Secretion Management

1. Problems experienced with secretion management

Introduction
Living with a neuromuscular disease brings challenges that can affect your movement and daily activities. While some symptoms are expected, others may be surprising and hard to explain, causing distress, such as difficulty with too much saliva.

Saliva plays a crucial role in keeping your mouth moist, aiding in chewing and digestion. However, in neuromuscular disease, it may accumulate, leading to issues like drooling and dribbling. Constantly using tissues or towels to keep your face dry can be embarrassing and make you feel self-conscious. The saliva itself can vary in consistency, being either thin and watery or thick and sticky. Interestingly, even with an apparent excess of fluid in the mouth, your throat and tongue may feel dry.

The increased volume of saliva can make swallowing and talking more challenging. Additionally, people with neuromuscular disease may encounter difficulties in clearing sputum (phlegm), which can also be thick and sticky.

Problems with saliva:
Experiencing a large volume of saliva, known as sialorrhea is a common challenge in neuromuscular disease. We typically produce 0.5 to 1.5 litres of saliva in 24 hours, but the weakness in the tongue and swallowing muscles can make it difficult to cope with this volume efficiently.

The delay in swallowing the saliva as quickly as it is produced, leads to pooling and if the muscles cannot create a strong seal, you may experience drooling and dribbling. We produce different types of saliva, which may be ‘serous’ – thin and watery and/or mucous – thicker, each serving different functions.

Treatments aimed at reducing the volume of saliva can make it stickier in an effort to dry it up. Due to impaired swallowing, the saliva may not be evenly distributed throughout the mouth, causing certain parts of your tongue and throat to feel dry.

Problems with sputum:
People with neuromuscular disease may have a weak cough, making it challenging to effectively clear saliva and sputum (phlegm) produced by the respiratory system and lungs from the throat. The sputum can stick in your throat or create a ‘rattling’ sensation in the chest.

As the sputum becomes thicker and stickier, it becomes increasingly difficult to remove through coughing, resulting in a sensation of chest congestion. The compromised ability to clear your lungs heightens the risk of developing chest infections. In cases where the cough is weak, these infections may lead to more pronounced symptoms than would occur if the cough were strong and efficient.

Nasal problems:
The same muscles that play a role in a strong cough are also essential for sneezing and maintaining a clear nose. When these functions are compromised, your nasal passages can become filled with nasal and sinus fluid. This fluid is naturally produced to keep the lining of the nasal passages moist. However, when there’s a challenge in sneezing or keeping the nose clear, this fluid may overflow into the throat, a condition known as postnasal drip.

2. Medications to Manage Secretions

Before starting any medication, it’s crucial to discuss potential side effects and individual tolerance with your healthcare provider. They can help determine the most suitable medication for your specific situation while minimising the risk of unwanted effects.

Excessive secretions

The most-commonly prescribed medications for secretion management are:

  • Glycopyrronium bromide
  • Atropine
  • Hyoscine

These are in a group of medications called anticholinergic agents that block the effects of the neurotransmitter acetylcholine which controls functions in the brain which play a role in memory, learning, and involuntary muscle movement. Several anticholinergic drugs are used to manage excessive saliva, including hyoscine hydrobromide, hyoscine butylbromide, glycopyrronium bromide, ipratropium, amitriptyline, and atropine.

However, these drugs are not specific to the receptors in the salivary glands, posing a risk of side effects in other parts of the body. Common side effects may include urinary retention, constipation, increased pressure in the eyes, reduced sweating, and double vision. In older individuals, there’s a higher likelihood of experiencing adverse effects like confusion, disorientation, memory problems, sedation, and nausea. In such cases, glycopyrronium may be better tolerated.

Atropine, on the other hand, is more likely to cause confusion as it easily crosses the blood-brain barrier.

It’s important to note that topically applied hyoscine patches can also lead to skin irritation.

Another drug which may help manage excessive saliva is clonidine which works in a different way to glycopyrronium, atropine and hyoscine.

