Home for Christmas!

Breathing treatment with Mommy.
Making room decor with Aunt Jenn.
All dressed up and ready to go.
Thumbs up for going home!

Dr. McKean came into Elena's hospital room and asked her, "Do you want to go home?" to which she replied, "no". His response, "Never heard that before."

However, once she was home, she was very happy to be there. She was all smiles in her own bed surrounded by her favorite stuffed animals. She was welcomed with hugs from her siblings. We all slept well that first night reunited!

Elena's final diagnosis was Pneumonia and Staph infection in her lungs. Both of these could be treated at home once we got her stable. She has been doing great! She take her meds like a pro and she is doing her new vest routine without complaint (most of the time.)

We thought her weight might plummet from being so ill and dining on hospital food, but that was not the case. She maintained and told me that she preferred the hospital food to my cooking! So, we have been trying to replicate the hospital meals at home and she is actually eating better. Who knew?

Thanks for the prayers, visits, food, and loads and loads of help. We are so thankful for our community of friends and family.

I could not have made it through the nights and days without my Sister Jenn, my "adopted sister" Andrea, and my Great Aunt Cindy who stayed with me for many hours away from their own family to help hold me up. Also, the many visitors at the hospital, My parents, my father-in-law, Elena's pediatrician Dr. Miller ( a dear friend of ours), Samantha (a CF Mom), Aunt Anne,
For those who helped Susie at my house with the kids and the food, thanks Amanda, Becky, Andrea, and Uncle Brian.

Thank you thank you thank you.

A diagnosis...maybe?

We have a possible diagnosis! Looks like Elena has a staph infection in her lungs. This is the best possible scenerio b/c it causes little to no damage on her lungs. We are so grateful for knowing. It will be confirmed tomorrow morning. If the results are positive, then we are going home tomorrow...maybe Saturday. We are cautiously excited.

Still no news

We are still unsure of a diagnosis. However, some tests run this morning show that the antibiotics are not working as well as they had hoped. We won't be leaving today or tomorrow for sure.

Elena was very excited to get out of her room today for some "play time" in the physical therapy room. She rode a bike up and down the halls like a crazy biker chick...it was very funny.

No news...but more smiles

Taking the IV for a walk to get her prize for the day since she completed all her treatments! Doing her breathing treatments like a champ!

Holding her new doggie "cookie" that her Great Aunt Cindy brought her today.

A giant thanks to all those who are praying, all those who have been by with food to the hospital and at our home, all those who have helped us at the hospital...you all know who you are and I am so thankful to have such fabulous friends and family.

Sitting Up and Looking Better!

Elena woke up this morning fever free!



Andrea came last night and helped keep an eye on Elena while I slept. Thanks Andrea!
Jenn came and relieved Andrea this morning and brought coffee and donuts! Mmmmm coffee!
Will let you know when we get a definite diagnosis.

ER and bacterial infection

Julie took Elena to the Children's Hospital at 2am when she spiked a high fever, was breathing quickly, and showed signs of hallucinating. We thought the cause might be food poisoning because Ethan threw up during the same hour, but it appears that was unrelated. After x-rays and bloodwork the current diagnosis is a bacterial infection. Her fever is down now, but the potential CF lung infection is weighing heavy on our hearts. Not sure how long she'll be in hospital. Prayers appreciated.

Change is good...VERY good!

Camping with Daddy....trying to catch a view of the Sunrise with Mommy.

Elena had a follow up appointment today with Dr. McKean. The results of all the changes have been better than we expected!

As many of you know, "Poop" is a popular topic of conversation in our home. When Elena has "sinkers" we all run (including Ethan) to the toilet to have a look and do a celebration dance. Sinkers have been so uncommon for her. Elena has also had yellow stool over the last 5 years, a sign of malabsorbtion. For the first time since birth, Elena has had BROWN sinkers without any excess oil! Not ONLY that but she is pooping far less now than usual. I know this may be TMI for many, but this is WAY exciting news. A sign that the new enzymes and added Nexium are working!!!!!!

The office noted a one pound weight gain. This is also another sign that the enzyme switch is doing it's job.

