THE IMPACT OF SICKLE CELL DISEASE ON THE NIGERIAN CHILD

Sickle cell disease is a multisystemic genetic disorder characterized by chronic anaemia, episodes of acute and possible chronic pain, progressive organ failure and a possibility of reduced life expectancy. It is originally a blood disorder that is inherited from one's parents but it can end up affecting all body systems. Although it has been declared a global disease, the highest burden of the disease is amongst the black race and Nigeria has the highest incidence of sickle cell disease in the world. The disease has a way of affecting every area of the lives of those that inherited it which has inspired this write up. I will be highlighting the health, psychosocial and financial implications of sickle cell disease on a Nigerian child with a focus on the interplay of these implications and possible solutions.
The health impact of sickle cell disease are the complications which remain the scariest implication of the disease. The red blood cells of people with sickle cell disease are sickle shaped instead of being biconcave. This abnormal shape makes the blood sticky and rigid , as a result it obstructs blood vessels and impairs blood flow to organs causing different complications in the process, the abnormal shape also causes blood to break down more easily and frequently. Pain crisis is the most distinctive feature of sickle cell disease . It is said to be the most common symptom in people living with the disease who are over 2years of age. This pain can be mild, moderate or severe, it can last for hours, days, weeks or even months. Another complication is chronic anaemia, the sickle shaped red blood cells breaks down easily and puts the body in a chronic anaemic state. Therefore, many people living with the disease usually need blood transfusion regularly depending on their disease severity. They are also predisposed to infections, impaired blood supply to the spleen caused by the sickle shaped red blood cells causes the spleen which is important for immunity in the body to shrink and stop functioning, thus predisposing people with sickle cell disease to numerous infections. Other complications include renal failure, retinopathy which can cause blindness, acute chest syndrome and pulmonary hypertension which are pulmonary complications, neurological complications like stroke, one of the commonest causes of stroke in children remains sickle cell disease. Not to forget complications like sensorineural deafness, leg ulcers, avascular necrosis, stunted growth, developmental delays, physical disabilities, enuresis, gallstones, heart diseases to mention a few, progressive organ damage due to inadequate supply of blood and oxygen to body organs occurs in sickle cell disease. Multiple organ failure and many of the other complications mentioned above can lead to early mortality in people living with the disease. It is important to note that manifestations of sickle cell disease and complications experienced can differ from person to person depending on the severity which is determined by various factors.
The psychosocial implication entails how people living with sickle cell disease are affected psychologically and socially by the disease. It is one aspect that needs more attention but is usually overlooked. First, I would like to discuss the stigma associated with the disease in Nigeria, many Nigerians wrongly believe everyone living with the disease will die before 18years of age and they are usually vocal about it even to the affected. This stigma makes people living with the disease hide their status from the world , it makes many of them feel abnormal and puts them in a bad psychological state. Apart from stigma the complications of the disease take its toll on the mental health of people living with the disease. Complications like developmental delays which makes people living with the disease feel abnormal when their body doesn't grow like their contemporaries, a 19yrs old female without breasts is most likely to develop psychological problems about it. Falling sick often is often associated with increased unemployment rate among people living with sickle cell disease , frequent absenteeism due to bad health usually costs many of them their jobs. Many people living with sickle cell disease are also single because people are scared that people living with the disease will die early which is not always the case and do not want to date or marry them, some are divorced due to marital challenges related to their health conditions,all these result in psychosocial problems like depression, anxiety, social withdrawal and loneliness. Psychological complications like depression and health complications like chronic pain syndrome can also result in social withdrawal and loneliness.
The financial implications of sickle cell disease especially in low economic regions like Nigeria also has an important role to play. Many countries in Africa have no health insurance schemes or free health for people living with the disease. These people fall sick very often and have to visit the hospital regularly , with the high rate of poverty in Africa , it will be difficult for people living with the disease to cope with the financial implications. Sickle cell disease is an expensive disease to manage, due to this a lot of people living with the disease die because of poverty and not necessarily because of the virulence of the disease. Many parents manage their children or family members with the disease at home because of the cost of taking them to the hospital. Several studies have shown life expectancy for people living with sickle cell disease in Africa is much lesser in comparison to developed countries where we have advanced medical care and health insurance schemes.
It wouldn't be wholesome to emphasize on the implications of sickle cell disease on a Nigerian child and not discuss possible solutions to them. One of the solutions that can be helpful will be increasing sickle cell awareness, this is to educate about genotypes and genotype compatibility because choosing a genetically compatible partner will prevent having children with sickle cell disease. Increased education about the disease, complications and management , correction of myths and stigmatisation associated with the disease will also go a long way. Introduction of newborn screening of sickle cell disease is one of the best ways to promote effective management and prevention of sickle cell disease, which is almost non-existent in Nigeria, Nigerian government needs to initiate this as soon as possible to help with the management and reduction of incidence of sickle cell disease in this country. Provision of standard health care and granting funds for sickle cell disease related researches will also help in managing and prolonging the lives of people living with the disease. New drugs like adakveo and oxbryta have recently been approved for the management of sickle cell disease abroad, these drugs are expensive and not available in Nigeria, Nigerian government should help make these drugs available and affordable to those that need them. Provision of regular psychological evaluation and management of the affected can be helpful when it comes to the psychological implications. Good family and social support will help with social withdrawal and loneliness. The government should also provide a free health care and health insurance scheme for Nigerians living with the disease to help with the financial implications.
In conclusion, we can see the grievous impact that sickle cell disease has on the health, psychosocial and financial life of people living with it. Hopefully the government can take to the proposed solutions and make life better for that Nigerian child living with sickle cell disease .
Dr. Adesoye Oluwatosin
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