Ethan's not so "tubular" adventure

I've not had the emotional energy to write much the last few weeks. I've been feeling pretty overwhelmed by the reality of the PBD-ZSD diagnosis on both our family personally and on my GFPD family at large.

Wednesday, March 20th
We took Ethan to our primary doctor's office because of his decreased intake and assumed that we would be heading to our local hospital for IV fluids. Our doctor thought that instead of staying locally we needed to head to the larger hospital about an hour away which has several of our specialists, including our GI. While we were actually waiting to see our doctor I got the call from the nurse at the GI's office with the date for Ethan's feeding tube surgery - May 8th. While the doctor was examining Ethan he said that he didn't think Ethan could wait that long, and that he was going to call the pediatrician/hospitalist at the larger hospital and see about getting us admitted for IV fluids that afternoon. So, we came home and packed our bags, preparing for a few days and headed to the hospital. We were admitted through the ER, so that took a while. Getting the IV fluids started was tough, and Ethan was very upset.

 

After a few hours we were up on the pediatric floor and getting settled in. We were seen by the GI and told that Ethan would have his tube surgery on Friday morning (just two days away). They continued to pump Ethan full of IV fluids and we decided that we would not even attempt bottle feeding for the time being. In addition to Ethan's decreased fluid and food intake which had lead us to being hospitalized and for the scheduling of the feeding tube their have been concerns about Ethan aspirating small amounts of his formula during bottle feeds, so we choose to stop bottle feeding in order to try to eliminate that possibility. 

Thursday, March 21st
We just spent the day hanging out in our room, Ethan was hooked up to the IVs so he didn't have a lot of mobility, but we took some walks around the floor, a few wagon rides, and visited the playroom. Ethan was looking a bit better, since he was getting pumped full of fluids and he began eating more of his applesauce and soy yogurt. Ethan's grandma and grandpa Marshall came back up for a visit and his grandma Chambliss and aunt Amanda also came up to see Ethan and so they could be with us the next day during and after surgery.

 

Friday, March 22nd was surgery day! Around 7:30am we left our room for the operating room. We spoke with the doctors and nurses and said goodbye to Ethan. Our pastor came up and prayed with us prior to Ethan going back for surgery. I was a mess. This was Ethan's first surgery and having him sedated is scary for me. We were really glad that one of the hospital's child life specialists was able to go back further than we were and also be in the recovery room with Ethan, since we weren't allowed to. After the surgery the GI doctor spoke with us and let us know that the surgery had went well and that Ethan was now in recovery. After a while we were headed back up to our room with Ethan. He was slowly waking up and getting the sedation out of his system. We just wanted to hold and comfort our little boy.

 

After a few hours Ethan started to be a more active as the sedation wore off and we had to make sure that he was wrapped up tight so that he could not pull at his surgery site and tube.

Here are some picture from when the nurse first unwrapped Ethan and we began using the feeding tube. For the first 18 hours Ethan got small amounts of pedialyte through the tube.

It was a tough day and night as Ethan recovered from surgery and we began learning how to use the tube and started to increase the pedialyte feeds to make sure that he was tolerating everything okay. Thankfully we all got some rest throughout the night.

 

When we woke up on Saturday, March 23rd we could tell that Ethan was feeling much better. He had continued to receive IV fluids throughout the night and most of the morning but got to come off of it by midday, which was great. We switched the feeds from pedialyte to formula and Ethan continued to eat small amounts of his purees. Ethan really enjoyed being off of the IVs!

 

After a long day of playing, we were all ready for some rest. We tried Ethan's first pump feeding that evening. Ethan fell asleep in his dad's arms!

 

We woke up on Sunday, March 24th hoping that we'd get discharged, but knowing that we wouldn't get the word one way or another until the afternoon rounds. So we spent the morning the just hanging out in our room, talking walks and wagon rides around the floor and just waiting to get the word if we'd be going home. Although Ethan was feeling really well, we couldn't go to the playroom because the fish tank in the room had flooded the room!

