It was a slow Saturday in the Children emergency unit of my hospital. The dark clouds meant a downpour was imminent, so the patient load was less as people prefer to stay in their homes except its life threatening. I decided to refresh my memory with my Oxford handbook of clinical medicine, but this was interrupted before I could finish one page. A 14-year-old boy was rushed in with excruciating pains in his chest, wrists and legs. It was so bad, that any slight touch or attempt to move him resulted in high pitched screams. Though this was my first time of encountering this patient, something within me already told me he was a sickle cell disease patient. My fears were confirmed when my colleague stepped in a few minutes later and greeted him cordially, inquiring about what brought him to the hospital this time. Apparently she attended to him in the clinic some days before, during his clinic visit. We carried out the necessary interventions and in a few days he was ready to go home, but he had already missed his exams. This is the bane of most sickle cell patients as they are always in and out of hospitals, and this negatively affects the quality of life they have, not to talk of the financial and emotional implications on their parents or caregivers.
Sickle cell disease is the most common genetic disease in the world. This means that it is not contagious and it is passed on from a person to their offspring by inheritance. For a person to have sickle cell disease, he has to have inherited the genes from both parents. Some people have the sickle cell trait, this means that they got one gene from a parent, these class of people mostly do not have symptoms of sickle cell disease. The problem of sickle cell disease is mainly due to the abnormal shape of the individuals’ red cells. They are shaped like a sickle and this makes it difficult for them to move easily within blood vessels, causing severe pain. There are conditions that can cause the red blood cells of sickle cell disease patients to assume this abnormal shape and these include dehydration, changes in body temperature and any situation resulting in decreased oxygen supply.
Sickle cell disease is a disorder of the blood and so it results in complications in different parts of the body. Some of these complications are very severe, while others are not so severe. These complications include anaemia, pain episodes or crisis and infections, other complications are leg ulcers, stroke, growth retardation, delayed sexual maturation. This disease could also progress to adversely affect vital body organs such as the heart, kidneys, liver, spleen, lungs, bones and eyes.
But one vital fact to note is that sickle cell disease is not a death sentence and all hope is not lost. Warriors as they like to be known have taken advantage of progress in modern medicine and go on to live fulfilling lives. I have come across people with the disease, whom I didn’t even know had it despite my medical acumen. Like all challenges in life, having a positive attitude is vital. Although the severity of the disease varies amongst individuals, one can hope to have a meaningful and enjoyable life if they manage it properly. Managing the disease involves several things, but first one has to know if he has it before he is able to manage it. So, screening for the disease is the first step. It involves a simple blood test and everyone should at least be aware of their genotype. Also regular clinic visits would help warriors adhere to prescribed therapy. Sickle cell disease is best managed by a specialist doctor called a haematologist, and so every warrior should ensure that they are regularly seen by haematologists. Other lifestyle modifications include staying hydrated, that means drinking a lot of water, have a goal to consume at least 3 litres of water every day. Also, eating healthy meals is vital, meals should include fruits and vegetables. Sleep and stress avoidance are two vital aspects of non-drug management of this disease. A warrior should have at least eight hours of sleep every day and avoid stressful situations as these tend to trigger episodes of crisis. These help to prevent a crisis.
The best therapy however is prevention. Since Sickle cell disease is inherited, prospective couples should be aware of their genotype. If both parents have the sickle cell trait, meaning that they carry one sickle cell gene each, there is a one in four chance that a child will be born with sickle cell disease per pregnancy. There are many other combinations that can be mapped out depending on the parents’ genotype, so premarital counselling and testing is very vital in the prevention of this disease. But the choice to carry on with the marriage still lies with the couple.
Sickle cell disease is a serious but manageable medical condition, though cure is not readily available. There is light at the end of the tunnel as recent medical advances such as stem cell transplant hold promises. We look forward to a time when sickle cell disease will be no more, but till then we soldier on.