Three Kenyans suffering from sickle cell anaemia share their experiences

In April last year, Denis Awich’s cousin, Rosemary, died of sickle cell anaemia. It was one of the lowest moments in his life because he, too, had been diagnosed with the condition.

They had battled the disease together for years, and when they were not giving encouraging talks to fellow patients, they found hope in each other.

Now Rosemary was gone, just a few months to her 26th birthday, and Denis wondered whether he would soon follow her to the grave.

Heartbroken and, he admits, a bit shaken, he decided to celebrate Rosemary’s life by starting the Denis Awich Sickle Cell Foundation (Dascaf), through which he spreads awareness about the disease.

“Before the death of Rosemary,” he says, “all I did was explain to my friends, classmates and relatives about the condition. But after she succumbed, I took it upon myself to let the world know about it.”

TAUNTED BY SCHOOLMATES

All his life, Denis has been in and out of hospital so many times that he views the brightly lit wards as his second home.

In the beginning, whenever he was in pain, he would go to various hospitals for consultation, and as sure as the sun rises from the east, the doctors would misdiagnose him.

Denis has thus spent a great deal of his life not only fighting sickle cell anaemia, but also popping drugs for ailments he did not have. Malaria, for instance, seemed the doctors’ easiest excuse to send him to the pharmacy.

His parents, worried that their son was always going down with malaria, decided to seek help further up the medical chain, and that was when Denis was diagnosed with sickle cell.

The good news was that they had finally figured out what was ailing their son, and the bad was that the son would be on medication for the rest of his life.

Sadly for him, pain was not his only headache. He was constantly taunted by his schoolmates and some relatives. The stigma was often worse than the pain, he says, and at one point he was tempted to drop out school.

“While in Standard Seven, a classmate succumbed to the disease. Everyone in school knew that she and I were suffering from the same disease and so when she died, my classmates repeatedly reminded me that I would be next. I could not handle it anymore and had to transfer to another school,” he recalls. But he outlived the taunts to celebrate his 26th birthday earlier this year.

MEN RUN AWAY

Catherine Odongo, another victim of the disease, is looking forward to her 29th birthday next week (October 12). For a person who has lived with the condition all her life, that would be a major milestone because most victims die young.

But, like Denis, Catherine has paid a high price for her condition, not least of all losing a job. “My condition was something my boss could not handle,” she begins.

“During the last Sunday of the month of July, after suffering a painful bout and asking for days off, I received a call from my boss telling me not to report to work anymore.”

Catherine, who was diagnosed with the condition when she was eight years old, reckons that her boss was not the only one who could not stand her being sick as she tells of how difficult it is for women living with sickle cell to interact socially.

“I have had men run away from me the moment I disclose my medical condition,” she says. “I remember an instance where I abruptly started feeling pain and walked into a restaurant to request for some water.

“In my pain, I couldn’t even afford an upright posture and people thought I was drunk. In fact, I heard one heckle ‘Leave that girl alone, she is just drunk’,” she adds.

VARIED DIAGNOSIS

Unlike Denis, she chose not to disclose her condition to anyone in school. Not even her relatives knew about it, save for her grandmother who brought her up and enlightened her about what was ailing her.

“I don’t think I could stand the stigma. And, to avoid it, I chose to hide my condition from everyone, apart from my grandma and brother. For the same reason, I never set foot in a boarding school.”

According to Prof Walter Mwanda, a haematologist at Kenyatta National Hospital and the chairman of the National Blood Safety Committee, diagnosis for sickle cell anaemia varies from one individual to another due to differences in the blood composition of individuals.

“Blood has a lot of elements, one of them being globin which, in simple terms, we refer to as chains,” he explains. “During conception, both parents contribute these chains that form the composition of the haemoglobin (an iron-containing protein in blood that carries oxygen from the lungs to body tissues, also referred to as Hb), an alpha chain and a beta chain.”

However, he says, there is formation of a third chain called the foetal chain, found in infants. A beta chain, responsible for the sickle cell trait at times, is usually suppressed by foetal chains which some children retain years after being born, making it difficult to detect the condition. However, as soon as the foetal chains are lost, the trait begins to manifest itself.

Prof Mwanda adds that when red blood cells are malformed, the person could be either a carrier of the disease or a victim, depending on the degree of the malformation.

PAINFUL EPISODES

“In extreme cases — where the patient is not just a carrier, but a victim — the red blood cells turn crescent shaped, making them stiff and fragile, hence clogging the blood vessels, especially around the joints, and depriving the body of oxygen. This causes pain,” he explains. “A normal red blood cell has a life span of about 120 days while a sickle cell has between 10-20 days,” he continues.

The low lifespan of the cells arises because of their sickle shape. Prof Mwanda says that due to the distortion of the cells, jaundice is likely to occur among patients.

Still, a lot of patients in this century live well beyond the age limit of 18 and, as Prof Mwanda says, not all sicklers suffer from painful crises, arguing that an individual is the sole determinant of how his or her body will react to the condition.

“As an individual, you cannot prevent the blockage in the veins, but you can reduce the number of obstructions by avoiding the causative agents.

