First week on Accutane

Jonathan has been on the Accutane treatment for a week now. The side-effects of extreme dryness has already begun, in fact it probably started soon after he started taking the tablets. The dryness is on his lips and around his mouth region. The dryness is so severe, that the discomfort is probably borderline pain. Jonathan spent the last few days going through a host of different creams - all recommendations from Gabrielle, especially as Gabby and Brandon both suffer from dry skin as well. Yesterday, he switched to a combination of coconut oil and vitamin E oil and that seems to work a bit better. The only issue is it only provides relief for about 5 minutes.

In Jonathan's defense, he was hesitant to use the cream(s) more frequently, because earlier in the week he was trying out a really expensive and thick cream that Gabby uses, and she wanted to make sure that Jonathan didn't waste it. Now that he is on the vitamin E oil (which, I believe works the best), we were telling him tonight that if he needs to put it on every five minutes, then go ahead and use it every 5 minutes. Maybe if he does that for an hour or so, it will gradually taper down in it's intensity. We're not sure, but trying to win an argument with a teenager can sometimes be difficult :-).

The good news is that we don't have to be constantly behind him, to remind him to take his Accutane tablets. With our (Gabby and Walter) heads all scrambled at the moment, preparing for our big move, we would frequently forget he has these tablets to take :-0.

We have a consult at LPCH (Lucille Packard Children's Hospital) for some blood work and a physical, to see check his levels and see how his body is handling the Accutane. The original plan was to get a two week prescription, which expires next week Wednesday, October 7th, and get a check-up to determine if they need to adjust the dosage, etc... I'm glad he is on the Accutane treatment, and now the major worry is to ensure a good transition of care when we move to Holland at the beginning of November. The good news is that the medical team at LPCH is working on ensure there is a smooth transition, so all I could do is hope for the best and trust in The Lord.

Treatment Started and Scan Updates

Jonathan finally started the Accutane treatment! He started on Wednesday night, to be precise, and has a prescription for two weeks, after which lab work and a physical is done to ensure he is handling the side-effects and is receiving the correct dosage. I'm just happy he finally started something, although I'm a little worried about continuity of care when we move from California to The Netherlands. We still have, at least a month before we need to figure that out, so hopefully the transition will be painless (from a red tape perspective).

We also got the results from Jonathan's MIBG and PET scans, and both came back negative! In one breathe, this is good news and I could just imagine that breathe is the exhale. In the other breathe, in this case the inhale, I'm cautiously optimistic because these two scans are not sensitive enough and we're not sure whether the PET scan is a true indicator or not, i.e. is it NB hot? Regardless of all the doubt, it is good to know there is currently no significant tumor in his body (at the moment).

When I look back to when we discovered his third relapse in June of this year, and the realization that the MIBG scan is not sensitive enough, I still remember all the worry and anguish on whether this 3rd relapse was going to follow the normal path or continue to be the anomaly that Jonathan is lucky to have, i.e. slow growth. A relapse with Neuroblastoma is usually quite aggressive, and I still remember the two fathers in Southern California who lost their sons in their battle with Neuroblastoma. Jonathan has and continues to be an anomaly with regards to this disease and I'm hoping he continues to be, especially in a good way.

In the early stages, we used to wonder how long we would have our son with us, usually measuring in single digit years. Now we feel his status has moved to more of a chronic case, in which it will always be part of his life, and we wonder whether Jonathan will outlive us or we would outlive him. For the moment, it's good to be in the position we are in, and we are definitely enjoying the "brief" respite.

PET Scan Scheduled

We learnt on Thursday that Jonathan's PET scan is scheduled for next week Wednesday. Unlike the MIBG Scan, which is either a two or three day affair, the PET scan is a one day event. Jonathan will get the contrast or some type of isotope at 1 PM, and the scan follows immediately at 1:30 PM. I'm not holding my breath that the PET scan will be extremely helpful because either news is a mixed blessing. If the scan comes back negative, we don't know if that means there are tumor cells that are undetected by the PET scan, or whether the tumor cells are detected and there are no more tumor cells. We don't (currently) have a way to tell whether there are any tumor cells, my suspicions are that they are probably too small.

Jonathan had another round of MIBG scans this week, i.e. on Thursday, September 10th, and my expectation, like the February scans, is for the results to come back negative. If the results (from the MIBG scans) do come back positive, then this would be good data to help us figure out whether the PET scans are a useful testing procedure or not.

Whew! So I go back to my earlier statement, any news we receive will be a mixed blessing. :).

We meet with the medical team next week Friday, September 18th. I'm hoping the meeting will be to review the results of both scans AND also start the 13-cis-retinoic acid, aka Accutane treatment. Here is looking forward to another "adventurous" week :).

Preparing for Accutane Treatment

We had the consult with Jonathan's oncologist on Wednesday afternoon. I couldn't help but feel that Jonathan wasn't a big priority for them, but I sort of understand why. Jonathan's stuck in this in-between state of not having enough (detectable) cancer for them to come up with a definite plan. Using chemo is not an option, a plan we both agree on, because it's like pulling out the big guns for something that truly appears localized in a very small area. It also means that a lot of phase 1 trials aka experimental treatment are not an option because a prerequisite is having detectable tumor, which he wouldn't have now that the lymph nodes has been removed from his neck region.

The "good news" is that he gets to start 13-cis-retinoic acid, otherwise known as Accutane.

