Friends

What does a friend mean to you?  Look at the person in this picture.  Soft brown eyes, mischievous smile, a go getter look on her face.  She is a mother.  A fun mom who gets on the level of her teen, a daughter that counts on her and loves her so. Shawna has Pulmonary Fibrosis.  She loves laughing, helping her friends smile, her baby dogs, her beautiful daughter, music, the beach, crocheting, geeking out and life.

Since my husband was diagnosed with this disease at age 49 and passed away from it in 2011, I know this is nothing to play with.  There are many (as many as breast cancer) that pass away from it every year in the United States.  Health care is difficult, even when there is is cure, because of finances many cannot afford to live.  In this case, the only cure, and it is not guaranteed, is a lung transplant.

I have been blessed to know Shawna, and now, she has made the huge decision to pursue this operation.  It takes a lot.  Bravery, Acceptance, Flexibility, and Finance.  Let's not forget Hope.  Can you imagine, putting your whole life out there for everyone to see, with the hopes of raising some money just so you can, live, Breathe?
https://www.gofundme.com/shawna-fetterolf-medical-fund?fbclid=IwAR3VyHnYdxrxNbvoyeqSkv_wGFyGjPHqF3q9SLfY-2ACRuUlXFtvdACo9e8https://www.gofundme.com/shawna-fetterolf-medical-fund?fbclid=IwAR1_RaAIb2INj5-0-ag769y6DJjxN47YG5bF_ju7P880cSItd4aAQEzoPn8

Pulmonary Fibrosis Patient and Caregiver Map

If you or someone you love has Pulmonary Fibrosis, you may have felt somewhat isolated as this is considered a rare-disease. A map has been formed to help assist in connecting both patients and caregivers, alike. Please check out this valuable tool.  ~Breathing  

to go there directly, click   https://www.google.com/maps/d/viewer?mid=zW_u6xjtaZtI.k2cqDBhhYmqs

Keep On Keeping On! ~Written By: Denise Queen-Sackinger

On this day, 7 years ago I underwent an open lung biopsy because a pulmonologist assigned to me a month earlier while I was in the hospital with double pneumonia didn't like what he saw on my x-rays. And the rest, as they say, is history!

Like most diagnosed with this mess, I had been seeing my PC doc for over 2 years about a chronic cough. Like most, I had never heard of IPF. Like most, I was scared out of my mind when I got the results of the biopsy. But here I am, 7 years and still stable. I sleep with O2 and use it with exertion. I had to quit working 2-1/2 years ago due to the high stress job and the bone degeneration caused by Prednisone. But, I've beat the statistical odds and for that, I am grateful. My 1st grand daughter was 3 days old on this day 7 years ago. I was sure I would not see her walk, much less be the young gymnast she is. I went to her 7th birthday party this past Saturday and best of all, I have 3 more grand daughters; her baby sister and her 2 cousins. I am blessed.

It's not lost on me that September is the month I was diagnosed and it is PF Awareness month. The year after I found out about this disease there was a PF Awareness WEEK. We are making progress! I've met some great people on FB the past 7 years. I've made life-long friends; most I'll never meet. I've lost far too many. If you have the energy to be an active advocate for PF, I applaud you. If you are at a point where you can only tell one person, one PF fact, I applaud you. Love, peace and happiness.  

“Keep on Keeping On!"  Written By:  Denise Queen-Sackinger

**Thank You Denise, for allowing me to share your experience.  You're spirit is amazing and may you have many more blessings to come!  ~Breathing

5 Things Every Person Living with a rare disease understands ~Written By: Rachel Wilson

5 Things Every Person Living with a Rare Disease Understands

Most people have heard the term “rare disease” but far fewer can name a rare disease let alone imagine what life might be like for those who have one. When it comes to rare diseases, including rare pituitary diseases like Cushing’s disease and acromegaly, what’s truly rare is the kind of public awareness and understanding that people with a rare disease truly deserve.

Rare Disease Day, which falls on February 28, aims to spread awareness about these conditions and the impact they have on patients’ lives.

How rare is “rare?” On one hand, people with a specific rare disease are statistically few and far between – in the U.S., a disease is considered rare if it is believed to affect fewer than 200,000 Americans. In the UK, a disease is considered rare if it affects fewer than 50,000. On the other hand, there are over 6,800 such diseases, according to the U.S. National Institutes of Health (NIH), so for something considered “rare” there sure are a lot of them.

