Breaking the Streak -- A Night in the ER

This past week took us down an old road. It took us to back to the emergency room, a large abdomen, blood in the stool and the realization that making even the tiniest of change to Caleigh's regimen would land us in a place we didn't want to be. 

Last Thursday we picked up and started an increased dose of compounded sulfazalazine medication that Caleigh takes for ulcers. Boston needed to increase the dose for her weight and they wanted to give it to her in liquid form which she would absorb better. We went back and forth with our pharmacist about what the compounded solution couldn't have in it. It was clean of things that Caleigh hasn't tolerated in the past. So we started it. 

One day later, May 8th, Caleigh wasn't feeling great. She had a small amount of bright red blood in her stool and her tummy was getting bigger. We had labs and a urine sample to complete that afternoon anyways, but I called Boston to tell them what was going on. We decided to stop the compound and go back to our regular dose and powder form. They also wanted Caleigh to be seen by a doctor just in case. No one in our GI office would call Boston back and our Pediatrician, who has been great with the transition, said she would see us. Looking at the time though, 4pm on a Friday, she decided to send us to the ER where we could get all the testing done in one place. 

So we went to the ER. We broke our streak. Caleigh hasn't been in the ER, much less the hospital since February 2010. It was a long break and after being in there for a ridiculous six and a half hours we remembered why we avoid it at all costs. Going to the ER was silly really, but I understand that Boston doesn't know Caleigh and they can't take any chances being so far away from us. It took three sticks for an IV just in case she needed fluids, which she didn't. X-rays were fine and so was blood work and urine. We got home around 11pm that night. 

The next morning Caleigh was really sick. Not tummy sick, but definitely head cold, flu-like virus... not feeling good. She slept upright next to me in the recliner most of the day. We slowed down her feeds, rested a lot and dumped tons of saline in her nose to keep things loose. Thank goodness our girl blows her nose like a champ. 

By Sunday she was much better and the one time blood in the stool didn't continue since we stopped the newer version med. It was all just a coincidence of events which is usually Caleigh's style anyways. She gave Eric the bug by Tuesday, I got a few days of nausea and exhaustion and we all continued to generally feel terrible most of the week. 

Boston has decided not to make any other changes other than increasing calories before we head back up to see them. They (and us) don't want to be sitting in the ER and back tracking until we see them again. We're not rocking that boat. 

Three Weeks Home

We've been home now for several weeks and we have fallen back into our routine... if you want to call it that. We don't have a solid schedule and that can make the weeks fly by. 

Caleigh went to a new eye doctor and got a new prescription. A little more near sided and a little more astigmatism. Her new glasses are on the way. 

We did her every 6 month dentist appointment. Everything looked great. 

We went swimming for the first time last weekend. We don't plan on stopping until we are headed to Boston. 

The quick update on Boston is that Caleigh's vitamins and minerals looked much better than they were expecting. We've increased her fluid amount and just yesterday upped her calorie intake to 24cal. We will be increasing her calories over the next couple of weeks and hopefully she will tolerate the change.

If she can adjust to the increase, we will plan on going to Boston in one trip. Head up for pre-surgery testing and then go ahead with the surgery in the same visit. This could be June or July. We aren't certain on timing yet. Caleigh wouldn't need to be on TPN before her surgery in this scenario. 

We're in the process of getting a few supplements compounded and changing home health companies. Boston has been incredible through all of the hiccups in transferring Caleigh's medical care. They get a huge thumbs up so far. 

So the plan is to keep increasing calories, swim and play as much as possible, and truly enjoy our time at home. 

Hello Boston

Caleigh's appointment at Boston Children's Hospital was at 12:30 on Thursday. We were told to expect a 4 hour appointment and we got that and then some. Which is a good thing. 

Eric and I have decided to transfer Caleigh's care to Boston. After the third hour with the very organized intestinal care team we knew that this was the place for Caleigh. As much as we connected with the surgeon in Pittsburgh, Boston was the complete package and that made all the difference. 

The traffic that we experienced in Boston was terrible, the weather isn't our cup of tea and the hospital is heavily under construction but we are here for Caleigh's care and that was the most important part of our decision.

