Dealing With Sickle Cell Disease In Ghana

Africa is confronted with a growing burden of Non Communicable Diseases (NCDs) coupled with a resilience of Communicable ones- a situation that has become known as the duality of disease burden.

In Ghana, Non-Communicable Diseases (NCDs) contribute significantly to illness, disability and deaths. These diseases are generally attributed to lifestyle and genetics. Among these genetically induced Non Communicable Diseases or hemoglobinopathies is Sickle Cell Disease (SCD). Sickle Cell Disease though an old disease, has received little public health attention especially in Africa and Ghana in particular.

Like most discoveries, the discovery of sickle cell disease was by accident in 1910 by one Dr. James Herrick in Chicago, USA. The first patient-Walter Clement Noel was a dental student of African descent. Noel suffered from respiratory problems which persisted for more than one month. It was discovered that his blood smear contained many sickled shaped and elongated cells. Later researches identified about nine variants of the genotype namely;

Sickle Cell SS
Sickle Cell SC
Sickle Cell S Beta plus Thalassemia
Sickle Cell S Beta Zero Thalassemia
Sickle Cell SD Punjab
Sickle Cell SO Arab
Sickle Cell S/Lepore
Sickle Cell S Sigma Beta Zero Thalassemia and

S/HPFH, known as Hereditary Persistence of Fetal Hemoglobin.

According Professor Kweku Ohene-Frempong-President of the Sickle Cell Foundation of Ghana, the disease is believed to have originated in West and Central Africa, India and East Saudi Arabia around 2000 to 1000 BC and spread to North Africa, the Mediterranean and the Middle East during the early migration in about 1000 to 200 BC. The disease later got the Americas and Europe between 1500 and 1900 AD particularly through the Trans-Atlantic Slave Trade.

Medical research has noted that among the numerous Non-communicable diseases (NCDs) and common adult health problems in general, Sickle Cell Disease (SCD) is found to be associated with most of them. Therefore to achieve a reduction in the burden of Non-communicable diseases (NCDs) there is an urgent need for a Comprehensive National Sickle Cell Programme.

Non-Communicable diseases (NCDs) such as stroke, hypertension, chronic pulmonary disease, retinopathy and blindness, hip and shoulder osteonecrosis, renal failure, diabetes, asthma, arthritis and cancers which are said to be associated with the disease present unique challenges for managing.

As at 2010, global estimates of children born with Sickle cell SS alone was 312 302 while 5 476 407 of newborns in that year had the trait of the disease (AS). Out of these estimates Sub Saharan Africa accounts for 75.5% of those with the disease (SS) and 65.4% of those with the trait (AS).

Frederic B. Piel and others projected that between 2010 and 2050 there would be a 32.2% increase in the number of newborns with the sickle cell disease SS (SCD SS) globally. It however heart rendering to note that out of this figure, the Americas, Arab-India and Eurasia would record a decrease ranging from 12.7% to 22.7% while Sub Saharan Africa and South East Asia would see an increase of 46.0% and 14.3% respectively.

A country by country disaggregation of the global projections shows that within the same period (2010-2050) Ghana would witness a 17.9% increase in the number of newborns with Sickle cell disease SS (SCD SS). Presently it is estimated that about 2% of children born every year test positive for sickle cell disease- SCD SS and SC, this translates to 1 in every 50 newborns- a figure that is relatively higher than the HIV/AIDS prevalence rate of 1.3%.

Unlike HIV/AIDS however, one cannot be infected with sickle cell disease by person except your parents. The two parents must be carriers or one is a carrier and the other has the disease. Where both parents are carriers, it is estimated that their newborn has a 25% chance of having the disease (SCD), 50% chance of also being carrier (AS/AC) and 25% chance being free of the disease that is neither a carrier nor a sickler. In Ghana 1 in every 4 Ghanaians is said to be carrying genes that can lead to Sickle cell disease when passed onto children.

Manifestations of the disease in children include but not limited to; Anaemia, Poor growth, Easy tiredness and Jaundice (Yellow Eyes). Some common complications include; Frequent attacks of severe pain also called crisis, acute severe anaemia, malaria and other infections, stroke (especially among children) acute lung damage and many more. In fact the experience of a person with sickle cell disease is aptly summarized in the words of Ashraf Kamulale- a Ugandan living with the disease thus; “All you need for a lifelong romance with anxiety is a diagnosis with sickle cell” Nike Opalemo- a Nigerian also living with the disease on her part describes her experience as ‘the height of child abuse for two lovers to bring forth child with the disease in the name of love’.

