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.

National Institute of Health Team Plan

January 10, 2015

The liver doctor called yesterday to review the team plan. They are suspicious of the blood vessels and immune system...they also want the panel of blood clotting disorders to be run. Patrick's liver function/ enzymes are good and not to blame for his health issues.  They want to review his liver biopsy slides and want a repeat ultrasound. 
Patrick will continue to need multiple endoscopes/bandings as this is the right therapy to prevent a serious bleed. He said the ultrasound will need to be done for consideration of either the Rex shunt or splenorenal shunt. He, in their evaluation, is a candidate but need to have those scans to see which direction they feel will be best for Patrick

They kept saying they need to do what is best for Patrick and that his condition is very complicated  which made me feel better that we didn't waste anyone's time. 

They really are focusing on the why for the portal hypertension and why was their a clot and what to do with it. The 3 other liver doctors felt it was not moving and would just stay there but this team has done research of the side effects of the portal vein clot. Even though Patrick's body has made the collateral vessels to help the blood flow around the blocked vein he can begin having bile duct problems and/ or growth development and cognitive issues. 
So thankful for our Charleston doctor not giving up on Patrick and passing everything off and pushing us to get where we needed to be. 
Thank you for all your vigilant prayers to open the doors of where we need to be and giving us the strength to keep going. And most importantly praying for Patrick ... In a sometimes scary place letting Patrick meet some really cool other kids and staff who make things a whole lot less scary.

National Institute of Health Day 2

January 8, 2015

Snow at the National Institute of Health

Liver/GI: it was great to get yet another insight into Patrick's portal hypertension. This doctor reviewed Patrick's reports and was surprised the varices from the first banding were so large for a child...and was a bleeder.  He felt the second bandings were a result of the first surgery because of the placement. They were presenting his case to the team of doctors and researchers. They want to review liver biopsy and possibly repeat. They know the clot is blocking the whole vein and need to get to the root of the problem (can I get an Amen)! He doesn't buy that we may never know and wants us to focus our attention on the liver even though the PH is a vascular issue. He wants to be sure the liver is an innocent bystander...try to resolve any of those issues before the liver becomes damaged. The Rex shunt (Chicago surgery) is a definite option but need to go down the road of how Patrick's varices will develop. The Rex shunt is very risky and although a cure not reversible. 
Hematology: had not planned on seeing them but the group wanted further evaluation and consult to begin treatment plan. She is urging that we work full panels for other clotting disorders...this will be done through Greenville. One treatment they wanted to consider was a blood thinner to help fevers/strokes...she would urge against it as his conditions are complicated. If he did this treatment it would put him at risk if he would have bleeding varices. 
They all said that Patrick is anomaly.
So plan..13 vials, urine specimen were taken for evaluation to get a control established. DNA tests being run with a year to review. Visit back in summer. Liver doctors will get back with us after meeting. Clotting disorders being run on Monday. 
Long week..hopefully some movement...

same place, different day

National Institute of Health Day 1

National Institute of Health

Email January 6, 2015

We met with nurses and doctor this morning as they drilled me on Patrick's Medical History... My charts of his fevers and the binder made them instantly impressed! Upon the arrival of the team of doctors...they were to discuss his case and then send one doctor back in...but they just piled in...9 doctors in a very small room. At this point it felt like I was in an interrogation room...very intimidated. I did feel a little star struck as well meeting the doctor whom is the leading researcher in this field. Patrick, in what this was the 5th hour, sunk in his chair and asked for Bear...this his security.  He was clearly not at all comfortable.
They repeatedly thanked us for joining the study where at this point I wanted to burst into tears thanking them for possibly some hope...
They did say he does not have TRAPS...he tested negative but 60% of the cases test negative. The reason the whole team wanted to meet with us is that there is a rare condition that echoes TRAPS but is not...they have 5 children who have portal hypertension, enlarged spleen, low platelets, diarrhea, and fevers...in order to determine if he falls into this is to do a whole genome/ exome sequencing (DNA test)...it will take a year for the study to be completed. And once this is completed it may not necessarily be a concrete answer being that there are only 5 cases. 
We are scheduled to see their liver doctor tomorrow morning  and hematology/ oncology in the afternoon followed by some blood work ups.
It is has been completely exhausting today and more of the same tomorrow but glad to have the opportunity to sit down with these specialists! 
Prayers for strength and calm...and for Patrick's will to continue...he told me he is going to crawl under the bed tomorrow so I can't get him.. Because I'm too big ya know!

EGD #2

December 5, 2015 (2 months after first)

Patrick had his second surgery today...thankfully his varices/bands from before had healed but new varices had formed since his last surgery in October.  Therefore, these needed to be banded today.  During recovery he experienced some bleeding problems.  It also took longer than anticipated for him to come out of it.  We will have to return for surgery #3 in a few months.  It appears that he will have to continue to have the varices banded because of the possible bleeding issues.  

