Tuesday, May 24, 2011

T+41: Going Gangbusters

Howdy all,

Caleb and Ethan in their Twins hats.
Next week will be the half-way mark on our trip to T+100.  They say the first 100 days are the most critical for complications and for graft failure.  So far, Ethan is doing great!  He's doing so well that it's becoming difficult not to look at the calendar and start tapping our feet waiting for July 22 to arrive.  As always, though, we know that his health is a fragile and precarious thing and it can change quickly.

Overall, Ethan is doing wonderfully.  The limp that he picked up the day after he was discharged and then held onto for a week and a half finally went away this past weekend, and he's back to his normal running knock-kneed--one arm swinging--self around the house.  Last Tuesday, after we dropped Grandma Lilianne at the airport for her return to Florida, Ethan had a sedated MRI, which showed no evidence of infection or avascular necrosis.

We met with Dr. Larson in Orthopedics here twice about the limp and we're still not sure what caused it.  Dr. Larson said it might be due to just general weakness in the muscles around his hip and thigh.  As with many hurler's kids, Ethan's pelvis is abnormal and the hip socket isn't as deep as it should be.  Ethan's isn't as bad as some, but his leg muscles still have to work harder to hold everything in place.

I made a run to the drug store the other day, but Ethan
didn't settle down for his nap as we expected.  Here's
Sarah multitasking on a phone meeting with her office and
trying to get Ethan down for a nap.
We're also happy to see that Ethan has not needed any infusions of platelets or red blood cells since he was discharged.  That means his body is back to making them again, as it should.  

Last Monday, we got some updated chimerisms and I think the message there is positive but too early to be definitive.  His CD15 marker, which they tell us is the marker that  signifies his ability to produce the missing alpha-L-iduronidase enzyme is 100% donor.  That's absolutely fantastic because it means that his body may now be starting to produce the enzyme necessary to break down the glycoamainoglycan (gag) or waste chains that cause all of the damage. 

We also heard last week that his CD3 marker was only at 42% donor. The CD3 marker signifies the body's ability to produce T cells to fight infection.  The transplant docs say that this is normal with the campath protocol that Ethan was on for this marker to take some time to come up. They also say that even if this marker doesn't come up, it won't drag the rest of the graft down.  Nor is it an indicator of potential graft failure, which is still possible, but unrelated here. Even so, after fighting mixed and declining chimerisms for a year and a half after Ethan's first transplant, we're a little nervous and really want to see this marker come up.

The kids organized a party in the gym last week.
Here are Caleb and Ethan playing twister.
In other news, last week we also met with Dr. Rothman in neurology to try to understand why Ethan has always had a hard time sleeping. Unfortunately, from the minute he came in the room it was clear that this wasn't his area of research.  In fact, when your doctor shows you how to look things up on the pubmed database, you can pretty much figure the conversation is not going to yield any new information.  Fortunately, the melatonin is working well.  Ethan's getting to sleep much, much more easily.  And we haven't had as many nights where he wakes up in the middle of the night and is up for 2 or more hours.

Ethan is doing so well, that the transplant folks have said that they only need to see us twice a week at this point.  Since we have some time, I'm filling up Ethan's schedule with speech therapy (twice a week) and occupational therapy (once a week).  Ethan will be 3 years old in July and at this point, he only speaks or signs about 40 or 45 words, so he really needs the speech therapy.  Occupational therapy focuses on his fine motor skills which are impacted by the gag buildup in his nerves and tendons.  They say that Ethan's fine motor skills are better than most for a hurler's kid, but the rehab therapists here have a lot of experience with Hurler's kids, so I want to take advantage of that expertise while we have it.  Not to mention the fact that it will give us good excuses for getting out of the house and keeping active. 

Theoretically, Ethan should start kindergarten in the fall of 2013. Given how delayed Ethan's speech is, I've started to wonder if we should hold him back from starting kindergarten, or if we should see if he can take kindergarten twice. If this transplant holds, I think his speech and hearing will improve.  I wonder if that extra year will help him catch up rather than just continuing to be behind if they lower his academic requirements and allow him to advance.  Obviously, we've got some time to think about this and see how he progresses from this point.  But I think I'm going to check in with some of the other Hurler's parents, especially those whose kids had late transplants or second transplants to see if their experiences tell us anything.

