This information is for health and social care professionals. You may also want to see our information for people living with or affected by MND on:
Motor neurone disease (MND) is a progressive and terminal disease that attacks the motor neurones, or nerves, in the brain and spinal cord. Respiratory muscle weakness occurs eventually in everyone with MND.
Difficulty in coughing can be due to weakness in inspiratory, expiratory and bulbar muscles. A respiratory physiotherapist can prescribe the most appropriate cough management regime for the person with MND and teach techniques to help them to cough more effectively.
Cough augmentation techniques may be offered to people with MND who cannot cough effectively. These techniques can enable expectoration of phlegm or mucus from the throat or lungs, thus reducing the likelihood of A&E admission and emergency intubation.
The NICE guideline on MND recommends:
- unassisted breath stacking and/or manual assisted cough as first-line treatment
- assisted breath stacking (eg using a lung volume recruitment bag with a one-way valve) for those with bulbar dysfunction or whose cough is ineffective with unassisted breath stacking
- if available, use of a mechanical insufflation-exsufflation machine (sometimes known by the brand name CoughAssist), if assisted breath stacking is not effective and/or during a respiratory tract infection.
MI:E is often referred to as CoughAssist. In the UK, there are two main MI:E devices used: the NIPPY Clearway (made by B&D Electromedical) and the CoughAssist E70 (made by Philips Respironics).
A mechanical insufflation-exsufflation (MI:E) device supports and enhances the ability of weakened inspiratory and expiratory muscles in order to improve a person’s cough and aid secretion clearance. Use of MI:E has been shown to reduce the incidence of chest infections, and furthermore reduce the likelihood of emergency hospital admissions. However, the benefits may be limited for someone who has severe bulbar impairment.
MI:E may be started once a person has experienced repeated respiratory infections. Once MI:E has been set up, people are advised to use the device on a daily basis to get maximum benefit and to prevent the onset of further respiratory infections.
MI:E is used with either a tight-fitting face mask, a mouthpiece, tracheostomy or endotracheal tube. Additionally, MI:E can be used in different modes, which helps to improve synchronisation between the patient and the machine, improves patient comfort and therefore treatment effectiveness.
A respiratory specialist will be able to prescribe the exact treatment regime needed. Training should also be provided to the person with MND and any family/carers who may operate the device. The provision or loan of MI:E machines varies from region to region and sometimes servicing and maintenance can be problematic. If problems are experienced, contact the local MND Association regional care development adviser (RCDA) who may be able to help.
The specialist respiratory team can advise on strategies to avoid infections. Repeated aspiration and poor lung function can leave a person prone to chest infections and pneumonia. It is important to have contact with a speech and language therapist who can assess and advise about swallowing ability. People with MND should be offered prophylactic pneumococcal and influenza vaccinations.
There may be a place for early use of antibiotics, if this is what the person with MND wants.
For information about managing saliva problems in MND, see:
An oral suction unit may be used to help with saliva and secretion management.