 

When the hospitalist/pediatrician came by he cleared us to begin discharge! He hoped that we could get discharged and home before the snow got too deep. We drove home in a snowstorm!

 

The drive home that usually takes about 45 to 50 minutes took over an hour and a half. It was a slow and scary drive home and we saw lots of cars in the ditch. I was so glad that we made it home safe. By the time we got home the snow was so deep that Jeff had to shovel the drive way so we could get in the garage.

 

Ethan was so glad to be home!

 

Monday, March 25th

Due to the snow Jeff stayed home with us. We were snowed in! It was in the midst of this that Ethan's site/feeding tube began leaking. We had been told not to change his bandages until the home health nurse came, but due to the snow they wouldn't be there until the next day. Our primary care doctor's office was close, the GI clinic was closed, and so we called the pediatric floor at the hospital and still couldn't get any answers. Thankfully, the dietitian through the medical supply company was able to talk me through the cleaning of the site and applying fresh bandages. We were just so scared. We had changed the gause 4 times due to the large amount of clear fluid that was leaking out.

 

Tuesday, March 26th

We had home visits with our EI dietitian and the dietitian from the medical supply company. We discussed the gradually increasing Ethan's feeds, keeping the site clean, feeding tube basics, etc. in addition to the leaking issue. Later in the afternoon the home health nurse came by and discussed some of the same things and talked about the leaking more. She was very concerned and I was grateful that she made a call to the GI for us, because I felt that when I had called I had a hard time explaining what was happening and why I was so concerned.

 

Wednesday, March 27th

The GI's nurse called and let us know that the GI wanted to see us. I took Ethan to the appointment and explained the large amount of clear fluid that was leaking from Ethan's site. The GI felt that much of the leaking was from Ethan's positioning and that we needed to try to keep him upright longer after his feedings. This is difficult since feeds aren't going all that well, since Ethan doesn't like his highchair (nothing new of course). The doctor also suggested that we cut back the water flushes a bit, to see if that would help.

 

Thursday, March 28th

The leaking was now formula, not clear! URG! Our EI feeding therapist came to our house and we discussed the change in Ethan's oral intake, and some of the challenges we are going to continue to have and those that will hopefully lessen over time as Ethan's body readjusts to getting the proper nutrition that he needs. She was also concerned about the amount of leaking.



 

Easter Weekend

Jeff had Good Friday off, and we spent the majority of the weekend as a family. On Sunday, Ethan and I stayed home - I didn't feel like a very full church nursery was a good place for Ethan with all that we are dealing with. We did have a nice Easter lunch with Jeff's parents' house though and Ethan got a new book and a cute bunny from his grandma and grandpa Marshall.

 



 

Monday, April 1st

Over the weekend Ethan's site began to change and enlarge. We were scared and so glad that we were getting to see our primary doc. We saw him and he agreed that the site didn't look right. I had talked with the GI's nurse that morning but was having a hard time explain my concerns. I was very glad that our primary agreed that something was wrong so he called the GI's office and spoke with the nurse who relayed our concern to the doctor. Before we had even left our primary doctor's office the GI's nurse called me to say that the GI would see us the next afternoon.

 

Tuesday, April 2nd

While Ethan and I were trying to get out the door to go pick up Jeff so we could all ride together to the GI appointment the nurse from our geneticist's office called to let us know that a few of the blood tests that were taken while Ethan was under sedation had come back. When I told her we were actually on our way up there to see the GI, she said she'd have a print out ready for us and she'd make sure she gave it to us and said hello when we got to there. So, Ethan and I picked Jeff up from work and we headed back to the GI. While at the gas station, filling up before getting on the interstate, I snuck in Ethan's 3:30pm feeding. I wouldn't call a tube feeding in the van (Jeff was driving) easy but it can be done and I will admit that at least this time it was far less messy than trying to spoon feed Ethan while the van was going down the road.

