Saturday, November 20, 2010

What's Normal?

I think this will be a short update (yeah, I know, I've said that before), then I've got a favor to ask.

The last couple of weeks we've been trying to figure out what's normal.

Ethan's 27 months old now, and until recently we'd never tried to get him to go to sleep by himself. We would always hold him and rock him while he laid on a big pillow on our laps.

I suppose it's silly that we never worked to teach him to go to sleep in his crib. But, with a kid who has spent so much of his life sick or in pain, or under the effects of drugs that screw with his sleep or upset his stomach, comforting and putting him to sleep have seemed two sides of the same coin.

We know he can go to sleep on his own because he does it during nap time at daycare, when all of the kids just curl up on their cots and go to sleep.

Now, we're starting to try to teach him how to go to sleep at home, and as natural as it may be for a normal kid, I find myself torn about working on this simple thing with him even now. Because I know that within two months his body is going to be torn apart by chemotherapy again, and I know that I will pick him up and comfort him until he can go to sleep, however long that may take.

Blood Pressure
Ethan had a check up with his primary care doctor a few weeks ago. She and we wanted her to double check Ethan ears and ear tubes after he came off of back to back ear infections. When they did his check in vitals, Ethan's blood pressure was 115 over sixty something. Dr. Oltmans commented that it was high for a kid his age, and my response was that it was ten points lower than it usually is because the docs at Doernbecher recently put him back on Amlodepine to counteract the effects of the steroids.

Dr. Oltmans raised the point that even though he's on steroids and has MPS and has been through transplant, there's no reason to allow a higher blood pressure, because it doesn't do anything for him except make his heart work harder. So, she gave us a referral to a specialist and we're scheduled to take Ethan in on Dec. 2.

Nobody at Doernbecher has seemed overly concerned about his blood pressures being in the 120s, so I never knew that pediatric blood pressures were typically in the 80s for kids Ethan's age.

So, the question becomes what's a normal blood pressure for a kid like Ethan? How do the combination of MPS and transplant affect his blood pressure? How aggressively should we treat it? Is it even worth treating it more aggressively at this point since transplant will throw it completely out of whack for the next six months? Add to that the effects of the steroids on his blood pressure are cumulative, at least until he comes off of them, and I wonder if anyone can answer any of these questions with any certainty.

Prednisolone and Rash
We're continuing to slowly taper Ethan off of the prednisolone steroid. He's coming off of it pretty well now. His rash recurs every few days, but it's nowhere near as fast as it was and there were a few days in the middle of the week where we were able to go without the steroid cream as well.

The steroids are still screwing with Ethan's sleep, but it's not as bad as it was. He's slept through the night the last couple of nights, which has been nice for all of us.

Donor Search and UMinn
We knew that UMinn had start the donor search 3-4 weeks ago, but we hadn't heard anything from them. We finally e-mailed them yesterday and learned that they'd made some progress. Dr. Orchard has decided to go with cord blood again, as opposed to an adult donor. That's consistent with the research that we've done which indicates slightly less risk of gvhd with cords.

They've lined up two units and I think those arrived at UMInn yesterday for secondary testing. Both units are 5/6 matches. Ethan's first transplant was a 6/6 match. A five out of six match is perfectly within the guidelines, but I think we'll have to wait till we get there to talk about what the clinical significance or consequence of the lesser match might be.

We were a little surprised that no one at UMinn had contacted us with the results of the search or to start scheduling things. After e-mailing and talking to them on Friday, Sarah and I got the sense that since they do so many Hurler's transplants, they may feel like they've got the playbook and don't take as much time to keep families informed or to explain.

Sarah and I are agreed that we will have to disabuse them of that style when we get out there. Maybe if we'd gone out to Minnesota originally, we could have just deferred to the doctors and wouldn't have felt as much need to have everything explained to us and wouldn't have ended up doing so much research on our own. But the last 17 months have trained us that we have to ask the questions and get the explanations and know what's going on. So the pros at Minnesota are just going to have to deal and make some extra time for us.

Ethan's Latest Trick
He seems to be able to whistle!

A Favor
Last but not least, we've got a favor to ask.

We would like to ask everybody reading this to put together a sign or poster that we can hang up in Ethan's hospital room and back at the Ronald McDonald House.

We would greatly appreciate signs from letter sized to poster board with pictures of you and your family and messages of love and support. They would be wonderful reminders of all of the people who are sharing this journey with us.

It would be nice if some of you could do signs or posters for Ethan at the hospital and others could signs or posters for Caleb at the house so he knows that he's important, too. Please feel free to get creative, whatever you have the time or inclination to do would be much appreciated.

