Today was a 4:30am wake up in order to get everyone ready and off to the Medical Imaging Dept at our local Kaiser.  Dylan's Kaiser GI doc ordered a MRCP (which is a MRI specifically for the liver).  

Dylan was super nervous but he handled it like the trooper that he is, no sleepy meds, no calming meds, just ole fashion tough guy stuff.  The technician even said he was awesome and "better than a lot of his adult patients".  

The results on the MRCP:

- No Dilation notated
- Renal Cyst notated 


So the long and short of this is no strictures were seen during this test, which is what we were concerned about but that he does now have a Renal Cyst (Kidney).  Dr. Rich is concerned and wants a liver biopsy done.  He wants to eliminate the potential for Vanishing Bile Duct Syndrome (although very rare).

Symptoms:

- Hyperlipidemia
- Fat-Soluble Vitamin Deficiency 
- Elevated: ALK Phos
- Elevated: GGT
- Elevated: Direct Bilirubin 


Dylan has lots of these issues listed but it's too early to freak out about it and I'm holding on that it's "very rare" to have that issue but yet we can't ignore any of these potential issues.

Amber spoke briefly to our transplant coordinator over at LPCH (Stanford), she mentioned she is going to discuss with the team about doing a ERCP, which is: Endoscopic Retrograde Cholangiopancreatography and is a technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems. Through the endoscope, the physician can see the inside of the stomach and duodenum, and inject radiographic contrast into the ducts in the biliary tree and pancreas so they can be seen on X-rays.

The quick thought is perhaps the connection that connects Dylan's liver to his intestine (which is also known as Roux-en-Y) is hardened and has a stricture (the MRCP wouldn't necessarily see this and by doing the ERCP they could visualize these area's and see if they see something the MRCP imaging does not).  She also mentioned about doing the liver biopsy at the same time.  For those that don't understand what Roux-en-Y is.  Think of having your exterior bile duct(s) hooked right to your small intestine...still doesn't make sense?  Here is an image (as you can see, he has altered anatomy b/c he didn't get a deceased liver which often times will come with a complete biliary tree and gallbladder which then hooks up to the intestine like everyone else, although not always...just depends on lots of other things from that liver donor and the need to section it, etc...I won't bore you with the details of that stuff.)

For now we wait to hear the final word from LPCH and see what they want to do, although we know its going to be some semi-invasive procedure unfortunately.  

I'm not going to lie, I have had my heart pounding in fear for the last week in anticipation of this test.  I'm happy the results are such that aren't super negative but leave more pondering than answers (as well as the added Kidney Cyst, which I haven't even had time to process in regards to what that all means...hoping its medication related and that's that but perhaps that's wishful thinking on my end).

As much as I love how strong Dylan is through all this, it also hurts to know the stuff he's forced to be strong about.  We often times only reflect back as adults and lose sight that he's only 8 years old, he's been doing this his entire life and as a sense of reflection on a very young life...that's a very crummy thought that I can never shake.

-Noah

It has been awhile since our last update. I am sorry for keeping those of you who don’t follow us on Facebook hanging. Dylan’s health has been “stable” since his hospitalization. The dose of IV steroids and tapering dose at home (which he is still on) seems to have corrected the rejection he was fighting. We have fought very hard the past 6 months to get his liver in the great working order it once was. Dylan continues taking the Prednisone and increased dose of his Prograf (anti-rejection medication). He has been getting labs done every two weeks to follow lab values and make adjustments as needed. It feels we live our lives two weeks at a time. Never really knowing what is in store next. If his numbers are good we get to stay on the course we are on, if they come bad our course is diverted in a different direction.

Dylan's liver numbers have consistently been getting better over the past two months. Each time they improve more and we are able to lower his Prednisone dose. Due to his rejection episode we have been running his Prograf at a higher level in his blood. Before the rejection his troft level (the level of medication in his blood 12 hours from his last dose) was kept in the 2-3 range. Since his rejection we have been keeping his level between 7 and 8. This dose has been higher than he was ever on, even immediately after transplant. His system just like lower levels of the drug. It has been necessary though. We have had to "reboot" his system into thinking his liver is his own. Not an easy task.

Sadly, during this fight, his kidneys were irritated in the process. One nasty side effect of the Prograf is it can be very hard for the kidneys to process.  It is known to cause nephrotoxicity (a poisonous
effect of some substances, both toxic chemicals and medication, on the kidneys). For the past six months Dylan's Creatinine (a kidney function test) has been creeping up. I noticed that Dylan’s urine output had slowed down drastically last week. He had only been urinating a couple times a day despite his intake. He was constantly thirsty and drinking water. Knowing his kidney function was leaving something to be desired it obviously concerned me. At that time he was also running a low grade fever. After consulting his transplant coordinator I took him in for labs and a urinalysis. I can't say I was shocked at the results. 

