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Archive for April 2015

The Way CFers Measure Health Status - aka, how is Bennett's CF these days?

Tuesday, April 21, 2015

Bennett had his 3-month Cystic Fibrosis Clinic appointment last week.  So, I thought I'd share a little bit about how that went and how that tells us how Bennett is doing with regard to his Cystic Fibrosis health.

There are five primary measurements that the Cystic Fibrosis Clinic clinicians typically use during a regular CF Clinic appointment to determine how a CF patient is doing.

These measurements include:

1.) Oximeter Reading
2.) Weight gain/loss
3.) Pulmonary Function Tests/PFTs
4.) Throat Culture Results
5.) Lung sounds

Each time we go to the CF Clinic (which is every three months when Bennett is healthy), Bennett has to go through a round of invasive testing to help the doctors determine whether or not he may be getting sick.  




One of the first tests Bennett must undergo are evaluation of his vitals.  Bennett's fever is taken (to indicate current infection/virus) as well as his blood pressure.  Bennett submits one of his little fingers for testing with an oximeter.  An oximeter measures the amount of oxygen in his blood.  Typically we want to see 100% but anything over 94% is best.  Bennett's last oximeter rating was 99%.



The second test often used to monitor CF weight loss/gain.  Due to malabsorption issues in CF, Bennett's lack of weight gain has been an ongoing problem.  At our last appointment, the doctor, dietitian and I decided to change his enzymes, increase his tube feeds and increase the amount of calories in his formula.  These changes look like they are benefiting him.  Bennett gained 2 pounds since our last CF appointment 3 months ago so this shows Bennett is doing well!




The third test is called a Pulmonary Function Test or PFT (see video above).  Children under 5 sometimes do PFTs for practice but CF patients under 5 typically do not do PFTs because they require good technique to provide an accurate reading.  It requires taking a breath and then, as fast as you can, pushing out as much air from your lungs as possible.  Our clinicians say a PFT FEV1 of anything over 80% is good.  So, Bennett's blowing 90% for his first real time is excellent.  He will get better at the technique in the coming years.  Bennett's PFTs showed us his lungs are working at full capacity! Woo-hoo!!

A fourth test we do at each CF Clinic visit is called a "throat culture."  This is when the respiratory therapist or nurse puts really long cotton swabs against the back of Bennett's throat in order to get a culture of any bacteria that might be in his throat.  This throat culture is sent to a lab.  It takes several days to get the throat culture back but we typically learn within a week.  Bennett showed no new bacteria or infection in his lungs (outside of typical Staph, which is normal in CF).  SUCH A BLESSING!!!  

The last test that Bennett often undergoes at his regular quarterly CF Clinic visit is a listen of his lungs by the CF Pulmonologist Doctor.  Our pulmonologist said Bennett's lungs sound great so we are not worried about any infections at this time.  

We go back to Dallas to the CF Clinic in July where we will go through all of these tests again.  But, for now, we are celebrating the small blessings of weight gain, clean throat cultures and good PFTs!

Like a seismograph to an earthquake, these tests indicate the beginnings of a lung infection and the progressive nature of CF.  So, every single good test result is a blessing. Every. single. one.

There are other measurements that tell us how Bennett is doing - such as chest X-rays, CT scans, bloodwork and his own feelings about how his body feels.  But, these are our main indications of how things are going when everything else is looking good.


The question I often get is, "How is Bennett doing these days?"  And my answer is: We've made it to 5 years and 7 months with no lung infections.  So, he continues to do amazing!

Tremendously thankful for today,

Seriously, how close are we to a cure? CFF State of the Science Update 2015

Monday, April 13, 2015

Several weeks ago, I attended the Cystic Fibrosis Foundation's Volunteer Leadership Conference in Virginia. 

One of my very favorite parts of VLC is the State of the Science because it is when the CF Foundation provides their annual science update.  I wanted to share what I learned there (feel free to watch the entire conference here: http://www.cff.org/GetInvolved/VLCLiveStream/).  

