Tuesday, May 22, 2018

Home sweet home!

We are so very, VERY glad to be home. The procedure this morning started much later than we anticipated.  We got there for 6:15 this morning and they didn’t take Parker back until 10:00. Thankfully this time around they gave him some happy juice before taking him to the back and he handled things much better. Two hours later, the nurse came out to take us to him, and while he was fine health wise, he was not coming out of the anesthesia well. He experienced a form of post-operative delirium which took us a while to get him out of. He was not a happy camper and he let everyone know about it both verbally and physically. Mom and dad definitely didn’t like this one bit; however, I’m happy to report that he’s back to his crazy, silly self and doesn’t remember a second of it. Now we wait for the doctors to review his results and make a plan for him.

Monday, May 21, 2018

A plan.... with a little more waiting

So today we met the team at Texas Children’s and let me just say... WOW! When you walk into a building and are impressed just by the waiting area and clinic organization, that speaks volumes. The entire place is efficient and organized! We spent a good bit of time talking with Dr. Himes today, and we officially have a plan of action. They asked us to stay one more night so that we can get an MRV done to take a look at the vessels in his belly to determine what type of surgical shunt he is a candidate for. This is a test that we cannot get done in New Orleans as they do not have the machines we need for this there. So tomorrow morning we will report in to the outpatient unit for 6:15 am, and he will be put under sedation to have this test done. 

Today we found out that essentially Parker’s liver itself functions just fine according to all of the tests that we have done. His portal hypertension, however, is the problem we need a solution for. Parker’s liver disease is Congenital Hepatic Fibrosis of the liver. Fibrosis basically just means hardened. His liver is SO hard that the blood cannot flow through the portal vein (which goes through the middle of the liver back to the heart). Instead of the blood flowing freely, the liver is constricting the flow, and the blood is backing up and pooling into the spleen. This is all stuff that we knew already, but to hear that his liver is functioning great otherwise and that his bile ducts are in good condition, makes us feel much better. His bile ducts are dilated but they are not misshapen, therefore the bile is flowing well and he is not at increased risk for cholongitis, which is a major infection of the bile ducts. The doctor did however confirm what we already knew. One day, Parker will need a liver transplant. Things will continue to progress to that point. The good news is that he feels that today is not that day. 

So far our plan of action is to complete the MRV tomorrow. The liver doctors, surgeons, and radiologists will the convene a meeting in the coming days once they have the results to determine which shunt is the best option for him. We will come back here after all of that to meet with the entire team, including surgeons and radiologists to discuss the plan that they choose. During the same visit, Dr Himes will perform an outpatient Endoscopy under anesthesia to get a better look at the esophageal varices that they found in the MRCP last week. He will also treat these varices in order to prevent any complications that could arise due to them. Esophageal varices are vessels in the esophagus where there is increased pressure. Basically the blood flow in these vessels is restricted due to the hardened liver, so the vessels are enlarged and full. If the pressure becomes too much, and the varices aren’t treated, those vessels could rupture causing internal bleeding. This could be very dangerous and life threatening, because sometimes the bleeding can not be stopped. 

Once the doctors have treated all varices and come up with a plan for the best shunt operation for Parker, we will essentially continue to wait. From our understanding, the shunt will not be necesssry until his platelet counts reach the 10s-20s. Right now, his platelet count is holding steady around 45. The concern, and reason we are formulating a plan now, is that Parker’s numbers drop dramatically each time. Last year at this time his platelets went from 120 to 75. Just a few months ago his platelet count went from 75-45. If he drops that dramatically this time, we are absolutely in range to need the shunt immediately. So the entire team will be on the same page so that when the time comes to execute the plan, we all know what to do next. 

So, yes we did get answers today. And yes we have a plan, which makes us feel MUCH better. However there is still a lot to do. 

Thank you to everyone who has helped at home and picked up our slack at work while we are on this journey with Parker. We truly appreciate all of it! Also thanks to everyone who has checked in for updates and sent prayers/thoughts/well wishes! It means the world to us! 

