Monica Amooti Nakibuuka used to spend more time in hospital than at home. This is because her son, Moses Kyazze has sickle cell disease and has suffered stroke three times.
After Kyazze had an apheresis there are fewer admissions now.
Apheresis is a three-hour process which involves red blood cell exchange. Here, the sickled red blood cells are sieved and are replaced with safe sickle free red blood cells. The procedure is not a permanent cure but makes a sickler’s life better with less hospitalisation.
According to Nakibuuka, previously, they would go to hospital twice a month because Kyazze used to fall sick all the time.
“These days, he rarely falls sick so we only go once in a while for review. The number of transfusions has greatly reduced and his heamoglobin count is usually between 10 and 12,” she says.
Sickle cell disease (SCD) is an inherited red blood cell disorder caused by an abnormality of the haemoglobin. It is characterised by chronic destruction of the red blood cells and painful crises, which in turn cause severe acute and chronic organ damage. There are about 5,000 sickle cell patients under the Sickle cell Association of Uganda (SAU).
However, many lives were lost during the first lockdown because of underlying problems which could not be identified since they died from home.
However, Ruth Nankanja Mukiibi, the SAU founder says the lack of transport cost many sicklers their lives.
People with sickle cell disease depend on daily medicines such as folic acid which is taken every day for a lifetime, anti-malarial drugs which may be taken on a weekly or monthly basis and some have to take hydroxyurea.
“Many of them ran out of these drugs and in the absence of blood boosters, we are advised to use beetroots but they took too much of it and an excess increases blood levels.”
With sickle cell disease, low levels of blood are dangerous but also when it is high, the vessels can pour out which increases the risk of a stroke if it happens in the brain and death if it is in the lungs. In the less severe cases, one can get a nose or ear bleed, urinate or cough blood.
“We tried to identify the extremely needy patients, did a needs assessment on the medicines they use when they get a crisis and we delivered them at their doorsteps using bodaboda but the numbers are many and we could not afford for everyone,” Nankanja says.
Kyazze was fortunate. His first apheresis procedure was done in January 2019, another in July 2019 and the third was in March 2021.
His immunity has also greatly improved.
“Every flu or cough previously meant an admission with cylinders of oxygen and very scary nights. Now I only get him tablets or syrups and he gets better,” his mother says.
Nakibuuka says apheresis is not a cheap procedure, but it is still cheaper than the amount spent on hospital bills in six months, the painful crises, the horrible six or even more blood transfusions. But the worst is watching your loved one in pain.
“It is worth it,” she says.
Moses’ procedure cost Shs2.8m and Shs300,000 for inserting the central line but the cost depends on the amount of blood one uses.
Apheresis is only used for sickle cell patients with persistent blood vessel blockage that bring out the painful crises or stroke.
In Uganda, the procedure is only done at the Joint Clinical Research Centre (JCRC) in Lubowa.
Besides the daily intake of folic acid and routine anti-malaria treatment, there are advancements the government has made when it comes to sickle cell treatment according to Nankanja.
The government has put hydroxyurea on the essential drug list and it is freely available at Mulago while in the pharmacies, a tablet costs Shs1,000.
“Hydroxyurea is a wonder drug for many sicklers. The pneumococcal vaccine has been introduced into the vaccination routine for all babies. One of the major causes of death among babies with sickle cell disease was pneumonia,” Nankanja says.
Penicillin injection during immunisation has also been helpful because children with sickle cell are prone to infections and this has improved the quality of life.
What can be done better?
According to the Ministry of Health, there should be a sickle cell clinic at every health centre IV. However, there are about 10 sickle cell centres in Uganda and Mulago, being the major one that opens from Monday to Friday is overwhelmed by the numbers.
Nankanja says, “Elsewhere in the regional referral hospitals, clinics operate once a week. Everyone will not fall sick on that day or even come for a checkup on that day because the numbers are many.”
The screening programmes should be taken up to the health centre IVs to increase awareness and screening among the people. The National Health lab in Butabika does outreach and mobilises for screening but we can do better by availing such services in the lowest level health units.