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. 

The Feather and the Transfusion

Yesterday, when I picked Philip up, he looked a little pale and my mom said my sister had mentioned it as well and that he was a little clingy to her the whole day.  I didn't think much of it because he's been having a rough time sleeping lately and figured he was tired or coming down with something. 

But when I changed his diaper this morning, his stool was black. I wasn't sure, but it was dark enough to cause concern and coupled with the paleness and dark circles he had that morning as well I  felt it warranted a call to GI.

The nurse called and said they felt it was a good idea to head to the ER. 

When we got to the ER they took us right back into a room and started his work up. They were concerned he was having a bleed from somewhere. When they took his blood it was very thin like water and his heart rate was rapid. He quickly declined and became very lethargic and mushy. His blood pressure was low. He was just out if it. Not himself at all. Scary to think how quickly he deteriorated and what if I didn't listen to my gut. The doctor came in explained that he was in fact having a bleed from somewhere. His hemoglobin was very low at a 6 and before they can do an endoscopy he needs to be fluid and blood resuscitated.  

Because there is more of a chance of bleeding going in and looking and if he had an active bleed his current blood doesn't give any room for more bleeding. 

So that meant he needed a blood transfusion. My eyes started to well up but I was still alone at that point and didn't want to lose it. He was doing so great I just never saw this coming. Especially not a blood transfusion. 

My sister arrived and as they were preparing him for the infusion we both caught sight of something and followed it

At the same time. It was a little tiny feather floating. We looked at each other and I started to bawl. We both felt it was Nana Bunny there saying everything was going to be ok. The nurses probably thought we were crazy as we tried to explain what just happened as I was crying out of nowhere. He also spiked a fever before the transfusion so we are trying to figure the cause of that too. We are in a step down PICU right now. He received 1 unit of blood and will get labs at 1am to see how everything looks and if he needs more. He's also on octreatide to help stop the bleeding and some antibiotics just in case. He can't eat or drink so he is not happy and keeps asking to go home. He even demanded to my sister that she " get me my baba right now". Endoscopy should be tomorrow. Hopefully early so we can get him drinking sooner. He's still spiking a fever and heart rate is high now so we are hoping that goes down too. Please say a little prayer for your friend Philip that this resolves quickly  and he can come home soon. 

The Ordinary Life

It's been a while since I've written anything and I wanted to write a year end post or new year post so I've been coming back and forth to my computer for a while now, trying to figure out what to write.

Type. Type. Type

Delete. Delete. Delete.

Repeat. Repeat. Repeat.

Then I saw one of those little meme/saying things on Facebook and it really spoke to me.

2015 was a pretty ordinary, routine year for us.   Generally, Philip's health this past year was so amazing that we had time to enjoy the ordinary life. We had maybe 2 hospital stays and the rest were routine visits and testing. Maybe that's why I've been a little too reluctant to post anything. I don't want to jinx it, but it really was so great to be able to take a little vacation this summer and spend the holidays with our families. I'm scared of getting too comfortable with this ordinary life that I'll be crushed if it goes the other way again.  So in the meantime, I will enjoy this ordinary, breathtakingly beautiful crazy life.

And because I also use the blog to sort of look back and see what his numbers are like here is a little more details about his health.
At year end, Philip's direct bilirubin went up to 2.9 then down to 2.5 and his other liver numbers remain about the same. (always elevated but not too bad) His liver is still functioning well but with the elevated bilirubin, the liver damage will keep progressing slowly.

His spleen is enlarged because of the portal hypertension and he is still at risk for esophageal bleeding and fluid in his abdomen. He will continue to be monitored with endoscopy surveillance with one coming up at the end of the month. He still suffers greatly with the itching, waking several times at night from it. Some weeks are better than others, but some are really bad. He has an amazing personality that I think stems from a lot of stuff he has dealt with in his 2 years. He is quite the character and makes us laugh everyday.