Yesterday he was so excited to come to Duke and play games with the doctors but this morning he became hesitant and upon being wheeled out he burst into tears.  The nurse said that he is now at that age where it is all starting to make sense to him and he is aware of what is happening to his body.

We are scheduled with NIH at the beginning of January.  He had this DNA blood sample drawn last week and sent back to them.  She said they do the test on site at NIH but they work it in to the schedule to test and has been taking a few weeks to get back.  If the results come back positive it will be urgent that he begins therapy immediately, even before our visit with them.

EGD #1

EGD (Esophagogastroduodenoscopy) is a surgery used to treat varices that form --  they send a scope down to look at the esophagus, stomach, and small intestine.  If varices are found (of a certain size, or "grade") they will band them to cut off blood flow.  

Sent: Fri, Oct 10, 2014 3:31 pm
Subject: Patrick Surgery

Patrick's surgery went well.  They found the varices and needed to band them in the lower third of his esophagus.  She saw varices with wale signs...which meant they were about to bleed...no telling as to when they would have ruptured.  We more than likely would not have made it until November.  She could not completely eradicate them but they should slough.

She told us that this would be the first in a series of endoscopies/banding and would need to return in 3 months again to do this.  She took a series of biopsies to send off to test for cancers, celiac (which has already tested negative), sugar disorders...we'll know these results in about a week as she still cannot explain the diarrhea. 

Last week I contacted a doctor at the Children's Hospital of Chicago (the first in North America to perform this surgery) and he contacted me within 40 minutes....this surgery is the only possible cure to Patrick's portal hypertension but has some pretty steep risks.  The surgery is about 5 hours long and he would have to be an ideal candidate.  This doctor has performed this 200 times and has had a 91% success rate.  We need to follow up when we get back and forward all medical records.  

Patrick is doing well...snuggled up next to me drinking hot chocolate....love these snuggle times with my sweet little guy!

Thank you for the prayers....

Danielle and Crew

Children's Hospital of Atlanta's alternatives

Email November 21, 2014 

no...the portal hypertension just won't go away; we can't relieve the blockage-it is there to stay-"it is of historical importance;" Patrick's portal hypertension would be classified as 'serious' since he has already had varices and varices with red wales (signs of bleeding); the diarrhea is not associated; had no results about the maltase/sucrose/palitanase (frustrating) so couldn't really know where she was coming from in biopsy-ing this and wants her to follow through with this and then will discuss and create a plan; spleen was enlarged; these surgeries will not reduce the size of his spleen but DO NOT REMOVE.

We discussed the Rex Shunt, Mesa Caval Shunt, and other alternatives.  He does not want to do the shunts right now.  He wants to take the time to see if he will continue having varices.  The risk of the Shunts are very steep and hopes that the banding will continue to improve his 'quality of life.' There are beta blockers that he can give him but never recommends this...he said it takes their childhood away.  They become cloudy.  He emphasized to let Patrick be Patrick...let him be a boy as much as we can but aware of when he needs to be restricted.

We have still sent the records to Chicago in hopes they can evaluate and see if he truly would be a candidate for the surgery.  NIH is also scheduling us to see their liver specialists to follow up.  

Road less travelled . . .

In all the research I have done the Rex Shunt appears to be the only procedure to 'cure' or give Patrick the best chance of having a normal childhood...eliminate the EGD procedures.  Granted, the risks are very high, but when do those risks outweigh the chance to have the best quality of life.

The leading doctor, of this type of surgery is at the Lurie Children's Hospital in Chicago, IL.  I decided to email him....it was one of those grasping at strings times when I felt like I had no other road to take...not really knowing where this one would lead or if we could even go this route.  Within 45 minutes on October 6, 2014 I had a reply:

Dear Mrs. Daniel,
Please do not apologize for e mailing me. You are just trying to help your son.
In the last 15 years, we have treated almost 200 children here with portal vein thrombosis. Many kids are brought here from other states and other countries.
Your son has a typical history, and physicians from other centers often are unaware of the Rex shunt, or are not sure how or when it should be done.
We would be happy to evaluate him for a Rex shunt. If you call my administrative assistant, he can make the necessary arrangements and get all the necessary information. 
It sounds like you already may have made contact with some of our past patients, but we could help you communicate with designated parental representatives who could give you a parents' perspective on the program. We have, I am sure, seen kids from South Carolina, Georgia, Alabama and Florida in the recent past who may be geographically near you.
What I would need from you is CD's containing the MRI and a summary of his medical history. 
You could also visit our website to find out more information. 
https://www.luriechildrens.org/en-us/care-services/specialties-services/liver-transplants/portal-hypertension-program/Pages/index.aspx

Factor Five Leiden

October 13, 2014

We went to hematology/oncology today and his blood counts were low but normal for him...based on the counts he feels that Patrick's body is healing well after his surgery.  The doctor was a bit more concerned about the confirmation of factor 5 leiden.  Not many children are tested for this as it is rare for children to have factor 5.  He does want to run a bigger panel of blood clotting disorders on Patrick as there were concerns of the factor 5 in addition to other clotting disorders that could wipe him out completely (like a double whammy) and cause some deeper issues later with bleeding and even within the liver and portal hypertension.  

