Liver Life Walk 2015

Family and friends came together once again for the Liver Life Walk Long Island on April 26th.

It was another amazing day and I can't really seem to find the words to convey how appreciative and thankful I am for everyone who showed support by walking with us, cooking food, coordinating drinks, setting things up, taking them down and monetary donations. And a special Thanks to the FDNY Corona Tigers and  Massapequa Fire Departments, your efforts and support go way beyond what anyone could expect. If I didn't get to thank you personally that day, Thank You! Know that each and every one of you are deeply appreciated.

Philip's health remains stable at the moment and we find ourselves just playing a waiting game majority of the time.

Waiting for his next fever.

Waiting in doctors offices and emergency rooms.

Waiting for lab results.

Waiting for the next endoscopy.

Waiting for him to get sicker and need a transplant…

Just stuck, waiting in one spot, not able to move forward.

Participating in this walk has made me feel like I am doing something really meaningful. Something that can really help. Moving forward in a sense, while we are just stuck where we are. Waiting.

Together, you helped raise $7,743 and the donations keep coming in. 

You have made this as successful as it has been. 

Thank you again from the bottom of our hearts. We really can't say it enough for the support that has been show to our whole family.

Scary Words. Scary Thoughts.

Thrombocytopenia. Hepatosplenomegaly.

Thrombocytopenia. (throm-boh-sahy-tuh-pee-nee-uh) 

Thrombocytopenia is any disorder in which there is an abnormally low amount of platelets. Platelets are parts of the blood that help blood to clot. This condition is sometimes associated with abnormal bleeding.

http://www.nlm.nih.gov/medlineplus/ency/article/000586.htm

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Hepatosplenomegaly. (hep-a-toh-spleen-o-meg-a-lee)

Abnormal enlargement of the liver and spleen. Hepatosplenomegaly is typically associated with chronic liver diseases.
http://www.medicinenet.com/script/main/art.asp?articlekey=3716

Thursday, I took Philip to his pre-surgical testing appointment at the hospital for his endoscopy next week. We were sent away last week because he presented with a rash and low grade fever. Which turned out to be a reaction to a medication he was on. This week, All went well and while we were there I had his blood drawn because they always check his coagulation profile among other stuff before they do the endoscopy.
Portal Hypertension (which Philip has) causes the liver and spleen to enlarge (hepatosplenomegaly). 
Because of the abnormal blood flow away from the liver with portal hypertension, more blood is shunted to the spleen.  The spleen, unfortunately, traps the platelets and the measured platelet count in the blood is reduced. A low platelet count may be associated with an increase chance of bleeding. (http://www.texasliver.com/en/cms/?207)

They also check his prothombin time which measures how long it take s for your blood to clot.

Normal Platelet levels are usually between 150,000 and 400,000.
Philip's usually hang out between 100,000 - 130,000.

I received a call from his doctor yesterday that his level had dropped to 28,000, Yet his clotting time remained within a normal range. This means he is at a higher risk for bleeding. So we will have to go in a few hours before the endoscopy to check the levels again. And go from there. Also we need to be extra vigilant with him playing. No rough housing, wrestling, running, or hard falls. How can you keep an almost two year old from doing these things? I mean not like he will be wrestling but he does like to play with his brother and cousins. He trips and belly flops like 10x a day, climbs on furniture and what not. 
The morning the doctor called to tell me, he took a header off the couch and banged his head on the floor. Now I couldn't get out of my head that he was internally bleeding or something.
The doctor reassured me that if he is acting fine after it he is probably ok. That is regardless of whether he has low platelets or not.
Platelets tend to go up and down so Im hoping it was a fluke and they go back up. 
I just feel like he was doing so good and the wind was taken out of our sails. I tend to not think about all the bad stuff that can happen on a daily basis. I try to live our lives as normal as we can, but when they call and tell you something not so good it's hard to not have the scary thoughts that come with it.

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Too close for comfort...

Last Wednesday Philip woke up and felt warm but his temperature only read 99.9 so I went on with our day as usual. As the day went on my sister called and said he felt hot to come home from work to see what was going on with him. I took his temp and my heart sank…102.5. So I made the phone call to the GI and braced myself for the instructions to head to the hospital because he had no other symptoms like a runny nose or cough. (This made my heart sink even more because Dan's brother was getting married Saturday and I knew it would be a very close call as to whether we would make it to the wedding because they usually keep us for 48 hours when this type of thing happens.)

They would call ahead and let them know we were coming. 

We got to the hospital and I took one look at the waiting room and knew it was going to be a long day, I just didn't think it would be as long as it ended up being. So I set myself and Philip up in a secluded spot so hopefully no one would bother us. They called us in and triaged us and said they were looking for a bed for him and then sent us back out into the waiting room, where we waited…and waited…for 6 hours…

It's hard enough to keep a 20 month old occupied for 30minutes let alone 6 hours all while dodging children coughing and vomiting. People were everywhere and literally "too close for comfort".  Kids kept coming up to play with Philip and I kept asking are you sick? Please don't get too close. I just wanted to run us out of there. Philip was a trooper though and handled the situation amazingly. 

