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

My Heart to You

It has been so long that I have truly written here, that I barely knew my sign-in name. 2011 held my most profound moments and emotions.  I do come here, to look at how many people have visited and from where in the world they came.  This was an amateur blog, written from the heart.  I also come here to read.  Sometimes, I can read only little bits at a time; other times, I read through everything like a novel.  First, I remember every feeling and moment.  Second, I see others that I approached to contribute their writing's and also know many have passed away.  Third, I notice my lack of editing and grammatical errors.  

Still, I notice that over 15,000 people have come here.  Some, just due to a google question that went wrong.  Even if that were half of the people who came, both halves matter.  

I have barely started writing again.  This place, I was so honest and raw, that it has been hard to write again.  I have been busy, trying to find my own spirit and supporting my children.  But, when I come here to read, I know that I was honest.  This disease.  Not everyone who has Pulmonary Fibrosis has the same experience and some make different choices, plus, it is a different time.  

For my husband, this has been the most truthful account from someone who loved him.  I still do.  Love him.  The words I write are not just for the sake of raising awareness, but instead; I am a writer, that writes about difficult experiences that have come to me personally.

Thank You for coming here.  Don't be discouraged.  No matter what, reflect outward in the way that is most creative and beautiful to you.

The things I tend to read on my blog are from this period of time.  If you want to go further back, hit- Older Posts, at the bottom of the page. This represents my heart the most and is what I have the hardest time reading, but am still thankful to have every experience associated with my husband and family. 

It is a love story that I hope you read...http://breathingair1.blogspot.com/2011

~~Breathing

Amazing Gift ~Written By Pamela De Loach

You know that moment in the morning before you are fully awake; I listen to my lungs - what a miracle! I can take a deep breath in and realize I can feel the air going in and filling up my lungs and then I can blow out my breath slowly. What an amazing gift!!!!!! Little over four years ago, I was fighting for every breath due to idiopathic pulmonary fibrosis (IPF). Today is my fourth lung anniversary and, as always, my donor is my hero. Today I think of their family and I hope they realize what an amazing person their loved one was and the fact they saved my life and perhaps the lives of others. What a gift they gave my family!! It’s still sad to think they left our world but left behind amazing gifts and allow others to live on. The best way you could help me celebrate this anniversary is make sure you are a donor. My family, doctors and friends are my greatest support system. Thank you all.

 ~Pamela De Loach

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

Study discovers biomarkers to predict the progression of idiopathic pulmonary fibrosis

Article via;   http://www.medicalnewstoday.com

A new blood test developed by experts at Royal Brompton Hospital could give patients with idiopathic pulmonary fibrosis (IPF) a better idea of their prognosis and whether or not treatments that can slow down the progression of the disease are working. 

The condition - thought to affect up to 20,000 people in the UK - causes progressive scarring of the lungs and is often fatal. 

The Prospective Observation of Fibrosis in the Lung Clinical Endpoints (PROFILE) study, the largest and most detailed observational IPF study of its kind, recruited 214 patients, who were identified by Royal Brompton Hospital and the University of Nottingham. The findings have been published online in the Lancet Respiratory Medicine this week in a paper written by IPF experts, including Dr Toby Maher, consultant respiratory physician at Royal Brompton and head of the Fibrosis Research Group at Imperial College London, Anne-Marie Russell, senior research nurse at Royal Brompton and Dr Gisli Jenkins at the University of Nottingham. 

The study, conducted at the National Institute for Health Research (NIHR) Royal Brompton Respiratory Biomedical Research Unit (BRU), took samples of blood and analysed the concentrations of several neoepitopes, which are types of proteins. These were measured at baseline and then at regular intervals throughout the following six months. Physiological measurements, including the amount of air which could be forcibly exhaled from the lungs and how much oxygen travels from air sacs (alveoli) in the lungs to the bloodstream, were also taken to detect how the condition was progressing. 

The research, which is the largest and most detailed observational IPF study of its kind, showed that the concentration of neoepitopes were higher in people with the condition compared with healthy controls. Some of the biomarkers were associated with worsening disease and outcomes and changes in their concentrations after three months appeared to predict the progression of IPF earlier than the physiological measurements. 

The results suggest that biomarkers could also be useful in the early stages of clinical trials for new treatments, as the measurements could indicate when the patient is responding to them.

Measurement of the neoepitopes may also be of use to clinicians when it comes to deciding what treatment to give patients, as it could potentially inform them if medication is working and thus help with the management of the disease. 

This could now have particular use because there are two antifibrotic drugs, pirfenidone and nintedanib, which have been approved for use in the UK within the last two years and both have been shown to slow disease progression. 

Commenting on the research, Dr Maher said: 
"These biomarkers have the potential to improve the treatment of IPF by enabling doctors to determine whether treatments are working or not at an early stage and before permanent lung damage has developed. Furthermore, the biomarkers may enable clinical trials in IPF to be much shorter, something which should speed up the process of making new treatments available for this devastating disease." 

