Sickle Cell Anemia - Case Management

CHILD AND ADOLESCENT HEALTH CARE MAINTENANCE

At the crux of effective health care maintenance is a strong patientprovider relationship built on trust, respect, open communication, and mutual understanding. The child and family should be encouraged to be active participants in the health care program, to be candid in expressing their concerns, and to work closely with the provider in all areas. In turn, the provider must be sensitive to the child’s development and be willing to offer counseling and education that is age appropriate, realizing that the child’s need for information will change with age. Providers who speak only to the parents and fail to incorporate the child or adolescent into the management plan will foster dependency in the child, ultimately hindering the transition to adulthood. The provider also must be knowledgeable about existing medical and psychosocial resources within the community and assist the patient in accessing these services when appropriate.

DEFINITIVE DIAGNOSIS

Once the definitive diagnosis is established, the parents should be provided with appropriate education and counseling about the specific form of sickle cell disease affecting their child. Providers must explain things carefully, avoiding medical jargon and allowing ample time for questions. Practitioners should not “overload” parents by providing them with too much detail during initial visits. More than one counseling/education session is required to ensure that parents adequately understand the information. A practitioner should not hesitate to refer the patient and family to a specialist for counseling. Many communitybased sickle cell organizations are available to help with patient education and the acquisition of social services. The definitive diagnosis should be recorded on the child’s immunization record and in other key medical records. A copy of the diagnostic information also should be given to the parents so that information can be shared with other health professionals involved with the infant’s care.

MAINTENANCE HEALTH CARE SERVICES

General Issues

Immunizations should be administered according to schedules recommended by either the American Academy of Pediatrics or the American Academy of Family Practice. In addition, all children with sickle cell disease should be immunized against hepatitis B virus and should receive the polyvalent pneumococcal vaccine at age 2 years, with a booster at age 5 years. Seasonal influenza vaccines are recommended.

Dietary counseling is an important part of routine health care. Mothers should be encouraged to breastfeed their infants, although ironfortified formulas are an alternative. Supplemental iron should not be prescribed unless the patient is documented to have reduced iron stores by specific assessments of the serum ferritin level or measurement of serum iron and ironbinding capacity. Children with hemoglobin SC disease are often microcytic in the absence of iron deficiency or concomitant alpha thalassemia. Similarly, the incidence of alpha thalassemia trait is quite high in the AfricanAmerican population and may produce microcytosis in the absence of iron deficiency. Routine administration of supplementary folic acid may not be necessary unless the dietary history reveals inadequate folate intake. Particular attention should be paid to the diet if education, unmet economic needs, or cultural patterns place the child at risk for dietary deficiencies.

All children, including those with sickle cell disease, need regular dental care. Supplemental fluoride given topically, in vitamins, or in the drinking water, should be provided when indicated. Cleaning and dental fillings do not require special care. Operative procedures such as extractions and root canal therapy should be preceded and followed by standard rheumatic fever antibiotic prophylaxis to lessen the risk of bacteremia/sepsis. If general anesthesia is required, the child should be hospitalized, and preoperative transfusion should be considered if the surgery is extensive.

Other health care maintenance services include routine hearing and vision screening and periodic skin tests for tuberculosis. Because of the ocular complications of sickle cell disease, adolescents and adults should be seen annually by an ophthalmologist. Providers must also counsel patients and parents about the adverse health effects of illicit drugs, including tobacco and alcohol. Teenagers should be counseled about safe sex practices, including abstinence. If sexually active, they should be encouraged to use condoms to prevent getting sexually transmitted diseases, including acquired immunodeficiency syndrome (AIDS). Adolescent girls should receive additional counseling on birth control practices and be provided with birth control pills or other effective contraceptives when requested, given that there are no specific contraindications.

Sickle CellSpecific Issues

When performing the physical examination, providers should pay attention to growth parameters and physical development. Physical growth and sexual maturation are delayed in sickle cell anemia patients compared with normal children, but the child eventually will “catch up” during the late teenage years. Common physical findings in children with sickle cell disease include mild generalized lymphadenopathy, functional systolic murmurs, and slight hepatic enlargement. Splenomegaly is often seen in infants and young children but is uncommon in children with SS disease and Sb o thalassemia after age 6 years. Splenomegaly, however, is frequently observed in patients of all ages with Hb SC disease and Sb + thalassemia. Because limitation of motion and pain are common signs of aseptic necrosis, the range of motion of the hips of older children and adolescents should be assessed. Scleral icterus is another common physical finding. Because children and adolescents are often teased about their “yellow eyes,” they should be taught the nature of this characteristic and reassured that it will not affect their visual acuity and does not necessarily reflect liver disease.

