MANAGEMENT OF SICKLE CELL DISEASE

NIH Publication No. 02-2117. 188 pages. Revised May28, 2002 (Forth Edition) National Institutes of Health, National Heart, Lung, and Blood Institute. Download the entire PDF  file for Adobe  Return  to table of contents

Chapter 7. ADULT HEALTH CARE MAINTENANCE

There have been significant improvements in the outlook for adults with sickle cell disease (SCD). The Cooperative Study of Sickle Cell Disease (CSSCD) and other observational studies have helped to define the prognosis and common complications that occur as the patients age. Improved management of infections and central nervous system (CNS) complications in childhood, active health maintenance for adults, new interventions, and improved psychosocial support have all contributed to a reduction in morbidity and mortality.

The prognosis in SCD has dramatically improved over the past 30 years. Estimates based on the mortality in the CSSCD estimate a median survival of 42 years for males and 48 years for females with SCD-SS disease and 60 years and 68 years, respectively, for SCDSC disease (1). Patients are now living into the seventh and eighth decades. More than 90 percent of patients of all phenotypes will survive past age 20, and significant numbers are older than age 50 (1). Risk of early death in adults with SCD is associated with acute complications such as pain episodes, anemic events, acute chest syndrome, chronic renal failure, and pulmonary disease (2).

Improved survival provides opportunities to improve the quality of life for patients with SCD; however, it also provides unique challenges in health maintenance. The frequency of pain episodes increases in early adulthood (3). Hydroxyurea provides the first pharmacologic intervention that reduces the frequency of pain episodes in adults (4). Proliferative retinopathy is a life-long risk that increases in prevalence with age (5). Vision may be compromised by these vascular changes that predispose to retinal hemorrhages, retinal detachments, and increased intraocular pressure (5). Renal glomerular disease is prevalent in adults and may cause increasing anemia, renal failure, and premature death (6-8). Prevalence of chronic pulmonary disease and pulmonary hypertension also increases as patients age, and may contribute to morbidity and mortality (9). Complications such as leg ulcers and osteonecrosis of the hips and shoulders cause chronic pain and disability, which require social and vocational adjustments (10,11). Essentially unstudied are the pain episodes that many women experience in association with menstruation and the increased frequency and severity of pain episodes near menopause. Geriatric challenges in patients with SCD are not well studied.

Health maintenance activities must also address interactions between SCD and other common health problems of the adult population. Individuals with relative hypertension and SCD have an increased risk of strokes and increased mortality, and therefore treatment for hypertension is an important aspect of health care (12) (see chapter 15, Cardiovascular Manifestations). Patients with SCD are not protected from developing cancer as they age (13). Diabetes, asthma, arthritis, atherosclerotic vascular disease, and other chronic illnesses may occur concurrently and provide unique challenges in managing patients with SCD.

Many adults are well adjusted socially and psychologically. Others, however, experience problems including anxiety and depression, and have difficulty forming and maintaining relationships, finding and keeping employment, and participating in usual daily activities (14-16). There is an increased need for social and psychological support services to maximize adjustment and productivity in adults with SCD (14-16).

Ongoing care of the adult patient includes preventive health maintenance, early recognition and treatment of complications, continuous assessment of social status, psychological assessment and support, and continuing patient education. Such services can be effectively accomplished only during regularly scheduled well-patient visits where effective doctor-patient relationships are established and medical, social, and psychological issues are addressed.

The initial visit provides an opportunity to establish rapport with the patient and his or her family and to determine the patient’s medical and social needs, as well as psychological strengths and challenges. A complete database should be developed that includes the information outlined in table 1.

Initially, a number of visits every 1 to 2 weeks will facilitate developing rapport, discussing laboratory and other test results, completion of the initial database, developing a problem list and care plan, and exploring active social or psychological issues. Routine medical evaluations are scheduled approximately every 2 to 6 months, depending on the patient’s phenotype and active problems. The database is updated at every visit. Blood counts, reticulocytecounts, and urinalysis are repeated at each visit to establish a baseline and detect problems. Pulse oximetry at each visit is also helpful. Routine chemistry tests should be repeated at least annually. Complications such as chronic organ failure, other medical problems, or complex psychosocial problems often require more frequent visits and more extensive laboratory evaluations.

Patients with hypertension, proteinuria, increased creatinine, renal tubular acidosis, or hyperuricemia should have more extensive and regular evaluation of renal function (see chapter 19, Renal Abnormalities in Sickle Cell Disease). Individuals, especially those with sleep apnea or chronic hypoxia requiring oxygen therapy with pulmonary disease or symptoms, should have regular pulmonary function studies and evaluation of pulmonary hypertension. Patients should have an annual ophthalmology examination for retinopathy, increased ocular pressure, and refraction errors. Followup examinations of patients with significant proliferative retinopathy is scheduled by the ophthamalogist at more frequent intervals (see chapter 14, Sickle Cell Eye Disease).