 

When we got to the clinic we checked in and were quickly called back. While they were getting Ethan's weight and height the geneticist's nurse stopped by and gave us the report. The GOOD NEWS is that Ethan's liver function tests have never been better! While it is true that often children with PBD-ZSD will have liver function tests that fluctuate, whenever something is better "for Ethan" and especially when something is closer to "normal" we celebrate! Many of the other tests will take weeks if not months to return.

 

Once he was weighed and measured we got to see the GI. It was our best visit with him to date. He agreed that the site did not appear normal and that it was indeed enlarging, although he wasn't sure why. He was glad to hear that the leaking had begun to slow, and he took that as a good sign -- that healing must be occurring on the inside, although the surface was not as it should be. He had the nurse swab it to check for infection, and told us that we were doing everything correct (keeping it clean, dry, etc.). He did not seem concerned about how much more of the tube was no on the outside of his body. He also spoke with us about the mickey button that Ethan would get in about 8 weeks, as long as everything gets straightened out and he heals properly. He also said he was going to speak with the pediatric surgeon the next day (we were the last appointment of the day) and see if we could get on the surgeon's schedule for a consult in case medical intervention was needed to "close" up the site/hole -- possibly via stitching.

 

Wednesday, April 3rd

Our furnace has been making some rather odd noises, so after Jeff talked to my Dad on the phone about it a few nights before, Jeff called and made an appointment for an HVAC company to come out and look at our furnace. From the conversation with my Dad we had some ideas about what might be wrong, etc. We were fortunate that Jeff's dad was able to come to the house to be with Ethan and I for the appointment with the HVAC company since Jeff was going to be at work. The short of it was that our 18+ year old furnace needed more repairs than it was really worth. BLAH! Not the news anyone wants to hear.

 

The home health nurse came by in the afternoon and was shocked to see Ethan's feeding tube site. She had not been here since in 8 days and could not believe the change and how much bigger the site was. She was glad to hear that the leaking had decreased, that we had been back to the GI, and that there were plans for us to see the surgeon if needed.

 

When Jeff got home we talked about the furnace situation and decided that we should just go ahead and replace it. It isn't fun, and we would have rather spent our tax refund on other things, but it needed to be done and we would rather not wait until it just gave out.

 

Thursday, April 4th

We had home visits with our EI feeding therapist and our EI dietitian today. Ethan did really well eating his soy yogurt for his feeding therapist, and we found out that Ethan had gained 6 oz. and grew 1/4 inch since the dietitian's last visit! Our dietitian has been working so hard for us! She has been fighting with our insurance, and writing letters, consulting with the supply company, speaking with the GI's office, etc. We are very thankful!

 

Friday, April 5th

The HVAC company came and installed our new furnace and air conditioner coil, and left with a check that was painful to write. We know that it is a good investment, needed to be done, and hopefully we will see at least a bit of a difference in our electric bill in the years to come.

 

Jeff's dad came by in the afternoon and helped keep Ethan busy and happy during his 3:30pm feed, and then Ethan and I headed outside for a walk. We only walked for about 20 minutes or so, but it was a nice little walk and he fell asleep in the stroller. I was thankful that Ethan remained sleeping while I transferred him to his crib. He needed a nap!

 

Tonight we wrapped Ethan's stomach in cling wrap and gave him a shower/bath. It didn't keep out all of the water, but did a good enough job, I guess. The use of cling wrap had been suggested by at least one of the nurses at the hospital. The joy on Ethan's face was worth the possible moisture --- we made sure we got the site dried off before getting him in his pajamas for bedtime. It took Ethan a few extra minutes to settle down for the night, but we were so happy that we haven't had to go driving for the last several nights. Since he no longer gets a bottle and besides getting a wipe down hasn't had a real shower/bath, everything about his nightly routine has been off since surgery. Thankfully, I think we are getting back into a routine, even if he does have to cry for about 5 minutes in his crib. It is still hard on Jeff and I to let him cry -- we don't let him go more than 10 minutes, because if he hasn't settled down by then, he isn't going to, but we know that he is tired and we really don't enjoy driving all over town at night just trying to get our little man asleep. We do it when we need to, but it doesn't me we like too.

