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Psychosocial Burden of Sickle Cell Disease on Parents.

Author(s) Ambroise Wonkam 1
Affiliation(s) 1Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, South Africa, Cape Town, South Africa.
Country - ies of focus Cameroon
Relevant to the conference tracks Chronic Diseases
Summary The chronicity of Sickle Cell Disease (SCD) could impair the quality of life of caregivers.
We studied 130 Cameroonian parents with SCD-affected children with a survey instrument including a 38-item stress factors scale.
Parents participants were urban dwellers (89 %), female (80 %), married (60.2 %), and employed (61.7 %). The majority (88.3 %) experienced moderate/ severe difficulty coping with SCD. >3 vaso-occlusive painful events per year was the stressor that most impacted their coping ability. Stressors scores increased with female, single, low education or > 3 children.
In Cameroon, there is a need to ensure affordable access to health-care and activities that would reduce SCD morbidity.
Background In Cameroon population carrier frequencies of SCD range from 8 % to 34 % with a birth incidence of 1.6 %. Although Cameroon has developed a national program for SCD, it remains unimplemented. There is no universal medical insurance coverage in Cameroon and parents and families play a central role in the care of children with SCD.
The chronicity of SCD and the associated frequent hospital attendance and poorer physical state of patients may contribute significantly to the impairment of the quality of life and wellbeing of parents, including marital disharmony, job performance and hindrance from the enjoyment of life.
We reported that Cameroonian parents (i.e. 62.5 %) with a SCD-affected child accepted the principle of termination of pregnancy if the fetus was to be affected. Prenatal diagnosis for SCD was initiated in Cameroon in 2007. In actual practice, to date, 90 % of parents with SCD-affected pregnancies elect for medical abortion. Parents reported that the negative family life experience of SCD, fear of having more affected children, and fear of the poor quality of the child’s health were their main reasons for acceptance of medical abortion for SCD.
Despite the high prevalence of SCD in Africa, there is a paucity of data on the psychosocial impact of SCD on their families
Objectives The general aim of the present study was to assess the perception and severity of various indices of psychosocial burden experienced by parents of SCD patients.Specific research questions were:
(1) How difficult is it to cope with the hospital factors related to the care of your SCD-affected child? 
(2) Has taking care of your SCD affected child made you lose any sort of revenue or financial benefits?
(3) How difficult do you find coping with the clinical manifestations of your SCD-affected child?
(4) Has the problem of caring for your SCD affected child caused any disharmony in your family life?
(5) How difficult is it for you to cope with your SCD-affected child's life?It is felt that this empirical approach towards the assessment of selected psychosocial areas of need, and its implications for care giving, are critical, as the findings could help to plan health resources and services appropriately and guide the provision of necessary support in the future.
Methodology Research Design
This research was a cross-sectional sociological study, using a quantitative approach.Sampling Method
The sampling methods were both purposeful and convenience sampling. Participants were recruited amongst parents of children with SCD. Parents and their affected children were invited to participate via the media. In addition, SCD-patients’ associations were contacted and engaged to collaborate with recruitment efforts.Participants and Eligibility Criterion:
The inclusion criterion was a parent residing with at least one living child with SCD.Questionnaire Format
Socio-demographic and Medical Information Demographic and medical information were obtained from the participants.
Psychosocial Impact of SCD
The general burden and burden of individual stressor factors was determined using a validated instruments for the study of caregiver burden in SCD. The survey instrument included a 38-item stress factors scale using Likert-type statements, evaluating general perceptions of stress and five main specific stressors: hospital factors, financial factors, disease factors (clinical severity), family factors (family life and dynamic) and child factors (perceived quality of life of the SCD-affected child). From individual stressors, the items pertaining to burden, there were four response options with increasing severity: 0, 1, 2 or 3.
A typical item to elicit responses about financial burden (stressors) was “Has taking care of the SCD-affected child made you (the participant) lose any sort of revenue or financial benefits for example loss of job, loss of working hours for business or going to farm or going to sell at the market etc, which has greatly affected the family? The response scale was 0=No such loss, 1=Minor loss of revenues or benefits, 2= Moderate loss of incomes, 3=Major loss of incomes.Setting and Procedure
The study was conducted at the Yaoundé Central Hospital, Cameroon. All face-to-face interviews were conducted in a private consultation room by two investigating physicians: a medical geneticist and a general practitioner.

