When did Cystic Fibrosis get here?

I know Ginny was diagnosed when she was 3 months old. It's a genetic disease, thus determined from conception. Before we knew it was CF, there were many tests done while in the hospital to figure out why she wasn't gaining weight, had anemia, vitamin E deficiency, etc. Once diagnosed and on the correct meds, things improved and life settled in.

Over the years, Ginny has had colds, coughs, strep, ear infections, etc. Those have been easily resolved with anti-biotics. Her lung function (PFTs) has always been in the 107% - 118% - ideally, you want your lungs to function at 100% so she's been in GREAT shape!

Ginny's weight was a struggle until they introduced us to "the hungry pill" (don't remember the medical name). The goal was to have her BMI above the 50th percentile. She was usually no higher than the 20th until that medication. They had started hinting at a G-tube, but we told them to come up with something else. They did. Within 4 months of being on "the hungry pill", her BMI% shot from 21% to 49%.

Don't get me wrong.....we've had our stressors and struggles with all of the appts, meds issues, germ warfare, insurance, trying to gain weight, trying to kick colds/coughs, etc. But, after the initial difficulties, we settled into daily life with CF......

...... That is, until her PFTs dropped to 89%....... we upped her meds, increased nebs, added Cipro and were to return in 1 month to check again............ they dropped to 81%...... *THUD*.....

She was admitted to the hospital -
She was in for 1 week of IV anti-biotics, respiratory therapy 4x/day, Chest PT 3x/day, and food galore. Just as she was finishing breakfast, snack would arrive - when that was done, lunch was delivered and all meals/snacks were wrapped around all of the other therapies. She has come home with a PICC line so she can continue the IV anti-biotics. There are 4 per day and we had to really schedule things carefully to fit them in with the proper spacing between - not to mention that thing called school, maybe even life.


Guess I can no longer live in my happy, little denial about CF. It's always been there, but now it's sitting at the table.

CF Appointment - 10/29/08

Good stuff at today's CF appointment. I'll provide stats, then some awesome research news...as close as ever to a cure!

Height: 54.7 - up 1.3 in.
Height %: 10% - up 1%
Weight: 76 lbs. up 3.75 lbs.
Weight %: 22% - up 1%
BMI: 17.83
BMI %: 49% - GOAL = 50%.......last visit 52%..... The kid grows which is what they want, AND gains over 3 lbs, but her height didn't go up as much as her weight.....Go Figure! Time to get out the stretching machine again :).
PFTs (breathing test) = 108...decrease of 7 from last appt., but still excellent!
Last throat culture showed light pseudamonas. OK. We are doing the TOBI every other month, so we are doing what we can for now. If it were to drop massively, then we worry (some of the other PFT stats indicate this could be margin of error).

RESEARCH NEWS:
Dr. Z was on rounds today. She heads up numerous research projects and reports out to the medical community and the local parent community on this at least yearly.

I asked if there was any research down the road for a DeltaF508 & R1162X person (Ginny's "mutations", as they like to call them!). She told us there had recently been a very promising research on the GG-something group. We all have CFTR channels which just happen to be blocked in folks with CF. Different genotypes lead to different manifestations.

So, this recent research caused the CFTR channels of the GG? group to act as the CFTR channels of "normal....non-mutant" people. WOW!!!! Basically, a cure of the issue, but not a way to be off of medicine and breathing treatments.

There is a research study of the same type coming for those that have the "x" codon. Lucky us!!!!!! She may be eligible to particpate, but she may also be too healthy to participate. Regardless, let's get this medication on the shelf so Ginny's CFTR channels function properly while she has no lung scarring and issues!!!!

I'm tired now.....

CF Appt - June 2008

Well, we had another CF appointment today, as we do every 3 months. It's physically and emotionally draining even when it goes well. We were there for over 3 hours today, which led to the 3 hour nap I took upon arriving home. That will lead to me being up much of this evening......bad cycle to get in :).
The reason it was 3+ hours today vs. the regular 2+ hours, was due to the fact that it was her bloodwork and x-ray appointment. Bloodwork is once a year, x-rays are every other year.
Here's the update:
Height = 53.4 inches = 9th percentile (can't imagine why she's short!!!)
Weight = 72.25 inches = 21st percentile
BMI = 17.73
And here's the big info about this one. They've wanted her to be at the 50th percentile for BMI for a long time. Today, we hit that goal!!!!!!!
BMI PERCENTILE = 52% YAYAYAYAYAYAYAY!!!!!!!!!
PFTs= 115%
PFT means Pulmonary Function Test. While a 115% lung function sounds good, she was at 134 last July. She went down over the last 2 appointments to a low of 101% at her March appointment. So, she is up almost 15%!!!! That's great news!!! Now, we just need to stay on top of her nebs (Pulmozyme, Saline, TOBI) and her chest PT to get that number back up to her norm of over 130%.

