Jonathan is in Remission

This is probably going to be my last post.  At least until three months from now.  I've been considering making the blog listable in blogger.com - more so as Jonathan is in remission.  Yep, you heard me correct!  Jonathan's last evaluation had all tests results as negative.  This is the third time his bone marrow aspirate tests were negative - even with the special staining tests from Stanford Hospital.  Also, the anomaly with the large uptake of the MIBG isotope does not appear to be related to tumor activity so the concerns from the second scan have been removed.

Here is the quote from an email from Jonathan's primary oncologist:

I think that ultimately we will call the MIBG scan as being ok.  the area of uptake in the liver has actually been there for a long time (almost a year, when i go back & look at the prior studies) and has not changed.  The nuclear med docs has been interpreting it as being activity in the bowel, but with the MIBG/CT images from last week it is pretty clear that it is liver.  However, the regular CT scan of the liver is normal (no abnormal lesions seen).  So I think the final answer from the nuc med docs will be that this is probably some kind of artifact or differential uptake in the liver, not tumor.

It's interesting that the timing of this news is at our two year Anniversary of when all this suffering started.  The main thing is that there is evidence to show that the cancer was steadily reduced and was getting less and less so hopefully this is it!  Although Jonathan is now in remission - we were given the opportunity to stay on active therapy for another six cycle ~ approximately 4 more months.  At the end of these six cycles Jonathan will have another full round of tests to evaluation whether he is still in remission or whether he has had a relapse.  Of course - if he has a relapse - I wouldn't consider it so.  For me, Jonathan must be NED (no evidence of disease) for at least a year before I consider that any results being positive a true sign it is that because the cancer came back vs a test from a previous evaluation being a false negative.

The timing of this news is good.  I think we - Gabby and I - have reached the end of our ropes with dealing with this treatment.  I had a big blowout with the primary oncologist a week after hearing the news - during a consult.  I was so mad I had to call a childhood friend to get his take on it - as he is a doctor as well.  What's funny is that I've always wished to be in the position where I can screw these doctors' over when they have a computer related problem, i.e. make them wait forever, don't take their advice, don't apologize when they are wrong, blah, blah, blah - but apparently other computer engineers are doing it for me :) 

I was telling this story to the family doctor that Gabby and I see and she was telling me that they are already experiencing it!  Their office recently went computerized and the transition has not been an easy one for them.  The few times I've been to the office after the transition - the actual visit takes longer because now they have to enter information into a computer during the visit - and of course - everyone is still getting accustomed to everything.  So, in a way, they are getting a taste, but it's not mean giving them the taste :( 

I guess this goes to show that it's really a good time for a break.  I know that if Jonathan's evaluation is negative in April - then we will definitely stop being on active therapy.  Although it's nice to be 'doing something' - it's more stress having to keep thinking about things -and we need a long break.

Results of re-evaluation tests

Interesting news!  This is the second time in a row that Jonathan's bone marrow aspirate (BMA) tests came back negative.  This is indeed a good sign - although I'm too numb to know whether I should be happy or not.  I think I don't hear enough (or any stories) about neuroblastoma survivors who have either not gotten a relapse and have lived to be an adult or survivors who may have had a relapse and is still able to live to be an adult.  My mind is too full of stories of neuroblastoma patients who are declared free of disease but develop a relapse - even after the major five year window has passed.  The only consolation I will have is that while Jonathan is declared 'free of disease' we will have a period of not having to do any treatments.  The only problem is that we could get lulled into a false comfort zone and hearing that he has a relapse later on would just be devastating.  I still haven't decided whether I will fight again if he does have a relapse.  I went through a period a couple weeks ago in which I was thinking that if his disease ever gets worse - we would do comfort care.  But, like all things, I'll cross that bridge when I reach it.

Although he has had a second negative result for his BMA - there is still one item that needs to be cleared up.  What follows is the text of an email from Jonathan's primary oncologist on her thoughts on his latest re-evaluation results.

The bone marrow was negative again - even with the synaptophysin staining - so we are 2 for 2 with clean results, which counts as a 'real' response to fenretinide, as defined by the study requirements - so that's great!

The MIBG scan is also probably fine, but there was a small spot of uptake that appears to be in either in the liver, possibly the gallbladder, or possibly retained within the bowel.  they needed to get SPECT images at 48 hrs to get a better view of it and unfortunately we couldn't get those pictures because they take longer to acquire & he couldn't hold still without anesthesia for that long.  When we went back & looked at his last few MIBG scans, this spot may have been there before, but it was chalked up to bowel activity (intestine running along the bottom edge of the liver) which is normal (the MIBG gets excreted via the bowel).  But the fact that it is present on several scans when you go back & line them up is both a little concerning and a little reassuring: active tumor usually does not stay exactly the same size over the course of months (that's the reassuring part) but its annoying that we have this spot now that we don't quite know what to make of.  Also reassuring that nothing abnormal shows up on the regular CT scan.  So to try to settle things, the next time he gets an MIBG scan we will do a special study called an MIBG/CT, where they get CT images at the same time as the MIBG & fuse the images so we can get 3-D localization of the spot that is hot on MIBG.  this test does not take the place of a regular CT scan, it just helps localize anatomy on MIBG scanning.

So its all good news so far, with the caveat that we need to sort out this finding on the  MIBG scan & we should be able to do that when we re-image him after the 6th cycle of fenretinide.

Jonathan has just finished his fifth cycle of the fenretinide drug and to date - he has had no visible or apparent side effects.  I'm so accustomed to having Jonathan go through some sort of 'hell' when receiving medicine that can threat him that it's a little strange to go through no hell with a drug that is suppose to help treat him.  I'm almost in disbelieve that this drug might actually be working - especially since everything is in an oral form and it's something we've been giving him at home.  I know we are slowly approaching our two year anniversary of fighting this disease and I'm curious to see whether Jonathan will be declared in remission when we reach that anniversary or not.  Regardless, I want to mark this anniversary with a special event - but I haven't figured out what it should be.  All in all it has been an interesting two years and I'm curious to see what 2007 would bring for us.

Struggling ...

I think we're biting off more than we can chew.  Or, maybe I was underestimating how difficult and time consuming it would be to have the kids in school.  Regardless, we are struggling to keep up.  When we had both kids going to daycare, before Jonathan was diagnosed with cancer, I couldn't remember it being this difficult.  I suspect part of the reason is that we are tapped out, but the hard part is that this is beginning to sound like a stuck record, i.e. being tapped out.  I almost feel like taking a month or two off from work and just spending time doing other things, for example, getting the house back in order, relaxing, exploring etc... but this is not something I would seriously consider right now.

In the past, and I still do it now, I've always been amazed at the ability of single-parents to raise and take care of their children and still go through life.  I wonder whether my situation is as difficult as their own or whether it is more difficult?  I think this is one of these times where not having family support is a disadvantage.  There is no - 'let's drop the kids off by the grandparents for the weekend', or 'let's ask the grandparents to take the kids to school for the next couple of days'.  The biggest thing that struck me was during our naturalization ceremony yesterday.  One of the presenters were reminding us (the candidates) how important this achievement is and to recognize the struggles that some people have had to endure to get naturalized.

Although the road to getting our permanent residency was not as difficult as most people, I do know our road to naturalization was a bit of a challenge.  We've had to go through two lay-offs - with the first lay-off happening during the 2001 recession and completely having all our finances wiped out.  I remember we almost had to leave California because no one was hiring and it was very difficult to get a job to bring in any income.  Does that mean our lives are any more harder than any other person?  I don't know!  The way I think about it is the stress that kids go through as they are growing up.  I remember, and what I see on TV helps re-enforces this, but when I was a university student (for example), I thought I knew what stress was - having to study for my engineering degree.  It's amazing how things are hidden from you as a child and you think your parents don't understand the stress you have to go through coupled with some parents telling you that you don't have any stress (at least compared to them) - is the picture that comes to mind when I try to put my life into perspective.  I frequently use this image when dealing with the kids.  I try not to belittle their life experiences and the stress that they are going through and I force myself to recognize that from their perspective they are dealing with as much stress that I, as a parent, have to deal with as well.

One of the things that puzzles me is how someone else story could provoke an emotional response.  When we (Gabby, the kids and I) went to the oncology (cancer) camp in the first weekend of September, there were morning sessions for just the parents.  I remember our first session on Saturday morning and each couple (or single parent) had to share their story, i.e. the story of their cancer child.  There was one other couple who had a child diagnosed with Neuroblastoma, and while the father started to go through his family's story, he started to cry.  For some reason that provoked a response in me as well - although I was good in holding back my tears.  I guess I was surprised that it would have provoked such a response and a part of me was a little disappointed that I was not able to bring it under control.

Besides myself, I can see how everyone else is struggling.  I feel it for Arielle as well and having gone through the oncology camp and learning about the effects on siblings, it has me more worried for her.  I guess this is one of those occasions where 'time will tell' and as with everything in the past I am just taking it one day at a time.

Another evaluation almost done

We've just completed all of Jonathan's standard tests to re-evaluate whether he still has cancer / tumor cells or not.  This time he had three tests done, the CT scan, the bone marrow aspirate and the MIBG scan (where he has the radioactive isotope injected into his body).  The CT scan and the injection was done last week Wednesday (11th Oct) with the first and second MIBG scan occurring on Thursday and Friday.  When we normally go for an MIBG scan, the second scan is always optional and we normally get a phone call at the night of the first MIBG scan to let us know whether we need to come back in for the second one.  This phone call is important not only to let us know that we need to come in, but to make sure that Jonathan remains NPO - doesn't eat anything after mid-night and stop drinking fluids about 6 hours before going under anesthesia.

