Home monitoring of fetal heart rhythm: Lived experiences of women with anti-SSA/Ro52 autoantibodies and their co-parents.

OBJECTIVE: The aim of this study was to explore the parents' experiences of home monitoring of the fetal heart rhythm. Women with anti-SSA/Ro52 autoantibodies carry a 2%-3% risk of giving birth to a child with congenital heart block (CHB), following transplacental transfer and antibody-mediated inflammation in the fetal conduction system during 18th to 24th gestational week. Early detection and subsequent treatment have been reported to decrease morbidity and mortality. Therefore, home monitoring of the fetal heart rhythm by Doppler has been offered at our fetal cardiology center. This study was undertaken to explore the lived experience of the routine. METHODS: Participants were recruited from a single fetal cardiology center. Consecutive sampling was used. The inclusion criteria were women with SSA/Ro52 antibodies who had undergone Doppler examinations within the last two and a half years at the hospital and had monitored the fetal heartbeat at home. A semi-structured questionnaire was created, and the participants were interviewed individually. The interviews were transcribed verbatim and analyzed according to qualitative content analysis. RESULTS: The overall theme was defined as "walking on thin ice," with six underlying categories: reality, different strategies, gain and loss, healthcare providers, underlying tension, and conducting the examinations again, all with a focus on how to handle the home monitoring during the risk period. CONCLUSION: Both the mother and the co-parent expressed confidence in their own abilities and that the monitoring provided them with the advantage of growing a bond with the expected child. However, all the participants described a feeling of underlying tension during the risk period. The results show that home monitoring is not experienced as complicated or a burden for the parents-to-be and should be considered a vital part of the chain of care for mothers at risk for giving birth to a child with CHB. However, explaining the teamwork between the different caregivers, for the patients involved, their areas of expertise, and how they collaborate with the patient continues to be a pedagogic challenge and should be developed further.

Parents caring and sham-feeding their child born with Esophageal atresia at home while waiting for reconstructive surgery.

PURPOSE: For children with Esophageal atresia who have to wait for reconstructive surgery, long hospital stay, delayed introduction of oral feeds and hampered oro-motor function has traditionally been draw-backs for this treatment as the patients have minimal training of oro-motor function while waiting for surgery. In this paper, we present the concept of sham-feed at home awaiting reconstructive surgery with the aim to obliviate these problems. The aim was to describe the characteristics of patients with Esophageal atresia waiting for reconstructive surgery sham-feeding at home by their parents and further describe adverse events that arose. METHODS: The study is a retrospective descriptive single center study on all children with a delayed reconstruction of Esophageal atresia who was sham-fed by their parents at home before reconstructive surgery between January 2010 and January 2023 at the Karolinska University Hospital, Stockholm. RESULTS: Nine patients where home waiting for reconstructive surgery for a median of 72 days. No adverse events were reported related to the sham-feed procedure at home. The children had reconstructive surgery at a median 120 days of age. Five of the children ate full meals orally day 8-27 after surgery. Two children ate fully before 1 year after surgery. Two children had surgery less than 1 year ago and were not eating fully orally at the time of data collection. CONCLUSION: Sham-feeding at home by the parents was safe and feasible with the benefits of a prolonged time out of hospital awaiting reconstructive surgery.

Parents' experiences of sham feeding their child with esophageal atresia at home while awaiting reconstructive surgery. A qualitative interview study.

PURPOSE: This study aimed to explore parents' experience of sham feeding their baby born with esophageal atresia at home, waiting for reconstructive surgery. METHOD: Semi-structured interviews were conducted with parents of six children born with esophageal atresia waiting for delayed reconstruction. The interviews were analyzed using qualitative content analysis. RESULTS: Parents experienced that sham feed reinforced the healthy abilities in their baby. They had faith in their own ability as parents to care for their child as well as to see to their baby's strength to cope with difficulties. Parents expressed that the health care system can hinder as well as be a major support on their way to a more normal life at home while waiting for reconstructive surgery. CONCLUSION: The experience of sham feeding at home while waiting for reconstructive surgery is characterized by positive aspects both for children born with esophageal atresia and their parents.

Health-related quality of life in children born with congenital diaphragmatic hernia.

