B.cepacia

Caleb's clinic just called and said that his last culture which was about a month ago grew B. Cepacia!!! They sent it to a few different places to confirm before calling me... They said out of the 5 different types this was the better of them.......


I would rather him not have any! I feel like I am going to puke! I thought this was something that they cultured when they were older.... NOT at 7!


Melissa

Pray for Stellan

If you get a moment please say a prayer for baby stellan and his family. He is in the NICU having heart problems (SVT). They are having a hard time getting his heart rate back to normal. His button is to the right.

Until next time.........

Sorry!

Sorry... I really didnt fall off the face of the earth! Having computer issues is no fun! That on top of sick kids and meds galore......... You know that drill. Wyatt has still been having a rough time! He just finished his 4th antibiotic for his on-going ear infection issue. Its just so weird. He has never had a ear infection until about 2.5 months ago and its been down hill ever since. I brought him for a re-check today and his ped said the left looked good but the right was red and full of fluid! She is going to give him 2 weeks and check again. If its still full of fluid or it gets infected again she is going to recommend tubes. She said the right ear is so bad she isnt sure he can fully *hear* out of it. I guess id rather go with the tubes than keep dealing with a miserable kid constantly on antibiotics! Caleb is doing good (knock on wood). He had a cold a few weeks back but with some antibiotics and increased treatments we were able to jiggle it out of him :) Dylan is also over his cold so hopefully they are all on the mend. Its been nice around here lately its so nice to open the windows and air out!


Until next time

VX-809

CF Foundation Announces Initiation of Phase 2 Trial of VX-809
March 25, 2009 The Cystic Fibrosis Foundation announced today the initiation of a Phase 2a clinical trial for VX-809, a compound designed to treat the basic defect in cystic fibrosis—a faulty gene and its protein product, called CFTR. Developed by Vertex Pharmaceuticals Incorporated, in collaboration with the Foundation, VX-809 is known as a “corrector” and aims to move defective CFTR protein to its proper place in the cell. The protein is then poised to move chloride out of the cell and potentially allow it to function as a healthy cell would. The Phase 2a trial is designed primarily to evaluate the safety and tolerability of multiple doses of VX-809 in patients with CF. In addition to assessing safety, the trial will also evaluate whether VX-809 affects key measures of how well the protein is functioning. The trial is expected to enroll approximately 90 patients with the Delta F508 mutation of CF, the most common mutation in CF patients. "Today, CF treatment involves the use of multiple drugs that address the symptoms of CF, however no therapy currently exists that targets the underlying cause of this disease,” said Robert J. Beall, Ph.D., president and CEO of the CF Foundation. “We are encouraged by Vertex’s rapid progress in advancing two novel therapies aimed at correcting the basic defect in CF. We believe that VX-770, which is expected to enter registration studies in the first half of this year, and VX-809, which today entered Phase 2 clinical development, have the potential to play a significant role in changing the future course of this disease for people with CF.” "The advancement of VX-809 into Phase 2 development demonstrates our commitment to improve care for patients with CF," said Freda Lewis-Hall, M.D., executive vice president, medicines development, and chief medical officer of Vertex. "This Phase 2a trial is designed primarily to provide a safety, tolerability and pharmacokinetic evaluation for VX-809. Any additional signals observed in this trial, including changes in sweat chloride and nasal potential difference, that indicate VX-809 has an effect on measures of CFTR function may be highly informative and encouraging for planning future clinical trials with VX-809."
VX-809 was discovered as part of a collaboration between Vertex Pharmaceuticals and Cystic Fibrosis Foundation Therapeutics, Inc., the nonprofit affiliate of the Foundation. Initiated in 1998, the Foundation has invested $76 million in CF research and development efforts with Vertex.
Read Vertex's press release.

