|Year : 2019 | Volume
| Issue : 2 | Page : 109-113
Age-related complications among individuals with sickle cell anemia attending a tertiary health facility in Northwestern Nigeria
Halima Bello-Manga1, Sani Awwalu2, Ifeoma P Ijei1, Livingstone G Dogara1
1 Department of Hematology and Blood Transfusion, Faculty of Basic Clinical Sciences, College of Medicine, Kaduna State University, Kaduna, Nigeria
2 Department of Hematology and Blood Transfusion, Ahmadu Bello University, Zaria, Nigeria
|Date of Submission||08-Mar-2019|
|Date of Decision||12-Apr-2019|
|Date of Acceptance||25-May-2019|
|Date of Web Publication||6-Dec-2019|
Dr. Halima Bello-Manga
Department of Hematology and Blood Transfusion, Faculty of Basic Clinical Sciences, College of Medicine, Kaduna State University, Kaduna
Source of Support: None, Conflict of Interest: None
Background: Sickle cell disease (SCD) is the most common monogenetic disorder worldwide. With an annual birthrate of approximately 150,000 in Nigeria, the burden is expected to increase by 75% in 2050. With improved care, patients live longer but may have more complications. This study seeks to determine the prevalence of some complications of sickle cell anemia (SCA) and how age relates to these complications. Methods: This was a 2-year retrospective review of clinic records of patients with SCA attending the hematology clinic of Barau Dikko Teaching Hospital, Kaduna State, Nigeria. Data on sex, age at last birthday, age at diagnosis of SCA, number of crises in past 6 months, proteinuria, steady-state hemoglobin concentration, chest infections, stroke, and oxygen saturation (SPO2) were collated. Statistical Analysis: Data were analyzed using SPSS version 21 (IBM Corp., 2012, Armonk NY, USA). Continuous and categorical variables were assessed using the Shapiro–Wilk test and percentages. Differences in presenting ages between variables were determined using Mann–Whitney U-tests. Level of statistical significance was set at P ≤ 0.05. Results: A total of 109 patients were retrieved, 68.8% (75/109) were females with a median (interquartile range [IQR]) age of 22(18, 29) years. The median (IQR) number of crises in the preceding 6 months was 0 (0, 3). The prevalence of proteinuria, chest infections in the preceding 6 months, severe anemia, low oxygenation, and stroke were 9.5% (4/42), 6.5% (5/77), 7.0% (5/71), 58.9% (33/56), and 1.3% (1/77), respectively. A positive correlation existed between number of crises and presenting age (r = 0.317; P = 0.005). Patients with proteinuria, severe anemia, chest infections, and low SPO2had higher mean rank presenting age while patients with stroke had lower mean rank presenting age. Conclusion: Older age is significantly associated with severity of SCD. Age-targeted interventions guided by evidence-based practices are important in slowing down disease progression and severity.
Keywords: Age, complications, sickle cell anemia
|How to cite this article:|
Bello-Manga H, Awwalu S, Ijei IP, Dogara LG. Age-related complications among individuals with sickle cell anemia attending a tertiary health facility in Northwestern Nigeria. Ann Trop Pathol 2019;10:109-13
|How to cite this URL:|
Bello-Manga H, Awwalu S, Ijei IP, Dogara LG. Age-related complications among individuals with sickle cell anemia attending a tertiary health facility in Northwestern Nigeria. Ann Trop Pathol [serial online] 2019 [cited 2020 Nov 26];10:109-13. Available from: https://www.atpjournal.org/text.asp?2019/10/2/109/272432
| Introduction|| |
Sickle cell disease (SCD) is the most common monogenetic disorder worldwide. It is a disease of major public health importance in sub-Saharan Africa; the region that harbors more than one-third of global disease burden and where access to basic health-care interventions is limited. With an annual birthrate of approximately 150,000 in some countries, unless specific actions are taken, the burden of SCD is projected to increase by ≥75% in 2050, particularly in Nigeria and the Democratic Republic of the Congo. Effective management of SCD involves genetic counseling, neonatal screening, and early diagnosis, combined with parental education and health maintenance. It is estimated that 90% of children born with SCD in sub-Saharan Africa will die before their 5th birthday. In high-income countries, newborn screening, penicillin prophylaxis, screening for stroke, and several interventions done as standard of care for individuals with SCD have changed the narrative of the disease from that of a life-threatening disease of children to a chronic disease of adults, with an overall improvement in health outcomes for this group of people. Unfortunately, the same is not replicated in sub-Saharan Africa, as there are very few newborn screening programs as well as a dearth of standard of care practices guided by well-documented recommendations incorporated into the care of all individuals with SCD. Few tertiary health-care facilities have made attempts at incorporating some of these standard care practices; however, this is far below the requirement, and those that present to such institutions represent only a small proportion of the true burden of SCD.