Apparently this time, someone dropped the ball and didn't call us so we didn't keep Jonathan at NPO.  Around 1 pm I receive a phone call inquiring whether Jonathan and mom were on their way - to which I replied - nope.  What followed was an ensue of phone calls between various medical staff and myself.  Of course the medical staff recognized that they dropped the ball but they still wanted Jonathan to come in for them to try and get the scan with him awake. As an aside note: the only reason Jonathan goes under anesthesia for the MIBG scan is that this scan requires the patient to be still for anyway between 60 to 90 minutes.  Kids of Jonathan's age wouldn't be able to stay still so long so the normal procedure is to put them under.

Unlike the CT scan - which Jonathan does without anesthesia - Jonathan is adamant that he wants the 'sleepy' medicine, i.e. go under anesthesia for his MIBG scans.  Friday was no different, and when Gabby asked him if he'll do the MIBG scan without the sleepy medicine he said no and started to cry.  This was unfortunate and one of the things I know is that once Jonathan makes up his mind about something it is very hard to get him to change it.  The additional challenge in this case was that the idea of 'holding him down' wouldn't work because in this case we needed him to stay still and plus it was a scan so it wouldn't have been possible.  At this point I was asking Gabby, "do you want me to fight this battle and tell the medical team he wouldn't be coming?"

Gabby didn't want to have to make a trip to the hospital just to prove to the medical team that Jonathan wouldn't stay still.  They are supposed to take our word for it.  I fought the 'battle' well but in the end we got word that our primary oncologist wanted us to do it (regardless) - so Gabby took the kids to the hospital for Jonathan to get his second MIBG scan.  Afterwards I found out that Jonathan did the scan very well.  He lay quietly for the 15 minutes.  Apparently the reason he didn't put up a fight was that he was very tired and his back was still hurting from the bone marrow aspirate biopsy from the day before.  All I can say is that I feel like such an arse now.  I don't know if I'm going to battle for Jonathan's defense in the future.

We have one more test to do - an eye exam - but this test doesn't add any results for confirming whether he has tumor cells or not.  This test is to study the effect of the drug on the rods in the patient's eyes.  The last time we did the eye exam it was a very traumatic experience.  Both tests required Jonathan's eyes to be dilated but one of them required that contacts be placed inside his eye and subjecting him to bright flashes of light (and yes he was awake).  It required both Gabby and I to hold him down.  The silver lining for the tests on Wednesday is that he shouldn't be getting the (same) test with the contacts so hopefully it should be a less painful experience.

Dealing with Stress

What is stress?  How does one know if the pressures of being under stress has been removed or is lessening?  Apparently, for me, it is still there.  My therapist thinks I am very strong, i.e. can handle stress well but is this true?  For a while I was thinking that my stress levels would be getting lower but my therapist raised an important point - that by Jonathan having a chronic illness and not knowing which way it will go will forever be on my mind.  Not having any control and the possibility of the disease getting worse are important factors that are keeping my stress levels high.  In a way - the therapist is right.  Not a week goes by that I don't occasionally wonder about Jonathan's (future) outcome or having to deal with some issue regarding Jonathan's treatment.  I guess if I didn't have the worry about a family member having a chronic illness would have been one less thing to worry about and thus less stress.

I hate having to deal with this issue.  I thought my body and my mind would have adjusted and life would move on - but apparently it does not completely adjust.  Of course having to deal with stress is almost like the vicious circle of life.  It goes round and round and doesn't seem to end.  As my therapist says - having to deal with the birth of another child might also be adding to it.  Yep - it definitely is.  This could not have come at a worse time.  For a while I considered a permanent method of contraception but I have not brought myself to actually doing so as yet.  The thing is - if my situation remains the same - I definitely cannot have a fourth child - but at least I don't have to worry about that for at least another six months.

Like I said, the cycle of stress is like the cycle of life.  I suspect that with Gabby and I trying to adjust also means there is more stress in the home environment - especially when compared to other families who do not have a child with a chronic illness.  What this means is that we have less patience when dealing with the kids - which does not help the kids.  Over the last couple of weeks I've been trying to follow my therapist's advice in that I should be giving Jonathan positive reinforcement.  Instead of pointing out when he does something wrong, I should also point out when he does something right and commend him more often.  I'm also following the same strategy with Arielle but fortunately the same level of worry does not exist.

I do know that this cycle of stress has immobilized me a lot.  Every so often I consider whether I should be making a career change and know I have additional things to think about.  This, indirectly, is a bad thing as well, and it adds more to the feeling of not being in control.  Any decision I make now must always factor in Jonathan and his chronic illness.

Waiting for round #4 of Fenretinide Study to begin

Jonathan's next bone marrow aspirate has been delayed.  Instead of having it done this week as was planned two weeks ago, it has been pushed to after cycle #4.  The main reason being that it was not necessary to verify the results so soon and there is another (general) re-evaluation planned at the end of cycle #4.  I'm happy that it got pushed because it means one less procedure for Jonathan.  For some reason I'm more nervous now about Jonathan going in for small procedures than I was a couple of months ago.  Even the thought of him going in for (minor) surgery to remove his Hickman catheter makes me nervous, when I know full well that I should not be nervous.  I guess, in this regard, I'm happy that Gabby is the one to take him in because then I don't have to worry about the actual event or having to bother the nursing or medical staff with 'trivial' questions.

Last week, we saw more problems with withdrawing blood from Jonathan's Hickman.  Although we could push fluid in, i.e. for his daily flushing of Heprin, we could not 'draw back' blood.  A couple of weeks ago, we had similar problems and we were fortunate that using TPA helped with breaking down the blood clots but we didn't know if it would have worked again.  The question is whether the blood clot was at the end of the Hickman (in his artery) and if it was the case, using TPA wouldn't have helped because the clot isn't in the Hickman.

As an aside note: TPA is a powerful anti-clotting agent.  When a Hickman gets clogged, just enough TPA is pushed into the catheter to fill the lumens (lines) with TPA but not enough so that it enters his blood stream.  After allowing the TPA to remain in the line for a certain length of time - the TPA is extracted  out of the Hickman, thereby preventing it from entering the blood stream.  From this explanation it's clear that if a blood clot is within the catheter then it can get dissolved but if it's at the end of the line then it's more difficult to dissolve.

In Jonathan's case, we suspected that the clot is at the end of the line and while pushing fluid will push the clot away so that the fluid could enter his blood stream, drawing back on his line would cause the clot to block the entrance to the lines thereby preventing any blood from being drawn back.  Thus, with this second occurrence of this problem with his line - we were not sure if they would have to remove it or if some other (invasive) method had to be employed.  In the end they tried the TPA again, letting it remain in the lines for about one hour, and that seemed to have fixed things (for now).

This afternoon, Jonathan goes in to get blood drawn for his immune reconstitution tests.  The results from these tests will tell us how the recovery of his immune system is proceeding and whether his immune system is at the same level as a normal kid.  Remember that last year Jonathan had a stem cell transplant which wiped out his immune system.  By using his own stem cells, his body is now regenerating his immune system.  It was estimated that it takes at least 12 to 18 months before his immune system will return back to normal.

Other than that, things are going well.  The problems with the insurance company seems to have been resolved.  Apparently there was a contractal issue between Aetna HMO (my insurance provider) and SCIPPA (the medical group that acts on behalf of Aetna HMO for more common procedures).  This issue was causing our medical providers, such as Stanford, to not get paid for their services.  I remember the day that triggered my investigating to find out what was going on.  It was a Friday afternoon (the worst time for this to happen) and I got a letter in the mail from Stanford billing department advising me that services on January 19th 2005 were not being paid (by SCIPPA or Aetna HMO) and that this claim has been forwarded to their attorneys who are pursuing the matter.  Depending on the outcome of their investigation would determine whether I would be held financially responsible (although they didn't phrase it exactly like that).  At the time, my first assumption was that a (normal) billing problem had occurred and I'll just have to make some phone calls on Monday to get this resolved.  Little did I know that up to two months later I would still be working on this, but by that time Aetna HMO had recognized what was going on and was taking steps to alleviate the situation.

Other than that, the kids and us are still adjusting to the new rhythm of things.  Waking up early, going to school, doing homework, etc...  We've been lucky so far that most of Jonathan's procedures have been scheduled to occur either after lunch or later in the day so that we don't disrupt his schooling as much as possible.  Both kids are enjoying school, making new friends and learning a lot.  Hopefully things continue along this uneventful way with no major (negative) surprises in the near future.

Fenretinide Study - Cycle #3 almost complete

Wow! A lot has happened since my last post.  For the first time ever, I now understand the difference between the marrow and the aspirate tests and how it relates to Jonathan's results.  When Jonathan goes for a Bone Marrow Aspirate - they are actually taking a piece of the soft bone (not the core) and some of the liquid (marrow) as samples.  The soft bone is the bone marrow test, while the liquid is the bone aspirate test.  When the previous set of tests came back positive it was the bone marrow that was coming back positive - not the aspirate.  So the way I picture it now is that there are NB (neuroblastoma) cells that have stuck on or clung to the inside of his bone and we're trying to clean (scrub) it away vs NB cells that are free flowing.

