Five Feet Apart Review: A Movie Embracing the Beautiful Experience of Life Lived with Cystic Fibrosis

By Beth Sufian

I saw the movie Five Feet Apart and LOVED it.  It was an incredible experience. It has a beautiful message of hope and the importance of friendship and accepting yourself and your situation.

Wipe movie reviews and online comments out of your mind. Create your own opinions about the film, but first, allow yourself to feel the incredible experience of sitting in a movie theater, watching a major motion picture with the three main characters who have CF, just like you.

Honor the memory of Claire Wineland who consulted on the movie and made sure the director and actors knew about how she lived her life with CF. The actress in the movie does an incredible job conveying what it is like to live life as a person with CF. I felt the spirit of Claire whenever the character Stella was on the screen. 

The male lead character, Will, reminded me of so many teens I met when hospitalized in the 1990’s. The character is affected in positive ways when he connects with others who have CF. The other character with CF, Poe, expresses his fears and shows how one can have a deep love for a friend. 

I thought I would cry a lot during the movie but actually I was so engrossed in the story I think my brain didn’t want any tears to block my view. At the end when a picture of Claire came up on the screen the tears started to flow. Today I saw the movie a second time with my staff who work tirelessly on the CF Legal Information Hotline.  My staff cried so much that their noses were red and their eyes were puffy. Everyone was deeply moved by the movie.

The depiction of what people with CF endure daily is extremely accurate and the director takes the time to show scenes with the characters doing breathing treatments, using the Vest, accessing their G-tubes, and wearing oxygen.

I thought the characters taking off their masks at different points in the movie was very realistic.  Many people with CF do not want to wear a mask in the hospital or in CF clinic.  This is a reality. Some people with CF follow cross infection rules set by their hospital or CF Center but other do not. I think the movie raises many important questions about the effect of cross infection rules/concerns within our community.  Some people are terrified of people with CF and others find safe ways to be friends like meeting outside or communicating online.

Our CF community is made up of all types of people with varying levels of health.  We need to share the stories of those who have serious health problems and not just stories of people who run marathons and climb mountains.

The movie shows us that a person with CF can be sick and still have a successful life.  In fact, Claire spoke about this often in her videos which can be found online.

Every day I speak to people with CF who call the CF Legal Information Hotline and tell me they think their life will be worthless if they stop working. I tell them their life is valuable regardless of their ability to be employed. Being employed should not be the way we measure success in the CF community. I think those who retire from employment to spend more time taking care of themselves are extremely successful! There are many ways a person with CF can help others in retirement. Join us at CF Roundtable and you can do great things.

In 2019, let us join together and support each other and embrace our differences.

*For those of you who would rather watch Five Feet Apart at home when it is released online but want to watch a CF movie now I suggest watching the Power of Two Movie. It is a documentary about the incredible twins Ana Stenzel and Isabel Stenzel Byrnes.

In CF, Low-dose Antibiotics Aid Bacterial Diversity in Airways, But at a Cost

As published on CF News Today, BY JOSE MARQUES LOPES, PHD

Using lower, or suboptimal, doses of antibiotics to treat lung infections in children and young adults with cystic fibrosis (CF) leads to fewer changes in airway bacterial diversity compared to therapeutic (higher) antibiotic exposure.

The findings also suggested, however, that patients receiving therapeutic doses had greater improvements in lung function.

The research, “Changes in microbiome diversity following beta-lactam antibiotic treatment are associated with therapeutic versus subtherapeutic antibiotic exposure in cystic fibrosis,” was published in the journal Scientific Reports.

People with CF often have recurrent lung infections that gradually worsen their lung function. Treatment of these infections, known as acute pulmonary exacerbations (APEs), is typically based on antibiotics directed at pathogens such as Pseudomonas aeruginosa. However, repeated dosing with antibiotics has been suggested as the cause of decreased microbial diversity in the airways, which, in turn, could worsen lung function.

