Thursday, March 31, 2011

Playtime's Over, Time to Get to Work

Hi all,

Ethan had his CT scan on Monday.  The lung nodules are unchanged, suggesting that it's not an active infection.  Ethan has been cleared for transplant and will be admitted tomorrow to start chemotherapy.

Ethan will be on a 12 day, reduced toxicity regime consisting of 11 days of chemotherapy and one dose of total body irradiation. He'll be inpatient from T-12 until he's stable and his white blood cell count returns to above 500 for at least three days. That could be as early as T+20, but a more realistic guess would be between T+30 and T+45, assuming everything goes reasonably smoothly.

One concern Sarah and I have wondered about is whether or not this reduced toxicity regime will kill off enough of his existing marrow to allow the new marrow to engraft.  Ethan's first regime, in Oregon, was a reduced toxicity regime, and his enfraftment peaked at 90% before slowly petering out over the last year. We're a little gun shy and don't want to go through another reduced toxicity regime just to have the same thing happen again.

We talked with Dr. Lund about this and he said that usually when a patient's transplant fails, it goes within the first 60 days or so.  In those cases, since the original donor marrow has already been removed, they're able to use a serotherapy to prepare the patient for a new unit of donor cells.

In this case, Ethan reverted to his own donor cells so that they need a reasonably strong preparatory regime to kill off his existing marrow. However, the toxicity from chemotherapy stays in a person's body for a period of 3-5 years post transplant and the toxicity is cumulative. They don't want to use the same full toxicity regime from the first transplant out of concern that his body couldn't handle the cumulative toxicity.  (Honestly, we forgot to ask how excessive toxicity manifests during transplant.  I assume that it would be problems with liver and kidney function, but don't know.)

Ethan is the third patient in this situation that UMinn has treated with this regime.  The first patient is post transplant with 100% enfraftment.  The second patient just had his transplant in January.  I believe Dr. Lund said that he's also 100% engrafted.  However he hasn't reached T+100 and his case has a number of other complicating factors.

Assuming that the nodules in his lungs are not active infections--and everyone agrees that is unlikely at this point--than Ethan is is really good physical shape to complete this transplant.  He's strong and his energy is good, and all of his major organs are in good shape.  Having said that, I expect that this transplant will be more difficult than his first.

Ethan came through his first transplant really well.  He never needed to be intubated, he was never in ICU, he only needed to be on IV nutrition (TPN) for three days.  Aside from the minor fact that it failed, it went really well. This time, I expect that he'll be in ICU at some point, I wouldn't be surprised if he needs to be intubated for some period of time.Add to that the normal concerns over kidney and liver function, blood pressure, heart rate, infections and everything else, I expect that it's going to be a bumpy ride.

In the end though, I've come to think of bone marrow transplants as educated crap shoots.  What we're trying to do to Ethan's body is completely unnatural.  The side effects of the chemotherapy and radiation, graft versus host complications, the side effects from the drugs they give him to treat the other side effects. It's impossible to predict or list what might happen while he's inpatient or what we'll be dealing with six months, a year or even 5 years from now. But he's healthy and strong going into this and he's at the best hospital and with the best doctors.  He's got the best shot he can have.

When we go in, I'm going to try to update this blog at least every other day.  And I'm going to try to take and post a lot of pictures. In fact, some posts may be mostly pictures.  It's hard to describe a transplant and side effects in words.  Some of the pictures will be fairly graphic. A lot of times, pictures can convey a level of immediacy and details better than 3 pages of text.  I hope and think that the pictures will be informative both for people who are following this blog to keep up with Ethan and for other families that are heading into their first transplant and wondering what it's like.

While Ethan is inpatient, I'll be with him six days a week.  Sarah and I will each take one weekend day, and Sarah will likely pull night shifts in the hospital once a week so that I can come back to the house and get some sleep.  Sarah's hands will be very full here at the house with work and spending time with Caleb.  We hope that she'll be able to post some updates "from the house" and let you know what's going on back here.

Tonight, Sarah and Caleb have tickets to the circus through HopeKids.  It should be a fantastic time. I've also posted some recent pictures on Facebook.

Okee dokee, Ethan's up from his nap and wants to climb on me.  Gotta go!

Friday, March 18, 2011

Spring break--Gettin' out of town for a few days

Minneapolis public schools--including the Ronald McDonald House charter school--are closed next week for spring break and Sarah has taken next week off from work so that I don't go nutso coo-coo from chasing both kids around the House next week.

So.... Since Ethan doesn't have anything scheduled until the end of next week, we're going to get out of town for a few days!

Sarah's colleague, Ann, has very graciously offered us use of her family's getaway on a lake in St. Croix Falls, WI, about an hour from here. Sarah just sent me some pictures of the house and it looks absolutely lovely, and just perfect.

