The Big Day

May 10, 2016. A day that we waited for anxiously, nervously, and feared came and went as fast as you can say transplant. 

Me and Philip headed in to the hospital on Monday night to get settled in and have blood work drawn and make sure all was set for the next morning. Philip made himself right at home but he kept saying I want something. When Dr. Chu(his hepatologist) came in, she heard him say that so both she and I asked him what he wanted and he decided on a muffin. A blueberry and chocolate chip to be exact. I told him I couldn't promise I could find one so Dr. Chu said she would ask the kitchen. Five minutes later she came back in the room with a box of muffins from Dunkin Donuts! It made his night! 

We were escorted early the next morning to a holding area where we met up with Dan. It's hard to explain the depth of emotions and anxiety that were going on. Philip fell asleep so he was the only one who was relaxed! There were tons of people asking different questions and saying how amazing what Dan was doing. So we would get very emotional and start crying, pull it together, then Someone else would start saying all these wonderful things and we'd lose it again. 

Then, before I knew it, with two swift kisses, both were being wheeled off into the unknown. 

Dan's coordinator came out a short while later around 8:30 to say they were both sleeping and that Dan had two requests right before he went under for when it was over..."warm food and back rubs". 

Ok, I'll give you "warm" food not "hot"! We were laughing so hard at that. And before I knew it she texted at 11:30 saying Dans piece was almost out! Then around 12, she came out and said Dan would be done in a half hour and they were ready to take Philip's liver out. 

1:30 came and his surgeon came out to say everything went well during surgery and we would likely be able to see Dan in about 2 hours. 

So I ran up to see Dan who looked good. And wouldn't you know, Philips surgeon came while I was up. I missed him by two minutes! He spoke with my sister and said everything went well. His liver was functioning, and he didn't need any blood products. He said it was great timing and that his liver was pretty cirrhotic. And it was going to happen sooner or later and better to do it now while he was fairly healthy and before he went over a cliff so to speak. My sister started crying so I was like why are you crying??!! Then 5 minutes later, it hit me and I was sobbing with my head in my hands in the middle of the waiting room. So much tension released at that minute. I ended up having a headache for three days straight due to all the emotions that day I believe. 

Then it was time to see him. This was a huge fear of mine! We spoke about how he would look very puffy like a Michelin baby and have the breathing tube so  I walked down the hall with clenched teeth waiting to see my puffy baby and as I turned into the room, I saw my sweet sleeping boy and he looked so good. I couldn't believe it. I yelled out "He looks so good!" He wasn't very puffy, he looked healthy, just asleep with a lot of wires hanging off of him. He was still intubated so it was really just sitting and watching him that night and the next day they started to wean him off the sedation so they could take the breathing tube out. 

Which they did around 5:45 on Wednesday night. The first word out of his mouth was scwatch, then I want my baba in a little groggy tiny voice. My heart sank instantly. Isn't this part of the reason we did the surgery to stop the itching and that's what he says first??!! So the itching was not only not gone but it got worse as the night went on. He was scratching like crazy!! I fell asleep for a few minutes and woke up to two nurses scuffling in the room and him crying so I put my glasses on to see him looking at me and he is covered in blood. All over his face and hair like the movie Carrie! I jumped up and they said he's fine mom he just pulled out his "A" line which is a catheter inserted into an artery so they could monitor blood pressure in real time during surgery and then draw blood after. 

So they had to bathe him, change bedding and all of his dressings because he was a mess. I'm surprised my reaction wasn't a little stronger but I had a feeling he would pull something important out even before surgery. So I felt I had to sit up and stare at him the whole night to make sure he didn't get to his central line which was in his neck. The nurse found these cute little mittens or more like boxing gloves for him to wear so he wouldn't get anything else.  

The next day he got to drink water and then eat some jello. His nurse Glynda was amazing. She was so wonderful with him. He didn't like her but she would get him out of bed, go for a ride in the stroller, clean him up, and make him walk.  

Dan was finally feeling well enough to visit the next day. He had a lot of pain and got tired really fast so it was a quick visit. 

By Friday he was allowed to eat, went for a long walk and even got to have a lollipop! And we were moved to a regular floor. 

