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.

Treatment

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.

Pain control

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.

Management

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.

Anemia Is Usually Well Tolerated

General Instuctions For Sickle Cell Anemia Patients & Family

Prenatal diagnosis