Quality of life among caregivers of sickle cell disease patients: a cross sectional study

World Health Organization estimated that over 5% of the world population carries genes of hemoglobinopathies. Approximately 23 out of 10,000 people are affected with sickle cell disease (SCD), with the highest prevalence in African countries, 110 out of 10,000 people [1]. However, SCD is also relatively prevalent in some cultures with high rates of consanguinity and large family size, such as in the Middle-East region, including Saudi Arabia, where hemoglobinopathies constitute real public health issues [2, 3]. SCD is one of the most common genetic disorders in Saudi Arabia, with an overall prevalence rate of 44.1 (42 carriers and 2.1 cases) per 1000 and marked regional variations reaching up to 134.1 per 1000 in the Eastern Region [3, 4].

SCD is a hemoglobinopathy characterized by a chronic anemia with various acute “painful crises” and chronic symptoms that require daily care and sometimes intensive medication; all negatively impacting physical functioning, sleep, school performance, and overall quality of life (QoL) of patients [5, 6]. An increasing interest to the various aspects of patients’ QoL is noted in the recent publications, highlighting the contribution of different factors such as complications, comorbidities, compliance to treatments, etc. [7, 8].

On the other hand, taking care of a child with SCD is a challenging experience for both the caregiver and the other family members [1, 2, 9]. Since, the management of SCD should be family-centered [10], besides the total dependence of the afflicted child on his/her caregiver for general care and treatment, parents, or caregivers, are subjected to continuous pressure; which may, in return, affect the patient’s QoL [11]. Consequently, caregivers are developing psychological disorders such as depression and anxiety [2, 11‐14]. Several studies have described the association between parenting a child with SCD and psychological distress; approximately 30 to 40% of caregivers had symptoms of psychological distress [15‐17].

Generally, common genetic disorders including SCD, constitute a considerable emotional and financial burden on patients and families, and even on the society, particularly in developing countries [18, 19]. A study from Nigeria, where the disease is highly prevalent, demonstrated that caregivers were exposed to intense pressure with the risk of developing psychological problems; which affected their ability to take care the afflicted children. Caregivers were in crucial need for attention and all kinds of support by both the physicians and authorities [19]. Another Nigerian study exposed a triangular relationship between the patient, the disease and the family. Exclusive caregivers, usually mothers, were the most likely to suffer from psychological problems and physical distress [18].

In developed countries such as Netherlands, female caregivers of children with SCD had significant impairment in the QoL, compared to healthy females and female caregivers of healthy children. All dimensions of the QoL were affected, particularly mood, sleep quality, feeling of happiness, and cognitive functioning [11].

In Saudi Arabia, despite high prevalence of the disease, no comprehensive data about health-related QoL of parents and/or caregivers of patients with SCD has been published so far. Such data will provide an insight that is necessary for optimizing the management of patients with SCD, by opening complementary perspectives to the purely medical follow-up and offering more targeted and specific solutions.

This study was conducted to investigate the QoL of caregivers of children with SCD in Western Saudi Arabia, and to assess the true impact of the illnesses on their overall well-being. Different dimensions of the QoL of caregivers were explored as well as the risk factors associated with deteriorated QoL. The dimensions of QoL that are more affected by the disease and the sub-groups of caregivers who are most exposed to poor QoL outcomes, with regards to the risk factors, were defined. We hypothesized that worse child conditions and lower satisfaction regarding different life domains may be significantly impacting the caregivers’ QoL.

The findings of this study highlight the impact of difficulties and challenges faced by close family members of children with SCD, who take care of the patients daily. Family-centered care is part of the continuous, effective and comprehensively coordinated standard health care of patients with SCD [21]. The quality of family-centered care in children with special needs is a critical determinant of healthcare services use [22].

The major observations of this study pointed at the relatively difficult demographic and socioeconomic conditions of caregivers, who were mostly housewife mothers (79.4%) of large families with low financial resources (64.5%) and no social assistance or health insurance (80%). Comparable profiles were described in African caregivers of children with SCD, where 80 to 89.8% were mothers with low occupational status, in households living with less than USD 120 per month in 50.7% of the cases; putting caregivers under huge stress due to inability to cover SCD patient’s needs [12, 17, 19].

Financially, the estimated cost of caregiving (400 SAR = USD 107 in average) was relatively high compared with the socioeconomic conditions of the participants’ families attributing the lowest satisfaction score for financial situation, out of 13 other life domains. Studies from Nigeria, where SCD is highly prevalent, reported an average USD 333 of monthly health expenditure in households with a child afflicted with SCD; which represented up to 34.4% of the family income and significantly impacted finances of 58.2% of the families [9, 23]. Financial stress was described as a significant factor for caregivers difficulty coping with their afflicted children; and the psychosocial impact of the financial burden was more remarkable during SCD crises [2, 23].

