Adventures in liver land 2013

March 22 2013:

Hi everyone! I know we have not updated in a very long time.
We are having way too much fun in toddler land to stop for updates! =) Hunter is doing great! He is running, jumping, and dancing his way through life. He is developing well and learning new words everyday. He is doing far better than anyone expected him to be doing at this point. His liver is still hanging on by a thread. Dr. Karpen says that his cirrhosis is as bad as it gets yet he has had no complications from it so far. The plan at this point is to wait for a complication to arise and then reconsider listing for transplant at that time. A complication would be ascites (fluid in the belly), severe itching that cannot be managed by medicine, or bleeding as a result to having portal hypertension. So far the only problem that Hunter has had is constant itching. Yesterday at clinic they prescribed him some medicine to hopefully relieve some of that. They do not think his itching is too bad yet. He is not causing a lot of broken skin and his still able to sleep comfortably. Hunter’s liver enzymes are still climbing but at this point there really is not anything we can do
about it.
We are still working on improving Hunter’s weight. He has not gained much in last five months so we are trying to add more calories. His goal for transplant was to be at least 10 months old and 22 pounds. Hunter is 16 months and 21.4 pounds. So we have exceeded our goal for age but weight is slow to come. However, he is extremely tall. The doctors are very pleased with his growth in height and say that his height is most important as it shows that his disease is not slowing his overall development. We also did a trial run with whole milk last month.Dr. Karpen was considering the possibly that Hunter had out grown his dairy protein allergy. Unfortunately the whole milk did not work out so he is still on his special formula. We are now trying some pediasure in hopes that his allergy is to lactose instead of dairy in general. We will also try whole milk again as he gets older. Otherwise, Hunter eats everything!!! He loves “ear-eee-ooos” (cheerios) and will eat a 4 piece chicken nugget and fries from chick -fil-a in one sitting with no problem. It seems unbelievable that he hasn't’t gained weight because he eats non stop!!

Now for some exciting news!!!!! As most of you know St. Louis Children’s has been following Hunter’s case so that he can be listed with them when the time comes. Children’s Healthcare of ATL is not covered in Humana’s transplant network nor is anyone else in Georgia so we were sent out of state… UNTIL NOW!!!! Yesterday at clinic, Dr. Karpen read us an email that stated, “Effective March 15, 2013, Humana will now be referring patients to Children’s Healthcare of Atlanta for solid organ and bone marrow transplant. Children’s Healthcare of Atlanta has now been accepted into Humana’s transplant network”!!!!!!!!!!!!!!!!!!!!!!!!! So Hunter will be staying in ATLANTA!!!! This is the best news EVER! We are sure that Dr. Karpen must have had some part in making this happen and are so grateful! Traveling 10 hours for appointments and then having to take life flight back and forth if anything were to happen is just crazy! We are beyond happy to be here with our transplant team where Hunter belongs!

Like I said, Hunter is GREAT and we could not be more thankful for that! Our story could have turned out so different. Making it this far is nothing short of a miracle. 

April 18 2013:

Hi all! Thanks to everyone who sent their support and love
this past week. Hunter is slowly getting better and his appetite is coming
back. Here is a play by play of what occurred this week. 

On Sunday Hunter was really sleepy. He napped pretty much all day and would not eat. Around six pm he started running a fever of 102+. I gave the on call transplant fellow a ring and explained his symptoms. She recommending bringing him in to the ED for labs to make sure everything was okay. By the time we arrived to Egleston he had gone down hill. His eyes were sunken in and he was not willing to get up and move around on his own. Luckily we are fast tracked so when we check in we were taken straight to a room. The liver doctor ordered labs and an ultrasound.The ultrasound showed that his cirrhosis and portal hypertension have progressed. His spleen had increased from 9.5 cm on 1/30 to 11.46. At first the team thought that this was an error because that is a HUGE increase in such a small amount of time. 11.46cm is bigger than a teenager’s spleen should be. However his labs verified that his platelets had dropped significantly and confirmed that his spleen had gotten much bigger. As his spleen continues to grow, it will eat up more and more platelets. His platelets were 119 four weeks ago and were 74 on Sunday evening. Low platelets cause him to bleed excessively. Any time he has lab work it takes a long time for them to get his bleeding under control. He also has small nose bleeds because of this. His neutrophil count was also really low, putting him in the category of severe neutropenia. This makes him very vulnerable to infectious diseases and bacterial infections. Also Hunter’s CBC and CO2 levels were low. He was very dehydrated. They started him on fluids while in the ER and the liver team decided to admit him. We were originally told it would just be a one night stay to give him a “tune up”. Unfortunately by the time labs were collected on Monday morning his platelets had dropped to 59 (not even 12 hrs apart). His red blood cell count and hemoglobin were also low along with his white blood cells. Most of his CBC was still abnormal. His bilirubin had also increased. On Tuesday they removed Hunter from fluids to try to encourage him to drink on his own. His fever was still spiking throughout the day. For a kid like Hunter this is bad news. He cannot have Tylenol like a normal child. He normally can only have a max of two half doses a day for a fever. However since his fever was not manageable they allowed him to have ¾ dose 4 times a day while he was in-patient.

On Wednesday they allowed us to take Hunter home under the condition that we call them and bring him back in if his fever continued more than two more days. After that time period he would not be able to continue on Tylenol due to the effects it would have on his liver. Also we are supposed to watch for a GI bleed. Hunter has always been at an increased risk for a bleed due to his portal hypertension. Now he is at an even greater risk because of his platelets and hemoglobin being low. So far things are going pretty smooth at home. His fever has stayed down since last night. He is still weaker than normal but he is attempting to play and eat. As long as nothing changes he will not have to follow up at clinic until June. His disease is progressing but hopefully it will slow down. He has beaten the odds this far and I know he can continue to do so. Please continue to keep him in your thoughts and prayers.