29 posts in the category “Idiopathic pulmonary fibrosis”

Shining the spotlight on pulmonary fibrosis

Posted September 17th, 2014 by

 

You may have seen our post on Pulmonary Fibrosis Awareness Day, but did you know September is also Pulmonary Fibrosis Awareness Month? The Pulmonary Fibrosis Foundation (PFF) wants everyone to shine the spotlight on idiopathic pulmonary fibrosis (IPF), and many PatientsLikeMe members have already shared their stories – Lori documented her journey from diagnosis to transplant in a 4-part blog interview series, along with Barbara and John.

We also wanted to pause for just a minute to recognize PatientsLikeMe member Bryan. He joined the community in 2013 after being diagnosed with IPF and was an inaugural member of our Team of Advisors.

We are sad to share that last month, Bryan passed away, and all of our thoughts are with his family, friends and fellow PatientsLikeMe community members. But even though Bryan may no longer be with us, his memory and data live on. The experiences he shared will help drive change in IPF and in healthcare. We can think of no better way to pay tribute than to share his recent video with everyone. Bryan, you will be missed.

In Memoriam
Bryan Kincaid


(1947-2014)


Let the world know about pulmonary fibrosis

Posted September 7th, 2014 by

On Rare Disease Day back in February 2013, we announced our partnership with Boehringer Ingelheim to help enhance the online idiopathic pulmonary fibrosis (IPF) community. And by September 7th that same year, the community had grown to just over 1,000 people. Today, on the next edition of Global Pulmonary Fibrosis Awareness Day, the community stands 2,500+ members strong, making it the largest online gathering of IPF patients anywhere in the world. In just a year, the community has almost tripled in size, and everyone is sharing about their experiences so that other patients, doctors and researchers can learn more about life with IPF.

But what exactly is IPF? Pulmonary fibrosis (PF) is a medical condition that causes lung tissue to thicken, stiffen and scar over a period of time, and “idiopathic” means “no known cause.” According to the Coalition for Pulmonary Fibrosis, there are over 100,000 Americans living with IPF at any given time, and an estimated 40,000 will die from the condition every year. And besides a complete lung transplant, there is no known cure for IPF.1

Today, the Pulmonary Fibrosis Foundation (PFF) is encouraging everyone to educate, share, fundraise and start conversations about IPF. You can learn more about how to get involved through the PFF’s toolkit and guidelines for September.  And if you or someone you know has been diagnosed with IPF, join the community at PatientsLikeMe – let’s change who knows about this condition and promote a better understanding of IPF all year round.

Share this post on twitter and help spread the word for pulmonary fibrosis awareness.


1 http://www.coalitionforpf.org/facts-about-idiopathic-pulmonary-fibrosis/


“No oxygen.” PatientsLikeMe member Lori shares about life after surviving idiopathic pulmonary fibrosis

Posted August 3rd, 2014 by

It’s crazy to think how fast things can happen. The last time we talked with Lori, she was telling us about life on the lung transplant list and playing what she called “the waiting waltz.” And now – everything has changed. Just two weeks after we posted her third interview on the PatientsLikeMe blog in mid April, Lori got the call – they had a set of lungs for her. We caught up with Lori one more time, and fifteen weeks post transplant, she’s nothing but smiles. Check out what she had to share and don’t forget to follow Lori on her own blog called Reality Gasps. (Thank you Lori for being so open about your experiences with IPF!)

 

 

What was your reaction when you got ‘the call?’ You mention a whole range of emotions on your blog.

The call took me completely by surprise. I’d been admitted to the hospital a week earlier because we just couldn’t meet my oxygen needs at home any more. I was literally 10-minutes away from starting a procedure to suppress my antibodies (to help increase my somewhat slim chances of finding a match) when the nurse walked in and told me they might have lungs for me.  Actually she said they “might, might, might, might, might” have lungs – normally they wouldn’t have contacted me that early in the process, but they had to cancel my procedure just in case. I felt an initial jolt of adrenalin and couldn’t help busting out in a huge grin. I quickly tamped down my euphoria, though. There was still a long way to go and anything could happen.

An hour later when I heard we were moving forward, I burst into tears. A year’s worth of pent up emotion poured out through those wracking sobs. I couldn’t believe my miracle was so close. But wrapped up in my joy was guilt that my chance to live was coming through the death of another.

Until the moment they put me under, my emotions continued to cycle from near manic joy, to fear that the lungs wouldn’t be right, to anguish for my donor and his family. Everyone says it’s a roller coaster, but like parenthood, you can’t really understand what that means until you live it.

What happens between getting the call and going into surgery?

Since I was already in the hospital, there was no mad dash for me. When the team decided to move forward, I was sent down for pre-surgery testing — a chest x-ray and blood work. Then we waited, and waited for a good 12 hours. Finally I was sent down to pre-op at 2:00 AM to get ready for surgery at 3:00. They inserted an arterial line and an IV. And they checked and rechecked my drug allergies and medical history. I was the only person in pre-op, but I still had to recite my name and birth date for everyone who came by!

My surgeon arrived and told me he’d heard about my surgery early enough to make sure he got a good night’s sleep with plenty of time for breakfast. I’d brought a disposable camera to get pictures during surgery, so he talked me through his typical “shot list”. I couldn’t wait to see those beautiful new pink lungs!

Just before I was to head to the OR, the doc got a call from the retrieval team that we were delayed for an hour. He said this was very common — they had to wait for all of the teams to arrive before they could start. We were delayed twice more as teams continued to fly in. When I finally made it to the operating room, I was surrounded by people and equipment. They explained each step as they got me situated on the table, and then the anesthesiologist placed a big mask over my face. Thankfully, I remember nothing until they removed the breathing tube three days later.

Fifteen weeks post transplant – how’s recovery and rehab going?

Full recovery takes about a year, but I am astonished at how far I’ve come in just three months. I went into surgery pretty weak because I didn’t have the energy (or the breath) to move much.  A lot of people told me to keep my legs as strong as possible and I quickly learned why. Post-surgery, I was on high-dose prednisone, which is very hard on the quadriceps – my thighs felt like jelly. Most people are up and walking shortly after their breathing tube is removed. I ended up needing a trach, so it was about a week before I took my first steps. I walked nearly 200 feet that first day and increased my distance and strength every time I hit the halls.

