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!,,

North Carolina Living

Being a California girl born and raised means my life has been filled with sun, busyness, traffic, countless brown dry fields, more sun, tons of places to shop, a diverse community, oh and lots of sun!!!  Every part of my life is rooted in California...my family, my friends, the place I said "I Do" to my husband, the hospitals where my precious children took their first breaths...everything.

 

On September 22, we packed up all our belonging and headed on a Five day cross-country adventure to our new home in New Bern, North Carolina...my heart was torn.  Saying goodbye to everyone and everything I've known my entire life was beyond difficult, but in the tears and heartbreak, God reminded me of His promises!!

When we began this adventure we bathed it in prayer...from the hospital and doctors that would treat Emma, to the company Matthew would work for, to the city we would live in, to the schools and church we would attend, to the beach we would play at...and the list goes on.  As a family, we sought God's direction for every step we would take.  We are confident in the Lord's leading and are excited to see what the future holds for us. 

 

After living in North Carolina for close to a month...we have learned some important lessons...

 

1. North Carolina is filled with the most friendly, loving, kind people I have ever met!!!  It has taken me by surprise to meet truly genuine people in the most unique places.  One thing is definitely true, New Bern was voted North Carolina's most charming city...and it is very deserved!!  However, with that being said...the "friendliness" of people in unnatural to me and can be very intrusive and uncomfortable.

 

2. Y'all is a staple in everyone's language...even the Asian man doing Abby's nails!!  The southern twang we hear everywhere we go is extremely catchy and strangely comforting. 

 

3. It rains here!!!  We have received over 12 inches of rain in the 3 weeks we have been here...and more is due to arrive.  We even had to prepare for our first potential hurricane, which was an interesting experience!!  But through it...we have learned the value of the storm and the joy of dancing in the rain...literally!!

 

4. We live on the border of New Bern and Havelock...both are small towns, under 30,500 people.  Havelock, NC is the home of the Marine Corp Air Station.  Our town is very patriotic...we actually live between. Two Marines and the woman across the street is retired from the Navy.  We constantly have jet fly by's which have come to actually be a loud yet beautiful reminder of the sound of Freedom!!! 

 

5. North Carolina loves festivals!!!  Since we have been there has been a Seafood Festival, MumFest, Mullet Fest (which is a fish festival, not a hair style festival...I was a little bummed to find out I wouldn't be able to see some "Joe Dirt business in front, party in the back" hair.) This past weekend was the Chili Festival.  I believe the Eastern Coastal County Fair begins this week...it's a lot of fun and always provides weekend entertainment. 

 

6. Most people attend church...since being here, every person I meet invites me to their church.  Also...we have A LOT of churches in this area...literally...my google yellow pages search yielded over 200 churches in the area!! 

 

7. Life is better with a big glass of sweet tea...and you will be happy to know that any restaurant you go to serves it!!  Just ask for tea and it's coming to you sweet!!!  If you want unsweetened tea, you actually have to ask and be specific...or you will be enjoying the sweeter things in life!!  Haha

 

8. North Carolina is one of the most beautiful places I have been to.  The sky is bluer, the grass and trees are greener...now this may be because I have lived in dirty, dusty, brown Bakersfield, California where we were in a drought most of my life...but I am like a kid in a candy store as I find new roads to explore and stop to take in God's amazing and beautiful creation. 

 

9. One word...mosquitoes!!!!!  I think these little blood suckers have scarred me for life!!!  Haha. The other day, on one of my explorations of the National Forrest I live in...I came across a place where I am convinced Heaven meets the Earth...it was the most beautiful sight.  When I jumped out of my car to run over and look a forrest ranger pulled up behind me.  After a little conversation with him about moving to NC, he warned me about these little pest and told me to stay in the sun if I didn't want to be eaten alive...I left with a few bites and plan to return covered in my favorite perfume...OFF bug spray!!!   

 

10.  Since arriving, I have been reminded of the joy of being a mother.  My sole focus has been my family since arriving in NC.  I have put my business on hold, in order to turn our house into a home and to reconnect with my kids.  Life passes us by so quickly...my girls have grown and matured so much and I have missed a lot due to my own busyness.  I have committed my time to them and in return God has blessed our relationships and have opened the door a new closeness and love.  My heart is full and I am so thankful for each of my daughters and their unique personality.

 

 

Moving across the USA is difficult...it challenges eveything about who you are and what you want to be.  It is lonely and isolating at times.  There are days when the silence is deafening.  I miss my family and friends dearly and struggle at times with the distance between us.  My girls have committed to praying every night that our family would relocate to North Carolina!!  haha  However, through the challenges...an amazing adventure awaits us...one that has brought us closer together as a family, that has reminded us of true love, that has shown us God's grace, mercy and loving kindness and an adventure that I am thrilled to be part of and look forward to the next turn in the road.  

 

A few nights ago we finally found a Chinese restaurant...my fortune said...

 

Remember at the end of the road, there's always a new path.

 

We were at the end of our road in Bakersfield, CA...today we are finding our way down our new path in New Bern, NC...here's to finding JOY in our JOurneY!!!