It is always hard for me to see the numbers come pouring in. When we have his levels checked at Kaiser I get the results before any doctor and usually in the middle of the night. The levels for his kidneys were above the "normal" range and were pretty high for a child in Dylan's age group. Oddly, he was also having abnormal levels in his red blood cells. He was extremely anemic. Now, I am going to admit something here. I suffer from some serious post traumatic stress disorder. Watching your child vomit blood is not something easily erased from your memory. When Dylan suffered his GI bleed I had his labs done just prior (not knowing what was wrong with him). The lab results from that horrible day and his current results were far too similar. I could feel the stress building up in me. Now, I didn't really think he was having another GI bleed , but seeing those numbers seriously made me question what was wrong with my little boy. I emailed over his results to our coordinator so she would have them first thing in the morning. It was a long sleepless night for me. Thoughts were racing and emotions were high.

We heard back first thing in the morning. I was told to lower his dose of Prograf immediately and she would get back to me after consulting his team. She also reassured me that he was not having a bleed. Her guess is that he was fighting a virus of sorts and that it was suppressing his bone marrow. Not exactly a comforting thought, but much better than the alternative. Later that afternoon I got a phone call confirming that he was to stay on the lower dose and that we would repeat labs the following week.  We all went on about our week. The kids enjoyed school activities, parties and some trick-or-treating on Halloween. Dylan was a skeleton and Tegan was a peacock.

Saturday morning we woke up to another surprise. I thought Dylan had been replaced with some other child. He was so swollen and puffy I could barely recognize him. I sent his coordinator a text and asked her to look at a picture of him. She told me to call  the on call doctor at LPCH right away. There was the usual back and forth one would expect on a weekend, but I did finally get in touch with the doctor at LPCH and we came up with a plan. She told me Dylan needed to be seen and that his labs should be repeated. I called and made an appointment in urgent care for later that afternoon. We needed to see where his numbers were and determine if he was going to have to be admitted or not. The Kaiser doctor was great. He walked in and asked me what I needed him to do. I guess he could tell I was on a mission. After a quick exam he ordered some labs for Dylan (STAT) and we were on our way.

After a short two hour wait at home I had results and emailed them over to LPCH. The on call doctor called me and said she was pleased to say Dylan's labs were stable and that all the "scary" possibilities were not a concern for her ( I already knew that). His liver enzymes were up a tiny bit and his red blood cells were even lower (increased anemia), but his kidneys had not gotten worse and she felt safe letting him wait until his clinic appointment we had scheduled Monday. It feels at times there is never a true break for Dylan. While driving to the clinic Saturday he had a complete break down. Tears, anger, and frustration overwhelmed Dylan. He doesn't want to do this anymore. He wants a break from all things medical and I don't blame him.

Monday we made a trip to Lucile Packard for a follow up. It was the usual 2 hour drive there. Tegan slept the whole way. Dylan and I just talked and sang some songs. He does travel really well. At our appointment we discussed what was going on with Dylan. His doctor felt that his kidneys took a hit from the high doses of Prograf, but was hopeful that with the lower dose he would bounce back unharmed. He also felt that his bone marrow was being suppressed by some type of virus. We are going to recheck his EBV and CMV levels next week to make sure it is not either of these. Both of these viruses can be pretty scary after transplant. We were also told to repeat that rest of his labs next week as well. If his liver is still happy we will lower his Prograf dose again and hopefully stop his Prednisone for good. We left and headed home with a solid plan. Little did we know we weren't done yet.

From Monday to yesterday Dylan started having breathing issues. I noticed that with every breath out Dylan was grunting and he had developed a funny cough. His lungs sounded very clear (Yes, I have a stethoscope. I also have an otoscope. You just never know). Now, here is where life is unfair for Dylan. He had been to the doctor twice and labs twice in less than a week, but I had to take him back in. Post transplant with water retention and low blood counts can be a very bad situation very fast. It is very possible to have fluid "push through" into the lungs and cause pneumonia. With all that he had going on I couldn't take a chance. I made him an appointment and took him in AGAIN. His regular doctor did not have any appointments, but I got him in with another one that I like. I have developed a list of "approved" doctors at Kaiser. There are some that I don't work well with. We did run into his doctor in the hallway and he said he wanted to see Dylan when were finished with our appointment. It was a quick appointment. The doctor had us do a chest x-ray to rule out pneumonia. I was really proud of Dylan. Since I had Tegan with me I couldn't go back there so he went back alone and did a great job. He is growing up so fast and amazes me everyday.

The results were "unremarkable". Fancy word for everything looks good. We were led to his normal doctor's office to wait for him. When we first met Dr. Morales Dylan was 10 months old. This
doctor has kept me sane and led us through so much. He never questions my feeling or thoughts and ALWAYS validates me. I have joked that he is the only one who gets "my crazy", but it's true. I remember being on the phone with him as Dylan vomited blood. I remember hanging up on him and I also remember him calling us in the ER to check on him. Dr. Morales is a remarkable doctor and person. We are blessed to have him in Dylan's life. Yesterday he just wanted to check in on Dylan. He told him that he was so proud of him for all he goes through and how well he handles it. He said he just wanted to let Dylan know that he is always checking in on him and that he is always on his mind. Like I said we are blessed to have this man in our lives.

For now we are starting up his asthma meds again. We will need to be watchful for thrush in his mouth since he is still on the Prednisone, but I hope it will correct the breathing problems. We will recheck labs either today or early next week to make sure everything is improving with the medication change. As always we would like to thank you all for following Dylan's journey. It really does mean the world to us.


-Amber