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.

Can you feel that?

....it's a shift ....it's a change ....can you feel it?
I can. I feel it and it may only get worse until the shift splits the path.
It's called adolescence.... or pre-adolescence for now.
What does this mean, how is it defined? Some see it as a time of stress, frustration, growth, independence. There is foot stomping, door slamming, the hand on that one hip that is sticking out, the crossed arms. There is yelling, blame, tears. Quotes may be "I hate you", "You're ruining my life", "You don't understand". That is what people think of or fear about what is coming.
For me, there is a different shift that is turning out to be more painful than that other adolescent stuff. It is the shifting of pill-counting, neb scheduling, worry, and stress over Cystic Fibrosis. For the first time, this last appointment was really hard on Ginny. They are always tiring, stressful, exhausting both emotionally and physically. But, there was something different this time. Cystic Fibrosis is shifting more to a problem she has to deal with. It's always been hers, but the burden has been mostly mine.
It is breaking my heart to watch her feel this more heavily, to cry that she "just wants to be normal!!!" It isn't fair, it does suck, it is annoying and frustrating. It always has been..... but, now it isn't just the physical issues for her. It is moving to a much deeper place for her, and that is pulling pain from deep within me.

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!

Support Ginny's Genes!!!!!

We are getting down to the final days before the Great Strides walk for CF. There are only 9 days left, so please go to the website. Donating is as easy as clicking a button online or writing a check to the Cystic Fibrosis Foundation with Ginny’s Genes in the memo section. Forward the link to other friends and family who may be interested in reading about Ginny and helping us out toward our goal for the big day:
http://www.cff.org/Great_Strides/HelenSmetheram
Anyone who wants to join us at the Navy Stadium in Annapolis on June 7th, just let me know. There are so many great activities and food for all to enjoy on that day.
Ginny is very excited about this and the it should be so much fun! I want to make this a really fun event for her! We can't wait to join in our first Great Strides walk and get more involved with the Cystic Fibrosis Foundation.
Thanks!
Helen

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

Life IS Stranger Than Fiction!

I've spoken a couple of times about Cystic Fibrosis. It is in our lives, and it's not our favorite part. Some days I deal with it better than others. I cringe when my daughter coughs, sniffles, has stomach aches, or gets tired from regular activity.
So often, I feel alone when dealing with this. There was no discussion or feeling of connection in my marriage with Cystic Fibrosis. My ex used to say "her issue" instead of actually using the words Cystic Fibrosis, disease, chronic condition.... so I felt alone there also. It was always better to ignore things because they might go away if you did.
The strangest connection came about today. I work with many people, some of who have been at CTE-JHU for a while, others who have been there a short time, like me. For the first time, I made up Christmas Cards and put candy on all of the envelopes. I've not really done this in previous jobs, I'm just so happy to be there, I was caught up in the excitement of the season. The cards I made up were the ones which I purchased from the Cystic Fibrosis Foundation (CFF). I even put the cards in so when they were pulled out of the envelope, that is what would be seen before the lovely face of the card. It's just my little way of putting it out there to get into people's minds.
Today, one of my co-workers said that she had noticed that my cards were from CFF and she wondered if I supported the charity. I told her that my daughter has it. She smiled and said that she also has Cystic Fibrosis.
WOW!

HERSTORY: Teaching Hospitals

HELEN QUOTE: "This is a teaching hospital, so I shall teach!"
This is long, but really worth it and is a lesson that we MUST be advocates for ourselves, our children and any in our care!!!

As a 3 month old, Ginny was hospitalized with a Nasal-Gastric (NG) feeding tube. Yes, it's just what it sounds like, a tube which goes up the nose and into the stomach and dumps nutrients into the stomach. And, yes, this was something which I learned to insert. That's something they taught me, Lesson #1.

While we were in the hospital, a Physical Therapist (PT) came in to teach me how to perform Chest PhysioTherapy (Chest PT) on a small baby by thumping the crap out of her.....okay, actually, cupping the hand and patting each lobe of the lungs. Lesson #2 for Helen. As I was learning this, an Attending Physician came into the room with his entourage. Said entourage consisted of a 3rd year Resident, 1st year Resident, and a Medical Student. It was the start of the new medical year so they all had new roles (never, EVER get hospitalized on July 1!!!!....Lesson #3).

The Attending was trying to be impressive to his latest set of groupies and thought he'd impress me with big words. His favorite was the word "sphincter". He told me that the tube was irritating the sphincter (the one between the esophegus and the stomach) and that it would continue to do so causing irritation of the sphincter, thus acid reflux and the sphincter wouldn't heal.....sphincter, sphincter, sphincter.....at the time, I had a 3 year old son who was not doing well with toilet training. Sphincter was not an impressive word to me!

Anyway, he continued running in circles telling me that the tube couldn’t stay in, but she probably couldn’t tolerate oral feed, taking a bottle by mouth. He wouldn’t listen to me, or explain what he really meant, or what his solution to this problem would be although I was pretty sure I knew. He wanted to put a feeding tube into her stomach directly. That way, we’d plug the food into a port in her stomach. Wouldn’t that be a really cool procedure to take his entourage through??? My, my what a great experience for them since they ARE in a teaching hospital.

LESSON FROM HELEN: I finally stopped the Attending and told him that he needed to leave the room. He tried to continue speaking, but I looked at him and said, “There are 4 of you looking at me and talking at me, but you are not listening to me. You say she can’t continue with the NG feeds, but you don’t think she can tolerate the oral feeds, but we haven’t even tried them. You are not listening to me and you need to leave.” When he tried to speak again, I told him he must leave and I turned my back on him to focus my full attention on the PT working with me.

They all left and I collapsed, handing Ginny over to the PT who told me I did the right thing. She told me that I need to be an advocate for my child and do what I believe is right.

A bit later, the 3rd year Resident came into the room. I’m pretty sure she was the one who drew the short straw. In a very intelligent (condescending) tone, she said, “Fine. We are going to try it your way.” She pulled out the NG tube and told me that a nurse would be in later with a bottle and they would try it “my way”.

END RESULT: That was the last time Ginny was on a feeding tube. Guess she could handle those oral feeds, and I’m sure her sphincter appreciated the break, I know mine did!

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!!!!