Kalydeco to come in 2012!

This is the first of its kind for CF drugs.  It has been said it is like the discovery of insulin for Diabetics.  It will not cure CF, but will make life a lot healthier.  It will hopefully be passed by the FDA in the next 6 months.  This drug is only for about 4% of the CF population.  It is not for Lexi's mutation, but definitely a step in the right direction...right:)

Kalydeco and hopefully the other drugs to follow will be meds that CF patients will have to take daily.  It is not like chemo, or antibiotics in that you take a course and things are fixed.  This med will cost patients approximately $200,000 per year, but I am sure insurance will help pay for part of that estimate.  Patients will still have to do breathing treatments, take enzymes, and do all the other things that keep these patients healthy.  Kalydeco will help correct the defective sodium/chloride channel at the cellular level. 

I spoke to a patient online that has been part of the study trial for this drug.  He is doing great.  He has not been sick at all since starting the drug.  He has gained weight and his lung function has increased for 60-80%. 

What we all need to keep in mind is the health of these patients until this drug is approved and all patients get it.  Patients with certain bacteria in their lungs will not be approved for this drug.  Also any lung or other organ damage done by CF prior to starting this med can not be reversed.  So we must keep at it...keep healthy, keep taking our meds, keep exercising...but most of all keep PRAYING!!!

http://www.cff.org/aboutCFFoundation/NewsEvents/11-2-NEJM-Features-Kalydeco-Study.cfm

Happy Halloween

I hope everyone is having a great day:)

I just wanted to update everyone on our family.  Poor Wittle Gage has been sick since last Wed am.  It started out as fever, congestion, and vomiting.  Now it is just a terrible cough.  I finally took him to the pediatrician yesterday.  He said Gage's chest was clear and his symptoms sounded viral, like a cold.  This, he said, could continue for another 3-5 days.  Poor guy, he wakes up in the morning, coughs and coughs, and says, "I feel bad again today, Mommy"!  I have to say, he is extra sweet when he doesn't feel good...isn't that terrible.  He is extra snugly:)

Lexi's cough is still not any better and she had several bouts of bloody stool last week.  So, I called UNC today...they decided that they need to check her stool for C-difficile, which is a type of infection.  If that is negative they want to go ahead and start her on oral antibiotics for her cough.  HoHummm.  Prayers, Please!!!

I also need some feedback from you.  If you had a child or had a family member with CF, how would you or how would you want someone to inform you about CF and all the many details.  Sit down and chat, or make you a little booklet on what is important, or give you a list of books or websites you can search yourself.  As you can see CF is a disease that effects almost all of the organs of the body and can be affected by many environmental things.  What do you think...give me you opinions, PLEASE.  I know most of our friends/relatives have been told a lot about CF, but I am sure after 8 years there has been a lot forgotten...



A little about our routine...

This video is a little about our daily routine and why it is important and how some of Lexi's meds work.


http://www.pulmozyme.com/understanding/treatmentregimens.jsp?c=MTPLCF7408&cid=pul_we_F012010_P012013&pid=9368998#

Finally an Update Blog...:)

Hi everyone...I know it has been a while, but I wanted to update you on Lexi's last several appointments.  Things have not been the best in my life lately, but I am so thankful for what God does for us every day. 

Lexi saw an ENT specialist in September.  Things went much better than we expected.  He went up her nose and into her sinuses with a scope and they looked pretty good.  He did see some thick mucus but not a lot of inflammation and NO polyps.  This is wonderful news to us.  We were thinking that she might need sinus surgery in the near future...PRAISE THE LORD!  He did increase her nasal spray and wants us to start doing sinus rinses several times a day...this we are still working on.  The sinus rinses irritate her nose so badly.  We are working on slowly increasing the frequency and will get there, I am sure.

Lexi had her regular check up with her CF Pulmonologist on Oct. 12th.  This appt did not so well:(  Her pulmonary function test was at her baseline normal...which is over 100%:)!!!  Dr. Davis, however said that her lungs had ALOT of congestion.  We decided to do an albuterol treatment and a second PFT to see if this made any difference.  The albuterol opened her airways a lot, and did bring up her PFT score.  This gave Dr. Davis the impression that Lexi has a lot more of an asthma component to her CF than we thought.  NO GOOD, says me!!!!  This means that all of those things, like fragrances, dust, animals, carpet, smoke....will just affect Lexi in a bad way....

