Health-Related Quality of Life and Distress of Parents of Children With Avoidant Restrictive Food Intake Disorder.

OBJECTIVES: Health-related quality of life (HRQOL) of children with avoidant restrictive food intake disorder (ARFID) is impaired. AIM: To measure HRQOL and distress of parents of children with ARFID. METHODS: Cross-sectional cohort study. Parents of children with ARFID, visiting our multidisciplinary feeding team, completed questionnaires on the online Quality of Life in Clinical Practice portal; the Questionnaire for Adult's Health Related Quality of Life to assess parental HRQOL and the Distress Thermometer for Parents. Reference groups of parents of healthy (HC) and chronically ill children (CIC) were used. RESULTS: Eighty-five mothers and 62 fathers of 89 children with ARFID (58% female, median age 1.9 years) were included (response rate 68%). No differences were found regarding HRQOL in 11 of 12 domains between parents of children with ARFID and HC. Mothers of children with ARFID reported significantly higher HRQOL regarding pain and fathers a significantly lower HRQOL on depressive emotions compared to HC. No differences were found in overall and clinical distress scores between parents of children with ARFID and HC/CIC. Mothers of children with ARFID had significantly higher distress scores regarding cognitive problems compared to HC and parenting problems in children <2 years compared to HC/CIC. Significantly higher distress scores on parenting problems in children <2 years were found in fathers of children with ARFID compared to HC/CIC. CONCLUSION: Most HRQOL and distress scores of parents of children with ARFID were comparable to reference groups. Since parents of children with ARFID perceive a lack of understanding and support from the environment, professionals should suggest peer support through patient's organizations. Furthermore, it is important to offer professional support since parents indicated that they would like to talk to a professional about their situation.

Health related quality of life of infants and children with avoidant restrictive food intake disorder.

OBJECTIVE: The aim of this study was to compare health related quality of life (HRQOL) in infants and children with avoidant restrictive food intake disorder (ARFID) to healthy and chronically ill controls. METHOD: A cross-sectional study was conducted in children who meet ARFID criteria at our tertiary care pediatric feeding clinic (September 2014 to July 2016). Before consultation, parents of patients (n = 100) were asked to complete questionnaires to determine HRQOL: the TNO-AZL Preschool Children Quality of Life (0-5 years), and "Pediatric Quality of Life Inventory" (6-7 and 8-10 years). HRQOL of ARFID patients was compared to both healthy (0-5 years n = 241; 6-7 years n = 61; 8-10 years n = 192) and chronically ill (0-5 years n = 79; 6-7 years n = 11; 8-10 years n = 26) controls. RESULTS: The prevalence of ARFID was 64%. HRQOL of ARFID patients aged 0-5 years (n = 37) was significantly lower on 6/12 scales (appetite, lungs, stomach, motor functioning, positive mood and liveliness) compared to healthy controls (P < .01), and on 4/12 scales (appetite, stomach, motor functioning, and liveliness) compared to chronically ill controls (P < .01). The ARFID patients scored significantly better on the problem behavior scale compared to healthy and chronically ill controls (P < .01). ARFID patients aged 6-7 (n = 9) had significantly lower scores in 3/6 scales (total score, psychosocial health, and school functioning) (P < .01), and aged 8-10 (n = 2) had a significantly lower school functioning scale (P < .01) compared to healthy controls. CONCLUSION: HRQOL of children with ARFID is decreased on multiple scales. The effect on HRQOL should be incorporated in clinical practice, and clinical studies should add HRQOL as an outcome measure.

Long-term efficacy of clinical hunger provocation to wean feeding tube dependent children.

