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!

Guilt and Adjustments

As parents we've all had those moments (I think) when you WANT your child to do something and they refuse to do it.
Put their diaper on.
Brush their teeth.
Take a bath.
Eat their dinner.
Go to bed...
But what if you NEEDED them do it? What if their health depended on it? Or if they didn't do it, the consequence would be much worse than the actual doing of the thing? Or what if it was for selfish reasons or a combination of both?
When it comes to the point of full out screaming and kicking and crying, do you use any and everything in your arsenal to get it done?
And although I am scared to admit it and am risking being criticized, yes, I have. And this is where the guilt comes in.
On our last day at the hospital, we were awaiting discharge and were informed that Philip's potassium was low and they wanted him to take a supplement before we could leave. Sure, no problem. So we waited, and waited and waited for 3 hours for the supplement to come up from the pharmacy. After 6 days in the hospital, we as well as Philip were drained. Physically, mentally and emotionally. They brought the supplement in and it wasn't just a teaspoon full but a giant syringe and then some. We tried to give Philip the syringe and he flat out refused to take it. Like gritting teeth, hand over mouth no way am I taking this shit refusal. I didn't blame him, after all the stuff he dealt with over the week. After a few squirts and his gurgling it out of his mouth we decided to take another approach. We put it in some apple juice so it would mask the flavor. One sip and he refused again. I didn't want to push too much but we were so close to him going home, and he needed to take it for us to go. His IV was already taken out (which should have waited till after he took this but that's neither here nor there at this point) The nurse said she had to get the resident and they would have to put the IV back in for him to get it or they would have to take a blood test for the potassium again and hope it went up. I knew that him being stuck again would hurt far worse than him drinking this stuff.  It was not an option in my book. He had been stuck well over a dozen times while we were there and it was getting hard to find places to stick. He had bruises and hematoma all over his hands, arms and feet. So we started bribing him with toys, cars, food, other drinks. Anything we could think of. Still refused. He even said he was staying there now and he didn't want to go home. So out of frustration in the moment, we said that he could stay but mom and dad had to go home. He immediately said OK I'll drink it, no crying or anything. It took him 1/2 hour to do it but he got it done with huge high fives and dances after every sip. We were out of there within 10 minutes of him drinking and he was asking to go in the big bathroom like a big boy, as if nothing ever happened. I immediately felt so guilty. I would never want him to think I would leave him there all alone. I hope that the saying actions speak louder than words is true because then he would see that I would never leave him and didn't leave him this whole stay. I am so mad at myself for saying that. Was I being selfish because I just wanted to get home too? The longer we stay the more germs he's susceptible to get and I wanted to get out! But at the same time, I feel like it was the only thing that I could do at that moment to save him from more pain of being held down by 4 people and being stuck. I'll just chalk this one up to a parenting fail and try to come up with a better strategy for next time.

And Danny. I feel so bad for him. I didn't see him for 5 days. I feel so guilty having to spend so much time away from him, but this time Philip did not do well with me away for any length of time. And he is such a good boy. He never complains about me being away, he just does what he has to do and gives me extra hugs and kisses when he sees me. He does want some extra attention but I don't mind giving him that. He's so sweet to Philip and will rub his arm and back. That's not to say they weren't fighting over a car within 10 minutes of being home and then play nice 10 minutes after that, but hey, boys will be boys. And He's been my shadow since we came home. I had some side work to do so I let him press the wireless remote when taking photographs and he pulled up his chair and sat in my office with me tonight while I caught up on some more work and fell asleep.

