Tuesday, November 24, 2015

"I've forgotten what it feels like to breathe"

Jane is one of my patients living with pulmonary fibrosis. She has agreed to share her story here on my blog. Please share Jane's story with your friends and loved ones to help raise awareness of pulmonary fibrosis.

"Ive forgotten what it feels like to breathe; Im preoccupied by it, and I am aware of every inhale and
exhale I attempt to make. I was diagnosed with interstitial lung disease (pulmonary fibrosis) in December 2012, right after undergoing surgery for my second collapsed lung that year. At the time, I was living in the San Francisco Bay Area attending culinary school. I was 25 years old and had recently left the corporate world of New York to pursue a return to the world of food and wine; I spent at least eight hours a day on my feet in the kitchens - lifting, running, butchering, constantly moving. But I had known something was not quite right with my breathing for about a year. I had a persistent dry cough, which several doctors attributed to allergy induced asthma or acid reflux, but none seemed too concerned. My first lung collapse in February 2012 had been alarming, but no one sensed there was anything more serious than a spontaneous event likely due to my tall and thin build. However, when my other lung collapsed in December and a biopsy was performed, interstitial lung disease was the diagnosis and I was sent to see a specialist in San Francisco. A few months later and no real understanding of why I had developed the lung disease or if there would be any viable courses of treatment, I returned to New York in order to have the support of my family while navigating these waters.

In the early stages of the disease, I was determined to keep it a secret - I didnt want it to take over my life or identify me. I was Jane, the 26-year old wine writer and buyer, who lived in New York and loved to cook, and also happened to be dealing with pulmonary fibrosis. However, as Ive gotten sicker, I feel as if Ive morphed into Jane, who has pulmonary fibrosis.

I would not recognize me today as the same person two years ago  - a 26-year old commuting five days a week from New York to Westchester - navigating the subways, climbing a large flight of stairs at the end of my train ride, walking the streets of New York on the weekend and going to the gym all while breathing the regular air around me. The disease has moved quickly and unapologetically through my body, and taken with it any element of an active lifestyle, a sense of independence, freedom, and most obviously, my breath.

Today, I am tethered to my oxygen concentrator 24/7, unable to perform simple tasks such as getting dressed while standing, showering, moving from one room to the next without needing to rest. I cough - often. I cannot recall the last time I set foot in a grocery store, I am no longer able to cook for myself or others - a task that once was the ultimate in enjoyment for me, and sometimes I will look out the window only to come to the realization that I havent left the apartment in two weeks. I get breathless from talking, and have mastered the art of avoiding any situation that would require I walk and talk simultaneously or laugh too hard for fear that my lungs couldnt handle it.

I have been on the transplant waiting list for a little over a year. The scariest realization I have had recently is that I no longer think of my situation as when I will get the call for transplant, but if.

On bad days, I am desperate for my phone to ring, but troubled by the fact that by nature, something I am hoping for is something that can only happen at the hands of grief and pain for others. Its hard to reconcile that my chance for more life must come from the death of someone else. Its a surreal feeling to be waiting for a transplant, and one I dont know Ill ever fully comprehend. But for now, I have learned to pace myself, to adjust my expectations, always plan ahead and, above all else, remain positive.
At my friend's wedding in New Hampshire

On good days, I am full of hope - hopeful that my call will come soon, and grateful for the incredible and generous support system I am lucky to have. Parents who were enjoying an empty nest, but have now reprised their roles of mom and dad, taking me to doctors appointments, preparing meals for me; four siblings who spend any free minute they have from work helping me - packing up my entire studio apartment when I moved last month, driving me five hours to New Hampshire and carrying my oxygen equipment for me so I could attend my best friends wedding, and bringing over sandwiches from our favorite Italian deli. An employer who has let me work from home for the past year, as my illness progressed. Doctors and nurses who are dedicated to caring and helping, and making a difficult process as painless as possible. Friends who visit me and never complain that I dont come to their homes, and offer to bring me groceries. They help preserve some semblance of normalcy for me - which may be the toughest non-physical attribute of this illness to reconcile - the loss of anything that is or ever was normal."

-- Jane
November 2015

Saturday, September 12, 2015

PFF Summit, Washington, DC, November 12-14, 2015

The Pulmonary Fibrosis Foundation will be holding its 3rd Summit in Washington, D.C. from November 12 to 14. I am planning on being there along with many other health care professionals and scientists who are passionate about raising awareness and finding a cure for PF. If you are a patient with PF or if someone you love has PF, I strongly encourage you to attend the Summit. You will have the opportunity to meet doctors, scientists, and other patients and caregivers. Special sessions are planned just for patients and caregivers. You can learn more about the Summit, including information about online registration at http://www.pffsummit.org/register.html.

I hope to see you there!

Wednesday, September 2, 2015

An update about IPF Treatment Guidelines

You might have heard that there are new “treatment guidelines” for Idiopathic Pulmonary Fibrosis (known as “IPF”). I was part of a webinar hosted by the Pulmonary Fibrosis Foundation last month where we summarized the new guidelines. Check it out.

If you have IPF, I think it is important for you to understand which treatments are currently recommended for IPF — and to also understand which treatments are not recommended for IPF. Before I begin, remember that everything on this website is informational in nature — please do not interpret anything I say as medical advice. Discuss everything with your doctor first before you make any changes to your medical treatment regimen. Also, you should be aware that these guidelines were only developed for folks with IPF. These guidelines do not apply to people with other forms of interstitial lung disease or pulmonary fibrosis.

In the next 4 paragraphs, I give a little bit of background about the guidelines. If you just want to skip ahead to the actual guidelines, scroll down to “Here’s what’s in the guidelines"

Clinical practice guidelines (typically just called “guidelines”) are a collection of formal recommendations to guide the diagnosis, screening, and/or treatment of a medical condition. Sometimes guidelines are developed by professional medical societies and sometimes they are developed by governmental organizations. In this case, the guidelines were developed by 4 professional medical societies: the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin-American Respiratory Society.

