Our journey gets longer…

February 2016

So much to write and think about after our week long trip to the CHARGE Clinic at Cincinnati Children’s Hospital for evaluations of both Isaac and Matthew. Some of the things we were looking at were the same for both boys, but there was a more substantial look at Isaac and his issues with his stomach, esophagus and airway.

First of all…the trip was well worth the drive, the logistics and planning for a family of seven to travel and stay for a blended “vacation” and hospital visit were tricky. We say “vacation” because were excited to find out that through the hospital we could get both a discounted hotel room and free tickets to many local attractions. Intertwined around day long appointments we were able to visit the zoo, the museum center, the aquarium and the Creation Museum.

We know that for the last year or so we have been living somewhat in a state of denial with Isaac. He has been relatively healthy, growing and thriving in development and mobility. The denial comes in what is underlying in his throat, lungs and belly. We know that there are problems, but we didn’t feel the urgency to do any intervention about it. He (and we) have been through so much we needed a break and a time to figure out a game plan for the future.

The following is the short (and long) of what we experienced and learned from the team of specialists, nurses and doctors that make up the CHARGE Clinic at Cincinnati Children’s Hospital (This is currently the only functioning CHARGE Clinic in the country). A side note, we loved the team approach and the head-to-toe evaluation of both Matthew and Isaac. We will use that format and update all of you on what we learned, as well as what you can pray for.

Psych Evaluation

Both boys were “interviewed” and their specific strengths and weaknesses were taken into account as far as where they are mentally and socially. The area they were leaning toward is if they would fit the criteria for being autistic. With that being said, there are two camps on if are they CHARGE behaviors or Autism behaviors. The answer is “yes”, actually both. CHARGE behaviors mimic and look exactly like Autism…the trending theory though, is that the root cause of the behaviors are different. On the strategy and the treatment side…the approach to addressing and minimizing the behaviors are the same. So, why would we look for and attach yet another “label” to Matthew and Isaac of Autism? It comes down to services and strategies to get the help that Isaac and Matthew could benefit from. There is much more awareness and thus more opportunities and services and strategies that therapists and doctors have access to in the Autism spectrum world. We can look at it in this perspective…a family unit is a family unit no matter how a family comes together. Natural birth parents, adoption, blended families, etc. are many ways to arrive at a family. To make a family unit work together like love, forgiveness, being there for one another, self-sacrifice and more are the strategies and “treatments” are the same and useful for any family to be successful. In testing and evaluation Matthew and Isaac both fit the clinical criteria for Autism and thus are open to the opportunities and services available to them. I have to throw out a disclaimer here…I have grossly simplified the parallels in between CHARGE Syndrome and Autism behaviors. They are both very complex but I will mention again…the strategies to dealing with the behaviors are beneficial to both.

ENT/Pulmonary

Isaac had a FEES (fiber optic endoscopic evaluation of swallow) which was very helpful. As the name says this test is to evaluate his swallow. While he is awake they insert a tiny camera down in through his nose that goes down to the voice box.  Sadly this tested confirmed that he had saliva everywhere and in large amounts. Not really surprising, but disappointing to find out that his larynx is not functioning like it should. It was not stimulating him to cough and clear the saliva. Under normal circumstances, one would be gagging and retching while this procedure is done. Isaac’s is either desensitized by all the reflux and secretions and/or he may have cranial nerve abnormalities in that area. Either way, not sure how optimistic we should be that he will ever have a strong enough swallow to eat by mouth. Plus it also indicates that he is aspirating his saliva. Which brings us to the next test.

They did a high contrast chest CT scan. This confirmed that he is aspirating and there are signs of lung damage as a result. This was very discouraging. It confirms that we do need to remove his largest salivary glands very soon. He will still have many salivary glands left, but by removing the largest it will reduce the flow from a firehose to a water fountain. Hopefully with a more manageable amount of secretions he will be able to handle them better and stop aspirating thus allowing his lungs to heal. We are grateful the damage is not worse at this point.

Whether or not we will remove his always large tonsils at the same time is still being discussed. It will likely not improve his aspiration, but it may be able to improve the potential for his swallow as it will open up the area. With the constant copious secretions his whole airway/esophagus tissue is just enlarged and irritated thus making the area narrow. As a matter of comparison an analogy of the proportional size of the back of his throat is was explained to us recently that if normal size of the opening was the diameter of the top of a soda can, Isaac’s opening is only the size of the tab to open the can. Elimination of four of the six major salivary glands is something that will be a priority when we deal with the following issue(s).

