WHAT'S HAPPENING Archive January 2004
January 30, 2004 "A Big Relief"Today started off on a very scary foot. Simon woke up and had some trouble formulating his thoughts. He seemed to be unable to find the words for some things. At one point, he said, "I'm confused!"
We called the on-call doctor, who said this behavior could be caused by the newly identified tumor on Simon's brain. Well, to make a long story short, we took him in for evaluation. As it turns out, the "new tumor" was a scary misinterpretation of Simon's CT on January 28. The tumor identified on that scan is in the BONE of his skull (frontal bone). The radiologists had used a head CT from November 23 for comparison (a CT that was done from the emergency room after Simon hit his head and got a huge swelling from his low platelets).
Our primary doctor (Dr. Lemons) is out of town at the moment, and the other doctors aren't able to stay up on the twisting details of Simon's case. We have now asked for a comparison to be done between Wednesday's scan and the scan done in New York on January 13. Our misunderstanding was that this disease site was new (since January 13) and growing extremely fast and that it was on the soft tissue of the brain, not on the bone.
We are VERY relieved to learn that it is "only" bone disease, which is probably causing enough swelling to give Simon a slightly blackened eye. We may even learn that it has not significantly changed since the January 13 scans. Dr. Lemons had assured me that the radiology team here had reviewed the scans from New York and that those scans had been put into digital format on the system here. Still, our experience is that radiologists always like to look at their own last scan and don't seem to "get it" that other institutions may be involved.
So, what explains Simon's muddled thinking this morning? We're not completely sure. Aside from pressure from a tumor on the brain, the other plausible explanation was a brain bleed caused by low platelets. Sure enough, Simon's platelet count was down to 6,000. His other counts have also plummeted. His ANC is 0.04 today (or 40 compared to Wednesday's 400). That's what's known as "profoundly neutropenic" (i.e., very immuno-compromised). His red cells were also way down, with hematocrit at 23.9 and hemoglobin at 8.4.
So, blood support was in order. Simon received a platelet transfusion (for the first time since July). We are gambling that his ITP won't kill off the donor platelets because he has seemed to hold a much higher platelet count since December. It is quite certain that the drop in all of Simon's counts is the result of last week's chemo. We must hope now that Simon's marrow can recover effectively from the chemo. I don't think it's going to bounce right back, given how low and how quickly his counts have dropped. Simon also received a transfusion of red blood cells today.
We are home resting now. What a bizarre and unnecessarily scary string of events. Not that the whole story isn't completely worrisome and unsettling, but the last week has had a couple of points where I have been glad that I'm paying attention. The first was our discovery last week that our homecare pharmacy had overbilled for Simon's chemo (irinotecan) in September. To the tune of $51,000 instead of about $10,000. It makes $10,000 sound like a bargain, doesn't it? And to think, our insurance company PAID the claim (that's how we even knew what was billed). It will be adjusted. But when you've got a lifetime insurance limit of $2 million, you need to watch every $50 grand!!
So, after chastising the insurance company for paying such an outrageous bill, I move on to a doctor reporting that Simon has a new tumor and that it is on his brain. Only to learn that it's probably "just" the same old stuff growing away in the bones. As I said, it's a huge relief. Of course we still have a lot of other cancer sites to worry about, but the idea of a brain tumor and a kid who gets cognitively muddled--I shudder to recall the worried feelings of this morning.
A second care package from Zingerman's arrived today! (The Cookie Fairies of Pebble Creek strike again!) Our friends in Ann Arbor are really keeping us going with this yummy stuff from a special delicatessen, reminding us of many times we shared Zingerman's food together in Ann Arbor.
We'll be staying in most of the weekend, eager to keep Simon away from germs and out of the hospital. The last time his ANC was this low was during transplant. That's the picture on the inside of Simon. On the outside, he's playing happily with his transformers, and he just went downstairs to catch Pokemon on TV.
