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Management of Sickle Cell Disease (2)
Date Posted: 17/Oct/2019
Treatment:
 
Treatment for sickle cell anaemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. A person with sickle cell anaemia will need to make regular visits to hospital to check the red blood cell count and monitor their health.
 
Treatment may include antimalaria, antibiotics, vitamins, daily folic acid, blood transfusions, pain-relieving medicines, supplemental oxygen, fluid therapy, and other medications; possibly surgery to correct vision problems or to remove a damaged spleen
 
Self/home management: It is advisable that a sickle cell patient drink plenty of water, eat  balanced diets 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.
 
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. Anti-malaria: Children with sickle cell anaemia should be given antimalaria 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 (adjuvants) 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. It is only administered under strict medical supervision.
 
Drepanostat: It is a nature product drug for sickle cell disease. It relieves sickle cell crises, lessens the pains and reverses the sickling of the blood cells. Blood transfusions: Blood transfusions increase the number of normal red blood cells in circulation, helping to relieve anaemia and stroke.
 
Vaccinations: Pneumococcal vaccinations are given routinely to children with sickle cell disorder because they are particularly susceptible to pneumococcal infection. The incidence of infection can be minimised through the administration of pneumococcal 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 and make informed decisions.
 
Cure
 
There is a cure for sickle cell in the form of bone marrow transplantation (BMT). BMT for sickle cell has been around since the late 1980’s – although still not widely available. BMT for sickle cell is a procedure whereby cells from the bone marrow of a donor (someone who is Haemoglobin AA or AS) are transplanted into someone with Haemoglobin SS. Bone marrow cells are usually taken from the hip bone of the donor – who must have either Haemoglobin AA or AS.
 
Usually, a brother or sister of the patient is preferred. The patient is cured because; they no longer have sickle cell, crises and other symptoms of sickle cell, they begin to lead life normally like people who are Hb AA or AS without frequent visits to hospital.  After the bone marrow transplantation procedure, when their genotype is checked by a blood test, they are found to have become Hb AA or AS – depending on the genotype of their donor. Although the patient is cured – as evidenced by cessation of crises and other symptoms of sickle cell, as wellblood test showing a change in their genotype, they need to be aware that they can still pass the sickle cell gene to their children. This is important.
 
BMT is expensive: Most people travel abroad for the procedure and the total cost of the procedure, air travel, food and board for about 6 months for patient and care giver runs into millions of naira. It is done in Nigeria at the University of Benin Teaching Hospital (UBTH and in one or two private hospitals.)
 
The first BMT in Nigeria was on the 29th of September, 2011, when the University of Benin Teaching Hospital recorded the first successful bone marrow transplant by transmuting young Matthew from HBSS genotype to HBAA genotype, using stem cells donated by his 14-year-old brother. Matthew made history as the first Nigerian to undergo bone marrow transplant successfully in the country. Mathew’s genotype is now AA. Few other families have also been able to have their children with genotype SS change to AA by the transplant team at UBTH.

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