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.

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.

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

Pushing Through the Fear – Guest Blog By Andrea Eisenman

By Andrea Eisenman

So many fears, where do I begin. Let’s start with my impending trip to Seattle from NYC. I like to travel but it gets complicated. How much room in my suitcase do I have to pack my myriad of machines and meds? And how much will I forget, despite my thorough list? I learned I had to put obvious things on my list like a hairbrush after I forgot that a few times. But when it is easily purchased at a drug store, no biggy. When it is my immune suppressants or a nebulizer, that is harder to replace.

I now have a lot more machinery to tote around when I leave home. I have my CPAP, my percussor and my inhalation machine and a facial steamer for my sinuses, plus my Neti pot for nasal lavage. These things become cumbersome and traveling light is not an option, I have to check my bag. So, planning is key for several days prior to take off. I am in that phase now. Packing it all. I bring enough meds for twice my travel time. My last trip to Seattle happened during 9/11. I could not fly home for a week. Luckily, I had an extra 10 days of medications to cover me.

My dad asked if I was up to the flight, it is a longer one than I have taken in many years. My answer is, I don’t know. I am fearful as I know I have lymphedema and even though I wear compression tights when I fly, it is less than comfy and I will swell in my upper body. I do have a compression machine for upper body swelling but it is way too big to bring. Will I be ok not using it for a few days? I am hoping the answer is yes. But because I do not know these things for certain, I have anxiety. And I worry I might get sick either from the flight or anytime during my trip. I do wear a mask in flight and try to stay as hydrated as possible in order to keep well. And of course, I will wipe down the area near my seat with cleaning wipes.

But in order to live a life, I have to take some risks. I had wanted to go to Seattle for a few years. It is therapeutic to get away once in a while and I had not traveled too far from home while my mom was alive. I wanted to be near enough if she needed me. I no longer have that worry. And maybe I used that as an excuse so I am now pushing myself to go on this trip. I know I can be resourceful and my doctors are only a phone call away if I get sick. There is a great CF center there and my friend is sensitive to my CF needs. When we were in college together she gave my CPT when I let her.

I find that when I push myself beyond my fears, I feel triumphant and am happy that I conquered them. Sometimes one has to get out of their comfort zone, even if it means wearing horribly tight pantyhose for six hours on a flight! I know it will be worth it and I can bond with my friend. I will feel like I accomplished something worthwhile. Maybe my next trip will be to Europe.

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.

You are invited! CF Transplant MiniCon

Another virtual event for our adult CF community!

About CF MiniCon: Transplant
This virtual event will explore all stages of the transplant process and allow those who are considering a transplant, preparing for transplant, or post-transplant to connect with others, learn more about the process, and share their experiences.

The CF MiniCon will feature a keynote presentation followed by storytelling panel discussions and small group video breakouts.

Check out the agenda at https://cff.swoogo.com/minicontx/agenda and register now, https://cff.swoogo.com/minicontx.

This event is open to adults with CF, their family members, and caregivers age 18 or older.

WEDNESDAY, AUGUST 15
6:30 – 10 p.m. ET | 5:30 – 9 p.m. CT | 4:30 – 8 p.m. MT | 3:30 – 7 p.m. PT

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!

Jerry Cahill’s CF Podcast: The Pre-Transplant Process with Dr. Emily DiMango

The latest video in The Path Forward with Cystic Fibrosis series, Dr. Emily DiMango, Director of the Gunnar Esiason Adult CF Program at Columbia University Medical Center, discusses the lung transplant process through the lens of a CF doctor.

First, she reviews the importance of CF patients participating in drug trials in order to start life-changing medications sooner. She then answers the following questions:

· What does pre-transplant management look like for a CF patient?
· When is the right time to be referred to the list?
· What is the referral process like?

Finally, she reiterates the importance of well-rounded treatment that includes physical health, nutritional health, and emotional health.

This video was originally posted on JerryCahill.com

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.