Sunday, December 25, 2016

Agents of Change at Mayo Clinic

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…

One by one we heard other people’s pagers go off in the waiting area. At 10:45, I realized everyone had been called that was there when we got there and now they had been calling people back that got there 30 minutes after us. I wandered up to the desk and started playing the mom advocate card to see what was going on. The man working registration looked us up in the computer and started making calls. I heard him say on the phone, “So you aren’t sure if you do this test? Should I be calling CT then?” After 5 minutes he hung up and told me they had to research some things and would be calling back. So we waited, and waited some more…

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.