September Clinic

We had clinic today which we had requested because Olivia's cough is so bad. Here are her numbers from the last two clinics:

9/2/15

50.6 lbs

49.3 inches

99% o2 sat

27 % BMI

1.40 liters FEV1

7/1/15

48.9 lbs

49.2 inches

98% o2 sat

18% BMI

1.42 liters FEV1

Her lung function was down a tiny bit, but she's had a really bad cough so we're really happy it was barely down. She has been put on 2 weeks of antibiotics for her cough, so hopefully that will eradicate the issue. Her respiratory therapist also measured her and ordered her a new vest. She chose a purple one this time. 

Her weight went up 1.8 pounds so her BMI went from 18% to 27%!  I'm so happy with this increase especially since she started back to school and has been dancing a minimum of 4 hours a week. We just need to keep up the good work!

They came in and asked if we are interested in being part of a clinical trial for Orkambi. It's being tested for people with a single copy of delta f508. We will definitely be giving consent and then hope she gets chosen. 

Well, that's all that occurred today. I will leave you with a few pictures. 

Hannah loves her big sister soooo much!

July CF Clinic

Olivia had her quarterly CF Clinic today. It was not bad but it was tiring as it always is. Here are her numbers from the last 2 clinics. 

7/1/15

48.9 lbs

49.2 inches

98% o2 sat

18% BMI

1.42 FEV1

1/21/15

47.9 lbs

47 7/8 inches

98% o2 sat

31% BMI

1.34 FEV1

So as you can see her lung function is good but her weight is an Issue AGAIN. So they talked to her about it for a little and talked about eating better and what foods she likes or would give a try. They told her that if this trend continued she would need a g-tube. I've told her that before but I think she thought I was using it as a threat so to have them say it to her freaked her out. 

Normally I post a percentile for her FEV1, but it was explained to me today that her liter number is a more important and accurate thing to watch. So from now on I will be posting those numbers instead.      Definition of FEV1: Forced Expiratory Volume in the first second. The volume of air that can be forced out in one second after taking a deep breath, an important measure of pulmonary function.

We will find out the results from her sputum culture next week. We've been clear for a while now so let's hope the streak continues!

Today was her pulmonologist's last time to see Olivia (moving to Alabama) so Olivia made sure to have the doctor check out her stuffed horse. The horse, Jesse, strained his leg doing a jump. Dr. Lowell said it's healing nicely and should be fine by tomorrow. 

Great Strides 2015!!

It is THAT time of year again...It is time for our yearly Great Strides walk!!

Click HERE if you want to donate!

Click HERE if you want to join Olivia's Outlaws!!

Our daughter, Olivia, has Cystic Fibrosis, a life shortening disease. It is our daily battle and greatest intention to keep her strong and healthy and her lungs disease free.

What is Cystic Fibrosis? CF is an inherited, life-threatening disease that has no cure – YET. People with CF have two copies of a defective gene that causes the body to produce abnormally thick and sticky mucus, which clogs the lungs and can result in fatal lung infections. The mucus also obstructs the pancreas, causing difficulty with absorbing nutrients. In 1955, people born with CF were not expected to live long enough to attend elementary school. Today the average life expectancy for people with CF is 37. I agree that this is extraordinary progress, but it is not enough and it is not the reality we accept for our little girl.  We're getting so close, but close only counts in horseshoes. The answers are within our reach and we need your help now more than ever. It is your donations and support that have gotten us here.  

This year, we will be walking in the Great Strides walk at James Island County Park on May 2, 2015. Please help me meet my fundraising goal of $5,000, by sponsoring me and/or join our team. Your generous gift will be used efficiently and effectively: approximately 90 cents of every dollar of revenue goes to support vital CF programs.

Making a donation is easy and secure. Just click the "Click to Donate" button on this page to make a donation that will support my team. Any amount you can donate is greatly appreciated. Your support will help fuel lifesaving CF programs.

Real progress has been made in the search for a cure, but the lives of people with CF are still cut far too short. There still is no cure for this devastating disease. By walking today, I am helping add tomorrows to the lives of people living with cystic fibrosis. Will you join me? Support me by making a donation to my Great Strides fundraising campaign today!

