Author Archive

Mar

URMC Pediatric Clinic Status

As our hospitals deal with all the implications of the COVID-19 pandemic, questions arise about the continuity of regular services. The staff of the URMC pediatric CF clinic have developed guidance for the CF community. The letter presenting this guidance was distributed by email and MyChart, but it never hurts use all available channels. You can read the advisory here: COVID-19 patient letter

Mar

Virtual Connection Opportunities

As we deal with the evolving COVID-19 pandemic, the isolation typical experienced by people living with CF is heightened. Two organizations have developed virtual gatherings to connect the CF community with each other - and with relevant information. CFRI Cystic Fibrosis Research, Inc. (CFRI) is offering a weekly COVID-19 support group on Thursdays (beginning 3/26/2020) to help the CF community cope. See details in their flyer CFRI COVID 19 support group. The group will be offered on Zoom and facilitated by Liza Sher, MD, a psychiatrist at Stanford with expertise in CF and lung transplant. To participate, go to: https://zoom.us/j/519241333 CFF Cystic Fibrosis Foundation's Community Voice announced a CF Community Happiness Hour. Here is their announcement: Join us for a virtual Community Happiness Hour on Friday, March 27! Everyone in the CF Community is invited to join us for a virtual CF Community Happiness Hour on Friday, March 27 from 7 - 8 p.m., ET, hosted by the CF Adult Advisory Council. Panel discussions and chat will focus on how we’re finding joy in the here and now, as well as tips and tricks for working from home, parenting, and self-care during these unprecedented times. To register, please click on the button below. Register Here We hope to see you there!   Sincerely, The CF Adult Advisory Council

Mar

CFFC Facebook Posts – March 2020 – Part 1

03/01/2020 - CFTR Amplifiers May Be Potent CF Mono or Combo Therapy, Study Says – Future CF treatments may include a new drug that helps the body produce more of the CFTR protein – either as treatment by itself or in combination with CFTR modulators. 03/02/2020 - Stepping Back as My Daughter Steps Forward – Parenting is tough, but guiding a child with CF into adulthood can be tougher. 03/03/2020 - Coronavirus (COVID-19) and Some of the Ethical Questions Facing the Cystic Fibrosis Community – In this blog post, Gunnar Esiason shows how he is trying to balance the fact-vs-fear atmosphere around the coronavirus outbreak. 03/04/2020 - Coronavirus Q&A – Since it is a major news story, here is more coronavirus information. This time from the UK, so you will have to translate some of the contact information. 03/05/2020 - We Need to Change Our Language When Discussing Mental Health – Tre Larosa takes the brave step of sharing her struggles with anxiety and depression to support her proposal to change the dialogue around this aspect of CF. 03/06/2020 - No One Should Be Left Behind – With all the excitement around the introduction of Trikafta, it is important that we keep our attention on the portion of the CF population that will not be helped by this (and other) CFTR modulators. 03/07/2020 - Research Corner: Translate Bio Recruiting Class 1 and Nonsense Mutation Patients – Yesterday's post dealt with the concern that 10% of the CF population may be left behind in the development of CFTR treatments. Today's article presents the research that holds hope for that portion of our community. 03/08/2020 - Plant-based relatives of cholesterol could give boost to gene therapy – An important step toward effective gene therapy with a dramatic increase in ...

Mar

Clinical Trial Alerts – March 2020

Cystic Fibrosis Foundation issued the following clinical trial alerts in March. March 4, 2020 ABATE: Study to evaluate IV gallium in adults with cystic fibrosis who have nontuberculous mycobacteria (NTM) Status: Enrolling Description: This study is taking place at multiple care centers across the U.S. It will look at the safety and tolerability of IV gallium, a drug intended to treat infections in the lung. Age: 18 Years and Older Mutation: No Mutation Requirement Fev1% Predicted: 25% or greater Number of Visits: 8 Length of Participation: 20 weeks ClinicalTrial.gov link: https://clinicaltrials.gov/ct2/show/NCT04294043 March 6, 2020 Phase 2 study of PTI-428 drug in people with CF ages 18 and older who have two copies of the F508del CFTR mutation Status: Completed with results Description: This study evaluated the safety and tolerability of the drug PTI-428. This study was for people with CF ages 18 and older who have two copies of the F508del CFTR mutation and were already taking tezacaftor/ivacaftor (Symdeko®). A key goal of the study was to determine if PTI-428 could increase the amount of CFTR protein produced in people who were already taking tezacaftor/ivacaftor (Symdeko®). Age: 18 Years and Older Mutation: Two Copies F508del Fev1% Predicted: 40 to 90% Number of Visits: 10 Length of Participation: 74 days ClincalTrials.gov link: https://clinicaltrials.gov/ct2/show/NCT03591094 Phase 1b study of inhaled AZD5634 in adults with CF Status: Completed with results Description: This study evaluated the safety and tolerability of the inhaled drug AZD5634 in people with CF. It also evaluated the effect of AZD5634 on improving the ability to clear mucus from the lungs (lung mucociliary clearance - MCC). Age: 18 Years to 60 Years Mutation: No Mutation Requirement Fev1% Predicted: 40% or greater Number of Visits: 4

