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    ​Recommendations for Pediatric

    Sickling Disorder Management for Health Professionals

    (FS, FSC, FSA)

    • These brief recommendations are in accordance with the recommendations of the National Institutes of Health Consensus Conference on Newborn Screening for Sickle Cell Anemia. They are intended to be used as minimal guidelines for disease management and are not intended to replace your individualized plan for the child's health care. For a chart of health maintenance for children and adolescents with sickle cell disease, click here. Ongoing careful pediatric medical follow-up, including both sick and well child care, is of the utmost importance. Parents must be educated about the increased susceptibility of these youngsters to infection and in the recognition of the symptoms of complications such as acute splenic sequestration crisis, pain crises and other vascular events. The importance of seeking prompt medical care for fever and/or pain must be stressed. Although the incidence of infection is decreased, infections may still occur when the child is being treated with prophylactic penicillin and the polysaccharide pneumococcal vaccine.
    • The use of prophylactic penicillin (as described in the New England Journal of Medicine 34:1593- 1599, June 19, 1986) is recommended beginning as soon as the child is diagnosed and continuing treatment at least until the age of 6 years.  Treatment beyond that age is best determined in consultation with a hematologist.

    The recommended dosage schedule is as follows:

    Penicillin VK Orally

    Age
    Dosage
    Frequency
    Under 3 years
    125 mg.
    2 times per day
    3-5 years
    250 mg.
    2 times per day

    L.A. Bicillin I.M.

    Over 2 years
    1.2 mill.u
    every 3 weeks
    • The choice of route is based on your professional assessment of family compliance, but the oral route is preferable.
    • It has been demonstrated that prophylactic use of penicillin, in conjunction with ongoing pediatric medical supervision, significantly reduces (by 84% or more) the high incidence of infection, and death from sickle cell disease in early childhood.
    • Children with sickle cell disease must be promptly evaluated for fever with prophylactic antibiotics used pending culture results.  Consult with pediatric hematology regarding specific guidelines if you have questions.
    • In addition to the standard age appropriate vaccines, immunization with pneumococcal conjugate vaccine (PREVNAR or PCV 13) is recommended when the child is 2, 4, 6 and 12 months of age, with pneumococcal polysaccharide vaccine (PNEUMOVAX 23 or PNU-IMUNE 23) being given at 2 and 5 years of age.  An influenza vaccination (flu shot) should be given each year prior to the winter flu season (October -early November) to children older than 6 months of age. Screening with Transcranial Doppler is recommended for all children with Hemoglobin SS or S-beta zero thalassemia (as described in the New England Journal of Medicine 339:5-11, July 2, 1998.   Children should receive screening with TCD at least yearly from age 2-16 years.  This procedure identifies children at high risk for strokes; > 90% of these strokes appear to be preventable with transfusion therapy.
    • Pediatric hematology consultation is needed to further support your ongoing monitoring of the child's hemoglobinopathy as recommendations regarding periodic laboratory evaluations are frequently updated. We recommend that each child with a hemoglobinopathy be seen by a pediatric hematologist at least once initially, and then at least once a year.  The following are resources for Pediatric Hematology in Maryland. :
    Johns Hopkins Hospital
    (410) 955-6132
    Fax (410) 955-8208
     
    Sinai Hospital
    (410) 601-5864
     
    University of MD
    (410) 328-2808
     
     
    Georgetown University
    (202) 687-2224
    Howard University
    (202) 865-4583
     
    Genetic Counseling is strongly recommended for the child's parents. At least one session at the time of definitive diagnosis and one follow-up session are recommended.  This will provide the family with an understanding of the child's hemoglobin disorder and support them in making informed decisions as to future pregnancies. Counseling is available at the genetic medical centers, genetic outreach clinics or community sickle cell programs (see enclosed list).
     
    Prenatal Diagnosis is available for "couples at-risk" of having a baby born with a hemoglobin disorder. These couples should be informed about prenatal diagnosis and be offered a referral to a prenatal diagnostic center.
    Johns Hopkins Hospital
    (410) 955-3091
    University of Maryland
    (410) 328-3815
    Sinai Hospital
    (410) 601-5853
    Greater Baltimore Medical Center
    (443) 849-3192
    Children's Medical Services at (410) 767-5585 will assist patients without insurance who are financially eligible to pay for needed services.

    Hemoglobin V:

    The identification of the less common hemoglobin variants is a side benefit of the sickle cell newborn screening program. The letter "V" (for variant) is used to indicate an unusual type of hemoglobin that cannot be definitively identified by our screening tests. Some variant hemoglobin cause health problems, others do not. A pediatric hematology consultation is required to determine the significance of the variant.


    Hemoglobin C Disease:

    Individuals with Hemoglobin C Disease have a mild anemia. This mild anemia usually does not cause any symptoms or problems. Iron and vitamins will not raise blood counts. No other therapy, treatments or special precautions are necessary. Other occasional features of hemoglobin C disease include hemolytic episodes, usually triggered by infection, splenomegaly and jaundice. Adults with Hemoglobin C Disease have an increased risk of developing gallstones. Many people with Hemoglobin C Disease have none of these symptoms.
     
    A pediatric hematology consultation is strongly recommended to establish a definitive diagnosis. Since our program is a screening program, our test results should not be considered definitive. A pediatric hematology consultation is recommended for each child with a hemoglobinopathy. Each child should have an initial evaluation and follow up visits as recommended by the consultant.
    Children with Hemoglobin SC disease require the same care as children with Hemoglobin SS disease, though they may have a more mild course.

    For More Information

    More detailed guidelines are available from the NIH National Heart, Lung and Blood Institute at http://www.nhlbi.nih.gov/health/prof/blood/sickle/sc_mngt.pdf and from the Mid-Atlantic Sickle Cell Disease consortium Practice Guidelines Workgroup at http://www.pitt.edu/~marhgn/guide.pdf