Day 5....The Diagnosis

Well, after a week here with lots of tests, consultations, blood work, procedures, etc etc etc.....

We have a diagnosis.

Bronchiectasis

(brong-ke-EK-tah-sis)

What is it?

Basically, it is a lung disease that causes the airways and lungs to become inflamed and widened. Some of them become misshapen. Once this happens, the airways and lining of the lungs begin to form pockets where mucus can settle. Air pollutants also settle in there.

This causes the airways and lungs to become weakened. As they weaken, they lose the ability to clear the mucus out on their own. This causes the body to recognize that a foreign object is in the lungs which should not be there. It begins to try to attack it. But it can't, because over time the immune system has become lowered to the point it cannot kill the bacteria. Then, it becomes an infection and you guessed it - pnuemonia.

 How did she get it?

Right now, we are still not 100% sure. Her (amazing) doctors here do not believe she has congenital bronchiectasis, but acquired bronchiectasis. This means they don't think she was born with it. Although at this point, it's hard to know for sure. Their best guess is she at some point was not treated appropriately for a pneumonia, leading to the bacteria remaining in the lungs. This caused the airways to become inflamed and weakened.

How serious is it?

It is pretty serious. Left untreated, it can lead to respiratory distress, pulmonary hypertension, loss of oxygen to vital organs...... 

BUT, we have a very aggressive treatment plan to beat it.

How do you treat it?

We have a game plan that is 2 parts: Healthy days and Sick days.

 Healthy:

She will have to do a hypertonic saline nebulizer treatment twice a day. After the treatment is done, we will do chest percussions on her for 20-30 minutes each time. We have little cup-like things that we use for this, and we use them on her chest, under each arm, and on her back. The entire time we rotate her position to try to get her lungs from every angle. She is also being placed on antibiotics for the next 3-6 months that she will take every Monday - Wednesday - Friday. The antibiotic they have prescribed her has an anti-inflammatory aspect to it that we are hoping will help the inflammation in her lungs and airway.

Sick: 

We will increase the nebulizer treatments to 4 times a day with manual chest percussions each time. She will be seen by her pediatrician at the first sign of sickness, who then will call her pulmonologist here at Mayo. She will decide which antibiotic to proceed with. 

Is it reversible / curable?

Yes and no. When you are diagnosed with bronchiectasis as an adult or when it is congenital, the answer is no. It is not curable or reversible. You have to work hard to maintain your health. 

As a child, when you develop acquired bronchiectasis, the hope and goal is that you can try to reverse the diagnosis - not the damage.

Meaning: Her lungs have 'significant' damage already. There was a stark difference in the CT scan taken here on Wednesday and the one taken at Vanderbilt in October. This is very concerning to both us and the doctors, especially considering there was only one diagnosis of pneumonia during that time frame.

This damage is permanent. There is no reversing what is already done.

However, we are praying and hoping that through the course of the aggressive treatments we are beginning, that we can reverse the diagnosis. Her doctors are hoping that as she continues to grow and develop, we will alleviate the build up of mucus and bacteria in her lungs. This should help the pockets to close and the inflammation to go down. It should also help her immune system to be given a break to finally be able to create some sort of a strength against air pollutants and viruses.

What did the sleep study tell you?

We discovered during the sleep study that during 9% of her sleep, she was breathing at a dangerous level... her pulse ox was WAY too low. A good level is 93+. Anything below that is bad, anything in the 80's is really, really bad (especially for her). She was remaining in the low 80's for 9% of the night. That may not seem like much, but the continued effect of this night after night can be detrimental to other organs. They are comfortable with a child being that low 2-3% of the night. So we will be getting oxygen at home for use at night. 

We will also be having a heart ultrasound done at home to make sure that her heart has not already been damaged by all of this

. If it has, the hope is that by having her on oxygen now it will repair itself.

What did the impedance probe tell you?