January 28, 2004 "Another Bruise"Simon's counts are dropping precipitously post-chemo. His platelet count was only 26,000 (after it was 73,000 on Monday). We are debating with the doctors about how to manage his platelet low. Do we give IVIG/vincristine to treat his ITP? Or do try a platelet transfusion? Back in July, his ITP consumed every tranfused platelet within half a day. Now, he might be able to hold onto some.
Simon's ANC (measure of immunity) is 400 today. That's officially in the range of required hospitalization if he gets a fever. So, he's staying home from school and enjoying excellent homebound instruction from his Grann.
We went into the clinic this morning for Simon to receive pentamadine (an inhaled antibiotic used to prevent "unrecoverable" pneumonia). For the past couple of days, I've noticed a darkening of his left eyelid, and I asked the doctor to take a look. I've seen other kids with neuroblastoma have tumors that swell into the eye socket.
Most unfortunately, the hastily ordered CT scan revealed a tumor that is now growing on Simon's brain, just behind his left eye. The CT report says it's .7 cm x 2.5 cm. To my knowledge, it was not apparent on the scan done January 13. That's a lot of tumor for 2 weeks' time.
Simon does not appear to feel any discomfort from this new tumor, but we are watchful. The chemo in his system may be able to stop the growth. We will also look into radiation for that site.
I do not think we are able fully to comprehend the spread of his disease right now. We are holding him close and still hoping the chemo will offer some relief, with its nasty negative effects passing quickly.
Numerous gestures of kindness shower down upon us. A surprise box of goodies from Zingerman's delicatessen in Arbor (thank you to our friends in the Business School OB department!). A lovely dinner yesterday at the Majors' home in Salt Lake City (our new church friends who somehow now feel like lifelong friends). A tantalizing mailing from Dotty Seiter, the ArtWarmers lady in Massachusetts. A surprise visit this evening by Noah, Tobias and Steven (the goat cheese and cocoa mix are delightful, but the play time for the kids is an unquantifiable therapy). Secret hands at work in the kitchen that make the dishes clean, wash the lettuce, cut the grapefruit, and put dinner on the table--actually, the hands belong to my mother!
We are grateful for all gestures (including the silent, prayerful thoughts of people who root for Simon everywhere). Your collective strength helps us face tomorrow as another good day.
January 25, 2004 "Busy Day"Simon has now received 5 days of topotecan/cytoxan (a moderate dose chemo that we hope will put a halt to the spread of his disease and turn the tide back toward decreasing disease). He has tolerated it beautifully and cooperated fully during the many hours of being hooked up to pumps, bags of fluid, and syringes of medicine. Our homecare company was surprised to learn that he didn't need the extra anti-nausea drugs they sent. To the contrary, Simon has chowed down vigorously at dinner each day of this chemo.
As it turned out, Markus became our primary at-home chemo nurse. The first day of chemo at home was too much for me--with both kids home and a huge array of pumps (3) and drugs (3) and fluids (2). I decided to back off on going it alone to get the IV fluids started before the homecare nurse came to guide the actual chemo part. Instead, I called Markus at his office and asked him to come home and help. He is an excellent chemo nurse. And he agrees, this is a really complicated process for us to manage at home. Altogether, the chemo involves 3 hours of pre-hydration, about 90 minutes of anti-nausea medicine followed by each chemo medicine over 30 minutes. Then we chose to do an optional 2 hours of post-hydration to help Simon's bladder clear the cytoxan as quickly as possible. I told you in my last post that we are "pretty good" at this stuff. Well, now we're even a little bit better!
For those "outside" the cancer world, all of this medical activity may sound overwhelming. But try to picture Simon with a little backpack holding his mini battery-operated pump and his bag of fluid/medications. He hoists the backpack easily on his own and walks around the house, engaging in his regular activities: building with Lego, playing Pokemon/Yugi-Oh cards, watching TV, etc. He has to run to the bathroom a lot because of all the hydration. He snacks. He laughs. It's amazing.
