Majorie and Marcel. Own picture
In our new section Life turned upside down we will talk to people who have experienced something that drastically changed the course of their lives. A tragic accident, kicking an addiction or the loss of a child. This week: Majorie (43), whose husband has the hereditary disease CADASIL, which means he can die ‘at any time’.
Name: Majority
Age: 43
Event: her husband has CADASIL, a hereditary disease, which means he could die ‘at any time’
Life motto: make every day a party
Life turned upside down: Majorie’s husband could die at any time due to CADASIL
Majorie (43) and her husband Marcel (50) have not been together very long when she notices that there is ‘a black spot’ that she cannot reach. When a conversation starts about his mother, she says she can never ask questions. Afterwards it turns out that this is because there is a hereditary disease in the family, which Marcel’s mother also had. It concerns CADASIL, a relatively unknown disease with an enormous impact.
Now, years later, it turns out that Marcel also has the disease and it has already manifested itself. Majorie changes from partner to informal caregiver and now sees her husband as someone ‘with increasingly childish behavior’. “For example, walking alone to sports is possible, but you have to check his bag,” she describes it. “The other day he accidentally took an ugly shirt from me and once he didn’t bring any sneakers.”
Back to the beginning of their relationship, a few months before they got married. A friend of Marcel makes a comment about what Majorie actually thinks about the fact that there is a hereditary disease in the family. Majorie: “Marcel had already hidden it so deeply away at that moment that he actually didn’t think about it anymore. I didn’t know anything yet. It was always the unspoken word.”
“At one point Marcel said he had to tell me something, but he kept crying. He couldn’t talk anymore and we went to sleep, the next day we both went to work. At one point I thought ‘this is too crazy for words, I’m going to call him now, he’s just canceling all his appointments, and I’m doing the same and we’re just going to talk. I don’t care how long it takes.”
‘You can still leave me now’
“At first he said, ‘How would you feel if you later walked alone behind a stroller?’” says Majorie. Eventually the monkey gets out of hand and it turns out that it is a relatively unknown disease, CADASIL, which Marcel himself could also have. At that moment Marcel does not want to know whether that is indeed the case or not. “Marcel said: ‘You can still leave me now, so do that. I actually wish I had never gotten to know you, because then we wouldn’t have had to make that choice and I wouldn’t have hurt you so much,” Majorie remembers well of that conversation. “But hey, if you have real love, it conquers everything, the storybooks sometimes say. Little was known about the disease, neither of us knew what it was exactly.”
To find out more, Majorie does research and calls all kinds of people. It turns out that a professor in Leiden is researching the disease and that she still has blood from Marcel’s mother. So she had participated in the study. Majorie: “At some point we started thinking about children, but I didn’t want to do this to a child. We have this knowledge, his parents did not. I couldn’t live with my children having the disease.”
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CADASIL
CADASIL stands for Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarctions and Leukoencephalopathy. It has an impact on the brain, which can manifest itself in migraines, dementia, Parkinson’s and general difficulty with thinking and motor skills.
It is not entirely clear how often the hereditary disease occurs in the Netherlands. More than 100 families are known to have CADASIL.
Ultimately they take the chance, bearing in mind that becoming pregnant might not work out at all, but Majorie quickly turns out to be pregnant. There is a test available to see whether an unborn child has CADASIL or not. But because Marcel did not want to know whether he had the disease, that was difficult. Ultimately, he gets tested, after which it turns out that he, but also their unborn daughter, have the disease. They decide to terminate the pregnancy.
“It all happened in silence. Marcel never wanted anyone to know about the disease, and I voluntarily went along with that. But that also means that people didn’t know that I had terminated a pregnancy in the first place, let alone why. We were able to transfer her to science, which was very special. This also freed up new subsidies for research.” Ultimately, Majorie and Marcel become parents of two healthy boys, who are now 18 and 14.
Family remains positive
CADASIL manifested itself in Marcel a few years ago, but at first it seemed like a burnout or symptoms of being overworked. “It was during Covid times and he was extremely busy,” Majorie remembers. “We actually thought it was some kind of overworked stress attack. He had difficulty talking and was suddenly very tired. He didn’t really want to know anything about it, because he worked day and night.” When Marcel’s face becomes half paralyzed at some point, they call the doctor. “He said it is a form of CADASIL. We were very down-to-earth about it. It will happen at some point, we thought, so we will notice that.”
I couldn’t live with my children having the disease.
Majorie also mentions that her husband sometimes had convulsions, precursors to epilepsy. In addition, he sometimes has difficulty waking up for days at a time. Now the disease is becoming more and more noticeable. “A few weeks ago he came home and said ‘well, this week everyone is going to bump into me on their bike’,” Majorie chuckles when she talks about it. The family remains positive and makes some things a joke, a way to deal with it. “Then one of my sons says ‘well dad, maybe that has more to do with your coordination’.”