Liprotamase

CF Foundation Finds Collaborator to Ensure Development of Important Pancreatic Enzyme
March 25, 2009
The CF Foundation announced today it will collaborate with a pharmaceutical company to ensure development of an important porcine-free pancreatic enzyme replacement therapy.
In a Foundation investment worth up to $3 million, Alnara Pharmaceuticals Inc., a Massachusetts-based pharmaceutical company, will complete the ongoing Phase III long-term safety study of liprotamase. Following completion of the study, Alnara will complete clinical and regulatory activities needed to file a New Drug Application with the U.S. Food and Drug Administration. The company is committed to bringing this important product to patients with CF.
Liprotamase was previously in development with Altus Pharmaceuticals Inc. and was known as ALTU-135 and Trizytek.
“The Cystic Fibrosis Foundation is excited to enter into a licensing agreement with Alnara for the development of liprotamase, which has the potential to be the first porcine-free pancreatic enzyme therapy for CF patients with pancreatic insufficiency,” said Robert J. Beall, Ph.D., president and CEO of the Cystic Fibrosis Foundation. “We look forward to working with Alnara to quickly finish the development of liprotamase and bring this promising new treatment option to patients with cystic fibrosis.”
The Development History of Liprotamase
Liprotamase, which has been in development since 2002, was originally discovered and tested by Altus Pharmaceuticals. Unfortunately, due to financial difficulties, Altus discontinued development in January 2009. Unwilling to let this important therapy languish, the Foundation retrieved rights to the potential drug from Altus, maintained its clinical progress and searched for a company to see the enzyme through the approval process.
The Foundation selected Alnara because of its unique experience with the pancreatic enzyme therapies: two key members of management were involved in developing the original product at Altus. Alnara also plans to develop a pediatric liquid form of the pancreatic enzyme.
Cystic Fibrosis and Pancreatic Insufficiency
Pancreatic insufficiency occurs in approximately 90 percent of people with cystic fibrosis and is treated with pancreatic enzymes that aid digestion and improve growth. Liprotamase is unique and important because, unlike other pancreatic enzymes currently available, it is derived from non-porcine sources.
In addition, liprotamase offers potential advantages over the current standard of care. Today, people with CF must take multiple capsules—sometimes four or five—with every meal or snack. In contrast, with liprotamase, patients can take one small capsule with every meal or snack.
Read Alnara’s press release.

Its late........

It is too late right now so part 2 will be posted tomorrow!

About Us........

My Name is Melissa and I have been married to jim for almost 10 yrs.....

We have a son dylan who is 1o and does NOT have cystic fibrosis.......

Our Middle son Caleb was diagnosed with Cystic fibrosis at 7 months old....... 7 yrs ago today....

I remember our wonderful ped. saying she just wanted to rule it out, after caleb had been sick off and on for several months................

I also remember my mom telling me that there was no way he had cf..... and when kids did have it they had to be pounded on several times a day.................

I remember the day caleb had his sweat test the person that did it said out of all the tests she had done no one had ever tested positive................

I remember the day caleb had his sweat test we had just gotten home (only 40 mins after the first test) I was called to come back in for another sweat test................

I knew in MY HEART SOMETHING WAS WRONG!!!

I brought caleb back in and they re-assured me that I was only there due to their error!

Another hr passed and the Ped's office called me and wanted me to come in ASAP to talk about the test results.....................

I demanded to hear the results over the phone!! I knew something was wrong and didnt want to drive an hr worrying......

After a few mins of hesitation they told me that calebs sweat test came back POSITIVE for Cystic fibroisis..........

It had been a long time since I saw my mom and gram cry.......................

Expecially MY mom!

We were told a few facts and pretty much sent on our way.....................

Later that evening I got a call from calebs ped (who was on vacation)

She was stunned with the results.........

She just couldnt believe it.................

No one could..................

Caleb was started on meds which he just wouldnt take................. NO matter what!!!!!!!!

A few weeks later he still wasnt getting better and he was rolling his eyes.............

It was found that he had a very low sodium level and was immediately transported to barbara bush childrens hospital.....................

He spent a week there getting his levels back to normal................... and for me it was learning more about his diagnosis.............

It took him a few yrs to get past the gag reflux in order for him to take his enzymes!

For him it came down to swallowing a piece of Hubba Bubba bubble gum!!!

So far he has only been in the hospital that one time.............

He is now 7 1/2 and on the following...........

2 creon 20.....

