**2. Theoretical consideration**

#### **2.1 The sickle cell anemia, a serious and deadly genetic disease**

Sickle cell anemia is the most prevalent genetic disease in the world, with approximately 500 million individuals with sickle cell traits and 50 million individuals with the disorder itself worldwide [3]. Originally spread across malariaendemic areas such as sub-Saharan African countries, historical migrations linked to the slave trade and the recent acceleration of migration flows have gradually changed its distribution worldwide [4]. It is found in almost all countries with large populations from Africa and regions around the Mediterranean. In France, for example, it is a rare disease, but nonetheless the most common genetic disease with a prevalence of one child per 1900 births [5].

With a prevalence rate of at least 2% in the general population and an estimated mortality rate of more than 70% among children under the age of 5 [6], the African continent is most affected by sickle cell anemia. Cameroon is one of the most affected countries, with a prevalence of 8.34% in the general population [7]. In sub-Saharan Africa, the unavailability of bone marrow transplantation—the only effective treatment for seizures—increases the risk of death in children under 5 years old [8].

The disease is an autosomal recessive pathology transmitted to the child by both parents. The presence of abnormal hemoglobin in the blood causes a deficiency in the supply of oxygen to various organs in the body by the red blood cells that have reduced life cycles. This leads to anemia and chronic, unpredictable pain [4, 9] that the patient identifies [1], resulting in multiple expensive hospitalizations and care organized by their parents [10] both in the hospital and among traditional healers.

#### **2.2 The sickle cell anemia, a persecution figure of the family group**

In the sub-Saharan African cultural context, sickle cell anemia is thought of as an "evil" that can attack any member of the family, even after the death of the patient [11]. The sick child is immersed in a society structured by traditional taboos, rituals and attitudes of which women are custodians [12]. The illness or handicap of the child is inscribed in this cultural structure, which gives it meaning and produces effects.

Sickle cell anemia is thought to be a manifestation of the possession of the sick child and his family by an evil spirit or bewitchment by a wizard [13]. It can also be perceived as a request of the ancestors to repair a transgression of an ancestral norm, addressed to the patient's family [14]. Generally, the mother is designated as responsible for this transgression and the overprotection of the sick child, by her and by the family members, constitutes a defense allowing them to feel guilt-free and to put the child in the family's history [14] alongside his brothers and sisters.

#### **2.3 The specificities of the medical care of sickle cell anemia in Cameroon**

The hospital is a place regularly frequented by sick children and their families. Described as the disease of hospitals in several African countries [15], sickle cell

**41**

*Sickle Cell Anemia, Representations and Care: Experience of a Brother of a Sick Child…*

anemia makes hospital services a second home for the patient and their families around them. The frequency of hospitalizations in Cameroon is estimated to be between three and four hospitalizations per month in children under five, between five and seven hospitalizations per month in adolescents and two hospitalizations per month in adults [16]. The reasons for these hospitalizations are sometimes varied in one subject and identical in others. In general, sick children are regularly hospitalized for pain attacks, severe anemia and/or chronic complications (stroke and heart attacks). Stroke is also a cause of hospitalization for sickle cell patients. For the latter, the probabilities of having a stroke before the ages of 20, 30 and 45 are, respectively, 11, 15 and 24% [17]. In Cameroon, the prevalence of stroke is

The medical management of patients has undergone an important evolution within the past 20 years due to the intensification of available treatments for children at risk of severe complications [19]. These treatments consist mainly of yoglycurea, transfusion programs and family transplants. Indeed, yoglycan significantly reduces the frequency of onset of occlusive seizures, acute thoracic syndrome and the degree of hemolysis. The establishment of transfusion programs for children detected as at risk of stroke by transcranial Doppler has significantly

Allogeneic transplantation is currently the only treatment that can cure approxi-

Sickle cell anemia is essentially a disease of the south whose treatment is in the north. This caricature seems more appropriate to address the lethal nature of this pathology in sub-Saharan African countries, including Cameroon, where it remains a chronic and orphan disease [21] because of the absence of hematopoietic stem cell allograft and the gene editing systems, the only treatments available against this disease [8, 9, 22]. Therefore, the therapeutic approach of the disease remains curative and focuses on the nature of the crises. The patient is supported in relation to the type of crisis he manifests. The primary purpose of medicine, in this case, is to alleviate the suffering of the patient by managing the symptoms. Crisis treatment incorporates several therapeutic products and postures. Severe attacks (mild pain, modeled fever) are often treated at home in collaboration with a doctor or by selfmedication. The patient is advised to rest, drink abundantly and is given an analgesic treatment including acetylsalicylic acid, paracetamol or Di-Antalvic. In case of severe attacks involving localized or generalized pain, rest and rehydration are recommended. If these measures do not calm the crisis, a transfusion is performed. The management of anemia, meanwhile, requires a transfusion of phenotyped erythrocyte concentrates, leukocyte depleted and filtered. The intervention in cases of severe pain attacks is based on the transfusion and/or hydroxyurea of the patient. Hydroxycarbamide is the only current attenuator therapy used in the management of vaso-occlusive seizures and severe anemias. The price of these products, combined with that of many hospitalizations, is relatively high for most Cameroonian

