I’m on the transplant list, now what?

In Jerry Cahill’s latest edition of The Path Forward with Cystic Fibrosis, Dr. Selim Arcasoy from Columbia University Medical Center discusses what happens once a patient is on the transplant list.
The first three major steps are:
  1. Create a strict exercise program with the hospital rehab center and integrate it into the patient’s schedule.
  2. Meet with a nutritionist in order to maintain proper weight.
  3. Educate! Meet with the care team in order to understand the entire process – both pre and post transplant.
The transplant process is a long one – and thoroughly detailed – in order to increase the chances of success. Tune in to learn more from Dr. Arcasoy.

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

Ground-Breaking Procedure. A major step for science, medicine, the human condition

by Mary Bulman; Independent UK

“Woman spends record six days without lungs thanks to ground-breaking procedure”

Yes you’ve read that correctly.
Yes, it reads six days.

A true miracle! Definitely an understatement.

Though it’s been over a year since this procedure was carried out, it’s one that I believe cannot be shared enough. A huge step for medicine and science- but perhaps a larger one for the human condition and the willingness to live and fight.

“I still don’t believe it happened. It seems very surreal.” says patient Melissa Benoit.
And that’s because it is, Ms. Benoit.

After coming down with the flu the last year 2016, Ms. Benoit was taken from her home in Burlington, Canada to the ICU in a nearby hospital located right outside of Toronto, Canada.  Doctor’s made the spilt decision to go through with a first time procedure in order to save her life. After becoming resistant to most antibiotics, bacteria began to move throughout her body, eventually causing her to lapse into septic shock. One by one her organs started shutting down, due to the decline of her blood pressure.

“Although it had never been carried out before, doctors decided to remove her lungs entirely.”

“What helped us is the fact that we knew it was a matter of hours before she would die,” said Dr Shaf Keshavjee, one of three surgeons who operated on her. “That gave us the courage to say — if we’re ever going to save this woman, we’re going to do it now.”

To learn more about Ms. Benoit and the new breed of surgery that was carried out please continue onto the article below:
https://www.independent.co.uk/news/world/americas/woman-six-days-without-lungs-waiting-list-donor-organ-burlington-ontario-melissa-benoit-world-first-a7547936.html

NuvoAir Launches Air Next spirometer– and it uses Bluetooth!

by- Market Insiders, PR Newswire

“The Air Next uses Bluetooth Low Energy, which is a more efficient and cost-effective form of wireless technology, to instantly forward this data from the spirometer to a smartphone or tablet.”

If you’re like me and you very much dislike the extra ten seconds it takes out of your day to write down and journal your spirometry numbers, keep reading. And too, if you’re like me and you forget to bring that journal sheet with you to your doctor to show him your numbers, fear not- you don’t even have to leave your house. Just share it through the cloud. Yes, I know… another cloud.

For those of us who have received a transplant– I believe you know this well. After your surgery you are to use spirometry everyday. Everyday. For a few reasons we are told. To check for rejection, if you’re spirometry numbers are declining. To see, for both personal and medical purposes where you live (what your baseline FEV1 is). Then if you want to brag and show someone. Me: “Look mom, I am taking care of myself. Today I went up 3%.”
It’s very important. My doctors use my home numbers as if I’m doing my PFT’s at their office.
And lastly, this new Air Next looks cool! It’s not like the one hospitals give you that looks like you’re blowing into a 1950’s portal, that’s designed like the inside of a pinball machine. Seriously, check this thing out!

To keep reading visit the article below; also make sure to check out the images:
http://markets.businessinsider.com/news/stocks/nuvoair-launches-air-next-revolutionary-new-home-device-to-help-those-with-serious-lung-conditions-1001941321

Study Links CF Patients’ Airway Bacteria with Disease Outcomes

By: Diogo Pinto

Researchers have linked variations in the mix of microorganisms in cystic fibrosis patients’ airways to their disease outcomes.

The findings in the journal PLOS One were in an article titled “Fluctuations in airway bacterial communities associated with clinical states and disease stages in cystic fibrosis.

CF patients typically have particular strains of bacterial and fungus in their airways. The usual bacteria suspects include PseudomonasAchromobacterBurkholderiaHaemophilusStaphylococcus, and Stenotrophomonas.

Other bacteria and fungi also inhabit CF patients’ airways, however. These include anaerobic species that do not need oxygen to grow and spread.

Not only do the microbial communities in CF patients’ airways vary by type of microorganism, but also in the relative abundance of each species.

Researchers decide to see if the prevalence and relative abundance of typical CF pathogens and anaerobic microorganisms play a role in the severity of patients’ disease and their lung function.

They analyzed 631 sputum samples collected over 10 years from 111 patients.

The team classified the stage of patients’ disease on the basis of their lung function scores. The yardstick they used was forced expiratory volume in one second, or FEV1. They considered an early stage of the disease to be an FEV1 score higher than 70, an intermediate stage a score of 40 to 70, and an advanced stage a score lower than 40.

Researchers classified disease aggressiveness — mild, moderate or severe — on the basis of change in FEV1 relative to age.

