We knew in June that Sara would need her next follow up visit at Mayo
Clinic near the end of the year. I wanted to coordinate with her Christmas
Break schedule to minimize the number of days she would miss school. Finally at
the beginning of October, doctor calendars were posted for us to set
appointments. Adam was also due for a routine annual ultrasound to make sure scar
tissue had not reformed from his uretero-pelvic junction blockage surgery in early
2013. After our local doctor told us he was out of answers with Sara and wanted
to wait for her kidneys to fail and just do a transplant, we lost all trust
with him and weren’t comfortable doing Adam’s follow up with him either.
Instead, we decided to take Adam with us to Mayo and meet with the doctors we are most
familiar with now.
December 21 is what we settled on, the first day of their winter break
from school. December 21. There was just something about that date. The date
and the appointments were on my mind often and I felt anxious about it. I
finally realized it was the same day Sara had her Boston surgery. An experience
that goes from a 4 hour surgery and overnight hospital stay to 11 hours and 4
days in the hospital, including Christmas, kind of stays with you. I will never
forget that image of seeing Sara for the first time in recovery. She was in so
much pain after that and didn’t smile for days.
I asked her a month ago what she remembers about her Boston surgery and
she just stared at me blankly. She doesn’t remember the aquarium and other fun
things we did. She doesn’t remember the hospital with fish tanks all over and
the cool trains that delivered meals. She doesn’t remember anything about
the pain or throwing up on the airplane home or how hard it was. I was thankful
and didn’t ask too many leading questions that would give her a memory that her
mind had already erased. Then I asked her if she remembered anything about her
first surgery at Mayo last summer. “What do you mean?” Well, Boston was a long time ago and you were very little but Mayo was not
that long ago. Do you remember anything from that? Again I got the blank
stare. She remembers playing Bingo in her room via video conferencing and
winning a doll. She remembers playing a video game with Adam where cows jumped
over bales of hay. That’s it. She doesn’t remember any ambulance rides. She
doesn’t remember the surgery. She doesn’t remember flying on the airplane and
having drainage bags attached to her back for days. As a mom, I will take on
the burden of remembering every little detail if it means she has no recollection.
I have learned even more the importance of keeping it light. I don’t even
recall asking for the gift of having Sara’s memory wiped from these difficult
experiences but what a wonderful gift that is!
So I have been breathing alot lately, trying to calm myself of the
anxiety that December 21 brings.
December 20 was an early dismissal day for the big kids but we pulled
them out of school in order to make the drive to Minnesota. We stopped by
school on the way out of town so Adam could pick up a present he had made us and didn’t
have a chance to bring home. Monday was a snow day, or rather cold temps and
icy conditions day. Sara’s teacher happened to stop in the office while I was
there and she asked if we wanted to take Sara’s gingerbread house with us to
decorate. Sara was heartbroken when school was called off Monday because she
knew she would miss that project that she had been looking forward to for a
week. The teacher headed to her classroom and came back with a graham cracker
house assembled, already attached to a cardboard disc and a bag full of candy
to decorate and an icing bag! She then brought down a second house for Adam. So
many kids were out sick that she had extras! What a wonderful surprise for the
kids! They decorated them Thursday night once we got back to Iowa. Adam decorated his and then we heard a giant crunch as he bit into the roof. I think it was a little challenging since he is missing his 2 front teeth!
We drove to Cedar Rapids to have lunch with Shannon’s parents and leave
Evan for 2 days. It was his first time being away from both Shannon and I.
Thankfully, he is so easy going that I wasn’t worried about it and I had plenty
of other things on my mind. Then we got back in the car and drove the rest of
the way to Rochester, about 6 ½ hours in the car total. A quick stop for dinner
and then we visited my aunt Robyn for a bit who had knee replacement surgery at
Mayo the day before.
Off to our hotel room and what a room it was! We have enjoyed staying
at TownePlace Suites a few times and this time they had the 2 bedroom suite
available. That’s 2 actual bedrooms with doors that shut, which is huge when
traveling with kids. So the kids had their own bedroom with a closet and a full
bathroom, we had a bedroom with closest and bathroom, a living room, and a full
size kitchen. Since it was late and they were both getting a cold, you would think
they would have been tired enough not to pester each other when sharing a bed.
