Saturday, December 20, 2014

~~~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.  

Thursday, November 27, 2014


Thanksgiving Day and so much to feel blessed for~ I feel blessed for nature and all of its beauty, for kind people with warm hearts who are thoughtful of others, living a life full of adventure, laugher~ I love laughter, my children~ that they are creative and healthy, and that I am here~ still Breathing.  I hope you have many reasons to feel blessed today  Happy Thanksgiving!

Monday, November 24, 2014

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.**  

Wednesday, October 22, 2014

Pulmonary Fibrosis and Panic Attacks

Over the years, I have heard many people with Pulmonary Fibrosis mention that they experience severe panic attacks.  It seems understandable that this would occur because many have difficulty breathing as part of their illness.  When I Google or look up panic attacks, many of the writings are geared toward people who generally have these experiences, but I cannot find much in writing or resources that specifically deal with this occurring while on oxygen and having a terminal illness.  

Though panic attacks can be severely debilitating, it seems that much of the literature indicates to face the fears you have and realize that the percentage of them actually coming true is very low.  With Pulmonary Fibrosis, I think this does not apply, as often the source of the panic has to do with the fear of not getting enough air, which is a very real scenario to PF patients.

I remember my husband experiencing quite a few of these attacks.  Some where quite severe.  He would flail his arms in desperation of getting more O2, but in reality, he was pulling the O2 out of his nose and knocking down canisters.  Another time he was so desperate for more air, that although he had a portable O2 container turned on and in his noes, that he picked up the portable and began trying to suck air out of the handle itself.  It was very heartbreaking to see.

As a caregiver going through this with him, I had to learn to keep calm and be very deliberate in my voice and actions.  No rushing, although he was in panic mode, I could not allow myself to rush about the room. Why?  If his surroundings were chaotic during these episodes, it only made the situation worse. If he could not be calm, then I had to for the both of us.  

Touching him during this time had to be thought out.  Rather than rubbing his back, I would place my palm flat on his back and hold it still.  Everything had to come down a notch, including my voice.  Speaking in a low, soft tone can be more helpful rather than an elevated voice.    

Helping him to focus assisted in bringing down the heightened nature of a panic attack.  If he was scared he didn't have enough air, I would put the pulse-oximeter onto his finger for both of us to check it.  Getting him to look at it helped gain focus.  If his SAT's were low, I turned up his O2 and gently let him know they will start rising soon.  Let's watch them rise together.  

These attacks sometimes came during a coughing episode, or would bring a coughing episode on.  By the time they passed, all of his energy was spent.  It took so much out of him to go through this.  I wanted to minimize this happening and looked at the different scenarios in which they occurred in the past.  I tried to notice patterns.

I noticed that many of them occurred before his showers.  Showering becomes a difficult task for someone with PF.  The moisture and steam in the air, the use of your arms to lather, the slipperiness of the tub, the water on the floor, the energy used to dry off, all become monumental.  There was no such thing as a quick shower anymore.  Understanding that and blocking out an appropriate amount of time eased the task.  As a caregiver, making sure everything was ready for him also helped.

We were provided a slew of drugs while he was on hospice. One of them was a liquid dose of Larazapam.  This was specifically provided to ease anxiety.  In this form, it did help but was something that worked in only the short-term and the dose might be given several times a day, depending on what kind of a day he was having.  At first, when all this was new to us, we administered a dose during his panic attacks as a way to help him calm down.  As time passed and we recognized the patterns of his panic attacks, we realized that the best time for him to take this was prior to his shower.  Before he felt the anxiety.

Everything we did had to do with preventing them in the first place.  Thinking ahead of any given situation, allowing the proper amount of time and preparing in advance seemed to help the most.  As he changed, we were flexible enough to change the preparations to assist his needs.

Everybody is different.  One thing I noticed and found rather unexpected, was that Morphine worked the opposite than what we thought it would do.  We thought it would help with his pains and help him to relax.  It did help with pain, although I noticed a pattern with that as well.  His form was a liquid Morphine taken orally.  At first administration, he would become somewhat sleepy, so we found that, for him, taking it after a shower and lunch would allow for a nice little nap.  I noticed that after an hour or two, he would become restless in his sleep and literally wake up in a panic.  While he was at this period, he suddenly felt he needed 5 things all at once and at first, not seeing the pattern of his reaction to this drug, I would run around the room trying to satisfy his every need.  It took a little time to recognize this pattern and having something soothing for him when he awoke, such as a cup of tea, seemed to decrease this.  

I have also read that certain foods may increase the possibility of panic attacks.  Too much sugar or caffeine might bring them on more frequently.  

Much of this is written from a caregiver's standpoint, yet, there are many people with PF who experience this and do not have any assistance.  This can be very frightening.  It might be a good idea to visit with your doctor to discuss these attacks if they are too frequent, or preventing you from daily enjoyment.  My husband was not on an anti-depressant, although some people say that this has helped them a great deal. Having a phone near-by or discussing this with your friends or family may give you additional support.  Of course, learning to change your focus from what is causing the panic to something else would help, but is one of the hardest things to do.  

Here are some links that may provide further insight (I will put a question mark after each topic, because not all of these will be helpful to everyone, but, maybe there is one for you.)  Be sure to speak with your doctor regarding any questions you may have before starting medications or natural supplements.  



Points of Focus? 



Drug options for treating Anxiety/ Depression-


Peer Support?  

Pulmonary Fibrosis Foundation Support Groups-

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:

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.