There was a lot packed in to a small amount of time.  But I narrowed down the most important parts of this update to four main points of interest:

1.) Kalydeco Update
2.) An update on the Combo Drug (hopefully to be out this year)
3.) VX-661
4.) 2nd Generation Modulators
5.) Stem Cell Biology/Gene editing
6.) Personalized/Precision Medicine


The "State of Science" at VLC was presented by (from L to R) the Cystic Fibrosis Foundation's CEO Bob Beall, Preston Campbell, the CFF's Executive Vice President for Medical Affairs, Michael Boyle, Director of Adult CF Programs/Associate Professor of Medicine at John Hopkins Hospital and Bill Skatch, the CFF's Vice President for Research Affairs.


This picture demonstrates the five ways the CFTR mutated genes work incorrectly.  At the bottom, it shows how many people have that particular gene.

Bennett's F508del gene is a Class II (87% of those with CF have at least one gene in this group). Bennett's 621+1G>T gene is a Class I (12% of those with CF who have at least one mutation in this class).


There are two ways that scientists are working to help fix the underlying reason for Cystic Fibrosis. These two ways or types of medicines are called "potentiators" and "correctors."  This explanation is super important to understand in order to follow the rest of the Scientific Update.

For those types of CF mutations where the protein is already sitting up at the surface, ready to turn on or ready to work but doesn't work correctly, "potentiators" are considered most helpful.  "Potentiators" are what we call medicines that can open that channel up or potentiate the channel because the channels are already there, they just need help with opening that gate up.   Kalydeco is a potentiator drug.

The more challenging problem in CF, but the one that scientists are making the most progress on, are those genes that benefit from medicines called "correctors."  "Correctors" are those medications that seem to benefit those with CF mutations in Class II - where the protein has not even made it up to the surface yet.  In order to fix this mutation, the protein needs to be moved up to the surface.  The reason it's called a "corrector" is because it corrects the folding so the cell allows it to get to the surface and work.  Lumacaftor is the first corrector drug.  It is believed to benefit those with F508del/F508del mutations.


KALYDECO UPATE: Kalydeco (also known as Ivacaftor) looks like it benefits those with CF mutated genes in Classes III, IV and V.  Another 20 mutations are just about to start being tested in clinicial trials for benefits of Kalydeco.  Kalydeco alone may benefit about 15% of those with CF.


COMBO UPDATE: There is excitement in the CF community because in a few months, the FDA will decide approval on the combination of Ivacaftor (Kalydeco) and Lumacaftor for those with two F508del mutations. Data shows that patients clearly benefit from the Combo drug (although, this drug is not strong enough to fix the condition for those with one delF508 mutation).  If approved, those with the most common CF genetic mutation would take a pill twice a day.  The FDA has set the date for May 12th to meet about the Ivacaftor/Lumacaftor combo.


VX-661 UPDATE: For those, like Bennett, who are not expected to benefit from Kalydeco or Lumacaftor, there is another set of CFTR Corrector, VX-661, that is hugely promising.

I learned during this talk that VX-661 is a drug that the CFF accelerated because of its potential.  Apparently VX-661 wasn't supposed to be in clinics until 2017 or 2018 but the CFF saw potential for this drug and decided to put in 25 million dollars towards getting this to patients faster.

Currently in clinical trials, VX-661 looks like Lumacaftor but works better, has less drug interactions and has a better safety profile overall.

This is a drug Bennett should benefit from. So, I'm very hopeful! They did not give us an idea of when it would be on the market but I do know they are in Phase III clinical trials so happening quickly!

Studies show that the benefit from VX-661 is even better Lumacaftor and may work with those with just one F508del mutation.  So, it could benefit up to 87% of those with Cystic Fibrosis.


Here are the current upcoming studies for those with one F508del mutations.  There is a study for those with two F508del mutations, a study for those with F508del and a Class I mutation (stop codons), a study for those with F508del with a Class V mutation and a study for those with F508del and Class IV mutation.


That covers about 90%+ with Cystic Fibrosis.  I am so very excited!  I have looked in to getting Bennett in one of these studies but, from what I found, these studies are happening in children and adults over the age of 12.  


2ND GENERATION MODULATORS UPDATE:  I'm not just excited for VX-661 for Bennett.  I'm also very excited about 2nd Generation Modulators.

This is a part of the Update that literally went so fast I neither understood it nor valued it.  In fact, I have had to spend several hours rewatching video from VLC over and over again so I could type what Dr. Skatch shared during this few minutes on stage.  This is amazing stuff but it's complicated.  So, as not to mess it up, I'm just going to write what he said had you listened to him speak.

