LIFE AFTER A TRACHEOSTOMY: Caring for your child before and after the trach

1. LIFE AFTER A TRACHEOSTOMY:Caring for your child before and after the trach RICHARD M. KRAVITZ, MD PEDIATRIC PULMONARY AND SLEEP MEDICINE DUKE UNIVERSITY MEDICAL CENTER

2. WORKSHOP OVERVIEW • SMA background • Respiratory care • Non-invasive ventilation • Tracheostomy • Invasive ventilation • Ongoing care • Your child’s future

3. SMA MYTHS(especially SMA Type 1) • You have no hope • You have no choices • There is nothing you can do • Your child is going to die • You have no support • A tracheostomy is a sign of failure

4. SMA FACTS • Treatment options are available (though they may be labor intensive and are more supportive, not curative) • Children with SMA of all types are doing better than ever before • There is currently no cure, but research is underway and therapies might yet be on the horizon • A tracheostomy represents just another a treatment modality

5. TREATMENT OPTIONS FOR SMA TYPE 1:The Big Picture • Take your child home and love them… • Aggressive respiratory support • NIPPV • Airway clearance and cough augmentation • Tracheostomy • Mechanical ventilation • Airway clearance and cough augmentation • Some combination of treatments

6. TREATMENT OPTIONS FOR SMA TYPE 2:The Big Picture • Supportive care is very similar to that used in Duchenne Muscular Dystrophy • Aggressive respiratory support • Airway clearance • Cough augmentation • NIPPV versus tracheostomy • Some combination of treatments • Good long-term outcomes

7. SPINAL MUSCLE ATROPHY BACKGROUND • For SMA Type 1: • left untreated, 90% die before 1 yo • Typical respiratory symptoms: • paradoxical breathing • pectus excavatum • (development of pulmonary hypoplasia) • Respiratory impairment (Bach data): • 10% develop respiratory failure < 6 mo • 70% develop respiratory failure with acute illness • undergo recurrent intubation • failure to be extubated is main cause for trachesotomy • 20% have symptoms between these 2 extremes

8. Scoliosis Restrictive lung disease Impaired mucociliary clearance Diminished cough Increased risk of aspiration/reflux Atelectasis Pneumonia Ventilation/perfusion mismatch Hypoventilation (asleep/awake) Obstructive sleep apnea IMPACT OFNEUROMUSCULAR WEAKNESS

9. Pulmonary function testing Spirometry Lung volumes O2/CO2 analysis Blood gas (arterial, venous, capillary) Pulse oximetry (SaO2) Capnography (EtCO2) Maximal respiratory pressures MEP and MIP Cough Peak Flow Chest radiograph Overnight oximetry/capnography Polysomnogram METHODS TO ASSESS RESPIRATORY MUSCLE FUNCTION

10. Inadequate cough Retained secretions Mucus plugging Atelectasis Hypoxia Respiratory distress Respiratory failure Inadequate airway clearance Quantity of secretions Quality of secretions Inadequate ventilation Hypoxia Hypoventilation Respiratory distress Respiratory failure Cough and Ventilation Worsen With Respiratory Illnesses! CAUSES OF RESPIRATORYMORBIDITY AND MORTALITY

11. INTERVENTIONS TO AIDAIRWAY CLEARANCE • Mucociliary clearance (non-pharmacologic) • Manuel chest physiotherapy • Intrapulmonary percussive ventilation (IPV) • High frequency chest wall oscillation (The Vest) • (bronchoscopy) • Mucociliary clearance (pharmacologic) • Albuterol; Pulmozyme; Mucomyst • Cough augmentation • Inspiratory phase; contraction phase; expiratory phase

12. COUGH AUGMENTATION • Inspiratory component Glossopharyngeal breathing (air stacking) Positive pressure assist (ambu bag) Intermittent positive pressure ventilation (IPPV) Mechanical ventilator • Expiratory component Abdominal/chest wall thrust (less effective with scoliosis) • Combined Mechanical in-exsufflator (Cough Assist)

13. AIRWAY CLEARANCE & COUGH AUGMENTATION

14. RESPIRATORY SYMPTOM PROGRESSION • Normal (asymptomatic) • Weakened cough Usually adequate when well Problems develop with infections Can lead to (recurrent) pneumonia/bronchitis • Nocturnal symptoms • Daytime symptoms

15. RESPIRATORY INTERVENTION PROGRESSION • Normal (asymptomatic) • No treatment needed • Weakened cough • Assist in airway clearance (required with viral illnesses) • Assisted cough • Chest physiotherapy • May require NIPPV (if hypoxic or in respiratory distress) • Nocturnal symptoms • Nocturnal BiPAP or volume ventilator when asleep • Daytime symptoms • Continuous ventilation • Non-invasive versus tracheostomy (in select patients)

16. ASSISTED VENTILATION • Negative pressure ventilation Iron lung/Portalung Cuirass Pneumowrap (Poncho) • Positive pressure ventilation BiPAP or volume ventilator Non-invasive versus invasive route

17. Advantages Non-invasive Reversible Safe Effective Preserves (natural) airway protection mechanisms Decreased risk of infection Comfort Preserves speech Less swallowing issues Disadvantages Lack of access to airway for deep suctioning Aerophagia (worsened reflux) Facial injury Facial bone remodeling from tight fitting mask Increased oral secretions Requires cooperation of the patient POSTITIVE PRESSURE VENTILATION:Advantages of the Non-Invasive Route

18. Nasal/oral dryness Epitaxis Nasal congestion Sneezing Rhinorrhea Sinusitis Claustrophobia Mask irritation Nasal abrasions Aerophagy Mouth leak Facial deformities (Decreased cardiac output) NIPPV COMPLICATIONS