Over the weekend he started perking up a bit here and there, eating more and being himself. Mostly he wants constant attention and is very demanding! Lol

Everyone keeps saying he looks so good and did this kid even have anything done?! And wouldn't you know his itching has basically disappeared! I haven't heard the word scwatch in 3 days! I'm still crossing my fingers it stays like this!!

Danny came to visit today and that was awesome. He was very nervous at first and didn't want to come in the room because he had to wear a mask to come in. So he hung out in the hall for a little bit then he slowly became comfortable and was talking to Philip. It was so nice to get a huge hug from him.

Unfortunately both Dan and Philip had some jumps in some liver numbers so Dan was not allowed to be discharged today. Philip had to get an ultrasound to to rule out vascular issues and that was found to be ok. We are just waiting to hear word on both so please keep saying some prayers for us. I really need them right now to keep me strong for both my boys. 

Old Worries, New Worries

I just put the boys to bed, and me and Dan were having our usual powwow about how we can't believe its so close. (I'm even hesitant to write anything because Ive been so paranoid someone will get sick beforehand, I can't keep enough anti bacterial in the house or on me. I'm driving everyone insane. )Anyway, back to the story.  So Dan said this is the last night at home before it, and it hit me. I could literally feel a sucker punch in my gut. I just put Philip in his little Thomas bed and it will be the last time pre-transplant. Next time we will spend the night at home, it will be a completely different ball game. Old worries will hopefully vanish but new worries will take their place. And to be honest I am terrified of the new worries, I'm comfortable in my old worries, I know what to expect and what to look for. But with the new worries I know he will have such a better life, and that makes all the worries in the world worth it.
It's a change we welcome as His itching has gotten even worse over the last couple of weeks and we can't wait to give him a chance to be itch free.
I Can't wait to say Bye Bye Biliary Atresia! And all the nastiness that comes with it.
At the same time, I find myself a teeny, tiny little bit sad. I've grown to love his little belly over the last almost three years. A little rub or pat here and there is one of my favorite things to do.

His scabs have been a part of him for so long I've grown to love them when most people look at them in disgust. Him saying "Scwatch" too many times a day to count is such a part of our daily routine I can't imagine life without it.

 But I can't imagine him living like this even more than that. And I am so excited that he will get the chance to hopefully experience that in such a short time thanks to Dan.
And speaking of Dan, how can I even put into words what he will be doing in a couple of days. I know there was never even a second thought to help Philip. He is amazing. I can't thank him enough or tell him how much I appreciate him. I know it is going to be a little rough in the beginning but he has held me up through the last few years so I know this will be nothing for him to get through. I usually rely on him for stability through these things and As crazy as I've made him over the last couple of days, I promise I will be strong enough for all of us.
And Danny, is the best big brother ever. I know he's scared  and he doesn't want us to be away for a while but he just goes with the flow and does whatever we need him to do. He's is such a great helper too. I hope knows how much we love him and what an important part of our family he is.

Thank you to everyone for all the continued support and prayers. I believe they have worked thus far so please keep us in your thoughts on Tuesday. I will try to update as I can.

Things I've learned while raising a kid with Biliary Atresia

Transplant is about a week away and I find myself reflecting on the last couple of years and what we've been through. I've been very emotional and anxious just waiting for the day to arrive. The blog has been a place I can come and let out my feelings and hopefully share my experience with others who may be in the same situation, but I can't really find the words to share with you how I'm feeling right now and I'm not sure there are any that are appropriate. Instead I am going to write a little about what I've learned over the last couple of years living with a kid with Biliary Atresia.

Not in any particular order.

1. Biliary Atresia Sucks!

         Not much more to add to that.

2. Google is your worst enemy!

         From the minute you hear those words Biliary Atresia, you will google the shit out of it.

And with all the medical pages you will happen to find every worst case scenario story with it. Websites, blogs, Facebook pages and groups, and the more you read the more you will drive yourself crazy. I took advice from our GI when she heard I was googling and joining groups on line. She said please stop! You will mostly find stories of the sickest of the sick and worst case scenarios so please try to limit yourself. Don't get me wrong, these pages are full of the most amazing kids and parents fighting to have a normal life but I found myself obsessing over them thinking all of these things will happen to Philip and becoming depressed. So, I limited myself and I joined only one group on Facebook and it is a great place to ask questions and get support from people in similar situations. But I don't dig anymore than that. I know my limits and I just mentally can't handle it. 

