Respiratory%20management%20in%20neuromuscular%20disease

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Respiratory management in neuromuscular disease
Teeradej Kuptanon,MD Division of Pulmonary and
Critical care Department of Pediatrics
Faculty of Medicine Ramathibodi Hospital
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Respiratory management in neuromuscular disease

• Medical problems
• Psychosocial problems
• Ethical problems

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Medical problems

• Specific treatment
• Respiratory
• Gastroesophageal reflex
• Constipation
• Pulmonary Hypertension
• Malnutrition
• Scoliosis

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Non-Cardiopulmonary Complications in Prolonged
Survivors of Duchene Muscular Dystrophy.
Complication Number of patients
Therapeutic interventions Malnutrition/dysphagia
15 Gastrostomy tube

placement Nephrolith
iasis 6
Lithotripsies, cystoscopies,

ureteral stents Diabetes mellitus 2
Insulin Deep venous thrombosis
2 Anti-coagulation Gall
stones 1
Potential need for
surgery Inflammatory bowel disease 1
Medical
Ped pulmonology411113-1117(2006)
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Respiratory

• Improve respiratory muscle function
• Mucus clearance technique

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Improve respiratory muscle function

• Non invasive positive pressure ventilation
  (NIPPV)
• Respiratory muscle training
• General nutrition
• electrolyte
• oxygen
• sleep position

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NIPPV

• Indication PSG
• FEV1, FVC1 lt 40
• PaCO2 gt 45 mmHg
• Base excess gt 4 mmol/L
• Indication ?????? NIPPV
• Symptomatic day time hypercapnia
• Nocturnal hypercapnia PaCO2 gt 50 mmHg, gt 50 TST

George CF, Guilleminault C. Sleep and
Neuromuscular Diseases. In Principles and
practice of sleep medicine, 20058318.
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Respiratory muscle training
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normal subjects, normal controls, NMD
subjects, NMD controls.
Praud JP, et al. Kendigs Disorders of the
Respiratory Tract in Children, 2006733-746.
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Mucus clearance technique

• Physical therapy
• Cough assist

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Psychosocial problems
Neurologist Pulmonologist Cardiologist Orthopedist Nutritionist Nurse practitioner
Care manager Social worker Physical therapist Occupational therapist Speech therapist
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Ethical problems

• End of life care

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Case conference ?.?. ???? 6 ?? ???????
juvenile rheumatoid arthritis with
encephalopathy ??????? admit ???????
pseudomonas septicemia ????????? ceftazidime
amikacin ?????????????? ??????????????
meropenem
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?????? 7 ?? ??. ?????????????? stridor ????????
??????????????? ?? fiberoptic bronchoscopy ?????
arythenoids ??? ??? ??????????????? arythenoids
collapse ??? vocal cord ?????? chronic
aspiration ??? gastroesophageal reflux (GER)
???????? PICU
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Physical examination inspiratory and expiratory
stridor, RR 36 /min poor air entry, prolonged
inspiratory phase ??? dexamethasone, adrenaline,
pulmicort neb ???????? upper airway obstruction
?????????
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Problem lists

1. Upper airway obstruction due to laryngomalacia
   secondary to GER
2. Encephalopathy with central hypoventilation

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???????????????????????? ?????????????????
A. CPAP B. BiPAP
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24 hour BiPAP spontaneous mode IPAP 20 EPAP 8
cmH2O
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CASE CONFERENCE

• 6-year-old girl with
• cyanosis during sleep

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• CC ????????????????????? 5 ?????
• PI 5 ???????????????????? ????????????????????
  ???? 18.00 ?. ?????????????????????????? ??? 30
  ???? ????????????????????? ????????????????
  ?????????? ????????????? ?????????????????????????
  ?????????????

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• Physical examination
• General Appearance Obese(W/H 169 ), eumorphic,
  sleepy girl with shallow breath, but decreased
  and paradoxical chest movement without snoring.
• Vital signs
• BT 37? C, PR 128/min,
• RR 28/ min, BP 100/ 60 mmHg
• fall asleep at OPD SpO2 64
• awake SpO2 95 (room air)

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• Physical examination
• HEENT not pale, no engorged neck vein
• HEART normal S1 S2 , no murmur
• LUNGS clear
• ABDOMEN no organomegaly
• EXTREMITIES no digital clubbing

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What do you plan to do next?
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negative inspiratory force -20 cmH2O
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Management On nasal BiPAP 15/5 cmH2O
RR 20 / min
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Thank you

• for your attention