Blessed

Pat and I have been absolutely overwhelmed with all of the love and support we have received through Addy's journey but probably not as much as we have been the last week.
Addy's wall is covered in cards that have been sent, out blog posts have been read constantly, and our transplant coordinator said her phone has been ringing off the hook with people asking about what they can do for Addy. On top of that the students at River View donated 105 units of blood in Addy's honor yesterday, and they have organized a shirt selling fundraiser and are having a bake sale next week! I have always cherished living at River View because it seems that our motto is "It takes a village to raise a child" and that is so obvious. I hope that everyone who has done anything for us knows how much we appreciate everything. We have had numerous people tell us that they can't believe how we are dealing with everything. It is because God has put so many amazing people around us! Thank you to everyone!

If you are interested in buying a shirt, they are $10 and are blue with orange writing that say Addystrong on them (picture below). You can email your orders to Darcy Miller at darcy.miller@omeresa.net.

Tuesday

Not whole lot of new information to present today. The D10 fluid overnight helped stabilize Addy's glucose so that is good. We are also allowed to give her baby food again. Starting tomorrow, they are going to decrease the medicine she was put on for the fluid in her belly because her stomach is back down to her normal size and it isn't hard anymore.

Addy has the nursing staff wrapped around her finger! Last night she didn't really want to sleep so the nurse asked Pat if she could take her for a walk. She ended up spending quite a bit of time at the nurses' station. Then today when Pat and I went to get lunch Addy was crying so our nurse took her out to show to everyone. The nurse told us she just watched everyone and was very interested in what everyone had. She is quite curious!

We also took Addy down to the playroom today so she could get out of the room a little bit. It is nice because every wing has a playroom for the kids. We didn't stay for too awful long because she got tired and was ready for her afternoon nap, but it was nice to get her out of the room for a little while.

When we came back from lunch, the transplant surgeons were in the room. There are quite a few here at the hospital and the doctor on call we had never met so he came to talk to us. He told us a lot of what we already knew, he can't predict when a liver will become available, Addy is high on the list, and it is very frustrating to wait. He did talk to us about a domino transplant (Here is an article about the first one they did). For a Cliff Notes version, what they would do is someone with the disease called Maple Syrup Urine Disease (MSUD) would get a new liver and then Addy would receive the MSUD liver. The MSUD would not get transferred to Addy.

The infectious disease doctor was just in as well and he says there is no reason, that he can tell, that she can't get the transplant. The reason he had to come was because of her rhinovirus. With the transplant and the anti-rejection medication, Addy's immune system will be very low after the surgery. If she were to have the transplant and have a virus, that could cause extra complications. However now that the rhinovirus is gone, he thinks it should be ok.

Thank you so much for all the thoughts, prayers and support! We can't believe how many of you are reading the blog and we are glad that we can keep you all informed. Please keep the prayers coming and hopefully we will get good news soon!

Monday

The GI doctors came in today and said that Addy's bilirubin has gone up and is now at 29. Her INR is staying steady and is at 7.5 but that is still very high. Her blood sugar is also dropping overnight even when we are feeding her every three hours, so tonight they will give her D 10 through her IV to help that. They scored her again on UNOS and her score is up to 54. Our transplant coordinator said that is very high and the phone should ring soon with a donor. However because there are no gurantees, they suggested we start seeing if anyone is interested in being a live donor. We realize that we have a lot of people willing to donate but we hate to ask anyone to go through it. In others minds, it may not seem like a big favor, but to us it is. We appreciate all of the offers but we are having a hard time with it. Everyone has other people depending on them and we don't want to compromise that or take them away from their families. We hope everyone understands that. Our transplant coordinator has given us the number for the live donor coordinator for people to call and get the process underway. It would start with a blood draw to make sure the blood type matches. Addy is O, so either O+ or O- will work, or if you have A there is something called A2 (what they tested Pat and I for) that will also work. If the blood type matches, the next thing would be a 2 1/2 - 3 day evaluation and more testing here in Pittsburgh. Another stipulation is the donor cannot be over 55 years old.

