Home Sweet Home!

I will preface this post by saying Thank you to everyone who has been reading my rambling goings on with Philip. Writing this blog I think is kinda like my therapy to deal with this situation better. When I found out about Philip's possible diagnosis I googled so much my eyes were crossing. I always gravitated to reading about peoples personal experiences rather than clinical and medical websites (Although I've read  my fair share of those too) So I guess I also figured that if my rambling can help other parents just finding out about this disease and  understand the process they will go through better, then it is worth putting out there.

Yesterday Dr. Prince came to see us in the morning. He said Philip would get his JP drain out that day and we could then go home!! We were so excited! We packed up our stuff and Dan started bringing things down to the car. Around 11:30am they came and removed the drain and the nurse was getting our discharge papers ready. She came in and asked if she could help get Philip dressed to go home so I said sure. Well she sat Philip up in bed and with that he sneezed and barfed ALL over. He sure scared the you know what out of that nurse. Dan ran out to catch the residents who helped remove the drain and with that we were told we had to stay for two more feedings to make sure he didn't do it again. So they hooked him back up to the IV to give him his meds he was due for. (Thank goodness the nurse didn't remove it, she just disconnected it.) We then waited and fed, and waited and fed. Philip did fine with these two feeds. This brought us to around 5:30 or so. So the nurse disconnects the IV and the residents come in again to start to explain our discharge plan. They leave and come back in a few minutes later and talk about giving Philip his first dose of his Prednisone through IV that night as opposed to me giving it to him orally. I would have to give him 20ml orally. It would delay us another 1 1/2 - 2 hours but We agreed that was probably the smartest thing to do. So back on the IV he went and judging how he acted taking his 13.5ml tonight I am glad we did it through IV last night. So we were finally discharged around 9:00pm with 4 prescriptions to fill and Philips med plan for the next few months.

Ahhh Freedom!!

Not knowing any better we stopped at CVS to fill the prescriptions on the way home.
Bad news. They can't fill 2 of the 4 prescriptions and Philip has to start taking some at 10am the next morning. Panic started to set in. So I went home with Prednisone and his Antibiotics in hand but not his Actigall(Which helps bile flow better) or his vitamins(AquADEK-fat soluble vitamins A,D,E&K)which are over the counter by the way but I guess not many places have it.
Thanks to the lovely pharmacist at CVS he told me I had to find a Compound Pharmacy who would make the prescription for us. He actually called the hospital and they told him where to send us.
So this morning was spent calling other local compound pharmacies and finding out most couldn't make the compound to be ready for today and that insurance did not cover one of the ingredients so they would not cover it. So we ended up with the pharmacy recommended by the hospital. So I faxed the prescriptions and had to run to queens that afternoon to pick it up.
Philip took his antibiotics like a champ in the morning as well as the actigall. Both doses are manageable. 7:00pm tonight he had to take 13.5ml of the prednisone. What a nightmare! It is inhumane to make a baby take that much orally. It smells like anbesol and I can only imagine what it tastes like.
They said not to mix it with formula or he will start to refuse it. The doctor suggested giving little drops here and there over the course of an hour. Well it took us two hours and that was filled with screaming, gagging, spit up, choking, sleeping,then some more screaming, gagging, spit up, choking, sleeping. My stomach is in knots knowing I have to do this with 10ml every day for the next 90 days. It is torture for all of us. Me and Dan had to take turns and Dan ended up having to do most of it because it made me so stressed. I dealt well with him in the hospital for 6 days getting surgery, then poked and prodded but this is more stressful and mentally draining than that. I am praying for the strength to deal with giving him the medicine I know he needs.
Anyone know a secret to giving a baby that much prednisone??!!

Green is my new favorite color!

