Close Call!

Last Sunday Philip got a fever around 8pm. So I called the GI doctor and as I assumed they sent us to the emergency room to get checked out and have some blood work done. This time my mom and dad had been sick with fever so we hoped it may just be viral and not cholangitis.
We got to the ER around 10pm and by the time they were able to get blood and the flu swab it was 2am. The rapid flu came back negative so the decided to admit us. We finally got in our room around 4:30am. Thank you to my sister for staying and keeping me company since Dan was at work.
They started him on IV antibiotics in the meantime. They took the blood before the antibiotics started and It turned out his blood work came back better than the week before when we saw the doctor. So this made them believe it was just a virus but We still had to wait the 48 hours for the full result of the flu test. So we were quarantined to our room and he couldn't come out til we found out the results.
His next blood work still improved so there was some question on whether or not it really was a virus or the cholangitis. Well Tuesday night they came in and said his Flu test was positive! So that was the source of the fever. In the meantime my mom, Danny and I came down with symptoms as well.
SO they released us Wednesday afternoon and we are all taking Tamiflu which by the way has helped me greatly. Philip was released on oral antibiotics because the IV was working on something so they figured as a prophylaxis to continue on for 8 days with amoxicillin.
Such a weird feeling to be happy that he had the flu. I was smiling like crazy knowing he wouldn't have to endure IV antibiotics for 21 days again.
Over the last couple of  months, Philip's platelet count had been dropping and While we were there his platelet count dropped significantly which we learned was from the flu. It did come back up a bit after a couple of days but we were told he is showing signs of portal hypertension.
We are still learning about it but in the simplest terms that way I understand it is as the liver hardens it causes a sort of traffic jam of the blood flow so it backs up into other veins and organs causing pressure like in the spleen which causes it to enlarge. There are other situations that can occur with this like esophageal bleeding but we are hoping that never occurs. Unfortunately this is the nature of the disease and although his bilirubin is the lowest it has ever been, the disease still progresses causing damage to the liver.
Philip still remains a happy little guy and is hitting all his developmental milestones. He doesn't like baby food much but who would I guess? lol

We will be walking in the LI Liver Life Walk this year at Eisenhower park on May 18th.
Please join us at the walk if you can and help us raise money for a great cause!
Our team name is Friends of Philip!

http://go.liverfoundation.org/goto/kimkennelly

Home at last!

After 23 days in the hospital we are finally home! Not much happened since the last update. Things were pretty quiet the last week beside them having to change the dressing on his head almost every day because Philip sweats too much and it started to fall off. He was the healthiest on the floor so not much was going on. We took our walks around the floor several times a day, went to the playroom and he would flirt with all the women on the floor. So, Saturday the 15th started like most days with them changing the dressing but this time they looped the line so it was sticking out a little bit in front of his head. Well one second left alone in the crib to get an outfit to change him and we heard a ripping sound. Yup! Philip finally ripped the whole dressing off and the line out with it. They came to check it and it was too far out so they had to remove it. We were in shock! 4 doses left and he pulled the line out!!! He was so proud of himself, smiling and laughing. So they went ahead and put an IV in his hand to finish off the doses. He had his last dose Sunday night around 6:30pm and we were free to go! Coming home never felt so good! Though it is proving to be a rough transition so far. He is very cranky and won't settle down. I guess it may take a few nights to get used to being home again. Thanks for all the good thoughts and prayers to help send Philip home!

Unique…

I just realized I never updated with what we/the doctors decided to do about the course of Philip's treatment. Last Thursday we decided to try a NICU Picc line. Meaning the line is smaller than the pediatric line. It is usually used in preemies but has been used in older babies before. So the Nurse practitioner from the NICU came in and said she could do it easily right there without sedation but my doctor said no, he needs to be sedated. So again no food for 6 hours and off we went to get the line in. The took him in and came out and said they were having trouble getting an IV in and they were going to try it un-sedated. Well she came out again and said he was fighting and moving so much they couldn't do it without sedation but they were finally able to get an IV in him to sedate him. What we thought was going to be a 20 minute thing turned into a 3 hour long process. They ended up getting it in a vein in the side of his head. Sounds worse than it is but the poor guy looks like he has an antenna coming out of his head. They had to X-ray him to make sure the line wasn't in too far risking it touching his heart and giving him arrhythmia. Sure enough it was in too far so they had to undress it and pull it out X-ray again. This process went on a couple of times to make sure it was at the proper length inside his body. The poor baby's hair is being pulled, cut and matted by all the taping and re dressing. They did originally shave some hair but not enough.
Unfortunately Philip is a mover and sweater and the bandage keeps puffing up giving room for the line to wiggle in and out. It came out quite a bit so they x-rayed again. The line was still good but it did move out a bit, So they kept his IV in and kept giving antibiotics through that till they could get it re dressed yet again. Once redressed and reassured it was ok to use, they started using the line in his head to administer the antibiotics. The dressing seemed secure but we kept noticing the line looking like it was going further into his head. And we were concerned because in the beginning they had to keep pulling it out because it was not safe to use. We expressed our concerns to the nurse, residents, GI docs, anyone who would listen. Several times. We kept being assured it was fine. They didn't see any movement. Now I had taken a picture of the line so I could reference the length for the doctors and there was an obvious change in the length yet we were still assured by the residents that it was ok. So yesterday, we noticed it looked a little farther in again so I complained again. They finally ordered another X-ray and what do you know…It was in WAY too far! In between, Dan spoke with another resident and said I know we may seem annoying but we are just concerned. She goes on to say "No we just think you are unique…"So Dan asked "what does that mean?" She said "Well, most parents don't question what we do, they just do what we say. You've been through a lot and seem more informed…"