Blood counts should be done frequently during the first year of life to establish the patient’s baseline, but after 12 months of age, the hemoglobin and hematocrit are relatively stable and need to be checked only once or twice a year in uncomplicated patients. For patients who are doing well, an annual urinalysis and measurement of blood urea nitrogen (BUN), serum creatinine, and liver enzymes are adequate for monitoring the patient for evidence of organ damage. Before the administration of the first transfusion, the patient’s red blood cell antigens should be determined and recorded in the patient’s permanent medical record.

Specific physical assessment skills that should be taught to parents include taking the body temperature and assessing respiratory effort, degree of pallor, and spleen size. Parents of children who have an enlarged spleen should be taught to palpate the spleen and may be given a tongue depressor that indicates the level at which the spleen extends below the left costal margin in the midclavicular line. The parent can use this “spleen stick” to determine if the spleen size has changed and can provide the stick to other health care providers who assess the child. The skin around the ankles of adolescents should be examined carefully for ulcers, and patients should be reminded to avoid trauma to the lower extremities because this may precipitate ulcerations. Parents should also be told that the child may have repeated episodes of enuresis or nocturia as a result of the renal concentrating defect and should be provided with suggestions on how to manage this rather difficult problem. Parents should remind school officials that their child will need access to fluids while at school and will also need to be excused periodically from class to use lavatory facilities.

One of the major advances in the care of infants with sickle cell anemia has been the recognition that oral penicillin given twice a day will reduce morbidity and mortality from pneumococcal infections. Prophylactic penicillin is recommended for all patients with SS disease and Sb o thal until they are 5 years of age. For patients who are allergic to penicillin, erythromycin ethyl succinate (20 mg/kg) divided into two daily doses can provide adequate prophylaxis. The importance of administering the prophylactic antibiotics should be addressed at all visits because parents may become lax in dispensing this essential treatment over time. Penicillin may be prescribed in either liquid or tablet form; finely crushed pills may be given to young children. Pills have an important advantage over liquid penicillin preparations because pills are stable for years compared with liquid forms of penicillin that must be discarded after 2 weeks. An alternative to oral penicillin is injections of 1.2 million units of longacting Bicillin ® every 3 weeks. A recently concluded randomized trial demonstrated that penicillin prophylaxis may be safely stopped after the patient’s fifth birthday. Concerns regarding the development of penicillinresistant pneumococcal infections should not prevent the use of prophylactic penicillin, but appropriate measurements of antibiotic sensitivity are required in treating patients who have pneumococcal infections.

The role of prophylactic penicillin in SC disease and Sb + thal is less well established. Although these latter two groups of patients are not at the same risk as children with SS disease for overwhelming infection by encapsulated organisms because they have better preservation of splenic function, a significant number of pneumococcal infections have been reported. Regardless of whether a sickle cell patient is receiving prophylactic antibiotics, these patients should be evaluated promptly when they develop a fever greater than 38.5 o C (101 o F).

Parents and patients should be instructed in the proper use of analgesia. Fear of addiction may prevent parents from administering adequate analgesics to their children. Similarly, older patients may undermedicate themselves for fear of becoming addicted to the analgesic. Discussing these issues with the patient and parents and using nonaddictive analgesics where possible should minimize the risk of drug dependency. Providers can explore various ancillary measures with the patient to assist with pain management, including biofeedback and relaxation techniques. The provider can also address health care practices that minimize the risk of painful events such as maintaining adequate hydration, treating infections promptly, and avoiding temperature extremes. Patients can often predict that a painful event is about to happen and can frequently help themselves by implementing treatment before the pain becomes too severe. Deterioration in school performance or changes in behavior may be early signs of cerebrovascular disease and should be evaluated promptly by physicians knowledgeable about sickle cell disease. Primary care providers should identify physicians with expertise in sickle cell disease to assist them in managing sickle cell patients under their care. Costeffective care can be provided when there is close collaboration and communication between primary care providers and consultant specialists.

Adolescents

The transition from a pediatric medical setting to the adult care setting is often difficult for the patient. Ideally, adolescents in the pediatric setting should be given assistance in learning how to use the adult care facility and be introduced to their new providers by those who have cared for them as children. In some hospitals, this transition is greatly eased by holding concurrent pediatric/adolescent/adult sickle cell clinic sessions.

COUNSELING

Academic and Vocational Counseling

Vocational counseling is very important for adolescents and adults with sickle cell disease. Gainful employment is possible, despite the unpredictable nature of vasoocclusive complications. The adoption of flexible work hours is an example of a strategy that will help patients in the workforce. Referral to community resources, including school guidance and vocational rehabilitation counselors, is another way to assist the patient in assessing and obtaining goals. Introducing children and adolescents to adults with sickle cell disease who have successfully coped with their illness has a very positive effect. Health care providers should be familiar with legal protection against discrimination in the workplace, provided by the Americans With Disabilities Act.

Recreation and Travel

Children with sickle cell disease may benefit from a summer camping experience, either in an appropriate regular camp or through participation in a special camp for children with sickle cell disease. If the camp staff members are knowledgeable about the disease and comfortable with the care of these children, the camper can learn selfreliance and share experiences with other children about sickle cell disease while having fun. Health care providers and others who attend camp with sickle cell children typically find this a rewarding experience.