Blessings

Faith is a funny thing. Most people have a strong pull either way. For me, it would come and go, always being tested on a daily basis until a recent event pulled me, no, shot me out of a cannon into certitude.
I'll start by admitting that I had been having a hard time lately. Philip is doing great health wise but he has some habits that need to be broken. He is addicted to his bottle and while his new medication for the itching seems to have helped a lot, he still wakes about 4 times a night yelling "OW, MOMMY, NO, CAN'T, NO MOMMY". I am just at a loss. I don't know what to do for him anymore. So with all that waking for scratching and bottles (and changing sheets because he drinks so many bottles) I am not really myself lately. I'm short with the kids and Dan and exhausted and just plain not 100% at anything I do. I was questioning my faith a lot. Asking for help and guidance and patience.
God, Give me a sign please!
...And I got it BIG TIME on September 24th.
My brother in law Jimmy, was able to get us to the airport to watch the Pope land in New York and had hopes of getting he and Philip Blessed by the Pope. Jimmy is battling Hodgkins Lymphoma. It was an exciting day that started with us being screened by the secret service. Me, Dan, Danny, Philip, Jimmy and his wife Tara all walked onto the tarmac and began to wait for the Pope's arrival. We saw the White House Helicopters and some Osprey's and Watched the airplanes take off and land. About 45 minutes before the plane landed, Jimmy and Philip had to switch areas in order to be in place to be blessed. Philip wasn't cooperating to the fullest, so I had to tag along with him and Jimmy. We made our way over and were stopped to do interviews with CBS news radio. Thy asked why we were there and what we thought of the Pope. I was so nervous I had no idea what to say! So I talked mostly about Philip and his situation. After that, we moved into the Blessing "pen" (for lack of a better word). We were standing on the edge of the crowd and somehow got pushed back out of and behind the blessing area. I thought the chance of them being blessed was out the window but Jimmy somehow made conversation with two gentlemen in front of us and it turned out one man's wife is a principal in our town and in a school that Tara worked at! Small World. It turned out they knew someone in charge and he came over and said the best he could do was have us walk to the other side of the pen and squeeze to the front and Give Philip to these two women in red shirts and they would hold him. Jimmy would not be able to get into the area. So we made our way over and squeezed our way up and handed poor Philip over! He was so good for a while then he started getting agitated. The ladies wanted me to hop the fence and come up there with them. I said No Way! I don't want to get shot by secret service! LOL The people around the area were so friendly and helpful it was amazing. Thankfully after going back and forth a few minutes, Jimmy agreed to hop the fence and be with Philip. During all this commotion the plane landed and the Pope was making his way over towards Jimmy and Philip. At this point Philip had enough. He kept screaming and crying and yelling for me. But Jimmy kept him with him and Pope Francis approached them laughing. Maybe because Philip was screaming so much! LOL Jimmy told him about them both and someone translated into his ear and he blessed both Jimmy and Philip. He even kissed Philip's head. The tears just poured out. There was No stopping them. Everyone in our area was so excited and emotional for us. It was incredible. After the Pope passed us, Jimmy handed Philip over the crowd back to me. You could see him crowd surfing on the news. LOL We walked back to our family and told them what happened. They were in awe! They couldn't believe it. I STILL can't believe it. Thank you so much jimmy for making such an amazing moment happen for us.
It was an incredible experience and one that I will hold in my heart forever. And when things get a little rough and I feel myself starting to question my faith again I just have to look at these pictures and know that without a doubt there is something greater out there and is watching over me and my family.

 

Sweet Relief

Wednesday, we headed to the hospital for Philip's endoscopy and colonoscopy. His stool had tested positive for blood so we wanted to investigate further to learn the cause of it. Whether it be varices or a milk protein allergy. 

So Tuesday, I miralaxed him up and battened down the hatches and got ready for the shit storm about to hit. (hahaha-I'm a sucker for potty humor).

He did great and only gave us a hard time overnight because he still wakes for his comfort bottle(s) when he wakes up itching. 

He also did great at the hospital and because they were running behind he only started to get cranky the last hour really. He kept asking for a snack or a bottle or a cup and saying I want to go home and head for the door. Poor thing. I felt so bad I didn't know what to say to him. Thank God for Child Life at the hospital though. They were very efficient in distracting him long enough to get through that hour even though he would intermittently get up to "go home". 

He was pretty agitated by the time they were ready and the anesthesiologist said he could give him something to calm him down before hand but it would take a little longer and they would stay in his system longer as well, or we could just bite the bullet and bring him in crying and the big breaths would help him breath in the anesthesia quicker. Child Life managed to distract him one last time and we walked down to the procedure room with her walking backwards with the i-pad and me holding him.

The anesthesiologist allowed him to pick a flavor for his mask this time and we picked watermelon. So I sat him on the table, and the anesthesiologist showed Philip the mask and had me smell it to show him, then on went the mask and the crying began again. One quick minute and he was sleepy. It always hard to see him go down like that but I try to never let him see me worried about it. I want him to see me smile and kiss him and say see you in a little bit! 

Both procedures went well and to our surprise they didn't have to inject any varices again! They are still there but still only Grade 1 which means they are not large enough to be treated. The colonoscopy went well too. He had some irritation and varices and she took biopsies, So we will wait to hear in about a week about that. We were able to see him right away and go home soon after. It's always such a relief when we get to go home right away and he gets to eat and drink normally. This also means we can wait longer between endoscopies now. He won't have to get another one for 6 months.

We also got blood work done and His platelets went up to 91,000 which is good. But his total bilirubin went up to 3.1 and his direct bilirubin went up to 2.6 from 1.9 in June. He's fluctuated in the past but mostly has stayed about the same for the last 6 months. Some other numbers were elevated as well so we will have to see what happens with the next blood work. I guess at some point it will start to trend upward as the disease progresses but I just pray it goes back down and it was just a blip on the radar.

So for now we will savor the feeling that we were able to come home and in his full night of sleep last night; even if it was just one sweet night...