The aim of this study is to assess the proportions of patients with proteinuria, severe anemia, chest infections, and stroke as well as to determine any differences in age among patients with these presentations. This will form a basis for instituting some recommended age-targeted health management interventions, as the standard of care with the hope of slowing down the progression of these complications.
| Methods|| |
This was a retrospective review of clinic records of patients with sickle cell anemia (SCA) attending the hematology outpatient clinic of Barau Dikko Teaching Hospital, Kaduna State, Nigeria. Records of SCA patients who attended the adult sickle cell clinic over a 2-year period (January 1, 2017–December 31, 2018) were utilized. Patients who met the following criteria were included in the study: (i) the diagnosis of homozygous SCD and (ii) at least three clinic visits during the period of review. Data on gender, age as at last birthday, age at diagnosis of SCA, number of crises in the past 6 months, proteinuria, steady-state hemoglobin (Hb) concentration, frequency of chest infections, stroke, and oxygen saturation (SPO2) were collated. Severe anemia and low oxygen saturation were defined as Hb concentration <6 g/dl and oxygen saturation ≤94%, respectively.,
Data were analyzed using SPSS version 21 (IBM Corp., Released 2012, Armonk NY, USA). The distribution of continuous variables was assessed using the Shapiro–Wilk test. These were summarized as means ± standard deviation or medians and interquartile ranges (IQRs) (25th percentile value, 75th percentile value [IQRs]) and modes. Categorical data were summarized as percentages. Differences in the distribution of present age between binomial variables were determined using Mann–Whitney U (MWU)-tests. Level of statistical significance was set at P ≤ 0.05.
| Results|| |
A total of 109 patients' records were retrieved [Table 1].
The median (IQR) number of crises in the preceding 6 months was 0 (0, 3). The mean steady-state Hb concentration was 7.8 ± 1.3 g/dL. The median (IQR) SPO2 was 93% (90.0%, 97.5%). The prevalence of proteinuria, chest infections in the preceding 6 months, severe anemia, low oxygenation, and stroke were 4/42 (9.5%, 95% confidence interval [CI] – 1.3%, 21.1%), 5/77 (6.5%, 95% CI – 1.3%, 13.5%), 5/71 (7.0%, 95% CI – 2.8%, 11.9%), 33/56 (58.9%, 95% CI – 47.4%, 75.0), and 1/77 (1.3%, 95% CI – 0.0%, 3.9%), respectively.
Age correlates positively with number of crises in the last 6 months
The number of crises in the last 6 months increased with increasing age of the participants as depicted by a positive, weak, and statistically significant correlation; r = 0.317; P = 0.005.
Lower steady -state hemoglobin concentration and oxygen saturation are associated with older age
Older participants had lower steady-state Hb concentration and SPO2(≤94%). Spearman rank correlation analyses revealed negative correlations between age of participants and steady-state Hb concentration (rho = −0.180, P = 0.133) as well as age of participants and SPO2(rho = −0.059, P = 0.661), respectively. However, both relationships were not statistically significant [Table 2].
Severity of sickle cell disease is associated with older age
Series of MWU analyses revealed that participants with proteinuria, severe anemia, chest infections, and low SPO2 had higher mean rank ages compared to those without these conditions; (34.50 vs. 20.13, MWU = 24.000, P = 0.023), (57.20 vs. 34.39, MWU = 59.000, P = 0.014), (42.30 vs. 38.77, MWU = 163.500, P = 0.741), and (31.65 vs. 23.98, MWU = 483.500, P = 0.083), respectively [Table 3].
|Table 3: Distribution of present ages of participants across categories of complications|
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Stroke is associated with younger age
Patients with stroke were younger than those without stroke (8.00 vs. 39.41, MWU = 7.000, P = 0.208) [Table 3].
| Discussion|| |
Our study demonstrates that the median age of diagnosis of SCD in our setting is 1 year; however, the majority (>70%) were diagnosed between the ages of 1–5 years similar to the report of Mukinayi et al. in the Democratic Republic of the Congo, as well as Southwestern Nigeria, with higher ages in Senegal; 4 and 9.8 years, respectively., The practice of newborn screening in other countries has given an opportunity for early interventions such as penicillin V prophylaxis and parental education, resulting in a decrease in under-five mortality from 25% to <3%.,, With the absence of newborn screening in sub-Saharan Africa, the disease has contributed significantly (~5%) to the overall under-five mortality in the African continent.
