Motor Neurone Disease – Saliva Control Guidance, Acute & Primary Care (719)

This guideline outlines the general management of patients with motor neurone disease (MND) who require pharmacological management of hypersalivation. General Practitioners may be asked to prescribe from this guideline on the advice of an MND specialist. Hypersalivation is often referred to as sialorrhoea or drooling. It affects up to 50% of people with MND and in 42% of these individuals the symptom is poorly controlled. In most cases, saliva problems are the result of poor lip seal and/or impaired ability to swallow, rather than increased saliva production. Patients may complain of excessive, watery saliva or thick, mucousy saliva and it is important to distinguish between the two symptoms as they are both managed differently.

Historically, a range of drugs with antimuscarinic actions have been used in an attempt to control hypersalivation. Blockade of cholinergic muscarinic receptors reduces salivary volume, but a lack of selectivity may result in widespread and undesirable central and peripheral effects, including drowsiness, restlessness, irritability, urinary retention, constipation, and flushing.

There are no randomised controlled comparative studies for the management of hypersalivation in MND, so prescribers should consider evidence for effectiveness, potential side effects and available routes of administration when choosing between them. Of the medicines listed in the following tables only Xeomin® is licensed for chronic sialorrhoea due to neurological disorders: all others are off-label use. It is common practice within treatment of MND patients to use a licensed drug for an unlicensed indication. This is supported by experience in clinical practice.

NICE guidance on MND was published in 2016. For sialorrhoea, this guideline recommends an anticholinergic as first-line treatment and referral to a specialist for botulinum toxin type A where first-line treatment is not effective, not tolerated or is contra-indicated. No specific antimuscarinic is recommended except where the patient has cognitive impairment and glycopyrronium should be used first-line as it has fewer central nervous system side effects.

There are various pharmacological treatments which have been used in the management of hypersalivation. The choice of drug should be based on its pharmacological and adverse effect profile. The table below aims to provide consensus based recommendations from the MND specialist team in the absence of a strong evidence base.

These medicines can be used in combination for additive effects. Monitor patient for side effects e.g. constipation, urinary retention, dry mouth, sedation, delirium. These medicines are contraindicated in: narrow angle glaucoma, myasthenia gravis, megacolon, pyloric stenosis, paralytical or obstructive ileus.

Refer to Summary of Product Characteristics (SPC) for each drug for full list of adverse effects, cautions and contraindications.

Hyoscine patches are often cut in practice, however the manufacturer cannot recommend this and state that efficacy and safety have not been evaluated when the patch is administered in this way. Patients and carers should be advised to report any leakage from the patch. Patches have also been occluded to prevent a portion of an intact patch coming into contact with the skin. Prescribers should be aware that cutting or occluding the patch would be off-label use of the medication. It is good practice to gain informed consent from patients. Patients and carers should wash their hands thoroughly after handling the patch to minimise the risk of transferring hyoscine to the eye.

Hyoscine butylbromide tablets are soluble in water and have been crushed however the manufacturer cannot recommend this. Prescribers should be aware that use would be off-label. It is good practice to discuss this with the patient where possible.

This can be very distressing for patients and may be caused by dehydration, mouth breathing, or saliva evaporating in the mouth. If patient is distressed, consider sublingual lorazepam 0.5-1mg if required or propranolol 10mg twice daily for anxiety. For thick, tenacious saliva, humidification, saline 0.9% nebulisers and carbocisteine 750mg TDS (reducing to 500mg TDS) are recommended as first line options (caution in patients with poor cough). Other recommended measures include:

1. National Institute for Health and Care Excellence. Motor neurone disease: assessment and management (Feb 2016) (NG42). London: National Institute for Health and Care Excellence [cited 2019. July 7th]. Available from: http://www.nice.org.uk

2. Ng L, Khan F, Young CA et al. Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2017, CD 0117776

3. Buscopan 10mg Tablets. Summary of Product Characteristics. Last updated 25/01/18. Accessed at www.medicines.org.uk on 03/07/19

4. Smyth J. The NEWT Guidelines (online). Betsi Cadwaladr University Health Board (East). Accessed at www.newtguidelines.com on 03/07/19

5. White R, Bradnam V. Handbook of Drug Administration via Enteral Feeding Tubes. Third edition. London, Pharmaceutical Press, 2018 (electronic version). Accessed at www.medicinescomplete.com on 03/07/19

6. Twycross R, Wilcock A, Howard P. Palliative Care Formulary. London, Pharmaceutical Press, 2017 (electronic version). Accessed at www.medicinescomplete.com on 03/07/19.

7. Sanofi. Scopoderm 1.5mg Patch. Summary of Product Characteristics. Last updated 24/01/19. Accessed at www.medicines.org.uk on 03/07/19.

8. Scottish Palliative Care Guidelines. https://www.palliativecareguidelines.scot.nhs.uk/guidelines/end-of-life-care/care-in-the-last-days-of-life.aspx [accessed 21 January 2020)

For further specialist advice, please contact the MND Clinical Nurse Specialists on Tel: 0141 201 2380 or 2381.