I guess it is just too tempting. He hit me. She kissed my butt. Really? We
moved Adam to our room to fall asleep and even then he laid there awake until
9pm for some reason.
Shannon and I ate breakfast in shifts to let the kids sleep. Adam had a
breakfast sandwich to go and Sara had to fast for her EXU test at 10am. We left
the hotel at 7:20am to get parked and stop at the Business Office to pay our
bill for the day since we don’t have insurance. First ultrasound appointment
was at 8:00am. Of course, even though Adam was scheduled for that appointment,
he volunteered his sister to go first. The tech was great and made the 1hr +
pass pretty quickly with all the questions she asked the kids. It was also
interesting to learn that they don’t let the sono techs work in the peds area
for more than 4 hours in a day or more than 1 day in a row. You never know what
kind of challenge you are going to get with kids and it can be very difficult
as a shift. It was nice to hear a small way that they honor their employees.
9:30 and we were off quickly to get to the parking deck and head down
the street to St. Mary’s for Sara’s EXU test. The EXU is a radiological test with contrast to check how things flow
thru the kidneys, into the ureters, and then the bladder. It was to help
see if the 3 areas of her left kidney that were a pinpoint size opening
at her last surgery in May had remained open or scarred shut again. We made it there right at 10:00
and checked in. We waited…and waited…
At 11:20, I went back to the desk and the man was gone. Another woman said
he physically went back there to see what was happening because he knew Sara
was fasting and he wasn’t hearing back. She hadn’t had anything to eat or drink
since 6:00 the night before.
At 11:40, the man came to tell me someone was on the phone that I
needed to talk to. The caller introduced herself and said we needed to come back
down to Mayo and they would do the test on 16 East where we were for the
ultrasound. I said okay and made sure they would still be able to draw blood
when they put the IV in to save an extra stick. She told me they would draw her
blood out front. I said that I didn’t want a separate blood draw and was assured
they could take blood at the time they put the IV in for the contrast. Then the
woman told me they didn’t do IV contrast in that department so they were going
to have to do more research where we could have the test done. She took my cell
phone number and said she would call me when she had it figured out. The man at
the registration desk told me they don’t do that test anymore and it shouldn’t
have been ordered. Unbelievable. We gathered up all our things and walked to
the lobby in order to get cell coverage. Shannon and Adam went to the cafeteria
for lunch while Sara and I played on the iPad.
At 12:15, the pager went off and we gathered all our things and headed
back down the hall. Multiple people met me and started apologizing profusely.
They were prepping a room and would be taking us back in a few minutes. Shannon
found us and then got me a granola bar before having to leave with Adam to go
back to the Mayo campus for Adam’s 12:45 urology appointment with Dr. Granberg
for his ultrasound results.
At 12:30, 2 people came out and apologized again, handing us a free
parking pass, 4 vouchers for free snacks, and $10 to be used in the cafeteria
for our inconvenience. I had explained to the kids 3 hours earlier that we were
going to have an attitude of patience and flexibility today because we didn’t
know how the day was going to go. Good thing I said it out loud and had a
chance to model it. I told them I was glad I said something to the registration
desk when I did and I knew it was not typical service from Mayo.
They pulled a nurse in from the CT/MRI department to start doing vitals
and coordinating the IV and labs. To add to the excitement of the day, they
forgot to put a hospital ID band on Sara. The nurse knew it was a rush to get
the labs processed in time for the nephrologist to analyze results before our
3:25 appointment so she had an extra person come right away to begin the
analysis on her cart. The IV port went in very well but I knew something was
off when he flushed the line a little and asked Sara if she felt any burning.
Then he asked the lab tech if any of the vials looked thick. Of course, one
did, and he pulled the IV out right away. He thought he had chosen a good vein
but it wasn’t strong enough to support it. If the contrast got into her body
instead of in her vein it would be damaging to her body and he didn’t want to
chance it. He found a new spot, redrew the one vial of blood and got her ready
for the IV.
