Day #14 {Thursday)
Compared to Wednesday...Thursday was a walk in the park!
Nephrology came bright an early to help manage Audra's new high blood pressure issue. Their plan is to start Audra on a Clonidine patch and then slowly wean off the oral clonidine she takes. They think this will give her more steady, reliable absorption of the clonidine given her current GI issues. The theory for her high blood pressure is that the frequent adjustments of her clonidine dose during her first week here and her persistent vomiting caused inconsistent absorption of the med and resulted in a rebound reaction of her blood pressure. So if we can get consistent absorption then her blood pressure should be more steady.
Audra's GI doctor came by later to review all that has happened in the past two weeks and where we should go from here. Currently she is receiving a small amount of pedialyte continuously through her g-tube and she has not vomited since late Wednesday night. The plan is to get her back to full formula feeds as soon as possible but with slightly smaller volumes. And as long as she has no further vomiting, the rest of her issues {H.pylori treatment, pancreatic insufficiency evaluation/treatment} can be handled as an outpatient once her current tummy troubles are resolved.
It is a bit frustrating that we have put 2 long weeks in here at the hospital yet we still haven't figured out or solved the reason that brought us here in the first place--her behavior concerns. And we have seen new issues arise that have taken the priority, however we are not certain about the reason for her tummy and blood pressure issues. I really want to believe that some underlying tummy trouble was causing her erratic behavior for all those weeks since she is unable to communicate when something is bothering her. And I really, really hope that her tummy just fixes itself and she is back to her old self...happy, giggly, calm, content...very soon.
I am anxious to get home, but I am also worried that once we get there these same issues will surface again. These past two week have felt like a cyclical roller coaster ride. I just want her fixed and able to go back to school so I can go back to work.
Friday, April 7, 2017
Thursday, April 6, 2017
Medical Mystery...the unexpected chapter
Unlucky Day #13
Yesterday was day #13 on the inside here at CHOP. Things were looking pretty good in the morning. There was even talk of Audra going home today if everything continued in the right direction. She had been off the IV nutrition for a couple days and her labs looked good so about 11:00am she had her PICC line removed.
Then, not even one hour later, she threw up...
The doctors weren't concerned at first. Everyone has a free pass to vomit once a day they said.
And then she threw up again...
...and again
...and again.
By late-afternoon her blood pressure was trending higher, she was vomiting nearly every half hour and she was feeling quite terrible. Things started getting very worrisome about 8pm when her blood pressure hit 170/100 then 217/115 !! Audra was so restless and anytime she moved or was disturbed she said "ow"...which she NEVER says.
It was a flurry of activity in her room for a while...lots of nurses and doctors coming in and out. A new IV was placed, bloodwork and cultures were sent, and extra IV fluids were given. Things were getting really close to calling the ICU team to evaluate Audra.
But first, the doctors here called to discuss their concerns with Audra's immunologist and GI doc. They also called the nephrologist (kidney doctor) to see what they should do for her blood pressure through the night. They recommended 2 doses of clonidine...the new extended release version they had just started the night before and an additional smaller dose of the faster acting clonidine.
About midnight things started to settle down a bit. Her blood pressure was trending down to a safer range, and she was able to finally fall asleep.
She and I were able to get some decent rest overnight and she is still asleep now. Her blood pressure came down maybe too much overnight...now it's 80/40.
I am sure today will be a busy day with lots of Audra's doctors weighing in.
Our plan from yesterday is out to window. It's looking like we will be here for a bit longer now. It's so frustrating that we are right back to where we were last week. But clearly we have not figured this sweet girl out yet. I think everyone was happy that the vomiting resolved, but we still never had a reason for it in the first place.
So this time we need an answer.
Yesterday was day #13 on the inside here at CHOP. Things were looking pretty good in the morning. There was even talk of Audra going home today if everything continued in the right direction. She had been off the IV nutrition for a couple days and her labs looked good so about 11:00am she had her PICC line removed.
Here she is looking good right before the PICC removal
Then, not even one hour later, she threw up...
The doctors weren't concerned at first. Everyone has a free pass to vomit once a day they said.
And then she threw up again...
...and again
...and again.
By late-afternoon her blood pressure was trending higher, she was vomiting nearly every half hour and she was feeling quite terrible. Things started getting very worrisome about 8pm when her blood pressure hit 170/100 then 217/115 !! Audra was so restless and anytime she moved or was disturbed she said "ow"...which she NEVER says.
It was a flurry of activity in her room for a while...lots of nurses and doctors coming in and out. A new IV was placed, bloodwork and cultures were sent, and extra IV fluids were given. Things were getting really close to calling the ICU team to evaluate Audra.
But first, the doctors here called to discuss their concerns with Audra's immunologist and GI doc. They also called the nephrologist (kidney doctor) to see what they should do for her blood pressure through the night. They recommended 2 doses of clonidine...the new extended release version they had just started the night before and an additional smaller dose of the faster acting clonidine.
About midnight things started to settle down a bit. Her blood pressure was trending down to a safer range, and she was able to finally fall asleep.
She and I were able to get some decent rest overnight and she is still asleep now. Her blood pressure came down maybe too much overnight...now it's 80/40.
I am sure today will be a busy day with lots of Audra's doctors weighing in.
Our plan from yesterday is out to window. It's looking like we will be here for a bit longer now. It's so frustrating that we are right back to where we were last week. But clearly we have not figured this sweet girl out yet. I think everyone was happy that the vomiting resolved, but we still never had a reason for it in the first place.
So this time we need an answer.
Tuesday, April 4, 2017
Medical mystery...Days 10-12
Day #10 (Sunday)
Today we were able to accomplish getting Audra to full strength continuous g-tube feeds...and she successfully held it all down!
Because of the reappearance of some challenging behaviors and body movements yesterday, today they added a second daytime dose of clonidine to cover her into the evening.
She seemed extra tired today and took two decent naps...2 hours in the morning and over an hour in the afternoon.
By late evening they were able to wean her off the IV nutrition.
