Thursday, January 27, 2011

Lung Biopsy-Follow Up

Quick update from Sarah:

Todd called this morning with an update on Ethan.  They had a fairly restful night (morphine will do that for you :-)).  They did a chest X-ray this morning and then the surgeon (Dr. Saltzman) removed the chest tube because things looked good.  He's got a dressing that needs to stay on for 2 to 5 days, but they may be able to come back to the House tonight or tomorrow depending on how Ethan's feeling. Yeah!

Wednesday, January 26, 2011

Lung Biopsy

Ethan is in recovery and doing well.

Purple pajamas in prep for biopsy

Todd reports that the surgeon was very happy with how the biopsy went, which we hope means he was able to do the simpler surgery and that he got a good sample of the gunk in the lung.  The anesthesiologist reports no problem visualizing the airway (often a problem in kids with Hurlers), and Ethan's O2 saturation levels in recovery are at 100%..perfect!  He was in some pain, so they've given him light pain meds and he's knocked out.  The plan is that he will be in-patient for a couple days on the BMT ward.  Then we'll be back at the House probably on an anti-fungal medication.

Thanks to all of you who were thinking of us today!

(By the way...the "boo-boo" on his head above his right eye...fell backwards down some stairs...nice to know he's still very much a 2-year-old!)

Wednesday, January 19, 2011

In which Todd quits his job

 I turned in my resignation today at work.

This is a big change for me and for us.  Our plan on coming out here was that I would borrow 2 months worth of leave through the beginning of March then go to half time telecommuting from here.  And that Sarah would take leave after mine.  And that we'd juggle it all somehow.

There are a number of factors that took us from there to here, many of which combined about the same time.  The big push was the 4-6 week delay in starting Ethan's transplant protocol.  Sarah and I have used up a lot of our leave over the last 15 months between the transplant itself and then subsequent doctors' visits and hospitalizations.  When the doctor called me to tell me about the granulomas and the delay, literally my first thought was what does this do to our leave schedules and our ability to juggle our jobs.  That was a heads up that my priorities might be out of whack.  

I also knew that this was the first of any number of potential complications and delays and that if we were watching our leave clocks and both trying to juggle our jobs, we'd go crazy. 

Another factor that played a part was seeing how Sarah's telecommuting is going and trying to imagine the two of us telecommuting on various schedules while juggling two kids, one of whom is going to be very sick even when he comes back to the house as an outpatient.  I just didn't see how either of us would be able to do our job halfway competently under those circumstances and it seemed to me that we'd both end up frustrated.

Add to that the expectation that the doctors here will be much more stringent about when (and if) Ethan would be cleared to return to daycare; together with the realization that at least two of Ethan's infections and hospitalizations this summer and fall were the result of viruses that he almost certainly picked up at daycare.  Which would mean that Sarah and I would be trying to juggle telecommuting full time for at least a year when we get back to Portland.

It just seemed like we were asking a whole bunch of people to juggle us and make allowances for us, and even with that we wouldn't be doing our jobs or taking care of Ethan as well as we should.

The reality is that there are a few families who make it through one transplant without having one of the parents quit their job.  But there aren't many.  I don't know of any who have made it through two transplants with both parents still employed.  

And in all honesty, I'm excited to be able to focus on Ethan's care and to work just for him.  Over the last year and a half, it's become increasingly difficult for me to switch back and forth from Ethan to work.  As sad as I am to leave the university and my good friends and colleagues there, I'm excited to be able to focus on Ethan and on caring for him. In the three weeks that we've been here, Sarah and I have both noticed new mannerisms and skills from him, and at some level we've been scratching our heads and wondering if he knew these things before, but we just didn't see them because we weren't with him as much?

So since it's been more like having two jobs, maybe it's not so much leaving my job as leaving a second job to really focus on the first.

In other news, everyone here is doing really well. Sarah's work is going well.  Caleb seems to be getting more homesick, which is certainly understandable.  But he's coping with it well, and Sarah and I are seeing him become increasingly independent and take on more responsibility.

