Question: I asked you a question a few days ago, but was unable to answer your follow up questions promptly due to the holidays and the questions was closed. I would like to submit them and get an answer prior to me submitting my review. Here is a transcript of our exchange and the answers to your questions:

Original Question:
My daughter is age 6 and has had very poor pulmonary function test results for 2 years. She's been seeing a pediatric pulmonary doctor after she had 4 bouts of pneumonia in one year 3 years ago. She had a bronchoscopy that found nothing significant other than a small nodule on her vocal chord that indicated she had some reflux. Her allergy testing came back normal. Her immunology testing came back normal. She was recently tested for Cystic Fibrosis and that was also normal.

She's now on: Advair HFA 115 twice daily; Singulair 5mg once daily; Nexium granules 10mg once daily; Flonase 50mcg once daily; Ventolin HFA 90mcg as needed 20-30 minutes prior to activity and Albuterol breathing treatments via nebulizer as needed every 4-6 hours. Despite this her pulmonary function is still poor. We've had to follow up with the doctor every 30 days for the last 7 months. This seems to be very frequent, as though there is something the doctor is concerned about, but not saying.

Are there any other tests you would recommend?


Your answer:
Hi...By what you quote the history and the medicines you are using are fitting into uncontrolled sever persistent asthma or Interstitial Lung Disease (ILD)
I have a few questions for you -
Questions:
1. How many days per month does she cough or feel breathless?
2. How many nights per month does her sleep get disturbed due to above symptoms?
3. Does she feel breathless when she runs around or plays with other kids?
4. Are the symptoms when there are seasonal changes?
5. Is there any family history of asthma or any other sort of allergies like skin allergy etc.?
6. Is the cough always associated with fever?

Kindly get back to me with answers to above questions. I will further proceed based on that.
Regards - Dr. XXXXXXX

ANSWERS:
1.     She coughs often after activities. She’s really unable to tell us how breathless she feels. She develops purple circles under her eyes and we can see them.
2. She seems to sleep, but snores at times. She's pretty restless when she sleeps and always wakes up tired. No sleep studies have been done. She sleeps alone.
3. She is breathless when running around with other kids and gets flushed when doing a lot of activity. She hates sitting out and doesn't like to accept her limitations. She's still participating in Physical Education and recess.
4. The symptoms don't appear seasonally related other than she does seems to have some seasonal allergies, although no identified allergies when tested. We live in a desert in the Southwest US, so we don’t have significant change in seasons.
5. Dad has asthma and breathing related problems. Dad has eczema too pretty significantly. Dad is allergic to cats, most dogs, guinea pigs. Paternal half sibling is allergic to seafood and appears to have allergic reactions to cats although the allergy testing came back on the low spectrum for pets. Same sibling has a lot of coughing associated with physical activity. Paternal grandmother spent time in the NICU after birth due to reported lung collapse. We just found this out recently. We aren’t sure what the circumstances were for sure as she recently passed away at age 58 suddenly after Septic Shock after a gallbladder surgery. As her mother, I also went into Septic Shock in 2011 after a surgery at age 34 and ended up on a ventilator. I ended up with a paralyzed diaphragm after the ordeal due to phrenic nerve damage, but this is believed to be from a PICC line and was not present from birth. I was recently tested and had my test for ANA antibodies come back positive. I also have another autoimmune disorder, but it’s not related to allergies in any way.
6. Cough is not always associated with fever. It's usually not. When she gets sick, she gets sick quickly if we don't intervene with nebulizer treatments immediately. She had RSV as an infant. Then she was well for 3 years. Later, she got a cold that quickly escalated to pneumonia and a hospitalization within a day or two. Within that year, she had 3 more instances of illness turning to pneumonia quickly. That's what led to me taking her to the pulmonary doctor on my own as I had a PPO insurance and I wasn’t getting answers about why my young child was getting so ill. She was then given an Advair discuss and Ranitidine for 1 year. At the annual pulmonary check up in XXXXXXX 2014 it was found her PFT was getting worse despite the Advair with no identified reason why. Doctor has added med after med without significant improvement. Doctor said recently he thought for sure it was CF, but the test was negative. Now, we are baffled.

Not sure if meds are right and it the tests that can be done have been done have been done. Thank you.