Category: Uncategorized

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

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”

An argument for treating youngsters while they’re healthy

Another terrific video from CFRI (Cystic Fibrosis Research International) with Jeff Wine who presents a must watch session on why its so important to treat youngsters’ infections aggressively even while they’re healthy. Jeff Wine has a CF daughter with a severe mutation (who is currently healthy in her 30s), he himself is a  Professor at Stanford and his presentations are incredibly informative.

Has your youngster  had re-occuring psuedomonas infections?

Are your doctors suggesting your child might have a chronic infection?

Are you doing everything you can to eradicate the infection?

Have you tried absolutely everything?

This is an absolute must watch for any parent facing these kinds of challenges with a young CF patient.

Support your CF Clinic before June 30th 2015

donateAs we are approaching the end of the financial year, we are appealing to individuals and businesses who may want to minimise taxes by making a donation to the Sydney Children’s Cystic Fibrosis clinic. All donations are guaranteed to be spent on resources that will enhance the quality of care for CF children in our clinic.

How you can help

Make a donation – your donation will help us lead the way in paediatric cystic fibrosis care at the Sydney Children’s Hospital.

To claim your tax deductible donation in the 20014-15 financial year, we must receive your donation before June 30th 2015.

How do I donate?

The best way to donate is by phone (02) 9382 1188 (Visa, Mastercard, Amex or Diners) and specify that your donation is to support the Sydney Children’s Hospital Cystic Fibrosis clinic. They will allocate your donation directly to our clinic and you should receive acknowledgement on your receipt that you have donated to the SCH CF Clinic.

Thank you in advance from all of us at Sydney Children’s Hospital Cystic Fibrosis Clinic.

Are donations tax deductible? Yes

Will I receive a receipt for my donation? Yes, it will be sent to you by email when approved and should note that you have a made a donation to the CF clinic.

Lumacaftor / Ivacaftor combo for DF508 > 12yrs

The Pulmonary-Allergy Drugs Advisory Committee of the U.S. Food and Drug Administration (FDA) held a hearing on May 12 to discuss Vertex Pharmaceuticals’ new drug application for approval of the lumacaftor/ivacaftor combination therapy for the treatment of cystic fibrosis in patients age 12 years and older who are homozygous for the F508del mutation.

This is one issue we should be following closely. We’ll keep you updated as soon as we have some news on the outcome of that meeting.

Introducing the Cystic Fibrosis Parents Advisory Council [CFPAC]

As many of you know, we kicked off our first meeting of the Sydney Children’s Hospital Cystic Fibrosis Parents Advisory Council on the 4th of June 2014. The cystic fibrosis parents advisory council (or CFPAC for short) is comprised of a bunch of volunteer parents who are working in partnership with the Sydney Children’s CF medical team to improve outcomes for our children across a range of key areas.

The medical team at Sydney Children’s Hospital recognises that we as parents can play a crucial partnership role in supporting and improving the health outcomes for our kids. We are the ones who are keeping track of every sniffle; we’re monitoring food and nutrient intake, we’re listening to changes in breathing and we’re overseeing physio. We’re the ones who are thinking about our child every day and asking ourselves “What could we do better today?” or “What difference can I make?”.

If you’re interested in joining the Sydney Children’s CF PAC please drop us a line and say hello.

All hellos can be emailed to

The more of us involved the better!

Jen Stumbles

Current Chair CF Parents Advisory Council