But the most exciting update of the day for Audra was that they took her off isolation precautions so she could finally leave her room for fun!! We went on lots of walks {well, rides for her in a little push car} but the rest of us got plenty of steps in ! She was also able to visit the playroom where she played with the toy kitchen and had a quick attempt at painting. Messy but fun!
Day #11 (Monday)
Audra's tummy troubles seeming to be heading in the right direction so today we got to work on a better plan for her behavior concerns. She has still been receiving her night time dose of clonidine for sleep, and since we have been here they have added a small 8am dose and another small 1pm dose to help with her behavior. But then she developed the new issue of spikes in her blood pressure that they think are a rebound effect each time a dose of clonidine wears off. So the new plan is to switch her to an extended release dose of clonidine at bedtime that will cover her for 24 hours and hopefully not result in the blood pressure spikes. But...the hospital doesn't carry this drug so the doctor had to order it as an outpatient med that I will pick up at the CVS downstairs. When I went down to pick it up, it turns out CVS needed to special order it, so I will arrive tomorrow afternoon.
The biggest news of today was that Audra was allowed to EAT by mouth! Sweet girl hasn't had a taste of anything in 9 days! Her face lit up at the sight of food in front of her...
Day #12 (Tuesday)
Today's goal was to get Audra back on her home g-tube feeding schedule of two daytime bolus feeds an then 12 hours continuous overnight....which would make us one step closer to HOME!
Audra's very lovely GI doctor stopped by to visit...turns out she has been away this whole time. I told her she missed a whammy of a week with Audra! But she had been kept in the loop by the GI doctor who saw Audra last week. She was very interested in the new genetic diagnosis of ZTTK syndrome that Audra received last week and she has already been doing her own research. She found it quite interesting that some of the other ZTTK kids have pancreatic insufficiency...something Audra has seemed to have for years without an explanation as to why. She has plans for future scoping with specific enzyme testing to more fully evaluate her insufficiency which will help us determine if med and/or formula changes are needed. But all that can be done after she has been discharged from this hospitalization.
With Audra moving in the right direction and no big plans for her medically today I decided to head upstairs and get a few hours of work in. It was nice to get away for a while and see the world outside of the four walls of her hospital room. But those 6 hours were the longest I have been away from her in the past 12 days! I couldn't wait to get back and see her. Turns out she was pretty sleepy the whole time I was gone. Lucky me...I guess she wanted to save up all her energy to share with me.
The new medication they ordered came in and I was able to pick it up from the CVS in time for her to receive her first dose tonight...fingers crossed that it helps with her behavior and blood pressure.
Today we were able to accomplish getting Audra to full strength continuous g-tube feeds...and she successfully held it all down!
Because of the reappearance of some challenging behaviors and body movements yesterday, today they added a second daytime dose of clonidine to cover her into the evening.
She seemed extra tired today and took two decent naps...2 hours in the morning and over an hour in the afternoon.
By late evening they were able to wean her off the IV nutrition.
But the most exciting update of the day for Audra was that they took her off isolation precautions so she could finally leave her room for fun!! We went on lots of walks {well, rides for her in a little push car} but the rest of us got plenty of steps in ! She was also able to visit the playroom where she played with the toy kitchen and had a quick attempt at painting. Messy but fun!
Day #11 (Monday)
Audra's tummy troubles seeming to be heading in the right direction so today we got to work on a better plan for her behavior concerns. She has still been receiving her night time dose of clonidine for sleep, and since we have been here they have added a small 8am dose and another small 1pm dose to help with her behavior. But then she developed the new issue of spikes in her blood pressure that they think are a rebound effect each time a dose of clonidine wears off. So the new plan is to switch her to an extended release dose of clonidine at bedtime that will cover her for 24 hours and hopefully not result in the blood pressure spikes. But...the hospital doesn't carry this drug so the doctor had to order it as an outpatient med that I will pick up at the CVS downstairs. When I went down to pick it up, it turns out CVS needed to special order it, so I will arrive tomorrow afternoon.
The biggest news of today was that Audra was allowed to EAT by mouth! Sweet girl hasn't had a taste of anything in 9 days! Her face lit up at the sight of food in front of her...
Day #12 (Tuesday)
Today's goal was to get Audra back on her home g-tube feeding schedule of two daytime bolus feeds an then 12 hours continuous overnight....which would make us one step closer to HOME!
Audra's very lovely GI doctor stopped by to visit...turns out she has been away this whole time. I told her she missed a whammy of a week with Audra! But she had been kept in the loop by the GI doctor who saw Audra last week. She was very interested in the new genetic diagnosis of ZTTK syndrome that Audra received last week and she has already been doing her own research. She found it quite interesting that some of the other ZTTK kids have pancreatic insufficiency...something Audra has seemed to have for years without an explanation as to why. She has plans for future scoping with specific enzyme testing to more fully evaluate her insufficiency which will help us determine if med and/or formula changes are needed. But all that can be done after she has been discharged from this hospitalization.
With Audra moving in the right direction and no big plans for her medically today I decided to head upstairs and get a few hours of work in. It was nice to get away for a while and see the world outside of the four walls of her hospital room. But those 6 hours were the longest I have been away from her in the past 12 days! I couldn't wait to get back and see her. Turns out she was pretty sleepy the whole time I was gone. Lucky me...I guess she wanted to save up all her energy to share with me.
The new medication they ordered came in and I was able to pick it up from the CVS in time for her to receive her first dose tonight...fingers crossed that it helps with her behavior and blood pressure.
4 years ago today...April 4, 2013
We boarded a plane {or two} bound for China and our sweet little treasure, Audra.
Little did we know the wild journey we would continue to take with her these past 4 years since welcoming her into our arms and hearts!
Saturday, April 1, 2017
Audra's medical mystery...Part 2
Audra slept well Wednesday night, had no vomiting and woke up Thursday morning looking a little bit perkier. We were actually starting to see a few smiles for the first time in many days.
We were blessed with a huge basket of goodies from my friends at work upstairs in the PICU. They have been stopping by often to visit, checking in multiple times per day via text, dropping off snacks and meals and even doing my laundry. I really do have the very best work friends ever!