Thick secretions
Dealing with thick and stubborn secretions has fewer options compared to managing excessive saliva. If thickened secretions are linked to treatments for too much saliva, lowering the dosage to the least amount that still works can be helpful. Before starting any medication, it’s a good idea to talk to a respiratory physiotherapist or a healthcare professional with the right knowledge. They can check how well you’re coughing and work out the best plan for you.

The process typically involves an evaluation of how well the cough is working and the burden of secretions. Following this assessment, a trial of medication can be considered.

Mucolytics
Mucolytics are medicines made to break up and thin the mucus in your lungs, making it easier for you to cough and clear it out. Some commonly used and helpful drugs in this category are carbocisteine, acetylcysteine, and erdosteine. These medicines can be really useful when thick mucus is causing trouble in your breathing, helping your lungs work better and making it simpler to get rid of the mucus from your airways.

Saline
If you’re dealing with more difficult symptoms, your healthcare provider might suggest using a nebuliser with saline solution (either normal or hypertonic strength). This is done to help loosen and thin out the mucus, making it easier for you to clear. It’s worth mentioning that there isn’t a lot of evidence on whether nebulised saline can change the properties of the mucus. While it might be helpful for some people, its effectiveness can be different for each person.

Bronchodilators
Nebulised bronchodilators, such as salbutamol, and medications with aminophylline can help your lungs clear mucus better by giving a boost to the tiny hair-like structures called cilia. These cilia team up in a wave-like motion, guiding mucus from your airways toward your mouth so you can get rid of it. But it’s good to know that there’s not a lot of evidence on whether these medications can change the properties of the mucus. They might be helpful for some people in making it easier to clear mucus, but how much they help can vary from person to person.

Antibiotics
When your muscles are weak, and you’re not coughing enough to clear out mucus, it can put you at a higher risk of getting infections in your respiratory tract. Antibiotics should be saved for treating these infections specifically. If you keep getting infections, using nebulised antibiotics might help, especially if specific bacteria like pseudomonas are found in your mucus.

For some people who often get chest infections (more than twice a year), taking preventive antibiotics, like azithromycin, could be helpful.

3. Physical Techniques to handle secretions

Mechanical treatment of secretions

Impaired Cough
Certain conditions, like stroke (CVA), spinal cord injuries (SCI), or neuromuscular issues such as muscular dystrophy, myotonic dystrophy, or motor neurone disease (MND), can affect how well you cough.

A cough happens in three phases, and if any of them are affected, it can make your cough less effective:

  • Phase 1 : Inspiration – You need to take in a good amount of air (more than 1.5-2 litres).
  • Phase 2 : Glottis Control – This involves closing your vocal cords to build up pressure.
  • Phase 3 : Expiratory Muscle Strength – You need strong muscles to create pressure in your chest and abdomen (over 100 cmH20).

Just by listening, we can’t tell how effective your cough is. For ongoing conditions like neuromuscular diagnoses, it’s important to regularly check how well your cough is working. There are simple ways to measure this using a peak flow meter and a cushioned mask, as shown in the picture.

A Normal Cough Peak Flow (CPF) is 360l/min upwards

Green – Able to clear secretion effectively is above 270l/min

Amber – May struggle at times of infection below 270l/min

Red – Below 160l/min – will struggle to clear secretions effectively

Anyone with a CPF in the Amber & Red zone may benefit from assistance to clear secretions and a referral for an assessment is recommended.

If difficulties clearing mucus arise or you keep getting chest infections, it’s crucial to act right away.

If the problem is thick and sticky mucus, we might improve things by making sure you’re drinking enough, using humidifiers, or taking medications like carbocisteine.

But if you notices changes in the amount or colour of the mucus, or if there’s a fever and they feel generally unwell, it could be a sign of an infection. In this case, it’s important to see a doctor urgently. For those who frequently get infections or are at a higher risk, some GPs may provide rescue medications to use during infections.

There are several options to improve cough effectiveness.
These include, breath stacking via an LVR (Lung volume recruitment) bag, glossopharyngeal breathing (GPB or frog breathing), manually assisted cough (MAC), or cough assist devices (previously known as MI/E).