This will be a fantastic chance for us to spend some family time, in what will (hopefully?) be the final few days before Ethan starts chemo. He's got a sleep study scheduled back at the House on Thursday evening and we're going to try an unsedated CT scan Friday morning. (Neither Sarah nor I are hopeful that this unsedated CT will be any more successful than the last one. We just don't think that you can stick a 2 1/2 year old in a CT scanner and expect him to hold perfectly still for 60 seconds. But the transplant staff don't want to sedate him again if they don't have to, and we certainly understand that.)

If the unsedated CT doesn't work on Friday, we've got a sedated scan penciled in for the following Monday. IF the results from the CT come back okay, then Ethan will likely be admitted Thursday or Friday (two weeks from now) to start chemotherapy.

While we've been on hold we've watched two  boys with Hurler's that arrived at the same time we did (Zach and Derick) go through transplant and be released from the hospital to come back to the House for their outpatient monitoring period. I am glad to say that they are both doing very well!

Some small part of me wishes that we'd gone in on schedule and, like them, were already through the worst of it. But mostly, I'm incredibly grateful for the last three months. I think we've all enjoyed the time, even Sarah, who is still juggling work and her three boys. I think we've all become closer since we got here; that's especially true for the boys.

I suspect the delay has been the most difficult on Caleb. He's made a handful of good friends here, only to watch them leave. I think that churn rate in finding and keeping good friends has to have an impact on him, but it's too early to say how it might manifest.

We joined a local organization called HopeKids that puts together events for sick kids and their families. We've taken Caleb to a few of their events: an NBA game, a movie, and snow tubing. Sarah and Caleb did the snow tubing together and that seems to have been the most fun.

The other day, Caleb asked me to teach him karate. I took karate classes for a few years when I was a kid, but I am hardly qualified to teach it and provide the structure that a little kid needs when learning it. But I've been hoping that Caleb would find a sport or activity that he likes enough to pursue, so I was really happy to hear he want to start an extra curricular activity

Yesterday, I took Caleb to a local karate school so he could watch class and see if he wanted to take lessons. Caleb is VERY slow to embrace new things, but he seemed to like it, in his own way. I told him if he wanted to take classes, I would practice with him at the House. And I even told him that after we got back to Oregon, I would sign up for classes, too. He seemed to like that and said, yes, he wanted to give it a try.

But when I had to amend that and say that Sarah would have to take him to class and practice with him once I went into the hospital with Ethan, Caleb changed his mind and said he didn't want to take classes. I was really sorry to hear that, both because I want him to try new things that he might like, and because a class like that might help him make some friends who will be around for a while.

But anyway, Sarah and I are really looking forward to getting out of town. Once Ethan goes through transplant and gets released, he'll need to stay close to the hospital for regular monitoring. So, this is likely to be our last chance to get away for some time to come.

At the end of next week, we'll post some pictures of what we've been up to this month.

Speaking of pictures, there is a freelance photographer who volunteers his services and takes pictures of families at the House. We spent about 20 minutes last weekend and he got some great shots! You can see them online here:   Many thanks to Jim Bovin and the Ronald McDonald House for making them possible. 

We'll post again whenever we get the results of the CT scan, so next Friday or early the following week.

Thanks for being there.

Thursday, March 3, 2011

Still on Hold (2 of 2)

Okee dokee. It seems as if we've achieved clarity and have a decision on the biopsy.

The clarity is that it is only three sites that we're looking at, not four or an infinite number.

The decision is not to do the biopsy. We'll continue on the Posaconazole anti-fungal and re-CT at the end of March.

Ethan and I are in the infusion center today, and Ethan's transplant doc, Dr. Lund came by and we reviewed each of the CT scan reports.  Dr. Lund confirmed that there are only three sites we're looking at.

I explained to Dr. Lund that I must have confused the small granuloma that's at the apex of his right lung, thinking it was on the bottom of the lung.  Dr. Lund was kind enough to confirm that I was mistaken.  (Though when I later talked to Sarah, she remarked that she'd been at that meeting to and that Dr. Lund had presented it as being at the bottom of the lung.  But Dr. Lund's got more letters after his name, so he must have a better memory. ;-)  )..

Anyway, since it's been my practice with this blog to put you all to sleep with a whole bunch of medical details you don't really care about and can't keep straight, here's the history of the three sites from each of the scans.

(margin of error +/- ~2mm)

                                               1/10          2/9          2/28
-  lacy area bottom                  described as lack of full inflation
both lungs                              

- right upper lobe                    4.2mm       3.7mm     4mm

- middle upper                        3mm           6mm       4mm
left lobe

Okay, does that make sense.  We're dealing with really tiny nodules and the growth noted in the left lobe between 1/10 and 2/9 is just outside of the margin of error.

