Aid Program for Lung Transplant Recipients

Some info on a financial aid program set up to help people who are having to receive a lung transplant. The program is through the Boomer Esiason Foundation

From The Boomer Esiason Foundation's Website: Link

BEF Lung Transplant Grant Program
While the cost of transplantation is typically covered by most insurance companies, travel and relocation costs are typically absorbed by the patient’s families. The BEF Lung Transplant Grants Program is designed to help families cover the expenses that are not covered by their insurance.

Possible expenses to the recipient and family include, but are not limited to: Patient and family transportation costs for evaluation, surgery, and clinic visits after transplant; and housing, food, and living expenses associated with relocation to the transplant site.

Candidate Evaluation:
Applicants need to provide a letter from a social worker stating their needs as well as a detailed cost breakdown from the family specifying where the funds would be allocated. All grant requests are reviewed by a committee that includes: Jerry Cahill, Volunteer Director of Education and Team Boomer; Dave Rimington, President; and Boomer Esiason, Chairman. Grant recipients are required to provide a copy of official receipts to the Foundation for all expenses that are covered by the grant. All donations made by BEF are made to the CF clinics directly, which closely monitor the grant recipients’ expenses.

Call 646-292-7937 if you have question about the program or would like to make a donation.

FDA approval needed for CF drug

I would like to encourage all of you to please copy and send the letter below to the FDA at druginfo@fda.hhs.gov

The CF community needs to get this drug approved by the FDA. We need your help to be heard loud and clear. Please sign your letter and include your city and state then ask all your family and friends to do the same.

If you are interested you can read the press release from Gilead the drug maker at www.gilead.com/pr_13646662

Thank you,
Lori
Co-Founder of the Living Breath Foundation

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I am writing to you today about a product that is attempting to get FDA approval, Aztreonam Lysine for Inhalation. The product is manufactured by Gilead and will serve the purpose of fighting against Pseudomonas Aeruginosa infections in the airways of Cystic Fibrosis patients. It has already been approved in both Europe & Australia under the name Cayston(R) and the US has been lagging behind in its approval. Our Cystic Fibrosis patients with Pseudomonas Aeruginosa are running out of antibiotic options to help fight infections in their lungs. Effectively treating infections in patients with CF is very challenging, and new treatment options are urgently needed. This medicine offers some new hope in their battle to maintain the health of their lungs. About 60% of people with Cystic Fibrosis have a chronic respiratory infection caused by the bacteria called Pseudomonas Aeruginosa that settles into the thick mucus trapped in the airways. Once it sets up house in the respiratory tract, Pseudomonas Aeruginosa is almost impossible to get rid of. Respiratory failure caused by the infection is often the ultimate cause of death in many people suffering from Cystic Fibrosis. The FDA has established a target review date, under the Prescription Drug User Fee Act (PDUFA), of February 13, 2010. I would like to urge you to please grant this medicine the go ahead for approval. The FDA Advisory Committee vote 15-2 in favor of this medicine and 17-2 on the correct dose and regimen.

UPDATE 2-US FDA panel says Gilead drug aids cystic fibrosis

An Interesting Article on Aztreonam: http://www.reuters.com/article/idUSN1023955020091210

From Reuters.com
By Jackie Frank

GAITHERSBURG, Md., Dec 10 (Reuters) - A U.S. Food and Drug Administration advisory panel on Thursday said it found Gilead Sciences Inc's (GILD.O) aztreonam was an effective new treatment for life-threatening lung infections in cystic fibrosis patients.

The drug won marketing approval in September in Europe and Canada under the brand name Cayston.

The anti-infective drugs panel's finding on a vote of 15-2 that the drug is safe and effective serves as a recommendation that the FDA approve the drug. While the agency is not required to follow an advisory panel's recommendation, the panel's opinion carries great weight.

The panel said the safety and efficacy involved a 75 milligram dose administered three times a day to aid in improvement of respiratory symptoms and lung function.

Azteronam is designed to treat lung infections in these patients caused by common pseudomonas aeruginosa bacteria, for which there are few inhaled antibiotics available.

Dr. Bruce Marshall of the Cystic Fibrosis Foundation urged FDA to approve the drug swiftly and told the panel: "There is a desperate need for additional inhaled antibiotics."