Mar

CF Foundation Statement on COVID-19 Preparedness

The following statement was released on March 11, 2020 by Dr. Michael Boyle, Cystic Fibrosis Foundation, President and CEO. We take our responsibility to the CF community seriously. For the past few weeks, the CF Foundation has been closely monitoring the spread of COVID-19 throughout the United States, following guidance from the CDC and local health authorities, and consulting with CF experts to ensure we have the right plans and resources in place to safeguard your health and well-being and that of all people with CF.Nothing is more important.In cities where there has been the highest incidence of community spread of COVID-19, the Foundation has postponed local CF Foundation events and asked staff to work from home. To minimize unnecessary risk, we have suspended work-related travel for all CF Foundation staff, as well as in-person meetings with external parties in Foundation offices, encouraging virtual meetings whenever possible.Out of an abundance of caution, we are now taking the extra measure of postponing or canceling all in-person CF Foundation events scheduled to occur between March 16 and April 12, regardless of location. With the COVID-19 situation evolving quickly, we believe we must act decisively in order to minimize risk for the CF community and do our part to slow the spread of this outbreak across local communities. We are monitoring this closely and will assess future events as the situation evolves, always putting health and safety first.At this time, based on the CDC’s guidance for people who are at higher risk, we also recommend that people with CF not attend any large gathering and stay home as much as possible, though we recognize that decision is up to each individual.We recognize that the facts about COVID-19 are still emerging, and that the uncertainty can be unsettling. We are committed to providing updates to you, your families, and our volunteers as new information is available. Please check cff.org frequently for the latest guidance for those at a higher risk for complications from COVID-19.The Foundation is prepared to take all necessary steps to help safeguard the health of the CF community as we continue to focus on our mission.We appreciate your continued support. Thank you for your patience and commitment as we respond to this evolving ...

Feb

An Evening with Gunnar and Darcy

The CFFC planning committee has added more details this year's education event. We will be spending the evening of June 4, 2020 with Gunnar Esiason and Darcy Cunningham. They will share their perspectives on relevant issues in both the keynote presentation and the discussion groups. To acknowledge Gunnar's concern about infection control, individuals with CF should not attend the event. We are still investigating options to make the presentation and discussions available to those who cannot participate in person. We have designed a program that offers a mix of socializing and information exchange. Here's the agenda: 6:00 PM – Welcome – create or renew acquaintances while enjoying complementary snacks and beverages as well as learning the latest news from our sponsors 6:30 PM– Keynote - Gunnar and Darcy will present a topic that will be announced later 7:30 PM – Break – a second opportunity to enjoy the refreshments and mingle with the attendees and sponsors 8:00 PM– Two concurrent discussion groups Learning to advocate for your care – Gunnar will guide a discussion on building the capacity within the support team to advocate for your needs Navigating the emotion of life with CF – Darcy will lead a discussion on the less-recognized aspects of living with CF 9:00 PM – End of program Our Evening with Gunnar and Darcy will be held at the Pittsford Hilton Garden Inn, 800 Pittsford-Victor Road. The venue is located near the I490 Bushnells Basin exit. We will be creating on-line registration to help us plan food and seating space. Check our calendar entry (An Evening with Gunnar and Darcy) for details and updates.

