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.

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.

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. 

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!

Please consider Paying It Forward in these six ways:

 

  • Unrestricted Gifts – your contribution will go to the program that needs it most.
  • Milestone Celebration: for a transplant anniversary, birth of a child, wedding, or a birthday. There is no greater reward than celebrating YOU and YOUR accomplishments.
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  • USACFA Endowment Fund – consider contributing which will get CF Roundtable closer to be self-sustaining forever! Please contact us if you are able to contribute.
  • Matching Gifts – if your employer has this program, then let us know!
  • Bequest – A simple and easy way to remember CF Roundtable in your estate planning.  To establish a bequest, please contact us.

 

To make a donation, click here DONATE NOW!

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USACFA proudly publishes CF Roundtable and all its associated programs; USACFA is a 501(c)(3) nonprofit organization. All donations are tax-deductible.

Thank you!

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.