The interesting news was that the results from his last bone marrow aspirate test came back negative - even after the staining test.  Again - similar to what happen in May - I am being cautiously optimistic.  Our oncologist has already scheduled another bone marrow aspirate test in three weeks - which happens to be next week Wednesday (20th September) - so this will give us more definite news on whether this is a false negative or whether it looks like it might finally be clearing.  What's going to be interesting is that we may have the bone marrow and aspirate taken from a different spot.  My concern is that the same area is being sampled all the time, so that area must be getting soft.  With the requirement of six months for the sample area to heal, then it means the areas on his back is not getting healed.  So this time the samples will be taken from the front of his hips.

Other than - we went to camp Okizu over the labor day weekend - 1st to 3rd of September.  All I can say is that I'm glad we went.  It complete blew my mind.  Arielle loved it so much she was crying when we had to pack up and leave.  She was a little upset the next day (for a little bit) that we had to leave.  I'm glad the kids had so much fun.

So Camp Okizu had a family camp for families where someone is either undergoing active treatment or has had cancer in the past.  The facilities have lodges but we didn't sleep inside but on the porch.  So the kids got to sleep in sleeping bags with the sky as our ceiling.  I think that was the most awesome part because with the hustle and bustle of life it's very easy to forget what nature is like and just being able to slow down a bit.  Our meals were taken care of as well - so yes - it wasn't a real camp but it did serve as a good introduction to what camping might be like.  We also learnt that they run an Oncology camp - just for the patients - and a SIBS (Special and Important Brothers and Sisters) camp - just for the siblings of patients.  We're hoping that Jonathan will go to the Oncology camp - the major challenge being able to convince him to leave mommy and daddy behind and spent a week in a strange place.  Arielle is too young though - the minimum age is six so she'll have another year or two to go.

We also had support sessions on both days (Sat and Sun) for the parents.  It was interesting hearing everyone's story and the challenges they had to go through but the eye opening was learning about the problems that siblings experience.  Either feeling like the second wheel, or that their parents don't love them, or that they are not important and that the cancer patient gets all the gifts.  We suspect that Arielle has some of those issues so it makes us realize that this is something we have to mindful of and try to treat them the same.  The classic example given was the example of when you hear your kids cough.  It's almost second nature that if the non-cancer patient was coughing then there wouldn't be much concern - sort of like "oh, it's only you that's coughing".  If it was the cancer patient that was coughing then a totally different story.

First week of school & re-evaluation

Wow! A first week of school is almost complete!  The kids are doing great!  I think between the two of them - Arielle is having a lot more fun at school than Jonathan.  On the first day - Arielle was all bubbly and smiles, while Jonathan was Mr. Serious - as usual.  I took the video camera with me to record the events of the morning - from waking up from bed to going to class.  I even recorded a little bit of morning assembly.  When we had first arrived at the school, we dropped Jonathan off first - because his classes start at 8 am and then we went over to drop Arielle - her classes start at 8:15 am.  What Jonathan didn't know was that we were sticking around for morning assembly.  It was interesting to see Mr. Serious in the line for morning assembly and then when he looked around and spotted me.  At first there was this look of surprise and then you saw this little crack of a smile.  The Pre-K class doesn't attend morning assembly - it just the kids in Elementary school.

On Tuesday, Jonathan had his CT scan and also got the injection of the isotope for the MIBG scan.  This was the first time Jonathan did not have to drink any oral contrast.  He only got the contrast that they inject into his Hickman during the actual scan.  On Wednesday, Jonathan had his bone biopsy aspirate and MIBG scan done.  This was the first time, in a long time, that he didn't have to go for the re-scan / second scan - which would have taken place today.  What is interesting is that he has been complaining about his back hurting a little bit though - both last night and this morning - but I suspect it's because he is not keeping himself quiet and his being active is aggravating the incisions from the biopsy.

The other interesting thing is that for almost each day we have had to give 'instructions' to Jonathan's Kindergarten teacher.  For example, on Tuesday and Wednesday he couldn't eat any food or drink any liquids because he was going under anesthesia later in the day.  What struck me was that I was wondering whether Jonathan would have gotten the same level of care and concern if he was in a public school.  This helped me realize that there are other advantages to the kids going to a private Catholic school - especially a relatively small one like St Lawrence.  I'm just glad both kids have teachers that care for them and that Jonathan's teacher is able to handle Jonathan's extra special needs as well.

Tomorrow we get ready to go to camp Okizu.  It's a special camp for families with kids with cancer.  I've never been on a camping trip - at least none that I could remember - but I figure if I like this weekend's experience then we could look at making more camping trips next year.  The cool thing is that we don't have to worry about food and there will be lots of people around to lean on for support so it will definitely make our first time experience of camping an easier one.

Fenretinide Study - Cycle #2 almost complete

Today is the last day for Jonathan to receive Fenretinide for cycle #2.  Nothing significant to report - no side effects (that we can see) and Jonathan is 'chugging' along just fine.  You can get more information about the study Jonathan is on by visiting the NANT (New Approaches to Neuroblastoma Therapy) website http://www.nant.org/nant2004-04.shtml

Next week is going to be interesting.  Besides starting school, Jonathan is having a full re-evaluation next week.  He gets the full works - CT scan, bone marrow aspirate and MIBG scan.  A couple of weeks back I was asking the medical team to consider skipping this evaluation - I just didn't want to have Jonathan go through the hassle with the bone marrow aspirate - having to get two needles stuck in his back to withdraw bone marrow.  Unfortunately my 'request' was denied as having the re-evaluation is part of the study / protocol.

I don't expect much changes to occur in the results.  I'm very skeptical that he is free of disease (at this early phase) and I'm a little concern about the fact that they were able to see tumor cells without using the special staining tests.  I guess we'll know more in about two weeks time.

Fenretinide Study - Cycle #1 almost complete

We've started the Fenretinide study.  Actually, we're almost finished the first cycle.  The treatment plan is that Jonathan will receive the drug for one week, then get a rest for two weeks and then repeat the cycle.  He is going to have six cycles in all.  If after the sixth cycle he still has tumor cells but the disease has not gotten worse, then he'll probably repeat this study - provided there were no adverse effects and the study is still open.  The cool thing about this study is that it is - by far - the easiest treatment plan we've had to date.  There are no hospital stays, no worries about his blood counts dropping and no worries about his immune system being affected.  The hardest part is the first and last day while he is receiving the drug because Gabby has to spend the entire day at the clinic for blood draws through-out the day, but I believe this only happens for certain cycles.

The first day that Jonathan received the drug was Thursday July 27th, and we were required to be there at 8 am in the morning.  This was a little tough because Gabby has been feeling more nauseous of late (for those of you that didn't know - we are expecting our third child) and we knew it was going to be a rough day for her.  Since this was an early morning event, it coincided nicely with the time I like to leave for work.  This meant I could have driven everyone up to LPCH so at least Gabby didn't have to deal with the additional stress of concentrating on driving - especially when a nausea episode hits her.  So, I drove to the train station and then Gabby and the kids headed over to LPCH.

For this particular study, the main goal is to test a new delivery mechanism, i.e. the drug is now in a powdered form vs being in a tablet form.  The powder form is mixed with Slim Fast and then the solution is ingested.  The problem is that the powder does not dissolve completely, so there are 'lumps' in the Slim Fast.  Well, Jonathan did not like the lumps and he behaved so badly when he had to take the medicine on the first day.  In order to get him to take it, it had to be forced down.  So, the usual process was adopted, i.e. we had three nurses, plus Gabby, hold him down to try and force feed him.  Of course, during the process, he was spitting it up, and my concern was that he was spitting it up on Gabby.  Although there isn't any clear evidence for Fenretinide, we were treating it just like Accutane (from his previous treatment), i.e. a pregnant person coming into contact with the drug could cause very, very, severe birth defects for unborn children.

After that episode in the morning, Gabby was stressed out.  We spend the day trying to decide whether to put an NG tube in him so we wouldn't have to deal with this struggle.  A huge portion of me was against this idea and I firmly believe that Jonathan is getting to be a big boy and must be allowed to make his own choices.  Thus, if he doesn't listen, then he'll have to accept the consequences.  Since Gabby's nausea was still acting up, and probably got worse because of the episode with Jonathan earlier in the day, the plan was for me to meet them in Palo Alto (after work) and then drive everyone at home.  Then I can give Jonathan his second dose (at home) and verify whether he was going to be a 'nice boy' and take his medicine without much fighting.

The second dose went down much easier than the first, but it involved daddy being more strict with him :) By the next day we had got it down really cool and then by the weekend I figured out that I didn't need to use the syringe to feed him but could spoon it instead.  Now, he has three more doses to go before we finish cycle #1 and it's going really well.  I've come to realize one thing with Jonathan, he is just like his daddy when it comes to trying out new things.  Sometimes, there is this huge mental block and the challenge is getting over the mental block and moving on.

Preparing for Fenretinide Study

The dice has been rolled, the decision has been made ...  After a very hectic week (which hasn't finished as yet) we're on our way to almost starting the Fenretinide study.  We're hoping by early next week to get final confirmation on when Jonathan can start the study.  On the plus side, this study can be given at home so there aren't any planned hospital stays and clinic visits are just to check in or get lab work done.  On the negative side, the testing for Jonathan's eyes are a painful procedure.  There are two sets of tests, the first one is where they dilate his eyes and measure the point at which he cannot see light in a dark environment.  The second test, which is the more painful one, is where they put contact leads in his eye.  The leads forces the eye lid to remain open and Jonathan is exposed to bright flashes of light - similar to the flashes from a camera,  The leads in Jonathan's eye, measures the eye's response to the light.  So, he has to deal with the pain of the contact leads in his eye plus having a dilated eye that is exposed to very bright (flashes of) light.

