Problems of parental care for sickle cell children at the ''Gracia Fondation'' treatment center

 Corresponding author: Akondji Bainakofota Dieudonné Copyright © 2025 Author(s) retain the copyright of this article. This article is published under the terms of the Creative Commons Attribution Liscense 4.0. Problems of parental care for sickle cell children at the ''Gracia Fondation'' treatment center Buruga Bhanduni Emmanuel 1, Akondji Bainakofota Dieudonné 2, *, Lilemo losembe Jérôme 3 and Lokonda Nome Moïse 4 1 Department of Anesthesia, Higher Institute of Medical techniques of Kisangani, RD Congo. 2 Department of Private and Judicial Law, University of Kisangani, Faculty of Law, RD Congo. 3 Department of Pediatrics, Higher Institute of Medical techniques of Kisangani, RD Congo. 4 Department of Epidemiology, Higher Institute of Medical techniques of Basoko, RD Congo. World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 Publication history: Received on 30 March 2025; revised on 16 May 2025; accepted on 18 May 2025 Article DOI: https://doi.org/10.30574/wjarr.2025.26.2.1382 Abstract Introduction: Sickle cell disease is a complex disease, both historically and medically, at the collective and individual levels. It is the world's most common genetic disease, affecting around 50 million people, and its treatment is multidisciplinary, comprising 2 components: analgesic treatment and treatment of factors promoting sickle cell disease. The sole objective of this study is to identify the various problems related to the care of sickle cell children encountered by parents at the ''Gracia fondation'' Center. Methodology: This is a descriptive, cross-sectional and quantitative study whose population is made up of 20 parents of sickle-cell children followed up at the ''Gracia fondation'' center in the city of Kisangani from 15 /10 to 15 /11 2024. We used an interview technique to collect data, based on a pre-established questionnaire. Results: After tabulation and analysis of the data, the following result was observed: insufficient financial means and abandonment of children by their fathers are the main difficulties experienced by parents in caring for their sickle-cellaffected children, according to 100% and 80% of the respective subjects. Keywords: Problem; Care; Child; Sickle Cell; Parent. 1. Introduction According to the International Covenant on Economic, Social and Cultural Rights, States Parties to the Covenant must take “the necessary steps to ensure the reduction of the stillbirth-rate and of infant mortality, and the healthy development of the child” (Article 12). Sickle-cell anemia is a genetic disease that can be described as global, and which seriously affects children's health, even leading to their death. This transcontinental and trans-ethnic disease has been forgotten by the international community, yet it was recognized as a public health priority by the WHO in 2005 and by the UN in 2008 (Resolution A/63/237) [27]. Sickle cell anemia is a complex disease, both historically and medically, at the collective and individual levels. It is the most common genetic disease worldwide, affecting around 50 million people [1]. World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2721 Hemoglobin abnormalities caused by point mutations in a heme chain lead to red blood cell falciformation in stressful situations (infections, hypoxemia, acidosis), resulting in occlusion of blood capillaries and particularly painful bone infarctions, which explain the vaso-occlusive crises that bring patients to the emergency room [2]. The structural anomaly in hemoglobin is represented by a DNA point mutation characterized by the substitution of an amino acid, glutamic acid, by valine, in position 6 of the ϐ chain. The anomaly is located on chromosome 11. Genetic transmission is autosomal recessive. When only one chromosome carries the HbS gene (transmitted by the mother or father), the subject is said to be heterozygous; a healthy carrier. However, when both chromosomes are carriers of the gene (transmitted by the mother and father), the subject is said to be homozygous and the carrier is ill [4]. Depending on the genotype of the parents, the following combinations are possible: 1. AA children born to parents who are both AA, who are not ill and cannot transmit sickle cell disease to their offspring; 2. AA and AS children, if one parent is AA and the other AS. AS children are not sick, but can transmit sickle cell disease if they marry another AS or SS person; 3. AA, AS and/or SS children, if both parents are AS and AS; 4. SS children if both parents are SS and SS [1]. Until the 1990s, sickle-cell anemia was considered a fatal disease, with studies reporting a life expectancy of no more than 48 years even in developed countries. However, major advances in our understanding of the still highly complex pathophysiology of the disease, and the growing codification