Parents of sick children, supported by the members of their extended families,

are frequently searching for ways to relieve their suffering. With the advent of globalization, they resort to several therapies. The therapeutic route is, in this sense, a sort of mosaic between traditional therapies and imported Western and messianic therapies. In this way, the traditional therapist, the doctor and the Imam, pastor or

mately 95% of children with sickle cell anemia [8, 20]. This treatment involves grafting from a brother or sister of the patient, based on their genetic compatibility

with hematopoietic stem cells located in the bone marrow of the patient.

families who generally do not have social security [16].

**2.4 Balancing between traditional care and medical care**

*DOI: http://dx.doi.org/10.5772/intechopen.90995*

6–7% in patients aged 7 months–35 years [18].

reduced this risk from 11 to 2% [19].

*Sickle Cell Anemia, Representations and Care: Experience of a Brother of a Sick Child… DOI: http://dx.doi.org/10.5772/intechopen.90995*

anemia makes hospital services a second home for the patient and their families around them. The frequency of hospitalizations in Cameroon is estimated to be between three and four hospitalizations per month in children under five, between five and seven hospitalizations per month in adolescents and two hospitalizations per month in adults [16]. The reasons for these hospitalizations are sometimes varied in one subject and identical in others. In general, sick children are regularly hospitalized for pain attacks, severe anemia and/or chronic complications (stroke and heart attacks). Stroke is also a cause of hospitalization for sickle cell patients. For the latter, the probabilities of having a stroke before the ages of 20, 30 and 45 are, respectively, 11, 15 and 24% [17]. In Cameroon, the prevalence of stroke is 6–7% in patients aged 7 months–35 years [18].

The medical management of patients has undergone an important evolution within the past 20 years due to the intensification of available treatments for children at risk of severe complications [19]. These treatments consist mainly of yoglycurea, transfusion programs and family transplants. Indeed, yoglycan significantly reduces the frequency of onset of occlusive seizures, acute thoracic syndrome and the degree of hemolysis. The establishment of transfusion programs for children detected as at risk of stroke by transcranial Doppler has significantly reduced this risk from 11 to 2% [19].

Allogeneic transplantation is currently the only treatment that can cure approximately 95% of children with sickle cell anemia [8, 20]. This treatment involves grafting from a brother or sister of the patient, based on their genetic compatibility with hematopoietic stem cells located in the bone marrow of the patient.

Sickle cell anemia is essentially a disease of the south whose treatment is in the north. This caricature seems more appropriate to address the lethal nature of this pathology in sub-Saharan African countries, including Cameroon, where it remains a chronic and orphan disease [21] because of the absence of hematopoietic stem cell allograft and the gene editing systems, the only treatments available against this disease [8, 9, 22]. Therefore, the therapeutic approach of the disease remains curative and focuses on the nature of the crises. The patient is supported in relation to the type of crisis he manifests. The primary purpose of medicine, in this case, is to alleviate the suffering of the patient by managing the symptoms. Crisis treatment incorporates several therapeutic products and postures. Severe attacks (mild pain, modeled fever) are often treated at home in collaboration with a doctor or by selfmedication. The patient is advised to rest, drink abundantly and is given an analgesic treatment including acetylsalicylic acid, paracetamol or Di-Antalvic. In case of severe attacks involving localized or generalized pain, rest and rehydration are recommended. If these measures do not calm the crisis, a transfusion is performed.

The management of anemia, meanwhile, requires a transfusion of phenotyped erythrocyte concentrates, leukocyte depleted and filtered. The intervention in cases of severe pain attacks is based on the transfusion and/or hydroxyurea of the patient. Hydroxycarbamide is the only current attenuator therapy used in the management of vaso-occlusive seizures and severe anemias. The price of these products, combined with that of many hospitalizations, is relatively high for most Cameroonian families who generally do not have social security [16].

