Why sickle cell disease need not be a death sentence

By Kiundu Waweru

The news of an 80-year-old man who died in Uganda last year may not have made it to any news pages or television. But to people living with sickle cell anaemia, his life and death is more important than any breaking news as he lived to that ripe age with the sickle cell disease.

For many years, the disease was called a ‘paediatric disease’ because many did not live to adulthood. But now there is hope that people born with the disease can lead as near normal a life as any other person.

Sickle cell anaemia, an inherited and chronic blood disorder, claims many children annually specifically due to late diagnoses.

Dr Kennedy Wafula, a paediatrician at the Aga Khan University Hospital, says the disease is caused by deformity of red blood cells. The red blood cells contain haemoglobin, a protein that carries oxygen in the blood.

Dr Kennedy Wafula talks about available new treatments. Photos: Maxwell Agwanda/Standard

"Normal red blood cells are round and flexible, which enables them to travel through small blood vessels to deliver oxygen to all parts of the body. Sickle cell disease causes red blood cells to form into a crescent shape, like a sickle. The sickle-shaped red blood cells break apart easily, causing anaemia," Dr Wafula explains.

The damaged red blood cells also clump together and stick to the walls of blood vessels, blocking blood flow. This can cause severe pain and permanent damage to the brain, heart, lungs, kidneys, liver, bones and spleen. 

Knowing this is disturbing to parents raising children with the condition. But the worst part is when they are told what their children are suffering from. Some doctors are insensitive.

When Christine Omollo’s son, who is now aged 13, was diagnosed with the disease at four years old, the doctor told her: "He won’t live past the age of five." Speechless, she dragged herself to a shade at the hospital and wept for hours.

Second opinion

If it were not for a mother, a nurse, Christine says she wouldn’t have known how to grieve her son’s certain ‘death’.

"We sought a second opinion and pored the Internet for information about the condition. I read books — and still do — on the subject. When I look at my son who is preparing to sit his Kenya Certificate of Primary Education later this year, I thank God," says Christine.

Although her son is on drugs everyday and avoids strenuous activities his age mates enjoy, he leads a near normal life, is jovial and performs well in school.

SMILE OF HOPE: Selina Ogweno’s son lives with sickle cell disease.

Just like Christine, many Kenyans don’t know much about the disease. And while the rest of the world set aside June 19 as the Sickle Cell Awareness Day, here the day passed just like any other. The Nairobi-based Children Sickle Cell Foundation (CSCF) chairperson, Selina Ogweno, says there are many people who need to be reached and told about the disease as well as how to care for their loved ones with the condition. Only then can Kenya join the rest of the world in the fanfare of marking the day.

Peggy Odoyo, now 28, was diagnosed with the disease when she was aged three. She is optimistic and jovial.

When we meet, she says with a wide smile, "There is hope for ‘sicklers’."

Peggy who is also the secretary of the CSCF says she came to learn about her being a ‘sickler’ when she turned 16.

"I had come up with serious illness, and was admitted for about one month. After that, I went into denial, especially after my school mates nagged me about my eyes being yellow, and having a thin frame (these are some of the tell tale signs of the disease)."

Selina whose six-year-old son was diagnosed with the disease is hopeful after learning of the Ugandan man who was a ‘sickler’ (as people with the disease are commonly referred to) and died last year at 80.

"Sickle cell is not a death sentence, you just need to manage it well and you can live to see your grandchildren," Selina says.

Selina’s son, Aggrey, became ill at six months. Three months later, he was taken ill again with the same symptoms. When the doctor told her that her son had anaemia, all Selina thought of was death.

Luckily, the same day, she went to the CSCF where she was helped to embrace acceptance. Today, she is the chairperson of the organisation which has members from all over the country. 

A person with sickle cell disease may be healthy one minute and then writhe in pain the next.

"Severe pain is caused by dehydration, or an infection," says Dr Wafula.  "The pain is migratory, from hands to feet, thenmoving to the joints. It must be arrested with pain killers because if it goes to the chest, it can kill."



Painful attacks

Christine says: "The painful attacks happen regularly. But when you know how to handle them, you get to accept your situation. I massage my son’s joints and encourage him to do the same when in pain. This makes it bearable."

Dr Wafula says, when the attacks happen, patients should be rushed to hospital, and taken straight to the nurse, a measure not followed in Kenya where many patients wait at the casualty for long and eventually succumb to a painful death.  Health workers are also reluctant to use painkillers such as morphine because they fear the child might get addicted. Yet this is "the standard way of treatment."

Sickle cell may also manifest as acute chest syndrome, massive bleeding, stroke and priapism. Priapism is a condition where boys suffer a very painful erection that stays for long hours. Parents and patients ignore this thinking it is embarrassing until it is too late. It is treated through surgery, while antibiotics should manage infections.

If anaemic, the patients should be transfused with blood that is free of the disease.

In children, acute chest syndrome manifests like pneumonia. Children are susceptible to the pneumococcal bacteria. The government is currently vaccinating children below the age of one against this bacterium.

"The government should extend this service and vaccinate children with sickle cell disease even if they are above five years," says Dr Wafula.