How Mentorship Helped Me Through the Process of My Lung Transplant

Note from Ella at CF Roundtable: We wanted to let all of our readers know that the upcoming issue will be all about organ transplants! It is full of information from pre and post transplant experiences by those of us with CF. Personally, I am very much looking forward to reading these stories as I have been just recently told I am in the “window” for transplant. It’s terrifying and joyous and a mixture of everything in between. I also will be signing up for this mentorship program because I want talk to those who have been through it! Thank you to the Lung Transplant Foundation for this perfect blog introduction to our next issue.

By Eirik Gumeny

Receiving a lung transplant can be an isolating experience, and no one should have to go through that alone. Mentorship programs are an excellent way to help.

When my wife and I started our journey towards my double lung transplant in 2014, we found ourselves overwhelmed and slightly adrift. We’d been to the orientations and met with the doctors, but something seemed like it was still missing, something we weren’t quite able to put our fingers on.

In hindsight, the answer was simple: pamphlets and professionals are only one half of the transplant experience. Input from other patients is equally – if not more – important. Transplant patients need to know the technical stuff, the ins and outs, yes, but there are things that only someone who’s actually lived through a transplant can help you with, things that only another person in your same position can understand.

That’s where the Lung Transplant Foundation Mentorship program can help.

They were among the first institutions to implement a mentorship program for lung transplant patients, and they were the first to reach out to my wife and me.

Founded as a non-profit organization in 2009 by a group of lung transplant recipients, The Lung Transplant Foundation (LTF) has been raising funds and acting as an advocate for lung transplant research ever since. Nationally recognized, the LTF is a member of the American Thoracic Society’s PAR Council of Public Representatives, and, currently, the only national lung transplant organization that sits on this prestigious council.

Their Mentorship program provides education and emotional support for transplant recipients and their caregivers, via confidential, individualized, one-on-one support for people at all stages of lung transplantation through personal contact with a trained Mentor.

When it comes to receiving a lung transplant, having someone who intimately understands what you’re going through its crucial to the experience. Though mentors can’t – and shouldn’t – replace professional medical advice, just having someone to talk to, someone who has faced the same problems as you, can help immensely.

The Lung Transplant Foundation can be found online at lungtransplantfoundation.org, and you can find more information on their Mentorship program by clicking here.

An artist who sees on his own terms

By CATHERINE SHAFFER

Detroit artist Brendan Patrick lost his vision, and nearly his life, to cystic fibrosis. Now he’s making the most of the time he’s been given by creating art and raising awareness of his disease.

At first glance, Brendan Patrick’s studio looks like any artist’s studio. It’s full of paints, supplies, and canvases filled with bright colors. But look a little closer, and you’ll see everything is labeled in braille. Brendan is banging a jar of red paint against his desk to get the lid open. He’s wearing paint splattered overalls and dark sunglasses.

He’s showing us his technique: “I dip my paintbrush in some paint. And I just kind of follow the line with my left hand while the brush is in my right hand. And, the key is, hopefully I stay in the lines. We’ll see if that happens. Well, you guys will see if that happens,” Brendan says, laughing.

Brendan lost his vision a decade ago. Before that, he was a tattoo artist. And in his free time he learned to play the guitar and started a band. All while living with a serious illness: cystic fibrosis.

Cystic fibrosis causes progressive damage to the lungs. Brendan was in the hospital repeatedly. By the time he was twenty-six, it seemed like his time was up. In 2008, he spent five months in intensive care waiting for a lung transplant.

“While I was on my ventilator, all I was doing was painting. Twenty-four seven, I probably painted maybe a hundred paintings. I was in the ICU, in my own private room, and it looked like an art gallery,” Brendan says.

Brendan’s lung transplant was a success. But he had a rare complication. He lost his vision.

Rob Cousineau is a longtime friend of Brendan’s, and he’s filming a documentary about him. He remembers the aftermath of Brendan’s devastating loss.

Cousineau says, “Brendan has all of this time now gifted to him with this new lung transplant and he can’t see, so he has nothing to do with the time he has left.”

Brendan’s friends and family stood by helplessly as he spiraled into depression. This went on for about two years. Then a friend suggested he use puffy paint to outline his designs. And that got him painting again.

Something really interesting happened to Brendan’s art after he was blind: a new style emerged on the canvas.

“I can create rooms in my brain. I can create images, and I can kind of paint a person’s face that I’ve never seen…If I’m dipping my paintbrush into yellow, I can see the yellow come onto the canvas. I guess seeing in my own terms now,” says Brendan.