Dr Maher and Dr Jenkins are involved in continued research which aims to make these blood tests available in specialist clinics. Further research, to search for other biomarkers of IPF and potential new ways of treating the disease, is ongoing.

 It is not known why IPF occurs, but it appears to affect cells that line the alveoli in the lungs, causing them to become damaged and die. In response, the body tries to repair the damage by releasing fibroblast cells, but the over-production of these cells leads to scarring and hardening (fibrosis) of lung tissue. The scarring means the lungs cannot work properly and patients are often short of breath and have a persistent dry cough, fatigue and gastric reflux. Patients with the condition have an average life expectancy of three years and the number of cases in the UK is increasing by around five per cent a year. 

Dr Maher said:
 "Although newly available treatments may help, this ongoing research is required to ensure better outcomes for patients with IPF." 

The research was funded by GlaxoSmithKline and the Medical Research Council and sponsored by Royal Brompton & Harefield NHS Foundation Trust and the University of Nottingham. Royal Brompton has the only unit in the UK solely dedicated to the management of patients with interstitial lung disease (ILD), the term used for more than 200 lung diseases that affect the tissue and space around the air sacs in the lung, including IPF. Experts at the hospital care for the largest number of IPF patients in the UK and receive around 1,000 new referrals every year. 

Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study, R Gisli Jenkins, PhD, Juliet K Simpson, PhD, Gauri Saini, MD, Jane H Bentley, PhD, Anne-Marie Russell, MSc, Rebecca Braybrooke, RGN, Philip L Molyneaux, MD, Tricia M McKeever, PhD, Athol U Wells, MD, Aiden Flynn, PhD, Prof Richard B Hubbard, MD, Diana J Leeming, PhD, Richard P Marshall, PhD, Morten A Karsdal, PhD, Pauline T Lukey, PhD, Dr Toby M Maher, PhD, Lancet Respiratory Medicine, doi: 10.1016/S2213-2600(15)00048-X, published online 11 March 2015.
Source: Royal Brompton & Harefield NHS Foundation Trust