On top of all this as well Pat woke up this morning with extreme back pain. Two weeks ago he had this as well and we went to the emergency room and found he had a kidney stone. He never thought he passed it but the pain subsided so he thought maybe it had gotten small enough, he didn't realize it was passed. That wasn't the case. After getting sick from the pain, he drove himself to the hospital where they confirmed he still had the kidney stone. They pumped him full of IV fluids until it was in his bladder. They sent him back with pain medicine and hopefully it will pass and he will feel better.

Morning Update

We just met with the GI doctors this morning. Dr. Schieder (hepatology doctor) doesn't feel safe sending Addy home with such a high INR. Her number has gone down a little-to7.6- but that is still not good.

Today she will need to get a blood transfusion because they have taken so much blood for all of her tests. She is going to get 10/ kilo over 4 hours. After tomorrow morning, they won't draw blood everyday. They also agreed to not do as many glucose tests since her numbers have been pretty steady.

She is almost completely off the IV fluids. She is only getting 5 cc's an hour and that is just to keep the PICC line open since she is getting medicine every 6 hours. The doctor thought we could decrease her Albumin since her numbers are getting better. That means she will get that medicine every 12 hours rather than every 6.

In other news, Pat and I visited the library yesterday. It was a pretty nice library and had movies as well. The set up in this room makes it a little hard for parents to watch TV do we got a movie to watch on the computer. I had already started Jodi Picoult's Between the Lines so I didn't get a new book, but found plenty I will check out if we are here longer. Pat decided to start the Hunger Game series. Bob and Denise (Pat's parents) came to visit but Denise wasn't able to come back and see Addy since she is still getting over a sinus infection. The doctors don't want anyone with cold like symptoms around Addy right now. Darcy and Chad also came and visited for a little bit.

* UPDATE * Our nurse just came in and said Addy's respiratory panel came back and it was negative so that means no more rhinovirus and we can take her to the playroom so she can  move around a little bit!

A little good with the bad

Let's start with the bad news...

Last night our nurse woke Pat and I up to let us know they wanted to do an x-ray of Addy's chest. Her heart rhythms were showing PVC's and they were worried this was caused by the PICC line that was put in. When the doctors came in today they believe it is just because the PICC line is resting on the wrong area so the IV team will be here later to pull it out just a little and see if that resolves the problem.

When the attending doctor, Dr. Kahn (our third Dr. Kahn along our journey) came in for the morning report she told us that Addy's INR (blood clotting) score was up to 8. It had been 7 when we first arrived but then dropped down to 6 yesterday. A normal INR is 0.9-1.3.

Another concern had been her glucose. It was extremely low when we got here (around the 20's) so they have been giving her glucose every 4 hours. This morning it was 80, which is a pretty good level.

There is still some ammonia in her body but the doctors think that is just from her previous GI bleed she had. They are going to put her on Lactulose to help that.

The biggest concern is getting her over the rhinovirus. If a liver would become available, it is a major risk to do the surgery while she has the infection. The anti-rejection drugs that she would be on would cause her immune system to go way down so any infection is detrimental to her.

On to the good news....

They have finally agreed to let her eat! Pat was giving Addy some Pedialite when the doctor was in and she watched how mad Addy got when she was done. She agreed that if Addy kept down the three ounces she took in the morning, the next time she got hungry she could have Pergestamil. So far that has gone really well. She is only taking 4 ounces at a time for now and they have decreased her IV fluid to almost nothing.

Some more good news was that the blood cultures they took yesterday haven't shown anything, so while the bacterial infection may not be completely gone, they are on the right track. We are still waiting on the nasal swab they took to check for the rhinovirus.

We also found out yesterday that Pat and I could be donors for Addy. There is a blood type called A2, which most people don't know because it takes a special screening to find it. Pat and I both got tested yesterday but have not heard the results yet. 

