Study on ventilator cases

1. Challenging
Pediatric
Ventilator Cases
Angela King, BS,
RPFT, RRT-NPS

2. Disclosures
In the past 12 months:
• Consulted for Breas Medical
• Consulted for Hamilton Medical
• Consulted for Ventec Life Systems
• Owner Mobile Medical Homecare
• My husband owns a ventilator repair company

3. Consensus Statements
Disease Published in Title Year
Duchenne
Muscular
Dystrophy
Am Journal Resp Crit
Care Med
Respiratory Care of the Patient
with Duchenne Muscular
Dystrophy
2004
SMA Journal Child
Neurology
Consensus Statement for
Standard of Care for Spinal
Muscle Atrophy
2007
CCHS Am Journal Resp Crit
Care Med
An Official ATS Clinical Policy
Statement: Congenital
Hypoventilation Syndrome
2009
Congenital
Muscular
Dystrophy
Journal Child
Neurology
Consensus Statement on
Standard of Care for Congenital
Muscular Dystrophy
2010

4. Distribution of Dystrophies

5. Duchenne Muscular
Dystrophy (DMD)

6. Best Reference
www.ATSjournals.org
“Although respiratory disease in DMD is its major
cause of morbidity and mortality, there is
inadequate awareness of its treatable nature.
Recent advances in the respiratory care of the
DMD patient have improved the outlook for these
patients, and many caregivers have changed
from a traditional non-interventional approach
to a more aggressive, supportive approach.”

7. Guillaume Benjamin Amand
Duchenne de Boulogne
Drawing of pt with DMD Duchenne with DMD pt

8. Duchenne Muscular Dystrophy
• 1:3000 live births
• X chromosome
• Affects limb and
trunk strength
• Respiratory
insufficiency
• Cause of death 80%
respiratory causes
Bach JR. Noninvasive Mechanical Ventilation,
Hanley & Belfus, Inc. Philadelphia, PA., p. 5

10. DMD
Bach JR. Noninvasive Mechanical Ventilation,
Hanley & Belfus, Inc. Philadelphia, PA., p. 5

11. DMD Milestones
Task Median Range
First walking 18 m 11-24 m
Lordosis 3 y No data
Gowers sign 5 y 1.5 – 7 y
Calf hypertrophy 5 y 3 – 7 y
Waddling gait 6 3 – 9 y
Parker, Analysis of a DMD population, Oxford Journal, 2005.

12. Gower’s Sign

13. DMD Milestones
Task Median Range
Toe walking 7 y 2 – 11 y
Lost walk w/o
assistance
10 y 5 – 13 y
Wheelchair 11 y 7 – 13 y
Scoliosis No data 7 – 13 y
Noc NIV 16 y 13 – 26 y
FVC peaks after the
patient is confined to the
wheelchair full-time,
then progressively falls
Parker, Analysis of a DMD population, Oxford Journal, 2005.

14. DMD Respiratory Progression
Loss of respiratory muscle
strength
Ineffective cough
Nocturnal hypoventilation &
SDB
Daytime Respiratory Failure
Death from Respiratory Failure

15. My Experience
• Rural community – limited Pulmonologists with 24 hour NIV
expertise
• Transportation issues
• Insidious progression
• If nobody told family about MPV, they think only option
beyond nocturnal mask ventilation is a trach
• Often still meet patients on CPAP and/or oxygen

16. What’s Wrong with CPAP & O2
• CPAP makes it harder to exhale
• There is no back-up rate with CPAP
• Supplemental oxygen does not increase ventilation!
• Unless these boys are smokers, there is nothing wrong with
their lung tissue! They don’t have low saturations because
they have COPD!

17. Subtle Signs of Respiratory
Failure
• Reduced energy, weight loss and poor school performance
• Sleep disturbance may increase requests for position
changes
• Chest infections become more frequent
• Once hypercapnia becomes established and is not treated,
symptoms progress to include headaches and more general
malaise
• There is a high risk of death from respiratory failure during
intercurrent infection

18. Mean Age of Death
10
12
14
16
18
20
22
24
26
28
30
Vent +
SS
Vented
in
1990s
1990s
1980s
1970s
1960s
Cardiom
yopathy
Age
at
Death
Bushby K. Current Pediatrics
(2005) 15, 292-300.

