Neutropenia

Neutropenia (noo-troe-PEE-nee-uh) is an abnormally low level of neutrophils. Neutrophils are a common type of white blood cell important to fighting off infections — particularly those caused by bacteria.
Some people have lower-than-average neutrophil counts, but not an increased risk of infection. In these situations their neutropenia isn't a concern. Neutrophil counts less than 1,000 neutrophils per microliter — and especially counts of less than 500 neutrophils per microliter — are always considered to be neutropenia, where even the normal bacteria from your mouth and digestive tract can cause serious infections.
© 1998-2016 Mayo Foundation for Medical Education and Research. All rights reserved.

I received a call from Philips doctor this week that his liver numbers were good but his white blood cell count was extremely low. This means he can catch something very easily. They asked if he was feeling ok or any fevers but Philip was fine. They said any fevers or anything g else out of the ordinary and we should go to the ER. They had hematology look at the blood and he had a little protections from another component of white blood cells but they were pretty concerned.  He had a ear infection about a week earlier and they seems to think it may be that it was viral and it caused his numbers to tank. They were only 120. So we've been repeating bloodwork every other day to see if it is recovering and to check for some specific viruses that can be pretty harmful for transplant patients. Those are still pending but his numbers went up from 120 to180 then jumped to 480. Which is good but still not normal. So we have to be extra cautious with him in general and around people and no crowds. Which is difficult this time of year because there is so much to do. We had to miss a Christmas party this weekend and He can't even go to school for now. I hate that he still has to miss out on things but it could be much worse. We are so thankful his liver is doing good for now. We repeat blood on Tuesday and hopefully it still recovers and he can resume activities as usual next week!

Thankful

Well this year has been crazy, and I'm not sure where to start
But there's so much to be thankful for
And all of you played a part.

In February, Philip became more sick
and we had some bad news
He needed a new liver
And now there was no time to lose.

It was time for a transplant,
 but so many kids were more sick.
So what could we do
to make his time on the list go quick?

Living Donation, they said
Could it be?
That Dan is a match
We'd have to wait and see!

Everyone sent us good thoughts
And some prayers
We couldn't believe how many people cared.

Then
Late March came around
Dan was a match!
Could this be the moment
That would make Philip not scratch?

It was May 10th
When they both went in
I held my breath
And my head would spin.

Again
You all came to my side
Lifted me up
And filled me with pride

They both did great
We count our blessings
Everyday is a gift
That keeps on giving.


I always worry I don't say Thank you enough for all of the support I receive from family, friends and strangers. Thank you!
I am thankful for my family, friends, Philip's doctors, surgeons and nurses.
I am thankful for every good day Philip has.
I am thankful for no more scratching, no more endoscopies, and no more Biliary Atresia!
I am thankful for modern medicine.
I am thankful for organ donation.
I am thankful.


If you're looking for a way to give back this holiday season, consider registering to be an organ donor. It is the ultimate gift.

https://www.donatelife.net/

6 Months

Six months ago today, we hopped in the FDNY Family transport van for the first time and headed into the city to face the unknown. Emotions were running wild, stomachs turning but we held on to hope and faith that both Dan and Philip would come through with flying colors. And boy has Philip emerged over the last six months as quite a colorful character.
Some things are becoming faded memories, like the waking every hour for itching. And watching videos like this one from the night before surgery, make me remember how marked his skin was and how his cheeks weren't always as big as they are now from the prednisone. He seems like such a baby in the video. He's really grown in leaps and bounds since surgery.

Some people have said, well now you can move on, forget it ever happened, and while he certainly acts like nothing ever happened and Dan feels like nothing ever happened, and I've wished and hoped it would be as easy as that for me, but these types of things stay with you. I live every day with the memory of all the discomfort he had to endure the last three years.
I mean, doctor's appointments are  becoming less frequent, less hospital visits and procedures, less medication, and it seems like everything is back to normal and I revel in every minute of good health, but I will forever have a child that has a life threatening condition. I continue to pull Philip back to me and look in his eyes to see if I saw a little yellow or if it is just the color of the room thats making them look like that. Or is his belly looking a little bigger than usual? Why is he bruising so easily? Does he feel warm? Did I wash that piece of fruit good enough? Did I cook the chicken long enough? When are the next labs so I can have a little piece of mind...
I wish there was some sort of live feed of blood work in him so I can make sure all is going ok all the time.
And as much as I limit my internet searching, the last couple of months, I've read about a few children with Biliary Atresia passing away. It breaks my heart that there are still children passing away from this disease. Some just get too sick too fast before they can get a transplant, and others pass waiting for organs because unfortunately there aren't enough and they are not eligible for living donation.
But with all that said, we couldn't ask for a better outcome so far and we thank our lucky stars every day that he was given the chance to lead a normal life. Prayers continue to be answered everyday, and we continue to be thankful for each and every blessing.