On Wednesday (19th) we had our consult for the Fenretinide study.  What is interesting is that there is a page for patients who are not adults, to sign off on, but Jonathan is still to young to be able to make decisions so right now Gabby and I make all the decisions about whether to start a study, etc...  I was just reading a post from a Dad whose daughter was diagnosed at 6 (six) years and she is now 13 years.  Here is a portion of it:

Anyway, as suspected she has a 3cm tumor that developed in her shoulder, this is where it get's real CHALLENGING, Bre is 13 yrs old and is VERY head strong, for those of you who know her, knows what I'm saying, We have been floating around in relapse land for about, oh a year and a half at least, for the most part she has felt pretty damn good. Now that we have a new development Dr. Kushner gave us some options 1) IV chemo ( top/cytox) 2) oral fenretinide 3) oral VP-16 and topotecan. I know he prefers option #1 and of course, I want the most effective approach for her but given that she is so determined about NO MORE IV CHEMO, NOT ANOTHER BROVIAC ( Hickman), I'm not losing my hair again, ect, ect... I know I can't convince her that it's the right thing to do. Then again, is it?!?! It was much easier when she was 6yrs old, I just made the decisions for her, when it comes to treatment. Now I think she asks more questions than us, and of course she is SO ANGRY and feels like she's had enough, At the same time, she hasn't had aggressive chemo for almost a year and is pretty damn strong,  never ending, What to do.

What is scary is that there is going to become a point in time when Jonathan will be old enough to make decisions AND those decisions may not align with Gabby or my own.  It will definitely be  interesting in the next couple of years, given that Jonathan is so head strong himself.

It's so funny, as part of our consult on Wednesday, Jonathan was getting checked out to make sure everything is going fine.  The nurse wanted to look inside his mouth but Jonathan refused.  He wanted her to check his ear.  He has this pattern that 'must' be followed.  The amusing thing - while I was watching him - was that he doesn't say anything, he just turns his head to give the ear and if you are not aware of his antics you wouldn't understand what he is doing.  So, like I said, Jonathan can be very head-strong himself.

It's interesting, at our last visit to the therapist I was talking about how headstrong the kids and be - both Jonathan and Arielle - and he was saying that was a good thing!  His point is that when they become adults they will be assertive, ask questions, not be pussies (sorry cannot think of the clean word right now).  The disadvantage is that headstrong kids make it hard for parents, i.e. more effort required, but, for the longer road, it's better.  I could see his point but it means a lot of parenting so that they don't deviate from the 'straight and narrow' road.

I must say that with us recognizing that we needed to spend more time with Arielle - things have improved tremendously.  No more writing on the walls :) which is definitely a good thing.  I can feel my relationship with my daughter slowly getting back on track and growing.  She is not just a mommy's girl anymore.  Of course, Jonathan and I are close and will probably be for quite some while.

The last interesting thing from our consult with our oncologist on Wednesday was regarding his bone marrow aspirate test.  We are hoping to use the results from the bone marrow aspirate tests to start the Fenretinide study, but if we cannot start the study by next week then we may have to do the bone marrow aspirate tests over.  The significant think about this last test was that they were able to get a cluster of cells BEFORE doing the staining tests.  Part of the requirement for getting on the study is that the patient must have measurable disease BEFORE the staining tests and if we have to repeat the bone marrow aspirate test then we may not get those results.  Now, your initial reaction could ask whether his cancer is starting to come back - because this is the first time (in a long time) we have see tumor cells without the staining tests - but for now we are going to chalk it up to due to sampling.

In my next post I'm going to write about my adventures with the insurance company :), getting authorization and working with Stanford.

Preparing to start treatment ...

A lot has happened this week.  Earlier in the week we got confirmation that Jonathan is going to take part in the study and that the earliest the study may re-open is the 24th July.  This is great news and in anticipation of him starting the Fenretinide study, we have begun his preliminary testing.  The big issue concerning me about starting this study is that one of the side effects of this drug causes night vision loss.  Initially, when I heard this, I didn't have a good handle of what this meant.  When Jonathan had his autologous peripheral blood stem cell transplant, I accepted the fact that he'll have permanent hearing loss, but for some reason, having vision loss is something I was not swallowing very well.  I've always said that if I had to loss any of my senses, let it be anything but my vision.

The first set of testing to be done was to get a baseline of his eye sight.  Last week Wednesday I got an email from my oncologist to setup an appointment to see an Opthalmologist.  Setting up the appointment was an interesting experience.  Apparently the number I got from my oncologist was a doctor's line, so half way into the conversation I was asked to hang up the call and call the 'regular' line :)  Then, when we reached the part to setup the appointments, the person at the Opthalmologist office was going to take a look at his schedule and give me a date but I stopped her and said that according to my email the appointment was suppose to be this Friday 

Let me pause here for a sec.

For those of you familiar with dealing the medical staff, especially the front end people, they always live to feel like they are in control.  Wait, that's an unfair assessment, but we've met so many different types of people that my tolerance is quite low.  I'm a no nonsense kind of guy now.  I don't expect you to like me, but I expect you to do your job.  Maybe I'm more cynical now and less friendly, but you know what, in the normal world of medicine, it's like pulling teeth to get any where, so it's my turn to pull teeth.  I truly understood, during my conversation with the person at the Opthalmologist's office that she was 'not in control' because my son was coming in as a new patient and she didn't have any background on his issue, but I was not going to let her give me any crap.  The last thing I was told was that both parents had to come, to help hold Jonathan down.  One parent was to hold his body, while the other was to hold his head.  Little did I know how important that request would be, but little or no explanation was given either so I said that Gabby would be the only parent present.

Pictures from Make a Wish Trip

We're at MGM Studios (I think) and the kids and I are taking a picture with Goofy. Gabby wasn't into hugging up the characters so she is taking the picture of the kids and I with Goofy. Good thing too, since I'm usually the one taking pictures so this time I get to be in one :)

Playing golf at Give Kids the World

The kids are playing miniature golf at Give Kids the World - a non-profit organization that works with wish organizations like Make a Wish to help fulfill a wish child needs. Miniature golf happens to be one of Jonathan's favorite games - and Arielle's too - except in this case everytime the ball goes in the hold - there is a surprise :).

Miniature golf surprise

One of those surprises I was mentioning in my previous post. The 'dragon' pops up and breaths steam. Notice that Arielle is no where to be seen, because she got a terrible fright.

Riding the roller coaster at Seaworld

Jonathan went on this roller coaster with Gabby and he loved it so much he insisted that I had to come too. For this ride, we had him wear a rain coat to protect his chest from getting wet. Jonathan is truly a trill seeker and loves the 'wild rides'.

Some good news - might be getting on the Fenretinide Study

Hot off the press this morning!  It seems that the Fenretinide study MAY reopen at the end of July and that Jonathan MAY have a spot to get on the study.  We'll get the final answer by Wednesday but this is some nice positive news to start the weekend.  At least we don't have to look at re-starting the accutane / cis-retinoic acid right now.

Just a little explanation on the whole study process.  All / most of Jonathan's treatments are experimental treatments.  None of them are tried and true and what this means is that most of these treatment options are not generally available to the public or 'most' hospitals.  Since the treatments are experimental, it means that they are conducted as a study since the patients taking part need to be closely monitored.  Just like any 'scientific' study, there are more rigid rules to follow - to get into the study, while doing the study and when the treatment is finished.  As in any study the purpose is to figure out whether this treatment plan works.  Some of the typical questions they are looking to get answered include: what is the correct mix of medicines or dosages to use, what are the possible side effects, are the drugs effective, etc...

As I was getting ready to submit this post - I got another reminder - and a good one at that - that until we know for sure - we have to be prepared for the possibility that Jonathan may not actually start the study at the end of July.  We just have to wait and get the final verdict next week Wednesday.

Results from 2nd biopsy

This is going to be my shortest post to date.

I got the results from Jonathan's biopsy this afternoon.  Unfortunately some tumor cells were still detected which means that Jonathan is not in remission.  The results from the test were the same as the last time his tests came back positive.  Thus, the tumor cells were only detected via the special staining tests and it was a small number of cells.  Normal tests did not show any tumor cells.

Not sure what are the next steps.  The study that Jonathan would have started is currently closed - Fenretinide.  Over the next couple of days we'll get more information from our primary oncologist on what the next course of treatment would be.

General Update

Yep ... it's been a long time since I've posted any news about Jonathan.  I've written about two to three entries but didn't quite like the flow of thoughts, so never bothered to post them online.  Hopefully this one flows much better :) I think it is a case of writer's block, coupled with just being exhausted!

We came back from our Make a Wish trip on May 22nd, which also coincided with the last dosage of Accutane.  This meant we had reached the end of the ch14.18 (immuno-therapy) protocol and Jonathan was to begin his re-staging / re-evaluation tests later that week.  This time he needed four tests - the CT scan (on the 24th), the Bone Marrow Aspirate (on the 25th), the Bone scan (on the 30th) and the MIGB scans (the 31st to the 2nd).  On the 1st June, we had found out that his CT scan and Bone Marrow scans came back negative.  We had expected all the tests to come back negative - except for the Bone Marrow - so it was interesting that the bone marrow came back negative - since the special staining tests have been really good about showing tumor cells - even though they were in very small quantity.