Here is 2nd Modulators explained by Dr. Bill Skatch (pretty much verbatim):

"It's very clear that alot of effort has been put in to CFTR.  And the patients that have these mutations are going to have tremendous benefit.  But some of these things are going to take time and certain mutations are not going to benefit from these correctors. So to reach that 100%, there are several paths we are taking.  One path is to look at other types of treatments or channels.

To explain, you have nice cilia beating and mucus is moving along in the lungs of a healthy patient. But in a CF patient, where there is a defect, that water layer shrinks so cilia can't beat and mucus piles up up.

One of the strategies is to think: are there other ways we can improve that water layer?  Can we restore moisture to the lung that doesn't require CFTR modulator compounds?

So, the CFF is working on several projects that are designed specifically to target for other proteins, not just CFTR.  One of those proteins is a sodium channel.  

As you know, chloride moves through CFTR.  But sodium moves through another channel, that you can consider to be a partner of CFTR.  And it turns out, one of the reasons that water layer shrinks down and collapse is because the sodium channel absorbs too much sodium.

So we are working several companies to develop molecules that will specifically target the sodium channel (it's called ENaC).  If we can shut down that channel, there is good evidence, in the laboratory, that the water layer can be restored. We believe that that will help cilia beat better and help move that mucus up.

The nice thing about this is: it doesn't matter what your CFTR mutation is.  This type of therapy has the potential to work in all CF patients - because all CF patients share that problem with same dehydrated mucus and this very thin layer of water.

So the CFF is working with several companies such as Parion that have very exciting new molecules that can specifically attack the sodium channel."


"The corrector molecules were once a crazy idea - to take a broken protein, that doesn't move through the cell, to repair its folding, get it to the surface and then turn it on.  That seemed really impossible 15 years ago.  But now, in the test tube/laboratory, this 2nd corrector molecule is showing that is as good as Kalydeco.

What's happening now is there is a very large effort, in terms of 2nd generation screening.  There are multiple companies looking at new and better chemical compounds.  Millions of compounds were screened last year.  We are looking at very diverse compounds.  We are thinking, the more we look, the better chance we have to finding the best molecule.

We also got much smarter, in terms of how we are screening.  We are using human airway cells to try to screen how these molecules work.  We are targeting these molecules to specific parts the CFTR protein, because we now have an idea of how the protein is broken.

Our goal is develop modulators - correctors - that will be as good as Ivacaftor in the F508del patient population.

What does this look like?  We are thinking, as we move in to the future, we are probably going to need, for many of the folding mutations, two correctors plus a potentiator - a three drug combination.

That will hopefully to get us where we need to be.  This will hopefully open the door for a large number of CF patients."

"If everything goes perfectly (15% of potentiators + 50% F508del homozygotes + 40% F508del Heterozygotes), that covers about 95% of those CF patients.  But it leaves 5% of CF patients that don't make CFTR that can be potentiated.  

This 5% is very complicated - nonsense, stop-codon, splicing variants, deletion variants, etc.  These are going to require a completely different type of therapy. One way to go after these is to develop therapies, therapies that are applicable to all patients (such as the 2nd generation modulator idea). Another way is we can target these mutations very specifically.

If we look ahead, what do we want to do?  The goal is to take the therapies we have and make them better until we have a cure for all patients."



"We know what the problem is.  The problem is in the gene, the DNA.  It's a mutation, small deletion, which is altered.  That mutation is translated into RNA. And the RNA in the cell is a template from the DNA.

So, if you have a mutation in your DNA, that mutation is then translated in to the next step of the biological problem, which is RNA.

RNA is used to make protein.  So the defect of the the RNA is then translated in to the protein defect. And the protein, if it's CFTR, is what causes symptoms.  So, this paradigm is very basic in biology.

For the past 50 years, the CFF has been working on the symptoms - better therapies for the the mucus, better therapies for the infection, better therapies for nutrition.  This has had a tremendous burden on the patient in terms of the amount of effort and time.  But it was had tremendous affect on  lifestyle and lifespan and making life better.

We are now entering in a very early stage, of moving backward along a path to get to the more basic defect.