Gastrointestinal (GI)

Isaac’s GI issues were our main reason for getting the second opinion before moving on to yet another major surgery. Isaac’s esophagus and tummy has probably been his biggest problem from the beginning. As you may remember his esophagus was not connected to his stomach at birth and he had a very difficult and delicate surgery at just two days old. He has since needed over 20 dilations of the scar area where the two sections were joined together. Each time undergoing anesthesia as they insert a small balloon to inflate and thus breaking up the scar tissue. He has also had two Nissen Fundoplication surgeries in which they take part of the stomach and wrap it around the bottom of the esophagus to prevent reflux. Both times this surgery appears to be fine, intact and textbook as far as the procedure…but, neither of these surgeries worked well for Isaac. During our visit the Cincinnati Isaac had a number of scopes (one each by ENT, pulmonary & GI). While showing us the pictures the GI doctor explained that Isaac has a “typical CHARGE stomach”. When we looked puzzled he asked if we knew what a normal stomach looked like and then proceeded to show pictures and explain that Isaac’s stomach was completely smooth where it should have many folds allowing it to expand and contract. There have been a number of previous times that the surgeons have been inside his tummy and taken photos, but we don’t remember ever being told about this or having it explained. This basically confirmed for us that his stomach would never be able to function normally. He has a stomach, but it is far different from a normal one.

So, we now know that it is indeed in his best interest to proceed with a procedure called an esophagojejunostomy (that is a mouth full isn’t it?). Basically it is like a gastric bypass surgery. The surgeon will disconnect his esophagus from his stomach at the diaphragm (top of the stomach). They will then pull up a section of the jejunum (intestine) and attach it directly to the esophagus. That way any secretions or tastes of food will go directly to his intestines. The stomach will then be re-connected lower “downstream” in the intestine. We will then be able to feed him through his g-tube into the stomach without him being able to reflux and continue to damage his esophagus. Isaac will be able to taste, eat and enjoy food in very small quantities with this surgery. Although, as we discussed above, only if he is able to get his throat to cooperate.

Summary

This is a scary surgery for us emotionally. We have endured somewhere around 30-40 sedated procedures/surgeries but there are some that are “big ones”. This is a big one and with it comes many fears, anxiety and planning of logistics of the when, where and how do we still live life with the rest of our family. Decisions are hard but in planning this next surgery it is our hope and prayer that it will increase Isaac’s likelihood of living a longer, healthier life. Isaac will likely be in the hospital for up to two weeks, more if there are complications after this surgery.

Where, when and how do we make this happen?

We have great surgeons at Devos Children’s and at the University of Michigan that we have full confidence in to do this surgery. At this point though we are leaning toward having the surgery a Cincinnati Children’s due to the complexity of the surgery and of Isaac. We are more comfortable with the CHARGE Syndrome team approach in Cincinnati. Isaac will be scheduled to have the salivary surgery at the same time.

We have decided that it is best for our family as a whole if we do this surgery before Matthew’s school schedule changes to summer mode. He will stay home in hopes of maintaining a more normal routine and hopefully avoid behavioral issues and stress on the rest of us. Theresa will stay with Isaac the whole time while in Cincinnati with me only going down for the surgery, maybe a visit in between and the glorious ride home at the end. Hannah and Megan will join in to help with Matthew and Sarah Anne. Sarah may get the chance to spend some time with grandma in the Detroit area…she is super excited about this. The timing for this is currently being planned according to “our” schedule. Of course, there are always things that make our plan go differently. God’s plan is always the best and He may have something different in mind as far as when this all takes place! We are shooting for the mid to end of April, 2016.

How you can help? Prayer!

For the logistics involved in the family preparing for and being separated during this time.

Obviously for the success of the surgery.

Health both before and after surgery for the whole family.

Safety in travel back and forth.

God’s provision for the finances involved in all of this (we have great insurance PRAISE GOD!) but there are a lot of uncovered expenses such as travel, lodging, food, etc.

And just that everything goes as smoothly as possible…no car problems, appliance problems (we came home from first trip to Cincinnati and our furnace was broken), Matthew will sleep well, Theresa will get good rest while staying with Isaac in his hospital room for the duration of his stay. Pray that normal little life problems will not be abundant during this time.

For Hannah who will be traveling to Africa on a mission trip shortly after the surgery….please pray for the mission, and of course health and safety (no cooties brought back home). She is traveling back to Mozambique where she went three years ago.

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