By now it was 1:00 and 2 ladies poked their head in who seemed like they
didn’t really know where they were supposed to be. They verified Sara’s name
and said they were going to take her to x-ray. They wheeled her in the hall and
then had a discussion about which direction they should go to get there. I made
a comment like do you really know what you are doing? They said they are used
to someone else bringing them their patients instead of getting them
themselves. So these were the 2 x-ray techs that were going to be doing Sara’s
EXU.
We got into the room, transferred Sara to the x-ray table, switched out
pillows, and started getting supplies ready. Their story was that although the
registration desk could see Sara’s name and the test that was ordered, it never
showed up on radiology’s to-do list because radiology doesn’t do that test
anymore. They have changed for them to be with a CT for adults but don’t want
kids to get that much radiation. More pieces to the puzzle as to why this all
went wrong today.
There were 2 “balloons” that they placed on Sara’s lower abdomen that
looked like a flat, square Ace Bandage. When the tech squeezed something like a
blood pressure cuff bubble, they expanded. A flat foam board went on top of the
balloons and then a velcro belt to hold it all tight. The balloons would be
expanded to push on her abdomen so the contrast would stay in her kidneys until
they filled and images could be taken. Then the belt would be released so the
contrast could drain thru her ureters and into her bladder while taking more
images.
They were ready to start the test and went to find a free nurse from CT
to put the contrast in. For someone reason there were no orders in the system
for contrast and Sara had no ID bracelet so the nurse had to find someone to
give verbal authorization. Finally at 1:30, they took their first x-ray image.
Then we waited. Another image at 1:45. I could tell we weren’t going to make
our 2:15 urology doctor appointment with Dr. Granberg. I had no cell signal but
did have wi-fi so I emailed Shannon hoping he would get it and coordinate
rescheduling the appointment. He got it and actually had just been called back
for Adam’s appointment, 1 hour late!
Every 5-15 minutes they took another x-ray image, sometimes putting a
wedge under Sara’s side so it would lay her kidney out more. Finally they took
the Velcro belt off and asked her to sit up and cough, hoping the contrast
would drain more. She went to the bathroom and then they took another x-ray. At
2:10, they wanted her to sit up on the table until 2:30 and then take another x-ray.
The 2 techs were absolutely wonderful. Sara loved answering all their questions
and telling them stories. They found a teal colored felt owl that the volunteer
team made to give Sara before their shift ended at 2:30 and another person took
over. She was finally released to have the IV shunt taken out. You could tell
by how red and irritated it was that it was going to bruise pretty bad. We
found our way back to our room a little before 3 and she dug into the banana I
brought and gulped down some water—21 hours without food or water and not
one complaint out of her! Off we went to the cafeteria to track down more food,
only to be disappointed. All the hot food was put away until 4:30 dinner so
Sara settled on a bowl of cereal and I had a bowl of broccoli soup.
We caught the 3:30 shuttle back downtown to the Mayo building and
headed to the urologist for our 2:15 appointment. They called us right back and
Dr. Granberg came immediately with 2 other staff. She apologized for the day and
expressed her frustration. When the radiology department wanted to cancel all EXU tests, she
told them they were still needed for pediatric cases and they promised whenever she
ordered one they would do it. But there are only certain staff who know how to
do them and certain radiologists who know how to read them so you just hope
those people are working on the days you need them. Apparently they had been
calling her throughout the day to discuss the test, how to administer it, how
often to take the x-rays, etc. No wonder she was running an hour late in seeing
Adam! She said they were having a clinical symposium the next day and everyone
important would be in the room to discuss what happened and how to change
procedures so it never happens again! It makes me feel better that we were part
of a process to make sure other families that follow behind us experience
something better than we did.
Dr. Granberg moved on to Sara’s test results. The ultrasound revealed
that stones were still present but her burden had not increased, so there were
not more stones than previously noted. The largest was 0.7cm, or a little over 1/4". She pulled up the
EXU x-rays and drew a picture to explain just how unique Sara’s anatomy is. The
new diagnostic phrase we have heard this time is infundibulopelvic stenosis bilaterally
(both sides). This is a “rare renal malformation”. When doing some reading on
the internet about it, I found the results of one study that was done over a
period of 17 years. 11,500 EXUs were performed as part of that study and only 3
children had infundibulopelvic stenosis.