19. TRACHEOSTOMYIndications • NIPPV no longer effective at treating hypoventilation • NIPPV not tolerated by the patient • Excessive oral secretions puts patient at risk for aspiration • Care for patient with NIPPV beyond the capabilities of the family • Resources for outpatient management with NIPPV not available in the community • Failure to extubate patient who has been intubated (for what ever reason). This assumes proper methods of extubation were employed

20. TRACHEOSTOMY

21. TRACHESOSTOMY

22. TRACHEOSTOMY TUBES

23. TRACHEOSTOMY:Advantages for Patient • Allows for assisted ventilation • Airway is protected from aspiration • Increased ease in suctioning excessive secretions (if needed) • No NIPPV mask pressure sores or facial remodeling • Treats obstructive sleep apnea (if present) • In select cases, may be a temporizing measure until extubation is possible

24. TRACHEOSTOMY:Advantages for Family • Freedom • night out for the parents • more mobility • getting to doctor’s office/hospital • shopping • vacations • Increased support in the home • more skilled nursing care • more nursing aid hours • (make sure you have a good case manager)

25. Unsightly Diminished cough Risk of infection Secretions Speech problems trouble understanding child never develop speech loose ability to speak Dysphagia Bradycardia Desaturations Ventilator dependency Loss of airway (if the tube comes out) Increased hospitalizations Suprastomal damage granulation tissue airway collapse Subglottic damage granulation tissue tracheomalacia tracheomegaly tracheal erosions fistula formation Subglottic stenosis Bleeding suction catheter trauma arterial erosion TRACHEOSTOMY:Disadvantages

26. Tracheostomy < 3 years old 0.58 admissions/year > 3 years old 0.21 admissions/year NIPPV < 3 year old 1.52 admissions/year > 3 years old 0.2 admissions/year > 5 years old 0 admissions/year HOSPITALIZATION RATES(Bach Data)

27. TRACHEOSTOMY:Infections • Keeping the tracheostomy site clean • Colonization versus infection (tracheitis versus bronchitis versus pneumonia) • Pseudomonas • Other organisms • Use of antibiotics • Topical versus oral versus IV versus inhaled • Changing out the tracheotomy tube • How frequently (usually weekly) • Indications

28. TRACHEOSTOMY:Mechanical Considerations • Indication for tracheostomy will help determine choice • airway protection • assisted ventilation • Size of tracheostomy tube • small enough to allow for speech • large enough to allow for adequate ventilation • Cuffed or uncuffed (or fenestrated) • uncuffed preferred for children • cuffed with deflated cuff is another option • Humidification • humidifier • artificial nose (HME) • Speaking valve (Passy-Muir Valve) • Swallowing function • speech therapy needs to be involved

29. PASSY-MUIR VALVE

30. TRACHEOSTOMY:Ventilation • Duration of ventilation • Nocturnal (capping tracheostomy tube during the day) • On demand daytime ventilation • Continuous daytime ventilation • Portability of ventilator • Easily attaches to the wheelchair

31. PORTABILITY

32. TRACHEOSTOMY:Ongoing care • Continue airway clearance (as before) • Continue cough augmentation (as before) • Continued aggressive care with URIs (as before) • Continue aiding ventilation • Supplemental O2 is (in general) still not to be used • Routine ENT follow up • Follow up every 6-12 months • Routine bronchoscopy

33. TRACHEOSTOMY:Decannulation??? • More likely to occur in SMA Type 2 • Close ENT follow up required • Two methods of decannulation: • gradual tracheostomy tube downsizing • bronchoscopy and one-step decannulation • Closure of stoma • Acute versus late onset airway obstruction

34. TRACHEOSTOMY:Quality of Life Issues • The family unit • limited data (especially with children) • the perspective varies: • the patient • the parents • the siblings • The medical team • health care providers ≠ the family • health care providers frequently underestimate the patient’s quality of life!

35. EQUIPMENT NEEDED FOR THE HOME • Mechanical in-exsufflator (Cough Assist) • Pulse oximeter • (Airway clearance): Chest PT or The Vest or IPV • BiPAP or volume ventilator (when needed) • Suctioning equipment • Tracheal supplies • (nebulizer)

36. TRACHEOSTOMY:Required Home Equipment • Extra tracheostomy tubes • current size and smaller • Humidification • Suctioning (pre-measured catheters) • (Monitor) • apnea-bradycardia monitor • pulse oximeter • Caregiver education • basic tracheostomy care • CPR

37. TRACHEOSTOMY:Long-term follow up • Otolaryngology • airway care • bronchoscopy • Pulmonary Medicine • Speech Therapy • speech • swallowing function • Respiratory Therapy • equipment review • (Home) Nursing • Social Work

38. Pulmonologist Neurologist Orthopedist Sleep physician 1o Care physician Nursing Nutritionist Respiratory therapist Physical therapist Occupational Therapist Speech Therapist Social work (Palliative care) MULTIDISCIPLINARYHEALTH CARE TEAM

39. KEY POINTS REGARDING A TRACH • Elective tracheostomy is better than emergency tracheostomy • Long-term treatment plan is best determined in times of health. The middle of an acute decompensation (especially one which was predictable) is not the best time to be making these types of decisions

40. THINGS TO REMEMBER • Every child is unique • You are your child’s best advocate • The decisions you make regarding your child’s care are the correct ones for your family • You are part of team caring for your child (actually, you are the captain, so the call is yours), but don’t be afraid to ask for input and be open to your health care team’s recommendations • You are not alone. There are many support groups out there to help you. Get involved!