Author: Isaac Boadu (BSc Biochemistry, MPhil Nutrition)
Department of Zoology and Environmental Biology (Parasitology and Public Health), University of Nigeria, Nsukka. (Email: [email protected])
Sickle cell disease (SCD) is one of the common chronic genetic disorders affecting the Africa populace. The disease presents a severe burden on sufferers and it is associated with increased ill health and death. It occurs because of a change in DNA composition (point mutation) which results in the production of abnormal haemoglobin. The point mutation results in the substitution of a single polar amino acid (glutamic acid) by a non-polar amino acid (valine) in haemoglobin (Hb) S .
There are different types of SCD depending on the type of haemoglobin gene inherited from parents. SCD is described within four major genotypes: homozygous sickle cell (SS) disease, sickle haemoglobin C (SC) disease, sickle cell β+ thalassemia (Sβ+ thalassemia) and sickle cell β0 thalassemia (Sβ0 thalassemia) . These different genotypes are associated with adverse health outcomes with varying severity. The most common and clinically severe form of SCD is haemoglobin SS, whereby a sickle S gene is inherited from both parents often referred to as sickle cell anaemia and Sβ0 thalassemia . People suffering from sickle cell anaemia (SS) experience severe complications such as pain, serious infections, chronic anaemia and organ failure compared to the other heterogeneous types of the disease (HbSC, HbS/β0, HbS/β+) .
In Ghana, the common genotypes identified are ‘SS’, ‘SC’, ‘SD’ and Sβ thalassemia . SCD differs from sickle cell trait (SCT) or carriers of the sickle cell gene. People who have SCT do not have the disease. They inherit a normal haemoglobin (A) from one parent and an abnormal haemoglobin (S/C) from the other parent. Carriers often do not exhibit signs and symptoms of the disease but are capable of passing the gene to their children.
Globally, it is estimated that about 400,000 children are born annually with SCD and over 80% of these births occur in Africa especially West and Central Africa . In Ghana, a recent report indicates that 2% (15000) of annual newborns are affected by SCD and 25% of the Ghanaian population are carriers of the sickle cell gene. That is, one in three Ghanaians have the haemoglobin S and/or C gene . These statistics raise serious concern about the disease epidemiology, awareness and management options. The question is ‘How far has the level of awareness been raised in Ghana, what management options are available and what happens to the reported 15000 infants born with SCD?’- Update of these questions are highlighted here.
Since the recognition of SCD as a public health concern by WHO in 2006  and the UN in 2008 , efforts have been made by governmental and non-governmental organizations in Ghana to increase awareness and public knowledge. Specific examples are ‘Sickle life’, a non-governmental organization, and the advocacy group, ‘Sickle Cell Anemia Foundation GH (SCAFGH)’ who provide information on the potential chances of giving birth to a child with SCD via social media and other platforms. They also organize tests for the genetic disorder. For example, on the 18th of June, 2017, ‘SICKLE LIFE’ in collaboration with Vodafone Ghana organized testing and education for the public in Accra to increase awareness . This was done prior to commemorating the World Sickle Cell Awareness Day (19th June) which is held annually to help increase public knowledge and raise awareness of the disease.
The SCAFGH was inaugurated in 2004. The organization was tasked by the Health Ministry in 2005 to lead the piloted newborn screening programme with the aim to develop a sickle cell counselling and health education to the public . The foundation recently (2016) secured funding of $4.5 million from the Ghana National Petroleum Authority (GNPC) to establish a Sickle Cell Treatment and Research Centre at Komfo Anokye Teaching Hospital (KATH), Kumasi . Though significant progress has been made to achieve the mission of SCAFGH, their activities are limited to the major cities of Ghana (Accra and Kumasi) and performance in terms of awareness creation and testing can be concluded to be inadequate. At the time of writing this article, the last update of SCAFGH on Facebook was 11th September 2014. Secondly, updates of the activities of SCAFGH are silent in the Ghanaian literature.
Other NGOs such as the ‘Bone Marrow Transplant Foundation Ghana International’ has recently been established to provide a curative solution to SCD. The foundation has liaised with Ridge Regional Hospital where a centre will be provided to carry out bone marrow transplant, the only curative option. Although there is a dearth of literature on the level of awareness and knowledge of SCD in Ghana, the few studies   have clearly pointed out the significant misunderstanding and inadequate knowledge of Ghanaians on SCD. Literature from US and UK presents extensive information on awareness and knowledge level of SCD yet a disease of such epidemiological and public health importance has been relatively unexplored and often taken for granted in Ghana.
A large body of evidence has been established on the importance of newborn screening coupled with penicillin prophylaxis as an effective way to significantly reduce ill health and death from SCD. In 2010, Ghana started a national newborn screening programme with support from the Ministry of Health and the Brazilian Government as an effort to manage SCD . Genetic counselling and education are essential components of NBS programme for SCD to ensure that results are effectively communicated to sufferers and their families. In the UK, newborn screening is shortly done after birth. Once a haemoglobin disorder is detected, a neonatal screening team visit and educate the affected family of the disease . This is not so different from the newborn screening programme in Ghana where babies are screened at birth or at well-baby visits within days or a few weeks after birth . However, mothers are asked to come for results within 4 weeks instead of a home visit. Visiting the affected family is reported to be only done when the family fails to come for results which rely solely on information provided by mothers at the time of screening. This is a major challenge to achieve the goals of the programme since most mothers do not believe their children are affected by the disease.
