“When you’re up to your neck in manure, don’t duck.”
Within the hour of sending “Webby” (Eric) my journal entry gathering my thoughts on Wednesday afternoon, we received word back from Dr. Maris regarding Christi’s MRI. He stated that he is now convinced 100% that Christi has tumor growing in the back part of her left 2nd rib (second rib from the top). Needless to say, we are crushed, shocked and brokenhearted beyond belief to learn of her progression. Our minds are a whirlwind and our bodies feel numb. I knew Shayne was trying to be brave, but his tears wouldn’t stop as we sat at the kitchen table Wednesday after dinner absorbing the shock while Christi played innocently on the floor with toys her cousin outgrew totally oblivious to the recent blow. We love our precious daughter and her cancer progression is a horrendous setback. We knew there wasn’t a cure, yet we were hopeful she’d continue marching along on the ABT somehow despite all odds. (Wishful thinking!)
Yesterday Shayne’s sweet father drove up 8 hours and spent the day and evening with us. When it was time for the conference call with Dr. Maris, the girls went shopping for horse riding helmets and off to a restaurant with Grandpa. As I posted to our NB support group at this morning, this is what happened:
51 minutes and 5 pages of notes later, there is still no plan of what to try next. As always, Dr. Maris was so very kind, compassionate and professional during our phone conversation tonight; yet, no recommendation has been given. After we discussed some options, he'd like us to respond with our decision in the next 24 hours (OK, we're now down to about 14 hours - smile) so that we can move on and get her started with something with the goal of getting it under control for hopefully a while. Meanwhile, as I type, sweet Christi is currently cuddled up in my bed sleeping so peacefully - soooooooo clueless as to what all has just happened regarding this new curve her life has again taken.
Dr. Maris is of strong opinion that we don't bury our heads in the sand, but that we do something. He believes its systemic disease. Some of his possibilities / combinations for new treatment included: CEP-701, increase ABT, external radiation to new site, irino/temozolomide/cefixime combo, among others - all hoping to buy us some time while maintaining the quality of life she's been so blessed to have enjoyed the 15+ months. Any thoughts?
Thanks for letting me vent/post/whine/cry/brainstorm/question, etc., etc., etc.
A grateful Thomas team Mom (Angela) - looking forward to seeing you in C
Other things shared in our conversation with Dr. Maris from my notes included:
What is the new tumor? a bone lesion? Bone marrow clumps? (It’s like the marrow in that rib is resistant to ABT- now starting to get in the bone itself, actually expanding her rib outward now. It started near the spine part of the rib and is now moving out all along the rib.)
The amazing and brilliant, NB expert Dr. Maris had already consulted with: Dr. Kushner (MSKCC) Dr. Mattahy (
His recommendation won’t be cut and dry. What are we willing to put up with? Christi has had a great quality of life. What is she willing to do? We now have possibilities that we didn’t have before. She now has normal blood counts, never been through transplant, has four bags of stored stem cells, never had external radiation (and in my mind has the fire in her eyes to fight this beast) so she will have other clinical trials she now qualifies for that she didn’t before.
If the goal is to maintain her quality of life, allowing her to keep her hair, take just oral chemo concoctions and let her be in school every day - a temporary plan would be to try the Irino/Temozolomide/ Cefixime Phase I study through NANT. She may respond to that and we could have some months to gather our next plan. She did irino for three months exactly two years ago and it worked well for her. She went to school every day and came home and got her IV chemo. NOW, two years later, it’s available in a pill form. Yahoo! We could try it with radiation, or without (although I’m leaning toward “just” the chemos to see if they work and if they don’t then moving on to radiation of the rib).
Dr. Maris reiterated the fact that we’ve known all along: with Christi’s cancer, eventually the disease breaks through and perhaps we’ve let our guard down because she was doing so well. Her cancer is very difficult to treat and it’s a tough pill to swallow to now once again change her life.
We discussed external and proton-beam radiation attempts.
She is in a CEP spot in case we want to go with that Phase I study.
Now I will cut out pieces of private info regarding another one of our NB fighting friends who also treats at CHOP, I’ll share Shayne’s email back to this child’s awesome father. (Since Shayne rarely emails I always treasure his words.) Here are Shayne’s thoughts:
……………Well we thought we had a tough weekend, but you trumped us there. When things get wacky at CHOP sometimes Angela will look at me and say "This is the best children’s hospital in the country," implying just how bad is it at the worst?
So how was the scan? Any deviation from the prior study? Angela is notorious for playing amateur radiologist, me I prefer to just watch Sponge Bob. I get too crazy trying to read that maze of dots on a monitor that seems to have the resolution of 1979 vintage IBM.