BACKGROUND & AIMS: The incidence of tube feeding dependency seems to increase worldwide, and these children may remain on prolonged tube feeding for many months to years. The multidisciplinary clinical hunger provocation (CHP) program is an intensive inpatient intervention of usually 2-3 weeks, aimed at weaning children from tube feeding. CHP has been proven highly effective on the short term (80-86%), particularly when applied before the age of two years but long-term data are lacking. The aims of our study were to determine the long-term efficacy of the CHP program and factors associated with success or failure and to assess anthropometrics, feeding behavior, and medical outcomes at long-term follow-up. METHODS: All tube-dependent children who underwent CHP at a tertiary hospital in Amsterdam, the Netherlands, between 2001 and 2014, and had a minimum follow-up of 12-months in 2015, were eligible to participate in this retrospective cohort study. During the CHP program, tube feeding is ceased stepwise to create appetite, according to a strict protocol. The program was defined successful if patients achieved oral intake and could be fully weaned from tube feeding following the CHP program. Acute malnourishment was defined as weight for height <2 SD or loss of >1 SD within 3 months, chronic malnourishment as height for age <2 SD and both acute and chronic malnourishment as both a height for age and weight for height <2 SD. Long-term efficacy (tube free at varying follow-up periods), anthropometrics (height for age, weight for height), feeding behavior and medical outcomes were assessed by a structured cross-sectional parental interview. RESULTS: In total, 57 patients were admitted to the CHP program. Fifty-two patients could be contacted of whom 42 participated in the study (response rate 81%) with a median age at admittance of 19 (IQR 13-22) months (62% female). The program was initially successful in 36/42 (86% (Bca CI 95% 75.0-95.2)) patients. A younger age upon initiation of tube feeding was negatively correlated with success (p 0.016). At follow-up, a median period of 67.0 (IQR 37.0-101.5) months after discharge, long-term efficacy was 32/41 (78% (Bca CI 95% 64.1-90.0)) (1 missing data). Patients with a successful CHP had beneficial outcomes compared to those with an unsuccessful CHP, showing less selective eating behavior (p 0.025), nocturnal feeding (p 0.044), forced feeding (p 0.044) and hospital admissions (p 0.028). However, 44% of successfully weaned patients fulfilled the criteria for malnourishment at long-term follow-up (13% acute, 22% chronic, and 9% both acute and chronic (compared to 22% at admittance: 13% acute, 6% chronic, and 3% both)). 59.4% of successfully weaned patients showed signs of developmental delays or were diagnosed with new medical diagnoses (43.8%) at long-term follow-up. CONCLUSIONS: The multidisciplinary CHP is a highly effective short-term (86%) and long-term (78%) intervention to wean young children from tube feeding, with beneficial feeding outcomes. However, at long-term follow-up, many successfully weaned patients were malnourished, showed signs of developmental delay, and were diagnosed with new medical diagnoses. For these reasons, patients should be monitored carefully during and after tube weaning, also after successful CHP. Tube dependency might be an early expression of medical diagnoses.

Prevalence and side effects of pediatric home tube feeding.

BACKGROUND & AIMS: Tube feeding ensures growth, but can have negative effects on health and psychosocial functioning, resulting in health related costs. The aims of this study were to determine the prevalence of pediatric home tube feeding in the Netherlands and to assess the clinical characteristics of tube fed children and side effects of tube feeding. METHODS: The prevalence of pediatric home tube feeding was calculated using data (2010-2014) of both the Medicines and Devices Information Project of the National Health Care Institute, and Statistics Netherlands. Subsequently, a cross-sectional parental online questionnaire was used to obtain data regarding clinical characteristics of tube fed children and side effects of tube feeding. Children aged ≤17 years receiving tube feeding ≥2 weeks were included. RESULTS: The prevalence of pediatric home tube feeding was 83-92:100,000 children/year. Parents of 279 children (53% boys) completed the questionnaire. Most children (88%) had ≥1 medical diagnosis, of which congenital abnormalities (42%), perinatal problems (38%) and neurologic diseases (16%) were most common. They had gastrostomy (60%), nasogastric (33%), or other tube types (7%). Parents of most children (74%) mentioned ≥1 side effect due to tube feeding. Vomiting (37%), lack of appetite (29%), and gagging (29%) were reported most frequently. Nasogastric tube placement resolved in negative experiences (94%). CONCLUSIONS: The prevalence of pediatric home tube feeding varies between 83 and 92:100,000 children/year in the Netherlands. These children are characterized by various underlying medical diagnoses. Side effects of tube feeding are frequently reported by parents. Further studies should focus on methods reducing side effects.

Neonatal screening for congenital hypothyroidism in the Netherlands: cognitive and motor outcome at 10 years of age.

