What's New?

It's been quite awhile since we've done a full update.  Although we post frequently on Facebook, we don't go into a lot of detail on there.  We have made some big changes, and there has been quite a bit going on over the last 8 months or so.

Since our last full blog post, Sam has faced quite a few challenges.  His cyclic vomiting has been regular, frequent, and debilitating.  We are almost at the end of 2nd grade, and Sam has missed approximately 55 days of school this year.

It's been a rough year.  I would even go so far as to say that it has been the most challenging and stressful year that we have had since Sam was born.  Sam's mom had gone back to work full time when Sam started full days of school. With so many issues going on and with Sam being out of school as often as he has, we had to make the difficult decision to have Deb go back to working part time in the evenings so that she was available to be home with Sam when he was ill and be available to support Sam while he was in school.  His interest level in school has really been a challenge this year.

With school being such a challenge, we have decided to remove Sam from his current school beginning next year.  Both of our boys have attended a dual language immersion program since kindergarten.  With the amount of school Sam has missed he was really struggling with Spanish.  They move so fast with vocabulary that there was just no way he could stay caught up.  This let him feeling lost in Spanish and added to his anxiety and dislike of school.  This was a really difficult decision for us to make as it was such a good opportunity for him.  In the long run though, it will be better for him to be able to focus on learning in English which makes it easier for us to help him with make up work.  His school has been extremely supportive and we will really miss being involved with our districts bilingual program, but we are excited to have Sam hopefully be more successful and happy next year.

Our frequent trips to Boston have also begun to catch up with us as well.  For 8 years we have been making regular trips to Boston for medical care.  They have done a fabulous job of managing Sam's medical care and it is difficult to imagine that we could find another team that is as knowledgeable and as easy to work with.

But there has been a cost to having them manage his care besides the financial cost, the missed days of school and work, and the stress of frequent air travel on Sam and I. Boston has struggled to find a cause or treat Sam's frequent cyclic vomiting.  This is partly because it wasn't like we could just make a quick appointment with another specialty in Boston when needed.  Trying to have them manage his care, but using specialists in Wisconsin was not working the best. as there always seemed to be a break down in communication and we just weren't progressing fast enough.

This was also a challenge when we had other issues in between our regular appointments in Boston as we were relying on our team in Madison.  They weren't as familiar with Sam's care and weren't managing his TPN.  It made for a lot of confusion when we needed to be inpatient and added another layer of stress.

With everything that is going on, we have made the decision to discontinue our trips to Boston and allow our doctors at American Family Children's Hospital in Madison to take over Sam's care.  This was an agonizing decision to make especially because it means moving away from the hospital that saved his life when Children's Hospital of Wisconsin gave us a bleak outlook and mediocre care. There is also the fact that Boston Children's Hospital is providing our Omegaven. That is frustrating as well as it has been three years since we shared our story on NBC and there really has been little to no progress on getting Omegaven approved by the FDA.

So far the transition looks promising.  We are aggressively trying to rule out causes of Sam's vomiting.  Now, instead of just having Gastroenterology make the call and try medications that we hope will help, we  have several teams of specialists helping us.  Neurology, Urology, Neurology, Endocrinology, and Gastroenterology are all running tests and monitoring Sam's labs to try to come up with something that will get him some relief.  We have an awesome new Intestinal Failure team as well that is managing Sam's TPN.

American Family Children's Hospital has committed to bringing Omegaven to Wisconsin for Sam.  We are hopeful that this works out.  This is a little nerve wracking as, once we finish using our supply of Omegaven from Boston, if they aren't ready to provide it for us we will be left with no choice but to have Sam go back on Intralipid.

This sounds scarier than it really is.  Yes, this is the lipid that was killing him  years ago.  Sam is older, bigger, healthier, and stronger now.  If we have to go this route hopefully his liver will tolerate it better now.  This has happened with other kids who went back to Intralipid, so it is more than possible.  It is definitively not what we prefer - but the worse case scenario ends with us being back in Boston.

In other news, Sam had to have a new central line put in this week.  His line that was placed in Boston in late 2015 has been an issue almost since it was placed.  In the past we have dealt with trying to keep the exit site of his line intact and healthy.  Go figure that we finally manage this and Sam throws us a curve ball.

We were inpatient for a few days earlier in the year because there was an area close to Sam's clavicle that began to look puffy.  This was an area where his central line tunneled under the skin.  An ultrasound showed some fluid under the skin but it never really looked infected.  It subsided and they decided we should take a wait and see approach.  We never want to pull a line unless absolutely necessary as there are limited veins that these types of lines can be placed in.  With the multiple replacements we have had to have over the years we are always at risk of losing the ability to reuse those spots.

This area came and went a few times, but last week he developed what looked like a blister over this area which kept getting larger.  Our surgeon was no longer comfortable leaving the line in and we had it replaced on Thursday.

The old line was a mess and the decision to remove it was a good one.  Even though his exit site was healthy, there was infected fluid and granulation tissue throughout the tunnel of his line.  This was most like caused by contamination when the line was placed.

As frustrating as it is to have to replace this line, we did get some good news.  When Sam was an infant he had a line in his right jugular.  When they removed that line due to infection the surgeon tied off the vein.  We didn't find this out until a few years later.  This is a very old school way of thinking and especially not something that should be done on someone who is going to be on TPN long term because it essentially eliminates that vein being used for a line placement ever again.  In Sam's case enough of a "nub" has grown off of the vein that they were actually able to use it for this line placement.  This is a result of our surgeon being super thorough and utilizing Interventional  Radiology to help him identify the best possible location before starting the surgery.

So now you are all up to date.  A lot of good change going on that will hopefully allow Sam to have less stress, feel better, and spend more time being a kid.

"Family" Reunion

Recently our family traveled together to spend a few days in Gloucester, MA ahead of a regularly scheduled appointment in Boston.  We shared a house with four other families made up of some of the most amazing people in the world.

When you look at this group of people, they don't have much in common.  They all have different political views, differing religious beliefs, come from different professions, and have varying heritages. None of them live in the same state as one another, in fact, they don't even all live in the same region of the country.

A stranger walking into our group would have thought we were having  a family reunion or were friends who had known each other since childhood.

All of these families are living with Microvillus Inclusion Disease.

I don't mean that someone in each of these families has a horrible, incurable, life threatening, disease though.  I mean that these families live life to the fullest every single way they can.  They have bucked the conventional thinking that MID is a death sentence or something that you "suffer" from.

We spent that weekend living.  Having fun.  Laughing at each other and ourselves. We may also have consumed copious amounts of food and alcohol - but what happens in Gloucester, stays in Gloucester.

Sometimes the conversations turned serious.  We talked about challenges, disappointments, and frustrations we have experienced.  But these were sidenotes and not what anyone will remember about that weekend.

Many of us have children who we were told would never live to the age of 2, and that we were fooling ourselves by thinking there was an alternative to having a transplant with poor long-tern survival rates.  We were told that you can not have quality of life and live long term on TPN.

We have learned through experience and from each other that this is not the case.  We are not fighting a disease, but fighting to make sure that our children see past  it and know it doesn't control their lives.

We want to thank them all for sharing their lives with us and being the awesome people they are. They truly know how to Dream Big, Live Life!