The day after I left the hospital, I returned for my first out-patient rehab appointment. I walked 15 minutes on the treadmill at a smokin’ 0.5 mph. Within a few days, I was up to 30 minutes and started to increase my speed. Now I’m walking two miles a day around the neighborhood and am working hard toward a 15-minute mile. It’s the hardest work I’ve ever done, but it is so worth it!

The one thing that really took me by surprise was the mental part of rehab. Before transplant, my body worked very hard to breath.  Shortness of breath meant my body needed more oxygen. But after transplant, neither one of those was true anymore. It was difficult to accept that I didn’t need to consciously breathe – my lungs could take care of that on their own.  After a week, I would periodically “forget” to breathe and be surprised to discover my lungs were working just fine. When I was walking laps around the halls, I would periodically get short of breath and start to panic – there was no valve to turn up my O2!  The pulse oximeter showed my sats were well into the 90s. I was short of breath because my body was weak, not my lungs. Thankfully, breathing is once again second nature!

What comes to mind when you think back on everything you’ve gone through – diagnosis to transplant?

I went through the same fear that most people do when they are diagnosed with IPF. The prognosis is pretty bleak: no treatment, no cure, progressive. But I decided early on that I was going to live my life as normally as possible – and do everything I could to prepare myself ready for transplant.

I continued to work for nine months, using oxygen at the office. But after two pneumonias, it was clear that my body was too fragile for the daily grind.  Without work distracting me, priorities and perspectives shifted. Everything suddenly had a sense of urgency. There might not be time “some day” to get back in touch with that friend, or tell my brothers how much they really meant to me. I had to let go of the things that had consumed me, like worry and regret, so I would have time and energy to do work on my life “to do” list.

I worried that after transplant, when things returned to normal, I would soon forget the lessons I’d learned. This whole experience has been a journey toward gratitude, and I realize now I will never be the person I was before – physically, emotionally or spiritually. Every morning I give thanks for my first waking breath. Throughout the day, I find the most delightful surprises – like watching a mama squirrel move her babies to a safer home. And sometimes I just stop and marvel at the air passing in and out of my lungs, lungs that once breathed in someone else’s chest. I spent three years quietly saying goodbye to all the things and people I loved. Now I get to spend the rest of my life saying “hello” again!

Additional comments from Lori

We just returned from our celebration trip to Sanibel Island, one of my favorite places in the world. For the first time since my diagnosis, I was able to walk the beach without tanks in tow. The image of Sanibel’s shell-strewn beaches and the memory of that rich briny air kept me pushing forward as first I struggled to breathe in ICU, and then sweated my way through PT and rehab.

At least once a day while we were there, my husband whispered to me “No oxygen”. It felt like a dream, a wonderful, amazing dream. We’ve been going to Sanibel for 25 years. It’s a huge part of our family tradition, and making it back there was an important milestone for me. A plaque on the wall at our beach condo read “If you’re lucky enough to be at the beach, you’re lucky enough.” That’s pretty much the way I feel about everything now!


The Patient Voice- PF member Bryan shares his story

Posted July 10th, 2014 by

 

Since we announced #dataforgood back in March, many PatientsLikeMe members have been sharing about why they donate their own health experiences. Becca (fibromyalgia) and Ed (Parkinson’s) already shared their stories, and now we’re hearing from Bryan, an idiopathic pulmonary fibrosis (IPF) member. Check out his video above. Miss Becca or Ed’s? Watch them here.

Share this post on twitter and help spread #dataforgood.


“Gee, doc, ya think?” – Barbara speaks about her diagnosis and life with IPF

Posted May 19th, 2014 by

PatientsLikeMe member Barbara (CatLady51) recently shared about her journey with idiopathic pulmonary fibrosis (IPF) in an interview with us, and she spoke about everything from the importance of taking ownership of managing her condition to how she hopes to “turn on the light bulb” by donating her personal health data. Read her full interview about living with IPF below.

Some PF members report having difficulty finding a diagnosis – was this the case with you? What was your experience like? 

My journey started back in 2005, when after my first chest cold that winter, I was left with severe coughing spells and shortness of breath. An earlier chest x-ray didn’t indicate any issues, so I was referred to a local community-based respirologist (what we call a pulmonologist here in Canada) who wasn’t concerned with my PFT results. I also had a complete cardiovascular workup, again with no alarming results.

Then, in 2008, I had another chest cold. Growing up in a family of smokers and being the only non-smoker, I seemed to have managed to miss having chest colds, but 2005 and 2008 were definite exceptions. Again, a normal x-ray, another visit to the respirologist and another PFT that didn’t send up any alarms [although looking back at both 2005 and 2008, I can see where there was a definite indication that I was heading towards restrictive breathing problems]. Inhalers only made the coughing worse. The respirologist said I had “sensitive lungs” – gee, doc you think?

Then, in November 2010, I was laid out with another chest cold, coughing my lungs inside out, barely able to walk 10 feet. So the new family doctor calls me. This time, the x-ray report came back that I was showing signs of interstitial lung disease (ILD). What? So onto the computer and in to see the family doctor. When the doctor suggested sending me back to the local community-based respirologist I had previously seen, I said NO BLOODY WAY!

Instead, through a friend who is a thoracic surgeon at the University Health Network (UHN) in Toronto, I got a quick appointment at the ILD Clinic at Toronto General Hospital (TGH) in January 2011. Since I hadn’t yet had a HRCT, I was sent for one and returned to the clinic in June. The initial diagnosis was probably IPF, but maybe NSIP since my HRCT didn’t show the UIP-pattern. The decision was made to treat as IPF so no harmful treatment was undertaken. A biopsy was discussed, but was considered too early for that invasive test and that instead my disease would be monitored via non-invasive tests.

My ILD/PF specialist continued to monitor me and after another exacerbation early in 2012 and the PFT showing a progression of my lung disease, we decided to send me for a VATS biopsy. The September 2012 biopsy clearly indicated the UIP-pattern of lung damage and the IPF diagnosis was confirmed.