Dr. Davis increased Lexi's Hypertonic Saline neb to 7% and I am to call back in the next week to let them know if it makes a difference in her cough....

Please keep us in you prayers that this works....not sure what the next step will be, but I do not want to be in the hospital for Thanksgiving or Christmas....

It's BAAACCCKKK...

Lexi's cough its back:(  It seems to have worsened over the last week and a half.  At this point I am not sure if it her sinuses or her lungs.  She sounds very raspy, her voice is hoarse, her cough is wet, and her sinuses are congested.  Please be in prayer for her.  I spoke to UNC and we are going to try some prednisone for a couple weeks and some Bactrim antibiotic.  I sure hope this clears things up...

The Can't are Overwhelming...

Cystic Fibrosis is probably one of the hardest diseases I know of.  It is the number one disease in the US that kills children.  There is only a 50% chance that people with CF will live to the age of 37.  If children go undiagnosed by the age of two, then they will most likely not live to see their preteen years. 

All of these statistics are hard to read, but to me the hardest part of CF is the CANT'S.  Yes even harder than the daily fight that CFers go through.  Even harder than the hours of treatments, hundreds of pills, tens of thousands of dollars in medical bills each year, and weeks of hospitalizations each year.

THE CANT'S ARE SOOOO HARD ON EVERYONE!

* Lexi can not have an indoor pet, no dogs, cats, fish, bunnies, hamsters...ext.

* We can not spend an extended period of time at a home that has an indoor pet. When it is unavoidable like b/day parties, I am on her like a hawk.  Making sure she doesn't put her hands in her face and washes her hands before she eats or drinks.  We can't tell folks not to have indoor pets and we hate to alienate her from everyone, but immediate family members that we would visit a lot can not have them at all.  Not even for short periods of time.

* We can not have any carpet especially in Lexi's room.  She does not need to spend the night at a          home where there is wall to wall carpet in the bedroom.

* We have to have allergen covers on our pillows and mattresses.

* She will never be able to go to a bonfire, or roast marshmallows over a camp fire.

* She can not be outside while people are grilling, unless the grill is far..far away and you can not smell the smoke.

* She will never be able to plant flowers or a garden.

* We can not have any indoor plants.

* We have to have a whole house filtration/purification system to remove any bacteria from the water.

* She can't enjoy a hot tub.

* She can't mow grass or be outside when someone is mowing grass.  Imagine how hard this is living in a neighborhood.  Seems like someone is always mowing.

* We can't be around cigarette smoke at all.  This is easier now b/c there is no smoking in public facilities in NC, but we still have problems at outside public places.  Seems like every time we go to the pool, someone is always smoking around us...uugghh.

* She can't be around 1st, 2nd, or 3rd hand smoke.  The first 2 are obvious, but we just recently learned about 3rd hand smoke.  It is the carcinogens and harmful debris left on smokers clothes, hair, and in their homes.  So no hugs if you smoke and have not showered:(

* Lexi can not clean...no vacuuming or sweeping b/c of the dust that is stirred up.  No mopping, tub cleaning, or toilet cleaning b/c of the chemicals used.  She can not even wash dishes or a car unless an unscented detergent is used.

* She can't be outside in the pollen or when it is dry and dusty.

* The heat makes it hard for her to breath and can cause her airways to close.

* She can not swim in lakes or ponds.

* We have to be very, very careful when swimming in public or private pools to make sure there is no bacteria or fungal growth and that the chlorine level is accurate.

* No one around Lexi can wear fragrances.  This is very, very hard!  Probably the hardest part, b/c these days all hygiene products have fragrances.  Her caregivers, friends, and teachers that will be in close contact have to wear/use unscented everything.  Fragrance free soap, deodorant, hair spray, hair mousse/gel, lotion, clothes detergent and fabric softener, and as lightly scented shampoo as possible (can't find unscented that works or that would be used too).

* We can not use any scented laundry detergent or fabric softener.

* We can not go in a home where they have used Fabreze, air fresheners, plug-ins, candles, warmers, ect.  And this is really hard b/c you have to remove these things weeks to months to get the smell out of your home and fabrics.  Sometimes longer if you have used a lot of fragrances like Yankee or Scentsy.  This has lost us a lot of invites, b/c most people want their homes to smell nice...and well we just can't go!

* Lexi will never be able to use Bath and Body Works, wear perfume, wash with nice smelling hair/body products.  This as a girl will be brutal. 

