I think it's fair to say that Ethan is an obsession with me.

Over the last few months, as Ethan's schedule of medical check-ins and check-ups has gotten lighter, I've added new appointments in a number of other areas.  And I've started to wonder if dragging Ethan to more appointments is a) fair to him or b) accomplishing anything?

Since the start of the year, we have gone to talk with local orthopedic surgeons and a new local geneticist to see if they are a good fit to work with Ethan, we've gone to see his PCP other specialists proactively to head off problems before they occur, we've broadened our discussion with Ethan's IEP team to make sure he's getting the services and help that he needs, and we've planned out a comprehensive schedule of evaluations at Ethan's one year Minnesota review next month.  Not to mention signing him up for karate classes, which, truth be told, he's really too young for.

Through all of that, I spend about 10 hours each week, talking with other MPS parents, reading about MPS, or learning about the school system special education system.  (Not to mention additional time paying medical bills, contesting bills, etc..)

Basically, my obsession manifests in three main areas: keeping Ethan healthy, putting together his medical team and mastering the IEP process.  The first one is probably the part that I have the most clarity on, so let's start there.

Keeping Ethan Healthy
Take care of the kid he'll stay healthy. Keep the kid healthy, voila, fewer pesky doctor's appointments.  Pretty self-evident, right?  Maybe to you, but in obsession land, it's not that easy.

For almost three years now, and not counting his inpatient stays, Ethan has seen a doctor on average once a week.  Even then, there were some health issues (for example his month long bout of adenovirus (and the exploding diapers from hell), and an ear infection or two) which we should have caught easily and early, that went on for far too long.

Now, I literally get nervous if Ethan goes more than a couple of weeks without seeing a doctor. I sometimes find myself suggesting additional check-ups a couple of weeks from now, "just to be sure he's doing okay."  (Though it is also possible that I crave the adult interaction, especially when we can talk about Ethan (it's a two-fer).)

Until recently, Ethan's always needed someone who knows what they're doing to diagnosis the whatever-it-is and prescribe the cures-whatever-it-is.  His medical conditions never just went away by themselves if he got some rest, OJ and chicken soup.

Then a few weeks ago, he got a cold, ran occasional fevers for a couple of days, coughed a lot, and slowly got better.  I was left wondering, "what the hell just happened?"  Ethan doesn't just get better.  Does he?  (I should add that at one point I did call the nurse coordinator at his PCP's office to double check the symptoms for pneumonia and ask if I should bring him in.  It turns out that the pneumonia signs that I needed to watch for had already passed a couple of days ago.  (Oops. I probably should have known that.))  Then the next week, Caleb and I got the same cold/flu thing and we had a harder time getting rid of it than Ethan seemed to.

Ethan's Cold Not Requiring Hospitalization (CNRH) highlights the fact that his health is stabilizing.  That is a milestone for us and, in truth, that simple cold highlights the fact that Sarah and I need to change our thinking and our conditioned response.

There will still be any number of sudden health issues that require a trip to the doctor for a diagnosis and medication (dx&rx).  But, there will also be an equal number of new health issues for which we will need to ad hoc a response at home. Many of these issues will be chronic issues that are a direct result of the disease.  Because of the rarity of MPS, there will be many times where there isn't a clinical response based on a well researched, controlled, double blind study.  In a good many cases, there won't be anything at all in the medical literature at all.  And there certainly won't be doctors here who have worked with whatever-it-is before.  The primary guide for Saran and I will be conversations we've had with other MPS parents and what we think will work for Ethan.

Sooner or later, we'll be cobbling together treatments that may include any number of herbal supplements and medicines, massage therapy, swimming, dietary experimentation, and even acupuncture.

In other words, Sarah and I are going to need to learn how to "wing it" and to learn by trial and error.   It's going to be a big change and one that will require ongoing self-education, mental gymnastics and adaptability.

Coming up with appointments when there are none.
Unless you've got an MPS kid yourself, It's unlikely that you've met anyone who has been poked, prodded, x-rayed, scanned, eyes, ears, nose and throated as much as Ethan has.  We're long past the point where he's lost patience with most of it.  Sure he'll hold still if the doctor wants to examine his skin or torso, but all bets are off if he brings out that little flashlight on a cord.  And don't even try to get him to hold still while that big, scary x-ray machine hovers over him.  Inconsolable.