When we talk about correctors and modulators, we are talking about protein-based therapy. Those molecules work on patients that have specific types of protein abnormalities.  But some mutations don't allow the protein to be made.  In some patients, they don't make the so modulator therapy will never work.  Those patients need different types of therapies.  One of those therapies is to move back on more layer is the RNA.

That RNA molecule may be manipulated in a couple of ways.  One, in a project we are working with Shire, is to simply take normal RNA and put it back to patients.  If we can take normal RNA in to cells, then those cells will make normal protein and that normal CFTR will function in any patient.

So there is a lot of challenges for this.  But we are very encouraged by the results of Shire.  Shire is moving very quickly. We hope to have RNA therapy coming in to patients within a year to two, maybe by the end of this year, but hopefully in 2016.  We just committed 15 million dollars to that."



STEM CELL BIOLOGY/GENE EDITING:: "The CFF is also working on ways of addressing stop codon mutations, specifically.  The CFF recently put in 6.7 million dollars into a company called PTC to try to develop a molecule that will help the ribosome read through to those stop codons to make the protein.

It was unfortunate that that molecule did not work as well as had hoped.  But have not given up. We are going back and starting several collaborations with new companies to look for better and more effective molecules that will work on these stop codons and nonsense mutations, specifically, to allow the protein to be made, which then can be potentially augmented by potentiators and potentially correctors, if the protein doesn't fold correctly.

How about the one time cure? If we could go back to the DNA...if we could prepare the DNA in the cells that need to make CFTR, then those cells would make normal CFTR and all of this process would be completely normal.

Five years ago, this was an absolutely impossible dream. But what has happened in the last five years is there is some very exciting technology, where you can go into the cell and highly specifically target regions of the protein, regions of the gene, that are defective and repair those defects.  This is called gene editing.

There are a couple of ways to go back about this.  There are a lot of technical difficulties to make this work.  But this is tremendously powerful and really provides, for the first time, the ability to go into the cell and remove the CFTR defect.  Instead of treating the disease, this is essentially removing the disease.

One way this might work is to take cells out of the lungs of patients, identify which cells are the critical cells needed to restore the airway, go in to those cells and edit the CFTR gene so that now it makes that normal protein and put those cells back in to the lungs of patients - have those cells grow, reproduce and populate the airways, then it would be possible to basically eliminate the need for correctors, potentiators or any other kind of therapy

Just last week, we brought 30 investigators who are experts in stem cell biology to the CFF.  We had a fantastic discussion about what cells we need to target, how to the cells behave, how do we grow them so they can actually graft in lungs etc.

I will say there are many obstacles but it was a very encouraging discussion. There are a group of scientists out there that are really excited to work on this problem.

In December, we invited a group of scientists to the Foundation to talk about gene editing.  We are bringing those people into the CF field.

This is where we would like to go.  This is a long project. There are many obstacles. So, we see this as a long term goal but as a way to totally transforming the disease.

PERSONALIZED/PRECISION MEDICINE:
As we look forward, we are looking personalized medicine or precision medicine.

One of the key roles is the maximize the function of CFTR.  This means we take the patients particular mutation, their genotype, and we target therapies that will maximally allow that genotype to function.

We want to combine the best types symptomatic therapies with the best type of genetic therapies (protein-restoration therapies).

Many patients today are going to continue to need symptomatic therapies.  We are going to continue to work on better therapies.

We hope as we bring these new types of therapies to younger patients in children and restore CFTR function, then those individuals will never need any type of symptomatic therapies.  This is our overall goal."

This was a fantastic update.  Here's the bottom line: My heart is overwhelmed with hope.  For most of those with CF, we are SO close!!  And no one is going to be left behind.

The CF Care Center Accreditation Process

Monday, April 6, 2015

I recently had a chance to learn more about the Cystic Fibrosis Foundation's CF Care Center Accreditation Process, the process that makes sure the care that Bennett is getting at his quarterly CF Clinic appointments is at or above standard, as a part of the work I've been doing with the CF Care Model Redesign and during my trip to the CF Foundation's Volunteer Leadership Conference.

I found learning about this CF Care Center Accreditation process to be incredibly valuable to me as a CF caregiver.  The more I know about what goes in to Bennett's care, the better I can be about paying attention to whether or not he's getting the best of care.