Drug resistance is a complication in treatment for these patients.

Scientists restore some function to cells from cystic fibrosis patients

In an encouraging new development, a team led by Scripps Research Institute scientists has restored partial function to lung cells collected from patients with cystic fibrosis. While there is still much work to be done before the therapy can be tested in humans, the discovery opens the door to a new class of therapies for this and a host of other chronic diseases.

Scientists restore some function to cells from cystic fibrosis patients

Proper use of Nebulizers and Inhalers

I found a short course through Pari about the proper use of nebulizers and inhalers. It is actually for clinicians, but I think it is very informative for all people using these devices. It takes about 30 minutes, you listen and answer a few questions before moving on to the next chapter. Well worth your time and a good refresher of information.

http://www.pari.com/ceu/pgm1/program/player.html?agree=agree_terms&Start+Program=Start+Program

Some Info on PFT's

I recently came across this explanation of what exactly they are testing for during PFTs. It is pretty interesting reading if you don't know it already!

From Web MD: http://www.webmd.com/lung/lung-function-tests

Lung function tests (also called pulmonary function tests, or PFTs) evaluate how well your lungs work. The tests determine how much air your lungs can hold, how quickly you can move air in and out of your lungs, and how well your lungs put oxygen into and remove carbon dioxide from your blood. The tests can diagnose lung diseases, measure the severity of lung problems, and check to see how well treatment for a lung disease is working.Other tests such as residual volume, gas diffusion tests, body plethysmography, inhalation challenge tests, and exercise stress tests may also be done to determine lung function. Spirometry is the first lung function test done. It measures how much and how quickly you can move air out of your lungs. For this test, you breathe into a mouthpiece attached to a recording device (spirometer). The information collected by the spirometer may be printed out on a chart called a spirogram.

The more common lung function values measured with spirometry are:

• Forced vital capacity (FVC) . This measures the amount of air you can exhale with force after you inhale as deeply as possible.
• Forced expiratory volume (FEV) . This measures the amount of air you can exhale with force in one breath. The amount of air you exhale may be measured at 1 second (FEV1), 2 seconds (FEV2), or 3 seconds (FEV3). FEV1 divided by FVC can also be determined.
• Forced expiratory flow 25% to 75%. This measures the air flow halfway through an exhale (FVC).
• Peak expiratory flow (PEF) . This measures how quickly you can exhale. It is usually measured at the same time as your forced vital capacity (FVC).
• Maximum voluntary ventilation (MVV) . This measures the greatest amount of air you can breathe in and out during one minute.
• Slow vital capacity (SVC) . This measures the amount of air you can slowly exhale after you inhale as deeply as possible.
• Total lung capacity (TLC) . This measures the amount of air in your lungs after you inhale as deeply as possible.
• Functional residual capacity (FRC) . This measures the amount of air in your lungs at the end of a normal exhaled breath.
• Expiratory reserve volume (ERV) . This measures the difference between the amount of air in your lungs after a normal exhale (FRC) and the amount after you exhale with force (RV).

Carmel Magazine

The Living Breath Foundation's "In a Garden of Sixty-Five Roses" event can be seen in the new issue of Carmel Magazine. The event was featured as part of the "Peninsula Scene" section of the magazine, where local events are showcased.


(holiday 2009 issue)
Check out the page here: http://www.carmelmagazine.com/peninsula/ho09.shtml

Monterey Herald publishes article on The Living Breath Foundation

Recently the Monterey Herald published an article on The Living Breath Foundation. Take a look!

A Breath of Fresh Air
Family deeply affected by an unforgiving disease makes it a mission
to help others burdened by cystic fibrosis

By MARC CABRERA

Herald Staff Writer

October 30, 2010

The Pappageorgas family, from left: Melissa, 24; father Chris; Nick, 26 and mother Lori (ORVILLE MYERS/The Herald)

Having one child grow up with cystic fibrosis, the debilitative disease that primarily affects the lungs, would have been difficult enough for the Pappageorgas family.

But when both of their children were diagnosed in their early teens, Chris and Lori Pappageorgas faced the challenge with determination. Now, they are working to pass on that determination to other families who are dealing with the disease through their Living Breath Foundation.

