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Hello. Hard to believe it's been about two months since our last update,
and that Arielle is three months now. Since most people want photos,
here's one of the first capturing her smile (hello adorable!!!):
We've received mixed results since our last update, but good definitely outweighs bad. We'll start with the tough stuff. The most recent MRI revealed another portion of Arielle's brain didn't form properly: the right cerebellum, which is responsible for things like controlling movement and processing language. This now means two different parts of her brain didn't form properly because of PHACE Syndrome, the rare disorder linking a large, infantile hemangioma (abnormal mass of capillaries - usually of the face), and birth defects of the brain, heart, arteries, eyes and/or skin.
As was the case with the other brain defect (complete agenesis of the corpus callosum), it will likely be quite some time before we understand what this means for Arielle, her growth, development and future. Translation: please continue to keep her in your thoughts and prayers!
In the short-term, we've been attempting to stop the growth of the hemangioma on her face. The picture above does the best job of explaining what a hemangioma looks like. When Arielle was born, there were only faint pink marks in areas that quickly exploded into the large, red masses that have now taken over the better part of a quadrant of her sweet little face. In an attempt to prevent any long-lasting facial deformities and worsening impact on her right eye, Arielle went on steroids about two months ago. Thus far, the medicine seems to have helped slow the progress, but has yet to fully put the brakes on it.
One of the best pieces of news is that the echocardiogram determined Arielle's heart is structurally and functionally normal. Yippeee! And, another test revealed the bulk of her right optic nerve formed, meaning there may be hope for some sight in the eye that was previously diagnosed with permanent blindness.
As many tests as she has endured, many more are in store for her future, and we're beginning to understand regular tests and monitoring are going to be a part of our family's life. Arielle still needs an MRA (magnetic resonance angiography), a special type of MRI test designed to evaluate arteries, veins, and the flowing blood within them. Another MRI focusing on the eye area should tell us more about what's possible in terms of restoring any vision to her right eye. Soon, we travel back to Texas Children's Medical Center in Houston to visit one of the leading doctors specializing in PHACE Syndrome, and are eager to receive any new insight. We also plan on attending the inaugural PHACE conference for the condition in November.
We're extremely pleased to report that thus far, Arielle has met all of the developmental milestones on time, and even early in some cases. She regularly coos and smiles, and has taken a particular liking to: FOOD, music, FOOD, being outside, FOOD, bright colored toys, FOOD, and Big Bird. Did we mention the girl likes to eat?
Thank you for caring about our family!
- Mary Alice, Travis and Baby Arielle
We've received mixed results since our last update, but good definitely outweighs bad. We'll start with the tough stuff. The most recent MRI revealed another portion of Arielle's brain didn't form properly: the right cerebellum, which is responsible for things like controlling movement and processing language. This now means two different parts of her brain didn't form properly because of PHACE Syndrome, the rare disorder linking a large, infantile hemangioma (abnormal mass of capillaries - usually of the face), and birth defects of the brain, heart, arteries, eyes and/or skin.
As was the case with the other brain defect (complete agenesis of the corpus callosum), it will likely be quite some time before we understand what this means for Arielle, her growth, development and future. Translation: please continue to keep her in your thoughts and prayers!
In the short-term, we've been attempting to stop the growth of the hemangioma on her face. The picture above does the best job of explaining what a hemangioma looks like. When Arielle was born, there were only faint pink marks in areas that quickly exploded into the large, red masses that have now taken over the better part of a quadrant of her sweet little face. In an attempt to prevent any long-lasting facial deformities and worsening impact on her right eye, Arielle went on steroids about two months ago. Thus far, the medicine seems to have helped slow the progress, but has yet to fully put the brakes on it.
One of the best pieces of news is that the echocardiogram determined Arielle's heart is structurally and functionally normal. Yippeee! And, another test revealed the bulk of her right optic nerve formed, meaning there may be hope for some sight in the eye that was previously diagnosed with permanent blindness.
