Snakes and Ladders

Nothing seems easy.  We were hoping for a “homerun”, but no such luck.  The interim report can be summarized thusly.

The biopsy from the tumor consisted mostly of ganlioneuroblastoma.  This is partially matured neuroblastoma cells.  It is still considered malignant, but it does not have the  malignancy of neuroblastoma.  So, this is an encouraging piece of information.  Dr. Merchant has suggested a “wait and see” approach on these tumors.

Four cores were taken from Maya?s pelvic bone.  The samples from the bone marrow contained even more matured cells, ganglioneuroma, which is considered benign. Good news on the bone marrow side.

So, we are in the nether realm. On one hand, it is very encouraging to see that Maya?s neuroblastoma is maturing.  However, there are still active neuroblatoma cells in her body, and we can’t get to it to put Maya on the study.

From the samples, while rich in stroma, there were just enough cells to get one micro-assay tests run; only 3/4 or what’s required to do the test.

The tumor board will meet next Thursday to make a clinical judgement.  Molecular study will not help to drive the treatment. Although we will gain some perspective on what Maya’s gene signature is, as Dr. Merchant puts it, “there is a worry that molecular lesson may not apply to the neuroblastoma cells.”

So, we’re not eligible for the study and we will not be going back to NIH.  Dr. Merchant did suggest that there may be a couple of options to pursue; namely, the fenretinoid study and DFMO study. We will have a discussion with Dr. Sholler or Mr Merchant subsequent to the tumor board meeting.


Nothing is easy with this kid!  But we love this kid. Whatcha gonna do?

The biopsy

8 AM:

It?s Monday morning. As is the case with every procedure, there is a knot in my stomach and nervousness that accompanies the day. ?We?ve done this before?, never seems to add any comfort.

The surgeon told us that there were two areas where the biopsy could be taken from. There?s an area in the front, well below Maya?s heart, or at the back. Neither of these two areas lit up on MIBG. MIBG is up taken by 94 ? 95% of the cancer. So if it lights up, it definitely means cancer activity. Two spots did not light up. That could mean a couple of things. The tumor simply did not take up the MIBG, or the tumor does not have viable cancer cells. Non viable cells would mean that the cancer cells have matured or have died.For the first time I wish there was some activity which we can get to.  We need a viable tumor sample from which we can run the genetic tests on.

On the other hand, if there are matured cells, this means Irenotecan / Tazolomide is working. But there are areas that are still lighting up and there are spots that we weren?t aware of before. So, is it working or is it not? One area that is lighting up is in the interior part of Maya?s spine, roughly mid back. But getting to this tumor is difficult and performing the biopsy may cause damage. No one wants to go after this tumor.

Based on the ultrasound, we found out that the tumor on the back is 1.25cm across and the one in the front is 1.8 cm; not very big. Also, getting at the tumor on the front will mean ?suspending? Maya?s breathing so that the movement of Maya?s diaphragm does not get in the way of harvesting the biopsy. Maya will be intubated just incase lung is “nicked” and her lung collapses.  Not something I want to think about.  Neither do the surgeon and the anathesiologist, but nevertheless, the precaution must be taken.

The plan now is to get to the tumor on the back. It is 10:30 AM now. The procedure has just started. As usual, we wait.  Both Indira and I had a quick cry.  It does not get any easier.


12:30 PM

We get word that things are taking a bit longer.  The biopsy went well, but looking at the sample in the OR did not yield conclusive results.  The lab will have to do their work to see if there are any cells that can be used for micro-assay.  Darn it!

The surgeon is taking bone marrow aspiration samples.  Maya is doing very well and she should be done very soon.  She was out around 1 PM.  And as ususal, groggy and cranky.  But both Indira and I are very happy to see her. And deep inside, I quickly allow a moment where I wonder why we have to put our lovely daughter through all… this.

After about 30 minutes of observation, Maya was transported back to the clinic where she can recover.  Within half an hour, Maya was sitting up and wanted to play. So, Maya and I did a “hello kitty” puzzle.  Of course Maya did get a good dose of pain medication, but even the clinicians and the nurses were impressed.  Maya also downed some pancakes and bacon.

So that was pretty much the day.  We spent the afternoon hanging out in the room and playing.  Maya loves her (indira’s) ipad.  I must say that it’s been nice to have around for Maya.

Maya will spend another night in the clinic, but for now, we’re back at the Children’s Inn.  Since the entire area is NIH, the center seems more relaxed about kids roam around. It’s definitely more relaxing to be here.  Since Maya seems to be doing very well, it’s likely that she’ll be discharged tomorrow.