We have found that she in fact does NOT have reflux. There was no evidence in the airways or the lungs of reflux. During the probe, they did see reflux but not a significant amount that would be treated. They said that basically we all have a small amount of reflux. No one would have a completely negative probe, especially after having the procedure done that she went through. They have taken her off the meds she was on, because long term these can cause recurrent infections in the lungs (ummmm....WHAT??). Imagine our surprise to hear that we have never been told of the long term risks of the combination of the medicines they had her on. The risk is the EXACT thing we are fighting. Here again we just thanked God to be here at Mayo with her.

Other findings:

Cystic fibrosis: negative!!!! Thank you, Lord. A large percentage of children with this condition also have CF, so we are so, so thankful to not be traveling that road.

She is also anemic and Vitamin D deficient. We are putting her on supplements for both. Although they don't feel this has contributed to her condition, it does explain some other issues we have noticed. And it's also just not good for her overall health to be anemic. 

Do you have to go back to Mayo?

Yes, we do. We will be back in 3 months to follow up with immunology and pulmonology.  They will repeat blood work, and we will try to see if we are progressing in the right direction. At some point in the next year, they will repeat the procedures done Wednesday in the OR to make sure no more damage is being done to her lungs. 

It's a lot. It's hard. It's scary. 

But, it is FINALLY an answer. We cannot even explain the peace we have just knowing what is wrong. We have always said we didn't care what it is, just having the knowledge to know how to accurately treat her is all we want. We feel prepared to go home for now, thankful to be able to have been here because of all of you.

Without our amazing family and friends, we could not have gotten here twice. We couldn't have missed work and been able to still pay the bills at home while paying to stay here. We cannot thank you guys enough for your roles in all of this - you have prayed us through the hardest time in our lives. Addy is an amazing little girl. She is going to have an unbelievable testimony one day. She is blessed to be loved so much by so many. 

I will still be blogging here and there! I will keep you all posted on how treatments are going at home and also of the next travel dates to get back up to (the hopefully much warmer next time) Minnesota once we get them.

We fly out tomorrow morning! We can't wait to hug some necks (Ella!!) and love on our family. 

See you soon!

 David and Laura (and Addy Brynn)

Days 3 & 4

Wednesday / Day 3:

We got up and headed to St. Mary's Hospital for Addy's procedures. She was having a triple scope (pulmonary, ENT, GI), a chest CT, lab work done, and two biopsies.

We were told to be there at 8:15, and the procedure would start at 9:15. So we got Addy changed into her super-cool Dr. gear to get the show started.

They took this picture of us before we headed downstairs to CT waiting. I love it. So thankful they do things like this.

Dr. Addy is ready to go! 

Unfortunately, the procedure before hers hit some snags, which left us waiting and waiting and waiting... Like 1 1/2 hours waiting. And she was hungry. Everyone knows

Addy LOVES to eat. She wakes up 'so hungy' and asking for food. So waiting until 10:40 to get started was HARD.

Finally, they got her back. They asked which one of us wanted to walk her into the CT room and stay with her while she went to sleep. Obviously, I can't stand not

 being with her (sorry Daddy) so I went. 

Oh. my. goodness. 

That was so hard. Watching her be so scared while they held the mask on her face was 

hard and extremely emotional for me. She held my hand really tight until she was asleep.

They let me kiss her before I was escorted out, a blubbering mess.

David wasn't sure what happened when I got back to the waiting room, because he couldn't understand what I was saying. Mostly, I never want to have to do that again.

We waited for about 2 hours before we got to meet with the Drs. They placed the impedance probe in and told us that they would feel more comfortable with us staying overnight for observation rather than staying in our hotel. Then we returned to the waiting room for about 30 more minutes until she was in recovery and we could go back.

 The top two pictures are recovery room pictures.

The bottom two are in the room we stayed in for the night. She did not move for a good 6-7 hours. We tried everything to wake her up.

At one point, she sat straight up and said "I wanna get up!!!!" and then flopped back down. It was about 4 more hours until she actually got up.