Here are two links: the new treatment guidelines from 2015, which are an update to previous guidelines from 2011. Many of the recommendations in the 2011 guidelines still stand. The new guidelines only addressed 10 recommendations and of these they left 2 recommendations unchanged. I will try to present a unified overview of the current recommendations (whether from 2015 or from 2011), but I will also mention when a recommendation was new or changed in 2015.

The final bit to know about before we talk about the actual recommendations made in the guidelines is that there are two kinds of recommendations: STRONG and CONDITIONAL (referred to as WEAK in the 2011 guidelines). Even though STRONG sounds very different from CONDITIONAL or WEAK, the truth is that they are quite similar. A STRONG recommendation means that almost everyone would want the treatment. A CONDITIONAL recommendation means that the majority would want the treatment.

I should also point out that the guidelines also make recommendations against certain treatments. A STRONG recommendation against a treatment would mean that almost no one would want that treatment. A CONDITIONAL recommendation against a treatment would mean that some people might still want that treatment, but that the majority would not.

Here’s what’s in the guidelines:

Here is what is currently recommended to treat IPF

STRONG Recommendations for the treatment of IPF (Almost everyone will want this)
1. Oxygen use (when medically indicated by low oxygen levels)
2. Lung transplantation (when medically indicated)

CONDITIONAL (or WEAK) Recommendations for the treatment of IPF (the majority of folks will want these)
1. Pirfenidone (Esbriet®)
2. Nintedanib (Ofev®)
3. Antacid therapy (such as Prilosec® or Pepcid®) even in the absence of symptoms
4. Pulmonary rehabilitation

There is also a CONDITIONAL recommendation to use steroids (like prednisone) during a severe sudden worsening (called an “acute exacerbation”). Most folks who have an acute exacerbation need to be hospitalized — this is often a serious event.

Of the above recommendations, the only change in 2015 were the recommendations for pirfenidone and nintedanib.

I think all of the above recommendations are good ones  — and in my own opinion, I don’t see any distinction between the “strength” of the STRONG and CONDITIONAL recommendations. These are all STRONG recommendations in my mind.

As I mentioned above, there are also recommendations against using certain treatments:

STRONG Recommendations against treatment in IPF (almost no one will want this)

1. Steroids (such as prednisone) unless an “acute exacerbation” develops (see above)
2. Steroids combined with azathioprine and N-acetylcysteine
3. Steroids combined with other drugs that act on the immune system
4. Anticoagulation with warfarin (coumadin) — please note that folks who need blood thinners like warfarin for a different condition, such as atrial fibrillation or blood clots, should stay on their blood thinner. This recommendation is only meant to recommend against using blood thinners to specifically treat IPF.
5. Other medications that aren’t typically used nowadays for IPF. They include:
    Interferon gamma

CONDITIONAL (or WEAK) Recommendations against treatment in IPF (some people might still want these therapies)
1. N-acetylcysteine alone (NAC)
2. Mechanical ventilator (a breathing machine) if respiratory failure (lung failure) develops.
3. Treatment of pulmonary hypertension
4. Sildeanfil
5. Other medications that aren’t typically used nowadays for IPF. They include:

Again, I tend to lump the STRONG and CONDITIONAL recommendations here together. They are all STRONG recommendations in my mind.

I imagine there are still many questions about these guidelines. Go ahead and post your questions and I’ll do my best to answer.

Monday, August 10, 2015

I have PF. What can I do about my cough?

Cough is one of the most troublesome symptoms for people living with pulmonary fibrosis. I would like to share my thoughts about treatments that seem to help cough in many of my patients with PF.

First, remember that I can't give medical advice here. Always discuss this information with your doctor before starting, stopping, or changing any of your treatments.

Most people with PF who have cough suffer from a bothersome and persistent cough without much phlegm (also known as sputum, which is any liquidy stuff you cough up). When there is phlegm, it tends to be thin and clear or white. Many people with PF report that the cough occurs in the morning (when the phlegm may be thicker) and might be worse with talking (particularly on the phone) or after exertion. Be sure to talk to your doctor if you cough up thick or yellow sputum, or if you cough up blood or blood clots, or if you have fever or chills, or if your cough changes all of a sudden.

Here are the things that seem to help my patients with PF. Nothing works 100% of the time. Some people respond to some treatments better than others. And, remember, discuss all of this with your doctor first.

1. Appropriate oxygen use. Many people with PF cough when their oxygen levels drop (particularly below 90%) during exertion or talking. See my previous posts about monitoring oxygen levels.

2. Treatment of postnasal drip. Postnasal drip (or chronic rhinosinusitis) is extremely common even in otherwise health people, and in my experience the vast majority of my patients with PF have postnasal drip. Typical symptoms include a sensation of mucous in the throat, tickling in the throat, frequent throat clearing, and the need to frequently swallow mucous. In my experience, one of the most effective treatments for this is a saline nasal wash (which is inexpensive, safe, and available over the counter in the cold and flu aisle of your pharmacy). Saline nasal washes can be administered with a netipot or a squeeze bottle. Your doctor might also recommend that you combine a saline nasal wash with a nasal steroid spray and/or an over the counter non-sedating antihistamine.

3. Treatment of acid reflux. Acid reflux (also known as gastroesophageal disease, or GERD) is pretty common among folks with PF -- even without symptoms, and is a common cause of cough in folks with or without PF. Talk to your doctor about whether you should take medication for GER. Also check out WebMD's section on treating GER without medication.

