Steven's Rhabdoid Cancer Blog

Diary of Events

Previous Month January 2007

Thursday February 1, 2007
Steven had a brain scan today, with a MRI, and a blood test to check his counts before going into the second chemo round. The scan of his head to is check there's no cancer there. Rhabdoid's typically occur in one of two places, the brain and the abdomen. On initial inspection there appears to be no tumor in his brain - a great result if confirmed as we've not heard of a single living person having recovered from Rhabdoid's in the brain.

The second chemo course is set to start tomorrow, this round of drugs being called ICE (Ifosfamide, Carboplatin, and Etoposide (a.k.a. VP-16)) and Mesna. It will take 3 days to administer, so we've got a 4 day hospital visit at a minimum. Common side effects of this course include hair loss, vomiting, and low blood counts ranging from 1-4 weeks from the chemo. Less common, but far more severe side effects include sores, abnormal liver function, kidney damage, and hearing loss.

Saturday February 3, 2007
The chemo procedure is dramatically different than last time where he was on a constant feed for 48 hours. This time the chemo drugs only take 2 hours per day, but for three consecutive days. Following each chemo round there's three rounds of recovery drugs every three hours. So it's pretty much non stop switching of drugs for about 11 hours a night.

Friday night was pretty tough, Steven received all three of the ICE drugs which must have been pretty nasty as he seemed to be in lots of pain. Saturday and Sunday he only gets Ifosfamide and Etoposide, leaving out the Carboplatin which is the nastiest of the three, he seemed much happier last night and even slept pretty well.

Steven - Feb 2 2007

Monday February 5, 2007
Steven returned home in the afternoon having finished the second round of chemo. 

Tuesday February 6, 2007
He seems to have handled the chemo pretty well, although he threw up twice today so is on anti nausea medicine. He's back to receiving Neupogen shots to raise his blood counts. However, one side effect of the ICE chemo course is that it lowers blood pressure, so he's off the Enalapril which reduces blood pressure which was high due to the kidney damage. He's going to the nephrologist tomorrow to check his blood pressure and hopefully everything will have stabilized and we'll be able to finally stop giving him anything for blood pressure after 7 weeks of Enalapril.

Wednesday February 7, 2007
Steven was again sick today, and considered a little anemic by his nurse so they took an extra blood sample. The sickness is an expected side effect of the chemo, this second round has been much harder on his body.

Today was Nikki's 4th birthday. We all had a nice time, she chose Fats Asian Bistro for her birthday dinner. We had birthday cake at home with presents. Nikki was really pleased with  getting own computer, along with a princess pink keyboard and mouse. 

Saturday February 10, 2007
Steven was sick once more on Thursday, but has been fine since then, he now seems to have gotten over the chemo. Certainly this round of chemo was significantly harsher on his body, either it was just that it's the second round and it's going to get progressively worse, or it was the different chemicals this time versus the first time that were harder to deal with.

His blood test from Wednesday came back strong, but we expect his counts to start dropping over the weekend, or in fact may have already done so, this will leave him in a very low immunity state next week. Currently he's only on the Neupogen shots on a regular basis, and at this point off the blood pressure medicine.

Nikki had her birthday party today and we brought Steven along to watch everyone, he gets bored quickly, so this was something different and fun for him to see. 

Steven's getting his first tooth, making it hard to tell whether he's fussing for that or other reasons, but he's been pretty happy the last few days.

Wednesday February 14, 2007
Steven had a blood test today, the results indicate significantly low red blood cell counts, and dropping platelet counts, however his white and ANC counts are climbing, and are up from 2.2K to 15K, so well out of the danger zone. He'll be receiving several blood transfusions later this week. Although this isn't totally unusual it's disappointing as it wasn't necessary after the first round of chemo.

On the positive side his blood pressure was measured and is very good, now that he's been off any medicine for adjusting it this is a great sign that his kidney damage has repaired and is now fully functional. Also, Thursday will be the last shot to his leg with the Neupogen medicine until the next round of chemo.

Steven now has his first tooth, he's been particularly fussy recently, and it's been hard to tell how much is from the teething and how much from the cancer.

Thursday February 15, 2007
A day of reflection and love for lost friends and family...

Today Jenn went to a funeral while I went to a cremation service.