Other completely awesome news: Elena did PFT's (Pulmonary Function Tests) to see what the status of her lung function is. Dr. McKean explained that the percent average for children her age with CF is 93-94%. Elena's was 116%! Needless to say, the Dr. and I were quite pleased with this fairly uncommon high result for her age at this stage in her life.

Elena has been a different child since the change in her meds. She has more energy (which we didn't think was possible). She is eating on her own without constant poking and prodding from us. She is ASKING for snacks and begging for larger portions!!!!!! Really, we ask...who is this child? It is completely awesome!

I was so stunned with all the good news and positive results that I wasn't sure what to think as I left the Dr. Office. We feel so blessed with good fortune in our lives and this is yet another thing to give abundant thanks to God for!

Lots of Change

And then a little more!
The time that Joel and I spend dealing with Elena's disease is truly overwhelming. The daily effort of medication, weekly planning sessions, monthly budgeting of cost, and emotional energy invested is a full time job. We appreciate everyone's prayers. They mean very much to us and especially Elena. We haven't been talking about it much lately, so thought a thorough update would be nice...

It has been a rough year for Elena. After months of thinking, examining, and contemplating where Elena needs to be receiving care, we decided to change doctors. Dr. Caplan has been Elena's CF Doctor since her birth. He was teamed up with Dr. McKean at that time. Dr. McKean is the one who walked us thru Elena's diagnosis those vital months following her birth and surgeries.

It was a difficult decision to leave Dr. Caplan, but he was very encouraging when we told him our decision. So, now we are at Dr. McKean's private practice. He works with Atlanta ENT, but specializes in treatment of Cystic Fibrosis patients. Yesterday was Elena's first appointment.

We had a lot to tell Dr. McKean. SO, here are the changes that we are making in Elena's care and why:

Pancrecarb MS-16 verses Ultrase MT12

(This is the drug that Elena has to take with every meal to mimic the function of her pancreas. They break down fats and proteins so her intestines have a chance to absorb them.)

1. Elena is currently taking Ultrase MT12 and has been taking a form of Ultrase since her birth. It has been extremely effective until this year.


2. Her stools have been full of oil/ fat and rarely are the much desired "sinkers" that we should all have.


3. Her abdominal pain has been excruciating at times, but mostly a constant pain that disrupts her meal time. Several times during a meal she will either get up to run to the bathroom or she has to sit back and relax until the pain passes so she can eat.


4. A child with CF should have 3-5 stools a day. On a regular day, Elena is having 7-10. NOT good.


5. She has been very uncomfortable because of the increased time on the potty. Sometimes it is so bad, she keeps crying that her butt hurts.


6. SOOOO, this change in enzyme brand is a good trial because this enzyme releases a different chemical (if that is the right word) that aides in the effectiveness of absorption. Apparently, the Ultrase does not have that.
   

Nexium verses Prevacid or Omneprezole

(this is a drug to help increase the effectiveness of the enzymes by changing the pH level of the digestive tract)

1. Elena was originally on Prevacid, it seemed to work, but it was very expensive on top of all the other expensive medicines she takes.
2. Next we tried Omneprezole which partially did it's job, but caused her more abdominal pain. Dr. McKean informed us that this drug has numerous side effects, thus explaining her increased pain and the lack of effectiveness.
3. Now, we are going to try Nexium if the new enzyme doesn't help her fat absorption. We are preparing for a battle with the insurance company to try to offset the cost.

Continue Cyproheptadine for increasing appetite and aid in her sinus allergies

1. Elena calls this her hungry pill. She will continue taking this, but will be split in half for morning and evening consumption.

Start Aquadeks for vitamins instead of ADEKS

1. Elena was taking ADEKS chewable vitamins because she got them thru a program with Ultrase. Now that she is no longer on Ultrase, we will use Aquadeks for her vitamin supplements.

Continue the vest treatment twice a day

1. Elena has been wearing her airway clearance vest at least once a day. We are going to add the evening in more routinely to be done in conjunction with the new meds listed below.
   