It sounds like Ethan's lung biopsy will be scheduled for some point early next week.  The surgeon says it will be 1-2 days inpatient in recovery if they are able to use the scope or 3-4 days inpatient if they have to go in between the ribs with their hands.  After that he'll be on an antibacterial or antifungal--whatever is needed--on an outpatient basis.  Sarah and I were very happy to learn that the insurance company has approved the procedures and care to be done here, and are not requiring us to come home to Oregon for a month because these are not, strictly speaking, transplant related.

Wednesday, January 12, 2011

Workup Results, the First Issue, and the Hurler's Gang

At the Minnesota Science Museum
Sarah, Ethan and I met with Dr. Lund this morning for what's called the pre-transplant "exit interview."  This is where they walk through the results from all of the pre-transplant evaluations and chart the course of the admit, transplant preperatory regime, etc..

Basically, everything looks good, except for the one thing that doesn't look good.  And we're going to have to deal with that thing before we can move to transplant.

The CT scan showed that Ethan has two nodules in his lungs.  The nodules are "presumably infectious, possibly [granuloma]."  A granuloma forms when the body is unable to fight an infection, typically bacterial or a fungal.  When the body is unable to fight the infection, it forms a protective wall around the infection and just leaves it there.  The trouble is, you can't tell from the scan if the infectious agent is still active or not.

During a bone marrow transplant, when the body's immune system is wiped out, granulomas with active infectious agents are, so Dr. Lund tells us, 100% fatal.  


Chew on that number for a while.  I know we did.

So, we've got to deal with it before transplant by finding out what the infection is and then killing it.  To find out what it is, they are going to perform chest surgery.  The surgeon will go into the lung with a scope--and I assume something to collect a sample.  They'll take a sample and culture it, which means put it in various growth media to see which ones it responds to and thus what it is.  Once they know what it is they'll put Ethan on a course of medication to wipe it out. 

In typical doctor fashion, Dr. Lund was sure that they could kill the infection, whatever it is.  It's just going to take time (could be four weeks or so if it's a fungus--they take a long time to culture), and some range of drugs.

After the surgery, Ethan will have a chest tube placed and will be inpatient for 2-3 days. And then he'll be outpatient on some course of drugs for a number of weeks, because they'll take a guess at what the granuloma is hiding and treat him for that. If we're lucky, once they figure out what it is, they will have guessed right and it will be dead and gone. 

To me, this just goes to show that there is no such thing as an uncomplicated bone marrow transplant.  BMT's are utterly complex and completely unnatural procedures where the body's natural protective response is hammered into submission through drugs and radiation so new DNA can be secured in place with a hot glue gun.  Each step brings its own range of potential complications and side effects.  Thereupon additional processes and medications are utilized to lessen those complications and side effects. And so on, and so on.  This will undoubtedly be the first of a number of complications.  Some may be major; hopefully most will be minor.

There are two pieces of good news.

To repeat myself, the first piece of good news is that Dr. Lund is sure, in typical doctor fashion, that they can kill whatever it is that's causing the granuloma.  

The second piece of good news is that Ethan is otherwise in very good shape.  Aside from the adrenal insufficiency, which seems manageable, all of the other departments said that everything looked fine.  His heart's in good shape, his vision and hearing are in reasonably good shape for a Hurler's kid, his neuropsych results were at least as good as they were over the summer (though it's hard to be precise, because he was uncooperative for some of the testing), his fine motor skills are on par with a normal 29 month old.  

Just as important as all of the medical opinions though, may be the fact that in our opinion, he's strong enough to get through this.  If the science works, Ethan's strong enough to come through the other side.
Big brother, little brother

In non-medical news.  Ethan is behaving more and more like a typical two year old.   He knows what he wants and what he wants to do, he doesn't mind throwing fits--though I won't say they rise to tantrum level--when he doesn't get his way and one of his favorite words is "mine".  But even with the terrible twos, he's still a happy, fun and smiling kid.  He loves to interact with folks, kids and adults alike, he likes to play games, he's got a great sense of humor,  And slowly, but surely, he's learning new words and communicating just a little bit better.

