Managing sickle cell disorder (2)

Rotimi Adesanya
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Treatment

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

Treatment may include antibiotics, vitamins, daily folic acid, blood transfusions, pain-relieving medicines, supplemental oxygen, fluid therapy and other medications; and possibly surgery to correct vision problems or to remove a damaged spleen.

Self/home management: It is advisable that a sickle cell patient drinks plenty of water, eat balanced diets that contain 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-malarials: Children with sickle cell anaemia should be given anti-malarial prophylaxis from time to time in order to prevent 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 new 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, the ultimate treatment

On September 29, 2011, 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. His 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.

A Nigerian couple, Mr. and Mrs. Ola Majekodunmi, based in England who reads my column on a weekly basis also shared their family experience and promised to render useful information to Nigerians who also want their children’s genotype SS changed to AA.

Their story: “My wife and I are more than happy to help in our own little ways. You can drop us an email for any specific questions regarding the new procedure that our daughter had, perhaps some parents may want to take that route too! It is liberating.

“My Nigerian family lives with me in England; our only child was, until June 2013, a full blown sickle cell patient! My wife and I have decided to share our experience and we think many Nigerian families may benefit from recent advances in medical technology.

“Our sickle cell journey was rough, unexpected and stressful, but we are now proud parents of a child that is free of sickle cell complications. Our daughter, after several years of sickle cell complications, was referred in June 2012 to the haematologist at the University College Hospital London.

“He agreed to perform a half-match (haplo-identical) Blood Marrow Transplant as a cure for our sickle cell child and the whole process started in December 2012. I am happy to report that the half-match BMT was successful, but as expected, not without some challenges.

“We have a whole new era ahead of our family and, especially our daughter whose childhood had almost been taken away as a result of the then sickle cell nightmare and life changing complications. We think many more affected children can benefit from this new cure for sickle cell.”

The Majekodunmis shared this experience from Dagenham, England, and they can be reached via delemajek@yahoo.com for any clarifications and information.

Meanwhile, on behalf of the Dabma Sickle Cell Foundation, I hereby invite my numerous readers to one-day summit on sickle cell disease drug management on the occasion of the World Sickle Cell Day, tomorrow Thursday June 19, 2014. The venue is NTA Network Centre, Ahmadu Bello Way, Victoria Island, Lagos.

•Concluded

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