Jenn took Steven and attended the funeral of Jim Patton, the husband of one of Jenn's closest co-workers. In a particularly touching surprise, Jim's wife, Lynn had decided that in lieu of flowers to make a collection for pediatric cancer research at St Jude's Children's Hospital. This is the hospital that Dr. Dome used to work at where he developed the treatment protocol that Steven is currently undertaking and where much cutting edge research continues to occur. 

I attended Julia's cremation service in Cambridge, England, it was a very nice service which I'm sure Julia would have appreciated. Chris, her boyfriend, and Oliver our brother both spoke in the service with light hearted but deeply touching tributes. Also in lieu of flowers at Julia's service a collection was made for pediatric cancer research in the UK.

As I sit here in tears 6000 miles from my wife and son in so much pain I feel the love of family, friends, and now hundreds of people I've never even met before. We thank you all.

Friday  February 16, 2007
Jenn posted some questions about where to find kids activities, places to play, and about pre-schools, in the Richmond VA area to Craigslist. She received a tremendous response from people with lots of information and many generous offers of help. Showing what a small world it is, this posting went full circle to a help group that Jenn's mum, Sue,  is a member of who then was forwarded our Craigslist posting to her - not realizing she was related to us. Sue has been of phenomenal help to us already having entirely given up her normal life to spend the vast majority of the last two months looking after us out in California.

Steven had 9 hours of transfusions today, two packs of red blood cells, and one of platelets.

Wednesday February 21, 2007
We all few in the night before and met at Dulles. Today was the first meeting with Dr Dome the doctor with the most experience with Rhabdoid tumors, and head of the Pediatric Hematology and Oncology department at Children's National Medical Center in DC. The reception area didn't have a record of our appointment with Dr Dome listed, they had just converted their scheduling system and obviously made some mistakes, in fact it was eventually found to be scheduled for next week - not a good start.

When we did get to talk to Dr Dome we were totally impressed with him and his experience in this field. He has personally owned the treatment of 7 patients and assisted with the treatment of as many more. We quickly decided that we wanted to work with him. At the end of the first meeting with him we were pretty much assuming we were going to move the entire treatment to DC. We asked if he had had any patients survive who were as young as Steven, unfortunately not. Although he did reassure us that there were patients Steven's age who had survived, but alas his chances are still only 17%.

Dr Dome managed to dramatically increase the pace of things. He started talking about resection (surgical removal of the tumor). We had thought this typically occurred after 6-8 rounds of chemo. However, that's not the case with Rhabdoid, he recommended it occur after the 2nd or 3rd round of chemo. As Steven has already had two rounds and responded to them very well he might be a candidate for surgery already. Dr Dome scheduled a CT scan for Friday so he and the surgeons could see how things were looking.

Next after the meeting with Dr Dome we went on a hospital tour, visiting the infusion centers, the ward, and several other places. None were particularly inspiring for a leading hospital. In pretty much every respect we were much more impressed with UC Davis.

Thursday February 22, 2007
Developing our plan of action: we all had flights booked for Saturday to go back to California so we were wondering what to do based on how things went on Friday, and how impressed we were with Dr Dome, but also balancing the fact that there was no value in getting Chemo in DC versus Davis where it will be far more convenient for us, and seemed so much better. Dr Dome confirmed that infusion and chemo are totally standard so it didn't matter where we got that done.

We decided that we'd go for the CT scan Friday and hopefully get a call from Dr Dome Friday evening. If he was ready for surgery Jenn and Steven would stay behind and get it done, Nikki and I would return to California on Saturday. If we either got a "not ready message" or no call at all, we'd all go back home.

Friday February 23, 2007
Steven's CT scan appointment was at 1pm, we arrived at noon (you have to arrive an hour before to get prepared for the scan) By about 2:30 none of the preparations had been started only debates as to whether Steven needed to be sedated for the CT. We were getting very unimpressed, and given the late time in the afternoon we were beginning to consider whether to cut our losses and leave - we had a CT scan scheduled for Tuesday at Davis anyway.

Jenn spoke to the receptionist saying that Dr Dome was waiting to review the CT results this evening, and then everything started moving. At about 5pm we got called into the PACU (a recovery ward), and shortly after that Dr Dome appeared.

