Preparing for MIBG

Today I don't have much to say. Tomorrow will be a far more exhaustive review. I just did not want to leave everyone hanging. I really don't feel much different than I did yesterday. I am happy to see the results from Sydney's bone scan. I am still scared out of my wits for the upcoming MIBG. However, today should be relatively uneventful. We have an MIBG injection and a few labs in preparation for tomorrow morning's scan. Hopefully, this will go smoothly for Sydney. She has had so many bad experiences with IVs we are just hoping that this one will go as smoothly as the last. They used a J-Tip on her during the last injection and it worked flawlessly. She did not feel a thing. I just pray that these little successes will reduce the fear that I know is swimming around in her little brain.

My thought for today is just trying to ensure that Sydney is comfortable in her own skin.

That is my purpose.

A clean skeleton in her closet

First off, I have not forgotten everything. I know that when things aren't going your way you have to celebrate your small successes. Yesterday was just that. In fact, it greatly surpassed by expectations. Here is how it happened.

At about 9:00 AM I heard from the oncology team. Everything had been moved into fast forward. Sydney needed to be at the hospital at 10:30 AM for our first day of what was quickly turning into a scan whirlwind tour. No problem for Lynley and I but this presented some big concerns regarding Sydney. We had to call the school to get her out of class but, with a little girl who was becoming more and more self aware, we were concerned with what would be going through her mind. She knew she had just had a scan on Friday. She knew she was done with scans. What would be going through her mind when she realized that Mommy and Daddy would be picking her up? Would she be scared?

Lynley and I quickly game up with a game plan. We would both be simple and honest. Daddy would focus on being funny and silly. Lynley would concentrate on rolling her eyes at Daddy. If we did it just right we could get out of there without scaring her too much. Simply put, we told her that there was something on her scan that they just couldn't figure out. So, they needed some other scans to help tell them what it was. Sydney was comfortable with that. Lynley and I skated by this particular incident but I must admit we are in a totally new world with her. She is of age and starting to ask some very tough questions. It is the subject of a diary entry unto itself. But, suffice it to say, Sydney is aware.

On to the scan.

By about 2:15, Sydney was on the table in nuclear medicine. The scan took about 15 minutes. Sydney was in the zone on the narrow table slowly sliding between the 2 large plates that do all of the magic. Lynley and I stood closely by and chatted with the technicians Steve and Margaret. It was familiar for all of us. It was the same people and the same room. We have been coming here every 3 months for the last 7 years. Everyone in the room has been there with us from the very beginning.

We all know.

Oddly enough, the conversation was light. In fact, the only thing making anyone nervous was the fact that I was constantly walking back and forth to the monitor to get a better view of Sydney's scan results. The news was good though. This was the best skeleton I had ever seen. It looked absolutely clear. However, I also knew that I was looking for a 5 mm spot on Sydney on a monitor which was at about a 1:16 scale. I did not even truly know if I would be able to see the lesion on a screen so small.

Regardless, the scan looked good. My biggest fears of diffuse bony turnover or riddled little skeleton were alleviated. She still may have a little disease but judging by this scan it certainly wasn't widespread.

That was incredible news and worthy of a deep exhale.

The rest of the day was pretty family centric. We picked up the rest of the kiddos from school. Daddy put his little twerplet collating army to work at the local Mail Stop to get out a mountain of Lunch for a Cure materials. We then made our way home and then back out to Tae Kwon Do. For all practical purposes, it was a relatively normal night.

Later though, the phone rang.

It was Dr. Eames. She was ecstatic and called to let me know that the bone scan was absolutely clear. I might even say she was downright giddy about the news, but I certainly would not want to give the impression that she was anything less than perfectly professional.

In the big scheme of what lies ahead, this was a small success. But, make no mistake, it was a huge step in the right direction. It is worthy of some temporary celebration while we prepare for the next one.

Much more lies ahead.

So what does this all really mean? Well, the real key here is that we did not see several spots of concern. Furthermore, the fact that there is not activity at this spot would seem to indicate that this probably is not an aggressive process. Could it still be neuroblastoma? Absolutely. But, is it becoming less likely? You betcha.