Inhale hypertonic saline and Pulmicort in her nebulizer twice a day

(The hypertonic saline is a liquid med put in a nebulizer with Pulmicort. The hypertonic saline is used to help keep things moving in the lungs by reducing and slicking the mucous and the Pulmicort will help to reduce inflammation which in turn helps increase the effectiveness of the hyper tonic saline.)

1. Some of you may remember when the hypertonic saline was introduced into the CF community after research results from people with CF that lived on the beach saw much improved lung function.
2. We decided to go the "aggressive" route with Elena's lung care. Dr. McKean reminded us of some info we already knew and shared with you after the last CF education day. Children Elena's age with no outward signs of lung problems can still have lung disease. Elena could very easily be one of those children.
3. So, we are going to take both medicines to help reduce the risk or help improve damage that could already be present. Once lung disease settles in, it can not be removed. "Aggressive" is the way to go.

We are VERY excited about the changes that are being made and hopeful that Elena's quality of life will improve.

A giant thanks to the Dr. Caplan, Nurse Renee, Dietitian Liz, and Receptionist Joy from the CF Center at Emory for being wonderful with Elena and more than understanding about our move. Of course, a thanks to Dr. McKean for taking us into his practice!

New Drugs Update...and request

As many of you may remember from a previous post a year ago, we wrote about some exciting new drugs that were on their way to helping those with CF. One of those drugs is now in clinical phase trial. Elena's Grama, Susie Goodrow, sent out a prayer request with the following info:

We thought we would pass this info on to you for prayer of this new drug treatment for CF. It looks quite good.

Elena, however, is a Delta F508 patient and this drug will focus on G551D. Hopefully, the VX-770 or VX-809 will also work to help Elena in the future. See the info below and in red the area about Delta F508 and this drug.

Either way our prayers will not only help Elena but others and every step of research for CF leads in the right direction we all want to see.

For more information or answers to questions you may have go to www.cff.org

Please pass on to your family, friends, and prayer groups.

Thanks,
Praying Grandparents,
Susie and David

VX-770 Trial Begins in Children as Part of Phase 3 Program
August 5, 2009
Today, the CF Foundation announced the initiation of a clinical trial in children as part of the Phase 3 registration program for VX-770. VX-770 is an investigational oral therapy aimed at treating the underlying cause of cystic fibrosis. The drug is being developed by Vertex Pharmaceuticals with support from the CF Foundation.
This is the second of three clinical trials underway in the Phase 3 registration program for VX-770. The registration program is designed to generate data that the U.S. Food and Drug Administration can use to determine if VX-770 is safe, effective and acceptable for approval.
“This potential drug is one of the most promising therapies in our pipeline that aims to treat the underlying cause of cystic fibrosis,” said Robert J. Beall, Ph.D., president and chief executive officer of the CF Foundation.
“We’re excited that enrollment is open for this second trial. The initiation of this study is an advancement in our efforts to bring a new therapeutic option to CF patients.”
The trial is a two-part study of VX-770 in patients with CF age six to 11 years who have the G551D mutation of CF. It will enroll approximately 30 children with CF.
The first trial, which was initiated in May, is examining VX-770 in people with CF age 12 and older who have the G551D mutation of CF.
Why wasn’t the Delta F508 mutation chosen for the Phase 2 VX-770 clinical trials?
Because G551D is already located at the cell surface, these patients have the greatest potential to benefit from treatment with VX-770. Most Delta F508 protein is not at the cell surface and therefore it is unclear whether the patients with this mutation will benefit. The Phase 3 registration program of VX-770 will help determine if it is effective in this patient population.
It is possible that Delta F508 patients may need to have the location of their CFTR protein corrected to achieve the most benefit from VX-770. Vertex is developing another compound, VX-809, which is currently being studied in patients homozygous for the Delta F508 mutation. VX-809 is designed to increase the concentration of CFTR proteins at the cell surface.

Joel followed up with:

Thanks, Mom, for sending this out. Here are a couple other notes...

