At the end of the day on Friday Adrienne and I met with the professor hematopathologist who is responsible for the conclusions of my brain biopsy specimen (they looked at these with a panel of experts).
She made time for us to talk about the outcomes of my brain biopsy specimen. It is not a standard practice that a pathologist explains their findings to a patient. So it was nice that the hematopathologist made time available to talk to us. The professor that we spoke to is experienced in ultra rare T-cell lymphoma. The first thing that she told us was that I was the most exceptional case that she saw in her entire career.

Like I said in my post of two days ago, the biopsy specimens of my brain showed massive amounts of oligoclonal T-cells. Strictly speaking, cancer would by monoclonal, healthy manifestations of T-cells are polyclonal (and do not appear like tumors). What I have is somewhere on this spectrum, leaning towards malignant disease and requiring treatment.

These “oligoclonal T-cell expansions in lymphoproliferative disorders” are ultra rare. They tend to appear more in immunocompromised patients (although they are also super rare in this group). For example AIDS patients, but also patients treated with immunosuppressive drugs, such as transplant patients or sometimes patients with serious autoimmune diseases, can be affected.

You can imagine why they point their finger at Fingolimod, the MS drug that I had been using, as a possible culprit. In cases where an immunosuppressive drug is thought to be the cause, the treatment choices are as follows: 1. Stop the immunosuppressant and see if the disease goes away 2. Reduce the lesion size with steroids and see if the lesions go away afterward 3. In the case of B-cell proliferation treat with a drug against B-cells (rituximab) 4. Use chemotherapy to try and eradicate the disease.
I am now at step four since steps one and two failed, and step three is not applicable because I have a T-cell and not a B-cell problem.

World wide 130.000 patients have been prescribed Gilenya (Fingolimod, the MS treatment), and a case like mine has never been reported. Also with other immunosuppressants, these kind of T-cell proliferations limited to the central nervous system have not been documented. Let’s hope that my unique disease reacts exceptionally well to the treatment.

Adrienne and I had an hour long meeting with the hematologist yesterday. The rest of the treatment is now more or less clear. I drew the schedule below, but as you can see there are still a lot of ± signs. Many durations are estimates, they depend on the speed of recovery, which varies between patients and can’t be predicted. The hematologist made clear that the chemo’s I will receive in the VUmc, make the ones I already had in the Jeroen Bosch hospital feel like a walk in the park. You can imagine that I am not looking forward to those coming weeks in the hospital. The exact date when it will start is not yet known. In the week of the 24th they will call me one day in advance to tell me that treatment will start the next day.

I hadn’t mentioned it before on the blog, but aHSCT (autologous hematopoietic stem cell therapy) can be a very effective treatment for MS. I had been studying this treatment long before this whole cancer story started. The results appear to be remarkable in most cases, but there are some serious possible side effects as well that have to be considered. This treatment is currently not offered in The Netherlands for MS, so as a patient who wants to get this treatment, you would have to go to a foreign country with a lot of money. You can imagine that since I knew aHSCT was going to be part of my therapy plan, my question has been whether the treatment had to be adapted such that it would be most effective for the lymphoma but could also treat MS. We discussed this yesterday with the hematologist. In the case of MS, the treatment is to eradicate the immune system before building it up again with the help of your own stem cells. This establishes a ‘reset’ of the immune system that in most cases causes a stop in the progression of the autoimmune disease. This reset is partly done by using chemo agents that kill lymphocytes specifically, their suppression of these lymphocytes lasts for months, which is no problem in a healthy person, or a person with an autoimmune disease. However, to fight cancer you need your immune system, as it is essential in destroying the cancerous cells. Therefore the specialists at the VUmc hypothesize that long term inhibition of the immune system could have a serious adverse effect in my specific case. Their reasoning sounds logical. Hopefully, the altered schema is still able to induce an “immune system reset” that is beneficial for MS. As you can imagine, there is no scientific evidence that this would or would not be the case. So let’s hope that in the end, this intensive treatment will prove to be super effective for both diseases.

Four times they had to try to stick me with an infusion needle (needed to administer contrast agent during the scan). Luckily the second nurse that tried, after the first nurse had attempted three times, a second one managed to succeed in one try. Results of the scan will be communicated to me on the 20th of July.