In many populations, tuberculosis screening should be done annually. Tetanus immunizations are kept up to date, hepatitis vaccine is given, and influenza vaccines are administered annually based on recommendations of the  Centers for Disease Control and Prevention (CDC). Pneumococcal vaccine is repeated every 5 years. Annual testing for HIV and hepatitis C may be indicated for patients who are sexually promiscuous. Women should be taught to practice breast self-examination, have an annual breast examination by a physician, and have mammograms with a frequency based on family history and age as recommended by the American Cancer Society (ACS). Males should be screened for prostatic specific antigen after age 50. Screening for colon cancer is based on family history and age as recommended by the ACS.

Initial evaluation should include assessment of the patient’s and family’s understanding of SCD. Educational activities should focus on correcting deficits in knowledge about more common complications such as infection, gallstones, aseptic necrosis, acute chest syndrome, leg ulcers, and priapism. Patients should be taught to seek medical care for persistent fevers greater than 38° C (100° F); chest pain, cough, and shortness of breath; symptoms of acute anemia including weakness, dyspnea, or dizziness; abdominal pain with nausea and vomiting; respiratory infection with a productive cough; symptoms of urinary infection; or unusually severe headaches. Preventive care includes learning physical limits and regularly participating in health maintenance, which includes following medication and immunization recommendations, protecting lower legs, and practicing safe sex. It is extremely important to discuss the specific risks to individuals with SCD associated with use of alcohol, cocaine, marijuana, and cigarettes.

All patients require education about appropriate management of pain. They must be taught to recognize the sources and intensity of their pain and to use appropriate therapeutic interventions. Headaches, menstrual cramps, strain, and muscle pains are best managed with aspirin, nonsteriodal anti-inflammatory drugs (NSAIDs), and acetaminophen. Treatment of SCD-associated pain is initiated with these agents. Physical therapy interventions—such as use of rest and heat, drinking fluids, and using relaxation or distraction—are taught. If the pain is not controlled by these measures, the patient is taught to seek medical care.

Patients and families should be reassured that appropriate use of narcotics to adequately control pain does not lead to addiction. They should be encouraged to use only the required dosages of medication, follow medical directions, and not use the medication for stress, anxiety, or other purposes.

The occurrence of chronic pain from avascular necrosis, leg ulcers, and other complications requires intense education so that patients understand that the goals of therapy for chronic pain are different from those for pain control during acute episodes. These goals are to minimize pain, increase pain coping skills, and maintain maximum social and physical functioning. In addition to pain medications, treatment may require nonpharmacologic interventions such as occupational and physical therapy, behavior modification, and other neurocognitive interventions.

Nutritional counseling is also important, with the goal of maintaining ideal body weight. Underweight individuals may require nutritional support. A number of patients need weight reduction, especially if they are overweight and develop complications such as avascular necrosis and diabetes. Folic acid supplementation is recommended for patients with  sickle cell anemia, although the optimum dose to normalize homocysteine levels has not been determined. Many patients take other vitaminsand nutritional supplements, but there is no strong scientific evidence for their use.

Young adults with SCD must be taught to approach activities in a way that minimizes excessive stress, exhaustion, dehydration, and extremes in temperature. Moderation and selfmonitoring of exertion level is the rule. Thus individual, rather than team, sports generally are preferred. Exercise is encouraged, but activities should be regular and slowly progressive.

Hydration with water is important before, during, and after physical activities. Cold exposure is minimized by adequate dressing and avoidance of swimming in cold water. Patients with avascular necrosis should maintain ideal body weight, avoid vocations that require prolonged standing or heavy lifting, and refrain from power weight lifting and exercise that involves running or jumping.

With proper hydration, most patients can tolerate air travel in planes pressurized to 2,200 meters (17). Patients going above this altitude in the mountains or in unpressurized aircraft may experience pain episodes and other complications(17). With advance arrangements, most airlines will provide supplemental oxygen for patients who have experienced problems in the past or who have other medical problems that may contribute to risk of complications. There is anecdotal evidence that persons with splenic function may be at higher risk during air travel.

Childbearing and birth control should be discussed with patients and their partners (see chapter 23, Contraception and Pregnancy). Discussions should include the risks during pregnancy, the potential for spontaneous abortion, and the physical and emotional challenges of raising an infant. Resources for patient support during and after the pregnancy should be explored. Preconception education should include genetic counseling and testing of the partner. Pre- or postconception genetic counseling should include discussion of prenatal diagnosis.

Individuals with SCD should be encouraged to complete their education and pursue vocations. Jobs requiring strenuous physical exertion, long work hours, exposure to hypoxia, or extremes in temperature may not be tolerated and should be discouraged, especially if the patient has increased symptoms when engaged in the vocation. Higher education and advanced vocational training can provide vocations and professions that are ideal for individuals with SCD. Many young adults seem to have more frequent and severe pain episodes during the first years of college. This may be related to the rigors of academic pursuits, excesses because of increased independence, or perhaps, directly related to the natural history of the disease (3).