Waiting to hear about the results of the biopsy is constantly hanging over my head.  The thing is, this time, we are wishing for viable tumor sample.  What crazy crazy world this is.   As Greeks would say, Opa!

Chincoteague Island

Is in Virginia, but it also runs north to Maryland as well.   As you can see in the satellitephoto, it runs good 40 KM or so.  And the beach is quite lovely. It is a sleepy little town not really touched by the corporate machine.  I guess it has a lot to do with the fact that it is a national wild life refuge.  If anything, there is a tired and old, if not a wee bit decaying feel to it.  But personally, I prefer this over the blinking lights with main strip full of McDonalds, Pizza Huts etc etc.

I took a chance this morning and decided to head East.  We had the choice of hitting the typical beach out this way or go a bit further and hit the national park.  I think it was not such a bad move.

We arrived here mid afternoon and spent the afternoon at the beach. The claim to fame around here is the wild horses of Chincoteague.  We haven’t seen them, but everyone raves about them.  Since it is a wild life refuge, there are a  lot of birds and  birders walking around with expensive camera equipment. Oh and crab is also big around here.  Apparently, you just take a string, tie a piece of meat on one end (or chicken neck) and just pull them out of the water.  Indira had “all you can eat crab” for dinner.  Personally, I didn’t think she ate enough. I was happy enough chomping on grass.   This is one place I could easily spend a week or more in a tent on the beach.  If you walk far enough (1 – 2 KM) , you could easily find a spot away from people that you could have the prime beach front realestate all to yourself.

Luckily, we found a motel on the main strip for a reasonable price.  We decided to stay the night so we can spend part of the day at the beach.  You could easily unleash Maya on the beach and she’ll keep herself content for hours on end. We’ll be here until the early afternoon tomorrow before heading back to Bethesda.  I will upload some photo’s as soon as we get back to “white America”.

Day 2 in Bethesda

All of us passed out relatively early yesterday.  It was a long day and all of us by 8 PM were barely hanging on.  As soon as we closed our eyes, it was 9 AM.  Feeling refreshed, we headed to the Clinic.  As mentioned yesterday, we are staying at the Children’s Inn.  The clinic is only one block from us… no, it’s more like 100 M or so.

What seemed funny to me was yesterday, when we were first heading out, the nice lady at the reception area, that the shuttle would be here in 5 minutes or so.   When we asked where the clinic was, she said, “Oh, it’s just across the street”.    Anyways, this morning we headed to the radiology clinic and started the MIBG scan.  Two technicians, Delise and Katrina were beaming at Maya and it wasnt’ long before they were Maya’s best friends.

The scan seemed long to me.  Something like 2 1/2 hours.  Both Delise and Katrina were amazaing.  Both of them held Maya in position in awkward positions, both at the head and at the feet for well over an hour.  Their dedication to their patients were comforting to say the least.  For your kindness to our little girl, thank you Delise and Katrina.  It wouldn’t be too difficult to just do what’s required of you.  You didn’t have to crawl up on the machine and hold Maya and comfort her was extraordinary.  Even so, Maya squirmed around and the first scan did not turn out as well as we had hoped.  Of course, the second scan for her mid section, Maya fell asleep.   We are likely to sedate Maya for the subsequent scan, which if all goes according to plan should be in another 4 weeks or so.

One thing that I found interesting here is that NIH has the equipment that can directly correlate one type of scan to another.  For example, they can take a CT scan and directly map it to the MIBG or X-ray(I think?)  What this means is that by matching one scan(say CT) to MIBG, they can get much better visual than one can by looking at the scans individually.  Why is this important?  The scans provide different information.  For example, CT provides the gross visual of the body.  MIBG shows the area where there is cancer activity.  So, by merging the two together, you can get a better understanding of what the tumor looks like and how much activity there is.  (May not be exactly correct, but I think it gives you some idea why being able to merge the two provides more information)

After the scan, we met with the anathesiologist for prescreening.  Dr. Mo Yousef is a young anathesiologist. All I can think of was that he looked like Young Joe Rogan (UFC MC) very unassuming and talked to us in a very unassuming way.  We liked him right away.  Maya? Not so much.