This study demonstrates that older age is associated with an increased number of pain crises. Although pain crises are a hallmark of SCD and one of the foremost causes of hospital presentation in individuals with SCD,, what is not established in this study is whether the crises can be associated with either standard of care or other social interventions. Frequent painful episodes are a predictor of disease severity and mortality in the landmark Cooperative Study of SCD study. Despite improvements in the management of SCD, including the use of disease-modifying therapies such as chronic blood transfusion and hydroxyurea, frequent pain episodes still remain an independent risk factor for premature death in individuals with SCD. In low-resource settings, pain management is suboptimal, and the use of disease-modifying therapies is not frequent. Initially, pain management begins at home with the use of “over-the-counter” analgesics. However, adopting the stepladder approach to pain management by developing a pain management plan that enables patients to move up the analgesic ladder with increasing intensity of pain, the use of nonpharmacologic methods such as keeping warm and massage and knowing when to present to the hospital may help in ameliorating the intensity and frequency of painful crises in our setting.
Data from this study indicate that the older the patient, lower the Hb concentration, and lower the SPO2(≤94%). A cardinal manifestation of SCD is chronic hemolysis that occurs as a consequence of HbS that polymerizes when deoxygenated. The concomitant effect is that of chronic anemia and its related complications; strokes,, severe cognitive deficits, renal insufficiency, as well as left ventricular diastolic dysfunction. Severe anemia is an independent risk factor for death among children with SCD in low-resource settings, where the burden of the disease is highest. Furthermore, low Hb concentration has been associated with poor lung function that is progressive with a restrictive pattern exclusive of SCD patients, especially in low-resource settings., Although low SPO2(SPO2≤94%) is not a true reflection of hypoxia, in a low-resource setting, it could be the one valuable marker of hypoxia, and its demonstration with age in this study may be as a result of chronic anemia or other cardiopulmonary complications including pulmonary hypertension, acute chest syndrome, or as components of chronic sickle lung disease.,
Our study demonstrates that participants with proteinuria are older than those without proteinuria. This is an expected finding as proteinuria is a predictor of chronic kidney disease, which develops with repeated renal infarction that results from sickling, ingestion of nonsteroidal anti-inflammatory drugs, and renal infections among others.,, The prevalence of proteinuria in our study (9.5%) is lower than that reported among other SCD populations; 11% in South West Nigeria and 60.9% in Cameroon. The retrospective nature of our study may explain this lower prevalence as many cases of proteinuria may have been missed in the records. However, our finding of a positive correlation between age and proteinuria corroborates the reports of Arogundade; Geard; and Powars. The relative risk for mortality in patients with SCD and renal insufficiency has been demonstrated to be 1.42 (95% CI, 1.12–1.81; P = 0.02). Early detection of renal disease in SCD and institution of preventive strategies have been shown to decrease the progression of renal disease.
Our study indicates that stroke occurs more in younger individuals with SCD. SCA is one of the leading causes of stroke (overt and silent) in children. Approximately 11% of children with SCA have a 10% annual risk of developing stroke, if no primary stroke prevention intervention is done. In addition, in Sub-Saharan Africa, it is estimated that 11% of children will develop a stroke before their 20th birthday. Based on hospital data, the prevalence of stroke in Nigeria is about 6%–8%., The finding of stroke occurring in younger age in this study supports the need for instituting the evidence-based practices (EBPs) for primary stroke prevention, which involves screening for elevated transcranial Doppler (TCD) ultrasound velocity coupled with regular blood transfusion therapy for those with elevated velocities for at least 1 year or the use of hydroxyurea., This strategy has worked well in high-income countries by decreasing risk of stroke by 92%, but yet to be readily available in low-income regions like Nigeria as demonstrated by Galadanci et al. in 2014. This gap was largely attributed to the lack of trained TCD-ultrasonographers and shortages of TCD machines. However, strategies to address some of these gaps are being developed in sub-Saharan Africa, and with appropriate government support, stroke prevention may soon become incorporated into the usual care for eligible individuals with SCA.,
Being a retrospective study, our findings are unable to establish at what point these complications occur. This will be addressed in a longitudinal prospective study, where the incidence and evolutions of these parameters can be further characterized.
| Conclusion|| |
Older age is significantly associated with the severity of SCD. Age-targeted interventions guided by EBP may be important in slowing down disease progression and severity.
We thank Nurses Monica Shuaibu and Gloria Bahago who helped ensure that all patients attending the sickle cell clinic have their vitals taken and recorded. Our gratitude also goes to Miss Victoria Luka who entered all data into an Excel spreadsheet.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]