More than half of my patients have significant improvement in their cough with one or more of the above treatments. Of course, the scarring and/or inflammation still contributes to the cough. Some people with inflammatory forms of ILD have improvement in their cough after treatment with prednisone. For some folks, the cough improves only marginally or not at all. 

I've heard some of my patients tell me that N-acetylcysteine (NAC) has helped their cough. I'd be interested in hearing what has worked best for you.

Wednesday, November 19, 2014

Should the PFF and CPF invest in pharmaceutical companies?

The New York Times is reporting that the Cystic Fibrosis Foundation stands to receive $3.3 billion in royalties resulting from their investment in a biotech company years ago. That initial investment led to ground-breaking science and a "blockbuster" drug that has a pretty amazing effect in a small fraction of people with cystic fibrosis. Critics are saying that "a higher price means higher royalty payments, [and perhaps] the foundation did not do enough to bring the cost down." 

I see that side of the argument, but it seems laughable that a non-profit foundation would have any meaningful impact in price setting by the pharmaceutical industry. The factors that determine price have little to do with advocacy. I hope people aren't missing the real message here: Investment in science and research can have a huge return. If other non-profits follow the CF Foundation's lead, we have a real chance of finding cures for terrible diseases (like Pulmonary Fibrosis!) in our lifetime. I can't wait to see how the CF Foundation uses it's $3 billion to cure CF.

What do you think? Should the PFF, CPF, and other non-profits in the PF world invest in research being done by pharmaceutical companies? 

Wednesday, October 15, 2014

UPDATED: The FDA approve pirfenidone and nintedanib to treat IPF

The U.S. FDA approved both pirfenidone and nintedanib to treat IPF on October 14, 2014. This is wonderful news, since as you probably know, we've had nothing available to treat IPF in the United States other than oxygen, exercise, and lung transplantation. Both drugs should become available in the US by the end of October, and both pharmaceutical companies are offering financial assistance programs.

Don't forget that you can always call or email the Patient Communication Center at the Pulmonary Fibrosis Foundation at 844.Talk.PFF or pcc@pulmonaryfibrosis.org

You can read more about these medications on the following websites:

Pulmonary Fibrosis Foundation's announcement:

My blog post about these drugs:

Dr. Swigris' blog post about these drugs:

A Pulmonary Fibrosis Foundation webinar about these drugs

Pirfenidone (Esbriet®)

The FDA's announcement of pirfenidone approval (Brand name Esbriet®)

Patient information on Esbriet® from the FDA:

Esbriet's® website including information about financial assistance through CareConnect:

Nintedanib (Ofev®)

The FDA's announcement of nintedanib approval (brand name Ofev®)

Full prescribing information about Ofev (with patient information at the end):

Information from Boehringer-Ingelheim about the availability of Ofev (source):
"To support people living with IPF who will be prescribed OFEV, Boehringer Ingelheim has developed OPEN DOORS™ – a comprehensive patient support program that will provide a broad range of financial and nursing support services. Accessible through www.OFEV.com and at 1-866-673-6366, OPEN DOORS™ services and resources include:

  • Access to nurse support 24 hours a day, 7 days a week
  • Assistance in finding financial resources to support access to OFEV
  • Access to information about IPF and assistance in identifying local resources, such as support groups"

Sunday, July 27, 2014

A Pulmonary Fibrosis Primer for Doctors

This is a post for health care professionals. Many doctors, nurses, and other health care workers have limited experience with Pulmonary Fibrosis. These are my own recommendations, not those of any organization I am affiliated with. This is not intended as a comprehensive review -- instead I provide highly focused learning points that can be used in clinical practice. 

When should I suspect that my patient might have pulmonary fibrosis?
There are four typical and overlapping presentations of PF:
1. Subacute or chronic cough
2. Exertional dyspnea
3. Concerned patient with a family history of pulmonary fibrosis
4. Incidental PF found on CT imaging

The initial evaluation should include careful chest auscultation for crackles. Please listen carefully halfway down the back at both lung bases as well as in the mid-axillary lines. Drs. Cottin and Cordier have written about the importance of careful auscultation in the early detection of PF. You can listen to crackles online here. If you have access to the NEJM website, you can also listen to crackles here. Examination of the fingers for clubbing can also be helpful in raising the suspicion for PF. 

If crackles or clubbing are present, order the following tests at a minimum:
1. PA and lateral chest x-ray
2. Spirometry and DLCO

I also recommend walking the patient in the hallway briskly for ~200 feet or until dyspnea develops. Check pulse oximetry before and after the walk. A decrease in SpO2 of 3-4% or more indicates exertional desaturation and should raise the suspicion of PF. Performing this informal test on a staircase is probably more sensitive than a hallway walk.

The bottom line is that you should have a low threshold to look for PF. Late diagnosis, early misdiagnosis, and suboptimal management strategies are common. PF should be investigated if there is one historical feature suggestive of PF plus one feature from the physical exam or from preliminary testing. Take a look at the figure below. Dyspnea, cough, or a family history combined with one of the "ACES" findings should be enough to move forward with an evaluation for ILD or PF.

What should I do if I suspect my patient might have pulmonary fibrosis?
If you suspect PF, please order a high-resolution CT scan of the chest. I discussed the details of an HRCT in a previous blog post about lung biopsies. Here is the bottom line: 

  • Supine inspiratory scan with slice collimation no greater than 2 mm (preferably < 1.25 mm)
  • At least a few supine expiratory images should also be performed
  • High spatial frequency reconstruction algorithm ("Bone" recon on GE machine)
  • No IV contrast
Remember, that a CT angiogram is not an HRCT -- yes, the images are thin, but the reconstruction is not "high res. Thin images alone do not make an HRCT.