Caleb News
Caleb has been doing great.  School is almost out, I think he's only got a couple of weeks left, so we'll have to see how things shape up for the summer.  The house staff says that they do a lot of activities for the school-agers

Caleb and Sarah at the Science Museum.
Sarah and Caleb went to the Science Museum this past weekend to see the King Tut exhibit.  Sarah says that the Science Museum here has a number of half day summer programs each week.  We're going to look into that and find out more about what the RMH staff have in store for us.  Hopefully, we can put something together that he'll enjoy.

Yesterday, the RMH staff organized an outing to see the Minnesota Twins play (our almost home team) the Seattle Mariners.  Caleb and I went and had a good time.  Considering Caleb's handicap--that his dad isn't a big baseball fan, and doesn't watch it at home--he did a great job paying attention.  We made it to the end of the fourth inning before Caleb said that he was ready to go home. It didn't seem to bother him that the Twins had just scored three runs to tie things up.  According to the box scores the game went to extra innings with the Mariners winning 8 to 7 after a run in the top of the tenth. Sounds like it was a great game.  (I may not be a huge baseball fan, but I'm not comatose.)

Tonight, I think we're going to go to Izzy's Ice Cream after dinner. Ironically, one of Sarah's cousins from Oregon introduced us to Izzys, and we're glad she did.  It's some of the best ice cream you've ever had.  I HIGHLY recommend having a scoop the next time you're in Minneapolis.  Thanks, Cara!!

Well, thanks for letting me ramble for a while.  Assuming that chimerism number picks up (and we think that it will, then it's all good news at this point.  We're hoping for lots more good news over the next 59 days.

Saturday, May 14, 2011

Sunday, May 15 is MPS Awareness Day

Sunday, May 15 is International MPS Awareness Day. The mucopolysaccharidoses are terminal diseases for which there are no cures. Please wear purple tomorrow to help raise awareness and to honor Ethan and all of the others who have fought and continue to fight these terrible diseases. Please consider supporting essential research for a cure by donating to the National MPS Society at Thank you very much.

Ethan is continuing to do very well. His blood counts are holding strong. He's making enough red blood cells and platelets that he hasn't needed recent infusions of either.

He's vomiting once or twice a day right now. That's unfortunate and a drag for everyone, but pretty much expected. He's still on IV nutrition and he's not eating much. His stomach is naturally pretty fragile right now, and the only thing in there is stomach acid, then we're throwing in tons of oral meds that cause nausea. It's understandable that there's a whole lot of puking going on. We're slowly reducing the calories in the IV, hopefully that will make him hungry and help him to want to eat.

Ethan had a sedated CT on Friday to check his granuloma. They look good. The docs have been keeping him on a stronger IV anti-fungal because he's still immune compromised. I think they may move him down to an oral med next week if his blood counts continue their steady improvement.

The day after he came home from the hospital, Ethan developed a bad limp. He doesn't let it slow him down, he can walk on it. But it is causing him some pain, so we've started giving him regular small doses of oxycodone to help with the pain. He had x-rays last week and everything was okay. He's got a sedated MRI scheduled Tuesday and we're working on an appointment with orthopedics. Hopefully between the two we can figure out what's going on.

In other news, Ethan's been having a real hard time sleeping. Ethan's had

chronic sleep issues since he was a few months old. They've been worse since we got here in January, and worse still since he was discharged. We've started him on melatonin which many MPS kids and post transplant kids use. That is helping some, but it's not a panacea. His sleep issues are complicated by the possible pain issues from his leg or continued irritation/pain in his mouth, stomach and GI tract and nausea from the Meds. In addition, I think the neurological changes from the Hurler's affect his sleep. We're meeting neurology on Monday to explore that last possibility.

In Caleb news, he's been really happy to have us home. He's been hanging out with us a lot rather than going down and playing with his friends. We've all enjoyed spending time together again.