19. DMD Survival
Bach, Respiratory Care, 2011
Some patients
used NIV as long
as 25 years

20. DMD
Bach, Respiratory Care, 2011

21. Key Findings Bach Study
Bach, Respiratory Care, 2011
Age 13
• VC peaks
• Daily air-stack, Max insufflation
Age 16
• PCF drops < 300 lpm
• Oximetry & MAC with respiratory
infections
Age 19
• Hypoventilation noted, VC 640
• Nocturnal NIV started
Age 21
• Continuous NIV, VC 320

22. Respiratory Interventions
Step 1: Volume recruitment/deep lung inflation technique
Step 2: Manual and mechanically assisted cough techniques
< 270 LPM
SpO2 < 95% = Hypoventilation, atelectasis, mucus!
Step 3: Nocturnal ventilation
Span > 10, low EPAP, generous set rate, easy trigger
No oxygen!
Step 4: Daytime ventilation
– Needed when can’t speak full sentence
– 2 mask interfaces or mouthpiece + 1 mask
– Mouthpiece 10-15 ml/kg
– Teach breath stacking -- alphabet
Step 5: Tracheostomy??
Lancet Neurol 2010; 9: 177–89.

23. Why Do We Often Get CPAP
Rx?
• All hypopneas are NOT evidence of an obstruction
• Hypoventilation due to muscle weakness results in
decreased saturation; increasing the EPAP will not help!
• Rarely, the patient does have macroglossia, which
increases the likelihood of airway obstruction
– Usually, a high span and generous rate will correct it!

24. Macroglossia
Acta. Neurol. Belg. 2010

25. MPV
• Allows patient to expand their lungs when
needed without having to be attached to a
mask all day
• Using volume mode facilitates stacking
• Avoid the risks associated with invasive
ventilation
• Easier eating & speaking, etc.
• More cosmetically acceptable
• Enhanced quality of life

26. MPV

27. MPV – Safety!

28. Take Home Message
"In general, virtually nobody who can speak should ever
receive a tracheostomy tube because of muscle
weakness; and those who have them should consider
having them removed."
www.doctorbach.com

29. Spinal Muscle
Atrophy

30. What is Spinal Muscle Atrophy?
• Autosomal recessive
neurodegenerative disease
• Lack of protein SMN
(Survival of Motor Neuron)
• Occurs in approximately one
in 6,000 -10,000 live births.
• Kills more babies than any
other genetic disease.

31. SMA
No ethnic or gender
preference

32. Characteristic Head Lag

33. Clinical Classification of SMA
•Symmetrical
•More proximal than distal
•Sensation preserved
•Leg weakness > arm
•Severity correlates with
age of onset

34. Best Reference
Wang C et al, J Child Neurol 2007
Encompasses:
• Diagnosis
• Respiratory Care
• GI and Nutrition
• Orthopedic Concerns
• Palliative Care

35. Type 1 SMA
Clinical Classification of Spinal Muscular Atrophy
SMA Type Age of
Onset
Highest
Function
Natural Age of
Death
Type 1
(severe)
0-6 mo Never sits <2 y
Type 2
(intermediate)
7-18 mo Never stands >2 y
Type 3 (mild) >18 mo Stands and
walks
Adult
Type 4 (adult) 2nd or 3rd
decade
Walks during
adult years
Adult
Wang C et al, J Child Neurol 2007

36. SMA Type I

37. SMA Type I – In the News
The parents of a severely disabled baby boy at the
center of a right-to-life case have thanked the judge for
ruling that he should be kept alive.
They were fighting a hospital's bid to turn off the ventilator
that keeps the child, known only as Baby MB, alive. The 19-
month-old boy has genetic condition spinal muscular atrophy
- which leads to almost total paralysis.
"No court has yet been asked to approve, against the will of
parents, the withdrawal of life support with the inevitable and
immediate death of a conscious child with sensory
awareness and cognition, and no significant evidence of
brain damage.“
News.bbc.co.uk, March 2006

38. Bach Survey SMA Type I QOL
Bach, Am J Phys Med Rehabil 2003

39. Physician Practice – SMA Type I
Hardart, Pediatrics 2002

40. Type 2 SMA
Clinical Classification of Spinal Muscular Atrophy
SMA Type Age of
Onset
Highest
Function
Natural Age of
Death
Type 1 (severe) 0-6 mo Never sits <2 y
Type 2
(intermediate)
7-18 mo Never
stands
>2 y
Type 3 (mild) >18 mo Stands and
walks
Adult
Type 4 (adult) 2nd or 3rd
decade
Walks during
adult years
Adult
Wang C et al, J Child Neurol 2007

