By the 12th week of summer, my true love gave to me:
One new old house in midtown
Two AC unit repairs (including a brand new unit for one of them!)
Three fridge failures (still working on resolving that one--but it's under warranty! Whew!)
Four family members heading to Chicago for a fake vacation this coming weekend/week. (Brooklyn's next GI clinic checkup is next week, and since we haven't been able to stray too far from Chicago or out of phone range this summer in the event that we might get the call we've been waiting for, we figured if you can't beat 'em, join 'em...)
Five years old and ready to start Kindergarten in eleven days--that's Rhet!
And a partridge in a pear tree...and by partridge, I mean Brooklyn...and by pear tree, I mean a new diagnosis.
I'll expand on that one...
(And it's going to be long, sorry about that. I owe you a long post!)
Back in May, Brooklyn and I made a quick 24-hr trip to Chicago for her GI clinic checkup. While we we were there, they took labs, checked her out, and issued her a new PELD score. To refresh your memory, the PELD score stands for Pediatric End-Stage Liver Disease. The transplant list isn't a "waiting list" where the person at the top of the list gets a liver and you move up in line and take your turn. Instead, your PELD score ranks your "priority." The higher the number (up to 40), the higher your priority over lower numbers. There are more factors involved when a liver becomes available, such as your blood type or whether the liver is a good "match" for your body, but the PELD gives you first dibs. We've been on the list for 6 months, sittin pretty in the lower middle. Someone could be listed today and jump to the top. It all depends on your PELD score.
Brooklyn started out in February with a PELD of 13. Her bilirubin (one of the numbers involved in determining her PELD score) had risen by the time we came in March/April for her hernia surgery--it had been steadily, almost rapidly rising at times since we had seen her first labs back in October. Then, something strange happened in May. For the first time, her bilirubin had dropped. It was enough to make her PELD score drop, although the change wasn't significant. They gave her a new PELD score of 12.
(They are required to re-PELD every 3 months. We get labs done every 6 weeks--if the numbers are significantly worse, they will request a new ["better"/worse] PELD score. If the labs numbers are the same or better, they just wait it out until they get worse or until they are required to re-score.)
So I came home in May a little bummed. Why had we rushed with all that lightning speed back in the fall only to be sitting around six months later, stable and waiting? Liver failure is so weird. Compared to you or me, Brooklyn is a very sick little girl. Compared to other kiddos in liver failure, she's not sick enough to take top priority. It feels so strange to be hoping for a "better"/worse score. Like, if she's going to be very sick, let's be very very sick really quickly for as short a time as possible so that she can get her transplant and be healed! I know, this transplant world is so weird.
The doctors had also talked to me about something else. They were questioning her biliary atresia diagnosis. They wanted to take extra labs to do some genetic testing for a rare condition called Alagille Syndrome. When they had done a CT scan immediately after her hernia surgery, they had found some abnormalities that had peaked their interest. They would send in the genetic test and wait for the results--if it came back positive, that gave some answers. If it came back negative, it didn't necessarily rule out the new diagnosis--the genetic test only tested certain markers.
I felt fear and discouragement when we arrived home in Memphis...what if this changed everything? What if a whole new can of worms or just a new can of uncertainty was opening? And Googling Alagille Syndrome (which is what every smart person should do when faced with a new diagnosis, right?) only made me feel worse. Biliary atresia is congenital, meaning Brooklyn was born with it--it wasn't passed on to her and she wouldn't pass it on. But Alagille Syndrome is a genetic disease...it would change the story about what had been and what will be...
A week later, Rhet finished up preschool, we moved to our new house, and we had plenty on our minds to keep us busy for awhile. (Including Brooklyn's 6-month post-placement report...can you believe she has been home for HALF A YEAR?! I am in awe. And 2015 is and always will be a blur.)
In July, the results of the test came back. Brooklyn tested positive for Alagille Syndrome. They were officially dropping biliary atresia from her diagnosis and changing it to ALGS.
It changed everything, but it also changed nothing.
Alagille Syndrome can include other complications--some children's symptoms are so severe that they are inoperable or need multiple organ transplants. Because Brooklyn had already received a heart echo, an abdominal ultrasound, and a CT scan as part of her transplant evaluation, the doctors had already determined that she had no additional complications. Her kidneys and heart look fine, and this condition is not degenerative, meaning these complications won't arise later in life. Her symptoms/presentation for ALGS include: some narrowing in her vascular system (not enough to cause heart complications), a few of her vertebrae are butterfly-shaped (super weird, but that is one of the symptoms), she has a broad forehead and triangular face shape (common in ALGS patients), she is small for her age (we had always attributed this to liver failure, but because she has ALGS, she will probably always be teeny tiny for her age), and of course, the bile flow in her liver is decreased/blocked. The only symptom needing correction is the liver failure, which can be fixed with transplantation.
