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Cognitive & Behavioural Changes; Life Expectations - ChAc

"Progressive cognitive and behavioural changes resemble those in a frontal lobe syndrome."

What changes occur with Chorea-Acanthocytosis?

Life Expectancy is extremely confusing for me. I was in traction when I was six for six months, which the specialists diagnosed as toxoplasmosis, and after this incident there was no indicators that brought any signs that indicated anything major, aside from some writer's cramps and facial ticks when I was 39 in the Spring of 2007.

I thought that I caught a very bad flu at the end of November, 2007. My fever rose to 40.5℃, and I was soaking wet. When I woke on the morning of 27 November, 2007, my ChAc commenced with am inability to speak or swallow.

It appeared initially as a potential beginning of Dystonia, and my descent, with attributes of Dystonia-Parkinsonism, accelerated and something felt very wrong.

We are over eight years on, and I was finally informed that my definitive diagnosis is Choreoacanthocytosis, Generalised Idiopathic Familial Dystonia-Parkinsonism this past Thursday.

My legs and feet are incapable of walking, as my tendons are so tight that tendon extension is not viable. DBS is useless. Botox can't be used due to severe anaphylaxis.

The specialist that came to Oxford's Nuffield Hospital to see me, freed my life with a definitive diagnosis. I require a mobility scooter, use leg braces that do not effectively stop the destruction with my feet, and the tendons are ripping out the cuneiforms from my feet. The appearance brings the perception that I have five ankles.

Since it has been eight years, and according to your information "Life expectancy is reduced, with age of death ranging from 28 to 61 years...Mean age of onset in ChAc is about age 35 years, although ChAc can develop as early as the first decade or as late as the seventh decade." It doesn't give an estimated lifespan from onset.

Does anyone have an estimate of one's expected lifespan from onset? I just prefer to know so I can find a way to beat this. I never give up hope. Thank you!

louise_d
moderator

Hi Gary
Thanks for sharing all this information.

Such lifespan statistics have a very limited value – disease progress is a very individual matter. However, your question is understandable.

I noticed here in the NA community under the section “Learn”, subsection “Documents”, there is an interesting article (link) of relatively recent date.

Several patients can continue to live an active and varied lifestyle for many years. Despite general similarities, every patient does not necessarily suffer from all symptoms, and not to the same degree either.
I hope this helps a bit.

Do you have plenty of support, family, etc.?
Best wishes, Louise

Dear Louise,

Thank you for the link and your suggestions.

I am man blessed with amazing levels of intelligence. I'm MENSA, yet I am also intrinsically bound by compassion and love. Perhaps this became the bardo for which my life created a balance. (I'm joking in this; self-deprecating humour can co-exist!)

I returned to Oxford because of my fiancé was incapable of handling a chronic illness from the breadwinner of the house. We had seven out of eight years of amazing love that everyone celebrated. The last year of our relationship turned into a nightmare based on my sudden change, and knowing him I knew why. Both of his parents died before his Senior Year of High School in Brighton, New York (just outside of Rochester); his father of brain cancer, and his mother of lung cancer. We suddenly saw the foundations on a relationship collapse quickly and frightening for all to watch.

So the cat is out of the bag. I'm gay, and probably the only openly gay man with any form of NA. I had a job ready for me from a former employer after our marriage at his grandfather's estate. My biological family has far too many dysfunctions and the only close bonds were from my grandparents, which all four are deceased, and my half-sister from my mother's second marriage. I love them all, but there is no familial bond. I was sent to Public Boarding School. Fell in love with my best mate. Tragedy struck, and I travelled globally trying to understand how something so beautiful could be lost. My family is my friends.

Regrettably they are not here in Oxford. They are around the world, and I am very alone, in a country that is viewing me as a feckless, work-shy, DLA-desiring Claimant that has an opportunity to meet up with my family for a reunion of friends in Miami Beach next month.

