Adventures in liver land 2012

Jan. 31 2012: 

Okay so here is our attempt to catch everyone up. On January 19, Hunter had his normal liver clinic appointment. During this appointment Dr. Karpen was still concerned about Hunter’s weight as well as him still be jaundiced. In an attempt to help him gain some weight the decision was made to place him on a NG tube. If/when it becomes time for Hunter to be transplanted he will need to be as big and strong as possible. This was the first appointment where Dr. Karpen started to give us a timeline on how soon he could need the transplant if things did not turn around. Right now this could happen as soon as three months from now. However there is still a chance that if Hunter improves we could hold off for longer. It seems to be inevitable, at some point he will need to be transplanted. The question is when.  Dr. Karpen does not want to wait too long and risk damage to the rest of his body.  

We were admitted to the hospital straight from clinic. That night an IV was placed and his NG tube was inserted. Somewhere during this time Hunter’s liver team raised concern that there might be a possibility of him having biliary atresia (another liver disease) on top of having alpha one. Liver damage incurred from biliary atresia is caused by injury and loss (atresia) of the bile ducts that are responsible for draining bile from the liver. The loss of bile ducts causes bile to remain in the liver. In most cases of biliary atresia they can perform a procedure called a Kasai. In the Kasai procedure, the bile ducts are removed and a loop of intestine is brought up to replace the bile ducts and drain the liver. As a result, bile flows from the small bile ducts straight into the intestine, bypassing the need for the larger bile ducts completely. In Hunter’s situation, this would not be an option. The doctors discussed this and decided that the risk was too great.  So to figure out if Hunter even had BA they scheduled a HIDA for Friday morning. This involved placing dye into his IV. The dye goes into his liver and typically should secrete within 24 hours. In children with severe liver damage or biliary atresia this does not happen. This did not occur with Hunter. So the decision was made to do a liver biopsy. Hunter was taken down to the OR Tuesday afternoon and placed under general anesthesia. The liver biopsy took only about 35 minutes. Results came back the next morning. Hunter did not have BA. They found that he has too few bile ducts that are slow to process. This is nothing that can be fixed. The goal is to help him gain weight and see if the bile ducts kick into gear and start working better. Otherwise we just have another reason for transplant. So they taught us how to work the feeding tube and sent us home. We will have check ups weekly now. We appreciate everything that everyone has done for us over the past few weeks. Hunter has an awesome support system. Please continue to keep him in your thoughts and prayers over the next few months. 

Feb. 17 2012:

February has been another crazy month here in liver land. Hunter has had two more ER visits and a six day admission. Two weeks ago we started having even more issues with Hunter keeping his formula down. He was also having 10- 12 loose stools a day. Then his sleep pattern shifted. He would barely wake up to receive a bottle and was over all not acting like himself. That night we drove him to the ER. They ran a liver panel and sent us home. Two days later we returned to clinic. He was still showing all of the same signs. His liver levels were continuing to go through the roof. His glucose was a little low but not horrible. They felt that it was safe to take him home for the time being. Over the weekend things continued to get worse so we finally got admitted. During this stay they determined that Hunter has a severe dairy protein allergy. We have now switched him from Pregestimil to an amino acid based formula called Elecare. He has only thrown up three times over the past week on this. (Woohoo!) He is acting happy again and is awake and alert. I do not think I have ever seen him smile this much! They ran another liver panel at the end of our stay and his levels decreased for the first time since he was born! Also during our stay we met with occupational, physical, and speech therapy. Egleston has a great therapy team to ensure that Hunter does not fall behind during the time that he is sick. Speech said that he is doing great with eating. Occupational and physical therapy showed a little concern about his muscle tone being mildly low. They think this is caused by him not processing proteins correctly. We are working with him and doing special exercises to help him along. Other than this he is developing just fine. Dr. Vos thinks that this nutrition change might do the trick. As long as he gains weight on Elecare we might get by for a longer time without transplant. We can only hope that our hepatologist will have the same opinion. We are so glad to finally have some positive news though!!! Thank you for all of the thoughts and prayers! 

March 1 2012:

Hunter has gained a little more than a pound over the last few weeks. The new formula seems to be helping. This is a great improvement; however, this does not mean that he is getting better. Hunter has to be healthy enough to survive transplant. Right now nutrition is our main focus. The liver team decided to increase his night time feeds. We will still have the NG tube for a while. Transplant is still expected soon. We are not being placed on the list until he is healthy enough to make it through. Previous to this appointment, Hunter’s liver was enlarged but still soft. As of yesterday he has scarring of his liver and spleen. We should have results back from his lab work soon.

