Home ventilatory managementin 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 ventilatoryassist) 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

morbid obesity 5

CF 6

CCHS 3

0.00%

5.00%

10.00%

15.00%

20.00%

25.00%

30.00%

35.00%

40.00%

45.00%

NMD OSA Lung CNS

Diag.

Thoracic cage

9 (18%)CF 4

NMD 3Scleroderma 1

CNS 1 5(10%)FD 1

SMA II 2OSA 1

V.c. paralysis3 (6%)

29 (59%)

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 program-CHOP

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

Wallis & al. Arch. Dis. Child. 2011

Total n=993

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

Paulides & al. Intensive Care Med.2012

Aims: To describe trends in pediatric home mechanical ventilation (HMV) and their impacton the use of pediatric intensive care unit (PICU) beds.

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

Amin & 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

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

• 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

Trends

• 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

Pediatric Long-Term Home Mechanical Ventilation:Twenty Years of Follow-Up From One Canadian Center. Amin R & al. Ped. Pulm.2014

conclusion

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