Tubie Friends

I participate in a forum designed for parents of children with complex medical issues (parent2parent), which has been a lifesaver for my sanity. They have forums for children with ridiculously severe reflux, forums for kids with respiratory issues, forums for kids who have many issues but no comprehensive diagnosis, and forums for tube-fed kiddos (among others).

When I mentioned that we were moving forward with the fundoplication, I received an email from one of the fabulous moms, who offered Nolan a Tubie Friend. Tubie Friends is a wonderful group of "surgeons" who place feeding tubes into Build-a-Bears (and other stuffed toys), then ship them to a child who will be getting a feeding tube. The toys are free, and the parent requesting the Tubie Friend pays the shipping cost.

I was still bewildered by the news of the pathology report and our solid decision to proceed with the surgery, and her email of support and kindness was very needed at that point in time.

As a mommy, I am at a complete loss for how to talk about the surgery with Nolan. It is too soon for him to know that he is going to have surgery, since it is an entire month away. At some point, however, we'll have to discuss it with him. We can't have him waking up and discovering a plastic valve jutting out of his stomach without a prior explanation.

How in the world do you explain a g-tube to a four year old?

Nolan's Tubie Friend has made our job as parents much, much easier. Nolan still knows nothing about his upcoming operation, and we don't plan on telling him until we are only a few days away from the actual event.

His Tubie Friend arrived in the mail on Saturday, and we gave the toy to him on Sunday morning.

Nolan was delighted, and asked what the "plastic thing" was in the puppy's tummy. "It's a button. The puppy has reflux and a sick tummy, and the button lets him get medicine in his tummy or his mouth."

Nolan immediately connected the extension set to the Tubie Friend and gave the puppy "Nexium."

He played with the button for quite some time, until he was satisfied with how it worked.

He also gave his Puppy Nexium in the more traditional way. He played for quite some time with the syringes (already a big part of Nolan's life with his daily meds) and with the main valve on the MicKey button. Tubie Friends outfits the toy with the exact type of g-tube or j-tube (or g-j tube) the child will be receiving, so Nolan's has the MicKey g-tube.

Nolan named his puppy February.

When we get closer to the actual surgery (probably a few days out), we'll start talking to Nolan about the fact that he will have a surgery to fix his tummy. When he wakes up, he'll have a button like February the Puppy.

Because of the Tubie Friend, the idea of a "button" will be less strange and less scary. We can demonstrate things like tube flushes and balloon fills on the puppy before it is done to Nolan.

The Tubie Friend has helped this scared Mom and Dad, too. We're able to see what it looks like before it is placed in our son. We can see that it isn't all that big, and it is simple to operate.

To all the surgeons at Tubie Friends, we love you, and thank you from the bottom of our hearts.

You have made a really difficult time in our lives a lot easier.

Struggling, And Trying to Avoid Platitudes.

Last week was a little rough. I'm not the type to break down in hysterical tears when things aren't looking up, but I was (and still am) struggling to process and deal with the upcoming surgery and terror-inducing biopsy of Nolan's esophagus. Last week, I didn't even want to get out of bed.

Not an option, of course, with two small boys and a rambunctious dog, and I am grateful that life surges on and forces me to keep moving. That feeling of extreme exhaustion was replaced by obsessive googling (my go-to coping mechanism, and yes, I'm aware that it isn't exactly healthy). Now I'm just a little stressed, a little sad, and able to keep things in (better) perspective.

In general, I don't write about my feelings or the profound sadness and stress (and anxiety/worry/panic) that accompany any new diagnosis or revival of an old one. It's too personal, and I honestly cannot stand the platitudes that people (who do not have sick kids) throw around in these situations.

God doesn't give you more than you can handle.
He doesn't look that sick.
I couldn't do what you do.
It could be worse.

These things add stress to an already stressful situation. The "it could be worse" statement is probably the cruelest thing anyone can say to a parent struggling with a sick child. It makes light of the current situation - a situation that is difficult, painful, and stressful. Of course it could be worse. It could always be worse. But that doesn't make the situation we're going through now easy. That statement makes the claim that what my child is going through is nothing. Not a Big Deal. And I hate it.

These statements implicate that you must accept every situation with, "WOW, what a great day! Everything is beautiful and it is such a wonderful thing that my child has XYZ! What a blessing!" Trust me, not everything is a beautiful, sunshiny gift - some things are tough as nails. We're getting through it, but we're terrified, stressed, and uneasy about the decisions and surgeries that are ahead of us.

