Alexx here. Sorry for the scarcity of blog posts the past 4 days but there were a lot of procedures going on and some scary possible diagnoses. So we wanted to keep it within the family until we had more information. Mom is doing okay, she is such a fighter. We still have a long road ahead of us but we are all on it together.
May 24th 2016 Day +47
Today I am getting a bronchoscopy. They want to sample my lungs to see if anything grows out–bacteria, viruses, fungus, and to check the types of cells. I’m still having fevers and they don’t know what. I tolerated the procedure without issues. It appeared “normal” and there wasn’t any blood or secretions.
Im still having nightly fevers. I also found out that that they saw newly enlarged lymph nodes and an enlarged spleen on my most recent CT scan. This is new when compared to a scan from a week ago. Because of this they want to get a PET scan to see which lymph nodes light up and which are the brightest; this way they know which one to biopsy. I’m scared and frustrated and I don’t understand why everything seems to be going wrong. Everyone I read about through FB and the MS blogs sailed through. Nobody had these problems.
May 25th and 26th 2016, Day +48 and Day +49
Alexx slept over last night. We watched a few episodes of Frankie and Gracie and it was a quiet night. Then in the morning I had a fever and it got better with Tylenol.
I had the PET scan done and we are awaiting results. It took 4 hours in total: transport, injecting the dye, waiting it for distribute through my body, drinking constraint, and then the scan, and then transport back.
Then I found out they want to do a bone marrow biopsy. Apparently they are concerned because I have an elevated ferritin, triglycerides, and cytopenias (low platelets and hemoglobin), as well as ongoing fevers without evidence of infection and splenomegaly, which can be indicative of a disease process called HLH.
We got the PET scan results in the evening which showed that many of my lymph nodes and spleen were “hot”, meaning they show a lot of activity. This could be from a type of lymphoma called PTLD but it could also be from infection with EBV or HLH inflammation. We are all very scared about the possibility of cancer but trying to stay positive and take it day by day, result by result.
HLH is when your body’s immune system overreacts and you get out of control systemic inflammation. I have 6/8 diagnostic criteria. HLH can be primary or secondary. Primary is related to genetics and is worse. Secondary is caused by a trigger–sometimes infection (EBV, CMV, bacteria) and sometimes from a hematologic malignancy such as lymphoma. After transplant and receiving ATG, people can develop a type of lymphoma called PTLD. So they need to figure out if the HLH is caused by the EBV or if I also have PTLD because of the new lymphadenopathy. For this reason I am going to get a lymph node biopsy. The easiest lymph node for them to reach is in my chest so I need to get an EBUS (endobronchail ultrasound), very similar to the bronchoscopy except they use a small needle to aspirate fluid from my lymph node.
Kai outside the BMT unit
Risa, Ian, and Ariel came to visit. It was wonderful to see them. They brought Kai with them but he couldn’t come onto the BMT unit because they don’t let kids under 12 visit because they have lots of germs. So he stayed outside and Alexx and Ricky played with him. It was a huge boost to their spirits. Kai is hilarious and Alexx introduced him to all of her coworkers.
I tolerated the EBUS without issue. Dakota and Ricky came in the morning. Alexx finally went home after 45 hours at the hospital. Gail and Mitch came to visit which was wonderful. Now we are anxiously awaiting the results of the bone marrow biopsy and lymph node biopsy.
May 27th Day +50
Alexx was on the phone with the attending Doctor around 9pm last night. She heard that after consulting with the pathologist, the BMT Doctor, and a lymphoma specialist that they do not believe I have lymphoma. Hooray! Finally some good news. Based on these preliminary results they decided to start Dexamethasone last night, which is part of the treatment regimen for HLH and brings down the inflammation. I haven’t had a fever since!
Dr Mapara (the director of the BMT unit and Alexx’s boss) came to speak with us today and explained everything. The bone marrow biopsy showed a hematophagocyte which is typical for HLH, however neither the bone marrow nor the lymph node showed evidence of lymphoma which is great news. NO CANCER!!!! So it seems the HLH was triggered by the EBV. Thankfully they caught the EBV and HLH very early. The EBV had peaked as high as 380,000; I received 2 doses of Rituxan and now it’s down to 1100. I’ll get 1-2 more doses of the Rituxan. He also said that typically patients with HLH are very sick and they get very sick very quickly. I’ve stayed stable the past few days, really only fevers; so he thinks that this is a good sign. There is a very specific protocol for HLH. It includes steroids daily and Etoposide (a type of chemotherapy) 2 times a day for 2 weeks and then once a week for 6 weeks. However, because I’m so stable he thinks I may respond to steroids alone. He is going to give me one dose of Etoposide tonight just to be precautious. Then they monitor response: they trend my fevers and inflammatory markers (Ferritin, CRP). This will dictate how long I will need treatment for. Obviously, with more chemo comes risk for infection, but hopefully I only need one dose. I have already received Etoposide before as part of BEAM.
Overall I’m feeling more optimistic than I have in a while. We know why I have fevers, we know I don’t have PTLD, and we have a plan. It totally sucks that I got the EBV/HLH but it could have been much more catastrophic. I am a warrior and I know that with the love and support of my friends and family and the expert care I am receiving at Columbia, I can get through this. I will fight.