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)