Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Those with sickle cell disease should be evaluated periodically to establish a normal baseline for the patient, identify impending problems, to update immunizations and maintain nutrition support, and to provide patient and parent education and support. A schedule table provides broad guidelines for the frequency of visits and interventions to be stressed throughout life. There is wide variation in the severity of the disease between individuals and these guidelines provide suggested minimum health maintenance activities.

Prevention of infection is one of the major methods of improving prognosis in sickle cell disease. Initiation of prophylactic penicillin should be begun as soon as sickle cell anemia is suspected. Individuals with Hb SS, S Beta ⁰ thalassemia, and perhaps, Hb SC, should continue on penicillin until age five. Immunization for usual viral illnesses, Streptococcus pneumonia, Hemophilus influenza, and hepatitis B is indicated as outlined in Table 3.

Individual immunization schedules will need to be developed based on the child’s past immunization record, reactions to vaccination, presence of febrile illness, and local school requirements. The parent should be provided a permanent record of immunizations that also records the hemoglobin diagnosis, allergies, medication, other medical problems, and the phone number of the primary care provider. Parents should always present this during follow-up care.

Parents and patients should be taught how to read a thermometer. It is very important to provide these for parents or patients that do not have them available. Inexpensive digital thermometers are now available that are accurate and easy to use. The patient and parent need specific guidelines on how and when to seek immediate medical attention when a fever develops or signs of infection appear.

Parents of small children with palpable spleens should be taught to palpate the spleen and instructed to do this on a regular basis and at any time that the infant appears ill. This task can be facilitated by the use of a spleen stick. A simple tongue blade is marked with the position of the spleen tip with one end on the left nipple in infants or costal margin on the left nipple line. This line is dated and limits can be set at clinic visits by the use of red ink. The mother should keep the stick in the purse and bring it to each clinic visit. The physician’s number and other information can be written on the back. This is an excellent way of increasing the accuracy of observations that may detect early splenic sequestration and provide information on base-line spleen size no matter where the infant presents for health care.

Sickle Health Maintenance Schedule

Birth to 6 Month - Retrieve infant for examination. Parental disease education. Examinations Once a Month. Start Penicillin 125 mg. P.O. B.I.D by two months. Teach importance of fever, how to take temperature, importance of PCN.Confirmation of diagnosis. Initiate immunization. Assist with family grieving.

6 Months - 1 Year -Start folic acid 1 mg. Discuss nutrition, prevention of complications and accidents, hand-foot, recognizing illness, and sequestration. Clinic visit every two months. Continue immunizations. Establish a clinical baseline. Do extended family testing. Do genetic counseling.

1 to 2 Years - Continue immunizations. Discuss pain control, growth /development,and issues related to chronic disease. Clinic visits every 3 months. Give Pneumococcal vaccine, 23 valent at age 2. Monitor history, physical, CBC and reticulocytes with each visit. SMA 18 twice a year. Reinforce monitoring for signs of infection, CNS complications, and increasing anemia. Stress importance of hydration and diet.

2 to 5 Years - Increase penicillin to 250 mg. P.O. B.I.D and continue folic acid. Stress growth and development, hydration, avoidance of over dependence, setting limits. Clinic every 3 months. Complete immunizations. Reinforce pain management principles. Educate school nurses/teachers. Educate school nurses/teachers.

6 to 12 Years - Consider stopping penicillin. Stress academic achievement, prevention of complications, setting limits and health maintenance. Begin sex education. Monitor psychosocial development. Discuss delayed puberty. Clinic visits every 4 to 6 months. Start regular eye examinations. Do CBC and urinalysis. Rule out gallstones.

12 to 18 Years - Monitor clinical status, sexual development. Assist parent and child in progressing to independence stressing self-management coping skills, and academic achievement. Discuss birth control, prevention of complications, learning physical limits, sexually transmitted disease, and substance abuse. Provide peer support. Provide realistic vocational counseling. Clinic visits every 4 to 6 months.

18 Years and Up -Monitor history, physical, CBC, Every 2 to 6 Months, retics, and UA with each visit. SMA 18, eye exam, PPD once a year. Screen for gall stones and aseptic necrosis if symptomatic. Assess knowledge and educate about sickle disease, pain management, prevention of complications, response to emergencies, and avoiding substance abuse. Provide access to birth control and genetic counseling. Teach the importance of learning limits, choice of vocation, hydration, diet, and dental care. Discuss disease variability, prognosis, and prospects for future therapy. Define psychosocial problems and support resources. Teach breast or testicle self examination.