So, things are different, but not really. She has a new diagnosis, but her current condition of health, the way they'll monitor it, and the way to fix it are essentially all the same. It was all earth-moving and anticlimactic at the same time, if that makes sense.
The Alagille diagnosis clicked a puzzle piece in place. We may never have known if she had biliary atresia--her liver is too damaged to tell with a biopsy or during transplant at this point. But with a positive genetic test result, that mystery has been solved. We know definitively what has caused the liver failure. And we also know why we're sitting around in July without the same rate of rapid decline that was happening October - February. Alagille Syndrome patients have no anticipated rhythm or pattern to their liver failure. While biliary atresia patients tend to decline steadily and/or rapidly at different times, ALGS patients can plateau, decline steadily, plateau, decline rapidly, etc all at varying times and speeds. This plateau that Brooklyn seemed to have hit this past spring was just that. And it explains why she had seemed to be declining so rapidly in the fall--because she was. She is still in liver failure and still needs a transplant, but her decline has transitioned into a period of stability for an unknown length of time. (It won't be years, though--her body wouldn't be able to sustain that--it will be more like an unknown period of months.) At some point, the decline will pick up again...steadily or rapidly, we can only wait and see.
So, that's the latest. And we're about to have more of the latest after our next GI clinic next Tuesday.
Please pray for our family as we continue to deal with change and transition in many areas.
Pray for our trip to Chicago, that we can experience sweet time together as a family--we are really thirsting for some happy memories to be made with all four of us together.
Pray for Rhet's first day of Kindergarten! I want her to feel brave and joyful and confident. Actually, I want to feel that way too. ;)
Pray for Brooklyn's liver...and for her new liver, however and whenever we will receive it. It is a terrible thing to be waiting on that life-saving gift. Honestly, I have trouble wrapping my mind around it and have to push that part away from my thoughts most days.
Pray for God to prepare our hearts and schedules and finances and physical endurance/health for liver transplant time, whenever it will be.
And thank you, friends. We couldn't do this life without you. We have chosen to follow the Lord in faith, and you have lifted us up and encouraged us every step of the way, even as we've felt tired and discouraged. God is for us! He is with us! And He is using you to remind us...
One new old house in midtown
Two AC unit repairs (including a brand new unit for one of them!)
Three fridge failures (still working on resolving that one--but it's under warranty! Whew!)
Four family members heading to Chicago for a fake vacation this coming weekend/week. (Brooklyn's next GI clinic checkup is next week, and since we haven't been able to stray too far from Chicago or out of phone range this summer in the event that we might get the call we've been waiting for, we figured if you can't beat 'em, join 'em...)
Five years old and ready to start Kindergarten in eleven days--that's Rhet!
And a partridge in a pear tree...and by partridge, I mean Brooklyn...and by pear tree, I mean a new diagnosis.
I'll expand on that one...
(And it's going to be long, sorry about that. I owe you a long post!)
Back in May, Brooklyn and I made a quick 24-hr trip to Chicago for her GI clinic checkup. While we we were there, they took labs, checked her out, and issued her a new PELD score. To refresh your memory, the PELD score stands for Pediatric End-Stage Liver Disease. The transplant list isn't a "waiting list" where the person at the top of the list gets a liver and you move up in line and take your turn. Instead, your PELD score ranks your "priority." The higher the number (up to 40), the higher your priority over lower numbers. There are more factors involved when a liver becomes available, such as your blood type or whether the liver is a good "match" for your body, but the PELD gives you first dibs. We've been on the list for 6 months, sittin pretty in the lower middle. Someone could be listed today and jump to the top. It all depends on your PELD score.
Brooklyn started out in February with a PELD of 13. Her bilirubin (one of the numbers involved in determining her PELD score) had risen by the time we came in March/April for her hernia surgery--it had been steadily, almost rapidly rising at times since we had seen her first labs back in October. Then, something strange happened in May. For the first time, her bilirubin had dropped. It was enough to make her PELD score drop, although the change wasn't significant. They gave her a new PELD score of 12.
(They are required to re-PELD every 3 months. We get labs done every 6 weeks--if the numbers are significantly worse, they will request a new ["better"/worse] PELD score. If the labs numbers are the same or better, they just wait it out until they get worse or until they are required to re-score.)
So I came home in May a little bummed. Why had we rushed with all that lightning speed back in the fall only to be sitting around six months later, stable and waiting? Liver failure is so weird. Compared to you or me, Brooklyn is a very sick little girl. Compared to other kiddos in liver failure, she's not sick enough to take top priority. It feels so strange to be hoping for a "better"/worse score. Like, if she's going to be very sick, let's be very very sick really quickly for as short a time as possible so that she can get her transplant and be healed! I know, this transplant world is so weird.
