Assessment of the Quality of Life of Parents of Children with Hemophilia in Yaounde, Cameroon ()
1. Introduction
Hemophilia is a rare disease caused by a hereditary deficiency in coagulation factors VIII (hemophilia A) or IX (hemophilia B). The incidence varies depending on the type of hemophilia, with 1 case of hemophilia A per 5000 live male births per year, compared to 1 case of hemophilia B per 30,000 [1]. The complications of hemophilia and the complexity of care surrounding this condition are a challenge for patients and their families worldwide [2]. The daily lives of children with hemophilia are marked by repeated bleeding, which causes pain, vascular-nerve compression, chronic joint disease, anemia, and sometimes death, depending on the site of the bleeding [3]. Financially, although the World Federation of Hemophilia regularly provides clotting factors in the form of donations to countries with limited resources, all other healthcare expenses related to this disease, including the purchase of hemostatic drugs, painkillers, mobility aids, the cost of blood transfusions and physical therapy, multiple hospitalizations, and additional paraclinical tests depending on the site of bleeding, are the responsibility of the parents [4]-[7]. Like any hereditary disease, hemophilia often causes parents to feel guilty, affecting their emotional well-being and causing emotional stress, anxiety, and depression [3] [4] [7]-[11]. Social relationships are also disrupted due to restrictions on physical and recreational activities, as well as multiple hospital visits requiring the constant presence of parents at their children’s side [2] [7] [10] [12]. All of this can affect quality of life. Numerous studies have examined quality of life as perceived by children with hemophilia, but very few have focused on the quality of life of parents of children with hemophilia [1] [2] [11] [13] [14]. The overall objective of this study was to assess the quality of life of parents of children with hemophilia and thereby contribute to the development of targeted health policies and interventions focused on medical and psychosocial support for these families.
2. Methods
A cross-sectional and analytical study was conducted from March 1, 2024, to July 31, 2024 (5 months). Children with hemophilia aged 1 to 18 years who were being treated at the Yaounde University Teaching Hospital, and their parents who agreed to participate in the study, were included. The recruitment was non-probabilistic and consecutive. The parents’ quality of life (QoL) was assessed using a tool called “Hemophilia Associated Caregiver Burden Scale (HEMOCAB)” [15].
This scale was developed in the United States and linguistically validated in various European languages. In our context, it has been translated into English and French, which are the two official languages of Cameroon. It is a self-assessment questionnaire, comprising 54 questions in 13 areas, nine of which relate to the frequency of the burden (emotional stress, financial burden, personal sacrifices and limitations, employment-related burden, parents’ interactions with others, the child’s interactions with others, interactions with school, the child’s perception, and managing the child’s hemophilia) and four relating to the intensity of the burden (medical care, professional situation related to the child’s hemophilia, interaction with the child’s father, and the impact of the child’s hemophilia).
Responses for each domain were reported on a five-point Likert scale. Responses relating to the frequency of the burden ranged from “never” to “always”, and those relating to the intensity of the burden ranged from “not at all” to “a lot”. High values corresponded to a high burden. For each domain, the total score was calculated by adding up the responses to the questions. The overall quality of life score was calculated by combining the scores for the 13 domains. This overall assessment, which ranged from 51 to 255 points, was converted to a scale of 0 to 100. In our study, we considered an average score below 50% as a good quality of life, while a score of 50% or higher indicated a poor quality of life. The children’s sociodemographic and clinical variables (age, sex, school level, place of residence, type of hemophilia, severity of hemophilia, type of current treatment), as well as the parents’ sociodemographic and economic data (age, sex, occupation, marital status, level of education, weekly working hours, change of job), were collected.
2.1. Operational Definition of Terms
“Low income” was defined as an income below the guaranteed minimum wage in Cameroon (<$79).
“Important bleeding” referred not only to the volume of blood lost but also to the location of the hemorrhage, which can be life-threatening.
“Poor care” was defined as inadequate treatment that could lead to disabling sequelae or even death.
A HEMOCAB domain was considered “good” when the average score was less than 50%.
