Sickle cell, the neglected killer
Apr 20, 2008
A WAVE of hot air wafts from the rotting ply-wood that seperated two cubical wards housing eight old beds. The cubicles serve over 60 patients at Mulago Hospital’s Sickle Cell Clinic. The two rooms with shuttered window glasses and no ceiling, serve as an injection wing, store, kitchen and office.
By Conan Businge
A WAVE of hot air wafts from the rotting ply-wood that seperated two cubical wards housing eight old beds. The cubicles serve over 60 patients at Mulago Hospital’s Sickle Cell Clinic. The two rooms with shuttered window glasses and no ceiling, serve as an injection wing, store, kitchen and office. That is the Sickle Cell Centre at Mulago Hospital.
The sickle cell disease is very easy to treat and can be reduced easily, but little or no attention has been paid to it. According to the officials at the Sickle Cell Foundation of Uganda, the health ministry does not fund sickle cell programmes.
“We asked the ministry to fund sensitisation programmes to encourage people to come for testing and treatment. They told us to look for a way of relating sickle cells to HIV/AIDS, which sounded unrealistic,†Ruth Makumbi, the foundation chairperson, says.
She adds that several people may be having sickle cells and since they do not carry out medical check-ups, they end up marrying carriers like them. “This means they will produce a real sickle cell child; who is likely to die before the age of five,†Mukiibi adds.
However, the director of health services, Dr Sam Zaramba, says money is sent as a consolidated fund, and “it is unfair for people to claim that the ministry does not care for sickle cell patients.â€
Makumbi says the majority of children born with sickle cells in Uganda die before the age of five.
The sickle cell disease is an inherited blood disorder that affects red blood cells. People with the disease have red blood cells that contain mostly an abnormal type of haemoglobin.
Sometimes the red blood cells become sickle-shaped, and have difficulty passing through small blood vessels.
Its main symptoms include anaemia, yellowing of the whites of the eyes, severe joint pains, swelling of the hands and feet, chronic wounds on the ankles in adolescents, chest infections and enlargement of skull bones.
Unlike other diseases, sickle cell has no treatment. However, there is free supportive treatment for every patient at the sickle cell clinic in Mulago, which also houses the association offices. But very few people in Uganda access the treatment.
About 5,000 sickle cell patients are registered with the association. Being the only one in Uganda, the clinic operates every day, receiving 200-250 patients a week.
At the clinic patients are treated for ailments resulting from the disease such as malaria, anaemia and pain. The clinic has no in-patient facility and only affords a day care room for critical cases. Admission cases are referred to the general hospital.
Zaramba says every hospital in the country has testing facilities for sicklers. However, he admits that there is lack of enough advocacy and counselling for patients.
“There is need to have all people tested before childbirth to avoid producing more sicklers,†Zaramba adds.
Dr. Deborah Nakiboneka of the Sickle Cells Clinic at Mulago Hospital says last year, they received 4,500 cases.
Sickle cell is the most common genetic disorder in Uganda, with one out of five people carrying the trait. Its spread varies among regions.
The Bamba in Bundibugyo are affected most with a 40% frequency. Among the Acholi, Iteso, Banyoro and Baganda, the trait frequency lies between 16% and 20%. The Lugbara, Bagisu, Basoga and Bakiga lie between 20% and 28%. The complication is lowest among the Bahima, Banyankole and Karimojong at 1% to 4%.
However, Nakiboneka says, there is no scientific explanation to this variation in the disease among tribes or regions.
She says its persistence in some areas is caused by endemic malaria.
It is shocking to note that over 25,000 babies are born with sickle cells a year and many of them die before they are diagnosed because there is no screening upon birth.
“Public hospitals say they cannot afford the tests yet each costs sh20,000 in private labs,†she says.
Sickle cells affect the haemoglobin, which is the part of red blood cells that transports oxygen in our bodies.
Under some circumstances, like reduced oxygen, cold weather, dehydration or illness, a sickler’s haemoglobin forms stiff fibres, which change the shape of the red blood cells to a sickle shape.
These are rejected by the body and are automatically destroyed, causing anaemia (lack of blood). If a lot of the red cells are destroyed in a short time, the patient suffers a haemolytic crisis.
This abnormal shape cannot move easily through the blood veins, thus causing blockage. Blocking of the blood vessels is what causes damage to tissue and severe pain among sicklers.
Blood is needed in sicklers when they become anaemic. Blood is also given when there is need to dilute the sickled red blood cells, such as in cases of a stroke and severe pain. Sicklers need blood transfusion during surgery or childbirth.
Sometimes an attack occurs when the blood gets pooled into the spleen and liver, which enlarge, leaving the person anaemic.
Since carriers have a mixture of normal and abnormal cells, they do not get any serious problem and go through life normally. They may never know their status unless tested.
Sickle cells can cause a stroke due to low blood levels. Unlike here in Uganda, in some countries, tests are done at birth to ascertain levels that may need blood transfusion every month.
The good news is that today, sicklers can live longer than they could 20 years ago. This is due to malaria prevention, vaccination and better nutrition.
Home care
Home treatment for sickle cells involves reducing pain and preventing complications.
Make arrangements with teachers or a tutor to help your child keep pace with his or her classmates when illness causes absence from school.
Explain to teachers that children with sickle cells may need to use the bathroom more often than others.
Educate teachers and other school employees about the signs and symptoms of the disease.
Written instructions will help school personnel know what to do and who to call in an emergency.