The doctors had also talked to me about something else. They were questioning her biliary atresia diagnosis. They wanted to take extra labs to do some genetic testing for a rare condition called Alagille Syndrome. When they had done a CT scan immediately after her hernia surgery, they had found some abnormalities that had peaked their interest. They would send in the genetic test and wait for the results--if it came back positive, that gave some answers. If it came back negative, it didn't necessarily rule out the new diagnosis--the genetic test only tested certain markers.
I felt fear and discouragement when we arrived home in Memphis...what if this changed everything? What if a whole new can of worms or just a new can of uncertainty was opening? And Googling Alagille Syndrome (which is what every smart person should do when faced with a new diagnosis, right?) only made me feel worse. Biliary atresia is congenital, meaning Brooklyn was born with it--it wasn't passed on to her and she wouldn't pass it on. But Alagille Syndrome is a genetic disease...it would change the story about what had been and what will be...
A week later, Rhet finished up preschool, we moved to our new house, and we had plenty on our minds to keep us busy for awhile. (Including Brooklyn's 6-month post-placement report...can you believe she has been home for HALF A YEAR?! I am in awe. And 2015 is and always will be a blur.)
In July, the results of the test came back. Brooklyn tested positive for Alagille Syndrome. They were officially dropping biliary atresia from her diagnosis and changing it to ALGS.
It changed everything, but it also changed nothing.
Alagille Syndrome can include other complications--some children's symptoms are so severe that they are inoperable or need multiple organ transplants. Because Brooklyn had already received a heart echo, an abdominal ultrasound, and a CT scan as part of her transplant evaluation, the doctors had already determined that she had no additional complications. Her kidneys and heart look fine, and this condition is not degenerative, meaning these complications won't arise later in life. Her symptoms/presentation for ALGS include: some narrowing in her vascular system (not enough to cause heart complications), a few of her vertebrae are butterfly-shaped (super weird, but that is one of the symptoms), she has a broad forehead and triangular face shape (common in ALGS patients), she is small for her age (we had always attributed this to liver failure, but because she has ALGS, she will probably always be teeny tiny for her age), and of course, the bile flow in her liver is decreased/blocked. The only symptom needing correction is the liver failure, which can be fixed with transplantation.
So, things are different, but not really. She has a new diagnosis, but her current condition of health, the way they'll monitor it, and the way to fix it are essentially all the same. It was all earth-moving and anticlimactic at the same time, if that makes sense.
The Alagille diagnosis clicked a puzzle piece in place. We may never have known if she had biliary atresia--her liver is too damaged to tell with a biopsy or during transplant at this point. But with a positive genetic test result, that mystery has been solved. We know definitively what has caused the liver failure. And we also know why we're sitting around in July without the same rate of rapid decline that was happening October - February. Alagille Syndrome patients have no anticipated rhythm or pattern to their liver failure. While biliary atresia patients tend to decline steadily and/or rapidly at different times, ALGS patients can plateau, decline steadily, plateau, decline rapidly, etc all at varying times and speeds. This plateau that Brooklyn seemed to have hit this past spring was just that. And it explains why she had seemed to be declining so rapidly in the fall--because she was. She is still in liver failure and still needs a transplant, but her decline has transitioned into a period of stability for an unknown length of time. (It won't be years, though--her body wouldn't be able to sustain that--it will be more like an unknown period of months.) At some point, the decline will pick up again...steadily or rapidly, we can only wait and see.
So, that's the latest. And we're about to have more of the latest after our next GI clinic next Tuesday.
Please pray for our family as we continue to deal with change and transition in many areas.
Pray for our trip to Chicago, that we can experience sweet time together as a family--we are really thirsting for some happy memories to be made with all four of us together.
Pray for Rhet's first day of Kindergarten! I want her to feel brave and joyful and confident. Actually, I want to feel that way too. ;)
Pray for Brooklyn's liver...and for her new liver, however and whenever we will receive it. It is a terrible thing to be waiting on that life-saving gift. Honestly, I have trouble wrapping my mind around it and have to push that part away from my thoughts most days.
Pray for God to prepare our hearts and schedules and finances and physical endurance/health for liver transplant time, whenever it will be.
And thank you, friends. We couldn't do this life without you. We have chosen to follow the Lord in faith, and you have lifted us up and encouraged us every step of the way, even as we've felt tired and discouraged. God is for us! He is with us! And He is using you to remind us...
1 comments:
Thank you so much for this update. We care about all of you and are praying!!
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