I am one of the South Beach Pioneers, that saved the Art Deco District from demolition. After our work and Liberal postcode saw the best of Life clash with the realities of so many losses from AIDS. They inspire and love me limitlessly, and I share the same for every one of them. A Documentary about us is holding its Premiere in Miami, and I am invited to it, but I can't walk, my larynx has been gone since Day One, but I can speak by training my epiglottis that the Tibetan Buddhist Monks use for their prayers. Regrettably I am financially unable to attend this Reunion of thousands that I love beyond all measure. The costs of transport for me and my mobility scooter from Heathrow to Miami and back, along with the required insurance, cost of living expenses and even clothing makes this a very sad time for me.

Given last week's confirmation in having ChAc I had relief for a fleeting moment. Now I am so aware of my dream of a Love feels like a cure for what we have: very unlikely. I now feel the weight of this on my heart, because I've watched friends that died on the daily for years, and I was a man that had every opportunity at sexual liberation. I have always felt that my intimacy is Love based on monogamy, mutual respect, and an ever-strengthening bond as part of my sexuality for my entire life.

It is a beautiful expression of Love, but I do not take this as a moral high ground. Like all sexuality it is just there. No choices. Just part of the fabric of every human on the planet, past, present, and future. I have no desire to have children, and with the Planet unwilling to take care of its six billion and rapidly ascending population I'm doing the planet a favour.

Do I want an intimate relationship and marriage? Absolutely. Would I adopt if this occurred? Yes, if my now hypothetical husband would understand of my dx.

My Chosen Family are many throughout the World. They are in NYC; Miami; London; Christchurch, Wellington, Hamilton and Auckland in New Zealand; Copenhagen; Los Angeles; and Berlin.

Here in Oxford I have my two cats, living in a Sheltered Tenancy very close to where I lived off campus when I read Law, and frighteningly alone. I should've gone to Købehavn (Copenhagen), as the UK is in a maelstrom of Political warfare on the Disabled, which is contrary to my Hygge-Centric Life.

I am alone, lonely, with two cats that keep my life with unconditional love just a short reach to me. I have a policy that if I would die they would be excellently housed with a compassionate, loving home for the remainders of their lives. I gave my promise, and I never break my promises.

So as you can imagine I'm suddenly feeling the full weight of it on me. I'm overwhelmed with sadness. I gave up everything for love, and I still hold hope, but with a rational, logical understanding of our species. I'll be 49 in less than a month from now. I do not take this life for granted, and I have no limit as to what can happen in Life. We can beat the odds. I miss and love my 'family' with every strand of my being. It is limitless and greater than our primitive abilities to fully appreciate. My life is insignificant when I see our ever-expanding Universe. As I was taught "If you cleansed the eyes of Perception everything would appear as it is: infinite." I was surprised by this. There is another bloke that is the piece of our lives' puzzle. We will see.

Thank you.

Hi Gary I was just reading your status about the life expectancy of someone with choreacanthocytosis and I like you am wondering the same thing. I'm 32 now and was diagnosed in March 2015 but I have always been a fidgety person so I probably had choreacanthocytosis from birth. My symptoms became more apparent 2 or 3 years ago as my speech became slurred and I couldn't walk properly. Now I can't swallow or hold a pen either and I'm in the process of being tested for epilepsy and narcolepsy as I fall asleep in random places at random times. I'm also waiting for the results of my test for Tourettes as I tic and swear at the most inappropriate times.
I have a cat she's my only source of companionship and I will be devastated if I die and she's taken to a cat and dog shelter. I also have a 14year old daughter who refuses to be seen in public with me or come to my house to see me. She lives with my mum at the moment so I can only see her when I go to my mums which isn't very often as I can't walk properly even though they only live 30-45 minutes away. My 1 wish is that I can get to see her grow up,maybe get married and have children of her own 1 day but the rate this illness is progressing I really don't think I will be alive to see that happen