On the other hand, most of the tasks and responsibilities related to the caregiving were home-centered and undertaken by the exclusive caregivers. The activities included helping patients to take medications, assisting them during recurrent disease complications and intermittent crises, carrying them to the clinics and hospitals for recurrent blood transfusions or periodic visits, as well as communicating with hospital and school. Consequently, more than half of the interviewees declared lack of time for to take of themselves, exhibiting low participation rates in various daily activities. Similar results were reported in Netherlands, where data indicated a limitation in daily activities of caregivers in relation with the extensive care schedule; including hospital visits, emergency crises and other duties related to SCD child condition [11]. If the importance of the caregiver’s role seems apparent in case of young patients, it was demonstrated to be a significant factor for improving adherence to care even for adolescent patients, who have greater level of autonomy and involvement in their own care [24]. Paradoxically, although being challenging for caregivers, frequent hospital visits for regular blood transfusion therapy are associated with a better QoL of the diseased child. All these factors constitute a permanent pressure on caregivers, which may affect their mental and physical wellbeing, compromising their quality of life and negatively affecting their behaviors and self-respect [19].

Majority of the interviewees (80.9%) declared being fairly or highly satisfied of the level of care provided within the family to their afflicted child. In a society like Saudi Arabia, which is characterized by strong family and spiritual values, home and family-centered care may be a determinant of success in the continuous care of patients with special needs. This is demonstrated by psychological (and financial) support received by caregivers mostly coming from their natural social environment; especially from partners, other family members, friends, and neighbors. Further, life domain satisfaction questionnaire revealed that caregivers are best satisfied of their relations with their children, other relatives’ partners and friends. The relationship between parents and their other, non-diseased, children (siblings of the diseased child) could be dominated by a shift in attention toward the diseased child, a protective attitude regarding the siblings from the pain of their brother/sister, and or their initiation to his/her care; and siblings may develop supportive and responsible attitude in that regard [25].

Assessment of the QoL showed that caregivers of children with SCD have impaired sleep quality and unsatisfactory emotional and sexual life but relatively conserved social and professional achievements. In addition, emotional assessments showed high incidence of anxiousness and feeling of exhaustion and negative trends regarding fitness and energetic feeling; although, a positive trend was observed regarding both happiness and joyfulness feelings. Comparable data were found in two Nigerian studies, where authors reported high proportion of feeling of unhappiness, expressed depressive symptoms, and 2% of suicidal ideations in caregivers. A close monitoring was then recommended with anti-depressive medication [17, 19]. A French study conducted in 2007 evaluated the incidence of post-traumatic stress disorder in 11 children with SCD and their parents following painful crises episodes. The study demonstrated the presence of post-traumatic stress disorders (PTSD) in both afflicted children and their parents. The authors recommended to conduct few more similar studies to address the relation between SCD painful crises and PTSD [15]. In Netherlands, a higher incidence of depressive moods and feeling of unhappiness was found among caregivers which was associated to marked guilt feeling and fear from having another sick child [11]. Furthermore, the same study reported decreased motor and cognitive functioning in caregivers, which was attributed to the lack of sleep resulting from the frequently continuous caregiving, associated sometimes to interrupted sleep pattern, a lack of vigor and vitality [11]. These data are consistent with our findings showing a positive correlation between sleep quality and cognitive skills supposing significant impact of bad sleep quality on cognitive functioning. On the other hand, sleep quality of the caregivers significantly improved in families with more number of children; which was also significantly correlated with more positive emotions among caregivers. These observations further support the effective role siblings can play in taking care of their diseased brother/sister; and suggest that sharing the caregiving with the other family members may be a crucial factor to preserve the QoL of the caregivers, in the long term.

The primary limitation of our study was difficult to reach all patients’ caregivers, due to unavailability of contact details in the patients’ files, in addition to the relatively short timeframe of the study; which resulted in the low participation rate and small sample size. Other aspects limiting generalization of the results include the single-center design, convenience sampling, and non-validation of the Arabic version of the tool. Further, approaching the QoL dimensions with interview-based survey may increase subjectivity in responses and expose to social desirability bias.

This study was an extension to the previous studies that highlighted the impact of chronic diseases on the families & caregivers of SCD children. The findings of this study demonstrated the huge financial and emotional burdens on the caregivers, who are in need of significant attention and focus in solving their psychosocial difficulties, in order to allow them to give more attention to their sick children and help them to cope up with their illness. The rich social and cultural features of the Saudi society constitute helpful resources for the caregivers of children with SCD, with potentially effective financial and psychological support; however, this should not downplay the role of public institutions and authorities in providing these families with the helping facilities and the required support.

Physicians and healthcare providers should be aware of the huge burdens on caregivers and orient them towards appropriate solutions, to help them meet their needs and improve their QoL and that of their diseased children.

The creation of organizations, groups and networks should be encouraged to support the SCD patients and their families, offering them an environment of free exchange and expression about their daily problems and sufferings and providing them with the useful advice and eventual financial support. Further studies are warranted in Saudi Arabia to investigate the psychosocial burden of SCD on the caregivers.