Kinley Walker's Journal
January transplant/liver clinic
Written Jan 9, 2014 10:11pmKinley had her transplant/liver clinic on Wednesday, January 8th. She has been doing pretty well and everyone was very pleased about that. She continues to grow and is now over 29 lbs and was 33 1/2 inches tall.
The size of her liver and spleen measured the same as her visit in November, so although it is enlarged, it wasn't any worse. We discussed how her platelets have dropped and that is a sign of Portal hypertension which we are already aware of. Because of her increased pressure in her portal vein, Kinley is at risk of having varices around her esophagus, stomach, and intestine. Those are smaller blood vessels that end up carrying a large amount of blood since the blood does not flow through her liver like it should. Sometimes these vessels leak or rupture and bleed out which could be life threatening. They want to schedule a scope in the next few months to look and see if this is becoming a problem or not. There are some things that they can do to try to prevent these bleeds, but they did say that they will only be able to see the ones around the esophagus and if or when they become a major threat, it will be time to talk about reactivating her on the transplant list. With that being said, they do not believe she is at that point yet, it is just something we will watch.
Dr. Weymann listened to Kinley's heart for a long time. Last time we were there he said he heard a slight murmer, so I thought he was listening for it again. He said that Kinley's heart sounded really fast. I told him about one night when I put her to bed and went back in to check on her once she was asleep. I could see her heart beating so I put my hand on her chest and I had a hard time counting the beats because it was fast. I went to grab a stethoscope and when I listened to it, it was slower so I thought maybe I was just not feeling it correctly. He said it was possible that Kinley's heart could be over stressed because of her bad liver and wanted her to have an Echocardiogram. He said that usually when the heart is too stressed it will have a "gallop" sound and he didn't think he heard that, but wasn't sure. She had the Echo and it was good. It did show that she still has a small PFO which is a small hole in the heart that should have closed as an infant. The PFO had shown up on an Echo she a little over a year ago and it is smaller now, so they are not concerned about it. They decided that the episodes of Tachycardia are not anything to worry about either. Her heart seems to be in good shape.
Her labs were again, not good, but stable. Her vitamin D is pretty low and we adjusted her meds because of it last time we were there, but the team is going to discuss it and get back to me with a plan. Ugh, the Vit D med is Kinley's absolute worst medicine. She sees it coming and shakes her head "NO!!" Poor girl.
So, overall, Kinley's condition remains stable. We do have some things to watch as we always do. The plan is still to hold off on transplant as long as she is doing well, and reactivate her on the list when we start seeing signs of a decline in her condition, or if the risks of keeping her liver start to be higher than the risks of the transplant. We are still hopeful that transplant could be a little ways off because she seems to be doing so well. She definitely amazes us and her transplant/liver team!!
Written Nov 30, 2013 1:17pmTomorrow, December 1st, is the 4th annual National Day of BA awareness. Early diagnosis is key in the survival of a baby with BA. You could be the one to detect something is wrong with your child, grandchild, niece, nephew. Maybe you could even save the life of a complete stranger's baby or the baby of a family friend. Since Kinley has been doing well you may not realize that BA is fatal without surgical intervention.
Let me start by telling you about Kinley's early days. She was born full term at 40 weeks 4 days. She was 8lbs 5 oz. The only complication I had while pregnant was anemia for which I took iron pills. She was a little jaundiced at birth, but so was Reagan so I didn't think anything of it. We took our 3rd "healthy" daughter home and I thought the biggest obstacle I faced was getting my 13 year old to accept the new baby. I took Kinley to her 2 week well baby visit and if she was jaundiced, it wasn't very noticeable. I was asked if she had yellow stools and I answered "yes" not realizing that the yellow he was asking about was a bright yellow, not the pale yellow stools like Kinley had. Everything checked out good. Since I bottle fed Kinley, she did not have to go back until she was 2 months old. I scheduled the appointment, but for some reason that I cannot remember, she couldn't get in the week she turned 2 months, so she went the next week when she was 9 weeks old. About 2-3 weeks before she her appointment I started to think she looked more jaundiced, but she didn't act like anything was wrong, so I didn't really worry about it. She ate well and slept well. I couldn't remember how long it took Reagan's jaundice to clear since she was born 10 years earlier, but I knew it did and she was fine, so Kinley's would too. Then an OB nurse that we know asked Troy if Kinley had always been jaundiced and he told me about her asking. I thought, well, I will just asked the doctor about it when I take her in for her check up in a week or 2 since she is doing well otherwise.
When I took Kinley in for her 2 month check up I told the doctor I was concerned that she was still jaundiced expecting him to say it wasn't a big deal...until he felt her abdomen and said that her liver was enlarged and we needed to do labs immediately. I looked at him and said, "She doesn't have Biliary Atresia, does she?" He said he wasn't sure but felt like she would be more sickly if she did. I already knew about Biliary Atresia because there is another case of it in my extended family, my second cousin's son. I read all of his Caring Bridge updates as he was going through his battle and transplant.