41. Chest Wall Changes
Schroth M K Pediatrics 2009;123:S245-S249

42. How Do Kids With SMA
Breathe?
Bell Shaped Chest Sunken Chest or
Pectus Excavatum

43. High Span Ventilation
> 10 cm ∆, low peep, + rate

44. Chest Wall Development After
NIV
6 mths
18 mths
Courtesy of A. Simonds, Royal Brompton Hospital, UK

45. Type 3 SMA
Clinical Classification of Spinal Muscular Atrophy
SMA Type Age of
Onset
Highest
Function
Natural Age of
Death
Type 1
(severe)
0-6 mo Never sits <2 y
Type 2
(intermediate)
7-18 mo Never stands >2 y
Type 3 (mild) >18 mo Stands and
walks
Adult
Type 4 (adult) 2nd or 3rd
decade
Walks during
adult years
Adult
Wang C et al, J Child Neurol 2007

46. Type 4 SMA
Clinical Classification of Spinal Muscular Atrophy
SMA Type Age of
Onset
Highest
Function
Natural Age of
Death
Type 1
(severe)
0-6 mo Never sits <2 y
Type 2
(intermediate)
7-18 mo Never stands >2 y
Type 3 (mild) >18 mo Stands and
walks
Adult
Type 4 (adult) 2nd or 3rd
decade
Walks
during adult
years
Adult
Wang C et al, J Child Neurol 2007.

47. Treatment
Clinical Classification of Spinal Muscular Atrophy
SMA Type Age of
Onset
Highest
Function
Treatment
Type 1
(severe)
0-6 mo Never sits NIV, Trach
Pallative
Type 2
(intermediate)
7-18 mo Never stands NIV, 12-24
hpd
Type 3 (mild) >18 mo Stands and
walks
NIV with resp.
illness
Type 4 (adult) 2nd or 3rd
decade
Walks during
adult years
Usually none
Wang C et al, J Child Neurol 2007.

48. Assessment and Monitoring of
Respiratory Status
Evaluate
Cough
Effectiveness
Direct
observation
Respiratory
muscle
function tests
• MIP, MEP,
peak cough
flow
Physical Exam
Vital
Capacity
Respiratory
rate
WOB*
Paradoxical
breathing
Wang C et al, J Child Neurol 2007.

49. Results of Respiratory Muscle
Weakness in SMA
• Difficulty coughing
• Small, shallow, fast breaths during
sleep
• Chest wall underdevelopment
• Lungs underdevelopment
• Recurrent infections that contribute to
muscle weakness
Pediatrics Volume 123, Supplement 4, 2009

50. Normal
breathing
REM related
sleep disordered
breathing
Non-REM and REM
sleep disordered
breathing
Daytime ventilatory failure
Chest infections
Weak cough,
reduced peak
cough flows
Breathing and swallowing muscle weakness
Physical
examination
Chest xray,
Sleep study
Airway
clearance
with cough
assistance
Swallow difficulty
Swallow function
evaluation
Nocturnal
non-invasive
ventilation
Cotinuous
non-invasive
ventilation
Pulmonary function,
peak cough flow,
respiratory muscle
strength
Death
Assessment
Natural History Intervention
SMA Pulmonary Natural History

51. Do You See the Problem?

52. Nocturnal Bilevel Settings
• Provide backup respiratory
rate
• True respiratory muscle rest
• Set EASIEST trigger
Recommend ST Mode (+/- PRVC)

53. Nocturnal Bilevel Settings
Example settings
• Inspiratory Positive Airway
Pressure (IPAP):
• 14-20 cm of H20
• Expiratory Positive Airway
Pressure (EPAP):
• 3-6 cm of H20

54. Nocturnal Bilevel Settings
Respiratory rate
• Set to capture breathing effort and rest child
• Example settings:
• Infants, often > 30 (SMA I)
• 1-3 years old, RR= 25
• >3 years old, RR= 20-25
• Teenagers to adult, RR=14-16 and
recommend sleep study to titrate.

55. Nocturnal Bilevel Settings
Inspiratory time
• Time over which the breath is
delivered:
• Infant 0.3 – 0.5
• Toddlers to child: 0.8 seconds
• School age: 1 second
• Teen age: 1-1.5 seconds depending
on comfort

56. NIV Challenges
Swallowing difficulty
Excessive secretions
Gastroesophageal Reflux &
Aspiration
> 12-14 hrs/day of ventilation
(vent?)