of its management, have made it possible to reduce mortality considerably by adhering to certain simple rules, and to transform sickle cell disease from a “fatal disease” into a “chronic disease” [3]. Sickle cell anemia is a genetic disease, inherited from both parents. It is the most widespread genetic disease in the world, affecting over 50 million people, with a predominance in Africa, India, the Mediterranean Basin and the Middle East. From these regions, it has spread to other countries as a result of population movements migration [5]. In France, sickle cell anemia is the most common genetic disease, affecting 20,000 patients by 2020, making it the leading genetic disease ahead of cystic fibrosis. However, over 50% of sickle cell patients live in the Île-de-France region [6]. Sub-Saharan Africa is particularly hard hit: one in every 65 newborns there has sickle cell disease. sickle cell disease. In Mali, the prevalence of sickle cell disease is estimated at 12%, including 1-3% for the homozygous form, making it a public health problem due to its frequency and multiple complications [7]. In Burundi, the prevalence of sickle cell disease is unknown. In 2016, the WHO estimated that it was at least 2% and that 10% to 40% of the Burundian population were carriers of the sickle cell gene [1]. Sickle cell disease presents as hemolytic anemia interspersed with vaso-occlusive crises, often complicated by severe bacterial infections. Pallor and subicterus, indicative of chronic hemolysis, are almost constant. The clinical signs of sickle cell disease are synonymous with complications. Sickle cell disease is manifested both by noisy acute manifestations and silent chronic complications: splenic sequestration crises, hyperhaemolytic crises, aplastic crises, osteo-articular vaso-occlusive crises or bone infarcts, extremity syndrome (hand-foot syndrome), cerebral crisis due to cerebrovascular accident (CVA), abdominal crisis or diffuse abdominal pain, priapism and clitorism, hypersplenism or splenomegaly, growth retardation and delayed puberty, severe infectious episodes, etc. [26]. In the Democratic Republic of Congo, sickle-cell anemia is a serious chronic disease responsible for early mortality and reducing personal, social and family quality of life, and family life. Patients may be experiencing great social difficulties, family conflicts, professional problems (precarious work and sometimes unsuitability for the job and repeated absences) or school problems (repeated absences, misunderstanding of classmates and the school system), and are therefore likely to have a greater number of attacks [8]. The main factors triggering vaso-occlusive attacks are cold, altitude (air travel), stress (school or university exams, social life, etc.), infections and dehydration. Sickle cell vaso-occlusive crisis is not usually accompanied by a major drop in hemoglobin levels. The occurrence of acute anemia in the course of sickle cell disease should raise the possibility of central complications such as bone marrow necrosis, parvovirus B19 infection and acute folate deficiency, or peripheral complications such as acute splenic sequestration and immunological transfusion accidents. This crisis is the most frequent acute complication of sickle cell disease, with an estimated prevalence of 95%. The painful vaso-occlusive crisis may sometimes herald another, more serious complication, or conceal other differential diagnoses [9]. World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2722 Spontaneous mortality from sickle cell stroke can be as high as 20%, reflecting the extreme severity of this complication. The relative risk of developing a stroke in sickle cell disease is much higher in children than in adults, with a predilection for strokes between the ages of 2 and 10, and a peak after the age of 30 [10]. Therapeutic management is multidisciplinary, with internists, emergency physicians and resuscitators all involved in the management of sickle cell complications. It includes symptomatic treatment of vaso-occlusive crises, transfusion, or even exchange transfusion, and organic support depending on the severity of the crisis. Patients with severe complications are admitted to intensive care [7]. The treatment of a simple vaso-occlusive crisis comprises 2 components: analgesic treatment and treatment of factors favoring falciformation. The vaso-occlusive crisis is the cause of very intense bone pain at a level superior to that of a bone fracture. The therapeutic response must therefore be at the same level, and in all cases requires major analgesics such as morphine [11]. According to the African Society of Pediatrics (2018), in sickle cell disease, there are only palliative treatments. The bulk of treatment consists of managing the symptoms resulting from crises. Sickle cell patients need rest, good oxygen therapy in the case of thoracic syndromes, good