So as the doctors hoped, the last couple of days were pretty uneventful. We were able to start him on pedialyte yesterday. 1oz every 3 hours. Now this is for a baby who was eating 6 oz every 4 hours before his surgery. Needless to say that Philip did not like being teased with such a little amount of  liquid. He was pretty unhappy all day and hard to comfort. It was a really hard day and night for me. I mean, it isn't natural to not feed your baby when they are hungry. But, They let us increase to 2 oz later in the day and today he was able to start his regular formula. He was so happy and talkative today. It made my day. The doctors may remove his JP drain this evening or tomorrow morning.
They even said we may go home tomorrow!!!! Now your probably wondering why my new favorite color is green...Philip had two dirty diapers yesterday and they were GREEN!! For those who don't know, one symptom of Biliary Atresia is acholic stool(Stools that are pale, clay, or putty-colored may be due to problems in the biliary system (the drainage system of the gallbladder, liver, and pancreas)
We switched Philips formula so many times I never thought the change in stool color signaled something else. His surgeon said this is a good sign that bile was flowing but we can't be sure if it is due to the surgery itself or the real deal as he called it. Ill just keep praying for green poop!
I really can't say enough about our surgeon. He has such a special way of keeping us grounded about  this working but at the same time keeping us hopeful and optimistic. So hard to put into words. A lot of people (visitors, nurses, etc) have been telling us how Philip's color looks so much better, it is hard not to get excited. But we brought it up to Dr. Prince and he said a lot of times people want us to feel better so they say it. He said they may actually truly believe they see a difference because they want to feel better as well. And as doctors and nurses it is in their nature to want to help too and want us to feel better and they don't know what else to say.  But the truth is that his skin and eyes will be the last thing to change back most likely. And we are ok with that. That being said I totally think his feet and hands look more normal color today. Lol

Another good day

Yesterday was a good day. They removed Philip's catheter and tube that drained his stomach. The only things left are his JP drain(tube inserted in his side to drain fluid) and IV. I held him for most of the afternoon. He is so hungry and is getting very irritable because if it. He doesn't settle easily but was comfortable in my arms. He started wetting diapers on his own which is great.
I came home last night to spend time with Danny so Dan stayed at the hospital. It was really hard to leave but Danny is still adjusting to having a new brother and on top of that I think he senses something about what is going on. I'm getting xtra hugs and kisses from him! It could be that he just misses me though. It didn't hurt to sleep in a regular bed either or get more than 4 hours of broken sleep. Hopefully today we can start to feed Philip. The doctors say it should be a boring couple of days.
Lets hope for boring!

Movin' on up!

Out of the PICU that is!!!!
Philip did great today. He went most of the day without morphine and I got to hold him!
That was a little traumatizing but at least I held him. He kept squirming which obviously caused him pain so I only held him for a couple if minutes. They also told us Philip would possibly be moving to a regular room. Dr. Prince came to see him and was very happy with how he was doing. He said he looks like what other patients at 3-4 days post op look like. So he didn't see a reason for him to be in the PICU either. So around 9:00pm we moved to a regular room. Still no feedings and still has catheter, fluid drain on his side( don't know what it is called) multiple IV's and the tube up his nose to drain his stomach. But tomorrow they think they may be able to remove some of them. Keep the prayers and thoughts coming because they are definitely working!