#### **2.4 Balancing between traditional care and medical care**

Parents of sick children, supported by the members of their extended families, are frequently searching for ways to relieve their suffering. With the advent of globalization, they resort to several therapies. The therapeutic route is, in this sense, a sort of mosaic between traditional therapies and imported Western and messianic therapies. In this way, the traditional therapist, the doctor and the Imam, pastor or

*Human Blood Group Systems and Haemoglobinopathies*

with sickle cell anemia.

**2. Theoretical consideration**

a prevalence of one child per 1900 births [5].

Cameroonian family and tries to understand its effects on a brother of a sick child. It shows the complexity of the experiences of children growing up with a sick sibling and its close interweaving with the family experiences as they care for the child. The objective is to build on this knowledge to open up the design of care devices that better take into account the specificity of the experiences of siblings of children

Sickle cell anemia is the most prevalent genetic disease in the world, with approximately 500 million individuals with sickle cell traits and 50 million individuals with the disorder itself worldwide [3]. Originally spread across malariaendemic areas such as sub-Saharan African countries, historical migrations linked to the slave trade and the recent acceleration of migration flows have gradually changed its distribution worldwide [4]. It is found in almost all countries with large populations from Africa and regions around the Mediterranean. In France, for example, it is a rare disease, but nonetheless the most common genetic disease with

With a prevalence rate of at least 2% in the general population and an estimated mortality rate of more than 70% among children under the age of 5 [6], the African continent is most affected by sickle cell anemia. Cameroon is one of the most affected countries, with a prevalence of 8.34% in the general population [7]. In sub-Saharan Africa, the unavailability of bone marrow transplantation—the only effective treatment for seizures—increases the risk of death in children under 5 years old [8].

The disease is an autosomal recessive pathology transmitted to the child by both parents. The presence of abnormal hemoglobin in the blood causes a deficiency in the supply of oxygen to various organs in the body by the red blood cells that have reduced life cycles. This leads to anemia and chronic, unpredictable pain [4, 9] that the patient identifies [1], resulting in multiple expensive hospitalizations and care organized by their parents [10] both in the hospital and among traditional healers.

In the sub-Saharan African cultural context, sickle cell anemia is thought of as an "evil" that can attack any member of the family, even after the death of the patient [11]. The sick child is immersed in a society structured by traditional taboos, rituals and attitudes of which women are custodians [12]. The illness or handicap of the child is inscribed in this cultural structure, which gives it meaning and produces effects. Sickle cell anemia is thought to be a manifestation of the possession of the sick child and his family by an evil spirit or bewitchment by a wizard [13]. It can also be perceived as a request of the ancestors to repair a transgression of an ancestral norm, addressed to the patient's family [14]. Generally, the mother is designated as responsible for this transgression and the overprotection of the sick child, by her and by the family members, constitutes a defense allowing them to feel guilt-free and to put the child in the family's history [14] alongside his brothers and sisters.

**2.2 The sickle cell anemia, a persecution figure of the family group**

**2.3 The specificities of the medical care of sickle cell anemia in Cameroon**

The hospital is a place regularly frequented by sick children and their families. Described as the disease of hospitals in several African countries [15], sickle cell

**2.1 The sickle cell anemia, a serious and deadly genetic disease**

**40**

priest are consulted at the same time. This system of "round care" [23] is one that perfectly summarizes the therapeutic path of most African families in sub-Saharan Africa. At each source, they seek the healing of a specific aspect of the child's suffering. The quests for meaning of the child's illness, ancestral protection and reconciliation with the ancestors lead parents to traditional healers. With the priest, the pastor or the Imam, families seek the divine therapy to ensure protection from God. They usually do this because they yearn for the healing of the child under the mercy of God. With the medical doctor, they seek the somatic healing of the child. These families are part of a permanent search for identity reconstruction [24, 25]. The identities of Africans remain very complex as they continually attempt to find their identities, following the effects of globalization [14, 26].

The traditional treatment of sickle cell anemia is based on cultural representations of this disease that people have. It does not aim to repair physiological disorders. It focuses on restoring social order by reconciling the patient and their groups with their social and supernatural environments [14]. This therapy considers the sick child as a messenger, a person who talks about the transgressions of ancestral norms by a member of his family or the persecution of his family by a wizard. This is the reason why parents, uncles, aunts, brothers, sisters and cousins of the patient are all also patients of the traditional therapists.

#### **2.5 Cameroonian families oscillating between modernity and tradition**

African families have retained certain intrinsic cultural values such as polygamy and the maintenance of family life through the births of several children. They have also opened up to modernity by gradually applying family planning practices.