-Notice that these groundbreaking drugs are being developed with heavy support from the CF Foundation. In fact, this work would not be possible without the CFF pouring over 90% of every dollar it receives into this type of research. Drug companies will not do it alone because it costs too much to develop a medicine that only a few thousand people will need to buy. For this reason, Julie and I thank everyone for their support of events that benefit the CFF.

-Phase 3 FDA trials of these drugs could last many many years. We need to pray for the proper political support and leadership that can accelerate the process.

-Elena is too young for most clinical trials, but it's possible that she could be on the cutting edge before we know it. Prayer is needed that she be at the right place at the right time with the right criteria to potentially benefit from (and tolerate) the trial.

This is all very exciting news. LOTS of information to digest, but wanted to be sure all of Elena's support is aware of the awesome strides that are being made because of the prayers and donations you have all made over the past five years. We are MOST grateful!

THANK YOU!!

"Bottom" Issues

Elena has been struggling with an ouchy bottom, many "oopsies-poopsies", and extra oil/ fat not quite making it into the "storage facility" in her body. We think we might be at a plateau in her weight gain because it is time for an increase in her enzymes. Usually, enzymes are increased according to weight gain. Elena has had no gain. However, she has been consuming more calories within the last month thanks to her Mom's ever-more creative cooking.

Would anyone like some fat with a side of fat?

We look forward to seeing what changes will be made in her enzyme consumption as well as what this will do for her lack of weight gain.

Time will tell! Thanks again for the prayers and words of encouragement. Those rough days are a little easier when we reflect on the kindness of our friends and the grace of God!

Cruise and Weight

This is called "3 pieces and 6 pieces" of pizza before dinner!

As many may have heard, you tend to gain weight on a cruise ship since there are about 15 different opportunities to eat everyday. However, somehow Elena remained the same weight. She did, however, have a "boat load" of fun.

Elena's friend Giselle

Elena is SO thankful for the Disney Cruise fun that Nana and PaPa provided for her, her family, her cousin Will, and Aunt Jenn...what FUN! She has talked about many adventures like going under water at the beach, meeting all the Disney characters (especially Captain Jack Sparrow), and of course the friendly Staff on board. Her favorite staff was surprisingly the food staff. We are surprised since eating is her least favorite thing to do. The one that made the biggest impression was named Giselle like the princess from Enchanted. This was a friendship meant to be!

Good news is...she did not loose weight. So success is still ours!

New Goal!

Elena has her sights set on Six Flags! Joel and I have told her that if she weighs 45 pounds, then she can go to Six Flags. This is very excited for her b/c she has been begging us to take her to a REAL roller coaster.

Our little timid girl is now a VERY brave 5 year old. She decided to start swimming and diving this summer (last year she would scream if you asked her to blow bubbles in the water), she is jumping over waves at the beach (last year she left scars on our arms from the scratches she left trying to scamper up our bodies out of the moving water), she smiles at the dentist while he fills a cavity (last year she cried when the dentist said her name), and NOW she wants to ride roller coasters (last year I took her on a small kiddie coaster and she cried for an hour). Marked improvement in just one year!

Join us in praying for Elena's weight gain. Weight gain for Elena means that her health is much more stable...it means that her lung function remains high...it means that she fights off illness MUCH faster...it means that she continues to grow strong...which means that her life expectancy continues to grow as well!

Go Elena! We are all praying for you and your continued good health!

What? Weight Gain???

So, we don't want to jump to any conclusions, or get too excited, but....Elena got on the scale this morning and was up to 42 pounds! That is a little under a two pound weight gain while also over coming a nasty cold. She usually vomits her way through sickness. We are so happy with this turn around!

She decided to start drinking milk again, but only if it is in a certain cup with three ice cubes. I'll feed it to her while standing on my head if that is what will work!

This morning, we all sat down and had a ramin noodle party for breakfast with Elena. Ethan thought he was in HEAVEN! He sat at the table with us while wiggling a little jig of glee as he ate his noodles. Elena ate her entire bowl ( a bowl worth 700 calories after we added butter and olive oil). Andrea, I realize that the sodium count in ONE bowl would do you in for the month. Of course, you all know that Elena needs the extra sodium...so on top of the 1560 mg of sodium...she added more salt! Sometimes it is hard not to gag while making her meals:)

We may have finally overcome the 40 pound plateau! As usual, we will keep you posted.

