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.