Steven's Rhabdoid Cancer Blog

Diary of Events - December

Wednesday Dec 13 2006
We’re on vacation, our 7 month old, Steven throws up for the first time in his life. At this point it’s a minor inconvenience, Nikki our 3 year old has throw up many times, in fact on most vacations so we’re used to it.

Monday Dec 18 2006
Back at home we take him to his pediatric doctor. They have “seen 500 kids in the last six weeks with the fever and throwing up.” The hard tummy, which is bigger and harder than the day before is seen as slightly unusual, but presumed to be gas.

Sunday Dec 17 2006
Steven is still throwing up and has done so every say since Wednesday once or twice a day, including on the flight home. Although he’s looking pretty sad about it when it happens he seems to bounce back pretty quickly. Even though it’s now five days of throwing up, Nikki still has him beat with a prior round of 10 days. However, what seems strange is his tummy is a little enlarged and a bit harder than usual.

Tuesday Dec 19 2006
Surprisingly we get a call from his doctor, checking about his tummy, being worse they schedule an ultrasound for Friday morning.

Wednesday Dec 20 2006
Although he has not thrown up since Sunday Steven’s been having diarrhea about once an hour. Jenn had seen him growing only incrementally with every diaper change. When I came home from work I was horrified at how much larger he was than when I had last seen his tummy on Monday night. Immediately Jenn took him to Folsom Mercy ER at about 4pm. At this point we still figured we were going to be told it was nothing, just gas perhaps.

By 6pm Jenn called me and told me to come to the hospital the doctors were acting like there was something wrong. Minutes later after I arrived they were telling us they had done an ultra sound and he had a “huge malignant tumor.” Our world changed forever.

They needed to get him to UC Davis Medical Center in Sacramento by ambulance, but before they could do that they needed to get an IV hooked up. This was horrible, attempt after attempt failed, he was screaming as they poked around to find a vein. Eventually they gave up on attempting to find a vein for the IV and told us they needed to hook up the IV to his bone marrow, this was going to be painful and pretty harrowing. We couldn’t take it and had to leave as they had six people pinning him down to hook up an IV to his bone marrow.

He was transferred to UC Davis by ambulance and sent to the medium severity pediatric intensive care unit (PICU). Several stabs later he had an IV hooked up to his arm, looking much like a pin cushion now. Jenn stayed with him over night, where they took several blood tests, urine tests, a second ultra sound, and a CT scan. At this point we were hopeful that we’d have results to work with the next day and figure out what course of action would need to be taken.

Thursday Dec 21 2006
Cancer was confirmed, a 12cm diameter tumor. Things were going pretty slowly until about 4pm, he had been moved from the PICU to the ward (lower severity). However, unlike we had expected, the CT scan did not clearly indicate the tumor type, and a biopsy would have to be surgically taken. This was scheduled for Friday afternoon.

Around 4pm he seemed to be in more pain, and having trouble breathing, I alerted one of the residents, by 5pm he returned. Steven was now asleep but they wanted to run several tests, by 6pm things were trending downwards at an alarming rate. His hemoglobin (the red bloodcells) was at a level 4 (not sure of the measure of parts per whatever) but this was a third of what it should have been. The tumor was growing at an alarming rate – we were measuring the rate of increase by measuring the circumference of his abdomen, which was growing at the rate of an inch per 12 hours. What was happening was that the tumor was internally bleeding, sucking up his blood supply to feed the tumor’s loss of blood within itself and starving his body.

By 7pm the two attending doctors we had been waiting to see, his primary pediatric oncologist, and his surgeon were there. Things were going really badly, the tone of discussion rapidly changed to one of having a desperate problem that needed to be fixed right now. He immediately needed some major transfusions of red blood cells, plasma, vitamin K, and Novo 7 (a clotting agent). After several failed IV attempts. He was taken over to the high severity PICU, at this point he was at severe risk of heart, liver, kidney and lung failure.

Grammy arrived from Virginia around 10pm. The PICU staff had the multiple IV’s working by then, and the necessary drugs and transfusions were flowing. As I was getting a nasty sinus infection and didn’t want to risk getting Steven infected with my cold so went home to get some rest. Jenn stayed at the hospital overnight.