Data Analysis
Data analysis was performed using the SPSS (Statistical Package for Social Sciences, Chicago; version 17.0). In addition, the skewness of the data informed the use of nonparametric tests. Level of statistical significance was set at p

Results Socio-demographic Characteristics of Participants.
A total of 130 participants responded and all were interviewed. The vast majority (89 %; n=115) lived in the urban areas of Yaoundé, the capital city of Cameroon; median age: 38 years (Range: 22–67), were female (80 %; n=104), Christian (93 %; n=119), married (77.1 %; n=86), living in monogamous households (81.1 %; n=73), employed (61.7 %; n=79), with at least a secondary or tertiary education (82 %; n=105).
Conclusion In conclusion, this group of Cameroonian parents with affected SCD children appears to experience major burden due
to the condition. The financial burden and the frequent vasoocclusive painful crises contributed significantly more than
other measured factors to the psychosocial burden of the caregivers. The results of the study could help to improve
counseling information when advocating for various screening policy, as well as insight for anticipatory counseling
guidance to affected families and patients in psychosocial areas that could need careful planning or specific attention.
The findings emphasize the need for the implementation of policies and practices that ensure easy access to health-care at
affordable costs to SCD-affected families.Policy and Practice ImplicationsThe study provides alternative measures to evaluate caregivers relative to their psychosocial stress. The identification
of those who are not coping effectively is valuable in order to provide appropriate support and benefit from any public
health interventions. The data suggest that high risk caregivers’ groups are monoparental families, especially single
females, unemployed, and caregivers with a low level of formal education. The data reveal the urgent need for schools
to consider adopting flexible tutoring and educational plans involving families with SCD, and for hospital administrations
and parents’ employers to be more aware of the needs of such families.With respect to genetic counseling services, the findings of this study provide insight regarding factors which could
motivate parents to pursue prenatal genetic diagnosis for SCD and may facilitate greater empathy among service
providers. The study also provides data for anticipatory counseling guidance for families with SCD-affected children
in psychosocial areas that may need careful planning or specific attention and support. In addition, the study provides some evidence to enhance counseling information when advocating for screening policies for SCD (neonatal, premarital
or population screening) that are encouraged by the Cameroon National Control Program for SCD.

Would you Terminate a Pregnancy Affected by Sickle Cell Disease? Views of Doctors, Parents and Patients in Cameroon.