In support of the rare disease community, Novartis will be launching an educational initiative called “A Day in My Shoes” which aims to tell the stories of people living with acromegaly. We spoke to several individuals for this post, and, as part of this effort to educate, they shared five things almost every person living with a rare diseases knows:

1. Getting properly diagnosed is one of the biggest challenges. Rare diseases are so rare that the symptoms are often misunderstood and as a result, people with rare diseases often spend years trying to get properly diagnosed. In the case of acromegaly, getting a correct diagnosis can take anywhere from six to 10 years and for Cushing’s disease, it can take about six years on average. By the time they’re diagnosed, many patients are just relieved just to put a name to their symptoms.
2. Your friends may know about your diagnosis, but only a few gems will really get what a chronic illness is or what it means. Many people are so uninformed about rare diseases that they expect your rare disease to clear up like a lingering flu. Blogger Rachel Wilson has Cushing’s disease, an endocrine disorder caused by a noncancerous pituitary tumor which ultimately leads to excess cortisol in the body. “There’s not a lot of empathy,” she notes. “Even some people that know me kind of get annoyed. ‘You’re sick again?’ or ‘What do you mean you can’t walk with us? But you walked last week!’”
3. You choose whom to tell very, very carefully. Most people living with rare diseases agree that once a diagnosis is public knowledge, people treat you differently. “I want them to know I have serious health issues but… I don’t want people to look at me like I’m disabled,” Rachel explains. There’s a paradox that patients face – wanting to tell but knowing that the people they tell are likely not to truly understand without a lot of effort on their part to explain…and then still, they probably won’t get it like they do with more widely known diseases such as cancer or multiple sclerosis.
4. Rare disease patients often play a large role in educating their doctors. Rare diseases aren’t just rare to the general public, they’re often rare to the physicians who treat them, even specialists. You’ve tried what seems like every available treatment, read medical journals, and done your own research. With all this, plus just living with the condition, you are the world’s foremost expert on how your rare disease affects you.
5. People will try to cure you. Not just your doctors. Everyone. Your Aunt Sally swears by a green smoothie and its healing properties. Your son’s third grade teacher has these supplements you simply have to try. “Everyone knows everything about anything,” is how Rachel puts it. “People like to diagnose you, or treat you, or, since they heard about this on a TV show, they know it’s not as bad as you make it out to be.” Many rare disease patients feel that people equate “rare” to “not really understood by the medical community.”

And while some of these realities for people living with a rare disease may indicate that they want both privacy and just to be treated like everyone else, most are strong advocates for public education efforts. Cushingstories.com co-founder Rae Collins notes, “Educating was key. To help others understand the disease, for me to understand it more, to help doctors even understand what I was going through. The more people who understood in my life, the better it became to me.”

Check out Novartis’ The Voices of Acromegaly and Voices of Cushing’s disease, a three-part video series that feature advocates, caregivers and people living with rare diseases on the Novartis Rare Disease YouTube Playlist.

For additional information on rare diseases and Rare Disease Day, visit Rare Diseases: More Common Than You Think? or the Rare Disease Day 2015 website.

via;  http://www.novartispharmaceuticals.com/en/stories/detail/5-things-every-person-living-with-a-rare-disease-understands

L O V E

"If you are not too long, I will wait here for you all my life."

Oscar Wilde

Dear Darling, Happy Valentine's Day.  It has been three years and two months since I have kissed you.  I miss you, Babe.  I dream of you often.  As the years have passed~ Spring, Summer, Autumn and Winter continue to flow as scheduled.  The children are growing, fine young people.  Our home, still safe and comforting.  The river so surreal, a beautiful gift everyday.  Sunrise and sunsets, with birds flying and making sounds.  The fish jumping upon waves of diamonds.  Clouds, kissed by light, whisper and beckon .  

The Stars, though, as beautiful as they are, To me, do not shine as brightly.  However, the Moon, is as mystical and glowing~ as ever before.  Blooming and ever-changing, like a Rose.  

I could go on and on.  Words really can't explain the transformations that occur moment by moment in the daily life.  For You, words do not have to.  You penetrate my heart, existing in all that my senses allow. ~~~Breathing~~~

“There is a time for departure, even when there is no certain place to go.” 

Tennessee William

****Hello, this is Breathing, I just wanted to Thank You for all the support you have given to Pulmonary Fibrosis, as well as me.  My 'counter' indicates 13,208 people have come to my blog, from many different countries.  I hope our family's experience has helped to raise awareness.  I do know this blog has helped me through such a difficult time.  I have shared with you our experience and have never held back, because most of this I typed in 'real-time'.  Now, I have transformed a bit since my husband's passing.  Not too much, but just enough to somehow realize that I have many thoughts that are better realized in my own time.  I will not post as much here, but anything that seems newsworthy to our cause will not be ignored.  Thank You my beautiful friends.  You show support just by coming here and reading this:

The word "pulmonary" means “lung” and the word "fibrosis" means scar tissue – similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. But, pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.

Also, pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike (see “Causes and Symptoms” below). The PF family of lung diseases falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don't include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.

