Home ventilatory management in pediatric neuromuscular and other lung diseases Dr. Huda Mussaffi-Georgy Pulmonary Institute Schneider Children’s Medical Center • • • • • • Case presentation Schneider’s Pulmonary clinic Experience World Experience Trends Guidelines Future plans Study case • L.S., baby male diagnosed with SMA type I at the age of 2/12 • At 6/12 age: ineffective cough, started cough assist • At 8/12 respiratory distress & atalectasis; hospitalized & started BIPAP at night • At 10/12 clinical aspirations & respiratory deterioration • At 10.5/12 gastrostomy was performed under spinal anesthesia after preparation with: Intensive physiotherapy Stopped feeding for 1 week Antibiotics NIV • Was stable for 12 hrs after procedure • 12-20 hrs later respiratory distress and difficulty to connect to BIPAP. New infiltrates Study case-cont. • • • • • Started invasive ventilation Intensive physiotherapy After 7 days extubation & swiched to BIPAP First difficulty to synchronize Connected to NAVA (Neurally adjusted ventilatory assist) for 2 days • Switched to BIPAP: P14/4 for 24 hours • Day 14 discharged from PICU • Day 20 discharged from hospital on 16 hrs/D NIV Schneider’s Data • Patients on home ventilation followed by the Pulm. clinic Jan.2007 to Jan 2015 (not ICU). • Diagnosis: 1. Neuromuscular Diseases (NMD) + Thoracic cage abnormalitis 2. OSA + Upper airway anomalies 3. CNS/CCHS 4. Lung disease/CF Results • • • • • • • 49 patients: M 24 (69%) Current age (n:40): median 13.4 yrs (1.1-44) Onset age: median 9.7 (0.1-42) Onset age < 2 → 10 (20%) Onset age > 18 → 8 (16%) Tracheotomy 5:CCHS 3 + Vocal cord paralysis 2 Currently Ventilated 29. 13 (45%)>16 yrs Diagnosis 45.00% 40.00% Diag. Thoracic cage 35.00% 30.00% 25.00% morbid obesity 5 20.00% 15.00% CF 6 10.00% CCHS 3 5.00% 0.00% NMD OSA Lung CNS 9 (18%) 29 (59%) CF 4 NMD 3 Scleroderma 1 5(10%) CNS 1 FD 1 SMA II 2 OSA 1 V.c. paralysis 3 (6%) Died Did not use the NIV Lost for f-U Doing well Stopped (obesity) Indications for ventilation • • • • • Hypoventilation Respiratory failure Recurrent atalectasis & pneumonia Upper airway obstruction OSA Conclusions • Need of Better registry with clear indications, follow up measure.. • A computerized file for NMD & ventilated patients (on way) • Need for written instructions for home care givers (on way) • Better contact with home medical staff Multidisciplinary Center for Neuromuscular Diseases(NMD) at SCMCI • Doctors: Pulmonologists (24/7). Neurologists-Orthopedics • Secretary • Physiotherapist: respiratory + neurodevelopment • Nurse • Social worker • Dietician • Ventilation technicians • Consultants: Genetics, Gastro, Cardiology, Intensive care, anesthesia, radiology. Speech & occupational therapists NMD clinic • • • • 50 pts followed at the pulmonary clinic 20 on cough assist 12 on NIV ventilation+1 tracheotomy (SMA I) Most of NIV started on department or day care • Bach’s protocol is used: Guidelines for pediatric home ventilation • American for children: Panitch HB, Downes J. & al. Ped Pulm. 1996 • Canadian for adults: Douglas A & al. Can Resp J.2012 • German (adults & Pediatric): Windisch W & al. Pneumologie: 2010 ATS pediatric assembly meeting 2013 ATS pediatric Assembly project-2013 • Pediatric chronic home ventilation: Symposium on non-invasive ventilation at ATS. Workshop to develop guidelines on Pediatric chronic home ventilation at ATS. Document to be finished later in the year German Guidelines German Guidelines A hospital to home programCHOP Long term surveys & trends • Children on long term ventilation(LTV) : 10 years of progress, UK. Wallis & al. Arch. Dis. Child. 2011 • Thirty years of home mechanical nentilation in children: escalating need for pediatric intensive care. Netherlands. Paulides & al.Intensive Care Medicine.2012 • Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 Children on long term ventilation(LTV) : 10 years of progress, UK. Wallis & al. Arch. Dis. Child. 2011 • Objectives: To identify the number of Children receiving LTV in the UK. Establish diagnosis & ventilatory requirements. Compare to 1999 data • Subjects: Stable children who needed ventilation after failure to wean for 3 months • Design: Single time electronic questionnaire filled by all UK pediatric LTV party (30) Wallis & al. Arch. Dis. Child. 2011 Total n=993 Wallis & al. Arch. Dis. Child. 2011 Wallis & al. Arch. Dis. Child. 2011 Children on long term ventilation(LTV) : 10 years of progress, UK. Wallis & al. Arch. Dis. Child. 2011 Conclusions • Significant increase in No of children on LTV particularly In NMD pts • This reflects: improving technology & increasing expertise in pediatric NIV ventilation • Substantial number of pts will require transition to adult services Thirty years of home mechanical ventilation in children: escalating need for pediatric intensive care. Netherlands. Paulides & al.Intensive Care Medicine.2012 Aims: To describe trends in pediatric home mechanical ventilation (HMV) and their impact on the use of pediatric intensive care unit (PICU) beds. Paulides & al. Intensive Care Med.2012 Thirty years of home mechanical ventilation in children: escalating need for pediatric intensive care. Netherlands. Paulides & al.Intensive Care Medicine.2012 Conclusions • Impressive increase of HMV • Most obvious in the youngest age with invasive HMV • Needed long stay in PICU Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 • Aims: To report clinical characteristics & trends of children on LTMV followed in Sick Kid Children’s Hospital, Canada. Retrospective study 1991-2011 • Long term mechanical ventilation (LTMV): Daily use of IMV or NIPPV for at least 3 mon. in home or Long term residual facility. • Children on CPAP were not included Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 Results Amin & al. Ped Pulm.2014 Total n= 379 Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 Results Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 Trends • More NIV ventilation • Escalating number of NIV initiated in sleep labs • NMD pts was the most common reason for LTMV • Younger age of ventilated pts Pediatric Long-Term Home Mechanical Ventilation: Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014 conclusion • Exponential growth of children on LTMV with favorable outcome • A registry is needed to design & implement future programmatic change for this medically complex population to ensure best practice for these children & their families Future needs • Establish a national data base for HMV pts • Working group with PICU, Alyn & medical home staff to establish indications & standard of care for children on home ventilation • Special meeting & workshops to improve knowledge & share experience • Transitional programs for adults • Other suggestions? Thanks • Pulmonary Institute staff • PICU staff
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