Tuesday, December 15, 2015

Since You've Been Gone~ 4 Years

Hey Babe,

It is hard to believe that 4 years have passed since you left us.  In a way, I know your not very far away and I can feel your gentle support as I make my way through life.  

When you first left, I remember sleeping.  I slept as I never had and someways, now that I think about it, I probably sleep-walked through the entire first year.  It was probably my body's way of recovering.  As time passed, I, then had trouble sleeping without you.  Staying up to the wee hours of the night and awakening before dawn.  Each day was always a reminder that you were not there.  

I made it a point to take road trips.  Less about the destination and more about the desire to prove to myself that I was a capable woman.  During the second year, I got myself a camera.  Nothing too expensive, but it suits me fine.  Since then, my camera has been my little companion.  I have always been creative, but when we went through your illness, I wondered if that part of myself had been lost.  It might sound strange but the camera saved my life.  I enjoy the beauty that unfolds each day and I enjoy the patience of photography. Being still.  

I still have a hard time with what seems like our shrinking family.  My oldest is now out on his own, following his dreams and working hard at it.  The youngest, is a teenager now.  Very thoughtful and attentive, although, as you know with teenagers, that only can go so far as they really need their space.  I try to remind myself not to rely too much on him and to learn to find contentment in my own space.  It is something I work on daily.  It is scary to think that I am completely on my own.  I think the hardest part about it is not being able to share things with another who understands me.  Certain thoughts and experiences to only be kept to myself.  I have found though, there is a beauty in learning to thrive within the serenity of my own soul.  

I have taken a few chances.  Recently, I bought a very old cottage to renovate.  One day, it might be my downsized home.  When I am there, I feel happy.  There is something about having a home that is just mine.  It is the first place that I have owned that I have not shared in raising a family, or as a married person. Lately, I go there and paint on a canvas.  Just simple abstract stuff.  But, again, that is another thing that I stopped doing when we went through your illness. It feels really good to blast music and just mindlessly paint.  It seems cleansing and healthy to me.  

I am trying to make the conscious effort to be a healthy person.  Not necessarily physically, although that is also coming along.  But more about, up here, in my head.  One of the best things I did recently is decide, that I do not want to be a martyr.  Yes, I am a widow, and yes, only you and I know the ways in which I still grieve.  At the same time, I have chosen not to be "In Mourning" my entire life.  It was a personal choice that needed to be made.  Since for whatever reason, I happen to still be walking on this Earth, should I allow my entire existence to be determined by your death?  I want to celebrate in my existence.  For instance, everyday, I have been playing music.  Recently, I started to dance to the music.  Really dance.  To feel my body come alive, to feel sexy, female again, elevated my heart.  I did this without guilt.  I found myself happy for a moment and I have decided that is okay.  I am still here, still ~Breathing.

I love you, Baby.     

Sunday, November 22, 2015

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

Sunday, October 18, 2015

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  

Thursday, September 24, 2015

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

Monday, September 14, 2015

5 Ways to Help the Caregiver In Your Life Written By: Ann Napoletan

Article Via; Caregiver's Blog 
Written By: Ann Napoletan
If you think you are too small to make a difference, try sleeping with a mosquito.  ~His Holiness, the 14th Dalai Lama
It’s true – there are those who insist one person can’t make a difference in this world. However, having recently been through an extremely difficult time, I have to respectfully disagree. In fact, I can say with complete confidence that each one of us holds the power to do wonderful, often simple things that can categorically change the course of another human being’s day.
If you have ever tried to extend a helping hand to a caregiver, you’ve likely found that they have a very hard time accepting assistance. Nurturers by nature, they’re used to offering support, but really struggle when they find themselves on the receiving end. Most caregivers don’t want to be a bother or appear the least bit needy, so even when people willingly offer, they have a natural tendency to smile and politely decline.
That doesn’t mean you should stop trying. Here are 5 simple things you can do to bring a smile to the face of the caregiver in your life.
  • Wintertime is perfect for sharing comfort food. Just about anyone would welcome a crock pot of hearty soup or a steaming hot casserole. These things not only make for a wonderful dinner, but will likely provide enough for a few lunches as well. Other ideas: Next time you’re cooking a meal, double the recipe and deliver the extra portions to your friend with reheating instructions. Don’t cook? How about sending a gift card for a favorite local eatery?  Be sure to take any special dietary needs into consideration.
  • Would your favorite caregiver enjoy a relaxing massage? Consider going in with a friend to purchase a gift certificate, and present it along with a “coupon” offering to provide respite care while she enjoys an hour or so of pampering.
  • Is your caregiver friend a member of the ever-growing sandwich generation? If so, make a play date with her kids. Perhaps you can pick them up from school for a movie and dinner, or how about a Friday or Saturday night slumber party with pizza, popcorn, and board games?
  • No matter the season, most caregivers could use a little help around the house. Some ideas include shoveling snow, raking leaves, trimming, or mowing the lawn.  Have you heard your friend mention any household “fix it” jobs that are screaming for attention? Maybe you and a few others can pitch in and hire a handyman service for an afternoon.
  • It doesn’t get much easier than this! Next time you’re out, simply pick up a heartfelt greeting card. Write a personal note inside and put it in the mail. Everyone loves finding a card in the mailbox; knowing that someone is thinking about you during a difficult time can make all the difference in the world.
These are just a few things you can do for the caregiver in your life; there are lots of other ideas. Remind them to take care of themselves. Get creative! Bring them a new journal, a pretty indoor plant, or just a good cup of coffee and their favorite indulgent dessert. Not only will it make someone smile to know you care, but you’ll be fulfilling a need they may not have realized they had. Most importantly, never underestimate the power of a kind word or caring gesture, no matter how simple. I promise it will make a difference.