Author(s) Ambroise Wonkam1, Jantina de Vries2, Charmaine Royal3, Dora MBanya 4, Jeanne Ngongang 5, Fru Angrafo III6
Affiliation(s) 1Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa, 2Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa, Institute for Genome Sciences & Policy, Duke University, Durham, United States, 4Department of Medicine, Faculty University of Yaoundé I, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Cameroon, Biochemestry, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon, Surgery, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon.
Country - ies of focus Cameroon
Relevant to the conference tracks Women and Children
Summary We studied the views of 110 doctors, 130 parents with one living child with SCD (Sickle Cell Disease), and 89 adults patients suffering from SCD, regarding prenatal genetic diagnosis and termination of a SCD-affected pregnancy.
The majority accepted the principle of prenatal genetic diagnosis for SCD (78.7%, 89.8% and 89.2%). The majority of parents accepted the principle of termination of SCD-affected pregnancy (62.5%) as opposed to doctors and patients where this group were in the minority (36.1%, and 40.9 %). Parents and patients who rejected termination of pregnancies claimed ethical reasons (69.1 and 78.1%) while those who accepted it feared having another SCD-affected child (98.1 and 88.9%) with a poor quality of life (92.6% and 81.5%).
Background Patients with Sickle Cell Disease (SCD) can suffer from anemia, painful episodes, susceptibility to infection, stroke, and chronic organ damage (kidneys, lungs, heart, brain). There is currently no cure available for SCD, but the condition can be managed using a variety of therapies. When the condition is not managed patients tend to die in early childhood as occurs in many African countries.
In Cameroon it is possible to test for sickle cell homozygosity before birth, and in fact Prenatal Genetic Diagnosis (PND) represents one type of preventive strategy, as it is offered as a reproductive option to at-risk parents. PND provides parents with a reproductive option to test at-risk pregnancies and make decisions regarding medical abortion.
However, legal bans on abortion exist in virtually all African countries and when allowed medical abortion is often restricted to direct threats to maternal health. This raises important ethical questions regarding the desirability of terminating affected pregnancies.
Many parents currently caring for a child with SCD opt to abort a fetus that is also suffering from SCD. What has not yet been investigated is the comparative views of parents to those of health care providers and patients living with SCD.
Objectives We examined the attitudes of a sample of Cameroonian medical doctors, parents with at least one SCD-affected child, adult SCD patients towards PND and TAP. There were two major research questions: (1) their agreement with prenatal genetic diagnosis and pregnancy termination in general (2) their agreement with prenatal genetic diagnosis for SCD, and pregnancy termination for SCD, and the reasons for their attitudes.
Methodology Design
This research was a quantitative social science study administered by structured questionnaires.
Sample Population and Eligibility Criteria.
The sampling methods used included both purposeful and convenience sampling.
Medical doctors were recruited from a National Medical Conference for continue medical education. In an attempt to ensure inclusion of parents and adult SCD-affected patients and incorporate the entire spectrum of this illness, we issued a call for participation using the national Cameroonian media. We also approached two SCD Patients’ Associations in Cameroon. Participants needed to be at least 18 years old with a diagnosis of SCD that was confirmed by a laboratory documentation of their hemoglobin electrophoresis.
Questionnaire Format.
The data were collected by means of a structured questionnaire consisting of three sections of closed-ended questions. These were (1) Socio-demographic characteristics; (2) Attitudes towards SCD screening policies; and (3) Attitudes about principles of SCD- prenatal diagnosis and termination of an affected pregnancy if the participant’s unborn child were proven to be affected. Response options were “Yes,” “No” or “Undecided”.
Research Setting and Data Collection.
The study was conducted at the Yaoundé Central Hospital where face-to-face questionnaires interviews were conducted. Informed consent was also obtained at this stage. In addition to the introductory explanation, each patient was given full non-directive genetic counseling with neutral information concerning PND and its reproductive options. Images were used to explain the obstetric procedure of PND and risks (specifically 1% induced miscarriages). Information on the available therapeutic options and follow up for patients with SCD was reviewed and the participants were given an opportunity to ask questions. The information provided during this counseling session was equivalent to the information that prospective parents would have received had they been seeking PND for SCD.
Data Analysis.
Data were analyzed using SPSS (Statistical Package for Social Sciences, Chicago). A comparison between two or more variables was evaluated by non-parametric tests (H test of Kruskal-Wallis or Z test of Kolmogorov-Smirnov, when applicable). The p values were considered significant if they reached 95%.
Results The majority of parents participants lived in urban areas (89%), were female (80%), Christian (93%), married (60.2%) in monogamous households (81.1%), were employed (61.7%), and had at least a secondary or tertiary education (82%). Similarly, the majority of the patient participants were urban dwellers (84.3%), female (57.3%), Christian (95.5%), single (90.9%), with a secondary/tertiary education (79.5%).
The clinical profile of participant children and patient participants indicated that they suffered from (relatively) severe forms of SCD. The majority of research participants received poor treatment for their SCD. Only 4.4% of participants received hydroxyurea treatment, the only treatment currently available to manage SCD. Nearly 90% (89.7%) had received traditional medicine for their conditions on at least one occasion in the past.
The majority accepted the principle of prenatal genetic diagnosis for SCD (doctors: 78.7%; parents: 89.8% and patients: 89.2%). The majority of parents accepted the principle of termination of SCD-affected pregnancy (62.5%), but doctors and adults patients were less comfortable with this principle (36.1%, and 40.9 % acceptance, respectively). The acceptance of the principle of medical termination for SCD increased with unemployment status. (missing data here)
Conclusion Differential views regarding medical abortion for SCD in Cameroon could lead to societal, ethical and legal conflicts. Our finding may well reflect the failure of professional stakeholders to provide adequate care services to patients with SCD in Cameroon.
The patient participants in this study indicated a surprisingly high (40.9%) rate of acceptability of TAP. This is surprising as one could argue that a decision to terminate a pregnancy where the future child would suffer from the same condition that is affecting the parent seems to imply a value judgment about the individuals’ quality of life. Patients who participated in this study presented with severe forms of SCD. We wonder whether our results mean that approximately 4 out of 10 of the patients included in this study did not find their quality of life worth living and did not want to allow a child to experience it. This is a disturbing finding that requires the further attention of policy makers and medical professionals in Cameroon.
Our finding may well reflect the failure of professional stakeholders to provide adequate care services to patients with SCD in Cameroon. For instance, the average late diagnosis of the condition in our participants leads to greater clinical severity. In addition, the very low number of people who receive adequate medical care to manage their condition, as well as the large number of people who received traditional medicine, may also indicate the failure of medical professionals in Cameroon to adequately manage SCD. Many patients with SCD require the expertise of specialized centers. Lifelong medical care and surveillance are not yet available in Cameroon where provision of healthcare services is hampered by major economic, organizational and infrastructural difficulties.These differential views of patients, physicians and parents also indicate potential ethical conflicts between various components of the Cameroonian society regarding TAP for SCD. Additional studies among various groups may provide detailed insight into the range of moral, legal and social perspectives held by the public and the healthcare community regarding genetic technology and prenatal diagnosis in Cameroon.