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KEEPING YOUR LUNGS HEALTHY: SMA TYPE I. Mary Schroth MD Pediatric Pulmonary American Family Children’s Hospital University of Wisconsin School of Medicine and Public Health. Objectives. Understand how SMA affects breathing Learn techniques to help your child’s breathing

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keeping your lungs healthy sma type i

KEEPING YOUR LUNGS HEALTHY: SMA TYPE I

Mary Schroth MDPediatric PulmonaryAmerican Family Children’s HospitalUniversity of Wisconsin School of Medicine and Public Health

objectives
Objectives
  • Understand how SMA affects breathing
  • Learn techniques to help your child’s breathing
  • Realize that you have choices in your child’s respiratory care
slide3

Encompasses:

    • Diagnosis
    • Respiratory Care
    • GI and Nutrition
    • Orthopedic Concerns
    • Palliative Care

Wang C et al, J Child Neurol 2007; 22:1027.

respiratory function in sma
Respiratory Function in SMA
  • Features:
    • Very weak muscles between the ribs
    • Chest wall: very soft and flexible during the first year of life
    • Diaphragm: primary muscle used to breath
results of respiratory muscle weakness in sma
Results of Respiratory Muscle Weakness in SMA
  • Difficulty coughing
  • Small shallow breaths during sleep
  • Chest wall and lung underdevelopment
  • Recurrent infections that contribute to muscle weakness.

Wang C et al, J Child Neurol 2007; 22:1027.

slide7

SMA Pulmonary Natural History

Natural History

Assessment

Intervention

Physical examination

Normal breathing

Breathing and swallowing muscle weakness

Pulmonary function, peak cough flow, respiratory muscle strength

Airway clearance with cough assistance

Dream related sleep disordered breathing

Weak cough,

reduced peak cough flows

Chest xray,

Sleep study

Nocturnal non-invasive

ventilation

Swallow function evaluation

Non-dream and dream sleep disordered breathing

Swallow dysfunction

Chest infections

Nocturnal or continuous non-invasive ventilation

Daytime ventilatory failure

Death

Wang C et al, J Child Neurol 2007; 22:1027.

assessment and monitoring of respiratory status
Assessment and Monitoring of Respiratory Status
  • Evaluate Cough Effectiveness
    • Direct observation
  • Physical Exam
    • Respiratory rate
    • Work of breathing
    • Paradoxical breathing

J. Child Neurol 2007; 22:1027.

assessment and monitoring of respiratory status cont
Assessment and Monitoring of Respiratory Status (cont.)
  • Chest xray
  • Swallow study
  • Polysomnography or sleep study
  • Scoliosis xrays

J. Child Neurol 2007; 22:1027.

chronic management
Chronic Management
  • Discussion of family goals
    • Balancing:
      • Caring at home for as long as possible
      • Long term survival
      • Quality of life and comfort
      • Resources to support above

Wang C et al, J Child Neurol 2007; 22:1027.

chronic management goals
Chronic Management Goals
  • Normalize oxygen saturation and CO2
  • Improve sleep
  • Facilitate care at home
  • Decrease hospitalizations and PICU stays
  • Decrease burden of illness on families

Wang C et al, J Child Neurol 2007; 22:1027.

based on experience and consensus
Based on Experience and Consensus:
  • Early aggressive and proactive intervention may prolong life without compromising quality of life.

Wang C et al, J Child Neurol 2007; 22:1027.

chronic management13
Chronic Management
  • Recognizing changes from baseline
  • Airway clearance
    • Assisted Cough
    • Secretion mobilization
  • Respiratory support
  • Palliative care

Wang C et al, J Child Neurol 2007; 22:1027.

assisted cough
Assisted Cough
  • Manual cough assist
  • In-exsufflator cough machine or Cough Assist
mechanical in exsufflation
Mechanical In-Exsufflation
  • In-exsufflator cough machine improved cough expiratory flow rates
    • Mean peak expiratory flow rates of 21 patients with NMD
      • Unassisted 1.81 ± 1.03 L/sec
      • Assisted cough 4.27 ± 1.29 L/sec
      • Exsufflator 7.47 ± 1.02 L/sec
      • Normal PCF 6-12 L/sec
      • Critical PCF is 2.7 L/sec