Ten years later, Brendan’s style is basically the same as it was before he was blind. It’s full of bright colors and characters from horror and science fiction. But there’s something different about it now. Like you’re seeing directly into Brendan’s mind. And people love it.

“Some of the stuff he does, it’s ridiculous how good it is for the fact that he’s blind, you can see that he’s blind. The stuff all just works.”

That’s another artist, Evans Tasiopoulos, at a fundraiser for cystic fibrosis featuring Brendan’s art. Dozens of artists are painting blindfolded in Detroit’s Tangent gallery, trying to copy Brendan’s process. They’re doing this for the first time, and it’s mostly going hilariously wrong for them.

Tucked away in a corner, Brendan sits on the floor with his paints and canvases. He says he’s painting a fluffy bunny rabbit. It’s grotesque — and cute. He makes it look easy.

Brendan’s not exactly grateful he lost his vision. But he is having a lot of success because of it.

“It moves some paintings, so that’s positive, right. But, with that being said, my story isn’t really about me dwelling on my misfortunes. It’s about what I’ve done after,” says Brendan.

And what he’s done after is create a tremendous amount of art, and music. Most people think they have plenty of time to accomplish their goals in life. Brendan’s gift is that he has never taken his time for granted.

Original article here.

Nutritional Well-Being After Transplant Measure of Likely Lung Health

By Joana Carvalho

The study, “Impact of nutritional status on pulmonary function after lung transplantation for cystic fibrosis,” was published in the United European Gastroenterology Journal.

CF is the third most common cause for lung transplants worldwide (16.8 percent of all cases). Although the disease is mostly associated with respiratory symptoms, gastrointestinal complications are also known to afflict patients, such as diarrhea, constipation, malnutrition, and inflammation in the pancreas, liver and intestines.

Previous studies suggest that malnutrition is linked to a poor prognosis in those needing a lung transplant. However, data on the impact of nutritional status on pulmonary function in those who have received a transplant is still quite limited.

In a retrospective study, a team of researchers at the Medical University of Vienna set out to evaluate the impact of nutritional status on pulmonary function of CF patients who underwent a double lung transplant within a median of 2.3 years.

Patients’ nutritional status was assessed using two different criteria: body mass index (BMI; kg/m2), and body composition measured by bioelectrical impedance analysis (BIA) — a technique that allows researchers to estimate body composition, especially fat content, by calculating the resistance posed by body tissues to the passage of an electrical current.

Lung health was analyzed by spirometry, a common test based on the amount of air a person can inhale and quickly exhale.

Investigators analyzed a total of 147 spirometries and BIAs performed on 58 CF patients (median age, 30.1), who were divided into four groups depending on their BMI scores. These groups were set according to BMI the guidelines defined by the World Health Organization (WHO), were: malnutrition (less than 18.5 kg/m2), normal weight (18.5–24.9 kg/m2), overweight (25.0–29.9 kg/m2), or obese (more than 30 kg/m2).

Data showed that malnourished patients (27.6%) had a significantly poorer in lung function than those of normal weight (63.8%) or overweight (8.6%), as measured by the percentage of forced expiratory volume in one second (FEV1% predicted, 57% vs 77%), and the percentage of maximum vital capacity (percent predicted, 62% vs 75%).

Investigators also found that lung function measured by FEV1% worsened over time in malnourished patients (decreasing by up to 15%), unlike normal weight and overweight individuals. In these patients, FEV1% remained stable throughout the observation period (median of 10.3 months).

Further analysis also showed that the ratio of extracellular mass (ECM) over body cell mass (BCM), as measured by BIA, accurately predicted lung function over time in CF transplant recipients, suggesting that BIA is superior to BMI in predicting patients’ pulmonary function.

The team concluded “nutritional status assessed by BIA predicted lung function in CF transplant recipients,” and suggested that “BIA represents a non-invasive, safe, fast, mobile, and easy-to-use procedure to evaluate body composition. Thus, it may be used in everyday clinical practice and bears the advantage of repeatability at every patient follow-up.”

The researchers also emphasized the importance of multidisciplinary patient care provided by dietitians and gastroenterologists to try and prevent or diminish malnourishment in CF patients, and so help preserve lung function after a transplant.

Original article here. 