Table 3. Sickle Immunization Schedule

1 Month Hepatitis B vaccine #2 (or 4 weeks after #1)

2 Months DPT, OPV, HibTITER Pneumococcal conjugate vaccine (PCV)

4 Months DPT, OPV, HibTITER, PCV

6 Months DPT, HibTITER ,Hepatitis B vaccine #3 (or 6 months after #2), PCV

7 to 11 Months HibTITER two doses 2 months apart

12 Months PPD, Influenza*

12 to 14 Months HibTITER one dose, PCV

15 Months MMR HibTITER Booster if initial 12 -15 months

18 Months DPT, OPV

24 Months Pneumococcal polysaccharide vaccine (PPV) and PCV if not given before

4 to 6 Years DT, OPV, PPD

5 Years Pneumovaccine Booster Pneumovaccine

12 to 16 Years Td, PPD, MMR booster

> 16 Years Every Year - Influenza*, PPD

Every 10 Years - Td

* For details and contraindications see package inserts or recommendations of the American Academy of Pediatrics or U.S. Public Health Service.

DPT = Diphtheria, Pertussis, Tetanus; HibTITER = Haemophilus B Conjugate vaccine

OPV = Oral Polio Vaccine; MMR = Measles, Mumps, Rubella; PPD = Tuberculin test

PCV = Pneumococcal conjugate vaccine 

PPV = Pneumococcal polysaccharide vaccine 

Td = Tetanus, Diphtheria - Adult dosage

*Sickle cell at high risk for influenza. See annual recommendations in Morbidity and Mortality Weekly Report

New Pediatric Immunization schedule from the CDC http://www.cdc.gov/nip/recs/child-schedule.PDF

Georgia Comprehensive Sickle Cell Center

Pneumococcal Conjugate Vaccine (PrevnarÔ ) Schedule 

Adequate nutrition with a balanced diet containing adequate protein should be stressed at all ages. Unless the diet is abundant in fresh fruit and vegetables, folate should be administered daily to all sickle cell patients to prevent bone marrow failure because the dietary intake of folate may not meet the increased requirements caused by the increased production of red cells by the bone marrow. Hemoglobin, hematocrit and reticulocyte counts should be checked periodically to identify pending problems. Iron preparations are to be avoided unless serum ferritin, iron, and TIBC levels document iron deficiency. Iron is recycled by the reticuloendothelial system and reused. Iron overload may become a problem later in life for the sickle cell patient who receives repeated blood transfusions, aggressive oral supplements, or parenteral iron administration.

It should be stressed that the life expectancy is unknown and that all individuals are expected to live long and productive lives despite their disease. Children and adults should be encouraged to develop hobbies and physical activities that they enjoy. They need to be educated about learning and respecting limits in activities that regularly precipitate complications such as pain episodes. Patients should be educated about the importance of drinking 2 to 3 liters of water or fluid per day. They should avoid extreme temperature changes by dressing properly in hot and cold weather. Excessive physical exertion without aggressive hydration should be avoided. Swimming in cool water often precipitates pain episodes. The patient should be encouraged to find their physical limits in sports and outdoor activities. Education about the specific harm smoking, alcohol intake, and recreational drugs do in sickle cell disease should be provided early and avoidance stressed. Educational and vocational goals should be set and actively pursued with positive reinforcement. Over protectiveness, family and health care over dependence, and chronic illness behavior should be discouraged.

Patients with sickle cell disease may be followed by primary care practitioners with knowledge about care of patients with sickle cell syndromes. If practical, all patients should also be seen periodically by hematologists with interest in sickle syndromes or at established sickle cell centers where specialized resources can be obtained when needed. These hematologists and sickle cell centers should have access to facilities for evaluation, treatment, counsel, and education of patients with any complications of the disease. Centers should have support staffs which include knowledgeable physicians, patient educators, genetic counselors, psychiatric support, vocational rehabilitation, occupational therapy, physical therapy, and other care providers working as a team to solve the variety of multidisciplinary problems this disease can present.

Special Concerns

Air Travel - There is definitely a connection with sickle crisis and flying in some patients. The major problem is a decrease in oxygen in the cabin air. The aircraft is only pressurized to about 7,000 feet which is low enough to get some people with sickle cell in trouble. The other problem is related to dehydration. The humidity in the aircraft is very low and fluid intake needs to be markedly increased before and during the flight. To prevent problems in patients who have experienced pain or complications on past flights and those with concurrent lung disease, supplemental oxygen at 2 liter per minute should be administered. Most airlines are willing to provide this but there will likely be a charge and arrangements will need to be made well in advance. The airlines usually require two weeks notice and a doctors letter that establishes the need for oxygen and also specifies the rate of flow. The patient should have a supply of all their medicines for the duration of the visit. The patient should carry a letter that summarizes the disease complications and the most recent laboratory results so that treating doctors will know their usual values. It is also important that all vaccinations are up to date. Taking in adequate fluids is important. Brief exercise will help but should be short in duration because of being disconnected from oxygen.

