SGH-RTs

1. Ching-Lung Liu, MD
Department of Chest Medicine
Mackay Memorial Hospital
劉景隆 醫師
胸腔內科
馬偕紀念醫院
NIV in
Neuromuscular Disease:
From acute to chronic

4. Chronic NIV in
Neuromuscular Disease
Overview

5. Timeline of development in NIV from the 1980s
MD: muscular dystrophy ERS practical handbook of NIV, 2015

6. Duchenne muscular dystrophy survival by
intervention
ERS practical handbook of NIV, 2015

7. Interface types for NIV
Oronasal Mask Total-Face Mask
Nasal Mask Nasal Pillows
N Engl J Med 2015; 372:e30

8. Respir Care 2006;51(8):896–911.
Bi-level ventilator used for NIV
• Single hose
• Ability to function correctly with leaks
• PSV (or PCV), setting the IPAP (≤ 30 cmH2O) and EPAP (≤ 15 cmH2O)
• Rebreathing, resolved by PEEP ( ≧4 cmH2O) or using a valve
• Supplemental oxygen is usually not necessary in NMD

9. Common parameters and their relationship to the
normal respiratory cycle
ERS practical handbook of NIV, 2015

10. To maintain patient-device synchrony
Trigger
• Auto-triggering: leaks
• Failure to trigger: muscle
weakness, intrinsic PEEP, or a
high level of support
• Back-up rate: recommended
Rise Time
• In patients with NM: a slower
rise time is often better
tolerated
Cycle
• Mask leak should be minimized
• Maximum inspiratory time is
useful
Humidification
• Addition of heated
humidification was associated
with fewer symptoms
Respir Care 2006;51(8):896–911.

11. N Engl J Med 2015; 372:e30
Real-time data in a standard NIV

12. Cause of respiratory failure
ERS practical handbook of NIV, 2015

13. Cause of respiratory failure
ERS practical handbook of NIV, 2015
OHS
Chest wall
disorder
NMD
Obesity
hypo-
ventilation
Scoliosis
Pompe
disease

14. Chronic NIV in
Pompe Disease

15. Brief History
• Female, born on 1974/July/19
• Age 16 (1991), proximal lower limbs weakness (first recognizable
abnormality)
• Age 24 (1999), repeated respiratory infections and respiratory
failure
• Age 30 (2005), necessitated mechanical ventilation through a
tracheostomy
• Age 30 (2005), late‐onset Pompe disease (LOPD) was diagnosed
• Age 22 (1997), difficulty climbing stairs, positive Gowers’ sign
(first symptom)

16. “Pompe Disease”
Carbohydrates from food
Glucose
Glucogen
Energy
GAA
Glucose
Energy
lysosome
Glucogen
build-up
Pompe
Disease
Glycogen storage disease type II, also called Pompe disease, is an autosomal
recessive metabolic disorder, identified in 1932 by the Dutch pathologist JC Pompe.
Yuan-Tsong
Chen
Recombinant enzyme
replacement therapy,
at Duke University in 1999.
“Myozyme”,
FDA approval in 2006.

17. Brief History
• Age 33 (2008), enzyme replacement therapy (ERT) with
alglucosidase alfa (Myozyme) with 20‐mg/kg infusions biweekly
• 3 months after ERT, she could walk short distances independently
and climb 18 standard 6‐inch stairs
• 4 months after ERT, tracheostomy was closed and then removed
• 12 months after ERT, she could climb 24 standard stairs and do
domestic activities
Myozyme was adjusted to 20 mg/kg every 2-4 weeks
• But, she still needs a noninvasive positive pressure ventilation
(NIPPV), especially during sleep

18. X-ray Imaging
2018/4/19
2018/5/82017/3/25
2017/3/23
2016/5/24
2016/5/192007/10/29
2008/1/7

19. Lung Function Testing
2012/3/14 2014/7/22 2015/4/7 2016/5/20 2017/3/24 2018/5/8
Spiromtery
FVC, % 32 28 28 38 34 29
FEV1, % 36 33 33 45 38 35
FEV1/FVC, % 94 97 97 99 91 97
PEF, % 47 39 51 69 56 53
Lung Volumes
VC, L (%) 1.27 (33) 1.06 (28) 1.25 (33) 1.39 (38) 1.29 (34) 1.10 (29)
RV/TLC, % 64 60 60 36 60 48
Diffusion
DLco, % 35 38
DLco/VA, % 92 106
Restrictive lung disease, extra-pulmonary, severe

20. Q1:
Why does the patient need NIV
during sleep?
A: There is an insufficient breathing during sleep.
B: There is an insufficient breathing in the supine
position.
C: There is an insufficient breathing in the supine
position during sleep.