4 months post transplant(don't mind sticker marks from his hospital stay)

 Working on his car

School Picture

Community

I've been trying to find the right words to convey how I've been feeling the last few days but I can't seem to find anything that could do it justice. Words just seem flat and can't convey all of it.
People always say to me I don't know how you do it. You're so strong. But the truth is, you go into survival mode. You do what you have to do to get things done and take care of your child and family, regardless of how you feel or the cost. We've been very fortunate to be able to heavily rely on family and friends for support. Every one of you, our family, friends, and people in the community have lifted us up and carried us through the last 3 years. I can Picture the 4 of us kind of crowd surfing on all of your thoughts and prayers, just rolling over all the crappy stuff. That is how you get through it, and when you get to see it in physical form, it is truly overwhelming.
On October 1st, our family, friends and community came together and had a fundraiser to help us with costs we have incurred and continue to incur due to Philip's illness. It was amazing to say the least. We are so lucky to have everyone of you in our lives. The amount of people who came out and donated, to the friends, family and businesses who donated goods and services, it was truly a beautiful day.
We can't thank everyone enough. Know that we don't take a single thing for granted and all the help and donations are deeply appreciated. Know that we count our blessing every day.
Philip had an amazing day running around, playing with new friends, meeting new people. Watching him be able to run and play and have so much fun fills my heart with so much joy, its hard to keep it from spilling out of my eyes half the time. He loves life and is doing good. He loves school and looks forward to going. We are still trying to get his anti rejection levels back where they need to be but we are on the right track.
I want to say a special thanks to my sister for all of her hard work on the fundraiser and for always being there for me the last three years. Always by side and I definitely couldn't have gotten through a lot of stuff without her. And if we didn't get to thank anyone personally for coming, I apologize.
Massapequa is an amazing place to live and we are very fortunate that so many people in the community showed their support.
Special Thanks to The Heather Pendergast Fund, Massapequa Fire Dept., FDNY 138/289, Anne Marie Roth, Stacey Shortell, Beth Collins, Joe Maneri, Our families, friends and so many businesses in the community who donated. Please consider patronizing them while out and about.
Thank you again!!!

http://www.heathersfund.org

 Philip joining the band

 Enjoying the Joe Saladino Band

The best family pic we could get by the end of the night. LOL

Still here

We are still here at the hospital waiting for Philip's last dose of antibiotics tomorrow morning. The plan is to be discharged after that tomorrow. 

They determined he had a bacteria called klebsiellla. Luckily it was sensitive to antibiotics. They switched from the original antibiotic to one more tailored to this bug. It's fascinating stuff that they can figure out what each bacteria is sensitive too and what it is resistant too and tailor each patients treatment of antibiotics. I am thankful everyday that we live in these times and in a country where we have access to amazing healthcare. I am also so thankful for our team of doctors and their communication with each other between Mt. Sinai and Cohen's. We are so lucky they all go out of their way to check up on him and make sure he is getting the best care. We are fortunate that we have met basically everyone in the GI department at Cohen's so we feel comfortable with whoever is on call. His normal GI who has followed him since he was 6 weeks old and worked with us through finding out that he had Biliary atresia wasn't on this week but she took time out to stop by to check in on us. Philips pediatrician who also does not round at the hospital anymore, stopped by to check in. And when I emailed his hepatologist at Mt. Sinai frantically on a Sunday night with some fears hoping to hear from them the next day, she called me that night to put me at ease. He is on contact and droplet precautions so he can't leave the room, so it's been a long week trying to keep him busy. He gets frustrated easily and is just plain crabby at times. Who can blame him. Being sick has caused his prograf( anti rejection med) levels to go out of whack. It was 18.6 when it should be around 8-10. Levels like that can be very dangerous. It can affect the kidneys so it needed to be reduced fast. So they stopped it for a few doses, which is very nerve wracking in itself. His kidney function so far seems ok. Now his dose is all messed up and we will have to figure it all out again. They've had to replace his IV 4 times and he has to get blood drawn every morning which ensues in lots of screaming and croc rolling which usually takes about 4 people to help get the task done. When I say screaming, I mean horrific screams that has surely terrified all the other patients and staff on the floor. People literally come out of their rooms to see if he is ok.  Every morning we are usually woken up by the phlebotomist and I have to sit in bed with him in my lap and cross my legs over him to pin him down and basically put him in a modified sleeper hold with my hand around his neck and grabbing his opposite hand to avoid him seeing when they stick him and him grabbing the needle out of the phlebotomists hand. I'm sweating just thinking about it. But these things are necessary. There is no choice in the matter. That is the only way to truly know what is going on inside. All we can do is try to make the rest of the time a little enjoyable and less clinical for him. This is how we spend some of our time. 

We have visitors.

We play pet vet.

We hide.

Where's Philip?