Getting the news that all of Jonathan's tests came back negative was received with mix emotions.  For (almost) any other cancer - I would be jumping for joy and celebrating that Jonathan had been 'cured' - or at least his cancer had gone into remission - but given the likelihood that the bone marrow aspirate might have been a false negative meant I couldn't celebrate as yet.  We had already discuss (in the past) the next steps in the event that all his test results came back negative - and the action plan was to repeat the bone marrow tests more frequently for at least two or three more times.

It's now been about a month, and Jonathan is scheduled for his next bone marrow aspirate test later this week - June 29th.  I'm anxious to get these results because another negative result will give me a better comfort factor for believing that his cancer has truly gone into remission.  The strange thing is that if his test comes back positive - then the medical team will see that as a relapse - when in actual fact (in my mind) it is not a relapse but that the tests done in June were false negatives.

If this test is negative, then Jonathan has two more bone marrow tests to do.  One at the end of July and the other at the end of August, with the caveat that the one at the end of August is a re-staging / re-evaluation round, i.e. all four tests would be repeated - CT scan, Bone scan, Bone marrow aspirate and MIBG scan.  I think if the results from the July test remain negative - then we'll throw a big party in August - but I'll have to remember to separate that party / celebration from Arielle's birthday party so that Arielle gets her time to enjoy her event.

General Update

It's the first of May.  Amazingly what comes to mind is that this is the month of Mary - probably because I read it in the church bulletin last night.  Should be an interesting month.  We have two birthday's this month - Jonathan's own on the 11th and my own on the 29th.  It's funny - but this is the first year that I got included into the monthly birthday celebration at work.  The previous (two) years - there was always something going on - so I never got the 'honor' : ).  This is also the month that we go on our Make a Wish trip - a much needed vacation that we are all looking forward too.  Lastly, this is the month that I 'officially' turn pro with my photography : ).  So it is definitely shaping up to be an interesting month.  I'm hoping to truly cherish each day and enjoy it to the fullest.

Jonathan has been going well.  On the 20th of April - Jonathan got sick (again).  He developed a fever (suddenly) on Thursday - with chills - and was admitted to the hospital.  He got another bacterial infection - but this time it was different - it was Ecoli (spelling?).  This was an interesting development - and although I wasn't any more concern that his other bacterial infections - Gabby was more worried - primarily because of the particular bacteria.  What was making me/ us more nervous was that we had finalize arrangements to leave San Jose on May 3rd to head over to Orlando - and with this latest bacterial infection it was definitely going to affect our plans.  We were not sure whether we would have to postpone for a couple of days or cancel the trip entirely.  We had these 'hard' requirements of wanting to go in May because we wanted to avoid going to Disney World during the summer, partly because of school closing thereby having lots of people, and partly because it would be hotter and during the hurricane months.  As much as people (outside of California) make fun of our earthquakes, I think I'll take my earthquakes any day over the hurricanes in Florida : ).

By Saturday (April 22nd), Jonathan's blood cultures (from Friday) were clear - so we knew the antibiotics were working and it was just a matter of finishing his treatment - which actually ends today (10 days later).  Last week, some more blood cultures were done and everything is still clear - so all looks good for our leaving on May 6th - our new date to start our vacation.  What's interesting is that his antibiotics finish today, he gets blood cultures done on Wednesday, and then on Friday we get the okay to leave on our trip which is on Saturday.  I know, I for one, will be very disappointed if I geared up the week to leave and I find out the day before that the trip is off.  Yes, I know, I wouldn't have a choice, because Jonathan's health is the primary focus - but you know - human nature - when you are looking forward to something and then it gets pulled right under you.

Jonathan also had his immuno-studies done.  This was a 'first' look to see how his immune system is recovering.  During his stem cell transplant - one of the goals was to wipe out his immune system - so about six months after transplant they test to measure how much his immune system is recovering.  So - it more than six months - eight months to be exact - and his results were promising.  All his indicators are showing activity but his immune system is still not there yet.  What it does mean is that we can start relaxing some of the restrictions we had including, not wearing the mask as much, meeting more people, going into some enclosed environments, etc., but he is still immuno-compromised so we still have to be careful.

Wait - almost forgot - some significant happened two weeks ago.  Jonathan does NOT have his NG / NJ tube anymore!  When he got sick two weeks ago - he vomited out his tube and he was insistent that we don't put another one back in.  Since then he has been taking his oral medications without too much fuss, and his appetite has been slowly improving.  We still have a lot of work to do with getting his appetite back on track - but one of the reasons I didn't want to press putting in another NG tube was that I wanted to get him more into the habit of eating on his own and not having to depend on his 'liquid' diet.  As we were telling the nutritionist last week, we have to 'strongly encourage' Jonathan to eat all during the day.  The only time he doesn't need any encouragement is in the night - and late into the night - when his appetite picks up.  The hard thing is that Jonathan needs at least 1400 to 1500 calories a day - more than the average person.  This is to ensure that his body is getting enough nutrients so that it can heal - but if we don't keep it up - then Jonathan starts to lose weight.

I can definitely say that although we have our good days and our bad days - it seems to be slowly getting better.  I know it's more difficult for Gabby to get him to eat than when I'm at home with the kids, but with our vacation right around the corner, the timing works out well so that I'll be able to spend the next 2.5 weeks working on his eating.

The only other medically (significant) event was that we (Gabby and I) got chewed up last week by our primary oncologist and primary nurse practitioner : (.  We even got a 'visit' by our social worker : ).  The entire issue stemmed around a prophylactic antibiotic medication that Jonathan is supposed to get on the weekends.  By prophylactic it means a medication that is taken to prevent disease from occur and not that there is a disease that requires treatment.  Well, we haven't been giving him his medication, Septra, for months and the medical team was a bit concern (to say the least) that we had stopped giving it to Jonathan.  I think a lot of reason was due to our forgetting, primarily because it was not a 'regular' medication that he gets everyday, and partly because by it being prophylactic there is less concern / priority about giving it to him.  Needless to say - we've started back giving it to him now - so all should be well.

Sad news on the ACOR message boards

I read today that Nick Snow - a neuroblastoma survivor died yesterday (or recently).  His story and his mom were a source of strength and I was thinking that if Nick could have survived then Jonathan has a chance.  Now I'm not so sure.  Please remember angel Nick Snow.

Here is the email confirmation sent to the group:

I have been asked by Shannon to confirm to the list that Nick passed away "peacefully" with no pain and surrounded by people that loved him. Cause of death was a perforated bowel and severe pancreatitis.. I am sorry I cannot write more, but words just are not in me now.

Iris

Here is an email I sent to Nick's mom last year November:

Hi Shannon,

Sorry for the intrusion but I didn't realize you son went through so much!  I don't know how you survived 84 months of treatment - but I've only been through 11 months and I don't know if I can go through another year - much less 6 months.  It's good to hear about a survivor of NB - it sure does put a little light on at the end of the tunnel.

Best Regards,

Walter ...

----- Original Message -----
From: SHANNON SNOW
Sent: Sunday, November 27, 2005 1:47
Subject: Re: Med: bone marrow not  clearing

Hi Christina,

It sounds like you have thrown just about  everything at Michael's tumor.  How much is still in his bone  marrow?  Has Michael taken the Accutane yet?  Have you looked into  Fenretinide?

Now seems to be a reasonable time to check out some  clinical trials.  Especially if Michael doesn't have rapidly progressing  disease.  You never know with a clinical trial............maybe your kid  will be the first to benefit from something new, and then down the road, that  something new will be standard treatment for other kids.

I snagged this  off my son's website so I don't have to retype it:

Nick (age 16 1/2)  underwent 84 months of treatment (chemotherapies, surgeries, 4 types of  radiation, a bone marrow transplant, vaccine therapy, gene therapy, many phase  1 experimental therapies), and is finally in remission! 

He has been  off all therapy since December 2002 and is still well. 

Almost  everything Nick did was a clinical trial, and some of the therapies are now  standard treatment.

You can check out www.NANT.org <http://www.NANT.org>   and then click on 'clinical  trials' and see what is new.  Sometimes it's really hard to decide what  to try, but you just have to follow your gut feelings and go for  it.

Please keep us posted about Michael.  Does he have a  website?

Shannon: mom to Nick Snow

http://www2.caringbridge.org/ca/nicksnow

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Delays for round 5 - bacterial infection

This has been an interesting week.  The original plan was for Jonathan to be admitted on Sunday - 26th March - to start his immunotherapy treatment on Monday - 27th March - but, unfortunately, that didn't happen.  On Sunday afternoon we 'discovered' that Jonathan had a fever.  Just after 12:30 pm, Jonathan's temperature was around 102.3 F.  We took his temperature again just after 1 pm and it had risen to 103.7 F.  This wasn't good.  It meant that we had to call it in.  Since we were already going to be admitted to 2 North - I thought we could have just checked in earlier.  What I found out instead is that the 'Fellow' (and the Attending) wanted us to go to ER to have them treat Jonathan first, prior to being admitted to 2 North.

In the past, we've been told that one of the reasons why we go to ER is that 2 North cannot admit directly.  My thinking was that we could go directly to 2 North, this time, because Jonathan was already scheduled to be admitted.  Unfortunately that didn't happen.  The reason told to us is that ER would be more prepared to deal with a kid 'crashing'.  Now, the reason I was opposed to going to the ER was that it takes so long, and the doctors there are not pediatric doctors so their manner of treating kids is not the same, etc, etc.  But I was assured that it wouldn't take very long and that there was a new section in ER for kids.

