Reflections...

Well I can't say that 2014 was "our year" and that I'm not anxiously awaiting it's demise in less than 24 hours.   But Thank you 2014, for opening my eyes and teaching us so many things in the wake of your passage. 
January - February taught Endurance, Courage and Faith.

March -April brought Frustration and Relief. 

May taught me about Generosity, Community and Support. 

June taught me to Live Life and Have Fun and about Love and Loss.

July - August brought Fear and taught Perseverance.

October brought More Frustration and Relief. 

November taught Patience, Humor and that Sometimes you just have to Let It Go! 

December taught me that Family is everything.  

Holidays come and go so quickly we shouldn't wait for them to show or tell the people we love how much they mean to us. We would not have gotten through this year without our family and friends. You've been there at the drop of a hat when we needed you and if I don't say it enough THANK YOU for everything you have done this past year and all that you continue to do for us.

I will try to be better this year at showing how much you are appreciated.

We Love You!

Sidelined...

Yup, Philip was sidelined.
We met with Mt. Sinai last week and they said this was a possibility. We were trying to go forward with Living Donor and they had said they didn't want to do it this early as well.

So, Just as he got into the transplant game, and was listed, we got word today that he has been de-activated from the list. Now don't get me wrong, this is a good thing. This means he is too healthy to be considered for a transplant at the moment but I can't help but feel disappointment and frustration. Its hard to put into words, but having him listed gave me a feeling that things were moving forward. Things were being done. We had a plan. I mean I fully understand not wanting to do it too early so he can grow more but we are pushing off the inevitable. I don't want to trade one set of problems for another but its hard to not feel like "Lets get this show on the road" and start our next chapter and give him some relief. Why wait till he gets more sick?
You would think it would be better to do while he is fairly healthy. But they don't want to put him or the living donor at any unnecessary risk.

And by too healthy, I mean by the numbers. The parameters for listing don't take into consideration the varices in his esophagus that had to be treated again after the last free and clear one or that he needs to have yet another endoscopy again next week.
It doesn't take into consideration how the poor kid rips his poor legs apart from them itching so bad, or the sheet burn on his nose from rubbing his face back and forth to scratch his face from the itching.
Or how he is up multiple times at night crying, sitting in the dark scratching himself.
The doctors need to appeal for these to be considered, and they don't feel it is necessary at the moment.
So we will continue to wait it out and learn more about the transplant game and just be grateful and enjoy that he is still happy and healthy at the moment, as we've learned that it could always change with a  drop of the hat with this disease.

The List...

I love this time of year. I used to look forward to the shift in weather, school starting, new clothes. A new beginning so to speak.

But this year, with the shift of the weather, it brings up some different feelings.

A year ago this past week was when I first heard the words Biliary Atresia.

I remember standing in the doctors office with Dan and my sister and listening to the doctor explain what they thought Philip had and that ultimately he would most likely need a liver transplant. 

I remember breaking down crying in the office, not knowing what the future held for my newborn baby boy. I remember seeing the doctors eyes tear up. I remember my sister holding me and saying we'll all get through this together. There were so many questions racing through my head. What kind of life will he be able to lead? Will he be normal? Is he in pain? Why him? Why us? How the hell am I going to deal with all of this? I remember handing him over to the OR nurse when they did his surgery, then Dr. Prince coming out and saying it all went well, checking each diaper with bated breath hoping there would be some color to his poop to show that the surgery worked.

…And here we are a year later, facing the exact thing I most feared when we first heard about Biliary Atresia. 

A liver transplant.

And to be honest, it hasn't been as dramatic as I had envisioned the whole process. We have been SO blessed that Philip has done so well this past year. Dont get me wrong, he has had some major hiccups but compared to most kids with his disease, he is doing great.

The last two fridays were spent at Mt. Sinai going through a transplant evaluation, which consisted of meeting with Social Work, Nutrition, Infectious Disease, Cardiology(EKG and Echocardiogram), Surgeon and will be getting a CT scan in the next couple of weeks. 