Bach J Chest 1993; 104:1553.

cough assist device
Cough Assist Device
  • SETTINGS to use by mask, mouth piece, tracheostomy tube or endotracheal tube.
    • INHALE
      • Start at +30, increase to +40 cm H2O for 1 sec.
    • EXHALE
      • Start at –30, increase to -40 cm H2O for 1 sec.
    • PAUSE TIME
      • 1-2 sec.
secretion mobilization techniques
Secretion Mobilization Techniques
  • Manual Chest Physiotherapy or Mechanical Percussion
  • Postural Drainage
  • Use pulse oximetry to guide respiratory therapy.
  • Do before eating or at least 30 minutes after eating.

Wang C et al, J Child Neurol 2007; 22:1027.

other techniques not proven
Other Techniques Not Proven

Vest Airway Clearance

Intrapulmonary Percussive

Ventilator (IPV)

frc relative to position
FRC Relative to Position

From Nunn’s Applied Respiratory Physiology, 2000

pulse oximetry
Pulse Oximetry
  • Acute decreased oximetry (< 95% while AWAKE)
    • suggests atelectasis or mucus plugging.
    • may be the first evidence of respiratory compromise.
  • < 95% while ASLEEP
    • suggests hypoventilation or mucus plugging.
secretion management
Secretion Management
  • May improve mucociliary clearance
    • Albuterol nebulized
  • May thin secretions
    • Acetylcysteine (Mucomyst™) nebulized
    • DNase (Pulmozyme™) nebulized
secretion management cont
Secretion Management (cont.)
  • Alter upper airway secretions
    • Medications
      • Glycopyrrolate (Robinul) by mouth/G-tube
      • Scopolamine patch
      • Steroid nasal spray
      • Antihistamines/Decongestants by mouth/G-tube
      • Botox injections of the salivary glands
    • Surgical Management
      • Salivary gland resection
respiratory support
Respiratory Support
  • Non-invasive ventilation
    • Bilevel positive airway pressure
    • Mechanical ventilation
  • Invasive ventilation
    • Tracheotomy
  • Palliative care

Wang C et al, J Child Neurol 2007; 22:1027.

non invasive ventilation niv
Non-Invasive Ventilation (NIV)
  • Short Term Goals
    • Rest the muscles for breathing
    • Improve exchange of oxygen for carbon dioxide in the body
    • Optimize patient comfort
    • Minimize risk
    • Avoid intubation

Mehta and Hill, AJRCCM 2001; 163:540

non invasive ventilation niv26
Non-Invasive Ventilation (NIV)
  • Long Term Goals:
    • Improve sleep
    • Maximize quality of life
    • Enhance functional status
    • Prolong survival

Mehta and Hill, AJRCCM 2001; 163:540

chronic respiratory failure bilevel positive pressure airway effects
Chronic Respiratory Failure:Bilevel Positive Pressure Airway Effects
  • Sustained reduction of daytime PaCO2
    • 3 Theories for NIV effect:
      • Rests chronically fatigued respiratory muscles
      • Reverses collapse of small air sacs in the lungs
      • Alters the CO2 “set point”

Mehta and Hill, AJRCCM 2001; 163:540

indications for niv
Indications for NIV
  • Standard:
    • Hypoventilation with oxygen saturation <90% and CO2 >45
    • Obstructive apnea
    • Post-operative care
indications for niv29
Indications for NIV
  • Specific to SMA
    • SMA type I
    • Oxygen saturation <94% while asleep and or elevated carbon dioxide level during sleep
    • Needing intubation or BiPAP during a cold
    • Pneumonia or atelectasis
    • Chest wall collapse/pectus excavatum
    • Poor sleep quality
chest wall development after niv
Chest Wall Development After NIV