21. Peak Expiratory Flow Rate Measurement
before and after sleep study
Lie down
Pompe disease Obese and OSA
Before sleep study at night (2018/5/9)
PEFR in sitting upright, L/min 239 ± 10 336 ± 26
PEFR in lying down, L/min 125 ± 7 283 ± 13
After sleep study in the morning (2018/5/10)
PEFR in sitting upright, L/min 208 ± 8 235 ± 43
PEFR in lying down, L/min 177 ± 51 216 ± 50
52% 84%
85% 92%
PEFR decreased in lying down, characterized by diaphragmatic weakness
Sit upright

22. Peak Expiratory Flow Rate Measurement
morning and evening
0
50
100
150
200
250
300
PEFR,L/min
Pompe disease
Moring PEFR, L/min 212.4 ± 15.4
Evening PEFR, L/min 187.1 ± 12.3
PFER decrease in the evening, characterized by disease activity
5/3
ERT
5/31
ERT

23. BPAP Titration Algorithm
↑EPAP to open
the airway
↑IPAP (∆PAP)
to maintain
ventilation
Switch to auto
mode
(iVAPS/ASV)
Switch back to
BiPAP mode

24. Q2:
To maintain ventilation, the tidal
volume (VT) ~
A: Either wake or sleep, VT are the same
B: Ventilation falls and VT decreases during sleep

25. Ventilation falls during sleep
Thorax. 1982 Nov;37(11):840-4.
MV during sleep in normal man
Minute ventilation falls during sleep, the
greatest reduction occurring during REM
sleep.
VT during sleep in OSA
S1 S2 SWS REMS p value
Tidal
volume,
mL
426.6 ±
32.5
416.9 ±
32.3
361.5 ±
17.4
285.8 ±
21.8
p <0.05
Tidal
volume,
mL/kg
IBW
6.8 ±
0.6
6.6 ±
0.5
5.7 ±
0.4
4.5 ±
0.6
p <0.05
0
100
200
300
400
500
S1 S2 SWS REM
Tidalvolume,mL
Sleep Lab, MacKay Memorial HospitalSet target VT: 5 ~ 7 ml/kg

26. BiPAP Settings
EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower

27. Q3:
In neuromuscular disease, the
settings of “Rise Time”, “Trigger”,
and “Cycle” ~
A: Rise time as 250 ms, Trigger as Medium, and
Cycle as Medium
B: Rise time short as 150 ms, Trigger Medium,
and Cycle as High
C: Rise time prolonged as 300 ms, Trigger High,
and Cycle as Low

28. BiPAP Settings
Normal
Ti 0.3 ~ 2.0 s
Rise time 250 ms
Trigger Medium
Cycle Medium
COPD
Ti 0.3 ~ 1.0 s
Rise time 150 ms
Trigger Medium
Cycle High
Restrictive/
NMD
Ti 0.8 ~ 1.5 s
Rise time 300 ms
Trigger High
Cycle Low
EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower
Medical Devices: Evidence and Research 2015:8 425-437

29. BiPAP Settings
EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower
Disease
IPAP/EPAP
RR
Rise time
Trigger
Cycle
Tidal volume
Pompe Disease
15/5 cmH2O
15
300
H
L
0.38

30. Chronic NIV in
Scoliosis

31. Disorders causing chest wall deformities
ERS practical handbook of NIV, 2015

32. Survival in patients with chest wall disorders
treated long-term mechanical ventilator is good
ERS practical handbook of NIV, 2015HMV: home mechanical ventilation
LTOT: long-term oxygen therapy

33. Brief History
◆Chief Complaint:
Both lower legs swelling for 2~3 months.
◆Present Illness:
She traveled to Australia and had herpes zoster, 2015/12.
SOB for 1 month; symptoms progressed in 1+ week
Orthopnea and night cough occurred.
◆ Surgical history: Scoliosis(+), surgery twice
◆ She visited Nepho OPD on Jan/12/2016.
Lasix and NTG pump was given
She admitted to ward.