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

~~~A Pulmonary Fibrosis Merry Christmas~~~

Merry Christmas to You~ 

The One with Pulmonary Fibrosis fighting each day to live a life fulfilled.  

The Caregiver, tirelessly assisting and supporting your loved one.  

The Child, holding the hand of your loved one and letting them hear your laughter.  

The Friend, who offers a shoulder to lean on and an ear to listen.  

The Bereaved,  bravely taking one step at a time forward, living to honor the spirit of their loved one.  

The Doctor or Nurse, trying their best to understand and treat the symptoms.  

The Researcher, searching to discover more about the causes and cure. 

The Advocate, creatively seeking to spread the word about Pulmonary Fibrosis.  

We All make a beautiful team, a family.  

To You~ My Pulmonary Fibrosis Family may you experience the magical love of this season 

in the most profound way.  

Many more treasured memories with each day of the New Year.  

~Breathing

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. 

Can I get paid to be a family caregiver?

In my particular state, I was told in order to receive a wage to be my husband's full-time caregiver, we would have to do two things:  A) get a divorce B) live in separate homes. I really never thought that made much sense. Perhaps, your situation is different. The link below offers some good places to start if you find yourself caring for a loved one.

The Care Givers Space

http://thecaregiverspace.org/blog/paid-family-caregiver/

This site is wonderful for anyone who is a caregiver.  Great information and support, so please feel free to visit them.  

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.

What Would We Do Without Support?

I wonder where I would be without finding the support of someone who understands?  ~Breathing

Find your support group by clicking the link below.  

Support Groups / Pulmonary Fibrosis Foundation

Don't have one in your area?  Contact the Pulmonary Fibrosis Foundation and find out how to get started.  There are also many groups provided online.

Rapid Progressors Speed to End Stage Pulmonary Fibrosis

Article Via; http://www.medpagetoday.com/pulmonology/smokingcopd/5805

Published: May 31, 2007

Download Complimentary Source PDF 

By  Crystal Phend , Staff Writer, MedPage Today

Reviewed by Zalman S. Agus, MD; Emeritus Professor at the University of Pennsylvania School of Medicine

save

|

A

A

Action Points

• Explain to interested patients that the study suggests that idiopathic pulmonary fibrosis may affect patients differently depending on gene expression and environmental factors, such as smoking.
• Caution patients that the study was preliminary and further study will be needed before physicians can prospectively determine which patients could experience rapid disease progression.

MEXICO CITY, May 31 -- A difference in genetic patterns may explain why some idiopathic pulmonary fibrosis patients, especially men who smoke, die more quickly after diagnosis than others do.

These rapid progressors were 6.5-fold more likely to be men and seven-fold more likely to have been smokers than slow progressors, found Moisés Selman, M.D., of the Instituto Nacional de Enfermedades Respiratorias here, and colleagues.

In the retrospective study, gene expression patterns differed between fast and slow progressors, implying biologically-distinct phenotypes of the disease, they wrote online in the journal Public Library of Science ONE.

The findings suggest that physicians should pay more attention to the time of onset of symptoms to identify these patients who are at greater risk, they said.

Most idiopathic pulmonary fibrosis patients have symptoms long before diagnosis, then slowly progress, with death coming within five years of diagnosis, they noted.

But, they said, distinct patterns of disease progression have become increasingly clear clinically.

To characterize these patterns, the researchers reviewed the charts of 167 consecutive patients with the disease who were evaluated at a single center between 1995 and 2004. Seven healthy volunteers as well as lung samples from autopsies were also studied as controls for immunohistochemistry, cellular and genetic profiling.

Rapid progressors were defined as those with no more than six months of symptoms before seeking medical attention.

From symptom onset, these 26 patients had a median follow-up of 13.5 months and median survival of 27 months. From diagnosis, median follow-up was 10 months and survival was 25 months.

Slow progressors were defined as those with at least 24 months of symptoms before presentation.

From symptom onset, these 88 patients had a median follow-up of 60.5 months and median survival of 93 months. From diagnosis, median follow-up was 17 months and median survival was 32 months.

In a multivariate analysis, significant factors in survival among the overall cohort included time from symptom onset to first consult, smoking, male gender, and lung function as measured by forced vital capacity.

Among the 80% to 85% of patients with known vital status, rapid progressors had significantly lower survival rates than slow progressors (hazard ratio 9.0, 95% confidence interval 4.48 to 18.3, P<0.001) or intermediate progressors (P=0.045).

Mortality determined from the time of diagnosis also tended to be higher in the rapid progressors (HR 1.5, 95% CI 0.81 to 2.87, P=0.18).

Among rapid progressors, significantly more patients were:

• Male (odds ratio 6.5, 95% CI 1.4 to 29.5, P=0.006).
• Ever smokers (OR 3.04, 95% CI 1.1 to 8.3, P=0.04).
• Current smokers (OR 7.1, 95% CI 1.2 to 40.9, P=0.02)

These rapid progressors, though, were not simply patients presenting at a different stage of disease or an acute exacerbation, the researchers said. Their physiologic, radiologic, and histopathologic parameters were similar to those of slow progressors.

Socioeconomic and educational background -- which can influence when patients seek treatment -- as well as initial treatment were similar between groups, they said. And there were no differences between rapid and slow progressors in pack-years smoked.

Nor were there baseline differences in age, lung function alterations, oxygen saturation, extent of changes seen on high resolution computed tomography, or bronchoalveolar lavage cellular profile, the researchers noted.

Lung biopsies done on 31% of patients showed no differences in baseline morphology for interstitial inflammation, pulmonary hypertension changes, smooth muscle hyperplasia, type 2 cell hyperplasia, or extent of fibrosis or honeycombing.

However, Dr. Selman wrote, there were important differences showing that "rapid progressors appear to represent a distinct biological phenotype among patients with idiopathic pulmonary fibrosis."

In a global gene expression analysis in a subset of patients, the researchers found that 437 genes were expressed differently between groups.

Rapid progressors overexpressed genes involved in morphogenesis, oxidative stress, migration and proliferation, and fibroblast and smooth muscle cell function.

This upregulation was seen on immunohistochemistry for the adenosine-2B receptor, which is involved in a key process of fibrotic remodeling, and prominin-1/CD133, which is found in hematopoietic stem cells and embryonic epithelium.

Furthermore, bronchoalveolar lavage showed that rapid progressors had more than a twofold increase in active matrix metalloproteinase-9, which may contribute to abnormal tissue repair and remodeling, compared with slow progressors.

Rapid progressors also had higher fibroblast migration than slow progressors (238% versus 123%, P<0.05) or controls (238% versus 30%, P<0.01).

While these subgroup studies were of limited size, "the relatively stringent selection of genes, the protein verification by immunohistochemistry on additional samples, and the biological relevance of the genes suggest that our results are biologically meaningful," the investigators wrote.

They also noted, however, that their study was preliminary and limited by retrospective data collection and dependence on patient recall of symptom duration.

However, "taken together with reports of the impact of acute exacerbations of idiopathic pulmonary fibrosis on morbidity and mortality, our results further highlight the variability in the progression and outcome of [the disease]," they concluded.

"These findings may explain, in part, the difficulty in obtaining significant and reproducible results in studies of therapeutic interventions in patients with idiopathic pulmonary fibrosis," they added.

The study was partially supported by a grant from the Universidad Nacional Autónoma de México. One of the researchers was supported by grants from the National Institutes of Health and by a donation from the Simmons family. The researchers reported no conflicts of interest.