Today was a busy Sunday for all of us. I got up early to sing in choir at church, and I used the early hour to start Simon's pre-hydration before he even got out of bed. An hour later, I attached the second bag of prehydration fluids and then headed to church to rehearse. While I sang at the 9:00 service, Markus and the kids did the chemo at home. They finished in time to come to the second service, and Simon gamely "wore" his bag of post-hydration fluids in his Sunday school classroom.
The church services today were momentous and emotional, as the church says goodbye to our Minister of Religious Education, Silvia Behrend. We met Silvia about a-year-and-a-half ago, and she has greatly eased our transition to Salt Lake City. We will miss her very much.
After church, we had a lovely lunch with Barbara and Justin Creek, fellow choir members who also happened to have tickets to the 2:00 matinee of Utah Opera's Hansel and Gretel. Thank you, Barbara and Justin, for your company and for the delicious lunch.
We are also grateful to Tamber from Kaleidoscope Kids/Community Nursing Services for getting us tickets to the opera. This was the children's first full-length opera, and (after some first-act fidgets) they did great. For me, it was a trip down memory lane, recalling my college experience of playing the role of the Mother in an Oberlin Conservatory production.
You would think that the day was already long enough, but we went back to our lunch spot for a little dessert. While driving home, we got a call from friends Bart and Tina, reminding us of our plan to join them for supper. So, we did a U-turn and headed their way instead. It felt great to hang out at their home and relax. The kids ran around with Carter and Granger (and tried to avoid big, slurpy kisses from Silky the dog).
Looking back, this Sunday was something of an extreme sport. We all had a great family day. The kids are sleeping soundly, and the parents are headed to bed, too. Somehow, chemo was able to fit in just fine.
But before I close, let's go back to Simon's cancer. It's time to think thoughts of massive attack and destruction of invasive cancer cells in the marrow and bones of Simon's body. Platelet dances are also in order. We will check counts on Monday and begin neupogen shots to boost Simon's white cell production. We hope he will remain healthy and in good spirits, and we REALLY hope the chemo does its job.
January 21, 2004 "Chemo Started Today"Simon had his first day of topotecan/cytoxan today. It went smoothly at the clinic, and we will administer the remaining 4 days at home. On Thursday, a homecare nurse will come to teach us how to program the pumps for the chemo agents. The rest of it (IV fluids, an IV anti-nausea medicine) will be "old hat", but with two chemo agents and two different fluid types, we'll need to be able to reprogram the rate that the pumps put the medicine into Simon.
It probably sounds very intense to anyone who's never hooked a pump and a bag of fluid up to a patient. But we're getting pretty good at it by now!
Simon is doing well. He feels good, he's eating well, he's talkative. He's Simon. He is walking about uncomplainingly, and we believe that is a sign that he is not in pain.
It is time to send your very best in hopeful, healing, loving, prayerful energies to Simon so that this chemo can beat back the cancer lurking in his bones.
The platelet dances are working. Simon's count is holding today at 130,000.
January 19, 2004 "Gearing Up"Simon's test results from New York continue to come in. The news continues to be worrisome. His bone marrow biopsies and aspirates are bilaterally positive for neuroblastoma. Now we are back to a situation that's about as precarious as at diagnosis. Only now Simon's body has been through so much. We must proceed with caution as we begin stronger therapies.
On the completely opposite side of the coin, Simon's platelets are on a rally. His count was 133,000 today. Woo-hoo! It's 4 weeks now since his last ITP treatment, and he has previously fallen off again after 10 days. Right now, he just keeps going up. Watch out normal range! Simon is just 17,000 points away! (These sound like big numbers, but think of platelets as a currency with huge inflation. A few thousand is a trifle.)
We continue discussions with Dr. Lemons about the next best step. We are gathering input from Simon's doctors on both coasts, and we will weigh the various suggestions to make our plan. I'm guessing chemo will start Wednesday at the latest.
