Defining chronic Pseudomonas aeruginosa infection in cystic fibrosis

By Valerie Waters and Keith Grimwood

Cystic fibrosis (CF) is a genetic, multi-system disease due to mutations in the cystic fibrosis conductance regulator (CFTR) gene, leading to ineffective anion channel activity [1]. The resulting impaired mucociliary clearance permits initial acquisition of Pseudomonas aeruginosa and, if untreated, the establishment of persistent infection in the CF airways. It has long been recognized that chronic infection, often characterized by a mucoid P. aeruginosa phenotype, is associated with more rapid lung function decline and earlier death in individuals with CF [[2], [3], [4]]. Defining chronic P. aeruginosa infection is, therefore, an important step in identifying CF patients most at risk of lung disease progression. Traditionally, the Leed’s criteria has been used to define chronicity (as having >50% of sputum cultures being P. aeruginosa positive in the preceding 12 months), as it is the only clinically validated definition [5]. However, the Leed’s criteria are difficult to implement in young children unable to provide sputum and further limited by the required number of sputum samples and follow-up time [6].

In this issue of the Journal, studies by Heltshe et al. and Boutin et al. aim to re-define what chronic P. aeruginosa infection means in CF. In a retrospective cohort study using data from the US CF Foundation Patient Registry, Heltshe et al. followed close to 6000 early-diagnosed CF children for approximately 6 years [7]. Two-thirds acquired P. aeruginosa infection and of those, 6% had an initial mucoid phenotype. Furthermore, the majority (87%) of children who developed mucoid infection did so before meeting the definition of chronic infection (at least 3 yearly quarters P. aeruginosa positive in the preceding year). Initial P. aeruginosa infection with a mucoid phenotype has been previously described and is a recognized risk factor for failure of antimicrobial eradication therapy [[8], [9], [10]]. Whether this initial acquisition of a mucoid phenotype represents prior adaptation of P. aeruginosa in the CF host (either undetected or transmitted from a patient with chronic infection) or simply infection with an environmental strain particularly well-suited to the CF airways, is as of yet unknown [11]. It is clear, though, that mucoid P. aeruginosa does have an adaptive advantage in early CF infection as mucoidy was associated with an almost three-fold increased risk of transition to chronic infection in this current study. Despite the presence of this risk factor, however, only 13% of P. aeruginosa infected patients went on to develop chronic infection. Although Heltshe et al. did not provide details as to eradication strategies used in this cohort, this low incidence of persistent infection does speak to the overall effectiveness of current antimicrobial treatment for early P. aeruginosa infection.

Boutin et al. took their investigation a step further by using molecular methods, specifically quantitative polymerase chain reaction (qPCR), to define chronic P. aeruginosa infection [12]. In their study, patients with chronic infection had significantly higher levels of P. aeruginosa as measured by qPCR compared to those with intermittent infection. A single P. aeruginosa qPCR measurement in sputum had a sensitivity of 84% (with a specificity of 85%) in detecting chronic infection using a threshold of 103.4 colony forming units (CFU)/ml. A single sputum PCR measure had the advantage of not requiring 12 months of culture results as per the Leed’s criteria [5]. Furthermore, in their small study sample size, PCR was more discriminatory than mucoidy status in predicting chronicity, not surprisingly, given that alginate production (conferring mucoidy) is only one of several virulence factors contributing to the establishment of persistent P. aeruginosa infection in CF [13]. When used in throat swab samples, qPCR had a considerably lower sensitivity (82%) and specificity (56%) in detecting chronic infection, likely due in part to the lower bacterial burden observed in this specimen, compared to sputum. The low specificity of PCR in this setting (positive PCR, negative culture) may reflect the fact that a molecular signal may precede culture positivity. Early detection of P. aeruginosa infection, before culture conversion, in CF patients was originally suggested decades ago using serologic and, more recently, molecular methods [[14], [15], [16]]. Serology, however, has proven disappointing at identifying early P. aeruginosa infection [17]. Nevertheless, early detection may still be possible using highly-sensitive PCR techniques for identifying lower airway P. aeruginosa infection in a young, non-expectorating child. In the study by Boutin et al., P. aeruginosa detection in throat swabs by PCR alone was linked to a positive culture in sputum in three-quarters of cases. Previous studies comparing oropharyngeal cultures to bronchoalveolar lavage (BAL) cultures in children with CF demonstrated that oropharyngeal cultures had a positive predictive value of only 44%, but a negative predictive value of 95% in diagnosing lower airway P. aeruginosa infection [18]. Performing P. aeruginosa qPCR on culture negative throat swabs may further improve the diagnosis of lower airway infection in young children with CF who are unable to produce sputum, but this approach will still need to be validated by comparative studies employing BAL fluid samples. Unfortunately, using confirmatory induced sputum samples as suggested by Boutin et al., may produce unreliable results as these specimens are poor predictors of lower airway pathogens cultured from BAL specimens in young children with CF [19]. Finally, it is yet to be determined whether an earlier diagnosis of P. aeruginosa infection leads to improved eradication success rates and superior clinical outcomes.

