Motor Neuron Disease

WHAT IS MOTOR NEURONE DISEASE?

A description by the Motor Neuron Disease Association:

Motor Neurone Disease (MND) is the name given to a group of related diseases affecting the motor neurones in the brain and spinal cord. Motor neurones are the nerve cells along which the brain sends instructions, in the form of electrical impulses, to the muscles.

Degeneration of the motor neurones leads to weakness and wasting of muscles. This generally occurs in arms or legs initially, some groups of muscles being affected more than others. Some people may develop weakness and wasting in the muscles supplying the face and throat, causing problems with speech and difficulty chewing and swallowing.

MND does not affect touch, taste, sight, smell or hearing, nor directly bladder, bowel, or sexual function. In the vast majority of cases, the intellect remains unaffected

MND is generally a steadily progressive disease, but the rate of progression varies greatly from one person to another.

Invasive Ventilation the Right to Choice

The prospect of living with the aid of ventilation is not always an easy decision to make, especially if we are talking about invasive ventilation.

Many people whose breathing is affected are offered Noninvasive positive pressure ventilation (NPPV) such as using BiPAP or VPAP ventilators. This usually increases survival by 6 -24 months (sometimes longer). This is limited by progressive bulbar impairment; when bulbar problems become severe NPPV, such as BiPAP, is no longer effective or tolerated. Some people have difficulty tolerating NPPV for other reasons. In the past the safety of NPPV has not been as good as invasive tracheostomy ventilation (specially if used 16-24 hours/day), although this is controversial.

With the option to have tracheostomy ventilation it would possibly extend survival to around 15 to 20 years or more, depending on the level of over-all care.

With a high standard of care, good nutrition, and good social involvement a person with ALS using tracheostomy ventilation can survive until another medical problem (such as a heart attack, cancer, etc.) occurs, just like other people.

However although this looks an attractive option as far as survival goes, there are other factors that need to be considered. These include Quality of Life for both the person with MND and also that of their Carers. Of course other factors come into the equation such as costs that include the package of support required.

Quality of Life

You will sometimes hear of awful stories where people have become locked in their bodies whilst supported by invasive ventilation. Although I must admit I communicate with a family where the husband on invasive ventilation can only move his left lower eyelid and despite this he is mentally still as sharp as a razor and maintains he still gets satisfaction from his life. You also hear of carers whose life is purely centred around the care of the person with MND; it seems the longer the person survives the more the burden of care. Of course people can survive longer anyway without ventilation if their breathing is not affected, or when on non-invasive ventilation, so it is not just down to invasive ventilation. Having said this I know of families in this position where their love and gratefulness in still having the person with them far outweighs the burden of care. The quality of life issues seem to be about the extra burden and pressure upon the carer by the management and care around the vent, plus the survival time being extended, which increases the chances of greater disability, hence a higher need of support.

Of course there can be an argument for better support for carers and shared care, especially if there is a medical requirement. However we have to face the reality of the society in which we live, not only for ourselves but also for those who care for us. In the UK the availability of outside professional support with general day to day care is somewhat limited.

It was my own personal experience that I was given by a professional a very bleak picture of invasive ventilation. This is I believe partly due to wanting me to take on the realisation of life on a vent.

Other people with MND have also told me that when they have asked about this they have been given a mixture of negative responses such as total avoidance, claims that it is not available, or that it is not the way forward. I personally suspect this is partly down to costs and personal bias as well. Of course life is not easy with MND anyway and life on a vent is not easy for you or your carer, although for a time it can improve quality of life.

There are people who do survive and have a reasonable life on a vent, one person I know even goes camping still and gets out and about a fair bit. What is one person's quality of life is not necessarily someone else's. There were several people with MND from Japan on invasive vents that attended the conference in Milan.

Henrik Wendt Jensen has this to say : "Quality of life becomes the central issue. To me it is associated with three closely related aspects: a basic well-being, meaningful activities, and the partaking in a community as an equal partner.

When you are seriously disabled each one of these aspects are affected. Previous values are lost and new ones will have to be defined through a shared effort in the family. A meaningful life is not a given thing but something that you create."

In my view it takes an extremely motivated person to live on a vent and be able to find ways to enjoy life. Life on a vent is described to good effect on the following web page: http://tpals.org/vent4.htm#david

The Dilemma of Choice

I feel it is important that the patient and their carer are not removed from the decision making process and so leaving it solely for the professional involved to 'play God' as it were. However I do feel there is a danger that the carer can be pushed into a situation of almost emotional blackmail. No one's partner wants to say 'no' especially if the person wishes to hang onto life for as long as possible. It is therefore so important that everyone is clear about what they are getting into beforehand and that good honest communication is established. It is with this in mind that I feel it should not necessarily be the sole decision of the person with MND. With better support packages being available such as there is in Denmark this would not be such an issue.

However, Henrik Wendt Jensen states: "To me the decision to accept respiratory assistance - when needed - was an easy one. Because I never for one moment doubted that my life was valuable. I have experienced myself as an active, important and accepted member of my family. They have fully supported my decision and have at no time discussed it or tried to influence it. Not only could the decision be mine alone - but it would have been impossible to discuss it with my family, had their attitude been doubting. Any suggestion of doubt concerning my decision would be a questioning of my life and that would inevitably mean an irreparable damage to our relationship. The same would be the case, had I been met with any expressions of doubt from friends or doctors. Any conflict between my life and the views of others would be immensely traumatic. It is possible to learn to accept your disabilities but you cannot learn to accept that others don't find your life worth while."