hydration in hot weather, and analgesics in the case of pain. Emergency transfusion remains the only treatment for profound anemia. Folic acid is given for life to facilitate cell renewal [12]. Around 40% of adult sickle-cell patients present with intermittent or acute priapism at some stage in the course of the disease. Attacks are most often nocturnal and favoured by the presence of sleep apnoea syndrome. The major functional risk is definitive fibrosis of the corpus cavernosum. Systematic anamnestic screening and management of intermittent priapism are essential to prevent the onset of acute priapism, often with permanent sequelae [11]. Any sickle cell complication should a priori be treated in hospital, due to the unpredictable risk of aggravation or association with other conditions that may have been masked at the outset: any bone pain crisis that is abnormal in terms of duration, intensity, resistance to usual initial treatments, fragility of the patient's underlying condition, or the impossibility of providing adequate analgesic treatment and hydration on an outpatient basis, should be referred to hospital. Furthermore, all extra-osseous complications are systematic indications for hospitalization, with hyperthermia leading the way, as well as thoracoabdominal pain and vaso-occlusive syndromes, and severe anemia. The efficacy of curative management of acute complications of sickle cell disease depends on the quality of initial management of vital distress and proper diagnostic evaluation of patients [13]. The criteria for returning home during a vaso-occlusive crisis are absence of fever, absence of chest pain, Respiratory Rate below 20 movements per minute, no morphine injections for more than 8 hours [14]. Sickle-cell anemia can lead to the death of the child, especially when the disease is not detected and treated from birth, as is the case in many African and developing countries such as the Democratic Republic of Congo. In most countries where sickle cell disease is a major public health problem, there are no national programs to combat it. The basic structures needed to care for patients are generally lacking. Systematic screening for sickle cell disease is a simple blood test, yet this is not common practice. In developing countries, the disease is usually diagnosed only when a serious complication occurs. As a result, over 50% of children with the most severe form of the disease die before the age of five, most often from infection or severe anemia. Early detection of the disease is essential, so that couples can benefit from information, education and appropriate care [15]. Effective ways of improving the health of sickle-cell children include: 1. standard treatments: antibiotics, vaccines, folates; 2. treatment of vaso-occlusive crises: analgesics and oxygen therapy; 3. prevention of factors triggering crises (cold, altitude, infections, dehydration); 4. Take folate-rich medication for anemia; 5. Vaccination to prevent pneumococcal and meningococcal infections; 6. Blood transfusion in cases of severe anemia or infection; 7. Blood transfusion to reduce the proportion of hemoglobin S [16]. Sickle cell disease is one of the most common diseases in the Democratic Republic of Congo, as recent epidemiological data demonstrate. They show that 2% of newborns are homozygous for hemoglobin S, and that an estimated 40,000 sickle-cell children are born each year, half of whom die before the age of 5... although early and appropriate treatment enables patients to control their symptoms and avoid serious crises [17]. Sickle-cell anemia is linked to the representation of death in the African context. Families live in uncertainty in the face of this tragedy. Faced with this situation, sickle cell disease remains hidden from the family circle; only the parents World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2723 manage the situation, even during the child's vaso-occlusive crisis. Given the sensitivity of the information we were seeking, a quantitative approach was used to highlight the problems associated with the management of sickle cell disease in our study environment. This approach is facilitated by the community diagnosis process, which enables a community to identify its own problems and needs, and the resources available to address them. In view of the above, we pose the following research question: What are the various problems related to the care of sickle-cell children encountered by parents at the ''Gracia fondation'' Center? 1.1. Objective Our sole objective is to identify the various problems encountered by parents of sickle-cell-affected children at the ''Gracia fondation'' center. 