My favorite item was absolutely the full-sized plush towel. After 4 days of showering in the hospital with hospital grade towels...which are scratchy and more like the size of a hand towel...having a large, soft towel was Heaven!
And now for the medical details:
Thursday started early with an 8:30am trip to radiology for a barium study to evaluate her stomach and intestines. Then we had to go back every 30-60 minutes for follow up x-rays. Which meant lots of wheelchair rides through the hospital. It was so nice to get out of her room for a while. She was finally done around 2:30pm.
She continued to have high blood pressures throughout the day, especially later in the afternoon. The running theory was that she may not have seen any of her usual nighttime clonidine dose for the past 5-6 days because of all her vomiting. And the high blood pressure could be the result of withdrawal from the medication. The day after she was admitted she was started on a small dose of clonidine in the morning (for her behavior issues) in addition to her nighttime dose (for her sleep disorder) so hopefully her blood pressure will normalize soon.
Later in the day the results of her barium study were available and there was no obstruction or structural reason for her vomiting. She did have very delayed gastric emptying which absolutely could be the reason for her vomiting. But we kinda knew that even before the study. So a normal study and no new vomiting meant they wanted to try again running pedialyte very slowly through her g-tube in addition to the IV nutrition. Her weight is down 3 pounds since coming in {which puts her way-way-way below the growth chart} so every calorie helps.
We were really looking forward to Friday...Daddy, Grandma and Grandpa were coming to visit! They were all here by 10:00am and luckily it was a lazy day for Audra with no tests scheduled. Grandma brought more balloons for Audra...including a butterfly that is as big as she is! We had a nice visit, Audra was so happy to see them! Sean and I were able to go down to the cafeteria for lunch and to catch up...which mostly meant talking about what needed to be done around the house and which kid needed to do what and go where this weekend :)
Later in the afternoon Audra started showing some of the behaviors that brought us here in the first place. She has felt so miserable the past 6 days, just been laying bed or sitting on my lap, sleeping and rolling around in discomfort. And, while we would be happy if those behaviors never came back, now is the time if they are going to. We are in the right place to get her help.
Saturday came and the doctors had big plans for Audra's belly. Around lunch time we changed her continuous G-tube feeding from just pedialyte to 1/4 strength formula. The IV nutrition was doing it's thing and her weight was creeping up a few ounces every day. After consulting with the GI doctors we had a good back pocket plan in case the addition of formula didn't go well.
Because it was Saturday again Audra was due for her weekly immunoglobulin infusion. We got that done without any issues. She was a tangled mess the whole time though, rolling around in bed with 5 tubings connected to her: 2 IV nutrition, 1 G-tube feeding, 1 IV med and the immunoglobulin infusion. It took me on one side of her bed and the 1:1 tech on the other side to constantly keep her from pulling something out.
Now that her belly is feeling better it is taking more effort to keep her distracted and occupied in bed. When she's happy she melts your heart with her beautiful smile and contagious giggle. She was happy to color for a little while in the new book she got from our PICU friends:
By mid-late afternoon Audra was really starting to struggle with her behavior. Her body was in constant motion...stiffening, arching, screaming, etc. And she was chewing on everything in sight. I went in search of a teething toy at the hospital gift shop and CVS but had no luck. We kept giving her empty feeding bags and IV tubing for her to play with and chew on so she would leave her real ones alone. Both the 1:1 tech and I were constantly trying to redirect and calm her. It was exhausting. But eventually the sun set, and once she took her nighttime meds she was asleep in 20 minutes. With her nighttime dose of clonidine she falls into a deep sleep, so her nurse was able to turn the lights on, draw all her blood work off her PICC line (including the extra blood for Genetics research) and hang her new bags of IV nutrition without waking her.
And that was the end of a very long week in the hospital. We are looking forward to a new week, with more progress in the right direction, and being steps closer to getting home.
Snuggling with the new fleece blanket she was given when she got her PICC line
My favorite item was absolutely the full-sized plush towel. After 4 days of showering in the hospital with hospital grade towels...which are scratchy and more like the size of a hand towel...having a large, soft towel was Heaven!
Audra got a special delivery from Aunt Kristy on Thursday...
...and oh boy did it bring a huge smile to her sweet face!
Thank you to everyone for the calls, texts, emails, well wishes and prayers.
The support we have received from everyone makes it so much easier to get through these long days and nights in the hospital.
And now for the medical details:
Thursday started early with an 8:30am trip to radiology for a barium study to evaluate her stomach and intestines. Then we had to go back every 30-60 minutes for follow up x-rays. Which meant lots of wheelchair rides through the hospital. It was so nice to get out of her room for a while. She was finally done around 2:30pm.
She continued to have high blood pressures throughout the day, especially later in the afternoon. The running theory was that she may not have seen any of her usual nighttime clonidine dose for the past 5-6 days because of all her vomiting. And the high blood pressure could be the result of withdrawal from the medication. The day after she was admitted she was started on a small dose of clonidine in the morning (for her behavior issues) in addition to her nighttime dose (for her sleep disorder) so hopefully her blood pressure will normalize soon.
Later in the day the results of her barium study were available and there was no obstruction or structural reason for her vomiting. She did have very delayed gastric emptying which absolutely could be the reason for her vomiting. But we kinda knew that even before the study. So a normal study and no new vomiting meant they wanted to try again running pedialyte very slowly through her g-tube in addition to the IV nutrition. Her weight is down 3 pounds since coming in {which puts her way-way-way below the growth chart} so every calorie helps.
