Coping with sickle cell disease: Unwarranted agony of innocent ‘sinners’

THE pain was indescribable; the scenario was pathetic. I couldn’t find any rationalised way to justify why an innocent child should suffer the consequence of genetic “sins” committed, knowingly or unknowingly, by his parents. That was my first experience with a sickle cell victim. Although efforts have been geared towards curtailing the genetic traits of the factor in recent time, it is sad that relatively little has been done in terms of creating an intensive and extensive awareness.

Epidemiologically, it is unfortunate that every person that inherits the haemoglobin SS from carries parents (Hb S) inevitably suffers the devastating condition (SCD), characterised by excruciating pain scenarios. For a child to possess the Hb SS, both parents must have been carries of the Hb S. Thus the child’s red blood cells (RBC) are determined by the two haemoglobins types the parents possess.

The common types of the Sickle Cell Trait are AS and AC; the two are carriers, not disease conditions, though there are other types. For instance, findings have shown that in a situation whereby a child inherited the altered haemoglobins from both parents, the genotype of such a child could be homozygous SS, CC or EE. On the other hand, the child’s genotype could be heterozygous (AS, AC or AE) if he or she inherited the mutant haemoglobin from either of the parents.

Despite the fact that the sickle cell anaemia is neither contagious nor transmittable, the traumatic experience of sickle cell victims is nothing but needless pathological hereditary inflictions on innocent souls. The scenario is pain, pain, and pain in virtually all parts of the body.

Acute Chest Syndrome (ACS) is one of the commonest pains a sickle cell person undergoes, often. Also, due to chronic anaemia (deficiency of red blood cells), these people are susceptible to all manner of cardiovascular attacks, ranging from pulmonary arterial occlusion (obstruction of blood flow), hypertrophied myocardial fibres (excessive enlargement of the heart muscle), infarcts (localised necrosis – death of living cells) resulting from obstruction of the blood supply.

Relatedly, avascular necrosis (AVN) is a life-threatening disease, resulting from a temporary or permanent loss of oxygenated blood supply to the bone, particularly the humerus (arm bone) and the femur (thigh bone). Cerebrovascular incidents (CVAs), associated with deficiency of oxygenated blood to the brain and the adverse effects of vaso-occlusive pain (sickled blood that causes blood clot) are other plights of people with SCD.

Other complication diseases include, but not limited: splenomegaly (an abnormal enlargement of the spleen); retinopathy (a disease of the retina that can result in loss of vision); nephropathy (disease affecting the kidneys); Leg ulcers; Priapism (a persistent painful erection of the penis); Jaundice; Cardiac dysfunction; Pulmonary hypertension (lungs infection); cholecystitis (inflammation of the gall bladder); Osteomyelitis (an inflammation of bone and bone marrow, usually caused by bacterial infection); sequestration crisis ( the pooling of a large proportion of blood in the spleen or in the liver).

Sadly, it is a major cause of death in children with SCD aged 0-2 years, according to medical experts. Now imagine a child vulnerable to all the aforementioned medical complications just because he or she inherited two abnormal haemoglobins (Hb SS) from both parents during conception. Imagine Nigeria losing 100,000 children yearly to the SCD, representing eight per cent of child mortality, having recorded the highest cases of SCD in Africa (Federal Ministry of Health, WHO).

It is sad that most of these children do not usually live to celebrate their fifth birthday. It is equally lamentable that about 3.4 million Nigerians are victims of the sickle cell anaemia, while about 40 million people possess the Hb S gene. Apparently, this development poses a serious threat to the actualisation of the Millennium Development Goals (MDGs, 4, 5 and 6). In the bid to creating awareness, the UN General Assembly in 2008 at the 63rd session of the organisation, reached a resolution that member states and the UN organisations should embark on social awareness of SCD every June 19.

Although the development is commendable, the once-in-a-year campaign is relatively inadequate to achieve the desired result on such public health problem that has moral and religious undertone in the diverse society across the globe. In Nigeria, under the former President Goodluck Jonathan’s administration the Federal Ministry of Health in collaboration with the MDG office in 2011 and 2012 empowered six Federal Medical Centres in the six geopolitical zones with a view to running dedicated clinics and programmes for the management and control of SCD. Also, the Sickle Cell Centre at Idi-Araba, Lagos, is a laudable one but inadequate for the 3.4 million SCD victims and the 150, 000 children who are born with the disease yearly.

The situation demands that more of such centre be replicated across the country. Under the present administration, the wife of the President, Mrs. Aisha Buhari expressed her commitment in the fight against SCD at the 2015 World Sickle Cell Awareness Day Campaign in Abuja. Unfortunately, Mrs. Buhari lost her step daughter, Zulaihat Buhari to the disease in 2012. Similarly, in October 1, 2014, the former Speaker of the House of Representatives, Mr. Dimeji Bankole lost his seven-year old daughter, Yewande, to SCD in a London hospital. Also, April 20, 2014, was a sorrowful day for the family of the former Minister of the Federal Capital Territory (FCT), Mallam Nasir el-Rufai, whose cousin, Zainab el-Rufai Mohammed, succumbed to SCD after a prolonged battle with the disease.

It is undoubtedly that treatment and management of sickle cell disease requires great financial endeavours. If Nigeria is losing the children of those who apparently have the wherewithal, what happens to the fate of children of the poor, whose parents do not have the financial muscle to provide comprehensive clinical care for their children with the disease? Therefore, the need for legislative apparatus germane to the improvement and upkeep of people living with SCD cannot be overemphasised. Every means of social communication should be deployed with a view to providing clinical facilities for the victims, and an intensive and extensive health education for Hb S carries in the society.

Also, the role of genetic counselling cannot be played down in this regard, whereby accurate and balanced information on genetic compatibility is provided for prospective couples. The vigour and intensity with which HIV/AIDS, malaria and cancer are being publicised should also be given to drive home the message of sickle cell disease awareness. The collective will in which Nigerians fought and won the Ebola Virus Disease (EVD) should also be demonstrated in the fight of SCD. The inputs of the media, religious and social organisations, NGOs and relevant government agencies at all levels are extremely needed. Tunde Kelani’s new film, “Dazzling Mirage,” is a welcome development.

The movie which projects a message of hope for people living with SCD should be immortalised. Scientists are yet to discover an easy method for the cure of sickle cell disease, except the bone marrow transplant which is extremely risky. It is high time aggressive efforts were made in sensitising the populace on the management, treatment and prevention of SCD. Also, clinical facilities and accessible prophylactic vaccines should be provided with a view to putting an end to the suffering of these innocent “sinners.” The various medical, social and emotional challenges these people face are extremely burdensome for them and their relatives to bear alone. The society, as a matter of obligation, ought to rise in unison to their aid. • Bakare is a public affairs analyst in Lagos