Role and management of tracheostomy in the care of ventilator-dependent patients with slowly progressive neuromuscular diseases

Context

The Association française contre les myopathies-Téléthon (AFM (French myopathy association)-Téléthon), asked the HAS to conduct a study relative to the role of tracheostomy in the management of ventilator dependence in neuromuscular diseases.

The Société de pneumologie de langue française (SPLF - French-language Pulmonology Society), the Société de réanimation de langue française (SRLF - French-language intensive care society), the Filière nationale de santé des maladies neuromusculaires (FILNEMUS) and the Association nationale pour les traitements à domicile, les innovations et la recherche (ANTADIR) support the request for inclusion in the HAS work programme.

The request was included in the HAS work programme.

The term “neuromuscular diseases” designates diseases that affect the muscles or their innervation system (motor unit disorder), which can appear at any age of life, be temporary or permanent, and progress at variable rates.

There are more than 200 different neuromuscular diseases (including muscular dystrophies, peripheral neuropathies, dysimmune neuropathies, spinal amyotrophies, myasthenia, myositis, amyotrophic lateral sclerosis), with various causes and consequences. However, all neuromuscular diseases combined, tens of thousands of individuals are affected in France.

In some diseases, the respiratory muscles are affected. Respiratory capacity and cough are reduced, leading to bronchial congestion and a risk of lung infection. Swallowing difficulties related to upper airway muscle dysfunction represent an additional risk factor for respiratory obstruction and lung infection.

Evolving knowledge about neuromuscular diseases and the management of patients with these diseases has led to real improvements in terms of both life expectancy and quality of life for these patients.

Objectives

The recommendations aimed at the professionals concerned are designed to support decision-making in terms the choice of care and to improve and harmonise practices. The ultimate objective is to improve the management of patients, and therefore the quality and safety of their care, as well as their quality of life.

Scope

Patients or users concerned by the subject

Any individual with respiratory failure due to a slowly progressive neuromuscular disease.

All age groups are concerned, with the exception of neonates (< 28 days).

Patients with spinal cord injuries and rapidly progressing neurodegenerative conditions, such as amyotrophic lateral sclerosis, are excluded from the scope of this best practice guideline.

Professionals concerned by the subject

The professionals concerned are those involved in the care of patients with neuromuscular diseases: paediatricians (paediatric neurologists, paediatric respiratory medicine specialists), respiratory medicine specialists, breathing and swallowing therapists, sleep specialists, anaesthesia and resuscitation specialists, intensive care and resuscitation specialists, surgeons, ear, nose and throat (ENT) specialists, radiologists, physical medicine and rehabilitation specialists, neurologists, general practitioners, nurses, speech therapists, physiotherapists, mechanical ventilation technicians.

Key messages

The following recommendations were considered as being those that should be implemented as a priority to improve the quality of care delivered to patients at the time of publication of the best practice guideline.

• It is recommended that tracheostomy only be envisaged following the failure of non-invasive ventilation (NIV) and cough assistance techniques. It requires the written informed consent of the patient, their person of trust or their legal guardian, following a multidisciplinary team meeting to consider the issue.
• Except in emergency situations, it is recommended that tracheostomy be exclusively surgical and performed by a practitioner with expertise in the procedure, either a surgeon or a resuscitation specialist.
• The post-surgical follow-up of tracheostomy should be carried out in an intensive care unit until the first tube change.
• Any early post-surgical bleeding requires immediate assessment by a competent professional and as soon as possible by the practitioner having performed the procedure.
• After the first tracheotomy tube change, transfer of the patient to a high-dependency unit or equivalent structure may be envisaged and rehabilitative management for swallowing and speech is necessary.
• In situations of bronchial congestion, bronchial drainage techniques are recommended in addition to tracheal suctioning. These are systematically tested and optimised in hospital first.
• Patient education and caregiver training in everyday procedures and measures to be taken in the event of certain emergency situations must be delivered before the patient is discharged from hospital.
• Tracheotomy tubes should be changed at least every 28 days.