They discovered a link between variations in the prevalance of the six typical CF pathogens, plus nine anaerobic species, and changes in a patient’s disease stage and lung function.

To continue reading, click here. 

Antibiotic resistance evolution of Pseudomonas aeruginosain cystic fibrosis patients

By Francesca Lucca, Margherita Guarnieri, Mirco Ros, Giovanni Muffato, Roberto Rigoli, and Liviana Da Dalt

Below is a study hoping to define and answer the questions of Pseudomonas aeruginosain, its evolution and the resistance from different antibiotics. The study took place between 2010-2013. Though the study may have some time clauses I believe there are some strong findings for the CF community moving forward.
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Introduction

Pseudomonas aeruginosa is the predominant pathogen responsible of chronic colonization of the airways in cystic fibrosis (CF) patients. There are few European data about antibiotic susceptibility evolution of P aeruginosa in CF patients.

Objectives

The aim of this study is to evaluate the evolution of antibiotic resistance in the period 2010‐2013 in CF patients chronically colonized by P aeruginosa and to highlight the characteristics of this evolution in patients younger than 20 years.

Methods

Clinical and microbiological data were extracted from two electronic databases and analyzed. Antibiotic resistance was defined according to European Committee of Antimicrobial Susceptibility Testing for levofloxacin, ciprofloxacin, meropenem, amikacin and ceftazidime. The between‐group comparison was drawn with the Chi‐square test for proportions, with the T‐test for unpaired samples for normally distributed data and with Mann‐Whitney test for non‐normally distributed data. Significancy was defined by P < .05.

Results

Fifty‐seven CF patients, including thirteen subjects aged less than 20 years, were enrolled. P.. aeruginosa antibiotic sensitivity decreased significantly for fluoroquinolones, mainly in patients aged <20 years, while it increased for amikacin and colistin. The analysis of minimum inhibitory concentration confirmed these trends. In pediatric patients treated with more than three antibiotic cycles per year, greater resistance was found, except for amikacin and colistin.

Conclusion

An evolution in P aeruginosa antibiotic resistances is observed in the 4‐year period studied. Responsible and informed use of antibiotics is mandatory in CF.
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Read the whole clinical journal here. 

Antibiotic resistance evolution of Pseudomonas aeruginosa in cystic fibrosis patients (2010‐2013) Francesca Lucca,Margherita Guarnieri,Mirco Ros,Giovanna Muffato,Roberto Rigoli,Liviana Da Dalt. First published: 1 April 2018. https://doi.org/10.1111/crj.12787

Lessons From the Road: Avoid the ER

By: Sydna Marshall

A few weeks ago I found myself in the ER at midnight in a new city.  Let me backup the story a bit. I had planned a road trip to Dallas with my bestie (we’ll call her “A” for now) for a beauty conference. Two days before the trip I ran a 102.6 fever for the better part of the day. The morning of, as I was doing my treatments and finishing my packing, I felt the familiar band of pain around my chest. I mentally ruled it out as merely remnants of pain from my blockage earlier in the week and carried on with my morning. I drove the hour north to pick up A and start our trek up to Dallas. We stopped for tacos and queso along the way. I took some pain meds for that persistent and annoying band of pain. A few hours later, we checked into our hotel, picked up our beauty boxes for the conference, and had a happy hour cocktail in the bar followed by sushi in the rotating tower.

Fast forward to 9p: CF changes on a dime. Suddenly, that pesky band of pain that I’ve absently noticed and ignored for the day is front and center. I can barely take a breath, much less a full breath, post-treatment. By 11p, I’ve laid in bed silently crying as the pain spikes up to an 8 and then back to a 6. It dawns on me that the band of pain is pleuritic pain. After texting multiple Cysters and weighing the pain with the inability to breathe, my recent 20% drop in lung functions and the fever I ran earlier, I finally make the decision to wake up A (who is for once sleeping peacefully, without interruptions, in the absence of her four kiddos) and have her drive me to the ER.

We arrive at the ER with this naïve idea that my CF clinic, albeit on-call at this late hour, will communicate with the CF clinic in Dallas. I’d already given A all of the information, phone numbers, and instructions for getting everyone, including my husband Adam, on the same page. Over the course of the evening and early morning hours, hundreds of texts and calls between A, Adam, and the on-call care team at home transpire in an effort to expedite the process.  Since it’s not my first rodeo with pleuritic pain, I’ve already determined before we even got settled in the ER that I desperately need instant-relief pain meds and a chest X-ray. Am I the only one who self-diagnoses? When you’re in and out of the doctor for the litany of health problems in addition to CF, you become the expert on your own body. I digress.