Despite current guidelines recommending higher antibiotic dosing regimens in people with CF, data from the U.S. indicate that beta-lactams — which include penicillin — were given in doses below the guidelines 38–53% of the time.

Although CF patients often do not achieve therapeutic doses of antibiotics to clear infections, which means that their blood levels of antibiotics do not increase sufficiently for effective treatment, it remains to be determined whether short courses of subtherapeutic doses alter microbial diversity, compared to therapeutic doses.

Aiming to address this question, researchers recruited 20 patients, ages 1-21, who were treated for APEs with intravenous infusion of beta-lactam antibiotics at Washington, D.C.’s Children’s National Health System.

Four samples of respiratory fluid were collected from each patient — when they were experiencing an APE, when they were doing well, right after antibiotic treatment, and at least 30 days later. Genetic testing determined the type and relative abundance of bacteria in each sample.

Blood samples and data on lung function were also collected during antibiotic treatment. Plasma antibiotic levels and bacterial minimum inhibitory concentrations (MICs) were used to determine therapeutic versus subtherapeutic antibiotic exposure.

Of note, to achieve effective bacterial killing, the serum concentration of an antibiotic must be above the MIC of the bacteria for a certain amount of time. Subtherapeutic exposure was thereby defined as insufficient time above MIC.

A total of 31 APEs were reported over the study period, from March 2015 to August 2016, and only approximately 14 (45%) of the antibiotic courses given were considered therapeutic. Most treatment regimens (25 out of 31 antibiotic regimens administered) included a single beta-lactam antibiotic.

Patients in the therapeutic group (11 patients, median age 9) had better lung function, and were less likely to receive inhaled antibiotics at study start, compared with the nine patients (median age 14) receiving subtherapeutic doses — 45% versus 100%, respectively.

At exacerbation onset, people in the therapeutic group showed a greater trend toward having a normal flora, compared to the subtherapeutic group (43% versus 12%), as well as increased abundance of Gemella, and a decrease in unclassified Enterobacteriaceae.

There was no significant difference in the presence of Pseudomonas aeruginosa or Staphylococcus aureus between the therapeutic and subtherapeutic groups.

As for antibiotic use, participants in the therapeutic group received ceftazidime (a more narrow spectrum beta-lactam) more often (86% versus 41%), and less frequently meropenem (a broad spectrum beta-lactam; 21% versus 59%) than those in the subtherapeutic group.

Those in the therapeutic group also had a significantly shorter time from end of treatment to post-recovery — 51 versus 79 days.

At both end of treatment and post-recovery, patients in the therapeutic group had decreased bacterial diversity in their airways, which contrasted with those receiving subtherapeutic doses — who showed minimal changes or higher diversity more than one month after treatment.

Unlike participants in the therapeutic group, who showed increased or decreased relative abundance of specific bacterial genera compared to baseline, those in the subtherapeutic group showed no changes.

“With the subtherapeutic treatment group, this could represent a ‘basement effect’ where it is harder to decrease diversity when it is already low to start,” Andrea Hahn, MD, the study’s lead author, said in a press release.

Also, “patients in the subtherapeutic group had more advanced disease than those in the therapeutic group, which may influence the findings,” Hahn said.

Data further showed that receiving therapeutic antibiotics was associated with a trend toward greater improvement in lung function.

“Thus, the conclusion should not be drawn that because subtherapeutic antibiotics have less impact on changes in microbial diversity, it could be used as a strategy to prevent declining lung function,” the scientists said. “This is likely a reflection of disease severity, antibiotic exposure, and antibiotic resistance.”

The researchers believe that repeated subtherapeutic courses of antibiotics could lower microbial diversity without clearing infections, causing progressive lung dysfunction. Closer monitoring of antibiotic levels in blood to ensure that each exacerbation is treated with therapeutic-level dosing could be an effective answer, Hahn said.

“What this study shows is that levels of the antibiotics we give probably play a role in patients’ ability to recover baseline diversity,” Hahn said. “If we pay more attention to drug levels when using these types of antibiotics to ensure that dosing is sufficient, we could potentially improve patients’ clinical outcomes over time.”

