Taking the joy away – food and Huntington’s Disease

This blog is all about food – not about its make-up, calories or scientific health benefits, but about the joy that comes with food. The pleasure of taste and perfect presentation, and the social experience of sharing food with family and friends. I’ve spoken often about good restaurants being those where that social experience is highlighted, or about the wonderful fun that can be had by preparing, cooking and eating food together at home.

So, what if someone took that all away from you? What if, slowly but surely, your ability to enjoy food diminished until it has to, by necessity, become fuel? Sounds awful doesn’t it? And that, ladies and gentlemen, is what happens to many people with Huntington’s Disease.

Picture of people enjoying a restaurant
What if you eating out became a terrifying experience instead of something fun and enjoyable?

Huntington’s, or HD, is an inherited, genetic disorder of the central nervous system. Symptoms can vary, but they tend to start with mood and cognition, moving to lack of muscle coordination and jerky body movements, along with a decline in mental abilities into dementia, and behavioural symptoms. In other words, it’s a pretty horrible disease. There’s currently no cure, and because it’s caused by a defective gene that can be inherited from just one of your parents, there’s a 50:50 chance that each child they have will inherit that gene, and so it’s passed from generation to generation.

It’s a subject that’s close to my heart, as my family has that gene. Although I was lucky enough not to be affected, I’ve seen several relatives not only go through the process of being diagnosed, but start to suffer the symptoms of this awful disease.

Last week was Huntington’s Awareness Week, and it was suggested to me that since I spend a lot of time writing about food, perhaps I should look at how HD affects this area that is so central to our lives, not just because of our physical need for sustenance, but the role it plays in our social lives.

As Huntington’s sufferers lose muscle coordination, they often develop problems with chewing and swallowing. At some point, their diet will have to be modified to adjust to this, with a move to softened foods like mashed potato. Eventually, they’ll probably have to move to a liquid diet, or even a feeding tube.

The NHS advises that people with HD need a high-calorie diet, and food should be easy to chew, swallow and digest. It suggests cutting it into small pieces or puréeing it to prevent choking, but doesn’t go much further.

The Huntington’s Disease Association (HDA) has some helpful fact sheets outlining the problems sufferers will experience, from the physical complications that loss of muscle control and coordination bring, like spilling food and choking, to the symptoms like the dementia that comes with HD that prevent people from choosing the right foods, or eating at a manageable speed.

Issues can include: trying to eat too quickly; loss of motor control in the face and neck that stops chewing and swallowing; spilling food out of the mouth; irregular diaphragm spasms that increase the risk of inhaling food; swallowing too soon; plus the embarrassment of choking or making a mess; or the mental effects of the disease leading to things like choosing inappropriate foods or trying to cram too much food in the mouth at one time.

There are plenty of practical suggestions to help, from choosing easy-to-chew and easy-to-swallow foods, foods that are high in nutrition or calories. There’s also advice on how to help the actual mechanics of eating – double handed cups, thicker cutlery, using non-slip mat and so on.

But struggling to eat is often about far more than the practicalities, isn’t it? For many people in this position, it means that the days of tucking into a steak with your friends or enjoying a roast dinner with your family are long gone. The textures and tastes of food become obstacles to be overcome, not delights to be savoured.

For HD sufferers, a simple meal with family can become a minefield of problems
For HD sufferers, a simple meal with family can become a minefield of problems (Photograph courtesy of Bill Branson / National Cancer Institute)

The HDA recognises this as an issue, saying: “The selection, preparation and arrangement of food are important in most cultures. Many of our memories of family life and friends are tied to eating food and drink. A pleasant family meal is an important part of that family’s life, a chance to talk, show love and concern, play roles on the family, catch up on the news.

“Yet this important part of family life can become a frustrating, tearful, angry time when someone is unable or unwilling to select, prepare and eat food in the usual way. Someone who for years has shopped, cooked and enjoy food may now feel lost and worthless because all or some of these things are difficult, tiring or even impossible.”

It’s not until you think about what it would be like to miss out on all the things that surround the act of eating, that you start to realise what a central role food plays in our lives – not just for our physical wellbeing, but as something that is at the heart of most societies and communities.

Cath Stanley, chief executive of the HDA, told me:”Every family event, every event in fact, tends to involve food, whether it’s a wedding, a christening or a funeral. It has huge implications.

“People become quite embarrassed, they have a tendency to drop things, and to cough during a meal. If they are in quite a social environment, it can be very difficult.

“People with HD lose the ability to do things by automatic pilot. Eating and swallowing becomes something they need to think about, so if someone is talking to them while they are eating it can be incredibly difficult.

“It further isolates them, at a period where they are already very isolated.”

Most Huntington’s sufferers will be referred to a dietitian who can help them adjust what they’re eating and give them ideas. But one relative told me that there’s not only a dearth of awareness about the effects of Huntington’s when it comes to food, but there’s also a marked absence info on what foods might suit members of the HD Community. Not just nutritionally-valuable foods, but things that are safe yet tasty. After all, just because you lose the ability to physically chew something, does it mean you should be reduced to a world of tasteless mush?

Blended food is one option for HD sufferers
What tastes good blended, and what doesn’t?

So, while I’m no expert, I did a bit of digging into a few hints and tips and a few ideas for food stuff. They might be useful for people who are dealing with this disease, or who are looking after relatives or friends who are, or they might just serve to illustrate the huge impact Huntington’s can have on the social event that is eating.

I, too, struggled to find a definitive list of imaginative foods HD sufferers can eat rather than basically resorting to babyfood. Obviously, there are ranges of high calories and high protein shakes on the market, and dietitians probably have a plethora of suggestions. But I was surprised that there wasn’t more high-profile stuff on an issue that probably doesn’t just affect those with HD, but sufferers of many other diseases. I did, however, find this blog, which had some interesting suggestions for meals that will still taste good, whilst being easy to eat.

As Emily explains, it’s not just a case of blending every meal you like when it’s solid – some things taste great in liquid form, and some things don’t. But it gives some interesting suggestions from soups to main meals and desserts. Blended waffles sound good to me, while blended pizza is certainly intriguing. While this may not be a comprehensive list, it gives an idea of how there may be ways to keep food safe, but still enjoyable, for people who have issues with chewing and swallowing.

I feel I’ve only scratched the surface of what is a surprisingly important issue that so many of us, myself included, probably take for granted. In the same way that those who enjoy live in countries where food is easily obtainable and affordable often take it for granted, so do many of us who experience no issues with food take for granted how isolating it must be if our ability to enjoy a meal was taken away from us.

An awareness of this issue is all Cath Stanley asks. “I think it’s about people being aware, if you’re out in a restaurant and someone is maybe coughing or dropping food, it’s actually being aware of the fact that that might be due to an illness and being more empathetic.”

In a world where for many eating is as much a pastime as a way of fuelling our bodies, the removal of the ability to enjoy this is yet another blow for those suffering from HD, or indeed any other disease that affects sufferers’ ability to take part in this most basic of human acts. And while we may not be able to do a great deal, the least we could do is spare a thought for how that might feel.

The Huntington’s Disease Association supports people affected by the disease, as well as providing information and advice to professionals who are trying to support families hit by this disease. Its work is invaluable to those dealing with Huntington’s – not just my relatives, but between 5 and 10 in every 100,000 people in the UK affected by HD.