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Duchenne Muscular Dystrophy: Pulmonary Management

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Title: Duchenne Muscular Dystrophy: Pulmonary Management


1
Duchenne Muscular DystrophyPulmonary Management
2
Introduction
  • Ambulant boys normally have few respiratory
    difficulties
  • Progressive loss of muscle strength leads to risk
    of respiratory complications over time
  • Ineffective cough
  • Nocturnal hypoventilation
  • Sleep disordered breathing
  • Daytime respiratory failure
  • Staged progression planned/proactive approach to
    respiratory care, aiming to prevent/manage these
    complications
  • Team to include a doctor and therapist skill in
    initiation/management of
  • Non-invasive ventilation and associated
    interfaces
  • Lung-volume recruitment techniques
  • Manual and mechanically assisted cough
  • Specific guidelines for respiratory care in DMD
    have also been published

3
Surveillance Ambulatory
  • Minimal assessment to include pulmonary function
  • e.g. sitting Forced Vital Capacity (FVC) at least
    annually
  • Enables familiarity of patient with equipment
  • Allows care team to assess maximum respiratory
    function achieved

4
Surveillance Non-ambulatory (clinic
measurements)
  • Main need for pulmonary care is after loss of
    independent walking
  • Figure 2, TLN p181
  • Clinic measurements at least every 6 months
  • Sitting FVC
  • Peak cough flow
  • Oxyhaemoglobin saturation by pulse oximetry
  • Maximum inspiratory and expiratory pressures
  • Awake end-tidal CO2 level should be measured by
    capnography, if patient non-ambulatory and has
    any of
  • Suspected hypoventilation
  • FVC lt50 prediceted
  • Current use of assisted ventilation

5
Surveillance Non-ambulatory (home measurements)
  • See Figure 3, TLN p181

6
Family Awareness
  • Family should be aware of the symptoms of
    hypoventilation or a weak cough, which should be
    reported to medical caregivers
  • Prolonged, apparently minor upper respiratory
    infections (e.g. recovery from common colds is
    slow, with colds progressing to chest congestion
    and bronchitis often requiring antibiotic
    therapy)
  • More tiredness than is usual
  • Shortness of breath, difficulty catching breath
    or finishing sentences
  • Headaches all the time or in the morning
  • Sleepiness for no reason
  • Trouble sleeping, frequent waking from sleep,
    nightmares
  • Wakes trying to catch breath, or can feel heart
    pounding
  • Trouble paying attention
  • Family should keep copies of the latest breathing
    test results to show to attending doctors

7
Prevention of Problems
  • Immunisation with 23-valent pneumoccocal
    polysaccharide vaccine for patients 2 years
  • Annual immunisation with trivalent inactivated
    influenza vaccine for patients 6 months
  • Both can be given to patients on steroids, though
    immune response to vaccination may be diminished
  • Detailed information on immunisation indications,
    contraindications, and schedules can be obtained
    from national sources
  • It is essential to keep up to date with
    vaccination policies as they can change regularly
    according to new threats
  • If chest infection occurs, then in addition to
    manually and mechanically assisted cough,
    antibiotics should be prescribed early

8
Interventions
  • Specific interventions are dependent on the
    disease phase
  • Staged progression
  • Volume recruitment/deep lung inflation techniques
  • Manual/mechanically assisted cough techniques
  • Nocturnal ventilation
  • Daytime ventilation
  • Tracheostomy

9
Step 1 Volume Recruitment Deep Lung Inflation
Techniques
  • By self-inflating manual ventilation bag, or
    mechanical insufflation/exsufflation
  • When FVC lt40 predicted

10
Step 2 Manual and Mechanically Assisted Cough
Techniques
  • Necessary when
  • Respiratory infection present and baseline peak
    cough flow lt 270 L/minute
  • Baseline peak cough flow lt 160 L/min or max
    expiratory pressure lt 40cm water
  • Baseline FVC lt 40 predicted or lt 1.25 L in older
    teenagers/adults

11
Step 3 Nocturnal Ventilation
  • Indicated in patients who have any of
  • Signs/symptoms of hypoventilation (patients with
    FVC lt 30 predicted are at especially high risk)
  • Baseline SpO2 lt95 and/or blood or end-tidal Co2
    gt45 mm Hg while awake
  • An apnoea-hypopnoea index gt10 per hour on
    polysomnography or four or more episodes of SpO2
    lt92 or drops in SpO2 of at least 4 per hour of
    sleep
  • Optimally, use of lung volume recruitment
    assisted cough techniques should always precede
    initiation of non-invasive ventilation

12
Step 4 Daytime Ventilation
  • In patients already using nocturnally assisted
    ventilation daytime ventilation is indicated for
  • Self-extension of nocturnal ventilation into
    waking hours
  • Abnormal deglutition due to dyspnoea, which is
    relieved by ventilator assistance
  • Inability to speak a full sentence without
    breathlessness and/or
  • Symptoms of hypoventilation with baseline SpO2
    lt95 and/or blood or end-tidal Co2 gt45mm Hg while
    awake
  • Continuous non-invasive assisted ventilation
    (with mechanically assisted cough) can facilitate
    endotracheal extubation for patients who were
    intubated during acute illness or anaesthesia,
    followed by weaning to nocturnal non-invasive
    assisted ventilation if applicable

13
Step 5 Tracheostomy
  • Indications for tracheostomy include
  • Patient and clinician preference
  • Patient cannot successfully use non-invasive
    ventilation
  • Inability of local medical infrastructure to
    support non-invasive ventilation
  • 3 failures to achieve extubation during critical
    illness despite optimum use of non-invasive
    ventilation and mechanically assisted cough
  • The failure of non-invasive methods of cough
    assistance to prevent aspiration of secretions
    into the lung and drops in oxygen saturation
    below 95 or the patients baseline,
    necessitating frequent direct tracheal suctioning
    via tracheostomy

14
Surgery
  • Lung function should be checked before surgery
  • DMD patients should never be given inhaled
    anaesthesia or succinylcholine

15
CAUTION Supplemental Oxygen
  • In later stages of DMD, supplemental oxygen
    therapy should be used with caution
  • Can apparently improve hypoxaemia while masking
    underlying cause (e.g. atelactasis or
    hypoventilation)
  • Might impair central respiratory drive,
    exacerbating hypercapnia
  • If patient has hypoxaemia due to hypoventilation,
    retained respiratory secretions and/or
    actelectasis, then manual and mechanically
    assisted cough and non-invasive ventilatory
    support are necessary
  • Substitution of these methods by oxygen therapy
    is dangerous

16
References Resources
  • The Diagnosis and Management of Duchenne Muscular
    Dystrophy, Bushby K et al, Lancet Neurology 2010
    9 (1) 77-93 Lancet Neurology 2010 9 (2) 177-189
  • Particularly references, p186-188
  • The Diagnosis and Management of Duchenne Muscular
    Dystrophy A Guide for Families
  • TREAT-NMD website www.treat-nmd.eu
  • CARE-NMD website www.care-nmd.eu
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