Most kidneys contain wide passageways that lead to small sacs inside
the kidney. The image below is an EXU of a normal kidney. Look at the white tree branch-like images in the top quarter of the photo. Those are the insides of the kidneys illuminated with contrast.
Sara’s passageways are very tiny and she has numerous sacs that are
enlarged. Think of a large sucker with a small stick. Those large bright white areas are the insides of Sara's kidneys, which is what makes the malformation of her kidneys very rare.
The EXU showed the contrast was moving thru Sara’s ureters and into her
bladder, which is a good thing. Dr. Granberg had been on the phone with Dr. Sas
to coordinate communication about the results. She had also called Dr. Krambeck
at her new hospital to give her a report. Because Sara is asymptomatic and her
kidneys are functioning well right now, Dr. Granberg feels it is best to just
monitor Sara for now. She did want one more x-ray to see whether that left
kidney had drained all the way but she didn’t want us to sit at the hospital
waiting any longer. While we were meeting with her, a page came thru that
because we are self pay, they wanted to charge us for the additional x-ray. All
3 staff thought that was ridiculous because it should have been included as
part of the EXU. They finally got it worked out and we had to go back to Mayo 16
East where our ultrasound was for a quick x-ray and then go see Dr. Sas in
nephrology.
We headed up to see Dr. Sas around 4:30 (our appointment was supposed
to be 3:25). Sara is on track with her growth rate and her blood pressure and
other vitals are good. Her bloodwork and urine analysis look great. Her protein
levels have improved and that would be an early indicator of kidney failure. Her
calcium levels have improved. She hasn’t had any UTIs, no pain, no symptoms,
and her stones have not increased in size or burden. Dr. Sas feels we are on
the right track with diet and medications.
The broad spectrum genetic test we have been waiting on is now in the
testing stages. Once the stones are under control, our goal is to preserve the
kidney function. Sara’s teenage years will be the next taxing time on the
kidneys due to extreme growth spurts. Dr. Sas is hopeful that adulthood is
easier to manage because her urine content should change to produce less
calcium. However, Sara is not and never has been a textbook case so she will
have to continue to be closely monitored. In the words of Dr. Sas, “Even the
great Dr. Amy Krambeck is amazed!” and she is seen as the world’s leading
expert on kidney stones.
Sara’s next follow up will be in 6 months for a 24 hour urine test,
bloodwork, and ultrasound. Adam’s report was that his ultrasound still showed a
small amount of dilation in the kidney that had the blockage where it drains,
really only a 1 on a scale of 10. This was no change from his ultrasound 2
years ago. Dr. Granberg feels he is doing so well that he can wait 2 years for
the next ultrasound, instead of just 1 like we originally thought.
We are still breathing a sigh of relief with Sara’s results. This is
the first time in 2 years that we have gotten results showing a status quo or
improvement! It really is an extreme amount of stress to manage her food and
water. Some days are easy and others she fights me all day long. I am so
thankful we got a win for those efforts finally, even if it may only be a
short-term win.
The day of testing turned out to be so exhausting that we just went out
for dinner and then back to the hotel to put the kids to bed. I was even in bed
at 8:30. The next day we did a little swimming before packing our suitcases. It
was fun to see them just be kids for a while, running in the water and going
down the slide together over and over and over again.
Then we headed out for a little Christmas shopping, had lunch, and left
for the return trip to Iowa. Evan was so excited to see us—I got a great big “MAMA!”
Of course, he loved the giant penguin we bought him. He did wonderful with Grandpa George and Grandma Sue and they thoroughly enjoyed their time with him.
Thursday night we relaxed and Shannon’s sister and her family came in
preparation to celebrate Christmas the next day (Friday). Back to Peoria Friday
afternoon. We were only gone 4 days but it sure felt like more than a week.
