Fungus Amongus

Dear Family and Friends,

The past 3 days have been so extremely stressful, please bear with me as I try to get you all updated on the current situation.

Molly has been having fevers for the past week or so (could be longer, but I'm losing track of the days exactly). Obviously this is extremely concerning because she has no immune system (specifically neutrophils) to fight infections. They have been doing virus panel after virus panel and everything keeps coming back negative. They have been doing fairly regular bedside x-rays to check her lungs, which on 2/4 looked nice and clear on the x-ray. Well on 2/6 everything changed - the x-ray came back and looked really bad- cloudy, some spots, fluid, etc - from an initial reading, they diagnosed it as PJP (pneumonia). Due to the fact that it came back so badly they FINALLY ordered another CT scan. Note here, Molly, myself and Aaron had asked for another CT scan about 10 days ago but every time they said no with reasons why of course.

So based on the x-ray the immediately put her on IV Bactrim which is the go to drug for PJP. She had only been on a prophylactic dose (meaning a dose to help prevent) of Bactrim on weekends. Anyway, they ordered the CT and we got the results of that back and they are really bad. So they have found that on her left lung, specifically there is fungus growing on her lung - they can see this as white spot(s) on her lung and there is even one growing where she has been complaining about her upper back shoulder area bothering her. 

So in the middle of these x-ray and CT scan results they decided that they wanted to do another bronchoscopy. Again, the major issue is here that Molly is not holding platelets at all - so when they give her 2 units of platelets and she is only getting a very small bump from them. She has literally had a slow running bloody nose for weeks. Obviously a procedure where they stick a tube in your airway can lead to bleeding so we were very hesitant but the issue is that they really do need to try to identify the organism, so we agreed and signed the paperwork.

The timing of all if this has been so intense - so now that we finally said okay to the broncho they changed their minds 2 hours before she was supposed to go in. The reason why is after looking at the CT scans more they decided that there was no good way to get a sample of the organism that is growing on the outside of her lung from the inside. That when they did this procedure before, they never got any result from it, so they decided not to do it....but they want to do a biopsy of the spot on the lung instead!

Ugh, so after a day of lamenting over this situation, they explained the process of the biopsy to us which involves the lung collapsing and her having to have a tube placed in her lung and would be in the ICU for at least 1-2 weeks. This pretty much freaked us out because she can't fight off any infection and then we put tubes in her, needles, and collapse a diseased lung - not something we are very hip with. We do get the point of it though - that without identifying the organism, they cannot target the treatment.

So, in the meantime of this decision process, they put her on 2 more anti-fungals (and removed the batrim because at this point they know that it is not PJP). So now she is on 3 anti-fungal medications all extremely bad for the kidneys and liver.

Alright, so in the process of doing the CT scan - they went ahead and did the full chest and pelvis CT and they also found that her kidneys and spleen are enlarged!! BAD NEWS for sure. Given Molly's situation - they told us that this can be either the fungus has spread to these organs or.... she may have leukemia in these organs. With this news, I/we am having a difficult time keeping it all together. 

So the story continues.... with the news of the proposed biopsy and now organ involvement and possibly leukemia as well if things weren't serious enough before well they sure are now (this was last night).  So we talked to a handful of our close family and friends and we all concurred that if they already have her on the anti-fungals that she would be on regardless of the findings of the biopsy that we do not want her going through the biopsy procedure due to her condition and the seriousness of it. So, last night as we are finally getting ready to fall asleep my phone dings - and it is a UofM Health Test Result notification - I open it up and a PCR (blood test) result has come back - the fungus has been identified - Aspergillus AG! So on one hand this is super bad because this means the fungus is in her blood stream and on the other hand it is super great because an organism has been identified!

So now we have made it to today! WHEW! Aaron and I have decided that there is no way in h-e-double hockey sticks that we are going to let her undergo a biopsy right now. We have her on the drugs, they would not change the drugs anyway and now we have an organism identified. I effectively communicated this with the doctors today (leaving all drama out here) and they eventually all agreed that there was not a compelling reason to put her through the biopsy. YAY - BIG WIN here.

So now, what do we do with Molly that she has a systemic fungal infection that can only be gotten rid of with 2 things - 1, the anti-fungal medication and 2, neutrophils. She will (statistically) not be able to survive the fungal infection without neutrophils and she definitely cannot get a transplant with it.  So as of this afternoon the plan is to have her undergo a bone marrow biopsy tomorrow and possible lumbar puncture to test her spinal fluid to see if there is leukemia there. The issue is that her platelets have to be at 50 in order to the the LP, so we may or may not be able to get her there because she is having a resistance to the platelets. But they can for sure do the bone marrow and this will tell us the health of her marrow. If her marrow is healthy enough, they will immediately give her the baby cord. It is on deck, waiting to be shipped. This will assist her marrow in recovery and allow her to produce her own neutrophils. In addition, they have ordered granulocytes which come from a special Red Cross facility (and donor) in Illinois. These are someone else's white blood cells which would ideally help her fight the infection. Of course, these are not without their issues as well (as is everything in this department) but these are the only 2 ways to possibly save her life. We should know the timing of all of this in about 2 days.

As for the leukemia situation, we are going to deal with this when we get the results of the marrow back (hopefully Friday). There are some additional options on the table for treating the leukemia, but unfortunately she doesn't qualify for CAR-T or Chemo at all with the fungal infection because everything they can do suppresses her immune system and then she would surely die from the fungal infection.

As you read this, please please pray for Molly and our family. We need you right now. This is the time, this is the place. Send us that energy so that she can get through this. Please reach out and let us know you are there. We are scared, mad, sad and overall stressed beyond what anyone can imagine. We are trying to keep it together on a daily basis and sometimes it is so overwhelming. I have cried buckets of tears yet again these past few days and today had the hard conversation with Molly about what she wanted at the time of her death. I am not being morbid here, I am being realistic. During our very sweet discussion, Molly says, Mom, if I die, I want my body to be made into dirt and a tree planted in it up north on our property. She also wants a celebration of her life, not a mourning.

Up north

I love her so much.

More updates to come on Friday.

Peace and Love,

Emilie