3. There really is no rhyme or reason to the   disease. Every kid is different.

         There really isn't. One kid may have the Kasai and never have a problem till they are 18 years old. Another might have the Kasai and it will fail immediately, making the child need a liver transplant quickly. Some kids like Philip's Kasai works ok but their liver sustains damage over time causing other issues like portal hypertension and GI bleeds. Some kids just itch. Some need feeding tubes, some don't. Some have major vitamin deficiencies, some don't. And just because it happens to one kid, it doesn't mean it will happen to yours. Who knows why it manifests in such different ways. Just focus on what might or might not happen to your kid, just take it day by day and deal with the next issue as it comes. 

4. While your world feels like it is crumbling, life goes on for everyone else.

        From the minute Philip was diagnosed, I felt like the world was crumbling around me. All my hopes and dreams for our little family of four were forever changed. Yet everyone around me was still living their lives like normal. Going to work, going on vacation, doing things that I had to put on hold for a little bit. I wanted to yell at everyone and make them feel the pain I was feeling. Don't you know what my newborn son is going through and you are just standing there like everything is fine!!

Looking back, I'm not sure what else I expected. Life does go on and it eventually did for us as well. Back to work, activities, and even a little weekend vacation thrown in. You'll find that some people might avoid you or not know why to say, friends you thought would come visit the hospital might not. Don't take it personal. They're probably uncomfortable and don't know how to deal with it, and that's ok.  I don't hold it against them. Heck, I don't know what to say sometimes when someone is going through something difficult. 

5. Trust your gut!

         If you feel something isn't right with your kid, trust your gut and bring it up to the doctor. Most of the time you will probably be right.

6. Speak up!

         This was a hard one for me. I am typically a shy person when it comes to asking questions or confronting someone. If you're not sure of something or something doesn't seem right, whether it be medications, hospital stays, anything, please speak up!! If you're not comfortable with your childs care, speak up! In hospitals there are nurse managers, patient advocates, a lot of resources to use if you're not comfortable with a situation. Ex. We always have a bad experience when residents try to take blood from Philip, so now I speak up and request transport or phlebotomy to do it. They may think Im being pain, but I am saving my son from undue pain.

7. Be Nice 

        I know this sounds obvious, but when you are in the moment and frustrated because your child has been crying for 2 hours and they can't eat, or when I get another hospital bill for $4,536 when the same procedure was covered last time in full, it is easy to snap at the person you are speaking with. But I have found when I am super nice on the phone with billing issues they are nicer in return and willing to help a lot more.  I'm guilty of it but have learned to just smile through it all. Even a simple chat with housekeeping can make your day brighter.  

8. The nurses are your lifeline

You will most likely see your nurse more than anyone in the hospital. They bring the food, the medicine and basically everything you need. Appreciate them and cut them a little slack if they take a little longer bringing you something you requested, they are often over worked and under appreciated. 

9. Make time for yourself (and significant other) I know we feel like we need to be there 24/7 for our little BA fighters, but we need time too. Don't forget to take care of yourself. Go get a pedicure, go to a movie, get lunch with a friend. Get out and take a break. It's OK. And don't forget about you're significant other too. Go on a date night and talk. Don't stop communicating with each other!!

10. It's OK to laugh

This is the most important to me. Yes things might suck and you feel like you want to throat punch someone but its ok to smile and laugh even though your child is sick. I completely feel that they feed off of our energy and if they see us laughing and smiling, then they will know everything will be ok. 

Dan and I try to find the humor in most of our situations. They might be politically incorrect and just wrong on so many levels but if something makes us laugh and gets us through a rough time and lets us forget about all the crap going on, then I'll laugh at it.

And just like that...

A date is set.

May 10, 2016. Seems like a pretty normal date, and to anyone else it will still remain just another day in the life. Another day to go to work, or a baseball game or to eat pizza...

But now, this date for me, will mean something. A re-birthday of sorts, a celebration, the day my husband gave my son a chance to live a better life. The day I watch not only one but two people I love dearly be wheeled/walked away to the operating room. It's really a crazy feeling of excitement, fear, and anxiety all at once. Excited that Philip will be on a road to good health, fear that all may not go as planned, and anxiety, well, you name it and I'm anxious about it. 