* There are lots of stores that we can not go to b/c of their fragrances.  One that we love, love, love is Cracker Barrel.  We were letting her wear a mask until we entered the restaurant side, but even a mask could not remove all of the fragrance. :(  We have also had to turn around and leave stores before b/c of the smell.

* I can't use most cleaning products.  I do still use some chemicals in the bathroom to remove mold/mildew, but I have to wait until she is asleep.  I close her door and leave the fan on for hours and know she will not enter that room for at least 6-8 hours.

*  We have to be persistent about hand washing and sanitizing. 

* We make waitresses re clean our table while I watch.

* I am the runny nose detective during cold/flu season.  Big family get togethers are very hard on us.  Everyone is rarely well all at the same time.  Is is fair for us not to go or them not to come????

* We are careful not to wear our shoes all over the house, b/c of the germs they carry.

* Gas powered four wheelers, golf carts, scooters can be harmful.  We try to eliminate these, but sometimes it is hard.  Those times we wear a mask.

* Nail polishing is a careful ritual.  We have to go outside, it has to be a breezy day to diffuse the fumes, and Lexi has to wear a mask.  Not too fun then, huh!

* There can be no visible mold or mildew in our home.  I know that sounds like it would be gross, but look around your tub or shower door.  There is mold there unless you clean almost daily.  This takes a great toll on me.  I clean 1 bathroom every night, so that means my bathrooms and kitchen sink are cleaned at least every other day.

* I have to vacuum my wood floors everyday to remove dust.  I mop them a couple times a week.  I have to dust 2-3 times a week, clean my bathrooms every other day, spray my sink and counter tops daily, spray Lysol on phones, remotes, door knobs, toys, couches, light switches, ect on a weekly basis (again all while Lexi sleep b/c of the fumes)

* Lexi has to be careful about markers, paint, glue, and any other art supplies that have an odor.

* She can't use some make up and lip glosses b/c of the fragrance.

* She can't have toys that smell.

There are more, but I am tired and you get the point.  This is so hard.  I mean, how do you tell your child that they can't go, or do this, or come inside there is smoke... And how do you tell your friends and family to abide by these rules?

WHAT WOULD YOU DO?

Some might say, let her live her life and be a child.  Don't be over protective.  Oh, and my favorite one is....you can't protect her from everything forever.  But how can you willingly let your child do something that is going to ultimately lessen her life span.  Can you sit by and watch knowing...  Please let me know... I struggle with this part of the disease more so than the med schedule.  It has literally made me OCD!





We are FREE...well a little.

I want everyone to know how great our God is.  I know He is the healer of  Lexi.  I know that everyone has been praying these past 4 weeks for us, but these last few days we have felt God's power.  You know we were getting a little more and more concerned this last week b/c Lexi's cough had not improved at all.  We were not sure how this week would end and what direction we were going to take next.  I even placed a call to UNC on Tues to let them know that Lexi was no better.  That is how concerned I was...  I was ready to maybe try a course of steroids, which I HATE.

Yesterday rolled around as it has the past few weeks.  I left work around 2pm to come home and start our afternoon treatment.  I started Lexi's IV and sat her between my legs to do her pat pats.  I usually do her back first.  After about 5 minutes, I always make Lexi cough....AND HER COUGH WAS CLEAR.  Again, I tell her to cough.  She coughs several more times...again HER COUGH IS NOT CONGESTED AT ALL!!!!  Wow, up until this moment when she huffs or coughs she sounds very, very congested.  Once she starts this cough, she usually has to cough several times to get clearance again.

Again at her evening breathing treatment, we go through the same ritual.  I make her huff and cough again, and once more her cough was clear.  I was excited, but hesitant to get my hopes up. 

Today, as most of you know was the test.  We had to go back to UNC for her check-up and decide what to do next.  On the way Reed and I several times asked Lexi to cough for us.  Each time the same.  A little congestion, which sounded like some sinus drainage...but nothing major at all.  PRAISE THE LORD!!!

We had an appt for her pulmonary function test first.  She blew 106%....THE BEST EVER!!!! And this is without having done any albuterol to open her air ways and without having done any airway clearance.  (Cause we didn't have time before we left this am!)  Dr. Davis came in and her lungs sounded clear.  Oh how excited we all were.  Finally...the PICC can come out, her little raw arm can heal, we can all get some much needed sleep, and return to our normal/abnormal life. 

Thank you all for all of your prayers, concern, and support.  We NEEDED every call, text, email, or question of concern.  It blesses our heart that we are so loved.

Love and Hugs,
Melissa