Friday Dec 22 2006
To my horror I was awoken by Jenn, given how Thursday night had gone, only one reason came to my mind as to why she was home. Fortunately she was home because things had actually improved substantially. Around 6am they had managed to get a second IV in, located in his right arm. His blood levels and clotting levels had returned to normal, although he was still on a transfusion to keep them normal.

I got to the hospital a couple of hours later to find the second IV that had been put in only 4 hours early had burst the blood vessel in his arm, and caused a horrible amount of hemorrhaging within his arm. He was deeply bruised from his wrist to his elbow for about a third of the diameter of his arm. For half that length it looked like a huge bleeding burn or blister. We were getting opinions on his arm ranging from "it’ll clear up pretty quickly" to "he’ll be scared like a burns victim for life."
 

Surgery was scheduled for the afternoon, we waited outside for four hours. They were taking a bone marrow sample, and intending to take a small biopsy for pathology to study, and installing a broviac (a high volume IV that’s hooked up to one of the veins going to your heart). It’s the main IV line used for all the chemo chemicals that are so toxic that they can’t be put in through a regular IV as they would damage the veins. It’s something he’ll keep for the entire duration of the chemo therapy.

Surgery wasn’t as expected, the tumor was highly necrotic (dead) and due to the extreme tension on his abdomem it ruptured when they opened him up. They scooped and sucked out 1.5 liters (3 lbs) of tumor from him, this was about 20% of his body weight. The surgeon spoke to us after the operation and told us this was rather strange and unusual that tumors aren’t typically this necrotic.

Having removed such an enormous amount made a huge difference to his internal organs, his lung capacity doubled, and his right kidney which had been significantly displaced moved back into a much more normal location, although was severely oversized (about triple the size of the left kidney). We hope this is only bruising rather than cancerous.

Coming out of the surgery he was looking pretty messed up, his arm damaged as above, an IV in the other arm, an IV in his skull (they have been looking everywhere for veins), a bigger one in his groin, and the big one now in his chest. Also he’s on a ventilator, an oxygen monitor, and a blood pressure monitor.

I asked the head surgeon how often he sees tumors like this, as he had mentioned it as unusual earlier. Bearing in mind he’s a 20 year veteran, and does several cancer surgery’s a day, it’s a bit unnerving that his response was “never, this was the first.”

They did an X-Ray of the broviac installation, unfortunately it showed some form of irregularity, upon testing it however it did work. A second X-Ray was done, this received mixed opinions as to whether it was correct or not. We’re ever so hopeful that the broviac is installed correctly, and that pathology will get some results on the biopsy quickly.

Saturday Dec 23 2006
The broviac was confirmed as correctly installed, and they were using it for a blood transfusion when we arrived in the morning. He looked really peaceful. By noon they had removed the IV in his head. He’s still on a ventilator, mostly for precaution, but that’s been extended through either Sunday or Monday, and I suspect they will keep him sedated until they remove the ventilator.

We heard that either the burns or the plastic’s department would be visiting in the next few days to look at his arm and figure out what can be done for the damage he endured there. It seems so unfair that even beyond the cancer other traumatic things are happening to him.

The initial scan of the bone marrow looked pretty good, Dr Ducore said that there was only one very small cluster of semi suspicious cells (out of millions of cells scanned). He and we are hopeful that this is a negative test, and will be confirmed as negative when the full bone marrow pathology test is completed next week. It will be excellent news if it hasn’t spread to his bone marrow, but also provides no further clues as to what the type of cancer is, and thus the treatment.

The pathology testing of the removed tumor will take until next week, so we’re basically on hold for the weekend and Christmas. Dr Ducore has been pushing the pathology department to get on with the analysis, but we’re at the mercy of their schedule.

In discussing with Dr Ducore the likelihood of when pathology will find results, he mentioned his biggest concern that too much of the biopsy may have been necrotic to get any results. At this point we’re still hopeful that pathology will have results by Wednesday and that we might start Chemo Thursday before new years day.

Today was a new health low for all of us. I took Nikki to urgent-care who confirmed she had an ear infection. Jenn has something wrong with her eye which she’s been putting off far too long, and I threw up twice in the evening not sure if it's stress or a bug, but I'll not see Steven tomorrow.
 