CF Foundation asking for Public Comment on Lung Transplant Referral Guidelines

Dear CF Community,

The CF Foundation seeks your input on the draft of the Lung Transplant Referral for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Consensus Guidelines. This guideline is one in a series of guidelines for advanced stage lung disease; the other guidelines in progress on this topic include: Advanced Lung Disease Consensus Guidelines and Post Lung Transplant Consensus Guidelines.

A committee of CF health care providers, transplant healthcare providers, and an adult 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 for lung transplant referral for individuals with cystic fibrosis.

To systematically collect feedback from the CF community, we have created an on-line tool which is linked below. We recommend that you review the attached document, Lung Transplant Referral for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Consensus Guidelines PDF, and use the link below to enter your comments.

Submit comments here: https://www.surveymonkey.com/r/LTxReferralPublicComment

The deadline for comments is Wednesday, October 10th, 2018, 5:00 PM, Eastern.

We value your input. 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,
Albert Faro

Albert Faro, M.D.
Senior Director of Clinical Affairs
Cystic Fibrosis Foundation | National Office

Bioengineers Are Closer Than Ever To Lab-Grown Lungs

By Robbie Gonzalez

The lungs in Joan Nichols’ lab have been keeping her up at night. Like children, they’re delicate, developing, and in constant need of attention, which is why she and her team at the University of Texas Medical Branch at Galveston’s Lung Lab have spent the last several years taking turns driving to the lab at 1:00 am to check that the bioreactors housing their experimental organs are not leaking, that the nutrient-rich soup supporting the lungs is still flowing, or that the budding sacs of tissues and veins have not succumbed to contamination. That last risk was a persistent source of anxiety: Building a lung requires suspending the thing for weeks on end in warm, wet, fungus friendly conditions—to say nothing of the subtropical climate of Galveston itself. “In this city, mold will grow on people if they sit still long enough,” Nichols says.

But their vigilance has paid off. In 2014, Nichols’ team became the first to bioengineer a human lung. A year later, the researchers implanted a single lab-built lung into a pig—another first. They’ve grown three more pig lungs since, using cells from their intended recipients, and transplanted each of them successfully without the use of immunosuppressive drugs. Taken together, the four porcine procedures, which the researchers describe in this week’s issue of Science Translational Medicine, are a major step toward growing human organs that are built to-order, using a transplant recipient’s own cells.

Bioengineering a lung is a bit like modeling with clay: Like a sculptor uses a wire armature to lend his creation form, Nichols’ team grew the tissues and blood vessels of their lab-grown lungs atop a framework of tough, flexible proteins. The researchers got that scaffolding secondhand, harvesting whole organs from dead pigs and bathing them in a concoction of sugar and detergent to strip them of the cells and blood of their previous owners like a coat of varnish from an old table.

Nichols calls the milky mass that remains the organ’s skeleton: It’s made mostly of collagen, which lends the lung strength, and elastin, which makes it flexible. Each scaffold goes into a bioreactor—one of the containers Nichols and her team built from scratch to house each of the proteinous blobs. The earliest models were little more than spruced-up fish tanks; the latest iterations still incorporate parts purchased from Home Depot.

Its humble origins notwithstanding, each bioreactor plays a vital role. “It lets you provide the organ with growth factors, media, mechanical stimulation,” says pediatric anesthesiologist Joaquin Cortiella, who co-leads the Lung Lab with Nichols. Its job is similar to that of a placenta, allowing the lung to develop in a warm, cozy, nutrient-rich environment for 30 days before it moves to the thoracic cavity of a living, breathing pig, nestled neatly beside the animal’s original lung.

Growing a lung in a bioreactor for a month is a significant accomplishment, says bioengineer Gordana Vunjak-Novakovic, director of the Laboratory for Stem Cells and Tissue Engineering at Columbia University, who was unaffiliated with the study. In an email to WIRED, she said that previous lab-grown lungs have spent a lot less time in culture before being transplanted. The extra time allowed Nichols’ and Cortiella’s bioengineered lungs to grow more blood vessels, the underdevelopment of which “is a major current limitation of lung survival,” said Vunjak-Novakovic. In past studies involving smaller animals, transplant recipients have died within a matter of hours due to fluid accumulation in the lungs. By contrast, the vasculature in Nichols’ and Cortiella’s organs allowed the pigs who received them to survive as long as two months post-transplant without any observable complications.

It’s unclear how the pigs would have fared beyond two months. The four animals in this study were euthanized 10 hours, two weeks, one month, and two months post-surgery, so the researchers could examine how each bioengineered lung had developed inside its recipient following transplantation. All signs pointed to the lungs integrating seamlessly—they continued to develop blood vessels and lung tissues and were colonized by the microbes specific to each animal’s native lung microbiome, all without respiratory symptoms or rejection by the recipient’s immune system.