On Friday, all the kids in the charter school here got a special tour of the Minneapolis/St. Paul airport. (The charter school just received a naming gift from Southwest Airlines. (Thanks, Southwest!!)) So, the kids got to go behind the scenes. We sent Caleb with our camera and he took more than 80 pictures. Almost all of them are pictures of the airport fire station. The kids also went up into the control tower, but Caleb says that he forgot to take pictures up there because he got distracted by looking through the binoculars.

If you want to see the pictures of the tires and wheels on the airport fire trucks, just let me know and I'll email some to you. For the rest of you, here is a picture of Caleb driving the fire truck.

So, day by day we move forward in small steps. Adjusting meds and timing to try to reduce the vomiting and interest Ethan in food, working to get him to sleep through the night, pondering what new issue might lie behind his limp...we live MPS every day. We know that those of you who travel this road with us also think about MPS often. Tomorrow is National MPS Awareness Day...perhaps as we go through our day tomorrow, we can bring a little knowledge of MPS to each person we meet. The more people know, the more support we can get for research to find treatments and maybe someday cures for these diseases.

Saturday, May 7, 2011

T+24: Discharged!

Brushing teeth at home.
(And that's a Nerf gun in Caleb's hand.)
Wow!  Ethan was discharged from the hospital Thursday evening and my head is still spinning.  Yeah, I know, it's been a couple of days.  Sorry for the late update, the boys are keeping us hopping.

I am completely surprised and amazed that we got discharged at T+22. I never expected it. He was discharged at T+28 the first time and I expected this to be much worse. I thought he would end up in ICU at some point and was very worried about GVHD.  In fact this transplant was easier than the first, so far..

A few things that have made the difference.  Even though Ethan was on a reduced toxicity regime for both transplants, I think that this regime was easier on him then the last.  We didn't see nearly as much tissue breakdown or GI tract breakdown as we did with the first.

In addition, this donor unit was larger than the first one. There were more donor cells to get in there and start multiplying. I think that he engrafted faster and started healing sooner as a result.  They drew blood for engraftment levels on Thursday.  Hopefully we'll have some early numbers by Wednesday.

Finally, I can't tell you how relieved I was to hear that GVHD wasn't a real concern with this protocol.  This cord was a 5/6 HLA match.Ethan's first was a 6/6.  I definitely wondered if we'd already wasted our best shot with the first transplant.  And I was afraid that we were due for some significant complications.  Ethan developed acute GVHD during his first, but it hasn't been a problem at all this time.

Ethan is really, really happy to be home.  He's so happy to be home that he will tolerate putting his mask on to go out of the room because he wants to go play around the house.   His energy and mood are really good.  Friday afternoon, he and Caleb went down to the gym and ran around for 20 minutes.  I couldn't believe it.

Medically, Ethan will have clinic check-ins every day or every other day for a while yet.  Some of these will be quick visits where they do a blood draw and to check his blood chemistry and then we go home.  Some will be all day affairs where they'll need to give him blood or platelets or immune globulin. Ethan's immune system is also still very, very weak.  At some point in the next 2 and a half months, it's likely that he'll develop an infection and have to go back inpatient for treatment.  For his first transplant, Ethan did not have to go back in during his first 100 days.  But as they say, past performance is no guarantee of future returns. 

Grandma giving some oral meds.
This is a frequent scene. 
For Sarah, Grandma and I, this is one of our busiest periods.  We've all become home care nurses.  Each day, Ethan receives a total of 23 doses of 8 oral meds. Simply drawing and administering his morning and evening oral meds can take an hour and a half, because if we give him the meds too quickly it will inevitably upset his stomach and he'll trow up--in which case we need to redo any meds he's taken in the last 15 minutes. 

In addition to his oral meds, he receives two night time IVs, a one hour caspofungen (an anti-fungal) and a 12 hour TPN nutrition IV.  Each evening, in addition to drawing his meds, we need to prep his TPN.  Getting the TPN ready consists of injecting 3 different vitamins into the nutrition bag and adding insulin because of his hyperglycemia.  Then we need to do a finger stick and check his blood glucose level both before we start the TPN and four hours into it.  Tomorrow and Sunday we'll add IV doses of GCSF a stimulating factor for white blood cells.

This is the busiest health care period for us.  As his condition stabilizes further, he'll come off of various IV and oral meds.and things will settle into a more manageable routine. 

