How symptoms of MND are managed
Progressive muscle weakness is a feature of MND, but can be variable in its severity, progression and distribution. In the lower limbs it may present as foot drop, a tendency to trip, difficulty in rising from chairs and excessive fatigue on walking. It can be equally disabling in the upper limbs, causing flail arms if proximal muscles are involved, or, with distal muscle weakness, difficulty with fine movements eg opening bottles and turning keys, and a tendency to drop things. It is common to see wasting in the web space between the thumb and index finger. Sometimes an individual limb can have more severe and widespread weakness at an early stage, but unilateral weakness will usually spread to involve both sides and to variable degrees. Difficult lifting the head, especially when tired, indicates the involvement of the extensor muscles of the neck and spine, and can gradually create postural difficulty (See separate information re neck weakness)
Physiotherapists will give advice regarding the role of exercise.
- Exercises should be individualised and non fatiguing.
- Work on core stability, balance and flexibility should be considered.
- Patients with very early symptoms and no respiratory compromise may manage moderate intensity and moderate resistance exercise to maximise general fitness and exercise tolerance, but always with consideration to the risk of fatigue
- Approach to exercise needs to be adjusted in the presence of increasing weakness, fatigue or respiratory compromise
- Exercise which is excessive for an individual is at risk of damaging weakened muscle fibres
- In later stages, passive exercise can be appropriate, to help reduce the risk of fixed deformities. These should be done with great care to avoid trauma to vulnerable structures
This is often gradual and can lead to malalignment and neck pain. Postural advice and core stability work can help with alignment of head/neck/shoulder girdle/trunk. Simple neck exercises to maintain available range can be useful and specific mobilisation techniques may be of benefit to patients with pain and stiffness.
Resting and sleeping postures need to be considered, with the timely provision of a riser recliner chair offering periods of neck support.
As things progress the role of collars becomes more significant. There are limitations to what can be offered by any collar and the consideration of comfort, pressure points, and access for NIV, progressive weakness and personal preference are all important.
Pain can be a problem for several reasons:
a. Joint pain and stiffness:
As muscles become weaker there is more strain on the ligaments and tendons around the joint, which can cause pain, and occasionally can result in a joint dislocation. However, reduced movement can result in stiff joints and potentially painful contractures.
Maintaining good posture and positioning reduces strain on the joints and helps to reduce pain. Physiotherapists can provide expert advice to patients with MND to help with this and sometimes recommend exercise techniques to optimise working muscles, reduce stiffness and maintain flexibility of muscles and joints. They can also help with positioning and ensuring adequate and effective support. Physiotherapists and other members of the multidisciplinary team can provide advice and support to family and friends so that patients can mobilise safely and effectively at home.
Other approaches you may find useful include:
- The application of heat and the use of a TENS machine
- Paracetamol and Non-steroidal Anti-inflammatory drugs (NSAIDs).
- NSAID gel can be applied to a localised area.
- Occasionally stronger painkillers are needed. Some of these come in a patch preparation which can be particularly useful if you have difficulty swallowing.
b. Muscle cramps and spasm:
Damage and degeneration of motor neurones can cause the muscles to spasm and cramp. A range of medications (such as baclofen, tizanidine and benzodiazapines amongst others) can be prescribed to help with this. The medications work in different ways to help to relax the muscles and relieve pain. Physical therapies can also sometimes be effective in reducing cramp and spasms – some people find TENS machine useful. If spasticity is very severe and localised then sometimes botulinum toxin injections can be helpful.
c. Skin pressure and damage:
If the skin is placed under pressure (for example by sitting on a chair or lying in bed) for too long the blood supply can be reduced. This can cause the skin to breakdown. Breakdown of the skin can cause pressure sores and can be painful. Because people with MND can be immobile, they are at risk of developing pressure-related skin damage and pain.
The key to managing this symptom is to prevent it from happening. The use of specialist cushions, mattresses and other equipment can relieve pressure on the skin. The type of equipment used is tailored to each patient depending on their situation. Occupational therapists and physiotherapists are experts in identifying these problems and selecting the most appropriate equipment to use. Advice can also be provided to ensure patients with MND are re-positioned regularly to help prevent the pressure building on skin.
If damage to the skin does occur the pain can be managed using a number of different medications. Doctors can discuss pain relief with patients depending on the particular situation. District nurses can visit you at home and help with dressings, seeking support from specialist 'tissue viability nurses' if needed.
Management of Nutrition
People with MND may have difficulties eating because the muscles of the mouth and throat are weakened, or because arm muscles are affected making eating physically difficult, or simply through fatigue or a lack of appetite.
Difficulty swallowing (dysphagia) is a common problem in MND, affecting up to 87% of patients with the disease. This can limit the amount and range of food which you are able to eat, leading to weight loss and poor nutrition. It can also increase the risk of aspiration, that is food 'going down the wrong way' (into the lungs rather than into the stomach), which can lead to infection (pneumonia). Good nutrition is vital for maintaining good quality of life as it helps to maintain muscle strength, improves healing and keeps the immune system healthy. If you have problems swallowing, tube feeding can help to maintain good nutrition. However, it is best to consider if you would like this at a relatively early stage while you are well enough and your respiratory function is adequate to undergo the procedure.