The last part of the day was signing consents and meeting with Dr. Javed Khan. He is a researcher who studies “Oncogenomics”.  We had agreed to participate in a study which looks at the genetic and proteomics of children with neuroblastoma (and other types of cancer).  As you know, our position has always been, always learn from the patients.  If nothing else, honor the children by learning as much as possible.   And since participating in the study does not add any additional discomfort or significant risk to Maya, we agreed to the study.

And somehow, we spent all day in the clinic and were freed from the confines of the hospital setting.  One benefit of driving here is that we are mobile.  So, we spent the evening having dinner downtown Bethesda in what is comparable to the theatre district in Toronto; lots of restaraunts and night life.  Oh, I thought I got ripped off… I had to pa y 4 dollars parking !  🙂  We were also lucky enough to catch the new Harry Potter.  Both Maya and I loved it.  Indira fell asleep.

Tomorrow morning, we are heading out to Ocean City.  It’s about 3 hour drive.  That’s OK.  We’re here with nothing much to do and the choices were, take the city or take the beach.  Not  much of a contest to me.  So, if all goes well, in the next twelve hours or so, Maya and I will be trying our luck catching Crab and .  Indira may be sleeping under the sun.

Sunday evening, we will be checking in at the clinic for the procedure monday.


Hello from Bethesda, MD

Well, we’re here.  It somehow took heck of a long time to get here.  We picked up Maya from camp around 3:30 PM and somehow, through traffic and pit stops and dinner break, pee break etc etc, we checked in at NIH around 6:00 AM.  Wow… that was painful.   Apparently, aside from pentagon, NIH/NIC is one of very few places that can be easily identified from satellite images.  Subsequent to 9/11, access to this place has tightened down.  Everyone who has not been vetted must go through the security check similar to what one expects at the US customs with dogs and bomb detection kits and everything.   The gentleman who ushered us through, apparently a long time attendee of caribana made this process a bit easier for us.  So, thank you Mr. Security man!

So, first things first.  We’re staying at the Children’s Inn.  It’s a cross between … a hotel and a play ground.  There are common areas, such as kitchens and play area etc, but we have a room to ourselves, which is nice; not that we’ve seen much of it today.

Our day started with a bit of confusion.  Due to haste on my part, we were late for our initial administration.  But, Chis Graham (Maya’s Nurse Practitioner) was kind enough to help us through the process and we were off.  Today was getting the base line info. So, it consisted of getting a CT scan, prepping for MIBG for tomorrow and a physical. We also met with Dr. Melinda Merchant, Maya’s clinician for the study as well as Dr. Gissell  Sholler.

The day looked like this:

  • Get blood and urine analysis done.  Check!
  • Get a CT scan done.  Check.  This meant that Maya had to take the contrasting agent that must be taken prior to the scan.  Of course, it does not taste like candycane or popsicle or any of the things which Maya likes.  Trials and Tribulation has begun.
  • We also had to prepare for the MIBG scan for tomorrow, which meant that Maya had to take the Iodine for her thyroid gland.  Check! They call it SKSI or something like that.  It’s just iodine, plane and simple, just like the “lugol’s” iodine.  It also does not taste like   candy or cake.
  • And there is a lot of waiting around amidst the confusion of receiving a new patient on the NCI’s part, and confusion on our part trying to find the radiation clinic and the children’s clinic and the cafe to pick up a lollipop to sweeten the bitter medicine.

And at last, we had a chance to sit with Dr. Merchant and Dr. Sholler.  Over all, we discussed how Maya’s been the last little while.  We provided them with all the information that we’ve collated over the past year and a bit.  They also asked us to consent to a couple of studies and allow access to Maya’s tumor.  Take them all.  What ever you need, if it will help Maya or if it will help you solve this dreadful disease, please take them.  Matter of fact, I insist.

In terms of the course of action, well not much I can add to what’s been already written.  But, I guess I can add the following details:

  • CT looked consistent with what’s they saw from previous scans.
  • There does seem to be a couple of spots where the tumors can be taken from. We will discuss details after MIBG.
  • There was a softer looking spot in Maya’s chest, which I was not aware of.  This spot seems necrotic.  If so, it is not a good candidate for biopsy sample.
  • In principle, we agreed to perform another bone marrow biopsy. This will not really hurt Maya, as she will be out for the soft tissue biopsy and the information gathered could potentially be helpful to her.
  • Nifurtimox is off the table right now, as it is still a trial drug.
  • However, Dr. Sholler felt that Maya had a lot of options open to her.  (She did say “quality of life”.  I hate that phrase.)
  • DFMO might be a good option for her, say after the personalized medicine trial, as it has very low side effect profile.