Even so, I feel like a shop-a-holic: if I see a pretty dress in the window, I've got to go in and try it on.  Or have Ethan try it on. As the case may be.

Is it fair to Ethan to drag him to appointments that aren't absolutely necessary?  I wonder about it.  And that wonder may slow me down a bit, but it doesn't stop me. (Maybe there's a twelve step program.)
In late January, Sarah and I took Ethan to Shriners Children's Hospital of Portland to talk to talk to their ortho docs about Ethan's orthopedic issues.  In addition to his upcoming carpal tunnel surgery, over the next few years Ethan may have some number of orthopedic surgeries for his lumbar kyphosis, his knock-knee (ism?), and potentially for hip displaysia.

Frankly, we were there to see if they were good enough to treat Ethan.

A large number of MPS families get orthopedic care in Minneapolis where a whole MPS medical industry has developed to support the MPS patients get major care through the University of Minnesota Children's Hospital.  However, when it comes to orthopedic care, the longish recovery times and potential for post transplant complications can mean unpredictably long stays in Minnesota, away from our support here, and disrupting both family and jobs.  In addition, I've seen a number of families who end up traveling back to Minnesota if their kid develops complications after he/she goes home.  Clearly we can't make the 36 hour drive to Minnesota straight through with a sick kid.  And the thought of taking a sick kid in a body cast on an airplane so he can go back to Minnesota for treatment I think is both scary and unrealistic.

While we do like Minnesota and the wonderful folks there, we don't want to spend any more time there than is absolutely necessary. We would prefer to cobble together a qualified team here in the Northwest.  The key word there is "qualified." By this point, it is likely true that Sarah and I know more about MPS than 99% of doctors.  But we think that we have the right to expect that Ethan's doctors know or learn about the impact of MPS within their specialties. Sarah and I have become quite neurotic about making sure that the doctors who work with Ethan either have direct experience working with MPS patients or demonstrate that they're willing to research and do their homework about MPS as they work with him.

It all comes down to trust.  Before we put Ethan into their hands, we need to be able to trust their  judgment and advice.

To some extent, I think that Sarah and I feel responsible for the failure of Ethan's first transplant.  I don't think we asked all of the questions that we should have and could have; and I think we were scared of the risks of transplant and that fear seemed to grow quite a bit larger when we thought of going to Minnesota to have it done.

I know that's not really a fair self-criticism.  The period from diagnosis to ERT to transplant is a depressing, scary, dizzying time.  Invariably, parents are still trying to come to terms with the disease.  Trying to comprehend the risks and process of a bone marrow transplant on top of that is hard enough.  It's mind numbing to try to figure out the comparative risks and benefits from one center to another.   But even with Ethan's second transplant, it took a doctor with direct experience to kick us in the pants and tell us to get to Minnesota.

Without a doubt, that feeling of guilt has contributed to the research we do, the questions we ask, and why we're trying to cherry pick a team.  However, it is equally true that we've seen too many doctors who seem not to care about the underlying pathology of Ethan's conditions and who can't be bothered to research or look at the larger picture.

Wow.  What a long preface to our consult at Shriners.  Sorry for the ramble.

Sarah, Ethan and I spent a couple of hours at Shriners, but in the end I don't think that we'll use them.  We were told that they might be prone to fairly aggressive surgical interventions and timing.  If anything, we want just the opposite.  We know he has issues that might be corrected by surgery.  But given all that he's been through and the risks of the surgeries, we want to give him time and intervene only when it's truly necessary to save mobility and function.  We simply can't subject him to immediate surgery for every single issue.  He'd spend most of the next year in casts and fighting post-op infections.

Our ortho plan at this point is to get complete spine, hip and leg exams in Minnesota next month.  Then this summer we're planning to go up to Seattle to talk to the docs at Seattle's Children Hospital and compare evaluations and recommendations.  (As I've noted before, there's a small multi-disciplinary group in Seattle working with MPS patients.)  Yes, it's basically the same plan as we had before Ethan's 6 month last October.  But I made the mistake of only scheduling the hip and leg doc, and not having a full set of scans and x-rays done ahead of time to inform that discussion.  As a result, we really didn't get the full picture that we needed during that trip.

In the end, Ethan's team may be spread out over three states.  We'll need to go back to Minnesota for, at least, the next few years for transplant follow-up and for a couple specialties where we can't find the expertise anywhere else.  We will almost certainly have some local Portland docs in a few specialties, and we may travel to Seattle for some other things.