Kinley's pediatrician called me back that evening after hours to tell me that her labs were very bad and that we needed to take her to a specialist immediately. You see, with BA, the only treatment other than a liver transplant is a Kasai surgery which is usually only successful to buy some time before the liver transplant, but a Kasai surgery is most successful before 8 weeks old because the longer you go without it, the more damaged the liver is. Since that was a Thursday, we kept Kinley home over the weekend and she was admitted to the hospital on Monday with testing and then had her Kasai on Thursday, at 10 weeks old.
We have been lucky that Kinley's Kasai has been partially successful because she was diagnosed fairly late. If she would have been more than 2 weeks older, they would have not even performed the Kasai and she would have been listed for transplant. She may not have even lived long enough to get a transplant.
Please speak up if you notice that a baby is jaundiced after 2 weeks old. If it is your baby take them to their doctor and request labs. A high direct bilirubin is a sign of BA and often total bili is only checked. Please feel free to tell anyone Kinley's story or give them my contact information if I can help in anyway. Also, if you change diapers light stools are also a sign of BA as without bile flow stools do not have color. Babies with BA also have trouble gaining weight.
Many babies born today with BA have a good chance of survival also because of liver transplants. We are very fortunate that Kinley is a candidate for a living donor transplant and we have Andrea, a special friend, and Daddy who have both been through testing and could be a living donor for Kinley if necessary. For some kids, living donor is not an option, and they do not all receive organs in time or by the time they do, they are just too sick to undergo surgery. Please think about organ donation and sign up or let your family know if you want to be an organ donor.
Thank you for listening to me tell our story and you can always check baaware.org for more information.
I hope you all have a Merry Christmas!!
November 2013 tests/liver clinic
Written Nov 14, 2013 1:18pmHi everyone. Kinley had appointments yesterday for her 3 month check up. We started the day with and ultrasound. Kinley could not eat or drink for 6 hours before this test, but it didn't seem to bother her a bit. I woke her at 2:30AM for a pediasure and she never even asked for a drink or anything to eat until after her test!! She laid very still for her US (not what I was expecting from and 18 month old, lol). Once finished, the tech said she was going to check with the radiologist to see if they needed anything else. She came back in with 2 radiologist doctors saying they wanted a few more pictures. They then began to question Troy and I. "Has she been doing ok? Is she seeing her doctor today?" I answered "yes" to both questions. They continued to perform the US and my eyes started to become engorged with tears. I thought that something must be terribly wrong. They were talking about a shunt to each other so I thought maybe they felt she needed surgery or something right away. Then they turned to us and said, "everything looks about the same as last time."
We then went to see her doctor's appt. Her Transplant Coordinator and Transplant Nurse Practitioner were the first 2 in to see us. They explained that over all the US was unchanged from last time. It showed fibrosis/cirrhosis of the liver and splenomegaly (enlarged spleen signifying high blood pressure in the Portal Vein) and a small amount of ascites (fluid in the abdomen). These are all things that we already know about, but the fact that they are unchanged is great because they do not seem to be getting worse right now. They said that the US did show a splenorenal shunt. That means that because of the high blood pressure in Kinley's Portal Vein (because blood does not flow through her liver like it should), she has grown a new vein from her spleen to her kidney. They said that they feel like this is taking some of the pressure off of her Portal Vein and making it less likely for her to have a bleed at this time. I am a little scared about what I have read about this on the internet and how it can cause some complications during transplant, but I trust that her surgeon is very skilled and will know how to handle it when the time comes!! They said they usually start doing scopes to check for varices at 1-2 years. They feel that it is safe to put it off with Kinley until 2.
They also felt that growth is no longer an issue. That means no more expensive formula, we get to switch to regular milk. This is so exciting for us since last year she was labeled as "failure to thrive" because she wasn't growing and dropped to the bottom of the growth chart. Now she is near the top!!
We have not gotten lab results yet, but they do not expect so see a big change in them. Overall they were very please with how Kinley is doing and even said for the first time that it is possible that she could go "years" before transplant if things continue this way. They are very careful in saying that though, because liver disease can be very unpredictable and complications could arise at anytime. For now we are just so thankful that Kinley has such a good quality of life and that transplant is an option once that is no longer true. Thank you to all who consider organ donation and to our living donor match who keep hope alive for us. Thanks to all who pray for Kinley also.
Troy and I also had blood drawn yesterday for DNA samples. It is part of a study that Kinley is in that is ongoing in several hospitals throughout the country researching biliary atresia.
Since Kinley is doing well, my mom and I are going to take her and Reagan on a trip to see Aunt Rachel and Uncle Dylan in Corpus Christie, Tx. It will be Kinley's first time flying on an airplane. I hope you all have a Happy Thanksgiving.