Sunday Dec 24 2006
Good news from the plastic surgeons, they think the damage to his arm will heal on it’s own over time, and didn’t think it necessary to perform any work on him.

He’s still on the respirator mostly as a precaution while his protein levels are recovered. He’s puffed up due to osmotic action of protein loss resulting in water loss from his blood to his skin, he’s rather bloated at this point, but he’s been improving today as his protein levels have risen. The primary reason he’s still on the respirator is incase his lungs get flooded.
 

Christmas Day 2006
He’s off the forced respirator (oxygen line down his throat) and was switched to a respirator mask by the time we left today.  

Today he was mostly stable, although his blood pressure is high so he’s on medicines for that.

Today he was hooked up to at least 13 things that I saw, two are simple monitors (Blood Oxygen and Blood pressure), one was the respirator, one is a stomach pump (fluids and air), urine-analysis, and 8 different IV lines that were merging into the two connected to him. There’s also heart rate, respiration rate, blood oxygen levels, and blood pressure monitor, and some narcotic pain killer. .

When we arrived in the morning his abdomen had inflated to a size larger than the peak size at which they performed surgery, they had already done an X-Ray to check the tumor hadn’t regrown at some insane rate or that there wasn’t horrendous internal bleeding – it turned out to be fluid retention and air in his stomach due to the forced respirator, and a continued fluid loss from his blood (due to protein deficiency). It’s got to be significantly uncomfortable as he looked like a beach ball. By the time we got there this morning he was getting something to drive the water retention down, and later in the day had a line hooked up to pump his stomach out through his nose (you’d be shocked how long a pipe you can simply shove down someone’s nose), after this his tummy became somewhat smaller and softer.

Tuesday Dec 26 2006
Some progress on the respirator, the mask is gone, now it’s a small air line that goes around his ears and into his nose only about a quarter inch, it’s not bad at all, and only bothers him a little.

The stomach pump is still connected, although not being used all the time, his skin is softer, but he’s grown quite a bit, he’s larger than yesterday, even more bloated. He now has a significant blood clot in his leg, and there may be issues with one kidney which would explain the bloating problem. He’s now 3 lbs heavier than he was before the surgery when they also removed about 3 lbs of tumor.

In general he continues to stabilize, he started feeding orally today for the first time in six days, he threw up the first time, but kept it down the second.

The broviac was determined as incorrectly installed today. It has been installed into a small vein, rather than the much larger vein it should be installed into, this will mean either adjusting it or installing a new one.

The IV into his groin has been mostly abandoned due to the blood clot, but as the broviac surgery is only a few hours away they decided to stop using it, but not remove it incase they need it during surgery. Instead, they switched over to a new IV that they put in his skull. The doctor putting that in tonight did a stellar job of getting it in the first time without hardly a whimper, thank you Cheryl. However, there’s a pretty bulky and heavy junction box attached to it, and far too close to his head – there’s only about an inch of line between his head and the box, such that it's basically sitting on his head. Every time he moves his head it tugs on this junction box of IV lines. They didn’t want to risk adding an extension tube to the line to make a lightweight connection to his head because the speed of flow of drugs to the line is so slow now that it would take 15 minutes to fill an extension tube, meaning he wouldn’t receive medications for 15 minutes and they thought that was too long.  As he could reach it with his hands, his hands were restrained.

Again nothing from pathology today.

Wednesday Dec 27 2006
What a difference one day can make, Steven had a wonderful day today.

He’s off any form of respirator, breathing normally, and has good blood oxygen levels.

He went to surgery again, his broviac was replaced: they put a new one in and removed the mis-installed one. They also removed the IV in his groin that had caused a blood clot. The IV that was installed in his head last night had already failed by this morning (he had pulled it out) so that’s gone too. So now he’s down to just the broviac which he’ll have to have connected permanently throughout the chemo for the next several months.

He’s still got the line going to his stomach, but it’s no longer being pumped, he breastfed today and had kept it down by the time we left this evening. Assuming there’s no problems tonight, the pipe should be removed by the morning, which will be great as that’s the last thing still taped to his face.

