Friday, November 20, 2009

The week’s motto: Any day without a major catastrophe is another day of progress and healing.

It’s been a very tough week, with a lot of worry, but we’ve avoided the major catastrophes and Ethan is continuing to heal. 

The week started with a good deal of concern about Ethan’s high blood pressure. Ethan had already been on a couple of stage one blood pressure medications, but they weren’t doing the trick.  Ethan’s normal systolic pressure is in the one-teens. His pressure has been climbing over the last couple of weeks as a result of the anti-graft versus host disease (GVHD) drugs that he’s on.  By the beginning of the week his systolic was peaking in the high 150s and he was averaging 140 or so. 
High blood pressure can damage the kidneys.  Late Monday, the kidney specialists recommended adding a stage 2 blood pressure medication called labetalol.  But labetalol isn’t recommended for people with asthma or obstructive respiratory diseases, and no one could tell me if the respiratory issues that Hurlers’ kids have are would qualify as an obstructive respiratory disease.
Tuesday morning, at 530, the nurse came in and started hooking Ethan up to the room’s heart monitor, explaining that Ethan’s potassium counts had come back high and that the night time resident wanted to monitor him in case he went into cardiac arrest.  But the nurse, couldn’t provide any real additional information about Ethan’s condition: how serious it was, what we could do to treat the potassium, quantifying the risk, etc.. 
After all of our requests to the doctors that we be given as much information as possible about Ethan’s condition, concerns, and treatment options, I was pretty ticked that the resident had ordered this without coming in to talk with me about what was going on.  I asked the desk to page the doctor and when she came in proceeded to grill her.  She said that Ethan’s potassium counts were coming back in the high 5s, low 6s and that they start worrying about cardiac arrest with levels above 5.3 or so, even though the real risk is for levels around 7.  She told me that the higher potassium levels, like the high  blood pressure was a result of the anti-GVHD drugs he’s on, and that at this point they just wanted to monitor him.
I explained to her and later to the daytime team, that when they feel a need to change Ethan’s medications, routine, monitoring or order new tests, we expected them to come talk with us before or during those procedures so that Sarah and I understood his condition, his options, and as much as possible about why the doctors were pursuing one option over another.   I also explained that our communication with them was an ongoing source of frustration and that Sarah and I didn’t feel that we were getting the level of detail we needed.  I think they finally got the message this time, and our communication with them over the next few days improved a good bit. Sarah and I well understand that we’re not doctors and much of the reasoning behind things may be beyond us, but we also feel strongly that the more we know and understand the more we can work with the doctors to anticipate and respond to problems.
The other problem with the heart monitoring is that the monitor wires are pretty short, so while Ethan was on the monitor, he was pretty much limited to his crib or the room’s recliner.  He could get down and play a bit, but it was pretty challenging with his IV lines going one way and the monitor leads going the other way it got complicated.  The doctors also ordered a full EKG for him, and the tech came by with the machine, attached 12 little sticky pads to his chest, but Ethan was so upset that they couldn’t do the test.  Then we couldn’t peel the lead pads off without hurting him too much, so he looked like he had a bunch of bumper stickers on his chest.
Also on Tuesday, the kidney specialists stopped by and we had a good conversation about Ethan’s blood pressure and his medication options. The doctors had not gotten a definitive answer about whether or not labetalol might be a problem for Ethan, but they did recommend another drug, hydrochlorothiazide.  Hydrochlorothiazide has a side effect of lowering potassium levels in patients.  For most people this can be an unwanted side effect, but in Ethan’s case since his potassium was high, it’s quite a good thing and has helped to bring his potassium levels under control.
After a tense Monday and Tuesday, Tuesday evening was pretty calm, and I figured things were getting under control. Unfortunately, Ethan had other ideas. 
When I woke up Wednesday morning, Ethan was a happy camper and ready to play.  But when I picked him up I realized that something was wrong, he wasn’t hooked up to his IV.  In fact, Ethan had pulled his central line all the way out at some point between 4 and 630.