Formed in 2008, the foundation (www.thelivingbreathfoundation.com) has already raised more than $285,000 and distributed more than $200,000 to further research and provide financial assistance to cystic fibrosis patients and their family members.

They started the foundation after recognizing the need for financial assistance based on their own experiences as parents. Both of their children, Nick, 26, and Melissa, 24, were diagnosed with the disease in high school.

"We wanted to provide for those others' needs," said Chris Pappageorgas, a licensed carpenter who works with Harvest Construction in Monterey. His wife Lori has worked in the dental field for 35 years and is currently on disability.

According to the Cystic Fibrosis Foundation Web site, cystic fibrosis is an inherited, chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. It can also obstruct the pancreas and stop natural enzymes from helping the body break down and absorb food.

It's a complicated disease, and a big part of the Pappageorgas' push has been to inform the local community.

"You've heard the name, but don't really know what (cystic fibrosis) is," said Lori Pappageorgas.

The Pappageorgas' case of having two children with the disease appears rare considering the relatively small number of patients in the United States, but it's not improbable.

According to the couple, 1 in 29 Caucasians are carriers of the gene that causes the disease. Both Chris and Lori are carriers, so their probability of having a child with cystic fibrosis was 1 in 4.

"The odds were the same for each child," said Lori Pappageorgas. "It's the odds of being with someone who has the gene as well (that are low)."

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

The Pappageorgas' children were diagnosed later than most, so the family got a late start on the demand of treatments.

Their children have routinely made hospital visits up to three or four times a month. In September, Nick was admitted early in the month, while Melissa had a stay toward the end of the month.

"It's a tremendously time-intensive treatment. Up to three hours a day of treatment," said Lori Pappageorgas. "It's a very difficult disease to manage. You can't manage it and maintain an eight-hour workday."

Knowing the ins and outs of treatment, the costs of traveling to out-of-town specialists and the out-of-pocket expenses involved, the Pappageorgases figured their best bet for helping was to give individual assistance.

Pappageorgas contributions have helped 15 individuals and families in Monterey County and throughout the state over the last two months.

This year, they were able to help a 9-year-old girl from Oakland who was in the advanced stages of the disease. They provided her with a portable machine that converts room air into pure oxygen.

The family's insurance company would only spring for a stationary device. The mobile machine, which runs for roughly $5,000, will allow her to leave the house while she battles the disease.

"It's going to allow her to attend school. We're allowing her some freedom," said Chris Pappageorgas. "When you're in the advanced stages like she is, who wants to be stuck at home?"

A social worker who had heart about Living Breath sought out the Pappageorgases and referred the girl. Lori Pappageorgas said they are now starting to get more referrals that way.

"We've been inundated. It's not only the word that we're out there, but that we're able to help," said Lori Pappageorgas.

That help has been extended to college students, in the form of three scholarships totaling $12,500. A single mother raising a child with the disease has gotten some support. Another young adult, recently unemployed and trying to keep bills, was given financial assistance.

The Pappageorgas did not reveal the names of the recipients, but said they hand over the money individually after getting a referral.

They have not turned anyone away seeking help, but they know they can only do so much. Chris Pappageorgas said their assistance is at best a temporary fix.

"Everyone of them are behind. Just trying to make ends meet. This helps so they can at least catch up," he said. "At least it relieves a lot of stress for them at the time."

Locally, the family knows of about a dozen local carriers. The family has worked to meet with them and help those that seek out assistance.

"Those are just the ones we know of," said Chris Pappageorgas. "We're trying to be active in the local cystic fibrosis community."

Francis Collins Interview with Bob Beall

Some video you may find interesting.

Cystic Fibrosis Foundation President and C.E.O. Robert J. Beall, Ph.D. interviews the Director of the National Institutes of Health, Francis S. Collins, M.D., Ph.D. at the North American Cystic Fibrosis Conference (NACFC) at the Minneapolis Convention Center on 10/15/2009.

Documentary film

This is a short film documentary film done about 9 months ago by Nate Olives a student. I thought it was worth sharing with everyone again. Nate is now in college studying film.

The Living Breath Foundation documentary from Nate Olivas on Vimeo.