Monday, May 14, 2018

Texas, here we come...

So as most of you know, Parker’s labs the last few weeks haven't been so hot. His kidney function still looks great; however, his immune system left the building, and another major concern has been the fact that all of his blood counts are super low. Platelets, hemoglobin, hematocrit, white blood cell counts and red blood cell counts aren't even close to the normal level. They are even lower now than before the CMV diagnosis.

On Tuesday of this week, we were called into an emergency transplant clinic appointment where Jason and I planned to go in with our guns blazing. We felt like the big picture was being pushed aside for all of these "maybe" situations. For a year now we have been dancing around the fact that his liver disease has been progressing. We completely understand and respect that his team is being thorough, and ruling out all of the minor causes before doing any invasive procedures, but we were ready for answers. We have always known, and have been told, that CHNOLA cannot accommodate Parker's liver needs now that we have progressed to this point, as they do not have the technology or expertise here for the proper testing and procedures that he will need. So before going into this appointment, we researched the best hospitals for us to seek second opinions on, not only based on their level of expertise, but also what was best for our family. We had our top 3 picks ready to discuss with them, and ask their opinions on. Well to our surprise, they beat us to the punch. Our transplant doctor quickly came in and said the same things we had been thinking. He agreed that we could be tip-toeing around the bigger picture AND he began recommending to us one of the hospitals already in our Top 3! It was like the heavens had opened and everything aligned. 

On Wednesday, Dr. Arias, his liver specialist in New Orleans made the appropriate phone calls to Texas Children's and discussed Parker's case with them. They spent a lengthy amount of time going over everything from the beginning, and Dr. Himes feels like Parker needs to be seen in Texas immediately. By Thursday I was on the phone with Denise, the coordinator, who is amazing! She is about as thorough as they get, and definitely my type of lady. She gets things done and doesn't leave any stone unturned. We got records from all of the necessary fields of expertise who have seen Parker or run tests on Parker, including the Operative Report and Discharge Summary from transplant. We ran insurance approvals and got Parker registered in their system. We also found out which tests that Dr. Himes wants done here in New Orleans before we see him. So while this is all going on, Jason and I thought we had a few weeks to get everything together and done based on what they were telling us. Then today happened, and it's almost like a tornado came through! 

Dr. Arias' nurse called this morning to let us know that we would need to be admitted to have these tests that Dr. Himes wants done and that they would need to be done by Thursday as Dr. Arias would be out of town after that. After several phone calls and e-mails to coordinate things, it is now confirmed that Wednesday night we will be admitted to CHNOLA and Thursday morning Parker will be having an endoscopy and MRCP of his liver done under anesthesia. Next, Denise from Texas Children's called several times to coordinate Parker's appointment, and while originally she thought we had weeks before he could see us due to his schedule, Dr. Himes had other plans. He opened up a new clinic time frame just for Parker, because he wants to see us a week from today. In SEVEN days!! 

So, hopefully by next week we will have lots of answers to our questions. Dr. Himes will be able to tell us, Monday, whether Parker is a candidate for a shunt, which type of shunt he qualifies for, or if he will have to move directly to a liver transplant. Hopefully we will also get answers as to how long we have before any of these actions need to be taken. 

So, as always, we are accepting any good vibes, thoughts, prayers, or whatever you're willing to give. As you can imagine, this is all a lot to process, we are having a bit of de ja vu, and things are moving much faster than we expected. 

Saturday, April 7, 2018

Just another ride on this wild roller coaster we call life...

Most of you know by now that if I'm writing a new blog post, it's usually not a good sign. When things are simple, it's easy to quickly post to FB and keep people updated. But when things get complicated I need a little more room to explain.

