MANAGEMENT OF SICKLE CELL DISEASE

NIH Publication No. 02-2117. Revised May28, 2002 (Forth Edition) National Institutes of Health, National Heart, Lung, and Blood Institute. Download the entire PDF  file for Adobe   Click Here to return to Contents

Chapter 9. PSYCHOSOCIAL MANAGEMENT

Sickle cell disease (SCD) is a complex condition that affects the patient, the family, and the patient’s and family’s relationship with health care providers and the community. It is imperative that teaching the skills necessary for coping with this illness begin at the time of diagnosis and continue throughout the life of the patient, and that providers recognize that including the extended family and the community in the education process will ensure the most positive outcome.

SUMMARY OF THE STATE OF THE ART

No interventions are proven to work with all patients in all situations (1-15). While a team knowledgeable about chronic illness and existing intervention models is ideal, available staff sensitive to the special needs of these patients can provide effective interventions even in primary care settings. Providers must recognize that the complexities of SCD necessitate a team approach to management. Clinical management, pain control, coping skills, genetic counseling, and community interactions, including school and work intervention, require different expertise. The establishment of Comprehensive Sickle Cell Centers introduced the concept of comprehensive care for SCD and refined the multidisciplinary team approach to health care. Psychosocial support was recognized as a necessary, integral function of the health care team. However, psychosocial interventions should be woven across the spectrum of medical care.

Historically, psychosocial interventions were reserved for emergency situations. Psychologists or social workers were called for acting-out behaviors, housing emergencies, noncompliance issues, and other crises. Crisis situations may be minimized by identifying specific points at which psychosocial interventions may be necessary and planning for them, thus eliminating the frustration and ineffectiveness often experienced by patients and caregivers.

Pain, the most well-known symptom of SCD, is the reason for most hospitalizations and precipitates many psychosocial crises. Skills for coping with pain and other complications of SCD must be taught early and reinforced often (8,9). Which patients will have mild diseases and which will have a more severe course is not predictable; however, stress is known to play an important role in the severity of chronic illness and pain. Absence of the physical appearance of trauma in severe SCD pain episodes can confound a patient’s ability to cope. In addition, many health care providers are not knowledgeable about sickle cell pain, its causes, and the best management options. This can lead to poorly controlled pain, continued treatment failure, and frustration of patients and providers.

The availability of prenatal diagnosis offers the family choices regarding the continuation of the pregnancy. When the decision is made to continue, the practitioner has time to assist the family in preparing for the arrival of a child with SCD. This time must be spent educating the parents and family about the disease and the need for family and community support. The better educated the family and the community, the better care the patient will receive. Many communities have SCD support groups that provide an avenue for sharing anxieties, as well as helpful information (5).

Whenever possible, satisfactory housing, accessible optimal medical care, and reliable transportation must be planned before the baby arrives.

Health insurance, emergency transportation, housing, and anxiety about recognizing symptoms are some of the issues new parents may need help acknowledging and addressing. Frequent clinic visits and home visits will allow the opportunity for parents and providers to establish a comfortable relationship to address these issues and help establish an ongoing pattern of compliance. Although advances in medical research have increased the chances for longevity, lack of understanding on the part of providers may result in inappropriate treatment plans that defy adherence. The establishment of clinical practice guidelines in SCD has decreased—but not eliminated —preventable deaths. In addition, noncompliance may undermine the best care plans.

In many cases, a concerted effort to understand the causes underlying noncompliant behaviors is necessary. Skepticism of the health care system, as well as barriers to access—such as location of clinics, laboratories, and pharmacies—  can all contribute to noncompliance (11).

The most important task of childhood is obtaining an education. Teachers must understand that children with SCD should be expected to perform as well as their peers, although special education is often needed.

Although undetectable micro-infarcts may cause brain injury, many of the learning difficulties these cause can be overcome with appropriate assistance. Failure to perform in school could be a function of neurological complications of SCD, but the lack of school success could also be confounded by socioeconomic factors. Disabilities and limitations must be acknowledged but, more important, strengths must be identified and inspired.

Pediatric health care professionals, in their desire to protect children with chronic illness, often inadvertently erect barriers to normal childhood behaviors and accomplishments. While addressing special needs and routine childhood health care, allowances must be made for regular school attendance (by flexible scheduling of appointments), for activity (by offering alternatives to inappropriate sports), and for learning (by providing support and education to school staff ).

ADOLESCENT HEALTH CARE AND TRANSITION

For a discussion of the transition from pediatric to adult health care, see chapter 6, Adolescent Health Care and Transitions.

As longevity of SCD patients increased, the need for continued comprehensive care became evident; however, while pediatric centers thrive, adult providers are scarce. Longterm management needs to focus not only on health care needs but on the other goals of psychosocial well-being—education, independence, and (eventually) marriage and family.

