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ORIGINAL ARTICLE
Year : 2022  |  Volume : 25  |  Issue : 2  |  Page : 52-56

Clinico-Demographic characteristics, morbidity and mortality patterns of sickle cell disease in a tertiary institution


Department of Paediatrics, Hematology/Oncology Unit, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

Date of Submission02-Jul-2020
Date of Decision19-Nov-2020
Date of Acceptance10-Dec-2020
Date of Web Publication22-Aug-2022

Correspondence Address:
Dr. Hafsat Rufai Ahmad
Department of Paediatrics, Haematology Oncology Unit, Ahmadu Bello University Teaching Hospital, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/smj.smj_78_20

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  Abstract 


Introduction: Sickle cell disease (SCD) is the most common hereditary hematological disorder in Nigeria with an annual incidence of more than 100,000 new births which contributes to the high Nigerian under-five childhood morbidity and mortality. Sufferers of the disease are frequently admitted into emergency rooms for presentations and complications such as pain crisis, anemia, stroke, and acute chest syndrome, in addition to other childhood infections such as severe malaria, sepsis, and acute respiratory tract infections. The aim of the study was to describe the clinicodemographic features and morbidity and mortality patterns of children with SCD in a tertiary institution. Methods: The study was a retrospective review of case records of children with SCD admitted with various diagnoses. Results: Complete records of 460 patients were reviewed and this constituted 10.1% of all new admissions during the period. There were more males, 286 (62%), than females, 174 (38%). The mean age was 6.3 ± 5.1 years and 249 (54.1%) were under the age of 5 years. The most common admitting diagnosis was a pain crisis comprising 168 cases (36.5%). The mean packed cell volume was 20.6 ± 4.1 inclusive of hemoglobin SC phenotype, and 197 (42.8%) had simple top-up transfusion, while 28 (6.1%) had exchange transfusion. A total of 438 (95%) patients were discharged, 21 (4.6%) died, and the highest mortality was from severe anemia (47.6%). Conclusion: This study describes the high burden of SCD constituting 10.1% of admissions, as well as the pattern of morbidity and mortality largely from severe anemia in the area under study.

Keywords: Morbidity, mortality, sickle cell disease


How to cite this article:
Faruk JA, Adebiyi MN, Ahmad HR. Clinico-Demographic characteristics, morbidity and mortality patterns of sickle cell disease in a tertiary institution. Sahel Med J 2022;25:52-6

How to cite this URL:
Faruk JA, Adebiyi MN, Ahmad HR. Clinico-Demographic characteristics, morbidity and mortality patterns of sickle cell disease in a tertiary institution. Sahel Med J [serial online] 2022 [cited 2022 Oct 2];25:52-6. Available from: https://www.smjonline.org/text.asp?2022/25/2/52/354190




  Introduction Top


Sickle cell anemia (sickle cell disease [SCD]) is the most common hereditary hematological disease in Nigeria, with a trait prevalence of 24%[1] and as high as 40.3% in Kano.[2] Nigeria has the highest global burden of SCD,[1] affecting about 2% of the population and an annual incidence of more than 100,000 new births.[1] The autosomal recessively inherited abnormal hemoglobin S when exposed to hypoxia undergoes polymerization to produce needle-like structures that result in the typical sickled red blood cells responsible for the pathogenesis of the disease.[3] The disease is characterized by chronic hemolytic anemia, chronic tissue hypoxia with ischemia, and infarction that gives the clinical crisis complications affecting all parts of the body.[4] The high incidence of vaccine-preventable diseases such as pneumonia,[5] malaria, and dehydration due to infections and acute diarrheal diseases are some of the common triggers for development of various crises in our setting.[6] These triggers frequently result in a crisis that may require hospitalization for pain relief, blood transfusions, and other forms of intervention.[7] In children, the major reasons for hospital admission include severe pain, anemia, infections, stroke, and acute chest syndrome.[8] The treatment measures usually include hydration, pain relief, antibiotics for infections, antimalarial treatment and prophylaxis, blood transfusions, folic acid, and anti-sickling agents such as hydroxyurea.[9],[10] Due to the high burden of SCD, it is therefore an important public health problem that impacts negatively on the quality of life of the sufferers and also exerts high demand on the health-care system.[11] The morbidity and mortality due to complications of the disease are enormous, especially in resource-poor settings where hospital admission rates reache up to 2.5/child/year.[12] Expectedly, this contributes to the high under-five and childhood morbidity and mortality rates in the country.[10],[11]

