Respiratory Physiotherapy in Neuro-Muscular Weakness

Kath Sharp Paediatric Respiratory Physiotherapist

Evidence Based Practice

Evidence base

EBM is the conscientious, explicit and judicious use of current best evidence in making decisions about the care of the individual patient. It means integrating individual clinical expertise with the best available external clinical evidence from systematic research.

Why develop a guideline?

• • • • • Prevalence ~1/3000 Majority of conditions genetic and manifest in childhood Likelihood of respiratory impairment varies greatly among different conditions Most likely to occur in those who are globally weak Most frequent reason for unplanned admissions

Aim of Guideline

• • Summarise the available evidence Provide recommendations which can be applied to all children with neuromuscular weakness

Appraising the literature

Guideline Covers…

• • • • • • • • • Identifying children at risk of respiratory complications Airway Clearance and respiratory muscle training Assisted ventilation Planning for surgical procedures Scoliosis Other problems and interventions that impact on respiratory health Transition to adult care Quality of life and palliative care Special considerations

Respiratory Complications

• • • • Compromise to gas exchange and pump function of the respiratory system Bulbar function Efficiency of muco-ciliary clearance Spinal support

Consequences

• • • • • Hypoventilation Upper airway obstruction Aspiration lung disease Secretion retention and recurrent infection Mechanical effects of progressive scoliosis

Identifying a child at risk of respiratory Complications

Clinical assessment of respiratory health should be part of every medical consultation

• • • • • • • • Extent, pattern, progression of weakness Degree of ambulation Degree of muscle fatigability Change in strength of voice or cough Problems with drooling Difficulty clearing secretions Choking with food Difficulty with chewing

Clinical assessment of respiratory health should be part of every medical consultation

• • • • • • Posture, seating Development of kyphosis / scoliosis Growth Nutritional status Frequency and severity of respiratory infections Sleep disordered breathing

Evidence level 3 Grade of Recommendation D

Vital Capacity should be measured in all patients with neuromuscular disease who are capable of performing spirometry as part of the respiratory assessment.

• • • Vital capacity can be reliably measured in almost all weak subjects by using a slow manoeuvre Has been shown to be a valuable predictor of susceptibility to infection and need for respiratory support Vital capacity correlates with survival in children and adolescents with DMD

Evidence level 2 + Grade of recommendation C

Methods

• • • Retrospective chart review 46 patients referred for respiratory assessment Mean age 12.7 ±3.6 years range 6-20

Diagnosis

DMD SMA CMD HSMN Pompes Nemaline Myopathy Congenital myopathy 1 1 3 1

Number

16 14 10

Information collected

• • • History of chest infection: How often had the child had chest infections in the year prior to review?

How many days of antibiotics had they had to treat chest infections?

If they child had had any hospital admissions for chest infections since age 2?

• • • • Lung Function: Inspiratory Vital Capacity FEV 1 / FVC PIP /PEP PCF

Results

• Average rate of chest infection was 0.84 ± 0.94

• Average number of days of ABs 5.5 ± 6.0

• 22/46 had had at least 1 admission to hospital with pneumonia

Cough Peak Flow should be used as part of the assessment of effective secretion clearance in children with neuromuscular disease over the age of 12

• • No studies looking cough peak flow thresholds in children There are standard values for normal children age 4-18 Bianchi (2008) Am J Phy Med Rehabil: 87: 461-7

Children with ineffective cough (including children over 12 with CPF <270l/min) particularly if they have episode of deterioration with respiratory infection, should be taught augmented cough techniques

• • 2 parts: Increase inspired volume Increase the expiratory forces applied to the inspired volume

Level of evidence 3 Grade of recommendation C

Manual cough assist and air-stacking methods to achieve MIC are effective methods of improving cough efficiency and should be used where appropriate

• • • MIC = Maximum Inspiratory Capacity Maximum volume of air “stacked” in the lungs beyond spontaneous vital capacity Relies on intact bulbar function Breath stacking with bag