As many tests as she has endured, many more are in store for her future, and we're beginning to understand regular tests and monitoring are going to be a part of our family's life. Arielle still needs an MRA (magnetic resonance angiography), a special type of MRI test designed to evaluate arteries, veins, and the flowing blood within them. Another MRI focusing on the eye area should tell us more about what's possible in terms of restoring any vision to her right eye. Soon, we travel back to Texas Children's Medical Center in Houston to visit one of the leading doctors specializing in PHACE Syndrome, and are eager to receive any new insight. We also plan on attending the inaugural PHACE conference for the condition in November.
We're extremely pleased to report that thus far, Arielle has met all of the developmental milestones on time, and even early in some cases. She regularly coos and smiles, and has taken a particular liking to: FOOD, music, FOOD, being outside, FOOD, bright colored toys, FOOD, and Big Bird. Did we mention the girl likes to eat?
Thank you for caring about our family!
- Mary Alice, Travis and Baby Arielle
I'm obviously going to have to post here more regularly than just on the major holidays.
This will be kind of short as I don't have Mary Alice's wordsmithing skills. Hopefully I can free time for her to write a more detailed update.
At the moment, Arielle is receiving prednisolone (an oral steroid) to arrest the growth of her hemangioma. It does appear to be helping, although we did back off the dosage a little too quickly two weeks ago. We've since increased it to the maintenance level. We go to Houston later this month to visit with Dr. Denise Metry, who specializes in PHACE syndrome. We're looking forward to what she can tell us about Arielle's case.
Thanks for checking in on us - it's hard to believe tomorrow is Arielle's three month birthday!
This will be kind of short as I don't have Mary Alice's wordsmithing skills. Hopefully I can free time for her to write a more detailed update.
At the moment, Arielle is receiving prednisolone (an oral steroid) to arrest the growth of her hemangioma. It does appear to be helping, although we did back off the dosage a little too quickly two weeks ago. We've since increased it to the maintenance level. We go to Houston later this month to visit with Dr. Denise Metry, who specializes in PHACE syndrome. We're looking forward to what she can tell us about Arielle's case.
Thanks for checking in on us - it's hard to believe tomorrow is Arielle's three month birthday!
Yesterday marked Arielle’s one month
birthday, and what a month it’s been. After quite a few doctors
appointments and tests, it has been determined that Arielle has PHACE
Syndrome. This is a rare disorder linking a large, infantile hemangioma
(abnormal mass of capillaries - usually of the face), and birth defects
of the brain, heart, eyes and/or skin.
We believe this explains and gets to the bottom of all of her health issues to date, including a key one that emerged since our last update. We already know Arielle is afflicted with three of the four key areas PHACE affects, namely the brain, her right eye and most recently her skin. In utero, it was determined the key pathway connecting the left and right sides of the brain didn’t form. Shortly after birth, an ophthalmologist concluded she is blind in her right eye. About two weeks ago, a large, bright pink mark began appearing on Arielle’s face and quickly grew to encompass the upper right quadrant of her head as well as her lips and tip of her nose. That ultimately triggered a visit to a pediatric dermatologist who happens to be very familiar with the disorder and immediately diagnosed it as PHACE.
Needless to say, this has been a very tough month for our family, but not without progress. Arielle has been examined by a pediatric neurologist, ophthalmologist, dermatologist, radiologist and will likely soon see a cardiologist. She has had an MRI and needs other key tests (MRA, echocardiogram, etc.) to fully evaluate the situation and help make decisions for her future care. In particular, upcoming tests will focus on her heart.
Arielle has undergone a myriad of exams, poking and prodding, and proving to be one tough little lady with incredible strength and endurance. We are determined to see, understand, and appreciate this child beyond her health complications and luckily, her amazing disposition is making that one of the easiest things for us to do right now. She can fight like hell when she wants, but instantly forgives as soon as the discomfort dissipates. And to the extent the doctors can do their jobs while letting her stay in the safety and security of her family’s arms, she remains amazingly at peace. She is a very good baby, with seemingly advanced physical strength and precious nature.
Mary Alice has come a long way in recovering from the difficult C-section and associated complications. She still lacks stamina and strength, but is definitely headed in the right direction. And Travis has secured a good supply of lotions to treat his dishpan hands from all the housework he’s been doing.