Our next step is MIBG which they were thankfully able to schedule for this week. We will have a day of normalcy before our injection on Wednesday. In the meantime, though, there will continue to be more planning and scheduling.

But, for right now, your prayers are working. Thank you.

Yesterday, was a victory for purpose.

Out, out damn spot

Yeah, the results from Sydney's scans weren't great and the air is thick with irony. You may recall from as recently as my last diary entry that I was not particularly concerned about the chunk of tumor sitting in Sydney's retro crural area. I was not concerned because it was not Sydney's biology to have this kind of solid tumor relapse. What I mentioned was that I would expect bony disease - bony lesions - multifocal sclerotic lesions and guess what - damn it?

I was right. The retro crural fullness was not even remarked. But, that was not where the news ended. They found a bony lesion.

Yep, we have a new sclerotic lesion on her breast bone. When taken with other findings we now have a collection which makes them multifocal. The are all kinds of possibilities in the differential diagnosis but none is more likely than recurrent neuroblastoma for a child like Sydney.

So, there you have it, the awful, bad, ugly news.

Is there any good news you ask? Well, neither myself nor the medical team are sure this is neuroblastoma. And, in this vortex of bad news, there is actually still some hope. First off, this is a sclerotic lesion by CT. Frpm that we do not know it is neuroblastoma. Furthermore, while neuroblastoma is a likely culprit, for Sydney, we have seen some pretty strange bony abnormalities which appear to have nothing to do with neuroblastoma. So with a healthy dose of skepticism, here is why I am not freaking out all over the place.

First, while this is a new lesion, Sydney has had several of these lesions in her pelvis and spine. Over the years they have remained stable and any changes have always been attributed to the way the CT scan sliced the images. Sydney probably has 5 or 6 (or more) of these lesions, all in the 1 - 5 mm range. As I have said they have been stable and non assuming and, interestingly, they have not been confirmed by MIBG. The only thing frightening about this new addition is the fact that it is new.

While a bony relapse may look just like this, in Sydney's case, something just does not add up. It is not exactly what we would suspect. For this reason, it has us all a little skeptical. We have a "heightened sense of awareness" but no one seems to be screaming relapse -- yet.

None the less, this is Sydney, and she does have stage 4 neuroblastoma. We can't rule it out and we have to go find more. For this reason, we are now lining her out for a full work up. Today she will likely get a bone scan and it we will try to work her in for an MIBG scan assuming we can get some for her this week. Unfortunately, we may have to wait another week. They order MIBG on Friday for a Wednesday delivery so the only way we will get scanned this week is if they have some extra.

You can probably also expect a bone biopsy in the very near future. We need to know what it is and I am guessing we will just go in to get it. I know this will happen if we have a negative MIBG result but it is possible that we may go in either way.

So, what else could this be. Well, I am skeptical of some kind of autoimmune process. This is the same nebulous culprit that I have blamed for the other lesions that we have discovered on her body over the years. Don't forget the strange lesion we had on her tibia or the bonus we found on her radius. I think, and I am hopefully praying, that this is all one in the same. The problem is that we may never know what it is. The good news is that it would not be neuroblastoma and that is about all we can hope for right now.

So, we are praying with all of our might that this is not neuroblastoma. We are looking for a clean MIBG. I am expecting the bone scan to be positive but that does not mean neuroblastoma - just that something is causing bone turnover. Regardless of the cause, I think we already know that is happening. Although, I would suspect that a negative finding would tend to point to a less aggressive process. Given Sydney's history and lack of MIBG lesions I am hopeful and guessing that her MIBG will be negative. While certainly not a definitive finding, a negative result by MIBG would sure make me feel a heck of a lot better.

I know, it sucks.

But, this is the reason I have so darn much purpose.

Pray on.

Twerp #1 Scan Day

Good morning! Well, as you can tell from the title, today is scan day for Sydney. It has been six months since radiologists discovered the chunk of tumor sitting inside of Sydney's belly. Today we will hopefully get some insight as to whether it is dead or alive and growing or not.

