What Minnesota Had To Say

Well, we are home now and I am still processing all that was said and everything, but here is what the decision is from Minnesota.  I posted it on Facebook as we were on our way home.

We are on the road heading towards home and we just got the call from Minnesota. They want to wait and see how the next few months go. If he has a lot of attacks in the next 6 months, then the surgery would be something they would do for him if we decided we were ready, but if he goes another 5 months between attacks like he did last time we can wait a while to make that decision. If anything changes with having pain inbetween or if he starts to lose endocrine function(insulin) in addition to his loss of exocrine function (enzymes which he already has to take for the rest of his life), then plans might change as well. I'm ok with this. We learned some new information and got answers to questions that other doctors would just ignore or BS us about. Right now his endocrine function is great and that function of the pancreas declines much slower than the exocrine function for people with atypical CF. That being said, pancreatitis and atypical CF affect each person differently. There are no definites or guarantees. His future (other than having to take enzymes the rest of his life) is one huge question mark. That was already the case because of his Autism Spectrum Disorder, but it is even more so now. I just hope and pray for his happiness. That is all I've ever wanted for him.

I will post in more detail soon after we are back into our daily routine and I don't feel like a zombie.  Also, Shade starts 2nd grade in a couple of days, so I will have time to think then.  Thanks for reading!

Pancreatitis for the First Time in 5 Months!

In the ER on July 13, 1013 before being admitted

     Well, we had a good 5 month break from hospitals and severe pain, but Pancreatitis reared it's ugly head again on July 13, 2013.  It started out like a normal Saturday morning with Dan at work and me and Shade lazily rolling out of bed around 1:00.  haha  We are night owls and love to stay up late and get up late on the weekends and during the summer.  Anyway, I didn't realize that anything was off at first.  He was waking up and watching TV on the couch.  Then at about 1:30 he told me that his tummy hurt and he asked for an Ibuprofen.  I had that familiar sinking feeling, but I tried to stay optimistic.  I gave him the medicine with a little juice and tried to assure him and myself that he would feel better in a few minutes.  At about 1:40, he pooped and I was more hopeful that things might settle down.  Well, at 2:00 he was literally screaming in pain.  Dan had the car, so I called my Mom and Dad in tears and asked them to come take us to the ER.  I gave Shade a half of a hydrocodone (which he was prescribed just for this reason) to help him until we could get to the hospital and then I packed and got things ready to go.
     When we arrived at the ER things went as they usually do.  We waited and waited and answered a million questions and then it was time for an IV so they could draw blood and give him pain meds.  It took forever and it was very difficult to distract him from the pain.  My Mom was such a huge help and my Dad was a trouper waiting in the waiting room due to only two adults being allowed in the ER room.  Of course, his levels were elevated and he was admitted very late that night.
     Things proceeded as they normally do and he was at first allowed no food or drink and only IV hydration and pain meds.  His pain was managed much better this time as we are learning to speak up more and demand they change the dose or the time intervals between so that he is suffering the least amount possible.  We have also learned that even if it is out of the nurse's hands, we can always demand to speak with a doctor so that orders can be changed.  We have become better at utilizing the Child Life workers and all of their resources and after 7 hospitalizations we have finally settled into the rhythm of life at the hospital.  It is not easy and it is still very exhausting, but there is comfort in familiarity.
     He was allowed to drink and then eat and his pain became less and less.  He loves to tell jokes to the nurses and we've found that he likes to ride up and down in the bed and "surprise" the nurses when they walk in by having the bed as high as it will go.  He is such a trouper and so resilient.  I die a little inside every time I have to watch him suffer, but he just bounces right back as if it's nothing.  He was given an abdominal ultrasound to make sure that there were no gallstones even though Dr. Palermo said that there were.  We were relieved, albeit frustrated with human error, to find out that he does NOT have gallstones at all.  If he had, they would have wanted to do an MRCP and then probably surgery to remove it.  No surgery is good news and we were allowed to go home on July 18.  Then all we had to do was prepare for our trip to Minnesota to see what the experts had to say about everything.  Details coming up!

Some Good News and Some Bad News- Let's Get Caught Up

In the ER again in June 2013

I have not updated in a while and I want to get caught up so that I have all of Shade's journey recorded.  Back in the beginning of June, we found out some great news.  Here is how I posted it on Facebook:  "We are going to Minnesota at the end of July for Shade's evaluation!! The experts will talk with us and review Shade's records and do tests and they will determine if they think that Shade needs the TP-AIT. So happy because this is what we have been waiting for, but terrified all at the same time. Also, insurance has not been completely worked out so we may end up having to pay out of pocket for this, but it will be worth it to finally find out if he needs this surgery or not."   I was super scared about how the whole trip would play out and how Shade would behave for the 3 days of tests and doctors and talking.  He did so much better than I thought he would do and I held up better emotionally than I thought I would as well.  I went in with no expectations and a few questions.  I was fully prepared to accept whatever they thought was right for Shade.  Ultimately, how we proceed with our son's treatment is up to us, but if they won't do the surgery, it doesn't matter if we think he needs it. 