We’re grateful to finally feel like the root cause has been identified and hope no more new “bombs” drop. We’re grateful for the meals, flowers, cards and notes of encouragement that keep the wheels of our world turning and sorry time doesn’t permit us to respond to everyone individually. We’re grateful for the spiritual support that has no doubt carried us thus far. We ask that you continue to keep our little one at the foremost of your prayers. Many thanks.
Mary Alice, Travis & Arielle
PS: For more information on PHACE Syndrome, visit: www.phacesassociation.com.
We believe this explains and gets to the bottom of all of her health issues to date, including a key one that emerged since our last update. We already know Arielle is afflicted with three of the four key areas PHACE affects, namely the brain, her right eye and most recently her skin. In utero, it was determined the key pathway connecting the left and right sides of the brain didn’t form. Shortly after birth, an ophthalmologist concluded she is blind in her right eye. About two weeks ago, a large, bright pink mark began appearing on Arielle’s face and quickly grew to encompass the upper right quadrant of her head as well as her lips and tip of her nose. That ultimately triggered a visit to a pediatric dermatologist who happens to be very familiar with the disorder and immediately diagnosed it as PHACE.
Needless to say, this has been a very tough month for our family, but not without progress. Arielle has been examined by a pediatric neurologist, ophthalmologist, dermatologist, radiologist and will likely soon see a cardiologist. She has had an MRI and needs other key tests (MRA, echocardiogram, etc.) to fully evaluate the situation and help make decisions for her future care. In particular, upcoming tests will focus on her heart.
Arielle has undergone a myriad of exams, poking and prodding, and proving to be one tough little lady with incredible strength and endurance. We are determined to see, understand, and appreciate this child beyond her health complications and luckily, her amazing disposition is making that one of the easiest things for us to do right now. She can fight like hell when she wants, but instantly forgives as soon as the discomfort dissipates. And to the extent the doctors can do their jobs while letting her stay in the safety and security of her family’s arms, she remains amazingly at peace. She is a very good baby, with seemingly advanced physical strength and precious nature.
Mary Alice has come a long way in recovering from the difficult C-section and associated complications. She still lacks stamina and strength, but is definitely headed in the right direction. And Travis has secured a good supply of lotions to treat his dishpan hands from all the housework he’s been doing.
We’re grateful to finally feel like the root cause has been identified and hope no more new “bombs” drop. We’re grateful for the meals, flowers, cards and notes of encouragement that keep the wheels of our world turning and sorry time doesn’t permit us to respond to everyone individually. We’re grateful for the spiritual support that has no doubt carried us thus far. We ask that you continue to keep our little one at the foremost of your prayers. Many thanks.
Mary Alice, Travis & Arielle
PS: For more information on PHACE Syndrome, visit: www.phacesassociation.com.
Just a quick note to let everyone know Arielle Elizabeth Kaspar arrived February 15th at 4:49 PM. 6 lbs 8 oz, 19.5 inches via c-section. While we know we're biased, lots of other nurses at the hospital indicate she is, in fact, extra adorable.
Delivery and recovery has been more trying than anticipated. Bleeding problems occurred, prompting a more intense c-section, and a blood transfusion followed. As many of you know, Arielle was diagnosed in-utero with a birth defect that we were warned could impact a wide variety of functions. One of those is sight. An ophthalmologist concluded she is likely blind in one eye, which both didn't form properly, nor does it communicate correctly with the brain, making it something that is not fixable. While such news is tough, we are grateful all appears well with the other eye and she should be able to live life as a "seeing" person. Other than that, all functions are high. And mama can tell you she seems to be eating well, that's for sure!
Once again, thanks to everyone who has reached out with calls, thoughts, prayers, etc. Keeping up with everything is very tough at the moment. Email is the best way to communicate with us for the time being. Thanks!
Mary Alice, Travis & Arielle
“Every baby is a blessing.” - Author
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Quick recap: At our 20-week sonogram in early October, it was discovered our daughter’s brain wasn't forming properly. The initial diagnosis was hydrocephalus, but a subsequent MRI determined the main pathway that connects the two halves of the brain didn't form. It’s called complete agenesis of the corpus callosum, or C-ACC.