We were really looking forward to Friday...Daddy, Grandma and Grandpa were coming to visit! They were all here by 10:00am and luckily it was a lazy day for Audra with no tests scheduled. Grandma brought more balloons for Audra...including a butterfly that is as big as she is! We had a nice visit, Audra was so happy to see them! Sean and I were able to go down to the cafeteria for lunch and to catch up...which mostly meant talking about what needed to be done around the house and which kid needed to do what and go where this weekend :)
Balloons from Grandma and Aunt Kristy
Friday afternoon the Genetic team came back to talk. They wanted to know if I had any questions after a couple days of processing the news of Audra's ZTTK syndrome diagnosis. They also requested my consent to include Audra in research regarding this new syndrome. They wanted to draw additional blood work and enroll her in a genetic database. They said that all journal publications, articles or papers that include information about her case will be shared with me. Audra and the few other children with this genetic disorder are essentially "writing the books" on ZTTK syndrome. What is learned from their medical issues and developmental progress will help determine the course and prognosis for future children who receive the same diagnosis.Later in the afternoon Audra started showing some of the behaviors that brought us here in the first place. She has felt so miserable the past 6 days, just been laying bed or sitting on my lap, sleeping and rolling around in discomfort. And, while we would be happy if those behaviors never came back, now is the time if they are going to. We are in the right place to get her help.
Saturday came and the doctors had big plans for Audra's belly. Around lunch time we changed her continuous G-tube feeding from just pedialyte to 1/4 strength formula. The IV nutrition was doing it's thing and her weight was creeping up a few ounces every day. After consulting with the GI doctors we had a good back pocket plan in case the addition of formula didn't go well.
Because it was Saturday again Audra was due for her weekly immunoglobulin infusion. We got that done without any issues. She was a tangled mess the whole time though, rolling around in bed with 5 tubings connected to her: 2 IV nutrition, 1 G-tube feeding, 1 IV med and the immunoglobulin infusion. It took me on one side of her bed and the 1:1 tech on the other side to constantly keep her from pulling something out.
Now that her belly is feeling better it is taking more effort to keep her distracted and occupied in bed. When she's happy she melts your heart with her beautiful smile and contagious giggle. She was happy to color for a little while in the new book she got from our PICU friends:
And a delivery from her home care nursing agency brought a big smile to her face.
She loves balloons...and the ribbons they are attached to !
The balloons were tied to a bunch of lollipops which she absolutely loves.
I had to quickly hide them in the closet, but at the very first word that she can put some food in her mouth again those lollipops will be the first thing she gets!
By mid-late afternoon Audra was really starting to struggle with her behavior. Her body was in constant motion...stiffening, arching, screaming, etc. And she was chewing on everything in sight. I went in search of a teething toy at the hospital gift shop and CVS but had no luck. We kept giving her empty feeding bags and IV tubing for her to play with and chew on so she would leave her real ones alone. Both the 1:1 tech and I were constantly trying to redirect and calm her. It was exhausting. But eventually the sun set, and once she took her nighttime meds she was asleep in 20 minutes. With her nighttime dose of clonidine she falls into a deep sleep, so her nurse was able to turn the lights on, draw all her blood work off her PICC line (including the extra blood for Genetics research) and hang her new bags of IV nutrition without waking her.
And that was the end of a very long week in the hospital. We are looking forward to a new week, with more progress in the right direction, and being steps closer to getting home.
Thursday, March 30, 2017
Audra's current medical mystery
I'm a few months behind on posts so this one is out of order. But I wanted to get an update posted on Audra's health.
(I have quite a few posts for January-March started but not finished yet so be sure to stop back and catch up once I get them posted)
Here's what's been happening {it reads like a true mystery novel!}...
Audra has been having behavioral changes for the past few months. But the past 6 weeks have been particularly challenging both at home and at school. Really this all goes back as far as early September. Audra had a UTI and was very sick while we were on vacation in late August. Once we got home and school started we noticed that she just wasn't the same. She had a flat affect, wasn't very interactive, hardly spoke the few words she usually does, and generally just seemed very zoned out. She was sleeping more than usual and seemed to have more frequent low grade temps. She was being sent home from school frequently for the low grade temps so I had her to the doctor a few times. At first we did the usual...blood work and urine tests. Her inflammatory markers were elevated which usually indicates an infection somewhere for Audra. They were thinking maybe the recent UTI she had was undertreated but multiple urine cultures came back negative. Over the multiple doctor visits in September and October we noticed that she was slowly losing weight. So with the abnormal bloodwork and weight loss her GI doctor decided to do upper and lower scopes in early November. Unfortunately they did not reveal a cause for her symptoms and she continued to lose weight so we had to add 2 daytime bolus G-tube feedings in addition to her usual overnight continuous feed. Slowly we saw her weight improve a bit, but not up to where she had been before all these mysterious symptoms started.
Then in December/January we noticed behavioral changes in Audra. She was easily agitated and even aggressive. She would scream, hit, kick, bite and have "tantrums"...all things we had not seen her do before. School started using the word "regression" to describe her. She was not doing things in school that she had been able to do at the end of the previous school year. And we noticed that she had a curious new interest in putting things in her mouth.
In mid February all of these behaviors escalated. She was disruptive in school to the point that I was being called to pick her up early multiple times. She was throwing her body, arching back and hitting her head to the point that we were worried she would hurt herself. Her body was constantly in motion to the point that she seemed uncomfortable in her skin. Things that never bothered her before seemed to bother her now. She was constantly pulled her glasses and hearing aids off, pulling on her G-tube and taking her cloths off. She was putting EVERYTHING in her mouth...even leaves, sticks, mulch, her g-tube dressing, paper, rubber bands...you name it. She was constantly shoving her fingers into her mouth and chewing on them.
We took her to urgent care to rule out the "easy" things like an ear infection or strep throat. And she checked out just fine. Then I had her back to her pediatrician who ordered more bloodwork and urine tests....which did not help in solving the mystery. So then I started touching base with each of her specialists individually...which is not the most efficient process. She continued to have new behavioral changes/regression including feeding herself with her hands instead of using utensils and daytime incontinence. So on Friday, March 17th she had an MRI of her brain and lumbar spine.
I was in communication with her neurologist the following week and her brain MRI didn't show anything acute that could be causing these symptoms...just the usual abnormalities that we already knew about. So she consulted with some of her colleagues and we made the decision to stop her ADHD med and start a new type of medication. Unfortunately, by later in the week her behavior had escalated to the point of risking her own safety. I was called to pick her up from school on both Thursday and Friday. So that Friday (3/24) I decided that she needed to be taken to the ER for an evaluation.