Thursday, September 3, 2015

Idiopathic pulmonary fibrosis: now a treatable disease and other highlights from the 2014 American Thoracic Society Annual Conference

by Matthew Stanbrook, Deputy Editor, CMAJ, in San Diego, U.S.A.
Article via; Healthy
Idiopathic pulmonary fibrosis is a chronic progressive disease in which the normal connective tissue of the lungs (the interstitium) is replaced by scar tissue through mechanisms we are rapidly understanding much better. The disease causes the lungs to become stiff (decreased lung compliance), small (lung restriction) and impaired in their primary function of gas exchange (impaired diffusion capacity). It affects an estimated 20,000-30,000 Canadians and has a median survival of 2-4 years from diagnosis, although some patients live much longer. While its onset is typically late in life, the disease can rarely occur much earlier, as attested by my hospital’s lung transplantation program, whose graduates include triumphs like social media superstar and Ellen DeGeneres guest Hélène Campbell, as well as tragedies like Cambridge, Ontario’s organ donation advocate Kayla Baker, who despite getting a new lung died earlier this year at the tender age of 15. I have had the privilege of crossing paths with both of these young women, whose stories are inspiring, yet serve as a stark reminder of the desperate need for effective treatments for this disease.
Patients with idiopathic pulmonary fibrosis, and those of us who care for them, have long been frustrated by the lack of such treatments, and more so by the fact that most innovative therapies tested to date have failed to show benefit and have often increased rather than decreased mortality. All this has suddenly changed with new research presented last week at the 2014 American Thoracic Society Annual Conference. These studies have now documented clear and compelling benefits of two medications, one of which is already in clinical use in Canada. Another equally important study has shown the lack of benefit of an older but widely used treatment.
Let’s start with the bad news. For decades, we had been treating idiopathic pulmonary fibrosis with potent systemic anti-inflammatory medications such as prednisone, azathioprine, and cyclophosphamide, based on the observation that chronic inflammation is a consistent feature of the disease, but likely the wrong mechanism on which to focus, as it turns out. We did so well in advance of obtaining any good evidence of benefit with this approach, which as usual turns out to have been a bad idea. In 2011, the large PANTHER IPF trial, evaluating combination therapy with prednisone, azathioprine and acetylcysteine, was stopped early by its data safety monitoring board due to findings of increased mortality with this regimen. In addition to a placebo arm, this trial had a 3rd arm that was randomized to acetylcysteine alone. After a brief hiatus of 3 months, the PANTHER IPF trial was allowed to continue with these latter 2 arms.
Acetylcysteine (also referred to as N-acetylcysteine or NAC) was thought to be possibly beneficial in idiopathic pulmonary fibrosis based mainly on theIFIGENIA study, which in 2005 showed that patients randomized to acetylcysteine, prednisone and azathioprine had less of a decline in vital capacity (a validated surrogate outcome measure in idiopathic pulmonary fibrosis) than those randomized to prednisone and azathioprine alone. Tellingly, IFINGENIA did not have an arm that was untreated, and in retrospect it seems more likely that acetylcysteine may have decreased the tendency for prednisone and azathioprine to kill patients, although given PANTHER IPF’s initial findings, clearly not well enough. Acetylcysteine’s purported beneficial mechanism lay in its property as the biological precursor of glutathione, which functions in the lung as a major antioxidant. That should have been a red flag right there—more often than not, “antioxidant properties” seems to be scientific code for “we really have no idea how this treatment would actually work” and denotes a treatment that turns out not to once proper studies are done. The final results of the PANTHER IPF trial, presented at the conference by Ganesh Raghu, Professor and Director of the Interstitial Lung Disease/Sarcoid/Pulmonary Fibrosis Program at the University of Washington, proves that acetylcysteine doesn’t work, either: there was no difference between patients receiving acetylcysteine or placebo in vital capacity (the primary outcome), death, or other clinical or surrogate endpoints that might denote benefit. There was an excess of non-fatal cardiac events in the acetycysteine group—of unclear meaning given the large number of outcomes evaluated, but certainly not encouraging.