My main concern going in was the results of his fecal elastase test in June.  If you recall, I had practically begged Dr. Palermo, his GI in Cincinnati, to do a fecal elastase test in April because he was already at a 99 back in November and had more attacks of pancreatitis since then.  He assured me that it was unnecessary because "the damage would not happen that fast" and it would be pointless to do another test so soon.  Well, when we had our appointment in June, practically the first words out of his mouth were that he wanted to do a fecal elastase test.  What difference that 2 months made, I don't know, but I am glad that he did it.  The results came back as 56 and anything below 100 is considered severely pancreatic exocrine insufficient.  This means his pancreas is shutting down it's production of enzymes.  He will need to take enzymes with everything he eats for the rest of his life.  I had no idea what this meant for his endocrine function and I was concerned that if we waited too long for the surgery, that he would not have enough good islet cells left to harvest.  Luckily, these concerns were addressed and I will fill you in on our trip in one of the next posts! 

I don't want to get too ahead of myself though.  Also during that visit with Dr. Palermo he mentioned that he has seen some small gallstones on some imaging from Kosair and that if Shade has any stomach pain that we should take him to the ER to get his amylase and lipase levels checked to rule out pancreatitis.  He had actually had some mild pain in May and we almost took him to the ER, but he was eating and drinking and only slowing down every now and then.  This lasted for 4 days and then he was completely back to normal.  Upon hearing about the possible gallstones though, we decided that if it happened again, we would take him to the ER.  Well, it did happen again on June 27, just a week after our appointment in Cincy.  We took him to the ER and they tested his levels and they were normal.  We were relieved, but still concerned about the whole gallstone issue.  We figured we would just ask at his next doctor's visit if he needed another abdominal ultrasound to confirm or what.  The docs in the ER did not seem concerned when we mentioned it to them and even Dr. Palermo had not mentioned surgery or anything, so we didn't know what to do.  Well, this brings us to Shade's next attack in July which I will make a separate post for and then the one after that will be our MN trip.  Stay tuned!!

Spread Chronic Pancreatitis Awareness!! Foundation for Childhood Pancreatitis.org

The Doctor Called Me Back

Here is a little bit of back story first.  Shade does not have pain in between attacks, but he has been hospitalized 6 times in less than a year.  His last ERCP was in August (3 hospitalizations ago). His fecal elastase test was done in November (2 hospitalizations ago) and the results show that he is severely pancreatic insufficient.  When I spoke with the GI at Cincinnati Children's, Dr. Palermo, and his nurse to ask if this changes anything, this is what I got.

 His nurse said that at this time, the results of his fecal elastase test would not change the course of his treatment because there is nothing we can do to prevent future attacks and any procedures might cause more damage. They might be willing to do a procedure if we come back again when he is having another attack.
As long as he has some funciton left in the pancreas, they won't do the TP-AIT because natural is better.  They want to keep the whole organ as long as they can.  She said that the islets are in the head of the pancreas and because that is where there is the most tissue, it is the last part to be damaged.

Then Dr. Palermo called back himself and asked if I was clear with the answers to my questions.  I asked about the damage and he said it doesn't happen that quickly.  He said that it is a slow process and that unless there has been trauma to the pancreas like from an accident, the damage happens slowly.  He said that for them to do the TP-AIT, the main criteria is how he does in between attacks.  It doesn't matter how many times he is hospitalized with attacks.  His fecal elastase test results don't really play a part.  His age doesn't play a part (they formerly said they don't like to do the TP-AIT until puberty).  What matters is that he is fine between attacks.  If he gets to a point where he is in pain in between, then we will see if surgery is an option.  As long as he has some function in his pancreas, they don't want to remove it.  I asked about atypical cystic fibrosis and if it causes constant damage and he said it might, but that there are many ways to have pancreatic damage and that we all probably have some damage to our pancreas. I asked if he would like us to come back when Shade has another attack and he said that he is comfortable managing his care from there because of the driving distance for us and because he would be receiving the same course of treatment at Kosair, so it doesn't matter.  We are, of course, welcome to come back to Cincinnati Children's whenever we want to though, and we should still set up an appointment for sometime this Summer unless he sees us before then.

I want to believe the doctor.  I really do, but I don't know if he is 100% correct.  I am a member of a few support groups on Facebook and it is crazy how often doctors are mistaken or some just don't have a clue about pancreatitis because it is rare.  I suppose I won't feel comfortable until we have our consult in Minnesota because UofM is the number one hospital in the country for performing the TP-AIT on adults and children.  If they tell me the same thing as Cincinnati, then I will just have to accept it.  Until then, I remain skeptical.