6 mths

18 mths

Courtesy of A. Simonds, Royal Brompton Hospital, UK

niv tolerance
NIV Tolerance
  • 40 children with NMD, 9 months-16yrs
    • Hypercapnia (n=17), CMV weaning (n=3), nocturnal hypoventilation (n=18), frequent URI with sleep-disordered breathing (n=2)
  • Results: 38/40 tolerated mask ventilation
    • PaO2 increased significantly
    • PaCO2 decreased significantly

Simonds AK et al, Eur Respir J, 2000; 16:476.

non invasive positive pressure ventilation devices
Non-Invasive Positive Pressure Ventilation Devices
  • Bilevel positive airway pressure (BiPAP)

2. Home mechanical ventilator

bilevel positive airway pressure bipap devices
Bilevel Positive Airway Pressure (BiPAP) Devices
  • Recommend ST (spontaneous timed) devices
    • Provides backup respiratory rate
    • True respiratory muscle rest
    • Synchronizes with efforts
bilevel positive airway pressure bipap st devices
Bilevel Positive Airway Pressure (BiPAP) ST Devices
  • Respironics
    • BiPAP Synchrony – being phased out
    • BiPAP AVAPS – average volume assured pressure support
      • Guarantees tidal volume breaths within range of inspiratory pressure
      • Minimum tidal volume 200 ml
        • Smallest child approximately 20-25 kg.
    • BiPAP ST
    • Trilogy 100
bilevel positive airway pressure bipap st devices40
Bilevel Positive Airway Pressure (BiPAP) ST Devices
  • ResMed
    • VPAP III ST
      • Can set breath trigger and cycle sensitivity
bilevel positive airway pressure bipap st devices41
Bilevel Positive Airway Pressure (BiPAP) ST Devices
  • Example settings:
    • Inspiratory Positive Airway Pressure IPAP (PIP):
      • 14-20 cm of H20
    • Expiratory Positive Airway Pressure EPAP:
      • 3-6 cm of H20
bilevel positive airway pressure bipap
Bilevel Positive Airway Pressure (BiPAP)
  • Respiratory rate
    • Set to capture breathing effort and rest child
      • Example settings:
        • <1 year old, RR=30 (highest rate on BiPAP)
        • 1-3 years old, RR=25
        • >3 years old, RR=20-25
        • Teenagers to adult, RR=14-16 and recommend sleep study to titrate.
bilevel positive airway pressure bipap43
Bilevel Positive Airway Pressure (BiPAP)
  • Inspiratory time
    • Time over which the breath is delivered
      • Example:
        • Infants and young children 0.5 seconds
        • Toddlers to child: 0.8 seconds
        • School age: 1 second
        • Teen age: 1-1.5 seconds depending on comfort
bilevel positive airway pressure bipap44
Bilevel Positive Airway Pressure (BiPAP)
  • Rise time
    • Determines how fast the pressure increased to the peak inspiratory pressure
    • Lower numbers = faster rise time
home mechanical ventilator
Home Mechanical Ventilator

Examples: LTV (laptop ventilator, LP-6+,LP-10, Newport HT-50

Modes:

- Assist control

- SIMV (Synchronized intermittent mechanical ventilation)

  • pressure ventilation
  • volume ventilation
nippv disadvantages
NIPPV Disadvantages
  • Gastric distention and vomit especially if children are constipated
  • Nasal bridge discomfort and other skin irritation
  • Face changes
nippv challenges
NIPPV Challenges
  • Swallowing difficulty
  • Excessive secretions
  • Gastroesophageal Reflux
  • > 16 hrs/day of ventilation
invasive ventilation
Invasive Ventilation
  • Tracheostomy placement
    • Not an acute intervention
    • Controversial in non-sitters

Wang C et al, J Child Neurol 2007; 22:1027.

palliative care
Palliative Care
  • NIV can be used as palliative therapy.
  • Goals:
    • Prevent PICU stays and tracheotomy.
    • Provide symptom relieve
      • pain, dyspnea, agitation, nausea, anxiety
    • Provide psychological, social and spiritual support for child and family