Ok. There is evidence of ILD/PF on the HRCT. What do I do next?
Based on the HRCT, you have diagnosed your patient with ILD. The next step is to figure out which kind of ILD your patient has. Here's what I do in my practice:

1. Take a thorough history
In addition to establishing the narrative history, asking the usual questions about dyspnea, cough, and exercise tolerance, and the other standard components of a medical history, I find out about the following:
  • History of cancer, chemotherapy, or radiation therapy
  • History of cardiac arrhythmias and amiodarone use
  • History of prostate, urinary, kidney infections and nitrofurantoin (Macrodantin, Marcobid) use
  • All current and prior chronic medications -- don't forget that you can check pneumotox.com to see if your patient's medications might have contributed to their lung condition.
  • Symptoms of connective tissue disease (Raynaud's, rash or other skin changes, joints, finger tip changes, heartburn, regurgitation, dry eyes, dry mouth, morning hand stiffness, muscular weakness or pain)
  • Exposure to mold sources
    • Forced air heating
    • Hot tubs
    • Humidifiers
    • Visible mold, water damage, or musty odors in the home
    • Renovation
  • Exposure to birds in the home or in cages; down pillows or comforters
  • Exposure to farming or argiculture
  • Occupational history with possible dust, fume, vapor, gas, or particulate exposure including:
    • Mining
    • Excavating
    • Quarry work
    • Stone work
    • Foundry work
    • Ceramics
    • Abrasives
    • Heavy metals
    • Asbestos
    • Construction
    • Aerospace industry
  • Family history
    • Anyone with pulmonary fibrosis/interstitial lung disease or sarcoidosis?
    • Anyone ever on oxygen at home?
    • Anyone with "autoimmune" diseases, such as lupus, scleroderma, rheumatoid arthritis, or "myositis"
  • I also ask about snoring and sleep apnea symptoms.
2. Physical exam findings
It is not unusual for me to have a pretty good idea about the specific diagnosis after completing a history and physical exam. Here's what I focus on during the exam:
  • Narrow oral aperture (scleroderma)
  • Heliotrope rash around the eyes (dermatomyositis)
  • Telangiectasias on face & chest (scleroderma)
  • Tightening of the skin on the face, chest, and hands (scleroderma)
  • JVD and hepatojugular reflux
  • Lungs
    • Crackles
    • Squeaks (hypersensitivity pneumonitis and bronchiolitis)
  • Heart
    • RV heave
    • Loud P2
  • Fingers
  • Arms & hands
    • Synovial thickening on MCPs and wrist
    • Joint deformities
    • Rheumatoid nodules
  • Legs
    • Erythema nodosum (sarcoidosis)
    • Pedal edema
  • Muscle tenderness (myositis)
  • Proximal or distal arm or leg weakness (myositis, steroid effect, deconditioning)
3. Serologies and other blood tests
I find rheumatologic serologies to be helpful in quite a few cases. There are certainly many patients I've seen where their rheumatologic serology results helped us avoid a surgical lung biopsy. In many cases, negative serologies help push me to have my patient undergo a surgical lung biopsy. I recommend checking a least a few serologies before performing a surgical lung biopsy.

At a minimum, the following should be checked:
- CBC with differential (for eosinophilic pneumonia)
- Anti-nuclear antibody titer (ANA)
- Rheumatoid factor (RF)
- Anti-cyclic citullinated peptide titer (CCP)

I strongly suggest also performing the following tests in most patients:
- Basic metabolic panel, including calcium
- Creatine kinase (CK)
- Hypersensitivity pneumonitis panel
- Extractable nuclear antibody (ENA) titer (includes antibodies against Scl-70, Jo-1, SSA, SSB, U1RNP, and Smith at my hospital)
- Anti-centromere antibody
- Erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- Anti-PR3 and anti-MPO antibodies (ANCA antibodies)

Some patients should also have the following tests performed:
- Full myositis panel (if myositis is suspected)
- HIV testing
- Urinalysis and microscopy (for pulmonary-renal syndromes)
- Anti-phospholipid antibodies (anti-cardiolipid antibodies, lupus anticoagulant)
- Anti-RNA polymerase 3 antibodies

All of the tests are back. What do I do know?
Now comes the hard part: you need to combine the history, physical examination, serologies, and HRCT appearance and come up with a unifying diagnosis. If a unifying diagnosis has not been clearly established by this point, you have a few options (listed here from most to least preferred):

Option 1: Refer to an ILD center
I strongly recommend referral to an ILD center -- even for a one-time consultation -- at this point in the evaluation. An ILD center can often help confirm a diagnosis without requiring a surgical lung biopsy. We can also provide guidance on management and help facilitate enrollment in clinical trials. You can find a list of ILD centers on the Pulmonary Fibrosis Foundation's website.

Option 2: Surgical lung biopsy
I think most ILD docs would prefer to provide input before a biopsy is performed, but if you do decide to proceed with a biopsy, please make sure the surgeon takes multiple large biopsies from 2-3 lobes (not just the lingula or middle lobe). Please also have the biopsy reviewed by a pulmonary pathologist with expertise in ILD.

Option 3: Prescribe prednisone
In most cases, I think this is a suboptimal management strategy for ILD that has not been fully characterized. There are some patients (e.g., very sick patients with ILD characterized by ground-glass infiltrates who do not have bronchopulmonary infection) where an "empiric trial" of corticosteroids is reasonable. In most patients, however, this approach clouds the diagnosis, makes subsequent management decisions challenging, and puts the patient at risk for all of the side effects of prednisone (including the 7-fold increased risk of death seen in idiopathic pulmonary fibrosis treated with prednisone).

What else can I do for my patient with PF?