The most common symptoms of PF are cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.

Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to IPF and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth.

Other common symptoms of pulmonary fibrosis include:

• Chronic dry, hacking cough
• Fatigue and weakness
• Discomfort in the chest
• Loss of appetite
• Unexplained weight loss

The Pulmonary Fibrosis Foundation is here to help you understand what it means to have pulmonary fibrosis. You can always reach us through our Patient Communication Center at 844.Talk.PFF or by email at pcc@pulmonaryfibrosis.org.

~~~~For my Baby, On Valentine's Day~~~~

"FIELDS OF GOLD"

You'll remember me when the west wind moves
Upon the fields of barley
You'll forget the sun in his jealous sky
As we walk in fields of gold

So she took her love
For to gaze awhile
Upon the fields of barley
In his arms she fell as her hair came down
Among the fields of gold

Will you stay with me, will you be my love
Among the fields of barley
We'll forget the sun in his jealous sky
As we lie in fields of gold

See the west wind move like a lover so
Upon the fields of barley
Feel her body rise when you kiss her mouth
Among the fields of gold
I never made promises lightly
And there have been some that I've broken
But I swear in the days still left
We'll walk in fields of gold
We'll walk in fields of gold

Many years have passed since those summer days
Among the fields of barley
See the children run as the sun goes down
Among the fields of gold
You'll remember me when the west wind moves
Upon the fields of barley
You can tell the sun in his jealous sky
When we walked in fields of gold
When we walked in fields of gold
When we walked in fields of gold

It's The Simple Things That Are Important~ Written By: Christy Mccullough

Five years....five years that my life was changed forever. The day I was told and crying coming out of that office and not even knowing what exactly it was I had but knew I would have to have a lung biopsy. How could this be happening to me? What would my life be like? So many things running through my head and not knowing where to turn. I didn't even know what Interstitial Lung Disease was and what life was going to become. It took me until after the New Year to even realize what was happening and longer to tell family how bad it was. 

I went through stages of denial, grief, anger. The thought of never seeing my children graduate, not being there to help my daughter pick a wedding gown, never seeing my grandchildren. Why was this happening to me? I thought my life was over. My children, family, and friends would watch me die slowly and there was nothing I could do. But I was wrong. I was not raised to give up and I have been through many things that I came out of and I would keep fighting not for me, but for my kids. To know that mama doesn't give up and show them you keep fighting no matter what. 

Days are not always easy. I gained weight from meds and lost my self confidence of not just as a person but as also being a woman. I can no longer breathe as easy and do things like I could. I don't like asking for help, never have. And hate when I can no longer do things as I could before without having to stop cause I can't breathe. I would never be the same person as I once was. 

I found support though with family and friends and also support groups in which those people have now become like family.  I can't say that it's not hard as it was but I have come to terms that one day my time will come. We all leave this world one day but somehow it's different when you know that you only have so long and there's nothing you can do about it. The one thing you do know is that you live. Live everyday. It's funny that at times I forget that I'm sick and have a moment like why am I coughing so much and remember~ oh yeah "lol"! Five years of coughing so hard you break your ribs, Five years of changing how you do things, five years to learn that it's the simple things that are important. 

So many people take for granted the little things, little things as just being able to breathe. Be grateful for the little things and never take life for granted. I have made the five year mark and plan on fighting till the end. I thank God for giving me the chance to wake up and try again everyday. I thank God for the family and friends who love me and give me the strength I need at times and a husband who has done more than support me in every way everyday.

**Thank You, Christy for allowing me to share your thoughts and to help lend hope for those diagnosed with Pulmonary Fibrosis to know that it is a learning process and perhaps, somewhere within that process is a deeper understanding of the things that are truly valuable in our lives.**  

~~~Breathing~~~

September Pulmonary Fibrosis Awareness ~My Wish

On this last day of September, having looked back at Pulmonary Fibrosis Awareness month, I can honestly say that this year seemed to be one of the most successful in spreading the word about this disease.  I have seen posts full of beautiful people with streaks of blue in their hair, listened to songs with blue in the title, watched touching videos of personal stories, read facts and blogs.  So many amazing people trying to do their part to let the world know how PF touched their lives; from patients themselves, to foundations, caregivers, friends and family members who lost someone to this disease.  

In trying to do my little part to contribute, I can say that it often is not the easy thing to do.  Can you imagine having this disease, yet posting and reading about the short life expectancy, or the terrible symptoms you may one day experience?  Or, as in myself, and many others that have lost a loved one, to relive the experience with every bit of research you do or the moment you tell your story to someone else?  It is not easy, but I see the strength of all of those who continue to advocate and I feel stronger, too.  

It is my wish that our collective stories have touched and informed some of those who have never heard of the disease.  That the general public becomes aware of Pulmonary Fibrosis and that one day, that awareness may lead to more funding for research and alternatives in medications, prevention, or a cure. 