PURPOSE: The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH). METHODS: Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children's hospital in Stockholm. In 2012, long-term survivors (n = 77) were asked to participate in the present study, which resulted in a 46% (n = 35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed. RESULTS: The study participants did not differ from the non-participants in terms of prenatal diagnosis, gender, side of lesion, method of surgical repair, time to intubation, need for ECMO support, or way of discharge from the hospital. Children born with CDH considered themselves to have a good HRQoL, as good as healthy Swedish children. There were only a few significant HRQoL differences within the group of children with CDH, although several median scores in ECMO-treated patients were somewhat lower. Correlations between child and parent scores on HRQoL were low. CONCLUSIONS: Health-related quality of life in children born with CDH is good overall, however, a correlation between the severity of the malformation and HRQoL cannot be excluded.

Behavioral, emotional and social functioning in children born with congenital diaphragmatic hernia.

PURPOSE: The aim was to investigate social competence and behavioral and emotional problems in children and adolescents born with CDH. METHODS: All children born with CDH, treated in Stockholm 1990-2009, were invited to participate. After written consent, the Child Behavior Checklist or Adult Self-Report questionnaires were sent to participants. Of the 145 long-term survivors, 51% returned a completed questionnaire. Both the syndrome and competence scales were used and open-ended questions were analyzed with manifest content analysis. RESULTS: All parents of children aged 1.5-5 years and 90% of parents of children aged 6-18 years reported a normal range on the syndrome scale. Five parents indicated internalizing, but none externalizing behavior. All young adults achieved a normal score on the syndrome scale. Eighty-five percent had normal school achievement, 79% had normal social scores and 40% had normal activity levels. Significantly fewer boys (23%) were in the normal activity range compared with 67% of girls. CONCLUSIONS: The vast majority of all parents of children born with CDH scored no behavioral or emotional problems, furthermore, they reported normal social and school competence. However, the activity levels seemed to be reduced in children born with CDH.

Parenting stress among parents of children with congenital diaphragmatic hernia.

PURPOSE: The aim of this study was to examine parental stress among parents of children with congenital diaphragmatic hernia (CDH). METHODS: Between 2005 and 2009, a total of 51 children with CDH were treated at Astrid Lindgren Children's Hospital. The survival rate at discharge was 86% and long-term survival rate 80%. One parent each of the long-term survivors (41 children) was included in the present study, and 34 parents (83%) agreed to participate. Participants received the Swedish Parenthood Stress Questionnaire (SPSQ). The questionnaire was supplemented by data from case records. RESULTS: Parents of children with CDH, who had been supported by ECMO or had a long hospital stay, showed significantly higher overall parental stress. Mothers scored an overall higher parental stress compared with fathers. A prenatal diagnosis of CDH or lower parental educational level resulted in significantly higher parental stress in some of the factors. CONCLUSIONS: Parental stress in parents of children with CDH seems to increase with the severity of the child's malformation. Mothers tend to score higher parental stress than fathers.

Self-assessed physical health among children with congenital diaphragmatic hernia.

PURPOSE: The aim of this long-term follow-up study was to investigate the current self assessed physical health in a CDH birth cohort at a single center. METHODS: Between 1990 and 2009, 195 children born with CDH were treated at Astrid Lindgren Children's Hospital. The primary survival rate was 85 %, and in 2010, 78 % were still alive. Data from medical records were supplemented by a questionnaire consisting of questions about perceived physical function. Patients were divided into groups according to time for intubation and need for extracorporeal membrane oxygenation. RESULTS: Children born with CDH reported themselves to be having greater problems with asthma, developmental delay, seizure disorder, poor vision, and scoliosis in comparison with normal Swedish children. They also described a sense of having less strength and becoming breathless more often than healthy friends. Symptoms of gastroesophageal reflux and abdominal pain were also reported. CONCLUSIONS: The majority of the children perceived their physical health as being overall good, but there was an increase of reported symptoms correlating with the severity of the malformation.

Prevalence of Mental Health Problems, Associated Factors, and Health-Related Quality of Life in Children with Long-Gap Esophageal Atresia in Sweden.

