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.

Unbelievable!

I was trying to think of the perfect title for this post but I think the best word to describe the news we got yesterday is Unbelievable!
We saw Dr. Prince (Surgeon) on Friday and he was so happy with how Philip looked. He ran some blood tests to see where we were at with Philip's numbers.
We had an appointment with the GI on Tuesday so they gave us the results.
Drumroll please………………………………………………...
His Bilirubin numbers are 1 / .7 !!!!!!!!!!!  Basically within a normal range! Just a few 1/10ths off.  His other numbers are still a little elevated but are dropping so they are happy with that. He also gained weight which has been a struggle for him in the past. He is up to 13lbs 6 oz.!  He is also being weaned off his steroids which is awesome. Can't wait to have one less medication to give him.
I could barely contain myself after that appointment. I had to run some errands after and I wanted to tell everyone I saw about the news. I would just think of it and start to choke back tears of happiness.
It's really been amazing the last few weeks watching his skin color slowly turn more pink. And especially his eyes become white because that is always the part of him I would gauge his yellowness by.  Dr. Prince said we just have to wait and see how long his body will sustain this. And the GI's said our focus now will be for him to continue to gain weight and grow.
When this all began it was hard to believe that things could go this smoothly. I am afraid sometimes of the rug being pulled out from under us or jinxing it by talking about how good things are going.
All I know is he is one strong baby and the room lights up when he smiles.
Thank you to everyones continued support and thoughts and prayers. Who knows, but I think all of those positive thoughts and prayers have somehow worked and helped him get through this. I know they have helped us.

1/3 is my new favorite number!

Thank heaven this week finally came!! We were waiting for this week to see Philip's doctors to get a sense of what was happening on the inside. 

Well, Tuesday we saw his GI doc and she was very happy with how he looked. She said it seems that his Kasai is draining!! She switched some of his medicines around so he takes 6 doses of meds a day.

The most exciting switch was that of this steroids. She was able to put him on a more concentrated solution so now he only has to take 3.3ml instead of 10ml. Only 1/3 of what he was taking!! So much easier now. No sweating for me and not much gagging or crying from Philip. LOL

She also ran some blood work so we can see how his bilirubin is. She told us to call today for the results.

On Thursday we saw Dr. Prince(surgeon). The first thing he said when he walked in was "Is he less yellow or is it me being really hopeful?" It was good to hear him say that because people keep saying it but we didn't want to get too excited if it was just our eyes playing tricks. He was very pleased with how Philip looked and how he is recovering. He asked if we had heard the results of the blood work yet and we said no. He was so excited to be the one to tell us the results. A 5/3.

Meaning his Total Bilirubin is a 5 and his Direct Bilirubin is a 3. When Philip went into the hospital before surgery, his numbers were 13/7. They have dropped by almost a half!!!! Nowhere near normal but he was pleased with the numbers of this as well as his other liver functions, which are still elevated but that is to be expected with Biliary Atresia and one just having liver surgery.

Just for reference, Normal levels are usually:

Total bilirubin: 0.3 to 1.9 mg/dL
Direct (also called conjugated) bilirubin: 0 to 0.3 mg/dL

In the beginning Dr. Prince told us about how 1/3 of Kasai's work, 1/3 work for an unspecified amount of time, and 1/3 never work. Well he said we can pretty much rule out the third where it never works!! So 1/3 down!! Unfortunately we still have to wait to see what the future will hold. We just have to keep praying for colored stool and lessening of jaundice.  I guess we should get used to the idea that we will never have a definite answer on whether the Kasai will work forever or he will eventually need a liver transplant. That is something I will always struggle with I think. In the meantime we will take it day by day and try to live each day to the fullest and try to go on with our lives as usual.

Here is a picture of Philip 3 weeks after his Kasai surgery. I think he healed up great.

He finally hit 12lbs too! He is only in the 19% for weight but at 25" is in the 95% for height. LOL
I'm hoping he will pack on the pounds over the next few weeks and catch up a bit.
So that is where we stand right now and only time will tell.
We want to thank everyone again for showing us so much love and support over the past few weeks. From texts and phone calls, to flowers, to fruit baskets, to gift cards, to dinners, to babysitting, to coming to appointments with us, prayers and so much more... we appreciate every single one of them. We would not be able to get through this without everyone's support. 

Waiting is the hardest part...

So it's been a while since I updated but to tell you the truth not much has been going on.  We saw the new pediatrician last Monday and she said he looks good. She even commented on his color and that he didn't look as ashy as he did before the surgery. We have also been impatiently waiting for Philip's doctor's appointments this coming week. We will see the GI on Tuesday and the surgeon on Thursday. Im assuming they will do some blood work to see where we are at.
Philip has been doing great this past week. He smiles, laughs and is talking up a storm. Sometimes when I'm giving him his medicine he will talk to me like he is complaining. It is too funny. He has been taking the meds pretty good. The prednisone still remains a challenge for me somedays. It is the most stressful part of the day for me. Especially when Dan is working and it is up to me to get it done alone. It takes me about 45 minutes while he's crying and gagging and coughing and Danny is playing in the dog food or standing on the open dishwasher door or demanding a cookie or yelling at me to get him Tea! LOL Needless to say I am breaking quite a sweat by the end of it. I feel so bad for Danny. He must sense something is going on. It must be hard to adjust having a new brother and having to share me with him but I cant imagine how he feels with me having to give Philip extra attention. But I just have to keep in mind it is temporary. Slowly he is coming around and will play this little piggy with Philip or give him kisses on the head in between covering his ears when Philip is crying. LOL
Several other people have also commented on Philip's color and how he looks better and he is not as yellow. I do see it somewhat, but his eyes still look yellow to me. It is hard to not get too excited about everyone noticing but I don't want to create a sense of false hope just yet. Don't get me wrong, We do remain very hopeful and are keeping the faith but I won't be jumping for joy just yet. I want to see some hard proof in the numbers. Hopefully this week will bring that.