Tomorrow are my appointments at the VUmc in Amsterdam. The meeting with the hematologist should bring clarity about the remainder of the treatment. I will start with an MRI scan. Hopefully, it will show a positive effect of the first two months of chemotherapy at the Jeroen Bosch hospital. The results of this scan will only be communicated to me on the 20th. They told me that the outcome of this scan does not alter the remainder of the treatment.
I will also meet the professor of pathology who only recently completed the final assessment of my brain biopsy specimen. She offered to meet me so that she could inform me about their final judgments and explain why they concluded that a primary central nervous system T-cell lymphoma is considered to be the most appropriate diagnosis. Apparently, the biopsy specimen revealed oligoclonal T-cell proliferation. The definition of cancer is that it is monoclonal (a single cell divided, resulting in clones of itself), oligoclonality implies that a few different cells multiplied themselves. Oligoclonal T-cell proliferation is super rare, and occurrence in the brain is undocumented. I am curious what the pathologist will tell Adrienne and me tomorrow. Most importantly they assured me that they believe that the treatment that I am following is the most effective.

I saw the image on the left in a comment on a fellow MS patient’s Facebook page. It reads “Be brave enough to hold on to the hope that life will be beautiful again.” Typically I ignore pictures of quotes on Facebook because I despise them, but this time I felt I had to reply. So what better way to respond than by posting another picture of a quote. My cheesy quote reads “The trick is to enjoy life. Don’t wish away your days waiting for better ones ahead.”

The reason why I had to respond is that I disagree with the aphorism in the first image. The most important thing that I learned from growing up around family members with progressive illnesses is that you should never stop trying to enjoy life now. I understand that days can be sh*t and that you hope that things will be better tomorrow. There are plenty of days in my own life that I wish will be better in future. Nonetheless, waiting for better days does not require bravery. I believe that finding a ray of light on even the darkest days is a real test of one’s spirit.

I feel better, most of the time I no longer feel extremely exhausted and my stomach ache has slowly disappeared. That is great news as it enables me to do more things, meet friends, attend Thomas’ diploma ceremony in school, bike around, etc.

Back in February, I started this blog to keep my friends, colleagues, and family members up to date, without having to tell the same story over and over again. At the same time, writing down what was happening gave me the time to structure my thoughts and to process all the things that were going on. The only downside to continuing this blog is that people can be very interested in my situation, and can keep up to date with every detail without me knowing about it. When someone calls, visits, sends an email, card, or text message, I am instantly reminded that they care. Unfortunately, I can’t tell if someone obsessively reads my blog. I enabled ‘comments’ on my posts so that people could leave a note, but I understand that this is somewhat cumbersome (I enjoy reading them though).

On Sunday I decided to post my story, with a link to my blog, on Facebook. I had been thinking about sharing my story on Facebook since I started my blog, but I hesitated. Facebook normally is a place to share happy events. Pictures of babies, travels, food, kittens, and misplaced quotes usually fill my timeline.
Facebook tells me that I have 288 ‘friends,’ and among these are people that I have never even met in real life. I could not imagine that these ‘friends’ were interested in reading about my unfortunate situation. There are no pictures of adorable cats on my blog, and my ‘food picture’ is not nearly as appetizing as the photos that usually fill my timeline of fancy dishes served at exotic restaurants.

Regardless, the replies I received to my post were heart warming. Thanks to everyone that took a moment out of their day to read my story and leave me a message.

The second course is over, I am home again. If everything continues to go fine, this was my last stay in the Jeroen Bosch hospital. The first half of the treatment is done. The harsher part in Amsterdam at the VUmc is next. I still don’t have any details of this treatment. I don’t even know when it will start. I will have a meeting with the hematologist there on the 14th after my MRI scan and hopefully, I will know more then. Not knowing when I will be in the hospital is also difficult for planning the kid’s vacation. They will stay in Manila with their grandparents for a few weeks, Adrienne will bring them, but we just have to guess what would be the best dates for this trip. For now, I still have some pain in my stomach, but it is not too bad.

Thomas came over for a long visit. Adrienne brought him and he stayed the entire afternoon. We folded a paper snake for Nils, watched a bit of Netflix, played Mario Kart on the Nintendo Switch and we went to the hairdresser in the hospital. Unfortunately, I wasn’t feeling too great today. I have a bit nauseous feeling in my stomach, perhaps caused by the antibiotics I’m getting as a prophylaxis, perhaps caused by the chemo. Nonetheless, we had fun.

The last course of methotrexate is running. This is the only chemo agent that remains of course two. Hopefully, the MRI of the 14th will show a positive effect of these two courses. If so then this is my last stay at the Jeroen Bosch hospital for the treatment and then the rest will be done at the VU. I will have to stay here until Friday or Saturday, depending on my blood level of methotrexate. As long as this is above a certain level they have to continue with hyperhydration and the administration of folic acid, a sort of antidote.