My sister drove Audra and I to the hospital and Audra was quite unsafe on the hour ride down. Even though we had her strapped into my niece's 5 point harness car seat she managed to get her arms out multiple times and even reached the door button once and got it ajar.
While we were walking into the triage area Audra was screaming, arching and flailing her body all over the place. The staff later told us that at first they thought she was just another child who was not happy about coming to the hospital...then they saw her smiling and laughing and noticed the way my sister and I alternated between holding her and catching her from the other's arms and they knew something wasn't right. That bought us a ticket back to an exam room within seconds.
After a long few hours in the ER it was confirmed that Audra would be admitted for a behavioral/psych evaluation. Which was a relief...because we needed help. Our sweet little Audra was not, and had not been herself for quite too long. In the beginning we had to make sure it wasn't a complication of one of her many medical problems that was causing the severe behavioral issues. She is so complex medically and her lack of language skills makes it impossible for her to let us know what she's feeling. But when test after test didn't reveal an answer we were left with only the behavioral/psych department as a possible cause.
Luckily CHOP has a brand new unit for kids with this exact issue. Their Medical/Behavioral Unit is for kids with underlying psychological or behavioral diagnoses who also have medical issues. But unfortunately the unit was full. So, Audra was admitted to a unit she has been on multiple times before...home of the Immunology service. Which was a good fit since she was due for her weekly immunoglobulin infusion the following day and they would know exactly how to do it :) Audra was assigned a 1:1 tech to sit in the room with her 24 hours a day to observe her behavior and help keep her safe.
Our first night was fairly uneventful after she got her usual nighttime dose of sleeping meds. Saturday morning she woke up happy and I ordered her breakfast. She seemed eager to eat but after a few bites she threw up. I gave her a break, then later after having a drink she threw up again ?! And as the day went on it continued. Then I remembered that her school nurse had said she threw up twice on Friday before she called me to pick her up. At the time I was thinking it was related to being so agitated. And she threw up after having a drink in the ER the night before...but again she had been so agitated.
By mid-morning we got word that the Medical/Behavioral Unit had a bed available for Audra and the doctors covering that unit came to see Audra. Both a General Pediatrician and a Pediatric Psychiatrist partner in the care of patients on this unit. Audra was finally able to get her immunoglobulin infusion started later Saturday evening and then we moved to the new unit. The plan was to run some pedialyte through her G-tube overnight and then get started on the behavioral stuff in the morning. But Audra had other plans...
She vomited shortly after the pedialyte was started so they decided to rest her belly overnight. Sunday morning came and she vomited after every sip she took. That bought her an IV and then she had an abdominal ultrasound and an x-ray. Neither of them revealed anything concerning so they continued to challenge her tummy with pedialyte. And each and every time it failed. By this time sweet Audra was starting to feel really terrible. She was nauseous and lethargic nearly all day long, except for short periods after vomiting when her belly was completely empty. Then the cycle started all over again.
Monday brought a new team of doctors and a fresh set of eyes. It also brought the abundance of resources available in this amazing unit....psychologists, social workers, behavioral analysts, developmental pediatricians...It was a revolving door of people coming into our room! However by this time it was clear that Audra's GI issues were a priority and the whole reason we were admitted in the first place (her behavioral issues) took a back burner. Audra continued to vomit persistently and another symptom became persistent also...the right corner of her mouth twitched nearly constantly. This led to an EEG for Audra and a neurology consult. Poor Audra's vomiting was only eased by continuously draining her G-tube to a bag and giving anti-nausea medicine around the clock.
In somewhat completely unrelated but very eagerly anticipated news...
We received word that Audra's long awaited genetic testing results were completed. And the Genetic team would be coming to see us the following day to discuss the results. Nearly 3 years ago our insurance company had denied this very extensive {and expensive} genetic testing for Audra (whole exome sequencing). But at that time CHOP was conducting a research study that involved the same exact genetic testing so Audra was enrolled in the study instead. When 2.5 years had gone by without any results from the study I asked Audra's Genetic team to resubmit a request to our insurance company for this genetic testing. By that time we had a new insurance company and Audra had added to her laundry list of medical diagnoses. This time the insurance company approved the testing and Audra had her blood drawn 12-28-16 with results expected in approximately 8 weeks. And well, here we are 12 weeks later, in the hospital for an entirely new issue and the results were IN !!!
To say I slept restlessly Monday night is an understatement. The anticipation of what was happening currently with Audra's GI illness AND the anticipation of genetic testing results was a lot and weighed heavily on my mind and heart.
8 very long hours later Tuesday morning came. First it brought another ultrasound for Audra, which again did not reveal a reason for her persistent vomiting. This led to "the talk". Audra's doctors mentioned to me that it was time to start thinking about giving Audra IV nutrition (TPN) since she hadn't had any food/nutrition since Thursday...which was 5 days ago. And if she needed TPN then she needed to have a central IV placed, a PICC line. And even though this sounds like a lot, my heart was a bit relieved. This meant that they were finally accepting the fact that Audra's little tummy just was NOT going to tolerate having anything in it. And although we didn't have a reason yet, they knew they needed to rest her belly for a period of time. Which meant I wouldn't have to see my sweet girl writhing in discomfort from nausea anymore and vomit every couple hours from the repeated attempts to get even tiny amounts of pedialyte into her.
Then 1:00 came and it was time for the genetic testing results that were nearly 3 years in the making. Two people from the Genetics team, 3 people from the Neurology team and one of the doctors from the unit Audra is on came in to see me. They got right to the point. They had found a mutation in one of Audra's genes that they think explains her multiple medical and developmental issues. The gene with the mutation is called the SON gene and the specific mutation is called ZTTK syndrome. It is a very newly identified genetic disorder that has only been identified in approximately 20 children in the world so far. As of now the information is still very limited but a paper published recently that describes 20 children with the syndrome noted ALL of Audra's medical and developmental issues in most/some of the 20 children:
Developmental delay
Non-verbal
Hearing loss
Structural brain abnormalities
Failure to thrive with G-tube
Pancreatic insufficiency
Immunodeficiency
Vesicoureteral reflux and kidney issues
These 20 children sound like little Audra clones !!