In house news, there are now three other Hurler's kids in residence, not including Ethan, and we met another one in clinic today.  You can't spit without hitting a kid with a rare disease.  One kid is post transplant and they've been here basically for a year dealing with complications.  Hopefully, they'll be able to go home soon!  The other two kids are 13 and 17 months old and are here for transplant.

I think we need to get leather jackets for the kids with a logo on the back!  Now what would a biker gang of Hurler's kids be called...

Saturday, January 8, 2011

Pre-Transplant Workup

The mask.
It's been a busy week for everyone here. Ethan and I have been going from appointment to appointment from 8 or 9am till 4 or 5pm every day, Caleb's started school, now going to full-day kindergarten as opposed to the half-days he's used to, and Sarah's been working and juggling the rest of us in and out of her day and workspace. Added to which, she's been getting over a cold.

Evenings have been full of a tired and fussy two year old, a six year old who wants to do whatever activity is going on in the house, and two tired parents.

Before we left Oregon, I saw the schedule for Ethan's workup, and thought that it was a ridiculous schedule for a two year old. But I figured, they work with a lot of young Hurler's kids they must have factored in his age. I was right, it was a ridiculous schedule. Each day has had 4-6 appointments in a row, each running from an hour to three hours, leaving us an hour or so for lunch, and no time at all for Ethan's nap.

Tuesday morning's schedule started with three hours of testing in neuropsychology. By the end of the first hour, it was clear that Ethan wasn't focusing. I couldn't blame him, he hadn't had a nap the day before and didn't make up for the sleep overnight.  He was just tired.  From that point, I started asking that they take the appointments for occupational therapy and physical therapy off of this week and move them onto next week so that Ethan would have more rest this week and we wouldn't be wasting everyone's time asking him to work through things that he was just too tired for.  That also opened up some windows in the schedule for Ethan to get more naps.

Other schedule snafu's include scheduling Ethan for a head and chest C/T scan and expecting a two year old to hold his head completely still for one minute while they shove him through this big machine. Needless to say, we didn't get those shots, and we've rescheduled them with sedation next week.

Aside from the mad schedule and the resultingly mad kiddo, it's been a pretty good week for Ethan. So far he's been evaluated by cardiology, dermatology, pulmonology, endocrinology, audiology, neuropsychology, otolaryngology, ophthalmology and radiology. He's had electrocardiograms, echocardiograms, chest x-rays, hearing and vision tests. He's had a skin biopsy to determine if his ongoing rash is eczema or GVHD (turns out it's eczema, at least at this point).  And, he's had his central line evaluated, and we've met with other doctors, coordinators and social workers.

Still to come, Monday 5:40 a.m. check in at the OR for a sedated CT scan, ear check (EUA), eye check (ERG), and lumbar puncture. Also next week, he's got completion of the neuropsych evaluation, occupational therapy and physical therapy appointments that were moved from this week, as well as a neurology consult.

On Thursday, I wasn't feeling well--I think something I ate was coming back to haunt me.  So Sarah took over the afternoon appointments.  Today, Ethan's having his enzyme replacement therapy, so it's his quietest day all week.

All of this is intended to ensure that Ethan is okay to undergo transplant, to identify any issues that may arise during transplant, and to provide baseline info for p/t and o/t therapy. There is some concern about the RSV he had in December; pulmonology wants to review the CT scan to ensure there are no lingering signs of infection and endocrinology wants to ensure that his adrenal system hasn't been impacted. But, so far his tests and evaluations look very good and nobody has raised any significant concerns.

I will say that this workup is far, far more extensive than what he received in Portland, and each of the doctors who has seen him has extensive experience evaluating Hurler's patients within their specialty, which is a nice change.

We did learn that they've changed their mind about his transplant regime. When we talked with Dr. Orchard by phone in November, he said he thought that they would decide to go with a fully ablative, full-toxicity regime, as they would with a first transplant because it had been a year since Ethan's first transplant. On Monday we learned that they've decided to go with a partially ablative or reduced toxicity protocol. (A fully ablative regime aims to clear out all of the marrow from the bone, and a partially ablative regime just seeks to clear out enough of the existing marrow for the donor cells to find space and begin to engraft.