Our handsome Captain Underpants had a fine day today. It was "Civil Rights Day" (that's Martin Luther King, Jr. Day for the Salt Lake City School District), so there was no school. Simon and Miriam played happily about the house. The dreary fog/smog is beginning to lift, and we saw some sunshine this afternoon. The kids and I had an uplifting trip to the toy store in the afternoon. I had promised Simon the Lego bug Creator set after his hard MIBG scan (it was a LOOONG time to lie still) last week. Miriam grabbed her piggybank and was able to purchase doll number 3 zillion in her collection--but this one floats in the bath!! They were both delightful companions at dinner, and we had a family game of Apples to Apples (junior) before bed.
Simon is eager to head back to school tomorrow, and he's bringing copies of a coloring book "for friends of children with cancer". It's called Sammie's New Mask, and we received it at no cost from the National Children's Cancer Society. Simon has reviewed the book, and he thinks it is a very good presentation. It will help his friends understand more of what he will be going through in his next round of treatment.
I know Simon's wide caring community is taking a huge gulp right now as we brace for the next steps in Simon's care. We truly appreciate all the support and all the comforting messages on Simon's MESSAGE BOARD. We have heard from other families on our neuroblastoma support listserv about their success in treating disease that sounds similar to Simon's current status. We fervently hope that we can turn the tide and move Simon back into a place of diminished disease. He does not appear to be feeling pain, although he is tired sometimes.
January 15-16, 2004 "Sobering Results"January 15 (on the plane, coming home)
When the consultation with the doctor about test results starts with, "It's not good", worst fears begin to feel confirmed. It is too soon for us to have processed the information and reformulated our plans. However, Simon's tests have shown substantial disease progression throughout his skeleton.
I find these words extremely difficult to convey. I find myself hoping that we will learn it is less dramatic than it sounds and that we will discover therapies that address this situation.
Not all of the results are in, but so far we know the following: the bone scan and CT scan both show active disease in Simon's skull. On the good side, the CT did not detect any new tumor masses in Simon's body. The MIBG scan (which was lengthy, iterative and somber yesterday, giving me my tip-off that things were not going well) revealed spots of active disease, presumably in bones and marrow, from "head to toe". Although the bone marrow test results are not back yet, the MIBG scan makes them "beside the point".
It turns out Dr. Kushner was already alerted to the disease progression on Monday when he saw an abnormal level on Simon's serum LDH (normal is 200, Simon's was 500). I need to learn more about this marker for disease. So, I guess they knew right away that Simon's disease was pretty active, but they kept us in "suspense" until today. I wonder if it's better to head through the battery of tests in oblivion, or to proceed nervous of finding something scary. Either way, the results are in, and they're not reassuring. Our hope to prep Simon for more antibody therapy will have to wait in the wings for now.
January 16 (at home)
The trip to New York was tough in many ways (Simon's virus and isolation status, the wintry weather, the "solo parent" travel, the dismal medical news). Still, we were able to connect with friends Barbara Blodgett, Ethan Steinberg and Rona Rodrig, and at least by phone with Bethany Reeves. (Barbara, we still have your scarf--thank you for the extra cushioning for Simon's sore back!) On the return trip, we encountered many warm and open spirits on the planes and in the airports. Simon and his new 8-month-old buddy Harrison lit up the Denver airport with their charming interactions, and Harrison's parents offered gentle curiosity about our reasons for travel and provided caring ears to hear our newly sharpened worries. We were also spirited along by the lady from the plane (with whom we never exchanged names), but who didn't mind that Simon spilled some water into her purse and who helped us move our stuff from the arrival gate to the next departure. And to the kind flight attendant who gave the lady and her companion a complimentary cocktail when I offered to buy her one after the mishap with the water. There are so many ways in which the world continues to sparkle, no matter what.
After breakfast this morning, Simon conversed briefly about the scan results. He was on his way to the TV room, but he turned, as if on an afterthought (or maybe on a twinge of something uncomfortable). He told Markus, "My mom was hoping that the bone scan results would show that everything is OK. But they found new cancer in my body." His eyes may have teared, but he turned and went off to the business of watching his new Bionicle movie. I had taken the news on Thursday tearfully, and I did not want Simon guessing about what was up. I had told him that I was really hoping for good results, and that I was feeling sad that they found more cancer. I told him it probably means he needs to get ready for more chemo.
