Life Inventory Family Impact Module Research Articles

Supporting caregivers of children living with disability in a humanitarian context: realist-informed evaluation of the ‘Mighty Children’ programme in Afghanistan

IntroductionTo ensure that humanitarian action is disability-inclusive, evidence is needed to understand how different strategies to support children living with disabilities and their families can work in these settings. Evidence...

Beyond the Diagnosis: Evaluation of Quality-of-Life Measures and Family Functioning in SLC6A1-Related Neurodevelopmental Disorder

BackgroundSLC6A1-related neurodevelopmental disorder (SLC6A1-NDD) is a rare genetic disorder linked to autism spectrum disorder, epilepsy, and developmental delay. In preparation for future clinical trials, understanding how the disorder impacts patients and their families is critically important. Quality-of-life (QoL) measures capture the overall disease experience of patients. This study presents QOL findings from our SLC6A1-NDD clinical trial readiness study and the Simons Searchlight SLC6A1-NDD registry. MethodsWe compiled QoL data from participants with SLC6A1-NDD enrolled in our clinical trial readiness study (n = 20) and the Simons Searchlight registry (n = 32). We assessed the distribution of scores on the Quality-of-Life Inventory-Disability (QI Disability), Quality of Life of Childhood Epilepsy (QOLCE-55), and Pediatric Quality of Life Inventory Family Impact Module (PedsQL-FIM) administered to caregivers. ResultsIn our cohort of 52 participants, the mean QI Disability total score was 73 ± 12.3, the QOLCE-55 mean total score was 49 ± 17.1, and the mean total PedsQL score was 51 ± 17.6. Longitudinal QoL scores for a subset of participants (n = 7) demonstrated a reduction in the Family Relationship domain of PedsQL-FIM (Δ-10.0, P = 0.035). Bootstrap resampling of total scores displays nonoverlapping 95% confidence intervals for the 10th, 50th, and 90th percentiles on all three measures. ConclusionsThis is the first study to investigate QoL measures for SLC6A1-NDD. Findings suggest that scores within the 10th percentile's confidence interval could be clinically significant, referring to QI-Disability scores of <61, QOLCE-55 scores of <46, and PedsQL-FIM scores of <42. Future validation studies are needed.

A quantitative cross-sectional study of the burden of caring for patients with Lennox-Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex-associated epilepsy in Japan

IntroductionLennox-Gastaut syndrome (LGS), Dravet syndrome (DS), and tuberous sclerosis complex (TSC)-associated epilepsy are rare conditions associated with severe childhood-onset epilepsy. Caregivers play a critical role in the patients’ care and may experience significant psychosocial and socioeconomic burden. This cross-sectional study determined the burden of caring for patients with these rare epilepsy conditions in Japan. MethodsA quantitative online survey was used to assess patients’ and caregivers’ characteristics and the caregivers’ emotional state, among others. Several validated questionnaires were used: the Hospital Anxiety and Depression Scale (HADS; 0–21 score) assessed the caregivers’ emotional wellbeing, the Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM; 0–100 score) assessed the health-related quality of life (HRQoL) of the caregivers and their families, and the Work Productivity and Activity Impairment General Health (WPAI:GH; 0–100 % score) questionnaire assessed work productivity. ResultsA total of 36 caregivers responded (median [interquartile range (IQR)] age 43.5 [39.5, 48.3] years; 33/36 [92 %] female; 13/36 [36 %] working part-time and 13/36 [36 %] not working). Participants cared for 7/36 (19 %), 19/36 (53 %), and 10/36 (28 %) patients with LGS, DS, and TSC, respectively (median [IQR] age, 11.0 [6.8, 16.3] years; age at first seizure, 0 [0, 0] years). Patients received a median (IQR) of 4 (3, 5) treatment drug types. Patients experienced median (IQR) 3.0 (0, 21.0) epileptic seizures in the previous week; 28/36 (78 %) had severe intellectual disabilities, and 34/36 (94 %) had developmental delays. Caregivers reported stress (17/36 [47 %]), sleep problems (13/36 [36 %]), and anxiety (12/36 [33 %]). They spent a median (IQR) of 50.0 (17.5, 70.0) hours caregiving in the previous week, with 3.0 (1.0, 11.0) hours of seizure-specific care. Caregivers reported that their lives would be easier with a median (IQR) of 1.5 (0, 5.0) hours fewer per week caring for patients during/following seizures. Median HADS scores were 9.5 (‘suspected anxiety diagnosis’) and 7.5 (‘no depression’) for caregivers, and PedsQL FIM Total median score was 60.1, indicating HRQoL impairment for the caregiver and their family. WPAI:GH scores for paid workers indicated important work impairment. Higher caregiving hours (≥ 21 h vs. < 21 h in the previous week) resulted in higher caregiver burden as indicated by the HADS Total score (p = 0.0062) and PedsQL FIM Total score (p = 0.0007). ConclusionsCaregivers of patients with LGS, DS, or TSC in Japan experience a significant time burden, reduced HRQoL, and high level of work/activity impairment. Caregivers provide round-the-clock care to patients and rely on family and specialized caring services to help manage the increased caregiving time, which tends to be associated with greater emotional burden and HRQoL impact.

