Oluwagbemiga Adeodu, Morenike Akinlosotu, Samuel Adegoke, Saheed Oseni
  • Oluwagbemiga Adeodu
    Department of Paediatrics and Child Health, OAU, Ile-Ife, Nigeria
  • Morenike Akinlosotu
    Department of Paediatrics and Child Health, OAUTHC, Ile-Ife, Nigeria
  • Samuel Adegoke
    Department of Paediatrics and Child Health, Obafemi Awolowo University, Ile Ife, Osun state, Nigeria., Nigeria |
  • Saheed Oseni
    Department of Paediatrics and Child Health, OAU, Ile-Ife, Nigeria


Background: Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. Relationship between HbF and SCD severity among affected children is also poorly studied.

Methods: In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 – 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined. The latter was assessed based on the frequency of significant painful episodes, blood transfusion, and hospitalization in the preceding 12 months; lifetime cummulative incidence of SCD-related complications; degree of splenic and hepatic enlargement; current haematocrit and leucocyte count, as previously described. Foetal haemoglobin levels were quantified with high performance liquid chromatography.

Results: The mean HbF level of the 105 children with SCA was 9.9 ± 6.0%. Male had significantly lower mean HbF levels than females, 8.0 ± 5.6% vs. 12.2 ± 5.8% (p < 0.001). None of the children had severe disease. However, those with moderate disease had significantly lower mean foetal haemoglobin levels than those with mild disease (7.7 ± 5.6% vs. 10.8 ± 6.0% respectively). The mean HbF level was also significantly lower in children who had history of acute chest syndrome and stroke compared to those without these complications,   p = 0.002 and 0.010 respectively.

Conclusion: Children with SCA who had moderate disease and those with history of life threatening complications such as stroke and acute chest syndrome had significantly low HbF. Therefore it is recommended that facilities for early quantification of foetal haemoglobin and HbF inducement be made available in order to reduce the morbidity and mortality among these children.


Children, sickle cell anaemia, foetal haemoglobin, acute chest syndrome

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Submitted: 2017-07-30 16:03:57
Published: 2017-11-01 00:00:00
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Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86(6):480-7.

Sickle-cell anaemia. Agenda item 11.4. In: 59th World Health Assembly; 2006; Geneva: World Health Organization.

Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. Multicenter Study of Hydroxyurea in Sickle Cell Anemia: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332:1317.

Adegoke SA, Adeodu OO, Adekile AD. Sickle cell disease clinical phenotypes in children from South-Western, Nigeria. NigerJClinPract. 2015;18(1):95-101.

Serjeant GR. Fetal hemoglobin in homozygous sickle cell disease Clin Haematol 1975;4:109-22.

Oyedeji GA. Socioeconomic and cultural background of hospitalised children in Ilesa. Niger J Paediatr. 1985;12:111-7.

Ballas SK, Lieff S, Benjamin LJ, Dampier CD, Heeney MM, Hoppe C, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol 2010;85:6-13.

Adegoke SA, Kuti BP. Evaluation of clinical severity of sickle cell anemia in Nigerian children. J Applied Hematol. 2013;4:58-64.

Mpalampa L, Ndugwa CM, Ddungu H, Idro R. Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda. BMC Blood disorders. 2012;12:11.

Norman NH, Nie, Hull CH. Statistical package for social sciences for Windows. 17.0 ed. Chicago: SPSS Incoporation; 2008.

Akinsheye I, Alsultan A, Solovieff N, Ngo D, Baldwin CT, Sebastian P, et al. Fetal hemoglobin in sickle cell anemia. Blood. 2011;118(1):19-27.

Brown B, Jacob N, Lagunju I, Jarrett O. Morbidity and mortality pattern in hospitalized children with sickle cell disorders at the University College Hospital, Ibadan, Nigeria. Niger J Paed. 2013;40(1):34-9.

Brozovic M, Davies S, Alison I, Brownell A. Acute admissions of patients with sickle cell disease who live in Britain. Br Med J. 1987;294:1206-8.

Wasil J. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med. 2011 31:289-93.

Adewoyin AS. Management of Sickle Cell Disease: a review for physician education in Nigeria (Sub-Saharan Africa). Anemia. 2015;2015:e791498.

Isah IZ, Udomah FP, Erhabor O, Aghedo F, Uko EK, Okwesili AN, et al. Foetal haemoglobin levels in sickle cell disease patients in Sokoto, Nigeria. Br J Med Health Sci. 2013;1:36-47.

Tshilolo L, Summa V, Gregorj C, Kinsiama C, Bazeboso JA, Avvisati G, et al. Foetal haemoglobin, erythrocytes containing foetal haemoglobin, and hematological features in Congolese patients with sickle cell anaemia. Anemia. 2012;2012:e105349.

Olaniyi JA, Arinola OG, Odetunde AB. Foetal haemoglobin (HbF) status in adult sickle cell anaemia patients in Ibadan, Nigeria. Ann Ibadan Postgrad Med. 2010;8:30-3.

Durosimi MA, Salawu L, Ova YAA, Lawal OO, Fadiran OA. Haematological parameters in sickle cell anaemia patients with and without splenomegaly. Niger Postgrad Med J. 2005;12(4):271-4.

Uko E, Useh M, Gwanmesia F. Frequency of foetal haemoglobin and haemoglobin values in various haemoglobin genotypes in Calabar, Nigeria. East Afr Med J. 1997;74(12):809-11.

Omoti CE. The value of foetal haemoglobin level in the management of Nigerian sickle cell anaemia patients. Niger Postgrad Med J. 2005;12(3):149-54.

Rao SS, Goyal JP, Raghunath SV, Shah VB. Hematological profile of sickle cell disease from South Gujarat, India. Hematol Reports. 2012;4:e8.

Nagel RL, Fabry ME, Pagnier J, Zohoun I, Wajcman H, Baudin V, et al. Hematologically and genetically distinct forms of sickle cell anemia in Africa. N Engl J Med 1985 321:880.

Aimola IA, Inuwa HM, Nok AJ, Mamman AI. Induction of foetal haemoglobin synthesis in erythroid progenitor stem cells: mediated by water-soluble components of Terminalia catappa. Cell Biochem Funct. 2014;32(4):361-7.

Falusi AG, Esan GJ. Foetal haemoglobin levels in sickle cell anaemia in Nigerians. Afr J Med Sci. 1989;18:145-9.

Mouele R. Haemoglobin F (HbF) levels in sickle-cell anaemia patients homozygous for the Bantu haplotype. European Journal of Haematology. 1999;63:136–7.

Alsultan A, Solovieff N, Aleem A, AlGahtani FH, Al-Shehri A, Osman ME, et al. Fetal hemoglobin in sickle cell anemia: Saudi patients from the Southwestern province have similar HBB haplotypes but higher HbF levels than African Americans. Am J Hematol. 2011;86(7):612-4.

Dover GJ, Smith KD, Chang YC, Purvis S, Mays A, Meyers DA, et al. Fetal Hemoglobin Levels in Sickle Cell Disease and Normal Individuals Are Partially Controlled by an X-Linked Gene Located at Xp22.2. Blood. 1993;80(3):816-24.

Adekile A, Al-Kandari M, Haider M, Rajaa M, D'Souza M, Sukumaran J. Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients. Med Princ Pract. 2007;16(4):286-90.

Trompeter S, Roberts I. Haemoglobin F modulation in childhood sickle cell disease. Br J Haematol. 2008;144:308–16.

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