It looks like we'll be leaving Portland around December 27 and it will probably take four days to drive out, so we should get there on New Years Eve. I think you can send them to us at the Ronald McDonald House even before we get there. The address is:

Sarah Aaserude & Todd Waddell
Ronald McDonald House
621 Oak St. SE
Minneapolis, MN 55414

Thanks very much for being there.

Friday, November 5, 2010

Working the Process

Sarah and the boyos with
Elizabeth & Ryan and their mom, Nancy.

Halloween Stuff
Starting on a fun note, we had a great Halloween!  The boys went as matching pirates.  A couple of Caleb's friends came over to our neighborhood, so we had four kids and three kids-at-heart trick or treating.

The older kids had a blast running from house to house.  We had Ethan in his stroller since we knew he wouldn't be able to keep up with them.  At each house everyone did the candy thing, then the older three would run off and we'd plop Ethan back into the stroller and Sarah or I would run with the stroller trying to get Ethan there before the others finished up.  When we got there, we'd pull Ethan out of the stroller and he'd amble up to the house.

This was Ethan's first real Halloween because he was in-patient at T+2 for his first transplant at that point last year.  But he quickly figured out the drill, and soon was holding out his bag, mumbling a one syllable something or other that we'll assume meant "trick or treat" and looking at what goodies were being dropped into his bag.

Everyone had a great time, even Caleb's friend, Ryan, who tripped and sprawled on a concrete driveway when making a hasty post house exit. He recovered pretty quickly after a hug and snuggle with his mom.

After about an hour, Ethan got tired, and Sarah started to head back to the house with him.  She'd gotten about a block away when the others realized that Ethan was heading home and decided that they were done too.  Caleb took off running down the block shouting "Mom!", "Mom!", loud enough to wake the dead.

Ethan Stuff


Medically, Ethan's condition has been reasonably stable over the last month.  The steroids he's taking have been doing their job, damping down his immune system.  Ethan's anemia was caused by anti-bodies that attached themselves to red blood cells.  White blood cells attacked the anti-bodies and in the process destroyed the red blood cells too. With his immune system suppressed more than usual, his white cells aren't fighting any anti-bodies, so the anemia hasn't recurred.

Last week at his
new fighting weight
Ethan's doctors wanted to get him off of the steroids as quickly as possible because the steroids have all kinds of negative effects on his internal organs.  They also, at times, make him more irritable than usual, make it hard for him to sleep, and make him pack on weight.  (He's put on 6 pounds since he was admitted, and is a big hunka love at the moment!)

They had him on a quick taper of the steroids, but that caused a really fast GVHD rash on his arms and legs.  So, we've backed the dose back up and we're on a slower taper schedule that will run through the end of November assuming the hemolytic anemia doesn't reoccur. His skin looks great now, but it's anyone's guess what his final fighting weight will be by the time he finishes the steroids.

We've also learned some more about the autoimmune hemolytic anemia. When Ethan was in-patient, the doctor (not Ethan's regular doctor) told us that the anti-bodies had probably formed in response to a virus or the amoxycillin that Ethan had been taking for his ear infections.  They said that it didn't have anything to do with his transplant.  That didn't sound quite right, so I did some more digging and found out that this type of anemia does sometimes occur in transplant kids and so went back to talk with the head of Ethan's team.

It turns out that the anemia is likely related to the transplant, either indirectly or directly.  The current thinking is that Ethan's month long fight with adenovirus in July and August resulted in a strong immune system response where his white cells were stoked up and ready for a fight.  The anti-bodies they were fighting may have developed in response to the amoxycillin, but it is perhaps more probable that the anti-bodies developed in response to his donor graft itself.  If that's the case, it means that Ethan's developed a hemolytic (tearing up red blood cells) response to what remains of his bone marrow graft.  That's not a good thing, and as we try to taper Ethan off of all of the immune suppressants to get him ready for the second transplant, there's a real concern that it might recur, and would be a significant complication that he doesn't need.

Transplant Stuff
Our insurance company has been busy negotiating the contract with UMinn over the past couple of weeks.  The process has moved reasonably quickly, but we just received the final approval to begin the pre-transplant screening and donor search last week.

A couple of weeks ago, Sarah and I had an hour-long phone conversation with the head transplant doc there, Dr. Orchard.  He said that they will likely do a full ablative, full toxicity chemotherapy regime with Ethan to clear out all of his existing bone marrow to make room for the donor cells.  He said that the pre-transplant workup will be a full week of tests done on an outpatient basis the week before starting chemo (which means no pre-transplant trip to Minnesota).  Then, they typically start administering one prep-drug on T -21 for a day or two, also on outpatient basis, then finally start the other chemotherapy drugs at T -9 in-patient for the rest of the process.

During that call, it became clear that we would likely be in Minnesota closer to six months than to four.