Over the last few years, I’ve learned a great deal. I know that the road to diagnosis is often long and complex with not all the pieces of information presenting at the same time — seldom with one test or series of tests taken at one point in time. I feel I’m fortunate that first I had that very unsatisfactory experience with the local community-based respirologist and that through my husband’s work we had met and become friends with a thoracic surgeon who is on the lung transplant team at TGH.

So even though I “naturally” followed the recommended course of action to get myself to an ILD/PF expert, my path to diagnosis wasn’t instantaneous. My biopsy could have just as easily shown that I had a treatable form of PF — still not good news but a different path.

Now with a confirmed IPF diagnosis, I’ve been assessed for transplant (June 2013) and found suitable but too early. But another winter of exacerbations and my ILD/PF specialist is now talking about going on the waiting list.

Another PF member, Lori, spoke about her “new normal” – how did your diagnosis change daily life?

Yes, life with PF has certainly been a series of adjusting to the “new normal” but up until February 2013 when I started oxygen therapy, the changes were small. I had to explain to people why I broke into coughing fits while talking on the phone or in person. I had to explain to people who offered water that thank you but it didn’t help since it was just my lungs telling me to talk slower or shut up. I had to explain to people that I wasn’t contagious when coughing. I had to explain to people that the huffing and puffing were just the “new me” and that they didn’t need to feel they had to jump in — that I would ask when I needed help.

But since going on oxygen therapy with my new facial jewelry and my constant buddy, I don’t have to explain that I have a disease but some people still like to ask questions and I enjoy answering them.

Life with PF and supplemental oxygen is definitely more complicated. I started with high-flow for exertion (6 lpm) and liquid oxygen (LOX). So I can’t spontaneously take off overnight (I would have to make arrangements about a week ahead to have  equipment and supplies delivered at my destination) and I probably can’t fly. But I’m a homebody so that has affected me very little. But I can’t leave the house without considering how long I will be and how many of my LOX portables to take with me.

I still do my own driving, shopping, cooking, housework, and one or two 2-mile walks per day on the farm property — over hilly landscape — because I’m de-conditioned after this past winter. I’m currently having to use 8-10 lpm for those walks but I’m doing them. Use it or lose it!

What made you decide to get so involved in the PatientsLikeMe community and how has it helped you better understand your own PF?

Involvement with PatientsLikeMe was more of a knowledge-based decision. I believe that knowledge is power and knowing as much as I can about my disease helps me to manage the disease. For me, support is sharing what I have found and providing directions to that information for others. Then it is up to them to read the information and decide how, or if, it applies to them.

I believe in being my own medical advocate in charge of my medical team. I’ve probably had a natural propensity for that but my way of thinking in not being a traditional patient was affirmed by Dr. Devin Starlanyl, a doctor with fibromyalgia who wrote The Fibromyalgia Advocate. Fibromyalgia is a matter of living with and managing the symptoms and dealing with different medical specialties to achieve that BUT also accepting that you as the patient are central to treatment and management.

I believe that living with PF is that way as well. The doctors can only do so much. There is no single silver bullet that they can give us, no matter what type of PF, to make it all go away. We have a core set of symptoms BUT we don’t all have all the same symptoms. We have to take ownership for our disease management.

So at PatientsLikeMe, I seek to not only learn but to share what I’ve learned. If I can help one other person shorten their learning curve then perhaps I’ve helped.

On your PatientsLikeMe profile, you reported using a pulse oximeter in 2013 – how did you like it? What did it help you learn?

I found that I was having to slow down too much or struggle too much to breathe. My walking test was not yet indicating that I qualified for oxygen therapy but rather was on the cusp of requiring supplemental oxygen. I was concerned about the damage to my body.

I purchased an inexpensive pulse oximeter to check my saturation. I soon realized that being short of breath was not a reliable indicator that my oxygen saturation had dropped below 90%. Having the oximeter to give me a measure of my saturation helped me to better interpret and listen to the other biofeedback that my body was giving me.

The oximeter helped me to manage my activity so that fear didn’t turn me into a tortoise that either slowed way down or seldom moved. I got a better handle on just how much and how fast I could do things to keep active, to keep my body healthy, to exercise all the parts of my respiratory system, and yet to do it SAFELY!

Looks like you use your profile tracking charts and reports a lot on PatientsLikeMe- why do you donate so much health data, and how do you think that will change healthcare for people living with PF?

Again, my propensity. I love learning! I love sharing what I learn! I keep my own spreadsheets with my medical data but that only benefits me. I know that one of the problems for researchers is accessing a sample population large enough to make meaningful inferences from their findings. And finding a large population in a given geographical area for a rare disease is difficult. Going outside the geographical area is expensive. So hopefully the remote sharing of information will be the answer.

We are all so very different and so many of us also have other health issues on top of the PF. So who knows what comparing us will show? But throughout life I’ve been amazed at how seemingly inconsequential, seemingly totally unconnected pieces of information can come together at a later point and TURN ON the light bulb!

So why not share my health data? It really is anonymous. Unless I provide more identifying information, I’m just a name and a face but maybe with enough names and faces we can get some answers that will benefit us all.

 Share this post on twitter and help spread the word for pulmonary fibrosis awareness.


Patients as Partners: The Perceived Medical Condition Self-Management Scale questionnaire results

Posted April 18th, 2014 by

Back at the beginning of April, we launched a new blog series called Patients as Partners that highlights the results and feedback PatientsLikeMe members give to questionnaires on our Open Research Exchange (ORE) platform. This time around, we’re sharing the results of the Perceived Medical Condition Self-Management Scale (PMCSMS), a health measure that looks at how confident people are in managing their own conditions. More than 1,500 members from 9 different condition communities on PatientsLikeMe took part. They worked with our research partner Ken Wallston from Vanderbilt University to make the tool the best it can be. (Thank you to everyone that participated! This is your data doing good.) Check out the PMCSMS results and keep your eyes peeled for more ORE questionnaire results as we continue the series on the blog.

