Long Surviving Sickler Dies At 62
One of Uganda's longest living sickler is dead. Nteyafa Masembe died on Sunday at his home in Bugolobi, Kampala at the age of 62.
Dr Ndugwa, an expert on sickle cell, said Masembe was one of the longest surviving sicklers known in Ugandan medical records. Doctors also believe he could have been one of the oldest sicklers in the world.
However, while talking to Bukedde, Ruth Nankanja, the president of Uganda's sickle cell association, said their longest living member is Blasio Wamala, 79, from Luweero.
Sickle cell is an inherited disease. Studies done in the 1960's say it is a growing problem in Uganda, with up 39% prevalence in some regions like western Uganda. Few studies have been carried out in the recent past. Ndugwa said more patients are reporting to the sickle cell clinic in Mulago.
The Prime Minister, Prof. Apolo Nsibambi, was yesterday among hundreds of mourners at a funeral service at Namirembe Cathedral in Kampala. Nsibambi talked of Masembe as a true professional civil servant.
Before he retired in 1993, Masembe was the Executive Director of the Uganda Commercial Bank main branch in Kampala.
"At one stage, he was in charge of credit. He executed his duties with professionalism and without favour. He was one of the handful of Ugandans who knew time management," Nsibambi said.
In his condolence message, the Kabaka of Buganda, Ronald Mutebi, said Masembe loved his kingdom. He talked of him as a developmental and true gentleman. Masembe's father was a chief in the Buganda kingdom.
Other mourners talked about Masembe as a respectable father, husband, civilised, humorous and a smart person in all his dealings. Mourners particularly referred to him as a good example to many youngmen to live a responsible and God-fearing life.
Masembe is survived by a widow and a daughter. His body was laid to rest yesterday in Mityana.
25,000 Sicklers Born Annually
OVER 25,000 children in Uganda are born with sickle cell disease every year, health officials disclosed on Monday.
"It is sad that the majority of them die within the first year of life," Dr Jacinto Amandua, the Commissioner for Clinical Services, said.
He was speaking at the opening of the Sickle Cell exhibition at the British Council in Kampala.
The exhibition is one of the week-long activities planned to create public awareness on the disease.
Amandua attributed the deaths to lack of proper care at the grassroots for the sicklers within Uganda's health delivery system.
Experts want the care and sickle cell tests on pregnant mothers to be integrated within the antenatal services and the primary health care system.
"If someone knows they have the disease it becomes easier to care for themselves," he said.
He added, "Most of the deaths can be avoided by simple interventions if the communities are sensitized."
Prof. Christopher Ndugwa, an expert on the disease said, "We are seeing an increment in the numbers. At least three new cases are diagnosed at Mulago Hospital sickle cell clinic weekly."
Mzee Bulasiyo, Uganda's known longest living sickler attended.
A new body, The Sickle Cell Association of Uganda (SUA) will be launched.
The chairperson, Ruth Nabbanja, said the association would offer counselling and sensitisation to reduce the stigma among the sicklers.
Uganda has one of the highest sickle cell prevalence rates in Africa although the exact numbers are not known.
Sickle cell is an inherited disease that affects the red blood cells and causes painful episodes to the patients when affected cells disrupt the flow of blood. Prof. Graham Serjeant, an expert said many people are carriers.
Kampala Tops in Sickle Cell
UGANDA has the highest number of people with the sickle cell disease trait carriers world-wide, an international expert on the disease has said.
Prof. Graham Serjeant from the University of West Indies said this on Tuesday while launching the Sickle Cell Association of Uganda at Kololo, Kampala.
Serjeant said the Bamba tribe in western Uganda has up to 40% trait prevalence according to studies. "This is the highest trait prevalence ever recorded anywhere in the world," he said.
25,000 Babies Die of Sickle Cell Annually
About 25,000 babies are born with sickle cell in Uganda every year and up to 80 percent of these do not live to be two years, Prof. Graham Serjeant from Jamaica has said.
Serjeant also said Bamba of Kasese and Bundibugyo have the highest frequency of sickle cell in the world, at a 39-45% frequency.
They are more likely to suffer from the hereditary disease that affects blood levels and is highly fatal.
Serjeant is in Uganda for 10 weeks on a sickle cell sensitisation drive. The disease causes enlargement of the spleen, leading to severe anaemia and death.
Serjeant was addressing journalists at the weekly cabinet briefing in Nakasero Thursday.
Serjeant said 16-20 percent of Baganda, Iteso, Acholi and Banyoro suffer from the disease; 20-28 percent of Bakiga, Basoga, Bagisu and Lugbara are likely to have it, while the Karimojong and Banyankore (1-4 percent) are least likely to catch it.
Doctors attribute the differences in prevalence rates to relatives' intermarriages, among other factors.
Serjeant said Uganda has done very little against the disease, indicating that the 50-year-old statistics by the colonial government could be much worse today.
He said even Mulago, the national referral hospital, had no study into the disease.
He said the methods of diagnosis were too old, making treatment harder.
"This should not be happening at a teaching hospital. It is the teaching hospital that determines the standard of medicine practised in Uganda," he said.
Minister of State for Information Basoga Nsadhu said if the disease could be tackled, the infant mortality rate could go down.
Today, of every 1,000 live births in Uganda, 88 infants die of preventable diseases.