In adulthood, reproductive issues are of major concern and often are not addressed in a positive manner. Most individuals, including those with disabilities, hope to achieve normalcy, (e.g., independence, a meaningful job, and a family) (13). One barrier to care may be providers’ lack of understanding of the multiple layers of learning needed to live with SCD and the realities of trying to be "normal" and fitting in. The pain experienced by many patients with SCD can be demoralizing and overwhelming. In addition to the psychological effects of inadequately treated pain, patients have the added stress of continually searching for effective pain relief, resulting in frequent emergency room visits and episodic care. This cycle can lead to depression, which is highest among the chronically ill and in the 20-40 age group, and is often not recognized or addressed. Continued comprehensive care—including a strong psychosocial component—for adults with SCD is most important, since prevention of complications is the key to longevity.

Psychosocial issues confronting patients, families, providers, and the community, though multiple and multifactorial, can be addressed and result in positive patient outcomes.

1. Vichinsky EP. Comprehensive care in sickle cell disease: Its impact on morbidity and mortality. Semin Hematol 1991;28:220-6.

2. Vichinsky EP, Johnson R, Lubin BH. Multidisciplinary approach to pain management in sickle cell disease. Am J Pediatr Hematol Oncol 1982;4:328.

3. Adams RJ. Lessons from the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study. J Child Neurol 2000;15:344-9.

4. Britto MT, Garrett JM, Dugliss MA, et al. Risky behavior in teens with cystic fibrosis or sickle cell disease: A multi-center study. Pediatrics 1998;101:250-6.

5. Campbell MK, Motsinger BM, Ingram A, et al. The North Carolina Black Churches United for Better Health Project: Intervention and process evaluation. Health Educ Behav 2000;27:241-53.

6. Maunder R, Esplen MJ. Facilitating adjustment to inflammatory bowel disease: A model of psychosocial intervention in non-psychiatric patients. Psychother Psychosom 1999;68:230-40.

7. Schultz A, Liptak G. Helping adolescents who have disabilities negotiate transitions to adulthood. Issues Compr Nurs 1998;21:187-201.

8. Gallagher RM. Treatment planning in pain medicine. Integrating medical, physical, and behavioral therapies. Med Clin North Am 1999;83:823-49, viii.

9. American Pain Society. Guideline for the management of acute and chronic pain in sickle cell disease. August 1999.

10. Ross-Lee B, Kiss LE, Weiser MA. Should health care reform be "color-blind"? Addressing the barriers to improving minority health. J Am Osteopath Assoc 1994;94:664-71.

11. Lillie-Blanton M, Hoffman SC. Conducting an assessment of health needs and resources in a racial/ethnic minority community. Health Serv Res 1995;30:225-36.

12. Bussing R, Aro H. Youth with chronic conditions and their transition to adulthood. Findings from a Finnish cohort study. Arch Pediatr Adolesc Med 1996;150:181-6.

13. Gatchel RJ, Gardea MA. Psychosocial issues: Their importance in predicting disability, response to treatment, and search for compensation. Neurol Clin 1999;17:149-66.

14. Telfair J, Myers J, Drezner S. Transfer as a component of the transition of adolescents with sickle cell disease to adult care: Adolescent, adult, and parent perspectives. J Adolesc Health 1994;15:558-65.

15. Betz CL. Adolescent transitions: A nursing concern. Pediatr Nurs 1998;24:23-8.

SICKLE CELL DISEASE BOOKS (FOR ADDITIONAL REFERENCE)

¦ Shapiro BS, Schechter NL, Ohene-Frempong K. Sickle Cell Disease Related Pain: Quick Reference Guide for Clinicians. New England Regional Genetics Group (NERGG), 1994.

¦ Hill SA. Managing Sickle Cell Disease in Low- Income Families. Temple University Press, 1994.

¦ Brandon WE. Disabled: My Life, the First Fifty Years with Sickle Cell Disease. Philadelphia: VACS Books, 1997.

¦ Nash, KB. Psychosocial Aspects of Sickle Cell Disease: Past, Present, and Future Directions of Research. New York: Haworth Press, 1994.

¦ Mindice K, Elander J. Sickle Cell Disease: A Psychosocial Approach. Oxford, UK: Radcliffe Medical Press, Ltd, 1994.

¦ Franklin I. Sickle Cell Disease: A Guide for Patients, Caregivers and Health Workers. London: Faber & Faber, 1990.

¦ Bloom M. Understanding Sickle Cell Disease: For General Readers a Guide to Understanding a Debilitating Genetic Disease That Affects Tens of Thousands Who Are of African Ancestry. University of Mississippi Press, 1995.

¦ Ballas SK. Sickle Cell Pain, Progress in Pain Research and Management, volume 11. International Association for the Study of Pain, 1998.

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Last modified: October 27, 2002