Information on Managing your symptoms of Idiopathic Pulmonary Fibrosis~ via; Lungs and You

This article is via; Lungs and You, please check out their website for more great information at:  www.lungsandyou.com

There are no FDA-approved medicines that treat IPF. However there is a good deal of research being performed and several clinical trials are underway to investigate potential treatments for IPF. These treatments are experimental and the impact they have on the course of IPF is currently being studied.

Despite the lack of medicines approved to treat IPF, there are still things you can do to help manage IPF symptoms and try to sustain your ability to perform daily activities for as long as possible.

The approaches used to manage the symptoms of IPF are designed to meet each patient’s unique needs. Every person’s medical history is different. In addition, people with IPF frequently suffer from other medical conditions. These other conditions may have an impact on the course of IPF (See "Managing other conditions" below.)

It’s also important to remember that each patient experiences IPF differently, and while some people with IPF don’t live long after getting their diagnosis, others may live longer than the often-quoted averages. Working together, you and your doctor can develop a plan to help you manage your symptoms in an effort to sustain your ability to participate in daily activities for as long as possible. Common approaches to managing IPF symptoms are listed below.

Summary of options for managing IPF symptoms

	Pulmonary Rehabilitation	Includes a range of conditioning and breathing exercises The goal is to help patients function to the best of their ability
	Oxygen Therapy	Recommended for patients who have low oxygen levels May help reduce breathlessness, enabling the patient to take part in pulmonary rehab exercises
	Lung Transplant	Can improve both life expectancy and ability to participate in daily activities Reserved for patients who have no other significant health problems, such as cancer; heart, liver, or kidney disease; or chronic infection, among others IPF is now the leading reason for lung transplantation in the US Lung transplantation has significant risks, including illness or fatality from the surgical procedure itself, infection, and cancer due to the use of drugs that suppress the immune system; you should discuss these risks with your doctor before considering a lung transplant
	Clinical Trials	Taking part in clinical trials may be an option for some people with IPF Talk with your healthcare team about your condition and your options

Managing other conditions

As mentioned above, it is common for people with IPF to also have other medical conditions (called “comorbidities”). These may include obesity, diabetes, pulmonary hypertension, obstructive sleep apnea, coronary artery disease, and emphysema.

These conditions will often require their own treatments and medicines. They may even have an impact on the course of IPF. Remember to always take your medicines as prescribed by your doctor.

If you have any questions about other health conditions you have, or the medicines you are taking for them, be sure to talk to your doctor.

For the Caregiver~~~

With September being PF Awareness month, I just wanted to say a few words to all caregivers. Thank You. We see you. Many times you are behind the scene, making meals, filling prescriptions, researching, advocating, loving and caring for your loved ones every need~ physically and emotionally. Often ignoring any aches or pains that you may have, worries, sadness or sense of loss. Maybe you are feeling isolated and helpless to the situation you and your loved one are in. You are true warriors and your spirit shines through everything you do. Thank You, Dear Ones!

Pulmonary Fibrosis Trust

Please visit another great website from the UK:  Pulmonary Fibrosis Trust at www.pulmonaryfibrosistrust.org

InterMune Reports Phase 3 ASCEND Trial Results of Pirfenidone in Idiopathic Pulmonary Fibrosis (IPF)

BRISBANE, Calif., Feb. 25, 2014 /PRNewswire/ -- InterMune, Inc. (ITMN) today announced that top-line data from ASCEND, a Phase 3 trial evaluating pirfenidone in patients with idiopathic pulmonary fibrosis (IPF), demonstrated that pirfenidone significantly reduced IPF disease progression as measured by change in percent predicted forced vital capacity (FVC) from Baseline to Week 52 (rank ANCOVA p

(Logo:http://photos.prnewswire.com/prnh/20120827/SF62570LOGO)

"We are pleased to report these top-line ASCEND Phase 3 results," said Dan Welch, Chairman, Chief Executive Officer and President of InterMune.  "Based on the strength of the ASCEND results, InterMune is preparing a resubmission of our New Drug Application for pirfenidone to the U.S. Food and Drug Administration (FDA), which we expect to submit by early third quarter of this year.  We would like to thank our collaborators, patients and their families for their participation in ASCEND and their contributions to IPF research."

Primary Endpoint

The magnitude of the treatment effect of pirfenidone was measured by comparing the proportion of patients in the pirfenidone and placebo groups experiencing either a clinically meaningful change in FVC, or death.  A 10% decline in FVC in an individual IPF patient is considered clinically meaningful and strongly predicts mortality.  At Week 52, 16.5% of patients in the pirfenidone group experienced an FVC decline of 10% or more or death, compared with 31.8% in the placebo group, representing a 47.9% reduction in the proportion of patients who experienced a meaningful change in FVC or death.  Additionally, at Week 52 the data demonstrated that 22.7% of patients in the pirfenidone group experienced no decline in FVC, compared with 9.7% in the placebo group, representing a 132.5% increase in the proportion of patients whose FVC did not decrease between Baseline and Week 52.