Since Parker's last stomach virus at this time last year, things have been....off. Some of his blood work never really bounced back, including his platelets, hemoglobin, and hematocrit. Just recently we started having more issues. No matter how much he ate, he never gained weight. He had unexplained diarrhea, which just recently led to malabsorption. In the last month or so, labs got worse, so we started running more and more tests. We've done umpteen stomach ultrasounds, over $2,000 worth of stool studies, and countless labs with no answers. Just last week his team decided to re-test him for EBV, BK, and CMV again. These are tests they do at least once a year because of how serious they can be for transplant patients and they have always been negative before, so I thought.. okay just ruling one more thing out, no worries.

Yeah, not so much.

Yesterday Parker's team called to let us know that his blood work came back and it tested positive for CMV virus or Cytomegalovirus. We were quite shocked and scared to hear this. Prior to transplant one of the requirements was that Patty- our hero- be tested for CMV and the doctors were mind blown to find that she was CMV negative. This was a HUGE deal because had she been CMV+ Parker would have been at a much higher risk of contracting the virus, as her kidney could have transmitted it to him. That is actually the most common way that transplant patients become infected with this virus. For kids like Parker, who have a CMV- kidney, it's actually quite rare for them to contract this virus over 180 days after transplant, so this means that at some point in time recently he has likely come in contact with someone who actively carried the virus.

While all of this probably just sounds minor up until this point, this is where I'll get VERY real with you. Most times I sugar coat things as to not panic everyone, but I think it's time some people get a true glimpse at our life. Maybe then it will sink in when I say that a simple virus for you is not the same for my kid. This is why we stay in our bubble, and this is why I preach about staying away when you are sick, and having respect for others who aren't as fortunate as you. We are EXTREMELY lucky that we caught this when we did. As of now, the levels in his blood are relatively low (435) even though they should be undetectable. BUT, the problem is that this virus has also caused his WBC and ANC levels to be so extremely low that he doesn't really have an immune system to speak of right now. At this point, the doctors are very concerned with whether or not he will be able to kick this virus on his own, even with the help of medicine. They are actually talking about discontinuing one of his daily anti-rejection medicines in an effort to help his immune system build up, and if you know anything about organ transplantation, you know that this is something they never do. It's a HUGE deal and BIG risk to his kidney. Without those medicines, his kidney is more susceptible to rejection. REJECTION!  

The reason his team is even considering being this aggressive is because this virus is extremely dangerous to post-transplant patients. For people with compromised immunity, especially due to organ transplantation, a severe case of CMV infection can be fatal. Like I said earlier in this post, we are fortunate that his levels are currently low, however, if his body can't fight this off and those levels continue to increase, things could get much worse in time. The concern in their voices when they called to let us know about this, has us extremely worried right now.

We spent most of the day yesterday on the phone trying to track down a very strong, specialty anti-viral medication, which he will have to take for a minimum of 30 days and until the levels in his blood are undetectable again. Not a single pharmacy in Houma had it, and only one pharmacy in New Orleans could get it for today. So this morning we drove out to NOLA to pick this up and he got his very first dose tonight. Hopefully we don't experience many of the side effects, because that list isn't too pretty. We go to clinic on Tuesday to get more labs, see our transplant team, and discuss the plan of action from here on out. We are keeping our fingers crossed that we are able to come home that night, and that maybe his levels will have magically decreased on their own. Yes, I know that's just wishful thinking. So in the meantime, if you could say extra prayers for Parker, and maybe Dad and I's sanity too that would be much appreciated! We will keep everyone updated as we find out more.