A HEMOCAB domain was considered “poor” when the average score was 50% or higher.
2.2. Data Analysis
The data were analyzed using SPSS version 26.0 software. Qualitative variables were expressed as numbers and percentages, while quantitative variables were expressed as means and standard deviations, or medians and interquartile ranges. Comparisons between the different variables were performed using the Chi-square or Fisher’s exact test. The strength of association was estimated using the odds ratio and its 95% confidence interval. Quality of life was reduced to two categories (good or poor). To identify the factors associated with parents’ quality of life, a bivariate analysis was conducted with quality of life (good, poor) as the dependent variable, and the psychosocial variables from the HEMOCAB were considered as predictors. A value of p < 0.05 was considered statistically significant.
2.3. Ethical Considerations
Approval was obtained from the Institutional Ethics and Research Committee of the FMSB at UY1 (Reference: No. 0667/UYI/FMSB/VDRC/DAARS/CSD dated June 10, 2024). The free and informed consent of parents and the assent of children aged 10 and over were obtained. Anonymity and confidentiality were strictly observed.
3. Results
During the study period, of the 76 eligible parents, 23 who could not be reached by phone were excluded, leaving 53 parents with 65 children with hemophilia selected for the study.
3.1. Sociodemographic and Clinical Characteristics of Children with Hemophilia
More than half of the children were aged 10 years or older (58.5%), with a mean age of 10.6 ± 5.3 years, ranging from 1 to 18 years. Forty nine of them had a secondary education level (49.2%). They were carriers of hemophilia A (86.2%), most of whom had severe hemophilia (67.7%). Nearly half had had at least two bleeds in the past 12 months (49.2%). The majority were on on-demand treatment (73.8%) (Table 1).
Table 1. Distribution of children with hemophilia according to their sociodemographic and clinical characteristics.
Variables |
Number (N = 65) |
Percentage (%) |
Age groups (years) |
|
|
[1 - 3] |
3 |
4.6 |
[3 - 5] |
9 |
13.8 |
[5 - 10] |
15 |
23.1 |
≥10 |
38 |
58.5 |
child’s grade level |
|
|
None |
5 |
7.7 |
Primary |
26 |
40.0 |
Secondary |
32 |
49.2 |
Tertiary |
2 |
3.1 |
Type of hemophilia |
|
|
Hémophilia A |
56 |
86.2 |
Hémophilia B |
9 |
13.8 |
Gravity |
|
|
Mild |
2 |
3.1 |
Moderate |
19 |
29.2 |
Severe |
44 |
67.7 |
Total number of important bleeding events during the last 12 months |
|
|
≥2 |
32 |
49.2 |
<2 |
33 |
50.8 |
Treatment |
|
|
On demand |
48 |
73.8 |
Prophylactic |
12 |
18.5 |
No treatment |
05 |
7.7 |
3.2. Sociodemographic and Economic Characteristics of Parents
The main respondents were mothers (83%). The most represented age group was 31 - 40 years old (43.4%). The type of family is biparental in the majority (64.1%), and the educational level is secondary for 54.7% of parents. Twenty-one had at least three dependent children (39.6%), most of whom had hemophilia (81.1%). Two-thirds (69.8%) had low incomes. Just over half worked part-time (n = 27, 50.9%), and for 11 of them, this was due to their child’s illness (40.7%). Twelve parents reported having changed jobs because of their child’s illness (22.6%). Parents reported spending a great deal on transportation to the hospital (92.5%), medication (88.7%), and physical therapy sessions and physiotherapy (30.1%) (Table 2).
Table 2. Distribution of the parents according to their sociodemographic and economic characteristics.