34. 2016/01/12
醫囑時間 : 2016/01/12 12:35
簽收/採檢時間 : 2016/01/12 12:48
報告時間 : 2016/01/12 12:57
備註: ER採血
項目名稱 結果值 單位
-------------------------------------------
Blood Gas
pH 7.360
pCO2 HH 81 mmHg
pO2 LL 25 mmHg
HCO3 HH 46 mmol/L
B.E. H 16.5 mmol/L
SaO2 42.0 %
ABG DATA
檢驗日期：2016/01/12 20:15
執行項目：83017 動脈血
檢體編號：0112212825
氧氣治療種類：Aerosal Mask
濃度：100% %
項目名稱 結果值 單位
----------------------------------------
pH 7.218
PaCO2 HH 125.4 mmHg
PaO2 LL 38.5 mmHg
HCO3 49.9 mmol/L
BE 22.1 mmol/L
SaO2 57.2 %

35. 醫囑時間:2016/01/12 12:35
簽收/採檢時間:2016/01/12 12:48
報告時間:2016/01/12 13:17
備註:ER採血
項目名稱 結果值 單位
-----------------------------------------
[Blood]
Hemoglobin H 16.4 g/dL
Platelet 231 10^3/uL
WBC H 10.1 10^3/uL
醫囑時間:2016/01/12 12:35
簽收/採檢時間:2016/01/12 12:48
報告時間 : 2016/01/12 13:09
備註:ER採血
項目名稱 結果值 單位
-----------------------------------------
Glucose AC H 126 mg/dL
AST(GOT) H 66 IU/L
Troponin-I 0.027 ng/mL
BUN H 25.7 mg/dL
Creatinine 0.80 mg/dL
Potassium 4.57 mEq/L
Sodium 138.3 mEq/L
BNP H 731 pg/mL 醫囑時間:2016/01/12 15:06
簽收/採檢時間:2016/01/12 15:0
報告時間:2016/01/12 16:07
備註:ER採血
項目名稱 結果值 單位
-----------------------------------------
CRP 0.51 mg/dL
Lab Data (2016/01/12)

36. 2D Echocardiogram
RV, RA dilatation

37. Estimating pulmonary artery pressure
PA systolic pressure: 81.9 mmHg

38. 醫囑時間:2016/01/13 04:44
簽收/採檢時間:2016/01/13 04:49
報告時間:2016/01/13 04:55
備註:病採
項目名稱 結果值 單位
--------------------------------------------------
Hemoglobin H 16.6 g/dL
WBC H 17.2 10^3/uL
WBC-DC
Seg H 85.8 %
Eosin 0.0 %
Baso 0.3 %
Mono 8.4 %
Lymp L 5.5 %
醫囑時間:2016/01/13 06:00
簽收/採檢時間:2016/01/13 04:42
報告時間:2016/01/13 05:35
備註:病採
項目名稱 結果值 單位
------------------------------------------------
Glucose AC H 137 mg/dL
Albumin 4.27 g/dL
Total Protein L 5.81 g/dL
Direct Bilirubin 0.22 mg/dL
Total Bilirubin 1.05 mg/dL
AST(GOT) H 68 IU/L
ALT(GPT) H 229 IU/L
Total Cholesterol H 231 mg/dL
Triglyceride 103 mg/dL
Uric Acid H 8.44 mg/dL
LDL-Cholesterol H 169 mg/dL
HDL-Cholesterol L 37 mg/dL
Ammonia 55 ug/dL
BUN H 25.9 mg/dL
Creatinine 0.93 mg/dL
Potassium 3.84 mEq/L
Sodium 142.3 mEq/L
檢驗日期：2016/01/13 04:37
執行項目：83017 動脈血
檢體編號：0113064928
氧氣治療種類：Cannula
流速：3L
項目名稱 結果值 單位
--------------------------------------------
pH 7.274
PaCO2 104.7 mmHg
PaO2 67.1 mmHg
HCO3 47.4 mmol/L
BE 14.9 mmol/L
SaO2 89.1 %
Lab Data
Day 2 (2016/01/13)