Lung Volume Recruitment bag (LVR) / Breathstacking
This device is a self-inflating bag, also known as an ambubag. It has a special one-way valve that allows a technique called breath stacking. To use it effectively, you need to have good control of certain muscles in your throat, called bulbar function. The idea is to take several small breaths while someone guides you with the words “breathe in, breathe in, breathe in.” You hold your breath until you reach your maximum breath capacity. This helps you build up more air in your lungs, allowing for a stronger and more effective cough. Usually, a mouthpiece is used, but if you need a mask, it’s crucial for the person helping you to keep a close eye on you for safety. You might also have a pre-agreed sign with your helper.

When combined with something called Mechanical Assisted Cough (MAC), this method becomes even more effective in helping you clear your airways.

Manually assisted cough (MAC):
This technique involves using either a Heimlich/abdominal thrust manoeuvre or bilateral costo-phrenic compression (a side-to-side compression, as shown in the diagram) to boost the airflow when you exhale. It can work on its own, but it’s even more effective when paired with a maximal insufflation, like breath stacking using a self-inflating bag (LVR bag). This combined approach helps maximise the airflow out of your lungs and can be a helpful method in managing respiratory challenges.

Glossopharyngeal breathing (GPB) otherwise known as frog breathing
Glossopharyngeal breathing (GPB), sometimes known as “frog breathing,” involves using your mouth and throat as a sort of pump to push air into your lungs. You take successive gulps, holding each one until your chest is filled as much as possible (referred to as maximal insufflation). This technique offers various advantages, such as a louder voice, maintaining the flexibility of your chest wall, and enhancing the effectiveness of your cough to clear out mucus. In some cases, patients who depend on a ventilator can use GPB to take breaks from their ventilator.

Cough Assist device (previously known as Mechanical Insufflation Exsufflation or MI/E)
The provision of this treatment can be variable across the UK. Some HMV centres provide cough assist devices for their HMV patients. Some HMV centres will assess and provide cough assist devices as a stand-alone treatment option for new referrals. Unfortunately, not all HMV centres provide cough assist devices.

Cough assist devices work by giving you a deep breath (positive pressure) and then quickly pulling the air out (negative pressure), mimicking a cough. It’s most effective when you sync your breathing with the device—breathe in during the deep breath and breathe out or cough when it’s pulling the air out. Many people use a soft mask for the best results, but you can also use a mouthpiece with a nose clip. You can use the cough assist machine sitting, lying down, or on your side, but standing isn’t recommended as it might make you feel dizzy.

If you’re already using HMV and need a cough assist device, it makes sense to start with a slightly higher pressure than your regular breath in. The breath out or cough pressure should be at least equal, but ideally a bit higher for the best results. Your healthcare team can adjust the pressures to make sure it’s effective and comfortable for you.

For those with weakness in the throat muscles, starting with a lower breath in pressure and a slower breath in time can help prevent discomfort and bloating. Most people don’t need extra oxygen when using the cough assist, but it can be added if your healthcare team thinks it’s necessary.

Using the cough assist in the morning and evening is common to prevent chest infections, clear out mucus, and keep your chest flexible. If needed, you can use it more throughout the day for extra help. There’s no strict limit on how much you can use it, but taking breaks between sessions prevents feeling lightheaded from breathing too much.

The main devices used in the UK are the Clearway 1 & 2 from Breas and the E70 from Phillips Respironics who have user videos available on their websites.

Other devices
In certain experienced healthcare centres, the vest and the percussionnaire are occasionally used to help mobilise chest secretions. While there isn’t strong evidence proving their effectiveness, some patients may find them helpful for managing symptoms.

Humidification
When dealing with tough and sticky mucus or too much thick mucus, using heated humidification can help. Increasing the temperature adds more moisture, but it’s essential to consider your personal comfort and preference.

However, if you have a lot of saliva or thin watery mucus, using HMV without humidification can be helpful because it has a drying effect. It’s all about finding what works best for you.