2 puffs of flovent twice daily

albuterol twice daily

azithromicin M/W/F

Cefzil twice daily

Pulmozyme once daily

ADEK vitamins...

culturelle

Chest PT twice daily

prevacid once a day

2-3 pediasure daily

High calorie diet.........................................


Until next time...............

Melissa

Never Bribe a 15 month old :)

Well, Lets just say I learned my lesson! Wyatt being on tobi is a big pain in the butt! There is no way he will sit still long enough to do it while he is awake so I have been doing it when he is napping in the morning and after he falls asleep at night. The other day I had a bunch of errands to do and he was getting sleepy and I couldnt keep him awake so I decided I would give him a peppermint patty. It was about the size of a quarter! I was about 5 mins from my house and figured he'd just gobble it up and then id be home..... Well this was the scene when I got home! I have no clue how he could have made such a mess with a teeny piece of chocolate... As you can see he was pretty proud of himself :)

Yup! lesson learned!

Until next time......

Melissa

Slacking on updates :)

Sorry I havent written anything in a week! Between the 3 boys keeping me busy and my father in law being in the hospital with stroke symptoms, I just havent had the chance... Never dull around here! Wyatt is still junky even after being on cipro..... Im at a loss with that sweet boy of mine. I just dont understand how he can always sound so junky. Its weird too. When he coughs he sounds to me like he still has a lot of inflammation and mucus. When I take him to the doctors they listen and say wow he sounds great! Perfectly clear! He does also have laryngomalacia, which accounts for some of the upper airway noise. I cant wait for him to out-grow it so its no longer a issue. Wyatt cultured pseudo last fall when they did a bronch. He was immediately put on the cipro/tobi treatments. even after 28 days of tobi and 14 of cipro he was still sounding clear to them and junky to me. So we went to clinic and they admitted him for iv anti's (on oct 31) and even after that he was still clear as a whistle to them and junky when he coughs (the coughing is usually when he is crying or his brothers get him wound up) Everyone seems to think if it was bacteria related all of these anti's would have taken care of it but it bothers me to no end that they have nothing to say about the inflammation/mucus issue! He does have a clinic appointment next wed and I will be putting my foot down for answers... It just doesnt make sense! Caleb can go on anti's and sound great within a few days.... I just worry that wyatt is going to suffer lung damage the longer it goes on!

If anyone can offer any advice on this id greatly appreciate it! Also if anyone has any hypertonic saline experience I was thinking of talking to them about it to see if maybe that would help!

wyatts current meds are-

Tobi nebs every 28 days
pulmozyme daily
albuterol
flovent
creon 10
adek
axid twice daily
culturelle
cipro- he just finished!
vest twice daily

I will try to post a few pictures later when the kids calm down!

Until then..............
Melissa

Vest time.......

Caleb fell asleep half way through :)


Wyatt on the other hand.....

Until next time...........

Wyatts 15 month well child check!

Well Wyatt had his 15 month wcc today and 3 shots :(.. He was not impressed to see anyone there! He fell asleep on the way so he was out cold through the first part of the appt. He slept through me undressing him and taking him to the scale! I layed him down and everyone was laughing that he was still passed out! I took it diaper off and all hell broke loose :).... He looked around and flipped out! He has never liked the doctors office and the trip to Barbara Bush childrens hospital didnt help the situation at all. Dr. H said he looked great! He weighed in at 26.6 and was 32 inchs tall. He actually lost a few oz. but she said considering the massive diarrhea he's had the past 2 weeks on cipro thats great! She said she would have thought he'd have lost a pd or more! His lungs were clear and she gave me a script to help his diaper rash.. He's had it for 2 weeks now and the nystatin cream makes it hurt worse. It is a yeasty type rash so he kinda needs the nystatin.

Until next time.........

You know you've been dealing with CF too long when....

I didnt actually write this but I think its so true :)



You Know You've Been Dealing With CF Too Long When ....

- You've used puppets, toys, sung, danced, pleaded and generally made a fool out of yourself all in an effort to get your child to eat.

- You've stockpiled enough applesauce to start your own factory

- You find loose enzyme beads literally everywhere, the car, your clothes, -the ceiling (not joking)

- You count more calories than sheep

- You add salt to everything and use butter as a general cooking base even when a recipe doesn't call for it

- You panic when your child sneezes

- Your child coughs and you immediately chant "Xopenex four times a day!"

