Sickle Cell Anemia Health Article

Treatment

Treatment usually includes frequent monitoring of red blood counts, antibiotics for infections, transfusions for aplastic crises and splenic sequestration when required, and oxygen as well as respiratory support for chest syndrome. Some patients with severe symptoms receive regular blood transfusions to prevent crises and/or other complications such as stroke and organ damage.

Children with sickle cell disorders are at risk of developing severe infections, and penicillin is usually prescribed to prevent dangerous pneumococcal infections.

Sickle cell pain can be managed with a variety of measures including the following:

• warmth, to increase the blood flow, by massaging and rubbing and by heat from hot water bottles and deep heat creams
• bandaging to support the painful region
• rest
• getting the child to relax, by deep breathing exercises and distracting the attention, and by other psychological methods
• use of pain-killing medicines (analgesics)

Analgesics should only be given as recommended by the treating physician. The gentlest analgesic usually prescribed is paracetamol, given three times a day (62.5 mgm under 12 months; 125 mgm 1–4 years; 250 mgm 4–10 years; 500 mgm 10–14 years; and 1 gm 15 years upwards). The next gentlest is codeine phosphate, given four times a day, at 1–2 mgm for every kilogram of body weight.

Bone marrow transplantation has been shown to provide a cure for severely affected children with sickle cell disease, but the procedure is not entirely without risk. In addition, the marrow must come from a healthy matched sibling donor and only about 18 percent of children with sickle cell anemia are likely to have a matched sibling.

Alternative treatment

Research contributed a great deal about sickle cell anemia from 1970 to the early 2000s concerning what causes it, how it affects the patient, and how to treat it. Scientists were as of 2004 starting to be successful at developing drugs that prevent the symptoms of sickle cell anemia and procedures that they hope should eventually provide a cure. Drug research is focused on identifying drugs, such as hydroxyurea, that can increase the level of fetal hemoglobin in the blood. Fetal hemoglobin is a form of hemoglobin that all humans produce before birth, but most stop producing it after birth. It has been observed that some children with sickle cell anemia continue to produce large amounts of fetal hemoglobin after birth, and studies have shown that these children have less severe cases of the disease. Fetal hemoglobin seems to prevent "sickling" of red cells, and cells containing fetal hemoglobin tend to survive longer in the bloodstream. Butyrate, a substance widely used as a food additive, was also being investigated as of 2004 as an agent that may increase fetal hemoglobin production.

Nutritional concerns

Thirst and dehydration caused by not drinking enough, even if thirst is not felt, are known to trigger sickle pain. Parents should accordingly monitor fluid intake closely.

Children with sickle cell anemia are anemic to various degrees. Most of the time they feel quite well, but if the anemia gets worse, they may feel very tired. Folic acid, a vitamin found in fruit and vegetables, supports making blood and anemic children especially need it to prevent them from becoming run down.

Prognosis

Sickle cell anemia is an inherited disease and lasts a lifetime.

Prevention

Both sickle cell trait and sickle cell anemia are inherited. Therefore, parents can pass it to their offspring. It is important for parents to get tested. If one partner has sickle cell trait and the other does not, their children each have a 50 percent chance of having the sickle cell trait, and a 50 percent chance of having normal hemoglobin. If one parent has sickle cell trait it is extremely important that the other parent be tested. If both parents have sickle cell trait, there is a 25 percent chance that the child will have normal hemoglobin, a 50 percent chance that the child will have the sickle cell trait, and a 25 percent chance the child will have sickle cell disease. If both parents have sickle cell trait and want to know whether the unborn child has sickle cell anemia, testing can be performed as early as the tenth week of pregnancy. If the results are normal, the parents can be reassured. If the tests show that the baby will be affected, the parents can be better prepared and they can make an informed decision concerning the pregnancy.