What is Sickle Cell Disease ?

    Sickle cell disease is an inherited disorder in which red blood cells (RBCs) are abnormally shaped. All the problems in sickle cell disease are due to its shape, which resembles a shaped farm tool called a sickle. Normal red blood cells are flexible and flow easily but in sickle cell disease due to its deformity, they are stiff and can get stuck in a tiny blood vessels cutting off the blood supply to nearby tissues. This abnormality can result in painful episodes, serious infections, chronic anemia, and damage to body organs. These complications can, however, vary from person to person depending on the type of sickle cell disease each has. Some people are relatively healthy and others are hospitalized frequently. Today with early diagnosis and treatment, most kids born with this disorder grow up to live relatively healthy and productive lives.

    The goals of Sickle Cell treatment are symptom management and management of disease complications. These include management of vasoocculusive crisis, management of chronic pain syndromes, management of chronic hemolytic anemia, prevention and treatment of infections, management of complication.

    Administration of hydroxyurea as a treatment at a dose of 10-15 mg/kg body weight Hydroxyurea increase the production of HbF, which retards gelatin and sickling. The hemoglobin level rises and the frequency of vasoocculusive crisis declines. A decrease in chronic pain and the reticulocyte count occur. Hydroxyurea is a potentially leukemogenic and carcinogenic agent requires CBP monitoring every month. Selection of patient for Hydroxyurea. Patients having frequent painful crisis (6 or more per year) Severe unremitting chronic pain that cannot be controlled by conservative measures.

    • Long-term follow-up is required for patients with SCD. This is a life long disorder
    • The frequency of out patient visits depends on the patients clinical status
    • For patients with minimal symptoms visit with blood every 3-4 months is reasonable
    • Others may need more frequent observation
    • Plenty of fluids to be given around 125 ml per Kg per day
    • Treat all infection even trivial one very promptly and vigorously
    • Institute pain medication at the earliest symptoms of a vasooculusive crisis
    • Patients on a chronic regular transfusion need iron chelation therpy
    • Tab. Folvite one tablet daily
    • Tab.Sodamint 1 to 3 tablets 3 times in a day

    There are a variety of approaches that can be used to treat pain associated with Sickle Cell Anemia. They included such medications as paracetmol, codine and tramadol. Consult your physician before beginning any medical regime. The addition of tricyclic antidepressants may reduce the dose and need for opiates by interfering with pain perception. Some patients with chronic pain can become depressed and managing depression has a statutory effect on the pain. There are also a variety of non-pharmacological approaches including support groups, heat and cold applications, acupuncture and acupressure hypnosis and transcutaneous electric nerve stimulation (TENS). In addition, a mild antibiotic regimen is suggested for children, especially for those between the ages of 4 months to 5 years.

    • Because of high turnover, folate stores are depleted. Folic acid supplementation must
    • Women who are menstruating should checked for co-existing iron deficiency if found give iron supplements
    • An overall balanced diet is essential
    • Blood transfusion indicated only in specific situations
    • Acute chest syndrome
    • Stroke
    • Pregnancy
    • General anesthesia
    • The aim is to decrease concentration of HbS to 30 % or less
    • Chronic and repeated transfusion leads to alloimmunization and iron overload
    • Surgical care is limited to treat disease complications
    • Skin grafts can help heal chronic leg ulcers
    • Hip replacement or other orthopedic procedure can be used to treat a vascular necrosis
    • Resistant priapism may require surgical draining of the penile corpora
    • Cholecystectomy may be needed for gall stones

    • Prenatal diagnosis also is available which enable one to know whether the unborn child is affected with disease or not. The laboratory procedures employed in prenatal testing are sensitive and rapid. Such prenatal testing must be accompanied with genetic and psychological counseling
    • DNA prepared from a biopsy of chorionic villus can be obtained at 8-12 weeks gestation. The procedure is safe and low-risk
    • DNA from amniotic fluid cells can be examined at 16 weeks
    • Investigational attempts are ongoing to isolate fetal cells from maternal blood for DNA assay