Since SCD is a chronic illness, families and sufferers face not only physical challenges due to the disease but also significant psychological and financial burden due to stigma and cost of care.[12],[13],[14] The impact on the family is worse in resource-poor settings due to lack of awareness about the disease, poor attitude, inadequate health-care facilities, inefficient health care systems, lack of social support, and almost nonexisting health insurance.[6],[14]

This study aimed to document the clinical and demographic features as well as outcomes of children with sickle cell anemia admitted into the pediatric medical wards (PMWs) of a tertiary institution in northwestern Nigeria.


  Methods Top


This was a hospital-based retrospective descriptive study of SCD subjects, aged 6 months to 17 years, admitted with various clinical diagnoses. Admission and discharge registers were used to identify children admitted over 18 months between January 2016 and June 2017 at the PMW and emergency pediatric unit (EPU) with a clinical diagnosis and laboratory confirmation of SCD. Laboratory confirmation of diagnosis was by alkaline gel electrophoresis which is performed at or above 6 months of age. Case records of these patients were manually collected from the central record library, hematology/oncology clinic, or pediatric/EPU record office. Case records without documentation of the laboratory confirmation and/or an initial full blood count (FBC) were excluded from the study. Information extracted included the biodata, phenotypic diagnosis, manual FBC reports, treatments given, duration of admission, and outcomes of management. For anonymity, patients' names were omitted from data entry. Data were coded and stored in a passworded file and shared only among research participants. Being a retrospective study, ethical permission to review clinical data, share the data among study participants, and publish the review was obtained from the Health Research Ethics Committee (HREC) of Ahmadu Bello University Teaching Hospital (ABUTH), Zaria, numbered ABUTH/HREC/K23/2019 dated May 24, 2019. All procedures were carried out according to the Declaration of Helsinki guidelines 2013. Data were entered into Excel 2017 version for the analysis. Data were presented in texts and tables, and averages were calculated as percentages and proportions. Student's t-test was used to determine the level of significance of the mean values at 0.5 significance level.


  Results Top


There were 515 admissions of children with a clinical diagnosis of SCD during the study period. This constituted 11.3% of 4562 total new admissions into the pediatric wards excluding the neonatal ward. Laboratory confirmation and initial FBC of 55 of the cases could not be retrieved and were therefore excluded from the analysis, while the 460 (10.1% of all admissions) cases had documented laboratory diagnosis and an initial FBC on admission and were therefore reviewed and analyzed. The age and gender distribution is as shown in [Table 1]. The male-to-female ratio was 1.6:1 with the total male population at 286 (62%). Two hundred and forty-nine (54.1%) of the children were under 5 years old. The mean age was 6.3 ± 5.1 years, range 7 months to 17 years.
Table 1: Age and gender distribution of patients admitted with sickle cell disease

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[Table 2] shows the socioeconomic status (SES) using the Oyedeji classification,[15] type of family, and ethnicity of the subjects. One hundred and twenty-one subjects (26.2%) were from upper SES (8.2% Class I, 18% Class II); 280 (60.9%) were from a monogamous family setting, while 392 (87.2%) were of Hausa–Fulani ethnicity. Only 2 (0.2%) of the studied patients had hemoglobin SC phenotype, and the rest had Hemoglobin SS phenotype. Eighteen (3.9%) children were admitted three or more times during this period.
Table 2: Demographic features of children admitted with sickle cell disease