1.2. Aim of the study With a view to improving the health of sickle-cell-affected children in the Democratic Republic of the Congo, this study has two interests: Scientific and educational: this study is intended to make a scientific contribution in terms of new knowledge, as a library that can help researchers in their future studies. On a practical level: this research is intended to be a frame of reference, raising awareness both for administrative and health authorities to think about improving the care of sickle-cell children, and for parents to bring their children to specialized centers for proper care. The results of this study will enable us to formulate a number of concrete actions (possible solutions) that could help decision-makers to draw up and implement national programs to combat sickle cell disease throughout the Republic. 2. Methodology 2.1. Type of study This is a descriptive, cross-sectional and quantitative study on the problem of caring for sickle cell children encountered by parents at the “Gracia fondation” treatment center in the town of Kisangani, in the Democratic Republic of the Congo, from 15/10 to 15/11 2024. 2.2. Study population The population of this study is composed of parents of children with sickle cell disease followed at the ''Gracia fondation'' Sickle Cell Treatment Center in the city of Kisangani during the period from 15 /10 to 15 /11 2024. Given the importance of this study, our sample included 20 subjects representing the parents of children who were followed up during this period. 2.3. Data collection methods and techniques In view of all these considerations, and taking into account the nature and purpose of our study, as well as the different levels of education of parents of children with sickle cell disease, we opted for an interrogatory method based on a questionnaire that we developed and administered or submitted to the respondents, in the form of a structured interview to obtain information on the difficulties associated with caring for children with sickle cell disease. After finishing with the administration of the ''Gracia fondation'' center, we were accompanied by a nurse to collect data from the parents. Due to time constraints, data collection took place every Tuesday and Friday from 10am to 2pm. 2.4. Data processing The data collected were analysed and encoded using Microsoft Office Excel 2013 and SPSS 20.1. The results obtained were presented in the form of tables. Data analysis was made possible by calculating frequencies and percentages. World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2724 3. Results 3.1. Socio-demographic data 3.1.1. Gender Table 1 Gender distribution of study subjects Gender f % Female 13 65 Male 7 35 Total 20 100 Analysis of Table I shows that 65% of study subjects were female, compared with 35% male. 3.1.2. Age Table 2 Age distribution of study subjects Age range in year f % 20 - 29 5 25 30 - 39 8 40 40 - 49 4 20 50 and over 3 15 Total 20 100 Analysis of Table II shows that the study population is made up more of subjects in the 20 to 39 age bracket, i.e. 61.1%, while those aged 50 and over represent only 15% of cases. 3.1.3. Level of education Table 3 Distribution of respondents by level of education. Level of education f % University 8 40 Secondary 12 60 Total 20 100 For the “level of education” variable, analysis of Table III shows that the majority of study subjects were at secondary school level (60%), while those at university level accounted for only 40%. 3.1.4. Marital status Table 4 Distribution of respondents by marital status. Marital status f % Married 14 70 Single 6 30 Total 20 100 This study revealed that 14 subjects, or 70%, were married, compared with 30% who were single. World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2725 3.1.5. Socio-economic level Table 5 Distribution of respondents according to socio-economic level. Socio-economic level f % Low 11 55 Medium 7 35 High 2 10 Total 20 100 The data in Table V show that 55% of the subjects in the study had a low socio-economic standard of living, while the socio-economically average represented 35% of cases. On the other hand, two subjects (10%) were of high socioeconomic status. 3.2. Variables studied 3.2.1. Circumstances of discovery of sickle cell disease in children Table 6 Distribution of subjects according to circumstances of discovery of sickle cell disease Circumstance of discovery of sickle cell disease f % After child's second blood transfusion 8 40 Child's sickly state 6 30 Following pronounced pallor 4 20 Following growth retardation 2 10 Total 20 100 In Kisangani, episodes of blood transfusion and a sickly child are the circumstances in which sickle cell disease is discovered in 40% and 30% of cases respectively. 3.2.2. Number of vaso-occlusive crises per year Table 7 Distribution of subjects by number of vaso-occlusive crises per year Number of attacks per year f % 1 to 4 times 2 10 5 to 8 times 5 25 Several times 13 65 Total 20 100 The data in Table VI show that 65% of subjects report several vaso-occlusive attacks per year, while those who have 1 to 4 attacks represent 10% of cases. 