We were impressed with the staff at the PACU, and again with Dr Dome, he had already reviewed the CT scan with the surgeon who would be doing the resection. They think he's ready, and are preparing for surgery on Tuesday. One surgeon is a senior attending pediatric tumor removal specialist, and the other a senior fellow. It seems that Dr Dome is moving this at light speed with excellent surgeons. However, there's never a day with only good news: The tumor appears to have encapsulated around his ureter (pipe from the kidney to the bladder) they need to remove the ureter where the tumor is attached to it.

This leaves three options - try to stretch out the remaining parts of the pipe from the kidney to the bladder and reconnect it, put some artificial pipe in, or remove the kidney. Dr Dome spent some significant time explaining that they won't know what's best until they are in the surgery so we need to be prepared for a kidney removal.

We also talked extensively about radiation therapy. Of all the side effects we've discussed with the doctors thus far, these are the worst impacts and highest probability. There are major risks of introducing new cancer due to the radiation and creating stunted growth problems in the areas affected by the radiation, with some severe side effects. Unfortunately through Jenn's extensive research with other Rhabdoid patients the only survivors are ones who have had radiation therapy, Dr Dome confirmed this, so it's not optional. The only question is when to do it, and how strong the radiation should be. The ideal point for radiation is straight after resection, however there are extremely serious concerns with using it on a baby under a year old, so we may hold off until the end of the chemo before using the radiation. It's at least not planned for this visit, so we'll likely be back to DC at least one more time to get the radiation done here.

The final discussion of the day with Dr Dome was fantastic news. We had submitted Steven for an extremely special kind of blood test. We had had major problems finding anyone willing to even draw his blood in California for this study, but had eventually managed to get the sample to Dr Biegel in Philadelphia for the test. She was looking for the deletion of the INI-1 tumor suppressors in Chromosome 22 of his DNA. Typically everyone has two of these INI-1's in Chromosome 22, which is what prevents cancer growth. A rhabdoid tumor is where both INI-1's have been deleted in a cell and it starts multiplying with the modified DNA structure. The huge fear that Dr Dome eliminated today with Dr Beigel's result was that although the tumor cells do indeed have both INI-1 structures deleted (as is the defining case of a Rhabdoid tumor), his regular blood (and thus regular DNA) doesn't have this deletion. This means many things: Steven only has to beat this tumor once which is hard enough, but at least reoccurrence of the tumor isn't guaranteed. It also means that Jenn and I aren't carriers of this dangerous gene and thus Nikki isn't at any increased risk.

Monday February 26, 2007
We met with Dr Philip Guzzetta the lead pediatric surgeon for Steven's operation tomorrow. He had spoken to the Urologist and unfortunately with the need for removal of so much of the ureter it seems the kidney has to go as although nothing is believed to be wrong with the kidney there's just no way to connect it back to the bladder. Apparently the other kidney will enlarge to make up for the other one being absent, picking up 80-85% of the capability of a perfectly functioning pair.

Steven - Feb 25 2007 - Snow in DC

Tuesday February 27, 2007
We started the day starving Steven from 10am onwards ready for his operation, we arrived at the hospital at noon for the preparations for Steven's operation at 2pm. We were "standby-scheduled" for 2pm. By 4pm someone came out to tell us that there would probably be no space in the OR for us until probably 9pm, we could wait until then, or there was a cancellation at 7:30am tomorrow that was a scheduled (rather than standby) time. So after 6 hours of travel time and waiting we gave up for the day and fed him. We'll be back at 6am tomorrow to prepare for his 7:30am operation.

Wednesday February 28, 2007
The surgery was a lot bigger than we expected. Preparation started at 7am, and he was done five hours later. The magnitude of the surgery became clear during the preparation he was going to be even more connected up than ever before, he has an epidural for pain management, a breathing tube, a nasal tube for gas removal from his stomach, catheters for urine and bowel movements, several things connected through the broviac, and an additional IV was needed too. 

Things went well, but they removed lots more than originally planned. The tumor, the size of a large pear was removed, it was indeed encapsulating the ureter so the ureter and kidney were taken out. Additionally there were two new cancer focus areas, likely caused by a spill of the cancer cells during taking the biopsies in the earlier surgeries. One was attached to muscle tissue near his naval which was scraped off, and one attached to his appendix, so his appendix was also removed. The tumor was touching some of the lymph nodes in his right leg, a few of these were removed too. Finally the tumor had been resting on a large muscle in his hip so a segment the size of an adult finger was removed from that too (I think about 10-20%) of the overall muscle size.

Main Page
March 2007