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[Table 3] shows the various admitting diagnoses of the subjects. Pain crisis defined in this study as pain and/swellings affecting the upper and lower limbs, back, chest, and sternum was the most frequent presentation accounting for 168 (36.5%) cases, of which 36 (21.4%) had pain crisis and evidence of hemolysis and 23 (13.7%) had pain crisis, hemolysis, and severe anemia. Of the 81 (17.6%) children with severe anemia, 21 (26%) presented with hemolytic crisis, while 60 (74.1%) presented with other anemic crises at the time of admission. Of the 63 (13.7%) cases with osteomyelitis, 42 (66.7%) were acute, 5 (7.9%) acute on chronic, and 5 (7.8%) chronic osteomyelitis. Eleven cases (19.5%) had multifocal osteomyelitis. There were 10 clinical diagnoses of typhoid fever, the presentation of which in this study included fever of up to 2-week duration, abdominal pain, vomiting, diarrhea or constipation, headache, and anemia; however,  Salmonella More Details typhi was isolated in only two of them. Similarly, there were four culture proven cases of sepsis with Klebsiella accounting for three and one case of Staphylococcus aureus. Urine cultures yielded  Escherichia More Details coli in two cases and Klebsiella spp, Proteus, and pseudomonas in one case each. Malarial parasite was demonstrated in 51 (11.1%) cases.
Table 3: Admitting diagnoses of sickle cell disease subjects

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[Table 4] shows the means of the weight, heights, packed cell volume, white blood cell counts, platelets, and reticulocyte counts. There were no statistically significant differences in the means of these values between males and females.
Table 4: Mean values of anthropometry and haematological parameters of subjects with sickle cell disease

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Treatment and outcomes

One hundred and ninety-seven patients (42.8%) had simple top-up blood transfusions and 28 (6.1%) had exchange blood transfusions for stroke, acute chest syndrome, and priapism. Although all the patients had antimalarial treatment, malarial parasites could only be demonstrated in 51 (11%) of the cases. Three hundred and thirty-two (72.1%) patients had antibiotics prior to admission. Seventy-three (15.9%) patients were started on hydroxyurea treatment for reasons that included stroke, recurrent admissions and blood transfusions, acute chest syndrome, avascular necrosis of head of femur, priapism, and chronic osteomyelitis.

Four hundred and thirty-eight patients (95%) were discharged to follow-up, one discharged against medical advice, and 21 died giving a hospital mortality rate of 4.6%. [Table 5] shows the mortality according to the diagnosis. Eleven of 21 dead patients (52.4%) demised within 48 h of admission. The highest mortality was from severe anemia accounting for 10 patients (47.6%%).
Table 5: Outcomes of subjects admitted with sickle cell disease and complications

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  Discussion Top


In this review, admissions due to SCD and related complications were 10.1% of the total admissions in the department. This underscores the burden of SCD in the region. Although more than half of the children were under the age of 5 years, more than one-third of the subjects were also aged 6 years and above, which could mean an improved survival of these patients beyond 5 years and or better health-care-seeking behavior of the parents. This could further be corroborated by the observation that more than half of the patients belong to upper and middle SES (I-III) and may imply better care and good health-care-seeking behavior. Interestingly, males were significantly higher in number, probably a true reflection of SCD in the community or the males might have more preponderance to having a crisis or perhaps a reflection of the proportion of males in the community. Gladwin et al.[16] and Ilesanmi[17] had implicated the antioxidant nitric oxide which was noted to be twice more available to females than males both in steady state and during crisis, suggesting that males are more prone to crises and attendant complications. Yauba et al.[18] reported a male preponderance of 54.2% in a study from the same institution that included SCD subjects in steady state and crisis who presented to our teaching hospital and a community outreach primary health-care center. This could also be a reflection of health-seeking behavior of parents favoring males over females. The Hausa preponderance is a reflection of the setting of the health facility.[18] The predominant sickle cell phenotype of SS is in keeping with previous documentation in the region.[19]