“Mommy, I can’t do it anymore”
One of the most difficult things about CADASIL is that someone is no longer who he or she was. The disease also includes vascular dementia. “Marcel was always very positive. He has become very negative, he has vascular dementia, seizures, Parkinson’s may be lurking. You see an ordinary person, but behind it a lot is happening.” However, this is not always as clear to the outside world as it is to his family. “It was our son’s birthday last weekend, and we celebrated it small, because Marcel doesn’t do it too big. Then he’s talking to someone and he comes up to me and says, ‘Wow, Marc is doing really well.’ And then I think yes, you don’t want to know. I constantly have to explain what’s happening behind the front door. Every day is a struggle.” Majorie does mention that antidepressants helped reduce Marcel’s negative expressions.
But Majorie illustrates with an example that this battle does not only take place behind the front door. “We recently went to a Christmas market and that should be fun and enjoyable, but Marcel only says negative things. He doesn’t want that either. Something suddenly snapped with our youngest, he was standing in the crowd roaring. And that’s a guy I last saw cry when he was 6, when he fell off his bike. He just said repeatedly ‘mommy I can’t do it anymore, I really can’t do it anymore’. That goes through my marrow and bone.”
Sometimes the things Marcel does are not appropriate, says Majorie. She had to learn to see it differently and to have “no vicarious shame.” “But of course you sometimes have that,” she explains. “Marcel has been the financial director of a very large company and has guaranteed millions, and now we have had to say that the banking app and his card have been taken away because he can no longer handle it.”
Own picture
‘Your partner is no longer your partner’
“You notice the disease in everything every day. Marcel was very well-groomed, he wore a suit and tie, had well-groomed hands and nails, went to the hairdresser once every three weeks, that kind of person. For example, now we are eating and I see that he is wearing a sweater that he has been wearing for two weeks. You can see for yourself that you are wearing a dirty sweater, right? But he doesn’t see that. And if you look at his nails, he just has mournful edges. Not only has he become a different person internally, but so has what you look at. So you are already saying goodbye to what was there. Love does not become less, but it is different.”
For example, Majorie mentions that the relationship between her and Marcel is different. “Your partner is no longer your partner, that disappears. You are also losing your father and your sparring partner in raising children.”
‘We don’t know when Marcel will die’
“We make jokes and try to keep it positive, but we don’t know when Marcel will die. I may come downstairs after this phone call and he’ll be dead in the chair.”
Dr. Saskia Lesnik Oberstein about the disease: “CADASIL is a hereditary disease of the small arteries. Over the course of life, a protein accumulates in the wall of the small arteries. This happens in all the small arteries of the body, but only the cerebral arteries are affected. Therefore, the problems in people with CADASIL are limited to the brain. The brain tissue chronically receives too little blood supply.”
I may come downstairs after this phone call and he’ll be dead in the chair.
Majorie: “It turns out that there are many more people who have CADASIL than thought. It often starts with symptoms of burnout or stress. Few general practitioners recognize it, which is why it should become better known. If people know this, they can investigate much more quickly whether it is familial. It can also start with a bad migraine with aura. How many people have that? That does not necessarily mean that everyone has CADASIL, but it could be one of the symptoms.”
Book about CADASIL
The couple has also written a bookprecisely to make CADASIL better known and fellow sufferers to assist. “The book started with farewell letters that Marcel wrote for the boys when he was traveling for work, because we didn’t know when he would die,” says Majorie. “There were so many that we thought ‘we should make a book out of this’. Because I couldn’t share anything of what I was experiencing, even at the age of 25/26, and that it was so drastic, I started writing. We still have all that, so at some point we decided to release that. I don’t wish anyone what I had to experience, that it was so unknown and I didn’t know where to go, I couldn’t talk to anyone.”
“I thought: if there is just one person, and that also helped me in the difficult writing process, who can benefit from it, I think it is enough. We received so many messages from people who indeed said ‘wow, how great, I thought we were on our own, it strengthens me’. There was a lady who emailed us and said ‘I’m not going to read the book, because I think it’s too intense, but it’s on my shelf. When I wake up I see the book, it gives me the strength to get up.’ Many more people need to know the name CADASIL,” Majorie concludes.
Chanice (31) was sexually abused in her youth: ‘I hid it away, but after the accident everything came to the surface’
Raisa (34) started wearing a headscarf: ‘A man attacked me on the street, he kept shouting’
Koen (34) was addicted: ‘At a low point, 7 grams of pure coke per day’
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2023-12-24 07:01:59
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