There he is!

We play cars.

And watch videos.

And play more cars.

And that's not all...

While staying overnight for observation, the doctor woke me up to tell me that Philip's blood culture had come back positive for bacteria. Meaning he has bacteria in his blood. This explains more why he was so extremely sick yesterday. It was not just a virus but bacteremia as well. I feel relieved I decided to come to the ER instead of just the pediatrician and they started the antibiotics right away even before the positive culture.  He's been in IV antibiotics every 6 hours and it seems to be helping. His clotting was a little off so they gave him a vitamin K shot to help with that. Today we repeated labs this morning and are waiting to see if his blood grows bacteria again. He had an ultrasound to rule out and absess or liver issue that might be causing the infection but that looked all good. His liver numbers remain good as well. They have to now isolate and figure what the particular bacteria is in his blood so they can fine tune the treatment if needed. But we heard that anytime bacteria is in the blood they would automatically treat for 7 days of IV antibiotics. I'm hoping I heard wrong but looks like we will be in for a few more days. I'm worried that maybe he ate something that was contaminated or I wasn't diligent enough watching what he ate or played with and washing his hands. He is 10x better than yesterday and almost back to himself so I am hoping the bacteria has responded well to the antibiotics and it is not a resistant strain. Continue to pray for Philip and that he can bounce easily back from this. 

Well that didn't go as planned...

I used to love going back to school as a kid. Going back to school shopping at the flea market with my mother and Nana getting my share of leggings and of course My bright white Keds for good measure. Wearing my fall colored and way too warm outfit on the first day of school even though it was 89degrees. But it changed when I had Philip. September is when I heard the words Biliary Atresia the first time and when Philip had his first surgery. Those feelings from those moments swiftly changed my perception of September. Before I got to know more about the disease I didn't think Philip would be starting school with his peers. As I read about it more I readied myself for developmental and milestone delays that come with malnutrition and other issues that come along from Liver disease. But he surprised us at every turn and grew and developed and hit every milestone. 3 years later and after the crazy journey we've been on I started feeling excited again about Back to School season. I did my supply shopping early for the kids, but there's always that one item that gets you. Before you know it, It's the first week of September and all the stores are plucked clean of school supplies. I mean, Good Luck if you dropped the ball and didn't get the Paw Patrol lunch box your kid saw at the beginning of the summer as you said to yourself, I have plenty of time to get it. Now you've been to every store you can think of imaginable that would carry said lunch box, along with every other slacker mom only to find 500 of the OTHER Paw Patrol lunch box that makes your kid lose their shit when you suggest it as an alternative. How dare you! You pick out the perfect outfits for your boys, Your Facebook feed starts to be filled with First Day of School pictures and you can't wait to take yours. You plan for you and your husband to drop off and pick up your son for his first day of kindergarten and go to orientation for your other son going to Nursery school for the first time. You don't feel as nervous as other moms for your kindergartener. He's strong and adaptable, he'll get through it just fine. 

The one in Nursery is another story. He's been through hell and back. He's really never been in the care of someone other than family. And now, not only do I have to entrust him to his teachers, I have to entrust him to all the parents of the children in the class and now that I think of it all the parents in the community for that matter. I pray they keep their kids home when they are sick, pray they taught them to sneeze in their elbow, cover their mouth when they cough, and clean their hands often. I deeply pray they have vaccinated their children against so many illnesses that can cause real problems for an immune compromised child like Philip. Yes most of those illnesses were once "common" childhood illnesses but "common" is the important word. My child's health isn't "common" and these diseases can spell disaster in an immunocompromised person. Their bodies can not fight as well and rare complications may be more of a  reality than not. We rely on herd immunity. It's a very real thing my child depends on. Then the morning comes

and your little guy wakes up with a fever. You go solo to drop your other son off at kindergarten, and watch the other parents head into nursery orientation without you and your son. And realize he will have to go it alone for his first day at school now. You bring your sick son to the hospital to be checked out where they keep him overnight for observation, and you miss getting to pick your other son up from his first day at kindergarten. I am still so angry with Biliary Atresia. We've had to miss countless things due to this disease. I still hear of kids dying from this disease either waiting for a transplant or complications from transplant. Transplant has been an indescribable blessing that has given Philip a new better life, but is by no means a real "cure" for Biliary Atresia. It comes with its own demons as well. The virus he has really kicked his butt because he is immunocompromised. It's really just a normal cold virus but he was definitely not himself earlier. Fever, shaking, whimpering, aching all over. His coagulation numbers increased meaning it takes a little longer for him to clot. They had to give him a Vitamin K shot to help. We think maybe the stress of the virus caused that. His heart rate was fast and blood pressure low. He slept most of the day. They have him on IV antibiotics just in case. He is finally feeling better now. And hopefully we get to go home tomorrow.  This is definitely not how I saw this day going.