Well Sunday, we got a call from our transplant coordinator that Philip is now listed for a transplant. His PELD score is -4. He is inactive at the moment because they are waiting for the CT scan but he will gain points for just being on the list.

What is PELD? How does it differ from MELD?
Candidates age 11 and younger are placed in categories according to the Pediatric
End-stage Liver Disease (PELD) scoring system. Again there is a Status 1 category
for highly urgent patients, representing about one percent of those listed.All other candidates in this age range receive priority through PELD.
PELD is similar to MELD but uses some different factors to recognize the specific
growth and development needs of children. PELD scores may also range higher
or lower than the range of MELD scores.

The measures used are as follows:

•bilirubin, which measures how effectively the liver excretes bile;

•INR (prothrombin time), which measures the liver’s ability to make blood clotting factors;

•albumin, which measures the liver’s ability to maintain nutrition;

•growth failure; and whether the child is less than one year old.

As with MELD, a patient’s score may go up or down over time depending on the
status of his or her disease. Most candidates will have their PELD score assessed
a number of times while they are on the waiting list. This will help ensure
that donated livers go to the patients in greatest need at that moment.
SOURCE:http://www.unos.org/docs/MELD_PELD.pdf

So since PELD scores are based solely on those measures, Philip's score is so low because he is fairly healthy. But these measures don't necessarily show how sick a patient is. So they can appeal for more points if Philip's health starts to take a turn for the worse.

Unfortunatley, New York has one of the lowest rates for organ donation in the country. So who knows how long Philip will have to wait or how sick he would have to be before he could receive a liver. So we will be going forward with hopes that me or Dan will be able to be a living donor for him. We will start that process in the next few weeks. The screening process for living donors takes 6-8 weeks and if one of us is a match, he could be transplanted soon after.

Some Organ Donation Facts:

• 22% of New Yorkers age 18 and over have enrolled in the New York State Donate Life Registry as organ. tissue and eye donors. Nationwide, the average is 47%.

• On average, 18 people die every day while waiting for organ transplants in the U.S., and every 10 minutes, another name is added to the waiting list. In New York State, someone dies every 15 hours waiting for an organ transplant.
 See more at: http://www.donatelifeny.org/about-donation/quick-facts-about-donation/#sthash.X6jINCoC.dpuf

A Small Victory!

When I was younger I used to like to jump the waves at the beach, but this one time, I guess the water was too rough and a wave came and blind sided me, knocking me off my feet, sending me tumbling under the water. I remember the pressure of the water pushing me down but I wasn't in that deep of water so I kept hitting sand. I finally caught my footing, stood up and BAM! another wave sent me ass over head again under the water, tumbling, fumbling to gain footing. I got my balance yet again and was able to outrun the incoming waves to the shore. This past year, particularly the last few months had me thinking about this feeling.  Just this feeling like I couldn't catch my breath, one hit after another. The bleeding episode, the endoscopies, hospital stays, doctors appointments, the passing of nana.

Then Tuesday morning we headed back to the hospital for Philip's third Endoscopy in a month. It seemed to take longer than usual but we were elated to hear that they did not find any more varices they had to treat. There is one small one but it is not raised so they didn't treat it. So Philip was able to drink right away and we were able to come home about an hour after the procedure. A small Victory that felt so sweet! Finally, a break in the waves even if it is short lived, it is just enough to make it to the shore and catch my breath a bit.

Groundhog Day

It's starting to feel like that movie Groundhog Day over here.

Tuesday, Philip had to have another Endoscopy done to see if there were any more varices in his esophagus.

So the Friday before I had to take him for blood work, then Monday for Pre-Op testing because his procedure needs to be done in the Operating Room because of the risk of bleeding I guess.

So again, no food, but this time we got to wait in the ambulatory surgical waiting area so he was able to play with toys and walk around so he wasn't as irritable. Just the last half hour before.