Are you going to press for another stem cell harvest? My understanding is that COG was reviewing the standards for # of Cells needed for rescue and that it was going to be lowered substantially. I am sure you will express your desire to use the stem cells judiciously.
Thanks for the tutorial on Irino. I anticipate getting first hand knowledge real soon. Sorry for making you repeat your email to me. Angela an I sometimes live parallel lives in the same household. We usually share whatever comes across electronically; however, it has just been exceptionally crazy around here this past week with our setback.
Christi has had modest responses to almost every treatment she has ever taken; yet, has never had a clinical response. (I think the definition of a response is a reduction of 50%.) So we started with a paraspinal mass and almost complete displacement of the bone marrow. Her initial treatment was per COG 3891 which was a lower dose induction with a variety of agents. We transferred to MSKCC and they escalated the dose. Each round of chemo maintained or reduced her disease until we got to a smaller tumor and 5-10% marrow. With mouse antibodies we cleared a few spots and then we did a surgery to get the remaining tumor out. We followed surgery with Irino which basically cleared the remaining spots until she was just faintly MIBG positive. We did the MIBG x2 but the marrow continued to be positive. With her good marrow wiped out we could only do cis-retoinic acid after her stem cell rescue. We think she progressed a bit on Accutane but we needed the rest from the heavy treatment. After that it was ABT 751 and we had been stable for over a year until Friday. This is the first new uptake that we have seen on an MIBG.
While we have known that she was not cured, we had hoped that we had the disease stabilized and it would last for a long period of time. There is no devastation like that of disease progression….well diagnosis and another “d” word that I won't mention.....anyways here is what Maris had to say today:
His list of course reflects our desire for Christi to hold onto her quality of life and for us to have a life. Somehow he sensed our uneasiness about plunging back into full time cancer parenting so he softly spoke of PZA, IL12, and antibodies which all would require long hospital stays. Don't get me wrong we are willing to sell the farm to get her what she needs, but most of these things are just not that clinically interesting to me right now so why torture ourselves.
Maris is a beautiful mind and has more clinical experience than 50% of the ped oncs combined; however, he just has not yet come forward with a definitive plan for us yet. He is painfully aware that we tend to do our homework, referred to our knowledge of trials, etc. and I sense he is trying to take his cues from us. The next step should be decided before the weekend (I hope).
Strength to you my friend,
So, that’s the medical update!
Today my Mom came over with
While we don’t have a plan yet determined I thought I’d better send this update so that Christi’s sweet prayer warriors weren’t left hanging. I’ll send a brief entry to Eric when we know what we are doing about this latest progression of her cancer and how we plan to attack it.
Regardless, like the quote at the top of this journal entry hints at, we won’t be ducking, but will continue fighting as long as she’s able. I could just scream at the unfairness of it all, but I believe it is in God’s plan and one day I will understand. I can only sob into Shayne’s shoulder and cry, “Why not me?” I’ve lived a great, full life she has not. One day, we shall understand. Until then, we’ll fight with all our might to save Christi’s life and thank you for your continued support and love.
With gratitude, Angela
Christi’s Joke: She’s busy playing so I’ll not bother asking her for one right now.
What’s next? She’s continuing on with her 15th round of ABT even though she’s been officially kicked off the study. Soon we shall depart for the Neuroblastoma conference driving off to Chicago all compliments of the NCCF. Thank you! Attending the third annual conference, we will once again learn a ton from brilliant doctors and researchers around the country who work tirelessly to find a way to beat this beast. We also hope to have free time to tour some great
Even though this now may change, I am still planning to fly directly from
FINAL TESTING RESULTS (
BONE MARROWS: “…..no evidence of tumor in the bilateral biopsies; however, the aspirate smears show several small cohesive clusters of cells which are morphologically consistent with minimal involvement by metastic neuroblastoma. This discrepancy between the aspirates smears and the biopsies are due to differences in sampling.” (Basically, meaning even though decreased: bone marrow is positive and it’s always that fishing analogy we refer to. “Just because you didn’t catch any fish, doesn’t mean the ocean doesn’t contain any.”)
CT: Originally reported as clear despite the fact they were looking for something in that rib area; well, after the MRI the CT was modified. “…..When correlated with a prior CT scan performed on
MRI: ……….”In the area of abnormal MIBG uptake, along the posterolateral aspect of the left second rib, there appears to be expansion with increased T2 signal intensity, appreciated on image #8 of series 3……….”
MIBG: …….”The images demonstrate a focus of increased radiotracer activity in the left hemithorax superiorly and posteriorly that is linearly shaped, possibly in the left upper rib…….”
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