Please note: This post discusses how Sarah and I evaluate medical recommendations for Ethan. In presenting this, we share our laypersons' understanding of the medical issues involved. We are not medical professionals and invariably our presentation here will, at best, be incomplete. We offer this information simply to illustrate how we've approached this problem. Nothing here should be construed as medical advice or applicable to anyone other than Ethan.
|Look, dad! I found a marker. A blue marker!|
I've long said that one of the most difficult parts of caring for Ethan is not knowing what normal is. That's absolutely true on a day to day basis. When he's in the hospital, it's incredibly difficult to gauge his condition and his progress because you've never seen 'this' (whatever this happens to be). The doctors and nurses have typically seen patients at the same stage of 'this'. If you've been reading this blog for a while, you'll know that we spend a lot of time trying to get the doctors and nurses to give us the information, the context that we need in order to better understand Ethan's condition and progress.
This struggle for context continues outside of the hospital, too. Ethan is the only developmentally delayed, non-verbal, physically disabled kid that I've ever cared for and worked with. I don't have any context to understand how he will do in kindergarten next year (whether in a special ed. classroom or mainstreamed in a typical class. More importantly, I have no idea, given where he is now, how much progress is reasonable to expect, or what areas might be particularly challenging for him.
In November, Ethan, Caleb and I traveled to Seattle Children's hospital to have Ethan seen by Dr. Klane White, an orthopedic surgeon there who has experience with MPS and works with many NW families. The primarily purpose of the visit was a six-month check-up on Ethan's spinal kyphosis to ensure that it hadn't gotten worse. This was the first time that Dr. White examined Ethan. Dr. White said that Ethan's spine is stable, but, he said, Ethan will need surgery to place growth plates in his knees to help correct his knock knees, and he will need bi-lateral hip replacement within the next two years. That last recommendation was a big surprise for Sarah and me because the last orthopedic doctor had summed to suggest that he might not need hip surgery.
Bi-lateral hip replacement is a big surgery. It is our understanding that the surgery itself is not technically difficult, but when complications develop, they can be significant and permanent. The recovery period is long and difficult, including 6 weeks in a body cast, followed by a longer period in a brace. Recovery requires a lot of physical therapy, and even then, Dr. White says, most kids limp for about a year as they continue to recover.
|Our Tuesday commuting setup. With the half-bike to pickup Caleb after school.|
For a number of weeks after getting this recommendation, we didn't know what to do with it. At various points, I found ways to rationalize ignoring the earlier recommendation and proceeding with the surgery, then I was equally able to rationalize ignoring the new surgery recommendation and going with the earlier evaluation.
If Dr. White had been any other surgeon, we probably would have just written him off, "Hey, surgeons are surgeons, if they see the littlest thing wrong, they want to operate. But since the first doctor said it wasn't that bad, this new guy must be over reacting." In fact, I said almost exactly that at then end of his exam. The truth was, though, that we had taken Ethan to see Dr. White precisely because Dr. White is supposed to know what he's talking about.
Now we had two contradictory recommendations from two orthopedic surgeons, both of whom have meaningful clinical experience with MPS. Great. Now what?
We started working through this pretty much the same way we've worked through anything with Ethan, by trying to understand what normal is. We started by looking through the available medical literature. The National Institute of Health Pub Med website is a great resource for checking to see if there are any published articles about, well, about anything health related. Some of the articles on PubMed offer free links to the full text. Most just offer an abstract with a link to the article, which is sitting behind some Internet pay wall. A lot of times, I can get the information I need from one of the free articles. Occasionally though, there's a promising abstract but it'll cost $40 to get the article. In those few cases, I've had really good luck sending looking up one of the authors and sending them an email to ask if they will provide a copy of their article for free. I've never had an author decline to send me a copy of their article. I think they realize that it's hard for families to get good information about MPS and they're using our population of kids as research subjects, so they don't fuss when one of us asks for a copy of something.