Dr. Talmadge King, Chair, Department of Medicine, University of California San Francisco and Co-chair of the ASCEND protocol steering committee, said, "IPF is an unpredictable, debilitating and ultimately fatal disease, and safe and effective treatments are desperately needed to alter the course of this challenging and complex condition.  The ASCEND data demonstrated that pirfenidone significantly reduced decline in lung function and significantly increased the proportion of patients who had no decline, which is an important advance in the field.  The results for 6MWT distance, PFS and mortality provide important supportive evidence of pirfenidone's efficacy." 

Key Secondary Endpoints

The ASCEND protocol pre-specified 6MWD and PFS as the two key secondary endpoints.  Change from Baseline to Week 52 in 6MWD is a measure of exercise tolerance.  A 50-meter decrement in walk distance is considered an independent predictor of mortality in an individual patient with IPF.  In ASCEND, pirfenidone reduced by 27.5% the proportion of patients who experienced a decline in 6MWD of 50 meters or greater (p=0.0360). 

PFS is a measure of time before death or a disease-progression event.  A PFS event was defined in the protocol as any of the following: death, percent predicted FVC decrement of 10% or greater or 6MWD decrement of 50 meters or greater.  In ASCEND, pirfenidone reduced the risk of death or disease progression by 43% compared to placebo (Hazard Ratio [HR]=0.57; 95% confidence interval, 0.43-0.77; p=0.0001).

Additional Secondary Endpoints

Three additional secondary endpoints were pre-specified in the ASCEND protocol: all-cause mortality, treatment-emergent IPF-related mortality and change from Baseline to Week 52 in dyspnea (shortness of breath).  The two mortality analyses were pre-specified for both the ASCEND study and the pooled population of the ASCEND study and the previous Phase 3 CAPACITY studies through 52 weeks.  Due to the relatively low overall mortality rate in patient populations in the time frames studied in a single IPF study such as ASCEND, pooled analyses of ASCEND and CAPACITY data provide more statistical power and a more precise estimate of the treatment effect of pirfenidone on mortality.

In the pre-specified mortality analysis of the ASCEND study alone, there were fewer events of all-cause mortality (HR=0.55, log rank p=0.1045) and of treatment-emergent IPF-related mortality (HR=0.44, log rank p=0.2258) in the pirfenidone group compared to the placebo group.  ASCEND was not powered to show a difference on these endpoints.  The relationship of death to IPF was determined in ASCEND by a blinded adjudication committee. 

The pre-specified analyses of the pooled population (N=1,247) across ASCEND and the two Phase 3 CAPACITY studies (taking CAPACITY mortality data through Week 52) showed that the risk of all-cause mortality was reduced by 48% in the pirfenidone group compared to the placebo group (HR=0.52, log rank p=0.0107).  Additionally, in the pooled population the risk of treatment-emergent IPF-related death in the pirfenidone group compared to placebo was reduced by 68% (HR=0.32, log rank p=0.0061).

The secondary endpoint of dyspnea, measured by the UCSD SOBQ questionnaire, was not achieved (p=0.1577).

Safety and Tolerability

In ASCEND, pirfenidone showed a favorable safety profile and was generally well tolerated. 

A total of 93.5% and 94.6% of patients completed the study, died or had a lung transplant by study day 365 in the pirfenidone and placebo groups, respectively.  The percentage of patients discontinuing treatment due to an adverse event was 14.4% in the pirfenidone group and 10.8% in the placebo group.  Serious adverse events (SAEs) were reported in 19.8% of patients in the pirfenidone group and 24.9% in the placebo group.  Hospitalizations due to respiratory, thoracic and mediastinal SAEs were reported in 3.6% of patients in the pirfenidone group and 11.2% in the placebo group.

The most common AEs with higher incidence in the pirfenidone group were primarily gastrointestinal (e.g., nausea and dyspepsia) and skin-related (e.g., rash).  The GI and rash AEs were generally mild to moderate in severity, manageable, reversible and only infrequently led to treatment discontinuations. 

Elevations of aminotransferase levels at least 3 times the upper limit of normal occurred in 2.9% of pirfenidone patients (including one case associated with a bilirubin increase) vs. 0.7% of placebo patients.  In general, these elevations occurred early, were manageable and reversible, and were similar to those observed in previous pirfenidone studies.

The safety and tolerability profile of pirfenidone was generally consistent with observations from the previous Phase 3 CAPACITY studies, open-label extension studies and post-marketing experience.

