World Sickle Cell Day: Towards better life for patients

The World Health Organisation (since 2006) and the United Nations (since 2008) set aside June 19, of every year as World Sickle Cell Awareness Day. This is with the goal of increasing the public awareness and understanding of the Sickle Cell Disorder (SCD) and for all stakeholders to recognise SCD as a global health concern thus giving it priority attention in the healthcare ecosystem.

Sickle Cell Disorder (SCD) is a chronic lifelong debilitating inherited blood disorder affecting millions of people worldwide. It affects hemoglobin production, causing red blood cells to be sickle-shaped, rigid and prone to clotting. The resultant effect of this abnormality is the blockage of the small blood vessels thus causing severe pains and causing damaging complications.

This pain often referred to as “sickle cell pain crisis” is the hallmark of the life of a typical sickle cell patient. If not properly handled, this crisis can result to other severe complications, organ damage and sometimes death. To say that sickle cell disorder has brought sorrow, tears and pains to many homes will be an understatement.

It is estimated that 300,000 children are born annually with the SCD with half of this number contributed by Nigeria. Of these numbers half may not survive to five years, due to infections, malaria, severe blood loss and other childhood diseases which vaccination and diligent management could have taken care of.

For the SCD patient that reached adulthood, the journey is always marred with excruciating pains, uncountable hospital visits, rejection and stigmatisation by the larger society. More often than not all these culminate into serious psychological issues to both SCD patients and their immediate family.

The foregoing underline the importance of the setting aside of a particular day in the year to mark this onerous health condition. According to the Africa Health Organisation (AHO), its objectives for this year’s world sickle cell day are:

·       Increasing awareness of community of the global burden of SCD

·       Urging health institutions to pay attention to sickle cell anaemia through developing health programs at the institutional level and operating specialized centres to facilities access to treatment.

·       Raising awareness of the importance of pre-marital screening to reduce transmission of SCD among generation

·       Promoting and Supporting research to improve quality of life of those affected.

The Africa Health Organisation could not have come with a better objective for this year’s World Sickle Cell Day as the enumeration below of each of the set objectives will reveal.

Even though there has been improvement in the awareness about SCD from what it used to be some years back, the awareness campaign is mostly championed by sickle cell patients themselves. There is still a lot that needs to be done at the institutional level. The society on the general level still lacks the basic awareness about the intricate involved with living with this disorder. To make it more pathetic there are some health workers, who still lack basic knowledge about sickle cell disorder management protocol.

To improve the awareness of this disorder, all and Sundry must key into the awareness campaign. The National Orientation Agency of the ministry of information in conjunction with the ministry of health can have a special unit to educate the general populace on this genetic disorder, our media houses can have special jingles and publications to raise awareness about it especially on how to avoid giving birth to children with the disease.

The public needs to know that SCD is not contagious and the stigmatisation of people living with this disorder must stop. Employers of labour should stop their discrimination against people living with this disorder and give them equal opportunity when seeking for employment.

The second objectives which relates to the health institutions developing special health programmes on the disorder is very germane to me who is a sickle cell patient, I believe the first thing to be done is the training and retraining of our health professional in our various hospital on the general and specific management protocol for sickle cell disorder. Thankfully, most teaching (and some) general hospitals now have a haematology department, which is a specialist department for the management of sickle cell disorder and other blood related ailments. It will be good to replicate this unit in all the general and teaching hospitals in the country and for them to be manned by personnel who are well trained, motivated, compassionate and knowledgeable in the treatment and management of SCD. These departments should have “day care units” where SCD patients in emergency situations can be quickly attended to, instead of taking them to the general Accident and Emergency unit of the hospitals.

Thirdly, is the raising of awareness on the importance of pre-marital screening to reduce the transmission of SCD. In my almost two decades of active advocacy on sickle cell disorder, what I have come to realise is the irreparable harmful effect of the lack of professional due diligence of our laboratories. In more than 50% of couples that I know with children with SCD, they went into their marriage because of the false laboratory result, which pronounced one or both of the couples as genotype ‘AA’ when in fact they are both ‘AS’. The resultant effect of this is giving birth to children with sickle cell disorder. The Medical Laboratory Science Council of Nigeria needs to step up its game in the setting of standards and monitoring of the laboratories of their members. There should be well spelt out penalties for laboratories dishing out false results, which has made many couples go through avoidable crises in their marriage.

The fourth objective of this year sickle cell Day as set out by the AHO is the promotion and supporting research to improve the quality of life of people living with SCD. The unfortunate reality about research on sickle cell disorder in Africa is that though the continent has the highest burden of sickle cell disorder, research on this disorder is mostly championed by the western countries. It is high time we refocus our search for a solution to SCD on African Herbal and traditional solutions which may in the long run be cheaper and more accessible than the orthodox solution.

Even where breakthrough has been made in research on SCD, the outcome is hardly shared with the general public. I still recall a publication in one of our national newspapers in December 2022, where it was reported that the Minister of Science and Technology says his ministry has developed anti-sickle cell vaccine. All my efforts as a sickle cell patient to confirm the veracity of this claim and get access to this vaccine prove abortive. One then wonders what is the essence of a solution that cannot be accessed. Our research institutions and the government need to show more dedication and seriousness to finding a solution to Sickle Cell Disorder.

One area of research that could have a great impact on improving the quality of life of the sickle cell patients and which is often overlooked by medical practitioners and caregivers is the psychosocial issues related to SCD.

Our clinical psychologists, sociologists, public health practitioners and other specialists in the area of mental health have a lot to offer to the sickle cell community on providing solution to conditions such as depressive feelings, abnormal habitus, suicidal ideation, poor self-worth, stigmatisation, substance abuse, relationship issues and other emotional burdens associated with SCD.

On a conclusive note, my appeal is that as we mark this year’s World Sickle Cell Awareness Day, we should not limit ourselves to the ‘Shows’ and ‘talks’ associated with the occasion. Instead, we must start to walk our talk. Sickle Cell Disorder is real with lifelong devastating effects on the sufferer and his/her family. The solution to this scourge is multifaced. Our government at all levels, corporate organisations and the whole society must resolve to be a solution to ensuring better qualitative life for all SCD patients and discourage relationships that could result into giving birth to SCD children. Truly, prevention is better than cure to Sickle Cell Disorder.

Shehu O. Mohammed, a sickle cell survivour, writes via [email protected]