39. 檢驗日期：2016/01/13 04:37
執行項目：83017 動脈血
檢體編號：0113064928
氧氣治療種類：Cannula
流速：3L
項目名稱 結果值 單位
--------------------------------------------
pH 7.274
PaCO2 104.7 mmHg
PaO2 67.1 mmHg
HCO3 47.4 mmol/L
BE 14.9 mmol/L
SaO2 89.1 %
檢驗日期：2016/01/13 13:59
執行項目：83017 動脈血
檢體編號：0113141529
氧氣治療種類：Cannula
流速：3L
項目名稱 結果值 單位
--------------------------------------------
pH 7.335
PaCO2 94.8 mmHg
PaO2 72.5 mmHg
HCO3 49.5
BE 18.0 mmol/L
SaO2 92.5 %
檢驗日期：2016/01/14 06:01
執行項目：83017 動脈血
檢體編號：0114074717
氧氣治療種類：Cannula
流速：3L
項目名稱 結果值 單位
--------------------------------------------
pH 7.311
PaCO2 114.6 mmHg
PaO2 64.8 mmHg
HCO3 56.5 mmol/L
BE 23.0 mmol/L
SaO2 88.6 %
檢驗日期：2016/01/14 15:41
執行項目：83017 動脈血
檢體編號：0114154555
氧氣治療種類：BiPAP
濃度：ST/5L/IP16/EP5/RR20
流速：5L
項目名稱 結果值 單位
--------------------------------------------
pH 7.450
PaCO2 81.5 mmHg
PaO2 75.6 mmHg
HCO3 55.4 mmol/L
SaO2 94.9 %
ABG Day 2 (2016/01/13) ABG DAY3(2016/01/14)

40. 檢驗日期：2016/01/15 09:20
執行項目：83017 動脈血
檢體編號：0115092426
氧氣治療種類：BiPAP
濃度：ST 流速：7L
項目名稱 結果值 單位
-------------------------------------------
pH 7.402
PaCO2 79.1 mmHg
PaO2 93.7 mmHg
HCO3 48.2 mmol/L
BE 23.4 mmol/L SaO2
96.8 %
檢驗日期：2016/01/15 14:16
執行項目：83017 動脈血
檢體編號：0115141953
氧氣治療種類：Cannula
流速：3
項目名稱 結果值 單位
-------------------------------------------
pH 7.333
PaCO2 100.1 mmHg
PaO2 97.8 mmHg HCO3
52.0 mmol/L
BE 26.1 mmol/L SaO2
96.5 %
檢驗日期：2016/01/16 09:28
執行項目：83017 動脈血
檢體編號：0116093236
氧氣治療種類：BiPAP
濃度：IP14/EP5/7L
項目名稱 結果值 單位
--------------------------------------------
pH 7.400
PaCO2 80.5 mmHg
PaO2 80.7 mmHg
HCO3 48.7 mmol/L
BE 23.9 mmol/L
SaO2 95.3 %
檢驗日期：2016/01/17 22:18
執行項目：83017 動脈血
檢體編號：0117232535
氧氣治療種類：Cannula
流速：2
項目名稱 結果值 單位
--------------------------------------------
pH 7.352
PaCO2 91.4 mmHg
PaO2 92.9 mmHg
HCO3 49.6 mmol/L
BE 24.1 mmol/L
SaO2 96.2 %
ABG DAY 4 (2016/01/15) ABGDAY5(2016/01/17)

41. Polysomnography
AHI:48.9,
TcCO2:56.2~80.0mmHg
CPAP/Bi-level titration
IPAP/EPAP:22/7cmH2O,
RR:25/min, Ti:0.6-1.0sec

42. CHEST CT SCAN (2016/01/29)

43. CHEST CT SCAN (2016/01/29)

44. Lung function test (2016/9/19)
Restrictive lung disease, extra-pulmonary, severe

45. Diagnosis
• Respiratory failure on Bi-level PAP
• Sleep Related Hypoventilation Due to
Medical Disorder
• Scoliosis
• Pulmonary hypertension
• Polycythemia (erythrocytosis)