Community Center for Health & Wellness

Everyone with CF or has someone in their family with CF knows the stress that can accompany having a chronic illness.

Here is a program worth checking into.

Community Center for Health & Wellness

http://www.itp.edu/currents/editorials/cf-support.php

The Institute of Transpersonal Psychology’s initiative to be of meaningful support and service to the community informs the Center’s overall mission, and is realized through meaningful collaboration with other community health providers.

One example of this commitment is the relationship between The Institute of Transpersonal Psychology (ITP) and Stanford School of Medicine Center for Education in Family and Community Medicine/Peter Judge Cystic Fibrosis Quality of Life Program. The goal of this program is to improve the quality of life for those who face Cystic Fibrosis every day, including giving assistance to caregivers and family members. This program offers help with such life skills as time-management, effective communication, self-advocacy, and help with issues related to stress, anger, grief, and loneliness.

To make an appointment or for further information call (650) 493-5006.

PARI Respiratory Launches PEP S

Here is a bit of info on Pari Respiratory's new PEP S which was developed to decrease medication delivery time and aid in airway clearance:

PARI Respiratory Launches PEP S

An Upcoming Stanford CF Discovery Series

There is another gathering in Stanford CF Discovery Series this October 13. This is the fifth in a series of monthly gatherings for the CF community, held on the second Tuesday evening of each month. Below is all the info!

Moving from the Moody Blues to a Golden Smile
Coping with Anxiety and Depression

An evening of support for the CF community

When: Tuesday, Oct 13, 2009 from 6pm-8pm

For: The CF Community: adult patients* and caregivers; adult and pediatric clinics
*Patients with CF and have ever cultured B. Cepacia or MRSA in the last 2yrs, or have a bacterium resistant to all antibiotics, will not be able to attend at this time.

Where: Adjacent to Stanford Hospital – in the Alway Building, Rm 104
Located next to Lane Library, on the ground floor, near a sandwich shop;
behind Stanford’s hospital main entrance/fountain area

Cost: FREE! Refreshments will be served.

Why: Because all of us could have brighter days; learn strategies and discuss difficulties with other CF patients and their families

Parking: FREE only at Structure 4 in front of Stanford hospital main entrance, near the fountains.

Questions? Camille Washowich, NP at the Stanford Adult CF Center

Presentation will be given by
Dr. Craig Schlarb & Students of Institute of Transpersonal Psychology

Stanford Hospital & Clinics & CFRI

More Photos from In a Garden of Sixty-Five Roses '09

Here's another set of photos from In a Garden of Sixty-Five Roses annual dinner auction, 2009.

Angels speech at In a Garden of Sixty-Five roses event

Angel's speech at In a Garden of Sixty-Five Roses annual event.

Photo Video from In a Garden of Sixty-Five Roses

This is a photo video put together by Tiffany Bell, one of our volunteers who worked at the event. The photos were taken by local photographers: Valerie Brower and Alex Geller. We will have more photos to post later. Hope you enjoy!

In a Garden of Sixty-Five Roses 2009

On August 25th 2009, The Living Breath Foundation held it's annual fundraiser at Tehama Country Club in Carmel, California. 220 guests came to the annual event benefiting those living with Cystic Fibrosis. A silent auction was held in Tehama's court yard while guests mingled and enjoyed wine provided by Ventana Vineyards, after which a fantastic dinner was severed in Tehama's main ballroom. Sandi Eason was the Mistress of Ceremonies and Angel Haynes was this years guest speaker. Angel is a 25 year old living with Cystic Fibrosis who spoke on her life and the challenges of living with CF. Bob Rosenthal, the auctioneer, kept the live auction fun and entertaining.

We are still working on the final total for the evening but we feel great about what has been tallied so far. We are looking forward to putting these funds to use in helping patients with CF!

Cystic Fibrosis Physical Therapy of Toddler

I found this video on youtube on how a parent does physical therapy on their toddler. I thought it was very informative.

What is Cystic Fibrosis?

The Following information comes from The Boomer Esiason web site.

What is cystic fibrosis?

Cystic fibrosis is a life-threatening disease that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria (or germs) to get stuck in the airways, which causes inflammation (or swelling) and infections that leads to lung damage.