As respirologist and New England Journal of Medicine Editor-in-Chief Jeffrey Drazen stated at the conference, there was never a good scientific reason to think that acetylcysteine alone would work in idiopathic pulmonary fibrosis; instead, “this was a straw at which people grasped”. Unfortunately, it seems a few are still grasping: while most of the IPFnet investigators were balanced and appropriate in describing the trial results, PANTHER IPF investigator and Executive Vice Chair of Medicine at Weill Cornell University Fernando Martinezdevoted much of a separate session at the conference to highlighting observed differences in “trends” between patients enrolled before and after the 2011 alert that temporarily halted the trial—notwithstanding that none of the differences reached statistical significance. This is specious and unscientific. As PANTHER IPF investigator and Chair of Medicine at the University of California, San Francisco, Talmadge King—arguably the world’s leading authority on interstitial lung diseases—stated, “I don’t know how you make a big deal over something meaning nothing”. Right you are, Dr. King. Acetylcysteine has fallen into common use, in part because it can be widely obtained without prescription as a supplement, and perhaps because it is thought at least to be harmless (which, as it turns out, may not be the case). We all need to move on and to encourage our patients to move on as well, not to waste their money and their hope on a treatment that doesn’t work.
And we finally have an effective therapy towards which to move: pirfenidone. Pirfenidone was previously tested in the 2 large CAPACITY randomized trials. Unfortunately, only 1 of the 2 trials showed a significant difference in the primary outcome, vital capacity. This was good enough to get the drug approved for clinical use in Canada and Europe, but not good enough for the U.S. Food and Drug Administration, which refused to approve the drug and mandated a new study. The results of this study, the ASCEND trial, were present at the conference by Dr. King. Unlike its predecessors, the ASCEND trial featured greater attention to quality control, with centralized adjudication of pulmonary function measurements and clinical events. I have been skeptical of pirfenidone to date, given the past track record of treatment for idiopathic pulmonary fibrosis that I described above, and also because we still don’t know the mechanisms by which this treatment acts in IPF (remembering the example of acetylcysteine). However, the results of ASCEND seem quite convincing: the proportion of patients who died or experienced a significant decline in vital capacity after 1 year was lower in the pirfenidone group by an absolute difference of 15% (equivalent to a number needed to treat of 7). When the results of ASCEND were pooled with those of the two CAPACITY trials, the findings were homogeneous and revealed a statistically significant 48% reduction in the hazard of all-cause mortality and a two-thirds reduction in mortality attributed specifically to pulmonary fibrosis.
In addition to pirfenidone, we will soon have a second therapy with demonstrated efficacy in idiopathic pulmonary fibrosis: nintedanib. Unlike pirfenidone, we actually know how this one works: it’s a tyrosine kinase inhibitor, a class of medications that has spawned several new anti-cancer drugs recently. Its mechanism, as helpfully explained at the conference byMartin Kolb, Associate Professor of Respirology, Pathology and Molecular Medicine at McMaster University, is that, as with all tyrosine kinase inhibitors, it mimics ATP and thereby prevents cellular signaling from the cell membrane to the nucleus. Of specific relevance here, nintedanib does so in fibroblasts, inhibiting their migration, proliferation and transformation to myofibroblasts—all key steps in the pathogenesis of idiopathic pulmonary fibrosis, which best resembles a misdirected process of wound healing and repair. Nintedanib has now been evaluated in the paired INPULSIS-1 and INPULSIS-2 trials, presented at the conference by Luca Richeldi, Professor of Respiratory Medicine and Chair of Interstitial Lung Disease at the University of Southhampton, UK. As with pirfendione, nintedanib showed a clinically and statistically significant different in vital capacity at 1 year. Deaths were also fewer with nintedanib, although differences did not reach statistical significance (the trials were not powered to evaluated mortality).
These new findings have immediately sparked much well-justified optimism within the respiratory community. For the first time, we can now confidently regard idiopathic pulmonary fibrosis as a treatable disease. There remain many substantial hurdles to overcome for our patients: as Dr. Drazen pointed out, these drugs have shown they can slow the progression of disease, but in most cases haven’t stopped it. There is thus immediate interest in studying these drugs in combination with each other and with other promising agents in development that have been derived from an improved understanding of disease mechanisms (such as simtuzumab, an inhibitor of lysyl oxidase like-2, an enzyme whose activity has been implicated in the formation of fibroblast foci, which are a pathologic hallmark of idiopathic pulmonary fibrosis). Both drugs have common side effects, particularly involving the GI tract, although these seem tolerable in severity. And both, of course, will be expensive. However, with clear benefits and a mortality reduction now documented for a frequently fatal disease that has previously had no clearly effective treatments, regulatory authorities, insurance companies and government third-party payers will have a hard time saying no.