We're happy to report some positive developments over the past month...
Two key visits
Our most recent sonogram appointment on Thursday revealed that not only have the enlarged ventricles with the excess fluid remained stable, they now measure NORMAL. The sonogram technician didn’t even think she was getting accurate images at first based on Arielle’s charted history! Additionally, her head circumference, which has consistently been above the 90th percentile, has also fallen smack dab in the middle of the NORMAL range.
These developments are positive, as they mean there’s likely no additional pressure on her brain and we’re more likely to be able to have a normal delivery. Had her head remained anywhere above the 80th percentile, our OB/GYN was recommending a C-section.
Just before Christmas, we were finally able to get an audience with our new neurologist with Dell Children’s Medical Center here in Austin. We spent more than a month and a half on a waiting list being told we were a top priority for a cancellation slot that would seemingly not come. A friend/board member got wind of our situation, and within days, an after-hours appointment was created on our behalf. We’re grateful beyond words to that person for the behind-the-scenes nudge.
The visit was incredibly productive, informative and helpful. The doctor was able to answer a long list of questions we had prepared, or in some cases, explain why an answer wasn't known. The neurologist recommends moving forward as you would with any newborn, as Arielle’s early life needs likely won't differ from other children’s. Mary Alice had intended to continue working part-time from home, and he didn't see any reason for her not to progress as planned.
Grateful to know
One of the more interesting things we learned from the most recent doctors visits is C-ACC is most often NOT diagnosed before a child is born. In our case, the enlarged ventricles and excess fluid acted as a tip off to warrant an MRI. Ironically, it is those very two things that are now normal.
We assumed because a sonogram helped us with a diagnosis, the same held true for others. So we asked the neurologist what the latest stats were for prognosis now that detection was more common than say 30 years ago (What percentage of kids lead normal lives? Experience minimal impairments? Suffer more severe problems?). The reality seems to be experts don't really know because more often than not, it isn't detected in the womb...so most aren't tracked since birth. That’s also why it’s commonly misdiagnosed as other conditions, like autism.
We feel all the more grateful to have an accurate diagnosis as we know this increases our awareness, knowledge and ability to best care for our daughter. While nothing “corrects” C-ACC, individualized therapies have been proven to help.
Focused hopes/prayers
Many of you have been very gracious in offering prayers and other demonstrations of support, or asking how you can help. We’re mindful that within our audience are people from a variety of religions, or lack thereof, but we share a common intention that our daughter will lead a healthy and productive life. We are convinced all the positive energy has helped our spirits remain high. We furthermore credit all of you and the good Lord himself with the latest progress Arielle has made.
With that in mind, we ask everyone to keep up whatever it is they’re doing with the following intentions in mind:
- That Arielle’s ventricles and head size remain normal in size.
- That the two hemispheres of Arielle’s brain find other ways to communicate without the pathway its missing (it’s unclear why, but some brains seem to cope/compensate better than others).
- That Mary Alice’s challenges with anemia and fatigue not worsen (doctors were never able to get it to improve, and it’s known for worsening near the end of the pregnancy).
- That we’re able to find an experienced pediatrician for Arielle. The one the neurologist recommended no longer accepts new patients with our insurance, so our quest on that front continues.
- That delivery goes smoothly. Only six weeks to go!
Thanks to you all once again. Our next update may very well be a photo upon delivery.
- Mary Alice, Travis and Baby Arielle
Quick recap: At our 20-week sonogram in early October, it was discovered our daughter’s brain wasn't forming properly. The initial diagnosis was hydrocephalus, but a subsequent MRI determined the main pathway that connects the two halves of the brain didn't form. It’s called complete agenesis of the corpus callosum, or C-ACC.
We're happy to report some positive developments over the past month...
Two key visits
Our most recent sonogram appointment on Thursday revealed that not only have the enlarged ventricles with the excess fluid remained stable, they now measure NORMAL. The sonogram technician didn’t even think she was getting accurate images at first based on Arielle’s charted history! Additionally, her head circumference, which has consistently been above the 90th percentile, has also fallen smack dab in the middle of the NORMAL range.
