An inpatient again

When I woke up around 4 am in the night, I noticed some headache. In the morning and during the day this headache didn’t go away, and it got worse when I bent over. A bit worrisome as I also had this kind of a headache before, when the lesions were large and obstructed the flow of CSF (cerebrospinal fluid) in the 4th ventricle. Only now it was by far nowhere close to
When my neurologist called to inform about further diagnosis and a hospital admission on Wednesday, I told her about my mild headache. She told me to come to the first aid of the VUmc, where they would make a CT scan to see if the lesions had grown and the situation could become acute fast.
After being stuck in traffic, I and Adrienne finally made it to the VUmc in Amsterdam after an almost two-hour long drive (we live in ‘s-Hertogenbosch). We arrived at the first aid at 18.30; only at 22.00h, the CT scan was made. Clearly, I wasn’t really urgent.
Luckily the CT scan showed no growth since the MRI of last Friday. Now I am staying overnight at the neurology department, tomorrow the exams continue.

What the neurologist told me they would do: 1.) A lumbar puncture, in the CSF they might see what the lesions are specifically. 2.) A new eye exam, perhaps there would be a lymphoma visible there now. 3.) A talk with the hematologist.
And 4.) what scared me, she told me that in case these exams are inconclusive, they would consider a new brain biopsy. I really can’t understand what they think they can learn from a new biopsy that justifies the risk of this operation. The previous biopsy was not taken wrongly, the samples were very abnormal, but they were not bad samples. A brain biopsy is an invasive procedure with serious risks. Also, it would mean that treatment is further postponed while waiting for the pathology results. I also don’t know what they think they can learn from such a new biopsy that could radically change the treatment. Or whether a treatment that is more radical and perhaps more risky would outweigh the risks of a biopsy. I have a lot of questions for the hematologist tomorrow.

I am writing this after just having had a meeting with my neurologist about the MRI scans of last Friday (7-4-2017). The MRI shows that the lesions have grown and are occupying more mass (30×25 mm for the biggest lesion vs 20×20 mm) than on the MRI of a bit less than three weeks ago (20-3-2017). This was very unexpected as I just stopped with the corticosteroids. Now the hematologist will call me for an appointment this week. A lumbar puncture will follow as soon as possible. Treatment, probably chemo, will likely start soon.

Up to now, this website contains mostly factual information, updates about exams and doctor’s visits. Emotions or feelings have not been part of it so far, but today I wanted to write something that doesn’t have anything to do with the exams, with future treatments, or with the medical facts. I would like to take a moment to write about all the support I have gotten, and what that means to me.

It has been, and continues to be, a long and uncertain journey. It gives me a lot of positive energy to know that so many people care about what is going on with me. I have gotten numerous emails, messages, cards, flowers and even balloons. At the same time, medical specialists from around the world are keeping an eye on my case, take their time to answer questions and provide me with feedback. There is no doubt in my mind that the best professionals are working on my complicated case at the VUmc, but it never hurts to get as many opinions as possible.

I am trying to view these new, and old, developments with an open mind. From many friends, family members, or fellow patients I have often heard: “why does this happen,” “it is unfair,” “I will never accept my limitations,” “why me,” “you have to fight,” “it is a battle that you will win,”, and “it can always be worse.” All these words are meant in the best ways possible, but my feeling is different. I don’t think that there is such a thing as a “fair” and an “unfair” amount of suffering. If there is, me having all the support, an excellent medical care team and a great health insurance, contrast sharply with a baby born with AIDS to a sick mother in a hut in a famished village in Afrika. At the same time, I don’t think it is helpful to minify your own grievances by telling yourself “things can always be worse”. I believe that whatever occupies your mind, whether it is something seemingly insignificant like a tooth ache, an ingrown toe nail, or a presentation at work; these things are important.

Acceptance to me isn’t sitting back and doing nothing. It’s having the clarity of mind to know when to act and when to focus on other things. To me, spending time with family and friends is more important than wasting it on negative thoughts about unfairness, non-acceptance, and future images of what may or may not happen.

Very often I read and hear about how patients are “fighting” and “won” their battle with a disease. It is not that I think people should “give up,” and “throw in the towel.” I believe that “surrendering to” and “accepting” the circumstances can help make the best of the current situation. Of course, this isn’t easy and it helps me enormously to see how many people care and show their support. Thank you!

The hematology department at the VUmc.

Today I met with the hematologist. In an hour-long meeting, we discussed the treatment options. I summarized the follow-up process in the flowchart below. What he told me was that the biopsy samples were very difficult to interpret and that the experts had never encountered anything this before. Even though the biopsy samples showed no clear “monoclonality” this could not be excluded (cancer cells are clones of a single cell, therefore monoclonality means cancerous). He couldn’t rule out, that after stopping with the Dexamethasone, the lesions would grow again. Of course, the hope is that the lesions continue to shrink, without the help of steroids. This could be a sign that a healthy immune system (which should turn back to ‘normal’ after having stopped the fingolimod -MS medication-, is able to clean up the access of lymphoid cells. Treatment will depend on what will happen in the coming month(s).

The new MRI scan of last Monday looks as expected. The lesions did shrink and the fourth ventricle is no longer as compressed and displaced as in February. What the cause of this shrinking is is not clear, it is likely that the Dexamethasone has caused the shrinking. I am now reducing this medication (I took 8 mg up to now, from tomorrow onward it will be 4 mg and 2 mg for one week after that). In about two weeks a new MRI will be scheduled, and if this shows again that the lesion is shrinking, another one will be scheduled after that. The reason is that they want to see whether the lesion shrinks by itself after I stopped with my MS medication, and with the absence of Dexamethasone. This Friday I have a meeting with the hematologist to discuss treatment options.