To refresh your memories (considering the fact that we are all a bit older now), at our scans six months ago they discovered a 17 mm x 21 mm chunk of what looks like tumor deep within Sydney's abdomen, tucked up under her diaphragm in the retro crural space. Woah, you may say! Relax, I may say. It turns out this chunk of "tumor likeness" has been there for years - at least 3 and maybe since diagnosis. While we have seen changes in the size of the tumor over time these are thought to be changes due to the way the CT scan acquire images, not to actual changes in the tumor(or whatever it is). Outside of neuroblastoma this could also just be the result of "postoperative changes" or due to some other type of extremely slow growing tumor.

Given the fact that we have not seen any real change over the years I must admit that I am feeling pretty good - all things considering. I mean, if you are going to find a chunk of tumor in your daughter that has had stage 4 neuroblastoma, this would have to be about the best thing you could find.

Now, I just hope it is not growing. In fact, the best thing we can probably hope for here is that we so no change. A nice nondescript, non assuming, and non transforming chunk of tumor would all suit us just fine I think. That would be best.

I always hate to speculate about scans or relapse because I fear rocking the karma. But, for your information, here is why it does not bother me so much. A solid tumor relapse really isn't Sydney's biology. That just isn't what we would expect from her. From her, I would expect a bony relapse with disease riddling her skeleton. That is what her disease is "supposed" to do (nice, huh?). That is not to say that a solid tumor relapse is impossible, just less likely.

Ah, the trials and tribulations of knowing too much about a disease you wish you had never heard of in the first place.

Regardless, this morning Sydney has a CT scan. That is pretty much it for this set of scans. (Yes, we are all trying to reduce the unnecessary radiation from a full work up.) She will also have an ECHO and an EKG just to make sure her ticker is in working order. Yet, another area we are watching closely for late effects. You know, when you have an Olympic athlete you have to worry about that stuff. (Okay, fine, we would have to worry about it even if she wasn't.)

So, there you have it. We have a busy day ahead. If you need a purpose today, we could use some prayers thrown in Sydney's direction.

You know what our purpose will be today.

Twerp Status Quo

I know it would not surprise anyone if I told you that this week was hectic. Here it is Thursday morning and the week has gone by so fast I can't really tell you what happened up until this point. It has been a whirlwind - both personally and professionally. Out of the depths of my mind (it is fairly shallow so it makes it easy) I can recall a few things.

First, the kiddos are all doing pretty well. In fact, Ainsley even brought home a blue sticker from school. It is funny. That still actually shocks the hell out of me. Regardless, once again she achieved greatness and we were all treated to a 49 cent vanilla ice cream cone on the way home. I truly am thankful for McDonald's this year. Ainsley has been so good that anything more than a 49 cent ice cream cone could put us in the poor house at her frequency of good behavior.

The others are doing pretty well, too. Tae kwon do is still a 3 night a week hurdle but the kiddos seem to be enjoying it. Oh sure, they complain that they would like to stay home and ride their Razors around the block for a few more hours but that doesn't phase me. Another hour of unmanaged chaotic behavior can never be a good thing when it comes to those 3 . Yes, the organized structure of tae kwon do class suits me just fine. At this point, it may be the only thing that preserves our parental sanity on those evenings.

Well, enough of the update. I have cookbooks to get out the door.

There is still time left. Don't forget to get your Christmas Lunch for a Cure cookbooks - it is 2 gifts in one - http://lunchforacure.org/cookbook

Back to shipping purpose.

The Holiday Hustle

You know, this time of year would be much more enjoyable if it could just slow down a little bit. While I would never ever want to go back to the days of living in the hospital, there were a few pretty good things that came out of that experience. Sure, Sydney was fighting cancer and our future was scary and unknown, but, we were together. At that point in our lives the Christmas parties weren't all important. There was little reason to spend a small fortune to decorate the house as we weren't really there that much. The only thing playing on the television was Bambi or Snow White so we were not inundated with ads or the realities of the world around us (the news was not being watched). Shopping seemed less important too. In fact, we didn't even really need Christmas presents. Sydney was too sick and the only thing anyone wanted was for her to feel better.