In summary, the recent studies by Heltshe et al. and Boutin et al. further our understanding of how chronic P. aeruginosa infection develops in CF and how to better recognize it [7,12]. Ultimately, prevention of chronic P. aeruginosa infection and its deleterious effects on lung function and survival is the goal.

Original article in Journal of Cystic Fibrosis here.

Lessons Learned Through Parenting and CF

Guest Blog By Jeannine Ricci

Back in 2001, when I became a mother, resources on parenting
with CF were practically nonexistent. Thanks to all of the
research advancements, it’s so exciting to now see more and
more people with CF exploring the possibility of parenthood.
Because this topic is becoming more prevalent, I thought it might
be a good time to resurrect an article that I wrote 5 years ago. It
discusses my experience with talking with my children about CF
and the possibility of a shortened life expectancy. Hopefully it will
help other parents as they face this challenging subject.
At the end of the article, I give an update on my daughters
and how their experience with my CF continues to impact their
lives.

***Lessons Learned- Parenting with CF***

My CF has shaped my children’s lives for as long as they can
remember. As toddlers, they developed patience. After many
temper tantrums, they eventually learned that I had to finish my
treatments before we embarked on our day’s adventures. As
preschoolers they learned empathy. They would run to get me
tissues and water if I was having a coughing fit. Their teachers
would comment on the level of empathy they showed their
classmates and how unusual it was at such a young age. During
their school years, they are learning to become more
independent. Just last week, my older daughter surprised me by
making their lunches and helping her sister with her homework
because she knew I was not feeling well.

Since CF had been woven into their everyday lives, I took their
comfort level with my CF for granted. They knew that CF meant
coughing, treatments, and IVs. What they did not know was that
it is a life-threatening disease. I regret having not broached this
subject with them as soon as I felt they were mature enough to
handle this information. Last year my daughter’s teacher called
to tell me that he was concerned about her. She was not herself
at school. She was very distracted and seemed depressed. I
knew immediately what was bothering her. I was on IVs because
of an especially difficult exacerbation. The side effects of the
antibiotics left me on the couch most of the day. It was the
sickest she had ever seen me. We had a long discussion after the
phone call and she opened up to me and told me how she had
read something at the CF walk that stated that the life
expectancy of someone with CF was 37 years old. I was 40. It
truly broke my heart to know that I was not there to answer her
questions and ease her fears when she read this critical piece of
information. She deserved to hear this from me. As I spoke to
her, I tried to give her realistic but hopeful answers. I told her
that I wanted nothing more than to watch her and her sister
grow up and that I hoped to be there for her college graduation,
her wedding, and the birth of her children. I explained that there
are so many new medicines being discovered that would help to
make this a possibility, but I also told her that there are no
guarantees, and that is why I try so hard to keep myself as
healthy as possible.

Just as my children have always incorporated important life
lessons from my illness into their lives, I believe that this
discussion will only further strengthen their character. It will
teach them the value of treating every day as a gift. I hope my
experience encourages other parents with CF to be prepared to
have this pivotal discussion with their children. No matter how
difficult it may seem, it will be worth the peace of mind knowing
that you will be the one delivering this information, ready to help
them cope with their fears.

Here are some guidelines from Lisa C. Greene, a mom of two
children with CF and co-author with Foster Cline, M.D. of the book
Parenting Children with Health Issues (www.PCWHI.com)

• Pivotal parenting moments can take us by surprise, so be
prepared ahead of time. Our answers should be honest, calm,
matter-of-fact, and hopeful. We shouldn’t use terms like “fatal”
or “life-shortening” nor should we make empty promises. Use
terms like “healthier” rather than “healthy,” “more likely to live a
long time” rather than “will live a long time.”