  1. Assess oxygen requirements at rest, with exercise, and with sleep. Prescribe oxygen (and instructions on its use) to maintain SpO2 > 90% all the time. Tell your patients to buy a pulse oximeter (less then $30 without a prescription) and monitor oxygen saturations right after exertion. Teach your patients to avoid desaturation by dialing up their flow before they exert themselves.
  2. Prescribe pulmonary rehabilitation
  3. Screen for sleep apnea
  4. Vaccinate for influenza and pneumococcus
  5. Help your patient achieve a healthy weight
  6. Encourage your patient to join a PF support group (including online support groups, like the one at inspire.com).
  7. Refer your patient for clinical trial enrollment
  8. Refer your patient for lung transplantation (it is never too early)
  9. Tell your patient about all of the PF information available online
That's a good start. 

Thanks for reading
Dave Lederer

Wednesday, July 16, 2014

Does Sleep Apnea Cause Pulmonary Fibrosis? Help me found out...

I have a theory. I believe that sleep apnea causes idiopathic pulmonary fibrosis. Maybe not in everyone -- and there are definitely other risk factors that contribute to IPF. But I believe it.

Here's why:

It might surprise you, but upwards of 90% of adults with IPF have sleep apnea. Only about 25% of middle-aged men and 9% of women have sleep apnea. This link between IPF and sleep apnea doesn't prove anything, but if sleep apnea were a cause of IPF, this is what you would expect to find.

There are a lot of reasons to think that untreated sleep apnea might injure lung tissue, including excessive stretching of lung tissue, low oxygen levels during sleep, and worsening of acid reflux. Sleep apnea happens dozens of times each hour...every night...for years. Even if only a little bit of injury happens each night, it can build up over a lifetime. And injured lung tissue can heal with scar tissue in people at risk for IPF.

I discussed the connection between sleep apnea and IPF in a 20 minute lecture I gave at the Pulmonary Fibrosis Foundation Summit in La Jolla in December of 2013. You can download my slides here. I have also written about the connection between sleep apnea and IPF in a brief letter I published in 2012 and in a small research study I published in 2009. Dr. Kevin Leslie has also written about this theory, although without referring specifically to sleep apnea.

So, it makes sense that sleep apnea might contribute to the risk of IPF, but no one has ever studied whether treating sleep apnea helps the lungs in people with IPF. Sounds simple, but it takes money to do this. Over the past few months, I've been designing a clinical trial to help us find out if sleep apnea contributes to lung injury and scarring in idiopathic pulmonary fibrosis. Now we need to raise the money. Please take a look at our video and read a summary of the clinical trial at Consano.org, where you can donate money to support our project. If you would rather not donate money, please consider sharing the Consano.org site with your friends and family by email or on Facebook, Twitter, or other social media websites.

Please help me make a difference.

Dave Lederer

Friday, July 4, 2014

Please participate in Dr. Swigris' online oxygen research study at National Jewish

Dr. Jeff Swigris at National Jewish Health in Denver has received funding from the Patient Centered Outcomes Research Institute to run the Participation Program for Pulmonary Fibrosis. study the benefits of using oxygen for people with all kinds of pulmonary fibrosis. This is a free online research study that is easy to participate in from home. He is looking for people with pulmonary fibrosis and their caregivers who meet any of the following criteria:

  1. People with pulmonary fibrosis who are NOT using oxygen during the day
  2. People with pulmonary fibrosis who ARE using oxygen oxygen during the day
  3. Caregivers (usually family members) of people with pulmonary fibrosis

You can learn more here: http://pulmonaryfibrosisresearch.org/current-studies

Email Dr. Swigris' team to let them know you want to participate: support@pfresearch.org

Thursday, July 3, 2014

"We the patients and caregivers live in the here and now.”

Barbara, I stole the title of this post from your comment to my last post. Your words crystallized the message in the comments so far.

I'll be honest. I expected everyone to say they wanted to spend the Pulmonary Fibrosis healthcare dollar on research for a cure. I was prepared to make the argument that we also need to fix the problems with delivery of healthcare to patients with PF. Very happy to see that we are largely in agreement.

Here's what I heard from your comments:

1. We need to educate healthcare professionals about diagnosis and management of PF. This includes internists, pulmonologists who do not practice at an ILD or PF center, nurses, respiratory therapists, and other allied health professionals.

2. We need access to better oxygen delivery systems to allow greater mobility and time away from an electrical outlet.

3. We need better/earlier access to pulmonary rehabilitation and supplemental oxygen.

4. We need better access to support services (grief counseling, social workers)

5. We need research that leads to drugs that actually make people feel better

I completely agree with each of these points.

What else should we add to the list? Also chime in if you agree or disagree with any of these needs.

Sunday, June 29, 2014

What do you think is important for the Pulmonary Fibrosis Community?

You may have already read that the FDA is holding a public meeting on September 26, 2014 to give patients an opportunity to tell the FDA which symptoms and treatments you think are important in idiopathic pulmonary fibrosis.

This is your chance to be a part of the drug development process at the FDA! It's a public meeting -- so that means you can attend the meeting live in person or via the internet. Just click over to the registration website. On the FDA website, you will see two sets of questions that the FDA wants patients to discuss at the meeting: One set is related to symptoms and the other is related to current treatment approaches.

Symptoms and treatment approaches are probably the right things to discuss at an FDA meeting, but we need to have a bigger discussion. "We" are a community of patients, families, doctors, nurses, allied health professionals, advocates, and researchers. I want to hear what you think our communities' priorities should be. Should we focus only on a cure? on new treatments that aren't cures? on improving the way that doctors diagnose and manage PF? on creating greater access to lung transplantation? on increasing awareness of PF?

If you were in charge, how would you invest the PF healthcare dollar?

I will start chiming in with my own thoughts after you post your comments...

