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Colin’s Story

Colin shares his thoughts, strategies and outlook on living with KD.

My name is Colin. I was born in 1953 and, along with my two brothers, I have Kennedy’s Disease (KD). In 1994 researchers from the USA came over to the UK to test members of the Hares family in Tredegar. My mother was tested and found to be positive as a carrier. Around this time Peter my older brother was suffering from severe weakness in the muscles of his arms and legs. He was subsequently diagnosed with KD. I decided not to be tested at this stage due to the negative aspects regarding insurance and job prospects. I was however fairly certain that I had KD as I had always suffered from muscle cramps and I now understood that this was a precursor to KD.

In 2010 I was officially diagnosed; basically I went to my GP and told him what I had. He then referred me to a consultant who made the diagnosis visually. This was subsequently confirmed with a blood test. During my youth, like many other sufferers, I had no idea that I had KD. I was active, fit and healthy. However, I was plagued with muscle cramps from adolescence up to the age of about 40, and had always had a small amount of breast tissue, which embarrassed me through my younger years. I didn’t learn that these were both early indicators of KD until much later.

Despite suspecting that I had KD in 1994, it wasn’t until 1998, when I was 45, that the disease really started to have a major impact on my life. I was working as a motor mechanic at the time and, despite being very fit, I started to have problems lifting things and getting up from the floor. It really struck me how bad things had become when I went to my son’s school sports day. I joined in the fathers’ race and was shocked to find myself last behind some very unfit looking dads. Although I probably could have carried on as a motor mechanic for a few more years, it seemed a good idea to change to a less physical job. As a result, I retrained and became a secondary school mathematics teacher.

For a while, with fewer day-to-day physical demands on my body, my KD became much less of a problem. But then, in 2002, my voice underwent a sudden change as a result of the disease, becoming much weaker and more nasal sounding. To combat the changes, I learnt to speak from my diaphragm and to slow down when speaking, but the challenge of using my voice to communicate with the class was still considerable. In 2004, I decided I needed to make more changes – switching to supply teaching to lower my workload and give me more flexibility. Unfortunately by 2009, it was getting difficult for me to climb even shallow stairs with any sort of load, such as books, so I had to leave teaching altogether.

Another task that has become increasingly difficult for me is maintaining the rental properties that my wife and I own. We have had rental properties since 1991, but purchased seven after 2000. My wife and I do pretty much all the maintenance and refurbishment on them, but as the strength in my arms and legs has reduced over the years, my wife now has to do most of the work with me assisting where I can. We have reduced the number of houses we have and are planning more reductions. If interest rates were higher, we would sell them all.

One of the most frustrating things about KD is not knowing what to expect when or how the disease will progress. Every sufferer, even those from the same family, seem to experience the disease differently.

Although my voice has not changed noticeably since it deteriorated in 2002, my throat is a continuing source of concern. My throat muscles, particularly the epiglottis, are weak, which allows food and spittle to enter my trachea giving rise to coughing or gagging. In the worst case, the trachea shuts altogether and you experience ‘dry drowning’ – it is as unpleasant as it sounds. My doctors have told me that if I pass out, breathing will resume – as yet I have not had to test this theory.

My arms and legs have lost strength gradually over the years to the point where I have now applied for, and received, a disabled badge for the car, but have not requested any other assistance from the local authority.

Although overall the decline has been gradual, what has been very frightening is that I have had a number of sudden, significant losses of strength, followed by a partial recovery. Every time I have a sudden downturn, there is the question, “will it come back?”

As my strength has decreased, so the chance of straining muscles and doing more long-term damage has increased. I am stubborn and lack patience – not good attributes for a KD sufferer. I push on physically when I shouldn’t, and although at the time I may be pleased with what I have achieved, occasionally I do serious damage and suffer later. For example, I went on a 14 night cruise in 2012. Each time we stopped at a new port, we did a lot of walking. By the end of the holiday, I could no longer use my right leg properly. My right-hand side is my weaker side and my leg became so weak that my foot just flopped to the ground when I tried to take a step. It took 10 months to recover and I was very lucky that the strength did come back.