Great Strides Success!

It was another fabulous year for "This Team is Brought to You By the Letter E". We raised just under $6000. The entire event brought in $600,000!!!!! It is overwhelming to see the amount of support our friends and family give us with CF. The generosity and support make the tough days so much easier.

Elena continues to do VERY well. Her weight had gone done, but is slowly creeping back up. She is steady around 40 pounds. She has not fallen below 40 in several weeks, so this may be a permanent thing! Praise God!

She has been doing her vest treatments daily and reeping the benefits. She has defeated colds and sinus allergies better than her healthy family members. God rocks!

Elena has a quality of life today that was not even available for kids her age 20-30 years ago. Most children didn't live past Elena's age of five. The money you raised goes directly into funding research that searches for a cure for CF. We are so close to a cure, all the while Elena is provided with more time because of all the new found treatments.

Thank you everyone for being a part of our team and prolonging her very precious life.

The walkers, the team leaders, the donators, the prayers...ALL are Elena's heroes.

Last Chance!

Last chance to join our team for Great Strides this Saturday at the Georgia Tech Burger Bowl.
http://www.cff.org/Great_Strides/JulieGoodrow & donate to
"THIS TEAM IS BROUGHT TO YOU BY THE LETTER E"!!!!!

Every dollar makes a difference. We are SO close to cure for Elena. Help us get there! Thank you and God bless!

Team 65 Roses: Elena Style

Elena was going to run in the ING Tot Trot again this year. The only difference was...she was sporting everything 65 Roses. She was so happy about being a part of the team. Unfortunately, the event was rained and thundered out. She did however get a medal. She said, "How can I get a medal when I didn't run?" Mommy and Daddy said, "Take the medal and run! You run your race everyday with CF!" This answer was good enough for her. She wore her metal the rest of the day (and today)!

Elena's weight is tettering back and forth, but has not gone up. The "hungry pill" continues to make her hungry...so it is still working. Ya-hoo for that!

Also a MAJOR news update on CF research. Below I copied what another CF mom wrote from her blog. Her daughter , Caroline, is not even a year old yet and was diagnosed with CF early like Elena. She simplified the good news perfectly.

"First of all, they are working on new medication for Caroline's digestive problems. Right now she takes 3 pills with every meal or snack (and that number is likely to increase soon). {Elena takes 4 and started with 1/2 when she was a baby..the amount increases with weight gain

}She would only have to take 1 pill with each meal or snack with the new meds. Plus, they are working on a liquid form for infants...you can imagine how much easier that would be!

Even more exciting is the development of a drug that could potentially cure the basic defect in CF patients. It makes the defected protein work properly, which means chloride passes through the cells and prevents all that troublesome sticky mucus from forming. This means better lung health, which is literally a life saver! I knew about the drug but did not realize they would be testing it on CF patients with Caroline's mutation, DF508 (There are over 1,000 mutations that cause CF). They started Phase 2 of the trial to test for safety and effectiveness of the drug. Keep your fingers crossed and bless the people who have volunteered to take the meds in the trials=)Caroline is so young and things already seem so promising for her future health. "

This is great news for Caroline, Elena, and ALL those struggling with CF. Thanks to Caroline's Mom for the simplified explanation!!!!

Another Pound and ANOTHER!

Today was Ethan and Elena's Annual check-up. The Pediatrician was more than thrilled to see how giant Ethan had grown. He is in the 95th percentile in height and weight at 26 lbs and 9 oz and 32 inches tall. He is our giant boy!

Elena had a MAJOR break thru this week. The hungry pill continues to work wonders! She is now weighing 39.2 pounds at home...at the Doctor she weighed 40 with shoes and clothes. Last weigh in at home was 37. SO...this makes a solid 2 pound weight gain for the month! Way to go Elena! We are so glad that she is back to 40 pounds again...even if it took 6 months to get there.

A Precious Pound!

