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

A Great Loss

At least 27 years ago, my ex-Father-in-Law, George, was diagnosed with Multiple Sclerosis and shared that information with his son. When I first met George he walked on 1 cane, then moved to 2 canes and was an Eagle-eyed father of a college-aged son.
He gave us heck as we dated, laughed when I "bippety-bopped" across the room and threw my fist into his son's shoulder when he made a "silly statement", and turned his head as he caught us getting to know each other better.
We visited him in Philly when we first got together, then Florida after they moved to Amelia Island, and in Vermont every summer for the family event.
He had the pleasure of seeing his first grandchild, Drew..........the grandson, at an early age. Drew was put on the tray of his wheelchair while grabbing his Grandfather's beard. Even up until this past February of 2008, he enjoyed the visits of his grandchildren. Ginny would try to kiss him, but was too short reach him. He used to say "Pucker up, Long Lips!" So, little Ginny would stretch her lips and give him kisses, sometimes with the help of her Dad lifting her up........but, she was always Long Lips!
For years, George has been close to the end of his life. He said goodbye last year, and allowed organ failure to rush his body to the hospital. He had cancerous tumors in his lungs which should've been the final straw for his weak body.........he made it through. He had organ failure, which caused his weak body to be rushed to the hospital so he could die. He managed to live through each of those.
Last Feburary (2007), George was admitted to the hospital with organ failure. He was inchoherent, and talking about being taken away. George was given a dose of medication which brought him about. The day after his goodbyes, George was eating Pork Chops. This is one of our favorite stories of him and his strength.

Today, Sunday, March 2, 2008, George was rushed to the hospital with organ failure once again. We all waited for word. His eldest son, and father of my 2 kids - Drew & Ginny, flew down once again to be with his Mother. We waited to hear that he had once again regained concisousness and was ready for his Pork Chops. We are still waiting to hear those words. ALAS, those words have not come.

Today, Sunday March 2, 2008 after 3:00 pm EST, George let go of the pain in his body. His wife was by his side and his grandchildren were waiting to hear that he was ready for his next meal.

George passed away today and has left all of us to deal with that sadness. While shocked that he finally let go, the family has to deal with the emptiness of life without him. George is finally able to rest in peace and support his family in the way he has been wanting to for years.

God Bless George Lavino - Husband, GrandFather, Father, Father-in-law, Amazing Strength...

It's really hard to be ........

It's really hard to be a great, technologically advanced, amazing parent. No, really, ......it is!

Due to my amazing ability to provide for my children technologically, networking my home computers for internet & printing purposes, networking my Tivos for sheer amazement, and being up on emerging technologies, I AM NO LONGER ABLE TO GET ON MY OWN COMPUTERS!!!!!!

I've provided so much for my children that they are now on the computers for homework, research, and mass amusement. This means that I can't get on my home computers. Time to make up a spreadsheet schedule for computer use in this house.............but, wait...........that will require that I have TIME ON MY OWN COMPUTER!!!!!!!!!!!

Love 'em both! Because as advanced as I may think I am, technologically speaking, they are light years ahead of me in brilliance.

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!

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!!!!

Pre-Adolescent Girls

Hypothetically speaking.......
If a 10 year old girl already has an attitude, rolls her eyes at most anything that's said, snaps at her brother to shut up when she thinks the parent is out of sight, and is starting to perfect the "stomp out of the room" move, is there any chance it won't get worse and will end by age 11???
I'm jus' sayin'........