Assuming that everything continues to go well, they anticipate admitting him and starting chemotherapy on Friday, 1/14, which will be day T-21. He'll continue chemotherapy through T-2. On T-1, he'll receive low dose total body irradiation, and his donor cells (from a donated and stored umbilical cord unit) will be administered on T+0.

If there are no significant complications, Ethan could be released from the hospital at about T+35. Or if there are complications it could be a week, a month, or many months after that.  We'll be here in MN until at least T+100 as that seems to be the magic date for transplant patients to be released back to their home hospitals for follow-up care.

The above was written Friday afternoon, while Ethan was napping during his infusion.  It's, now about 6:40 on Saturday morning.  Ethan woke up about 2:30.  Sarah worked with him until 4:30 when I woke up and relieved her.  I worked with him until 5:30 before dosing him with Benadryl to help him sleep.  Lemme see how much more of this I can get done before Ethan wakes up and wants to use his computer.

Yesterday evening, we got a call from the endocrinologist.  Ethan's been diagnosed with adrenal insufficiency, which is likely a reaction to the steroids he was on for his anemia.  He's got a new med prescription for a type of cortisone. It sounds like they can manage it with medication, and it will prove to be a temporary condition.

The vest.
On other Ethan News, the battle with Ethan's central line dressing has been continuing. Late last week, his skin was healing nicely, and we thought we might be able to do away with the gauze gladiator vest and go back to the normal dressing.  But that didn't last 12 hours. So we put him back into the vest.  The vest is kind a pain to put on and with so many people looking at various parts of Ethan, we had to cut it off and then put a new one on a couple of times in clinic in addition to daily dressing changes at home.

On Wednesday, Ethan had his central line checked out to see if the November '09 line was still in good shape for this transplant. The nurse who looked at his line also took ownership of the dressing issue and has been helping us work through different causes and options there.  We think that Ethan might have developed a sensitivity to the adhesive in the Tegaderm dressing.  Evidently, sensitivity or a developed allergy to these dressings is not uncommon.  Torie, one of the other Hurler's kids in the house has developed a strong allergic reaction.

As a first step, to get ahead of this, we changed the kind of dressing. So instead of the biopatch, tegaderm combination like this, we've covered the insertion site with a small primapore dressing and then covered the site and his tubes with a catheter wrap.

We also came to think that the scratching had become a behavioral issue as well.  Ethan's been doing most of the scratching at night.  Even though Sarah and I have been taking turns sleeping with him to keep him from scratching at it, it hasn't been enough. As Sarah and I were looking for answers and options on this, we called my mother and asked her if she would make a sleeveless vest that would be thick enough to dull the scratching but soft enough that it wouldn't irritate his skin. She hopped right on that and put something together on Thursday for us. Surprisingly, Sharon, the nurse we've been working with on this, likes to sew and also whipped something up.  She took a 3T t-shirt, added some quilting underneath the front of the shirt and added a velcro closure on the back so we could make it more snug.

Fortunately, the new dressing seems to be working better, so far.  He's not picking at it nearly as much.  The downside is that the primapore needs to be changed every other day, whereas the biopatch/tegaderm combination only needs to be changed weekly. Eventually, we'll need to go back to a dressing that we can leave in place for a full week.  There are some hypoallergenic options out there that we'll explore over the next couple of weeks.

Caleb and Sarah have had a busy week as well.

Caleb started kindergarten at the Ronald McDonald House charter school on Tuesday.   Unfortunately, the only other kindergarten student left just before Christmas break, so there's no one Caleb's exact age in the school. I worry about Caleb not having any peers around and how in his natural emulation of the older kids in school he may grow up more quickly than he should or is ready for.  Caleb is a good kid, and I think he'll do okay.  But it's just another way that his childhood isn't normal, and it tugs at me.  Regardless of all of that, Caleb seems to be settling in well.  His teachers say that he seems comfortable in school and he is making new friends.  His teachers have also been wonderful to let him bring things to share with the class each day (train posters, science kit, and, of course, his toy hovercraft---they ran it in the gymnasium during recess.)