Aug. 27 2012:

 

Life has been busy. We have had a few really GREAT months... and lately a few bumps in the road. So here is a little bit of what has been going on in our life- Hunter is currently 18lbs and 1 ounce. Still small but he is no longer being tube fed. He is crawling, standing, rolling, and playing like any other 9 month old little boy. He has caught up developmentally and is so strong! For a little while even his liver team was amazed by how well he is doing. For almost three months we felt like a completely normal family. About three weeks ago Hunter caught a normal cold. He had a high fever of 103 or above so we took him to the hospital. Unfortunately a "normal/ everyday" cold really knocked him down. His platelets dropped really low and he had a hard time kicking the illness. While admitted at Egleston he was diagnosed with portal hypertension. The portal hypertension is caused by the cirrhosis and can lead to serious complications (Feel free to research portal hypertension on your own. The information can be overwhelming so I will not post it on here.) Transplant is still in his future... as normal we still do not know when. He has lost a little weight through being sick but his energy is back. -------- Also wanted to inform you all of some major changes and decisions happening in our life currently. Our insurance switched over to Humana back in June. Unfortunately Humana does not consider Children’s Healthcare of Atlanta a "center of excellence" for transplant so we are unable to stay with our team. We are looking into new centers throughout the country. We will most likely be transferring Hunter's care to St. Louis Children’s Hospital. We have been in contact with the team there and will be traveling to meet them soon. We are asking for positive thoughts and prayers as we make this tough decision. As always we thank each of you for your continued love and support.

Oct. 26 2012: 

Hi everyone! It is update time once again! Everyone has been curious as to how our trip to St. Louis Children's hospital went. I also want to be sure and explain exactly why we chose to visit their team. I am sure most of you have heard about our battle with our insurance. Originally, we were told that Hunter would not have coverage at Children's Healthcare of Atlanta at Egleston. We have a great liver team here in Atlanta and trust them 100%. This is where he has been followed since birth so you can imagine our frustration. After our last admission in August, I called insurance about some claims and once again argued my case. This time after months of confusion... I was told that Egleston would be covered for general admissions. This would be any time that he is seen by his liver team for regular clinic visits or when he is admitted for observation. What they will not be covering is anything directly connected to transplant. For example, the actual surgery cannot be done at CHOA and admission prior to and after transplant for prep/recovery. This is why we made the decision to go ahead and meet a new team. We could not see waiting until it was time for transplant and have to rush around to find the right center to list him with. We wanted to know that we had a team set up that we could trust and be familiar with. So we called St. Louis for a second opinion/ meet and greet visit.

Dr. Turmelle and her team at St. Louis were phenomenal with Hunter. She was compassionate and blunt. Everything that I want in a doctor for Hunter. She went into detail about where we are currently with Hunter's disease and we feel very comfortable knowing that this is where we will be when it is time for Hunter to receive a transplant. I will try my best to explain that facts that she gave us last week. Remember that Alpha 1 does not take a set course. Every child with this disease will progress differently. Hunter is currently stable so there is no need to list him right now. The longer we can wait the better! The goal is for him to be as big and strong as possible so if we can hold off then GREAT!!!! =-). So here is the break down from our trip!!

 

                On Wednesday (Oct. 17th 2012) Hunter was scheduled to go for an ultrasound, labs, and clinic with the St. Louis team. We got to ultrasound at 7:30. The whole process took about an hour. From the ultrasound we could see his liver and spleen. His liver looked severely scarred. It actually looked like the moon. Almost like there were large pits in it. The edges were rough looking. It was smaller than it had been in the past. The radiologist explained that all of these things were due to scarring because of cirrhosis. His spleen was enlarged as we have known it to be for a while. On this day it measured 9.8 cm long. The doctors could feel it all the way down to his belly button. They sent the reports up to the liver team for further analysis. Next we had labs drawn and then proceeded to clinic. Dr. Turmelle did a great job explaining how alpha 1 typically progresses. Normally a child with Alpha 1 would develop cirrhosis at about 5 years of age... maybe have to be transplanted as a teenager if at all. This is obviously not the course that it has taken with Hunter. For hunter, she explained, alpha 1 has taken a rougher, more progressive course than most children that they see. She told us how Alpha 1 is so unpredictable. With cirrhosis he could stabilize and be good for years before needed a new liver. But she also told us that this would be unlikely. He would probably need a new liver sooner than later. His portal hypertension has progressed steadily over the last three months. The next three months will be very informative . It can continue to progress at this rate or slow down. She said there is nothing we can do to control it. It has to run its course. She wants him monitored closely to watch his development, nutrition and use physical therapy if he needs it. She also explained that hunter's immune system is not slow. The reason for keeping him away from sick people is because one illness or infection could put him over the edge and he may not recover. This would send him straight to transplant. But she also said that we cannot keep him in a bubble. He has to be a child as well. Currently the signs that it is time for transplant would be the following: he could have a bleed from having portal hypertension that they cannot get under control, or if he develops ascites and they cannot get it under control with diuretics. Hunter has also been sleeping 15-18 hours a day. The doctor said this is due to liver disease. It is normal for him. On a good note, Hunter was on the 10% for growth!!!! He weighed 19.7 lbs!!!! His labs were stable. So for now we wait... Wait for his progression to slow down or wait for a complication. Either way we are home and a family until something further happens.

We have lots of fun to look forward to! Fundraisers all weekend to help offset his medical bills. - What a huge blessing that is! & His first birthday is in 3 weeks and we are going to celebrate! What a first year of life it has been! He is one incredible little boy. When I reflect over the past year it is not Alpha 1 and our journey with liver disease that I see. I see an energetic baby boy who has been a blast to watch grow and play. From giggles to hissy fits.. he is my whole world. God has given us a huge gift and while I may forever be a "Liver Mom", I would not trade this ride for anything!!!!