Scott, Frederic and others opined that though there is insufficient data to make a definitive statement, there is consistent evidence with the early life mortality of 50%-90% among children born in Africa with the disease. The World Health Organization (WHO) on its part estimates that sickle cell disease contributes the equivalent of 5% of under-five mortality on the African continent with over 9% of such deaths in West Africa and up to 16% of under-five deaths in individual West African countries. What happens to children born with sickle cell disease according Professor Kwaku Ohene-Frempong depends on where the child is born and lives. Children born with sickle cell disease are special children who need extraordinary care. This includes professional medical care and familial care.

According to Dr. Ike Lagunju- a Paediatric Stroke specialist, 10% of children with the disease would suffer a stroke before their 20 birthday.

Gaston and others observed that neonatal screening, when linked to timely diagnostic testing, parental education and comprehensive care markedly reduces morbidity and mortality from sickle cell disease (SCD) in infancy and early childhood. Adegoke and Kuteyi – of the Departments of Paediatrics and Community health respectively of the Obafemi Awolowo University also opined that favourable familial care has a good prognostic index for children living with the disease. Truth be told however that, caring for a child with sickle cell disease in Africa can be expensive and depressing. Long term management of the disease goes beyond just the healthcare needs of the patient but includes other goals of their Psychosocial wellbeing particularly education, independence and eventually marriage and family.

A barrier however, is noted in providers inadequate understanding of the multiple layers of learning needed to live with the disease and the realities of trying to be “normal” and fit in.

So what happens to the estimated 15000 children born with sickle cell disease each year in Ghana?

So far the only known cure for sickle cell disease is Bone marrow Transplant which is not only expensive but also invasive and requires a matching donor.

The glimmer of hope is the National Newborn Screening Programme for Sickle Cell Disease; the work the Sickle Cell Foundation of Ghana has been doing over the years in the quiet. Though the Programme is presently only in Kumasi, The Foundation (SCFG) is determined to deliver on its mandate of implementing a National Newborn Screening Programme, the first of its kind in African, on behalf of the Ministry Of Health (MOH). It is projected that large scale universal screening could save the lives of over 9 million children with the disease, 85% of whom would be born in Africa by 2050.

Indeed there is growing evidence that contrary to earlier views expressed by persons within and without the medical fraternity that persons born with sickle cell disease would not make it pass ages 16, 25 and 30 respectively, people with the disease have made it to ages 55 and counting.

According to a Cooperative study on Sickle Cell Disease (SCD), it is estimated that the median survival age for a person with Sickle Cell SS (SCD SS) is 42 years for males and 48 years for females while Sickle Cell SC (SCD SC) is 60 years for males and 68 years for females.

Thanks to comprehensive care and the use of penicillin prophylaxis, Vaccination and recently the use of hydroxyurea to reduce the frequency of crisis.

This is the opportunity to alter the course of the disease for the thousands of people in Ghana, especially young children. However, like any other Public Health Programme, the success or otherwise of it depends heavily on the support and cooperation it enjoys from the citizenry and key stakeholders both local and international as well as government.

In this regard, let us unite in our efforts to lend support for the prevention and management of the disease through advocacy and participation in the activities relating to the disease. By this we shall be showing love for country and fellow. After today let us begin to see the involvement of high profiled personalities like our first and second ladies, ministers both of religion and government and celebrities as it pertains in other countries including our next door neighbor Nigeria as we wait for support from wherever it may come. For our brothers and sister who maybe living with the disease, this is the time to break the silence, shake of the stigma and speak out. Tell your story, let the whole World know what you are going through and let’s get more people to test for Sickle Cell Disease before saying “I do”

If these interventions and more have worked in the developed World why not our owned country Ghana!

After all persons with sickle cell disease are simply exceptional persons with extraordinary genes whose journey of living with the disease has many twist and turns. It could your child’s, brother’s/ sister’s or cousin’s life.

As we reflect upon these things lets us keep in mind that sickle cell disease is directly related to five out of the eight Millennium Development Goals (MDGs) and indirectly linked to the remaining three. Therefore if we lax in our collective effort at combating Sickle Cell Disease, we shall derail our forward match towards the attainment of these lofty goals.

Let’s hope a word to a wise is truly enough!

Adam Soale
Email: [email protected]
Tel: 0242318917/0207307714