And while this doesn't really solve or fix anything for Audra...there's no magic pill, potion or lotion that will "cure" any or all of her medical/developmental issues... it is somewhat of a relief to know that all of her many issues do fit together. And there are other children out there who are just like Audra.
After a few tears, tissues and some reassurance from the genetics team it was time to turn our attention to some of the current issues. Neurology let me know that her EEG from the day prior was stably abnormal, not different from her previous EEGs. And the mouth twitching that was captured was not seizure activity. But she did continue to have abnormal "discharges" and now that we know she has ZTTK syndrome (in which there is a tendency to have seizures) they felt she needed an overnight video EEG.
Our final doctor visitor of the day was GI. Audra's GI doctor is on vacation this week so one of her colleagues came by to consult on Audra. Many big bad GI things that could be causing her vomiting have been ruled out: pancreatitis, malrotation, volvulus, intussusception, etc. Which leaves the more standard things like gastroenterovirus (not mommy's vote) and ileus (mommy's vote). Audra has had an ileus twice in the past and this time she is acting exactly like the other two times. It's cyclical...she gets nauseous, irritable and uncomfortable anytime her belly has anything in it, then she vomits and is more comfortable until her belly fills up a bit again. The only way to make her comfortable is to keep her belly continuously draining to a bag and give her anti-nausea medicine. And that sounds exactly like the past few days! Either way doesn't matter, treatment really is the same....bowel rest. Don't put anything in, and keep her tummy drained. Then start up very, very slowly again in a few days. So that is what I pleaded with the doctors to do...and STOP trying to challenge her poor little tummy until after she gets the PICC line and starts TPN.
We finished off our very busy day with a trip up the neurology floor to have the EEG leads placed. Not a fun process for Audra but she made it through and we got back to her room just in time for her nighttime sleep meds and *hopefully* a good night's rest since she'll be getting bowel rest too.
Wednesday we woke up refreshed and ready to finally make some progress in getting her belly feeling better. Audra {and mommy} slept pretty well since she had bowel rest overnight so she wasn't nauseous or vomiting through the night. I just knew that once she had the PICC line in place and TPN infusing her doctors wouldn't feel so pressured to get some kind/any kind of nutrition into her tummy. Which meant my sweet girl would finally have some relief from the constant vomiting.
It was a long wait until we finally we got the call at 3:15 to go down. She was happy to get into a wheelchair and go for a ride. Being inside the same four walls for the past 5 days is getting to us!
(I have quite a few posts for January-March started but not finished yet so be sure to stop back and catch up once I get them posted)
Here's what's been happening {it reads like a true mystery novel!}...
Audra has been having behavioral changes for the past few months. But the past 6 weeks have been particularly challenging both at home and at school. Really this all goes back as far as early September. Audra had a UTI and was very sick while we were on vacation in late August. Once we got home and school started we noticed that she just wasn't the same. She had a flat affect, wasn't very interactive, hardly spoke the few words she usually does, and generally just seemed very zoned out. She was sleeping more than usual and seemed to have more frequent low grade temps. She was being sent home from school frequently for the low grade temps so I had her to the doctor a few times. At first we did the usual...blood work and urine tests. Her inflammatory markers were elevated which usually indicates an infection somewhere for Audra. They were thinking maybe the recent UTI she had was undertreated but multiple urine cultures came back negative. Over the multiple doctor visits in September and October we noticed that she was slowly losing weight. So with the abnormal bloodwork and weight loss her GI doctor decided to do upper and lower scopes in early November. Unfortunately they did not reveal a cause for her symptoms and she continued to lose weight so we had to add 2 daytime bolus G-tube feedings in addition to her usual overnight continuous feed. Slowly we saw her weight improve a bit, but not up to where she had been before all these mysterious symptoms started.
Then in December/January we noticed behavioral changes in Audra. She was easily agitated and even aggressive. She would scream, hit, kick, bite and have "tantrums"...all things we had not seen her do before. School started using the word "regression" to describe her. She was not doing things in school that she had been able to do at the end of the previous school year. And we noticed that she had a curious new interest in putting things in her mouth.
In mid February all of these behaviors escalated. She was disruptive in school to the point that I was being called to pick her up early multiple times. She was throwing her body, arching back and hitting her head to the point that we were worried she would hurt herself. Her body was constantly in motion to the point that she seemed uncomfortable in her skin. Things that never bothered her before seemed to bother her now. She was constantly pulled her glasses and hearing aids off, pulling on her G-tube and taking her cloths off. She was putting EVERYTHING in her mouth...even leaves, sticks, mulch, her g-tube dressing, paper, rubber bands...you name it. She was constantly shoving her fingers into her mouth and chewing on them.
We took her to urgent care to rule out the "easy" things like an ear infection or strep throat. And she checked out just fine. Then I had her back to her pediatrician who ordered more bloodwork and urine tests....which did not help in solving the mystery. So then I started touching base with each of her specialists individually...which is not the most efficient process. She continued to have new behavioral changes/regression including feeding herself with her hands instead of using utensils and daytime incontinence. So on Friday, March 17th she had an MRI of her brain and lumbar spine.
I was in communication with her neurologist the following week and her brain MRI didn't show anything acute that could be causing these symptoms...just the usual abnormalities that we already knew about. So she consulted with some of her colleagues and we made the decision to stop her ADHD med and start a new type of medication. Unfortunately, by later in the week her behavior had escalated to the point of risking her own safety. I was called to pick her up from school on both Thursday and Friday. So that Friday (3/24) I decided that she needed to be taken to the ER for an evaluation.
My sister drove Audra and I to the hospital and Audra was quite unsafe on the hour ride down. Even though we had her strapped into my niece's 5 point harness car seat she managed to get her arms out multiple times and even reached the door button once and got it ajar.