• We need to try our best not to let our own fears and worries
show, both in our words and in our body language. Children pick
up on (and tend to mirror) their parents’ emotional cues,
especially when they are young. If you are having trouble
controlling your own emotions about these tough issues,
counseling might be helpful.

• At some point, we do need to address the issue of life
expectancy. Hopefully, this will be clarified by around the age of
eight (around 3rd grade) depending on the maturity of your
child. One way to address this issue is to ask your child questions
to open up dialogue. Some examples are:
“How much do you know about CF?”
“How are you handling it?”
“Is there anything about CF that worries you?”

With a little awareness and preparation, you can make talking
about these difficult issues a positive experience. Relationships
can grow closer when people go through tough times together.

Update: 5 Years Later
It’s no surprise that this disease has continued to shape my
daughters’ lives over the past 5 years. There’s no denying that my CF, anxiety, and depression have caused many hardships for my family. And it’s
difficult not get swallowed up in the guilt of knowing that there
are times that I’m not able to be the mom that I desire to be, the
mom that they deserve. During these times, I try my best to
focus on the positive ways CF has touched their lives.

The attributes of fortitude and courage were fostered as they
watched my battle with CF progression as it inched its way closer
and closer to the center of my life–our lives–demanding more
attention. And then, in November of 2014, they witnessed hope
being transformed into tangible reality as I swallowed my first
dose of Kalydeco. This new reality has allowed them to more
confidently envision me by their sides in the distant future. They
have embraced this gift with a deep sense of gratitude that can
only be felt when someone has experienced the threat of the
unbearable alternative.

They have watched as this same gratitude has fueled my desire
to help others who are still waiting for their miracle. At the young
ages of 15 and 17, they possess a keen understanding of the
intrinsic value in every life, and that the amount of money in
someone’s bank account or what type of insurance they have
should not be dictating access to these life-saving medications.

They have learned the importance of taking action and
advocating for others, even if your voice is seemingly
overpowered by others. They have both participated in the Cystic
Fibrosis Foundation’s Teen Advocacy Days in Washington D.C.
the last few years, meeting with members of Congress and
stressing the importance of ensuring adequate healthcare
coverage for all. They recognize the gifts both given and received when you touch a person’s life indelibly and both of my daughters have expressed an interest in pursuing a career in the healthcare field. 

I’m so proud of them as I watch them develop into
compassionate, strong, young women, inspired to make their
mark on this world; a mark that undoubtedly would not be so
deep and impactful if it wasn’t for the valuable life lessons they
have gleaned from having someone they love with CF.

Traveling With CF: Plan Ahead, Be Flexible, and Accept Help

By Ella Balasa

Looking up at the rising wall of stone, sweat droplets beading on my forehead, I think about the hundreds of steps between me and the top of the walls of the city of Dubrovnik, Croatia. I want to see the view from the top, but I feel the discomfort of what-ifs welling inside me … what if I hold up the line going up the stairs because I need breaks? What if I pass out from shortness of breath? What if my lung collapses again from taking such heavy breaths with only 25 percent FEV1?

Those were my thoughts last August during my European adventure.

The first six months of 2017 had been difficult. I had three surgeries — each two months apart — on my lung because of a reoccurring lung collapse. I spent weeks in the hospital and then weeks recovering at home. I went from barely walking around my house to building up the strength to walk on the treadmill for 30 minutes a day, only to restart the process each time after the next two surgeries. There were moments I never thought I would get stronger, that I’d be confined to my house with 24/7 supplemental oxygen, chained to an oxygen concentrator that allowed me to breathe.

Slowly I got stronger and — after the third surgery — the lung held. I had been planning this trip since before my medical issues began, and I wanted to make it a reality. I already had to cancel a trip to Vegas for my 25th birthday and a Fourth of July get-together with my best friends. I would be heartbroken if I had to add this trip to that list.

Gabriella-Balasa-Traveling-Quote-Orginal

In the days leading up to my trip, the fear of another lung collapse (pneumothorax) still terrified me. A pneumothorax occurs when air is trapped between your chest wall and your lung. This trapped air pushes on the lung, allowing less room for the lung itself in the chest cavity, thereby collapsing it.