4. Other techniques to manage secretions and difficulty with intrasalivary

Intrasalivary gland botulinum toxin injections
Sometimes known as Botox, botulinum toxin can be injected into the salivary glands when medications for excessive salivation are not effective or have limited use due to side effects. Trained clinicians with experience in managing excessive saliva in patients with neuromuscular conditions receiving long-term ventilation usually perform these injections. There might be a wait to get these injections, and they are often done in a hospital setting. This treatment may not be suitable for everyone.

Each side of the face has two main salivary glands—the parotid glands in the cheeks and the submandibular glands under the jaw. The team doing the injections will discuss and agree with you on which glands to inject. Generally, the parotid glands contribute less to saliva production, while the submandibular glands contribute more.

The injection is done through the skin, not the mouth. The needles used are very small, and most people tolerate the injections well, even when all four glands are injected. You might not notice the effects for a few days or even up to a week. Some people may not experience a beneficial effect. Occasionally, there might be blood-stained saliva for a couple of days after the injection, but this usually settles down. The effects of the injections can last from 3-6 months. If they cause excessive dryness, this cannot be reversed and will only improve once the effect has worn off. When the effect starts to wear off, you may consider getting repeat injections.

Radiotherapy
If injections of botulinum toxin directly into the salivary glands are not effective or well-tolerated, another option is radiotherapy to the salivary glands. This procedure is usually performed by specialised oncology teams. However, there might be a significant waiting list for this treatment.

Salivary gland surgery
When other treatments haven’t worked, surgery is an option. This can involve procedures to block the flow of saliva into the mouth or the removal of one or more salivary glands. Like other treatments, there might be a significant waiting list. The surgery usually requires general anesthesia, which might be riskier for patients who need ventilation support. It’s important to note that removing a gland is a permanent solution, and if it causes excessive dryness, it can be challenging to treat.

5. Frequently asked Questions

What devices are available that I can use at home?
If you have a weaker cough and need help clearing secretions at home, there are two main devices: a cough assist device (formerly called a mechanical insufflator exsufflator or MI/E) and the Lung Volume Recruitment (LVR) bag. These tools are designed to support you in keeping your airways clear and managing mucus effectively.

How will I know when to use each treatment and when to take a break?
Both the cough assist and the LVR bag use positive pressure and room air, causing few side effects. When dealing with retained secretions, using these devices should be less exhausting compared to persistent ineffective coughing. It’s recommended to use these devices regularly as a preventive measure, especially if your cough is weak, to avoid infections and maintain the flexibility of your chest wall.

Ideally, using these devices at least twice daily when you’re feeling well is suggested, and more often during times of infection. There’s no set maximum number of uses. It’s important to allow adequate rest time between uses or cycles to prevent feeling lightheaded due to “over-ventilating.

Can breathing exercises decrease flare ups? What can be done to maintain breathing/swallow?
Breathing exercises can decrease flare ups. Simple measures such as avoiding people with infection, coughs and other illness symptoms, keeping adequately hydrated, maintaining mobility or frequent position changes and taking deeper breaths with an inspiratory hold can be advantageous. If on home ventilation, using this more frequently, or increasing pressures at times of secretion retention can be beneficial.

If you are a 24 hour NIV/HMV user, does it have more of an impact? Is the advice or treatment different?
Using positive pressure from Home Mechanical Ventilation can help clear secretions and make breathing easier. If needed, using Home Mechanical Ventilation more often or adjusting the pressure levels can be helpful. It’s crucial to prioritise and address secretion clearance, especially when facing recurring respiratory tract infections. This approach is essential to prevent further respiratory challenges and maintain overall respiratory health.

Useful Links
Respiratory Futures spoke to Dr Ben Messer about recommendations to treat cough in patients who have Duchenne Muscular Dystrophy (DMD). Dr Messer sat on the DMD Care UK respiratory working group, who developed this Guidance.

https://www.respiratoryfutures.org.uk/features/monitoring-cough-strength-in-people-with-duchenne-muscular-dystrophy-dmd/

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