Health‐related quality of life in children with myelomeningocele: a systematic review of the literature

Abstract

Myelomeningocele (MMC) is the most complex congenital birth defect compatible with life. To provide the best health care for children with MMC, clinicians and researchers have to understand health and functional status of their patients as well as factors influencing their quality of life (QOL). The objective is to review studies that assess health-related quality of life (HRQOL) and determinants of HRQOL in children with MMC. The data sources include MEDLINE, along with reference lists of relevant reviews and included articles. Study eligibility criteria are quantitative studies that assess HRQOL using validated HRQOL instrument. Participants include children aged 0-18 years with diagnosis of MMC or spina bifida. Risk of bias was assessed using the Hayden system for non-randomized studies. Narrative synthesis and tables of results of HRQOL studies were conducted. Children with MMC have decreased HRQOL compared with normative population. Physical HRQOL is the most exposed aspect of QOL, while discrepancies in psychosocial domains vary by study. Many determinants of QOL have been found, and it is still not clear which determinant can be described as the strongest predictor. Psychosocial factors are the most consistent determinants of HRQOL.Heterogeneous study sample size, study designs, determinant and outcomes measures limited synthesis of findings. The HRQOL in children with MMC is complex and mediated by a number of associated medical problems, together with a variety of psychosocial and environmental factors. Future research is needed (a) on the relation between complex medical problems, functional independence, parent's and clinician's expectation and HRQOL in children and (b) to assess the differences in HRQOL by social environment and healthcare system.

Language and Latino immigrants living with spina bifida: Social determinants of health – the missing dimension in quality of life research

“…Along with physical domains, a number of additional factors were associated with lower HRQOL scores; the investigation and understanding of these factors as a basis to develop interventions designed to improve QOL emerged as an important pursuit. Consistent with previous reviews of HRQOL in patients with SB [26,48], our findings demonstrated that most studies were cross-sectional in nature and described varying degrees of HRQOL divergence on parent and selfreported scores related to emotional, social, and school domains (Table 1). Moreover, a generic measure of QOL was utilized by the majority of studies (n = 15/18; 83%), of which two were supplemented with the use of the SB-HRQOL [43,44].…”

“…In the US, 25% of children live in immigrant families [23], and of the estimated 55 million Hispanics/Latinos, nearly three of every four speaks Spanish at home while remaining the fastest-growing demographic group [24,25]. In this setting, previous systematic reviews of health-related QOL (HRQOL) assessments have called for the exploration of social environments and HRQOL [26]. We sought to examine if appropriately validated instruments, which take into account cultural context, are available and the extent of their use to assess QOL in this historically underserved US population.…”

Estimates of global and regional prevalence of neural tube defects for 2015: a systematic analysis

“…Neural tube defects (NTDs) are a group of severe congenital disorders associated with substantial mortality, morbidity, long‐term disability, and psychological and economic costs . Many NTDs are preventable with folic acid, and long‐term survival and quality of life among those living with NTDs can be improved through access to appropriate clinical care and rehabilitative services . However, efforts for primary prevention and addressing the needs of those living with NTD have been hampered by a lack of transparent prevalence estimates to quantify the burden, especially in resource‐poor settings.…”

Ceramide: a novel inducer for neural tube defects

“…Anencephaly, failed closure of the anterior neuropore, and spina bifida, failed closure of the posterior neuropore, are the two most common NTDs. While nearly all babies affected by anencephaly die in utero or shortly after birth, those affected by spina bifida typically survive, but endure mild to severe intellectual, neurologic, and/or physical disabilities throughout their lives . The emotional and financial burden of such birth defects is often overwhelming, with average management costs for this lifelong defect exceeding $700 000 per patient…”