The liver doctors felt pretty certain that his clot, that completely blocks the portal vein (spleen to liver), had been there for many years ... this blood doctor is a little more concerned that it had not been apparent in previous ultrasounds and seemed to just turn up recently within the past few months on the MRI.  He is going to discuss this with radiology to see if it would be something that could not have been seen on the ultrasound.

And so it begins . . .

We went for a hike at Paris Mountain State Park.  Patrick was having a hard time and got real winded....even when I was carrying him.  And he tasted some blood and had some pain in his esophagus area.  They mentioned when the varices begin to bleed they may feel some pain...we called the Duke doctor.  Because of the recent symptoms and his blood panel results, Duke wants to have this surgery before November...she feels it is too long to wait so scheduled for 9/9 and 9/10 at Duke.  The biopsy will be done at the same time.  

Duke Hepatology and Hematology

Email September 11, 2014

The liver doctor at Duke agreed with the doctors in Atlanta.  Duke feels the endoscopy and banding of the varices should be performed as soon as possible.  She wants to do the endoscopy and while she is in there band if needed whereas Atlanta wants to see where the banding needs to be done and then come in another time and band.  She mentioned the high possibilities of ruptures while we are at home and would much rather us get him in to have this done to avoid this.  

She had put him on the schedule to discuss with the board tomorrow again where she wants to discuss him having the surgery on his portal vein.  The clot he has in his portal vein won't go away with medicine.  Right now his body is accommodating for this lack of blood flow but it also is putting a toll on his body and could have a high possibility of long term effects on his liver.  

We also met with the hematology/oncology team.  They feel that MUSC has been thorough and does not feel he is having any type of blood issues (except platelets).  His platelets dropped 40,000 since a month ago but still feel it is more related to the enlarged spleen and portal hypertension.  They said Patrick doesn't fit the factor 5 leiden profile and they don't normally run this on children but still worth seeing the results. He did not fit the criteria other than family history and it is not enough to go on.  They also are running another blood test to see how his bone marrow is functioning despite the bone marrow test....it wouldn't have shown up on this?  It is rare and not likely it will come back positive but is just one more thing to rule out.  We need to continue with a hematology/ oncology doctor to just keep monitoring his blood levels. 

Our biggest decision now is to figure out where we want to have the surgery done so pray for our decision.  

He slept halfway through the first appointment and halfway through the second appointment - 4 hours!  And then slept the whole ride home!  We still managed to get him to bed at 9.

Don't take pity on me . . .

Email August 29, 2014

I know all of you and everyone praying for us is concerned about Patrick, his doctors and our family.  It is the only thing that keeps us floating above the water.  Yesterday I had a really overwhelming feeling that I don't want to experience again...one of concern versus pity.  It's ok to be concerned for Patrick but please don't take pity on him...  

Patrick and best buddy Bear on the way to CHOA!

At the transplant center we were invited to go downstairs to a little event where they could build legos and even were able to get a lego kit to take home with them.  It was a great organization that was putting this on and these two guys were great with the kids...focusing on Patrick and not him being sick.  We had been so thankful for a great distraction of where we really were and what was really facing us.  As the nurse took him back up to our room, they patted him on the head said "see ya buddy" and saw it in their eyes...it was not that of pity but of true concern to give him a distraction from being sick at least for a day.  He didn't have to think of what he needed to avoid to not hurt his body or not be able to do something that could cause him to get hurt.  These big guys had tears in their eyes and wished for a day for all these kids to be not just healed but be normal.   Another kid that had been downstairs with us had severe burns on his face and these guys didn't take pity on him but made him normal for a brief moment....concerned with him and what happened but never to pity him....there is a difference between pity and concern.  Like I said...please be concerned for Patrick but do not take pity on him.  He is a blessing to us and everyone he meets.

Please keep us in your prayers as well as all the doctors and staff of these hospitals.....and say a thankful  prayer to God for these two big burly guys who are making such a difference to kids in the Atlanta area.

In addition I thank all of you for your outpouring of love and support...We have been blessed with great family, church, friends, employers (Grace and Sheriff's Department), and even people I don't even know. 

A great organization that offers Little Bricks of Hope
http://hopelaughplay.org/