Friday evening, Caleb and Sarah went to the Mall of
America with Caleb's friend,  Wes, and some
other kids from the house.  This is Caleb driving a semi, one
of the rides, there.
Caleb is certainly happy that we're all back together, too. Caleb's been a champ throughout this.  He asks a few questions about why Ethan does this or that, but I've never heard him complain about Ethan or about how much of our time Ethan takes.  Nonetheless, I think he needs some focused one-on-one parent and kid time. I just think he needs to know that he can be the center of attention, too. Hopefully, in 3-4 weeks, our routine will have settled down some so we can really start to give Caleb more quality time. 

Reflecting on the inpatient period, I will say that I'm very impressed by the BMT program here.  I know I spend a lot of time griping about doctors and nurses. I certainly stand by most of those gripes.  But, looking at the big picture, the team here does a very good job.

Most notably, perhaps, the nurses are very well trained.  It strikes me as a young nursing staff for a BMT ward.  I suspect that's due to the very high number of transplants that they do, it's a very busy place and I suspect there is a higher than average burn out rate.  But even outside of whatever classroom training about transplant care and protocols, nurses here do at least 3-4 months of shadowing experienced nurses.  So they get quite a lot of exposure to different clinical situations.  All of the training shows up in the consistency of care.  Sure, there are some nurses that we like more than others, and there are some who are less competent than average. Overall, though, UMinn's done a great job bringing their nursing staff up to a fairly consistently high level. 

In addition, the child life specialists here are also very good.  In fact, it's difficult to walk into any of the clinic or wards without having that department's child life specialist come and introduce themselves.  We haven't really needed to take advantage of their expertise because Ethan is still too young to grasp explanations of hickman lines and chemotherapy.  (The staff have offered to work with Caleb, but he has said that he doesn't have an interest in hearing more details about Ethan's treatment.). Nonetheless, they check in very frequently and were able to provide some additional toys and ideas for stimulation while Ethan was inpatient. And they've been good sources of ideas for things to do with Caleb.

The social worker we've got is nothing but impressive.  During Ethan's first transplant and follow up, we only saw Ethan's social worker about 3 times, and our conversations with her were brief and generally uninformative.  The social worker that we work with here we see about every other day.  She's been great in helping us navigate things here and in pointing us towards resources   And she's been a very concerned and effective resource in helping us establish better communication with that first doc when we went in and in ensuring that we're comfortable in our interactions with the medical staff.

The doctors and overall BMT expertise here is quite impressive as well. They do so many bone marrow transplants here that they are able to model and study outcomes for any number of different transplant protocols.They've even done studies on the number of days between line dressing changes and the materials to use.  Their expertise in BMT just reinforces my opinion that regional children's hospitals shouldn't try to do so many of bone marrow transplants outside of the established and more common hematology/oncology disorders. It's my impression that the regional centers just don't see enough patients in less common disorders to develop or validate effective protocols and that as a result their success rates will always be lower and their mortality rates are likely to always be higher.  That's nothing against the doctors and staff at regional hospitals at all.  It's just a question of having the numbers and resources to meaningfully study and improve outcomes and success rates.

Well, I'm going to wrap this up, here.  If you've made it this far through my ramblings, you get to enjoy the video below of Ethan and his physical therapists working (playing) while he was inpatient.   The therapists brought Nerf guns for Ethan to play with.  I don't think they knew that Caleb and Ethan have Nerf battles all of the time and that we have quite an impressive armory at the House.  As you can see, we all had a blast.

Monday, May 2, 2011

T+19: New Digs

As I was writing this, Ethan decided that he wanted to go for a walk on the ward.  And the big news is that he wanted to do it enough that he tolerated the mask he has to wear.  Here's Ethan and Grandma Lilianne, both with big smiles on their faces.

We walked around the ward for about five minutes, and then he got carried around the ward for another 10 minutes.  He had a great time, but it may be a few days before he's ready for a game of hide and seek on the ward.

We now return you to our regularly scheduled blog update.

The last few days have alternated between busy and hectic.  But Ethan's condition has been stable, he's continuing to heal, and overall he's doing very well.