Losing disability insurance is a possible nightmare for those with CF

Original article on CBS This Morning

Megan Willis lives with cystic fibrosis, a deadly disease that causes extensive lung damage. The 22-year-old said she spends around six hours a day administering medications and therapy, and that the disease frequently causes infections and other complications.

With the condition, Willis qualified as a disabled adult for Social Security benefits on living expenses. About 10 million other Americans too disabled for work also get the stipend, called Disability Insurance. More importantly, having Social Security gave Willis access to Medicaid, which paid her annual health care costs of over $100,000.

But in March, Social Security sent her a letter saying her health had improved since the last review of her case and that she was able to work. This was news to Willis.

“My health has only gotten worse in the past year,” Willis told CBS News chief medical correspondent Dr. Jon LaPook.

Despite that, her Social Security benefits were terminated and she lost Medicaid. Since she lives in Florida, one of 14 states without expanded Medicaid, she had no other way to get it. Her family can’t afford private insurance.

While the medical bills mounted, she began law school.

“I don’t want to just stay stagnant and you know, depressed, looking at the four walls of my room,” Willis said. “I want to move up in the world even if you know it’s going to be hard.”

Willis also contacted attorney Beth Sufian, who runs the Cystic Fibrosis Legal Hotline and has cystic fibrosis herself. Sufian said, “We’ve seen a five-times increase in the number of people with cystic fibrosis that have been reviewed in the past 18 months. And we think that Social Security is targeting young people with chronic illness in an effort to reduce the number of people getting benefits.”

By law, disability claims are periodically reviewed to see if recipients are still eligible for benefits. Over the last decade, to combat a backlog, full medical reviews quadrupled to an expected 900,000 this year.      

“It really is a life or death situation for all of our clients when they lose their benefits,” Sufian said.   

In Willis’ case, Social Security ultimately reconsidered and she was able to get back on disability in November. She was hospitalized around Thanksgiving, and Medicaid kicked in and the bills were covered, her mother Wendy said.

But Willis’s lawyer still has about 200 pending cases of people with cystic fibrosis who are first getting reviewed and those who are appealing.

***

Losing disability insurance can have devastating consequences for those with CF. Our very own Vice President, Beth Sufian, helps many individuals get on and stay on disability. For the full story go to https://www.cbsnews.com/…/what-happens-when-someone-loses-…/

Insufficient antibiotics available for cystic fibrosis patients: Study

Turns out, the majority of patients with cystic fibrosis may not achieve blood concentrations of antibiotics sufficiently high enough to effectively fight bacteria responsible for pulmonary exacerbations, thus leading to worsening pulmonary function.

Cystic fibrosis, a genetic condition that affects about 70,000 people worldwide, is characterised by a buildup of thick, sticky mucus in patients’ lungs. There, the mucus traps bacteria, causing patients to develop frequent lung infections that progressively damage these vital organs and impair patients’ ability to breathe.

A recent study led by researchers at Children’s National Health System shows that it’s impossible to predict solely from dosing regimens which patients will achieve therapeutically meaningful antibiotic concentrations in their blood. The findings were published online in the Journal of Pediatric Pharmacology and Therapeutics.

These infections, which cause a host of symptoms collectively known as pulmonary exacerbations, are typically treated with a combination of at least two antibiotics with unique mechanisms. One of these drugs is typically a Beta-lactam antibiotic, a member of a family of antibiotics that includes penicillin derivatives, cephalosporins, monobactams and carbapenems.

Although all antibiotics have a minimum concentration threshold necessary to treat infections, Beta-lactam antibiotics are time-dependent in their bactericidal activity. Their concentrations must exceed a minimum inhibitory concentration for a certain period. However, study’s lead author Andrea Hahn explained that blood concentrations of Beta-lactam antibiotics aren’t typically tracked while patients receive them.

