After a nerve wrecking day, they called me at 17.30h, and they informed me that the histology was inconclusive as of yet, the pathologist wasn’t sure what he was looking at and they had to wait for more stains to be completed. Today, no conclusion can be made. I don’t know what that means. I can only speculate that it is very uncommon. They will have a new multidisciplinary meeting on Wednesday, and they expect to have a final treatment plan on Thursday. The final call is now planned for Thursday around 17h…

Nils and Thomas in the mouth of a T-Rex.

I am feeling worse than last week before the biopsy, but not extremely. I don’t feel as dizzy anymore and I also do not have throbbing headaches. My balance is again a bit worse, and I am extremely tired. These symptoms are to be as expected. They of course destroyed some brain tissue with the biopsy, and the whole situation is not favoring good health. Nonetheless, I am trying to do fun things, like visiting dinosaurs with the kids in Boxtel, or eating with my parents and sister.

I went ahead with the biopsy. My bed was wheeled into the icy cold OR, I saw the operating table, their navigation system, everything was in place. The last thing I remember is the stinging feeling of the anesthetic in my arm. Afterward, I woke up, shivering from the cold, but feeling ok. Adrienne was sitting next to my bed and the nurses put some warm towels and something that felt like a huge hairdryer under my sheets to warm me up. The neurosurgeon came over to tell me that everything went fine. I had to stay in the PACU (post-anesthetic care unit) the whole night. They monitored my EKG and oxygen saturation continuously. They also did some physical tests every hour, to see if my pupils still contracted when they shone a light in my eyes and whether I could still move my arms and legs. I hardly slept.

On Tuesday morning, the day after the biopsy, I felt ok. I was tired but I also didn’t sleep in the night. I didn’t have any pain. After speaking to the neurologist and the neurosurgeon, I was sent home. They had to wait for the pathologist to stain the biopsy samples and to analyze them. Then in a multidisciplinary meeting on Monday the 6th of March, they would discuss the outcome and call me, probably for an appointment to discuss treatment options. I could wait for this at home.

Coming back on Sunday evening was hard. I felt fine and I didn’t want to come back to the depressing hospital ward and be emerged again in the nightmare. But I came back, we discussed our doubts but were urged to do the biopsy. I was feeling fine; they had ample time to plan the procedure and nothing needed to be rushed, this was the best, least risky, situation. In the morning before the surgery, I would still speak to my neurologist.

During her visit in the morning, she told me that the lesions resembled lymphoma. The VUmc’s most experienced neuroradiologist had no doubts. The lesions were in such a dangerous location that a small increase in their size could lead to acute problems. The bigger one in the cerebellum could easily cut off the liquor (CSF) circulation, causing hydrocephalus (a waterhead) and other serious problems. They also saw a smaller lesion that is close to the basal ganglia (a selection of important structures in the brain), so they all strongly recommended me to go for the biopsy.

In acute situations, you can reduce the swelling of a tumor or an MS lesion by giving steroids. However, giving steroids could render a biopsy useless. Therefore it was necessary to take the biopsy before they could start with any treatment (note that steroids reduce swelling, but do not treat MS or tumors).

I was sent home for the weekend. The biopsy was planned for the coming Monday (27th of February). Since I was feeling fine, and nothing medical was happening during the weekend, I could leave and come back Sunday evening.

Over the weekend I had some more time to think and discuss the situation. I started to doubt about the biopsy. Maybe waiting, a follow-up scan, and perhaps a lumbar puncture, would be a less risky approach. I was feeling fine, and what are the chances for this situation. It felt like an unlikely presentation of MS was still more likely than a very rare type of cancer.

Sagittal slice containing the largest lesion that they want to biopsy.

I finally mustered the courage to look at the MRIs, before I didn’t want to, I am no radiologist, and I couldn’t think of anything positive coming from this. To discuss the biopsy I wanted to see them, to understand where they would access it and plan their biopsy. I had been working on stereotactic neurosurgery in the Maastricht University Medical Center before I came to Nijmegen in 2012, so I had a good understanding of the procedure (at least about the techniques behind it).

The MRIs indeed showed a big (3.5cm) lesion in the cerebellum. This lesion showed no necrosis in the center (necrosis is a bad sign) and had a well-defined border (ill-defined margins are extra bad). The location of the lesion wasn’t difficult to access with the biopsy needle (a 3.5mm diameter needle), but the location in the cerebellum, made it that risks such as a bleeding or brain swelling could quickly escalate to a coma or death. Nonetheless, the risks were low, estimated to be about 1% for these adverse events.

After this talk me and Adrienne spoke to the neurologist and the hematologist. According to the young hematologist, the cancer type they suspected (primary central nervous system lymphoma) is incredibly rare. The fact that my symptoms were getting less baffled her, she told us that this normally does not happen. Also, the neurologist seemed to be surprised with the state I was in and even mentioned that MS, however unlikely, was not off the table.

A lot of exams were to follow. To characterize a tumor, they would need to look at its cells, obtained with a biopsy. A brain biopsy is a risky undertaking, and when it can be avoided that has the preference. Therefore they planned following exams to look whether the tumors were also to be found in other, more accessible places. Since they strongly suspect lymphoma, an eye exam was planned (apparently lymphoma can also occur in the retina) and a PET-CT to look for tumors anywhere else in the body. None of these exams showed any other places of activity. I didn’t know whether that was good or bad news. A talk with the neurosurgeon was planned on Friday, to discuss the subsequent brain biopsy.

