A Breath of Fresh Air

On Tuesday November 17, we took Emma to her first appointment at UNC.  We were nervous to enter into a new clinic and experience a new environment.

 We were greeted by Sherry the triage nurse...she was sweet and very welcoming.  Within minutes of stepping in the room after completing her vitals, Dr. Terry Noah entered...

Now a little background on Dr. Noah, he has been at UNC since 1987, he graduated from the University of Michigan and completed his residency at Yale-New Haven Hospital.  Terry is part of a successful research program investigating the effects of air pollutants in humans at UNC center for Environmental Medicine, Asthma and Lung Biology.  He was the Program Director of the Pulmonology Fellowship program from 1998-2007 and previously served as Division Chief from 2002-2009.  As of September 1, 2015, Terry was names Chief of the Division of Pulmonology.  Dr. Noah has a special interest in respiratory viral infections, environmental pollutant effects in the lungs, and lung transplantation in children.  He has numerous publications and awards.  In addition, he is listed on the prestigious list of The Best Doctors of America!!

Now why would I give you a history of this doctor??  I wanted to see how God orchestrated everything about our move...even Emma's doctor.  This doctor was assigned to her, I was not given the ability to "pick" a doctor.  Out of all the doctors at UNC, Emma was blessed with the doctors who's special interest includes environmental pollutant effects in the lungs and respiratory viral infections, her two biggest issues!!!

After a long conversation regarding her history, and my concerns (which he literally sat, took notes and just intently LISTENED to me), he shared his thoughts.  He believes she needs to be rechecked for Aspergillosis (ABPA) and while awaiting those results that she needed to come off all medications for APBA immediately.  He explained that she had been on these medications for far to long.  Then he shared his concerns about her steady lung decline in her small airways.  After explaining the importance of the small airways, we looked at her previous functions for the past 1 year at Kaiser Los Angeles.  In July, when she did her last lung function test, her overall numbers (FEV1) sat at 122 and her small airways were sitting at 90, still great numbers, but a steady decline.  Dr. Noah was wonderful, he took all my questions, concerns and input with ease and not once did I stump him...which was a miracle in the world of CF and doctors.  He did order numerous blood test, chest X-ray and wants her followed by ENT immediately, because the report from Kaiser has him concerned about the need for a third sinus surgery.

Next we met with one of the research coordinator, which informed me that they are beginning to open up the Orkambi research study for children ages 6-12 with the gene mutation Emma has.  She will be investigating Emma's history to see if she will qualify, which means we could have the coveted "miracle drug" in our hands much sooner than expected!!!!

We quickly met with the sweet dietician, and found out that Emma has gained weight and her currently BMI is in the 57%!!!!  As a CF mom this is music to my ears!!  Since leaving Kaiser in July, we have been unable to get the formula needed for her gtube nightly feedings...gotta love being suck between insurance and doctors.  Well, since she is doing so well the dietician and doctor feel if she can continue to gain weight between now and February, they will discuss removing her gtube permanently!!!!  On one hand this makes me nervous for many reasons, but they explained their logic and I understand their thought process.

After learning that good news, my heart was full...it was now time for Emma's PFT's (lung function test).  After my conversation with Dr. Noah I was nervous about what the numbers would say.  These numbers would be the first scientifically proven facts we had regarding our move...was the clean air helping Emma, were our trips to the beach and her ability to play in the salt water helping?  With butterflies in my stomach, Emma put the light blue nose clips over her tiny, adorable nose and began breathing into the machine.  With a fast deep breath she blew in the machine emptying her lungs of all the oxygen they contained until she had nothing left but the need to gasp a fresh breath of air.  My eyes flashed to the computer screen...the first set of "warm up" numbers were less than poor.  At that moment, her lungs sprung into action.  Every test from that point on improved...her numbers kept growing and growing...the respiratory therapist cheered in amazement with me.    After about six tries, he laughed and said with a big smile, he said "well sweetie, I don't think you can get anything better than this last one."  My eyes looked at the screen and immediately filled with tears...her overall lung function has improved from 122 to 128...which is a number we haven't seen in a very long time.  But her small airways improved from 90 to 157!!!!!  Yes you are seeing the right numbers...157!!!  I just sat there stunned.  The RT printed off a certificate for Emma for blowing such incredible numbers.  My precious warrior stood there in her yellow hospital paper gown, with blue plastic gloves, smiled, pulled on her face mask and turned to proudly walk back to her room, with a very noticeable hop in her step and even though her mouth and nose were covered by her face mask, I could see the smile on her face and the sparkle in her eyes!!

We finished the appointment with a visit from the social worker, and Dr. Noah's nurse who reviewed our plans for the next three months.  Emma received her flu shot and then headed to take two chest X-rays, and to see the hospital vampires, who sucked nine vials of blood from her skinny right arm.  After nearly six hours, we picked up our stuff and headed for the exit doors.

Overall, our appointment was fantastic.  We are still awaiting results from her lab work, chest X-ray and throat cultures.  I should know more about those on Monday.  Dr. Noah has a clear and precise direction he wants to take Emma and is confident in caring for her.  Before walking out the door, he turned and with a half smile (he is very serious, even though he likes SpongeBob) he said..."I love a good challenge!"

I think Dr. Noah and I will get along just great, and I believe with his help, Emma has a chance to thrive in a new and exciting way.    With that being said...We are never guaranteed tomorrow, and I do not want to sound naive...we are certain Emma will have bad days, we know we will find our home inside the walls of the UNC Children's Hospital sooner than we would like.  I will not claim that moving to North Carolina will keep her healthy for the rest of her life...the bottom line is we are fighting a progressive, terminal lung disease.  Until a cure is found, we must except reality.  However, after reviewing the numbers and seeing the HUGE improvement in her lungs, my heart is overwhelmed by God's grace and mercy.  I am astounded by His heart and passion for Emma, I am grateful for His provisions and I am humbled by His love and protection.  

Thank you to each and everyone of you who have decided to partner with us.  We are so blessed by your desire to "do life" with us.  We are forever thankful for your love, support and prayers.  Thank you to everyone who took a step of faith with us and financially helped get us to North Carolina...Emma's lung function improvement are because of YOU!!!  Your faith in us as parents and more importantly in the Creator of the the Universe has paved the way for amazing things in Emma's health!!

One last thing...We are constantly encouraged by your phone calls, text, emails, etc...without you the world of cystic fibrosis would be a lonely place. Thank you all for helping us breathe easy!,,