Since antibiotic dosing often doesn’t correlate with cystic fibrosis patients’ clinical outcomes, Dr. Hahn and other researchers examined whether patients actually achieved serum antibiotic concentrations that are therapeutically effective.

In addition, all the patients underwent pulmonary function tests at the start of their exacerbations and about once weekly until their antibiotic therapy ended.

Using the data points, the researchers constructed a model to determine which patients had achieved therapeutic concentrations for the bacteria found in their respiratory secretions. They then correlated these findings with the results of patients’ pulmonary function tests. Just 47 per cent of patients had achieved therapeutic concentrations. Those who achieved significantly high antibiotic exposure had more improvement on their pulmonary function tests compared with patients who didn’t.

Paradoxically, they discovered that although each patient received recommended antibiotic doses, some patients had adequately high serum antibiotic concentrations while others did not.

Another way to ensure patients receive therapeutically meaningful levels of antibiotics is to develop new models that incorporate variables such as age, gender, and creatinine clearance–a measure of kidney function that can be a valuable predictor of metabolism–to predict drug pharmacokinetics.

Using findings from this research, Dr. Hahn adds, Children’s National already has implemented an algorithm using different variables to determine antibiotic dosing for patients treated at the hospital.

Original article here.

Congratulations to Our Scholarship Winners!

The US Adult CF Association (USACFA) is excited to announce our recipients of the Lauren Melissa Kelly Scholarship for the Spring of 2019.

In our evaluation, we look for students who demonstrate tremendous academic achievement, community involvement and a powerful understanding of how having CF matched with these achievements places them in a unique situation to gain leadership roles within the community. Our scholarship is open to all pursuing any degree, from associates to Ph.Ds. We believe that any higher education is a strong foundation for advocacy and involvement in CF.

We are pleased to announce Rebecca Cedillo and Michael Miccioli as the recipients of this semesters’ scholarship. Congratulations to them! They will be awarded $2500 each.

Both of our recipients demonstrated the leadership, intelligence, and drive of Lauren Melissa Kelly. We at USACFA look forward to seeing them further develop their leadership and advocacy in the cystic fibrosis community.

We are excited to announce more scholarship opportunities coming soon! Please stay tuned for more information. For questions, please contact us at scholarships@usacfa.org.

Catastrophizing and Cystic Fibrosis: Fear of Breathlessness Impacts Quality of Life

By Carisa D. Brewster

A new study has found an association between the cognitive process of breathlessness catastrophizing (BC) and poor health-related quality of life (HRQoL) in patients with cystic fibrosis.

Breathlessness is common in patients with cystic fibrosis and does impact HRQoL, even when pulmonary function is in normal ranges. Catastrophizing is a cognitive distortion where irrational thought patterns dominate, and the worst outcome is expected regarding a real or anticipated issue.

Advances in treatment have increased life expectancy for people with cystic fibrosis, but there remains a need to better examine and understand the psychological issues related to quality of life, according to researchers of this study.

“Following in the footsteps of other researchers who have begun to examine breathlessness catastrophizing in respiratory populations, such as those with COPD, we were curious to examine breathlessness catastrophizing among adults living with cystic fibrosis and how it relates to quality of life,” Danijela Maras, MA, lead researcher and doctoral student in clinical psychology at the University of Ottawa, told MD Magazine®.

Participants were recruited from a small observational cohort within the pilot project, “The Ottawa Cystic Fibrosis Treatment Knowledge and Adherence Program”, from May 2011 to June 2013. Patients were excluded if they had received a lung transplant or had an expected survival of less than one year. Final sample size was 45 adults.

The following was assessed for all participants: lung function (FEV1%), depression (Center for Epidemiological Studies Depression Scale), anxiety (7-item Generalized Anxiety Disorder scale), pain (Cystic Fibrosis Symptom Scale, developed by researchers this for study), BC (Breathlessness Catastrophizing Scale, adapted from the Pain Catastrophizing Scale), and HRQoL (Cystic Fibrosis Quality of Life questionnaire).