While we were walking into the triage area Audra was screaming, arching and flailing her body all over the place. The staff later told us that at first they thought she was just another child who was not happy about coming to the hospital...then they saw her smiling and laughing and noticed the way my sister and I alternated between holding her and catching her from the other's arms and they knew something wasn't right. That bought us a ticket back to an exam room within seconds.
After a long few hours in the ER it was confirmed that Audra would be admitted for a behavioral/psych evaluation. Which was a relief...because we needed help. Our sweet little Audra was not, and had not been herself for quite too long. In the beginning we had to make sure it wasn't a complication of one of her many medical problems that was causing the severe behavioral issues. She is so complex medically and her lack of language skills makes it impossible for her to let us know what she's feeling. But when test after test didn't reveal an answer we were left with only the behavioral/psych department as a possible cause.
Luckily CHOP has a brand new unit for kids with this exact issue. Their Medical/Behavioral Unit is for kids with underlying psychological or behavioral diagnoses who also have medical issues. But unfortunately the unit was full. So, Audra was admitted to a unit she has been on multiple times before...home of the Immunology service. Which was a good fit since she was due for her weekly immunoglobulin infusion the following day and they would know exactly how to do it :) Audra was assigned a 1:1 tech to sit in the room with her 24 hours a day to observe her behavior and help keep her safe.
Tucked in for her first night in the hospital
By mid-morning we got word that the Medical/Behavioral Unit had a bed available for Audra and the doctors covering that unit came to see Audra. Both a General Pediatrician and a Pediatric Psychiatrist partner in the care of patients on this unit. Audra was finally able to get her immunoglobulin infusion started later Saturday evening and then we moved to the new unit. The plan was to run some pedialyte through her G-tube overnight and then get started on the behavioral stuff in the morning. But Audra had other plans...
She vomited shortly after the pedialyte was started so they decided to rest her belly overnight. Sunday morning came and she vomited after every sip she took. That bought her an IV and then she had an abdominal ultrasound and an x-ray. Neither of them revealed anything concerning so they continued to challenge her tummy with pedialyte. And each and every time it failed. By this time sweet Audra was starting to feel really terrible. She was nauseous and lethargic nearly all day long, except for short periods after vomiting when her belly was completely empty. Then the cycle started all over again.
Monday brought a new team of doctors and a fresh set of eyes. It also brought the abundance of resources available in this amazing unit....psychologists, social workers, behavioral analysts, developmental pediatricians...It was a revolving door of people coming into our room! However by this time it was clear that Audra's GI issues were a priority and the whole reason we were admitted in the first place (her behavioral issues) took a back burner. Audra continued to vomit persistently and another symptom became persistent also...the right corner of her mouth twitched nearly constantly. This led to an EEG for Audra and a neurology consult. Poor Audra's vomiting was only eased by continuously draining her G-tube to a bag and giving anti-nausea medicine around the clock.
In somewhat completely unrelated but very eagerly anticipated news...
We received word that Audra's long awaited genetic testing results were completed. And the Genetic team would be coming to see us the following day to discuss the results. Nearly 3 years ago our insurance company had denied this very extensive {and expensive} genetic testing for Audra (whole exome sequencing). But at that time CHOP was conducting a research study that involved the same exact genetic testing so Audra was enrolled in the study instead. When 2.5 years had gone by without any results from the study I asked Audra's Genetic team to resubmit a request to our insurance company for this genetic testing. By that time we had a new insurance company and Audra had added to her laundry list of medical diagnoses. This time the insurance company approved the testing and Audra had her blood drawn 12-28-16 with results expected in approximately 8 weeks. And well, here we are 12 weeks later, in the hospital for an entirely new issue and the results were IN !!!
To say I slept restlessly Monday night is an understatement. The anticipation of what was happening currently with Audra's GI illness AND the anticipation of genetic testing results was a lot and weighed heavily on my mind and heart.
8 very long hours later Tuesday morning came. First it brought another ultrasound for Audra, which again did not reveal a reason for her persistent vomiting. This led to "the talk". Audra's doctors mentioned to me that it was time to start thinking about giving Audra IV nutrition (TPN) since she hadn't had any food/nutrition since Thursday...which was 5 days ago. And if she needed TPN then she needed to have a central IV placed, a PICC line. And even though this sounds like a lot, my heart was a bit relieved. This meant that they were finally accepting the fact that Audra's little tummy just was NOT going to tolerate having anything in it. And although we didn't have a reason yet, they knew they needed to rest her belly for a period of time. Which meant I wouldn't have to see my sweet girl writhing in discomfort from nausea anymore and vomit every couple hours from the repeated attempts to get even tiny amounts of pedialyte into her.
Then 1:00 came and it was time for the genetic testing results that were nearly 3 years in the making. Two people from the Genetics team, 3 people from the Neurology team and one of the doctors from the unit Audra is on came in to see me. They got right to the point. They had found a mutation in one of Audra's genes that they think explains her multiple medical and developmental issues. The gene with the mutation is called the SON gene and the specific mutation is called ZTTK syndrome. It is a very newly identified genetic disorder that has only been identified in approximately 20 children in the world so far. As of now the information is still very limited but a paper published recently that describes 20 children with the syndrome noted ALL of Audra's medical and developmental issues in most/some of the 20 children:
Developmental delay
Non-verbal
Hearing loss
Structural brain abnormalities
Failure to thrive with G-tube
Pancreatic insufficiency
Immunodeficiency
Vesicoureteral reflux and kidney issues
These 20 children sound like little Audra clones !!
And while this doesn't really solve or fix anything for Audra...there's no magic pill, potion or lotion that will "cure" any or all of her medical/developmental issues... it is somewhat of a relief to know that all of her many issues do fit together. And there are other children out there who are just like Audra.
The official exome report with a diagnosis in writing
After a few tears, tissues and some reassurance from the genetics team it was time to turn our attention to some of the current issues. Neurology let me know that her EEG from the day prior was stably abnormal, not different from her previous EEGs. And the mouth twitching that was captured was not seizure activity. But she did continue to have abnormal "discharges" and now that we know she has ZTTK syndrome (in which there is a tendency to have seizures) they felt she needed an overnight video EEG.