Great Strides is a fun, family-friendly event that raises awareness and support for people with CF and their families.

January Clinic

Today was Olivia's quarterly CF Clinic appointment. 

Here are her stats. October's numbers are in parentheses. 

-Weight is 47.9 lbs (45.9 lbs)
-Height is 47 3/4 inches (47 1/8 inches)
-Height/weight ratio 31% (27%)
-O2 Sat 98% (98%)
-FEV1 103% (108%) before Albuterol 103% (119%) after Albuterol
-Sputum culture - Pending

So it was a good clinic. Her weight has been going up steadily each clinic and her lung function has stayed pretty good consistently as well. Since she hasn't cultured pseudomonas since September of 2012, they have taken her off of TOBI. No other medication changes were made.  She had her annual bloodwork drawn and will hear back about that. They have set up appointments for a sleep study (because she doesn't feel rested in the morning) and speech therapy  (because they think it might help with some textural food aversions). We got to meet our new Respiratory Therapist and he seems really nice.

Happy New Year's!!!

Happy New Year's from the Neals!!

Collecting seashells and cheesing hard!

Pretty sunset

Party on, Wayne. 

Olivia wrote "I love you mommy and daddy and Hannah."

Hannah was much more interested in the waves than having her picture taken with me. 

My beautiful and awesome daughters. 

My handsome and sweet husband. 

Gene mutations

I just got off the phone with the genetic counselor from Clinic. I asked her yesterday if they could get a copy of Olivia's mutations from Ambry Genetics because we've always had a hard time finding info on her secondary mutation. We've always thought she had 3859 delc but it turns out she has 3659 delc. There are about 200 people with her combination of mutations and 97% of them have pancreatic insufficiency issues.  It makes no difference in Olivia's treatment, but is fascinating to know.

October Clinic

Olivia had her quarterly CF Clinic appointment today. She got the flu shot and wasn't thrilled. 

Here are her stats. July's numbers are in parentheses. 

-Weight is 45.9 lbs (44.7lbs)
-Height is 47 1/8 inches (46 7/8 inches)
-Height/weight ratio 27% (23%)
-O2 Sat 98% (96%)
-FEV1 108% (95%) before Albuterol 119% (102%) after Albuterol
-Sputum culture - Pending

We're still battling her weight but it went up 4%. Her lung function increased 15% so that's AWESOME!!  Her genetic counselor is checking with Ambry Genetics to see what her mutations are. We know she definitely has Delta F508, but her secondary one is a question. It would be amazing to find out that it's something they're currently testing treatment for. 

I'll let you know what we find out. 

Here are some pictures of the day. 

    Olivia chilling out. 

Hannah truly chilling out. 

Right after the flu shot. 

Olivia smiling because she thought my face was silly when I was imitating her. 

Both girls out cold on the drive home. I find it hilarious that both heads are dropping to the same side. 

Tough questions

 Normally when Olivia has clinic, she does what they ask and plays during the "in-betweens". She doesn't ask questions or even pay attention when the people come through. July's clinic was different. She was very attentive and inquisitive so after we got home Chris and I talked to Olivia about what everything meant. She seemed happy with what we told her and she didn't have any further questions. 

A week and a half ago, Olivia asked how she got CF. It caught me COMPLETELY off guard so I proceeded to draw a Punnett Square and explain dominant and recessive genes. The whooshing sound of my explanation going over her head was almost audible. A few days later, she asked me again and I explained it in more understandable terms.

Last night, while we were doing treatment, she asked me "Mommy, how long am I gonna have CF? I'm sick of having it." I asked Chris to come up so we could all talk about it as a family. She was crying because she's sick of treatment and taking pills. We discussed drugs in the pipeline and told her we just have to stay as healthy as possible until they get here. She was upset that the medicines aren't available for her yet and doesn't want to wait anymore. We told her if we could take CF from her and put it in us, we would in a second. We all cried together. It was a hard thing to talk about. 

Tonight, we sat down and read her Mallory's 65 Roses to help her understand. It helped and she told us on each page the ways that she and Mallory are alike. 

I knew the day would come that she would start asking questions, but it has come sooner than I expected. 