I carried him to the OR and stayed till they put him to sleep. He wasn't happy when they tried to put the mask on and fought it so I put my face close to his to talk to him and give him a kiss. The nurse then said "don't breath in too deep Mom, we don't want you to sleep as well!." LOL Oops.

It went well but they did have to treat another varix, so that meant a stay overnight. 

When the varices are treated(collapsed), the blood looks for another path and in turn can cause more pressure and others to pop up. So they need to check every two weeks until they do not find any more.

The point I guess is to treat the varices before one can rupture and cause bleeding again.

They were slow with discharges, so we were stuck in recovery for about 4 hours. He was able to drink pedialyte that night and boy did he take advantage of that! The nurses were laughing and saying we should put a keg of it in his room or give him one of those silly beer hats with the straw tapped into the pedialyte. 

His belly was a little more distended than usual but I think it was from gas and all that pedialyte. He also had some blood in his stool so they checked his blood work to see if all was good, which it was. We were able to come home the follwing day.

We will now repeat this process in two weeks...
And every two weeks after that if more are found.

A lot has been going on lately and I'm finding it harder and harder to keep things in order and in place. So many appointments, medicines, people to call, questions to ask, my head is spinning. I feel bad because I feel like I cant give 100% to anything. I used to be one of the most reliable people at work but feel like a flake lately, coming and going unpredictably. And when I am there I am on the phone constantly with doctors or the insurance company. I've been very fortunate that my boss has been very understanding and allows me to come and go when needed.
And forget housework. Thank goodness Dan does most of it because we would all be wearing dirty clothes and mismatched socks if it weren't for him keeping on top of the laundry. The kitchen table is forever piled high with mail to sort through and I can never find my keys or phone. But to be honest, when I get home I'd rather lay on the floor and play with the kids or put the TV on after they fall asleep and binge watch Breaking Bad and I really don't feel guilty about it. LOL We like to say our house is "lived" in. So I'll keep tripping over some toys, searching for my keys and praying no one pops by for a surprise visit. I'll get to all that stuff eventually.

So if you can, please pray and think good thoughts that they don't find anymore varices the next time we go so Philip can take a break from this stuff for a bit and just enjoy being a curious 1 year old.

The Sixth Sense...

Last week I was on and off the phone several days with Philip's GI doctor (Dr. Webster) for various reasons about appointments and medications. And after all issues were resolved she asked is there anything else? Like she knew I had another concern. 
*Philip has portal hypertension and we've been monitoring him closely for that. We've been speaking about possible bleeding esophageal varices.

• Portal hypertension is an increase in the blood pressure within a system of veins called the portal venous system. Veins coming from the stomach, intestine, spleen, and pancreas merge into the portal vein, which then branches into smaller vessels and travels through the liver. If the vessels in the liver are blocked due to liver damage, blood cannot flow properly through the liver. As a result, high pressure in the portal system develops. This increased pressure in the portal vein may lead to the development of large, swollen veins (varices) within the esophagus, stomach, rectum, or umbilical area (belly button). Varices can rupture and bleed, resulting in potentially life-threatening complications. SOURCE:http://www.webmd.com/digestive-disorders/digestive-diseases-portal

Picture Source SOURCE: http://www.smcgi.co.kr/endoscopy/liver/liver.html

So at the last minute I said to her that I was starting to get paranoid about a bleeding episode and what I should do. So we spoke about it and had a plan in case it were to happen.
If it was a lot of blood and he was pale and not himself call 911 ASAP and go to the nearest hospital. If his poop was black but he was acting fine, I could drive to the ER myself. Assess the situation and make a decision. Fast.
I guess in some way(my sixth sense) I just knew it was going to happen soon...

And well, It happened. 