A big lingering question is how well the bioengineered lungs deliver oxygen. Though each of the pigs had normal amounts of the stuff pumping through their bodies, that could have been the work of the animal’s original lung. The researchers worried the implanted organs were too underdeveloped to risk stopping each research animal from breathing on its original lung, to test the lab-grown one in isolation. That’ll have to wait for future experiments, which Cortiella and Nichols say will involve pigs living for a year or more on their transplanted organs.

Such studies will also require more animals. “It will be interesting to see how robust this technology is, as the number of animals was very low,” said Vunjak-Novakovic. Still, the results are promising. With sufficient funding, Nichols and Cortiella think they could be transplanting bioengineered lungs into humans within the decade.

But first come more experiments—and better, more reliable research facilities. High on Nichols’ wish list is a clean room for the bioreactors, accessible only to researchers clad head-to-toe in bunny suits. She’d like more automated equipment too, which would translate to less manual labor and fewer opportunities for error. And of course, she’s looking forward to the day when she and her colleagues can monitor their lungs remotely via a livestream. Babysitting bioengineered lungs may always be a 24-hour job, but at least with a video monitor the members of the Lung Lab could work remotely.

The Hospital Comfort Kit Is Now Available!

The Hospital Comfort Kit Is Now Available!

When Rebecca Poole was admitted to the hospital in December 2014, she had no idea that she would not be discharged for 219 days. Her husband Ray focused daily on what he could do to make her more comfortable. Friends and family would ask what they could do to help and at the time he didn’t have an Continue reading The Hospital Comfort Kit Is Now Available!

Ex Vivo Lung Perfusion for Transplant

Cystic Fibrosis Podcast 186:
In the latest edition of The Path Forward with Cystic Fibrosis, Dr. Frank D’Ovidio – the Surgical Director of the Lung Transplant Project and Director of the Ex Vivo Lung Perfusion Program at CUMC – explains exactly what the Ex Vivo program is and what its end goals are.
Because so many donor lungs are damaged at the time of death, only 20-30% of donated lungs are usable for transplantation. The ex vivo lung perfusion (EVLP) is a process of evaluating and preparing donor lungs outside the body prior to transplant surgery. In EVLP, the lungs are warmed to normal body temperature, flushed of donor blood, inflammatory cells and potentially harmful biologic factors, and treated with antibiotics and anti-inflammatory agents.
Eventually, as this process is perfected, it could expand the available donor pool by restoring and repairing donor lungs that have sustained damage and eventually create a sort of ‘ICU for organs.’

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

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.

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.

SIX Ways to PAY IT FORWARD to CF ROUNDTABLE!

By Jeanie Hanley, President

Greetings CF Roundtable Subscriber!

May is CF Awareness month. What better way to “Pay It Forward” than by supporting CF Roundtable which has been vital to the CF community! Please consider making a tax-deductible donation today.

This is YOUR CF Roundtable and because of your generosity, YOU have made it possible for nearly 30 years. 100% of your donation goes into the newsletter and many outreach programs. All work is done by volunteers with CF like Andrea, our Executive Editor, whose inspirational words regarding her 18 years of transplant are below:

Eighteen Years of Life Post-Transplant

By Andrea Eisenman, Executive Editor of CF Roundtable

Reflecting back on my life for the last 18 years post-transplant, I am amazed I have lived so long. Way longer than I expected, considering the 50 percent median survival of 5 years after a bilateral lung transplant. I am grateful for this time in which I was able to get married, go back to school for various interests like film and cooking, and care for my mom in her later years, share my life with people I care about and never in recent memory felt this good.

While I have enjoyed a good quality of life, it came with a price of total compliance almost to the point of being neurotic at times (my doctors probably get sick of my calls and emails), a daily exercise regimen and lots of rest. But I found that if I did things I enjoyed like tennis, pickle ball or swimming, it helped get the exercise for that day done while it was fun and social.

I have been extremely fortunate as not only do I have this longevity with transplant and I feel pretty well. Aside from the last 12 months, I have had the ability to travel and do most things my peers do. While I had some setbacks recently, I am starting to feel better. I keep a positive outlook and do what is needed. I can see how precious this gift of life is and I hope that when my time comes to be a donor, the person who gets my organs enjoys them as much as I enjoyed these lungs.

DONATE LIFE!

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