Growing Up a Sickler

By Carol Tuimur

Silas Kiprono, 28 was diagnosed with sickle-cell anemia at the age of three. “Kiprono, my first born, was always a ‘normal’ baby, until his persistent non-stop cries begun” says Jannie, Kiprono’s mother. The doctor decided to run some tests on him during the visit and that was when he was diagnosed with this incurable condition. Being a fairly foreign concept, this was followed by frequent visits to the doctor. This condition was not only foreign to Kiprono’s parents, but also to the medical field in the country at the time.

What is Sickle-cell anemia?

According to Kiprono’s physician, Dr. Samuel Kamande, a general practitioner, sickle-cell anemia, is one of the most common severe monogenic disorders in the world. It is an inherited form of anemia where there are not enough healthy blood cells to carry adequate oxygen, throughout the body. Research has shown that, most people with sickle-cell disease are found in Africa where more severe cases are also found.

Dr. Kamande explains that a normal red blood cell is supposed to be round in shape and flexible for it to move easily throughout the blood vessel. However, a sickler’s (a person with sickle-cell anemia) blood cell is rigid, sticky and shaped like sickles (hence the name) or a crescent moon.

The irregularity in their shape forces them to get stuck in small blood vessels, which could then slow or block blood flow and oxygen to various parts of the body.

So far, there has been no known cure for this disorder. However, Kiprono enlightens me that there are clinical trials currently ongoing in the united states such as; the GBT 440 and the Gene therapy. He says that there has only been one case which could hardly be perceived as a success story. Despite their being no cure, medications are available to relieve the pain or help to prevent problems associated with the disease.

Dr. Kamande explains the general complications as follows; constant migrating chronic pains that could lead to a stroke due to lack of oxygen in the blood, acute chest syndrome which is difficulty in breathing and hence fatal, avascular necrosis which is the wearing out of the bones because the poor blood supply in the joints leads to friction and grinding out – could lead to hip replacement, difficulty in child birth due to it rigorousness and caesarian birth method is advised and last but not least, are the leg ulcers – this is an extremity which is brought about by poor blood circulation.

He says that they can be managed by; keeping hydrated, keeping warm during the cold seasons, staying away from too much heat, good balanced diets, following medications and last but not least, listening to your body and knowing your limits because sicklers are also human and not all pain is sickle-cell related.

Growing up with sickle-cell disease

“Growing up a sickler is tough, because you are constantly unwell but you cannot really comprehend what is happening to your body” says Kiprono. With Jannie’s desire for her first born son to enjoy his childhood, he says that during his later years in primary school, is when he begun to understand that he was a little different from his friends. This is because he was told not to play too much, to frequently drink a lot of water, to always keep warm, not to expose himself to too much heat and other precautions which came as a result of the disease. “I felt like my childhood was robbed away from me!” says Kiprono.

It took a genetics class in form 2 where he really took an interest outside from what his doctors were feeding him. One day, he says, he followed his biology teacher out of class, explained to his teacher that he has sickle-cell disease and that would like to know more about it. Though his teacher only had the curriculum perspective of the condition, this was the first time he really got a good grasp of what the disease entailed.

At this point, he learnt that both his parents are carriers (AS – their blood cells carry both the normal and the sickle-cell gene). This put his curiosity to rest as he now understood why he and his younger brother, Micah are of the same blood, yet different.

Kiprono says that, for the longest time, he was discouraged from taking risks or basically living his life. After much research on the internet, he learnt that sicklers could only live till 23 years of age. ‘Every attack felt like I was seconds away from taking my last breath” he says. His perspective on life however took a turn in the year 2011. He met a group of fellow sicklers. This group consists of over 100 people. When he inquired of their ages, he felt like he had just been born all over again. This is because he says the oldest person in the group was a 58-year-old man, with a decent job and a nuclear family unit. “I knew at that moment that I had a reason to live” says Kiprono.

Despite this, there are a lot of challenges that sicklers could endure. Growing up in Nairobi, which is considered an urban area, made Kiprono’s accessibility to facilities and the management of the disease possible due to the proximity advantage. However, he says, sicklers in the rural areas are either feared or cursed hence considered outcasts or have little to no access to facilities that can help their guardians in managing the disease.

Sicklers have also been in positions where they could visit a doctor and the practitioner has no idea what the disease is. Kiprono tells of a friend’s experience where she visited a doctor and she kept complaining of migrating pains yet she could be able to articulate herself. She requested for strong painkillers. The nurses and the doctor assumed that she was faking the pain, and was assumed to be an addict.

“Access to insurance is our biggest challenge as persons with chronic disease. They either give you half the cover or turn you down” says Kiprono. This is because most insurance companies perceive such persons as liabilities, he explains.

Giving an example of his own cover, he says that he has a cover with Jubilee insurance. This constitutes; a 2 million in patient cover and an 80K out-patient cover. He says that he however, receives the full out-patient and only 1 million, which is half of his in-patient cover. He emphasizes on the inconvenience of these policies as they constantly fall ill and their on-going medications are very expensive (Kshs. 40 per tablet) depending on the dose. This then leads to the cover running out and one is forced to either renew or pay in cash, not to mention the myriad impromptu services such as consultation, lab works, imaging services and the pharmacy. Larry Okinyo, a medical underwriter with APA insurance, partly concurs with Kiprono. He however says that the cover given may not necessarily be half of the package requested, but could even be lower depending on the gravity of the pre-existing condition. “When a person with a chronic disease applies for an insurance cover, they are obligated to take a medical report which is then reviewed by the insurance’s medical team. Afterwards, the underwriter in-charge is the one who decides on the specific in-patient amount that the person is qualified for, guided by the advice from their medical team” he says.