From 1993-2008, Ghana started a pilot newborn screening programme with support from US National Institutes of Health, in Kumasi and Tikrom, nearby rural community, to identify babies with SCD. These children were enrolled in a Sickle Cell Clinic at KATH . Despite 8 years of implementation of the national neonatal newborn screening, there has been no statistics on survival or death information of the children enrolled in the various Sickle Cell Clinics. This eluded statistics is important to identify areas of need, attract policy support and resources for measures to reduce the burden of SCD mortality.
Although impressive measures have been put in place in the management of sickle cell patients, the cost-effective and potent drug, hydroxyurea, which has been shown to decrease the number of days of hospitalization and transfusion, lower crises rate, and lessen acute chest syndrome  is yet to be introduced as a treatment option in Ghana. The top ten drug commonly prescribed to SCD patients in Ghana include folic acid, diclofenac, ibuprofen, B-complex, artesunate/amodiaquine, paracetamol, penicillin V, amoxiclav and zincovit .
As part of efforts to address the incidence of SCD and achieve the UN sustainable development goal of ‘good health and well-being’, there is a need to raise awareness and increase public education. Screening tests should be made available not only in urban centres but also in the rural and peri-urban settings where the SCD may be prevalent. Management options should also be multidisciplinary featuring medical and non-medical professionals. There should be available funds by the Health Ministry through SCAFGH for more research to be carried out to update public knowledge on SCD.
- Moheeb, H., Wali, Y. A., & El‐Sayed, M. S. (2007). Physical fitness indices and anthropometrics profiles in schoolchildren with sickle cell trait/disease. American journal of hematology, 82(2), 91-97.
- Al-Saqladi, A. W., Cipolotti, R., Fijnvandraat, K., & Brabin, B. J. (2008). Growth and nutritional status of children with homozygous sickle cell disease. Ann Trop Paediatr, 28(3), 165-189.
- Kanter, J., & Kruse-Jarres, R. (2013). Management of sickle cell disease from childhood through adulthood. Blood Rev, 27(6), 279-287.
- Hyacinth, H. I., Gee, B. E., & Hibbert, J. M. (2010). The Role of Nutrition in Sickle Cell Disease. Nutr Metab Insights, 3, 57-67.
- Osei-Yeboah, C., Rodrigues, O., & Enweronu-Laryea, C. (2011). Nutritional status of children with sickle cell disease at Korle Bu Teaching Hospital, Accra, Ghana. West Afr J Med, 30(4), 262-267.
- Dennis-Antwi, J. A., Dyson, S., & Frempong, K. O. (2008). Healthcare provision for sickle cell disease in Ghana: challenges for the African context. Diversity & Equality in Health and Care. 5:241-254.
- Edwin, A. K., Edwin, F., & Etwire, V. (2011). Controlling sickle cell disease in Ghana ethics and options. Pan African Medical Journal, 10.
- World Health Organisation. Sickle-Cell Anaemia Report by the Secretariat. Fifty Ninth World Health Assembly, 2006. [http://apps.who.int/gb/ebwha/pdf_files/WHA59/A59_9-en.pdf]. Accessed January 22, 2018.
- United Nations General Assembly. Recognition of sickle-cell anaemia as a public health problem. Sixty-third General Assembly, 2008. [www.worldlii.org/int/other/UNGARsn/2008/277.pdf] Accessed January 22, 2018.
- https://www.graphic.com.gh/news/general-news/15-000-babies-born-with-sickle-cell- disease-annually.html Accessed January 25, 2018.
- http://www.gnpcghana.com/press30.html, Accessed January 26, 2018.
- Orish, V. N., Onyeabor, O. S., Sanyaolu, A. O., & Iriemenam, N. C. (2014). Evaluating the knowledge of sickle cell disease and hemoglobin electrophoretic pattern among people living in Sekondi-Takoradi Metropolis, Ghana. Journal of Medicine in the Tropics, 16(2), 56.
- Ameade, E. P. K., Mohammed, B. S., Helegbe, G. K., & Yakubu, S. (2015). Sickle Cell Gene Transmission: Do Public Servants in Tamale, Ghana Have the Right Knowledge and Attitude to Curb It? Open Journal of Preventive Medicine, 5(06), 299.
- Cope, A., & Darbyshire, P. J. (2013). Sickle cell disease, update on management. Paediatrics and Child Health, 23(11), 480-485.
- Ohene-Frempong, K., Oduro, J., Tetteh, H., & Nkrumah, F. (2008). Screening newborns for sickle cell disease in Ghana. Pediatrics, 121(Supplement 2), S120-S121.
- Chawla, A., Sprinz, P. G., Welch, J., Heeney, M., Usmani, N., Pashankar, F., & Kavanagh, P. (2013). Weight status of children with sickle cell disease. Pediatrics, 131(4), 1168-1173.
- Nsiah, K., Osei-Akoto, A., & Ansong, D. (2014). The Drug Regimen Prescribed for Sickle Cell Patients Attending a Clinic in Kumasi, Ghana, in a Period of One Year. Open Journal of Blood Diseases, 4(04), 50.
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