Variables |
Number (N = 53) |
Percentage (%) |
Respondents |
|
|
Mother |
45 |
85 |
Father |
4 |
7.5 |
Others (aunts and uncles) |
4 |
7.5 |
Age groups (years) |
|
|
[26 - 30] |
10 |
18.9 |
[31 - 40] |
23 |
43.4 |
[41 - 50] |
15 |
28.3 |
≥ 50 |
5 |
9.4 |
Family type |
|
|
Biparental |
34 |
64.1 |
Monoparental |
19 |
35.9 |
Level of education |
|
|
Unschooled |
2 |
3.8 |
Primary |
10 |
18.9 |
Secondary |
29 |
54.7 |
Tertiary |
12 |
22.6 |
Number of children |
|
|
< 3 |
15 |
28.3 |
3 - 4 |
21 |
39.6 |
≥5 |
17 |
32.1 |
Number of children with hemophilia |
|
|
1 |
43 |
81.1 |
2 |
8 |
15.1 |
3 |
2 |
3.8 |
Profession |
|
|
Senior government officials, private sector executives, import/export traders |
4 |
7.6 |
Traders and middle-ranking civil servants |
12 |
22.6 |
Traders with little capital, workers, and laborers |
16 |
30.2 |
Farmers and casual workers in cities, housewives |
21 |
39.6 |
Working frequency |
|
|
Part-time work |
27 |
50.9 |
Full-time work |
26 |
49.1 |
Part-time work due to child’s hemophilia (n = 27) |
|
|
Yes |
11 |
40.7 |
No |
16 |
59.3 |
Changed profession due to child’s hemophilia. |
|
|
Yes |
41 |
77.4 |
No |
12 |
22.6 |
Healthcare spending |
|
|
Transportation |
49 |
92.5 |
Medical supplies |
47 |
88.7 |
Physical therapy |
12 |
22.6 |
Mobility aids |
7 |
13.2 |
Psychotherapy |
5 |
9.4 |
Physiotherapy |
4 |
7.5 |
3.3. Quality of Life of Parents of Children with Hemophilia
Poor quality of life was found in 66% of parents. The average overall quality of life score was 140.04 ± 31.72, with extremes of 51 and 197 (Table 3).
Table 3. Quality of life score according to the different areas of the HEMOCAB scale.
Domain |
Number |
Means ± DS |
Min |
Max |
Emotional stress |
53 |
69 ± 19 |
23 |
100 |
Financial burden |
53 |
66 ± 26 |
20 |
100 |
Personal sacrifices |
53 |
49 ± 24 |
20 |
100 |
Work-related burden |
53 |
31 ± 27 |
0 |
93 |
Relationships with other people |
53 |
43 ± 19 |
20 |
80 |
Interaction between the child and others |
53 |
43 ± 25 |
0 |
90 |
Relationships with the child’s school |
53 |
48 ± 32 |
0 |
100 |
Perception of the child |
53 |
72 ± 16 |
37 |
100 |
Managing the child’s hemophilia |
53 |
61 ± 14 |
30 |
95 |
Medical care |
53 |
72 ± 19 |
20 |
100 |
Work situation related to hemophilia |
53 |
32 ± 31 |
0 |
100 |
Interaction with the child’s father |
53 |
30 ± 31 |
0 |
100 |
Impact of the child’s hemophilia |
53 |
56 ± 23 |
20 |
100 |
Global quality of life |
53 |
140.04 ± 31.72 |
51 |
197 |
3.4. Factors Associated with Poor Quality of Life among Parents of Children with Hemophilia
Significant personal sacrifices [OR = 32.6 (3.8 - 275.3), p = 0.001], poor relationships with others [OR = 5.3 (1.1 - 26.9), p = 0.030], poor interaction of the child with others [OR = 4.9 (1.4 - 17.3), p = 0.011], poor interaction with the child’s school [OR = 9.6 (2.3 - 39.8), p = 0.002], poor management of the child’s disease [OR = 6.4 (1.1 - 36.9), p = 0.04], and the negative impact of hemophilia on parents [OR = 9.1 (2.4 - 33.3), p = 0.001] were factors significantly associated with poor quality of life for parents (Table 4).
Table 4. Associated factors of parents’ poor quality of life.