I know other CF families feel the same way.  So, I wanted to share my learning with other CF patients and families who might be interested in the nuts and bolts of how a CF Care Center is accredited.

This information came from a presentation by Bruce Marshall, director of clinical affairs of the Cystic Fibrosis Foundation.  Feel free to watch the webinar presentation yourself here: https://vimeo.com/122527397.  I'd love to hear comments at the end of this post as we continue to look for ways to improve it!


So, what is a "CF Care Center" and why it is important?
CF Care Centers are medical clinics within a local hospital partially funded by the CF Foundation that operates multi-disciplinary care for those with Cystic Fibrosis.  There are more than 110 cystic fibrosis care centers and 55 affiliate programs nationwide, including 96 programs for treating adults with CF. (Affiliate programs are smaller CF Care Centers who report to a larger CF Care Center.)

Typically, a CF Care Center includes at least one CF doctor (typically either a pulmonologist or pediatrician with CF training), a nurse, a respiratory therapist, dietitian and a social worker.  Those who work at the CF Care Center are considered part of the CF Care Team.

According to the CF Foundation, "this high quality of specialized care available throughout the [CF Care Center network] has led to the improved length and quality of life for people with CF."

Slide is from presentation from the CF Foundation's Volunteer Leadership Conference 2015.

The History of the CF Care Center Model:
CF Care Center Accreditation started in 1961 with the accreditation of two centers.  This Accreditation took place only 6 years after the Cystic Fibrosis Foundation was formed in 1955.  CF Care Center Accreditation was one of the first initiatives of the Foundation as a way to prolong survival.

In the 1960s, 1970s and 1980s, there was rapid growth of accredited CF Care Centers, mainly in pediatric programs as CF was primarily a pediatric disease.  In the 1980s, however, adult programs really begin to grow.  In the mid to late 90's, adult model programs were evaluated.

In 2000, the CF Foundation began mandating that all CF Care Centers provide adult programs.  These adult programs ranged in a spectrum from CF Care Centers providing adult-oriented CF physicians but sharing the pediatric CF clinic's multidisciplinary team all the way to true CF Adult Care Models, which included a separate and distinct adult CF Team.

In the last year or two, the last pediatric-adult shared programs have separated so that now all adults with CF at every CFF Accredited Center have access to their own adult CF Team.  Now in the US, we now have a mature pediatric and adult care model for all of those with Cystic Fibrosis.

The Accreditation Structure
There are 4 explicit standards that makes a CF Care Center an approved Cystic Fibrosis Foundation CF Care Center.  They are:

Physician Leadership - properly credentialed physicians and physicians experienced in CF care
A multidisciplinary team - such as a respiratory therapist, dietitian, social worker.  Other team members can include a pharmacist, physical therapist, psychologist, chaplain, a patient advocate, etc.
Participation of the CF Clinic to participate in the patient registry, an IRB patient-consented observational study that is used to evaluate much of CF care.
Meeting of clinical teaching and research requirements


The CF Care Center Accreditation CommitteeThese four standards are enforced by a CF Care Center Committee, made up of 9 pediatric and 9 adult CF Care Center Directors, elected representatives from pediatric, adult and affiliate programs. Committee members serve two year, renewable terms.

The CF Care Center Committee's Mission Statement is "to support the CF Foundation Mission by fostering exemplary care of all individuals with CF through: promotion of standards of care, accreditation of care centers, education of providers and advancement of research in all aspects of CF."

The Committee meets twice per year, typically in May and in December for 1.5 to 2 days each time. During these meetings, the Committee evaluates those CF Centers visited during the previous months.  All CF Care Centers are visited by the Committee and are evaluated for accreditation every 3-5 years.



The Accreditation Process
There are two core processes of the Accreditation Process.
They are:
1.) site visits are made by Center Committee members and
2.) annual updates submitted by centers.

The Site Visit Process
When a CF Care Center is visited by the CF Care Center Accreditation Committee, the in-person visit typically last a day or day and a half.  Typically there are two visitors who attend the site visit (usually a pediatric and an adult CF doctor).

