Doctors have asked patients with sickle cell disease to monitor their uptake of hydroxyurea, a common drug used to reduce the complications associated with the disease, after a TikTok user was admitted.
Timothy Kayanja, 23, also known as Jayden, was last Friday wheeled into TMR International Hospital, after a distress call was placed to the facility, suggesting that he was having ‘chest pain’ and ‘trouble with breathing’.
‘When he arrived, he was in severe pain, struggling to breathe, and his oxygen saturation was critically low at 70%. He was rushed to the intensive care unit (ICU) and placed on oxygen therapy. A healthy adult requires levels above 95%,’ Dr Erasmus Okello, his assigned critical care physician, said on Tuesday.
Tests revealed that he experienced an acute vaso-occlusive crisis, acute chest syndrome and had a history of using hydroxyurea, which was no longer responding to and had caused him ‘complications’.
Dr Okello described hydroxyurea as ‘a very vital drug in the care of people with sickle cell disease’, saying it helps the bone marrow produce a more resilient type of haemoglobin to assist patients to live a fairly stable life without complications or significantly reduce complications.
‘However, the drug comes with side effects, including depressing the ability of the bone marrow to rapidly produce certain cells, which preferentially increases the production of the fetal haemoglobin, the main oxygen-carrying protein in fetal red blood cells.
‘In the case of Jayden, when he was placed on hydroxyurea on four attempts, each time, his bone marrow’s ability to produce blood cells got significantly depressed, beyond what is expected, suggesting that his bone marrow is not very reactive in producing red blood cells,’ Dr Okello explained.
Adding: ‘This is a very delicate balance between having sickle cell disease and not being able to compensate by producing normal red blood cells; so, it was deemed that the drug was becoming far more toxic to him than would benefit him, and it was discontinued.’
According to Dr Okello, Jayden has been receiving folic acid, among other medications, to sustain his blood levels.
‘In the first world, there are other medications that have been developed but are still being studied and not widely used here; so, we don’t fully know their application in the clinical setting.’
Such medication, Dr Okello says, is meant to improve sickle cell disease; however, we don’t have them available in Uganda, and are not even used in the first world where they are available and are extremely expensive.
‘Hydroxyurea remains the cornerstone for treatment of sickle cell disease patients, but the side effects have to be monitored closely by your doctors, while the patients are supposed to periodically be reviewed,’ Dr Okello further explained.
The reviews will guide on how the drug is helping to build up the patient’s fetal haemoglobin in preference to sickle haemoglobin, if there are any side effects of significant bone marrow suppression from the drug, and other side effects that could cause a reduction or discontinuation of the dose.
It is unclear for how long Jayden used hydroxyurea, but he told this publication that he had previously been at Mulago National Referral Hospital and has been treating sickle cell disease for the past 20 years.
Shortage of haematologists
Dr Oscar Mugerwa, an attending physician at the same health facility, said Jayden no longer responds to hydroxyurea, and a bone marrow transplant is now the best chance to significantly improve his quality of life, and has linked him to haematologists.
Haematologists are medical specialists focused on diagnosing and treating blood disorders, including both cancerous and non-cancerous conditions.
Dr Mugerwa, however, says Uganda has not more than five haematologists, but they scheduled appointments with three, possibly on Wednesday (today), to prepare him for the bone marrow transplant.
Dr Daniel M. Talemwa, the Executive Director of TMR International Hospital, said they will continue supporting Jayden as he prepares for a bone marrow transplant, including conducting all necessary tests for him and the potential donor to maximise the chances of a successful outcome.
‘The cost of the bone marrow transplant ranges from between $ 40,000 (about Shs141.88 million) and $150,000 (about Shs532 million),’ Dr Talemwa said. However, as of Tuesday, Shs76.71 million and a similar amount in dollars ($21,677.3) had been raised through mobile money and a GoFundMe page, among other channels.
‘We encourage continued support as the fundraising effort aims for $100,000 (about Shs354.7 million) to cover the cost of both the transplant and the vital post-transplant care, which is crucial to reduce the chances of complications as well as rejection by Jayden’s body and ultimately, transplant failure,’ he added.
According to the World Health Organisation (WHO), globally, the prevalence of the sickle cell trait is estimated at 7% of the world’s population, translating to between 300,000 nd 500,000 children born annually with the disease.
Africa, according to the same agency, bears 80% of the global burden, while between 50% and 80 percent of the affected children die before their fifth birthday.
Uganda is said to rank fourth after Nigeria, the Democratic Republic of Congo (DRC) and Tanzania, with between 20,000 and 25,000 new-borns diagnosed with sickle cell disease each year.
The Ministry of Health has been rolling out awareness campaigns in both urban and rural areas, urging couples to include sickle cell testing in routine health checks.
Dr Roseline Achola, a technical officer at the Ministry of Health, says sickle cell is becoming a serious national health burden. ‘For those intending to marry, screening for the disease is critical to prevent further spread,’ she says.
In Uganda, the highest burden is in northern and eastern Uganda, where between 15 percent and 20 percent of the population carries the trait.