WARNING: Description May Be Graphic
Monday morning I heard Philip wake up on the baby monitor around 7:30am so I let him play in his crib for a little bit like I do every morning. I could hear him jabbering and cooing with an intermittent cough here and there. I got up and got myself dressed and ready because he had his 1 year well visit that morning. I finally went in to him around 7:50 and saw what I thought to be an exploded diaper. The "poop" was sooo dark though, like almost black. "Poop" on his face, arms, legs, all over the crib... EVERYWHERE.
---->Cue the guilt and frustration that I didn't go in earlier. So I turned on the light and Instantly saw there were blood stains on his sheets and large clots of blood mixed in with what I still thought was poop. (Variceal bleeding can show up in poop or vomiting) And to tell you the truth I didn't think it was vomited up because in my head I though when it happened it would be like that scene from the Shining with the blood pouring out the doors( I know. I'm nutty.) and he would be limp and pale. He just looked up at me with a smirk like "What. What did I do?" So panic was starting but He looked and was acting great so I grabbed him and threw him in the tub. I took off his diaper and to my surprise it had poop in it and it was his normal yellow color. Now the panic really started. Hands start shaking realizing that it was thrown up and this was it. He was having a variceal bleed. It was actually happening. I made the decision to drive him to the ER myself instead of an ambulance because he was not actively vomiting and he looked and was acting fine and I wanted him to be at Cohen's Children's Hospital so he could be treated right away instead of waiting for a transfer to there from a local hospital. So,  I grabbed a sample of the vomit and his diaper to bring, took a picture of the crib, grabbed Danny bare foot and all and started calling people on my way to drop Danny off and to notify his doctors we were on our way to the hospital. Special Thanks to my future sister-in-law Meghan for taking the crazy ride with me as we got stuck in traffic and missed the exit due to too many people calling me at once and climbing into the back seat mid trip to console Philip. (If this happens again I WILL be calling an ambulance, lesson learned) Docs said he was totally fine that I did the right thing but It was not easy trying to concentrate to get there. So they took us right in and I learned that generally when varices bleed it is a lot more blood than what Philip vomited. (Hence always thinking it would be like the Shining reference in my head) So they would be doing an endoscopy to see what was going on. They came with a crib to the ER to transport him to the OR for the test and we told them he would not lay down in the crib because he was very irritable from not eating. They said well that is the policy so we laid him in there screaming and lo and behold he tried to stand up and was almost falling over. So we laid him back down and they moved the crib and he did it once more and it was even worse this time. He literally stumbled over. So my sister yelled stop and we said this is not safe. You can't transport him in this. So we convinced them to put me in a wheelchair and hold him because we weren't going to go any other way. They took him in for the test around 2:30 and were done around 3:30. She had said it would take around 10-15 minutes or up to 45 minutes depending what they find. It could be just irritation, an ulcer or the varices. So when it took that long, me and my amazing sister who stayed with me all day figured something was going on. (Dan was in a class and we didn't want him to have to retake it so he stayed till it was finished then came after). She came out and said they found one esophageal varix that could have caused the bleeding. Its not always obvious that they have bled. But it was enlarged and angry looking. His was probably oozing which is why it wasn't bleeding profusely. He also has some smaller ones more toward his stomach opening but they don't treat those. So they injected something into the vein to collapse it and scar it so it wont bleed. That's not to say it won't bleed again or others might form and bleed. So after recovery we had to spend a couple of days in the Pediatric Intensive Care Unit because of a certain medicine he needed to be put on. He also was not allowed to eat until late Tuesday afternoon. Needless to say he was not a happy camper. Monday night into Tuesday was the roughest night and day I ever had with him. Dan was scheduled to work and we want him to be conservative with taking time off so he can take plenty off when transplant time comes. So I spent that night and day by myself. Thank God my sister was working Tuesday and got to visit me a few times. There was no consoling poor Philip. He was throwing his head back, arching his back and screaming. I didn't feel safe holding him but he didn't want me to put him down either. And he was connected to a zillion lines as well so he kept getting so tangled. And he was so itchy on top of all of that. They were trying to find a compatible medicine for IV use to help with it. He couldn't take anything orally yet so it had to work with IV and the other medications he was on. Finally at 3:30am we tried Benadryl and he slept for an hour or two. 
 Philip finally was allowed to eat that afternoon, He did a complete 180 and he was his normal self, charming all the nurses and everyone who saw him. Like nothing ever happened. He continued to eat well and they weaned him off of the medication and we were able to come home Wednesday afternoon. You would never know anything like this happened to him looking at him now. He is back to his smiley self.