After controlling for lung function, depression, anxiety, and pain, there was a significant correlation between breathlessness catastrophizing and poor HRQoL (P <.05). In addition, 40% had clinical depression and 13.3% had moderate levels of anxiety.

While results are preliminary and warrant further exploration, Maras said this highlights the importance of assessing and treating mental health difficulties in individuals with cystic fibrosis.

“For example, breathlessness catastrophizing could be targeted in psychological interventions to improve mental health, quality of life, and/or treatment,” she said. “Findings are also relevant for other populations that experience breathlessness, such as those with asthma and other respiratory diseases, neuromuscular conditions, and cancer.”

Maras said that further research should center on longitudinal designs to examine breathlessness catastrophizing in larger and more diverse populations and explore how it plays a role in treatment uptake and adherence.

The study, “Breathlessness catastrophizing relates to poorer quality of life in adults with cystic fibrosis”, was published in the Journal of Cystic Fibrosis.

Click here for original article.

CFReSHC meeting on Aging, Menopause and CF

What is menopause? I’m 30 years old and have heard it groused about for years, but I really don’t know what it is. I have heard from older women with CF that maybe it starts earlier in CF women and that the experience could be different from the non-CF population. I know it has something to do with hormonal changes, more estrogen, maybe? But I have no idea how it will impact my health in the future or what signs and symptoms I should be on the lookout for. On this one, I will follow the girl scouts’ mantra “always be prepared.”

Luckily, CFReSHC is hosting their next Patient Task Force meeting about the menopause experience. It is a great opportunity for any adult woman with CF to learn about what to expect in a few years’ time or to share your wisdom and questions if you’re experiencing or have experienced it.

The meeting will be December 10th from 2-4pm EST.  Laura Mentch, Health Educator, will be sharing her knowledge on the subject.

Please email info@CFReSHC.org, or follow us on facebook for more information.

Recapping the last month of podcasts!

CF Podcast 198: The Art of Healing

In the latest Cystic Fibrosis Podcast, Jerry met with Dylan Mortimer – a 38-year-old artist who lives in New York City with his wife and two sons. As a CF patient, Dylan uses his art to represent his journey with the disease – and to inspire others in their battles to never settle for their diagnoses and keep hope alive past the difficulties they face.
The video podcast was made possible through an unrestricted educational grant from Gilead to the Boomer Esiason Foundation.

CF Podcast 199: Living Life after a Double Lung and Liver Transplant

Jerry Cahill chatted with Kathryn Norris about her journey with cystic fibrosis in his latest podcast. Diagnosed at 3 months, Kathryn soon moved back to her mother’s home-country, Spain, where she had a different experience growing up with CF. Because of socialized healthcare, she had a great deal of access to specific medications, but no access to more recently discovered treatments. In her hometown, walking to and from school helped her fit exercise into her daily life, as well as a number of extracurriculars including tennis, swim, roller blading, and more.
Tune in to learn more about Kathryn – her path with CF to a double lung transplant and a liver transplant, why she is studying to be a personal trainer, and how she copes with her disease.
This podcast was made possible through an unrestricted education grant from the Allergan Foundation to the Boomer Esiason Foundation.

CF Podcast 200: Being a CF Mom

Today’s CF podcast features Megan Neville – a CF mother and caregiver. She shares her story – from learning of her son’s diagnosis to dealing with the guilt of that news to how she now deals with a teenager who has a chronic illness. She reflects on the importance of having an incredible support system of family and friends surrounding her and how raising a CF child can be a team effort.
Tune in to learn more about Megan and her journey as a CF mom.
This video podcast was made possible through an unrestricted educational grant from the Allergan Foundation to the Boomer Esiason Foundation.