So PLEASE, PLEASE, PLEASE. If you have a feeling or see something you're not sure about SPEAK UP to your doctor or caregiver. I will never be afraid to question a doctor again. You know yourself and child best.  Especially when they are not your usual doctors.
These are the same feelings and persistence that got Philip diagnosed in the first place. I'm not sure what makes doctors feel so easy to brush off a parents concerns these days. I'm glad my new pediatrician isn't like this.
Through all of this Philip has remained the happiest baby ever. He smiles at everyone who looks at him. He flirts with all the nurses and it seems like everyone on the floor knows his name. He is a little bored and it is hard to keep him occupied because he is hooked up to a heparin drip 24 hours a day to make sure the PICC line doesn't get clots in it because it is so thin. But he is holding strong.
What stinks is that he really isn't "sick" anymore. They just have to finish the antibiotics course because it is bringing his numbers down and he had been doing so well after the surgery. They don't want to risk any more fibrosis or scarring to his liver when it is in such good shape still. That is the reasoning on being so aggressive with the treatment.
Our tentative release date will be February 16th. It can't come fast enough.
Thank you for all the continued prayers and support!

PICC Line In. PICC Line Out...

Today was the Worst day ever.

Philip got his PICC line inserted yesterday and it went well. It wasn't the most stable position but in far enough where they were comfortable sending us home and having home care teach us to do the infusions of his antibiotics. This morning they changed the dressing and sent us on our way with the line out 3cm. We got home around 12:30 and the pharmacy dropped off the supplies by 1:00 and the home care nurse was over by 1:30 to start. Philip's dressing was saturated in blood so she had to change it again before we could start the infusion. So while my poor sister and Dan held him down she proceeded to take the dressing off. I won't  begin to tell you you the screams he was making from the tape being taken off. One word...Horrific. I thought I saw the line come out a little but she measured and said it was the same as what was in his discharge papers. She cleaned and redressed the site and tried to flush it but met resistance and saw that fluid was coming out of the entry site at the skin. So she had to undo the dressing again to evaluate it to make sure there were no kinks. More screaming for poor Philip. So after a few calls we were sent back to the hospital for the PICU team to evaluate it and see if it was salvageable. So off to the hospital we went at 2:45 where they undressed the site again. This time I held him down screaming. His poor skin was so raw at this point. They measured the catheter and it was 8cm outside now. Meaning only 10 cm were inside his body only reaching to his shoulder. Not far enough in to do its job properly so they had to take it out completely. The purpose is to have it snake far Into his body closest to the center like where his heart  is. So we were readmitted and brought up to our room, Which is in the new pavilion and is private so we were happy about that.  Now they had to put in yet another IV so he can get his antibiotics till we figure out what to do.  3 STICKS to get the IV in this poor baby.  Enough already!!!!  How much can this poor baby handle??!  I just can't anymore.  I wish they could stick me. He 's getting wise to what's going on and knows if I put him down someone will most likely be poking and prodding him.  Just frustrated all around.  Tomorrow we will decide  if we should try a PICC line again or  just go for the neonatal line and stay in  the hospital or  do multiple IV sticks over the next couple of weeks.  Let's hope whatever happens, things go a little smoother from here  on out.

Friggin' Bacteria!