57. NIV Disadvantages
Gastric distention and emesis
especially if children are
constipated
Nasal bridge discomfort and
other skin irritation
Face changes

58. Hypoplasia
Non-invasive Respiratory Support,
Anita Simonds, 2001, Arnold Press.

59. Sleepnet Mini Me

60. Nasalaire II Petite
Size E

61. Respironics Profile Lite

62. Resmed Mirage Micro for Kids

63. ResMed Pixie

64. University of Wisconsin Protocol
SMA I 2/day SMA II prn SMA III serious illness
1 Cough assist machine (4 sets of 5 breaths)
2 Oral suctioning
3 Secretion mobilization
4 Cough assist machine (4 sets of 5 breaths)
5 Oral suctioning
6 Postural drainage (Tredelenburg) 15-20 min
7 Cough assist (4 sets of 5 breaths)
8 Oral suctioning

65. FRC Relative to Position
From Nunn’s Applied Respiratory Physiology, 2000

66. When is a Tracheostomy Needed
for SMA?
Recurrent infection (after optimal management)
Severe bulbar involvement
Inability to tolerate NIV for the hours required
Ineffective therapy despite optimal management
Retained secretions

67. Scoliosis
• Common in children
with SMA type I & II
(50%)
• More common in
non-ambulators
• Can make sitting
difficult
• Can impact
respiratory function
67

68. Spinal Bracing

69. Neater Eater

70. On a 1-7 scale, rate your life-satisfaction:
1 = very dissatisfied
7 = very satisfied
Dr. Bach Study: 621 vent users, 256 HCW

71. Meet MJ.
On a 1-7 scale, rate
what you think her
life-satisfaction is:
1 = very dissatisfied
7 = very satisfied

72. Guide to the Evaluation and Management of Neuromuscular Disease
by John R. Bach, MD, FAAPMR, FCCP, pages 131 - 137
0
1
2
3
4
5
6
7
NPPV Trach HCW HCW re: Vent
User
5.04
4.68
5.33
2.42

73. MJ

74. Summary for Peds NMD
• If PCF < 300, start Cough Assist
Work up to +40, -40 or even higher
• Nocturnal ventilation: High span (> 10 cm), minimal PEEP,
generous respiratory rate, easiest trigger
– No CPAP! No oxygen!
• Daytime ventilation – best with MP (no rate, no PEEP, best
with volume breaths, teach breath stacking)
• If SpO2 < 94%, either:
– Mucus accumulation
– Atelectasis
– Hypoventilation
• Transition to ventilator @ ~ 12-14 hpd
• Consider alarms, even for NIV!

75. Congenital Central
Hypoventilation
Syndrome

76. American Thoracic Society Documents
An Official ATS Clinical Policy Statement: Congenital
Central Hypoventilation Syndrome
Genetic Basis, Diagnosis, and Management
Debra E. Weese-Mayer, Elizabeth M. Berry-Kravis, Isabella Ceccherini,
Thomas G. Keens, Darius A. Loghmanee, and Ha Trang, on behalf of the
ATS Congenital Central Hypoventilation Syndrome Subcommittee
Best Reference

77. Congenital Central
Hypoventilation Syndrome
• About 1 in 200,000 live born children have the condition
• While asleep, children with CCHS experience progressive
hypercapnia and hypoxemia
• Children with CCHS lack arousal responses and sensations
of dyspnea; they do not exhibit signs of respiratory distress
when challenged with hypercarbia or hypoxia
• Require life-long ventilation support during sleep

78. Modalities
• Positive pressure ventilation via tracheostomy
• Non-invasive positive pressure ventilation (bi-level
ventilation)
– After 6-8 years of age
• Negative pressure ventilation
– After 6-8 years of age
• Diaphragmatic pacers
Oxygen is not ventilatory support!

79. Negative Pressure Vent

80. CHRONIC VENTILATORY
SUPPORT
• All CCHS patients require assisted ventilation during sleep
• Weaning these patients off mechanical ventilation totally is
not a realistic goal
• Ventilators are adjusted to provide ETCO2 consistently
between 30– 35 and SpO2 greater than 95%
• Optimal ventilation avoids atelectasis and the development
of co-existing lung disease
• Children who are hyperventilated at night have better
spontaneous ventilation while awake
• Weaning of daytime assisted ventilation is best
accomplished by sprint weaning
– 2-4 x/ day. Stop: SpO2 < 95%, CO2 > 45-50, distress

81. CLINICAL COURSE &
PROGNOSIS
• CCHS infants may be very unstable
• All patients with CCHS will need supported ventilation while
asleep
• PPV with acute illnesses
• Children with CCHS can have prolonged survival, with
several patients now in young adulthood
• The outlook for these CCHS children is encouraging
65% come off daytime ventilation