Unbelievable Support

We cannot believe the generosity....the first day our www.GoFundMe.com/PenniesforPatrick went live and everyone helped us raise over $1000 - IN ONE DAY!!  We celebrated!!

 And so our challenge begins...for every $1000 we make, until we reach our goal, we will find a way to celebrate and share with you!

Pennies for Patrick

At a time when everyone is overburdened with their own finances, and bombarded with great causes and financial requests, I ask you to help Patrick.  

I remember my dad gave me a huge glass water bottle when I was little and said to put every penny in it . . . quickly those pennies turned into quarters, then dollars, and before we knew it we could barely lift it.  

I know every day you get requests for help and know your hearts tug at all of them but please consider helping us get Patrick the surgery he needs to possibly beat this portal hypertension for good.  There is no 100% cure but this surgery is a possibility of a normal childhood, and an indefinite postponement of a liver transplant.  Dig in those seat cushions, under your seats in the car, every penny counts for Pennies for Patrick....help us fill our glass bottle!

http://www.gofundme.com/PenniesforPatrick

Take Me Back to Chicago . . .

Little did I know that this patch from my dad, given to me over thirty years ago, would have so much meaning.....

I was born in Arlington Heights, Illinois.  And, even though I lived there a short time, there has always been loyalty to Chicago...especially the sports teams...Da Bears, Blackhawks, Bulls, and even though the rivalry with the White Sox and the Cubs, we liked them both!

About 40 years ago, my mom made a monumental choice....a choice that saved my life.

A few years before my mom had given birth to my best friend, and sister, and had been told she would never be able to have another child, but God had another plan.  She became pregnant with me while on a prescription medication.  The doctor shared with her that due to that medication, I would not be a value to this world...I would be born deformed and possibly have mental issues and should abort me.  Those words shutter through my bones 40 years later.  My mom chose me...

And as my mom made a choice to save my life, we again are returning to Chicago to make another choice at saving Patrick's life and his childhood.  As my sister says, "it has come full circle."

In May, we will head to Lurie Children's Hospital for a final evaluation for Patrick.  We will spend 2-3 days in evaluation to see if his body can withstand the 6-8 hour surgery.  If the doctor feels we can move forward we will make Chicago our home for the next month.   They would graft a part of his jugular vein and then place it to allow the blood to flow back into the liver from the spleen and stomach/intestines.  We will commit to visit Chicago every three months for a year, every 6 months for 2 years and then yearly visits.

I never thought I would return to Chicago...and the road has been hard...but we welcome the journey with hope in our hearts!

And so we meet again . . .

Hello Fever . . . And so we meet again!  

Patrick began a fever last Thursday and it is still lingering today.  He gets these fevers every month and normally last 5 days.  Sometimes they are just riding the line just above 100.6 and other times can peak at 104 but mostly are about 101/102.

Patrick was happy this morning but now you can tell he feels pretty cruddy....he has gotten pretty used to these fevers and normally can just ride them out but gets a whole lot more tired (more than usual) and pretty irritable when he is tired.  He doesn't ever have any other symptoms...no sore throat, runny nose, stomach aches...he just gets real pale and and dark circles under his eyes.  

Doctors have tried to link this with his painful and ache-y joints and diarrhea but the joint pain is pretty random and the diarrhea is all the time.  

Tylenol and Ibuprofen don't help his fevers....they either stay the same or increase the fever rather quickly.  There are other treatments for Patrick but nothing worth the risks as they would increase the size of his spleen (which is already too big) and can have some risks with clotting.  Plus...they can make him turn into a hazy lazy little boy....or a monster of a little boy....two things we just couldn't have.


Charleston doctors ran for TRAPs and it was inconclusive as one element in his testing was on the borderline and the lab left it up for doctors to make the clinical diagnosis. NIH ran another test and discovered he does not have TRAPS or Mediterranean Familial Fever.  NIH has decided to enter him into a clinical trial and had his whole genome exome sequencing completed along with Andy and I.  We won't see any connections to this for another year.  We return to NIH for further monitoring in the summer.

And so, my friends, the fevers continue...

Hope in a cure . . .

February 6, 2015

Lurie Children's Hospital nurse contacted us wondering if we still would like Patrick to be evaluated for the Rex Shunt.  The doctor in Chicago is the Director of Transplant Surgery and is one of a few pediatric surgeons to perform this surgery.  He has performed over 150 surgeries.  The rex shunt could restore the blood flow back to Patrick's liver.  It, by no means, is an easy fix or easy cure....we would have a really extensive pre-op work-up.  We made it past the first round of candidacy.  I gave them the latest update with Patrick's last varice bandings and am hoping this will not slow down the process...in some cases they want to have varices that are controlled...not sure they think these are controlled since he has had them banded every 2 months since October.