The Rest of The Story So Far

This is my 8 year old son, Shade. He's always been a very happy and healthy boy despite being diagnosed with an Autism Spectrum Disorder. He loves video games, telling jokes, bowling, and swimming. His life drastically changed forever beginning on June 11, 2012.  I wrote about our first hospital stay in my previous post.  The doctors told us that since he hadn't had trauma to his belly and had no prior history of pancreatitis that it was probably just a one time thing and sent him home on June 16, 2012. He was back to his old self and completely fine until July 7, 2012. He complained of stomach pain and refused food and drink so we took him to Kosair's ER. They checked his levels and admitted him for pancreatitis again. They did an MRCP and came back with normal results. No food or drink and checked his Amylase and Lipase levels daily. His levels came down and he was released on July 12, 2012. The doctors were still hopeful that he would have no more issues, but put him on a low fat diet of 15 grams of fat a day or less. We had so many questions, but we never got any straight answers. When he went to the ER a third time on August 7, 2012, I got mad and began to demand answers and any further testing they could do to stop this cycle. It was taking it's toll on all of us, but especially on Shade. He screamed every time somebody came into the hospital room because he was afraid they would poke him with a needle. Getting the IV in every time was a nightmare requiring several adults to physically hold him down while he begged me to make them stop. I had to help hold him down every time. They started him on enzymes (Creon) on 8/11/2012 and they agreed to genetic testing and told us to keep him on the low fat diet. That was much harder than you would think with a child who's food choices were already self limited due to sensory issues as a result of his Autism. The doctors also finally agreed to an ERCP which was done on 8/22/2012. The doctor who performed it found no anatomical anomalies or blocked or narrowed ducts, but he did find lots of scarring and diagnosed my son with Idiopathic Chronic Pancreatitis. Soon after this, the results of his genetic testing came back and he has 2 CFTR mutations. His sweat test came back normal, but he was sent to a pulmonologist and diagnosed with Atypical Cystic Fibrosis. Now we knew what was causing the Chronic Pancreatitis, but still had no way to stop it from causing repeat acute attacks. He remained on the low fat diet and enzymes and did not have an attack in September and we were skeptical but thrilled. Then came October 11, 2012. Back to the ER with the same symptoms and the same course of treatment at the hospital. This time the doctors finally admitted that they were in over their heads and did not know what else to do for him. One of them actually told us that this is our life now and we just have to get used to it. He said that Shade would learn to tolerate the pain better as he got older. :( I was so discouraged. He was released on the 15th and life went back to our new normal for a while. I decided that I needed to be more proactive and started searching all over the internet for information and help. I found several groups on Facebook, but only one for children with pancreatitis. I felt so alone, but I found out some info and had a phone number to call to a doctor in Minnesota. I was hopeful for at least a consult, but here it is April 2013 and I am still waiting for insurance to decide. Since then he has been hospitalized in December and February and I fully expect him to have to go back towards the end of this month. It's almost like clockwork. We also had meetings with doctors in Cincinnati, but they don't believe he is a candidate for the TP-AIT at this time and want to just "wait and see". They don't like to do the surgery before puberty. I have conflicting information from paperwork from Minnesota though. They have studies proving that children under 10 recover quicker and have an overall better outcome than older children and adults. I know that his pancreas is becoming more and more damaged with each attack and possibly in between attacks. I feel like he is a ticking timebomb. We found out the results of his fecal elastase test from November (two hospitalizations ago) and he got a 99 which is severely pancreatic insufficient. I'm sure if the test was repeated, his number would be even lower now. I don't want his pancreas to become so damaged that we can't get enough islet cells for the transplant, but I don't want to jump into such a serious surgery either. Still, my gut tells me that he needs this. We even went to Cincinnati during his last attack and had him transferred so they could see him during an attack per their request. They accused him of being drug seeking and still want to "wait and see". I don't feel like that is the right approach, but I am just the mother after all. haha I would like to have another ERCP or even an EUS done to see how much damage he has now, but I have been playing phone tag with the Cincinnati GI for 3 weeks now. He hadn't had the results of the fecal elastase test before and even suggested that we might want to take Shade off of enzymes. His nurse told me to definitely keep him on the enzymes (DUH) and that they would call me back with answers to my questions. I have called them again and it has been a week and all I hear are crickets. Meanwhile, Minnesota is still negotiating with Shade's insurance and we are just waiting. Waiting and preparing for the next inevitable hospital stay. Shade is fine right now. He is happily playing and enjoying the last few days of his Spring Break, but I know how quickly that can change and I also know that if his pancreas continues to get damaged, he may end up in constant pain or worse. I'm scared to death and stuck waiting while the time bomb keeps on ticking. So, there is our long story so far. Sorry it's so long. I left out some details of course, but this is the important stuff.