Month: July 2017

Adrienne left with Thomas and Nils to go to Philippines. The plan is for her to go there with the kids to visit her parents in Manila. After spending a bit more than a week there she will travel back on the 10th alone, leaving Thomas and Nils with her parents. On the 25th she will pick them up again.

In the mean time, I still haven’t been called, so I am still waiting until the VUmc has a bed for me. I hope I won’t have to be home alone for too long.

I called the hematology department at the VUmc this morning, I wanted to know if I could expect a call before next week or that in the weekend anyway nothing was going to happen. They let me know that the first time that I could be called would be on Monday. No guarantees, of course, it could be any day next week.

Yesterday the hematologist called, so I thought the chemo would continue today… Wrong! They called to inform me that it is very well possible that the hematology department will not have a bed available for me this week and that I will only be called-in next week. Unfortunately, they still couldn’t predict when they would have any space, so still, they will call the day in advance to ask me to come in before 11 am the next day. Only now, it can be either this week, but also next week… To quote the eloquent 45th President of the United States: “sad!”

I felt more like spending the energy that I had with the family than on updating this blog, which is why I didn’t write an update since Friday.
During the meeting with the neurologist this Friday we had an extensive look at the MRI scans. All lesions show a significant size reduction. They haven’t disappeared, but they also haven’t stayed the same or grew. These results mean that the treatment so far has been effective and things are progressing as planned. Still, there are more treatments to come, and the next one will be with Ara-C in the VUmc. Ara-C, also called cytarabine is a modern drug, it was first synthesized in 1959, well after the second world war. It is a great drug that will certainly cause immune system suppression and will most likely cause nausea, vomiting, and headache. I will stay in the hospital until my immune system starts to regrow (probably this will take around ten days), when this happens they stimulate this growth even more with a growth factor Filgrastim (Granulocyte-colony stimulating factor -G-CSF-), which will increase the number of hematopoietic stem cells in my blood. This stimulation will be done by injections for two days; these injections will likely cause nausea, fever and bone pain. After these two days, they will start collecting stem cells from my blood. I will be hooked up to a sort of dialysis machine that will extract these stem cells from my blood. This apheresis will take around 5 hours. Afterward, the stem cells are cooled in liquid nitrogen, and I can go home for a couple of weeks. Later these stem cells will be used to regrow my bone marrow after the final course of chemo therapy will destroy it.

You can imagine that I can’t wait for the call of the hematology department at the VU this week. They will call me one whole day in advance to inform me that they are expecting me the next day.

We just came back from the neurologist and the MRI of last Friday shows a big size-reduction of all lesions. Good news!

This week has been about trying to be not too nervous for the MRI results of tomorrow afternoon. The specialists assured me that unless the MRI shows “some sort of explosion,” it would not change the upcoming treatment plans. Now I don’t feel great, but I am quite sure that an explosion did not take place. Regardless, to me, the results of the MRI seem important. I hope that the two months of chemotherapy have led to measurable positive effects.

I find it difficult to keep my mind from worrying about useless “what if’s,” such as: “what if the tumors are bigger,” “what if there are more lesions,” “what if nothing has changed on the MRI?” In either case, there is nothing that I can do, except for continuing with the planned treatment. Worrying about an adverse outcome is pointless. Perhaps it will not happen, and everything is fine, in which case I worried for nothing. Or otherwise, the negative is true, and then that situation has to be dealt with, and being nervous is not an effective way to make the best of this situation either.

Letting go of negative thoughts is, of course, easier said than done.

This emoji describes best how I feel: Quite shitty, but still positive. I believe that I mostly feel MS symptoms, although it is impossible to tell what is causing how I feel. To me experiencing MS symptoms does make sense though, the chemo decimated my immune system. Last Friday the hematologist proudly told me that the blood levels of my lymphocytes are back to normal. For people that were healthy before their cancer, this is good news. When you have an autoimmune disease, unfortunately, the immune system malfunctions and can attack your own body. Hypothetically I can imagine that a strong immune system is what is causing me to experience more MS symptoms than ‘normal,’ and why I experienced them less after the chemo.

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.