Sports - Encourage activities that require concentration and skill rather than endurance. Golf, martial arts, skateboarding, bowling, table tennis, and fencing are all good choices. Swimming in a heated pool is also recommended because of it’s low impact on the hip joints. "Extreme" or endurance sports (long-distance competitive racing, for example), which push the body to exhaustion and cause dehydration are likely to cause problems. Sports that involve cold temperatures (skiing, sky diving) or low oxygen (mountain climbing) will probably trigger sickle cell pain. Common triggers for increased sickle cell pain are dehydration, temperature extremes (both cold and heat), low oxygen, exhaustion (lactic acidosis), infection, and stress. Sickle cell pains show up in certain places in the body because of vaso-occlusion. Generally, pain affects the long bones or the vertebrae of adolescents with sickle cell, and the shin is a common location as well. Active youngsters need to pay particular attention to the hip, as avascular necrosis is extremely common in young adults with sickle cell and can be accelerated by repetitive injury from high-impact sports. Pain in the hip or knee should be evaluated by an orthopedist with sickle cell experience. Hydration before and during sports activity is recommended. Rest breaks every 20 minutes to avoid acidosis should scheduled.

For sprains and strains, avoid using ice. Try instead applying a cool--not cold--compress to reduce the risk of vasoconstriction, which can aggravate pain in the area.

Patients with avascular necrosis of the hip should not participate in sports that involve repetitive jumping (basketball, dance, and gymnastics, to name a few) that may cause further injury to the hip joint. If the patient has a very enlarged spleen, contact sports (football, hockey, lacrosse, etc.) present a risk of rupturing the organ, but the level of danger depends on the level of sports competition. Most elementary-school-age children can't hit hard enough to actually damage the spleen.

References for Background Section

Charache S, Lubin B, and Reid CD. eds. Management and Therapy of Sickle Cell Disease. Third Edition. NIH Publication No. 95-2119, 1995.

Sergeant G. Sickle cell disease. Oxford: Oxford University Press 1985:199.

Bunn HF and Forget BG. Hemoglobin: Molecular, Genetic and Clinical Aspects. W.B. Saunders. Philadelphia. 1986.

Huntsman RG. Sickle-Cell Anemia and Thalassemia: A Primer for Health Professionals. The Canadian Sickle Cell Society, Newfoundland. 1987.

Herrick JB. Peculiar Elongated and Sickled Shaped Corpuscles in a Case of Severe Anemia. AMA Arch Intern Med 6:517, 1910.

Hahn EV, Gillespie EB. Sickle cell anemia. Report of a case greatly improved by splenectomy and further observations on mechanism of sickle cell formation. Arch Intern Med 1927;39:233-54.

Pauling L et al. Sickle cell anemia, a molecular disease. Science 110:543, 1949.

Ingram VM. A specific chemical difference between the globins of human and sickle cell anemia haemoglobin. Nature 178:792, 1956.

Motulsky AG. Frequency of sickling disorders in US blacks. N Engl J Med. 288:31, 1973.

Green D, Scott JP. Is sickle cell episode a thrombotic event? Am J Hematol 23(4):317, 1986.

Rickles FR, O’Leary DS. Role of coagulation system in pathophysiology of sickle cell disease. Arch Intern Med 133:635, 1974.

Embury SH, et al. Effects of oxygen inhalation on endogenous erythropoietin kinetics, erythropoie sis and properties of blood cells in sickle cell anemia. N Engl J Med 311:291, 1984.

Guy RB, Gavrilis PK, Rothenberg SP. In vitro and in vivo effect of hypotonic saline on the sickling phenomenon. Am J Med Sci 266:267, 1973.

Haynes J Jr, Allison RC. Pulmonary edema. Complication in the management of sickle cell pain episode. Am J Med 80:833, 1986.

Dwarakanath GK, Warfield C. The pathophysiology of acute pain. Hosp Pract April 15, 1986:64b.

Fields HL, Levine JD. Pain mechanisms and management. West J Med 347:141, 1984.

Vichinski EP, Johnson R, and Lubin, B. Multidisci plinary approach to pain management in sickle cell disease. Am J Ped Hematol Oncol 4:328, 1982.

Friedman EW, Webber AB, Osborn HH, Schwartz S. Management protocol for sickle cell disease patients with acute pain: impact on emergency department and narcotic use. Am J Emerg Med 4:267, 1986.

Benjamin LJ, Berkowitz LR, Orringer E, Mankad VN, et al. A collaborative, double-blind randomized study of cetiedil citrate in sickle cell episode. Blood 67:1442, 1986.

Dover GJ; Charache S; Boyer SH; Vogelsang G; Moyer M 5-Azacytidine increases HbF production and reduces anemia in sickle cell disease: dose-response analysis of subcutaneous and oral dosage regimens. Blood 66:527, 1985.

Barrett-Connor E. Bacterial infection and sickle cell anemia. Medicine 50:97, 1971.

Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. N Engl J Med 314:1593, 1986.

Solanki DL, Kletter GG, Castro O. Acute splenic sequestration episode in adults with sickle cell disease. Am J Med 80:985, 1986.

Zola EM and McLeod DC. Comparative effects and analgesic efficacy of the agonist-antagonist opioids. Drug Intell. and Clin. Pharm. 17:411, 1983.

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Copyright © 1997 Sickle Cell Information Center
Last modified: August 12, 2004