July Clinic

At the end of July, Olivia had clinic.  First, here are her stats (April's numbers will be in parentheses):

-Weight is 44.7 lbs (43.3 lbs)
-Height is 46 7/8 inches (46 inches)
-Height/weight ratio 23% (27%)
-O2 Sat 96% (98%)
-FEV1 95% (100%) before Albuterol 102% (103%) after Albuterol
-Sputum culture - NORMAL (YAHOO!!)

So her weight has been issue for a while.  It's not alarming because she is gaining and growing, but she needs to eat more to keep up with the amount of energy she is expending (which is A LOT!).  Her lung function has decreased some.  When listening to her they could hear popping and crackling.  She had a CT scan done in April which showed a lot of mucous and her lower left lobe looks blocked by a plug (which matches what they could hear with the stethoscope).  Her PFT showed improvement in the small airways again after receiving Albuterol.  They have been trying to decide over the last few clinics if she has asthma and they've finally concluded that she does.  We started an allergy inhaler, but I haven't really noticed a difference yet.  Her CT scan also showed minimal bronchiectasis.  It was the normal amount for a CF'er Olivia's age, but still upsetting to hear.  I want her to be the exceptional miracle case, but  I know this is the same for all CF parents.

We have a new genetic counselor, dietician and said goodbye to Mary Lester (Respiratory Therapist Extraordinaire).  She moved to California and we wish her well, but will miss her dearly.  So now it's basically like a whole new clinic (sigh).

Here are a few pictures I snapped that day...

Standard cutie picture

 Mary made Olivia's gown into an 80's dress.

 My girls snuggling after clinic.

Olivia's Outlaws Great Strides 2014

Another year of Great Strides has come and gone.  Thanks to the help of our awesome donors, we raised $5421!!! We had a wonderful day at James Island County Park.  Thank you so much to the Bryant's and the Wells for joining us on this very important day!!

Here is Olivia with Isabel, Caitlin and Ava.

 Everyone needs a balloon crown!

 See??  I told you.

 Olivia and Isabel

 Caitlin is WAY up high in the tree.

 Team Neal!

 Resting during one of the laps.

 Olivia posing next to a sign with a quote by Piper Beatty.  She's such an inspiration. 

 I won VIP parking!

 Representin'!

 The kiddos (Carter, Ava, Isabel, Olivia and Caitlin - Nolan was elsewhere)

Go Olivia's Outlaws!!!

April CF Clinic

Olivia had clinic today. I have been dreading clinic because she's been sick so many times lately and I knew her weight was going to be an issue. So here are her numbers for this clinic-I'll put last clinic's numbers in parentheses so you see what the changes are. 

Height-46 inches (44.25 inches)

Weight-43.3 lbs (42.7 lbs)

Height/weight ratio-27% (53%)

O2 saturation-98% (99%)

PFT FEV1-103% (127%)

So you can see that she gained a smidge of weight, but grew quite a bit which killed her BMI. Her lung function decreased but she's been sick and dealing with allergies so we're not concerned (too much) at this point. But they did say she sounds a little lung gunky.  She also had a bunch of blood drawn to see what her vitamin levels look like. 

We have a chest CT scheduled for April 10 and she's meeting with an allergist in May. We're changing her allergy medicine regime and adding in hand CPT (chest percussion therapy) to help clear the mucous. Clinic wants to see her in 6 weeks to make sure she's going in the right direction.

I'm ready for a nap now!!

Here's Olivia and Hannah before heading out. 

Olivia waiting for the fun to begin. 

Olivia with the AWESOME Mary Lester (respiratory goddess). 

Olivia prepped with the cream to numb the blood draw site. 

Post blood draw. She was an super brave girl!!

Playing on the swingset

I love being outside!!

Eewww, is that a bug? 

 Why are you not climbing with me??  What do you mean "You're tired?"

 Dirty feet are a sign that fun has been had

AUGH!!!  The grass has turned to lava!!

I feel a song coming.... 

 ...And IIIIIIIIIiiiiieeeeeiiiiiiii...

 ...Will always love....

  ..Yoooouuuuu...

Phew...nailed it.

 Its exhausting to be me.

 Its exhausting to be your dad.