Now for adjustments...
There is always a period of adjustment after a hospital stay. We basically do what we have to to get through the stay and deal with the consequences when we get home and hope we can adjust quickly back to our normal routine. We were on the right track of finally reducing Philip's bottle use but that went out he window in the hospital, especially that he couldn't eat for so many days and just could drink, so we have to start again with that. And he started hitting himself when he gets angry or frustrated in the hospital and talks through gritted teeth when frustrated, so we have to work on that. Sleep, well he never really slept good to begin with but the hospital throws that out of whack too. The day after we came home he whined and cried til 4:30pm.  He kept thrashing around and finally said his ear hurt. So, off to the doctor this morning and he has an ear infection on top of everything else. It was probably brewing in the hospital and what was causing the fevers. The poor kid can't catch a break. Today he is finally acting like himself and his appetite is back in full force.

I spoke with the doctor and asked again what could have caused the varices to get that bad so fast because 3 weeks ago when he had the endoscopy they were flat. There is some speculation that whatever virus/infection he had caused a strain on his body and liver and thus led to the varices bleeding but there is no real answer. Sometimes it just happens. It's very unnerving that it could happen that fast and that next time it could be even worse than this time. And that every time he gets sick it could take a toll on his body causing this to happen again. I'm afraid to take him anywhere now for fear of him getting sick. We are now waiting to hear from Mt. Sinai. They will be discussing his case at the team meetings to see what they want to do as far as transplant goes. We will most likely see them in the next 2-4 weeks and have more information.

Thank you for all of your continued prayers and support. Keep praying Philip stays strong and that this doesn't happen again anytime soon.

Ticking Time Bomb

Saturday morning, Philip was brought in to have his endoscopy. So off we went and we waited to hear what they found. The doctor finally came out and said he did great but they saw 4 very large varices, one which was actively still bleeding. So they had to do sclerotherapy on all 4. They responded well to the treatment and flattened out and the bleeding stopped. She said she was actually shocked by the findings because he was just scoped 3 weeks ago and the varices were all flat at that point. The covering doctor said she always refers to kids with esophageal varices as ticking time bombs because this is what happens. It's so unpredictable and could be worse too. 

He was put on restriction to have nothing by mouth which is one of the most stressful situations for a 2 year old to be in. He was not happy and as time went on became pretty combative when any doctor or nurse tried to take a look at him. He just repeated I want my Baba, I want to go home now, can you lay with me, can you hug me, which turned to get me my baba now, I gotta go home right now, can you scratch me, and just good plain old NO! For good measure. These were repeated over and over for hours and hours into Sunday. He had 3 IV's fail and had to be stuck multiple times for blood draws, including a not so fortunate incident with a resident who I had to ask to stop and take a break because Philip was thrashing around and compromising his IV's and she was coming at him like Norman bates in Psycho with the needle. Literally, me and nurse had to duck out of her way because she was trying to

follow his hand with the needle and then repeatedly stuck him about 5 times in the hand in quick succession with no success. She had no patience and caused him unnecessary stress. The transport team who I have to say was amazing was called in and they came in , treated Philip with respect, had this cool like infrared tool that shows where the veins in. We wrapped Philip in a blanket so he couldn't be so combative and 1,2,3 done! I also have to say we've had some of the best nurses ever this stay. A special thank you to our nurse Cristine 

Today. I've never experienced a nurse like her before in any of our stays. She had her shit together, was on top of everything and She went above and beyond to advocate for and help Philip make it through til he could finally start eating at 2pm. She was truly amazing.

Once Philip was able to drink and eat clears like water, juice and jello, his personality started to slowly come back. It's still mixed in with I want to go home. And even a smile and I I get to go home now! Like he's trying to convince us that he's allowed to go home. But it's a little more bearable now. He did have another black stool earlier so they did bloodwork and it was still stable so they believe it is just residual blood left over still making its way out. But if there are more, they need to recheck bloodwork every time. 

All in all he is doing well and improving and is stable at the moment. We are doing ok as well. A little exhausted emotionally and physically but it's nothing compared to what Philip is going through so we will push on through it. 

 We will be in contact with Mt. Sinai the transplant center to see what they think about getting exception points and moving forward with living donation. 

Thank you for your continued prayers and support. 

We know they are working and someone is looking out and watching over Philip.