Dr. Lund talked with pulmonary on Wednesday and pulmonary said that even if we were able to find and biopsy the right upper lobe site, they still wouldn't sign off until they got a stable or reduced scan of the middle left lobe at the end of March.  The left lobe site is is the one right in the middle of the lung.  There's no way to biopsy that without removing a significant portion of the lung.  Clearly, we're not going to do that.

So, we're going to stay the course on Posaconazole and rescan at the end of March.

As I've said before, while we do want to get this moving, we're okay with taking the time that we need to give Ethan the best shot.  The nature of lysosomal storage diseases like Hurler's is such that one month isn't going to make any measurable difference for Ethan.  As long as he keeps receiving enzyme replacement, he'll be fine physiologically.  And assuming that he's been producing some enzyme through last year, he should be fine neurologically for an additional month.

When we first came out, we figured we'd be out here until the end of June or so.  We'll have lost three months before we even start. If I had to guess, I'd say that there's not much chance we'll be home before mid to late August.  That's assuming that there are no post transplant complications--that's a big assumption given Ethan's complex history.  At this point the easiest thing to do is just to know that we're here for the duration, as long as that might take.  If we try to assign an artificial deadline to this, we'll go crazy.

(Of course there's always a possibility that our insurance company will tell us to come back to Oregon for the rest of March and then come back out to Minnesota after Ethan completes his Posaconazole and has a follow up scan in Oregon.)

The biggest impact of this additional delay will be on Sarah's parents who are taking care of our dogs; on our neighbors Steve and Susan who are caring for our house and lawn; Caleb's kindergarten teacher who is taking care of Caleb's fish (if any are left) and on Caleb.

At the moment, we're doing fine.  After running so hard for the last year and a half, this has been easy time for us.  Caleb's spring break is coming up the last week of March. Assuming, the insurance company doesn't tell us to come home,  I'm hoping that Sarah will be able to get at least three days off, and we'll likely explore options to get out of the city for a few days.

Thanks for being there and hugs to you all.

-t, s, c, & e.

Wednesday, March 2, 2011

Still on Hold (1 of 2?)

The boys at bedtime last night (with fresh haircuts!).
They'd both love to sleep together.  But a six year old
and a two year old trying to get to sleep in the same bed?
There's no way that would end well!
Sorry we haven't done an update recently. Before Monday, there wasn't any news to share.  Come to think of it, at the moment, we still don't have anything definitive to share; which makes me think that this post will be more in the "blog as therapeutic talking it out" vein, than a "here's where we are and what we've decided" kind of thing.

At the moment, we're on hold for another four weeks.  But we're talking about another biopsy, so that may change.  Hopefully, we'll get some more information today; so after three weeks of silence, we may end up putting out two updates within a day or so.

Ethan had his bronchial lavage on February 16th. The rate of success on lavages is generally very low, so no one was surprised that the lavage cultures came back negative. In other words, the lavage wasn't helpful in figuring out what type of infection the nodules in his lungs are and what specific medication would kill it off.

Monday morning, Ethan had his third CT scan. That showed that the granuloma in his upper right lung is still 6mm. (You may recall that this particular granuloma was 3mm in the 1/10 CT, and 6mm in the 2/19 CT.)

We met with Dr. Lund (from transplant) and Dr. Laguna (from pulmonary) together Monday afternoon to discuss the results. The doctors were hoping to see a reduction in the size of the granuloma that would indicate that the anti-fungal medication (Posaconazole) is treating it effectively. Since there wasn't a reduction, that leaves us with four options:
  1. We can assume that the granuloma is left over from a previous infection and is not currently live. That would assume that the difference in measurement between the 1/10 scan and the others would be due to where the CT bean bisected the granuloma. If we assume this, then can proceed to transplant ASAP. But, if we're wrong, a live infection will kill him when he starts transplant.
  2. We can check with surgery again and see if they feel they can do a biopsy of this nodule. When we've talked about this with pulmonary before, it was felt that this nodule is too far inside the lung to get at easily or effectively.
  3. We can continue the Posaconazole for another four weeks in the hope that we'll either see a reduction or that the Posaconazole just kills it in place.  Realistically, that would push the transplant back an additional six weeks: four weeks for the meds, two weeks for the CT, review, and if it's okay, then review of the final transplant protocol by the insurance company.
  4. We can put him on intravenous Amphotericin, a stronger anti-fungal, but one with potentially lethal side effects.

Our transplant doc, Dr. Lund, strongly suspects that we're dealing with the first scenario, and that this isn't an active infection. I'm pretty sure he feels comfortable starting chemo. But, he's deferring to the lung specialists in pulmonology. I think that's as it should be. There are any number of reasons why the BMT mortality rate here at UMinn is half of what it is elsewhere. Part of that success rate is experience dealing with post transplant complications. But part of it is the comprehensive workup Ethan went through and listening to the expertise of all of the various specialists.