These developments are positive, as they mean there’s likely no additional pressure on her brain and we’re more likely to be able to have a normal delivery. Had her head remained anywhere above the 80th percentile, our OB/GYN was recommending a C-section.
Just before Christmas, we were finally able to get an audience with our new neurologist with Dell Children’s Medical Center here in Austin. We spent more than a month and a half on a waiting list being told we were a top priority for a cancellation slot that would seemingly not come. A friend/board member got wind of our situation, and within days, an after-hours appointment was created on our behalf. We’re grateful beyond words to that person for the behind-the-scenes nudge.
The visit was incredibly productive, informative and helpful. The doctor was able to answer a long list of questions we had prepared, or in some cases, explain why an answer wasn't known. The neurologist recommends moving forward as you would with any newborn, as Arielle’s early life needs likely won't differ from other children’s. Mary Alice had intended to continue working part-time from home, and he didn't see any reason for her not to progress as planned.
Grateful to know
One of the more interesting things we learned from the most recent doctors visits is C-ACC is most often NOT diagnosed before a child is born. In our case, the enlarged ventricles and excess fluid acted as a tip off to warrant an MRI. Ironically, it is those very two things that are now normal.
We assumed because a sonogram helped us with a diagnosis, the same held true for others. So we asked the neurologist what the latest stats were for prognosis now that detection was more common than say 30 years ago (What percentage of kids lead normal lives? Experience minimal impairments? Suffer more severe problems?). The reality seems to be experts don't really know because more often than not, it isn't detected in the womb...so most aren't tracked since birth. That’s also why it’s commonly misdiagnosed as other conditions, like autism.
We feel all the more grateful to have an accurate diagnosis as we know this increases our awareness, knowledge and ability to best care for our daughter. While nothing “corrects” C-ACC, individualized therapies have been proven to help.
Focused hopes/prayers
Many of you have been very gracious in offering prayers and other demonstrations of support, or asking how you can help. We’re mindful that within our audience are people from a variety of religions, or lack thereof, but we share a common intention that our daughter will lead a healthy and productive life. We are convinced all the positive energy has helped our spirits remain high. We furthermore credit all of you and the good Lord himself with the latest progress Arielle has made.
With that in mind, we ask everyone to keep up whatever it is they’re doing with the following intentions in mind:
- That Arielle’s ventricles and head size remain normal in size.
- That the two hemispheres of Arielle’s brain find other ways to communicate without the pathway its missing (it’s unclear why, but some brains seem to cope/compensate better than others).
- That Mary Alice’s challenges with anemia and fatigue not worsen (doctors were never able to get it to improve, and it’s known for worsening near the end of the pregnancy).
- That we’re able to find an experienced pediatrician for Arielle. The one the neurologist recommended no longer accepts new patients with our insurance, so our quest on that front continues.
- That delivery goes smoothly. Only six weeks to go!
Thanks to you all once again. Our next update may very well be a photo upon delivery.
- Mary Alice, Travis and Baby Arielle
"The greatest wealth is health."
-- Virgil
The quote rang particularly true for us yesterday, when our unborn baby was diagnosed with hydrocephalus. For those as unfamiliar with the condition as we were, it refers to a build up of fluid in the brain. Normally, the fluid drains into the spinal cord, but in our baby's case, remains trapped in the lateral ventricles. That, in turn, causes pressure on the brain and an enlarged head, symptoms our baby shows.
What does it mean?
It means many things as we face an uncertain and frightening future. Most importantly, it means all three of us would very much appreciate being kept in your thoughts and prayers at this time. Initially, Travis and I wanted to wait to find out the gender of our child until birth. It was such a struggle for us to conceive, we weren't concerned with "girl" or "boy" or "pink" or "blue" - just a healthy child. Since the diagnosis, we decided we wanted people to be able to pray for our baby by name. We are having a girl, her name is Arielle, and her sonogram photo is attached. We have found two meanings for the name: Lion of God and ethereal. The combination sums up well the balance of strength, determination, and grace our entire family will need as we cope with the situation at hand.
Medically, what's in store?