During this meeting, the Committee representatives meet with institutional leaders (aka the CF Clinic director, the hospital directors, etc.).  The Committee does a comprehensive assessment of the center.  They look at the clinic, the personnel, the facilities, key labs to CF care (such as the sweat test and microbiology labs).  They do a chart audit and hear report outs of teaching and research, as well as quality improvement.  Sometimes, but not always, the Committee members will meet with the CF Patient Advisory Board.

Once the site visit is complete, the Committee members will get together to share and review their Site Visit findings.  During that meeting, the Committee will go through all centers considered in that 6-month cycle.  A decision is made on accreditation status of each Clinic evaluated.

Accreditation possibilities include:
* Accredit
* Accredit with contingencies (pending immediate action)
* Accredit with provisional status
* Disaccredit

Once a decision is made, critiques based on the site visit are written down and are sent in a letter form back to the CF Care Center, at which time the CF Care Center is notified of their accreditation status.

After revealing the accreditation status of each center, the Committee requests a response from the CF Center.  CF Centers are given a short amount of time to respond via letter regarding the Committee's findings. (aka, if the Committee states the CF Care Center is losing their accreditation, the Committee lists in the letter why the CF Clinic is losing their accreditation.  The CF Clinic then has some time to write a letter back to the Committee stating their challenges and how the Committee can expect the CF Clinic to respond.

Annual Updates
Since not all CF Clinics are visited yearly, there is an important aspect of accreditation that continues. This is done through "Annual updates." Annual updates are sort of like progress reports from the CF Center to the CF Foundation each year.  Questions are asked regarding the CF Clinic's strengths, weaknesses and challenges.

If the CF Foundation sees a problem with one of these annual updates, the institution will be flagged for an early site visit and the Center Committee is notified.  If there are no problems, the CFF will provide accreditation approval and they stay on their 3-5 year cycle of Accreditation.


So, once a CF Center is Accredited, then what?
So, center sites visited in the previous year are eligible for the "Quality Care Award" based on evidence of "sustained quality improvement resulting in improved outcomes.  The CF Foundation provides recognition of the Center/s who receive this award at the NACF Conference. 

Benefits of Accreditation
The benefits of a CF Care Center being accredited includes:
* Accountability - ensures standards are met

* Ideas for improvement - host centers and site visitors benefit from visits to other CF Care Centers
* Securing resources from institution - when a CF Care Center must meet requirements set forth by the accreditation process, this engaged the institution to support the CF Care Center in meeting these requirements for fear that otherwise, the CF Care Center will not longer be accredited (something that the host institution such as University or hospital would not want to see is the CF Care Center brings patients to its facilities)/
* Public recognition of excellent performers - CF Care Centers can be the pride and joy for awards and their institutions.  They can be model for all other centers.

Here's an example of how well CF Care Centers work for CF patients.  The picture below shows data from CF Care Centers with regard to their patient's FEV1 vs BMI percentile.  In 2002, CF Care Centers were scattered in all four quadtrants.  Some were doing amazing in one area or in the other.  Some were doing amazing in both.  Others were not.  But, as CF Care Centers worked to find solutions, they began to figure out better ways of caring for CF patients.  In 2012, the same data shows that most CF Care Centers were amazing in both areas.  This is the power of working together and fighting for a goal of better health outcomes for CF patients:

Current New Initiatives for Care Center Accreditation
The CF Foundation recently indicated there are several new initiatives for Care Center Accreditation.  They include:
* The Patient and Member Experience Care Survey - already 107 (about 40%) of the 265 CF Care Center programs have participated
* Revising accreditation criteria and bolstering struggling centers (such as allowing for independent pediatric and adult programs - as long as transition in place, relaxing the teaching criteria and allowing referrals for clinical trials to count towards research criteria
* Facilitation greater CF Foundation and core center oversight of affiliates

Slide is from presentation from the CF Foundation's Volunteer Leadership Conference 2015.

