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GP Respiratory Management for Adults with Neuromuscular Conditions

About respiratory management in neuromuscular patients

People diagnosed with progressive neuromuscular (NM) conditions, of which Motor Neurone Disease is the most common, have a high risk of developing respiratory symptoms and comorbidities.

It is difficult to predict when respiratory muscle involvement, swallowing difficulties or sleep disordered breathing will occur or the rate of progression.

People with progressive NM conditions should be screened regularly for early signs and symptoms of respiratory compromise. Upon diagnosis of a neuromuscular condition, all people should be referred to their nearest neuromuscular or MND clinic, or respiratory specialist (see referrals section).

People with progressive NM conditions who have any signs of respiratory compromise should be encouraged to discuss their plans and goals for end of life including respiratory care options and be supported in developing an Advanced Care Plan. These discussions can occur with the GP or by referral to a palliative care specialist. Early consultation with palliative care may also assist with respiratory symptom management.

Care for patients with neuromuscular conditions is best achieved through a multidisciplinary approach. The multidisciplinary team may include neurology, respiratory, speech pathology, dietetics, physiotherapy, palliative care, and the GP as the team coordinator.

Alerts

  • Low saturation ≤92%, unless previously known lung disease
  • Increased respiratory rate >30 breaths/minute
  • Persistent/recurring morning headaches
  • Aspiration
  • Acute or worsening orthopnoea
  • Rapid weight loss (approx. 10% or more over 3 months)
  • Serum bicarbonate levels >27mmol/L

Assessment

All patients with a diagnosis of a neuromuscular condition should be assessed for respiratory compromise at every visit.

  1. Assess for signs and symptoms of respiratory compromise.

    Signs and Symptoms of Respiratory Compromise

    • breathlessness (dyspnoea) at rest or when lying flat (orthopnoea)
    • using more pillows at night to help with breathing
    • excessive use of the muscles in the upper chest and neck
    • weakened cough, sneeze or voice
    • shallow, faster breathing
    • frequent respiratory tract infections
    • swallowing difficulty
    • coughing after eating or drinking
    • decreased appetite
    • increased fatigue
    • impaired concentration or confusion
    • disturbed sleep
    • excessive daytime sleepiness
    • morning headaches
    • excessive saliva or drooling
  2. Ensure all vaccinations are up to date
  3. Investigate respiratory infections

    Respiratory Infections

    • If the patient has recurrent respiratory infections, investigate the source of the pathogen.
    • Arrange for chest x-ray if chest infection or pneumonia is suspected.
    • Consider aspiration as a cause, and refer to speech pathology for further assessment.
  4. Obtain baseline respiratory investigations
  5. Respiratory Investigations

    • Spirometry
    • Compare with previous spirometry (if available).
    • Measure Forced Vital Capacity (FVC) with spirometry in erect and supine positions. A change >20% when supine suggests diaphragm weakness and the need for further investigation
    • Patient should be referred to a respiratory specialist if their FVC < 50% predicted
    • Serum bicarbonate
    • A venous bicarbonate >27mmol/L can indicate hypoventilation and should trigger further respiratory investigation
    • If >27mmol/L, consider arterial or capillary blood gases, further respiratory testing or referral to a respiratory/sleep specialist or centre urgently, especially if saturation SpO2≤92%
  6. Ongoing Review
  7. At every visit:

    • Organise spirometry and cough peak flow measures and compare to baseline
    • Assess signs and symptoms of respiratory compromise
    • Examine respiratory function

      Respiratory Examination

      • Central or peripheral cyanosis
      • Respiratory rate
      • Use of accessory muscles
      • Abdominal paradox
      • Auscultate chest for crepitations or wheeze
      • Perceived breathlessness at rest or on activity
      • Pulse oximetry*
        • SpO2 ≤92% at rest in the absence of coexisting lung disease may indicate likely respiratory failure. These patients should be assessed urgently by their either their specialist respiratory physician, neuromuscular clinic or at the emergency department of their local hospital. Patients will require arterial or capillary blood gases for exclusion of hypercapnic respiratory failure.
    • Record patient weight

      Weight Assessment

      • Change of weight can impact on respiratory function. Increasing weight adds to the burden on the respiratory system. Unintended weight loss can reflect problems with swallowing or breathlessness, and may contribute to further respiratory muscle impairment
      • Review dietary intake and any problems with eating and drinking
      • Referral to a dietician and speech pathology
      • Consider need for alternative long term nutrition planning in consultation with MDT
    • Assess for constipation

      Constipation Assessment

      • Constipation can exacerbate respiratory issues.
      • Increase fluid intake.
      • Consider increasing fibre intake or use of laxatives

Management

Points to Consider!