Once Ginny went on the "hungry pill" (as we call it since the name is something I can't remember), she jumped from a BMI in the 29th percentile to the 46th. How great was that!!!!!

CONGRATS GINNY!!!! You are an amazing young lady!

Absentee Blogger

Yes, I've been an Absentee Blogger. But, here's the quick and dirty version of some of the things I said I'd talk about:

The Gala - went to a most wonderful event for my nephew, Henry. It was a fund-raising gala for his school. There was a silent auction, raffle, and beautiful video/slide show presentation of all of the students at his school. Henry began the video sitting behind the Principal's desk with his glasses on (he doesn't need glasses, but has decided they are now a part of his character). If this video becomes public or I can get a hold of it, then I will post it here. He was amazing! He had a scripted part which was delivered with meaning and feeling. Then, there was his ad-libbing which brought the house down. Check out Alice's blog about the gala: http://thefairfax.blogspot.com/2008/03/weekend-to-remember.html

The Recovery - Drew's Kidney healed and his visit to the doctor after returning from Florida showed almost no blood in the urine. Hallelujah!

The Appointment - Ginny had her CF appointment. Her weight gain was, once again, beyond expectations. The "hungry pill" really made the difference in our lives and has taken her out of the "hmmm....she may need a permanent feeding tube" status.
Bad news is that her PFTs (Pulmonary Function Tests) have decreased from the 130% range to the 100 % range. Still very good, but significantly lower than her norm. She had a cold which contributed to the lower numbers. But, that is no excuse and we need to ramp it up a notch.
Good news is that we had a wonderful visit with one of the physical therapists who connected with her regarding the ability to "cough up stuff". Ginny calls it "Gunk-a-loogey" and has, generally, been disgusted when it comes up. We need her to embrace and be proud of her gunk-a-loogey. Since this visit, she has been much more productive with her cough.....I've never seen so much mucus, but I'm a proud Mama.

The Birthday Party - Ginny had the most fun & amazing birthday party. We had a room in the back of Ledo Pizza in Crofton. There was pizza, chicken tenders, mozzarella sticks and amazing entertainment. We had 2 dance mats plugged into the PlayStation2 which was hooked up to the flat screen on the wall. The karaoke machine with her iPod & TV were across the room, and we had a station for sand art. The girls sang, danced, ate, and made tropical fish & dolphin sand art keychains. My parents and brother were there, and Ward's Mother and sister + kids were there. A great time was had by all!!!!

Next up: Bahamas & Drew's Birthday Party

The Appointment - November 2007

Once every 3 months, we need to go through the ordeal of taking Ginny to her Cystic Fibrosis Clinic appointment at JH. I say ordeal because of the amount of time it actually takes for the appointment, the hassle it can be to drive and park, the stress for the couple of days leading up to the appointment and the collapse which occurs after.

Below is Appointment Summary for November 2007:
1. Height, Weight, Blood Pressure
November - 64.5 lbs - INCREASED 6.5 lbs!!!!!!!
2. PFTs - Pulmonary Function Tests
118% which is down from her normal 130%. Yes, over 100% sounds great and is fantastic. However, ANY decrease is NOT good!!!!!!
3. Doctor
Review of Height-Weight Ratio: Height - Weight Ratio: IDEAL = 50%+ PREVIOUS: 29% CURRENT: 46% !!!!!!!!!!!! WOW!!!!!!!! She is almost where they want her to be. Where they have been hounding her/us to get her up to. AMAZING!!!!!! Dr. R was so complimentary. He used words such as excellent, proud, good job and ended with "Keep up the good work!" Wow! We've waited a long time to hear that! Then, there's the throat swab which is done every time.
4. Nutritionist
Reviewed the eating habits and suggests how to increase calories. Not much to comment on this appointment due to the excellent increase in weight and height-weight ratio.
5. Nurse
Reviewed equipment, cleaning, directions and needs for assistance with insurance company. She gives us handouts to help at home, school and anywhere else as needed.
6. Pharmacist
Reviewed all medications, dosages, writes out new scripts as needed.
7. Respiratory/Physical Therapist
She came in to review the breathing treatments and Chest PT. This did not go well and we need to come up with processes to improve. This includes at her father's house, maybe there even more so than mine. He hasn't cracked the Vest out in a long time which the PT told Ginny was unacceptable. This portion of the appointment left her in tears and me feeling a bit nauseous.
8. Psych
She came in to give us ideas for PT incentive and to cheer Ginny up.
9. Research
This time, we participated in a research survey. It consisted of 3 questionnaire papers on my part and one on Ginny's part. The researcher read Ginny's and recorded her responses, I filled mine out at the same time. It was trying to gauge how the nutrition portion, self-esteem and feelings about the protocols by the family and the patient.

OTHERS - not this time, but once per year blood has to be drawn. Every other year, x-rays are taken. Then, there are the times that other doctors come in.....never sure who or why they are there, but it is a teaching hospital......one day I'll have to go over my theory about that!!!!