Issue 93, June 2006
The following is based on an article that appeared in HDA UK Newsletter
Feeding in the Late Stages of Huntington’s Disease
By Dr Keith Andrews MD FRCP,
Director of Institute of Complex Neuro-disability,
Royal Hospital for Neuro-disability, London.
Food in HD
Food is important to us for several reasons - to keep us alive and healthy and also as part of our social interactions (such as family meals). One of the consequences of Huntington’s Disease (HD) is that at some stage swallowing can become difficult. Another consequence of the involuntary chorea movements of HD is that they use up energy. Thus a person with HD will be faced with two problems - an increased need for food but a difficulty in swallowing that food.
Whilst it is uncommon for the stage to be reached where swallowing food becomes impossible the amount of time required in feeding can become very prolonged. How well the person manages to get sufficient food is therefore dependent on the amount of help that is available. Also the effort in feeding over such a prolonged period of time can be quite tiring.
Methods of Feeding
There are solutions to this problem. First changing the texture of the food can make it easier to swallow. Secondly ensuring the food is of a high nutritional value can decrease the volume of food needed. Where these are insufficient then there are methods of providing food through a tube directly into the stomach thus by-passing the difficulty of swallowing and allowing a good nutritional state to be achieved.
There are two main methods.
The first is using a naso-gastric (NG) tube. This is a tube inserted through the nose into the gullet and then into the stomach. This method is easy and the tube can be put in by a trained nurse. The disadvantages are that they are unsightly, they irritate the throat and can cause leakage into the lungs. NG tubes can, however, be very useful as a short-term measure.
The second method is known as a Percutaneous Endoscopically-placed Gastrostomy tube – more commonly referred to as a PEG tube. This is a tube that is passed into the stomach through the skin of the abdominal wall with the guidance of a gastroscope (a flexible ‘telescope’) which is placed into the stomach through the mouth. This ensures that the doctor can see that the PEG tube is pushed through the skin into the right part of the stomach. The advantage of this method is that once it has been inserted there is no discomfort and much less chance of food spilling into the lungs. Also, once in place, the tubes can function well for several years before requiring replacement. All nutritional and fluid needs can be given by tube using specially designed liquid formula. Some food or drink can still be given if it is safe to do so and the person wants it.
When should PEG feeding be considered?
Since the stage when one of these tubes is likely to be required is usually only found in the very late stages of HD this creates ethical questions of what is actually being achieved by using these artificial methods of feeding. Are we prolonging life or prolonging the terminal stages of HD?
Let us try to think this out. Let us assume first that the tube is required for someone who is fully mentally alert. What are the reasons we would give for putting in a PEG tube? First it would be to maintain good health and prevent the complications of being underweight. There is also some clinical experience that getting sufficient food and fluids improves the mental state, and relieves anxiety and agitation, with possible beneficial effects on choreic movements and psychiatric manifestations. Thus maintaining a good diet and hydration can improve quality of life.
But the need for feeding tubes rarely arises whilst the person with HD is sufficiently mentally able to make decisions. So what about the situation were, the person with HD is no longer able to make decisions about whether to be tube fed or not. To a large extent the benefits are the same as for the person with mental capacity to make the decisions.
The real concerns arise in the very late stages, where there is deterioration both physically and mentally, when the decision is whether putting feeding tubes in is unnecessarily prolonging the deterioration phase when there is little or no discernible quality of life. Of course, the only person who can really say whether it is worthwhile is the person who has HD and needs the tube - but they are unable to tell us.
Of course, it may be logical to put a tube in at a stage when the person is beginning to lose the ability to make decisions but still has the ability to have a reasonable quality of life. The problem then is whether there is any stage where it would be appropriate to remove the tube.
To remove the tube has enormous emotional concerns for family and professional carers and therefore not a decision to be made lightly. It is much easier not to put a tube in than to make a decision at some stage that it is inappropriate to continue tube feeding. But this may prevent the tube being used at a time when it would be beneficial. At present the law only requires the decision to withdraw tube feeding to be judged by a Court in the case of people in the vegetative state. So this leaves the doctors and the clinical team with a dilemma of having no clear legal guidance on what is legal in the case of someone with Huntington’s Disease.
So where does this leave us? Our own experience on the Huntington’s Disease Unit at the Royal Hospital for Neuro-disability in London is that people with HD can actually have a good quality of life until the very late stages. It is also our experience that the vast majority of people with HD can be fed by mouth until the very late stages - but it does require a lot of staff experienced in how to feed a person with HD. So in a very specialised unit there may not be the need for PEG feeding. Where staff and skills are not available it may well be appropriate to put a PEG tube in to ensure that the person does not starve. It is important to recognise that a PEG tube does not necessarily deprive a person of the enjoyment of having some food by mouth where swallowing is slow but safe enough for small amounts. Thus under some circumstances PEG feeding may very well be helpful in making up a short-fall in nutrition and/or hydration.
So what will happen if you do not get sufficient food? This will depend on how much difference there is between the amount of food you need and the amount of food you can take in. If there is very little difference between the two then there may be a slow loss in weight over many months. The greater the difference between how much food you can get in and the amount of energy your body is using then the greater will be the weight loss. The consequences of this is that you will be more likely to get pressure sores and will be more vulnerable to get infections, especially chest infections which may lead to pneumonia.
The only person who can really say what is appropriate is you and therefore it is important that you do think out what you want to happen to you in the future. But if you have reached the stage where you cannot make decisions, how can you have your say? This is where an Enduring Power of Attorney may be able to help.
Acknowledgement: HDA UK –Newsletter Issue 67 November 2005