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.