"These results from the ASCEND trial provide compelling evidence of a clinically meaningful treatment effect of pirfenidone with generally favorable safety and tolerability findings, which is very encouraging for patients suffering from this fatal and relentless disease," said Paul W. Noble, Chair, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, Calif. and Co-chair of the ASCEND protocol steering committee.  "Importantly, the overall safety observations from ASCEND complement and corroborate the robust safety database that already exists from the InterMune-sponsored clinical studies of pirfenidone and extensive post-marketing experience outside the United States."

InterMune intends to present additional data from the ASCEND study at the 2014 American Thoracic Society International Conference in May.

About ASCEND

ASCEND (Assessment of Pirfenidone to Confirm Efficacy and Safety in IPF) is a multinational, randomized, double-blind, placebo-controlled Phase 3 trial designed to evaluate the safety and efficacy of pirfenidone in patients with IPF.  Patients (N=555) were randomly assigned 1:1 to receive oral pirfenidone (2403 mg/day) or placebo and were enrolled at 127 centers in the United States, Australia, Brazil, Croatia, Israel, Mexico, New Zealand, Peru and Singapore. 

More than 95 percent of eligible patients (those patients who remained on blinded pirfenidone or placebo therapy) who completed the ASCEND study decided to enter the open-label RECAP extension study.  RECAP is a study in which all patients receive pirfenidone.  RECAP also includes patients rolled over from the company's prior CAPACITY studies which completed in late 2008 and enrolled 779 patients in two Phase 3 studies.  RECAP provides valuable long-term safety data that further expands the already large safety database for pirfenidone in patients with IPF.

About CAPACITY

Pirfenidone has been studied in multiple Phase 3 clinical trials in patients with IPF, including the two Phase 3 CAPACITY trials sponsored by InterMune.

The CAPACITY program consisted of two concurrent 72-week trials which enrolled a total of 779 patients.  Both trials were multinational, randomized, double-blind, and placebo-controlled. The studies were designed to evaluate the safety and efficacy of pirfenidone in IPF patients with mild to moderate impairment in lung function.  The primary endpoint in both studies was the change from Baseline to Week 72 in percent predicted FVC.  This endpoint was met with statistical significance in CAPACITY 2 (p=0.001).  The secondary endpoints of PFS and categorical change in FVC also achieved statistical significance (p

Pirfenidone demonstrated a favorable safety profile and was generally well tolerated in both CAPACITY studies.  The most frequent side effects reported were photosensitivity rash, gastrointestinal symptoms such as nausea and dyspepsia, and dizziness.

About Esbriet® (pirfenidone)

Pirfenidone is an orally active, anti-fibrotic agent that inhibits the synthesis of TGF-beta, a chemical mediator that controls many cell functions including proliferation and differentiation, and plays a key role in fibrosis.  Pirfenidone also inhibits the synthesis of TNF-alpha, a cytokine that is known to have an active role in inflammation.

On February 28, 2011, the European Commission (EC) granted marketing authorization for Esbriet® (pirfenidone) for the treatment of adults with mild to moderate IPF.  The approval authorized marketing of Esbriet in all 28 EU member states.  Esbriet has since been approved for marketing in Norway and Iceland.  In 2011, InterMune launched commercial sales of pirfenidone in Germany under the trade name Esbriet, and Esbriet is now also commercially available in various European countries, including key markets such as France, Italy and the UK.

On October 1, 2012, Health Canada approved Esbriet for the treatment of mild to moderate IPF in adult patients.  Health Canada designated Esbriet for Priority Review and completed the accelerated review according to target guidelines of 180 days.  InterMune launched Esbriet in Canada in January 2013.

Pirfenidone has been marketed as Pirespa® since 2008 in Japan and since 2012 in South Korea by Shionogi & Co. Ltd.  Under different trade names, pirfenidone is also approved for the treatment of IPF in China, India, Argentina and Mexico.

Pirfenidone is not approved for sale in the United States.

About IPF

Idiopathic pulmonary fibrosis (IPF) is an irreversible and ultimately fatal disease characterized by progressive loss of lung function due to fibrosis (scarring) in the lungs, which hinders the ability of lungs to absorb oxygen.  IPF inevitably causes shortness of breath, and a deterioration in lung function and exercise tolerance.  IPF patients follow different and unpredictable clinical courses and it is not possible to predict if a patient will progress slowly or rapidly, or when the rate of decline may change.  Periods of transient clinical stability in IPF, when they occur, inevitably give way to continued disease progression.  The median survival time from diagnosis is two to five years, with a five-year survival rate of approximately 20-40 percent, which makes IPF more rapidly lethal than many malignancies, including breast, ovarian and colorectal cancers.  IPF typically occurs in patients over the age of 45, and tends to affect slightly more men than women.