CF Podcast 201: Being a Lung Transplant Coordinator

Today’s Cystic Fibrosis Podcast features Nilani Ravichandran, current AVP for Cardiothoracic and Vascular Services at Beth Israel Medical Center, who spent over 17 years as a lung transplant coordinator at NY Presbyterian/Columbia University Medical Center. She sat down with Jerry Cahill to explain what transplant coordinators do, how they work to minimize infection and rejection, and how they teach their patients to care for their new organs. Nilani says that a transplant coordinator’s goal is to give his or her patients the best quality of life possible when they reach the end stages of their diseases.
This video podcast was made possible through an unrestricted educational grant from Chiesi to the Boomer Esiason Foundation.

CF Podcast 202: Being Grateful

With the holiday season approaching, everyone starts to think about why they are grateful. In this video, a number of post-double lung transplant recipients share their reasons for being grateful, how they honor their donors, and more.
Don’t forget – registering to be an organ donor can save a life! Register today: donatelife.org.
This video podcast was made possible through an unrestricted educational grant from Chiesi to the Boomer Esiason Foundation.

As Both Patient and Scientist, I’m Putting Nature’s Medicine to the Test

By Ella Balasa

I peered into one of the incubators that stored my petri dishes for 24 hours, anxious to see whether I would discover discoloration and unevenness on the surface, which would have indicated that my experiment produced favorable results. I wanted to see a visual representation of whether manuka honey kills the stubborn Pseudomonas bacterium, which dwells in nearly half of the lungs affected by CF.

I’m a microbiology lab scientist, plus an inquisitive writer. I also consider myself an informed, self-advocating realist. Life experiences have taught me that I am solely responsible for my health. I strive to keep my health stable through prescribed medications, healthy diet, and some natural supplements.

During my college years, I focused on the environment, especially the living parts that we can’t see but that are essential to the cycle of life — bacteria. It just so happens that certain ones are, understatedly, little pests for people with CF. The lung bacteria of people with CF birth many symptoms and infections.

I continually fight Pseudomonas aeruginosa, my nemesis bacterium that spikes fevers within days of overwhelming my immune system and that has caused countless infections, leaving my lungs with pockets of dead tissue. I take antibiotics frequently, but I also believe that naturally derived compounds can have positive effects. So, despite my disdain and nausea, I sometimes supplement garlic, which contains the antibacterial compound ajoene. I’ve also consumed manuka honey; this I’ve done more religiously, as it tastes more like candy than any “medication.” Manuka honey contains the natural antibiotic methylglyoxal, a compound that fights relentless Pseudomonas by causing its cells to burst and die. I took a spoonful a day for a few years until recently. Maybe I stuck to this exorbitantly priced, palatable remedy merely because of its taste and the flawed logic that expensiveness is indicative of effectivity.

I had the idea to test the effectiveness of the honey on my sputum. My mucus grows many species of bacteria, but Pseudomonas is a primary component, so it’s easy to propagate in the lab setting.

Yes, I took a sputum cup of mucus into work. When inoculating the vials with the bacteria, I was slightly anxious that my lab mates might freak out at the sight of the hazardous and vile-looking green blobs. Then again, they work with wastewater from treatment plants, so it really shouldn’t phase them.

I tested a concentration of 15 percent weight per volume of manuka honey, a choice informed by published studies. I tested half of the petri dishes with honey mixed into the nutrients for the bacteria and the other half without the honey. The dishes with the honey should have less bacterial growth if the treatment works. (If you want more detail on the process, drop a comment below this column.)

The yellow dish has the honey added and the white dish doesn’t. (Photo by Ella Balasa)

After the 24-hour incubation period, I was excited to see the results of science that we as patients typically do not participate in. We provide our sputum samples during doctor’s appointments, then labs perform antibiotic resistance tests, and results are returned as values on a piece of paper indicating resistance or susceptibility. We don’t see the process. I was doing this same research on my own, and in a sense, taking the utmost control of my health.

To continue reading, click here.

We Thank You!!

We sincerely thank each and every one of you who donated to CF Roundtable yesterday on Giving Tuesday.

Your contributions are immeasurable! Your support of our organization means the support of many many individuals with CF to help join us as a community and to help spread awareness and resources.

Thank you!

Happy Holidays!