When there is a decrease in air pressure at higher altitudes, air molecules expand, occupying more space. Because of my history of lung collapses, there was a chance that I might have a slight air pocket between my lung and chest wall. If so, the altitude change in an airplane could have expanded this air pocket, making the collapse much larger and dangerous.

Some might think it’s too risky to travel outside the country if you have a chronic illness, where the possibility of needing medical attention is high, and the constant awareness of symptoms and management of medications and treatments are a necessity.

There certainly are times when the risks outweigh the benefits. In my situation, there will always be a risk, but the level of potential pleasure to be gained makes an attempt worthwhile.

Planning for the Trip

Being prepared was important and eliminated some of the anxiety associated with travel. It was also necessary to relax about the parts that were not in my control.

I made sure I had my flight insured and bought travel insurance, and I carried the documents with me. I counted and packed the amount of medications I would need, plus extra.

I did not worry about packing light. I require the amount of luggage of a family of four. In the past, this has embarrassed me. We all stereotype women and their extra bags, but I need: A rolling luggage bag for my vest, a roller for my oxygen concentrator, my suitcase of clothes and personal products, and a carry-on backpack of medications. I do not check any of my nebulizing medications and machine, inhalers, enzymes, and antibiotics in case my suitcase gets lost. These are the items I have to have, and it would be a nightmare tracking them down in a foreign country.

I opted for special services through the airline for assistance with getting from one gate to the next between flights and to help carry heavy bags. Having 25 percent lung function, it’s tiresome to walk distances, and it’s not possible for me to carry anything remotely heavy. This was the first time I had used this service. I’ve never liked being seen as different or needing special accommodations. However, I have realized, as my disease progresses, that doing everything everyone else does is not always possible, and it’s OK.

And, it turned out to be a wise decision. As I got off one of my flights, I was met by an airline employee with a wheelchair and a sign with my name. I had 20 minutes before my next flight was to depart JFK airport in New York, and my gate was at the other end of the terminal. With only 10 minutes to go, this gentleman started running as he wheeled me through the airport. By the time we got to the gate, he was profusely sweating. I was the last one to board! I would have missed my flight without this assistance.

What I Learned

First, I learned to be comfortable with strangers seeing me doing CF-related stuff, like wearing a mask and using an oxygen concentrator on an airplane, and doing a breathing treatment on a park bench, while coughing and spitting into tissues. Here is a picture of me doing exactly that in Split, Croatia.

Gabriella-Balasa-Traveling-Nebulizer-Featured-Rectangle
To continue reading this article, please visit the CF Foundation Blog.

CF Patients, Especially Post-transplant, at High Risk of Gastrointestinal Cancers, Study Finds

By Patricia Inacio

Patients with cystic fibrosis (CF) are at higher-than-average risk of developing gastrointestinal cancers, especially those who underwent a lung transplant, a new study shows.

Transplant patients with CF were found in this retrospective study to have a five-times greater risk of gastrointestinal cancer than those who have not had a transplant, the researchers found, emphasizing a need for careful screening for small intestine and colon cancers particularly, but also for biliary tract and pancreatic cancers.

The study, “Risk of gastrointestinal cancers in patients with cystic fibrosis: a systematic review and meta-analysis,” was published in the journal The Lancet Oncology.

More effective therapies developed in the last 30 years has significantly improved life expectancy for CF patients, with 70 percent of all patients in developed countries expected to reach adulthood. Treatments now in use range from pancreatic enzymes, to antibiotics targeting the bacteria Pseudomonas (a major cause of CF lung infections), to lung transplant surgeries.

But improvements in life expectancy — prior to 1980, most CF patients died in infancy — has brought an awareness of comorbidities in this population. Reports of cancers in CF patients have also risen since 1980, the study notes.

An international team of researchers conducted a systematic review and meta-analysis of studies published in six different databases — PubMed, Medline, Google Scholar, Scopus, Embase, and Cochrane — to investigate the incidence of CF and gastrointestinal cancers. Additional sources included scientific meetings and studies mentioned in the bibliographies of selected studies.

Their search identified six cohort studies including a total of 99,925 CF patients with a gastrointestinal cancer diagnosis. The studies included those who had undergone a lung transplant and those who had not.