Variables |
Quality of life of
parents |
OR
(CI 95%) |
p value |
Poor |
Good |
Personal sacrifices |
|
|
|
|
Normal |
12 (34.3) |
17 (94.4) |
1 |
1 |
Important |
23 (65.7) |
1 (5.6) |
32.6 (3.8 - 275.3) |
0.001 |
Relationships with other people |
|
|
|
|
Good |
21 (60) |
16 (88.9) |
1 |
1 |
Poor |
14 (40) |
2 (11.1) |
5.3 (1.1 - 26.9) |
0.030 |
Interaction between the child and others |
|
|
|
|
Good |
12 (34.3) |
13 (72.2) |
1 |
1 |
Poor |
23 (65.7) |
5 (27.8) |
4.9 (1.4 - 17.3) |
0.011 |
Relationships with the child’s school |
|
|
|
|
Good |
12 (34.3) |
15 (83.3) |
1 |
1 |
Poor |
23 (65.7) |
3 (16.7) |
9.6 (2.3 - 39.8) |
0.002 |
Managing the child’s hemophilia |
|
|
|
|
Good |
2 (5.7) |
5 (27.8) |
1 |
1 |
Poor |
33 (94.3) |
13 (72.2) |
6.4 (1.1 - 36.9) |
0.04 |
Medical care |
|
|
|
|
Increased |
34 (97.1) |
11 (61.1) |
1 |
1 |
Normal |
1 (2.9) |
7 (38.9) |
0.1 (0.0 - 0.42) |
0.001 |
Impact of the child’s hemophilia |
|
|
|
|
Positive |
8 (22.9) |
13 (72.2) |
1 |
1 |
Negative |
27 (77.1) |
5 (27.8) |
9.1 (2.4 - 33.3) |
0.001 |
4. Discussion
4.1. Study Limits
This study assessed the quality of life of parents of children with hemophilia and identified factors associated with it. However, several limitations were noted, including the single-center setting, the relatively small sample size, the exclusion of parents who could not be reached by phone, and the potential for selection and information bias.
4.2. Sociodemographic and Clinical Characteristics of Children with Hemophilia
The mean age was 10.6 ± 5.3 years, ranging from 1 to 18 years. A similar average age was also found by other authors [4] [12] [13]. However, a review of the literature shows that average ages vary depending on the age groups used in different studies. Forty-nine percent of them were enrolled in secondary school (49.2%).
The majority of children had hemophilia A, and most cases were severe. This was also seen in other studies [3] [8] [11] [13] [14]. However, other authors found more children with a moderate form [12] [16]-[18]. Half reported having had at least two bleeds in the past 12 months (49.2%). Williams et al. found in their study that 5% of parents were anxious about their child’s risk of bleeding [3]. With regard to treatment, coagulation factors were administered on demand in the majority of children (80%), while only 20% received prophylactic treatment. Similar results have been found by Wiedebusch et al. [13]. On the other hand, Buckner et al. had only 17% of children with treatment on demand [12]. Some authors found that treatment on demand could alter quality of life, while prophylactic treatment, although considered chronic treatment, could improve quality of life [2].
4.3. Sociodemographic and Socioeconomic Characteristics of Parents
The main respondents were mothers, as confirmed by numerous studies [4] [6] [9] [10] [12] [13] [15]. This can be explained in cultural and social terms, as mothers are the main sources of support and care for their children, especially when the latter have a chronic illness [2] [11] [12] [17]. However, Ersoy et al. in 2023 in Türkiye found that fathers were the main respondents, which can be explained by the low literacy rate among mothers in their study environment [5]. The majority of parents were married. This has been found in other studies [9] [13] [15]. While it is well known that a stable family structure can provide essential support for parents who have to manage the complex and sometimes unpredictable care needs associated with their child’s illness [2] [14], couples with children with chronic illnesses may also experience relationship tensions due to the burden of care and financial concerns, resulting in a reduced quality of life [13]. The majority of parents had at least a secondary education level. This has also been found in other studies [4] [7] [9] [13] [15] [18]. A high level of education is often correlated with a greater ability to access medical information and make informed decisions about care [12]. Consequently, the parents’ level of education could have an impact on the quality of care received by children with hemophilia and, secondarily, on the parents’ quality of life.