- You check websites daily for news about a cure

- Your house has more medications than the pharmacy

- You've actually found yourself explaining a medication to the pharmacist instead of the other way around

- You've considered dying all your child's clothes yellow to hide the vitamin stains

- Your medical bills are looking more and more like the national debt

-You've prayed for your child to be fat

-You have a strong urge to smack whiny parents of "normal" kids

-You've actually found yourself exclaiming angrily in the grocery store upon discovering extra-cheesy mac doesn't have more calories than regular (sadly true)

-You know what the poopie dance is, and you've done it

-You've had long involved discussions with other parents about poop

-You've started referring to everything in acronyms (CPT, PA, MRSA...)

-You're seriously considering a medical degree, cause at this point you know more than most doctors

-You've reduced a complete stranger to tears by yelling at them not to touch the baby

-You can describe the hospital's daily menu in exact detail

-Purel and Lysol are your new best friends

-You've considered life-long quarantine for your child

-You've told someone off for coughing near you or your child

-You have multiple doctors on speed dial

-You refer to CPT as your child's nightly beating

-The pediatrician sees you more often than their own staff

-You tend to refer to life AD & BD, After Diagnosis and Before Diagnosis

-You've cried over half a pound

-Your child comes with an instruction manual if you leave them with a sitter

-You've forgotten what normal is

-You find empty enzyme capsules in your pockets

-Your child wears a neb mask more easily than they do a hat

-Your child has started trying to do their own CPT

-You own every Baby Einstein video made and are eagerly awaiting more just for something different

-You've forgotten to put meds in the nebulizer and actually let it run several minutes before discovering the problem

-Your after clinic routine at home looks like a hazmat excercise


Until next time.....

Older Photos.....

Wyatt the snow baby early 08
Wyatt making a mess with cereal :)

Wyatt Glow baby! 07

Caleb sneaking in some lovin!

He is so Proud to be a big brother!

Until next time....

Melissa

Wyatts 1st picc.. 11/3/2008

Mr. Wy checking out his 1st picc line Nov. 3 2008!

Melissa

You have to start somewhere!

Well, Im not really sure how to start this.. I have had a blog before and I never really shared it. I ended up losing it somewhere in cyberspace :) Hopefully this one wont end up with the same fate as I think this will be a great way to spread awareness, vent and meet some great people.. I am married and have 3 great boys. My 2 youngest both have cystic fibrosis. Caleb is 7 and wyatt is 15 months. Caleb was diagnosed when he was 7 months old after being sick for a couple of months and failure to thrive. Wyatt was diagnosed at a month old. We had planned on having him tested anyway but when he wouldnt gain a single ounce for the first month of life, I knew in my heart that he had it too. Both kids are doing fairly well. Caleb currently culture staph and wyatt cultures staph and pseudomonas. Each of the boys has been hospitalized once. Caleb when he was 7 months was transported to barbara bush childrens hospital from his dr's office as he was having mild seziures and was lathargic. He had been sick with everything under the sun and his sodium level dropped to a dangerous level. Wyatt has had a tough go of it! He started with some weight issues, then caught a cold... then another cold.. then another... you catch the drift! It got to the point where I walked into the clinic and said something is up! Nothing is working and he is still sick! We decided that a bronchoscopy was a good idea and during that they found that he was culturing pseudomonas. He was imediately put on cipro and 28 days of Tobi nebs, but even after all of that he still had a nasty wet cough! Thats when it was time for wyatts first admission! He spent a awful week in the hospital! It took over a dozen needle pokes to land a temp. iv in his foot! Then about 36 hrs later he blew that line and another unsuccessful 9 trys to get another one in... Then he went under and they inserted a picc line (after another dozen failed iv attempts while under sedation) He was sent home for a week of iv antibiotics. Wyatt was good for a few weeks and then he caught yet another cold.. he is currently on cipro and this is also his Tobi month. So far he is sounding good so I am hopeful that just maybe these poor kiddos can catch a break!

I will try to get some pictures up when the kiddos are settled long enough to do so! :)


Melissa