Medical record folders & pre-clinic prep forms

We haven’t met as a parents group for a while but I’m still getting a lot of requests for the medical records folder we set up some time ago. So much so that I thought I’d post it here again for those of you looking for the information.

This medical record folder is for you and your child. It will help you to take ownership over your child’s medical records so that you can be certain and accurate about your child’s medical history; what’s worked in the past and maybe even some notes of reflection as to why you or the medical team think something worked or didn’t work as well as you’d hoped.

Remember, although your doctors are experts in CF, you are the expert when it comes to how CF manifests in your child. And if you’ve just been diagnosed and you’re not an expert yet, you soon will be. It helps if you can be as descriptive as possible in your notes so you can compare symptoms and treatments from one exacerbation to the next. So try and fill out as much as possible each time. The doctors will keep their own notes but you will get to know your child’s health better than anyone. The best CF care comes when doctors and patients work together as true partners. As you go along this journey you will realise that there is never any one “right” way to treat anything – cystic fibrosis or otherwise.

There are many good opinions and many terrific doctors whose opinions differ on important topics. You will see this if you go to the conferences and read some of the medical literature on the web. The terrific thing about you taking responsibility for your child’s medical records and reflections / symptoms is that you will collect a bunch of insights along the way which are relevant to your child, and that is priceless. Remember the old English saying… “Opinion between good men (or women) is knowledge in the making.”

Keep on top of your child’s medical history and record your own notes & reflections about their experience with CF. Share it with your care team and participate actively in the discussions with your care team about your child’s care.

The notes you write in here and the discussions you have as a result of them, will be knowledge in the making.


You can download the inserts we created here: CF Medical Folder Setup

And don’t forget about the pre-clinic preparation pad to help you make the most of your time with your CF clinicians.


If you have any questions (or feedback), feel free to give me a shout.




VX445 gets the green light for FDA approval

For those of you who didn’t see the news a few weeks ago, On 30th May Vertex Pharmaceuticals  announced that it has selected the triple combination of the next-generation corrector VX-445 (elexacaftor), tezacaftor and ivacaftor to submit for potential global regulatory approvals for people ages 12 and older with cystic fibrosis (CF). Final data announced today from a 24-week Phase 3 study in people with one F508del mutation and one minimal function mutation and from a 4-week Phase 3 study in people with two F508del mutations will form the basis of these submissions. Vertex previously announced that both of these Phase 3 studies met their primary endpoints, and the company today announced the final results of these studies, including results for key secondary endpoints and safety data.

Great news.


Regular colon cancer screening is important

CFRI Community Matters, Summer 2017

By Jordan Dunitz, MD and Joanne Billings MD, MPH — Adult CF Center, University of Minnesota


  1. All CF patients are recommended to have a colonoscopy by age 40 (or 5 years post transplant whichever comes first).
  2. CF patients need a better colon prep (due to stickier stools).
  3. Doctors need to be aware to check the right side of the colon (and check entire thoroughly) as CF patients’ polyps tend to favour the right side.

Everyone who is touched by cystic fibrosis (CF) – including patients, families and care teams – is encouraged by the dramatic improvement in survival that has occurred in the past few decades. Most patients now live to adulthood, and many to middle and even old age. With improved survival, it is important to anticipate new health challenges that CF patients may face with advancing age. One such concern is colon polyps and colon cancer. From the 1960s to 1990s there were a number of individual cases of cancer reported in CF patients. In 1995, an analysis of large databases including CF patients from the US, Canada and Europe revealed an increase in cancers of the gastrointestinal (GI) tract including esophagus, pancreas and intestines. More recently there have been numerous reports of colon cancer in CF patients.

The risk appears to further increase after lung transplantation. In people without CF, periodic colonoscopy is recommended starting at age 50 in order to identify and remove colon polyps. Polyps are thought to be a precursor for colon cancer, and early detection and removal of polyps appears to reduce the risk of developing colon cancer. There is growing evidence that colon polyps and cancer develop earlier in CF patients than the rest of the population. At the University of Minnesota, we have started routine screening colonoscopy in our CF patients starting at age 40. We recently published the results of colonoscopies performed from 2008 – 2015. We had 82 patients who underwent at least one colonoscopy. Half of these patients had at least one polyp. One quarter of the patients had three or more polyps. Three patients had colon cancer. Thirty-two patients underwent repeat colonoscopies one to four years after their first colonoscopy. For those patients who had polyps on their first colonoscopy, 81% had polyps on the repeat procedure.