Well, It's been a while since I updated everyone on Philip so here it goes…
Saturday morning, Philip developed a fever. So I called the GI office and they sent us to the ER to get some blood work done but be ready for a 48 hour stay. Normal protocol for someone with Biliary Atresia. So after a ton of blood work, urine culture, and ultrasound it looked as if his Bilirubin and liver numbers increased as well a white blood cell count a little. The major concern with having had a Kasai is an infection called Ascending Cholangitis. Since his intestine is hooked directly to his liver, there is now a direct "highway" so to speak for the bacteria which we all have in our intestines and we need to aid in digestion to creep up(Ascend) into the bile ducts in the liver. Being his bile ducts are malformed this causes the backup of bilirubin and can cause liver damage. They admitted us and His fever persisted overnight and spiked at 104. So even before the final results they started aggressively treating it as if it were cholangitis. Because he is doing so well after the Kasai that is another reason to treat it so aggressively. The treatment consists of 21 days of IV antibiotic called Zocin. Yes, when I heard 21 days I about passed out. So we have been in the hospital since then and he is getting his antibiotics every 6 hours. We thought maybe it could be a virus but after starting the antibiotics and no fever since Sunday and the trend of his bloodwork, it looks like it is indeed cholangitis. Today he is having a PIC line put in which is sort of a more temporary permanent IV line that can stay in for the whole 3 weeks so they don't have to keep sticking him. They will try to put in a pediatric line so he can come home and a home care agency will show us how to administer his medicine at home. If not they may have to put a neonatal one in and he will not be able to come home because home cares will not care for one that small in. We are a little stressed out trying to figure it all out right now but we are praying the PIC line works and we can come home and he continues to respond well to the treatment. Philip on the other hand is charming the pants off of all the nurses and staff. And he rocks that hospital gown!

  bil·i·ara·noia
 noun \ˌbi-li-er-ə-ˈnȯi-ə,\

 paranoid thoughts that cause you to believe that your baby's eyes are getting yellow and therefore his bilirubin is rising.

 The past few weeks I've been having what I call "Bili-aranoia". I've become convinced that Philip's eyes are getting yellow. I continuously ask family "Do you think his eyes are yellow?"

"Am I crazy?" "You don't see it?" 

To which everyone says "Yes, you are crazy" LOL

I'm that crazy lady in the store that comes up to you and asks "Can I have your honest opinion on whether my baby's eyes are yellow. Go ahead tell me the truth." 

Ok, Maybe I haven't resorted to that yet but it does sound like a good idea right? You know, to get an unbiased opinion? LOL

We did see Dr. Prince last Friday and I brought it up to him and he didn't see it either, but he did blood work anyway. He said that is what will tell the truth. 

We then saw Dr. Webster the GI doctor on Christmas Eve.

She gave us the results and his bilirubin went up from .07 to .09. She said that was within the margin for lab error so she doesn't even consider that a rise and wasn't concerned at all.

Also the fact that his liver numbers decreased significantly, she

said that tiny rise in bilirubin would not cause his eyes to yellow unless I have some super bionic vision. LOL

She said he is doing great! What an amazing Christmas present!

She also did not see the yellow in his eyes. 

So I guess I'll just have to get used to this new sense of paranoia  that something is going to go wrong. Things are going so well I just don't want something to happen to that.

With the New Year upon us it's hard not to reflect on all that has happened this past year. What a roller coaster ride it has been.

Saying goodbye to 2013 will be bitter sweet. We welcomed beautiful Philip into the world and expanded our family. Danny turned two, became a big brother and has grown in leaps and bounds. We received the terrible news about Philip's condition. He went through surgery and is doing great now. We learned how strong we are as a family, a couple and as individuals. We learned how much support we have in family and friends. We learned how to enjoy the small things more, and to not take those small things for granted. We learned the power of laughter and a great sense of humor. Mostly we learned the power of faith and prayer and positive thinking.

This year could have been so much worse if it wasn't for every one's support and positive thoughts for Philip, us, and our family. Thank you to everyone again for all of that.

We hope this coming year continues to bring good health, happiness, laughter and love to everyone and their families.

Have a Happy and Safe New Year!

Love and Good Wishes

Kim, Dan, Danny and Philip

Congenital Malformations Registry (CMR)

So we received a letter in the mail a few weeks ago that Philip had been "reported" to NY State's Congenital Malformations Registry (CMR). Who knew there was such a thing. The role of the registry is to help better understand the extent and causes of birth defects in New York State's Infant population.
Hospitals are required to identify and "report" children who may have congenital malformations.
They include material to make you away of services that may be available to you and your family.
I understand the importance of such a thing and feel like its needed but I have to be honest that I was a little taken aback when I received the letter.
Now It  wasn't a surprise that Philip's disease is considered a birth defect but I felt maddened by the use of the word "report" in the letter. In my head being "reported" always came with a negative connotation. It brought up feelings from the beginning that I did something wrong to cause this. Also the fact that his name is out there on a "registry" (Maybe not literally but figuratively). It just makes me think of the sex offender registry or that he was being banished to the island of misfit toys.  I know a terrible thought but these are the things that first came to mind when reading the letter. No one wants to feel like their child is less than perfect and this made me feel like, well now it is permanently documented that my sweet perfectly imperfect baby is, well, imperfect.

That being said Philip continues to be doing great. We get more blood work the end of the month and we will see if his levels are still going in the right direction. He took his last dose of steroids Friday  night so that was an exciting day for me! I remember when he had surgery and they said he had to be on the steroids for 90 days and we said Wow it will be almost Christmas when he finishes. We couldn't ask for a more happy baby.