How a person with sickle cell disease can lead a healthy life

Ali Kibirango, 38, from Luwero District has three children living with sickle cell. When his first wife gave birth, and it turned out that the child had sickle cell disease, he separated from her, accusing her of bringing misfortune to his family. Then, he was advised to re-marry. Unaware that he was a sickle cell carrier, he married another woman who, would also give birth to two children with the condition.

Kibirango did not know that his children had sickle cell until he visited a doctor. “Several tests were done but the results would turn out negative until we went to Mulago Hospital and the results showed that my children were suffering from sickle cell,”says the father of 13. For Susan Kyambadde, four of her five children have sickle cell disease. She learnt about the condition four months after her first child was born.

“Until then, I had never imagined that I could be a sickle cell carrier. My husband and I were advised to test and to our disbelief, both of us were carriers,” she narrates.
They considered stopping having children but the doctors encouraged them, saying that they had a one in four chance, of having a child with a sickle cell.

Kyambade’s in-laws mounted pressure on her husband to marry another woman saying that her children were a curse to the family and all of them would eventually die.

Her husband however did not yield to the pressure.
“My first born is now 13 years old, and she will be sitting for her primary seven exams this year. And all of them are trying to live the best way they can, following doctors’ advice,” she says.

Inherited disorder
According to Dr Jane Aceng, the director general of health services, sickle cell disease is inherited, which means it is passed from parent to child.
To get sickle cell disease, a child has to inherit two sickle cell genes-one from each parent.

“When a child inherits the gene from just one parent, that child has sickle cell trait. Having this trait means that you do not have the disease but you are a carrier and could pass the gene on to your children,” she explains.

Sickle cell anaemia, the most common form of sickle cell disease comes about when two parents, both carriers of the sickle cell trait have a child.

How it happens
Dr Christopher Ndugwa, the head of the sickle cell clinic at Mulago Hospital says that the condition occurs when abnormal genes are inherited from both parents.
“When both parents have the sickle cell trait, there is a one in four chances that the child will have sickle cell disease,”Dr Ndugwa explained.

Medical experts say that the disease is considered a serious disorder in which the body makes sickle-shaped red blood cells instead of the normal disc-shaped blood cells.

The difference is that the normal blood cells move easily through the blood vessels, taking oxygen to every part of the body while sickled cells can get stuck and block blood vessels, which stops the oxygen from getting through. While sickle cell is a growing problem in Uganda, ministry of health says there is still no accurate data on the extent of its prevalence. Most health facilities are also not well equipped to handle patients with the disease.

Statistics based on selected districts in Uganda indicate that at least 30,000 children are born with the condition annually while 50,000 children under the age of die every year. According to Dr Ndugwa, 15 to 20 per cent of Ugandans are carriers of sickle cell anaemia.

Statistics
The prevalence is said to be highest among the Baamba in Bundibugyo District at 38 per cent, followed by Bakiga, Basoga, Bagisu and Lugbara at between 20 to 28 per cent and the Baganda, Itesots, Acholi, and Banyoro with the prevalence ranging from 16-20 per cent. The Uganda Sickle-cell Association says it has about 20,000 registered members while Mulago Hospital, sickle cell clinic has 9,000 registered patients, 90 per cent of whom are children between the ages of zero to 13 years.

How to cope with the ailment

According to Dr Christopher Ndugwa, most sickle cell patients can survive beyond the age of five with early intervention and treatment, adding that children with sickle cell usually die of other ailments like anaemia, pneumonia and bacterial infections.
Ruth Mukiibi, the president of the sickle cell association of Uganda says to minimize hospital or clinic visits, patients are usually advised on how to manage crises at home.
Food plan

She recommends that a combination of fresh fruits and vegetables be included on their daily meal plans mainly to boost their blood levels at all times since most of these foods and fruits contain folic acid.
She also says that sickle cell patients need to remain hydrated at all times.

Children below 12 years should take at least three litres of fluids, while those who are over 13 years should drink at least five litres of water every day.

The water not only helps them remain hydrated, it keeps them away from painful episodes since dehydration triggers painful crises. While blood boosting is a necessity for the survival of a sickle cell patient, Mukiibi warns that having very low blood levels may trigger strokes which can settle in any part of the body including the lungs or heart.

“A sickle cell patient should take blood tests every once in a while to ensure that they have enough blood,”says Mukiibi.
Dealing with pain

When it comes to diet, it is recommended that sickle cell patients stick to a balanced diet that includes half a kilo of beetroot every three months, beans and avocado leaves among others.

Sickle cell patients are also susceptible to very painful crises every once in a while depending on how well they are taken care of. This usually happens when the sickle cells block blood flow. When this happens, doctors recommend that the patient drinks a lot of fluids in order to flush out the cells that have blocked the flow of blood.

The other alternative is to immediately put them on intravenous fluid drip before they are taken to the hospital.
Mukiibi says that once the patient has drank enough fluids, they may not need to go to the hospital, unless the crisis persists.

For children, it is recommended that they take painkillers and keep in a warm place.

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