The Challenges of Sickle Cell Disease

While thousands of privileged Nigerians travel abroad for better education, one reason many who go abroad do not return is health. As the world commemorates the Sickle Cell Awareness Day today, I wish to draw attention to those living with the disease; a disease which has been pushed to the back burner since the arrival of other diseases considered more life threatening. Unfortunately, Sickle Cell Disease continues to ravage Nigerians but the focus on it has dwindled.

While speaking to a friend, Nancy Miller, in the United States of America about the challenge faced by those with the disease, she didn’t seem to have a clue that it was such a life-threatening disease because the care and management of SCD in the USA and the percentage of those affected have kept it out of the list of life-threatening diseases. For those living with SCD in Nigeria, the discrimination continues especially with regard to employment and marriage. The challenges keep mounting due to lack of awareness and information.  

The red blood cells are normally soft and round. This helps them move easily through the blood vessels. People with sickle cell anemia have an abnormal type of hemoglobin, main building block of the red blood cells. It causes the red blood cells to become hard and sickle-shaped. Because of their odd shape, some of the cells get stuck in the small blood vessels where they break apart. The broken parts can cause blockages in the vessels. This can happen in a blood vessel that leads to a major organ. The decreased blood flow can cause severe pain and organ damage. Since the body recognises that sickled cells are abnormal, it destroys them faster than they can be replaced. This causes anemia especially under a number of conditions which include fever and cold. People with sickle-cell trait have one abnormal hemoglobin gene and typically lead normal lives — many do not even know they are carriers. The trait is found in less than 1 percent of white Americans. Those with two mutated genes are said to have the sickle cell disease which can cause a lifetime of health problems like severe pain, anemia, stroke and damage to tissue and organs.

According to Mr. Olanrewaju Adigun, a 33 year old IT Professional, “It’s been an eventful experience, it has affected the way I handle a lot of things. I have my personal dos and don’ts and I abide with them; part of which is drinking a lot of water. I always have clean drinking water around me. To the glory of God, I rarely fall sick nowadays.” But while most men continue to own up to having the disease, a number of women (especially unmarried) prefer not to be named. Ms. Beauty Sunmola (not real name) a 35 year old student who is also involved with buying and selling in the UK had this to say, “I used to feel dejected and out of place especially considering how the treatment goes in Nigeria. The doctors in Nigeria need proper treatment orientation on the management of sickle cell disease. I almost lost my life till I got here”.

More and more people continue to seek for better health abroad while our health sector continues to go into disrepair. Ms. Sunmola added further that, “I came to the United Kingdom for studies and I am receiving treatment in the process. Treatment here is more detailed than in Nigeria. I remember then, even before any test was carried out, doctors concluded that it’s malaria! They treat malaria and prescribe pain killers (paracetamol etc) that may not suit one’s kidney or any organ of your body.” Mr Adigun supported this, “The fact remains that the developed countries have better social infrastructures, facilities and personnel and better support structures for people generally to help manage different health issues. Even people living with HIV-AIDS have a better chance at survival in those countries. I am looking forward to the day African countries will have adequate medical and social structures because we have better weather over here. Too much of cold isn't the best for people living with SCD”.

But it turns out that many Nigerians find cold bearable in the light of the benefits of survival. However, one must commend parents, siblings, spouses etc that continue to provide support to “sickle cell warriors” as an online activist chose to call those living with SCD. Commendation should also go to NGOs – The Sickle Cell Foundation, Touch A Cell, TAHF (Temitayo Awosika Help Foundation), Sickle Cell Warriors Inc and The Genes – who keep toiling to bridge the gap in our health sector.

One great quality of those living with SCD is that they are brilliant. Though this may be due to their medical condition, another believable reason is not farfetched. Those with SCD are usually exempted from play or work that may be exerting, though most people living with SCD try to avoid discrimination by going the extra mile where given the opportunity. Ms. Sunmola had this to say, “I’m not sure all SC carriers are brilliant but most are. If I can pass through degree courses of about 10 modules in a semester without a carry over, then I can attest to the brilliance.” For Mr. Adigun, “A book is a good companion on a sick bed”.