• Overview on Retinoid Therapy
• Detail Information from a Medical Journal

This has been a drug that Jonathan has taken in the past, i.e. when he was first diagnosed back in 2005, and this treatment and the follow-on treatment with Fenretinide were the drugs responsible for Jonathan being declared in remission for the next 5 years, i.e. 2007 to 2012. The plan is for Jonathan to start Accutane in the upcoming weeks, it takes a while to get all the paperwork completed as Accutane / 13-cis retinoic acid is a controlled substance, due to the recalls associated with (among other things) depression and suicides, as this drug was also used heavily in the treatment of acne in teenagers. Fortunately, as Jonathan has taken this drug in the past, he is already registered with iPLEDGE so hopefully the "red-tape" doesn't take too long.

• Information on Accutane
• Information on iPLEDGE

Other than treatment options, we also discussed detection options as well. I was sharing my concerns that the MIBG scans are not a good way to detect tumor cells, especially as we saw in 2014 that the CT scans were showing tumor growth. It wasn't until 6 months later when the tumor got large enough for it to register as tumor cells in the MIBG scans. Thus, when the MIBG scan came back clear in February 2015, I didn't put "2 and 2 together" to realize that we may have gotten a false negative.

During the consult, I learnt that there are some Neuroblastoma cells that are "MIBG cold" and the only other, possible, detection method is using a PET scan. We're going to try a PET scan as well, to see if the MIBG cold Neuroblastoma cells are responsive to the PET scan, but it's not a method we're hoping for a lot of success. We believe Jonathan had a PET scan when he was younger and it was unsuccessful - but not 100% sure.

Other than that, we got some education on medical terminology and the human body. Apparently, just like the blood system, the lymphatic system is part of the circulatory system in the body. Apparently tumor cells can spread via both systems. Somre more reading on the lymphatic system can be found on Wikipedia's website.

Another successful surgery!

Saturday, August 18, 2015 - 1:57 PM



Jonathan's surgery took about two hours. I was expecting it to be a little less, but cest la vie! All in all, no complications of any kind, and we were not expecting any. We are all, including Jonathan, "experts" in this now. His scar from the surgery is a bit longer, and I forgot that there is always nerve damage whenever this type of procedure is done. Jonathan experiences some numbness whenever certain parts around that area is touched, but luckily, nothing worse happens as there is always a possibility.

This picture was captured in the (post-op) recovery room. Jonathan just woke up from anesthesia, and part of the "recovery" process is to drink some fluid or eat / suck on one of those frozen lollipops. Here you can see the scar from the surgery on his neck, and also the scars from the exit point of his central line, when he has had chemo in the past. His scars are truly his badges of honor. When he met a very young Neuroblastma boy (less than 5 years old) at Camp Okizu's Oncology Family Camp, I was suggesting to Jonathan to show the scar from his surgery when he was five and the incision went from his button, to the side of his chest, and then up towards just under his arm! What an emotional time, and I still remember it like it was (almost) yesterday :).

Looking forward to the results and next steps!

Waiting in Pre-Op

The day that we’ve been waiting for has finally arrived! We’ve been looking forward to Jonathan having his surgery for quite some time now, as everything else was being “held up” until it happens. We can finally get pathology examining his tumor, learning and discussing his treatment plan, and on a more minor level, hopefully some discomfort relief for Jonathan for his arm, shoulder and back areas.

I decided to take Jonathan for his appointment and Gabby was going to meet us after dropping the other kids to school. Luckily, our next door neighbor took the day off so she could babysit Dakota and also keep Niko so he could have his potty breaks. For some reason, it was important to Gabby, to be present in the recovery room after Jonathan’s surgery, and since only two adults or kids over 18 (?) are the only ones allowed, it means that if Dakota was here, someone would have to be in the waiting room with her.

Although I think it was unnecessary for Gabby to extend herself, I respect it. Her friend and neighbor, also made it possible for this to be an option as well. I think path of Gabby’s drive is that she is in a dark but realistic place. We’ve come to a realization, earlier this year, that Jonathan’s disease is a chronic one, i.e. persisting for a long time or constantly recurring, and is the better path for us mentally, rather than getting disappointed every time he has a relapse. It will be interesting to see what happens over the next few months, and (hopefully) get some confirmation on whether his tumor is still a slow growing one or is following the more “traditional” path of being more aggressive.

Scheduling a CT Scan

It's almost two weeks later and the lymph node is still enlarged! When Jonathan had his last scans / evaluation in February, it was decided that instead of getting scans every 3 months, we would switch to scans every 6 months since the tumor growth was so slow. Even though his scans would now be every six months, he would now receive a physical at the half-way mark in the Oncology clinic. This means that Jonathan will still be at LPCH every 3 months but it wouldn't be for scans, at least, that was the plan.

Since his physical was coming up, July 10th to be exact, we decided to ask if a CT scan could be scheduled, so that way we would have those results to review as well. An appointment was setup for June 30th to see his nurse practitioner and he also had labs and a chest x-ray done. His CT scan was setup for the following Monday, i.e. July 6th, with his MIBG scans occurring later that week, i.e. injection on July 7th and scan on July 8th.

I could see that the folks at the Oncology clinic were a little bit concerned, and the fact that an MIBG scan was also scheduled, and relatively quickly, was further proof. Now I cannot wait to review the results on July 10th. We fear the worse but hope for the best :).