  • Low oxygen levels indicate respiratory   insufficiency. However, excessive amounts of supplemental oxygen can promote   carbon dioxide retention, creating negative impacts on patient outcome. If   oxygen is required, aim for a target saturation of 88-92%.
  • Pulse oximetry   readings can vary in their accuracy due to features such as temperature, drug   responses, incorrect sensor application and the condition of the skin.
  1. Management should occur in line with the patients current advance care plan
  2. Advance care plan

    • Where possible, the advance care plan should be discussed and determined prior to deterioration, and regularly reviewed.
    • Reviewing the advance care plan and the patients informed preferences will assist in determining the most appropriate management options.
  3. If there have been any changes in respiratory symptoms, complete respiratory investigations.
  4. Manage any common respiratory issues:
    • Risk of aspiration

      Risk of Aspiration

      Possible cause:

      • Disease progression
      • Pharyngeal and laryngeal dysfunction
      • Bulbar weakness

      Action:

      • Speech pathologist referral – urgent if patient has a current chest infection suspected to be caused by aspiration
      • Careful selection of food and drink until speech pathology assessment
      • Assess cough strength
      • Suggest safe swallowing strategies such as sitting upright during meals, eating and drinking slowly
      • Educate and encourage around good oral hygiene and regular mouth care
      • Consider thickened drinks
        • Thickened fluids can make some patients’ swallow less safe and more difficult. Thickened fluids can increase dehydration risk, affect renal function and medication absorption and reduce quality of life.
      • Consider need for long term feeding plan in consultation with MDT
    • Difficulty clearing secretions
    • Difficulty Clearing Secretions

      Possible cause:

      • Impaired cough effectiveness with increased risk of respiratory failure and death, even if using mechanical ventilation.
      • Secretions have become thick and sticky due to dehydration

      Action:

      • Referral to respiratory outpatient clinic for routine measurement of peak cough flow. A peak cough flow rate <270L/min places the patient at risk of ineffective cough, especially during a chest infection.
      • Increase fluid intake.
      • Consider regular normal saline nebuliser to assist with thinning and clearing secretions
    • Increased breathlessness

      Increased Breathlessness

      Possible cause:

      • Disease progression
      • Presence of respiratory infection

      Action:

      • Identify and treat any respiratory infections, and consider aspiration as a possible cause
      • Increase frequency of respiratory reviews
      • Educate patient about potential for ongoing respiratory deterioration.
      • Refer back to specialist clinic.
      • Consult with palliative care for symptom management.
      • Consider medications.
      • Review response to medications and adjust dose as required.
      • Hospitalisation
    • Hypoxia or hypercapnia

      Hypoxia or hypercapnia

      Possible cause:

      • Respiratory failure

      Action:

      • Arterial blood gas
      • Overnight oximetry- specifically if patients are reporting waking with headaches in the morning, or an increase in daytime sleepiness
      • If hypoxic, judicious use of oxygen therapy to maintain sats of:
        • 88-92% if hypercapnic
        • 92-96% if hypoxic only
      • Refer back to specialist clinic.
    • Excessive saliva

      Excessive Saliva

      Action:

      • Consider medications.
      • Review response to medications and adjust dose as required.
      • Need for suction equipment at home
      • Arterial blood gas
  5. Provide early antibiotics for signs of chest infection (presence of symptoms or raised white cell count) to reduce the risk of bacterial super-infection
  6. Provide information for the patient and discuss self-monitoring, self-management and end-of-life matters

    Discuss Self-Monitoring, Self-Management and End-of-Life Matters

    Discuss;

    • the signs and symptoms of worsening respiratory failure (orthopnoea, morning headaches, and worsening daytime sleepiness) and symptom management advice
    • palliative care involvement for additional respiratory symptom management and end of life care planning
    • advance care directives
  7. Ongoing Review.

Referral

  • Refer patients with signs of acute respiratory compromise to the Emergency Department unless contraindicated in the patient’s advance care plan.

    Signs of Acute Respiratory Compromise

    • Low saturation ≤92%, unless previously known for this
    • Increased respiratory rate >30 breaths/minute
    • Persistent/recurring morning headaches
    • Serum bicarbonate levels >27mmol/L
    • Aspiration
    • Acute or worsening orthopnoea
    • Rapid weight loss due to inability to eat
  • Consider referral for specialist respiratory assessment for:
    • Assessment of respiratory muscle weakness
    • Nocturnal hypoventilation
    • Risk of aspiration or swallowing difficulties (as well as urgent referral to a speech pathologist)
    • Determination of the need for, and specific type of ventilatory support.

      Ventilatory Supports

      • Continuous Positive Airway Pressure (CPAP)
      • Bi-level Positive Airway Pressure (BiPAP)
      • Oxygen
      • Cough assist techniques
  • Consider tele-monitoring if available in your area

Information

Clinical Resources

Patient Information

Download

Download GP Summary of this page (82Kb pdf)

References

  1. MND Australia. (2012). Factsheet - Living Better for Longer: MND Australia Fact Sheet EB5 Breathing and motor neurone disease: an introduction. https://www.mndnsw.asn.au/about-mnd/information/153-managing-with-mnd/living-better/231-breathing-and-motor-neurone-disease-an-introduction-eb5.html
  2. Agency for Clinical Innovation. (2012). Domiciliary Non-Invasive Ventilation in Adult Patients (A Consensus Statement). https://www.aci.health.nsw.gov.au/__data/assets/pdf_file/0008/159794/ACI-NIV-guidelines.pdf
  3. MND Australia. (2012). Factsheet - Living Better for Longer: MND Australia Fact Sheet EB7 Breathing and motor neurone disease: medications and non-invasive ventilation. https://www.mndnsw.asn.au/about-mnd/information/153-managing-with-mnd/living-better/233-breathing-and-motor-neurone-disease-medications-and-non-invasive-ventilation-eb7.html

These consensus guidelines were developed in consultation with respiratory and physiotherapy specialists, general practitioners, consumers, speech pathologists, neurologists, and the ACI Respiratory Network. ACI thanks all those who contributed to its development, especially A/Professor Amanda Piper, Dr Hima Vedam and Jane Burrage for their time and expertise.

Document to be reviewed June 2019

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