CONTEXT: Patients with thyroidal congenital hypothyroidism (CH-T) born in The Netherlands in 1981-1982 showed persistent intellectual and motor deficits during childhood and adulthood, despite initiation of T(4) supplementation at a median age of 28 d after birth. OBJECTIVE: The present study examined whether advancement of treatment initiation to 20 d had resulted in improved cognitive and motor outcome. DESIGN/SETTING/PATIENTS: In 82 Dutch CH-T patients, born in 1992 to 1993 and treated at a median age of 20 d (mean, 22 d; range, 2-73 d), cognitive and motor outcome was assessed (mean age, 10.5 yr; range, 9.6-11.4 yr). Severity of CH-T was classified according to pretreatment free T(4) concentration. MAIN OUTCOME MEASURE: Cognitive and motor outcome of the 1992-1993 cohort in comparison to the 1981 to 1982 cohort was the main outcome measure. RESULTS: Patients with severe CH-T had lower full-scale (93.7), verbal (94.9), and performance (93.9) IQ scores than the normative population (P < 0.05), whereas IQ scores of patients with moderate and mild CH-T were comparable to those of the normative population. In all three severity subgroups, significant motor problems were observed, most pronounced in the severe CH-T group. No correlations were found between starting day of treatment and IQ or motor outcome. CONCLUSIONS: Essentially, findings from the 1992-1993 cohort were similar to those of the 1981-1982 cohort. Apparently, advancing initiation of T(4) supplementation from 28 to 20 d after birth did not result in improved cognitive or motor outcome in CH-T patients.

Health- related quality of life and self-worth in 10-year old children with congenital hypothyroidism diagnosed by neonatal screening.

BACKGROUND: Much is written about cognitive and motor development; less is known about social and emotional consequences of growing up with congenital hypothyroidism (CH).The objectives of the study were: (1) to compare health related quality of life (HRQoL) and self-worth of 10 year old patients with CH with the general population; (2) to explore associations of disease factors, IQ and motor skills with the outcomes. METHODS: Children with CH and their parents completed several questionnaires. Patients were classified to 'severe CH, n = 41' or 'moderate/mild CH, n = 41' based on pre-treatment FT4 concentration.Differences between CH and the general population were tested by analysis of covariance and one sample t-tests (mean scale scores HRQoL and self-worth), chi-square tests and binomial tests (% at risk of impaired HRQoL and self-worth). Linear regression analyses corrected for gender were conducted to explore associations of the outcomes with disease factors, IQ and motor skills. RESULTS: Patients with CH reported lower mean HRQoL on motor, cognitive and social functioning, and on autonomy and positive emotions (p < 0.0001). Patients were also more often at risk for impaired HRQoL and self-worth. No differences were found between the severity groups. Lower IQ was only significant associated with worse cognitive HRQoL. Initial FT4 plasma, age at onset of therapy, initial T4 dose and motor skills were not significantly associated with HRQoL and self-worth. CONCLUSIONS: Negative consequences in terms of HRQoL and self-worth are prevalent in children with CH, independent of disease factors, IQ and motor skills. Physicians should to be attentive to these consequences and provide attention and supportive care.

Evaluation of cognitive and motor development in toddlers with congenital hypothyroidism diagnosed by neonatal screening.

OBJECTIVE: The Dutch neonatal congenital hypothyroidism (CH) screening procedure and treatment modality has been adapted several times since its national institution in 1981. These changes enabled us to investigate whether earlier treatment has resulted in improved cognitive and motor outcomes. The present study examined whether the advancement of treatment modality has resulted in improved cognitive and motor outcomes. METHODS: In 95 toddlers with thyroidal CH (CH-T), born in 2002 through 2004 and treated at a median age of 9 days, cognitive and motor outcomes were assessed with the Bayley Scales of Infant Development-II-NL at 1 and 2 years of age. This outcome was also analyzed in relation to treatment variables. RESULTS: The mean mental developmental index (MDI) scores of the severe (initial free thyroxine [FT4] ≤0.4 ng/dL (≤5 pmol/L), moderate (0.4 < initial FT4 ≤ 0.8 ng/dL (5.0 < initial FT4 ≤ 10.0 pmol/L), and mild (initial FT4 > 0.8 ng/dL (>10.0 pmol/l) CH-T groups at 1 year and the moderate and mild CH-T groups at 2 years were similar to the population mean. The mean MDI scores of the total CH-T group and severe CH-T group at 2 years were significantly lower than the population mean (p < .0001). In all 3 severity subgroups, significant lower psychomotor developmental index scores (p < .0001) were observed. No correlations were found between starting day of treatment and developmental outcome. Initial T4 concentration and initial T4 dose were weak predictors for developmental outcome. CONCLUSION: Essentially, comparable with our earlier findings, children with CH, especially those with severe CH, are still at risk for motor and cognitive problems, which are probably due to the consequence of the prenatal hypothyroid state or the thyroid hormone deficiency in early life.