What’s ORE all about again? PatientsLikeMe’s ORE platform gives patients the chance to not only check an answer box, but also share their feedback on each question in a researcher’s health measure. They can tell our research partners what makes sense, what doesn’t, and how relevant the overall tool is to their condition. It’s all about collaborating with patients as partners to create the most effective tools for measuring disease.


“Life is good” – PatientsLikeMe community member John_R speaks about his new life after being diagnosed with pulmonary fibrosis

Posted March 11th, 2014 by

Several people in the PatientsLikeMe community use the phrase “new normal” after being diagnosed with pulmonary fibrosis (PF), and PF member John_R doesn’t’ think his new normal is all bad. This month, he chatted with us about getting diagnosed with PF, bringing oxygen to the workplace, and how living with his Sweetie keeps him focused on the positive moments of his journey.

You were recently diagnosed with PF in 2013 – can you tell us a little about your diagnosis experience?

I was initially diagnosed with PF back in 2002 via a CAT scan with contrast. Around 2000, some haziness was seen on an x-ray, and my doctor recommended that I see a pulmonologist. I was getting ready to move to Texas, so I waited until I settled down and found a new GP. It was after my first physical with my new doctor that I was sent to see a pulmonologist. He sent me for a series of CAT scans from April of ‘02 to Jan ’03.

The first scan indicated “There are several patchy areas of infiltrate identified peripherally in both lungs. These are identified at the anterior and lateral upper lobes as well as in both lower lobes. Mild patchy infiltrate in the lingual and right middle lobe are also seen.” The third scan concluded “….these findings suggest mild fibrosis.” and “…mild stable interstitial airspace disease consistent with fibrosis given stability.”

The doctor indicated that my PF was “mild” and “stable” so not much to really worry about. Each time I visited, pulmonary function testing showed that my Forced Vital Capacity (FVC) dropped a few percentage points. My doctor did not seem to worry, so neither did I. In May of 2011 my father died from Idiopathic Pulmonary Fibrosis (IPF). That was when my pulmo started to show concern about my PF. In 2013 my FVC dropped to less than 50%. Off to another CAT scan with contrast. Insurance denied that procedure and required an HRCT (hooray for my insurance company!). The scans showed lots of damage, but were not conclusive for IPF. In August I had a VATS Biopsy performed that proved IPF.

Following the biopsy I was referred to my ILD Specialist at UT Southwestern.

You talked a little in the forum about your first day at work with oxygen – can you share a little about that for our blog followers? 

The first day I used oxygen was on Christmas Day. Since I had been out from work for the Biopsy in August, everyone knew I had lung issues. The week before Christmas I let my boss, the ladies in the front office and my guys know that I would be coming back from Christmas using oxygen so there would be no surprises.

I use the small M6 oxygen tanks in a bag that slings over my shoulder for my portable O2 use. That first day of using oxygen at work was the first day in a long time that I could climb the stairs up to the office without having to sit at my desk for a few minutes, gasping for breath and regaining my mental clarity. It was also the first time in a long time that I made it home with some energy left.

My oxygen use was quickly accepted at work. There were a couple of double takes when people who did not know I was going on O2 saw me for the first time. A quick smile from me was returned and all was back to normal.

How is life different with PF than before? What have been some of the hardest losses, and what have you gained?

“The New Normal” How do I explain how life has changed without sounding much more negative than I really am?

Life is different not just for me, but also for my Sweetie. Our new normal does not include some things that were important to us. We had to find a new home for our parrot, Tinker. Lighting a fire in the fireplace on a cold winter’s evening is part of our past. No more soaking in hot tubs or spending time at the shooting range.

We find other ways to be romantic, so the hot tubs and cozy fires are not that big an issue. I do miss Tinker and miss my time on the range.

Making sure I have plenty of oxygen has become a part of pretty much every decision we make. I am still the cook in our house. When I was first routing the tubing around made sure I could reach the stovetop with a bit of slack to spare. I also do the grocery shopping and, as we talked about before, still work.

Every chapter of my life that I get to spend with my Sweetie is the best chapter to date. Life is good. For the first time ever I have a year’s worth of vacation planned in advance. Vacation is going to be spent with family and I am really looking forward to those get-togethers.

Looks like you use your PatientsLikeMe PF Severity Score, track your treatments and chart a lot on your profile. What do you find most useful about these tools?

MyCharts is an awesome tool and more people should use it. The charts give a nice visual snapshot of how you are doing. The information is great to print out and bring with you to doctor’s appointments and I wish I had found PatientsLikeMe much sooner. I was just filling out paperwork for a genetics study and had to use several sources to collect information on medication history. It would have all been there if I had started charting sooner.

I also like to look at other peoples’ charts. I compare where I am to where they are and what they are going through. This helps me come to grips with my future.

What advice would you give to others who might be newly diagnosed with PF?

Number one, find an Interstitial Lung Disease specialist. Community pulmonologists are just not that knowledgeable about PF. The vast majority of their patients have COPD, Emphysema, Asthma and the like.  They just have little or no experience with PF.

Next I would advise to not read too much of the medical literature found on the Internet until after you have spoken with a specialist. There is a whole lot of scary stuff out there that probably does not pertain to you. I know you are going to read it anyway, so after you do, take a deep breath and remember that you are not average and that your circumstance is different from every other person with PF. My fibrosis was discovered a dozen years ago.

Finally, hang out in the forums. Ask questions, post ideas and help us support one another.


“Sleep has become a process.” Checking in with idiopathic pulmonary fibrosis patient and PatientsLikeMe member Lori

Posted November 11th, 2013 by

Some of you probably remember seeing her on the PatientsLikeMe blog before. Lori is living with idiopathic pulmonary fibrosis, and when we first chatted with her last July, she shared her experiences with blogging, the difficulty in finding the right diagnosis and how connecting with others has positively impacted her life. For our “Are You Sleeping?” initiative, the PatientsLikeMe community is taking a closer look at how sleep impacts our health, but also how our health affects sleep. Check out what Lori has to say about it in our follow-up interview with her.

lori2

Don’t forget to check out Lori’s blog too, called Reality Gasps. She balances stories of her daily struggles with dashes of humor that can make anyone laugh.

How have you been doing since the last time we talked? It looks like you said on your blog that you broke an ankle?!  