The Sickle Cell Association of Uganda is hosting Serjeant.
The association chairperson Ruth Nankanja, 27, showed off 4-year-old Aisha Tezigattwa, whom with the advice of Serjeant, was operated on in Mulago to remove the spleen.
"Before she was four years, she had 31 hospital admissions and 39 blood transfusions until July last year when the operation was done. We couldn't stay a week without rushing to hospital, but she is perfectly healthy now," Aisha's mother, Bulya Sauda, said.
The association called on the ministry of Health and well-wishers to help fight sickle cell, which has largely gone neglected.
"We are not doctors, but we saw the pain we went through when we were children. We are sacrificing our lives to make these children's lives better. We are the Philly Lutaayas of sickle cell," Nankanja, a sickler, said.
Serjeant said Uganda needs 230,000 Pound Sterling (approximately Shs 575m) to study the disease and screen births at Mulago for three years.
Serjeant and his wife are giving the association Shs 10m every year out of their pension, to aid and sensitise parents with sicklers.
Five Million Ugandans Risk Producing Sicklers
Sickle cell disease is an inherited blood condition which cannot be cured, so why bother?
Sickle cell disease only affects 25,000 Ugandan babies each year, so why bother?
You get the disease by inheriting sickle cell genes from both parents and since these occur in 20% of the population 5,000,000 Ugandans are at risk of having a child with sickle cell disease, so what!
Sickle cell disease is not new to Uganda. Fifty years ago, pioneering research in Uganda showed that the frequency of the sickle cell trait varied between different tribal groups from levels of 1 - 4% among the Karamajong, Banyankole, Bahima, and Bakiga to 40% among the Baamba (the highest frequency ever described).
Figures of 16-20% occur among the Baganda, Iteso, Acholi, and Banyoro and 20-28% among the Bakiga, Basoga, Bagisu, and Lugbara. When two people carrying the sickle cell trait have children together, the risk of having a child with the disease is one in four for each pregnancy.
Among the Baganda, this works out once in every 100 pregnancies but among the Baamba, once in every 25 pregnancies. From the birth rate and the gene frequency it can be calculated that 25,000 babies with the disease are born in Uganda each year, making it one of the most common genetic disease.
A mutation of the haemoglobin molecule in the red cell responsible for carrying oxygen to the tissues causes the molecules to form chains when oxygen is removed, and these distort the red cells into the sickle shape.
These cells are rapidly destroyed in the circulation causing anaemia, jaundice and gallstones but also block flow in blood vessels causing damage to tissues all over the body. These problems include damage to the spleen so the child is prone to serious infections and acute severe anaemia, the brain-causing strokes particularly in young children, the lungs producing a pneumonia-like problem, the bone marrow causing the painful crisis of the disease, and the skin which may result in chronic ulcers around the ankles.
The disease cannot be cured so what can we do?
Regular penicillin prevents the serious blood infections affecting sickle cell children in non-malarial areas. The spleen, an organ under the ribs on the left side of the belly can become suddenly enlarged trapping the baby's blood and leading to death. Teaching mothers to feel the spleen has reduced the death rate from acute enlargement of the spleen by 90% in non-malarial areas.
Simple education can inform the mother how to manage the child, ensure complete immunisation, improve nutrition, avoid many of the serious complications, and others can be treated more effectively if diagnosed early. In Jamaica, the median life expectancy for the most severe form of sickle cell disease, SS disease, now exceeds 50 years.
What are the figures for Uganda?
There are none because there has been little research on the disease over the last 40 years in Uganda. Having been at the forefront of sickle cell research 50 years ago, other communities have raced ahead in improving their management of the disease.
Other people have been doing research on the disease so why not apply the findings and interventions described elsewhere
The truth is that malaria markedly affects the expression of sickle cell disease and applying interventions from non-malarial areas may give a false sense of security.
Information from malarial areas, mostly from Nigeria but also one study from Uganda, show that the bacteria causing serious blood infections are not sensitive to penicillin so we would be wasting our time and money.
What about teaching the mothers to feel the spleen?
There is no data to show that this is appropriate here. Also, malaria modifies the spleen in sickle cell disease.
So either we study our disease in Uganda and develop our own interventions appropriate to our patients or we just let them die.
Most people would feel that the latter is unacceptable in 2003 and should have been unacceptable over the last 20 years when so much research on the disease has been done elsewhere.
There are limited resources in Uganda.
Of course there have been other serious epidemics such as HIV/Aids and malaria but these are being addressed by huge resources, multiple research groups, international agencies, charities both in and outside Uganda.
Unfortunately, the Sickle Cell Clinic at Mulago Hospital does not even have adequate haemoglobin electrophoresis which is the basic test for diagnosing sickle cell disease.
It does not have access to a blood analyser considered basic to the management of the disease elsewhere. Were it not for the clinical expertise of specialists such as Professor Christopher Ndugwa of the Department of Child Health, the mortality rate would be even higher than it is now.
Between 10,000 -15,000 of Ugandan babies born with sickle cell disease each year are dead by the age of two years and that the median survival is of the order or one to two years.
You have to decide whether this is important enough. Remember that one of the indices of civilisation is the extent to which society protects those who are most vulnerable.
Graham Serjeant is Chairman, Sickle Cell Trust (Jamaica); Professor Emeritus, University of the West Indies