In my view the carer should if possible try to find out what it is like supporting someone who is on a vent and if possible contact other carers who do or have experienced this.

My own personal experience

Not started yet

Other Factors

Sometimes people's progression is such that their breathing deteriorates at a disproportionate rate to the rest of their progression. It is in my view that these sorts of situations where the person is still fairly able that the possibility of an invasive vent becomes a real consideration, though not exclusively so.

Living Wills are a helpful consideration if you have MND especially if you are intending to try for invasive ventilation in order to avoid disputes regarding your wishes once communication becomes more difficult.

Of course I have been discussing choice here with partners and medical professionals but another factor in this situation is your area Health Authority's willingness to fund. It can seem that the reality of fighting the authority over funding is probably against you even if you take the matter to the European Court of Human Rights. For a start before you can do this you have to go through the court system in your own country, and the chances are that if your breathing is deteriorating by the time you get that far it would possibly be too late anyway. Costs are always a factor but the belief that there would be a huge demand for invasive vents by MND sufferers I feel is not realistic. Only a few people choose this option in other countries; as we have already said one person's quality of life is different to another's. I am sure only a few well motivated people would want to go for this option in the UK if it was more readily available. I again quote Henrik Wendt Jensen: The only public interest in the matter is - strictly speaking - the financial question and when it comes to it few people have the courage to deal with that question.

To the person directly involved, who alone can contemplate the problem, the question is quite different and far more simple. The key-question is not ventilator or not, but whether you find pleasure in life or not. If you have a zest for life in spite of your disabilities, then the ventilator is a minor detail which can never become decisive.

Is There a Culture of Paternalism?

There seems to be odds stack against those with severe disabilities in the UK especially around choice. It seems to many PALS that there is an almost paternalistic culture where everyone else knows what is best for them. There are many procedures that require mechanical interventions in order to retain life, for example pace makers, kidney dialysis machines etc. However once it comes to invasive vents it is often a reluctance due to the wish to protect the carer; 'enough is enough' so to speak. It appears to me that this is often before the carers' or patients' wishes have been explored let alone a care package.

Why should some people in other countries be given this option but in the UK there is almost a blanket refusal to consider it? Are doctors less caring in the USA Japan or Europe, are we suggesting that they just put people on invasive vents without considering quality of life issues?

I doubt it. According to the research document 'Non-Invasive ventilation in motor neuron disease; current UK practice' that was carried out in the year 2000 only a small proportion of eligible patients with MND were referred for NIV compared to the rest of Europe and the USA.

It also stated that the number of patients referred for tracheostomy over a 10 year period was 9.6 per year. However it was also stated that a number of these patients had undergone tracheostomy before the diagnosis or as a result of an emergency.

The "Motor Neurone Disease Association" Position

When I raised the question about the MNDA position regarding on the BUILD UK forum this was their reply:

"One of the MND Association's core values is to enable people with MND to make and act on informed choices. As a general principle therefore, we would support an individual's right to be mechanically vented, provided that their decision has been made in the full knowledge of all the likely implications for themselves, their families, and their carers."

The latest draft of the NSF (and it seems to change weekly) states in part:

"Some patients with progressive conditions will have written directives specifying those interventions they would or would not wish to have during the later stages of their disease. Some of these may be life saving e.g. tracheostomy and ventilation for respiratory failure. It is important that these directives are observed."

The Association is currently working with medical experts to produce clinical guidelines on 'The Management of Respiration in MND/ALS'. We expect to publish the guidelines in the summer.
The current version (and this shouldn't change materially) includes the following guidelines:

Invasive ventilation should be considered on the basis of a fully informed decision by the patient and carer including end of life decisions in order to avoid unplanned intervention.

Invasive ventilation should be considered electively when non-invasive ventilation is not appropriate due to bulbar symptoms and when non-invasive ventilation is no longer effective due to progression of disease.

Invasive ventilation improves quality of life and survival for patients and should be considered for all appropriate patients.

The negative implications of use of invasive ventilation should be fully discussed with patients and carers.

All of our Care Centres should adopt the guidelines and we will also be disseminating them to others involved in the care of people with MND.

We have also submitted the draft guidelines to the NSF team, and hope that the final version of the section on invasive ventilation in the NSF may reflect our view more closely.

Summing Up

Despite all the reservations the right to have the option of ventilation via tracheostomy should be available. It is not a question of saying 'I want it so I should have it'. There is the carer's quality of life to consider as well as the medical implications / suitability of the procedure etc. However there are many people in the UK and in other countries who wish to support their partners; some are elderly, some are extremely sick, it is not just MND where this occurs. Are we really going to say to these people that we are not going to treat them anymore as they are too much of a burden?

Clearly the decision to facilitate or go on a vent is not a decision that should be taken without a great deal of thought. However it is not something that should be unavailable to people who wish to live their life in their own way, as best they can.