Protocol for a randomized controlled trial to evaluate a year-long (NICU-to-home) evidence-based, high dose physical therapy intervention in infants at risk of neuromotor delay.

Developmental disabilities and neuromotor delay adversely affect long-term neuromuscular function and quality of life. Current evidence suggests that early therapeutic intervention reduces the severity of motor delay by harnessing neuroplastic potential during infancy. To date, most early therapeutic intervention trials are of limited duration and do not begin soon after birth and thus do not take full advantage of early neuroplasticity. The Corbett Ryan-Northwestern-Shirley Ryan AbilityLab-Lurie Children's Infant Early Detection, Intervention and Prevention Project (Project Corbett Ryan) is a multi-site longitudinal randomized controlled trial to evaluate the efficacy of an evidence-based physical therapy intervention initiated in the neonatal intensive care unit (NICU) and continuing to 12 months of age (corrected when applicable). The study integrates five key principles: active learning, environmental enrichment, caregiver engagement, a strengths-based approach, and high dosage (ClinicalTrials.gov identifier NCT05568264). We will recruit 192 infants at risk for neuromotor delay who were admitted to the NICU. Infants will be randomized to either a standard-of-care group or an intervention group; infants in both groups will have access to standard-of-care services. The intervention is initiated in the NICU and continues in the infant's home until 12 months of age. Participants will receive twice-weekly physical therapy sessions and caregiver-guided daily activities, assigned by the therapist, targeting collaboratively identified goals. We will use various standardized clinical assessments (General Movement Assessment; Bayley Scales of Infant and Toddler Development, 4th Edition (Bayley-4); Test of Infant Motor Performance; Pediatric Quality of Life Inventory Family Impact Module; Alberta Infant Motor Scale; Neurological, Sensory, Motor, Developmental Assessment; Hammersmith Infant Neurological Examination) as well as novel technology-based tools (wearable sensors, video-based pose estimation) to evaluate neuromotor status and development throughout the course of the study. The primary outcome is the Bayley-4 motor score at 12 months; we will compare scores in infants receiving the intervention vs. standard-of-care therapy.

Structural Relationship between Family Functioning and Parental Health-Related Quality of Life among Parents with Down Syndrome Children in Kelantan, Malaysia

Caregiving and living with a Down Syndrome (DS) child is distressing and has given a negative impact on the quality of life of parents. The study aims examine the parents with DS children on the health-related quality of life (HRQoL) and family functioning and to identify potential factors affecting them. The cross-sectional study involved 94 parents with DS children from the Paediatric Clinic, Hospital Raja Perempuan Zainab II, Kota Bharu, Malaysia, with a mean age of 46.7 ± 7.0 years. Demographic and socioeconomic data of the parents and DS children were collected, and their quality of life (QoL) was measured based on the self-reported Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM). Parents of DS children in the current study have shown good HRQoL and family functioning, with scores of 83.2 and 89.3 out of 100, respectively. The family functioning score shown significantly correlated with the parental health-related quality of life (p&lt;0.001). The number of family members was found to have a statistically significant effect on family functioning (p=0.015). The lowest score in the HRQoL was in the domain of ‘worry’. The findings of the current study verify previous reports of relatively good quality of life and family functioning in families with DS children and also with other disabilities.

