Saliva management

Sialorrhoea or drooling does not occur due to excess saliva production but rather a decreased ability to manage normal saliva production due to weakness of the tongue, lip and swallowing muscles. A build up of saliva and thick tenacious mucus can disrupt sleep and increase the risk of choking.  

Non pharmacological management:

Sialorrhea or drooling and pooled secretions:

• position body and provide head and neck support 
• prevent and reduce skin irritation
• portable suction device
• oral swabbing and syringing.

Thick tenacious saliva:

• check fluid intake
• natural mucolytic agents: 
  • papaya enzyme, juices and ice cubes - grape, apple, pineapple and papaya
• frequent swabbing of the mouth: 
  • using plain water or a mouth wash of one teaspoon bicarbonate of soda or one teaspoon salt in a glass of water, especially after meals (avoid harsh mouthwashes)
• nebulised saline may be helpful
• assisted cough technique can help
• extra water through the PEG, if available, can have a diluting effect.

Xerostomia or dry mouth:

• often related to mouth breathing during sleep
• sip of fluids regularly 
• oral lubricants: 
  • Aquaspray, dry mouth products from the chemist, or citrus lollies to stimulate saliva. Or try 100 parts grape seed oil to 1 part peppermint oil
• review medications - some such as anticholinergic medications may cause dry mouth 

Pharmacological management:

There are few randomised controlled trials to confirm the efficacy of drugs and interventions to manage this disabling and troubling symptom. A number of papers, however, provide recommendations based on good clinical practice. 

Please be advised that a number of papers suggest the use of atropine drops orally. A TGA safety update in 2018 advises health professionals to exercise extreme caution if considering off-label prescribing of atropine eye drops to treat hypersalivation. This follows the death of an adult patient in 2014 (NB not a person with MND) that has been attributed to accidental oral ingestion of a toxic quantity of atropine eye-drop, which had been prescribed off-label for sublingual administration. Hypersalivation is not an approved indication for atropine eye-drops in Australia.

A number of papers also refer to the use of glycopyrrolate. In Australia glycopyrrolate is listed on the TGA as ROBINUL glycopyrronium bromide (glycopyrrolate) 0.4mg/2mL injection vial, for use in a variety of conditions to control excess saliva. It is not listed on the pharmaceutical benefits scheme (PBS). 

Sialorrhea is amongst others, one of the most disabling symptoms in ALS. This is particularly relevant in patients with bulbar palsy experience in whom sialorrhea is associated with mucous secretions and saliva along with an impairment of ability to swallow secretions but not due to an increasing of saliva production: this pathological alteration is caused by tongue spasticity, orofacial and palatino-lingual muscle control failure, facial muscular weakness, as well as to an inability to maintain oral and buccal competence.

Currently, there are a number of drugs that may be, at least partially, beneficial, but side effects need to be considered. In this respect, it is important that treatment is tailored according to the severity and impact on the patient’s quality of life, balancing side effects (Garuti et al 2019). 

Common issues and management options in motor neurone disease:
Sialorrhoea:
•    Anticholinergic agents (eg combination preparations containing hyoscyamine, atropine and hyoscine), atropine drops orally, glycopyrrolate (nebulised or subcutaneous)
•    TCAs (eg amitriptyline)
•    Injections of botulinum toxin into salivary glands
•    Low-dose, unilateral, salivary gland radiotherapy

Viscous/tenacious sputum:
•    Frozen dark grape juice (contains mucolytic enzymes)
•    Nebulised normal saline
•    Mucolytic agents (eg bromhexine, nebulised N-acetyl cysteine)

Xerostoma:
•    Maintaining adequate hydration
•    Artificial oral saliva preparations
•    Oral pro-cholinergic drops (eg pilocarpine)
•    Cessation, where possible, of drying agents (eg anticholinergic, diuretics)

(Lau et al 2018).

Sialorrhoea treatments include suction, drug treatments and more invasive approaches, such as injection of botulinum toxin or irradiating the salivary glands, which may improve sialorrhoea and QoL. These interventions work by reducing the amount of saliva either through its removal (for example, by suction) or reduction of salivary output (for example, by anticholinergic medications and botulinum toxin injections).

A single session of botulinum toxin type B injections to parotid and submandibular glands probably improves sialorrhoea and quality of life at up to 4 weeks compared to placebo injections, but not at 8 or 12 weeks after the injections (moderate‐quality evidence from 1 placebo‐controlled RCT, N = 20). The review authors found no trials of other approaches (Ng et al 2017).