We finally were brought back and they started the IV and took some blood and a nose swab.

The nurse came in and said he was positive for corona virus(same thing he had when he was in the hospital for Thanksgiving) and his liver numbers were similar to what they have been. Phew! So it is Not Cholangitis. BUT…

The nurse also said His hemoglobin is low and asked if anyone ever mentioned that before?

Nooo…What does that mean?

Well the doctor came in and said they were concerned he might be bleeding somewhere…Wait. What?

His levels were fine last month when they took blood but somewhere in there something happened to make his levels drop.

Google.Google.Google. Low hemoglobin in toddlers...

One thing kept coming up.

Milk…Lots of it. 

And wouldn't you know my little Philip switched to Whole Milk just last month and was drinking lots of it.

Like Lots…30oz a day lots.

So the doctor came in and was explaining some things and trying to figure out the source and that they were admitting us to figure this out. So I mentioned the milk thing and he flat out said there is the source right there! He could possible have a milk protein allergy and it is irritating his insides or Toddlers who drink a LOT of milk tend to get iron deficiency anemia. SO they took some more blood to check iron levels and he is indeed iron deficient. So no more milk for my little guy. He has to go back to formula for now.

We stayed overnight in the hospital ER because there were no beds available. I actually didn't mind because it was a private room and if we went to the floor, I'm sure we would have had 1-3 roommates.

They were debating keeping us overnight again to check the hemoglobin but I told them about my brother- in-laws wedding and they decided to let us go home but return in the morning to get blood work redrawn but if his hemoglobin went down anymore, we would be re admitted right then and there. So I took Philip back to the hospital Friday morning for blood work and hung around to hear the results. After an hour, I called the doctor and he said Philip's numbers were stable and didn't get worse so we could go home! Never been so relieved! After spending Thanksgiving and Christmas in the hospital I didn't want the kids to miss another special occasion. We were all able to attend the wedding and had an amazing time. It was definitely the closest call we've had and by all means embodies the title of this post and was…

Too close for comfort...

Pillows, Pillows, Pillows!

This past week, Philip finally had his endoscopy that we have been postponing since October.
Unfortunately they found two moderate size varices they had to treat as well as a small one they left alone. So that meant another night in the hospital and a prolonged period without drinking or eating.
We knew this stay would be a little different because he has really started talking a lot and understanding things a lot more over the last few months. I knew it would be harder because he can express what he is feeling more like saying Ouch and scratch when he is really itchy.
We finally got into a room after waiting 5 hours in recovery then were switched to a different floor after a couple of hours in our first room. Once Philip was able to drink he wanted to walk around the hospital floor and didn't want to be in his room. So after making our 10th lap around the receptionist at the front said Hi Philip! Are you having fun? Well, Philip yelled NO! so fast and loud, all of the staff up front were laughing so hard and telling everyone about his come back.
Then our lovely nurse tried to put his baby lo-jack on and he told her to "goway"! I felt so bad but all the nurses got a kick out of him. He then proceeded to tell every doctor and nurse to "goway"! who came to see him. Except for one who came in with doctors and he was screaming to get out of the room and away from them saying "goway". She asked him if he wanted to go to the playroom and he put out his arms and went right to her. This is a kid who is extremely attached. He screams if I go to the bathroom at home and he doesn't see me. He did LOVE the playroom though so that was a major factor in that too. He went to sleep at a fairly regular time and I thought this is going better than I thought, but of course in true hospital fashion they have to come in and do vitals and woke him up at 10:30PM. Recently Philip has a "thing" for pillows and this was the first time they gave him a pillow in the hospital crib. So needless to say he was beside himself that he had a "piwow" and he wanted everyone to know he had a "piwow" at 10:30pm - 1:00am. He repeatedly yelled in excitement "piwow" over and over til he finally fell asleep on his "piwow". Our poor roommate. He probably kept them up half the night. LOL.
The next morning an Asian lady was outside our door speaking another language and I was holding Philip walking by the door. I can see he was interested and he all of a sudden started yelling back at her mimicking the language she was speaking! Like really loud!! I almost died!!
It stinks having to stay at the hospital but his emerging personality gave us some comic relief and makes the time go a little faster. The doctor said we can probably wait til the Spring to do another endoscopy, which is great because I can't do it every 2 weeks again. That was a nightmare.
His liver numbers continue to remain the same and fluctuate back and forth. It's his itching that is the most difficult to deal with at this point as nothing helps. We see the Liver specialists at Mt. Sinai next week so we will see if they will appeal for points or possibly remove him from the list all together. We are just stuck in a holding pattern right now as far as the transplant goes. We'll just keep enjoying his emerging comedic personality in the meantime!

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