Living on the edge with sickle cell anaemia

“I am the first born of four children but all my siblings are normal. My parents found out I had sickle cells when I was only six months.
Then, the doctor told my parents to break up because they were going to have only sicklers.

He advised them not to waste resources on me because I was going to die soon. All through my childhood, I remember the many dos and don’ts my parents subjected me to. I would be kept in the house whenever it rained while my siblings played. I attended only day schools and I thought that my parents never loved me.

I was always a special child who was kept on medicine, put in sweaters all the time and would go for routine medical assessments. My parents had never told me what my problem was but I would hear people say I was going to die anytime, until I was in Primary Four.

There was a time I hated my parents because they were being overprotective. When they told me I had sickle cells, I thought they did not want to waste any resources on me since they knew I was going to die soon.

Whenever I went for my routine assessments, I was scared because there were no adult sicklers so I had started to believe what people say that sicklers do not live after 15 years.

My worst disappointment was that at the sickle cell clinic, they never taught us how to prevent the attacks which were very painful.

Up to now, I still blame the department for keeping us in the paediatric department even when we were mature.

My worst blow came when I was in Primary Three. My class teacher had received a notification letter that I was not supposed to engage in vigorous exercises.

She shouted on top of her voice that I had sickle cells and was not going for physical education (PE) so I had to keep other children’s uniforms.

From that day, children stopped associating with me and no one sat next to me.

There were many stereotypes and stigma as children would tell me how their parents told them that I would not grow up. Since then, I vowed never to tell anyone that I had sickle cells unless they found out from someone else.

When I made a mistake, my mother would beat me like any of my siblings and she never minded me getting an attack afterwards.

She taught me to be hard working although my father told me not to force myself to do things that seemed hard for me.

The most stressful moments were whenever I was in a candidate class. I would have the stress of the candidate class and the fact that I was about to die. Sometimes, I felt hopeless and lost focus.

When I completed my A- Level, my parents insisted that I enrol at the university as an education student because they thought this was what I had to do. In my second year at university, I got an attack which was so painful and I had never told anyone that I was a sickler.

They heard me groan and cry in pain but when they offered to take me to hospital, I refused. Instead, I reached for my medicine kit and injected myself with painkillers under the blanket.

When I graduated, I taught for only one and half months and resigned because this was not my passion. In 2000, I declared to the public through the media that I had sickle cell disease because I was tired of hiding.

I received phone calls with insults and abuse from people telling me that no one was going to marry from my family but I got married in 2003. During our courtship, all we did was study about sickle cells because my husband is normal.

When I got pregnant, a certain doctor told me not to shop for the baby because sicklers do not give birth. I gave birth to a baby girl by C- section not because I would fail to push due to sickle cell but because my uterus was small.

Caring for a sickler at home

According to Dr Herbert Luyima, a clinical medical officer at the sickle cell clinic in Mulago hospital, children with sickle cell disease should be given folic acid daily to help them in the production of red blood cells.

Depending on the medical worker’s advice, Dr Luyima advises giving sicklers weekly or monthly malaria preventive drugs to protect them from malaria.

In addition, use mosquito repellants and ensure that a patient sleeps under a treated mosquito net.
“The patients must drink a lot of fluids; that is, three litres for children and five for adults. Oral rehydration solution is also vital to help keep them hydrated,” Luyima adds.

Watch out for the colour of the child’s palms, lips and tongue daily and if they turn pale or yellow, rush him to hospital immediately.
Patients must eat a balanced diet daily but more so fruits and vegetables.

Find the cause of pain and help them address the problem. In case of a fever, the patient should be given painkillers and rushed to hospital.
“If the patient is pregnant, she should attend two sickle cell antenatal visits in addition to the four normal visits,” says Dr Mutungi.

Facts about sickle cell anaemia

Sickle cell disease is one of the commonest genetically inherited blood disorders affecting both children and adults worldwide.

“The average prevalence of sickle cell disease in Uganda is 11 per cent although there are regions where there is high prevalence like the mid north Acholi- Lango region, Bundibugyo and some parts of the eastern region where the percentage is 20,” says Dr Gerald Mutungi the programme manager non- communicable diseases prevention and control at the Ministry of health.

He adds that the prevalence is as low as three per cent in the south western region of Uganda.

According to recent study estimates contained in a ministry of Health statement, approximately 10,000 to 15,000m babies are born with sickle cell anaemia annually in Uganda and 80 per cent of these children die before the age of five because of late or missed diagnosis due to infections such as sepsis, anaemia and malaria.

“The cause for the early deaths and devastating complications is due to the fact that very few people have access to early diagnosis and comprehensive medical care,” says Dr Mutungi.

For a very long time, people thought that having sickle cell was a death sentence where victims were thought to die at five years. However, with improved healthcare services, people with sickle cell disease can survive up to 60 years.

Dr Mutungi warns that sickle cell disease is a common, neglected but serious non-communicable disease and thought to be common in people from regions where malaria is endemic.

Sickle cell disease occurs in children whose parents are both carriers of the sickle cell gene. Sometimes, parents never realise that they are carriers (traits) because carriers never get ill.

The first and largest sickle cell clinic is at Mulago National Referral Hospital while three others have been opened at Nsambya hospital, Jinja hospital and Kasana health centre IV in Luweero.

Diagnosis and treatment

Sickle Cell Trait and inheritance
The sickle cell trait is an inherited condition in which a person is a carrier of the sickle cell gene but never suffers the sickle cell attacks. Such people are genetically healthy but may pass on the gene to their offspring.

If one parent has sickle cell anaemia and the other is normal, all the children will have sickle cell trait but will have normal genes.

If one parent has the sickle cell trait and the other has sickle cell anaemia, there is a 50 per cent chance of having a baby with sickle cell disease or trait with each pregnancy.

When both parents have sickle cell traits, they have a 25 per cent chance of having a baby with sickle cell with each pregnancy.

“Therefore, before you decide to have children, it is important that you go for screening as a couple so as to ensure you do not pass on the gene to your offspring,” warns Dr Mutungi.

How to prevent attacks
The patient should take plenty of fluids to avoid blockage of the veins by the sickle cells. Getting enough rest is good for a sickler as well as skipping strenuous exercises.

Dr Herbert Luyima, a clinical medical officer at the sickle cell clinic in Mulago, advises sickle cell patients to use a hot water bottle or warm piece of cloth to warm up the area where the pain has originated and avoid very cold places.

“Keep all medical appointments even when the patient feels normal and ensure the children get pneumococcal vaccination to protect them from infections,” Luyima warns.

Signs and symptoms
A person with sickle cell disease will suffer from rapid destruction of the red blood cells causing anaemia and jaundice (yellowing of the eyes)

In young children, there may be pain and swelling of fingers or toes. The swelling is caused by damage of the spleen whose role is to filter blood.

The patient gets frequent infections, becomes anaemic and there is stunted growth in children.

The complications
Sickle cells are destroyed rapidly in the body of people with the disease, causing anaemia, jaundice and the formation of gallstones.

The sickle cells block the flow of blood through vessels resulting in lung tissue damage, acute chest syndrome, sickle cell crisis which are characterised by painful episodes in the arms, legs, chest or abdomen, stroke and priapism which is painful prolonged erection in sickler men. The disease also causes damage to most organs including the spleen, kidneys and liver.

Damage to the spleen causes sickle cell disease patients, especially young children, to be easily overwhelmed by certain bacterial infections.

The numbers

10,000: Estimated number of children born with anaemia annually in Uganda.

80%: Of children born with sickle cell anaemia die before five years of age.