Mucus also can block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food, which provides important nutrients to help us grow and stay healthy. People with cystic fibrosis often need to replace these enzymes with capsules they take with their meals and snacks to help digest the food and get the proper nutrition.

Symptoms

The severity of cystic fibrosis symptoms is different from person to person. The most common symptoms are:

• Very salty-tasting skin
• Persistent coughing, at times with phlegm
• Frequent lung infections, like pneumonia or bronchitis
• Wheezing or shortness of breath
• Poor growth/weight gain in spite of a good appetite
• Frequent greasy, bulky stools or difficulty in bowel movements
• Small, fleshy growths in the nose called nasal polyps

Sometimes people are told that they have asthma or chronic bronchitis when they really have cystic fibrosis. New research shows that the severity of CF symptoms is partly based on the types of CF gene mutations (defects). Scientists have found more than 1,500 different mutations of the CF gene.

How does CF affect the lungs?

Scientists have many different ideas about what goes wrong in the lungs of a person with cystic fibrosis, but it all begins with defective CF genes. Normally, the healthy CF gene makes a protein — known as CFTR (Cystic Fibrosis conductance Transmembrane Regulator) — that is found in the cells that line various organs, like the lungs and the pancreas. This protein controls the movement of electrically charged particles, like chloride and sodium (components of salt) in and out of these cells. When the protein is defective, as in cystic fibrosis, the salt balance in the body is disturbed. Because there is too little salt and water on the outside of the cells, the thin layer of mucus that helps keep the lungs free of germs becomes very thick and difficult to move. And because it is so hard to cough out, this mucus will clog the airways and lead to infections that damage lungs.

Causes

Cystic fibrosis is a genetic disease. That means people inherit it from their parents through genes (or DNA), which also determine a lot of other characteristics, including height, hair color and eye color. Genes, found in the nucleus of all the body's cells, control cell function by serving as the blueprint for the production of proteins.

To have cystic fibrosis, a person must inherit two copies of the defective CF gene — one copy from each parent. If both parents are carriers of the CF gene (i.e., they each have one copy of the defective gene), their child will have a 25% chance of inheriting both defective copies and having cystic fibrosis, a 50% chance of inheriting one defective copy and being a carrier, and a 25% chance of not having CF or carrying the gene.

Who gets cystic fibrosis?

Approximately 30,000 people in the United States have cystic fibrosis. An additional 10 million more — or about one in every 31 Americans — are carriers of the defective CF gene, but do not have the disease. The disease is most common in Caucasians, but it can affect all races.

What is the life expectancy for people who have CF (in the United States)?

There is no way to accurately predict how long people with cystic fibrosis will live, as many different factors may affect a person’s health. Severity of disease and time of diagnosis are two such factors. Many people have a mild case of CF, while others can have moderate or severe cases. In addition, some adults with cystic fibrosis have only recently begun to use new treatments, while an infant now diagnosed at birth will have the advantages of starting specialized treatments that were not available even a decade ago.

In 2005, the predicted median age of survival rose to 36.5 years, up from 32 in 2000. The steady rise of the median predicted age of survival suggests how improvements in treatment are advancing the lives for those with CF. In 1955, children with CF were not expected to live even to first grade. Today, an increasing number of people with cystic fibrosis are living into adulthood and leading healthier lives that include careers, marriage, and families of their own.

Is cystic fibrosis fatal?

Currently, there is no cure for cystic fibrosis. However, specialized medical care, aggressive drug treatments, and therapies, along with proper CF nutrition, can lengthen and improve the quality of life for those with CF.

When will there be a cure?

Because CF researchers are blazing new trails in drug development and gene therapy, experts have no way of saying for sure when a cure will be available. Certainly many children today have the chance to live long and full lives. In fact, for the first time in the history of the disease, many people with CF are now living into adulthood — more than 40 percent of people with CF in the United States are age 18 or older.

The “aging” of the cystic fibrosis community is largely due to the increase in innovative new treatments and specialized medical care. But a better quality of life and partially increased length of life are simply not enough. That is why BEF supports the CF Foundation’s efforts to expand and strengthen the drug development pipeline of potentially life-saving new therapies while, at the same time, supporting a vital care center network.