I did bring up that there is some “evidence” of efficacy of Irenotecan and Tazolomide.  This suggestion was based on the fact that Maya’s bone marrow did show matured blastoma cells and possible necrotic spot in her chest area. Both Dr. Merchant and Dr. Sholler agreed that keeping Maya on that therapy should be considered, if it is shown efficacious.  All options are on the table presently.  How does one show conclusively that it’s the drug that is having the effect?  Well, I guess we’ll discuss.

We also mentioned that there were a handful of families that are in similar circumstances as we are. Both Dr. Merchant and Dr. Sholler were open to the idea of discussing possibilities with them, if they were interested.  So, we made a shout out for neuroblastoma families who are in our thoughts and prayers.  Since Maya is the second child to undergo biopsy, there is still room and next batch of children will be recruited in the September timeframe.  Neuroblastoma families, if you are interested, please contact us or Dr. Merchant or Dr. Sholler directly.  Our prayers are also with you.

In anycase, we’re here and made it safely;  all good.  So far, our day has been very interesting, albeit both Indira and I are running on fumes. Pictures to come soon…ish.

Washington DC, Here we go!

Well… the time is fast approaching.  We have our sight set on doing whatever we can for Maya and that means we’ll be travelling to Washington DC  (well actually Bethesda, MD) for a new clinical trial.    As many of you may be aware, we’ve been accepted for a “personalized medicine trial” at NIH.  Maya is doing quite well right now.  She looks healthy and she’s been having lots of fun hanging out with Indira and her cousins.  She is even starting to look a little rolly, as a child should.

The maintainence chemo has been relatively easily tolerated, but it has not reduced the disease.  So, we needed to do something.  Luckily, I was able to get in touch with Dr. Giselle Sholler just as she was starting her new position at the NMTRC, in Grand Rapids, MI.  For the first time, children with Neuroblastoma are able to enroll in a study where the child’s genomic signatures are considered in their therapy.  It is about time. This trial is touted as the “landmark” therapy presently and we are very grateful to be part of this study.

We set out to have Maya treated at the Van Andel Research Institute in Grand Rapids, where Dr. Sholler was.  However, given that Ministry of Health was not going to cover the cost, Dr. Sholler referred us to Dr. Melinda Merchant at NIH, as NIH would cover our costs.  It might have been a little effort on Dr. Sholler’s part, but the consequences are significant to us.  And for that gesture, we would like to say thank you Dr. Sholler.

So, after a few email exchanges and personally meeting both Dr. Sholler and Dr. Merchant, we’re all set to head down to Bethesda.  A couple of weeks ago, we weren’t so sure, as Maya’s platelett’s were down in the dumps.  Maya bottomed around 37 and the minimal requirements were 50 and ideally it should have been above 75.   We are happy to report that Maya’s plateletts are well above 75 and so, the last remaining hurdles were cleared.

The overall itenerary for the study is as follows:

  • We need to get Maya’s base line. That means we will get new scans done, both CT and MIBG at NIH.  We will also get tumor samples through her back.  We can do this during our initial visit.
  • Within two weeks, the “tumor board”, a collection of Oncologists will recommend a therapy for Maya based on her genetic signature. The drugs that will be recommended will all be FDA approved drugs.  The novelty comes in the form that some of the drugs may not have been used in conjunction before.
  • We then will fly down to NIH again so that at least the initial administration can be done on site.  The clinicians want to ensure that Maya does not have an adverse effect and wants to observe her at least for the first time.
  • If Sick-kids agrees to administer the subsequent therapy to Maya, we can do the rest of the treatments here at sick kids.  If however, sick – kids do not feel comfortable with it, we will have to fly down to Bethesda on a regular basis so that Maya can get her subsequent treatment.
  • On some regular interval (2 – 3 months),  new tumor samples may be taken to re-examine the genetic make up.  This task is performed to ensure that Maya’s cancer has not mutated to a point where the therapy is rendered ineffective.

Sounds great to me.  We will head down this Wednesday and will spend a week there to start on this exciting new course.  Our itenerary on this trip includes:

  • July 14th.  Meet the clinicians.  CT scan.
  • July 15th. MIGB scan.
  • Weekend in Washington.
  • July 18th. Biopsy.
  • July 19th or 20th.  Come back home.

While we’re there, we’ll be staying at the children’s inn at NIH.  I know we haven’t been updating the blog lately.  We will keep all of you informed on our trip and the details more often.


Thank you for sharing this journey with us.