On Saturday, we moved to a new hospital building.  The old building served both adults and kids.  The new building is a dedicated children's facility just across the river. The hosital staff had been planning this move for 18 months.  They had really thought things through and the move went really smoothly.

Sarah and I were most concerned that Ethan would rebel against being strapped to a guerney while wearing a mask (to him catching an airborne infection) for the 30-45 minutes it would take to transport him.  Fortunately, we'd learned our lessons from transporting him up and downstairs for the CT scans and when it came time to put him on the gurney, we just swaddled him up really tightly and he stayed pretty calm throughout the move.

The new hospital is amazing--or at least the patient rooms are amazing, since that's mostly what we've seen so far.  The room is easily twice as large as the one that we've been in over the last month, and it's state of the art.

The first third of the room is primarily for medical staff.  The supply cart and linens cupboard can be refilled from the hallway without the staff having to come into the room.

The middle third of the room is Ethan's space.  He  has four TVs he can see from his bed:  a big screen 55 inch with video conferencing capability, two smaller 30 inch screens next to that, and another flat screen on a swivel arm that he can pull into bed.   Each of the screens can run TV, in house movies, internet (w/ wireless keyboard), wii video games, DVDs, and an iPod dock.  He can control a video monitor outside of his door that says if he's asleep or awake or in a good mood, or on a restricted diet, and he can e-mail his doctors or nurses with questions.  Or at least he could if he could spell and type.  (Though he does make a good try of it!)  The LCD monitor on the swing arm is also a touch screen controler for all of the other gadgets.

Sunrise over Ethan's room.
On the ceiling, just next to Ethan's bed, is a row of computerized lights.  You can use the touchscreen to set them to different color patterns like a yellow and red sunrise, a purple sunset, a rainbow, green northern lights, etc.  It's really over the top.

The white board in our old room has been replaced by a floor to ceiling 3 foot dry erase board that we can write on or he can write on to keep track of meds and schedules.  We've reserved the bottom third of the board is reserved for Ethan to write on.

The other side of Ethan's room is thought of as parent and family space.  There is a computer worstation, a table and chairs, convertible sofa and a hospital recliner (as opposed to a home recliner, the former being about 1/2 as comfortable).

The whole thing is pretty stunning and it's quite a change from Ethan's last room.

Medically, Ethan is doing very well. We're at the point in all of this where we're trying to convert most or all of Ethan's IV meds to oral meds so that we can go outpatient.  UMinn tends to keep patients on IV nutrition when they first go outpatient, so Ethan's almost certainly going to be hooked up 16-24 hours a day for a while when we get back to the Ronald McDonald House.

Ethan's got 20+ doses per day of 8-9 medications.  His stomach is still very shakey, some of the meds cause additional nausea and some of them just taste yucky.  Morning and Evenings are the big times for meds.  We usually try to give him a couple of milileters, then wait a couple of minites before giving him more. Doing it this way means that it can take an hour or an hour and a half to do them all.  But spacing them out helps his tummy recover in-between and means that if he throws up, most of the previous meds have had time to absorb and we don't have to redo as many of them.

The only abnormality that we're still trying to get a handle on is Ethan's glucose levels.  His levels have been all over the place.  Sometimes he's normal without insulin, and sometimes he spikes to 200 or more and they have to start the insulin back up.  The endocrinologist, Dr. Moran, says that the infection he had likely stressed his pituitary gland, impacting his insulin production.  She says it's almost certain to recover in the next couple of weeks or so.  It may be that Ethan will be on insulin when he gets released, which is okay.  But right now, his levels are fluctuating too much for us to monitor and adjust to at home.  So we need to get a handle on that.

All in all we're doing great.  Ethan is getting stronger day by day, and now we are certainly comfortable in these new digs.   Some pics from the last week or so follow, below.

Thanks, Jen!!


Doing a search on the internet. I think he was researching
universities in Malaysia. Maybe he's thinking of
going to college there.

In the ambulance, moving to the
new hospital.

Rolling out of his old room.
The new room is huge!

This may be a little excessive.

Part of the parent/family space.

Caleb bowling with our wonderful Care Partner volunteer,
Sue, back at Ronald McDonald House during the hospital