Smith, Robyn2018-07-102018-07-102017https://hdl.handle.net/10539/24873A thesis submitted to the Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, in fulfilment of the requirements for the degree of Doctor of Philosophy. Johannesburg, 2017.Over recent decades medical and surgical advances have significantly lowered the mortality rate for children born with congenital heart defects. Congenital heart disease (CHD) survivors are at high-risk of growth retardation and developmental morbidity that negatively affect their health-related quality of life (HRQOL). In addition, caring for a child with a chronic health condition such as CHD places a considerable financial and emotional burden on parents, putting them at risk of ongoing stress and psychological morbidity including anxiety and depression. The outcomes of children living with CHD and their families in South Africa (SA) are unknown. Outcomes for children with CHD in SA are likely to be further complicated by social disadvantage and Human Immunodeficiency Virus (HIV) co-infection. The aim of this observational descriptive study (Phase I and II) was to determine the pre-cardiac intervention, and three-month and six-month post-cardiac intervention development, growth, HRQOL and parenting stress outcomes of young children with CHD in central SA. Outcomes were compared over time, and variables associated with development, HRQOL and parenting stress outcomes determined. In addition, the developmental needs of young children living with CHD in central SA were to be identified. In order to meet the Phase I and II objectives, forty-eight consecutive children, 30 months and younger, and their parents were recruited into this study at the Universitas Academic Hospital in Bloemfontein. Children who had previous or emergency cardiac surgery were excluded. Development was assessed using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III), HRQOL using the Paediatric Quality of Life Inventory (PedsQLTM) and levels of parenting stress using the Parenting Stress Index Short-Form (PSI-SF). Growth outcomes were determined by z-scores calculated for growth parameters. Medical severity of the cardiac disease was rated by a paediatric cardiologist using the Cardiologists Perception of Medical Severity Scale. Baseline data was collected for 40 children. The majority of children (n=26) underwent open-heart surgery in infancy with cardiopulmonary bypass. Most children (n=30) had moderate disease severity, with twenty percent (n=8) having cyanotic heart defects. A quarter of the children (n=10) had Down syndrome (DS). Surgical outcomes were comparable to those reported in developed countries, with a mortality rate of 15%. There was a high attrition rate during Phase II of this study, with 47.5% of children and their families missing one or more follow-up visit. Mothers fulfilled the role of primary caregiver, and carried most of the burden of care. The majority of families were from a low socioeconomic backgrounds (87.5%) and mothers had low levels of education, with only 40% having graduated high school. The majority (68%) of children had suboptimal growth prior to cardiac intervention. There was significant growth catch-up for both weight (p=0.04) and head circumference (p= 0.02) by the six-month post-cardiac intervention. Complete catchup growth had not yet taken place by the six-month post-cardiac intervention, with 40.9% of the children still presenting with malnutrition. The growth trends of children with CHD with DS were found to be similar to those of children with CHD without DS. Growth in children with cyanotic heart defects tended to be poorer both before and after cardiac intervention. There was a high prevalence of moderate developmental delay across all development domains. Motor delays (27.5%) were most prevalent prior to cardiac intervention. Motor performance improved with age and post cardiac intervention, but language and cognitive performance declined with age and increasing skill complexity. There was not a significant change in the developmental outcome of the children over the timespan of this study. The developmental outcome for children with cyanotic heart defects tended to be similar to those with acyanotic heart defects. The presence of DS was significantly (p<0.001) associated with developmental outcome across all developmental domains at all time-points of assessment. Children with CHD with DS tended to have considerably poorer developmental outcomes compared to children with CHD without DS. Disease severity (p=0.02) and maternal age (p=0.01) were significantly associated with cognitive development. Age at first cardiac surgery was found to be significantly associated with language development both before cardiac intervention (p<0.01) and at three-month post-cardiac intervention (p=0.04). Suboptimal growth prior to cardiac intervention (p=0.04) and maternal age (p<0.001) were significantly associated with motor development. Developmental performance was well below the test mean on all subscales of the BSID-III at all the time points of assessment. Although the patterns of development and the prevalence of developmental delays in the current study were similar to those reported in developed countries, children living with CHD in central SA performed below the expected developmental levels for children with CHD when assessed on the BSID-III. Hypotonia was the most significant abnormal neurological finding, with 45% of the children presenting with hypotonia prior to cardiac intervention. The hypotonia tended to resolve in the children without DS by the six-month post-cardiac intervention. Overall parents’ perceived their children’s HRQOL as being relatively good, and similar to that of their healthy same-aged peers and other children with CHD in developed countries. Parents’ perception of their children’s HRQOL improved significantly after cardiac intervention (p= 0.04). Perceived HRQOL tended to be similar for children with cyanotic and acyanotic heart defects. Parents of children with CHD with DS tended to perceive their children’s HRQOL as poorer when compared with parents of children with CHD without DS. Motor development (p=0.01) and levels of parenting stress (p=0.02) were significantly associated with parents’ perceptions of their children’s HRQOL prior to cardiac intervention. The majority of parents’ (60%) experienced clinically significant levels of stress prior to their children undergoing cardiac intervention. Parenting stress decreased significantly from pre-cardiac intervention levels at both three-month (p<0.001) and six-month (p<0.001) post-cardiac intervention as the child’s cardiac symptoms resolved or decreased, and their health status improved. Parents of children with cyanotic and acyanotic heart defects tended to experience similar levels of stress. Parents of children with CHD with DS tended to experience higher levels of ongoing stress when compared with parents of children with CHD without DS. Parenting stress prior to cardiac intervention was significantly associated with parents’ perception of their child’s HRQOL (p=0.02) and language development (p=0.04). Parenting stress at threemonth post-cardiac intervention was significantly associated with age at first cardiac surgery (p=0.03), language development (p=0.03) and level of maternal education (p=0.04). HRQOL and parenting stress outcomes were closely linked before cardiac intervention. Parents perceiving their child as having a poor ability to function in everyday situations experienced increased stress levels. Based on developmental performance on the BSID-III 59% of the children in the current study would qualify for referral to early intervention (EI) services including physiotherapy, occupational therapy and speech therapy, with many children requiring access to more than one service. A home-based parent-driven developmental activity programme would likely be best suited to meet the developmental needs of children with CHD living in central SA taking into account the geography of the area and service delivery challenges in the public healthcare sector. Phase III of the study resulted in the development of a home-based developmental activity programme to meet the identified developmental needs of children with CHD in central SA. Qualitative methods, including an expert panel of rehabilitation professionals and a focus group of parents, were used to gain consensus on the content of the developmental activity programme. In conclusion, it is encouraging that the longer-term outcomes of children with CHD in central SA were not vastly different from those of children in developed countries. The greater extent of the growth retardation and developmental delay of the children in the current study is however of concern. The findings in this study strongly support the implementation of a cardiac neurodevelopmental programme as part standard cardiac care in SA. Early developmental intervention and psychosocial support services are indicated to optimise the outcome for both children living with CHD and their families. A home-based parent-driven developmental stimulation programme provides an innovative approach to meeting the developmental needs of young children living with CHD. Keywords Congenital heart disease, neurodevelopment, growth, parenting stress, health-related quality of life, home-based developmental activity programme, Bayley Scales of Infant and Toddler Development, Third Edition, Paediatric Quality of Life Inventory, Parenting Stress Index-Short Form.enQuality of LifeHeart Defects, CongenitalChildThesis