•

Manual cough assist and air-stacking methods to achieve MIC are effective methods of improving cough efficiency and should be used where appropriate

Lung Insufflation Capacity volume achieved if patient cannot breath stack • Expiratory airflow will increase with manual compression to the chest wall or abdomen

Level of evidence 3 Grade of recommendation C

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MI-E should be considered in very weak children, those with loss of bulbar function, and those who cannot cooperate with manual cough assist or air-stacking or in whom these methods are not effective

No RCT showing MI-E to be more effective than other forms of physiotherapy in preventing respiratory exacerbation • Does increase PCF

Level of evidence 3 Grade of recommendation C

Design and Aim

• • • • Prospective clinical trial 17 patients (DMD 4, SMA 4, Congenital Myopathy 9) Age range 5-18 years Clinically stable

Analyse the physiologic effects and tolerance of mechanical Insufflation – Exsufflation for children with NMD

Methods

• • Intervention – MI-E 3 sessions all in one day 6 sets Insufflation – Exsuffalation • • • +15-15cmH 2 O +30-30cmH 2 O +40-40cmH 2 O Measured VC and CPF after each intervention All interventions happened on same day None of the patients had used MI-E before

Date of download: 4/29/2013 Copyright © American College of Chest Physicians. All rights reserved.

Physiologic Benefits of Mechanical Insufflation-Exsufflation in Children With Neuromuscular Diseases * Chest. 2008;133(1):161-168. doi:10.1378/chest.07-1615 Figure Legend: Individual data of the SNIP and the best PEF or PCF of the patients at baseline and after the MI-E applications at the three pressure levels (15, 30, and 40 cm H 2 O). The patients with Duchenne muscular dystrophy, spinal muscular atrophy and other neuromuscular diseases are represented by the black squares, the open squares and the black dots, respectively. See the legend of Figure 2 for abbreviations not used in the text.

Conclusion

• • CPF increased significantly after +40-40cmH 2 O Well tolerated in children

Oscillatory techniques such as high-frequency chest wall oscillation and IPV should be considered in children who have difficulty mobilising secretions or who have persistent atelectasis, despite the use of other airway clearance techniques

• IPV = Interpulmonary Percussive Ventilation Modified IPPB superimposes high-frequency mini bursts of air on the individuals breathing pattern • Creates an internal vibration

Oscillatory techniques such as high-frequency chest wall oscillation and IPV should be considered in children who have difficulty mobilising secretions or who have persistent atelectasis, despite the use of other airway clearance techniques

High Frequency Chest wall Oscillation = VEST • Inflatable jacket over thorax

Level of evidence 3 Grade of recommendation D

Airway clearance techniques should be used during respiratory infection when oxygen saturations levels fall below 95% while the child is breathing room air. If the techniques being used fail to result in an increase in oxygen saturation to 95% or above, different methods of airway clearance should be used

• No RCTs looking at airway clearance during acute respiratory exacerbation

Level of evidence 3 Grade of recommendation D

• • • • •

MI-E should be available in the acute setting in all hospitals that treat neuromuscular patients as an alternative method of airway clearance with the purpose of preventing deterioration and the need for intubation and mechanical ventilation

No RCTs looking at benefit of MI-E Does improve CPF Is well tolerated in children Does reduce treatment time Can prevent need for escalation of care

Level of evidence 3 Grade of recommendation D

Design / Aim

• Randomised 2 day cross over trial • • • • Group 1 – Day 1 morning treatment without MI-E afternoon with Day 2 morning treatment with MI-E afternoon without 8 patients (DMD =4, SMA =3, Congential myopathy=1) Age range 4-44 years

• All NMD patients admitted to ward with evidence of infection approached • Sputum retention, increased CRP, Increased WCC, increased temperature

Hypothesis: Airway clearance more effective with MI-E than without

Results

What should we take from these guidelines?

• Assessment is fundamental to planning treatment interventions • Use guidelines to help direct treatment plans • Each patients treatment plan should be patient focused