10/13/18 BMT Day +163 CMV and Foscarnate

Reese continues to struggle with nausea and a gut that just isn’t healing as quickly as her doctors would expect. She is still on .9 steroids, which is not a small amount. It is for these reasons that we have agreed to a scope on Monday. We have avoided this procedure for months, but now the doctors have determined that the information is worth the risk. In the past, it was decided that a scope would not guide treatment (nothing about her plan would change with the information they would gather.) Now it has been decided that they need the scope to form a plan to move forward. One of three things will happen. They could go in and not find any active gvhd, which will mean they can wean her steroids faster. This is what we want for reasons I will detail below. The second scenario is that they will find something “new”… a reason for her current problems. Then we can treat this new problem appropriately. The third situation is one that WILL NOT happen. They could find active gvhd. This would be devastating and not what anyone is expecting. As you know, long term steroid use is NOT ideal. Well, there is now a problem that is making us more anxious to get Reese off of steroids. Her CMV has continued to be active and she can not shake it without functional t-cells. She has been on Foscarnate to battle the CMV for months and the Foscarnate is hurting her kidneys. So a couple weeks ago, we switched her to a new CMV med, Ganciclovir. There is a catch to every medicine, and Ganciclovir is a count killer- it is very hard on the marrow. Marrow that is new to Reese. It was determined that relieving her kidneys was more important (the lesser of two evils, when there is no good option.) Well, it didn’t take long for the Ganciclovir to tank Reese’s hemoglobin and platelets.. she had transfusions of both the past two days. So, now we need to decide if we hurt her kidneys or her marrow. Neither is ok. What do we do about it? We get Reese OFF the steroids, so her own body can put the CMV to rest! I have come full circle, you can see why we need the scope. If Reese’s gvhd is PROVEN to be not active, we can peel the steroids off faster, and her own body can do it’s job.

Reese’s blood pressure did stabilize and she is now back on BP meds that are keeping it right where we want it. That was a crazy couple of days last week. She continues to be diabetic, and we also anticipate this will go away as the steroids drop. We had a scare this week when Reese started complaining about neck pain. Another side effect of high dose steroid use can be stress fractures… Reese is fragile right now. After lots of consults and some imaging it was (for now) determined that it is muscular, thank god. You may remember Reese lost the ability to hold her head up a few months ago. Even though she has regained strength, her neck took a hit. Reese will start working with an occupational therapist this week.

Reese and Quinn began art therapy this week. They meet with their new “teachers” every Tuesday morning and they LOVE it! I love to watch them learning and thriving. They also continue to work with Matthew, our band leader (music therapist) every week. You probably saw some of their greatest hits on my YouTube a couple months back. Both Reese and Quinn love to make up songs… it is a BIG creative outlet for them. So song writing with a musician is right up their alley.

I posted yesterday about Reese’s mutations being ZERO and her 100% chimerism (all donor!!) It has been brought to my attention that not everyone understands what this means. Sometimes I forget that I am confusing. I’ll try to explain it a little better now (disclaimer- I am not a doctor, but as always, this is how I understand it, myself!) JMML is a devastating disease, the worst of the childhood leukemias. There is less research, it being very rare, affecting 1 out of 1.2 million kids. The relapse rate is 50% and a second mutation, like Reese’s, makes it higher. As you know, it is for these reasons we traveled to the doctors who are specifically studying JMML. They happened to be in San Francisco. Reese has had 100% chimerism since May. This means that when you look closely at Reese’s bone marrow, you will see all donor cells, a complete hematopoietic replacement, no Reese cells. Mutation status is a little different. Let me try to explain why we are SO EXCITED about these results. Devastatingly, children can have 100% chimerism and still relapse at a later time. If the mutated gene is still there, in a very small amount, hidden, something can set it off again and it starts cloning. So, new technology that is VERY precise can now look at marrow for a specific mutation. This JMML exon panel was created here and has been available since January and it can spot the slightest bit of a gene mutation. After transplant in May, Reese was 100% donor. But, when the UCSF team looked more closely, they saw a teensy mutation left (<1%.) This is devastating, this is what causes relapse. But we were never devastated. Because, before the results came back (4 weeks) her gvhd had hit. This is when we thank God for gvhd. It was severe gvhd and the doctors assured us that the graft vs leukemia effect would knock out those last mutated cells. So we waited, held our breath… the silver lining of gvhd on our side, and it was true. Twice. She has had TWO clear mutation screens, and she is also 100% donor. This is what it is all about, this is why we are SO THANKFUL on this gorgeous San Francisco day!!!!!!! We thank God for guiding and protecting us, we thank God for Reese, we thank God for our doctors, we thank God for our nurses, we thank God for our family, we thank God for our village, we are so blessed and I will never stop being thankful!

Leave a Reply

Your email address will not be published. Required fields are marked *