Struggling with Being Negative

Struggling with Being Negative

My story with Huntington’s Disease started almost thirty years ago, when my mother was diagnosed. From teenager to this day, I have witnessed the many faces of HD. I’ve seen my mother lose her ability to do tasks she used to master and slowly fade away in a nursing home. I’ve watched my sister suffer from several psychosis, having severe delusions and rapidly decline until she passed away on my birthday. My other two siblings are in middle stages of the disease.
Except for my mother, everyone in my family have been completely unaware of their illnesses (anosognosia), which has made it very difficult and frustrating to help them. I have noticed the onset of cognitive and psychiatric symptoms years before the onset of motor symptoms. I’ve been the one who’s had to bring up the illness when they didn’t want to hear anything about it. All my attempts to help them get early support and therapies, have backfired on me.

Chain of Generations

Last year marked the 30th anniversary of the discovery of the Huntington’s disease gene. The discovery opened up the possibility for a predictive genetic test to determine if a person has inherited a repeat mutation of the HTT gene. Taking the test is a difficult decision that everyone should consider carefully for themselves.
25 years ago, I decided to get the predictive test and was given a negative result. I made the decision to get tested soon after my mother’s diagnosis. I didn’t think about my decision for long. In retrospect, I couldn’t have had the slightest clue, how a positive or negative result could affect my life. There was no way for me to understand what was to come. It took me years and years to understand the effect the result had in my life.
One of my siblings got tested at the same time and her result was positive. Two other siblings chose to live at risk. We used to be very close, but the presence of the illness, the risk of others and not talking about it, was an elephant in the room. Our individual choices and results were too difficult a topic to discuss. Some of us wanted completely to shut out the idea of their risk or talk about the positive result of our sister, while I found it difficult to rejoice in my own result.
Until today, I have already lost many family members to HD and will lose the rest in the coming years. Every diagnosis in the family triggers memories and anxiety from previous ones. As I am losing family members one by one, a piece of me disappears with them. I feel I’m losing a part of my own history by losing the only people who have known me all my life. Whether I choose or not, I can’t help but think about the future fate of the next generations, my niece and nephews and their children. Still, I try my best to get along with the growing shadow of HD.

Best Gift Ever

Nowadays I’m a mother of two wonderful sons. My children haven’t experienced how wonderful aunts and uncle they have. They never met my mother and that thought breaks my heart, every time I think about it. The constant sadness, fear for my loved ones and sleep deprivation during baby years, eventually wore me down. When my younger son was only 2,5 years old, I started psychotherapy, and was diagnosed with moderate depression. Going to therapy for three years, was probably the most important decision in my life. As my therapist once said, it was the best gift I can ever give my children.
In therapy, I had time to go through everything that had happened so far. I was able to sort through the “clutter” in my head: the sadness, frustration, anger, bitterness, disappointment, and fear. I understood many of the first HD symptoms that my mother had when I was just a teenager. In therapy I realised I had deprived myself of happiness. I had hidden my feelings because I thought I have no right to complain. I believed I must stay strong no matter what. I realised the impact HD has on my spouse and friends. Without the safety net they provide, I cannot survive.
Although I don’t have the mutated gene, I have lived and breathed HD just the same, every single day, decade after decade. I had to admit, that I also need help, support, and therapy to be able to take care of myself and my loved ones. I had to learn to set boundaries and put me and my children first. I had to understand that depriving myself of happiness, I deprive my children of their right to a happy mother. It hasn’t been easy, and I’m still struggling with it.

Survivor Guilt

My mother raised me to be a person who takes care of others. That person I’m trying to be. I have no right answers and I have certainly made mistakes, but I have done my best out of love for my family. I have felt a sense of inadequacy that I haven’t been able to help them enough, get them the best possible care, save them. Step by step I have learned to have mercy on myself.
Several years ago, I broke down in tears in a HD-conference when, for the first time ever, I heard someone say, “It’s not easy being negative”. It was the first time I knew someone understood what I was going through. It was the first time I knew someone understood that being HD negative in a family is constantly carrying a huge burden and suffering a great loss.
In another conference, I heard the term Survivor guilt for the first time, and it hit me. Survivor guilt is an experience which occurs after a traumatic event in which someone else dies. Although I couldn’t influence my own, or the inheritance of my siblings’, I felt guilty for surviving. Out of that guilt, I have not lived my life to the fullest. I am a survivor, but never felt like one.

Negative into Positive

Over the years I have done a lot of volunteer work for HD and rare diseases. I cannot help but wonder if volunteering is my way of attempting to compensate my survival and ease my guilt? Is it my way of trying to repair something? Is learning more and more about the disease my way of trying to control the unpredictability of HD? Is it my way of trying to emotionally accept the random chance of inheriting or not inheriting HD? Is it my way of trying to find answers to questions that have no logical answer?
Through my volunteer work, I have found a way to channel the sorrow and loss into something positive. Every now and then I wonder if it is still something that gives me more than it takes. In addition to work and family life, it may be too burdensome, but so far, volunteering still empowers me. Supporting others contributes to my own mental wellbeing and strengthens my resilience. And resilient I have become. That’s why I’m still standing.

Saija Ristolainen-Kotimäki

Member of the Board at the European Huntington Association
Member of a patient advocacy group at the Finnish Network for Rare Diseases


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