Per some requests here are some pictures of Addy from today. 

Her PICC line

Cuddling with Piglet

Piglet has an IV just like her

Her favorite sleeping position

Fever

On February 6th I took Addy for her bi-weekly check up at Columbus. She had started having a runny nose the weekend before and Wednesday morning developed a fever. When we got to Columbus she was up to 101 degrees. After seeing Dr. Kahn and Dr. Potter, they thought it would be best if they admitted Addy so they could determine what was causing the fever. They gave Addy a dose of Motrin to help with the fever and by the time we were in our room, her fever was gone. They still ran blood tests and we had to wait 24 hours for the blood cultures to grow something. They found that it was rhinovirus, the common cold. Since it is a virus, there was nothing to do for it other than let it run its course. The good news was we only had to stay in the hospital one night.

She continued to have the cold symptoms for two weeks and then a little a week over on the 16th, she started a fever. The hospital doesn't consider it a fever until it reaches 100.4 and she was only at 99. She also had teeth coming in so we just thought that was the culprit. Monday the 18th though Addy ran a fever throughout the night and at one time it reached 102. After talking to Dr. Kahn, she asked us to come to the ER at Children's. At 4:00 we left for the hospital. They drew blood cultures right away and then decided to admit us once again. They went ahead and put her on antibiotics in case it was a bacterial infection. I headed home Monday night so that I could go to work and Pat and Addy stayed at the hospital. It was the first night I had ever spent a night away from Addy and I was heartbroken, but I knew Pat  would take good care of her.

Wednesday they did a CT scan of her stomach because it had become very hard. It showed that there was fluid in her belly and also her intestinal walls were swollen. They stopped feeding her and she was only hooked up to IV fluid. They hoped this would help push out the fluid quickly.

When Addy was not responding to what Columbus was trying, they transported her to Pittsburgh by ambulance Thursday night. She was not a happy camper because she was so hungry. Friday morning the doctors came in to talk to us and wanted to do an ultrasound of her abdomen and also put in a PICC line so that her antibiotics and blood draws could be done through that rather than her hand IV's.

Back to Where it All Started

I am starting this blog for a couple reasons. First, we are hoping this is a way that everyone can stay informed. We have been absolutely overwhelmed with the love, support and concern everyone has shown through out Addy's life. She is one blessed little girl! Second, this will be a way for us to hopefully remember everything. Pat and I have learned more medical jargon than either of us ever cared to learn but there are still some things the doctors say that we aren't sure about, so it is nice to have it all recorded. Third, we hope that this may help another family going through this someday. When we first found out Addy had to have a transplant, a friend that I went to  high school with send me a link to a blog or someone she knew. While it was a little scary to read, it was also comforting to know what to expect.

For those that don't know all that Addy has gone through, I am going to give you a Cliff Notes version (or what we have to tell the doctors every time we have been admitted). I had a pretty easy pregnancy. I wasn't sick very much and wasn't overly uncomfortable. Delivery was normal and pretty fast (thankfully!). Her Apgar score was normal and her bilirubin was a little high, but nothing to be concerned about. We got to go home after two nights in the hospital. At her one week appointment her bilirubin still was a little elevated, but the doctors didn't feel that she needed the photo-therapy. We were told since she was nursing, she may be jaundiced a little more than a formula fed baby. At seven weeks I took her back to the doctor, concerned about her color. He ordered blood work and we went home. Soon I got a call from Pat that scared me more than I had ever been scared before. He told me the doctor called him and we needed to get to Children's as soon as we could. When we got there we waited in the ER as they decided what needed to be done. Her veins were so small that they had a hard time getting an IV in and had to poke her three times before they finally called someone else, who was able to get it on the first try. It was awful to watch her go through that. Next they wanted an ultrasound of her stomach. They did one but said it wasn't clear enough because she had just eaten. We had to wait until she hadn't eaten for four hours before we could do another one. If you know Addy, you know this wasn't easy. She was furious! Pat and I took turns walking around this tiny room trying to console her. Finally she fell asleep in Pat's arms. When we went to get the ultrasound Addy squirmed all over the table and just screamed, she was so hungry. This of course made the whole process a lot longer, but they finally got what they needed. After that we went back to the ER room and were told shortly after that we were going to be admitted. From the ultrasound and the blood work there was something wrong with her liver but they needed to do more testing.