 

Other things that have been going on over the last few weeks:

• We have been fighting insurance to cover Ethan's formula and feeding tube supplies. Our EI dietitian, primary doc, medical supply company, nurse at the GI's office, case coordinator at the hospital, etc. have all been part of the battle. It appear that we will have at least some real relief for at least the remainder of the year (since we have already met our deductibles and out of pocket max for the year). Our EI dietitian shared that our insurance said that they will cover the cost of his feeding tube supplies and 99 cans of formula a month (we haven't heard this from the medical supply company yet though). Once Ethan is up to his full daily goal, he will need around 120 cans a month (4 eight ounce cans a day will meet his current fluid and calorie needs) so while we will still be purchasing some formula on our own, we are very thankful that insurance is going to be covering just over 80% of the cost of his formula. Most insurance companies understand that formula for tube feedings is expensive and is a medical necessity, and most parents don't have to fight so hard to get things covered, so I am very very grateful to our team who went to bat for us and kept fighting to get these expenses and services covered. Thank you thank you!
• Three sets of very special and close friends of ours are having difficulties finalizing their adoptions. My heart aches for my friends and there is nothing besides praying that I or anyone can really do at the moment. Each situation is very difficult and unique, and I know I am very biased but I want to see these families officially united, ASAP! Each of these families love the Lord and are great parents who love the little ones who are already part of their families but aren't "officially" their's yet. Prayer greatly appreciated for my friends and for all families who are in the process of becoming forever families.
• Another little one with PBD-ZSD left the arms of her parent's for the arms of Jesus. She fought so hard but her little spirit was ready to be with the Lord. Please pray for comfort and peace for little Autumn's parents and for all parents who have lost a child.
• Other children and families in our group are experiencing difficult changes in their children and/or have children who are really struggling right now. One of Ethan's buddies had a feeding tube placed the same day as Ethan and has been having some complications as well, and two other of his little friends will be getting them soon -- so with all the difficulties that Ethan has been having I worry about his friends and hope and pray that they won't have any of these issues. 
• Ethan has been missing therapy because he isn't going to STEPS right now. We need to figure out what is going on with his site and get it healing properly before we go back. But, I hate that he is missing out on therapy. He is restless being with me all the time. This little boy loves people and loves "school" but I'm not willing to risk it yet. We have spoken with our EI Service Coordinator and we are going to make arrangements for at least a few of his therapists to see Ethan here at home for a while, which I think will be helpful.
• We are working on trying to get the word out about our 2nd Annual Pancakes for PBD-ZSD event that we are holding on Saturday, May 18th to raise money for our family and hopefully other families to be able to attend the 2013 GFPD Conference in Lincoln, NE this summer.
• There are lots of layoffs at Jeff's work. At this time we are thankful that he seems safe although there are lots of people, Jeff included, that will be "forced" to take nonpaid furlough days. We have been told to be prepared for up to 3 weeks. I know it is hard to complain about losing three weeks of income when many are losing their jobs completely, but to be honest, it is still going to be difficult for us (and I would think most people). We are praising and thanking the Lord for Jeff's job. Please pray for those who recently found out that they are going to be out of work soon. These layoffs are going to have a major impact on families throughout our community.
• The next few weeks shouldn't be quite as eventful but we still have lots of appointments coming up and we really need Ethan to heal so we can begin the next step -- changing the tube out for the mickey button, which should be an easy thing, but with Ethan, nothing is easy.

 

Thank you for your love, support and prayers. We are so thankful that there are so many people who care about Ethan and our family.