An MRI slice with contrast of 21st Feb (left) and 20th of March (right)

The poster at the VU ironically says in the right bottom: “I care about quickly knowing what we are facing.”

Today (Monday) a new MRI scan was made. It all went fine. The results of this scan will be discussed on Wednesday. I don’t know what to expect. I can only imagine that the lesions have become smaller. I feel better, no more headaches or dizziness and my speech is less dysarthric. Only I sleep badly due to the dexamethasone. I wake up often in the night, and around 4 am I don’t feel like sleeping anymore. During the day I am incredibly tired. This, of course, makes all my ‘regular’ MS symptoms worse.

Nils at the gym, a good distraction.

Of course, I was nervous today. I was up extra early, and we even went to the gym with Nils just like every Tuesday. Straight afterward we took a train to the hospital in Amsterdam. Yesterday, at the end of the day, my case was again discussed in the multidisciplinary council at the VUmc. Together with the hematologists, pathologists, neurologists, neurosurgeons and oncologists, they discussed the situation. Today at noon I and Adrienne were invited for a meeting with my neurologist to talk about their conclusions.

When exiting the tram, the first thing that caught my eye was the ominous “cancer center”.

The title of this paragraph was the first thing she said “it doesn’t appear to be as bad as we assumed”. So what does that mean? The whole situation is very complex and very atypical, that surely is the catch phrase. The pathology report reads: “Het geheel is uiterst lastig te interpreteren” which means “it is very difficult to interpret [the biopsy samples]. What they see in the biopsy is an abnormal amount of T cells. But these T cells do not resemble cancerous cells; they do not appear to all originate from the same cell that divides uncontrollably (cancer). It is puzzling why they are there. The hypothesis is now that it is a B cell problem that triggered all the T cells to be there. T cell problems are even rarer than B cell problems. Perhaps my MS medication might have triggered this somehow, as it is an immunosuppressive agent, but this has never been described before. She told us that these cells could be precursors of a lymphoma, so they need to be monitored very closely (additional MRIs).

An idea would be to treat with Rituximab, a monoclonal antibody that targets B cells. This also treats MS, which is a good thing. But since they can’t rule out a T cell problem, messing with the B cells might give rise to unexpected adverse events. That is why she told us that she first wants to discuss the options with the hematologist. They will schedule a new MRIs as soon as possible (this week). In this MRI they want to see what the lesions look like now, perhaps stopping Gilenya would have had a positive effect, although it is probably impossible to judge, the Dexamethasone has probably shrunk the lesions anyway. They also want to check whether the biopsy was taken in the right place, this should still be visible on the MRI (in the worst, but very unlikely, case they missed the mass and sampled only the boundary). Then if all looks ok, they will stop with the Dexamethasone and do another scan 2-3 weeks later. Then if all seems well, a treatment could be started, if the hematologist agrees this might be Rituximab.

All in all, it is the best news I could receive in this situation. Of course, things are not at all miraculously solved, but given the entire situation, knowing that it is not a definite lymphoma, or even something worse, is good news for now.

Playing in the sand dunes of the “Drunense Duinen”.

This morning I was called by the secretary of the neurology policlinic. Tomorrow at noon me and Adrienne have an appointment with my neurologist and the hematologist. During this meeting they will tell me the findings of the biopsy and the treatment plan. Of course, it is good knowing what is wrong and creating a plan to start treating it. But at the same time, it will also be the end of not knowing, of trying to ignore what is going on. Naturally, I have been wondering what made the pathological assessment of the biopsy samples so difficult. I do not know a lot about pathology, and certainly not about brain tumor pathology (through my work I know a bit about common breast and prostate cancer histology), so I don’t know what the difficulties to assess the samples could have been. I am happy that they have had a very thorough look together with their world-renowned MS pathologist involved, at the same time, I worry that something that is aparently that abnormal could turn out challenging to treat (hopefully the opposite turns out to be true).

Logically, the long wait has been a nervous one at times. Luckily apart from being tired and sleeping poorly (this could be a side effect of the dexamethasone -the steroids I am taking- probably amplified by the stressful situation), I am feeling relatively ok. Both my speech and coordination are worse than they were, but it is impossible to pinpoint the cause. It could very well be due to the mass in my cerebellum, but it could also be a result of the biopsy, my MS (speech and coordination problems are not new to me) or the lack of sleep. At this stage, I don’t know; it is most likely a combination of all of the above.
I filled my days with positive things, with the family. Hard times are surely coming, so I am trying to enjoy the good times as much as possible.

After another nerve wrecking day and night, my neurologist called me at 14.30h, and she informed me that the histology was still inconclusive. The main assumption is primary central nervous system lymphoma, but I got the feeling that the final conclusions about the specific type are still not yet known, and these are important for the treatment plan. The pathologists involved (my neurologist assured me that the best pathologists are working on it) have to wait for more stains again. It is without a doubt very atypical, but essential to correctly classify. The suggested follow-up treatment (chemo, radiation, etc.) is dependent on the tumor subtypes as far as I understand. The generic treatment I am using now (dexamethasone 8mg), is suitable for all types of lymphoma and does not need to be changed.

So again no definite diagnosis, and no treatment plan. My neurologist expects this to be ready on Monday or Tuesday. She will call me tomorrow, and make an appointment in the hospital, with her and a hematologist. She would rather meet me and Adrienne in person than have a phone call about this, I agree… More waiting to come….