Our final doctor visitor of the day was GI. Audra's GI doctor is on vacation this week so one of her colleagues came by to consult on Audra. Many big bad GI things that could be causing her vomiting have been ruled out: pancreatitis, malrotation, volvulus, intussusception, etc. Which leaves the more standard things like gastroenterovirus (not mommy's vote) and ileus (mommy's vote). Audra has had an ileus twice in the past and this time she is acting exactly like the other two times. It's cyclical...she gets nauseous, irritable and uncomfortable anytime her belly has anything in it, then she vomits and is more comfortable until her belly fills up a bit again. The only way to make her comfortable is to keep her belly continuously draining to a bag and give her anti-nausea medicine. And that sounds exactly like the past few days! Either way doesn't matter, treatment really is the same....bowel rest. Don't put anything in, and keep her tummy drained. Then start up very, very slowly again in a few days. So that is what I pleaded with the doctors to do...and STOP trying to challenge her poor little tummy until after she gets the PICC line and starts TPN.
We finished off our very busy day with a trip up the neurology floor to have the EEG leads placed. Not a fun process for Audra but she made it through and we got back to her room just in time for her nighttime sleep meds and *hopefully* a good night's rest since she'll be getting bowel rest too.
Sound asleep for the video EEG
And some serious bedhead in the morning!
Wednesday we woke up refreshed and ready to finally make some progress in getting her belly feeling better. Audra {and mommy} slept pretty well since she had bowel rest overnight so she wasn't nauseous or vomiting through the night. I just knew that once she had the PICC line in place and TPN infusing her doctors wouldn't feel so pressured to get some kind/any kind of nutrition into her tummy. Which meant my sweet girl would finally have some relief from the constant vomiting.
It was a long wait until we finally we got the call at 3:15 to go down. She was happy to get into a wheelchair and go for a ride. Being inside the same four walls for the past 5 days is getting to us!
Every other time Audra has had anesthesia I have suited up and gone into the OR with her. But she was feeling pretty calm and content and she willingly went to the nurse's arms from mine right outside the door. The nurse told me later that Audra was calm but wrapped her arms and legs around her with all her strength and wouldn't lay down. So they gave her meds in her IV while the nurse held her and she quickly fell asleep. The nurse fell in love with Audra's sweet self and so she came out with a shiny new PICC line AND a fluffy new blanket...one of the many that are donated to the hospital to give to kids who are experiencing way more than any kid should have to. Audra's recovery was a little rocky, she was still vomiting, her blood pressure was very high and she had a fever. The anesthesiologist was a little concerned and kept an extra eye on her for a while. She needed some oxygen at first and they said she had vomited while they were placing the breathing tube so they were worried some of the vomit could have gotten in her lungs. Over time her breathing settled out but her blood pressure remained high. And then it hit me...over the past few days the nurses were having trouble getting her blood pressure. It would be high and they wouldn't believe it, thinking instead that she was moving too much or fighting the cuff. But now she was perfectly still and asleep and her blood pressure was 160/100. I wondered if this had anything to do with her mysterious vomiting the past 5 days. By this time she was due for her nighttime dose of clonidine for sleep, which they thought would also help reduce her blood pressure. We made it back to her room about 8:00pm...got her clonidine in her, started the highly anticipated TPN and put this day in the books!
To be continued...
Thursday, March 23, 2017
Confirmed
Riley and Aidan both received the Sacrament of Confirmation this evening.
How special it has been for the two of them to share receiving the Sacraments together.
When Aidan started CCD in first grade we decided to try homeschooling Riley for CCD. We knew at that age that he wouldn't be able to handle the regular CCD classroom. It worked well for a few years and then they began offering a Special Needs CCD class and Riley transitioned beautifully to attending that.
Now, after seven years of Religious education, both my boys are being Confirmed...
together !!!
Many years ago I might not have believe this was possible for Riley.
Riley took the name of St. Peter
Riley Connor Peter Boyle
Aidan took the name of St. Christopher
Aidan Sean Christopher Boyle
My mom was Riley's sponsor...
And my dad was Aidan's sponsor...
The boys wore the same monogrammed ties that I had made for their First Communion four years ago.
Wednesday, March 22, 2017
March updates
Aidan is finally Cast-Free !!! It's been a long 8 weeks with him in a cast. His bones were a little slow to heal at first but they finally got themselves together (pun..haha!) and he graduated to a splint. Let the soaking and scrubbing and moisturizing of icky flaky skin begin !!
We had our first (and probably last) real significant snow of the season. Only one other time in February was there enough snowfall to actually suit up an go play in it (and that might have been pushing it!)
Riley had his last big Swimming Meet of the season.
I love watching him swim,
just wish the meets weren't so long,
with so much time in between each of his events.
Someone finds long swim meets in warm natatoriums exhausting...
We had our first (and probably last) real significant snow of the season. Only one other time in February was there enough snowfall to actually suit up an go play in it (and that might have been pushing it!)
Blitz's 1st time in the snow,
and he wasn't much of a fan...
My little Irish girl ready for school on St. Patrick's day
And my not-so-Irish girl playing the part too.
But she wasn't going to school,
She had a date with the MRI scanner...
Her nephrologist ordered an MRI of her spine to rule out certain causes of her sudden loss of potty training skills.
So her neurologist added on an MRI of her brain to look for causes of her recent drastic changes in behavior and loss of developmental skills.
She was a champ!
Got her IV in with no trouble and they were able to send off the massive amount of bloodwork that had been ordered by her various specialists.
We saw lots of familiar faces...
so many nurses that used to work with me in the PICU now work in Sedation,
including her Godmother.
When Audra saw Renee she clung on to her for dear life,
I think wishing she would take her away.
Sweet girl snuggled up and fell asleep in my arms before they even gave her any anesthesia!
Once we were all done she was rewarded with a big old Shamrock Shake!
Both Riley and Maura had pieces of their school artwork in the District Art Show
Subscribe to:
Posts (Atom)