Quality of Life and Family Impact in Down Syndrome, Autism Spectrum Disorder, and Co-occurring Down Syndrome and Autism Spectrum Disorder.

Families of children with neurodevelopmental disorders have developmental, behavioral, and social-emotional needs that affect quality of life (QoL). This study assesses the validity and utility of a caregiver QoL measure; characterizes QoL in families with children with Down syndrome (DS), autism spectrum disorder (ASD), and a dual diagnosis of DS and ASD (DS + ASD); and compares and explores differences in QoL based on diagnosis. Caregivers of children and adolescents with ASD (n = 610) and DS (n = 177) completed the Pediatric Quality of Life Inventory Family Impact Module 2.0, yielding overall, parent functioning, family functioning, and subscale scores, and a Parent Global Impression (PGI) rating. An ASD cohort (n = 177) was sex matched to the DS cohort (n = 177) to mitigate potential sex bias. Additional analyses compared these groups with children and adolescents with DS + ASD (n = 37). Analyses showed that the Pediatric Quality of Life Inventory was valid and reliable in DS, ASD, and DS + ASD populations. No differences were reported in PGI ratings among groups. Caregivers in the DS group demonstrated higher QoL and family functioning compared with the ASD and DS + ASD groups. The DS group reported significantly better Emotional Functioning and Communication and less Worry than the ASD group. Compared with the ASD group, caregivers of the DS + ASD group indicated more concerns with Physical Functioning. Notably, the DS + ASD group had significantly lower levels of QoL than the DS group in nearly all caregiver functioning domains. This study highlights differences in QoL within and between neurodevelopmental disorder groups, which may help identify families requiring additional support, advocacy, and community engagement.

Psychometric properties of the Spanish version of the Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM)

IntroductionThis study analysed the psychometric properties of the Spanish version of the Pediatric Quality of Life Questionnaire Family Impact Module (PedsQL FIM) in the Argentinian population. Patients and MethodsThe sample included 232 caregivers, of who 108 were parents of children with chronic diseases (mean, 9.54; standard deviation [SD], 4.43) and 124 parents of children in the general population (mean, 12.37; SD, 4.6). ResultsWe assessed the validity of the instrument with the known-groups method, finding significant differences between the case and control groups in the overall and subscale scores (P < .01). We also assessed test validity by means of exploratory factor analysis, which yielded an 8-factor model that explained 74.03% of the variance. We assessed reliability with the Cronbach alpha and found a high internal consistency (α=0.95). ConclusionThe PedsQL module proved to be a valid and reliable tool to assess the impact of a chronic paediatric condition on caregiver quality of life and family functioning.

Cardiac rhythm devices in pediatric patients: Impact on family functioning and parent's health-related quality of life.

This study compares the impact of children's cardiac rhythm devices and health status on their parents with that of healthy controls. Furthermore, it aims to ascertain whether sociodemographic characteristics and medical data are associated with parent-reported impacts. This cross-sectional study is part of a comprehensive single-center study of long-term psychosocial outcomes in pediatric patients with pacemakers and implantable cardioverter defibrillators. The study includes 69 patients with their parents: 69 mothers and 57 fathers. Parents responded to the Pediatric Quality of Life Inventory Family Impact Module and to open-ended questions assessing impact on family life and treatment satisfaction. Parents reported more negative impact on family life than healthy controls in all three summary scores. Among fathers, presence of a child's congenital heart disease and female sex is associated with lower family function. No group differences emerged regarding device type. Positive and negative cognitive aspects predominated for patients' mothers and fathers. However, one substantial difference is that mothers reported more positive and negative emotional impact than fathers. We conclude that parents' well-being should be addressed in clinical contexts, especially through emotional and practical support and open communication focused on parents' worries and concerns.

Predictors of family impact of juvenile localized scleroderma.