Wang C et al, J Child Neurol 2007; 22:1027.

the last straw for nmd lung function
The Last Straw for NMD Lung Function
  • Viral respiratory infections
    • Result in:
      • Increased muscle weakness
      • Increased airway secretions
      • More difficulty breathing
  • The answer is not supplemental oxygen!
acute care respiratory management
Acute Care Respiratory Management
  • Goals:
    • Normalize oxygen and carbon dioxide exchange
      • Reduce lung collapse
      • Enhance airway secretion clearance

Wang C et al, J Child Neurol 2007; 22:1027.

airway clearance
Airway Clearance
  • Assisted Cough
    • manual cough assist
    • mechanical insufflation-exsufflation
  • Suctioning mouth or airway
  • Chest physiotherapy
  • Postural drainage

Wang C et al, J Child Neurol 2007; 22:1027.

university of wi cold care protocol
University of WI Cold Care Protocol
  • Perform every 4 hours:
    • Airway clearance
      • 15 minutes
    • Cough Assist
      • 4 sets of 5 breaths
    • Suction mouth or airway
    • Postural drainage
      • 15 minutes
    • Cough Assist
      • 4 sets of 5 breaths
    • Suctioning mouth or airway
airway clearance cont
Airway Clearance (cont.)
  • Use oximetry feedback to guide airway clearance
    • SpO2 less than 94%, use assisted coughing.
    • If no improvement:
      • continue respiratory airway clearance treatment
      • place on respiratory support.
  • Assisted cough techniques PREFERRED over deep suctioning and bronchoscopy

Wang C et al, J Child Neurol 2007; 22:1027.

acute respiratory failure bilevel positive airway pressure effects
Acute Respiratory Failure: Bilevel Positive Airway Pressure Effects
  • Decrease respiratory muscle work
  • Greater respiratory muscle rest
    • BiPAP >> CPAP
  • Improved exchange of oxygen and carbon dioxide

Mehta and Hill, AJRCCM 2001; 163:540

acute care respiratory management56
Acute Care Respiratory Management
  • Respiratory support
    • Non-invasive ventilation with aggressive airway clearance may decrease the need for intubation.

Wang C et al, J Child Neurol 2007; 22:1027.

acute care respiratory management57
Acute Care Respiratory Management
  • Use respiratory support during the day and at night as needed to relieve fatigue.
    • REMEMBER: EVERYONE IS WEAKER DURING A COLD
  • CPAP is not indicated.

Wang C et al, J Child Neurol 2007; 22:1027.

acute care respiratory management58
Acute Care Respiratory Management
  • Oxygen therapy
    • Used to correct hypoxemia when:
      • Airway clearance techniques and respiratory support maximized.

Wang C et al, J Child Neurol 2007; 22:1027.

acute care respiratory management59
Acute Care Respiratory Management
  • Intubation and mechanical ventilation may be needed
    • Temporary
  • Tracheostomy placement
    • Not an acute intervention
      • Surgically placed tube through the neck and into the trachea
      • May be necessary for SMAtype I

Wang C et al, J Child Neurol 2007; 22:1027.

acute care respiratory management60
Acute Care Respiratory Management
  • Maintain hydration
  • Avoid prolonged fasting (>4 to 6 hours)
    • Continue to feed or provide intravenous nutrition.
  • Use antibiotics

Wang C et al, J Child Neurol 2007; 22:1027.

acute care respiratory management61
Acute Care Respiratory Management
  • Albuterol and/or Atrovent therapy should be reserved for patients with asthma.
  • Inhaled mucolytics, e.g., Mucomyst and Pulmonzyme (Dnase), albuterol and Atrovent and corticosteroids are areas in need of future research.

