Saturday October 1st 2022

The disease they won’t talk about – Huntington’s

Columnist Erica Southey explores a dark family secret which has haunted her from birth.

Huntington’s Disease (HD) is our ‘dirty family secret’ that still roams around, separating scared individuals from the outside world. Ignorance is not bliss.

Faith, a handful of close friends and the will to survive kept me going whilst I went through life in a dark haze with the odds stacked against me in the form of a question: “Do I carry the dreaded Huntington’s gene?”

My closing chapter was already written. It was just a matter of how many good years in-between I was allowed before the same cruel fate would finally finish me off like it did my grandmother and mother. This! the expectation from my family based on Huntington’s history on my mother’s side. I was marked to face the same cruel fate. A death sentence that would see a daughter and sister reduced to a heap of bones shaking on a mattress on the floor of a psychiatric institution.

That is almost the last mental picture I have of my mother. The lie would continue as it did for generations. Huntington’s Disease was a dirty family secret to be hidden at all costs. A cost that was dear to my dad, brothers, me and close family who couldn’t understand what was happening to my mom. The lie my dad was told when he popped the big question any woman on the brink of a new life wants to hear – was that my grandmother is in an institution because she fell off a horse. This explained her involuntary muscle movements and incoherent speech.

My dad didn’t realise that my grandmother was in the final stages of HD. My mom had it worse. The years went by. Three children later, my dad started realising something was very wrong. The woman he had married suddenly had fainting spells, involuntary muscle movements and turned into Jekyll and Hyde. She went from a gentle woman to this raging mad stranger who threw things around. That is when the horror dawned on him – his memory flew back to a few of the rare visits to his mother-in-law.

My dad confronted my granddad, who then let the cat out of the bag. My dad said he was horrified, angry and most of all felt guilty bringing us in the world subjecting us to a similar fate. My dad started to read up on the disease and got involved as far as Prof Hayden in Canada – a long and tedious process considering so little information was available, let alone the internet highway in those days.

A further challenge which he faced in South Africa was that so few people wanted to talk about “IT”. He came home frustrated, trying to understand this strange disease that played havoc with his family.

This is my experience of HD. From six years old, I was asking why my mom was making funny movements with her mouth. And to this day, the disease still carries an air of anonymity. I lost friends and was often mocked by people saying “your mom is a drunk” because of the way she moved. My dad took her to all our sports events despite her condition. This left me open to lots of nasty commentary, ostracising, pity and few friends who thankfully, despite the situation, were there for me. A boy I really loved and who loved me couldn’t face seeing me turn “like that”. A tough price for a young girl with dreams of happiness. There was no forever after, just a dark, pre-determined outcome drilled into me by others.

Before predictive testing became available, I took the big step and got married. My husband at the time (now ex) knew what he was getting into. He saw my mom and we explained what was wrong with her. He at least had a choice – one my dad was denied. I fell pregnant and had a miscarriage. Fears of HD resurfaced and I had myself sterilised – a decision at the time (1989) thought wise. I now regret it, considering I tested negative in 1996 for Huntington’s Disease.

I took the scary step of finding out my fate. Scientists made a breakthrough and predictive testing became available in the early 1990s. Time ticked by painfully slowly as I tossed and turned contemplating my fate, though faith kept me strong and I believed that my result would be negative for Huntington’s Disease. It was like a game of chance with the odds for Huntington’s Disease being 50/50. If the test is negative then your children and generations to follow are free.

Facing a team of specialists, a psychologist and a nurse at the Genetic section of Groote Schuur Hospital in Cape Town, I was put through a rigorous testing protocol before they deemed me fit/unfit to give me my results. Armed with faith, I progressed through the steps very quickly. I was given a new lease on life, but bore the scars of a family that in their own way distanced themselves from me.

How could I have planned any sort of life back then? I would never face what my mom did. I recalled what I saw many a time. A mother struck down by this cruel disease that stripped away every ounce of her dignity. MY mother, a BA LLB graduate and Graduate Librarian, a brainy woman, was reduced to a frail invalid.

This is but a glimpse into the dark world of Huntington’s – a disease that devastates families and individuals who test positive. Perhaps some of you may never have heard of Huntington’s Disease, a well kept family secret in an age of information and awareness campaigns. Maybe you are reading this and are trapped in the Huntington’s maze, seeing someone you love waste away. Speak out and expose this dark terror – you are not alone.

7 Comments for “The disease they won’t talk about – Huntington’s”

  • Lizette says:

    I was really touched by your article. It left me with mixed feelings, sadness and relief. First I thought how sad that it must be that any child has to go through such pain, seeing her mother suffering for all those years and not be able to do anything for her. And due to this “incurable decease” being forced to be deprived from having children of your own.
    But at the same time, I was also relieved that she was spared from the dreadful Huntington’s Disease and knowing she does not have to live in fear anymore. Thank you for making me aware of what a privilege it is to be blessed with health.
    God bless.

  • Erica says:

    Hi Lizette, it often takes something like this to realise the gift of life. Sad thing is – that so many people still suffer in silence and don’t talk about HD.

  • paul bailey says:

    well my darling erica !
    what a story ! I am so pleased you are finally being recognised for the simply amazing woman you are, one word describes you ” WOOLWORTHS ” !!!!!!!

  • Erica says:

    Paul, thanks. 🙂 Thanks for your support.
    This is a great site and very informative. So keep reading and let others over there know to log on and check it out. There are numerous others caught in the same environment with HD and don’t talk about it.

  • tsanko says:

    Wonderful ..thanks a lot for posting a good informitive blog

  • Erica says:

    Hi Tsanko, HD is still a closed subject. Getting it out there is the only way that it will spark more people to talk about it.

Leave a Comment