BACKGROUND: Children with long-gap esophageal atresia (LGEA) face a high risk of digestive and respiratory morbidity, but their mental health outcomes have not been investigated. We aimed to identify the prevalence of mental health problems in children with LGEA, associated factors and health-related quality of life (HRQOL). METHODS: Twenty-six children with LGEA aged 3-17 were recruited nationwide in Sweden. One of their parents and adolescents aged 11-17 completed information on the child's mental health (Strength and Difficulties Questionnaire), generic (PedsQL 4.0) and condition-specific HRQOL (EA-QOL). Parents gave information on current child symptomatology. Mental health level was determined using validated norms; abnormal≥90 percentile/borderline≥80 percentile/normal. Elevated levels were considered borderline/abnormal. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05. RESULTS: Twelve children with LGEA aged 3-17 (46%) had elevated scores of ≥1 mental health domain in parent-reports, whereas 2 adolescents (15%) in self-reports. In parent-reports, 31% of the children had elevated levels of peer relationship problems, with associated factors being child sex male (p = 0.037), airway infections (p = 0.002) and disturbed night sleep (p = 0.025). Similarly, 31% showed elevated levels of hyperactivity/inattention, and associated factors were male sex (p = 0.005), asthma (p = 0.028) and disturbed night sleep (p = 0.036). Elevated levels of emotional symptoms, seen in 20%, were related to swallowing difficulties (p = 0.038) and vomiting problems (p = 0.045). Mental health problems correlated negatively with many HRQOL domains (p < 0.05). CONCLUSIONS: Children with LGEA risk mental health difficulties according to parent-reports, especially peer relationship problems and hyperactivity/inattention, with main risk factors being male sex, airway problems and sleep disturbances. This should be considered in follow-up care and research, particularly since their mental health problems may impair HRQOL. LEVELS OF EVIDENCE: Prognosis study, LEVEL II.

Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia: a nationwide Swedish study.

BACKGROUND: In 10-15% of children with esophageal atresia (EA) delayed reconstruction of esophageal atresia (DREA) is necessary due to long-gap EA and/or prematurity/low birth weight. They represent a patient subgroup with high risk of complications. We aimed to evaluate postoperative morbidity and health-related quality of life (HRQOL) in a Swedish national cohort of children with DREA. METHODS: Postoperative morbidity, age-specific generic HRQOL (PedsQL™ 4.0) and condition-specific HRQOL (The EA-QOL questionnaires) in children with DREA were compared with children with EA who had primary anastomosis (PA). Factors associated with the DREA group's HRQOL scores were analyzed using Mann-Whitney U-test and Spearman's rho. Clinical data was extracted from the medical records. Significance level was p < 0.05. RESULTS: Thirty-four out of 45 families of children with DREA were included and 30 returned the questionnaires(n = 8 children aged 2-7 years; n = 22 children aged 8-18 years). Compared to children with PA(42 children aged 2-7 years; 64 children aged 8-18 years), there were no significant differences in most early postoperative complications. At follow-up, symptom prevalence in children aged 2-7 with DREA ranged from 37.5% (heartburn) to 75% (cough). Further digestive and respiratory symptoms were present in ≥ 50%. In children aged 8-18, it ranged from 14.3% (vomiting) to 40.9% (cough), with other digestive and airway symptoms present in 19.0-27.3%. Except for chest tightness (2-7 years), there were no significant differences in symptom prevalence between children with DREA and PA, nor between their generic or condition-specific HRQOL scores (p > 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p < 0.05. Days to hospital discharge after EA repair and a number of associated anomalies showed a strong negative correlation with HRQOL scores (2-7 years). Presence of cough, airway infection, swallowing difficulties and heartburn were associated with lower HRQOL scores (8-18 years), p < 0.05. CONCLUSIONS: Although children with DREA need more treatments, they are not a risk group for postoperative morbidity and impaired HRQOL compared with children with PA. However, those with a long initial hospital stay, several associated anomalies and digestive or respiratory symptoms risk worse HRQOL. This is important information for clinical practice, families and patient stakeholders.

Re-ECMO for congenital diaphragmatic hernia: Is it worth the effort?