To measure the impact juvenile localized scleroderma (jLS) has on family quality of life and to identify predictors of family impact in this population which may inform the development of tailored resources to enhance family functioning for patients with jLS. A retrospective cohort study of pediatric patients with jLS and their families was conducted. Five questionnaires were administered at each visit: Pediatric Quality of Life Inventory Family Impact Module (PedsQL-FIM), PedsQL 4.0 Generic Core Scales (PedsQL-Generic), PedsQL Rheumatology Module (PedsQL-RM), Child Health Assessment Questionnaire (CHAQ), and Children's Dermatology Life Quality Index (CDLQI). Linear mixed models with random intercepts for each patient were used to find relationships between family impact scores and clinically relevant variables over time. Variables of interest included disease activity status, methotrexate use, jLS distribution, and scores for PedsQL-Generic and PedsQL-RM. The median baseline PedsQL-FIM total score was 80.9 (IQR=76.6-97.4). Adjusting for age and sex, the most significant predictors of family impact were PedsQL-Generic scores and four of five PedsQL-RM dimensions (all P<.001); methotrexate use had borderline significance (P=.06). Family impact increased more significantly over time in older patients. In multivariable modeling, PedsQL-Generic total score and jLS "other" distribution were significant for predicting an increased PedsQL-FIM score (P=.003 and P=.03, respectively). JLS has a moderate family impact. Family impact is predicted by patients' general and disease-specific health-related quality of life (HRQL) and their jLS subtype. There is a trend toward increased family impact with methotrexate treatment. This study emphasizes the importance of family-centered care in jLS.

Health-Related Quality of Life and Family Functioning of Primary Caregivers of Children with Cerebral Palsy in Malaysia.

Caregiving for children with cerebral palsy (CP) has proved to negatively impact on the physical and psychological well-being of their primary caregivers. The aim of the current study was to examine the overall impact of caregiving for children with CP on the primary caregivers’ health-related quality of life (HRQOL) and family functioning, and to identify potential factors associated with primary caregivers’ HRQOL and family functioning. The cross-sectional study involved a total of 159 primary caregivers of children with CP with a mean age of 42.8 ± 8.4 years. Demographic data and information on the physical and leisure activities of the primary caregivers were collected, and their quality of life (QOL) was measured based on the self-reported Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM). Primary caregivers in the current study have shown good HRQOL and family functioning, with scores of 82.4 and 85.3 out of 100, respectively. Through multiple linear regression analyses, the mother’s level of education, family monthly income, sleeping problems in children with CP, and the existence of children with other types of disability have been identified as factors contributing to HRQOL and family functioning. The findings help set out the course for stakeholders to establish action to enhance the QOL of primary caregivers.

Quality of life of children with spinal muscular atrophy and their caregivers from the perspective of caregivers: a Chinese cross-sectional study

BackgroundSpinal muscular atrophy (SMA) is an autosomal-recessive motor neuron disease leading to dysfunction of multiple organs. SMA can impair the quality of life (QoL) of patients and family. We aimed to evaluate the QoL of children with SMA and their caregivers and to identify the factors associated with QoL in a cross-sectional study conducted in China.MethodsWe recruited 101 children aged 0–17 years with SMA and their caregivers from a children’s hospital in China. Twenty-six children had type I SMA, 56 type II and 19 type III. Each child’s QoL was measured by the Pediatric Quality of Life Inventory 3.0 Neuromuscular Module (PedsQL NMM), which was completed by the child’s caregivers. The caregiver’s QoL was measured by the Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM). Information on sociodemographic characteristics, disease-specific characteristics, and treatments were collected using the proxy-reported questionnaire. Two-sample t tests and one-way ANOVA were used to compare differences in average scores of QoL across subgroups.ResultsChildren with type III SMA had a higher average Total score of PedsQL NMM and higher average scores in domains Neuromuscular disease and Family resources than children with type I or type II SMA (p < 0.001). Caregivers of children with type III SMA reported higher average scores in the domains of Physical, Emotional, Social, and Cognitive functioning of the PedsQL FIM than those of children with types I or II SMA (p < 0.05). In addition, disease-related characteristics (e.g. limited mobility, stable course of disease, skeleton deformity, and digestive system dysfunction) and respiratory support were associated with lower average scores of PedsQL NMM and PedsQL FIM (p < 0.05). Exercise training, multidisciplinary team management and use of the medication Nusinersen were each associated with higher average scores in both PedsQL NMM and FIM (p < 0.05).ConclusionOur study has demonstrated factors that may impair or improve QoL of children patients with SMA and their parents. Particularly, QoL was relatively poor in children with type I and type II SMA as well as in their caregivers compared to those with type III SMA. We strongly recommend that standard of care in a multidisciplinary team be strengthened to improve the QoL of SMA patients. Our study called for increased attention from clinical physicians on measuring QoL in their clinical practices in order to enhance the understanding of impacts of SMA and to make better decisions regarding treatment.