In Cameroonian families, the cultural tradition is neither past nor "outdated" [14]. The behaviors of the subjects are always marked by identifiable traditional elements. These families have neither resisted nor surrendered to modernity. These are simultaneously modern and traditional families, not necessarily modern or traditional. In Cameroon, there are several types of families; nuclear families, extended families, polygamous families, monogamous families, etc. [27]. These categories reflect the diversity and complexity of family dynamics in Cameroon, and potentially the lives of children confronted by a sick sibling.

#### **2.6 The experiences of the brothers and sisters of sick children**

A child's illness has repercussions on their siblings who experience ambivalent feelings towards them. These relate specifically to shame, guilt, love, hatred, complicity, rivalry, anguish and the desire for the death of the sick child [28]. In order not to aggravate the suffering of their parents and that of the patient, brothers and sisters often attempt to prevent them from suffering [29].

Studies on sickle cell anemia patients indicate the psychological suffering of sick children and their parents [9, 14, 30]. The experiences of their siblings are often studied from the parents' stories. Overprotection of the child causes parents to progressively neglect caring for their other children [31]. The latter experience emotional breakdowns resulting in feelings of rejection, marginalization and exclusion on the part of parents and the extended family [32, 33]. They express jealousy towards the patient that they designate as the main person responsible for crises that destabilize the family financially and emotionally [34]. They feel guilty for having negative thoughts such as jealousy towards the patient; they consider themselves "bad siblings" [35]. They experience, at the same time, the desire for the death of the patient and the fear of this death [36]. Their experiences are usually influenced by parental intrusions in their relationships with the sick child because they are

**43**

the family.

**3.2 Jules and his family**

*Sickle Cell Anemia, Representations and Care: Experience of a Brother of a Sick Child…*

making them the person most likely to take care of children [21].

often parented by parents [36]. In the absence of the parents, they are the ones who supervise the sick child. Supervision ranges from daily monitoring of treatment and observation of medical instructions by the patient [2] to the bodily care of the patient and their assistance during hospitalization [37]. This has great implications on the sisters of sick children, particularly on girls' education. In many sub-Saharan African societies, the main objective is to bring up girls to be good mothers, thus

The methodology of this research is similar to that presented in a previous research [36]. In sub-Saharan African cultures, children who talk to professionals about themselves or anything else are supposed to have been given permission by their families to do so [38]. It is usually parents who talk to professionals about their children, who on their part are obliged to listen and to talk only when adults allow it [39]. Talking with children about sickle cell anemia is not easy and it requires the researcher to make an alliance with the family. This research, which took place in Cameroon worked to get parents to allow their children to talk with the researcher about the way they experience the care of their sick brother or sister. In fact, many children are not allowed to speak to strangers and to speak only to adults who are

The parents signed consent forms concerning the participation of the family and children in the research and designated which of their children would participate. These children were allowed by their parents to speak with the researcher about their family, their sick brother or sister and his/her illness. Indeed they had a mission to talk to the researcher who informed them about their freedom to participate in this research. The method includes an individual interview with each child and a drawing, preceded and followed by a group time. Before the interview, the mother, in front of the whole family, talks about the relations between her sick child and his siblings; the children listen and keep quiet. After the interview and the drawing, the adults question the researcher and the participating child about the content of their meeting. It is therefore an individual meeting, but it takes place in a group setting

We report in this article the case of Jules. With Jules, we talked about the illness, his relationship with the sick child and the family. In Jules' interview, there is an important place for treatment and its effects on the sick child, on his siblings, on his mother and father and on extended family members. This interview was done in the absence of the other members of the family whom we asked to leave the family room to allow for confidentiality. The interview was followed by the drawing session after which the analysis of the drawing was done. The family drawing test followed the approach of [41], taking into account the cultural referent as advocated by [42]. We asked Jules to draw his family on a sheet. The drawing, complementary to the interview allows the child to project on the sheet what he thinks and experiences about his family, his sick brother and his place in

In accordance with the ethical requirements, we gave fictitious first names to the

persons to guarantee the anonymity and the confidentiality of the meeting.

*DOI: http://dx.doi.org/10.5772/intechopen.90995*

**3. Material and method**

**3.1 Specificities of the method**

intimate with the family [40].

allowing children to speak as freely as possible.

*Sickle Cell Anemia, Representations and Care: Experience of a Brother of a Sick Child… DOI: http://dx.doi.org/10.5772/intechopen.90995*

often parented by parents [36]. In the absence of the parents, they are the ones who supervise the sick child. Supervision ranges from daily monitoring of treatment and observation of medical instructions by the patient [2] to the bodily care of the patient and their assistance during hospitalization [37]. This has great implications on the sisters of sick children, particularly on girls' education. In many sub-Saharan African societies, the main objective is to bring up girls to be good mothers, thus making them the person most likely to take care of children [21].