Coping in relationship is a major challenge for those living with SCD. As Adigun, who is looking forward to settling into marriage soon noted, “I guess it’s all about enlightenment. I've had previous friendships that didn't materialise into relationships because they couldn't cope with the fact that I'm living with SCD. But my fiancée and in-laws are just the best. They are very enlightened and they know that all is well in as much as I live right. And really, it’s all about the grace of God.” Though the peculiarity of the disease means carrier often search out mates with the AA blood type, but the challenge even here as Ms. Sunmola mentioned is that, “It takes the grace of God even for those with the AA blood type to stay and go ahead with you through thick and thin”.

While many marry based on ignorance, many more with the sickle cell trait continue to ignore the consequence of marrying someone with SCD. While genuine love is said to conquer all, the lack of support (especially where both partners have frequent crisis) and the grief of burying children based on the sickle cells has become a reason why people can no longer ignore the consequence. But haven’t our transport network and religious crisis created more deaths than SCD? Those who ignore the consequence must be prepared to live with it pending when medical science breaks the code to widen the marriage choice pool by ensuring that those with the trait (AS, AC) can always donate A for every try for a baby.

Another challenge is the issue of sexuality. I know women with SCD who have children, unfortunately research shows that men with SCD may have low fertility and most sperm banks still do not receive sperm from those with SCD but we continue to see men with SCD who have biological children. Exerting energy beyond what a carrier’s system can bear may lead to cardiac arrest or priapism which must be properly managed or else it could affect potency. Unfortunately, priapism does not only occur during intercourse, it may also occur in association with spontaneous nocturnal erections.

Though western countries celebrate the success of medications like Hydroxyurea and blood transfusion which prevents crisis and bone marrow transplant which cures SCD, these interventions is still far from reach in Nigeria.

People should learn to accept SC carriers as normal people; live, relate and associate with them as normal people and support them to get to the solution. SC carriers don’t need pity; they need love and care like every other human. While for people like Ms. Sunmola prayer is a key, those living with SCD, need to do proper medical check regularly. After doctors ascertain what is right, the carrier should stick to the routine medication as prescribed; take the painkiller when need be and live a healthy life by eating right and doing light exercise to keep fit. For those who believe in the power of a Supernatural Being, remember to confess to God what you want to see. Beneficial foods remain yam, beans and greens. But even these depend on individual carriers and other complicating ailments. But carriers are usually advised to stay away from sugar, white flour and cassava containing foods.

For all those who relate with those living with SCD, Mr Adigun has a word, “Empathy is the word. Don't treat them like something is wrong with them. Help their self esteem, especially the young ones, by speaking positively about their abilities. It helps to know that people you love believe in you. Also help them in being disciplined with regards to taking their daily supplements, drinking a lot of water, protecting themselves from extreme temperatures and maintaining a clean and mosquito free environment”.

On a day like this, we call on medical doctors not to support the choice of aborting embryos due to the presence of SC. Parents should also not be driven to support euthanasia as an option; every child is a gift. Those in the corridors of power should help pass a bill to accommodate people living with SCD as far as school and work is concerned. They should create opportunities for flexible work schedule for people living with SCD. Employers should understand that though those living with SCD may require flexible leave to make up for sick days, they are dedicated to their duties if given the chance and usually perform better than others. The schools should allow flexible workload for undergraduates; they could take fewer courses in a semester and take longer time, maybe 6 years instead of 4, to obtain their degrees. These would forestall resignation or school dropout due to stressful nature of their employment or academics. The government should also commit to exposing medical doctors to how SCD is treated and managed abroad.

To all the “sickle cell warriors”, I wish you strength and courage to become survivors.

A version of the story first appeared in Punch Newspaper. Mrs. Omoteso Famuyiwa can be reached via email