Another big factor that we will have to deal with is that our insurance company is willing to pay for travel and lodging for Pittsburgh where they aren't willing to do the same for Boston. It's all about coding and department specifics. Pittsburgh would classify Caleigh's case as a transplant candidate because their transplant and intestinal rehab departments are grouped together and use the same doctors. Boston isn't willing to code her case that way which is probably accurate considering her health right now.  Boston's transplant and CAIR departments are separate. So if we are in Boston for long periods of time we wouldn't have that added monetary support. We will definitely be fine without it, but it would have made the deal sweeter.  I'm glad we aren't talking transplant one hundred percent. 

Boston had already requested Caleigh's medical records including the operative notes from her STEP procedure in 2008. They had called me prior to us coming up and asked a lot of questions. I sent them her formula recipe, medical history timeline and medication/allergy list. They knew so much about Caleigh before we even walked in that it made the appointment really easy. The questions we were asked were basically to fill in the small gaps. The whole five hour appointment we were rarely left in the room alone. There was always someone with us learning more about Caleigh or giving their opinion on how we should proceed. 

They were immediately concerned with Caleigh's weight, growth and overall nutrition. We know she is small and I've noticed it more over the past six months or so that she is skinny. She's been 38 pounds for over a year now, but still growing in height. They are worried about her vitamins, minerals and bone health. 

The surgeon that we met with was extremely knowledgable. He immediately pointed out the surface veins that are all over Caleigh's torso. This could be a sign of an old blood clot. This gets complicated, but it goes back to damage to her liver from her being on TPN for so long. As the doctors in Pittsburgh suggested this could be causing the veins inside her intestines. A portal vein issue. 

Boston doesn't believe that the veins are rupturing causing the bleeding episodes that Caleigh is having. They are guessing that there are a lot of sources. They have recent research that some kids that have had the STEP procedure actually bleed along the staple lines. They think this may be her case as well as ulcers, irritation etc. around the known strictures. 

The "Granulation Tissue" that Caleigh has inside her g-button stoma that is so painful isn't actually that. Granulation tissue is something that happens on the outside of the body that is painful. It's a different kind of tissue that is, we are told, a different kind of pain to painless. In Caleigh's case she actually has a severe irritation of the gastric mucosa, which is the inner lining of the stomach. This makes me cringe because our GI has been cauterizing this off during scopes which must be extremely painful for Caleigh. They are going to give us some tricks for stabilizing the g-button and the option of moving the stoma to a different location if all else fells. Again, this is rare and they really haven't seen it. 

Hello Boston via Caleigh's Corner

Hello Boston via Caleigh's Corner

MTHFR... we recently found out that both Eric and I are carriers of this gene. Caleigh got two strands. In simple terms it means that you may have clotting issues and that you can't break down folic acid in its inactive form. Eric and I both are on methylfolate, which is the broken down version of folic acid, and a B12 supplement. It's made a huge difference in our energy. They want Caleigh on the same. 

I also found out in the last year that I have the auto immune disease Hashimotos. This is basically my body attacking my thyroid. Everyone told me that my symptoms were caused by stress and it took 7 doctors and 4 years to figure this out and that is the short story. Since this is an auto immune disease it means that it is genetic. Somewhere, although I don't know where, the auto immune genes were passed on to me.

All of this to say that Boston is concerned that Caleigh has something metabolic/genetic possibly happening. Mostly because of her ongoing dumping syndrome that raises its hand every now and then. Her blood sugars get into the 300s and within an hour of her feed being done her glucose would drop to the 40s-50s causing Caleigh to feel terrible. Typical dumping syndrome involves stooling out, but Caleigh doesn't budge from her normal twice to three times a day routine. With the MTHFR, premature adrenarche and my auto immune disease they want to investigate her endocrine system to see what is going on.

What was impressive was the fact that they were very aware of MTHFR and how it would affect Caleigh during surgery. I'm pretty sure that was the hook that pulled us in. It's still a fairly new finding and most doctors don't know about it yet so this was wonderful and reassuring to hear from them. 