Dodgeball in the bouncy house. 
Many of the evenings have been occupied by RMH events such as the WII gaming night they did on Tuesday, the bouncy house they set up in the gym on Wednesday or the weekly bingo night on Thursday, at which Caleb has won two or three times each week, resulting in new toys we try to find room for. We are getting better about pushing the kids back to the room earlier so that everyone can unwind and get ready for bed.  But it can be a challenge.

Sarah's had a busy and tough week too.  She's been fighting a cold since last weekend.  Having a cold around here isn't a good thing, since a lot of the kids in the house are weak or immune compromised, and you don't want to get them or their parents sick.  So, Sarah's been wearing surgical masks all week, even when she's asleep.  She takes it off when she's alone during the day, telecommuting from our room.  At dinner times, she's been getting some food and going over into the corner where she can remove her mask and eat without breathing on anyone. Fortunately, she's finally feeling better and we were all happy to see her face again yesterday.

Since it looks like Ethan will be admitted on Friday, this will be our last normal weekend for quite some time.  Later this afternoon, after Ethan's mid-day nap, I think that we're going to go to the Mall of America.  (Just shoot me now.)  The rides and attractions for kids should be fun.  But I'm really hoping we don't actually try to browse and shop.  (Argh.)  I'm not sure what we're going to do tomorrow.  But I expect that we'll try to get out and do something fun.

A huge thank you to everyone who has sent posters, cards, toys for the kids, and care packages.  I'm sorry that we haven't had time to thank everyone directly.  We really appreciate it, and so do the kids.

For those of you who were wondering about RMH, I've posted some pictures on FB.

Thanks for checking in.  I'll try to post a brief update Monday or Tuesday after Ethan's sedation and checks

Saturday, January 1, 2011

Happy New Year!

-          Ronald McDonald House, Twin Cities

The best way I can think of to start this new year’s post is to say thank you to everyone who has helped us a little or a lot over the last year and a half.  There are literally too many of you to thank individually, and I’m afraid we didn’t quite get to Christmas cards this year.  But your help and support at various times and in different ways has helped us to smile or relieved us of a worry or allowed us to focus on something else that needed doing.  To all of you who have helped, in big ways and little ways, thank you.

Caleb, at least, had a very good Christmas.  His six year old mind was filled with the expectation and excitement that is all Christmas should be for children.  As Sarah and I started thinking and focusing more and more about the trip the day after Christmas, it was Caleb who kept me centered and reminded me why Christmas was important, too.  Caleb had a great time handing out and opening gifts.  Ethan, on the other hand, wasn’t too impressed.  This was Ethan’s third Christmas, but he wasn’t interested in opening gifts.  Sarah’s mother, Pat, came by and we had Christmas brunch.  Unfortunately, Sarah’s father wasn’t feeling well, so he wasn’t able to come by. Nonetheless, it was a fun morning, filled with happiness, smiles and warmth, just as Christmas should be.

By two that afternoon, Sarah and I were packing the mini-van, and we got on the road a little after 9 the next morning.

The 2,000 mile drive was uneventful.  We ran into about an eighth of an inch of snow in the Blue Mountains in eastern Oregon, and some fog in Idaho and Iowa.  But most of the time, including our two days in the Rocky Mountains, were clear and sunny.  The weather was so nice that now I’m wondering if I can get a refund on these snow tires and chains!

We drove eight or nine hours each day.  By the end of each day, Caleb and Ethan were restless and tired, but they did great.  For me, the highlight of the trip was spending some uninterrupted time with Caleb.  He is such a bright and charming boy; I really enjoyed his comments and questions about things we saw and listening to him continue to process this extended trip and what it means.

Ethan had a good trip too, though for him that primarily means that he didn’t have any significant medical issues during the trip.  Up to two days before departure, we weren’t sure if Ethan’s health was going to be good enough to make the drive, and we were considering the possibility that he might have to fly.  The Wednesday night and Thursday night before we left, Ethan ran small fevers of 99.5 or so.  Both nights, the fevers went away without Tylenol or Ibuprofen, but we figured if they came back Friday or Saturday night, that there was a risk that he’d end up getting hospitalized for some length of time somewhere in Utah or Wyoming or something.   Fortunately, the fevers didn’t come back.

