Wednesday, December 23, 2009
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.
Thursday, December 17, 2009
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.
Wednesday, December 16, 2009
Tuesday, December 15, 2009
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.
Monday, December 14, 2009
Friday, October 30, 2009
Wednesday, October 7, 2009
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.)
- 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.
- Her stools have been full of oil/ fat and rarely are the much desired "sinkers" that we should all have.
- 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.
- A child with CF should have 3-5 stools a day. On a regular day, Elena is having 7-10. NOT good.
- 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.
- 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)
- Elena was originally on Prevacid, it seemed to work, but it was very expensive on top of all the other expensive medicines she takes.
- 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.
- 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
- 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
- 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
- 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.)
- 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.
- 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.
- 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!
Wednesday, September 2, 2009
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.
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.
-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.
Tuesday, August 11, 2009
Monday, July 13, 2009
Monday, July 6, 2009
Sunday, June 7, 2009
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.
Sunday, May 17, 2009
Thursday, May 14, 2009
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!
Monday, March 30, 2009
Tuesday, March 17, 2009
Friday, February 27, 2009
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!
Thursday, February 19, 2009
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"
Sunday, February 8, 2009
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.
Wednesday, February 4, 2009
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.
Tuesday, January 27, 2009
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!