The most common complication seen was pain crisis either alone or in association with other complications such as hemolysis, severe anemia, sequestration, and so on. This is similar to what was obtained in other places.[20] The different severe anemia syndromes ranked second in occurrence in contrast to Enugu where severe anemia was the most common complication encountered.[21] Interestingly, the cases of severe anemia without hemolysis or pain were many and may be a pointer toward contribution of other causes of anemia such as subclinical infections which could either be bacterial, parasitic, or viral, simple malaria, nutritional anemia,[22] or parvovirus-related aplastic anemia which is difficult to diagnose in our institution due to lack of facilities.

Although infections have been implicated as reasons for hospitalization in SCD,[21],[23] the culture yield in our patients was very low. This could probably be due to prior antibiotic use in these subjects and the lack of more sensitive diagnostic facilities. In the same vein, the evidence for malaria as a precipitator of crisis from this study was demonstrated in only 11% of subjects, perhaps due to prior antimalarial treatment. Makani et al.[24] in a 5-year prospective study in Dar el Salam had shown that children with SCD had lower level of parasitemia both at the clinic and during hospitalization compared with non-SCD children. However, parasitemia during hospitalization was associated with a higher incidence of severe anemia and death.

Blood transfusion either as simple top-up or exchange transfusion was given to 48.9% of the patients. Being a disease-modifying treatment, the use of blood transfusion in our setting becomes even more important as most patients presented with complications of anemia such as cardiac failure.[25] Hydroxyurea treatment was started for 15.9% of the patients indicating the extent of severe complications of the disease. It also shows the acceptance and relative confidence of the patients and caregivers bearing in mind the numerous providers, patients, and system-related barriers that challenge the efficient use of the drug.[26] The mortality of 4.6% was lower than that obtained in Enugu. However, this is just the tip of the iceberg as more than this are likely to die in the community without being audited for. A huge proportion of SCD do not present to the hospital for care because of financial constraint, stigma, patronage of private clinics and/or alternative medicine, and caregiver's fatigue.


  Conclusion Top


A high burden of SCD admissions with more than half being children <5 years old is demonstrated. The morbidity pattern is varied comprising SCD crises and other complications including bacterial infections and malaria. Mortality occurred in 4.6% of the subjects, half of which was due to severe anemia. Early infant diagnosis and comprehensive care inclusive of infection prophylaxis is a key to minimizing morbidity and mortality.

Acknowledgment

Drs. Hope Omeiza and Favour Lebari are hereby acknowledged for assisting with the collation of case notes and data. All the staff and other doctors involved in the management of these patients are hereby acknowledged.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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National Guideline for the Control and Management of Sickle Cell Disease 2014. Abuja: Federal Ministry of Health; 2014.  Back to cited text no. 1
    
2.
Mustapha OT, Abubakar FH. Study of prevalence of sickle cell disease in Kano metropolis and its suburbans in Northern Nigeria. Nig J Basic Appl Sci 2011;10:219-25.  Back to cited text no. 2
    
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Chakravorty S, Williams TN. Sickle cell disease: A neglected chronic disease of increasing global health importance. Arch Dis Child 2015;100:48-53.  Back to cited text no. 4
    
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Makani J, Ofori Acquah SF, Nnodu O, WonkamA, Ohene Frempong K. Sickle cell disease: New opportunities and challenges in Africa. Scientific World J 2013;193252. [doi: 10.1155/2013/193252].  Back to cited text no. 6
    
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McCavit TL. Sickle cell disease. Pediatr Rev 2012;33:195-204.  Back to cited text no. 7
    
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Serjeant GR. Sickle-cell disease. Lancet 1997;350:725-30.  Back to cited text no. 8
    
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Kanter J, Kruse-Jarres R. Management of sickle cell disease from childhood through adulthood. Blood Rev 2013;27:279-87.  Back to cited text no. 9
    