Patients and families often seek advice on the best methods of travel. Flying in a pressurized aircraft usually poses no special problems for sickle cell patients, provided that fluids are liberalized and the patient dresses warmly to accommodate for the low humidity and cool temperature of the aircraft. On the other hand, air travel above 15,000 feet in nonpressurized planes can induce vasoocclusive complications. Land travel by bus or automobile is not associated with any increased risk of sickle cellrelated complications, although frequent rest and refreshment stops should be included in the travel plans. Patients should be encouraged to consult their physician before traveling and should be advised to carry with them specific medical information that includes their diagnosis, baseline hematologic values, a list of current medications, and the name and telephone number of their physician. Providers should give their patients the names of physicians or care facilities to contact in the event of problems.

ADULT HEALTH CARE MAINTENANCE

Common practices and approaches to optimal health care maintenance of adults are reviewed, with an emphasis on developing a useful operational strategy for clinicians. Health maintenance includes prevention, early recognition and treatment of complications, and continuing patient education. In contrast to the stress of acute crises, wellpatient visits provide a better psychological setting for the development of effective doctorpatient relationships and effective coping skills. The development of such relationships may result in fewer visits to the emergency room and fewer hospitalizations.

INITIAL PATIENT VISIT

Patients with sickle cell disease are at high risk for developing sickle cell retinopathy. Patients should see an ophthalmologist for retinoscopy; the ophthalmologist should conduct followup examinations of patients diagnosed with significant proliferative retinopathy, at regular intervals.

Also during the initial visit, health providers should assess the patient’s understanding of the disease and its complications. Family planning and genetic counseling services should be discussed, and the availability of prenatal diagnosis should be mentioned when appropriate. Asymptomatic patients often have a false feeling of security about their disease. They should be cautious particularly regarding the choice of a profession and jobs requiring strenuous exercise and exposure to high altitudes.

PATIENT FOLLOWUP VISITS

Attention should be focused particularly on abnormalities of renal function such as renal tubular acidosis and hyperuricemia. During initial and followup visits, it is important to discuss the general nature and variability of the disease with the patient and to explain the significance of symptoms. Specifically, complications such as gallstones, aseptic necrosis, and priapism should be mentioned. Also, the issues of childbearing and birth control should be addressed, and the patient’s spouse or partner probably should be included in these discussions.

It is particularly important to instruct patients about how to respond to acute illness. They should be instructed to seek immediate care for events such as high fever, productive cough, and symptoms of acute anemia such as sudden dyspnea, weakness, or dizziness. Discussion with the patient should include the identification of available means of transportation to the emergency room. Painful episodes should initially be managed at home with rest, a warm environment, increased oral fluids, and mild analgesia or other modalities of pain management. If pain persists or fever develops, the patient should contact his or her physician or go to an emergency department. The procedure to be followed for a particular emergency department should be discussed in detail; if a prior call from the physician can facilitate the visit, the patient should know whom to call at night or on weekends and holidays.

Patients should keep a small supply of an intermediate strength oral narcotic analgesic (codeine, hydrocodone, or oxycodone) and should be instructed to use it for attacks of pain that do not respond to aspirin, acetaminophen, or ibuprofen. They should be informed that most pharmacists will not accept a telephone prescription for a narcotic and should be instructed on how to obtain another supply.

Certain matters of general health care are particularly important to patients with sickle cell disease; for example, it is important for the physician to discuss the hazards of cigarette smoking and excessive alcohol intake. Although strenuous exercise may have to be avoided in most patients, a wellplanned exercise program should be encouraged.

Most patients eat poorly during painful crises, and because patients with hemolytic anemia have an increased need for folic acid, daily supplements of 1 mg should be prescribed at those times. The danger of masking a vitamin B 12 deficiency is small, but even young AfricanAmerican patients are at risk. There is no evidence that any other form of vitamin supplementation is of value in sickle cell disease.

The patient’s employment should also be discussed. Arrangements for obtaining “doctor’s notes” after an absence from work, filling out forms, and refilling prescriptions should be outlined for all patients with a chronic disease. Medical providers should be familiar with procedures for seeking legal assistance if workplace discrimination because of disability is perceived.

VOCATIONAL GOALS

PSYCHOSOCIAL COUNSELING

DENTAL CARE

SURGERY AND ANESTHESIA

After surgery, it is extremely important to ensure hydration and, at the same time, prevent circulatory congestion. Adequate records of the amount of intravenous and oral intake should be maintained; urine output and fluid drainage should be measured and recorded, and patients should be weighed daily. Minor hyponatremia (130 140 meq/L) can be tolerated but care should be taken to avoid hypernatremia. Efforts should be made to ensure deep breathing and coughing, to avoid atelectasis.

EXPERIMENTAL THERAPY