Dagbladet has previously written about a “new” and deadly disease – the vexas syndrome (from vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) – which has previously only been registered in the USA.
After the disease was made known in a research article in New England Journal in October last year, it is registered five cases at Oslo University Hospital. Dagbladet is not aware of the total number in Norway.
The first registered case at Norway’s largest hospital was a man who had been ill since 2012. The man died at the age of 76 in 2020 – for the doctors found out what was wrong with him.
Dagbladet has spoken to the man’s wife, who wishes to remain anonymous.
– It was special to get a diagnosis post mortem, but it was nice to know that. The doctors were never conclusive in the diagnoses, and they did everything they could to find out what was wrong with him, she says.
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More symptoms
Now doctors have found that vexas syndrome is a sudden change in the x chromosome, and that it mainly affects men. No one knows at the moment why the change occurs.
The symptoms are fever, decreased general condition, inflammation of the ear and nasal cartilage, rash, changes in the lungs, blood clots, low blood platelets and often low blood percentage.
SEVERAL SYMPTOMS: Symptoms include rash, inflammation of the ear and nasal cartilage, changes in the lungs, blood clots, low blood platelets, low blood percentage and fever. Photo: New England Journal
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The wife says that the first symptoms were vague.
– After several examinations, they found that he had a blood drop of 80, which means that you are ill, she explains.
Immersion is a blood test that is especially widely used for the detection and investigation of chronic inflammatory conditions, according to Lommelegen.
– When he was admitted to Bærum hospital for the first time, the doctors thought it could be the heart, since he had a chest pain. Then a doctor suspected something else and he was transferred to Rikshospitalet, she says.
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– Went very fast
The man was hospitalized several times in the time before he died. He was put on several medications, which gave him many side effects.
She says that he went on a high dose of Prednisolone for many years, which is a drug that can cause strong side effects. Among other things, it can weaken the immune system so that you are exposed to other infections.
– In the end, his skin was as thin as paper, which made it difficult to put veneflon. For the past year, he was on medical day care at Bærum Hospital with a blood transfusion every week.
– There were many attempts, testing, medication, and bone marrow biopsy. They tried everything and had different theories. I have nothing to complain about in the health care system and what they tried to treat him.
She says that he was very ill with sepsis (blood poisoning). In the end, the pandemic also hit, so she was not allowed to visit him in the hospital. Eventually, he died of a complication of otitis media.
– It went very fast, she says.
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– Years of uncertainty
It was chief physician Øyvind Midtvedt at the Rheumatology Section at Oslo University Hospital who followed the man during the years he was ill and who became suspicious when he read the research article last year. The disease picture of the man was consistent with what was described in the article.
Genetic analyzes were then performed afterwards, with the wife’s permission, and they were able to establish that it was vexas syndrome.
– It was a strange case of illness. When we read about it, we understood that it was what he had to have. For the doctors who treated the patient for many years, and not least for the relatives, it was a relief that one could finally make the correct diagnosis, chief physician Midtvedt has previously stated to Dagbladet.
There is no treatment for the disease today.
– He did not get to get an answer. But if you had received that diagnosis, you would also have been told that the prognosis was very poor. That there is no treatment. So if it’s better to live in hope, I do not know.
– He was ill for eight years. To begin with, one had hope, and that this would pass by itself. But then he was constantly weakened by the medication. There were many years of uncertainty. He quickly became very ill.
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– What were you thinking when you finally got the answer to what went wrong with him?
– He was a curious person and he would find this interesting. I thought so. But then there was not much to do with it. I think it’s nice that they had the genetic material and got to test this.
– Keeped the mood up
She says that her husband was a physically fit and active man. He loved skiing.
– That was the saddest thing for him. That he could not bear to ski, she says.
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She describes him as curious and upbeat. He was sporty and active in the local community. On Friday, they were supposed to have celebrated a golden wedding. But that was not the case.
– Unfortunately we did not get to experience it.
– What do you think now in retrospect?
– If you had known this, you might not have needed all the bone marrow biopsies, which are painful, and you would have released some medication. It has been very demanding at times.
– But he kept his spirits up, she says.
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