Ethan was also on antibiotics at noon, 8p and 4a, during the first few days of the trip.  That didn’t turn out to be a big deal, though it was an interesting experience unhooking an IV line while standing under an overpass an a small Oregon side road during a light snow, or in a 7-11 parking lot in Salt Lake.  We weren’t sorry to end the antibiotics on Tuesday, though mostly because it meant the end of the 4a-5a hookups and a solid night’s sleep for all of us.

Medically, the main issue that we’ve been dealing with is that Ethan keeps scratching off the dressing that covers his central line.  Normally, his dressing consists of a small round patch (biopatch) that covers the insertion point, and a thin, clear plastic stick on dressing (Tegaderm) over that.  When Ethan was inpatient a couple of weeks ago, his skin got really dry and irritated, and he started scratching his dressing off.  The tegaderm is adhesive, and between Ethan’s scratching and the peeling off of the adhesive, his skin under the dressing became even more irritated.

His skin was so inflamed that the Wednesday before we left, we took him in to have him looked at, and everyone agreed that we needed to stop using the tegaderm for a while to give his skin a chance to breathe and heal.  But we still need to protect and cover his insertion point both to keep it clean and to make it harder for Ethan to scratch.   As a result, Ethan is wrapped in two rolls of gauze in a gladiator vest style. It does keep him from picking at it, but it’s a chore to do and we have to change it every day or two.   We tried to go back to his normal dressing last night, but he scratched it off again overnight, so it looks like we’ve got at least another week or two of the gladiator.

So, anyway, back to the trip.  We arrived in Minneapolis just after lunch on Thursday.  We got really lucky, not only did the Ronald McDonald House have room for us, but one of their long-term apartments had just opened, so we could go right in there and wouldn’t have to start out in a smaller, hotel like room and change later.

The room is decent, a one bedroom apartment probably about 500 sq feet.   The bedroom’s got two queen sized beds AND a set of bunkbeds (so Caleb felt right at home), as well as two good sized closets and a desk.  The living area has a small living room/dining table/kitchenette, and there’s a bathroom off of the entryway.  We’ve gotten most of our stuff unpacked and even got most of the posters up that folks have sent us..thank you again!  We’ve also got Sarah’s computer set up…thank you NWEA for helping make that so easy.  The internet connection leaves much to be desired, but we’ll figure that out next.  We’ll have to figure something out for a second work station when Todd gets back to work as well, but we’ve got a few weeks before we need to have that worked out.

So, we’re catching up on laundry, and getting to know our way around the House again.  Caleb loves the game room and the playroom where there is a place to do a good train layout, and the other playroom where there is a play grocery section and some toy castles.  Ethan also likes the playroom where there is a toy kitchen he can fix us imaginary soup.  When we were here last summer, we weren’t really here long enough to get into the routines around here, so now we’re learning about Bingo night and where the SuperTarget is, etc.

We’ve also met two other Hurler’s kids and their families who are here at the house at the moment.  Believe it or not, we’ve met kids with other MPS/ML types, but this is the first time we’ve ever met other Hurler’s kids.

At the moment, since Ethan hasn’t started his appointments, I feel a little like an interloper here at the house—using the facilities and services, but not really deserving it.  I know that will change quickly enough once Ethan starts chemotherapy and during his outpatient period.  It’s at that point that the house will be an irreplaceable resource, allowing us to concentrate our energies on caring for Ethan and helping him get better.

On Monday, Ethan starts his pre-transplant work up.  Here’s a list of his appointments.

Labs and Calendar Review, Chest X-Ray, EKG, History, Echocardiogram, Cardiology Consult
Neuropsychology, BMT Coordinator Mtg., Audiology, Ear, Nose and Throat Consult
Radiation Therapy Consult, Simulation, Occupational Therapy Eval, Ophthalmology Consult
Social Worker Mtg, Pulmonary Consult, Endocrine Consult
Monday 1/10
Physical Therapy Eval
Thursday 1/13
Neurology Consult

I think he’s going to start chemo the week of 1/10 on an outpatient basis, and then will probably be admitted a week or so later depending on how his body reacts to the chemo.

I've posted some pictures of the trip on FB:

Thanks for checking in on us.
Happy new year!