The Stacked Deck of the IEP process
The last major area that I'm obsessing about is the special education system and the individualized education plan (IEP) process.

According to the federal "Individuals with Disabilities Education Act," because of his special needs, Ethan is entitled to an individualized education plan designed to provide him with a free appropriate public education.

For those that don't know, IEPs for each qualified student are developed by individual teams comprised of the parents, the child's teacher(s), and relevant school system specialists.   In theory, they work in partnership to identify the child's needs and put in place appropriate modifications, adaptations and support. 

However, there are a number of inherent difficulties and conflicts within the IEP process.  Just in terms of evaluating a child, most parents are not special education or early childhood education experts; so most are not in a position to know what evaluation tools are appropriate, or if those instruments are implemented or interpreted correctly.

Second, most parents are not fully aware of the range or appropriateness of possible accommodations, modifications or supports.  As a result, it is very difficult to advocate for changes that a parent might feel would benefit their child. Further, even if they did know the full range of what's available, they likely would not know the criterion or standard practice trigger at which point those additional modifications or resources are typically applied.

Third, there is a conflict between the requirements of the law and school systems' budgets.  It is absolutely true that school systems are under-resourced and over committed.   It is not that educators want to withhold appropriate services, they honestly don't have all of the money they would need if they were to fully meet all of the special education needs within the system.

The resulting mess plays out in different ways.  Sometimes IEP team members may not initiate discussion of potential supports and will only begin discussion of them if the parent asks about them. But if the parent doesn't know what's available or appropriate, how could they know to ask?

In other cases, parents and school systems often argue about whether a student truly needs or would benefit from a particular support. (Again, parents are at a disadvantage because they don't know the accepted practice within that system or any system.  They don't know whether other children with similar issues might have been granted the support that they are seeking.  I've talked before about not knowing what normal is the context of discussing Ethan's medical condition.  The same is true here.  The parent's framework has been upended and they don't know what normal is in a special education context.

As if all of that weren't enough to create a lot of mutual head-banging, there are at least two more significant factors to add to the mix.

The IDEA law does not require school systems to provide special needs students with the best possible free education.  It requires school systems to provide a free and appropriate public education. So if parents' go in arguing that their child is due, X, Y and Z because it's the best possible support, they've probably lost the battle after that first sentence comes out of their mouth.

Oh, lest we forget, teachers today are underpaid and overworked and suffering under the weight of overcrowded classrooms.  For school systems out here in Oregon--at least--you may also need to throw in some resentment and push back from the teachers because he/she has taken a pay cut through mandated furlough days, or their colleagues have been laid off, and/or they may have been transferred from a school that they knew and loved due to consolidation measures.

Not only is the classroom teacher a member of the IEP team who helps shape the nature and extent of the accommodations, he/she is the one person most directly responsible for implementing the plan during the school day. But the teacher has 28 other students in an elementary school class.  How much time does he/she have to monitor your kid, let alone modify the lesson plan to meet your kid's individual needs?

Ethan's only 3 1/2, isn't this down the road for him?  How is all of this impacting him already?

For two and a half years now, specialists have been telling us that we need to focus on developing Ethan's language skills; that developing his ability to communicate will reduce his frustration, help him stay closer to the language learning curve for other students and provide tools for him to learn a lot of other essential skills.  Yet at 3 1/2 Ethan has essentially 10 daily spoken words and another 10 functional signs.

Even though Ethan does not speak much, he's very good at expressing himself nonverbally through getting your attention and pointing or using his few signs or words to accentuate his need.  Given that, and his ability to understand and respond to questions and directions, we've always thought that there is a significant and affective gap between Ethan's cognitive ability and his expressive ability.  In addition, we've come to believe that the gap significantly impacts our ability to properly test and assess his true abilities and needs.

After battling with Ethan's speech issues for a couple of years, three months ago, his speech therapist brought an assistive communication device in for him to work with.  As I discussed in a previous post, Ethan did very well with it and we are working through the school system to evaluate which particular model of adaptive communication device works best for him.

For more than two years, the specialists have been telling us how important it is to build his understanding of speech concepts and language concepts as quickly as possible. Now that we've asked for an evaluation, the school system staff seem sincerely interested in helping him.  That seems kind of odd since none of them had told us that this option was available.