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Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010–2050: Modelling based on demographics, excess mortality, and interventions. PLoS Med 2013;10:E1001484.  Back to cited text no. 10
    
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Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of sickle haemoglobin in neonates: A contemporary geostatistical model-based map and population estimates. Lancet 2013;381:142-51.  Back to cited text no. 11
    
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Adegoke SA, Abioye-Kuteyi EA, Orji EO. The rate and cost of hospitalization in children with sickle cell anaemia and its implications in a developing economy. Afr Health Sci 2014;14:475-80.  Back to cited text no. 12
    
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Ani C, Aranda AM, Kinanee J, Bolanle O, Kramer T. Trainee teachers' stigmatising attitude towards sickle cell disorders in Nigeria. Euro J Educ Stud 2012;4:349-60.  Back to cited text no. 13
    
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Afolayan JA, Jolayemi FT. Parental attitude to children with sickle cell disease in selected health facilities in irepodun local government, Kwara State, Nigeria. Ethno Med 2011;5:33-40.  Back to cited text no. 14
    
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Oyedeji GA. Socio-economic and cultural background of hospitalized children in Ilesa. Niger J Paediatr 1985;12:111-7.  Back to cited text no. 15
    
16.
Gladwin MT, Schechter AN, Ognibene FP, Coles WA, Reiter CD, Schenke WH, et al. Divergent nitric oxide bioavailability in men and women with sickle cell disease. Circulation 2003;107:271-8.  Back to cited text no. 16
    
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Ilesanmi OO. Gender differences in sickle cell crises: Implications for genetic counselling and psychotherapy. J Psychol Psychother 2013;3:123.  Back to cited text no. 17
    
18.
Yauba MS, Aikhionbare HA, Ogunrinde GO, Bugaje MA. Significant bacteriuria in children with sickle cell anaemia in a Nigerian tertiary institution. Nigr J Paed 2014;41:350-3.  Back to cited text no. 18
    
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Inusa BP, Daniel Y, Lawson JO, Dada J, Matthews CE, Momi SS, et al. Sickle cell disease screening in northern Nigeria: The Co-existence of B-thalassemia inheritance. Pediat Therapeut 2015;5:262.  Back to cited text no. 19
    
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Salman ZA, Hassan MK. Hospitalization events among children and adolescents with sickle cell disease in Basra, Iraq. Anemia 2015;2015:195469.  Back to cited text no. 20
    
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Ikefuna AN, Emodi I. Hospital admission of patients with sickle cell anaemia, pattern and outcomes in Enugu area Nigeria. Nig J Clin Pract 2007;10:24-9.  Back to cited text no. 21
    
22.
Bello-Manga H, De Baun MR, Kassim AA. Epidemiology and treatment of relative anemia in children with sickle cell disease in sub-Saharan Africa. Expert Rev Hematol 2016;9:1031-42.  Back to cited text no. 22
    
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Abhulimhen-Iyoha BI, Israel-Aina YT, Joel-Utomakili K. Sickle cell anaemia: Morbidity profile and outcome in a paediatric emergency setting in Nigeria. Afr J Med Health Sci 2015;14:79-82.  Back to cited text no. 23
  [Full text]  
24.
Makani J, Komba AN, Cox SE, Oruo J, Mwantemi K, Kitundu J, et al. Malaria in patients with sickle cell anaemia: Burden, risk factors and outcomes at the outpatient clinic and during hospitalization. Blood 2010;115:215-20.  Back to cited text no. 24
    
25.
Howard J. The role of blood transfusion in sickle cell disease. ISBT Sci Series 2013;8:225-8.  Back to cited text no. 25
    
26.
Brandow AM, Panepinto JA. Hydroxyurea use in sickle cell disease: The battle with low prescription rates, poor patient compliance and fears of toxicities. Expert Rev Hematol 2010;3:255-60.  Back to cited text no. 26
    



 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]



 

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