Parents' Perceptions of the Effectiveness of Music Therapy on Their Chronically Ill Children.

Chronic disease in a child, with the associated hospital stays, places considerable demands on the child and their family. The aim of this study was to investigate the parents' perceptions of the music therapy used with their child during a hospital stay and to determine whether they felt that it reduced the child's anxiety and stress generated by hospital admission. We hypothesized that the use of live music therapy from a music therapist would positively support these patients in everyday clinical practice, promote their wellbeing, and have positive impacts on their vital signs and blood pressure. Children with chronic gastroenterological and nephrological diseases included in this prospective study received live music therapy with a median duration of 41 min (range from 12 to 70 min) two to four times per week until discharged from the hospital. At the time of discharge, the parents were asked to complete a Likert-style questionnaire to evaluate the music therapy. Seven items were related to general questions about the patients and sessions, and eleven items evaluated the personal perceptions of the parents. Music therapy was conducted in 83 children, with a median age of 3 years (range from 1 month to 18 years). All parents (100%) completed the questionnaire at the time of discharge. Seventy-nine percent of the parents stated that their children were able to enjoy the music therapy sessions without being stressed. In addition, 98% of the respondents said that they were grateful for the music therapy their children received (97% fully agreed and 1% rather agreed). All parents considered music therapy to be beneficial for their child. The parents' responses reflected the view that music therapy is beneficial to patients. According to the parents, music therapy can be integrated effectively in the inpatient clinical setting and can support children with chronic illnesses during their hospital stay.

Effects of Music Therapy on Vital Signs in Children with Chronic Disease.

BACKGROUND: Recent research found evidence supporting music therapy for hospitalized children with chronic diseases. The aim of this study was to investigate the effect of music therapy on hospitalized children's vital signs. METHODS: In this prospective study, children with chronic gastroenterological and nephrological diseases received active or receptive music therapy two to four times a week until discharge from hospital at the pediatric special care unit (SCU) and pediatric intensive care unit (ICU). Baseline and post-therapy heart rate, oxygen saturation and blood pressure were recorded and analyzed as control values at three points on the same day when the children were alone in their patient room at rest. RESULTS: A total of 83 children, median 3 age of years (range one month to eighteen years) received music therapy. In total, 377 music therapy sessions were treated: 200 receptive therapy (78 ICU, 122 SCU) and 177 with active therapy (0 ICU, 177 SCU). Music therapy interventions showed changes in vital signs during music therapy sessions. After music therapy, heart rates decreased by 18 beats per minute (95% confidence interval (CI), -19.4 to (-16.8)), oxygen saturation increased by 2.3% (95% CI, 2.2 to 2.5), systolic blood pressure decreased by 9.2 (95% CI, -10.6 to -7.7) and diastolic blood pressure decreased by 7.9 (95% CI, -9.6 to -6.3). When music therapy was applied at the SCU (ICU), heart rates significantly reduced by 17.9 (18.9) beats per min, oxygen saturation increased by 2.4% (2.1%) and blood pressure reduced by 9.2 (2.8) mmHg (systolic) and 7.9 (0.3) mmHg (diastolic). Almost all control values were better than directly before the intervention. However, after music therapy intervention, the children showed better values in vital signs compared to being alone in their patient room. CONCLUSION: Music therapy is an added value for children with kidney and liver/gastrointestinal diseases during their hospital stay.

Expanding the Spectrum of FAT1 Nephropathies by Novel Mutations That Affect Hippo Signaling.

INTRODUCTION: Disease-causing mutations in the protocadherin FAT1 have been recently described both in patients with a glomerulotubular nephropathy and in patients with a syndromic nephropathy. METHODS: We identified 4 patients with FAT1-associated disease, performed clinical and genetic characterization, and compared our findings to the previously published patients. Patient-derived primary urinary epithelial cells were analyzed by quantitative polymerase chain reaction (qPCR) and immunoblotting to identify possible alterations in Hippo signaling. RESULTS: Here we expand the spectrum of FAT1-associated disease with the identification of novel FAT1 mutations in 4 patients from 3 families (homozygous truncating variants in 3, compound heterozygous missense variants in 1 patient). All patients show an ophthalmologic phenotype together with heterogeneous renal phenotypes ranging from normal renal function to early-onset end-stage kidney failure. Molecular analysis of primary urine-derived urinary renal epithelial cells revealed alterations in the Hippo signaling cascade with a decreased phosphorylation of both the core kinase MST and the downstream effector YAP. Consistently, we found a transcriptional upregulation of bona fide YAP target genes. CONCLUSION: A comprehensive review of the here identified patients and those previously published indicates a highly diverse phenotype in patients with missense mutations but a more uniform and better recognizable phenotype in the patients with truncating mutations. Altered Hippo signaling and de-repressed YAP activity might be novel contributing factors to the pathomechanism in FAT1-associated renal disease.