Kasai Surgery Day-"Prince" Charming

Since I can't really relax or sleep I decided to write a little to clear my head a little.
This was probably the most emotionally draining day of my life.
Our day at the hospital started at 11:30. We were admitted to pre op and started our day of waiting.
And it was a long day of waiting...
Philip's surgeon Dr. Prince came to speak with us and after reviewing all his background again he decided he didn't think we would gain anything by going in with the camera first. It would only waste time because he believed Philip really did have Biliary Atresia. So we agreed and they finally took Philip in at 2:45. I didn't get to bring him into the room, I handed him over to the nurse. One of the hardest things I've ever done. Off to the waiting area we went, tissues in hand. Around 4, they said there was someone on the phone for us. My heart skipped a beat but it was the nurse telling us they had just finished the cholangiogram(where they inject dye to kind of see where there are blockages in bile ducts and gall bladder) and were mapping out there plan. Around 6:00 the phone kept ringing in the waiting area but the volunteer left. We were joking that one of us should answer then A few minutes later a nurse came out. Our hearts skipped a beat again. She said they were just making the first connection but everything was going as planned.
7:45 Dr. Prince (our Prince Charming) came out. He said surgery went exactly how he would have wanted. No complications. Philip's Biliary Atresia was the second most common type. He indeed had a gall bladder and it did indeed drain into the small intestine. Unfortunately it did not have the upward plumbing of the liver to the gallbladder. They removed the gallbladder and ducts and attached the small intestine to the liver. His liver also shows signs of damage from the bile being backed up. We hope the Kasai works and it will reverse over time. I can't imagine having to do this on an infants body. Imagine how small your small intestine is at 8 weeks old!  Dr. Prince is the nicest most compassionate man. He really cares about Philip's outcome and I feel like he is with us 100% emotionally on this journey. He mentally prepared us that Philip would most likely be still intubated in the PICU but Philip had different plans and they were able to extubate him. He really is so strong.  No matter how much my sister and Dr. Prince tried to prepare me for how he would look after surgery, you will never be prepared enough to see your infant with tubes all over the place and a four inch incision across his abdomen. So sad. It was also surprising that he all of a sudden looked so much like Danny! He really is a little trooper.
He is on some pain medication and will be on steroids and antibiotics. He is resting comfortably though. He doesn't fuss much and when he does it is only for a second. He is definitely teaching me a thing or two about strength.
Unfortunately with the Kasai procedure there is no way to know if the surgery is a success right away. The surgery went as well as can be expected but we will have to wait and see over the next 4-6 weeks how his body does and if he actually has the bile ducts to drain into his small intestine. The waiting is the hardest part. How do I get through the next few weeks? How do I keep my sanity? How do I not drive myself crazy? I have absolutely no idea!
We're also so thankful to everyone for their prayers and good thoughts. The support we have been shown today is amazing. So overwhelming. Thank you to everyone especially our families who were with us today on this emotional journey. We know it isn't easy for you either but appreciate everything you do for us.

Philip's Story

Philip Michael, our second son was born July 23rd, 2013 at 1:40pm weighing 10lbs 5oz. and 22.5 inches long. We were so excited to meet him and add him to our family. After an uneventful pregnancy it was no surprise we were discharged from the hospital after two days with no issues.
His eyes seemed a little yellow to me but was reassured his bilirubin numbers were fine and that all newborns were a little jaundice and it would clear in time.

We started to settle in and get used to being a family of four. Philip had some feeding issues in the beginning and after many changes of clothes, a trip to the ER and many formulas later, he was finally gaining weight and doing well.
Yet his eyes still looked yellow to me. I brought this up to his pediatrician at our 2 week and 4 weeks appointments but was reassured he was fine. I even called the office at 5 weeks because his eyes were still yellow and I was starting to get concerned. I received a call back from the office and was still reassured that babies can be jaundiced for two months and I didn't need to bring him in, I could wait for our 6 weeks appointment.
Friday, September 6th came and we went to the pediatrician. I mentioned that his eyes still seemed yellow to me and the doctor finally conceded and re tested his bilirubin.
Dan called me later that afternoon and said the doctor called him and to head over to the emergency room, that Philip's bilirubin was high and we should get some blood work done. So off I went to the ER. They did some blood work and it turned out that his Direct Bilirubin was high. That day I learned there are two types of bilirubin:

• Indirect (or unconjugated) bilirubin. This form of bilirubin does not dissolve in water (it is insoluble). Indirect bilirubin travels through the bloodstream to the liver, where it is changed into a soluble form (direct or conjugated).
• Direct (or conjugated) bilirubin. Direct bilirubin dissolves in water (it is soluble) and is made by the liver from indirect bilirubin. Source: Web MD

In simplest terms, elevated Indirect causes your usual newborn jaundice where they put the baby under the lights. Direct bilirubin is related to your liver and elevated number may signal a problem with ones liver.

So then they did an ultrasound to see if any thing else stood out. It showed a contracted gallbladder but he had just fed and that can cause the gallbladder to contract. They also noticed his poop was pale and grayish. I thought the change was due to changes in formulas so it never alarmed me. Turns out it is not a good thing.

After a while the doctor came back and told us she spoke with the GI doctor to see what the cause could be and was told it could be a thyroid problem, trouble with enzymes, a cyst in the bile duct OR even worse ..this is when I heard it for the first time...BILIARY ATRESIA. Google, google, google....and I wanted to throw up.

I was told to NOT google but that is hard to do when you have no clue what is wrong with your baby and the name of the worst possibility sticks out in your head.

What is biliary atresia?

Biliary atresia is a life-threatening condition in infants in which the bile ducts inside or outside the liver do not have normal openings.