Of course, there was more than idiopathic pulmonary fibrosis on display at the conference. Other research highlights included:
  • Among women who continued to smoke during pregnancy, infants of mothers who were randomized to Vitamin C 500 mg per day had better lung function at birth and fewer episodes of wheeze at 1 year than those whose mothers were randomized to placebo and similar in both respects to infants of non-smoking mothers. Benefits were seen predominantly with mothers who carried a known nicotinic receptor polymorphism previously shown to be associated with smoking-related lung diseases. Noteworthy for being one of the few randomized trials to show that Vitamin C does anything worthwhile in anyone, but the biological rationale appears to have been much better worked out in this case and lends plausibility to the findings. Of course, the most important and effective treatment in this context is smoking cessation, but the evidence that the mechanism of neonatal lung impairment may be based on effects of nicotine would imply that pregnant women using nicotine replacement therapy for this purpose should perhaps also receive Vitamin C.
  • E-cigarette vapour increased the virulence of methicillin-resistant Staphylococcus aureus (MRSA) in a mouse model. This was similar to the effect of cigarette smoke, although the mechanisms were different. The results provide more evidence suggesting that e-cigarettes are not a harmless substitute for cigarette smoking.
  • Statins do not reduce COPD exacerbations, it seems. In the STATCOPE trial, a unique collaboration funded by both the Canadian Institutes of Health Research and the U.S. National Heart, Lung and Blood Institute, simvastatin 40 mg daily, given for 1-3 years to COPD patients who did not otherwise have an indication for a statin, showed no obvious benefits or harms. Similarly, in the RODEO trial, rosuvastatin 10 mg daily for 12 weeks did not improve pulmonary or peripheral artery endothelial function. These studies thus fail to confirm prior observational data suggesting that statins might be beneficial in COPD. Oh, and it seems thatstatins are of no benefit in ARDS associated with sepsis, either.
  • Lung volume reduction for COPD, attempted endoscopically with a gelfoam sealant, more than doubled the risk of respiratory adverse events, including 2 deaths, leading to the premature termination of the ASPIRE trial. Conventional lung volume reduction performed surgically has beenshown previously to be of potential benefit, but only in a small proportion of highly selected COPD patients. Hopes that this therapy might be expanded to more patients with less invasive approaches continue to meet with frustration—even if the idea is sound, the right technique just isn’t there yet, it seems.
  • Vitamin D supplementation in asthma patients who were Vitamin D deficient did not reduce asthma treatment failure or facilitate dose reduction of inhaled steroids, even though it did usually raise Vitamin D levels above the threshold for sufficiency. The hypothesis was based on prior studies suggesting that Vitamin D deficiency is associated with worse asthma outcomes. Vitamin D deficiency has been implicated in the pathogenesis of several diseases in which Vitamin D supplementation has subsequently failed to demonstrated benefit. Perhaps asthma should now be counted among these, although in fairness, the VIDA trial really shows only that Vitamin D cannot replace an effective dose of inhaled steroids, rather than ruling out any potential benefit of Vitamin D in asthma.

Saturday, July 25, 2015

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. 

Thursday, June 4, 2015

I'd Be Lying

If I said I had never been in love
I'd be lying, 
Sailing on an open road,
Beneath a blanket of stars,
Glimmering light reflect in our eyes,
Unspoken secrets dance in our hearts,
Whisper trust,
Between promising souls,
If I said I had never been in love,
I'd be lying.

If I said I didn't miss you
I'd be lying,
Mapping lines around your eyes,
Lost in the turn of your smile,
Intertwined and connected,
Your hand in mine,
If I said I didn't miss you, 
I'd be lying.

If I said I didn't believe in you,
I'd be lying.
Between the grey and blues,
A night bird coos,
Familiar melodies floating in air,
On softness of moonlight,
I know you're there,
If I said I didn't believe in you,
I'd be lying.

Happy Anniversary, Baby.  I miss you.

Saturday, February 28, 2015

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

5 Things Every Person Living with a Rare Disease Understands

Blogger Rachel Wilson
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.

Saturday, February 14, 2015


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~~~~


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