Wang C et al, J Child Neurol 2007; 22:1027.

extubation
Extubation

Extubate when the child is:

1. afebrile

2. not requiring supplemental O2

3. CXR is without atelectasis or infiltrates

4. off respiratory depressants

5. airway suctioning is 1 time/hour or less

extubation cont
Extubation (cont.)
  • Extubate from reasonable settings:
    • a rate similar to the optimal BiPAP rate
    • pressures that approximate BiPAP inspiratory pressure (15-20) and expiratory pressure (3-6)
    • ideally room air
  • Avoid low ventilator rates through ET tube  atelectasis (lung collapse) and fatigue.
    • E.g., CPAP trials are not indicated prior to extubation.
recommendations
Recommendations
  • In-home respiratory equipment:
    • Cough Assist machine
    • Suction machine
    • Spot check pulse oximeter
    • Method for secretion mobilization, e.g., palm cups, electric percussor
    • Ambu bag
    • Nocturnal respiratory support, e.g., BiPAP or ventilator with portable power supply.
    • Supplemental oxygen for emergency use.
recommendations65
Recommendations
  • Routine immunizations
  • Annual influenza vaccine
  • RSV prophylaxis

Wang C et al, J Child Neurol 2007; 22:1027.

recommendations66
Recommendations
  • Get to know your Emergency Medical Services
    • Who comes when you call 911?
sma international registry
SMA International Registry
  • https://smaregistry.iu.edu/
conclusion
Conclusion
  • Referral for respiratory care evaluation should occur shortly after diagnosis
  • Chronic respiratory management includes providing methods for:
    • airway clearance with assisted coughing
    • Noninvasive ventilator support
  • Routine immunizations

Wang C et al, J Child Neurol 2007; 22:1027.

conclusion70
Conclusion
  • Discussion should occur early:
    • options for respiratory care
    • goals for chronic and acute respiratory care

Wang C et al, J Child Neurol 2007; 22:1027.

conclusion71
Conclusion
  • Acute respiratory illness management requires:
    • increased airway clearance and secretion management techniques
    • increased respiratory support
    • nutrition and hydration management
    • low threshold to start antibiotics

Wang C et al, J Child Neurol 2007; 22:1027.

breathing support cont
Breathing Support (cont.)
  • Options:
    • Negative pressure ventilation
    • Positive pressure ventilation
      • Non-invasive
      • Invasive
negative pressure ventilation
Negative Pressure Ventilation
  • A device worn over the outside of the chest wall and attached to a vacuum pump
    • Examples:
      • Porta-Lung (Iron lung)
      • Cuirass or Poncho
    • Relative Contraindications
      • Upper airway obstruction
positive pressure ventilation
Positive Pressure Ventilation
  • Non-invasive
    • Preset air volume or pressure delivered by
      • Nasal mask
      • Full face mask
      • Mouth piece
non invasive positive pressure ventilation
Non-Invasive Positive Pressure Ventilation

1. Bilevel positive airway pressure (BiPAP).

2. Home mechanical ventilator (LP-6+, LP-10, LTV, Achieva) with volume or pressure ventilation.

technical aspects
Technical Aspects
  • Bilevel positive airway pressure (BiPAP)

Examples: Respironics Synchrony, ResMed VPAP III, Knightstar

IPAP: 14-20 cm of H20 EPAP: 3-6 cm of H20

Mode: ST (spontaneous timed)

Respiratory Rate: high enough to capture breathing efforts and rest child.

Inspiratory Time: depends on pt age and RR

Rise time: speed of breath delivery

technical aspects cont
Technical Aspects (cont.)