Friday, May 26, 2017

Good news... with whiplash

The last two weeks have been nothing short of an adrenaline junkies roller coaster ride. In March we were told that Parker's numbers were stable enough that at this rate of progesssion, we had years before needing to to take action. Two weeks later, Parker started with the beginnings of the stomach virus. Fast forward to May, and the news we got was heart breaking. In a months time, a single virus forced his portal hypertension to decline significantly. We were told that a specialist at CHOP needed to review Parker's file and evaluate him for a procedure. For a very major procedure that we thought we had years to deal with. Yesterday we spoke with the renal transplant team at CHOP, and our appointment dates were set for July 18th. We finally let it settle in, and accept the fact that this is our fate. Today I spoke with Dr. Arias, our hepatologist here in New Orleans. He and Dr. Rand had the opportunity to spend a lengthy time consulting and  reviewing Parker's records. After doing so, Dr. Rand has decided that Parker does not need to be seen or evaluated in Philadelphia on July 18th. She feels that we are not yet critical and that Parker needs to grow some more before a surgery of that capacity. Especially being post-transplant. The arteries that they would be redirecting have incurred a lot of trauma, especially during transplant. They need time to heal and settle before being cut into again. She also believes that there is still the possibility that his body could recover and numbers could improve the further we get away from the norovirus. She advised that at this time she would not do anything different than we are doing now. The plan is to continue monitoring every 6-8 weeks with blood work and ultrasounds, and to eventually get a CT angiogram locally to get a better look at things. There's no definitive timeline as to when we will need to take action, or what the plan of action will be at that time. We are extremely grateful that they feel Parker is stable at this time, and that surgery is unnecessary, but the whiplash from the last few weeks and the unknowns have us pretty wrung out. Thank you everyone for all of the prayer! We appreciate each and every one of them. We will keep everyone updated as we can. 

Friday, May 19, 2017

Just another day in the life..

So I realize it's probably been a while since you guys have gotten an update this way, but that just means that up until now things have been relatively quiet. We've had your typical illnesses: colds, flu, stomach virus, and so on, but nothing major happening. Until today. We've been seeing a liver specialist for Parker since transplant. Most of you remember that when transplant time came, we were shipped out to Philadelphia due to Parker's liver condition. The surgeon here wasn't comfortable with operating on Parker without a liver specialist first evaluating him and deciding if he needed a dual liver/kidney transplant, and then she also wanted a liver specialist in the operating room with her. The problem there was that there was no qualified hepatologist in our area at the time. 

Dr. Arias, our new hepatologist at CHNOLA, is a pretty amazing doctor and he quickly made it up the ranks to one of our favorites. He's that doctor that gives you his contact information and asks you to e-mail him anytime. And when you do e-mail him, he calls you back personally within 24 hours. After today's appointment he went as far as to give us his cell phone number and asked us to call/text him with any questions about labs or ultrasound. His bedside manner with Parker is awesome and he explains things to us in terms we understand. He has trained at some of the top facilities in our nation so he is knowledgeable, but he still tells us like it is. So when he called recently asking to see us immediately we knew something was up.

 Parker has always had portal hypertension, it comes hand-in-hand with his diagnosis of ARPKD/CHF (Autosomal Recessive Polycystic Kidney Disease/ Congenital Hepatic Fibrosis of the Liver) Portal hypertension means that the blood cannot flow through his liver properly because his liver is hardened. When the blood cannot flow properly, it backs up into the spleen. We've monitored it closely with blood work and ultrasounds regularly, and while his labs have always been slightly off, it was nothing to draw concern about. His spleen has always been enlarged, due to blood flow backing up, but we were told his spleen wasn't large enough to worry about yet. The problem is that any little thing can cause more damage, including infections and viruses, and Parker spends a good bit of time sick because of his immunosuppression. Throughout March and April we fought Norovirus- a particularly ugly stomach virus, for over 30 days. Unfortunately this virus did a number on his liver. Since then, his platelets have dropped significantly, his liver enzymes (AST,  ALT,  INR) are all elevated, and his spleen has grown a good bit, and the blood flow in his veins are showing resistence now, which is a new occurance. Since it's been well over a month since our last hospital stay, all of these things should have fallen back to their "norm" by now, yet they haven't. They've progressively declined further, and that progression has them worried. The team here believes it's time to make a plan. Things aren't emergent, but we need to tackle this before it becomes an emergent situation. It's better for him if the evaluations and procedures are done while he is well. The procedure will be less invasive that way, and recovery much easier. However, the plan that we think will be best for him is not something that CHNOLA or any surrounding hospital has the technology or the training to do. 