When to Consider a Tube
- When your weight goes 10% to 15% below your weight at diagnosis
- When eating or drinking leads to frequent choking and risk of aspiration pneumonia is increasing
- When it takes more than one hour to eat a meal
- When eating is no longer a pleasurable activity
- When respiratory function is decreasing
When NOT to Consider a Tube...
- When your breathing function is considered too poor
- When the risks outweigh the benefits
The Speech and language Therapist will assess for any weakness in the muscles which are involved in swallowing and recommend strategies to modify swallowing. (S)he will work closely with the dietitian, advising you on the types of foods and liquids which may be easier and safer for you to eat and drink, and looking at alternative methods to prepare food to maximize nutrition.
The respiratory (breathing) muscles are affected in most patients with motor neurone disease at some stage in the disease process. This can make breathing and coughing more difficult. It is important you have regular breathing tests to check for any respiratory muscle weakness. Sometimes, but not always, you may notice you are short of breath on exertion or talking. Overnight, if your respiratory muscles are weak, carbon dioxide levels may build up in your blood. This can cause daytime sleepiness, morning headaches, feeling confused or sleep disturbance. You should immediately inform a professional if you have any of these changes. Non invasive ventilation can be very effective in helping manage symptoms. This involves wearing a tight fitting mask or nasal tubes which ensures air is taken into the lungs. This is initially used at night but in time may also be needed during the day time as well.
As respiratory muscles become weaker coughing may become more difficult. Various pieces of equipment can be used to help clear any secretions from your chest. Sometimes medications can also be helpful with cough and breathlessness – your doctor can discuss these with you.
Weakness of the mouth and swallowing muscles can sometimes cause difficulties with managing saliva. This can lead to drooling which can be a distressing and embarrassing. It can be helped by supporting head position and using a suction device. Saliva may also become thick and sticky making the mouth feel uncomfortable. Some people also experience a very dry mouth. Fortunately there are several therapies which can help to relieve these symptoms
- Tablets or Patches. Medications (eg hysocine, atropine, amitriptyline, glycopyrronium) can be used to reduce the production of saliva. They can be given orally (tablets or drops) or via a patch (hyoscine) which sticks to the skin and needs to be changed every three days
- Botox Injections. There is some evidence that injection of botulinum toxin directly into the salivary glands can be effective in reducing saliva production. This is a temporary treatment which lasts for approximately 3-6 months and then will need to be repeated.
- Radiotherapy of salivary glands. This is occasionally used to reduce saliva production. It is a permanent option; Typically a patient will be offered radiotherapy to one side first, with the option of radiotherapy to another gland if there has been no significant benefit. It may produce some skin soreness, which may take a few weeks to settle.
Thick and sticky saliva
- Fluids. It is important to have enough to drink. You should try to have 1 ½ to 2 litres of fluid daily avoiding caffeinated drinks and alcohol.
- Pineapple juice or papaya juice can help reduce the stickiness of saliva.
- Steam inhalations can also help
- Medications can also help. These can be taken in liquid or tablet form eg carbocisteine (mucodyne)
A suction machine may be used to help remove saliva from the mouth.
Artificial saliva. Mouth gels or sprays can help moisten the mouth
This is a common symptom, and has many causes. Weakness, including reduced power of the abdominal muscles to push, loss of mobility, effects of medications, a low residue diet and dehydration may all contribute. Prevention is important, but laxatives may be required.
Anxiety can affect people living with MND. as they start to think about finances, family and future care issues. These anxieties may be eased by talking to a professional. At times of extreme anxiety panic attacks can occur. Relaxation techniques and learning how to control breathing may help with this. Medications can also be used to relieve panic attacks if they are very problematic
Fear. Many people fear what will happen in the future as this disease progresses. You may find it helpful to talk to a professional who has experience of MND about this. You may wish to talk about what is important to you and what types of treatments you would consider as your disease progresses
Emotional lability. Some people experience uncontrollable episodes of laughing or crying. This can occur when you do not feel particularly happy or sad and often stops within a few minutes. If this is embarrassing or causing you concern, medications can be used to help to manage this.
Depression and grief is a natural reaction following the diagnosis of MND and at intervals during the course of the illness. Both practical and psychological support is important in helping to relieve this. If these feelings are overwhelming you should speak to your doctor or nurse who can discuss this with you further. You may find it helpful to talk about the way you feel to someone who has training in offering support. Medications can sometimes be helpful.
Most people living with MND are fully aware of their situation and maintain good mental functioning even with increasing weakness. However, a few people (approximately 5%) develop frontotemporal dementia. In this situation they may have difficulties in planning complex tasks and decision making, or they may develop disinhibited or rigid behaviour or become withdrawn. A higher proportion have more subtle cognitive involvement (20-40%), which appears to be more common in patients with predominantly bulbar symptoms rather than limb involvement. Support is available from many members of the team, and a social worker and occupational therapist's input may be invaluable.