Going off topic - so that I remember the events clearly.

When I first called to report that Jonathan had a temperature of 103.7 F I was in disbelieve that the doctor actually wanted us to go to ER and not to 2 North.  I made sure that he was clear with explaining his logic to me, i.e. 2 North not being able to treat kids that are 'crashing'.  Although his logic made sense - his logic is based on the 'general' rules and IMO if you have proper continuity of care, then doctors would be familiar with the specific traits and previous history of the patient.

My next call was to the ward directly, 2 North, to verify if the room was already available.  I wanted to verify if there was another motive for having us go to ER without telling us.  The room was not ready, and it was going to be ready by 6 pm because a patient was checking out that afternoon.  During this call with the charge nurse, the 'Attending' came on the phone and I got the entire explanation again - but this time I was told that there is a new 'wing' just for pediatrics - so our experience would be much better.

In the end - I don't think there were any other reasons that the doctors just wanted to play it save - and given who the doctors were that night - conservative - it made sense for their decision.

By Monday we found out that Jonathan's blood culture had grew out the 'staff' bacteria.  This made it the fourth time that Jonathan has gotten this bacteria.  He got it in rounds: 1, 2, 4 and 5.  By Monday night or Tuesday - we found out that his urine sample had also grown out another bacteria - so Jonathan was on antibiotics to treat both types.

Originally, when we found out that Jonathan was having fevers, we figured that they were due to Jonathan 'catching' Arielle's cold from last week.  During the previous week, Arielle had fevers, runny nose and some coughing, and try as you might to keep the sick person separated, once one person in the family gets sick, the entire family gets it.

By Monday / Tuesday, Jonathan started to cough.  It was one of those dry, non-stop, coughs that goes on and on and on.  By Wednesday, Jonathan's daily blood cultures were still growing bacteria, and with his coughing, the Attending wanted an X-Ray to rule out any possible pneumonia.  His X-Rays came back clear, but the 'Attending' had already decided to bring up the topic of taking out his Hickman catheter.  The thinking was that his catheter may be 'seeded' with bacteria and hence the reason why he keeps getting these reoccurrences.  It was also discussed during the other rounds as well - but in the fourth round we found out that the IL-2 drug, in about 10 to 15 percent of the cases, made kids more susceptible for getting a bacterial infection.  Here is the text we got about the effect from IL-2:

... it may not be that his line is colonized.  if you have been using both lumens at home it is more likely that it's the immunotherapy.  IL-2 causes a reversible defect in neutrophil function and patients are at an increased risk of gram-positive bacterial infections.

In the end, I decided that I would take the recommendation of the doctors and have his Hickman catheter removed.  For some reason my gut feeling was that it should be removed and since my gut feelings have been the one entity that has helped me to make all my decisions with Jonathan's care - I could not ignore it.  Mind you, this was not an easy decision to make, although the procedure itself is very simple and only lasts a couple minutes.  I guess what I was hesitant about was having to go through the process of taking it out and then putting it back in ... but too late now ... the decision has already been made.

Results from Jonathan re-evaluation

Today's journal entry is going to be easy.  I'm going to cut and paste both the email I received from our oncologist and some additional comments I made when I sent the email out.

Here is my introduction to our oncologist's email:

Hot off the press (literally).  As you will read (see email below), no clear indication of what the results actually mean.  I know some of you may want to read into it, i.e. a sign that it ‘appears’ that the amount of NB cells detected is lower – but in the cancer world that really doesn't matter.  The fact that there is cancer cells is the problem.

The big question – which Dr Twist doesn't know – is whether the cells are differentiating, i.e. have the NB cells been converted to the ones that cannot reproduce or have been ‘matured’.  Keeping in mind there are different ways to conquer this disease, i.e. killing the cells or removing their reproductive capability.  In Jonathan’s case – it can be that he doesn't really have any disease and what is remaining are cells that have differentiated.

The original email from our oncologist:

Hi Walter,

the pathologists needed the 'special stain' (synaptophysin) to see a few neuroblastoma cells in the bone marrow biopsy - they were mostly rare single cells, since they could only detect with that stain, they could not get a real look at the morphology of the cells to see if they looked like they were differentiating in any way.  They could find these rare cells on both right and left side, but again i think they were rather surprised since they would not be able to pick out single cells like this with routine staining (usually the cells grow in clumps of several NB cells together, so they are easy to visualize compared to normal blood cells).  so, I think it is fair to say that we are finding rare cells that are below the level of routine detection - true minimal residual disease - but understanding the significance of this is a harder question ( no good data when we are in this realm).  the MIBG is negative and the CT looks fine too.

Clare

So - what does this all mean?  To me - it sounds like a positive step in the right direction.  It appears that the amount of cancer he has is continuing to decrease and that the disease is responding to the treatment.  The biggest positive is that the stain test is still proving to be a viable component of continuing to detect whether Jonathan has disease - since I'll rather know he has disease than live in a fantasy world thinking he does not have any disease and then he has a relapse within five years.

This leads me on to another topic.  We - Gabby, the kids and I, have been battling this disease for over 16 months now.  I remember during our discussions with various relatives - early on in Jonathan's treatment - that the general thought that it will be through God that Jonathan will be healed and - if he isn't healed - then it's God's will too and maybe there is another purpose, i.e. his suffering might help in some small way.  Well, my response is that this is a load of bull.

Don't get me wrong - I'm not saying that I don't believe in God - I do believe in God.  What I don't understand is that why does EVERYTHING have to be attributed to God.  We - as human beings - have free will - and it's our own free will that causes things to happen in our lives - so why can it not be that people live or die, get cured or not cured because of the luck of the dice, or the science of medicine!

Waiting for results - evaluation of cancer

We're slowly coming to the end of Jonathan's ch14.18 protocol treatment and occasionally my mind drifts on wondering what is the next stage?  Does he get declared NED - no evidence of disease - or do we have to go onto another protocol / another treatment plan?  For some reason I'm not really phased by it and this may be due to the fact that even if his results come back  negative and Jonathan is declared NED - we will never really know whether all the tumor cells have been eradicated.  We wouldn't know whether Jonathan will have a relapse and we'll have to go through all this treatment again - in the years to come.  So - instead of being ecstatic about any news - my mentality is to just move through life and take what comes - going with the flow.

Last week was the week for Jonathan's evaluation tests - to determine whether the remaining tumor cells in Jonathan's body has increased (not likely), stayed the same (more likely) or decreased (somewhat likely).  The medical team conducted three tests on him - a CT scan, an MIBG scan and a bone marrow aspirate (or biopsy).  The result that I'm really waiting on is the bone marrow aspirate.  Hopefully, we'll get those results by the end of today or tomorrow and it will be another data point to determine Jonathan's care.

On the good news side - we've finally decided to go on Jonathan's Make a Wish trip.  So - we'll be heading to Orlando, Floriday for two weeks in the beginning of May 2006.  Originally we were going to leave closer to mid-May and stay about three weeks - that way we would have had time to visit relatives in Florida, but since Jonathan's sixth round finishes on the 14th May - we have to be back during that week so the end of protocol tests can be done.  Also - we didn't want to travel in June because we wanted to avoid being at Disneyworld for the summer vacation - too much crowd.

So - our plan is to have two sweet and relaxing weeks in Orlando.  Make a Wish is covering 1 week and we're covering another week - to extend our trip.  We've been talking to the kids about the upcoming trip - but whereas Arielle is excited to go - Jonathan is a bit apprehensive primarily due to the fact that he has to get on a plane.  For some reason he's afraid of his ears hurting and I think it has to do with a time when he traveled as a baby - to Trinidad.  If it wasn't for the plane ride - Jonathan would be more gun-hoe.  In any event - we try to talk to him about it regularly and show our enthusiasm and assure him that everything will be alright.

This weekend we are taking the kids to see a Monster Truck rally (or show) and then Jonathan starts round 5 of his immunotherapy treatment.  On Friday he starts getting the GM-CSF - infused in his line for about two hours a day for 14 days.  The first dose is done at the day hospital while the other two - on Saturday and Sunday - will be done at home.  On Sunday night he gets admitted to start getting the ch14.18 over the next four days.  If all goes well - which it usually does for this type of round - Jonathan should be out by Friday and hopefully over the weekend we'll be able to go to the opening of Bonfante Gardens.

Recovering from Round 4 of Immunotherapy

Whew - last week is over!  On Wednesday night - when I came in for 'my shift' - I told Gabby to sleep in late on Thursday.  I know she was very tired and with her not sleeping well in the nights, the extra hours in the morning would definitely help.  So I told her that she could aim for 12 pm and then I'll head into the office.  Well, it's a good thing I stayed that late that morning.  While the doctors were preparing to round - I found out that Jonathan's liver enzymes were way off the charts.  The normal values are around 40 - Jonathan's were around 400 - ten times the normal amount!  Although we expected his liver enzymes to rise to maybe double the amount - ten times was a huge jump!

With the enzymes being so high - it meant that his liver had too much toxicity to deal with - so the team got the okay from the study panel to take Jonathan off the IL-2.  If his liver enzymes went down sufficiently the next day - then they will restart the IL-2.  Unfortunately (or unfortunately) it didn't go down so Jonathan just had his ch14.18 on Friday and we were discharged on Saturday.