Sunday, May 25, 2014

IPF Clinical Trials Webinar, May 28, 2014, 1pm Central Time

The Pulmonary Fibrosis Foundation is holding a free webinar at 1pm CT on May 28, 2014 to provide information about the results of the three recent IPF clinical trials. I will be participating in the webinar, along with Greg Cosgrove, MD and Kevin Flaherty, MD. You can learn more and register here.

Thursday, May 22, 2014

"Should I take NAC for my pulmonary fibrosis?"

This has been an exciting week in the world of Pulmonary Fibrosis. The results of three well-designed clinical trials were published online and were presented at the American Thoracic Society meeting in San Diego. I reported on the results of two of these trials in a previous post. In this post, I am going to talk to you about the results of the PANTHER-IPF trial, which studied N-acetylcysteine (or "NAC") for idiopathic pulmonary fibrosis (or IPF).

Monday, May 19, 2014

Clinical trial results for pirfenidone, nintedanib, and NAC for IPF

There have been some exciting developments recently. On Sunday, May 18, 2014, The New England Journal of Medicine published the results of three clinical trials that studied three different treatments for idiopathic pulmonary fibrosis (IPF): pirfenidone, nintedanib, and N-acetylcysteine (NAC).

Friday, May 16, 2014

Expanded Access Program: You might be able to get pirfenidone in the U.S. soon...

Intermune announced an Expanded Access Program for pirfenidone for people with Idiopathic Pulmonary Fibrosis. Below is a press release from the Pulmonary Fibrosis Foundation. For more information, contact the PFF's Patient Communication Center at 844.TalkPFF (844.825.5733) or email PCC@pulmonaryfibrosis.org.

Tuesday, April 29, 2014

"I was just diagnosed with Pulmonary Fibrosis. What do I do now?"

What advice do you have for people who were just told they have PF? Here's my list.

First, get yourself an appointment at an ILD center. You can find a list of ILD Centers on the Pulmonary Fibrosis Foundation's website. It is important that you work with your doctor to come up with the most accurate diagnosis and the best possible treatment recommendations early in the course of your disease. A few years back, I published a research study that found that people with IPF who show up at an ILD Center early after the onset of symptoms seemed to have better outcomes in the long-term.

Second, get yourself a pulse oximeter. You can get one in your local drugstore or online for less than $30. A pulse oximeter is a small device that you place on your fingertip to measure oxygen levels. It tells you the percentage of blood in your arteries that is carrying oxygen. Normal oxygen levels (or "sats") are over 97%. When oxygen levels drop below around 90%, we often prescibe oxygen to be used at home. Early on, most people with PF often have normal or near-normal oxygen levels 24 hours per day. As the disease progresses, people with PF initially maintain these near-normal oxygen levels during periods of inactivity (sitting or laying down), but during exertion, oxygen levels start to drop under the 90% mark. These low oxygen levels can contribute to breathlessness and can have other negative health effects (see this post and this one too). Using oxygen at home and when out of doors to help prevent oxygen levels under 90% can be beneficial in PF. Take a look at Dr. Swigris' post on using pulse oximeters at home.

Third, talk to your doctor about pulmonary rehabilitation. Pulmonary rehabilitation is a structured outpatient exercise program for people with lung disease. This is a critically important part of staying healthy and active for people with PF. Dr. Swigris has a great post on exercise in PF.

Fourth, get accurate information about PF. There's a lot of confusing information about PF online. I strongly recommend that you reach out to the Pulmonary Fibrosis Foundation for information online at www.pulmonaryfibrosis.org. You can also call the PFF's Patient Communication Center, which is staffed by wonderful people who can give you accurate and helpful information about PF. Their phone number is 844-TalkPFF (844-825-5733). You can also email the Patient Communication Center at pcc@pulmonaryfibrosis.org. My blog and Dr. Swigris' blog also have accurate information about PF.

Fifth, get support. Involving others in you disease can help improve health outcomes and your emotional well-being. This includes your family and close friends, but also includes reaching out to others with PF. There are support groups for PF throughout the US. If you are in the US, check out the PFF's list of support groups. There are also online support groups that you and your caregivers can join.

Sorry to everyone for the two-month lapse since my last post. Just busy at work. Will be posting more in May and June.

Go ahead and add your suggestions for the newly diagnosed in the comment section below. Also feel free to share this post online through Facebook, Twitter, and other websites so other people can find this information. 

Monday, February 17, 2014

More on Predicting the Future in IPF

John R. asked the following question:

"What is your take on using the GAP Risk Assessment System as a tool to help determine a basic prognosis for the IPF patient?"

Here's my answer:

Thanks for bringing up the GAP score, John. For those who don't know about the GAP score, it is a simple tool that combines Gender, Age, and Pulmonary function test results (see the G-A-P there?) to help doctors predict how likely it is that someone with idiopathic pulmonary fibrosis (IPF) might die over the next three years.

The GAP score is an accurate tool that helps us identify people with IPF that should be considered for enrollment in clinical trials of new treatments or who might need lung transplantation. But the GAP score, just like any other prediction of the future, can't tell us what is going to happen to an individual person. Here's an example of what I mean.

Imagine that someone's GAP score was "4". About 1 out of 3 people with a GAP score of 4 will die over the following two years. This might mean that we should consider lung transplantation or enrollment in trials of new treatments -- in this way, the GAP score is indeed helpful. But this information actually does not help us know how long someone has left. Are you one of the "1 out of 3"? We still don't know the future.

Bottom line: Risk prediction tools like the GAP score can be very helpful for making decisions about treatment options, but are not really helpful if the question you are asking is "when am I going to die?" They can't answer those questions unless they predict a very high (>95%) or very low (>5%) risk, and in most cases, we don't need risk prediction tools when your risk is very high or very low, because it is often quite obvious when someone is very sick or very well.

While I do NOT recommend calculating your GAP score (for the reasons discussed above), I know everyone has already googled it. Here is a link to the calculator.