To a lesser extent, I have found that my experience of KD has involved a lot of temporary lapses. Say, for example, I have a problem with an ankle giving way. This will last for a few days or weeks, then suddenly disappear almost never to repeat.

Coping with KD
There is no cure or treatment for KD but there are ways to minimise its effects on your lifestyle.

Here are some of the strategies and exercises that I have found useful:

To keep your throat muscles strong, look up and try to swallow. This is hard for anybody to do but it will strengthen swallowing. Sing – even badly – to get the diaphragm working.

If, like me, you experience your throat restricting your breathing, push your head back as far as you can and draw in as much air as you can – long breaths are what you need. The reflex is to cough but try to ignore it until your throat releases. My first experience of this led to sheer panic. Now it’s still frightening but manageable.

Voice and facial muscles
The tongue is used in the formation of sounds. Mine has been badly hit by KD and is wasting away. To strengthen the tongue, push it against the roof of your mouth and then against each cheek

Another ‘do anywhere’ exercise is to say the vowels, A E I O U, in an exaggerated way. This exercises the facial muscles and works the tongue.

As my walking has got slower, so opportunities to get my heart to work harder have receded. I now swim – about 24 lengths each time in a 30-metre pool – taking breaks during the swim.

While in the pool I use its buoyancy effects to take weight off my legs. This allows me to walk on tip toes and strengthen my ankles. Walking backward and sideways has also proved very useful for maintaining my balance.

Life without the use of my legs is a constant worry so maintaining what I have is crucial. However, my key piece of advice is “try not to overdo it”. This is much easier said than done. Even now I don’t know my limit as it changes from day-to-day, but pushing yourself too far can have devastating consequences. Every individual has to listen carefully to what their body is saying about how much they should try to do.

Swimming, as I have said, is excellent exercise. Slow walking reasonable distances is another way I keep my sanity – and knowing the wheelchair will have wait another day! For anything past 200 metres I use Nordic walking poles. They have been my saviour, giving me the ability to walk extra distance and giving me more confidence re falling and stumbling. I have read that the poles take 30% load off your legs and increase calorie burn by 45%, not to mention giving the upper body a workout while walking.

Remember the effects of cold weather are accentuated in KD muscles so try to warm them up before exercising. For me, stairs no longer lead to heaven – they just fill me with apprehension, so at home I go up on my hands and knees. It even seems normal now.

I have had several falls as a result of KD and it is amazing what damage you can do to yourself in the process. Once you lose your balance you are a passenger – your reflexes will not work fast enough to save you.

One obvious precaution is to stand by something to support you just in case, but good balance is a great asset. Standing on one leg for as long as you can – say 30 seconds – will improve feet muscles and the brains’ sense of balance. I do it while waiting for the kettle to boil. The core abdominal muscles also aid keeping us upright. Get down on all fours and pull up your tummy, hold it for 10/15 seconds and release. Repeat this until bored!

One of the toughest things for any sufferer to accept is that KD is a degenerative disease and, whatever you do, you will not get ‘better’. I have found that the best way of coping is to talk to people – friends, family, medical professionals and fellow sufferers. You may be the one with the disease but all those around you – that care about you -suffer along with you, so think of them once in a while and thank them for being there.

There is currently no medication for KD. We all are looking for some magic cure, but until it comes along I will tell you what I take.
Turmeric – 800 mg per day; Ginger 550 mg per day; Vitamin E: Vitamin B complex.

None of the above harm me and they may do me some good. Turmeric, for example, has had a very good write up in neurological circles, as well as oncology papers. It is said to encourage the production of neurons.

KD is a horrible disease that affects every sufferer differently. There seems to be no way of knowing if you’re going to have a good muscle day or not – believe me I have tried to work it out to no avail. So what I do is take every day as it comes and remember to smile every now and then.