Tackling sickle cell disease

That means sickle cell disease remains a grim reality for many. Sickle cell causes among others lameness, growth retardation, stroke, priapism – unwanted sustained erection and blindness.

Currently, over 300,000 sickle cell births occur in sub-Saharan Africa every year. However, if no action is taken, the World Health Organisation predicts that the number of children born with sickle cell may climb another 30% by 2050.

A 2014 surveillance study by the Ministry of Health found that 13.3% of the population had a sickle cell trait whereas 1.3% of the populations are sicklers.  The same study indicated, of the 112 districts, 49 had a sickle cell trait of above 15% of the population with the North, East and Central regions having the biggest burden. The Central Public Health Laboratories opened a sickle cell laboratory in 2014 to ensure that big numbers of people get access to screening services and knowledge on how to cope with the disease.

Coping with the disease

Jeremy is celebrating his ninth birthday this year. But between 15,000 and 20,000 children born with the disorder die every year, 80% of whom do not make it to their fifth birthday.

But Prof. Nduggwa says the disease is no longer a painful guarantor of a short life. He says those who manage the symptoms well live a full life like any other person.

“Before when research into the disease was still small, we focused on treating symptoms whenever a patient got a crisis. Now, our focus is not allowing these symptoms to occur”.

He mentions hydroxyruea, a drug that Evelyn calls ‘a savior’. Ever since her son was initiated on this drug about two years ago, the pain and hospital admissions have reduced. The doctor says this drug initially meant for cancer was introduced in Uganda in 2013 after an efficacy study was conducted at the clinic and found that it greatly reduces complications that come with the disorder.

Another pediatrician Dr. Nicholas Nanyenya tells The Independent that for each symptom, they have found a solution. He says because a sickler’s spleen which is normally responsible for filtering cannot perform its function because of the blood’s sickle shape, patients usually get an enlarged mass on the left side of their stomach and are prone to infections. To prevent this, doctors recommend a prophylaxis antibiotic regularly with Penicillin being the most appropriate drug for those below five years. The doctor says if children are confirmed to have the disease early enough, starting them on the antibiotic immediately greatly helps in countering severe complications caused by bacterial infection.

Since one of the biggest challenges for sicklers is anemia, the doctor says on a daily basis they should be given folic acid or iron rich meals in addition to keeping them rehydrated by taking at least three liters of fluids per day.

Also, malaria in sicklers is responsible for most admissions; doctors say an anti-malarial drug Fancidar should be taken on a monthly as prevention. Fancidar, unlike other drugs, persists for long in the blood. They also advise patients to avoid strenuous exercise in addition to keeping warm at all times.

Nanyenya says sickle cell needs constant reviews meaning parents should always consider going to hospital early enough because many times it can be quite unpredictable whether the crises will be severe or not.