About InterMune

InterMune is a biotechnology company focused on the research, development and commercialization of innovative therapies in pulmonology and orphan fibrotic diseases.  In pulmonology, the company is focused on therapies for the treatment of idiopathic pulmonary fibrosis (IPF), a progressive, irreversible, unpredictable and ultimately fatal lung disease.  Pirfenidone, the only medicine approved for IPF anywhere in the world, is approved for marketing by InterMune in the EU and Canada under the trade name Esbriet®.  Pirfenidone is not approved for sale in the United States but has completed three Phase 3 clinical trials to support regulatory registration in the United States.  InterMune's research programs are focused on the discovery of targeted, small-molecule therapeutics and biomarkers to treat and monitor serious pulmonary and fibrotic diseases.  For additional information about InterMune and its R&D pipeline, please visitwww.intermune.com.

Conference Call and Webcast Details

InterMune will host a live webcast of a conference call today at 8:00 a.m. EST to discuss the top-line ASCEND Phase 3 results.  Interested investors and others may participate in the conference call by dialing 800-738-1032 (U.S.) or +1-212-231-2905 (international), conference ID# 21709573.  A replay of the webcast and teleconference will be available approximately three hours after the call.

To access the webcast, please log on to the company's website at www.intermune.com at least 15 minutes prior to the start of the call to ensure adequate time for any software downloads that may be required.

A telephonic replay will be available for 10 business days following the call and can be accessed by dialing 800-633-8284 (U.S.) or +1 402-977-9140 (international), and entering conference ID# 21709573. 

Forward-Looking Statements

This news release contains forward-looking statements within the meaning of section 21E of the Securities Exchange Act of 1934, as amended, that reflect InterMune's judgment and involve risks and uncertainties as of the date of this release, including without limitation InterMune's expectations regarding the timing for resubmission of its new drug application with the FDA for pirfenidone; the potential to make pirfenidone available as a medicine to IPF patients in the United States and InterMune's intention to present additional data on the ASCEND trial at the American Thoracic Society meeting in May 2014.  All forward-looking statements and other information included in this press release are based on information available to InterMune as of the date hereof, and InterMune assumes no obligation to update any such forward-looking statements or information. InterMune's actual results could differ materially from those described in InterMune's forward-looking statements.

Other factors that could cause or contribute to such differences include, but are not limited to, those discussed in detail under the heading "Risk Factors" in InterMune's most recent annual report on Form 10-K filed with the Securities and Exchange Commission (SEC) on February 21, 2014 (the "Form 10-K") and other periodic reports filed with the SEC, including but not limited to the following: (i) the risks related to the uncertain, lengthy and expensive clinical development process for the company's product candidates, including having no unexpected safety, toxicology, clinical or other issues and having no unexpected clinical trial results such as unexpected new clinical data and unexpected additional analysis of existing clinical data; (ii) risks related to the regulatory process for the company's product candidates, including the possibility that the results of the new 52-week Phase 3 clinical trial (ASCEND) having an FVC endpoint may not be satisfactory to the FDA for InterMune to receive regulatory approval for pirfenidone in the United States; (iii) risks related to unexpected regulatory actions or delays, in particular in connection with our planned resubmission of a Class 2 NDA with the FDA seeking approval of pirfenidone or other government regulation generally; (iv) risks related to our ability to successfully launch and commercialize pirfenidone in the United States, if approved by the FDA and (v) InterMune's ability to obtain or maintain patent or other proprietary intellectual property protections.  The risks and other factors discussed above should be considered only in connection with the fully discussed risks and other factors discussed in detail in the Form 10-K and InterMune's other periodic reports filed with the SEC, all of which are available via InterMune's web site at www.intermune.com.

Source Via;   http://ca.finance.yahoo.com/news/intermune-reports-phase-3-ascend-120000665.html

My Donor, My Hero ~Pamela De Loach

It was November 10th 2011: It was a normal day for the De Loach’s (expect for me who has IPF and has to wear oxygen 24/7, this is as normal as it get for us). Bob and Rob were off to New York and I’m off to work thinking about the day and the long weekend with my girlfriend who is staying with me while Bob is away. It was a normal day at work as I ate my lunch in my office because I do not have the energy to walk to the staff lunch room. Back in the classroom we start lunch breaks for everyone else, one of the students’ needs to go to the nurse for a procedure so the other aide leaves.

Whoever said your life can change in a second was right. The phone rings, it’s the office, Tampa General Hospital (TGH) is on the phone they want to speak to you. I politely ask if I can call them back; the voice on the other end says "No, I’m coming to stay with your kids and you have walk to the nearest phone with an outside line." It finally dawns on me what it was- “the call”.