The preop would be CT of his abdomen, labs, and a venogram.  I don't think he will have to have a liver biopsy but that had been done over a year ago.  The venogram goes through his jugular vein and takes a good look at his veins.  After this is all done they usually schedule the surgery.  The surgery lasts about 6-8 hours and they graft a part of the jugular vein and sew to the portal vein (liver) and the vein in his abdomen.  Most children stay in ICU for a few days and then transfer to a floor...we would be in the hospital for about a week and would need to remain in the Chicago area for 3-4 weeks after surgery.  Patrick would have a 3" scar on his neck and a scar on his stomach that looks like the MercedesBenz logo.

Ideally, the shunt restores the blood flow to the liver.  This doesn't necessarily "eliminate the need for a future liver transplant but can postpone it indefinitely."

There are some other surgeries that could divert the blood from his liver rather than restoring the normal blood flow through the liver.  Patrick's little miracle body has created these collateral vessels...but the blood flow does not go through the liver...which what I seem to understand could cause some scarring in his liver which in the long term could harm the organ.  After our evaluation with the liver doctors at NIH, the doctor there said if we do not work at fixing the problem we are risking his blood clotting (doesn't help with Factor5), growth and cognitive functioning.

We would follow up with office visits, labs, and radiology exams every three months for a year, then every 6 months for two years, then annually.

The surgery risks include:  bleeding, infection, organ failure (need to check on this one as just heard this from another parent whose surgeon mentioned to them), failure of shunt, non-reversible. The shunt can sometimes narrow and there is also the possibility of thrombosis (clotting) but this is why we would be monitored quite often.

The risks of not doing surgery:  repeated EGDs and bandings, bleeding of varices, internal bleeding, no cure, esophagus issues.

I think the NIH doctor best summed it up for me, "I think you should go up to Chicago to be evaluated.  Once he has been evaluated for a clotting disorder, it would be reasonable to proceed with surgical intervention to avoid long term complications. We touched on it when we met, but long term portal hypertension can cause problems with growth and cognitive development. To prevent life threatening bleeding, endoscopy with banding is a good option, but each procedure carries risk, and while the individual risk of endoscopy versus surgery is low, the cumulative risk may exceed that of surgery."

I never thought we would get to this point...it seems like so long we have been told to wait and see what happens but now this is becoming a reality.  The nurse said it is a big commitment but I feel we owe it to Patrick to get him the best quality of life for a little boy his age.  

EGD #3

Email February 13, 2015

Patrick before surgery

Patrick had a really tough time today. He began getting nervous last week about the anesthesia and just got worse this morning. The nurse we saw first was shocked and surprised as he normally is so happy-go-lucky. The gown even stressed him out.  They even had to give him medication to relax him before taking him back.

Mom and I waited and thought he'd get by without being banded but we were wrong...he had to be banded. She left one varice because it was too small to band.  We are due back in another 2 months for surgery #4.

This time you can tell that these surgeries are taking a toll on his body.  It took him longer to come out of recovery and even now he looks so pathetic.  He just wants to come home and is in and out of sleep.

(They did not bring us back until after an hour after surgery...we went back to recovery and waited another hour before we started to wake him up.  They say it normally takes about 30 to 45 minutes to become awake).

After surgery we were discharged and came back to the hotel when Patrick's fever spiked and he just became disoriented and we could not keep him awake. I called the doctor and they were immediately concerned about internal bleeding...his blood pressure kept dropping during recovery but they thought he just kept stirring during recovery causing this drop.  

Patrick's vitals were good but she was able to see something was wrong and that he looked like he felt "punky."  She checked the charts and dosages were ok and was unable to determine what was wrong...and again Patrick's case is 'complicated.'  We need to continue to watch him tonight...and need to check in with the on-call liver doctor through the night.  As we left she said she has been doing this for 20 years and Patrick is the only patient she has brought back in because she was worried about him. 

He is now awake...has eaten a little bit of soup but his fever continues.

More Dead Ends - vascular and hematology

February 5 2015

Today we saw the chief vascular surgeon at Greenville Hospital.  In his 16 years he has never seen a case like Patrick...Patrick is his first one.  

He said the type of portal hypertension that cause the varices in his esophagus is the most dangerous and the banding is the only treatment.  The endoscopies/bandings are necessary since there is no vascular surgery to cure the portal hypertension/vein thrombosis.  He reiterated that portal hypertension in kids is very rare and hard to manage.  Since there is no vascular option he suggests we continue the treatment plans with Duke and NIH.  

Hematology doctor called today and said that genetically there was nothing abnormal that came up to indicate Patrick has any other blood clotting disorders besides the factor 5.  The interesting thing is is that  the Factor 5 will thicken his blood but the portal hypertension makes his blood thin which then sends all of his blood counts into distress -- I guess they don't just cancel each other out to make things normal!  He also told us that the ultrasound showed no difference in comparison to his last one.

We head to Duke next week for surgery #3...Patrick is not looking forward to his surgery at all...he has already been getting anxious.

Please keep praying for Patrick, medical staff, and for the knowledge that they need to give him the best treatment for him in helping to have the most 'normal' childhood possible....he has great siblings, cousins, friends who understand 'how' to play with him so pray for these little people too.