“When sickle cells have adequate supply of oxygen, they behave like the other normal cells, hence there is no pain. However, a slight depletion of oxygen leads to formation of the crescent shape which blocks the veins and results in painful episodes.”

The disease is a genetically inherited condition which is transferred to children if both parents are carriers of the sickle cell trait.

SWELLING AT TIPS OF FINGERS

“We are not all the same. Misconceptions, ignorance and misinformation are what ail us. Sicklers are normal people who can lead normal lives and perform normal activities.

“It is stupid to segregate them due to their condition. Early symptoms like jaundice (yellow eyes), pain and swelling at the tips of the fingers and toes (hand and foot syndrome) are related to the condition.”

Despite some advances in recent years, the sickle cell anaemia still takes a deadly toll on patients, according to Dr Donald Kohn, a researcher at the University of California Los Angeles (UCLA). He says that, despite all the improvements with medical care, the average patient only lives into their 40s.

Dr Kohn, however, hopes to change that through a clinical trial where the goal is not to just treat sickle cell disease, but to cure it.

The approach is to basically do a bone marrow transplant using the patient’s own stem cells and introduce a gene that will prevent their red blood cells from sickling and clumping together.

After the stem cells are removed, it takes three days to modify them in the laboratory. During that period, the patient will undergo chemotherapy to kill the remaining affected bone marrow cells.

POOR BLOOD SUPPLY

The hope is that when the modified stem cells are transplanted back into the patient, they will multiply and quite quickly create a new, sickle cell-free blood supply.

Using the patient’s own bone marrow stem cells means they no longer have to find a donor who is a perfect match, and that they do not have to take powerful anti-rejection medications that most transplant patients need to stop their body attacking the donor cells.

Dr Grace Kaguthi, a Centres for Disease control (CDC) research officer and co-investigator in the trial study, says patients with sickle cell suffer pain due to poor blood supply to different organs in their body, “which damages organs such as the brain, limbs and spleen”.

“With the damage of the organs, it reduces their life span as well as their quality of life.

“But with better health care, the patient’s life span can be increased to 45 years or more,” explains Dr Kaguthi, adding that the goals of treating the disease are to relieve pain and to prevent infections.

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THE BATTLE HAS LEFT ME BRUISED, BUT I SOLDIER ON

My name is George Obiero, and, at 53, I may be one of the oldest Africans living with sicke cell disease. As a teacher, I must admit that living with the condition is an everyday fight, but so far I seem to be winning the battle.

That is not to say that I am not bruised, though, because my body is living testimony to how fierce this battle had been. I do my best to fight the pain, but at times the bouts are so strong that they knock me out.

Now, at this age, I do not have the energy of youth, and so I call upon those close to me to help me when the disease attacks. It is a burden they have learnt to live with, and I am grateful to have a family that supports me in this tumultuous journey.

I inherited the disease from my father, who was a carrier. He was unaware that, by marrying my mother — who was not a carrier — and agreeing to have children without medical advice, the possibility of having sickler children was as high as 50 per cent, and I am living proof of that. My two elder siblings, however, came out unscathed.

I learnt I was unwell at the age of 12, when my parents took me to Kenyatta National Hospital to check what was wrong with me as I was always down with some ailment or the other.

In most cases, whenever I went to hospital I had to do an haemoglobin blood test to determine how healthy my blood was.

Doctors opine that when the blood level is above 4, the patient is safe, unless he or she exhibits signs of malaria that need treatment. However, when it’s below 4, then blood transfusion is recommended.

In most cases, my blood level was always 2.9, a sign that all was not well. And most times when I visited the doctors, the results showed I was suffering from anaemia and received four to six units of blood every month.

I had to discontinue my education at Ng’iya Mixed Primary School in Siaya to join my father in Nairobi to be near Kenyatta National Hospital for the frequent check-ups.

In Nairobi, I joined Heshima Road Primary School. I passed my primary exams and was admitted to Starehe Boys’ Centre. I was denied admission, however, because I was 15 when I sat my primary exams in 1978, yet Starehe was admitting boys aged no more than 13. As a result, I joined St Mary’s Yala instead.

In 1980, I had a serious attack that left me in a coma for three months. My father died while I was still in hospital, and when I came to, I had no one to continue paying my school fees at St Mary’s.

I sank into depression, but a local church came to my rescue and sponsored me through secondary school. Just as I was about to write my final exams in 1986, however, I had a serious attack that forced me to be hospitalised, and as a result I wrote the papers from a hospital bed.

I did not score enough points for university admission, and instead joined Kilimambogo Teachers’ Training College for a Diploma Course.

After graduation I was posted to Rambo Primary School in Siaya County, where I have been the head teacher for the past 10 years. In some countries, sicklers are discouraged to marry because they do not want the disease to spread to their children, but I had girlfriends who were very close to my heart, and I knew I would get married to either of them. But all of them refused to marry me when I asked them to.

In 1994, I met a woman named Joyce, and with whom I eloped and later got married despite my situation. She loved me unconditionally and never listened to what people said. We are now blessed with two daughters who are, unfortunately, carriers of the disease, and whose children will most probably have sickle cell.