I thought about “the family plan” developed for when the call came, but I was so excited and I couldn’t drive home. Some of the school folks offer to drive me to TGH. The new “family plan” is quickly developed: Rachel, Joshua, and Angela would meet me at TGH and my school buddies would stay with me until my family arrived. I arrived at TGH and the testing began to see if the donor and I were "the perfect match".

As we waited for the test results to come in, the doctors and I spoke to Bob and the rest of the family in upstate New York via Skype. Everyone was in a good mood and when my coordinator announces “IT’S A GO” the room breaks out in cheers and tears as our prayers have been answered.

The next hour is a blur, the family walks with me to the OR and we say goodbye. I really got scared in that second. The surgery started shortly after that and my new lungs are in on November 11th 2011.

My donor and their family are my heroes. In their grief they honor the request of their loved one and gave me a chance to continue on with my life. As we celebrate this second year, we have to remember my donor’s family. Please remember to check off that donor box on your driver’s license and tell your family your wishes.

**Thank You, Pamela for sharing your story with us!  What a beautiful gift that has been given. ~Breathing 

Care Giving For Yourself

It was in October that my husband was diagnosed with Pulmonary Fibrosis.  Looking back at the span of his diseases progression, I realize that at that initial point the impact of the disease was primarily emotional.  Physically, the only way he was diminished was by a slight cough which sounded like he was clearing his throat.  In all other ways, physically he seemed fine.  By December the following year he had passed away from this disease.  He remained active up until the two months prior to his passing, at which point fatigue seemed to overcome him and he became weaker and weaker.  

In the October of his diagnosis, I was physically fine, too.  We both were just two normal people.  Very active and seemed to have a beautiful life ahead of us.  I was his caretaker, confidant and friend.  The initial point of his being diagnosed, it was primarily emotional for me as well.  As he progressed in his illness, I progressed in my level of care for him.  Taking care of my beautiful husband has been the most profound and rewarding experience that I have ever had.  It has also been the most difficult.

Have you heard that over long periods of time spouses start to look like one another?  Or perhaps, an owner of a dog will start to take on a resemblance of their dog?  I think it is because we grow close to the souls we love.  We empathize.  If they are happy we are happy, and if they are hurt, we are hurt.  

When someone we love feels ill, we feel ill too.  If they cannot catch their breath, we feel light headed.  If they are scared of what the future holds, we are scared.  But, here is the catch....   If we are a caregiver to that person, then in that moment, we cannot just collapse, we have to swallow it down and get it together.  After all, logically, we are not the ones that are sick, right?  Furthermore, the person that you would normally run to when you are not well, cannot deal with or accept what you may be going through.  As a caretaker, it is a scary place to be to know that there is no longer anyone left to run to.

There are moments in care giving, that when the patients back is turned that tears begin to stroll down your face.  Or, when you are sitting with them and telling them words of love, that your heart feels gripped with fear and dread.  When your partner is sleeping and you listen to the rise and fall of their breath and you lay awake, sick inside.  

When a loved one passes away, people tell you that their suffering is over.  But, you are still here.  Is your suffering over?  

When you advocate for the disease, you meet others who have the illness your spouse or loved one had.  The disease that you witnessed first hand.  The patients will tell you that because you are just a caretaker that you could never understand the frustrations of having the disease first-hand.  But, you do not say anything because you do understand.  You also understand what it is also like to see it through to the full progression.  Past the point that many patients are aware of.  You swallow this down.  Meanwhile, your husband or loved one is gone.  

As I mentioned, it was in December, almost two years ago my husband passed away.  It gets cold in the winter here.  During that time, I spent most of my time in a blanket.  My primary source of socialization, sitting at my computer.  Dark circles under the eyes, but, always putting happy-faced icons at the end of my sentences.  Everyday, a struggle just to get through.  Time passed, not in sections, but by minute.  If I could just get through the next minute, and the next...  Then, something needs tending.  I tend to it.  I feel glad I did and wait for the next minute.  Then another something needs my attention, and it goes round and round.  Until, slowly I notice what needs attention before hand.  Somewhere along the line, counting time by minutes, turned into measured days and I realized that it was time to turn my attention to me.  

Eventually, I started to see the sunlight.  I almost felt guilty about that.  At first it hurt my eyes, but then it felt good on my skin.   I began to feel proud that I had made it through and continued to take care of what needed tending.  I am surviving.  I am to the point that I have realized that this is not enough.  I have realized that I am not the one who died.  I am still alive.  I still have a purpose in my life and I want to do more than just survive.  I have also realized that Pulmonary Fibrosis has made me sick.  I had forgotten myself, physically and emotionally.  As my husband became more ill, so did I.  As he began to let go of life, so did I.  When he died, I did not.

I have to Give Care to myself now.