The plastic surgeons came back to check on his arm, again they confirmed it looks like he has a decent chance of healing with only a superficial scar and don’t think work is needed on his arm at this point.

He was taken off all IV pumped medications, with only TPN (nutrition) and lipids (fats) being sent via IV. This is a huge improvement from the 8+ things he was receiving by IV only a little while ago. If the feeding continues to go well he may be off TPN and Lipids very soon.

He’s suffered some kidney damage to the right kidney, it may be limited to bruising at this point due to pressure from the tumor, but he’s got a bit of a blood pressure problem as a result of it. The issue has been stabilized to the point that he’s able to receive oral blood pressure medicine and no longer needs the two IV medicines he was receiving for it so is making good progress there.

Bloating is down a good bit, he’s about a pound less today and looks visibly better, although still huge compared to normal.

Again no news from pathology though.

Friday Dec 28 2006
Lungs are good, pneumonia has been handled.

Kidneys are improving, but he’s still on two blood pressure medicines, one orally, one by IV (although not continuously), there is consideration that perhaps by tonight both will become orally administered, and only twice a day.

Blood clot in his leg is now being treated with shots to his fatty tissue (mostly in his legs) twice a day, this isn’t being considered a big problem.

His arm is healing up at a fast rate and looks much better already, we’ll have to see how bad the scaring works out and whether he needs a skin graft later in life, but at this point it’s pretty promising.

He remains on TPN (food) and Lipids (fats) through IV, but only because he’s not being breast fed 24 hours a day and continues to refuse to bottle feed, if we were taking him home he could now be taken off TPN and Lipids immediately.

Yesterday he was removed from the intensive care unit, and moved to the ward, good news because he’s getting better, bad news because he gets less attention from the staff (nurse:patient ratio has changed from 1:1 to 1:3).

He’s vastly more awake and alert, we’re now trying to spend 24 hours a day with one of the three of us being with him at all times.

He’s off pain killers and sedation, although occasionally getting Tylenol, he had that twice last night while I was with him.

Pathology results are trickling in, the latest update on the bone marrow pathology is the tentative statement of absence of one of the key indicators of neuro blastoma, knocking that off the list, or at least removing it from being the most likely. Based on two results that came back on one of the tests the leading most likely is an Ewing family tumor, this is an extremely rare tumor something like 1 in 20 million for his age group or a Rhabdo Myo Sarcoma

The bad news is that the pathology results were not fully conclusive, are being sent to a few other major cancer centers, and that we’re not likely to know the final results until late next week. If his kidney heals well enough to get off the blood pressure medicine by IV, and move to an oral administration we are talking about bringing him home before the pathology results are determined, and then bring him back for the chemo once that has been decided.

At this point it has been confirmed to a reasonable level that the tumor type he has isn’t a viciously fast growing tumor (except when it collapses and internally bleeds like last week) so understanding the exact type of cancer, and the right treatment path is vastly more important than jumping on any treatment path that may not be appropriate. 

Saturday Dec 29 2006
Nikki's first visit with Steven in the hospital went really well. We put both of them in a cart and towed them around the hospital with his IV system in tow.

Sunday Dec 30 2006
He’s doing very well now, in fact well enough that if the appropriate staff were around at the hospital working over the weekend we would have been able to bring him home today. As it happens, with the long weekend there’s no one around to discharge him or to get the supplies we need to maintain his health at home. So we should be bringing him home Tuesday.

Although he’s doing really well, and had lots of smiles today, he’s still taking shots twice a day in his leg for the blood clot which he hates and fights the needle making it all the harder to administer. He’s orally taking blood pressure medicine due to the kidney damage, but this is improving day by day. His arm has improved tremendously; I think he might be lucky enough to get away with just a smallish cosmetic scar at this point.

However the serious issue: we still don’t have a definitive answer as to what the cancer is, results continue to come in, but nothing is certain. Today the doctor showed me the CT scans taken before and after surgery. Horrifyingly the tumor that’s left is still huge, taking up as much as 50% of the surface area of his abdomen in one of the CT cross sectional slices. This is down from around 80%, so the volume of the tumor is probably a half or less of what it used to be, but it’s still huge and until we know what it is treatment won’t start.

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