Feb

CFFC Facebook Posts – February 2020 – Part 2

02/15/2020 - Iron Deficiency Common Among CF Patients and Requires Routine Screening and Guidelines, Study Suggests – This study demonstrates the broad spectrum of effects CF has on body chemistry and the need for comprehensive analysis in determining CF care. 02/16/2020 - My CF family: love in all its forms – A belated Valentine's Day greeting from the other side of the Atlantic. 02/17/2020 - The Limits of Antimicrobial Sensitivity Testing in Cystic Fibrosis – Although a little dense, this article points out the difference in assessing antibiotic strategy between CF individuals and other populations. The researchers concluded that a partnership between pulmonologists and infectious disease doctors will produce the best results for the individual. 02/18/2020 - There's been a lot of talk about the medical benefits of Trikafta but not much about paying for it. CF Roundtable is hosting a webinar that will deal with Trikafta and Social Security benefits. [Webinar was held 02/23/2020.] 02/19/2020 - UK CF Medical Associations statement on coronavirus – There is some good, non-panic advice in this advisory from the UK. 02/21/2020 - Majority of Home Nebulizers Used by CF Children Contaminated with Microorganisms, Leading to Exacerbated Symptoms, Study Finds – Another instance where the headline seems to be common sense. The full results of the study show that all steps in the recommended cleaning protocol must be followed to be effective. 02/22/2020 - Empowering Your Outer Circle to Advocate – This blogger extends the definition of advocacy. 02/23/2020 - Public Comment: Post Lung Transplant CF Care Guidelines – Advances in CF treatments have created the need for additional standards for care. CFF is offering the opportunity to weigh in on post-transplant guidelines. The comment period is open now and runs through March 5th. 02/24/2020 - Noninvasive XV Technology May Lead ...

Feb

Clinical Trial Alert – February 2020

Cystic Fibrosis Foundation issued the following clinical trial alert in February. February 25, 2020 Study to evaluate CB-280 in adults with cystic fibrosis and chronic Pseudomonas aeruginosa Status: Enrolling Description: This study will look at the safety and tolerability of CB-280, an oral drug taken twice a day intended to treat infections in the lung. Multiple doses of the drug will be tested in adults with cystic fibrosis and chronic Pseudomonas aeruginosa to find the best dose. Age: 18 Years and Older Mutation: No Mutation Requirement Fev1% Predicted: 40 to 90% Number of Visits: 6 Length of Participation: 2 months ClinicalTrial.gov link: https://clinicaltrials.gov/ct2/show/NCT04279769

Feb

Public Comment: Post Lung Transplant CF Care Guidelines

Cystic Fibrosis Foundation has just announced the public comment period (February 20-March 5) for the recently developed Post Lung Transplant CF Care Guidelines. Here is their announcement. The CF Foundation seeks your input on the draft of the Cystic Fibrosis Foundation Consensus Statements for the Care of Cystic Fibrosis Lung Transplant Recipients.  A committee of CF and transplant health care providers, two adults with CF, and a spouse of an individual with CF researched and developed the consensus recommendation statements presented in the draft. It is our hope that you will add your input and feedback to ensure that our community issues actionable practice recommendations to guide CF care after transplant. The draft manuscript and recommendations are available for public comment from February 20th - March 5th, 2020 at 5:00 p.m., ET. We recommend that you first review the attached "Cystic Fibrosis Foundation Consensus Statements for the Care of Cystic Fibrosis Lung Transplant Recipients" document, and then use the button below to share your comments. Share Feedback Here Thank you in advance for taking time to review and provide us with feedback on this very important aspect of CF care. If you have any questions, please contact shempstead@cff.org. Sincerely, Erin Tallarico, RN Director, Lung Transplant Initiative  Cystic Fibrosis Foundation Sarah Hempstead, MS Practice Guidelines Manager Cystic Fibrosis Foundation

Feb

CFFC Facebook Posts – February 2020 – Part 1

02/01/2020 - Learning That CF Isn’t My Fault – This blog is an example about how complicated sustaining mental health can be. 02/02/2020 - S. pseudopneumoniae Bacteria Can Cause Pulmonary Exacerbations in CF Patients, Study Finds - You don't really need additional problems to worry about, but identifying a problem is the first step toward solving it. 02/03/2020 - The Harvest: Transitioning to life with Trikafta – We don't want to be repetitive, but it's likely that you can't get too many Trikafta stories. 02/04/2020 - Day 4 – Trikafta Power – Yes, another Trikafta post, but this one is special. It's observations shared by CFFC past-president Kori Tolbert Doherty! 02/05/2020 - CF Roundtable - Winter 2020 – The latest edition of CF Roundtable focuses on insurance issues. 02/06/2020 - Chronic Pain, Depression, and Anxiety in the CF Community – A thoughtful discussion about a subject that needs thoughtful discussion. 02/07/2020 - Research blast on gene editing – In their unique way, UK's Cystic Fibrosis Trust provides a primer on gene editing. 02/08/2020 - My (Almost) Liberation from Recurrent Cystic Fibrosis Clinic Visits – While this starts out like another Trikafta success story, this blog post from Gunnar Esiason takes a thoughtful look at the dual purposes of clinic visits. 02/09/2020 - Research Sets Out to Identify Mechanisms of Dormant, Antibiotic-resistant Bacteria – This research may find the answer to recurring exacerbations. 02/11/2020 - Poor Gut Microbiota in Infants with CF Linked to Stunted Growth in 1st Year of Life, Study Suggests – The information in the headline may seem obvious, but it is helpful when science adds the ...