The g-tube is the most stressful thing, I think, because it is a visible reminder that something is Not Right. Everything else is pretty much invisible (well, when he's not wearing his hearing aids or covered in a C-Pap mask), but the "button" on his tummy really bothers me for some reason. At first it was going to stay in for a period of three months, but after talking to the GI team, it will likely stay in for as long as Nolan needs help breathing at night. His stridor is still quite loud, so I doubt that the C-Pap is going anywhere soon. Which means the "button" will be sticking around for a while, too. And that makes me sad.

I am SO grateful for other parents who have "been there, done that" and assure me that the button is a Big Scary Thing when you first make the decision, but that it ends up being no big deal. The kids can swim with it, play with it, and no one even knows it is there. One group of parents is particularly amazing and has helped us immensely, but I'm saving that for another post. Tough times create beautiful people, and there are some really outstanding people out in the world.

A gift from some wonderful people - 
helping moms and kids to cope with tubes, one friend at a time.

Surgery Scheduled

Nolan's surgeon called on Monday. Nolan's case had been presented to the GI team, and they reviewed all of his records. Nolan's reflux is very severe, but his records are a little confusing.

His initial tests (in 2009, prior to diagnosis) were:

Upper GI Barium X-Ray: Entirely Normal (normal anatomy, no reflux noted).
Gastric Emptying Scintiscan: Delayed Gastric Emptying (gastroparesis) noted, normal for reflux.

The other tests from the ENT's office included:

Pharyngeal pH probe, done ON medication (Nexium): significant airway reflux detected.
Pharyngeal pH probe, done ON medication (Nexium + Zantac): significant airway reflux detected.
Upper GI Barium X-Ray (2011): Normal anatomy, one episode of reflux noted.

Fortunately, the remainder of Nolan's tests from the GI doctor's office arrived. Those tests included:

pH Impedence Probe (dual channel): Severe reflux, slow bolus transit time.
Endoscopy with Biopsy and Pathology report: Reflux esophagitis with cellular dysplasia (Barrett's Esophagus).

Whether Nolan has delayed gastric emptying or not is completely insignificant now, because he has cellular changes in his esophagus - these cellular changes are very, very rare in children. The severity of Nolan's reflux, coupled with the respiratory symptoms, is enough to warrant a Nissen Fundoplication. With the cellular changes evident at the age of two, a Nissen Fundoplication is the only real way to proceed.

Barrett's Esophagus is a condition that predisposes people to cancer. The cells exhibit specific changes, and over the course of time, can turn into esophageal adenocarcinoma. The average age of diagnosis for Barrett's Esophagus is 55 years - in other words, the average person with Barrett's is a senior citizen.

Since children with this condition are so very rare, many GI doctors will not see a child who has these changes in the esophagus. I'm not sure how Nolan's original GI doctor missed it, or why she didn't ever do a follow-up endoscopy and surveillance biopsy, but I suppose the point is moot. The GI team at Golisano's saw it, and that is what matters. His GI care will be in Rochester now, anyway, so the little guy will be appropriately cared for.

The surgery (Nissen Fundoplication with Mic-Key button placement) has been scheduled for March 7 at Golisano Children's Hospital (part of Strong Memorial). The surgeon will do a repeat endoscopy with biopsy prior to the surgery.

If the biopsy shows that his esophageal cells have reverted to normal, then we can rest assured that the Barrett's is gone. If the biopsy shows that the dysplasia remains, then Nolan will be scoped and biopsied every six months for the rest of his life. If a scope shows changes in the pathology of the cells (advancing stages of this pre-cancerous condition), then further action would be taken at that time (I won't go into that now).

I'm still flabbergasted that our original GI doc never did a follow-up scope. I know the condition is very rare, but surveillance is mandatory with dysplasia - the risks are simply too high to ignore.

I'm also kicking myself, because this was one of the very few reports that we did not have a copy of. We still don't have an actual copy of the report - I only remember being told (verbally) that the pathology report showed "ectopic gastric mucosa" and "blunted villi," not having any idea of what those two things meant (and searching the interwebs for useful information with those words was futile).

The good news is that there is now a team in place to follow Nolan and to monitor the histological changes. I can't express how grateful I am that we made the decision to transition to Rochester for Nolan's care. And, hopefully, the new biopsy will show healthy, normal cells that haven't been altered by repeated acid exposure.

There are a few research articles on Barrett's Esophagus in children - I'm posting this here for my own reference:

Barrett's Esophagus
http://www.nature.com/gimo/contents/pt1/full/gimo44.html

Barrett's Esophagus in Children: Diagnosis and Management
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1242875/

Endoscopic Surveillance of Children with Barrett's Esophagus
http://www.hon.ch/OESO/books/Vol_5_Eso_Junction/Articles/art282.html

Barrett's Esophagus in Children: A Study of 31 Children:
http://journals.lww.com/jpgn/Fulltext/2004/06001/P1048_Barrett_s_Esophagus_in_Children__A.1172.aspx