3.2.3. Cause of vaso-occlusive attacks Table 8 Distribution of subjects by number of vaso-occlusive attacks per year n=20 Cause of attacks f % Malaria, diarrhea and typhoid fever 10 50 World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2726 Cold and heat 7 35 Pregnancy if adult 4 20 Undecided 2 10 The data in Table VIII show that infections (malaria, diarrhoea and typhoid fever) and climate (cold and heat) are the main causes of vaso-occlusion in 50% and 35% of subjects respectively. 3.2.4. Management of children with sickle cell disease Table 9 Distribution of subjects by elements of care for children with sickle cell disease n=20 Management of sickle-cell anemia f % Pain control [treatment with analgesics (morphine)] 20 100 Transfusion in case of anemia 17 85 Ensuring good nutrition 15 75 Infection control (treatment with antibiotics) 14 70 Application of lifestyle hygiene measures 9 45 Hydration and oxygen therapy 7 35 Treatment with hydroxyurea 5 25 Treatment of priapism 5 25 Pain control, transfusion in case of anemia, good nutrition and infection control are the main components of sickle cell case management, according to 100%, 85%, 75% and 70% of cases. 3.2.5. Difficulties experienced by parents in caring for sickle-cell-affected children Table 10 Distribution of subjects according to the different difficulties experienced by parents in caring for sickle-cell children n=20 Difficulties experienced by parents f % Parents' lack of financial means 20 100 Abandonment of children by fathers 16 80 Socio-cultural problems 4 20 Insufficient financial means and abandonment of children by their fathers are the main difficulties experienced by parents in caring for their sickle-cell-affected children, according to 100% and 80% of the respective subjects. 3.2.6. Proposed solutions to improve sickle cell disease management Table 11 Distribution of subjects according to proposed solutions to improve the management of sickle cell disease n=20 Proposed solutions for improving sickle cell disease f % Government support for children with sickle cell disease 20 100 Separation of A/S and A/S or A/S and S/S couples 13 65 Sterilization of couples to avoid procreation 6 30 World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2727 Government treatment of sickle-cell children by setting up national sickle-cell treatment centers throughout the Republic (100% of cases) and separation of A/S and A/S or A/S and S/S couples (65% of cases) are the solutions to improve care. However, 30% of subjects reported sterilization of couples to avoid procreation of sickle cell children. 4. Discussion and comments 4.1. Gender Analysis of the study data shows that 65% of the subjects were female, compared with 35% male. This result can be explained by the fact that it is women, mothers in most cases, who care for and accompany children to hospital for treatment. They are the primary family nurses, while the men are the ones who provide for their families. 4.2. Age range The study indicates that the study population is made up more of subjects in the 20 to 39 age bracket, i.e. 61.1%, while those aged 50 and over represent only 15% of cases. Given that the study population is made up of parents of sickle-cell-affected children, the 20 to 39 age bracket is therefore the age of majority. Both men and women can marry, thus founding their homes. These are the ages with which young people want to identify and confirm themselves in life. 4.3. Level of education For the “level of education” variable, the majority of subjects in the study were at secondary school level (60%), while those at university level accounted for only 40%. Contrary to the study by Malumbu L, in her study of medical care for women in prison, the case of the Kisangani central prison, reported that the majority of inmates had lower or primary education, i.e. 33.3% had secondary education and 30.3% were illiterate. Those with secondary education accounted for 27.3% of cases [18]. In the Democratic Republic of Congo, despite the absence of a job market, the Congolese have become aware of the need to educate their children, based on Unicef's awareness-raising slogan: “All children, girls and boys at school”. As a result, we have a considerable number of high-school students, most of whom are limited to the secondary level for a variety of reasons: early pregnancy, lack of resources, voluntary drop-out, etc. 4.4. Marital status This study presents the results according to which 14 study subjects, i.e. 70% were married versus 30% single. These results clearly show that motherhood is an adventure for married people, although some fathers abandon their sickle-cell children. In principle, you can only give birth when you live as a couple. For this reason, more than half of our study population were married. 