Parker will need what is called a Distal Splenorenal shunt- IF he is a candidate. Essentially what this will do is bypass the portal vein that is backing things up from his liver to his spleen. During surgery they detach the splenal vein from the portal vein and reattach the splenal vein to the renal vein. This allows blood flow to go around the liver, relieving pressure from the swollen veins in his stomach and esophagus, without taking blood flow away from the liver. It also helps to control bleeding and clotting issues that portal hypertension can cause. 

In order to find out if Parker is a candidate for the procedure, and exactly how soon he will need it, there are a slew of tests and procedures that need to be done. Once again, CHNOLA doesn't have the machinery, technology, or training to even evaluate him for this procedure. In fact, it doesn't exist in the state from what we are told. So off to Philadelphia we go again in the next month or two. Dr. Rand's team at CHOP performs these tests almost once a week, so they are very well versed.

Our doctors here will begin coordinating information with the teams at CHOP on Monday. We expect it to take a while for the teams to consult, formulate a plan, and obtain insurance approval. Insurance approval will likely take the longest as it's not easy getting approval to cross state lines, and coverage drastically decreases when you do that. The transplant team at CHOP will also want to see Parker at this time since we are nearly 4 years out from transplant, so we have been told to expect to be there for at least a week. At this time there are still a lot of unknowns, but we know that we will be in the best hands possible at CHOP. The care there is truly unbelievable! In the meantime, please keep us in your prayers, specifically that the team comes up with the best plan available for Parker. 

Monday, January 2, 2017

ARPKD and what it means for the future of our family..

As most of you know, at 30 weeks gestation Jason and I received the devastating news that our first pregnancy was not going according to plan. Parker was diagnosed via ultrasound with Autosomal Recessive Polycystic Kidney Disease. It's a mouth-full right?

When Parker was born, and eventually transferred to Children's Hospital, we met with a geneticist. She ran extensive tests on Parker and thoroughly explained the details of ARPKD to us (mostly in terminology that we didn't understand). The odds of this happening to us again were likely a 1/4 chance, but since Parker's results weren't straight forward, Jason and I needed to be tested as well. At the time that didn't mean much to us. We were fighting day and night for Parker, so the distant future wasn't something we could concern ourselves with at the time. Not to mention, these tests were pretty costly. Our main priority was, and still is Parker's health and well-being, but he's 5 now and pretty stable, so we've been discussing expanding our family more and more. This Summer we finally moved forward with getting our parental genetic testing completed, and the odds were confirmed. Any child that Jason and I conceive would have a 1/4 chance of actively fighting ARPKD. We won the genetic lottery, right? We are in contact with many ARPKD and kidney related families. And we wholeheartedly respect each families choice of how they expanded their family, but for US we just can't see knowingly risking that with another child. So this new information, which we were sort of prepared for, has kind of thrown us for a loop on IF or HOW we would expand our family. The only conclusion that we can both agree on is adoption.

Adoption, however, is probably the most complicated thing I have ever tried to research. I mean I'm pretty sure that this could legitimately be a chapter in a college level Quantum Physics class. There is open adoption or closed adoption. There is domestic adoption and international adoption. There is the foster system, independent adoption, adoption agencies, and the crazy people on Craigslist trying to scam you out of your entire savings account. We have been researching this for 2 years now and are no closer to understanding what is best for our family, emotionally and financially, than we were when we started. We aren't even certain that there is an adoption route that is both emotionally and financially right for our family, but it's something we are determined to find out.

What we are asking of you guys is if you have had a personal adoption experience, know someone who has had a personal adoption experience, work in a field that gives you hands-on experience with adoption, know someone who works in a field with hands on adoption experience, know a church or social worker who has a program that can point us in the right direction, etc.. anything at all that will help us, please share your stories with us. We want to know the good, the bad, and the ugly. We want to hear pros and cons of every aspect. At this point, taking out a billboard sounds like the least complicated option (j/k...), so we appreciate all of the help!

You can message me directly on Facebook https://www.facebook.com/kristen.porche
or e-mail me at kldupre2@yahoo.com