A Prospective Study of Parent Health-Related Quality of Life before and after Discharge from the Neonatal Intensive Care Unit

To determine how infant illness and parent demographics are associated with parent health-related quality of life (HRQL) during and 3months after hospitalization in the neonatal intensive care unit (NICU). We hypothesized that parents of extremely preterm infants would report lower NICU HRQL than other parents, and that all parents would report improved HRQL after discharge. This prospective study of parent-infant dyads admitted to a level IV NICU for ≥14days from 2016 to 2017 measured parent HRQL before and 3months after discharge using the Pediatric Quality of Life Inventory Family Impact Module. Multivariable regression was used to identify risk factors associated with HRQL differences during hospitalization and after discharge. Of the 194 dyads, 167 (86%) completed the study (24% extremely preterm; 53% moderate to late preterm; 22% term). During the NICU hospitalization, parents of extremely preterm infants reported lower adjusted HRQL (-7 points; P=.013) than other parents. After discharge, parents of extremely preterm infants reported higher HRQL compared with their NICU score (+10 points; P=.001). Tracheostomy (-13; P=.006), home oxygen (-6; P=.022), and readmission (-5; P=.037) were associated with lower parent HRQL 3months after discharge, adjusted for NICU HRQL score. Parents of extremely preterm infants experienced a greater negative impact on HRQL during the NICU hospitalization and more improvement after discharge than parents of other infants hospitalized in the NICU. Complex home care was associated with lower parent HRQL after discharge. The potential benefit of home discharge should be balanced against the potential negative impact of complex home care.

Quality of life of the family of children with asthma is not related to asthma severity

The quality of life for the family is an important outcome of childhood asthma. The aim of the study was to describe the quality of life in families who have a child with asthma. The Pediatric Quality of Life Inventory Family Impact Module was completed by the parents of 527 children with asthma. The median overall score was 75.0 (interquartile range 63.9, 87.5). The following factors were independently associated with lower quality of life: additional difficulties such as anxiety and financial hardship (3.81 [2.45, 5.93]), waking with asthma symptoms one or more nights a week (odds ratio 2.53 [1.34, 4.75]), regular use of symptoms reliever medication (2.47 [1.57, 3.87]), and female gender (1.97 [1.27, 3.05]). Lower socioeconomic status of the family and exposure to molds at home doubled the odds for lower quality of life. Physician's diagnosed asthma severity and control were associated with quality of life in univariate, but not multivariate analysis.Conclusion: Multiple factors, several of which are not related to asthma, contribute to the family burden of having a child with asthma. Clinicians should be mindful of the impact of asthma on the child and the family, and consider exploring factors not directly related to childhood asthma. What is Known: • Childhood asthma as a chronic disease impacts the quality of life of the patient, but there is also an impact on the immediate family. • There are relatively few studies exploring the quality of life of parents of a child with asthma; the results are heterogeneous and none has been carried out in an Eastern European country. What is New: • This is the first study to describe caregiver's quality of life in an Eastern European population in the context of childhood asthma. • The quality of life of the family of asthmatic child depends not only on factors related to asthma, but also non-asthma related factors such as poverty which play even more important role.