If all goes as planned Philip will have about a 2 week stay and Dan will have about a week stay at the hospital. Both livers should start growing back right away with Dan's being pretty much 80-90% regenerated by 6weeks post surgery. His body will use all of it's energy trying to regenerate it so we anticipate him being very tired after surgery. He also needs to take it easy for several weeks. They will not clear him to go back to work for 12 weeks and then they will see how he is doing to put him back full duty or not. I will take several weeks of as well to care for them and to get Philip's medications down pat and full understand them. They said he will probably be on about 9 medications coming home from the hospital. 

It seems as if time is moving faster now and this now special, precious date is just around the corner. We have a lot of loose ends to tie up before surgery so days are full of calls to insurance, doctors and other various entities. Both Philip and Dan go for pre surgical testing May 2.

A lot of people have asked how I feel about a date being set and basically My whole mind and body is just a constant buzz, often accompanied by chest pains, heartburn and the BG's (bubble guts as Dan affectionately calls it).  But generally, all I can come up with and say is shit's getting real...

The Signs

Since we started this journey with Philip being diagnosed with Biliary Atresia, there have always been signs along the way,  assuring me that someone was watching over us.

When he was in the hospital with a liver infection, a wing mark appeared on his window...

After my Nana passed away, I felt it even more so. Birds remind me of her, She always loved birds, feeding them out of the back door of the house, even hearing stories from when she worked the bird show at the World's Fair. So when Philip needed the blood transfusion, when a feather appeared out of nowhere, floating in the air, it really hugged my heart to know she was there watching over us.

Then yesterday morning, I wasn't too surprised that while me and Dan were talking about waiting to hear about him being a match that we saw two Cardinals sitting outside our house in a tree in the front yard.

There's a saying that Cardinals appear when angels are near. They are also known to symbolize health, hope, rejuvenation, celebration and joy.
So it seems they had a double meaning as it looks like Dan is a MATCH!!!
They calculated and they would only need to take 13% of Dans Liver to give to Philip.
Dan just has to be cleared by cardiology, which he has an appointment tomorrow and Philip has a CT scan next Monday. Then just have to tie up some loose ends and hopefully we can schedule the transplant sometime soon.
In the meantime, I'll keep looking for the signs!

A New Journey

It's early, still dark outside, as we wake the kids up, gather all of their stuff and pack the car to drop them off at my moms before we get on the road for a two day visit with Mt. Sinai. In the car we talk a little about Philip and Danny, our nerves, (OK my nerves) and this new journey we're about to start. But it's mostly silent, both of us knowing the importance of the next two days and how the results will impact our lives, and not knowing what the many appointments ahead would hold. We just know that while Philip is still fairly healthy, he still has a life threatening illness. His PELD score does not reflect the risk he faces waiting on the list. And with his low score he can wait years to be called for a cadaveric liver. Do we wait for the call and risk him having an even worse GI bleed and get sicker or do we try to be donors ourselves and start to hopefully move forward with good health? The itching has gone from bad to worse. So it really was a no brainer for us. Move forward with Living Donation. We decided to have Dan try first because Philip is very attached to me and we think he may fare better in recovery if I am with him.

Living Donation is handled by a completely separate team than Philip's. So Wednesday started by meeting with the Transplant Nurse Coordinator, followed by Social Work, then a Nutritionist, and Financial Coordinator. And Lastly 16 vials of bloodwork. They test for everything. Their main concern is the health of the donor. If there is any little thing that can increase the risk of complications, they will rule you out as a donor. The team we met with seems very caring and attentive to all our needs. They are very strict about confidentially so I was not allowed in some of the appointments like Social Work. They want to make sure the donor is not being pressured into donating and that Philip is not paying him off in matchbox cars...lol
Thursday morning started with a chest X-ray, then MRI. After that we met with the surgeon who would do Dan's surgery, then we met with the hepatologist who will follow the donors care after the transplant. Finally Dan had to meet with the psychiatrist.
We will know on Monday if Dan is a match. If not, I will plan to go ahead and be tested next.