Over the course of the 12 hours in the ER, my port is accessed a total of four times, with one of them being a needle repositioning, before we get anywhere. To administer IV medication and run blood tests, two different nurses start dueling peripheral lines, one in my left hand and the other in my right elbow.  Meanwhile, other nurses attempt to get my port working, which won’t flush or draw back blood. My vein blows on one of the lines, and the other is dangerously close. I have a chest X-ray taken, a CT scan with contrast of my lungs, every blood test imaginable, an EKG, several rounds of morphine, two doses of vancomycin and two albuterol treatments.  I’m told I have a potential pulmonary embolism, a virus causing pleurisy, a mucus plug, or sepsis. Twelve hours in, and about 10 minutes after Adam arrives at the Dallas ER, my repeated requests to be moved to my home clinic, care team, and hospital are heard and I’m care-flighted from Dallas back home (Adam has to drive back home). Once admitted to my home hospital, they have me repeat nearly every test the Dallas ER did less than 24 hours prior as none of my medical records transferred with me from the ER. Five days later the medical records from the ER finally make their way to my home hospital and care team.  In the end, it was determined that I had a virus, which accounted for the difficulty in breathing, pleuritic pain, and fever. It was a very long, traumatic, stressful, and a trying 12 hours away from home. And, I missed my conference entirely, but that’s another story.

I’ve since had some time to reflect on this jaunt to the ER. The biggest takeaway for me – CF clinics do communicate but getting the ER to communicate with the CF care team is nearly impossible.  Having a port is a blessing, but it requires orders from your doctor, not just any doctor, to access and use heparin or cath flow in the event that it’s not working properly (or, in my case, repeatedly accessed incorrectly).  I learned that complaining of chest pain at a new hospital where none of my medical records are accessible means a round of tests to rule out heart problems, despite knowing that it’s my lungs. I learned that transferring medical records from one hospital to another is a royal pain in the you-know-what.

Hindsight is always 20/20, but I know I could have avoided the entire debacle if only I had heeded my inner voice the morning I left for Dallas when I first felt the band of pain around my lungs.  For me, it’s often hard to gauge when it’s important to say no and upend plans, especially when it impacts friends and family around me. If a trip to Walgreens completes a vacation in my house, am I an overachiever for my trip to the ER?

How One Conversation Led Me to Being More Intentional About My Life

By: Ella Balasa

Would I ever live long enough to fall in love? Would I be able to graduate college? Would I be remembered for making some kind of impact on the world before I was gone? Would I get to travel to destinations where the breaking waves crashed against a rocky shore and the sea mist sprayed as I breathed deeply, and beside me stood …

Gabriella-Balasa-Beach-Featured-Rectangle

I’m startled back to reality. I sit in a hospital bed, surrounded by my parents in chairs on either side of me. I’m on the lumpy foam mattress, where I sit cross legged and my butt sinks at least 4 inches straining my back and adding to the pain the past few weeks — and this conversation — have caused me. My dad sits, lips pursed as normal when he listens intently. We are all listening to my doctor talk about my declining health, about my recent episode of pneumonia, and what my future may hold.

“No one knows the future,” I think, as the doctor speaks. My mind jumps again to that ocean scene, only it isn’t me standing on the shore, I’m now observing the scene from above, as if in spirit. Observing a couple embrace and I feel a strange sense of sadness, anger, and jealousy.

“It’s time to consider a lung transplant.” Those words, uttered from my pediatric CF doctor 6 years ago, made me, in an instant, think about all the joys of life I hadn’t gotten to experience yet.

Why me? That’s the first thought many people have when they can’t accept the reality of what’s happening. We try to answer unanswerable questions.

Later that summer, my parents and I followed doctors’ advice and scheduled a week-long transplant evaluation. A week of what I still consider to be grueling medical tests, even compared to other lung complications I have developed since. In the end, the transplant evaluators concluded I was not quite in the transplant window at the time. That fall, my health started to stabilize. I started my second year of college and I felt myself withdraw from the world.

To continue reading, visit CFF community blog.

Home Spirometers: A Useful Tool in Tracking CF Symptoms and Progress

Guest Blog By: Meranda Honaker

Over the last several months my health has continued to decline despite being compliant and diligent with my healthcare routine. In July I developed a fever during a trip to Boston to speak to a biotech company about my journey with cystic fibrosis. I developed a fever and by the end of my visit, I was unable to walk up steps without severe dyspnea. I was so exhausted from feeling sick I would return to my hotel room to lay in the bed for hours to rest. My chest pain and shortness of breath became so severe in the coming days that I could no longer take a deep breath. I checked my SpiroPd home spirometer which displayed a definite decline in my lung function. I immediately contacted my CF clinic to see my CF doctor. Initially, despite feeling bad, I assumed I was feeling poorly and decided not to rush to CF clinic. Sometimes I have a bad day or few bad days health wise and begin to improve on my own. Once I saw my lung function had declined I knew I needed to be seen in CF clinic rather than waiting it out.
Continue reading Home Spirometers: A Useful Tool in Tracking CF Symptoms and Progress

Tell Congress to Oppose the American Health Care Act

The House of Representatives will soon vote on legislation that would jeopardize access to affordable and adequate health care coverage for people with cystic fibrosis.

The bills would effectively eliminate Medicaid expansion and alter Medicaid’s funding structure in a way that would reduce access to care Continue reading Tell Congress to Oppose the American Health Care Act

‘Stunning’ gap: Canadians with cystic fibrosis outlive Americans by a decade

‘Stunning’ gap: Canadians with cystic fibrosis outlive Americans by a decade

Continue reading ‘Stunning’ gap: Canadians with cystic fibrosis outlive Americans by a decade