Last we left off, I spoke of Elizabeth moving to an antibody therapy - called brentixumab vedotin (SGN-35, brand name Adcedtris). So here's where the roller coaster ride picked back up! As it turns out, Elizabeth seems to have been misdiagnosed from the very beginning. Dr. Safah would not accept the fact that she had such a dramatic mixed response to ESHAP. So, she ordered the pathology to be re-read - and fortunately for us, the second pathology analysis came from a doctor that trained in Maryland at Johns Hopkins. He had seen Gray Zone Lymphoma (GZL) before - and that's what Elizabeth has. It's a rare cancer - probably less rare that it seems because it is apparently misdiagnosed a lot. Anyhow, it's a mixture of Diffuse Large B-Cell Lymphoma (DLBCL) and Classical Hodgkin's Lymphoma, typically Nodular Sclerosing. What was Elizabeth's original diagnosis? You guessed correctly - Nodular Sclerosing Hodgkin's Lymphoma. Unfortunately, it appears that she was GZL from the start. The histology was re-examined from her "relapse" back in April and it was confirmed that this was GZL as well. We are in the process of getting the original slides looked at again - the ones from September 2011.
Once Dr. Safah figured out that she had GZL, she then ordered another PET/CT scan because the mini-BEAM regimen was not ideal for her type of lymphoma. She consulted with Dr. Dunleavy at the National Institutes of Health and National Cancer Institute. He told her that he recommended a regimen that NIH created - it's called Dose-Adjusted EPOCH-R. This regimen is primarily used for patients with DLBCL and GZL. Unfortunately, the data for someone using this regimen that has "relapsed" from GZL is rare - mainly because DA-EPOCH-R is used as front-line treatment. This regimen calls for a 5-day infusion of the following drugs: Rituxan, Etoposide, Vincristine, Adriamycin, Prednisone, and Cyclophosphamide. Rituxan is a monoclonal antibody specifically targeting CD20-expressing cells (which Elizabeth has on her tumors in her chest and lung). It has been one of the very best drugs to ever hit the cancer field and because she hasn't been exposed to it before, we feel really good about its chances of working. She has had etoposide before - in the ESHAP and mini-BEAM regimens. Vincristine is a chemical analog to the drug vinblastine, which she received in the ABVD regimen. She has also had Adriamycin in ABVD - the "Red Devil". This drug has a lifetime dosing limit that we will most definitely hit by cycle 7 so we need this damn regimen to work before then!!! Haha. Prednisone is a steroid - nothing to see here. Last but not least is the really fun drug - cyclophosphamide. It is a nitrogen mustard alkylating agent! Oooh fun! It was developed a long time ago - and is in the family of mustard gas agents used back in WWII. It disrupts DNA by crosslinking the DNA strands and causes programmed cell death. It also causes any number of two thousand side effects - ok, I'm kidding. But it is a toxic chemical. Oh who am I kidding here - all of these drugs are toxic and shouldn't be taken! But alas - here we are starting yet another chemo regimen. The beauty of this regimen is that it is given over 5 days - constantly, unlike the standard R-CHOP. The science behind this dosing schedule is that they feel it gives the drugs a better chance at hitting the cancer cells during their growth phase. Makes sense because cancer is just abnormal growth of human cells - kill them while they are rapidly dividing. The long-term data from DA-EPOCH-R is showing it to be a better treatment for DLBCL than the current standard R-CHOP. Hopefully this will play out well for us too! Dr. Dunleavy says that in his experience, GZL responds better to non-Hodgkin's treatment - good! Hopefully, she'll be a case-study that makes it's way to scientific articles and will save someone else's life one day! I know that she is going to be an example - a great example - for other's in her position.
We feel like this regimen will work. We have finally gotten answers to why things happened the way that they did. Elizabeth might not have "relapsed" in the traditional sense. She may have just suppressed the non hodgkin's aspects of her cancer to a point where it took the 14-16 weeks off of chemo to come roaring back. Interestingly, this is something that was missed twice by West Clinic. I won't say much about it other than if you are EVER in a situation where cancer is possible based on biopsy results - get a second opinion on the pathology/histology so that the proper diagnosis is given and that specific disease is treated properly. We look back and are disappointed in West Clinic and Dr. Pallera - not because he isn't a good doctor - because he didn't ask "Why" like Dr. Safah did. It was the "why" that has saved her from more chemo that would have yielded the same results. We are very lucky to have caught this now because failing transplants lessens the chance at a cure - something we are still trying to achieve.
Enough of the soapbox - Elizabeth is doing well. She finished her first cycle of DA-EPOCH-R last week - right after Hurricane Isaac hit. Told you this was a roller coaster ride, didn't I? I mean - how many people can say they got inpatient chemo while a hurricane hit? Sheesh! For the first time in nearly 3 months, she is feeling little to no pain in her left lung/rib cage area. This is where her largest mass is - about the size of a golf ball. No pain = Scott feels good about this regimen. Maybe we are killing this shit once and for all. She did get a little fluid build-up in her right lung and it made her uncomfortable for a couple of days this weekend. Her blood counts are also wiped out so she got a fever this weekend - but luckily we were able to avoid a trip to the ER as it stabilized and then went back down. Any type of infection could be fatal right now because her immune system is basically non-existant. She rebounded fairly well and is back to herself now (even though those counts are still 0). We had Anna's 6th birthday party on Sunday. Man, out little girl is growing up too fast. She is the love of my life along with her little terror of a sister, Lyla. I miss those little ones so much now that I am separated from them during the week. Oh well - whatever it takes to get Elizabeth healthy - that's what we'll do!
I will try and write more soon. We have lots more to share. But the take home is this: We finally got the right diagnosis and are on a more tailored regimen. She feels 100% better in her left lung which is fantastic news! We are super-hopeful now - even if she still faces transplant in her future. We'll see as the next scan approaches! She will be back at Tulane in the hospital from Sept 13-19 or 20th getting cycle 2 of DA-EPOCH-R. She will get another PET/CT scan at the end of September and we'll see how it's working. Hopefully we'll be able to do more chemo (Yay! - I'm sure that is what Elizabeth is thinking!) and then proceed to curative transplant.
Keep her in your thoughts and prayers! We certainly appreciate it!
My best,
Scott