All that being said, We will be going  to Mt. Sinai on Monday to meet the Liver specialist and start the transplant conversation. I will update when we figure out what the situation is as far as that goes. Him having had a variceal bleed pushes him a little closer to a transplant.

I also want to send out a Thank you to the lady who refilled the supplies in Philip's room Tuesday Morning. She asked how we were and I said well it was a rough night. She said "Well today is a new day and it will be great. Good things will happen today."
It was exactly what I needed to hear at that exact moment. 

525,600 Minutes...

How do you measure a year?  A crazy year at that!
Today Philip turns 1!
He loves to play with his brother and cousins, he pulls himself up and cruises along furniture.
He loves to bang things. And to nod Yes! He says mamamama, dadadada, and nananana. He doesn't really like baby food and is just starting to tolerate a little texture. He still loves his bottle! He is still very happy and has the best smile. He can light up a whole room with it. He has brought us so much joy and happiness and we can't imagine life without him.
It's bittersweet looking back one year today as we hurried to drop off Danny at Dan's parents house, my water breaking in the car, freaking out that I was going to have the baby on the side of the highway with no drugs. Getting to the hospital and telling everyone I had to push but was really only 4cm, The doctor delivering him and yelling he has to be 10lbs!, then the nurses weighing him and yelling that he was 10lbs 5oz! We were so happy, oblivious to the shit storm about to hit us just a few weeks later. Everything as we knew it would be turned upside down.
This year as a whole has been a rollercoaster ride full of too many ups and downs that nobody should have to endure, let alone a little baby. The hospital stays, the poking and prodding, the endless taking of medication, you name it…I know. I know.  This doesn't sound like my usual upbeat always look on the bright side self but I'll be honest … I'm mentally, physically and emotionally exhausted. The recent passing of Nana has been pretty rough on everyone in the family. It makes me sad that she wont be here to celebrate Philip's First Birthday with us and that he didn't get a chance to get to know her like Mike, Ava and Danny did, but she will be here in spirit and I'll make sure to have an extra piece of cake(or two) in her honor. LOL

Now for and update on Philip's health!
The past few months Philip has become very itchy. He scratches his arms, legs, chest, back, ears, eyes and head. He is up every 2 hours at night scratching himself. The GI sent us to the dermatologist and allergist who both said it was eczema. She wanted to rule out pruritus from anything liver related because his bilirubin wasn't really high enough to suggest it would be liver related.

Pruritus. Pruritus is caused by bile buildup in the blood and irritation of nerve endings in the skin. Prescription medication may be recommended for pruritus, including resins that bind bile in the intestines and antihistamines that decrease the skin’s sensation of itching.
Source:http://digestive.niddk.nih.gov/ddISeases/pubs/atresia/

So we slathered him up in creams and ointments to no relief. So our GI did another blood test and his bile acids came back elevated. So we need to start him on another medication to see if it can help relieve some of the itching. We do however get to stop his antibiotics TODAY!!!! He has been on them since his surgery at 8 weeks old. They have stained his teeth grey and I can't wait to be done with that sticky mess! His spleen is also more enlarged and his platelet count is a little lower (meaning the spleen is holding onto more platelets making it larger). He also gives us a really hard time eating solid foods. He will tolerate some baby food. But when he's had enough thats enough. He has a tendency to gag and throw up a lot. He is getting a lot better but if he has something with a little texture its not a good situation. He is just starting to try to bite into food and he wants it but if he gets a piece off and tries to swallow, he gags and throws up. I feel so bad for him because he looks at you eating and tries to grab it but he just can't tolerate it going down. We have an appointment with Early Intervention to see if he can qualify for some kind of feeding therapy but they said most likely he won't because his development has not been affected by it. So with all that being said we have been referred to Mt. Sinai in the city to see the liver specialists. We visit them mid August and will report back with more updates.

