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NIV in NM Disease
- 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
- 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
- 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)
- 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)
- 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
- 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