New Program for Affiliate CF Care Centers
To facilitate great CF Foundation and Core Center oversight of affiliates, the CF Foundation has recently begun a program called the Targeted Assistance Program (TAP).  This program provides:
* data-driven approach to selection (registry data, experience of care survey data, site visit reports and annual updates, staffing levels)
* intervention team comprised of peer clinician
* reporting to center committee
* improvement plan with timetable
* investment of resources of CF Foundation and the institution

In the slide below, you can see how CF Care Centers are doing in their measurement of patients (6-17 years old) with their FEV1.  Most CF Care Centers (represented by black dots) fall within the mean (represented by the gold line).  However, a few CF Care Centers are doing much better than the mean (see the green dots) and some much worse than the mean (see the red dots).  Note that 5 of the 6 red dots are those CF Care Centers who are CF Care Center affiliates.  This is just one of the reasons why the CF Foundation has put in to place the Targeted Assistance Program.  Nobody wants to go to a CF Care Center well below the mean.

Slide is from presentation from the CF Foundation's Volunteer Leadership Conference 2015.
This blogpost packs in a TON of information.  But I wanted to blog about this process for two reasons.  One, I wanted to blog because this information - information about the CF Care Center Accreditation Process is hard to find.  The CF Foundation's website does not list much, if any, of this information.  I wanted to share it with other CF families.  The second reason I wanted to share is because, as a part of my work with the CF Care Center Redesign, I have realized that while there are SO many wonderful processes in place to help our CF patients get and stay well, there are many aspects of CF care that need to be improved. The Accreditation Process is one of those.

Here are the things I'd like to see about the Accreditation Process improved:
1.) I'd like CF patients/caregivers involved in the accrediting our CF Care Centers.  The patient/caregiver voice is vital to improving CF care.  We need structured and integrated input from CF patients/caregivers when choosing to accredit CF Care Centers.

2.) I'd like more transparency about our CF Care Centers accreditation process.  When CF Care Centers lose their accreditation, it is simply taken off the website.  Passive communication about a CF Center's accreditation or lack thereof cannot help CF patients make informed decisions.  As much as I absolutely *loved* learning about the ins and outs of the CF Care Center process, I was disappointed that I had to be involved knee-deep in a project that the CF Foundation is supporting before I could get access to this extremely beneficial information.  I want information like this to be more accessible for all CF patients/caregivers, not just those involved in particular projects with the Foundation.

3.) Not only do I want to know how my CF Center is doing in the accreditation process, I'd like to know how other CF Care Centers are doing with their accreditation. The CF Foundation gives out Quality Care Awards at the NACFC.  But, the problem with NACFC is it's nearly closed to CF patients and caregivers attending.  So, I would like to know who is winning awards, why and how. Knowing my clinic or other clinics are doing well only helps me better understand the kind of care my child might be receiving at their CF Care Center.

3.) I appreciate the current Accreditation Process, but I'd like a Process that pushes my CF Care Center to go beyond minimal standards.  I am incredibly grateful for the process we currently have in place.  The CFF Accreditation Process helps to assure Bennett is at a CF Care Center that, at minimal, is up to CF standards in his care.  But I want more for Bennett and for all those with CF.  At this point, there is no direct way for CF patients/caregivers to know how their CF Care Center is doing compared to others around the country.   I'd like to see the Accreditation Process adopt some type of higher recognition of CF Care Centers that are providing care beyond the minimal accreditation standards.

Easter 2015

Sunday, April 5, 2015

"Hoppy" Easter from our little bunnies to yours!


The Texas Bluebonnets are blooming this week.  So, we decided to make our Easter pictures our Bluebonnet pictures, as well!  

While taking pictures of the children playing among gorgeous wild flowers, I couldn't help but feel God's closeness and recognize his faithfulness.  

Every Spring, God sends us flowers.  Every morning, a sunrise.  (Max Lucado)



“Because children have abounding vitality, because they are in spirit fierce and free, therefore they want things repeated and unchanged. They always say, "Do it again"; and the grown-up person does it again until he is nearly dead. 

For grown-up people are not strong enough to exult in monotony.  
But perhaps God is strong enough to exult in monotony.


"It is possible that God says every morning, "Do it again" to the sun; and every evening, "Do it again" to the moon. It may not be automatic necessity that makes all [bluebonnets] alike; it may be that God makes every [bluebonnet] separately, but has never got tired of making them. It may be that He has the eternal appetite of infancy; for we have sinned and grown old, and our Father is younger than we.” 
-- G. K. Chesteron, Orthodoxy



Oliver, 7 years old; Avonlea, 21 months old; Bennett, 5 years old