The doctor told us to prepare for a long roller coaster ride with a completely unpredictable ending. Worst case: the pressure builds, and Arielle dies before or shortly after she is born. Best case: the potential for a normal life. Everything in between is also on the table: surgeries, vision problems, learning disabilities, physical handicaps, etc. The outcome will depend on how the fluid behaves, on its own or through medical intervention. Naturally our prayer is for whatever is causing the build up to miraculously relieve itself, or for medical procedures to achieve the same result.
Mary Alice was immediately put through a series of tests yesterday that will hopefully help the doctors gain a clearer picture of the situation. To us laymen, the diagnosis and prognosis seems like a giant puzzle shattered into millions of tiny pieces. Every piece helps rule something in or out. Once the test results arrive, we will go to a specialized center in Houston, where they will perform an MRI, and hopefully be able to link more puzzle pieces together for us. That will be in about three to four weeks.
On a different front, Mary Alice's blood condition that prevents her from properly absorbing iron has been causing minor challenges from the beginning - primarily weakness and fatigue. Yesterday, her hematologist made adjustments to her treatment that we hope make more of a difference. We also ask for prayers on that front to help her body become as strong as possible to best support, carry and care for Arielle.
Future communication
It is with heaviest and humblest hearts we share this information with you. For those of you interesting in following our journey, Travis will be setting up a special blog page here on our family website where we will post information as it becomes available. We hope to have something up in the next few days.
We have already found comfort in our immediate family members, which are encircling us with their greatest love and support. We thank all of you for your friendship, and keeping us in your thoughts and prayers at this time.
- Travis, Mary Alice and Baby Arielle
The quote rang particularly true for us yesterday, when our unborn baby was diagnosed with hydrocephalus. For those as unfamiliar with the condition as we were, it refers to a build up of fluid in the brain. Normally, the fluid drains into the spinal cord, but in our baby's case, remains trapped in the lateral ventricles. That, in turn, causes pressure on the brain and an enlarged head, symptoms our baby shows.
What does it mean?
It means many things as we face an uncertain and frightening future. Most importantly, it means all three of us would very much appreciate being kept in your thoughts and prayers at this time. Initially, Travis and I wanted to wait to find out the gender of our child until birth. It was such a struggle for us to conceive, we weren't concerned with "girl" or "boy" or "pink" or "blue" - just a healthy child. Since the diagnosis, we decided we wanted people to be able to pray for our baby by name. We are having a girl, her name is Arielle, and her sonogram photo is attached. We have found two meanings for the name: Lion of God and ethereal. The combination sums up well the balance of strength, determination, and grace our entire family will need as we cope with the situation at hand.
Medically, what's in store?
The doctor told us to prepare for a long roller coaster ride with a completely unpredictable ending. Worst case: the pressure builds, and Arielle dies before or shortly after she is born. Best case: the potential for a normal life. Everything in between is also on the table: surgeries, vision problems, learning disabilities, physical handicaps, etc. The outcome will depend on how the fluid behaves, on its own or through medical intervention. Naturally our prayer is for whatever is causing the build up to miraculously relieve itself, or for medical procedures to achieve the same result.
Mary Alice was immediately put through a series of tests yesterday that will hopefully help the doctors gain a clearer picture of the situation. To us laymen, the diagnosis and prognosis seems like a giant puzzle shattered into millions of tiny pieces. Every piece helps rule something in or out. Once the test results arrive, we will go to a specialized center in Houston, where they will perform an MRI, and hopefully be able to link more puzzle pieces together for us. That will be in about three to four weeks.
On a different front, Mary Alice's blood condition that prevents her from properly absorbing iron has been causing minor challenges from the beginning - primarily weakness and fatigue. Yesterday, her hematologist made adjustments to her treatment that we hope make more of a difference. We also ask for prayers on that front to help her body become as strong as possible to best support, carry and care for Arielle.
Future communication
It is with heaviest and humblest hearts we share this information with you. For those of you interesting in following our journey, Travis will be setting up a special blog page here on our family website where we will post information as it becomes available. We hope to have something up in the next few days.
We have already found comfort in our immediate family members, which are encircling us with their greatest love and support. We thank all of you for your friendship, and keeping us in your thoughts and prayers at this time.
- Travis, Mary Alice and Baby Arielle