2. Home mechanical ventilator Examples: LP-6+,LP-10, LTV 900 or 950 Modes: AC, SIMV PC or SIMV VC

  • pressure ventilation
  • volume ventilation
  • Tidal volume approximately 13-20 ml/kg per nasal mask
nippv disadvantages79
NIPPV Disadvantages
  • Stomach may fill with air and the child may vomit especially if constipated
  • Nasal bridge discomfort and other skin irritation
  • Changes of midface growth
nippv challenges80
NIPPV Challenges
  • Difficulty swallowing
  • Lots of oral secretions
  • Gastroesophageal Reflux
  • Requiring > 16 hrs/day of ventilation
invasive positive pressure ventilation
Invasive Positive Pressure Ventilation
  • Requires an artificial airway
    • Endotracheal tube
      • Short term
      • Treatment For “Reversible” Respiratory Failure
      • Extubate to BiPAP
  • May be needed during a cold or illness.
invasive positive pressure ventilation82
Invasive Positive Pressure Ventilation
  • Tracheotomy
    • Surgically placed tube through the neck and into the trachea
    • May be necessary for SMA type 1
  • Mechanical ventilation
invasive positive pressure ventilation83
Invasive Positive Pressure Ventilation
  • Tracheotomy
    • Reasons
      • Child is not able to tolerate non-invasive ventilation
      • Child requires nose mask ventilation for 24 hours per day when healthy
      • Difficult to manage “death spells”
recommendations84
Recommendations
  • In-home respiratory equipment:
    • In-Exsufflator cough machine or Cough Assist
    • Suction machine
    • Spot check pulse oximeter
    • Nocturnal respiratory support
    • A consistent method for secretion mobilization, e.g., palm cups, electric percussor, The Vest, IPV
    • Postural drainage method.
care during a cold
Care During a Cold
  • Every 4 hours
    • Airway clearance for 10-20 minutes
    • In-exsufflator cough machine/Cough Assist
    • Postural drainage for 15-30 minutes
    • In-exsufflator cough machine/Cough Assist
care during a cold cont
Care During a Cold (cont.)
  • Use the In-exsufflator cough machine/Cough Assist as often as needed to clear rattley breathing and help with coughing
care during a cold cont87
Care During a Cold (cont.)
  • Use breathing support during the day and at night.
    • REMEMBER YOUR CHILD IS EVEN WEAKER DURING A COLD
care during a cold cont88
Care During a Cold (cont.)
  • Use the pulse oximeter to help guide you in room air.
  • When the oxygen saturation is less than 94%, use the cough machine.
  • If you cannot keep the oxygen saturation >90%, the child should be seen by a physician.
care during a cold cont89
Care During a Cold (cont.)
  • Keep your child hydrated
  • Try to continue to feed if possible.
contributing factors to respiratory muscle weakness
Contributing Factorsto Respiratory Muscle Weakness
  • Scoliosis
    • Alters chest wall shape and efficiency
  • Nutritional status
    • Suboptimal nutrition increases muscle weakness
    • Excess weight contributes to more difficulty moving
interventions for contributing factors to respiratory muscle weakness
Interventions for Contributing Factorsto Respiratory Muscle Weakness
  • Scoliosis
    • TLSO or Body Jacket
    • Spinal fusion
  • Nutritional status
    • Suboptimal nutrition
      • consider gastrostomy tube.
    • Excess weight
      • consider diet limits
choices
CHOICES

Choices

Choices

Choices

Choices

Choices

families of children with sma
Families of Children with SMA
  • Select community of people who share a common thread . . .

You all care and love someone with SMA.

choices94
CHOICES

Children with SMA type I are very weak in the first year of life and their prognosis is not clear.

recurring themes
RECURRING THEMES

All families struggle with:

  • the diagnosis of SMA
  • how to best care for their child
  • whether their child experiences pain as a result of their choices
choices96
CHOICES
  • Some children require a breathing tube when they become very ill.
choices97
CHOICES
  • Some children require a breathing tube when they become very ill.
    • Questions that families and medical care providers think about:
      • Is the condition reversible?
      • Will they come off the respirator/ventilator?
when the options are limited
When the Options are Limited
  • Families often have the difficult position of choosing the option they believe is best for their child and their family.
  • Because there are no right or wrong choices.

TRUST that you will make the right decision.

slide99

Palliative Care

  • Recognizes that this is a life threatening disease
  • Should be part of “Treatment” of disease process
  • Goals
    • Provide symptom relieve
      • pain, dyspnea, agitation, nausea, anxiety
    • Provide psychological, social and spiritual support for patient and family
  • Palliative care can be in the hospital, in the home or both
summary
Summary
  • Children with SMA type 1 are at great risk for poor cough and hypoventilation.
  • Many medical interventions are available to support breathing.
  • Not all interventions fit every child.