Children with cerebral palsy in Ghana: malnutrition, feeding challenges, and caregiver quality of life

To assess feeding difficulties and nutritional status among children with cerebral palsy (CP) in Ghana, and whether severity of feeding difficulties and malnutrition are independently associated with caregiver quality of life (QoL). This cross-sectional survey included 76 children with CP (18mo-12y) from four regions of Ghana. Severity of CP was classified using the Gross Motor Function Classification System and anthropometric measures were taken. Caregivers rated their QoL (using the Pediatric Quality of Life Inventory Family Impact Module) and difficulties with eight aspects of child feeding. Logistic regression analysis explored factors (socio-economic characteristics, severity of CP, and feeding difficulties) associated with being underweight. Linear regression was undertaken to assess the relationship between caregiver QoL and child malnutrition and feeding difficulties. Poor nutritional status was common: 65% of children aged under 5 years were categorized as underweight, 54% as stunted, and 58% as wasted. Reported difficulties with child's feeding were common and were associated with the child being underweight (odds ratio 10.7, 95% confidence interval 2.3-49.6) and poorer caregiver QoL (p<0.001). No association between caregiver QoL and nutritional status was evident. Among rural, low resource populations in Ghana, there is a need for appropriate, accessible caregiver training and support around feeding practices of children with CP, to improve child nutritional status and caregiver well-being. Malnutrition is very common among children with cerebral palsy in this rural population in Ghana. Feeding difficulties in this population were strongly associated with being underweight. Feeding difficulties were associated with poorer caregiver quality of life (QoL). Child nutritional status was not associated with caregiver QoL.

Benefits of Medical Home Care Reaching Beyond Chronically Ill Teens: Exploring Parent Health-Related Quality of Life

BackgroundCaring for teens with special health care needs places physical and mental health burdens on parents, which can be exacerbated by the stresses of transitions to independence. Medical homes can improve teen transitions to greater self-management and reduce health care–related time and financial burdens for families. We examined the association between parent-reported teen medical home status and caregiver health-related quality of life (HRQOL). MethodsThe study sample included parents or caregivers of teens with special health care needs aged 15 to 18 recruited from a pediatric Medicaid accountable care organization who participated in a survey (response rate, 40.5%). The primary outcome was parent HRQOL scores (0–100 points) measured using the Pediatric Quality of Life Inventory Family Impact Module. Medical home status was based on parent report of teen's health care meeting medical home criteria. Linear regression models were used to estimate HRQOL scores, adjusted for demographic characteristics, health literacy, and teen functional limitation. ResultsAmong 488 parents, 27% reported their teen received care consistent with a medical home. Adjusted parent HRQOL scores were significantly higher among those whose teens had a medical home (74.40; 95% confidence interval, 71.31–77.48), relative to those whose teens did not (65.78; 95% confidence interval, 63.92–67.65). Medical home subscale analyses showed HRQOL scores had significant positive associations with family-centered care and coordinated care, but not other subscales. ConclusionsTeen medical home status was positively associated with caregiver HRQOL, suggesting that the medical home may benefit overall caregiver well-being. In particular, receiving care that was family centered and coordinated appeared to be the most beneficial.

Family impact of acquired brain injury in children and youth.

To assess the parental view on the impact of pediatric traumatic brain injury (TBI) and nontraumatic brain injury (NTBI) on the family and its determinants. Follow-up study including parents of children with a hospital-based diagnosis of acquired brain injury (ABI) aged 4-20 years at onset of ABI. Parents completed the Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM), which measures Parent Health-Related Quality of Life, Family Functioning, Communication, and Worry. Additional assessments included the Pediatric Stroke Outcome Measure (PSOM), the Child & Family Follow-up Survey (CFFS), PedsQL General Core and Multiple Fatigue Scales, and sociodemographic and disease characteristics. Parents of 108 patients, median age 13 years (range 5-22), completed the questionnaires 24-30 months after diagnosis. There were 81 patients with TBI of whom 11 (14%) with moderate/severe TBI and 27 patients with NTBI of whom 5 (19%) with moderate/severe NTBI. The median PedsQL FIM Total Scale was 80.4 (SD 16.1). The PedsQL FIM Total Scale and 4 out of 5 Subscale Scores were statistically significantly better in the TBI group than in the NTBI group and in patients with severe NTBI than with mild/moderate NTBI. Moreover, in the total group, there were significant univariate associations between the FIM Total Scale and/or one or more Subscale Scores and age, preinjury patient health problems, and the PSOM, CFFS, PedsQL General Core, and Multiple Fatigue Scales. In the multivariable analysis, the FIM Total Scale was significantly associated with type and severity of injury and preinjury patient health problems. Two years after onset, the parent-reported that impact of ABI on the family as measured by the PedsQL FIM was considerable especially in patients with moderate/severe NTBI.