Happy First Birthday Philip! So, How DO you measure a year? Just like the song, measure it in LOVE!
Philip, You are SO loved and we have been so blessed this past year to have seen it through all of the support of our family and friends.
May the world always meet you with as bright a smile as your own!
We love you!

Nana Bunny

On June 15, 2014 the world lost one amazing person. It's hard to begin to describe what an amazing person my Nana was. She had this unique quality that made everyone she met feel special. She was warm and generous and caring. If you came into her house, you best believe you were going to be eating something. Whether it was her famous meatballs, pizzelles, eggplant, zeppoles or anything she could find to offer. And if you were a kid visiting, forget it. You'd be sure to leave with a goody bag full of candy and little toys from the "candy lady". She lived life to the fullest and had a great sense of humor. She loved a good party and was quite often the life of it. Always fun to be around. I'll always remember something would happen making her begin to giggle and it would be so contagious that me, my sister and mom would start in too and it would go on forever, the four of us giggling till we cried.
She was so special to so many people but be sure that so many people were special to her. Looking through pictures we found that she kept every picture anyone sent to her, whether it be a wedding photo, a child's school picture or Christmas picture, all in photo albums. She loved being with her sister and seeing her nieces and nephews. I'll miss seeing her sit at her table when I walk in the door at my mom's house or her saying to Philip "give me your hand!" We were so blessed that we got to spend so much time with her and I am thankful that my boys got to share in some of that time. I am comforted with the great happy memories I have with her and my family. She will forever live in my heart.

The Rally…in more than one sense of the word.

Rally 1...
On May 18th, our family and friends gathered together to walk for the Friends of Philip team in the Liver Life Walk Long Island at Eisenhower Park. When I joined up for the walk I never thought it would turn into what it did. I thought a few friends and family would join me and we would have maybe a 10 person team. Well our family and friends rallied together and close to 200 people came out for the walk and raised to date $15,282.14. And the donations are still coming in. It was an amazing day. The weather was perfect and we had an amazing picnic thanks to the FDNY and Massapequa Fire Departments. I was filled with emotion seeing how many people came out to show their support for us and our family. I still get teary eyed thinking about seeing the crowd of Friends of Philip shirts and Philip cutting the ribbon to start things off. It is so easy to feel alone with the whole situation and that just reinforced that we are and will never be alone on this journey. We even made some new friends and met some others who had Biliary Atresia and had transplants and are now 20 somethings in college. It was an incredible experience from start to finish and I hope to continue to raise money and volunteer for the American Liver Foundation. Maybe some day there will be a cure or a way to screen for the disease so children can get the help they need earlier, giving them a better chance of living a longer life with their native liver. Thank you from the bottom of our hearts again to everyone who participated and helped with organizing this amazing day. I am truly humbled and will never forget the day.

 Rally 2…
A few days after the walk we saw Philip's doctor and she noticed his liver and spleen were a little larger than before. I've also noticed him being a little more distended. So we had some blood work done and she told us that maybe it was time to pick a transplant center to meet with. If just to start the talk and get to know them and them know us. So there we were researching between Mt. Sinai and NY Presbyterian driving myself crazy because I felt that whichever decision I made I was deciding his fate. If God forbid something doesn't go right it will be my fault for choosing that center and doctor.
Anticipating his bloodwork would get worse, we waited for the doctor to call.  Well, we spoke last night about the results and somehow Philip rallied and it improved!
OK maybe improve isn't the right word but it got better by a few points on a few and stayed around the same on others. SO the doctor said with it staying the way it is, it is still too early to meet with the transplant team. Philip continues to be a fighter! Amazing news! We'll take it as long as we can.

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!