Sialorrhoea, the unintentional loss of saliva through the mouth, is the frequent complication of neurological disorders affecting strength or coordination of oropharyngeal muscles, such as motor neuron disease/amyotrophic lateral sclerosis (MND/ALS) or Parkinson’s disease. Sialorrhoea might affect up to 42% of ALS patients, with almost half of them having poorly managed symptoms. Sialorrhoea can impair patients’ social life, while dermatological complications, such as skin rashes, may arise due to constant exposure to moisture. Moreover, the excess mouth-retained saliva in ALS patients may lead to serious complications, such as choking, which causes anxiety, and aspiration with the consequent pneumonia.

The inclusion of a sialorrhoea-related item in the ALS functional rating scale testifies both the incidence and importance of sialorrhoea during the ALS clinical course. Because of the progressive nature of ALS, presence and severity of sialorrhoea should be assessed at every visit and, when present, active treatment pursued. Available treatments include behavioural therapy, i.e. techniques to enhance periodic swallowing of saliva, systemic or local anticholinergic medications, botulinum toxin injection, electron beam irradiation, and surgical techniques. This review paper briefly describes the available options with related side-effects and current guideline recommendations for managing sialorrhoea in ALS patients (Pellegrini et al 2015).

Shoesmith et al 2021, Canadian best practice recommendations for the management of amyotrophic lateral sclerosis - See table 1: symptom management recommendations

National Institute for Health and Clinical Excellence (2016) NICE Guideline: Motor Neurone Disease Assessment and Management – see recommendation:
1.8. Managing Symptoms, Saliva problems 
​​​​
Andersen et al 2012, EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force :

Sialorrhoea recommendations:

1. Treat sialorrhoea in ALS with amitriptyline, oral or transdermal hyoscine, or sublingual atropine drops (GCPP)
2. In patients with refractory sialorrhoea, botulinum toxin injections into the parotid and/or submandibular gland are effective and generally well tolerated (level B for botulinum toxin type B, level C for type A toxin).
3. Irradiation of the salivary glands may be tried when pharmacological treatment fails (GCPP).
4. Surgical interventions are not recommended (GCPP).

Bronchial secretions recommendations:

1. A mucolytic including N‐acetylcysteine, 200–400mg three times daily, may be beneficial (GCPP).
2. Beta‐receptor antagonists and a nebulizer with saline and/or an anticholinergic bronchodilator and/or a mucolytic and/or furosemide may be used in combination. Mucolytics should only be used if sufficient cough flow is present (GCPP).
3. The patient and carer should be taught the technique of assisting expiratory movements using a manual‐assisted cough (can also be performed by a physical therapist) (GCPP).
4. The use of a mechanical insufflator–exsufflator may be helpful, particularly in the setting of an acute respiratory infection (GCPP).
5. A portable home suction device and a room humidifier may be of use (GCPP).

Being a syndrome with low incidence and short survival, most recommendations are good clinical practice principles (GCPPs) based on the consensus of experts in the field of ALS. Further randomized and double‐blind clinical trials are urgently needed to improve the management of ALS.
 

Miller et al 2009, Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. See also - Respiratory management algorithm

MND Aware e-training program MND Hub – online training course for health professionals and service providers developed by MND NSW – Session 12: Symptom management - saliva and mouth care

The Southern Metropolitan Region Palliative Care Consortium (SMRPCC), Robyn Reid, MND Shared Care Worker- MND podcasts - Secretion management and dysphagia

International Alliance of ALS/MND Association, 17th Allied Health Professionals Forum 2019:

1. An interdisciplinary model for cough and secretion management in ALS/MND, Jodi Allen 
2. Taking the secret out of secretions developing evidence for the management of oropharyngeal secretions in ALS/MND, Marian McCarron 

Factsheets:

• Considering Gastrostomy - PEG and RIG
• Breathing and MND
• Eating, drinking and swallowing

Other resources: 

MND Decision Assist Tool, Should I have a gastrostomy for my nutrition and hydration in MND?

MND NSW, Living well with MND 2019 Staying in control of your speech, saliva and swallow - Clare McCluskey

Healthtalk.org stories collected by academic researchers who interview people in their own homes, using their own words: 

Eating, swallowing and breathing with MND