To end on a note that better represents the tone of Simon's day, he had great fun with his sister, with all the toys he'd left behind for a few days, with the friends who came to visit. He laughed and was buoyantly goofy. This afternoon, inspired by Miriam who stripped to her undies and called herself "Captain Underpants", Simon stripped to his, donned a red cape, and zoomed about the house shouting "Tra La Laaa". If you are unfamiliar with Captain Underpants, head to your favorite bookstore and check it out!
January 14, 2004 "Waiting for Results in New York"Simon and I (Mary) are in New York on a short trip for his January work-up (scans and other tests). We have completed all the tests and will have a meeting to learn preliminary results on Thursday before we fly back to Salt Lake City.
The folks here have been pleased to see Simon, and to see him looking so well. He's grown about 1.5 inches (3.5 cm) since his last visit to New York in July. Yesterday he arrived in his grey sweatpants and a long-sleeved Michigan shirt. The clothing is a bit on the big side, giving him an even fuller, taller look. With his short hair and his hands in his pockets, he looked so very cool and grown-up. One of the staff walked by and asked, "What are you, a freshman at Michigan?"
As always, a battery of tests opens the potential for learning bad news. It is hard to wait until tomorrow for reassurance or, if that is not possible, findings that indicate a need for new action. Please be thinking strong, healing thoughts for Simon. And strong nerves for me, as I go to learn what they see (or, hopefully, DON'T see) inside him.
January 3, 2004 "Happy New Year"We embark on the year 2004 filled with hope for a good future for Simon. Markus gave expression to his hopes at a recent church service, and his Hopes for 2004 are posted on this site.
It is hard to believe that I am now setting the year "2004" after the date as I write about Simon's life with cancer. He was diagnosed at the very end of 2001. We are now in year 3 of this intense endeavor.
Accutane so far, so good
We are also grateful to Mary's cousins Steve, Annette and Rowan Williams (frequent contributors to Simon's MESSAGE BOARD) for their hospitality in Oakland. Despite patches of heavy rain, the trip was easy and pleasant.
The consultation with Dr. Matthay and neuroblastoma nurse Janet Veatch took place on Tuesday afternoon. Our doctors and we had sent records and scan files ahead for her review, so we spent the first part of the appointment filling in the gaps in her understanding of Simon's case. With all the hospitals and the complication of ITP on top of neuroblastoma, Simon's case is not easy to explain.
Her overall take on Simon was very encouraging and supportive of his treatment to date. She classified him as having "minimal residual disease". Next to remission, that's as good as you can get. She agrees with the radiologists at Primary Children's that the spots of active disease (presumably bone) are in the right shoulder, the lower right pelvis, the right orbit, and along the top of the skull. She was glad we have him on accutane (and suggested we could have started that months earlier).
She took a good look at Simon and interacted with him enough to see that he's a happy kid with a high quality of life. She urged careful consideration of more toxic (but potentially curative) therapies, keeping Simon's quality of life in mind.
Simon's ITP is another reason to avoid more toxic therapies, since he's not currently eligible for most studies due to his platelet instability anyway. Dr. Matthay advised us to keep him on accutane for another 4-6 months and hope that the ITP resolves. She reviewed two more radical ITP interventions that we might consider only if and when Simon's neuroblastoma requires more intensive therapy. One is a splenectomy (removal of the spleen) and the other is radiation to the spleen to disable it.
Finally, Dr. Matthay recommended that Simon receive radiation to the site of his primary tumor (near the left kidney/adrenal). We will discuss with Dr. Lemons when that might be considered. She said that scans (CT and MIBG) and bone marrow biopsy/aspirate every 3 months are sufficient monitoring (unless we suspect something is going on), and that a monthly spot urine check for VMA/HVA levels is a good idea. All of these things are part of Simon's current treatment, but we can probably develop a more orderly schedule, including for his ITP treatments.