4.5. Socio-economic level The study data indicate that 55% of the subjects in the study had a low socio-economic standard of living, while those of socio-economic means represented 35% of cases. On the other hand, two subjects (10%) were of high socio-economic status. The African Society of Pediatrics, in its Guide to the management of sickle-cell disease in Africa, has shown that sicklecell disease mainly affects impoverished populations in countries with limited health budgets [19]. In our opinion, many of the advances made in the management of sickle-cell disease are not yet available in the areas where it is most prevalent, but are available to patients in rich countries or to rich patients in poor countries. These advances do exist. We need to make it accessible to as many people as possible. 4.6. Circumstances of discovery of sickle cell disease in children In Kisangani, episodes of blood transfusion in children and sick children are the circumstances in which sickle cell disease is discovered in 40% and 30% of cases respectively. World Journal of Advanced Research and Reviews, 2025, 26(02), 2720-2732 2728 In the Democratic Republic of Congo, people have no culture of medical analysis. They wait for the onset and severity of the disease before going to hospital for treatment. Over 60% of the population self-medicate. As a result, chronic illnesses such as sickle-cell anemia, cancer, HIV/AIDS, etc., in both children and adults, are discovered late. 4.7. Number of vaso-occlusive attacks per year The data from this research indicated that 65% of subjects report several vaso-occlusive attacks per year, while those who have 1 to 4 attacks represent 10% of cases. A study of sickle cell disease in children in Beni and Butembo, Democratic Republic of Congo, reported that 197 (1.5%) of the patients studied were sickle cell patients. There were 163 cases of severe vaso-occlusive crises (82.7% of sickle cell patients), including 26 not included in this study. A total of 137 cases were included. The mean number of patients hospitalized per year was 15.7 ± 16.5, with extremes of 22 and 30 [19]. In our opinion, the onset of vaso-occlusive crises in sickle-cell patients depends on several factors: hypoxia, cold, fever, dehydration, stress, intake of stimulants, infections, etc. Failure to take these factors into account can lead to vasoocclusive crises as many times a year. 4.8. Causes of vaso-occlusive attacks This study shows that infections (malaria, diarrhea and typhoid fever) and climate (cold and heat) are the main causes of vaso-occlusive attacks for 50% and 35% of the respective subjects. According to HAS, it is recommended to explain to parents the factors favoring painful vaso-occlusive attacks: • hypoxia: excessive and unusual exertion, altitude (from around 1,500 m), tight clothing, etc. ; • cooling: cold-water baths, etc. ; • fever; • dehydration: vomiting, diarrhoea, etc. ; • stress; • intake of stimulants, alcohol, tobacco or illicit drugs (more so in than in children) [25]. The vaso-occlusive crisis is the most frequent; it is provoked by the obstruction of microcirculations by sickle cell red blood cells, responsible for an acute hyperalgesic ischemic lesion of the irrigated organ (monoor multifocal osteoarticular crises, abdominal crises, thoracic syndrome, etc.) and the resulting pain. 4.9. Elements of care for children with sickle cell disease Pain control, transfusion in case of anemia, good nutrition and infection control are the main components of sickle cell case management, according to 100%, 85%, 75% and 70% of cases. According to Keza G.K, Diallo D et al (2023), medical management of sickle cell disease consists of monitoring anemia, preventing painful attacks and infections, and detecting complications early to treat them as quickly as possible. Patients are always provided with a prescription for pain medication [20]. Therapeutic management of sickle cell disease is multidisciplinary, including internists, emergency physicians and resuscitators. It includes symptomatic treatment of vaso-occlusive crises, transfusion, or even exchange transfusion, and organic supplements depending on the severity of the crisis. Patients with severe complications are admitted to intensive care [21]. According to Brillant Damus (2022), the treatment of a simple vaso-occlusive crisis comprises 2 components: analgesic treatment and treatment of factors favoring falciformation. The vaso-occlusive crisis is the cause of very intense bone pain at a level superior to that of a bone fracture. The therapeutic response must therefore be at the same level, and in all cases requires major analgesics such as morphine [22]. According to the African Society of Pediatrics (2018), there are only palliative treatments in sickle cell disease. The essence of treatment consists in managing the symptoms resulting from crises. The sickle cell patient needs rest, good oxygen therapy in the case of thoracic syndromes, good hydration in hot weather, and analgesics in the case of pain.