Overall, she did great with the probe. She woke up finally around 9 pm and went back to bed at 11:30. They woke us up around 8 to get some vitals. So we did get a decent night of sleep, although sleeping on cots and a couch that feels like concrete are not great for comfort :)

Watching Frozen for the 1,0000,0000,000 time

 

 

 

On Thursday, we were discharged from the hospital right at noon. We had to go straight to the other main building for a 12:30 sweat chloride test (Cystic Fibrosis).

During the CF test - they had sterile gauze under all that wrapping to collect her sweat. She HATED it.

 That test took an hour and a half, so we were finishing up right at 2. We had a 2:00 appt with an allergist/immunologist so David left the testing room early and went to check us in for that appt. When we got there, they were calling us back. That appointment took 2 hours!

 Whew, we were exhausted. 

By the time we got back to the hotel, we had about an hour and a half to eat, repack a bag, and leave for the sleep study. Addy and I headed back downtown leaving David at the hotel with a nice, comfy bed with big fluffy pillows sad and alone. :) 

When we got there, they were playing classical music and Addy decided to do a ballet performance for the other people waiting for their sleep study. 

Hilarious. Words cannot express how funny it was. I think at this point she had hit delirium?! She would usually get really embarrassed, but she just kept going even when she noticed people watching her.

Part of her performance :)

My little robot

It was another long night, but we got a lot of information from her sleep study. So it was worth it! The next post will have the diagnosis and info we were given today!

Day 2

Have I mentioned we love this place?

I mean, every single thing that makes a child feel more comfortable, less afraid, and keeps them occupied has been done. 

Like the waiting rooms....

They have tvs on the floor. Seems simple, but she loved it! She can actually see the tv while she plays and colors. They had a stage, which was perfect for our performer extraordinaire.

When they call you back into the rooms, there are no hard back chairs to be found.

Every room has a couch with a tv that has a video on demand service. There is just about every Disney movie ever created available. She got the pillow that was offered, turned on Monsters University, and snuggled up. It is the perfect exam room. 

They also have stuff on the walls, like this heat sensor. You press your hand against it and it shows the heat from your hands. She loved it 

{so did her daddy, for those of you who saw his facebook picture earlier. Yes, that really happened, yes I am embarrassed, yes he is just as bad as she is!!}

Now, onto the good stuff :)

Her first appointment was with the ENT and speech pathologist. They wanted to do a swallow study to re-confirm that she is no longer aspirating. It was done differently than at Vanderbilt. Instead of it being an active x-ray, it was done with a tube run up her nose, into her throat. It had a camera at the end so we got to watch as she drank, ate soft food, and had regular food. 

She was a champ!! 

She hated it, but she did everything they asked. They were shocked. They told us they get kids her age to cooperate around 30% of the time. 

We took her and got a prize when we were done :)

Result: no aspirations!! 

We kinda figured that would be the result, but it's nice to hear it again. They did say that from the angle the camera is placed we should not have been able to see her tonsils, and they were very visible. David and I even noticed them without them being pointed out to us. 

The drs. all agreed that because of this, they are so interested to see her sleep study.

Next appointment: GI. This was quite interesting. 

The dr was so kind, and she really listened to our concerns about the possibility of reflux and medication for life {this is what we have been told from her specialist at home}.

She said that she honestly doubts she even has reflux, from her symptoms and 

tests that they received from Vanderbilt. She even said that being on the medication she is on for as long as she has been could lead to increased infections in the lungs....

Really?

Again, can I just say that we are SOOO thankful to be here. 

We have never been told of the risks of these medicines long term, and we feel so thankful that she was willing to hear our concerns about it. 

She will be doing a part in her exploratory surgery tomorrow and then placing an impedence probe that will stay in 24 hours. There is a very good chance we will have to stay in-patient over night because of this. The probe will show reflux. We are conducting the study off of medication. She said she would be absolutely shocked if she does have reflux, because to her, she has no signs or symptoms of the type of reflux she is being treated for.

