Tuesday, September 30, 2014

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. 

Monday, September 29, 2014

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.

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

Sunday, September 28, 2014

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

Oxygen Therapy
Pulmonary RehabilitationIncludes a range of conditioning and breathing exercises
The goal is to help patients function to the best of their ability
Oxygen Therapy
Oxygen TherapyRecommended for patients who have low oxygen levels
May help reduce breathlessness, enabling the patient to take part in pulmonary rehab exercises
Lung transplant
Lung TransplantCan 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
Clinical TrialsTaking 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.

Saturday, September 27, 2014

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.  

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.

Friday, September 26, 2014

Rapid Progressors Speed to End Stage Pulmonary Fibrosis

Published: May 31, 2007
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.

Thursday, September 25, 2014

Pulmonary Function Test (PFT)

Many people diagnosed with lung restricted diseases are referred to take a Pulmonary Function Test (PFT). National Jewish Health gives an over-all description of what the test will entail.

Here is also a link that will help you understand the results of your PFT:

Tuesday, September 23, 2014

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!

Monday, September 15, 2014

More of us need to know about pulmonary fibrosis~ via; Letters to the Editor, AZ Central

September is Global Pulmonary Fibrosis Awareness Month.
It seems every dread disease has a special month — and pulmonary fibrosis is no different. Some stats about this disease:
• This disease is 100 percent fatal.
• Each year, 40,000 will die from PF, about the same number of fatalities as from breast cancer.
• There is no known cause or treatment, and the disease is relentlessly progressive; average lifespan from time of diagnosis is two to five years.
• PF patients gradually lose the ability to process oxygen as their lungs fill with scar tissue and become stiff. Oxygen can't pass into the blood.
• Four times as many people have PF as ALS or cystic fibrosis.
• Since 1999, the number of patients with PF increased by 156 percent, to more than 128,000, and more than 50 percent of the cases are misdiagnosed for a year or more.
• A lung transplant is the only option to extend life, but 50 percent of those on the list will die before receiving a transplant.
• There is only one PF support group in Phoenix and the surrounding area.
• St. Joseph's Hospital and Medical Center is doing research on medications for PF.
My husband was recently diagnosed with PF.
More information is available at pulmonaryfibrosis.org.
— Madeline Wollmer,

Thursday, September 11, 2014

Pulmonary Fibrosis Patients/9/11 Responders Represented at State of the Union Address for Second Year in a Row ~Via; Coalition For Pulmonary Fibrosis

Deadly Lung Disease Claims as Many Lives Each Year as Breast Cancer, Yet Largely Unknown
San Jose, Calif. – January 28, 2008 – For the second year in a row, pulmonary fibrosis patients will be represented at the President’s State of the Union address.  Just a year ago, Caesar Borja, Jr. attended President Bush’s State of the Union speech, just hours after his father lost his fight to live as he awaited a lung transplant for pulmonary fibrosis.  Tonight, another pulmonary fibrosis patient’s family member will attend the State of the Union address.

Joseph Zadroga will attend tonight’s address in memory of his son, James Zadroga who died in 2006 to pulmonary fibrosis.  Both Zadroga and the senior Borja were members of the New York Police Department during 9/11 and are believed to have been exposed to toxins that could have caused their pulmonary fibrosis, an incurable and deadly lung disease.  According to reports, two pathologists agreed that Zadroga’s death to pulmonary fibrosis was linked to ground zero contaminants.

“It is a tragedy that every day, hundreds of people lose their lives to this horrific disease,” said Mishka Michon, Chief Executive Officer of the Coalition for Pulmonary Fibrosis.  “It is our hope that these brave family members can help increase awareness of this deadly disease that receives little funding and little attention from the federal government.  We are working hard to find treatments to take this disease from terminal to treatable.”

Cases of pulmonary fibrosis are on the rise with more than 150 percent increase in incidence and prevalence of the disease since 2001.  There is no FDA approved treatment and no cure for the disease that claims 40,000 lives each year, the same as breast cancer.  More than 128,000 people suffer from pulmonary fibrosis and most will die within three years of diagnosis.

Monday, September 1, 2014

September 2014 Pulmonary Fibrosis Awareness

~~September is Pulmonary Fibrosis Awareness month. If you enjoy 'Breathing' the page or the actual, literal term, please join me in helping to spread awareness for this deadly disease that steals the breath of people~ young, old, and in between. There is no cure for this terminal illness and very little funding to find a cure. Although each year 40,000 people die from Pulmonary Fibrosis in the U.S. alone, many people have not heard of this disease. For more information on how you can help, please visit:http://www.pulmonaryfibrosis.org/ ~or~ http://www.coalitionforpf.org/