Results showed a significantly higher risk of gastrointestinal cancer in CF patients than in the general population, including site-specific cancers — namely, those of the small intestine, colon, biliary tract (the tubes transporting bile produced by the liver into the small intestine), and the pancreas.

In lung transplant patients, the overall risk a gastrointestinal cancer was five times higher than those without a transplant.

Compared to the public-at-large, all CF patients had 20 times higher risk of cancer of the small intestine, and 10 times higher for colon cancer.

“Our results support the CF Foundation Task Force recommendation to initiate colon cancer screening at age 40 years, with repeat screening every 5 years thereafter and 3-year surveillance intervals,” the researchers wrote, recommending the small intestine also be examined in the colonoscopy given.

They also proposed a screening strategy for biliary tract and pancreatic cancers with specific imaging techniques, and a blood test for a cancer protein (antigen 19-9) to be performed every two-to-three years for 40-year-old patients who have not had a transplant, and every one-to-two years who those who have.

For original article please visit CF News Today. 

26 Years and Counting with CF

By Ella Balasa

The day I was born, the median life expectancy of someone living with cystic fibrosis was 31. Although I haven’t reached that median yet, I feel like I’ve beaten the odds.

During past birthdays, my parents, brother, and I celebrated with cakes filled with raspberry layers and chocolate frosting. The cake always had my name written across the top in big, pink, block letters, and the number of candles matched the number of years lived. I remember my dad’s voice quivering just slightly by the time he sang the last “Happy Birthday” lyric. I think that he sheds an extra tear of joy, metaphorically, for each year I get older. He’s happier than the year before, that I’m one year closer to living the long life he hopes and prays his little girl would have.

I know my parents have always had a seed of heartache that they’ve kept hidden far in the back of their thoughts, watered by the knowledge that they may outlive their youngest daughter. It’s a feeling unknown to me; I can only imagine the fear.

I realize that my disease continues to progress with each passing year, causing a gradual decline in the intensity of accomplishable physical activities. My birthday is somewhat of a grim reminder of what I’ve lost over time. It’s marked by at least one less thing I can do.

Toward the end of my high school years, my brother was my exercise coach. He was always encouraging (sometimes nagging) me to do frog hops down the driveway and sprints from the mailbox to the stop sign on the corner. I also was running about 1 mile, or half of one, in my neighborhood on the days I felt extra motivated. One early summer day, at the end of my loop, about half a block before I reached the stop sign on my corner, I felt the urge to cough. When I got to the corner, I started coughing globs of pure blood while bracing myself against the sign. It was one of the last times I ran. That was the year I turned 18.

When I turned 21, I stopped working out at a gym and instead got a treadmill and weights at home because I had started to require supplemental oxygen while exercising. Without the extra oxygen, my blood oxygenation levels would dip into a range that could cause damage to my heart. My lungs began failing at the job they are required to do: supply oxygen from the air into my blood vessels and to the rest of my organs.

I was using a nasal cannula and carrying around a machine that puffed loudly with every breath, but I couldn’t allow people to see me as abnormal. I still have a hard time being in public with the supplemental oxygen, and although I don’t yet require using it constantly, it’s caused my illness to become visible rather than invisible, as it typically was — and I struggle with that.

Last year, when I was 25, I learned what it feels like to do a 500-pound deadlift. Except I wasn’t in a competition. I was bringing just two bags of groceries into my house from my car less than 50 feet away. During infection exacerbations in my lungs, I am unable to walk at a normal pace, much less carry anything, due to my airways feeling like they are the diameter of a toothpick, and the lack of oxygen my body is receiving. During these times, I feel my body needing the extra oxygen that I sometimes deprive it of because of my unwillingness to show the signs of my disease.

Based on this column thus far, it might seem as though I lament on the difficulties. Honestly, I don’t notice much when my breathing becomes less limited. It’s easier to notice when my breath is restricted and I feel my body producing less, functioning less.

Despite these reflections on my inabilities, I don’t remember my birthdays for all the things I couldn’t do in that year. I do remember everything I could and did do, both on that day and the 364 days in between. For my 10th birthday, I remember having a picnic in the park and running around the playgrounds playing hide-and-seek. For my 21st, I remember going to a local bar, Baja Bean, and getting the coveted sombrero so everyone would know I was celebrating my big day. For my most recent, the 26th, I rode in a small seaplane over the city, then landed into the river.