Although she recommends avoiding high-toxicity treatment, Dr. Matthay was intrigued by the idea of prepping Simon with high-dose cytoxan (very immuno-suppressive) to enable him to have more antibody therapy. One possibility there is that his ITP would be improved by the immuno-suppression. Of course, no one can predict the results of this riskier idea, and we are still thinking over our options of possible further antibody therapy in New York.
We are glad to have Dr. Matthay and Janet Veatch on Simon's team, and we expect Dr. Lemons and we will continue to consult with them as time passes.
Simon's story, and that of our family as we live on without him, appears on this Web site in reverse chronological order. Anyone who wants to see the whole story can scroll down to the bottom of this archive and start there. We have listed the treatments that Simon underwent in the short summaries for each archive link in case other families facing neuroblastoma want to take a look at Simon's experience as a point of reference.
The second half of 2005 has come and gone. We hit the one year point since Simon's death on August 6th. We celebrated Simon's memory with a concert by the terrific group Two of a Kind at Utah's Hogle Zoo. Miriam has started kindergarten, Markus continues to work hard as an assistant professor, and Mary is doing a little teaching (voice and German) and pursuing her interest in writing.
Miriam finishes preschool. We celebrate Simon's birthday on May 17, 2005 with an outpouring of community love and support during a Lemonade Stand in Simon's memory. We travel to Oberlin for Mary's 20th college reunion and on to Ann Arbor to hold a second memorial service for Simon on June 3rd. Mary attends a writers' workshop in Salt Lake City.
Acts of mourning continue as life also moves forward. Mary travels to Denver and meets the bereaved family of Becca Lockler.
The pace slows. We reach six months since Simon died, then seven. Mary and Miriam travel to Ann Arbor for the first time since Simon died.
Working through grief; working on life; trying a few new things.
Approaching "the holidays" without Simon and marking time with the moon.
Our fourth month without our dear Simon included a busy and rewarding celebration of Simon's legacy with the music duo, Gemini.
Life without Simon crawls along. Memorial tributes of all kinds are underway, from Simon's Memorial Fund to friends and family holding events in Simon's honor.
Life without Simon creeps along.
Simon died on Friday, August 6. We held his memorial service on August 28, and we established the Simon Craig Vodosek Memorial Fund to honor his beautiful spirit.
The month began with Simon's wonderful Celebration of Life Party. As his disease progressed, he slowed down more and more, but he hung in there for the whole month.
A wonderful end of Simon's first-grade year (June 4) leads quickly into a rapid progression of disease with increasing pain and weakness. Our focus is on pain control and other comfort measures to allow Simon to enjoy his remaining time.
Recovery from radiation side effects and enjoying good energy to get back to school. Second course of temazolomide/irinotecan. Simon turned 7 on May 17!
CEP-701 trial abandoned after 4 weeks because of further progression; begin palliative treatments to ease symptoms of jaw tumor and general disease, including radiation and chemo with temazolomide/irinotecan.
Chemo abandoned after further progression; begin CEP-701 phase I clinical trial at UCSF
More chemo (round 2 topotecan/cytoxan)
Disease progression (widespread in skeleton) discovered during work-up at Memorial Sloan Kettering Cancer Center; begin salvage chemo with topotecan/cytoxan
Begin accutane (vitamin A derrivative to fight neuroblastoma); consultation with Dr. Kate Matthay at UCSF
Irinotecan/vincristine at home
Onset of ITP (immune-mediated thrombocytopenia purpura) AND move to Salt Lake City
Exploring monoclonal antibody therapy; prepared with topotecan/cytoxan
Still recovering from MIBG therapy
Finally starting kindergarten!
Recovering from transplant
Mixed response to therapy--taken off COG A3973 after 4 chemo cycles
Stem cell harvest (to be purged)
Diagnosis and starting treatment