One more thing: the GAP score was developed in idiopathic pulmonary fibrosis (IPF) and may not apply to all of the other forms of Pulmonary Fibrosis. Many other forms of Pulmonary Fibrosis have better outcomes than IPF.

Sunday, February 2, 2014

Pulmonary Fibrosis Support Group at NYP/Columbia, Feb-June 2014

We will be holding our Pulmonary Fibrosis Support group at NYP/Columbia in Manhattan on the following dates during the first half of 2014:

February 20
March 20

April 17
May 15
June 19

Time: 3pm-5pm

Atchley-Loeb Conference Room
Presbyterian Hospital Building, 8th floor
622 West 168th Street
New York, NY 10032

Please try to bring your own oxygen, but we will tanks for those who need them.

All are welcome! Call Susanne or Julie in our ILD office at 212-305-8203 with any questions.

Tuesday, December 24, 2013

Am I Too Old for a Lung Transplant?

I'd like to talk about lung transplantation again. In this post, I'll try to answer the question "Am I too old for a lung transplant?" As you will see, it's not a simple thing...

Sunday, November 17, 2013

Pulmonary Fibrosis/ILD Support Group in Manhattan

We will be holding our next Pulmonary Fibrosis Support Group for patients and their caregivers at Columbia University Medical Center/New York Presbyterian Hospital on Thursday, November 21, 2013 from 3-5pm. I will be there along with our Pulmonary Fibrosis team. All are welcome!

The address is 622 W 168th Street, New York, NY 10032
Take the Presbyterian elevators to the 8th floor and go to room E107. 
Support Group is held in the Atchley-Loeb Conference Room. 
Contacts: Julie or Susanne at 212-305-8203

Hope to see you there!

Dr. Lederer

Sunday, November 10, 2013

"Should I take prednisone for my Pulmonary Fibrosis?"

I've received a number of questions about whether or not prednisone should be used to treat pulmonary fibrosis. In this post, I am going to try to help you understand the role of prednisone in the treatment of pulmonary fibrosis. I first want to remind you that you should not make any changes to your current medical treatments without discussing things with your doctor. In particular, if you stop taking a medication like prednisone,

Tuesday, October 29, 2013

The Honest Truth about Lung Transplantation: It’s a Wonderful and Terrible Thing

I want to begin a conversation about lung transplantation as a treatment for Pulmonary Fibrosis. This is a huge topic and the conversations that I have with my patients about lung transplantation range from “we need to move forward with evaluating you for lung transplantation right now” to “lung transplantation is a dangerous option for you, and we should avoid it.” There is no single conversation about lung transplantation. So writing this post has been a challenge. Perhaps this will be the first in a series of posts about lung transplantation for Pulmonary Fibrosis.

Thursday, October 24, 2013

Pulmonary Fibrosis Support Group in New York City

Hi everyone!

I wanted to alert everyone to a new Pulmonary Fibrosis Support Group that we started at Columbia University/New York Presbyterian Hospital in Manhattan. Our next support group will be held on Thursday, November 21 from 3pm to 5pm at 622 W 168th Street in Manhattan. We will be meeting on the 8th floor of the Presbyterian Hospital Building in the Atchley-Loeb Conference Room. I will be there along with the rest of our Pulmonary Fibrosis team at Columbia. We will talk about clinical trials, lung transplantation, and any other topics that people want to talk about.

Please call 212-305-8203 to RSVP.  More information available here.

New post on lung transplantation coming soon!


Saturday, October 5, 2013

“The Internet Said I Have 3 Years to Live”

I’ve been having a hard time writing this post about “prognosis”, or how long you might expect to live with Pulmonary Fibrosis. It’s a tough subject. After struggling for about 30 minutes, I realized two things:

Number one – my difficulty writing about prognosis is a pebble in comparison to your mountain of difficulty living with Pulmonary Fibrosis. So I’m going to stop complaining right now.

And number two – it’s difficult to write about prognosis because…. Well, because prognosis is a lie. A myth. Let me explain.

Friday, October 4, 2013

Dr. Swigris' Pulmonary Fibrosis Research Website and Blog

Dr. Jeff Swigris from the Pulmonary Fibrosis Program at National Jewish Health in Denver, Colorado has developed a new "patient-centered" research program for patients with Pulmonary Fibrosis. He is a world leader in the clinical care of patients with Pulmonary Fibrosis and he is also an outstanding clinical researcher studying new ways to improve the everyday life of patients with Pulmonary Fibrosis. He's got some great ideas and I encourage everyone to take a look at his website at http://pulmonaryfibrosisresearch.org. You can join his "P3F Registry" if you are interested in being considered for enrollment into research studies about Pulmonary Fibrosis.

Thursday, October 3, 2013

The Sixth Sense: Why you should use oxygen if you have Pulmonary Fibrosis!

Hi everyone!

I’m just completely blown away the overwhelming response to my blog. I’ve received comments, emails, and a few in-person thank yous. I’m pleased that you are finding the site useful. Please keep sending me your ideas for future blog posts.

Right now I’m flying at 30,000 feet on my way to Denver, Colorado for a meeting about Pulmonary Fibrosis. I’m a lung doctor, so I’m always thinking about how lungs are working (or not working), and right now I’m wondering if I’m harming myself by spending a few hours at altitude.

As you might know, the air is “thinner” at higher altitudes, which means there is simply less air to breathe. Now, I’m not breathing “30,000 feet” worth of thin air – which would be really bad. Instead, since the air in the cabin of this Boeing 757 is pressured a bit, I’m breathing about “8,000 feet” worth of thin air. For someone with normal healthy lungs breathing comfortably at sea level, about 99% of the hemoglobin in the blood leaving the heart might be carrying oxygen -- that’s a normal healthy percentage. But with the thin air up at 8,000 feet, only about 90% of the hemoglobin is carrying oxygen.