We've had a surgical consult Tuesday afternoon to discuss option 2. I'll come back to that in a minute.

With option three, the operative question is "if we do another four weeks of the Posaconazole, and there's no change, will everyone feel comfortable signing off on the transplant?". If, after four more weeks of medication, we do another CT and it shows no change, will pulmonary feel okay signing off on transplant?

Since the granuloma isn't growing, no one really wants to consider Amphotericin. Though I suppose if another CT scan shows that it is growing and the Posaconazole isn't working, then that we might reconsider that.

All things considered, I want to agree with Dr. Lund that it is most likely an old infection and not alive. (Though in truth, I have absolutely no knowledge, reason or expertise to base that belief upon.) Pulmonology responded to that by saying that there's no sign of calcification on the CT scan as might be expected if it were an old, inactive infection. Since I know essentially nothing about granuloma and less than nothing about the calcification of granuloma, I'll let them argue it out.  I'm in no position to argue the technicalities of this.

The discussion with Dr. Saltzman, the surgeon, yesterday was as helpful as it was maddeningly confusing. It was helpful because he said that they could do another biopsy with the same generally low risks as the first one and try to get a sample of the 6mm nodule at the apex of Ethan's right lung.

Well, that was good to hear.  But, wait a minute.  When did we start talking about a 6mm granuloma at the top of Ethan's right lung?  I thought the granuloma we were tracking was inside the upper center of the lung.  I don't even recall anyone mentioning another granuloma at the apex of his right lung. 
Ethan running around the infusion
center before his weekly enzyme
top-off.  It was "Superhero Day" at the
hospital, and Ethan got a
Superman balloon.
I mean, I assume the doctors all know which granuloma they're talking about right?  And how many of the damn things are there?  I'll leave that question aside for now, though it feels like I need to go in again and just sit down with them and go through the CT scan with them frame by frame.

Assuming that the granuloma in the right apex is the one that went from 3mm in scan 1 to 6mm in scans 2 and 3, then it seems like a second biopsy is the way to go.  If we biopsy that specific site (and they can actually find it and get a sample--not a sure thing), then the results of that would be definitive, right?

At the end of the surgical consult yesterday, I told the surgeon, Dr. Saltzman, that the risks of a second biopsy seemed acceptable, but 1)I wanted the head of his transplant team, Dr. Lund, to actually make a recommendation that the risks were acceptable, 2) we're all talking about the same site, and 3) that if we did it and it came back negative, that everyone would sign off on going to transplant.

After talking things through in this post, it seems clearer to me that we're likely to go with a second biopsy.  Though, obviously, we've still got a little work to do to confirm "what", "where" and whether or not it would answer the questions that are on the table.

I'll try to post an update as soon as we figure all of that out.

In House news, Caleb continues to do really well, though he says he's homesick, and Sarah and I think that will get a lot worse before it gets better.  Caleb's continuing to do very well in the charter school here, and Sarah and I are really happy with all of the progress that he's making.  He's learning to recognize more letters and words, and he's doing really well with his numbers and adding and subtracting single digit numbers in his head.

Wes (l) and Caleb (r) with life sized
cardboard cutouts of two of the Timberwolves.
We recently signed up with an organization called HopeKids.  They organize events for sick kids and their siblings and get tickets for them to go to sporting events, plays, and special movie screenings.  I've gotten more and more concerned about taking either of the kids out in public to places like the science museum or the kids' museum where there are lots of kids that may be carrying a bug that the boys might bring back into the house.  It's really nice to have opportunities to get one or both of the boys out of the house to an event where the other parents understand that and there's less risk of the kids bringing something home.

This weekend, HopeKids had gotten a bunch of tickets to see the Minnesota Timberwolves (the local NBA team) play.  I took Caleb and another kindergartener that's at the house, Wes. Unfortunately, the seats were way up there, three rows from the top.  If you've never been to see a particular sport, it's hard to understand what's going on, much less get into the action, if you're that far away.  Not surprisingly, both boys were ready to leave just seven minutes into the game--once they'd finished their soft pretzels.  Oh well.

In March, we've got Caleb and Sarah signed up to go see a special screening of the new kids movie, Rango, and for an evening of snow tubing.  Hopefully, Caleb will have more fun with those.

In more difficult RMH news, last week we lost one of the kids here.  Sanjay Baca died from complications of his bone marrow transplant.  Sanjay suffered from a genetic blood disorder called Thalassemia Major.  Sanjay and his mom, Karla, arrived at the house about the same time that we were originally scheduled to start transplant.  Sanjay was a lively, joyous, 10 year old with an infectious smile. He is survived by his mother and father and three lovely bothers and sisters.  We will very much miss the chance to share his smile again and to get to know him better.

That's all the news from here at the moment.  Thanks for letting me talk this through with you.  Our conversation has been helpful. Time to e-mail the docs.