There was so much information to take in it was head spinning. They really want to hit home that there is no medical benefit to the donor. You could in fact be putting yourself in a position of worsening your health. Being that Dan's job requires physical activity they said he could potentially be out of work for 3 months and then go back slowly to full activity at work. Your whole body's energy will be going to regenerate the liver so the donor will be very tired at first, not eating the same, pain. They said people can actually feel their liver growing back, with little twinges here and there. It's pretty amazing stuff, but is pretty terrifying at the same time.
For me at least. Dan seems to be fine with the whole thing. A little nervous but not a basket case like me. What if we go through with the transplant and Philip gets worse? What if Dan has a complication? What is Philip has a complication? How will I deal with the 2 of them in the hospital? How is Danny going to deal with having his whole family gone for a couple of weeks? Not just one of us but ALL of us? Poor little guy. I know he will be with family and be fine but it has to have some effect on him. I am going to contact social work to see if they have any resources for him.
Just endless questions in my head. But we've faced the last 2 years head on, what choice do you have really when faced with a situation like ours, and we'll keep facing it head on, whatever comes. Keep  smiling and trying to find the humor in things. And knowing all of the support we have from family and friends will carry us through any obstacle we face.

This is a picture Dan saw as soon as he walked in the office. Could it be a sign...?

The Inevitable and the Unknown.

I'm not really sure where to start this post but the last few weeks I've been overrun with so many emotions and feelings, it's taken me a while to actually want to put it all down in writing.

So many feelings...

Happy, sadness, fear, relief, anxiety, exhaustion, loneliness, paranoia, confusion just to name a few. Sometimes just one at a time but then a wave of all at once, the weight crushing at times, paralyzing me. Feeling the constant buzz in my chest and butterflies in my stomach. But, they have finally settled a little and I am able to move forward a bit onto a road we've known we'd likely have to take but when Philip's health stabilized for so long, we prayed and hoped it wouldn't happen for a while or that he may be one of the lucky ones. 

But after the bleed he had, we met with Mt. Sinai last week and transplant is inevitable. He is now actively listed for transplant. Philip is in a bit of a unique situation as his liver is still doing its job and according to the numbers of his bloodwork he is still very low on the list. This means he would not likely be called anytime soon for a cadaver liver.  It's not impossible but again, not likely. He unfortunately is dealing with issues having to do with the portal hypertension and these GI bleeds which are a result of the existing and on going damage to his liver. Every day he is at risk of a life threatening bleed. There is no way to predict if and when it will happen. Along with his severe itching that wakes him every couple of hours at night which has gotten worse, They have suggested we move forward with a living donor since we had expressed interest in that once before. So we asked if doing it in a month would be too soon because we don't want to rush into anything if he is too healthy; and the doctor said, listen, if we had known he would have had this past bleed, they would have suggested to do it a couple of months prior. So in their opinion doing it in say a month is not too soon. So, we went home with some literature to read about living donation and it is some scary stuff.

There are major risks with such a surgery and so many possible outcomes it is literally terrifying. The fear of the unknown is so crippling. Not only do you have to worry about Philip accepting the liver and rejection and infection afterwards but then you have to worry about complications with the donor like vascular issues and bile leaks and them possibly needing A transplant themselves and even death. There are so many possible outcomes,  It's makes your head spin. 

I was speaking with Philips pediatrician about my fears and she put me at ease a little. She said hey, do the testing, take it day by day. You guys may not even be matches and then it is out of our hands anyway. And she is right, don't worry until there's something to worry about, right?(insert nervous laugh here)

After we met with transplant, Philip was scheduled for an endoscopy to check for more varices which they did find. They had to inject two, one of which started to bleed when injected which has never happened before. The injection in theory is supposed to collapse the vein and stop it  from bleeding. She had to apply pressure with the scope and it did stop bleeding. They checked his blood count just in case and it was good. She feels that maybe it bled because the pressure from the portal hypertension is just that high in the varices. So we stayed overnight again but Philip took it like a champ this time. We got to go to the playroom to keep his mind off of not drinking or eating. He loved it. He talked to everyone and asked if they could help him play. He really has such an amazing personality and it's so fun to watch him interact with everyone.

Through all of the worries, we have decided to try to move forward for living donation. We're living in fear everyday that every time he coughs that it will be blood or every poop will be black. And the poor kid doesn't sleep anymore really because of the itching (nor do we lol) We've decided that it's enough. So Dan has decided he would like to try to be Philips donor and while we wait to hear back from living donation, We will take every day, minute by minute, and pray that he can hold off bleeding or having any other major issues until we can get him transplanted. 

 Yay Play Room!

                   I don't sleep at night :(

  Scratch Wounds

Yay, My Own room with cool blue lighting!

         Yay, Donut Ball!