Most of the articles I found on PubMed were about general orthopedic management of MPS issues; a couple of them were written by Dr. White, himself. The articles suggested that a very high percentage of kids with Hurler system need hip replacement, and they talked a little about the optimal timing for the surgery. But since, Dr. White had written those articles, it wasn't surprising that they agreed with his recommendation. Further, the articles didn't really tell us whether or not Ethan met whatever criteria exists for needing this surgery or if he really needed it this soon.
Once the holidays passed, I faxed a letter to the original ortho guy, Dr. Walker at Gillette Children's in Minneapolis. I included a copy of his most recent report about Ethan and a copy of Dr. White's report. A few days after getting my fax, Dr. Walker called me on the phone so we could walk through things together. That's one of the really wonderful things about MPS doctors, they are almost always willing to take extra time to help patients and families explore and understand their conditions and their options. Perhaps this is generally true of doctors who work with more complex patient populations. It is certainly something that MANY OTHER DOCTORS COULD LEARN FROM.
Dr. Walker and I talked for about 15 minutes. I came away from the conversation with the impression that, while there may be a small chance that Ethan doesn't need the surgery, more realistically, most Hurler kids do end up needing hip reconstruction. So why all the confusion? I would say that a good part of the difference in their reports and recommendations is due to differences in style. I think Dr. Walker's communication style is less direct than Dr. White's.
It also seems that they have different ideas about the timing of any surgery. Dr. Walker said that, in his experience, pediatric hip reconstruction can typically be completed a few years later than Dr. White had suggested without seriously increasing the complexity of the operation or of recovery.
While I was working through the articles and communicating with Dr. Walker, I was also talking with 6-8 different MPS families, trying to learn from their experiences. With MPS, the good news is that you can look at other families and see what's coming down the road. At least for us, we have come to rely on that information to help us think through things. Unfortunately, the bad news about MPS is that you can look at other families and see what's coming down the road. Every kid is different. But, there are a number of similarities across the MPS subtypes, and within the subtypes the typical issues occur with enough frequency that you can learn a lot from the families whose kids are older than yours.
Talking with those other families seems to confirm what we're hearing from the doctors. Where this surgery has gone well, the kids seem to have decent mobility. At least anecdotally, it looks like kids who wait longer tend to experience increasing mobility issues and, in some cases, increasing pain and discomfort. And there are some kids who have experienced significant complications with these surgeries; complications that can result in the child being confined to a wheelchair.
While I may generally prefer Dr. White's direct communication style to Dr. Walker's more indirect style, the are other aspects of our communication with Dr. White that I'm not wild about. Dr. White said that Ethan needed to have this surgery within the next two years. From his clinical experience and medical understanding he has a reason for that timetable. But, from talking with Dr. Walker it seems clear that Dr. White's preferred time frame is not as hard and fast as it was presented. Nor has Dr. White has done the best job explaining his rationale for performing this surgery within the next 1-2 years. Soon after we received his recommendation, I wrote to him and asked him to for more information about the timing and he wrote back and literally said "that works best for me."
One of the big challenges of working with complex pediatric patients who have multiple conditions and who undergo a seemingly endless series of medical exams, tests, evaluations, and procedures is that you need to look at the whole kid. In presenting his timetable as a firm medical necessity, Dr. White made it difficult to initiate a conversation about whether or not that timetable is ideal for Ethan. Dr. White even went so far as to say that he'd like to perform the surgery in April (of 2014, I assume) because he wants Ethan to be in the ICU for a day or two after the surgery and pediatric ICU beds are difficult to get during the summer. I would imagine that they are difficult to get because parents don't want to pull their kids out of school if they don't have to. Now, Ethan may be developmentally delayed, but that doesn't mean that it's any less important for him to be in school. It's just the opposite.
Sarah and I are pretty clear that unless a critical issue comes up, we don't want to subject Ethan to a major surgery until his communication skills improve. He is making progress, but they need to come a long way before we will be comfortable that he will understand an explanation of the surgery, will be able to ask questions and tell us about his worries and fears, talk to us about pain and discomfort and communicate with us during, what will be, a very difficult rehab.