The biggest concern that the doctors had at the beginning was that Addy had biliary atresia. Pretty much this is a blockage or disconnect from the liver to other organs. To check for this we had to do a hydascan but she had to be on special medicine for five days. They also started her on other medicine, which she is still currently on. She take Ursidiol, to help with the bile caused by the jaundice, AquaADEKS, a multi-vitamin, extra Vitamin D, and extra Vitamin K. They also started her on Pergestimil formula to supplement with nursing. Pat went home after the 4th day to go back to work and on the 5th day we did the hydascan and found it wasn't biliary atresia. However, they didn't know what it was. They allowed us to go home and we scheduled follow up appointments.

A little over a month later at an appointment, the doctors were concerned that she was not gaining any weight. They hospitalized us again so they could monitor her feeding and weight gain. They adjusted her calorie intake with the formula and told me I could no longer nurse. I was absolutely heart broken and had a really hard time with that. Again after 5 days, they were comfortable releasing us and letting us go home.

Another month passed and we were back at the doctor's office. They now wanted to do a liver biopsy because they told us that would be the most sure fire way to diagnose Addy. The doctors also wanted to do it now because, at the time, there was a world class hematologist (blood doctor) on service at the hospital. This was such a big deal because Addy's blood clotting numbers were really high, which means she was more prone to bleeding and he could monitor her to make sure she didn't bleed during or after the procedure. We decided to go ahead with it even though we had put it off because we didn't want her to have surgery if there were other ways to figuring out what was wrong. We were in the hospital 2 days before the actual biospy because the hematologist wanted to give her Vitamin K through an IV before the procedure to help with the bleeding. Finally they did the liver biopsy. She had to be put under so that was unnerving for Pat and I. When she was in the operating room, Pat and I spent our time playing Sonic the Hedgehog on the waiting room's Game Cube. The procedure didn't take longer than 20 minutes and when we went back to see Addy she was smiling. Once the doctors were happy with her levels we were released which was a day or two later.

Two week later we went back to the clinic to find out the results of the biopsy. We met with Dr. Potter who is the liver specialist at Children's in Columbus. She told us that there was scarred tissue in her liver but they didn't know why or what caused it, but it looked like she would need a transplant. I was shocked and didn't know what to say or do. Thankfully my dad had gone to the appointment with me because Pat had to work. After the initial shock wore off we realized that whatever we needed to do to get Addy healthy was what we were going to do.

To start the transplant process we were sent to Children's Hospital in Pittsburgh. The screening process was a three day long process where we met with a variety of doctors and other specialists. The worst of these was an education session where we were told all that Addy would go through if she had the transplant. It was terrifying. Looking back it is nice to know everything, but at the time we were both completely against doing the transplant. We didn't stay in the hospital for the screening process so we were able to explore Pittsburgh a little. While the traffic makes no sense, we found plenty to take up our time. Once we were done, we were told the whole team would meet on Friday and we may know something right after that or with it being right before the holidays it may not be till after the New Year.

We didn't have to wait long because they called us that Friday. Dr. Schnieder, the hepatologist at Pittsburgh, called while I was at our school Christmas Dinner. He told me that she was a candidate for transplant and that we should consider putting her on the UNOS (United Network for Organ Sharing) list. We would have her blood drawn periodically and that would determine her PELD (Pediatric End Stage Liver Disease) score.

This was all very overwhelming but like I have said, if it wasn't for the amazing support from our family, friends, co-workers and everyone else we would never have been able to make it through.