YES! Elena gained a pound! So, it has been a long road, but she has finally gained 1 pound. So know she is 37 pounds. We still need to get her up past 40, but any direction up on the scale is a success.

Also, she is finally understanding the value of her vest treatment that she does daily. Today when her treatment finished, she said," Mom, I can breathe better this morning. I think my vest helped." Yes...yes it does!

Wa-hoo!

My Hungry Pill

Elena started an appetite stimulant and WOW! She is hungry all the time! YEAH! Something is working! She has started drinking milk as well. She takes a half of the pill with breakfast and then the other half with lunch. She calls it her hungry pill. Still no weight gain, but at least she is eating more.

A funny story about CF...Elena was with a group of young girls at church the other night. She announced that she had CF. They asked what that meant, she said, "I'm not quite sure, but I have to take enzymes before I eat." Later she asked me, "What is Cf for?" translation "What does CF stand for?" I said, "Cystic Fibrosis". She replies, "Oh yeah! 65 Roses"

CF Education Day

This is lengthy, but loaded with info. Each section has notes that Joel typed during the 30-60 minute presentations. The notes don't represent a summary of the presentation, just things that we wanted to remember or that applied to Elena.

Marcus Institute - David Jaquess PhD, Director, Stephanie Powers
1-2% of all children require intervention to achieve adequate body growth
50% of children with CF need some sort of assistance (psychological or physical). There is WIDE range of severity.
Bad experiences & Feeding problems can cause eating difficulty. For example...
1) Choking incidents that causes long lasting fear
2) Disrupted parent-child interaction
3) History of pain or discomfort
4) Taking lots of effort to eat
The above list is for all children, but CF children usually overlap with all of them. This is especially caused by the CF child’s consistent need to eat until over-full. When someone becomes hypersensitive to eating challenges, motor & sensory “defects” develop (like being averse to the way a particular food feels in your mouth).
Pavlov thought applies to CF kids. They associate unpleasant sensations after eating just like the “ringing bell” effect on the dog. If we try to change the stimulus or environment of eating VERY gradual over time, the Pavlov effect would be decreased like the treatment of a phobia.
Completing routines is critical, but when and in what order – maybe not so much.
Punishment does not motivate change to new behaviors. Plus, children may not make the connection anyway. Negative side effects of punishment could be physical aggression by the child or disrupted family interactions.
Positive reinforcement idea: use selective attention by reacting fully to behavior that is desirable and reacting minimally to poor behavior.
Changing your method to positive reinforcement can be very difficult. If the child is used to getting their way by throwing a fit, they will only throw more fits when they see that the fit isn’t working the way it used to. For example, we know that Elena fakes tummy pain, and would be likely to fake more tummy pain in response to positive reinforcement that was causing her to eat more.
But Elena doesn’t “get” the idea of rewards – she just restructures her enjoyment. She recognizes the motivator, and is too smart when she alters her behavior without actually eating more.
The presenter’s examples confirmed that we’ve tried everything. We shouldn’t abandon a strategy after a week, but fine tune it and stick with it.
Modeling is a good way to display positive reinforcement. For example, rewarding Ethan when he reaches goals during mealtime to show Elena how it is supposed to work.
Find things that will motivate her daily, weekly, and the BIG DEAL like
Daily: later bed time, family game, movie rental
Weekly: park, bowling, date night with Daddy
Big Deal: Aquarium, Disney, Show at Fox etc.
We really need to put thought into what can motivate Elena and get a routine for a few weeks.
Use motivators during meal (draw a picture, one line per bite or color in a picture one square color in per bite).
Contact Meghan for some ideas (she has feeding experience with her work)
When to seek help – no progress for 3 months, disruptions getting worse over 3-6 months, nutrition becomes critical (feeding tube discussion), you feel lost.
We should call this doctor and review Elena’s case. He specifically said he would help brainstorm and would not charge and be honest about whether help was needed.