I did break my ankle and had to have surgery to put in a plate and 8 screws! I had refilled the bubbler on my concentrator and didn’t realize I hadn’t screwed it back on just right. As a result, I wasn’t getting enough oxygen and when I stood up, my sats dropped quickly.  I collapsed and must have twisted my ankle, dislocating it and breaking the tibia in the process.  Surgery was quite an adventure. Because of my IPF, I can’t go under sedation, so I had a spinal and was awake for the whole thing.  I have three weeks left in the cast, and then I’ll move to a boot. I’m basically chair-bound right now, so I am eager to get mobile again!

We’ve been talking a lot about sleep on PatientsLikeMe lately. What are your sleeping problems like? Have you been officially diagnosed with insomnia?

I don’t have an official diagnosis of insomnia, but it is definitely a side-effect of my medication. I am on prednisone, which can cause insomnia at higher doses. I was just getting over an infection when I broke my ankle, so I was on a higher than usual dose of prednisone.

In addition to periodically having troubling getting to sleep, I also have periods where I wake up several times during the night because my O2 sats drop. Everyone breathes more shallowly when they sleep, and if I am having a flare or an episode, this can be a particular problem for me at night. Since changes in weather can affect my breathing, I’ve been having more trouble during the past few weeks.

When I do wake up at night, I check my sats immediately. If they’re low, I do some deep breathing to bring them up and can usually drift back off to sleep. Though, I have unintentionally started my day at 4:30 or 5:00 in the morning more than a few times!

How do you think lack of sleep impacts life with IPF? Or is it the other way around for you, that IPF affects how well you sleep. Maybe both?

I think sleep and IPF are definitely intertwined for me. To make it easier to breathe, I sleep with my head elevated. I was never a “back” sleeper, so I’ve had to get used to a whole new position. It still doesn’t quite feel “natural” to me, and it becomes one more thing to obsess about on those nights when I just can’t fall asleep. And as I’ve mentioned, medication and O2 levels affect my sleep as well.

When I can’t sleep, I get sluggish, both mentally and physically. Mental fog is especially dangerous because I need to be aware of how my body feels at all times. If I am moving and my oxygen supply cuts out or is reduced, I collapse within seconds. That’s how I broke my ankle. More directly, I notice that I cough more and my chest generally feels tighter if I am fatigued. Plus, when I am tired, it’s hard to get up the energy to keep moving. And one thing I’ve learned is that remaining active is a key factor in fighting IPF.

You mentioned that you take Ambien sometimes, but that it might not work if you’re on other medications too. Can you tell us about that? 

I take a low dose of Ambien, which usually is just enough to let me drift off to sleep. But, when I am on higher doses of prednisone, Ambien often won’t cut it. My doctor gave me a dosage range of 5-10 mg so I could adjust for those situations. But I’ve found that when I take 10 mg, I wake up feeling really groggy and that feeling lasts for several hours. I also worry about taking too much sleeping medication because I don’t want to subdue my nighttime breathing too much. I’ve started using relaxation techniques in addition to the Ambien, and this seems to be helping.

I still long for those days, though, when my bedroom was quiet (no huff-chuffing machines) and I could flip onto my belly and just snuggle down for a lovely snooze! Like everything else with my disease, sleep has become a process.


It’s Global Pulmonary Fibrosis Awareness Day!

Posted September 7th, 2013 by

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Do you know someone with pulmonary fibrosis? Thanks to the Pulmonary Fibrosis Foundation, today – Sept 7th – is dedicated to raising awareness about this condition and sharing the stories of patients living with PF. It’s Global Pulmonary Fibrosis Awareness Day, and we want to do our part to help raise awareness of this lung condition. Pulmonary fibrosis (PF) causes scarring and thickening in deep lung tissue over time.

We recently announced that the PatientsLikeMe community has grown to include more than 1,000 patients with idiopathic pulmonary fibrosis (IPF). IPF refers to cases where the cause of the condition is unknown, and it affects over 100,000 people in the United States alone.[1] Our collaboration with Boehringer Ingelheim has made the acceleration to enhance our community for PF patients possible. So for Global Pulmonary Fibrosis Awareness Day, we’d love to share what we know about this condition, people’s experiences with it, and the stories behind the people living day in and day out with the disease.

More and more IPF patients are sharing their real world experiences on PatientsLikeMe, monitoring their own health while connecting with others just like them. We’ve done a series of blog interviews with our members, including Lori, Kim, Jeff and Ian, as well as other patients like Sarah and Keith.

Beyond the stories, our members are sharing experiences about what it’s like to live with their IPF. Here’s a snapshot of what we’ve learned about their real-world experiences, including a quote from Lori, an interviewee and amazing individual who was diagnosed with IPF in 2011 and has been connecting with PatientsLikeMe members ever since.

PatientsLikeMe-PF Community

As the PFF says, today is about sharing stories and raising awareness of a condition that affects over half a million Americans. You can help by sharing your story and experience here and through campaigns run by the Pulmonary Fibrosis Foundation and the Coalition for Pulmonary Fibrosis.

Every story, every voice, and every experience matters when coming together to make a difference for pulmonary fibrosis.


[1] http://ghr.nlm.nih.gov/condition/idiopathic-pulmonary-fibrosis


PatientsLikeMe creates largest open registry of IPF patients in the world

Posted August 22nd, 2013 by

Community One of Website’s Fastest Growing;
Reveals Real-World Picture Of Daily Life With the Rare Lung Disease


CAMBRIDGE, Mass.— August 22, 2013—
In a few short months, PatientsLikeMe has created the largest open registry of idiopathic pulmonary fibrosis (IPF) patients online, and some of the most current patient-reported data on the disease. The information the community has shared is providing new clues about the condition and giving a rare look into what it is like to live daily with a disease that has no known cause or cure.

According to the National Institutes of Health, pulmonary fibrosis causes scarring in deep lung tissue over time, making it harder for the lungs to move oxygen into the bloodstream. Cases where the cause of the disease is unknown are called IPF and usually affect middle-aged and older adults, more typically men, and an estimated 100,000 people in the United States.