Big Changes in The Ritter House

Since July I have been silent about a new issue with Emma.  Honestly, we needed time to process, pray and figure things out.  For a year Emma has been diagnosed with ABPA (Allergic Bronchopulmonary Aspergillosis) but recently things took a turn for the worst.  Here is a quick overview of ABPA:

ABPA is an allergic reaction or hypersensitivity reaction to a fungus known as Aspergillus fumigatus.  This is a fungi found in the soil.  Although most of us are frequently exposed to Aspergillus, a reaction to it is rare in people with a normal immune system.  However, in certain people, the immune system overreacts to the antigens of Aspergillus fumigatus found in the lungs.  This may damage the airways and result in permanent lung damage.  ABPA most commonly affects people with asthma or cystic fibrosis.

ABPA Symptoms:
* Coughing with brownish flecks or bloody mucous
* Fever
* General weakness
* Elevated IgE (allergy levels in the body)
* Wheezing
* Inability to exercise
* Exercise-induced asthma
* Decline in Pulmonary function
* Increased Sputum

When Emma was 6 years old we were told she had a very high allergy to Aspergillus fumigatus and would have to be extremely cautious with this.  She was treated with a low dose of steroids due to their findings and never spoke of it again.  Two years later, upon transferring to Kaiser Los Angeles in July 2014, we discovered a HUGE spike in her IgE levels.  Emma was put on a high dose of steroids to help bring her numbers down as well as Sporanox (antifungal) also known as itraconazole.  This drug helps to lower the chance of the disease getting worse over time.  Sporanox is normally used for 3-6 months or until the symptoms are gone.  However, Emma has never gone without the symptom in over a year, so she has remained on treatment.

In July 2015, her numbers spiked again, prompting a high steroid treatment once again.  Along with the high steroid use comes another onset of issues.  Many of you have noticed the most obvious side-effect...her puffy face.  This is known as moon face and it is very difficult on Emma emotionally and physically.  Since her skin is tight on her face, it does cause her pain.  The long term use of steroids is also known to cause CFRD (Cystic Fibrosis Related Diabetes).  Over the past 2 months, Emma has had very irregular blood sugar levels and is now being watched for CFRD, which, if she develops this it will require the use of insulin. 

In July we were forced to change clinics due to an insurance change.  This meant we had to leave Kaiser Los Angeles and return to Valley Children's Hospital in Madera.  After fighting with the Pulmonology team to get Emma seen, the doctor that saw her wants to remove all antifungal medications from her!!!  He disagrees with Kaisers care, and feels we will only treat her if she is symptomatic.  EVEN THOUGH SHE HAS BEEN SYMPTOMATIC FOR OVER A YEAR!!  He disagrees with my concerns, thinks she is doing well.  He doesn't feel that her symptoms are serious and treats me like I am being a dramatic mom!

A few days ago, I had the chance to speak with an AMAZING 32 year old CFer who shared her story about ABPA with me.  It is very similar to Emma's, in the fact that is was disregarded and left untreated due to her not having the "typical symptoms".  ABPA made her extremely sick, she ended up coughing up blood and developed bronchiectasis (which Emma already has).  In addition, she ended up in the ICU and nearly died due to hemoptysis (coughing up blood).  I am happy to say she is doing well today, after receiving the proper care. 

ABPA is rare and only affects 2-11% of cystic fibrosis patients.  Kern County has one of the highest Aspergillus fumigatus levels in the United States.  Now please don't misunderstand me...Aspergillus fumigatus is found EVERYWHERE, it is an environmental allergy.  However, Bakersfield has very high levels.  Three doctors have encouraged us to move.  With that being said...we have come to a few conclusions...

Due to Emma's health, we have decided to begin the process to move.  This is a very big step for us due to most of our family living in Bakersfield/Taft.  This is a hard decision for us, but one we feel is absolutely necessary for Emma's safety and future.  We have settled on moving to North Carolina, so Emma can be seen be the amazing team at UNC.  This is one of the top CF centers in the United States.  I spoke with the Cystic Fibrosis Foundation today who praised UNC and was in complete support of our transition.

We desperately need your help to make this happen!!  We have set up a gofund me account to help with moving expenses.  Many of you know, Matthew has been out of work since January and has most recently been working part time.  This is not enough income to make this move happen.  Matthew is in the process of securing work in the Jacksonville area.  

If you have connections to a moving truck, please let us know...the largest expense with moving is a moving truck and the gas.

It is very humbling to come to you and ask for you help...but without it, we will have no choice but to continue with the current care being provided to Emma, which means they will stop the treatment of ABPA, until something bad happens.  The fear of something serious happening to her is more than I can bear.  I have cried more times than I care to admit to when I consider the future Emma will have if we continue living around the Aspergillus fumigatus in Kern County. 

We sold Matthew's motorcycle to help with expenses, but we cannot do this alone.  Please prayerfully consider partnering with us as we strive to ensure Emma's future health.  We are confident that we serve a mighty God who can give us all we need, and He has blessed us with an amazing support system with all of you throughout the world.  Thank you to everyone who has helped us and walked beside us throughout this journey...you are the true soldiers of Emma's Army!

Please visit:

Help us Move for Emma's Health

http://www.gofundme.com/qmasqnu5