I would hope that a doctor who works with complex patients would recognize that the timing for a surgery like this is not necessary cut and dry and that he or she would initiate a conversation with the parents about what makes sense for the kid. I mean if an extra year could bring additional communication skills that means the difference between a successful rehab and having Ethan struggle through it and end up in a wheelchair.
So... That's as far as we've gotten in this.
What's NextEthan's pediatrician, Dr. Elia, has suggested that we have a care management conference call between Sarah or me, Dr. White, Dr. Elia and Ethan's nurse case manager. The call would be an opportunity for Dr. White to better explain his rationale about the timing of the surgery and for us to share our concerns about how and why Ethan's communication issues are a concern for us.
If you had asked me a week ago to describe the purpose of that phone call, I would have given you a radically different answer. I would have started with, "It's an opportunity for Dr. White to convince us that Ethan even needs this surgery at all. If he can do that, then we'll talk about the timing."
We've made a lot of progress on this in the last week. Or at least I have. I've worked more on this than Sarah has, because that's my job. I've tried to share a lot of this with her, email conversations with other MPS parents, articles, etc.. And I've tried to let her know where my thinking was at certain points so that she would have an idea where I'm coming from and where I might be going next. But I think, with something like this, you go through the seven stages of grief. Not those exact steps, but certainly similar levels of denial and acceptance. In any given day, I can have multiple opinions about whether or not we should subject Ethan to this surgery. Coming to accept it, accept the rationale, the promised benefit, and the pain that stands between here and there, has been and will be a process.
In the end, I expect that Ethan will have the hip replacement, and at the some time, they'll put plates in his knees to fix that. With luck, we'll be able to put the surgery off until summer of 2015, when Ethan will be 7. If we're unlucky, his hips will get worse and he'll have to have the surgery before that. And if we're really unlucky, we'll put the hip surgery off until 2015, then his spine will destabilize and we'll have to postpone the hip surgery to deal with the spine, the who knows where we'll be when we finally get back around to his hips.
Caleb NewsCaleb had his annual well-child check up a couple of weeks ago. During the exam, Dr. Elia discovered that Caleb's left ear tube, which fell out of his ear drum 3 or 4 years ago, is still sitting in his ear canal. It's been in there so long and the was has built up behind it to such an extent that it's impacting Caleb's hearing.
We tried to wash it loose at the doctor's office, but that didn't work. So we're doing about four weeks of a ear wax softener at home and then we'll take him back in and see if it helped.
If that doesn't work, we'll take him to an ENT to see if he/she can pull it out and if that doesn't work, well....
About a week after we found out about that, I got a call from Caleb's school saying that he was in the health office complaining about pain in his ear. Now, it may well be true that his ear hurt, but oddly, he never complained of pain in his ear before we found out about the tube.
Caleb's a great boy and he's great with his brother. But I think growing up with Ethan has given Caleb a touch of hypochondria. It's certainly not his fault, he has no way of understanding what normal childhood maladies are and can't tell what's a minor annoyance that we just kind of deal with and what's serious. And he also sees Ethan getting a lot of attention for being sick.
But still... When the school secretary put Caleb on the phone so he could explain what was going on, I really thought I could hear the smirk coming through the phone.
Ethan and I rode up to Caleb's school and I gave him some children's Tylenol. And I told him, "look, there are going to be times when your mom and I can see that you're not feeling well and we keep you home from school. And there will be times when you're not feeling well and we can't tell. That's legitimate. But school is really important. You've already had one 'maybe sick/maybe not stay home from school day' you can have one more of those this school year. If you decide to come home today, you'll have used your last day and from that point, if you're kind of not feeling well, you'll have to just tough it out. Have your Tylenol and then wait to see if it helps and then decide what you want to do.
Five minutes later, Caleb said he wanted to go home.
Before the school year is done, I expect I'll get another call from the school secretary. And that will be a learning experience for Caleb, too.
|Sarah's folks gave the boys this fantastic playhouse. Talk about physical therapy!|