The company’s focus on IPF accelerated earlier this year when it announced a collaboration with Boehringer Ingelheim to enhance its IPF patient community. Now more than 900 IPF patients use PatientsLikeMe to monitor their health and share information about their experiences with the condition. They can also connect with others like them and find IPF research studies in their area. In the process, members are contributing new, comprehensive data about the disease that will aid clinical research and the development of new treatments.

“We have become a magnet for people who want to learn about living with disease, and a central repository of real-world information on 2,000 common and rare conditions,” said Co-founder and President Ben Heywood. “It’s gratifying to see people gathering faster than ever before to support each other, live better, and contribute vital data to medical research.”

As the IPF community grows, PatientsLikeMe plans to study the challenges in diagnosing the disease and to ask patients about other top priorities for research. IPF patients interested in participating in research to improve understanding of IPF can sign up at http://www.patientslikeme.com/join/ipf/whatisipf.

The data shared on the website to date offer a snapshot of the IPF community’s members and highlight the effect the disease has on their lives:

  • The average age is 64; four out of five are older than 55.
  • 54% are male, 46% are female.
  • Approximately 15% report having familial IPF, a subtype of IPF thought to be genetically related.
  • An initial review of comorbidities shows that pulmonary hypertension, a condition that puts additional stress on the cardiovascular system, is most frequently cited. Other common co-morbidities include type-2 diabetes, gastro-esophageal reflux disease and osteoporosis.
  • Most report limitations to their activity and energy level. Over 90% experience shortness of breath during activities, while over 50% experience shortness of breath by just walking up a few stairs. About 7% are short of breath even when they are sitting down or at rest.
  • Close to 90% have noticed their energy level affected by IPF. About 35% say they often have to nap during the day or are always tired, no matter how much they sleep.
  • About 80% report having a cough. The majority report having a mild or moderate cough, but approximately 15% have severe coughing.
  • Approximately 11% have had unplanned hospitalizations as a result of their IPF in the past three months.

About PatientsLikeMe
PatientsLikeMe® (www.patientslikeme.com) is a patient network that improves lives and a real-time research platform that advances medicine. Through the network, patients connect with others who have the same disease or condition and track and share their own experiences. In the process, they generate data about the real-world nature of disease that help researchers, pharmaceutical companies, regulators, providers, and nonprofits develop more effective products, services and care. PatientsLikeMe is a trusted source for real-world disease information and a clinically robust resource that has published more than 35 peer-reviewed research studies. Visit us at www.patientslikeme.com or follow us via our blog, Twitter or Facebook.


“Retooling my attitude.” An interview with idiopathic pulmonary fibrosis patient & PatientsLikeMe member Lori

Posted July 7th, 2013 by

As part of our “Spotlighted Blogger” series, we’re talking with people who are sharing their personal health experiences to help raise awareness of disease and change healthcare for good. For our latest interview, we’re talking with Lori, an idiopathic pulmonary fibrosis (IPF) patient who started blogging about her journey back in October 2011. Her blog is called Reality Gasps and she balances stories of her daily struggles with dashes of humor that can make anyone smile. If Lori sounds familiar to some of you, it’s because she’s also part of the PatientsLikeMe community.  She recently took some time to talk with us about why she started blogging, the difficulty in finding a diagnosis and how connecting with others has positively impacted her life.

Lori

What made you decide to start blogging about your experience? What’s been the community response?
When I was first diagnosed with IPF, I started researching online (like everyone else). The medical sites gave me an idea of what was happening to my body, but they said nothing about how to live with this disease – and when I really thought about it, that’s what I needed. So, I turned to blogs written by other patients and caregivers. They were (and remain) incredibly helpful. I started my own blog as a way to make sense of what was happening to me. I’ve been a writer all of my life and the blog is my way of “talking it through.” I also see the blog as a way to give back to the amazing PF community. I hope that by sharing my experiences and passing along information, I’ll be able to help others cope with all that PF entails.

The response to my blog has been more than I ever hoped for. It’s a great way for me to keep family and friends abreast of what’s happening to me without managing an email list. Plus, other bloggers have discovered me and through them, I have also found several excellent blogs. Through the comments section, other PFers are sharing some really helpful insights for the whole community. And I love that we can all share a laugh or two about the craziness of our daily routines. What I’m happiest about, though, is that several readers have told me they forward some of my posts to others who they think might benefit from the message. IPF may be the source of my struggle, but what I go through on a daily basis isn’t unique. Fear, uncertainty, anger, stress, joy, motivation – these are the issues I deal with in my blog and they are universal.

When did you first know that something wasn’t quite right? Do you remember what your first sign or symptom was?
I was diagnosed officially in September of 2011. About 2 years before that, I started noticing shortness of breath when I climbed a flight of stairs or walked quickly. I shrugged it off as being overweight and out of shape. The shortness of breath continued to increase and my doctor prescribed a Flovent inhaler, figuring this was some kind of asthma or reactive airway condition. The Flovent didn’t help, and about a year later I developed a dry cough. It wasn’t much to start with, but over the next several months it got worse. I sucked on cough drops all day every day, and frequently coughed until I vomited. By the summer of 2011, I was so short of breath when I walked that I’d have to sit down and gasp for a while.

We’ve spoken to others with rare lung disease that had a hard time finding an official diagnosis. Was that your experience too?
Getting someone to understand that my symptoms went way beyond mild asthma was a long and arduous process. Even I had a hard time thinking it was anything more that just poor fitness and extra weight. But the coughing had gotten so bad that it was affecting my quality of life – I worried constantly that a coughing fit would cause me to vomit in public. My allergist thought the cough was caused by medication and started switching things around. As an afterthought, he ordered an x-ray. That’s when everyone saw the infiltrates throughout both of my lungs.

I didn’t know what infiltrates were, but I knew they were bad, so I got online and started researching pulmonologists. I wanted an experienced diagnostician who would help figure this out. I was fortunate because I unknowingly chose a doctor who belongs to the only non-university based IPF research center in the country. He ran extensive tests on me and did an open-lung biopsy to confirm the diagnosis. I talked with him extensively about his experience and felt comfortable that not only was he extremely knowledgeable about IPF, but that he would be an active partner with me as I learned how to manage and live with the disease.