Last appointment: Pulmonary / Sleep Specialist

We met with Dr. Baughn who is the primary pulmonologist assigned to Addy's case. She is also a sleep specialist and will be conducting her sleep study Thursday night.

We reviewed everything with her, both sleep and lungs. 

She decided to add a biopsy of the lung tissue to the surgery tomorrow.

She mentioned how interested she was in the results of the sleep study, too. So now we are! Even though we really don't know what they are looking for and how it contributes to her lungs. All we know is, it does. So we are thankful they are running every test they can. 

Overall, today went very well. It was longggg and bless it, Addy was over it. 

It was nothing a little ice cream couldn't cure :) 

 We are anxious and excited for tomorrow.

We have to be at the hospital at 8:15. 

The chest CT is first, which she will be completely sedated for.

After that is completed, she will be moved to the OR. 

They said it should start around 9:30-9:45 and last approximately

 2 hours as far as anesthesia goes.

Please pray for Addy and her doctors tomorrow! We are praying for good results, and also a diagnosis to come on Friday. We are so blessed to have such a great support system while we are here. We will keep you all posted throughout the day on Facebook of her progress, so check there if nothing has been posted here. 

Thanks again for the love and support. 

We love you all!

Laura

 

On the Road Again...And Day 1

Hello sweet friends and family! 

Well, here we are again in the beautiful, not-so-warm and sunny Rochester, MN. We had a great trip here with two perfect flights!

Getting very sleepy!

The Arch and Busch Stadium ~ Go Cards!!

 

Sweet princess couldn't make it to Minneapolis!

 Once we arrived in Minneapolis, we had a few hours before we could check in to our hotel. So we figured we were only 2 miles from the Mall of America so we headed to check it out. 

It. Is. Amazing.

You could spend all day in there for days and days and not get bored.

Addy got to meet Sharky the Shark from the Aquarium - she was only mildly petrified :)

After spending, well, not enough time in there, we headed to Rochester. Bright and early wake up time to make it to the clinic at 8:30. She had three appointments Monday morning : 8:30, 9:30, and 10:15. I was really wondering how in the world they could schedule three appointments so close together.... We actually got done early! We were taken to a room where we saw all three people we needed to see, without ever leaving that room. They come to you instead of moving you 3 times. It was so nice!

First appointment: we met with the nurse who is in charge of the aerodigestive clinic. She is the one we met with last time we were here. She is wonderful. She was very interested to see the difference in how Addy sounded last time and this time. 

Completely different.

Three weeks ago - completely clear, no rattle. Today - very loud, rattly chest.

Next, we saw the pediatrician who will be following Addy this week.

We love her! 

She spent a good amount of time with us, covering all the basics of Addy's history and care. 

She did an exam, and she said Addy's lungs were so loud and junky 

she had a hard time hearing her heart.

 {So thankful we are here}

 She asked a lot of immunological questions. She scheduled a repeat sweat chloride test to just be sure the one taken at Vanderbilt is correct. This tests for Cystic Fibrosis. Obviously, we need this test to be negative. But so many of her symptoms are aligned with this disease. So to be certain, they are repeating it. 

She also added in a large amount of bloodwork to be taken while Addy is in the OR. This will be sent to the immunologist/allergist we are meeting with Thursday. 

This dr. will already have everything she needs when she sees Addy!

 Have I mentioned we love this place? 

Next, we met with the aerodigestive clinic social worker. He basically wanted to make sure we had all the resources we need while we are here. He got us $15 off our hotel room every night and free parking passes just in case we have an appointment that the shuttle can't get us to. 

Checking out the helicopter pad on the other building

Oh ya know, just the normal hotel room activities - dance party!!

Bedtime FaceTime session with sissy

 

We feel this a great start to our trip! We are getting things accomplished that would have taken weeks and weeks at home. We so appreciate the love and support

 we are receiving from home.

 It is hard to be away {especially from our sweet Ella} 

but we know Addy is getting what she needs here. 

We will update again tonight when we return from our next two appointments :)

Lots of love,

Laura