Half of parents work part-time because of their children’s illness (50.9%). This has also been found in numerous studies [2] [5] [6] [12] [15] [18]. Khair et al. found that two-thirds of parents in their study worked part-time [2], and Ersoy et al. noticed that the percentage of parents of children with chronic illnesses working full-time was low compared to that of parents with healthy children [5]. Parents of children with chronic illnesses are often forced to stop working or reduce and adapt their working hours to meet their children’s medical needs [2] [5] [17]. Sometimes they are forced to change jobs, as found in our study and others [5] [15]. Although necessary, working part-time can have significant economic repercussions for families. Parents who reduce their working hours to care for their children run the risk of seeing their total income decrease, which can make it difficult to manage healthcare-related expenses [18]. In fact, in our study, most parents reported spending a lot on transportation to the hospital (92.5%), medication (88.7%), and physical therapy and physiotherapy (30.1%). Other studies have also shown that parents of children with chronic illnesses often invest a significant portion of their income in medical care, transportation, and medication, which causes financial hardship [5] [9]. It is also well known that limited activities can be a source of stress and anxiety [2].
4.4. Quality of Life
A poor quality of life was found in 66% of parents. Von Mackensen et al. found results similar to ours (66.2%) [15]. In Nepal, Shakya et al. observed moderate and severe levels of impaired quality of life in 60.5% and 9.2% of parents, respectively [18]. Mothers were our main respondents. Studies have shown that mothers of children with chronic diseases report higher levels of stress and impaired quality of life compared to mothers of healthy children [13]. Other studies have shown that the burden on mothers of children with hemophilia is higher than that on fathers due to their involvement in the child’s care, but also because of the guilt they feel [12] [13]. Indeed, caring for a child with a chronic disease, and more specifically hemophilia, requires many sacrifices on a physical, financial, socio-professional, and emotional level. In our study, high scores were found for both the frequency and intensity of the burden, namely high emotional stress (69 ± 19). Parents of children with chronic illnesses frequently suffer from increased emotional stress [4] [9]-[11] [13] [17]. The presence of inhibitors in children with hemophilia was a source of psychological stress [2]. According to Williams et al., 5% of parents were anxious about the risk of bleeding [3]. Kodra et al. found that 54% of parents had anxiety problems [8]. For Von Mackensen, emotional stress was one of the most affected areas among parents (37.4 ± 22.6) [15].
Healthcare and treatment costs can place considerable financial pressure on families. In the study, the financial burden was high (66 ± 26). In fact, 92.5% of parents said they had spent money on transportation and 88.7% on medical supplies. This was also found by other authors [2] [4] [18]. Ersoy et al. found that most parents had to travel for care and that many were not affiliated with social security [5]. Von der Lippe et al. suggested that home treatment could reduce the financial burden on parents [10]. For Von Mackensen, 26.8% of parents had a high financial burden [15]. Obeidat et al. attributed the financial burden to home schooling and the cost of food [17].
The burden associated with the child’s perception was high (72 ± 16). In the literature, parents’ quality of life is also strongly influenced by their perception of their child. Our results show that 100% of parents with a negative perception of their child report a poor quality of life. This corroborates the work of Dekoven et al., who found that a positive perception of the child’s abilities could reduce stress and improve parents’ quality of life [4]. Von Mackensen found that the perception of the child was one of the areas of quality of life most affected (37.9 ± 24.7) [15]. For Khair, activity limitations were a source of stress and anxiety for parents [2]. According to Williams et al., parental perceptions of their children’s hemophilia varied depending on the age and severity of the disease [3].
The management of hemophilia in children had a considerable impact on the quality of life of parents of children with hemophilia (61 ± 14). Indeed, parents’ quality of life is strongly influenced by how they manage their child’s hemophilia. Kish et al. reported that the feeling of having to do everything for their children had an impact on parents’ quality of life [16]. Other authors also noted that some parents said they did not have enough energy because their child’s illness required enormous personal sacrifices on their part to care not only for the child with hemophilia but also for the whole family, and that all of this had a negative impact on their physical health [4] [14] [17].