That’s my biggest piece of advice to anyone – do your research and find a doctor who understands the disease. You may have to travel a bit to find him/her, but your life is worth it.

You titled one of your recent blog posts, “Retooling My Attitude.” Can you tell us about that?
On the surface, “Retooling My Attitude” is an homage to my grabber. I’d been thinking about getting a grabber for a long time – to retrieve things off the top shelf or recover socks that some how wound up behind the dryer. After I got sick, when I realized that simple tasks like picking up my bedroom were getting more and more difficult, I saw the grabber as more than just a simple convenience. Suddenly, it was a tool that offered me a little bit of control in an out of control situation. My disease has given me a new perspective on many elements of my life, and that provides fodder for much of what I write about. It’s also shown me that a good laugh can brighten even the darkest day, and so I also try to inject as much humor into my posts as I can.

It looks like you have quite a following on your blog. How do you think connecting on a blog or through an online community like PatientsLikeMe can help others with IPF?
From the beginning, I’ve been impressed with how warm, strong and supportive the pulmonary fibrosis community is. With the help of online locations such as PatientsLikeMe, I have friends across the globe who are always available to offer insights about everything from potential side-effects with a new medication to ideas for dealing with painful muscle spasms. It’s also really comforting to be able to connect with people who understand exactly what I’m going through, like the guilt that comes along with being “the sick one” in a family. And it’s nice to know that I’m not the only one who manages to get my cannula caught in the oven door!


Keith & Sarah’s personal journey with rare lung disease. Part III, “Bonus round”

Posted June 22nd, 2013 by

Over the last few months, Keith and Sarah have been sharing their journey with us. In this final interview of our three-part series, they talk about how he got on a transplant list and their “phones at the dinner table” policy. If you missed our first two interviews with Keith and Sarah, you can find them here.

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What did you have to do to get on a transplant list? Did you have to meet certain criteria?
[Keith] The transplant assessment process is an intense and very time-consuming one. When you are contacted about being assessed for transplant, you are sent a large envelope listing out a weeks worth of testing, doctors visits, and appointments in Toronto at Toronto General Hospital. The hospital evaluates you on many things, and ultimately if you are deemed “healthy” enough (because you can actually be too sick, or too healthy) as a result of this testing, you are placed on the list. There were psychological assessments, nuclear cardiac testing, liver testing, kidney testing, pulmonary function testing, physical testing, blood tests (LOTS of blood tests) to name a few.

Can you talk about your “phones at the dinner table” policy and how it changed?
[Sarah] Phones allowed at the dinner table. Most families discourage this, as it is always nice to have the family come together at the end of the day and talk about their day. The one time when people are tuned to each other as opposed to their devices! Once Keith was placed on the list, we were waiting for that call to come which would signal the next phase of our life. This meant that our phones were even MORE attached to us, and yes, were placed on the dinner table until that call came.

SK$

How are you doing post transplant?
[Keith] I feel fantastic! Being able to breathe with deep breaths every minute has been the most life changing experience for me. I am still sifting through a bit of a fog with some pain medications but as they are reduced, I find myself feeling more and more enthusiastic about what lies ahead. Each day is a gift, and I am enjoying my “bonus round.” The realities of transplant are such that we never know what tomorrow will bring, so I am living my life to the fullest every day that I am here.

What’s the one thing you both think every rare lung disease patient should know?
[Sarah & Keith] There is hope. With awareness we can educate people on the necessity to be organ donors, which can ultimately get rid of the list! The transplant program at Toronto General is exceptional and if you do what they tell you, take care of yourself and do your physio…there truly is hope for a better life with new lungs. The hospital’s goal is to help you through your disease in any way possible, and transplant is the last option, but if you need it, this is the place to be.

Sarah and Keith are part of our “Spotlighted Blogger” series, where we talk with people who are sharing their personal health experiences to help raise awareness and change healthcare for good. Sarah started blogging about her fiancé Keith’s journey with a rare lung disease back in July of 2012 on her blog Taking a Deep Breath.


Keith & Sarah’s personal journey with rare lung disease. Part II, “Lungies”

Posted May 22nd, 2013 by

In this second interview of our three-part series, Keith and Sarah talk about how their daily lives changed and the importance of connecting with others. If you missed our first interview with Keith and Sarah, you can find it here.

S-K 2

What were the most noticeable changes you had to make in your daily life?
[Keith] My ability to enjoy time with family was impaired because I could no longer be active with my children or my wife. I could not work because when I tried to do the simplest task, I became out of breath. I could no longer carry a toolbox, go up a set of stairs, or do everyday tasks at home without becoming winded and requiring rest. I wanted to rest all the time and was never comfortable.

As a caregiver, what things could you do to help Keith the most?
[Sarah] Keith eventually got to the point where he needed me for many personal tasks as well as taking care of all of the home tasks. I showered him, and took over our business, and we hired a cleaning service every two weeks to try to keep the house in order. Keith really needed to know that I was there for him. I made lots of special food for him because he needed to gain weight in order to be active on the transplant list.

How did you connect with other transplant patients and why did that help?
[Sarah & Keith] From the beginning, we began to seek out others who had received lung transplants. We found one in our town and eventually met many others through our many visits to the hospital. The lung transplant “family” is a tight one and it really helped to have others to talk to who were waiting, as well as those who had received their transplants and could offer hope and advice. Facebook became a good way to find people through other friends from the hospital, and eventually we developed a close group of “lungies” that we keep in touch with.

Besides PatientsLikeMe, what would you suggest to others patients who are trying to connect with someone like them?[Sarah & Keith] Finding groups on Facebook that relate to organ donation / transplant and beginning conversations with others is a great way to find people. Starting your own Facebook group or blog to raise awareness about your situation is another excellent way to let people find you.

Sarah and Keith are part of our “Spotlighted Blogger” series, where we talk with people who are sharing their personal health experiences to help raise awareness and change healthcare for good. Sarah started blogging about her fiancé Keith’s journey with a rare lung disease back in July of 2012 on her blog Taking a Deep Breath.

