Implementation of a high fidelity simulation based training program for physicians of children requiring long term invasive home ventilation: a study by ISPAT team.

Introduction: The number of children requiring long-term invasive home ventilation (LTIHV) has increased worldwide in recent decades. The training of physicians caring for these children is crucial since they are at high risk for complications and adverse events. This study aimed to assess the efficacy of a comprehensive high-fidelity simulation-based training program for physicians caring for children on LTIHV. Methods: A multimodal training program for tracheostomy and ventilator management was prepared by ISPAT (IStanbul PAediatric Tracheostomy) team. Participants were subjected to theoretical and practical pre-tests which evaluated their knowledge levels and skills for care, follow-up, and treatment of children on LTIHV. Following the theoretical education and hands-on training session with a simulation model, theoretical and practical post-tests were performed. Results: Forty-three physicians from 7 tertiary pediatric clinics in Istanbul were enrolled in the training program. Seventy percent of them had never received standardized training programs about patients on home ventilation previously. The total number of correct answers from the participants significantly improved after the theoretical training (p < 0.001). The number of participants who performed the steps correctly also significantly increased following the hands-on training session (p < 0.001). All of the 43 participants who responded rated the course overall as good or excellent. Conclusion: The knowledge and skills of clinicians caring for children on LTIHV can be enhanced through a comprehensive training program consisting of theoretical training combined with hands-on training in a simulation laboratory.

The effects of nusinersen treatment on respiratory status of children with spinal muscular atrophy.

BACKGROUND: Respiratory involvement is the main factor predicting the prognosis of spinal muscular atrophy (SMA). Significant responses in motor functions have been demonstrated with nusinersen, but pulmonary outcomes are still varied. We aimed to explore the effects of nusinersen on the respiratory functions of patients with SMA. METHODS: Patients with SMA who were receiving regular nusinersen treatment in our tertiary care hospital were enrolled in this study. We evaluated the patients in terms of the necessity to ventilatory or nutritional support, presence of motor involvement and other comorbidities related with prognosis at three consecutive assessments. RESULTS: The study group consisted of 43 patients (18 type 1, 12 type 2, and 13 type 3) with SMA with a mean age of 27.8 months at diagnosis and 60.8 months at the beginning of nusinersen treatment. The respiratory function improvements were noted in six patients at third assessment. Early initiation of nusinersen was significantly correlated with reduced hospital admissions (P = 0.026). Nutritional support and weight gain were remarkable in the ventilatory-supported group. Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores were significantly higher in the non-tracheostomized group in patients with SMA type 1 (P < 0.005). CONCLUSIONS: We posit that nusinersen may change the natural prognosis of SMA and improve care of children with SMA. Following up children with SMA for longer periods under nusinersen may be beneficial for understanding the effects of treatment. Results of our study need to be supported by future long-term studies to reach a consensus on nusinersen, considering the overall genetic and environmental status as well as the cost-effectiveness of the treatment.

The Role of Telehealth Services in Children with Cystic Fibrosis During Coronavirus Disease 2019 Outbreak.

Objectives:This study aimed to monitor the health and nutritional status of pediatric cystic fibrosis (CF) patients via telehealth services during the novel coronavirus disease 2019 (COVID-19). Additional aims were to determine the level of anxiety in the patients and their caregivers and to determine the COVID-19 transmission status among CF patients.Materials and Methods:The CF team supported the patients via remote contact. During telehealth services interviews, in addition to obtaining information about the patients' anthropometric measurements, health status, and CF-related complaints, the State-Trait Anxiety Inventory (STAI) was administered to the patients and controls. The Hospital Anxiety and Depression Scale (HAD) was administered to their caregivers.Results:The study included 144 pediatric CF patients (74 males and 70 females). Mean age of the patients was 8.9 years. In all, 42 (29.2%) of the patients were tested for COVID-19, of which 4 were positive. The mean STAI score was significantly lower in the patient group than in the control group (p < 0.001). The mean HAD anxiety score was significantly higher in the caregivers of the CF patients, compared to the caregivers of the controls (p = 0.005). In addition, the mean HAD depression score was significantly higher in the caregivers of the CF patients (p < 0.001).Conclusions:Telehealth is an innovative method for providing health care services while maintaining social distance and avoiding the risk of exposure and spread of COVID-19. Telehealth services reduce patient and parental anxiety and increase the level of confidence in managing CF-related complications.