Half the patients who did not have polyps on their first colonoscopy were found to have polyps on their second colonoscopy. Based on our findings, we recommend that all CF patients who are medically stable undergo periodic colonoscopy starting at age 40. Those patients who have three or more polyps or polyps with advanced histopathology (higher risk of cancer) should have a repeat colonoscopy in one to three years. All other patients should have a repeat colonoscopy in three to five years. For CF patients who have undergone lung transplantation, we recommend the first colonoscopy at age 40 or five years after transplantation, whichever comes first. We recommend that follow-up colonoscopy follows the same guidelines as CF patients who have not undergone transplantation.

While the timing of a colonoscopy is important, the technique is equally important. Prior to a colonoscopy a patient has to complete a “colon prep.” This washes all of the stool out of the colon so the doctor can get a good look at the walls of the colon. Since CF patients tend to have sticky stool, they need a more thorough prep than patients without CF. At the University of Minnesota, we have developed a colon prep that has been quite effective but still tolerable according to our patients (see CF Colon Prep below). If a colonoscopy is recommended and you have CF, please consider reviewing this prep with your doctor. Many of the polyps in CF patients are found in the right side of the colon. It is important that the doctor performing the colonoscopy is aware of this so that your entire colon is thoroughly examined. Our understanding of colon polyps and colon cancers in CF is still evolving. It  is unclear if the increase in polyps and cancer are directly related to the cystic fibrosis transmembrane conductance regulator (CFTR), a difference in the bacteria in the CF colon, or some other cause. More research will be required to better understand why CF patients appear to develop polyps and cancer earlier than those patients without CF. As our understanding improves, recommendations for screening may change.

Instructions: CF colon prep

New Nutritional Guidelines for CF

For those of you interested in the new nutritional guidelines which were launched at this year’s Australian CF conference you can download a copy of them here. [This is the final draft but final copy not yet available online].

You’ll see these new guidelines not only promote a healthier more wholistic diet than previous CF recommendations. Moving away from any fat and sugar to make the calories add up  – to an emphasis on good fats and heart health where possible. The guidelines also touch on vitamins and supplements – namely glutathione, probiotics to name a few emerging areas which have not been formally addressed before.

How to build resilience

One of things we’ve been discussing at the 2017 Cystic Fibrosis Conference here in Melbourne is mental health. We’ve been lucky enough to have Dr Alexandra L Quittner who pioneered TIDES – the first large scale multi-country multi-centre mental health study in cystic fibrosis present to us; and it’s highlighted how mental health really needs to present alongside physiotherapists, dietitians and other care partners as part of a wholistic approach to CF health. Continue reading “How to build resilience”

Vertex reports great results for its latest trials targeting double DF508 and single DF508 combos

Hopefully by now you’ve all seen the fantastic results that Vertex reported last week regarding their latest trials with precision therapy for those with both double DF508 and a single DF508 mutation combo. Vertex shares are up 21% and the reported results are pretty exciting. Although the trials were for patients 18 and older, it sounds like they’re fast tracking the next late stage trials (earmarked for early 2018) so let’s hope pediatric trials follow swiftly. Continue reading “Vertex reports great results for its latest trials targeting double DF508 and single DF508 combos”

Diet Check: Heart Healthy Fats

Heart Healthy Fats [Standford University CF Centre]
Milk, butter, cheese — For most adults living with cystic fibrosis, those
food items are staples in an everyday high-calorie, high-protein diet.
Fat found in dairy products pairs well with most meals, and is excellent
at boosting calories. But don’t be fooled. Dairy is not the only option
available. You’re probably well aware of avocado and trail mix, but what
sets these fats apart from fats found in dairy and butter?

Continue reading “Diet Check: Heart Healthy Fats”