(A central line is a rubber tube placed that is placed surgically in a patient’s superior vena cava vein, near the right atrium of the heart.  The other end exits the skin and has one or two lines (lumens) which can be used to deliver IV medications or to take blood samples.)
Ethan had just pulled his right out and was sitting on my lap ready for breakfast.  There wasn’t any blood—I think these things are designed so that the vein just closes over the hole when they’re pulled out.  Don’t ask me how that works.  And don’t tell me, I don’t think I want to know.
The doctors came in and looked and confirmed that there didn’t appear to be any internal bleeding.  They confirmed that they did want to do surgery to replace the line-- as opposed to inserting a less robust line in through his arm.  They said they were working to schedule surgery that afternoon and Ethan shouldn’t eat anything so that he didn’t have complications when they anesthetized him later. 
The nurses inserted an IV in his arm so that he could continue to get his meds for the day.  About mid-day, we found out that the surgeon who had done his first placement was in the hospital that day and could do his replacement.  The surgeon showed up later and ran through the risks of the procedure and the message was basically, this is a routine, but high risk procedure.  If everything goes well, then everything will go very well.  If anything goes wrong, Ethan will probably die (I’m paraphrasing).
Unfortunately, they couldn’t get the surgery done Wednesday, because an emergency hernia case came in.  But they did do the surgery Thursday morning and everything went perfectly. Ethan was back in his room by mid day and eating and sleeping pretty well.
Putting the week in context, it’s all been about GVHD.  Even though Ethan’s doing well and the transplant is officially engrafted, GVHD will be a serious risk for at least the next six months.  Ethan showed some early signs of GVHD, a skin rash and some abdominal pain, so the doctors reacted very aggressively with medications and steroids, and those impacted his blood pressure and potassium levels.  Those side effects, while serious,  were still secondary to the risk of GVHD so the doctors stuck with the medications and treated the side effects as well as they could.
Late yesterday afternoon, the doctors started talking about the possibility of sending Ethan home on Monday.
Ethan’s blood counts are solid.  Today’s white blood cell count is 5.5 and his ANC is 3,095.  He’s taking most of his medications orally, he’s eating well, and his early GVHD appears to have responded to medication.
Anything can change at any moment, so he may or may not come home Monday.  Something could change and it could still be next week or next month or January.  But at this point, he looks stable enough to come home.
Once he gets home, he’ll still have a lot of medications to take to head off GVHD and various infections.  He’ll be back to the hospital every day or every other day for blood tests and monitoring.  He received red blood cells and platelet transfusions Wednesday and yesterday, so it’s not inconceivable that he’ll receive additional transfusions during those outpatient visits. He’ll also continue receiving weekly enzyme treatments for at least the next couple of months.   I’d have to say that I’m guessing it’s also probable that he’ll have to go back into the hospital at one point or another if his condition changes in any way. 
He’s by no means out of the woods.  Just Wednesday, I heard from the parent of a Hurlers’ kid from Bend, Oregon.  Their son, Benjamin, received a cord blood transplant at Doernbecher last year.  Benjamin was released from the hospital in September, but readmitted in October with severe GVHD.  Unfortunately, Benjamin didn’t make it.  He passed away from multiple systems failures after five weeks in the hospital.
Hearing from Benjamin’s mother and learning about Benjamin’s story brought the week into clear focus.  As stressful as the blood pressure and heart monitoring and another surgery have been, the doctors are doing exactly the right thing, treating any sign of GVHD aggressively and keeping their options open for future treatments with a new central line.  
I was very thankful to hear from Banjamin’s mother, and I will say again that our family owes a debt to the kids and families that have come before us.  We benefit from their experiences.  Their roads have always been more difficult than ours, and we would never have gotten this far without them.
We’re hoping Ethan will be able to come home next week.  It would be lovely to have the whole family at home for Thanksgiving.  But we’ll take it one day at a time and do what we need to do.