with greater facility in dealing with the issues in question. Btu the difficulties are always going to be present, Doulton acknowledges: “it is truly a delicate situation,” she concedes, “to assist the patient to strive for their dreams with consideration of the realities of having a debilitating chronic illness that is unpredictable.” But in terms of a first step towards the establishment of a broadly-accepted set of protocols for the establishment and maintenance of a more holistic approach to the transition between pediatric and adult care for those diagnosed with sickle cell disease, Doulton’s model seems admirable, and likely to be the basis for future refinements, perhaps based on research to be gleaned from Treadwell Telfair et al.’s proposed future research subjects.
Barakat, L.P., Lutz, M., Smith-Whitley, K., and Ohene-Frempong, K. (2005). “Is treatment adherence associated with better quality of life in children with sickle cell disease?” Quality of Life Research 14(2), 407-14.
Davies, S.C. And Oni, L. “Management of patients with sickle cell disease.” (1997). British Medical Journal 315(7109), 656-60.
Doulton, D.M. (2010.) “From Cradle to Commencement: Transitioning Pediatric Sickle Cell Disease Patients to Adult Providers.” Journal of Pediatric Oncology Nursing November 2009
Jenerette, C.M. And Brewer, C. (2010). “Health-related stigma in young adults with sickle cell disease.” Journal of the National Medical Association 102.11 (2010): 1050-5.
McPherson, M., Thaniel, L., Minniti, C.P. (2009). “Transition of parients with sickle cell disease from pediatric to adult care: assessing patient readiness.” Pediatric Blood Cancer 52.7 (2009),
Quinn, C.T., Rogers, Z.R., McCavit, T.L., and Buchanan, G.R. (2010). “Improved survival of children and adolescents with sickle cell disease.” Blood 115(17).
Slaughter, D.T. And Dilworth-Anderson, P. (1988). “Care of Black Children with Sickle Cell Disease: Fathers, Maternal Support, and Esteem.” Family Relations 3.7 (1988): 281-7.
Telfair, J., Alexander, L., Loosier, P.S., Alleman-Velez, P.L., and Simmons, S. (2004). “Providers’ perspectives and beliefs regarding transition to adult care for adolescents with sickle cell disease.” Journal of Health Care for the Poor and Underserved 12
Telfair, J., Ehiri J.E., Loosier P.S., Baskin M.L. (2004). “Transition to adult care for adolescents with sickle cell disease: results of a national survey.” International Journal of Adolescent Medicine and Health 16.1
Treadwell, Marsha; Telfair, Joseph; Gibson, Robert W.; Johnson, Shirley; and Osunkwo, Ifeyinwa. (2011) “Transition from pediatric to adult care in sickle cell disease: Establishing evidence-based practice and directions for research.” American Journal of Hematology 86.1: 116-20.
Wojciechowski, EA, Hurtig A, Dorn L. (2002). “A natural history study of adolescents and young adults with sickle cell disease as they transfer to adult care: a need for case management services.” Journal of Pediatric Nursing 17.1
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