Birthdays have always been, and always will be, a celebration of my life. It’s the progression over time, despite my best efforts to stay as healthy as I possibly can, that I’ve found to be somewhat discouraging at times.

When I blow out my 27 candles next year, there will certainly be a diminishment in my physical abilities. But I won’t be dwelling on it. I’ll be thinking about all of the new things I did, the places I went, and the people I met.

To read the original article, please click here.

CF Foundation ‘Venture Philanthropy’ Model Crucial to CF Breakthroughs

By Larry Luxner

When the Cystic Fibrosis Foundation (CFF) was established in 1955, most people with cystic fibrosis (CF) didn’t make it to their sixth birthday. Today, the average life expectancy of a CF patient is 47 years.

To date, the U.S. Food and Drug Administration has approved 12 CF therapies. Three of them are CFTR modulators that treat the basic disease-causing defect, benefiting 60 percent of all patients, and more therapies are on the way.

Preston W. Campbell III, the CFF’s president and CEO, directly attributes this dramatic improvement to the foundation’s philosophy of “venture philanthropy.”

“We are now in Phase 3 CFTR trials that, if successful, will mean that as early as next year, more than 90 percent of all individuals with CF will have a highly effective therapy targeting CF’s basic defect,” he said. “More therapies that treat the complications of CF are in the pipeline than ever before.

“It begs the question: how did all of this happen?”

Campbell answered that during his March 26 presentation, “Patient advocates taking a real stand in drug development: How the CFF worked with biotech and pharma to find a cure,” at the 2018 World Orphan Drug Congress USA in Oxon Hill, Maryland.

Back in 1960, the Bethesda, Maryland-based foundation broke ground by establishing a Care Center Network to provide multidisciplinary care. Within five more years, it had formed a patient registry.

With only $400,000 in the bank, it would also commit $11 million to research, Campbell said. “Five years later, in 1985, the basic CF defect was identified, and in 1989, the CFTR gene was discovered. That opened the floodgates,” he added.

Campbell’s predecessor, Robert J. Beall, created the Therapeutics Development Program — now called its Venture Philanthropy Model — in 1998 to entice industry to focus on CF, and specifically on CFTR as a target. Its three components were financial assistance, research tools and scientific advice, and a clinical trials network.

“We would lower the risk for industry to come into the CF space. We also made our research tools and scientific advice freely available, and we also embedded the best scientists in the world in these industry programs,” said Campbell, who took over from Beall as head of the CFF in January 2016. “Finally, in order to make sure clinical trials were safely and efficiently done, we created a clinical trials network that originally had seven centers and now has 89.”

In the beginning, CFF’s investments were typically in the $1.5 million range. Ultimately, the foundation invested more than $100 million in Aurora and its successor, Vertex Pharmaceuticals, whose headquarters are in Boston.

To date, the FDA has approved three Vertex CFTR modulators: Kalydeco (ivacaftor) for patients with the G551D mutation in the CFTR gene (2012); Orkambi (lumacaftor/ivacaftor)for patients who are homozygous for F508del, the most common mutation in the CFTR gene (2015); and Symdeko (tezacaftor/ivacaftor) for homozygous F508del patients as well as others (2018).

“Payments are milestone-based, so we pay for success,” Campbell said. “A scientific advisory committee determines if milestones are met and if the project should continue. Successful programs offer a return on our investment, so if the program is foundering, we shake hands and walk away.”

To continue to full article, please click here.

‘Sham’ or public interest? ICER suggests 70%-plus discounts on Vertex’s cystic fibrosis drugs

By Angus Liu

Vertex has often talked about its admiration for Gilead, setting the big biotech’s ability to roll out multiple antivirals as a model for its cystic fibrosis endeavor. Now, though, it faces the same pricing issue once pinned on Gilead’s hepatitis C franchise. But the company refuses to play sitting duck.

In a new report, the U.S. cost-effectiveness watchdog the Institute for Clinical and Economic Review argues that Vertex’s cystic fibrosis trio—Kalydeco, Orkambi and newly approved Symdeko—need a huge price cut to achieve cost-effectiveness. Its suggested discount? Over 70%. Continue reading ‘Sham’ or public interest? ICER suggests 70%-plus discounts on Vertex’s cystic fibrosis drugs

You have a new set of lungs! What should you expect next?

