Corrie de Jong talks about Huntington’s, the hereditary, incurable disease that many families would rather not talk about. Sick or not, the impact is huge. “And then you hear: you don’t have it. You have to get over that.”
Max and Verstappen. 55-year-old Gerrit Krikke is a fanatical fan of two drivers. He lives and works at Alliade in Leeuwarden, but he always watches Formula 1 elsewhere in the city, with his sister Corrie de Jong (56). On the couch with her, he enthusiastically encourages his two heroes. Gerrit and Corrie grew up in Hogwarts in a family with Huntington’s disease. The progressive, incurable brain disorder has been rampant in their family for generations.
Sword of Damocles
“A disease so bad that no one talks about it.” A poster with this tantalizing sentence hangs prominently in Corrie’s kitchen window, right next to the front door. In this way, she hopes that visitors will become curious and actually start the conversation. About Huntington and the consequences of the hereditary disorder, which hangs over families like a sword of Damocles. Corrie herself lived for years with the idea that she would slowly decline after her fortieth birthday.
In the Netherlands, about 1,700 people have Huntington’s disease, and about 6,000 to 7,000 people are at risk of getting it. The symptoms resemble a combination of dementia, ALS and Parkinson’s. The cause of the condition is a faulty gene that damages nerve cells in the brain. Corrie always uses the metaphor of Pacman, who bites his way through the brain. “How the disease progresses depends on how many Pacmans there are, how fast they go and which path they walk.”
Very scared
Her brother Gerrit – who has Down syndrome – was diagnosed with Huntington’s disease about ten years ago. ,,Gerrit does not know that he has the disease and I would like to keep it that way so as not to burden him even more. He does occasionally find that he is drunk. Because his muscles are constantly making uncontrolled movements. Gerrit gets very scared at those moments, because he loses control.”
Huntington Center of Expertise
Corrie receives guidance and advice from the Huntington Polyclinic in Grou. Her brother Gerrit is one of more than ninety northern patients who, together with their loved ones the outpatient clinic visits for a biannual check-up by a multidisciplinary care and treatment team. In the specialized range for daytime activities, attention is paid to common problems such as choking, falling, being inactive, reacting impulsively, being angry or having gloomy thoughts. The clinic is located in residential care center Friesma State van Noorder breed. This complex provides housing for ten people with Huntington’s disease. Noorderbreadth is in the process of becoming a Huntington regional center of expertise, where knowledge and expertise is shared with other healthcare providers in the region.
Huntington’s disease is passed from parent to child. If a father or mother has the abnormal gene, every child in the family has a 50 percent chance of inheriting the disease. In Corrie’s case, her beppe had Huntington, her mother too, and three uncles, one of whom is still alive. So for years she prepared herself for a slow and miserable death, as she had seen in her relatives.
Nothing to do about it
In her early thirties, Corrie decided to get tested for Huntington’s. This has been possible since the discovery of the abnormal gene in 1993. Only 10 percent of risk carriers do this. “Most people don’t want to know. There is nothing that can be done about it.” A child’s wish is often a reason to do it. This also played with Corrie. “Maybe I wanted kids. That never happened because I didn’t have a partner at the time.”
The DNA test showed that she was not a carrier of the wrong gene. ,,I always thought: I’ll get sick when I’m forty and then the decline starts. I lived by that too. On the one hand, tried to enjoy as much as possible, made long journeys. But subconsciously I also didn’t dare to enter into permanent relationships. And then you hear: you don’t have it, so you can also turn eighty. Very confusing. Suddenly your perspective is broadened. Has the deadline passed? You have to get over that.”
‘I didn’t invent this disease’
After the results, Corrie went to a support group for non-gen carriers for two years. “Huntington is a terrible disease, even for family members who test negative. How can you be happy if a family member who is very dear to you has the disease? People feel guilty. And within some families are really the ones who are not allowed to complain. Luckily I don’t feel guilty. I didn’t invent this disease.”
Corrie learned to accept that she still has her whole life ahead of her. She found love and married in 2015. Huntington is always in her life. Because her brother has it and her sister – who is in a process to get tested – perhaps too. “One family is not the other, but people prefer not to talk about Huntington. There’s a lot of shame. There is fear of reactions from others. You get ‘crazy’ symptoms when you have the disease, the outlook is not fun to say the least. At work I also kept it to myself for a long time that Huntington’s runs in the family. I didn’t want to be passed over for a promotion.”
Killed disease
It is therefore called the hushed disease by the Huntington campaign team. In recent years, this team has raised more than 4 million euros for research into a drug to cure the disease. The chances of a cure are promising. Money is still needed and that is why Corrie is writing a song about Huntington that she is willing to share via Spotify or YouTube for a fee. “I can’t cycle up a mountain or run a marathon, but I can sing a little. So yes… every little bit helps.”
Corrie’s action for Huntington