So the plan... Once we told them that we would like to transfer care to Boston they started processing everything. They ordered blood work for that afternoon and an abdominal ultrasound for the next morning. We will be planning on returning to Boston in the near future for about a week and a half for more tests. This will probably be inpatient so that we can easily add in hepatology (study of the liver, gallbladder, etc.), endocrine and genetics as well as the intestinal tests that she will need. Caleigh may also be put back on TPN at this time to get her body in the best nutritional condition possible before surgery. 

So yes, surgery is in the near future for Caleigh. They want to remove the strictures, lay out her intestines and do an intra operative scope to really see the inside surface of her intestines and proceed from there. It will basically be a very long exploratory surgery. 

They are very confident that Caleigh's food intolerances aren't really intolerances, but more of an anatomy issue. They feel like they can rehab her intestines and there was mention of attempting real food again after things are up and running again. We will have blood work back this coming week and we will have a plan of increasing calories and moving over to continuous feeds for more absorption. 

The ultrasound that they ordered was to see Caleigh's liver, veins and abdominal blood flow as well as veins for future central line access. Caleigh was nervous since she doesn't remember having an ultrasound. At the end I asked her if it was the easiest test ever and she said yes with a smile. I wish all medical tests could be like ultrasounds. We should have these results this week as well. 

Caleigh was in agreement with us about the Boston team and we happily finished off our visit with a trip to the aquarium which she absolutely loved. She shared so many smiles while we were there. As for Eric and I... we aren't on our short gut game like we used to be, but it is coming back fast enough. We're happy with our decision and glad that it was an easy and clear one to make. 

Hello New York - Day Two

Our second day in New York City was much slower paced. We woke up later, got ready a little bit slower and made it out of Brooklyn around lunch time. 

There was a ten minute period of time where I was convinced that I had lost my iPhone. In an embarrassing turn of events I found it safely zipped in a side pocket of my purse. We ate lunch at Roast Kitchen which we stubbled upon. The line was out the door so we knew it was a good bet and it turned out to be a great choice. 

When we finished up, we walked over to 5th Avenue and into the American Girl Store. We looked around the first floor, made it to the second floor where Josephina's collection is. Caleigh decided she wanted the Fiesta Dress and that she needed to use the restroom. So we went to the third floor where the cafe and a restroom is. Having a child with Short Bowel Syndrome requires a lot of bathroom breaks, but Caleigh did great while we were out and about. I'm pretty sure we saw more water closets than sights on this trip though. 

I've failed to mention that while we were in NYC it was their spring break. To say the store was crowded would be an understatement. We managed to push our way around and made a hair appointment in the salon for Caleigh's dolls. That appointment was for 6:30pm. Our tea time was at 4pm. So in between shopping and restroom breaks we went over to Rockefeller Center and walked around. 

At 4 we showed up in the cafe where they were completely accommodating to us. There is a flat pricey rate charged and little did I know it really isn't a tea party. The dolls get a plate and a tiny cup, but the whole experience is Americanized. Milkshakes, pigs in a blanket, tiny sandwiches and every sweet thing you can think of. Since Eric and I don't eat that stuff and Caleigh doesn't eat in general they gave us fruit and vegetable kabobs and drinks. It wasn't what I thought it would be, but now I know.  

Once we were done, we went back outside and walked down to St. Patricks Cathedral. It was very crowded and they are doing a lot of construction so we didn't stay too long, but it really is a beautiful place. 

Caleigh was pretty worn out by this time. Finally we made it back up for the doll hair appointment. Those ladies are artists for sure. Caleigh was really demanding about wanting black and white ribbon. This isn't something that American Girl Salons carry apparently, but our stylist winked at Caleigh and told her she had a box in the back full of stuff for Caleigh to choose from. They pulled off the black and white ribbon, Caleigh picked hair styles that I will never be able to recreate or even try and we were done. 

When we got outside it was pouring down rain. We had plans to walk down to Central park, but it was raining too much and by this time it was pushing 7:30. We called it a day. 

We got a car back to Brooklyn and got pretty soaked in the process and then spent a really long time in traffic. We learned all about our driver's life, family and distaste for the city he lives in. It was entertaining. 

That was it... Caleigh's first trip to New York. The next morning we got a fairly early start and made the short four hour drive to Boston. 

Our update from Boston Children's Hospital is next...