For his first transplant, chemotherapy started at T -10 and had a 35-40 day total inpatient stay. This time, between the workup and the 21 day chemo schedule, we're looking at essentially a full month before transplant (T+0).  Then at lest a month, probably closer to two, in-patient post transplant. Then probably two-three months there for outpatient monitoring.

In my last post, I said that if the stay looked to be more than four months then Sarah and I would have to make some decisions and changes to accommodate that.  Over the last couple of weeks, we've both been talking with our employers to put agreements and processes in place.

Work Stuff

With Sarah's office it's relatively easy.  Her team has staff all around the country, and even though Sarah's got eight people who work under her, the organization is used to long-distance and virtual teams. Sarah did talk with her HR department and they recently agreed to change the organization's leave sharing policy to allow leave sharing not just for employees' medical conditions, but for their immediate family as well.  That's a huge help since Sarah's only got about two weeks of paid leave available at this point.

For my job, we're looking at a couple of things.  As I said before, I'm going to take a couple of months of leave while Ethan is in-patient. PSU has a policy allowing employees to borrow sick leave. Technically, the policy only applies to employee's personal medical conditions, not to their dependents. However, before Ethan's first transplant, the university's HR department worked with the Oregon state government personnel people to approve an extension for me to use that when caring for Ethan.  I didn't have to use it at that point but will rely on it a great deal this time around.

I've also got a lot of fantastic colleagues both here at PSU and at Oregon State who are not only willing to give me some of their unused sick leave, they've appealed to PSU HR for a way to do that.  I have no idea if that will come to pass, nor do we know how much the change in leave sharing policy at Sarah's company might help.  But I do know that we are both truly grateful to our friends and our organizations for all of their support.

In addition for ways to maximize how much leave we have, I have also asked my boss for permission to go to half-time for the 2011 calendar year.  It was not an easy decision to ask for that change.  However, I think it's an acknowledgement of where we are now and the fact that we're going to be pulled in even more directions over the coming year.  We've been working full-time and burning through lots of leave at work.  At home, we are making things work.  We are finding time to juggle Ethan's medical needs, work, and building some time for the kids to have fun, "normal kid time".  But in doing all of that, we've made some choices to let some things go, such as exercise or Sarah's choir, or all but the most minimal amount of yard or housework.

I hope that going down to half-time next year will help us not just manage Ethan's care better but will allow me to do things that free up time for all of us so we have a little bit more time to breathe and attend to those things that have been necessarily farther down on our list.

Minnesota School Stuff

One of the happy things we discovered was that the Minneapolis Public School system has full-day kindergarten, unlike the half-days at Caleb's school here.  We've decided to have Caleb go to the public elementary school in the neighborhood near the Ronald McDonald House instead of the half-day kindergarten at the charter school in the house.  Hopefully, the additional activity will help him make more friends there and will pass the time better.

MPS/ML Stuff

Over the weekend, I was reading a newspaper article about a teenage Texas Rangers fan who has Hurler's Syndrome and was excited to be going to see the World Series.  When talking about how rare Hurler's is, the article mentioned that there are fewer than 1,000 active cases in the entire world.  I hadn't heard that number before and it kind of rocked me on my heels.

The often cited figure is that the incidence of Hurler's Syndrome is 1 in 100,000 live births.  We usually figure that there are 40-50 kids born in the U.S. each year.  But I'd never stopped to think about how many active cases there might be worldwide.

Hurler's and the other MPS/ML diseases are not specific to any race or ethnicity, they occur worldwide.  Most unfortunately, MPS/ML children in the developing world often receive an accurate diagnosis much later in life, if at all, when the diseases have progressed further and more damage has been done. It's also true that even with early diagnosis and treatment many Hurler's kids die young or die in transplant.   But 1,000 active Hurler's cases was still a staggering reminder of how rare this specific disease is.  It makes me more grateful that treatment options like BMT and ERT have been developed. (It's also worth noting that number does not include the other MPS/ML types or the number of patients suffering from lysosomal storage diseases as a whole.)

Looking at that larger community of MPS/ML patients and their families; over the last two months, the MPS/ML community has lost a number of our children.  Sarah and I want to take a moment to commemorate their lives and offer our sympathies to their families.  Each of these children is an inspiration on overcoming obstacles and living life to its fullest.  And each of these families is proof that each of us is as strong as we need to be and can live, laugh, hope and love amidst heartache.

Devon Clark, MPS II, Portland, OR, 17 years old
Kraig Klenke, MPS III, 17 years old
Joshua Kenneth Burt, MPS II, 11 years old
Ashton Whitaker, MPS IIIA, 7 years old

We never thought that we could grow so close or care so much about children we've never met.  But Sarah and I live, laugh and cry with the joys and heartaches of the entire MPS family.  We feel their loss personally and share their hopes that their children are flying on the wings of angels, free from the limitations and pain they experienced in life.