AIM: To evaluate the results in CDH patients subjected to a second course of ECMO at a single institution. MATERIAL AND METHODS: Retrospective review of medical charts of patients treated for CDH and ECMO in our center since 1990 to December 2018 was performed. For patients subjected to a second course of ECMO and who survived to hospital discharge charts from follow up visits were also reviewed. RESULTS AND DISCUSSION: From Jan 1990 until December 2018, 311 patients with CDH were treated in the department. 267 of these (86%) were discharged alive from the hospital and 81% (237/293) of the Swedish patients were alive by December 2018. 101 patients (32%) were subjected to ECMO treatment of whom 71 survived (70%). 22 patients underwent a second ECMO run and 13 of these survived to hospital discharge. Seven of the Swedish patients [19] were long-term survivors (37%). The vast majority was on V-A ECMO. CONCLUSIONS: It is possible to recannulate the right common carotid artery and internal jugular vein for a second course of venoarterial ECMO in CDH patients, who deteriorate severely after decannulation. Previous research has shown that long-term survivors subjected to ECMO twice reported similar frequencies of pulmonary, gastrointestinal, neurological and musculoskeletal sequelae as the long-term survivors, who needed ECMO support only once, and similar health-related quality of life. Regarding their psychosocial function, they scored within normal range in the behavioral, emotional and social scales domains. A second ECMO run may contribute to a higher survival and that the long-term morbidity among survivors is not more pronounced than among survivors after a single course of ECMO. It is therefore suggested that a second course of ECMO should be offered on the same indications as the first course. LEVEL OF EVIDENCE: III Case series.

Educational level and socioeconomic status in patients born with congenital diaphragmatic hernia: A population-based study.

BACKGROUND: Neurodevelopmental dysfunction is one of the most disabling outcomes for congenital diaphragmatic hernia (CDH) survivors and may have a long lasting impact in adult life. AIM: To evaluate to which extent being born with CDH has an impact on the educational level and socioeconomic status as a proxy for neurocognitive development. MATERIAL AND METHODS: Nationwide, population-based prospective study of newborn children in Sweden from 1982 to 2015. School grades, highest educational level and income were assessed through Swedish public registries. Children above 15 years of age with CDH were compared with randomly selected controls. RESULTS: A significantly higher number of cases (17% vs 10%) did achieve neither a school nor a university degree. Among those who achieved a degree there was no difference in the highest level of education. The qualification points in elementary school did not differ, but in high school female cases had significantly lower qualification points than female controls. There were no differences in individual disposable income between cases and controls. However, males had higher income compared to females. Prematurity and a long hospital stay had a negative impact on educational level. CONCLUSIONS: A higher proportion of children born with CDH compared to controls do not achieve a school degree. Among those who achieved a degree, the school achievements and educational level were similar to controls. Prematurity and a long hospital stay are risk factors for not achieving an educational degree. TYPE OF STUDY: Prognosis study (high-quality prospective cohort study with 99% of patients followed to the study end point). LEVEL OF EVIDENCE: Level I. I for a prognosis study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point.

Addressing the causes of late mortality in infants with congenital diaphragmatic hernia.

INTRODUCTION: Despite improvements of neonatal intensive care, mortality among patients born with congenital diaphragmatic hernia (CDH), remains high, and there is a significant late mortality in this cohort. OBJECTIVE: The aim of this study was to evaluate the causes of death among 251 consecutive CDH patients treated at our institution during the last 26years period. METHODS: Retrospective review of all causes of death between 1990 and 2015 of the CDH cohort prospectively collected in a database. RESULTS: Of the 251 CDH patients treated in our department since 1990, 49 were not alive by the end of 2015. Thirty-six patients (14%) died before discharge, and 13 (5%) after the first care event. The mean age at death was 262days (±653days, median 34days). Eighty six % (42 cases) of the fatalities occurred during the first year of life, more than half of the patients died before 1month of age and only 13% after the age of 1year (7 patients) (late mortality). The causes of early mortality (before 1year of age) were mainly cardio-respiratory, whereas GI complications occurred in the late mortality group. CONCLUSION: The most common cause of death among CDH patients is respiratory insufficiency and associated pulmonary hypertension, and most of the fatalities occur before 1year of age. Among older patients, gastrointestinal morbidity as cause of death is highly represented. LEVEL OF EVIDENCE: II.