Medical care was a burden for parents of children with hemophilia (72 ± 19). This has also been found by other authors [4] [15]. Obeidat et al. reported that frequent hospital visits to obtain clotting factors and for the management of complications had an impact on the physical health of parents of children with hemophilia [17].
The impact of the child’s hemophilia also proved to be a burden for parents in the study (56 ± 23).
The mean overall quality of life score for parents was 140.04 ± 31.72, with extremes of 51 and 197, reflecting the influence of psychosocial factors on quality of life. A high mean overall score was also found by Dekoven et al. (80.76 ± 20.14, with extremes of 35 and 144) [4], Manikandasamy et al. (63.7 ± 19.5 with extremes of 0 and 100) [14], and Shakya et al. (46.8 ± 12 with extremes of 0 and 88) [18].
4.5. Factors Associated with Poor Quality of Life
The significant personal sacrifices [OR = 32.6 (3.8 - 275.3), p = 0.001] observed in the study increased the risk of poor quality of life by 32 times. This result highlights the burden of responsibility that parents must bear. This has also been supported by other authors [4] [16].
Social relationships were also identified as a determining factor in parents’ quality of life. We found that poor relationships with others increased parents’ risk of having a poor quality of life by 5 times [OR = 5.3 (1.1 - 26.9), p = 0.030], and poor interaction between the child and others by almost five times [OR = 4.9 (1.4 - 17.3), p = 0.011]. Many other studies have shown that chronic diseases are often a source of poor social interactions with friends, but also sometimes with other family members, such as other healthy children or even the father, who feel neglected [2] [7] [12]-[15] [17]. Fear of bleeding, which is responsible for physical restrictions, leads to social isolation for children with hemophilia who cannot engage in the same activities as their peers. Rejection and stigmatization due to disability also cause withdrawal, with negative effects on the quality of life of children and their parents [1] [2]. Von Mackensen et al. indicated that positive social relationships play a protective role. They noted that parents with good social support reported less emotional distress and a better quality of life [15].
Poor interaction with the child’s school was found to be an associated factor of poor quality of life for parents [OR = 9.6 (2.3 - 39.8), adjusted p = 0.002]. In fact, children with hemophilia very often have disrupted schooling, which has an impact on their parents’ quality of life [10] [12] [14] [15]. Collaboration between parents and schools appears to be essential for the child’s well-being, but also for the emotional support of parents.
In our study, poor management of the child’s disease significantly increased the risk of impaired quality of life in parents by 6.4 times (p = 0.04). It has been shown that parents of children with chronic diseases, including hemophilia, experience increased emotional stress, which often results in a deterioration in their quality of life [15]. This study found that emotional stress was a key predictor of parental satisfaction, which corroborates our findings, where 100% of parents with high emotional stress reported poor quality of life (p < 0.001).
The negative impact of hemophilia on parents was also a factor associated [OR = 9.1 (2.4 - 33.3), p = 0.001]. In fact, the lesser impact of the child’s hemophilia on parents was more of a protective factor, reducing the risk of parents having a poor quality of life [OR = 0.07 (0.01 - 0.9), p = 0.041]. Parents of children with hemophilia felt considerable emotional strain, often due to the uncertainty associated with the presence of inhibitors [2] [4], the occurrence of bleeding episodes [4], the constraints of on-demand treatment [2], and disruptions to schooling [10] [15].
On the other hand, we noted a significant protective effect when medical care was not considered to have increased by parents [OR = 0.1 (0.0 - 0.42), p = 0.001]. This corroborates the findings of other authors on the impact of medical management on parents’ quality of life [4] [15] [18].
5. Conclusion
More than 60% of parents of children with hemophilia have a poor quality of life, and many factors are associated with this condition. Consideration should be given to implementing health policies and targeted interventions focused on medical and psychosocial support for these families.