Bile ducts in the liver, also called hepatic ducts, are tubes that carry bile from the liver to the gallbladder for storage and to the small intestine for use in digestion. Bile is a fluid made by the liver that serves two main functions: carrying toxins and waste products out of the body and helping the body digest fats and absorb the fat-soluble vitamins A, D, E, and K.

Normal liver and biliary system

With biliary atresia, bile becomes trapped, builds up, and damages the liver. The damage leads to scarring, loss of liver tissue, and cirrhosis. Cirrhosis is a chronic, or long lasting, liver condition caused by scar tissue and cell damage that makes it hard for the liver to remove toxins from the blood. These toxins build up in the blood and the liver slowly deteriorates and malfunctions. Without treatment, the liver eventually fails and the infant needs a liver transplant to stay alive.

The two types of biliary atresia are fetal and perinatal. Fetal biliary atresia appears while the baby is in the womb. Perinatal biliary atresia is much more common and does not become evident until 2 to 4 weeks after birth. Some infants, particularly those with the fetal form, also have birth defects in the heart, spleen, or intestines. SOURCE: http://digestive.niddk.nih.gov/ddiseases/pubs/atresia/

So we were sent home with an appointment to see a pediatric GI in a couple of days.

The GI doc called Monday the 9th and based on the blood work form the ER our next step would be to have a liver biopsy. WHAT??!! His vitamin K levels were low so she wanted to start him on vitamin k pills before the biopsy. Vitamin K helps our blood clot and when there may be a blockage in the liver, it hinders our bodies ability to absorb certain vitamins. SO we met the GI Tuesday and they mentioned this BILIARY ATRESIA again.

They seemed to think this is what Philip has. They actually said this is most likely what Philip has. He had some more blood work done and his coagulation numbers did not improve enough so we had to delay the biopsy one day and get him a shot of vitamin K.

September 12th, 2013

was biopsy day and he could not eat for 6 hours before the biopsy. Philip was a champ and slept most of the time and did not fuss much. They put him under light sedation for it and they were finished within 20 minutes.

He then had to wait another 4 hours to eat because they had to re check his blood for sign os bleeding.

He did great again but started to get fussy the last hour or so. He was kept overnight for observation.

September 13th, 2013

We heard from the doctor and the biopsy results came back...consistent with BILIARY ATRESIA.

Next step was to meet with a surgeon:

How is biliary atresia treated?

Biliary atresia is treated with surgery, called the Kasai procedure, or a liver transplant.

Kasai Procedure

The Kasai procedure, named after the surgeon who invented the operation, is usually the first treatment for biliary atresia. During a Kasai procedure, the pediatric surgeon removes the infant’s damaged bile ducts and brings up a loop of intestine to replace them. As a result, bile flows straight to the small intestine.

While this operation doesn’t cure biliary atresia, it can restore bile flow and correct many problems caused by biliary atresia. Without surgery, infants with biliary atresia are unlikely to live past age 2. This procedure is most effective in infants younger than 3 months old, because they usually haven’t yet developed permanent liver damage. Some infants with biliary atresia who undergo a successful Kasai procedure regain good health and no longer have jaundice or major liver problems.

The Kasai procedure

If the Kasai procedure is not successful, infants usually need a liver transplant within 1 to 2 years. Even after a successful surgery, most infants with biliary atresia slowly develop cirrhosis over the years and require a liver transplant by adulthood. Source: http://digestive.niddk.nih.gov/ddiseases/pubs/atresia/

September 16,2013

Today we met with the surgeon and scheduled the surgery for Thursday the 19th. Philip will be 8 weeks 2 days old. He will insert a camera first to confirm the suspicion of Biliary Atresia. There is a tiny chance it is not but most likely they will confirm it is and continue with the Kasai procedure as explained above.

There are so many mixed feelings getting a diagnosis for your child like this. Most are indescribable.

But the surgeon spoke today of a lot of feelings of having to mourn for the life you expected for your child and yourself and that struck a chord with me. It is so true on so many levels.

Our lives will forever be changed with a diagnosis like this and we will face many challenges over the days, months and years to come. We are so blessed though to have the family and friends we do who have shown insurmountable support, love, concern and caring over the last week. We thank you all from the bottom of our hearts.