Carefully teaching the individual to establish excellent study habits and to practice moderation in social activities usually will reduce the frequency of complications. These changes, along with discussions with faculty and advisors, almost always allow motivated students with SCD to complete their studies; however, more time may be required than is usual for students without medical problems.

Patients must also be encouraged to discuss their disease with their employers. Maintaining employment may require intervention by the health care provider to explain limitations to the employer, provide excuses for absences from work, and complete forms for the employer. Health care providers also should be familiar with legal protection against discrimination in the workplace, as provided by the Americans with Disabilities Act. Individuals with severe disease, cerebral vascular accidents, and avascular necrosis may truly be disabled.

In these situations, the health care provider should actively assist in obtaining benefits for the patient.

Social services and psychologic support activities are a critical component of comprehensive health maintenance in sickle cell patients (18). These activities are best accomplished if social workers and mental health professionals are integrated into the sickle cell care team. Social workers are invaluable in solving a myriad of social and family problems. Mental health workers can assist in managing psychiatric problems such as depression, teaching coping skills, and giving instruction in cognitive and behavioral management of pain. Nurses are an essential part of the support team because they have ongoing interactions with the patients and their families that facilitate identification of special patient and family needs. Nurses coordinate health care efforts and education about preventive health care, and provide ongoing psychosocial counseling.

Vocational rehabilitation services are also important in adult health maintenance. Patients with inadequate education can receive training in order to acquire satisfying employment that supports independent and productive life styles. This greatly improves their self-image and often has a positive impact on their health and utilization of health care resources. For example, occurrence of avascular necrosis or leg ulcers in patients with jobs that require prolonged standing often requires training to allow them to qualify for desk jobs.

Routine dental care is important to prevent loss of teeth and infections that may lead to other SCD complications. Dental procedures that require local anesthesia can be performed in the dentist’s office as with any other patient. Procedures requiring general anesthesia necessitate hospitalization and may require the usual perioperative care recommended for sickle cell patients (see chapter 24, Anesthesia and Surgery). Patients with a history of rheumatic heart disease, mitral valve proplapse, heart murmurs, or those with implanted venous access catheters and orthopedic prothesis should receive antibodics for subacute bacterial endocarditis (SBE) prophylaxis with tooth extractions, aggressive dental hygiene activities, gum surgery, or root canal therapy.

1. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994;3430:1639-44. 

2. Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle cell diseases in Jamaica. Br Med J 1982;285:633-5.

3. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med 1991;325:11-16.

4. Charache S, Barton FB, Moore RD, et al. Hydroxyurea and sickle cell anemia. Medicine 1996;75:300-26.

5. Charache S. Eye disease in sickling disorders. Hematol/Oncol Clin N Am 1996;10:1357-62.

6. Powars DR, Eliott-Mills DD, Chan L, et al. Chronic renal failure in sickle cell disease: Risk factors, clinical course, and mortality. Ann Intern Med 1991;115:614-20.

7. Falk J, Sheinmen J, Phillips GM, et al. Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensin converting enzyme. N Engl J Med 1992;326:910-15.

8. Gausch A, Cua M, Mitch WE. Early detection and the course of glomerular injury in patients with  sickle cell anemia. Kidney Int 1996;49:786-91.

9. Powars D, Weidman JA, Odom-Maryon T, et al. Sickle cell chronic lung disease: Prior morbidity and the risk of pulmonary failure. Medicine 1988;67:66-7.

10. Embury SH, Hebbel RB, Mohandas N, et al. Sickle Cell Disease. Basic Principles and Clinical Practice. New York: Raven Press, 1994.

11. Serjeant GR. Sickle cell disease. 2nd ed. Oxford: Oxford University Press, 1992.

12. Pegelow CH, Colangelo L, Steinberg M, et al. Natural history of blood pressure in sickle cell disease: Risks for stroke and death associated with relative hypertension in sickle cell anemia. Am J Med 1997;103:171-7.

13. Dawkins FW, Him KS, Squires RS, et al. Cancer incidence rate and mortality rate in sickle cell disease patients at Howard University Hospital. 1986-1996. Am J Hematol 1997;55:188-92.

14. Thompson RJ, Gil KM, Abrams MR, et al. Stress, coping, and psychologic adjustment of adults with sickle cell disease. J Consult Psychol 1992;60:433-40.

15. Faber MD, Koshy M, Kinney TR. Cooperative study of sickle cell disease: Demographic of adults with sickle cell disease. J Chronic Dis 1985;48:495-505.

16. Abrams MR, Philips G, Whitworth E. Adaption and coping: A look at a sickle cell patient population over age 30-an integral phase of the life long development process. J Health Soc Policy 1994;5:141-60.

17. Mahony BS, Githens JH. Sickle crisis and altitude: Occurrence in the Colorado patient population. Clin Pediatr 1979;18:431-8.

18. Koshy M, Dorn L. Continuing care for adult patients with sickle cell disease. Hematol Clin N Am 1996;10:1265-73.

Send mail to aplatt@emory.edu with questions or comments about this web site.
Copyright © 1997 Sickle Cell Information Center
Last modified: November 10, 2002