[YOUR HEALTH] Managing sickle cell disease (3)

On September 29, 2011, the University of Benin Teaching Hospital recorded the first successful bone marrow transplant by transmuting young Matthew Ndik from HBSS genotype to HBAA genotype, using stem cells donated by his 14-year-old brother. The parents were very excited because their son made history, though they complained bitterly about the high cost of the transplant. Matthew made history as the first Nigerian to undergo bone marrow transplant successfully in the country.

Treatment for sickle cell anaemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. A patient with sickle cell anaemia will need to make regular visits to the doctor to check the red blood cell count and monitor his/her health.

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Treatment may include antibiotics, vitamins, blood transfusions, pain-relieving medicines, anti- sickling drugs, blood transfusions, supplemental oxygen, fluid therapy, and other medications — possibly surgery — to correct vision problems or to remove a damaged spleen; and the ultimate ‘treatment,’ bone marrow transplant.

Self/home management: It is advisable that a sickle cell patient drink plenty of water, eat a balanced diet that contains all classes of food in the right proportions — including fresh fruits and vegetables; take regular  non strenuous exercises, keep warm in cold weather, and cool in hot weather.

They also need to take one tablet of folic acid daily, as well as malaria prevention medication regularly, as recommended by the doctor. They are also expected to use treated bed nets to reduce mosquito bites.

Antibiotics: Children with sickle cell anaemia may begin taking the antibiotic penicillin when they are about two months of age and continue taking it until they are five years old. It helps prevent infections such as pneumonia, which can be life-threatening to an infant or child with sickle cell anaemia.

Antimalaria: Children with sickle cell anaemia should be given anti-malaria prophylaxis from time to time in order to avoid crises.

Pain-relieving medications: To relieve pain during a sickle crisis, over-the-counter pain relievers and application of heat to the affected area should be deployed. Stronger pain medication can also be prescribed by the doctor.

Dietary supplements: Ciklavite, Trevor, Jobelyn and many more are some dietary supplements that play supportive role in the management of sickle cell disease.

Hydroxyurea: When taken daily, hydroxyurea reduces the frequency of painful crises and may reduce the need for blood transfusions. It may be an option for adults with severe case of the disease.

Hydroxyurea seems to work by stimulating the production of foetal hemoglobin — a type of hemoglobin found in newborns that helps prevent the formation of sickle cells. This drug is only administered under strict medical supervision.

Drepanostat: It is a new drug for sickle cell disease. It  is  fast  acting and relieves sickle cell  crises  within  a few days,  lessens  the  pains  and  reverses  the  sickling   of  the blood  cells. Drepanostat works by stimulating production of foetal hemoglobin.

Blood transfusions: Blood transfusions increase the number of normal red blood cells in circulation, helping to relieve anaemia. In children with sickle cell anaemia at high risk of stroke, regular blood transfusions can decrease their risk of stroke. In a red blood cell transfusion, red blood cells are removed from a supply of donated blood.

Oxygen supplementation: Breathing supplemental oxygen through a breathing mask adds oxygen to the sickled blood and helps the patient to breathe easier. It makes the sickled cells to have enough oxygen.

Bone marrow transplant: Bone marrow transplant offers the only potential cure for sickle cell anaemia; but finding a donor is difficult and the procedure has serious risks associated with it, including death. Bone marrow transplant involves replacing bone marrow affected by sickle cell anaemia with healthy bone marrow from a donor.

Vaccinations: Pneumococcal vaccinations are given routinely to children with sickle cell disorder because they are particularly susceptible to pneumococcal infection — the number one killer of children under five in the developing world. The incidence of infection can be minimised through the administration of pneumococcal conjugated vaccine.

Prenatal diagnosis: Couples for whom there is a real possibility of bearing children with sickle cell anaemia are able to find out the haemoglobin genotypes of their unborn child in early pregnancy.

Thus, timely and adequate information, support and counselling can be provided.

Those at risk of having an affected child have options. This condition can be diagnosed as early as the 12th week of pregnancy. Termination of pregnancy can then be considered. Others may choose to take the chance of having an affected child.

An alternate option to prevent having a child with sickle cell is through adoption and In-Vitro Fertilisation technology.

-Rotimi Adesanya (roayad@yahoo.com)

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