Rikki's story

Rikki and Myrna
My Story with Huntington's disease began in 1979, when I met Rikki's father. I was 18. We didn't stay together very long because of his behaviours, which I learned later were often typical of HD. For years I just thought he wasn't a very nice person.

During the time we were together he once told me his father had died of Huntington's disease, but he didn't explain much and I had no idea of it, so I didn't ask further & just accepted it. Never once did I consider HD or its consequences.

After we parted, I discovered I was expecting. I already had one little girl and though I liked the idea of another baby, I didn't want one on my own as a young single parent & I had chosen to have no more to do with the baby's dad because I didn't trust him.

I couldn't abort, so I decided it would be kinder to have this baby adopted. I believed this would be better for her, for Sarah and for myself too as I was hardly in a position to bring up the one I had by my self, let alone any more.

Family & friends who knew me well enough believed I wouldn't do it, but I was determined I would, right up to the moment I looked at her. When Rikki was born on the 3rd of July 1980, something inside me melted. I took one look at her tiny screwed up red face, screaming her lungs out & knew I couldn't go ahead with the adoption. So home she came with me & her big sister. Somehow I would find a way to cope and mostly I did.

Five years later, my next door neighbour, whose daughter was married to Rikki's dad's brother, told me he had been diagnosed with HD. At first I wan't worried until he went on to explain Rikki's 50% chance of inheriting it and its terminal outcome.

I don't remember anything between that conversation & going to talk to a specialist at the hospital. I recall the specialist telling me that one day a test would be available, but Rikki wouldn't be able to have it until she was 18. He said I should have her sterilised as soon as she was at a certain age, so that there would be no risk of passing it on to any more children.

He also told me that people under the age of 40 didn't develop HD & that most of them are around the age of 60. Rikki's dad was in his 20's.

My biggest initial worry was taking away Rikki's right to have children. I figured that if she wasn't going to get this disease until she was over the age of 40, then her children could have grown up by then & anyway, we, her family, would be there to take care of her children for her, if, by any chance, she couldn't.

Did I know or think about how a person could be affected by HD, how a child could suffer growing up with HD in their lives, or that her children could develop it too? No, nor did I have any information to tell me different. That was it, no input from anyone until Rik was about 12.

We just went home & got back on with our lives. I married and had two more daughters. After a lot of thought, I decided it would be better for the girls not to forget that Huntington's was a possibility for Rikki's future. I thought it would be easier for Rik to 'always' have known about HD than to find out when it could interfere with her dreams for her future or come as a terrible shock to her at a time when she was at a vunerable stage in her life.

I feel the knowledge of 'always' knowing, helped to soften the blow for her when it did come.

In 1993, I remember a lady from Oxford hospital coming to visit me at home to talk & update me on the progress of the test that was now available. While she was there a question suddenly came to mind, so I asked it. I don't know why…I had never considered it before, but I asked her if children could develop HD? She told me that it was extremely rare & quite unlikely.

As Rikki turned 13, her behaviour began to change. Yes there was typical teenage behaviour, but there was also something else I couldn't quite put my finger on. It was not just the change in her daily behaviour, moods & aggression, but I could see something else & to this day I can't say what it was exactly.

I watched her closely. I asked friends & family if they could see something. Their answer was always no. They said that it was typical of teenagers to behave as they did...its in their job description.

Having one teenager already that fitted the job description well, I tried to accept that reasoning, but yet something about her, still niggled me. I just felt it was more than teenage hormones.

I surprised myself one day by asking her out of the blue if she was drinking alcohol or taking drugs…but I surprised Rikki even more. The look on her face was total shock. I knew immediatly that that was not the reason for the difference in her smf I continued to puzzle over it.

I'll never forget the night I found out. You may think me strange for admitting this, but, as I was dozing off to sleep, I heard a voice clearly say "Rikki has Huntington's disease".

Suddenly I was wide awake and from that moment, I knew without a single doubt she had it. I didn't need for her to have a test to confirm it, but of course it did. I have to tell you, I had never heard voices before, nor has it ever happened since. It was strange!

I called Oxford hospital the next morning and told them Rikki was displaying signs of having HD. It was arranged for Rik to go and see a neurologist.

We arrived for the appointment and I tried to explain to the neurologist why I thought Rik had HD but I could still not describe what I could see because it was so slight. I also had no idea of any of the symptoms I should have been aware of.

I reckon when I told her of the voice as I was falling sleep, she must have thought I was a total crank, not that I blame her. I might have thought the same too if someone was coming to me for such a diagnosis of their child, with no actual hard hitting facts for thinking so.

After our 'chat', she did various balance tests with Rik, which she passed with flying colours, then she proceeded to tell me that she could see no signs of HD and advised me not to be paranoid and that I shouldn't wish this kind of disease on my daughter. I was mortified. I tried to protest, but no intelligible words would come out of my mouth. I left her office with tears falling down my face.

Within no time of leaving there, I wiped my tears away. I knew she was wrong and I knew that she would soon see that. My conviction was that strong. Fortunately the neurologist wrote to Rikki's school telling them of my fears for her and they replied saying they had not only noticed a decline in Rikki's school work, but Rikki was constantly falling out with her friends too. We were called back for a second appointment and the test was arranged.

February 4th 1994, I brought the girls a puppy. I have never been a big dog person myself, but the girls had been asking for a long time and I felt it would really help to soften the blow of the results of the test that we was to receive the next day. They were totally thrilled with Charlie, the new crazy, funny, family member.

Feb 5th, Rik and I sat in the reception room, waiting to be called in to be given the results. I still had no doubt what they would be. I had spent time preparing my daughters for the worst and Rikki and I had talked about it a lot. I told her all that I knew, not that that was a lot, really. Someone told me that they could become chain smokers, so I remember telling her that. Funny what things stick in your mind, isn't it?

I talked to her about death & tried to impress on her that it was nothing to be scared of. That it would happen to all of us, one day, somehow. I told her we were fortunate to be able to prepare for what we knew & to be able to make the time we had together count. I promised her we would do all we could to make her life happy & comfortable. I tried to make it feel that a positive result was not going to be devastating.

Anyway, back in the office, we were given the positive result.

I have never been so impressed with another being in all my life, as I was with Rikki that day. I had already shed my first bout of tears. Besides I wasn't going to fall apart in front of Rik. She needed to know we would be ok. Inside I was shaking and felt sick. I suppose I hoped I would be wrong, but on the outside I was calm & accepting.

But Rik...One tear. One single solitary tear trickled down her cheek...& her words? "What about you mum, how will you cope when I die?"

The only question she wanted an answer for was how long might it be before she had to use a wheelchair.

To this day, whenever I remember it, I feel my broken heart being filled with pride, all over again. That a person so young can be so brave and that her first concern was for me, her mum, and not for herself. It still never fails to amaze me.

The neurologist apologised for her wrong impression of me.

That was almost 14 years ago and I have learned so much through Rikki since then. If she could be so strong for me when just being given such devastating news, then I would try and remain that strong for her throughout her lifetime.

If she can so bravely cope and never have sympathy for herself, then I would try to be that way too. If she could continue to be happy and make the most of her life as she has, with a smile on her face, then I would try to be that way too.

I can't say I always succeeded, but I never forgot for too long to keep trying.

Update: Rikki passed away peacefully and comfortably this morning with all her family present. She asked that her life be celebrated rather than her passing mourned. October 25, 2007