46. Polysomnography
AHI:0.4,
TcCO2:33.0~49.8mmHg
CPAP/Bi-level titration
IPAP/EPAP:15/6cmH2O,
RR:22/min, Ti:0.7-1.4sec

47. Estimating pulmonary artery pressure
PA systolic pressure: 34 mmHg

48. BiPAP Settings
EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower
Disease
IPAP/EPAP
RR
Rise time
Trigger
Cycle
Tidal volume
Pompe Disease
15/5 cmH2O
15
300
H
L
0.38
Scoliosis
15/6 cmH2O
22
300
H
L
0.26

49. Chronic NIV in
Obesity Hypoventilation

50. ERS practical handbook of NIV, 2015
Severe OSA Lone OHS Combine OSA and OHS
Obesity-related chronic respiratory failure

51. ERS practical handbook of NIV, 2015

52. 2019/01/14 2019/01/222019/01/11 2019/01/16
• The patient admitted to 中興醫院 with influenza pneumonia, acute
respiratory failure, asthma with AE and obesity during Jan 11~24, 2019.
• She visited Chest OPD on Jan/24/2019
• Presenting Symptoms: big snoring, apnea during sleep for a long time
• Height:151cm, Weight: 144kg, BMI: 61.8
Friedman palate position: III
Tonsils size: 3
Brief History

53. 2019/01/31
2019/01/30
Lung function(2019/01/30)
Restrictive lung disease, extra-pulmonary, mild

54. Sleep study(2019/02/20)
Polysomnography
AHI:153.1,
TcCO2:50.8~74.9mmHg

55. Sleep study (2019/03/07 vs 2019/02/20)
CPAP/Bi-level titration
IPAP/EPAP:25/13cmH2O,
RR:18/min, Ti:0.8-1.7sec

56. BiPAP Settings
EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower
Disease
IPAP/EPAP
RR
Rise time
Trigger
Cycle
Tidal volume
Pompe Disease
15/5 cmH2O
15
300
H
L
0.38
Scoliosis
15/6 cmH2O
22
300
H
L
0.26
Obesity hypoventilation
25/13 cmH2O
18
300
H
L
0.44

57. SUMMARY & DISCUSSION
Bi-level mechanical ventilator therapy for
NMD, chest wall disorders, and OHS

58. Duchenne muscular dystrophy survival by
intervention
ERS practical handbook of NIV, 2015

59. To maintain patient-device synchrony
Trigger
• Auto-triggering: leaks
• Failure to trigger: muscle
weakness, intrinsic PEEP, or a
high level of support
• Back-up rate: recommended
Rise Time
• In patients with NM: a slower
rise time is often better
tolerated
Cycle
• Mask leak should be minimized
• Maximum inspiratory time is
useful
Humidification
• Addition of heated
humidification was associated
with fewer symptoms
Respir Care 2006;51(8):896–911.

60. BPAP Titration Algorithm
↑EPAP to
open the
airway
↑IPAP (∆PAP)
to maintain
ventilation EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower
VT
1 Rise time2 3 Trigger, Cycle

61. BiPAP Settings
EP
IP
Ti
Rise time
Flow Flow
Trigger sensitivity Cycle sensitivity
Higher Lower
Higher Lower
Disease
IPAP/EPAP
RR
Rise time
Trigger
Cycle
Tidal volume
Pompe Disease
15/5 cmH2O
15
300
H
L
0.38
Scoliosis
15/6 cmH2O
22
300
H
L
0.26
Obesity hypoventilation
25/13 cmH2O
18
300
H
L
0.44

62. Control
COPD
Obesity
Restrictive
NMD
• I time↓
• Rise time↓
• ∆PAP↑
• EPAP↑
• ∆PAP↑
• RR↑
• Rise time↑
• ∆PAP↑
• Rise time↑
Bi-level Ventilator Settings
Ti 0.3 ~ 2.0 s
Rise time 250 ms
Trigger Medium
Cycle Medium
Ti 0.3 ~ 1.0 s
Rise time 150 ms
Trigger Medium
Cycle High
Ti 0.8 ~ 1.5 s
Rise time 300 ms
Trigger High
Cycle Low

63. Thank You
Ching-Lung Liu, MD
clliu.5839@gmail.com
Division of Chest, Department of Internal Medicine
Mackay Memorial Hospital