Reasons for Consultation of Sickle Cell Patients at the Center for Mixed Medicine and SS Anemia ()
1. Introduction
Sickle cell disease is an autosomal recessive genetic disease characterized by a point mutation of the globin b chain in position 6 of the GAG-GTG codon, the replacement of valine by glutamic acid which induces the synthesis of hemoglobin s responsible for vaso-occlusive crises and chronic hemolysis [1].
According to the WHO, nearly 5% of the population carries a gene responsible for a hemoglobin abnormality. Africa is an original home. Recent estimates predict more than 400,000 births with Major Sickle Cell Syndrome (MDS) in 2050 [2], 75% of which will be recorded in sub-Saharan Africa and India [3].
The Democratic Republic of Congo (DRC) is the third most affected country in the world by sickle cell disease after India and Nigeria, respectively the first and second most affected countries in the world, and the second country in Africa after Nigeria. It is estimated that 20 million Congolese (i.e. 25% to 30% of the population) are carriers of the sickle cell gene. The prevalence of the HBss is 3% and represents a heavy burden for those affected and their families. In the meantime, around 40,000 newborns are born with sickle cell disease in the DRC [4] [5] [6].
In Kinshasa, sickle cell patients are treated in specialized centers such as the Center for Mixed Medicine and SS Anemia (CMMASS), which is a public utility center managed by the Ministry of Health and Hygiene. However, there is not data on the frequent reasons of consultation of the patients in the center. Hence the present study was carried out on the files of homozygous sickle cell patients regularly followed at CMMASS-Kinshasa, with the objective of determining the reasons for consultation of these patients.
2. Methods
Setting and type of study
This study was conducted at CMMASS, which is an institution specializing in the management of sickle cell disease in Kinshasa, DRC. It is a retrospective study carried out on the files of patients who came for consultation in the period from January 1, 2018 to December 31, 2020.
Study population
It was made up of the records of sickle cell patients aged 18 and over who came for consultation at the CMMASS during the period in question.
Inclusion criteria
Any well-kept records of sickle cell patients over the age of 18 with a documented electrophoresis result who consulted during the study period were considered for work.
Parameter of interest
The parameters of interest in this study were the different reasons for consultation of sickle cell patients (osteo-articular pain, abdominal pain, chest pain, pallor, dyspnea, jaundice, fever, cough and others) at the CMMASS during the period.
3. Results
In the period from January 1, 2018 to December 31, 2020, according to patients’ consultation files at the center, 2463 SS homozygous sickle cell patients had been seen in consultation at the CMMASS. One thousand three hundred and fifty (1350) patients (54.8%) were female while 1113 (45.2%) were male; thus giving a sex ratio of 1.21 in favor of women. The most represented age group is that of 18 to 25 years with 1148 patients (46.6%); followed by that of 26 to 33 years old with 719 patients (29.2%), from 34 to 41 years old with 586 patients (23.8%) and over 42 years old with 10 patients (0.4%). Table 1 presents the data mentioned above.
The most represented reasons for consultation, in the patients’ fines, in the series were grouped according to the syndromes: pain reasons with osteo-articular pain for 1521 patients (61.75%), infectious reasons with fever for 603 patients (24.48%), hemolytic patterns with pallor for 310 patients (12.59%), menongo-encephalic patterns with headaches for 192 patients (7.8%), and other patterns such as cough for 63 patients (2.56%). Table 2 presents the data mentioned above in more detail.
4. Discussion
The objective of this work was to determine the reasons for consultation of sickle cell patients at the CMMASS in the period from January 1, 2018 to December 31, 2020. According to the inclusion criteria, 2463 files of sickle cell patients had been selected for the work at the CMMASS.
One thousand three hundred and fifty patients (54.8%) were female while
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Table 1. Gender and age of the population.
1113 (45.2%) were male; thus giving a sex ratio of 1.21 in favor of women. This slight female predominance in the present study is also found in various studies in the DRC [7] [8] [9]. On the other hand, other authors find no predominance between the two sexes [10] [11]. These differences would be related to the demographic data of different countries because the transmission of sickle cell disease is not linked to sex [12] [13]. The most represented age group is that of 18 to 25 years with 1148 patients (46.6%); followed by that of 26 to 33 years old with 719 patients (29.2%), from 34 to 41 years old with 586 patients (23.8%) and over 42 years old with 10 patients (0.4%).
The most represented age group is that of 18 to 25 years with 1148 patients (46.6%); followed by that of 26 to 33 years old with 719 patients (29.2%), from 34 to 41 years old with 586 patients (23.8%) and over 42 years old with 10 patients (0.4%). In this series, the slight predominance of 46.6% compared to other age groups could be explained by the better understanding of the pathology by older subjects who better apply preventive measures and are therefore less affected by certain acute complications.. This argument corroborates the article by Chetcha Chemegni B et al. which reports a predominance in the age group between 15 - 24 years or 60% in their series [14] [15]. Even more, it is explained by the life expectancy of sickle cell patients. Indeed, in the United States, their life expectancy would be reduced by 25 to 30 years compared to the black American population in general [16]. In 1991, Latoudji et al. found in Benin a 30-year survival rate of 18.66% in homozygous sickle cell subjects [17].
The most represented reasons for consultation in the series were grouped according to the syndromes: pain reasons with osteo-articular pain for 1521 patients (61.75%), infectious reasons with fever for 603 patients (24.48%), hemolytic patterns with pallor for 310 patients (12.59%), menongo-encephalic patterns with headaches for 192 patients (7.8%), and other patterns such as cough for 63 patients (2.56%). Osteoarticular pain was the most common reason for consultation with 61.75%. Our result is superior to that of Ibrahim Keita [18], who found osteoarticular pain, pallor and jaundice with respectively 41.7%, 37.5% and 17.5% of cases.
5. Conclusion
This study reveals that osteoarticular pain was the most dominant reason for consultation in homozygous sickle cell patients, followed by fever and pallor at the CMMASS during the period from January 1, 2018 to December 31, 2020.