The funny thing was that since after his 2nd round - I kept telling everyone - we need to decrease the amount of IL-2 he will be getting for his fourth round.  I told our nurse practitioner, our primary oncologist and nobody agreed with me - even Gabby!  Gabby and nurse practitioner had some cock and bull story about they don't want to reduce his chances of being able to eradicate his disease, yadda, yadda, yadda.  Our oncologist didn't want to change it because they had no 'evidence' aka medical necessity, from the last round that it needed reducing.  Well NOW you have the medical necessity!

Yet again - I've made a decision that proved to be right.  This takes me back to the stem cell transplant when TWO Attending Doctors wanted to take out Jonathan's NG tube because they felt he was at risk for fungal disease and with his non-existent immune system - could possibly have killed him.  One of my concerns was that if we took it out - we wouldn't be able to give Jonathan any oral medication that he needed on a daily basis for his treatment.  In the end I stood my ground and we left the tube in and (fortunately) things went well.

So with this episode of the amount of toxicity - I'm concern about stressing out his liver so much so that it stops working (non-medical speak).  Jonathan is getting his labs done today - so we'll get a sense of where his liver enzymes are and whether he can start taking the accutane.  Although the protocol stays that he should start today - I've very opposed to it right now - so much so - that I deem this as a critical issue - an issue in which I MUST be convinced in order for Jonathan's treatment to move forward.  This time I would be putting my foot down - including risking taking him off the study - although I haven't quite reach there as yet.  Ok - enough ranting.

The other interesting thing that happen was during the time the doctors were rounding on Thursday - remember I was staying late that day so Gabby could rest.  I guess what made it interesting - but I only found out afterwards - was the team - especially the Resident that had to present - thought it was going to be an easy, i.e. step in the room, do their thing, and then leave.  The Resident didn't expect to be engaged in a conversation where the parent knew a lot about what was going on and can question the validity of their actions.  Afterwards I had to chuckle - because I did give the Resident stress - so much so that the Fellow had to jump in.  But in the end - it was all a load of bull crap.  The sad thing is - it would be nice if these doctors would come back and say - Mr Seepersad, you were right!

I must mentioned the bull for my own records: with Jonathan needing extra air to get his oxygen levels up - the team wanted to verify that he wasn't getting pneumonia.  They thought that maybe he had fluid in his lungs - hence the need for extra air.  The problem was that in order to verify, they needed to have a chest x-ray done but I'm opposed to having Jonathan do unnecessary procedures especially if the cumulative effect might have (negative) long term effects.  We decided that if Jonathan could stay off of the extra oxygen then we may not have to do the x-ray.  So around lunch time I turned off the extra oxygen and Jonathan never needed any more air.  We didn't end up doing the x-ray.

But life goes on.  That's why I don't waste my time trying to become friendly with the doctors.  I maintain a strict professional level with them and keep them on notice.  I think these problems arise because since Stanford is a teaching hospital - you never have the same group of residents for each stay.  For each of our stays, this year, we've had totally different groups of residents.

Next week Jonathan is being re-evaluated to see if any progress was made.  On Monday he has his CT scan.  On Wednesday he gets the contrast for the MIBG scan.  On Thursday he gets his MIGB scan and we're not sure if his bone marrow aspirate will be done at the same time.  So, by the following week we'll get the news.  I'm almost expecting it to be positive, i.e. still have tumor cells, but we'll see.  Even if the test results come back negative - there is no guarantee that it's correct because the negative result can be due to sampling error.

Day 2 of Round 4: Pressure ...

Here is the sequence of events as I remember them.  Sunday night was a bomber.  For some reason the day before I have to go to work - I usually don't sleep very well - although on Saturday night I was able to.  I had already schedule to take one of the vehicles to the dealer to check out the speedometer so the plan was that Gabby would take the vehicle in with the kids and then head up to Stanford for Jonathan to get a blood draw.  The blood draw was for labs for his admitting into the hospital later that day.  Of course with our not sleeping well (yep - Gabby too) the plan got screwed up.  I think I got up around 4:30 am and Gabby couldn't go to sleep until 5 am.  What ended up happening was that I took the vehicle in to the dealer and then drove up to the office.  On my way to the office I dropped off Jonathan's blood sample since we are (sometimes) allowed to do the draw at home.  This also means that Gabby got a chance to catch up with her sleeping while I was out.

Later in the day we found out that although Jonathan's counts were good - his hemoglobin level was less than 10 so he would have to get a blood transfusion when he gets admitted.  By 9 pm we had reached to the hospital - Gabby and kids coming from home and I was coming from San Francisco.  As is the (new) norm - I spend the nights with Jonathan - so I figure Gabby must have left around 10:30 pm with Arielle to head home.  I slept soundly until around 5:30 am when I found out that Jonathan already had the blood transfusion and that he had spiked a fever around 5 am.

Spiking the fever was a bit strange - especially since he didn't start the ch14.18 and I was hoping it was not another bacterial infection.  The two nurses I asked didn't have a clue what could be causing it and it wasn't until I had an informal chat with the pediatric doctor (senior resident) did I realize that it could have been a reaction to the blood transfusion.  I must mentioned that by Sunday - we had started round the clock dosage of Zofran to prevent Jonathan from having an episode of vomiting.

Tuesday night was a bit rougher - for Jonathan at least.  They didn't start his ch14.18 infusion until after 10 am - which meant he didn't finish until just before 8 pm.  By the time I arrived at the hospital it was just after 8 am and the interesting things I noticed was that his pulse was hovering around 175 and his pulse ox (amount of oxygen his body is getting) was hovering around 92.  Every so often his machine would beep - signaling that one of his measurements have crossed a threshold and for the next two hours I was thinking it was his pulse rate but later found out it was his oxygen rate.

As an aside - the interesting thing about the monitoring machines is that when it goes off in the room - it also goes off in the nurses station.  There have been some instances in the past where we could ignore the alarms - and usually what happens is that we change the threshold values - and depending on the nurse you get it's easier to get this done that others.  The funny thing is that if you get one of those hard-arse nurses who don't want to change the threshold value - after getting the alarms going off all the time in the nurses station - it normally 'annoys' (I use this term affectionately) the senior nurses which forces the hard-arse nurses to make the change.

Anyways - getting back on track - Jonathan's situation was not improving and his oxygen rate was hovering around 90 with his pulse rate climbing to 180 - sometimes peaking to 190.  The major concern was the oxygen rate because we had to make sure his body was getting enough oxygen.  I wasn't really concern until a nurse came in and started to check out Jonathan.  She decided that she'll start some oxygen going by sticking a tube in a cup and putting it near his face so his body will get pure oxygen to breathe to try and increase the pulse ox rate.  At one point in time Jonathan was actually holding the cup to his nose and I guess he got tired of it that he finally decided that we can use the breathing tube to deliver the oxygen directly to his nose.

I was kind of glad we were able to make the switch - but as with all things - it's better than Jonathan decided we should use it rather than forcing him.  We slowly got the oxygen rate up but it required Jonathan staying on oxygen for the entire night.  His pulse rate was still high and we believe that's because of the fevers he's having.  The other news I got last night - around 5 am this morning - was that Jonathan's blood culture grew out a positive for a bacterial infection.  So here we go again with the antibiotics and dealing with another infection right in the middle of ch14.18 transfusion.

I should point out that since yesterday evening - Jonathan has been sleeping - probably because of a combination of the morphine and adivan - and probably because of the amount of stress his body is under.  It's good that he's resting through this - but it's painful to see because I can clearly see he's weak and under stress with the treatment.  The other thing that happened early this morning was that he started to complain of leg pain again - but this time I know it's because of the ch14.18 and not because of increased tumor activity.

The concerning thing is that usually - Jonathan doesn't get hit with the side effects until the third day.  What's disconcerting is that it's happening only after the first day - so I'm not looking forward to tonight and the next two nights after that - because I know it's only going to get worse.  What's been interesting is that I'm not phased by any of this.  I was thinking to myself - last night - that a year ago if I found out that Jonathan had to get oxygen - I'll be freaking out - now it's like - no biggie.  The good thing is that I like the doctor we have in the nights - probably because he is a senior resident - so he isn't spanking new - and he doesn't feel like he knows it all : ).

Round 4 of Immunotherapy Treatment

Round 4 started last week Tuesday with Jonathan getting infused with a low dose of IL-2.  Since he did relatively well with IL-2 during round 2 - we were allowed to have the infusion take place at home.  Jonathan's infusion was for four days - so from Tuesday morning until Saturday he was hooked up to his machine that gave him 1 ml per hour of IL-2.  We weren't taking any chances with his nausea this time around - so at nights we gave him a dose of his anti-nausea (Zofran) medicines - especially since he is still on the machine that feeds him during the night.  His feed is five cans of Pedisure which is delivered over 10 hours.  The good thing is that his weight is keeping constant - about 48 pounds - although he can do with a bit more weight.

Jonathan is going to get admitted tonight to start four days of high dose IL-2 and ch14.18.  This time we'll have him on round the clock anti-nausea medicines including: Zofran, Adivan, and Benedryl (or another drug in the same family).  I don't expect any surprised this time around - especially since we learnt so much during round 2.  We're also coming up to another re-evaluation of his tumor - and both Gabby and I are not really looking forward to it.  Let's just say I wouldn't be surprise if his tests still come back positive - but we'll see what happens.

Prior to starting round four - we spent the weekend (President's Day weekend) at Disneyland (in southern California).  We left San Jose around 3:30 pm - when we really wanted to leave at 2 pm - and arrived in Anehiem around 10 pm.  I don't know how people could live in southern California - but the traffic there is really bad - and if I can say that as a Bay Area person - then you know it's really bad!  On the way back to San Jose - it took as two hours just to drive 30 miles!