 


“Make memories starting now!” An interview about idiopathic pulmonary fibrosis with Kim Durand

Posted May 17th, 2013 by

Many of you have seen our “Spotlighted Blogger” series, where we talk with people who are sharing their personal health experiences to help raise awareness of disease and change healthcare for good. Well for the first time on our blog, we’re talking with a “Facebooker.” Kim started her Facebook page, Pulmonary Fibrosis Awareness, after her father passed away from idiopathic pulmonary fibrosis (IPF). She recently took some time to talk with us about her experiences with the disease and the benefits of online patient communities.

Kim1

Can you tell us a little about yourself and your experience with IPF?
My father was diagnosed with IPF in July of 2006. He always had a chronic cough and never got it checked out until one day it got really bad and my step mom made him go get checked out. He had a chest X-ray and the doctor gave him the tentative diagnosis of IPF. Surely, the only true way to know if a person has IPF is through a lung biopsy so that’s what he had done, I believe in September of 2006.

There was a complication during the biopsy. His lung collapsed and he was in the hospital for about 6 weeks. The lung never did fully inflate. He ended up having 2 chest tubes while in the hospital. He was in good spirits though, always reassuring me that he was doing well and things would get better. Over the next few months he started the process of getting on the lung transplant list at Duke University. He did all the rigorous testing and lost the weight. I went to take care of him for a few days and while he had trouble with catching his breath when walking around, he was still walking on the treadmill to lose that weight. He was in good spirits even though he was exhausted. He was on oxygen therapy 24/7. At Christmas time, we spent the day together and he had a hard time talking because he couldn’t breathe very well. It was the last time I heard my father speak.

A few days later they moved to NC where they waited for the call. His health deteriorated quickly and about a month later he was rushed to the hospital because he couldn’t breathe. It was a Thursday when I got the call from my step mom that my dad was intubated and that my sister and me needed to go down to NC to be with him. She informed us that it wasn’t good and that it was the end. It was the hardest trip I ever had to make.

Kim2

We walked up to his hospital room in the ICU and I slowed down before we got to his room and I looked in and backed away and fell to the floor, crying. I couldn’t believe that my father, this strong man, was lying so helpless in a hospital bed, so dependent on machines to keep him alive. The kind staff at the hospital helped me go to my father in the room where I sat by his side. I didn’t hold his hand right away. Touching isn’t something he liked so I respected that. We spoke with the doctors and nurses and organ donation coordinators. We waited as long as humanly possible for lungs to become available. It was decided that Saturday he would be taken off the list because he had been intubated too long and his heart was working too hard. We could have extubated on that day but I know my father and I believed he wanted to be an organ donor. So we postponed his passing by one day so that the donor team could get ready with recipients.

The next day around 7:30am we arrived at the hospital to say our goodbyes. I remember checking his monitors pretty regularly and when we walked in his heart rate was around 70 and when my sister and me finally held his hand, his heart rate went up to 87. We talked with him privately about what his final wishes were. We knew he couldn’t talk but we wanted to talk to him anyway and try to get any sort of response out of him. We waited a couple more hours for the stuff to be put in place and then they wheeled him down to the operating room. We waited in the family room for a few minutes where the coordinator went over with us what to expect. They said that if he breathes on his own for 10 minutes after he’s extubated he will be intubated again and taken back to his room. But this never happened.

We were there for him during his final minutes. Telling him that it was ok to move on, that we would be ok. I am next of kin so it was my decision to make him an organ donor and my decision to take him off life support. It’s my signature on those papers. Something I will never be able to change. The hardest thing I’ve ever had to sign in my life. We watched as he peacefully tried to breathe. After his last breath, we all continued to sob and the doctor confirmed that he had passed.

We had approx 2 minutes to say our final goodbye before they wheeled him in to the OR for organ recovery. It wasn’t a good atmosphere for us to be in when he passed because there were teams of doctors and nurses standing in this big room with us waiting for him to pass. But I tried not to focus on that part too much. My step mom was at the helm, talking to him while he tried to breathe. At about 10am on Jan 28 2007 my father passed away waiting for a lung transplant due to IPF.

 

You’ve been very active in the IPF community for a long time. How have you seen online communities help patients and caregivers with IPF?
I created the Pulmonary Fibrosis Awareness (PFA) group on Facebook initially as a memorial to my dad; just a place for my friends and family to go to remember him. It now has almost 5,000 members. I have met so many wonderful people through this group. Many who are patients and care givers. All have been affected by IPF in some way. This online group has become a community where they can share their experiences, trials and tribulations. A lot of members go on there to just read about other people’s issues. We have several “regular” members that post quite often. I feel like if I hadn’t made this group, some of these members never would have met and that is so humbling to me. Having an online community really helps in this digital age because many of those struggling with IPF aren’t able to leave the house or have trouble getting around in the community, so they are able to reach out and socialize over the internet. I am proud to have helped in that way.

How do you think information sharing platforms like PatientsLikeMe benefit patients?
I think it’s a fantastic tool for those dealing with any illness to track their progress. It’s multifaceted, somewhat user friendly and very well put together over all. It’s another great outlet for those who can’t leave the house due to their illnesses or those who can but want to document their life.

What are some other helpful resources that you think IPF patients should know about?
There are so many communities and organizations available to IPF patients. The PFA group is partnered with the Pulmonary Fibrosis Foundation, so a lot of our members come from there and some of the members of PFA go there for resources. There’s also the Coalition for Pulmonary Fibrosis. I get a lot of newsletters from them. They were the first organization to tell me about the new drug pirfenidone when it was in its clinical trial stage and now it’s in the manufacturing stage.

What’s one thing you think every patient with IPF might not know, but should?
MAKE MEMORIES STARTING NOW! I will never be able to create memories with my dad anymore. Had I known he was going to pass so quickly, I would’ve spent more time with him, called him more etc. Each day is a gift and each day a new memory should be made. Cherish them. You never know when it’ll end.

If you’re living with IPF, find others just like you in our growing community of almost 400 IPF patients on PatientsLikeMe. Learn what they’re doing to manage their condition with symptom and treatment reports, and share your own experience with a personal health profile and in the IPF forum.

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