Home ventilatory management
in pediatric neuromuscular
and other lung diseases
Dr. Huda Mussaffi-Georgy
Pulmonary Institute
Schneider Children’s Medical Center
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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.
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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
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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
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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
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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