The trip to Disneyland was nice.  We felt we needed to do it - to give the kids something fun to do.  I think Jonathan feels his Make A Wish trip isn't going to happen because we keep pushing it out.  So this quick get-away was to give them a little taste and lift their spirits.  We went with my cousins from San Francisco - so the kids had their cousins to hang out with as well.

On the personal side - I think both Gabby and I are reaching our limits with pushing on.  Everything is robotic for me now - just get through the day at work - do whatever we have to do with Jonathan's treatment and rest whenever I can.  I'm toying with the idea of taking some leave (unpaid) but we'll see.

Putting Jonathan's remaining cancer into perspective

Jonathan continues to do very, very well during this third round of receiving ch14.18.  He's been active, hardly feeling any pain, and has been able to eat.  Today is the last day in the hospital, and hopefully all goes well tonight so that he can be release tomorrow morning, so that by tomorrow afternoon we'll be on our way to Yosemite.

We also had our consult with our primary oncologist this morning - to help put into perspective what Jonathan's remaining amount of tumor cells actually means.  As with all things in life - there is some good news and there is some bad news.  To sum it all up, the good news is that we can say with some certainty that Jonathan's life has been extended.  When Jonathan was finally diagnosed with Neuroblastoma a year ago - it was almost the end for him.  We have (now) reached the stage where most of the tumor cells have been eradicated; he has no solid tumors; the amount of tumor cells in his bone marrow is small; and he is recovering - physically, mentally and emotionally.

The bad news is that he still has cancer cells - so he is definitely NOT cured.  There is also no guarantee that he will be cured because one of the characteristics of Neuroblastoma is that even with just a little bit of tumor cells - it can become aggressive and 'explode'.  I've (personally) read too many stories of Neuroblastoma coming back with such a vengeance that it's frightening.

What does this all mean?  It means we have a reprieve.  It means we (finally) have hope.  It means a little more sanity in our lives - but for how long - we don't know.

Immunotherapy Round 3

I've finally gotten into a pattern for Jonathan's immunotherapy treatment.  It involves my taking him into the hospital to check-in, usually on Sunday evenings, then spending the nights with him during the week.  This is more convenient that my other pattern because I get to spend time with Jonathan, have a shorter commute to work (if I drive), and be more in touch with Jonathan's day to day treatment at the hospital.  Although I have a shorter commute, I still take a hit with going into the office because by the time I can actually leave the hospital for the office and depending on the particular mode of transportation I use, it usually still screws up my commute so it's not really an advantage.  But, on the bright side, I get to spend time with my son AND give Gabby a break - although (sometimes) she feels the break might not be necessary.

This third round has been proceeding quite well.  I've known that the 1st, 3rd, and 5th rounds are easier than the 2nd and 4th rounds, but so far Jonathan is handling it quite well.  As an aside, the 6th round is not a true round since there is no immunotherapy drugs given - just accutane.  The medical team has also decided not to ramp up to the full dose of ch14.18, so instead of going to 20 ml per hour, the maximum given is now 15 ml per hour.  This means the pain due to the ch14.18 does not become unbearable for Jonathan but the length of time it takes to transfused is longer.  I think the medical team has also figured out the right mix regarding pain control with morphine.  Now Jonathan gets 1 mg per hour with a 1 mg per push on the button with a 5 minute timeout.  Normally he doesn't have to start pushing the button until well pass halfway into the transfusion process.

An interesting thing happened during the first day and a half of this cycle.  When Jonathan was first admitted his hemoglobin level was around 10.6.  This blood sample was taken around 8 pm on Sunday night.  They took another sample at 6 am on Monday morning, and the hemoglobin level was around 9.8.  Since his hemoglobin level was below 10 - they wanted to transfused him with red blood cells but I pushed back saying that one of those tests was suspect and since they had to do another blood sample at 6 pm - I'll defer to making a decision till then.  Well, the 6 pm blood sample, taken on Monday evening, came back with a hemoglobin level around 12.4 which means Jonathan didn't need a blood transfusion.  I'm a bit puzzled by the vastly varying results for his hemoglobin level but not a critical issue for me to follow-up on.

Switching gears a little bit - we got some information last week that helps us put Jonathan's current tumor levels into perspective.  The amount of tumor cells remaining in Jonathan's bone marrow is less than 5%.  That means it's so small that it's hard to count - so there isn't another counter range below 5%.  What is significant is that in order to detect this small amount of tumor cells - the medical team are using a special staining test.  Tests that were not available a couple of years ago.  This means that if this was five years ago - Jonathan would have been (erroneously) declared in remission or free of disease - but then a couple years later he would have had a relapse.  One belief I've heard from our oncologist is that a relapse probably means that they never really killed ALL of the cancer cells ... so in a way it's good that they have these new tests.  Unfortuantely there may still be undetectable tumor that this staining test will not pick up - but it does provide new hope in trying to combat this disease.

So, the plus side is that the amount of tumor cells remaining in Jonathan's body is very, very small.  To me, this is significant news and a cause for a little celebration!  The negative side is that there is still a small amount of tumor cells in Jonathan's body so he's not out of the woods yet.  To be level headed about this - it means that there is still no guarantee that Jonathan will be cured - because this amount of tumor cells - even though it is so small - can still be resistant to treatment and / or become aggressive and start spreading and growing again.  We have a consult with our primary oncologist on Thursday morning to help me figure out what this news really means and how I can put this into perspective with the wider picture of what other neuroblastoma patients have gone through.

The biggest excitement will be at the end of the week when we make another road trip.  We're going back to Yosemite by my friend's parent's house and with Gabby's folks in town, we'll have a bigger crew.  So it will be Gabby's folks, our family friends in Hollister and us.  We're all looking forward to it, given the amount of fun we had the last time and with it being dead-on winter - the kids will have another opportunity to play in the snow.  I've already 'warned' Gabby's parents that the kids like to have snowball fights - so beware : ).

Findings from round 2 re-evaluation and moving forward

Wow - a lot of information over the last couple of days.  Last week Thursday - 19th - Jonathan was scheduled to have his bone marrow aspirate and MIBG scan done.  This involves him getting anesthesia which requires him to not eat anything solid / food from midnight.  Well, I don't know what happened but we - both Gabby and I - clean forgot about taking off his feeding pump and it wasn't until 7:45 am that I realized that he was still hooked up!  It meant that Jonathan couldn't get any anesthesia and I had already planned for Jonathan and I to (both) have hearing tests done on Friday (27th).  I was so disappointed because I was looking forward to the hearing tests that I had taken a day off - but with us forgetting to stop his feed it mean the bone marrow aspirate had to be pushed to Friday.  The only good thing that came out of this mistake was that Jonathan got sedation, instead of anesthesia, to do his MIBG scan.  Since he did so well on sedation then for future scans that's what they'll use.

The other significant thing that happened was that on either Tuesday night or Wednesday morning - I forget which one - Jonathan and I was having a discussion about whether he will stay still for the MIBG scan so that he doesn't have to get anesthesia or sedation (in this case).  Besides getting the usual response that he doesn't want to stay still and that he wants the sleepy medicine - I found out on Thursday afternoon - while preparing for the MIBG scan that one of the reasons for the push back was that Jonthan was having major issue with his biopsy. (On a side note - that is a really long sentence).  Apparently he wanted the sleepy medicine because he didn't want to feel the needles go into his back, he doesn't want to feel the cuts.

I was completely taken aback - because I don't know how he can feel any needles going into his back.  Normally he is put to sleep, the biopsy is done and he is woken up.  Of course I tripped out.  I immediately penned an email to our nurse practitioner and our oncologist with my concerns and to investigate whether he gets enough 'sleepy medicine' when the procedure was being done.  The problem was - I didn't know if the problem was that he goes in for a procedure and wakes up with pain in his back - where the two holes are done for drawing out the sample of bone marrow.  In any regards - the experience - perceived or not - needed treatment.  So we've in the process of getting Jonathan to see a psychiatrist.

Shifting gears completely - we got the results from his MIBG scan and biopsy results.  The scans came back clear - which was excellent news.  Keeping in mind that his previous two scans were also clear but the significance was that we didn't know if the (bone) pains he was getting up to five days after finishing the IL-2 and ch14.18 treatment were due to the cancer returning or due to the IL-2.  I think even our oncologist was a little concern and with the MIBG scan coming back clear (or negative) it means that there is no new tumor involvement.

His bone marrow aspirate results were a little interesting.  Everytime that Jonathan gets an aspirate done - it involves taking two samples from his lower back.  This is the first time that one sample came back negative while the other side came back positive with marrow involvement of less than five percent.  The thing is - we don't know if the sample that came back negative is due to sampling error or whether the ch14.18 protocol is working.  We've been told not to read too much into it and it's sound advice.  I'm just ecstatic that the pain he was experiencing during the second round wasn't due to cancer involvement - cause that would have just crushed me to find out that it's getting worse.

The last piece of good news (at least for this journal entry) is that Jonathan's appetite has returned.  Although Jonathan still gets his NJ feedings every night - we suspect that since it's an NJ tube vs an NG tube - with the feeds emptying into the upper regions of his small intestine vs his stomach means that he would get the feeling of being full.  In any regards, although his appetite has returned he doesn't eat enough for his body's needs since his intact has to be 1.5 times a normal person's daily allowance.  But - all in all - it's been a good week or so ...