In a sense, it was calm.

In retrospect, it was almost even slow.

Now, I haven't forgotten everything. Although, my rosy colored history spectacles don't allow much of the pain and fear back in. I know I don't want to return. I also know we were busy and life was hectic.

But still, there was something different.

The beauty of Christmas then was its simplicity. The simple fact that we were together. In fact, that was all that really mattered. Christmas was about focusing on each other.

Today I feel like things are going in a 1000 different directions and I find myself yearning for a return to the basics. I just want to relax and be with my family. I have grown weary of the 80 hour work weeks, the rush to spend more, be the life of the party at each and every Holiday party, and the challenge to decorate more than the neighbors.

It does not take long to forget what is important. I miss that gift from Sydney's treatment.

It is time to take a big deep breath of purpose.

A layman's perspective on the future of MIBG in neuroblastoma therapy

The last few weeks have been interesting on the neuroblastoma front. I have been part of several discussions on the future of MIBG therapy in neuroblastoma. Several of these discussions were in the research trenches but I also had the privilege of seeing Dr. Kate Matthay speak on the topic at Cook Children's the other night.

Secretly, I have always been a fan of MIBG therapy. I have seen many children with little hope for a cure have their lives saved in large part to this treatment. I know, without a doubt, that there are many children here today that would not have been without this treatment. For them, MIBG has been a miracle drug.

I am not alone with this insight.

There is a mounting army of survivors that can attest to the success of MIBG. Furthermore, there are many oncologists that have experienced these successes in children that they know they would likely have lost without the treatment. More and more are seeing the benefits of MIBG first hand.

As the research articles continue to flow from medical publications it is increasingly clear that MIBG will be an important part of neuroblastoma therapy in the future.

Many are aware of the toxicity of MIBG therapy. It is hard on counts and in many situations requires stem cell support. It is often this toxicity that has kept people from pursuing it as an option. However, now that the writing is on the wall that MIBG benefits may outweigh its side effects - especially for a certain group of ultra high risk patients - it should be a consideration. And, from what I am learning now, it looks as though it might be appropriate for all high risk patients.

As it stands now, MIBG therapy has a response rate of somewhere around 30 to 35%. That may not seem like a lot but, when you consider these response rates are based on the most heavily pretreated kiddos with the most resistant forms of the disease, that number starts to look better and better. Take this into consideration. Some of the best drugs we have for neuroblastoma, don't have a response rate as good as that. The old stand by, a combination of Topotecan and Cytoxan, has a response rate somewhere around 25% and our other relapse "go to" combination, Irinotecan and Temodar, only yields a response rate somewhere around 15%. Given those numbers, MIBG therapy begins to look really, really good - even considering the toxicity.

Make no mistake. MIBG is the most effective treatment we have in relapse when considering response rate. It is the toxicity cost that has to be weighed.

That is all fine and dandy but what really has intrigued me is the discussion of moving this treatment into up front therapy. Believe it or not, there is actually a study out of Europe that has looked at including MIBG at the very beginning of therapy. "Scarily toxic" you may say. However, "complete response" is what I have to say. It appears that MIBG may work even better in up front therapy and, as expected, response rates are even higher. Early data makes an argument that this could be the next big bump in survival.

Should MIBG therapy be one of the first things we do? Well, there are still some issues to work out. I think we need to get our stem cell collection completed before we hop into MIBG therapy to help reduce some of the secondary cancer risks. There are some logistical issues and other concerns as well. However, MIBG is on the horizon and it is closer than ever to being a mainstream treatment option for children with high risk disease.

Pilots are already offering MIBG in the up front window for ultra high risk kids and I would not be surprised to see MIBG as a component of a phase 3 trial as early as 2013.

Bottom line, if you have a child with high risk disease MIBG should be in the back of your mind. While today it is not for every child with neuroblastoma, it is certainly a strong option for a child with relapsed or refractory disease.

It is more ammo for your purpose gun.