It seems to me that the most likely conclusion is that due to time and resource constraints, the school system doesn't offer adaptive technology unless and until the parents ask for it.

But wait a minute.  If the purpose of the IEP team is to match the relevant specialists with the parents to develop a holistic education plan, how can they do that if the special education experts are withholding options?

I mentioned this to someone recently and said that I really felt like when it came to the IEP process I was in the dark about what supports were most suited and appropriate for Ethan.  She said to me that I was always going to feel that way.

I thought about that for a tenth of a second and said, "No.  That's not going to happen.  I will not always be behind the curve.  I will understand the IEP process and to advocate for the supports that will help Ethan."

And here's what this whole long post boils down to.

None of his doctors live with Ethan day in and day out.  None of them will ever truly understand the totality of how MPS impacts him or how their proposed treatment fits into the question of his quality of life.

None of his teachers live with Ethan day in and day out.  None of them will ever truly understand the totality of how MPS impacts him or which supports might help him the most.

Neither his doctors nor his teachers will ever truly understand what Ethan is capable of with the proper support.

And no one else is responsible for maximizing his potential, abilities, quality of life, not to mention his happiness, over a period of decades.

It sounds cliched, but we are his parents, and we have to be obsessed with every single aspect of Ethan's and Caleb's lives because no one else could ever care as much as we do.

Thanks for checking on us.



Marian said…
This is a really genuine, gut wrenching journal Todd. I applaud your honesty and I feel your "obsession". What honest parent would NOT feel this way? I am frustrated, for you, since the "systems" don't always address the needs of the child/family BUT you and Sarah are the very best advocates for Ethan and Caleb's health and education. Stay strong and know that you do have prayers sent your way. Hugs to all of you!
Karla in MN said…
Well said...we have also gone this road with our children. You are the best advocates for your children. Keep strong and know you guys are in our thoughts and prayers.
Cheryl said…
Todd when you have time, we should chat about IEP's. It is way to much to type it all out here. Lol. I will start by saying, with Andrew I have found that it is crucial for you to have the school nurse at every IEP meeting and be in constant contact with him or her. Everything we have ever got on his IEP, our nurse has helped us get. Their opinion is huge at IEP's. Also wanted to discuss Shriners Orthopedics.
Erica J. Thiel said…
Todd, as an adult with MPS I and no doctor can quite say where to place me in the spectrum (in other words am MPS I-unspecified due to my mutations and symptoms but at the same time my appearance) I agree that it is actually wise to 'cherry pick' doctors and pick those who you feel comfortable with, those who are willing to learn about MPS (one thing ive learned time and again even some drs who have quite a lot of experience with MPS really in reality know very little bc they cant realize that MPS isnt a solid and instead is a varied disorder meaning that no one person or child with this disorder presents the same and to many are not willing to accept that.) Good drs will look at Ethan and his symptoms and try to help him and not ocmpare him to every other MPS child's symptoms. Some of the best drs I have are not MPS drs but want to help, want to learn about MPS and may or may not have saw a few MPS kids in their training but what they care about is my symptoms and how can they help me. I to see drs at 3 different centers including UofMn, UWHC-Madison and FMLH/CHW and soon will likely add CHOP in Philly when that study starts more in-depth (I have thought about having that genetics team replace my own). Only you know what is right for Ethan and no provider can tell you what to do, nor can any other family, individual or article tell you what you will not know deep down. Sure its alot more complicated to have providers at different centers but if it means better care than I always think the complicated nature of this kind of care is worth it! As for where to have surgeries ive had some like my 1 of my 2 spine surgeries at UofMn and then had post-op care locally with xrays sent to the spine surgeon from my local surgeon/neurologist and they communicated/worked together + saved having to drive the 4 1/2-5 hrs back and forth numerous times. I just fup then at the 3 mo mark. Just a possible thought. All other surgeries like carpal tunnel, lumbar spine, etc., etc., have been local. My Cardiol talks about it may be the best option to have heart surgery done with Dr.Braunlins team (he knows her from his training at Mayo clinic) but I honestly dont know with that I may opt to stay local.
Erica J. Thiel said…
ps have you ever thought of programs like the autism sprectrum programs such as ABA which some families like Emma's and Mia's are using? I dont think you have to have autism specific dx and they focus on speech, OT, PT, and identifying eacg kiddos specific needs and therapists come to the home each day 5 days a week typically 4-5 hours a day...