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Living with KD

Personal Stories

John Ireland, Born 1939 - England

John Ireland, Born 1939 - England

I was born in Liverpool in 1939, had a normal childhood. I left school at 15 years of age and became an apprentice plasterer. On finishing my apprenticeship I was conscripted into the Army, in October of 1960, and spent over 2 years in Germany.

On release from the military I returned to my trade of plasterer.  Then in 1967, I met Janet and we married 2 years later.  As well as my occupation being very physically demanding, I did lots of work upgrading our house.
All in all I led a very physically active life.

Early in 1974, I started to notice that my legs were stiffening up and I was having to walk more slowly. Stairs were becoming a struggle, and work was getting harder to do.  I had difficulty lifting my feet up onto curbs, and sometimes the power would just go from my legs without warning (like someone had switched off the light) and I would fall where I was - sometimes downstairs. I also had difficulty in lifting my arms above my head.

I spent most of 1975 in the hospital. Initially, I was referred to an Orthopedic Consultant, who prescribed a corset for the back problem he thought I had! Then I was referred, at my request, to the Neurological Unit at Walton Hospital, where I underwent extensive tests. Lead poisoning was considered because I had very high levels of it in my blood, but there was no improvement when the levels were reduced. Ultimately I was informed that I had Spinal Muscular Atrophy, there was no treatment or cure, but the doctor said that I would be monitored annually, as the condition is progressive.

I had already given up plastering, and was doing less physical work. The doctor told me to keep moving as much as I could, so I went swimming regularly, and used a rowing machine and exercise bike. I then developed problems with swallowing, and used to wake up choking during the night. I had to eat soft, moist food, and needed lots of fluid to help get it down. My ability to walk reduced considerably, stairs were a huge problem. I avoided going anywhere I couldn't park very close. I was forced to give up work completely in 1979 because of my condition. We were fortunate that Janet had a good job.

We had to adapt our house by adding a ground floor bathroom, and I was having to use a wheelchair to get around outside the house. As things worsened, especially with falling downstairs, we finally moved in 1989 to our present home, which is a bungalow. Our first visitor was the local Occupational Therapist - Sally. She was a ray of sunshine in, what was for us, a very depressing time. She was so helpful with various disabled aids and equipment, and good advice, that it made life easier for me.

The choking episodes increased in regularity and severity, and it was very frightening. My doctor referred me to a throat specialist. He was a
disaster. He had no understanding of what might be the cause of the problem, and was very insensitive. My doctor decided to refer me back to the Neuro Unit at Walton, and in 1991 I was admitted for further tests. The diagnosis did not change, they found that I was weaker than in 1975, and I was again told that they could do nothing for me. I never heard from them again.

I had to stop driving, which was very depressing. Eventually I was unable to get out of the car on my own. I had to be lifted, which was very hard on my wife. We found a special wheelchair that becomes the front passenger seat of a car, so we bought one and had our car adapted to take it. This meant that I stayed in the chair throughout, from leaving home to getting back there.  It was horrendously expensive, but worth it.  Equipment provided by the local Council needed to be upgraded because of my deterioration, so we received further visits from the local O.T.  Unfortunately not Sally, who had moved to work in Lancaster, and happily is now a firm personal friend to us both.  She told us of a centre in Winsford, Cheshire, that treats people with neuromuscular conditions by means of physiotherapy and hydrotherapy. My doctor referred me, and they (being a charitable organization) agreed to treat me. It has been very helpful.

I was told by staff there, that I should have a Consultant involved in my case. They recommended one who is a Pediatrician, who has an interest in
neuromuscular conditions. I went to see her in January 2000, when she said very quickly that she thought that I was suffering from Spinal Bulbar
Muscular Atrophy - Kennedy's Syndrome.

A blood test was taken and confirmed it in just 2 weeks - 25 years after the original diagnosis. She arranged a full series of general health checks, referred me to a speech therapist (very helpful), a Genetic Consultant, and a Genetic Research Professor. I see her regularly and she is closely monitoring me. When I realized that the condition is genetic, I was very interested in how it had come down through the family to me. Genealogy had been a hobby for some years (to fill in empty hours) and I had tracked an extensive family tree.

I subsequently discovered two distant male cousins with Kennedy's and a female cousin who is a carrier. I have established that we share the same
ancestor - my great grandmother - who came to Liverpool from Ireland in the 1860's.

I can do very little without help now, I have problems with my neck and need to wear a brace sometimes. It is hard to lift my arms to shave or comb my hair. I feel the cold badly, especially in my legs and feet. I still use an exercise machine, but it is passive and operates by remote control. I use the wheelchair in the house now.  I do not think that I would have got this far without the help and support of my wonderful wife Janet, who until two and a half years ago held a full time job as a Police Officer as well as caring for me.