New Therapies in Pipeline – Nael McCarty PhD, Senior CF scientist for Emory Medicine
(Look at these notes in conjunction with the pipeline chart)
CFTR Modulation – Emory will soon be starting Phase 3 trials, this is big stuff and we need to look into Elena’s participation. “These are the most exciting things happening.”
VX770 – For G551D, but might be used in conjunction with other drugs that directly target DelF508. We need to cheer on this one too, and didn’t realize this before.
VX809 – Meant for us!!!!! DelF508. Trials starting at Emory when?
Denufosol – restores airway surface liquid, great progress, applies to all CF mutations. TIGER-2 trials are only open to ages 8 and older.
To prepare Elena for NPD procedures in the future, we should start using neti pots. Other CF parents we’ve talked to encouraged their use anyway.
Manage your expectations – no one knows what drug development programs will fail. We saw the drug pipeline from 8 years ago, and a bunch of them fell off the chart since then. 20% of Phase 1 drugs make it, 50% for Phase 2, 80% for Phase 3.
Even if Elena doesn’t start receiving newly developed treatments for another 10 years, it could still extend her life expectancy to beyond age 50-60.
Carriers of CF (Julie, Joel, etc) have a higher incidence of sinus problems because having just one DelF508 actually does affect the way that CFTR produces protein.
Joel spoke to Nael at a break, and learned more about what he does. Through our conversation I gleaned that the rate of CF research is increasing because the research being done applies to many other areas. Interest among scientists in working with CF related topics is generated because the developments have far reaching repercussions outside the relatively small population of CF patients.

Emory CF Center Update – Dr. Schechter
We should not be upset when Elena is hospitalized for a tune-up. It means she is getting the best care possible. Studies show that aggressive use of antibiotics to treat pulmonary exacerbation produces the best results, and that sort of treatment is done in the hospital. Plus, if she’s hospitalized we could reapply for Medicaid (hope & change).
Periactin (sp?) – the appetite stimulant we need to ask about
Elena’s BMI percentile should be over 50% to give her the best chance for good lung function in the long run. This isn’t just a “theory,” it’s proven. Mean BMI for patients under 20 years old at Emory CF Center is 52 percentile.

New and Better Approches to Airway Clearance – Mary Lester
CF is an awful sneaky disease. Even parents and family fall into the false thought process of “symptoms are low, so everything must be going fine.” But symptoms are always there and building over a lifetime. Consistent airway treatment is a must.
Active Cycle Breathing Treatment – introduced at around Elena’s age, we need to research this
It’s a family disease. If everyone is involved with the treatment, it works better!

Importance of CF Clinical Research – Jeannette Peabody RN
Common reasons why people stay out of research trials…
1) Fear of the unknown
2) Possible risks (study drug side effects, safety, procedures/tests)
3) Study is asking too much of you
4) Time off from work/school
5) Costs (time & travel also)
Clinicaltrials.gov and type in cystic fibrosis
Considering Elena’s existing challenges, doing trials just might be too much. We know what Elena can and can’t tolerate. There has to be some trials out there that could work.

Two girls with CF, one 18 & one 22ish, were part of presentations. They both shared that they were part of the original Pulmozyme trials many years ago. It was emotionally straining to see these young women. Why do they appear well? What is their life like? Why do others we’ve met not do as well? We can’t control CF and have to live with the unknown. All of our efforts and focus cannot save our child. We try to turn my feelings of grief and sadness to feelings of hope.

Tough Night

Sometimes Elena has long nights where she is up using the toilet a lot. Last night was one of those nights. She also threw up in her bed once. We are not really sure why this happens sometimes, but it does. In the morning she is exhausted but with very little memory of the night, thank goodness.

She spent the day resting and ate better than usual after a night like that. Weight still holding, no gain yet. We are just glad there is no more weight loss.

Weight Gain

In an attempt to make it easier to keep up with Elena's health, I have made this seperate link. I will update this as new things happen with Elena.

So, this morning I was looking at some picture of Elena from 5 months ago. I was noticing how round her face looked, how mooshy her arms looked, how soft her tummy looked. I realized that Elena is too thin. A 6 pound weight loss on her is SO huge. This would not show on most adults, but is catastrophic on young children.

As stated in my most recent post, Elena has been eating much better. However, there has been no weight gain.

Continued prayers are very much welcomed!