I was feeling a lot better by the end of the week. I no longer had any serious symptoms anymore, they didn’t have to wake me up in the night, and even my balance and speech were back to how they were half a year ago.

A slice of the contrast T1, containing the mass like enhancing lesions in the cerebellum.

On Monday the 20th of February 2017 a research MRI scan was made of my brain. I still felt a bit sickish but was feeling much better, and I could do the tests, and I was fine in the MRI scanner with my nose pointed to the ceiling. This position made me very dizzy on the days that I was feeling sick. I expected to see new MS lesions on the scans, probably aggravated by the flu, therefore I also asked whether a radiologist could look at the images, and my neurologist would call me back on Wednesday to discuss the new scans.

Not on Wednesday, but on Tuesday morning my neurologist called. She told me that they wanted to make new MRI scans with a clinical protocol as soon as possible. They found something in my cerebellum that appeared ‘space occupying.’ MS lesions, in general, do not occupy any space, they are inflammations. Tumors do, they are new cells… A stressful time began. I arranged daycare for Nils and Thomas (Tuesday is my day with the kids), called Adrienne (my wife) and took the train to VUmc.

I arrived at in the hospital around a 12.30h; I was ‘checked-in’ in section 2B, the neurology ward. My MRI had to be moved in between the regular MRI-scans that were planned for the day. The scans were made around 15h. The rest of the afternoon was waiting for the results of the scans. I assumed that it was just MS related and that the research scans from yesterday were inconclusive since they were made without a contrast agent and with a research protocol.

The amazing dinner I was eating when the neurologists came with the news.

During the evening hospital dinner, two neurologists came in, they notified a nurse to come along, told us to cover up the dinner and follow them to a quiet room. Adrienne and I were floored about what the neurologist told us. The other neurologist seemed to be close to crying and the nurse was silent and sad. He told us that there were multiple lesions visible in my brain that appeared to be tumors. With the biggest one in the cerebellum. According to the neurologist, this lesion was centimeters big. They needed to find out what it was exactly, what kind of tumors these were, (they were thinking about lymphoma, but without any further examinations they didn’t want to speculate). Just as us the neurologists were baffled. The chance for a brain tumor is incredibly small, especially at my age, and the medication I am using is not known to increase these odds.

I joined a research project at the VUmc (the medical center in Amsterdam that is specialized in MS) in which they want to study whether Gilenya reduces cognitive decline. For this study next to some cognitive tests, an MRI scan is made every six months. My first scan was made on the 28th of July 2016. (Note that Gilenya is a non-experimental, FDA approved drug, that has been on the market since 2010)

My second research exams were scheduled for the 30th of January, but I had to reschedule because I was (like so many other people) sick with what I presumed was the flu. The scan was rescheduled for the 14th of February, but I again had to cancel, because I again (not still) was feeling awful. A small movement of my head would make me very dizzy, and I had a lot of headache over the weekend. This mostly went away again, and finally, on the 20th of February, I traveled to the VUmc for the exams.

Me, a few months ago.

This is going to be a rather long story about my health and the current events.

My first MRI, showing a lesion in the brain stem (the white spot just off the center)

I expect that not everybody knows about my history with multiple sclerosis, so first of all, I will tell a bit about that.
In October of 2013, I had, what I found out later, were called ‘paroxysmal’ symptoms. Every few minutes, for about ten seconds, the right side of my face would feel icy cold as if I was pressing it to a cold window, at the same time, I had problems to coordinate my left arm and left leg. When I visited the GP, she referred me to the hospital.
Later an MRI was made, and a small white spot was visible in my brainstem. I remember that time of uncertainty. With me wondering “what is this spot,” “I hope it is not cancer,” “it is probably multiple sclerosis (MS).” MS is a disease that my father has had since I was almost two years old, and my sister has had for more than ten years.
The diagnosis was not easy to make; a lumbar puncture was made, but it showed nothing. The only thing to do was wait, a new MRI scan was made, and in August 2014 the diagnosis MS was finally made. The MRI showed new lesions in different places. From then on symptoms did get worse slowly. Balance got worse, speaking wasn’t as fluently anymore, my cognition became less, and I was more tired. Progression was steady, but not very fast.

Signing the UWV documents that state that I can’t do my work anymore.

There were periods when things were better, and periods when things were worse, but most of the time I was noticing a slow but steady decline. I tried different types of DMTs (disease modifying treatments), there is no cure, but there are medicines on the market that slow down the progression in some patients. They didn’t seem to work. After a long trajectory with the company doctor and the UWV (Employee Insurance Agency), I was found to be unable to work, since then I significantly reduced the hours I worked at the Radboudumc.

New scans were made, and in July 2016 I switched to Gilenya, what they call, a second line treatment. These medications are often more effective but somewhat riskier. Gilenya, a daily pill, was still relatively ‘mild’ with risks ranging from early heart problems (I had to be monitored in the hospital for a day when I started) and then some increased risks for benign skin cancer, and a minuscule risk for malignancies (between 1/1000 and 1/10000 patients). Since I didn’t expect to be on this medication for long, I didn’t ponder on these possible risks for too long.