Monday August 25 2014

Medical options for people with sickle cell anaemia

By Sandra Janet Birungi

Ten-year-old Hawa (not real name) had saliva running down her mouth. She is living with sickle cell anaemia, which her parents discovered when she was three.

“We did not know she had the disease. She is the only child in the family who has it,” Hawa’s father, Asuman (not real name) says, as he wipes off the saliva.

“She used to suffer strokes but the major one came when she was eight, and it paralysed her,” he says.
Sharif Kiragga Tusuubira is also living with sickle cell. “When I tell people about my condition, they get shocked. When I first told my wife before I proposed to her, she thought I was joking,” says Tusuubira, who is the executive director of the Uganda America Sickle Cell Rescue Fund.

In Uganda, there is an estimated 25,000 children born every year with the sickle cell gene, although they do not necessarily end up with the condition. Dr Phillip Kasirye, the head of the sickle cell clinic at Mulago National Referral Hospital, says they have over 10,000 registered patients, with 50 new ones registered every month.

“The clinic has many cases because each time a doctor from a different facility finds out a patient has the disease, they are referred here,” he says.

The disease also accounts for 16 per cent of deaths among children under the age of five in Uganda.
Prof Christopher Ndugwa, a researcher and one of the pioneers of the sickle cell clinic says the way the disease affects individuals varies due to the existence of the foetal haemoglobin (HBF). A low haemoglobin makes a sickle cell patient prone to infections and chronic organ damage, which may lead to death.

“Early diagnosis increases survival rates. The way each patient responds will depend on the level of haemoglobin in the body,” says Prof Ndugwa.
Dr Kasirye says part of the challenge in dealing with sickle cell is the limited number of treatment centres in the country. “There are only five sickle cell clinics, compared to the number of people who suffer from the condition.”
Doctors who handle the condition are also few, but Dr Kasirye says any trained doctor should be able to treat sickle cell.

Tusuubira says the America Uganda Sickle Cell Rescue Fund has trained about 15 counsellors to be able to educate parents, medics and patients on how to manage the disease, deal with the stigma, as well as help during severe attacks.“For example, boys who have sickle cell usually suffer from painful erections. Talking to the parents, doctors and the patient on how to manage the situation is important,” he says.

Public education
According to Dr Kasirye, sensitisation about the disease will help reduce stigma.

Medical options
Several medical options are available for people living with sickle cell disease. A new drug, hydroxyurea, has been successfully tested in Europe to treat the disease, but it is yet to be tested in malaria prone regions especially in Africa.
Used as a cancer drug, hydroxyurea reduces the formation of sickle cells.

According to Dr Irene Lubega, a consultant paediatrician at Mulago hospital, hydroxyurea reduces the painful episodes, which often lead to hospitalisation.

Prof Ndugwa said even though the drug is already on the market in Uganda, there is no data on its effects to people in in malaria prone areas.
“A study is underway to establish its effects in children under five in Uganda,” Prof Ndugwa says. The drug is available in 200 to 500 miligramme capsules and in liquid form, costing between Shs1,500 and Shs2,000 per tablet. Dr Gerald Mutungi , the programme manager in charge of non-communicable disease at the health ministry says they have applied to National Drug Authority to license hydroxyurea, and to have it distributed at all government hospitals.

Folic acid
This is useful for the development of red blood cells. Every person living with sickle cell should take a continuous supply of folic acid, to avoid developing severe anaemia.

People living with sickle cell should be immunised with penicillin prophylaxis, to reduce their risk of developing bacterial infections because of a reduced immune system.
Pneumococcal and homophiles are the common vaccines used. In Uganda, the pneumococcal vaccine is available for all children below the age of five.

Bone marrow transplant
Although this is one of the options to manage sickle cell anaemia, Prof Ndugwa says transplants are rarely successful. He says for instance, only 500 successful transplants have been undertaken in the US, which has more advanced technology than Uganda.