Children with sickle cells can usually exercise and play normally if they drink plenty of fluids during and after exercise.
Get regular rest breaks during rigorous exercise.
The child should always keep warm.
A WAVE of hot air wafts from the rotting ply-wood that seperated two cubical wards housing eight old beds. The cubicles serve over 60 patients at Mulago Hospital’s Sickle Cell Clinic. The two rooms with shuttered window glasses and no ceiling, serve as an injection wing, store, kitchen and office. That is the Sickle Cell Centre at Mulago Hospital.
The sickle cell disease is very easy to treat and can be reduced easily, but little or no attention has been paid to it. According to the officials at the Sickle Cell Foundation of Uganda, the health ministry does not fund sickle cell programmes.
“We asked the ministry to fund sensitisation programmes to encourage people to come for testing and treatment. They told us to look for a way of relating sickle cells to HIV/AIDS, which sounded unrealistic,†Ruth Makumbi, the foundation chairperson, says.
She adds that several people may be having sickle cells and since they do not carry out medical check-ups, they end up marrying carriers like them. “This means they will produce a real sickle cell child; who is likely to die before the age of five,†Mukiibi adds.
However, the director of health services, Dr Sam Zaramba, says money is sent as a consolidated fund, and “it is unfair for people to claim that the ministry does not care for sickle cell patients.â€
Makumbi says the majority of children born with sickle cells in Uganda die before the age of five.
The sickle cell disease is an inherited blood disorder that affects red blood cells. People with the disease have red blood cells that contain mostly an abnormal type of haemoglobin.
Sometimes the red blood cells become sickle-shaped, and have difficulty passing through small blood vessels.
Its main symptoms include anaemia, yellowing of the whites of the eyes, severe joint pains, swelling of the hands and feet, chronic wounds on the ankles in adolescents, chest infections and enlargement of skull bones.
Unlike other diseases, sickle cell has no treatment. However, there is free supportive treatment for every patient at the sickle cell clinic in Mulago, which also houses the association offices. But very few people in Uganda access the treatment.
About 5,000 sickle cell patients are registered with the association. Being the only one in Uganda, the clinic operates every day, receiving 200-250 patients a week.
At the clinic patients are treated for ailments resulting from the disease such as malaria, anaemia and pain. The clinic has no in-patient facility and only affords a day care room for critical cases. Admission cases are referred to the general hospital.
Zaramba says every hospital in the country has testing facilities for sicklers. However, he admits that there is lack of enough advocacy and counselling for patients.
“There is need to have all people tested before childbirth to avoid producing more sicklers,†Zaramba adds.
Dr. Deborah Nakiboneka of the Sickle Cells Clinic at Mulago Hospital says last year, they received 4,500 cases.
Sickle cell is the most common genetic disorder in Uganda, with one out of five people carrying the trait. Its spread varies among regions.
The Bamba in Bundibugyo are affected most with a 40% frequency. Among the Acholi, Iteso, Banyoro and Baganda, the trait frequency lies between 16% and 20%. The Lugbara, Bagisu, Basoga and Bakiga lie between 20% and 28%. The complication is lowest among the Bahima, Banyankole and Karimojong at 1% to 4%.
However, Nakiboneka says, there is no scientific explanation to this variation in the disease among tribes or regions.
She says its persistence in some areas is caused by endemic malaria.
It is shocking to note that over 25,000 babies are born with sickle cells a year and many of them die before they are diagnosed because there is no screening upon birth.
“Public hospitals say they cannot afford the tests yet each costs sh20,000 in private labs,†she says.
Sickle cells affect the haemoglobin, which is the part of red blood cells that transports oxygen in our bodies.
Under some circumstances, like reduced oxygen, cold weather, dehydration or illness, a sickler’s haemoglobin forms stiff fibres, which change the shape of the red blood cells to a sickle shape.
These are rejected by the body and are automatically destroyed, causing anaemia (lack of blood). If a lot of the red cells are destroyed in a short time, the patient suffers a haemolytic crisis.
This abnormal shape cannot move easily through the blood veins, thus causing blockage. Blocking of the blood vessels is what causes damage to tissue and severe pain among sicklers.
Blood is needed in sicklers when they become anaemic. Blood is also given when there is need to dilute the sickled red blood cells, such as in cases of a stroke and severe pain. Sicklers need blood transfusion during surgery or childbirth.
Sometimes an attack occurs when the blood gets pooled into the spleen and liver, which enlarge, leaving the person anaemic.
Since carriers have a mixture of normal and abnormal cells, they do not get any serious problem and go through life normally. They may never know their status unless tested.
Sickle cells can cause a stroke due to low blood levels. Unlike here in Uganda, in some countries, tests are done at birth to ascertain levels that may need blood transfusion every month.
The good news is that today, sicklers can live longer than they could 20 years ago. This is due to malaria prevention, vaccination and better nutrition.
Home care
Home treatment for sickle cells involves reducing pain and preventing complications.
Make arrangements with teachers or a tutor to help your child keep pace with his or her classmates when illness causes absence from school.
Explain to teachers that children with sickle cells may need to use the bathroom more often than others.
Educate teachers and other school employees about the signs and symptoms of the disease.
Written instructions will help school personnel know what to do and who to call in an emergency.
Children with sickle cells can usually exercise and play normally if they drink plenty of fluids during and after exercise.
Get regular rest breaks during rigorous exercise.
The child should always keep warm.