Cystic Fibrosis Podcast 183:
The Path Forward with Cystic Fibrosis
By Jerry Cahill
In the latest edition of The Path Forward with Cystic Fibrosis, Dr. Arcasoy from Columbia University Medical Center is back to explain what happens after a patient has a double lung transplant. He discusses pain management and the post-transplant care team in detail.
Here’s what to expect immediately pre and post-surgery:
  • Post-surgical care including pain management
  • Medical care that includes antibiotics, antirejection medication, and anti-infection medication
  • Psycho-social recovery assistance
Dr. Arcasoy also explains who your post-transplant care team is and what they do… it’s a lot, so here’s a cheat sheet:
WHO: Medical Transplant Pulmonologist and the Coordinator
WHAT:
Patients will meet with their Post-transplant team once a week for three months, then every 3-4 weeks for a year. At every meeting, the following occurs:
  • Chest x-ray
  • Lab work
  • Pulmonary function test
  • Physical exam
  • Conversation to review medications and overall health & wellness
  • Follow up lab review and medication changes
The schedule for bronchoscopies vary depending on the center, and additional testing can be added at any time deemed necessary.
Remember – every patient’s experience is completely unique! Do not get discouraged; and work with your care team to prepare both mentally and physically for the bumps along the way.

This video podcast was made possible through an unrestricted educational grant from Columbia University Medical Center and the Lung Transplant Project.

Lauren Melissa Kelly Academic Scholarship Due June 30th

CF Roundtable offers the Lauren Melissa Kelly (LMK) Scholarship award each semester, in honor of Lauren Melissa Kelly. The academic scholarships of up to $2500 are awarded to adults with cystic fibrosis who are pursuing career certifications, associates, and bachelor and graduate degrees.

Nancy Wech, Lauren’s mother, established this scholarship in Lauren’s name and memory. In Nancy’s own words:

Lauren Melissa Kelly was an extraordinary woman. Laughing, gregarious, spontaneous, fun, talkative, driven, thoughtful, smart, kind and loving — all descriptive terms for Lauren, who lost her battle with CF late in her senior year at the University of Georgia. In 1992, Lauren was chosen as one of ten Senior Leaders at University of Georgia. She had distinguished herself as a member of the Golden Key Honor Society, Mortar Board, Phi Upsilon Omicron, Gamma Beta Phi, the Tate Society and Delta Gamma sorority.

Lauren felt the most significant opportunities of her college career included participation in the reconstruction and formation of organizations, which will serve the university in the future. She acted as one of the re-founding members of the Phi Kappa Literary Society and was significant in the metamorphosis of the Z Club into the William Tate Society. Her other activities included Greeks Advocating Mature Management of Alcohol (GAMMA) in which she served as Secretary and Special Events Chair. She was also a member of the Women’s Glee Club for more than two years.

In recognition of her academic performance, Lauren’s degree of Bachelor of Science in Family and Consumer Sciences was awarded posthumously. At the time of her death, Lauren was engaged to be married and living off campus in an apartment. She lived life to the fullest!

Walt Disney said, “Don’t cry because it’s over, smile because it happened. It’s not the days in life you remember, it’s the moments.” As Lauren’s mother stated, “I smile because she happened to me. Now, I want you to smile because she has happened to you.”

Please visit our website for application and full scholarship criteria and details. http://www.cfroundtable.com/community-outreach/united-states-adult-cystic-fibrosis-association-scholarship/

Send any questions to scholarships@usacfa.org.

 

You got the call for transplant… Now what happens?

Cystic Fibrosis Podcast 182:
The Path Forward with Cystic Fibrosis
In Jerry Cahill’s latest edition of The Path Forward with Cystic Fibrosis, Dr. D’Ovidio and Dr. Arcasoy from Columbia University Medical Center explain what happens once a patient receives the official phone call for his or her transplant.
They explain dry runs, the transplant surgery, a patient’s first breath, and more! Keep in mind; the overall transplant experience varies greatly among patients, as each case is completely unique.
This video podcast was made possible through an unrestricted educational grant from Columbia University Medical Center and the Lung Transplant Project.