Should I be worried?

Maybe. I’ll explain why…

Tuesday, October 1, 2013

Should I join a Pulmonary Fibrosis support group?

Kathy Lindell, the nursing manager for the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease at the University of Pittsburgh is guest blogging today on our site on the importance of joining a Pulmonary Fibrosis support group. Kathy serves on the Medical Advisory Board for the Pulmonary Fibrosis Foundation and actively participates in the clinical care of patients with Pulmonary Fibrosis. Please share this with loved ones and friends and leave comments and questions for Kathy.
- David Lederer, MD

Kathleen O. Lindell, PhD, RN

Should I join a Pulmonary Fibrosis support group?
Kathleen Lindell, PhD, RN
Research Assistant Professor
Division of Pulmonary, Allergy, and Critical Care Medicine
Dorothy P. & Richard P. Simmons Center for Interstitial Lung Disease
University of Pittsburgh, Pittsburgh, PA


Hi everyone! I am thrilled to be a guest blogger on Dr. Lederer's website. As a reminder, this post represents my personal views, not those of the University of Pittsburgh.

Attending a Pulmonary Fibrosis support group is a crucial part of helping you and your caregiver(s) adapt to the many unknowns that come with a diagnosis of Pulmonary Fibrosis. The disease course of Pulmonary Fibrosis is variable, so there is no set timeline on how the disease progresses.  There is no way to predict your future, and this can cause a great deal of angst and fear of the unknown for you and your caregiver(s).

The support group should provide information so that you can learn more about your disease; topics might include:

  • What is Pulmonary Fibrosis?
  • Importance of Exercise
  • Use of Home Oxygen
  • What are the treatment options?
  • Is a lung transplant the right thing for me?

The support group members can provide you with a home where you can get help with the day-to-day life of IPF;  explaining what IPF is to your family/friends, how to travel with oxygen, etc. The benefits of attending an IPF support group include meeting other people who are going through exactly what you are going through.

Maybe you’re not the type who would attend a support group? We advise our patients (and actually write a prescription) to attend at least once and then make their own decision if it’s for them.

Maybe you do not have a support group near you. There are on-line support groups that are easy to join. Here is a link to see if there’s a support group near you or how to join an on-line support group.  http://www.pulmonaryfibrosis.org/supportgroups

Our experience has been that this disease is not patient friendly. I wish I could say it was.  BUT, our experience has also been those who attend and participate in support group live better with their disease because of their interactions with other patients and caregiver(s) who are going through the same journey.

Monday, September 9, 2013

Five reasons you should use oxygen if you have pulmonary fibrosis

Your doctor told you to use oxygen for good reasons. It's a difficult transition, but smart use of oxygen is one of the most important things you can do if you have pulmonary fibrosis. Here's why you should maintain your oxygen saturation >90% 24hrs/day:

  1. You will suffer less. Oxygen saturations < 90% make most people feel short of breath.
  2. You can do more with oxygen. Using oxygen during activities will allow you to accomplish tasks that you simply can't do any other way.
  3. You can avoid pulmonary hypertension. Avoiding low oxygen levels can help prevent a serious complication of pulmonary fibrosis called pulmonary hypertension.
  4. You can improve your chances of getting a lung transplant. Lung transplant doctors want you to be strong, active, and have a healthy weight. Using oxygen allows you to be active and participate in rehabilitation, which can turn you into a better surgical candidate!
  5. You might live longer. While not proven to be the case for patients with pulmonary fibrosis, oxygen use does help people with chronic obstructive pulmonary disease (COPD) live longer. 

In an upcoming post, I will discuss some of the practical aspects of using oxygen in and out of your home. 

    Thursday, September 5, 2013

    The Pulmonary Fibrosis Checklist: Do these 8 things!

    This is a quick post to introduce the Pulmonary Fibrosis Checklist. If you've been diagnosed with any form of pulmonary fibrosis, talk to your doctor about these 8 things...

    The Pulmonary Fibrosis Checklist

    1. Use enough oxygen to keep your oxygen saturation >90% 24 hrs/day.
    2. If eligible, participate in pulmonary rehabilitation.
    3. If you have idiopathic pulmonary fibrosis (IPF), avoid long-term use of prednisone.
    4. Consider a sleep study to check if you have sleep apnea.
    5. See if you are eligible to enroll in a clinical trial of experimental therapies.
    6. Obtain a consultation from an ILD Center for help establishing your specific diagnosis and providing management advice
    7. See if lung transplantation is an option for you. 
    8. Visit the Pulmonary Fibrosis Foundation online for information, resources, and support.
    What do you think should be added to this list?

    I will focus on each one of the Checklist items in future posts.

    Thursday, August 29, 2013

    Should I undergo a lung biopsy to diagnose my pulmonary fibrosis?

    Of course only you and your doctor can really answer this question. But there are some important things you should know before you go under the knife...

    Yes, sometimes lung biopsies are necessary in PF...
    First thing is that a lung biopsy is sometimes (but not always) needed to help diagnose the kind of PF you have. And it's important to try to figure out which kind of PF you have for the following reasons:
    1. Different kinds of PF respond to medications differently. For example, prednisone is widely used to treat PF when inflammation is present in the lung, but it turns out that prednisone is harmful if you have IPF. Sometimes a lung biopsy is needed to sort this out before deciding on a treatment plan. If I don't think the results will influence the immediate treatment plan, I sometimes still recommend a biopsy since the results will often influence future treatment decisions.

    2. Sometimes infections, serious inflammatory conditions (such as a "vasculitis"), and other conditions may be alternative explanations for the appearance of your CT scan. Lung biopsies can be very helpful in these settings.

    3. Different kinds of PF progress at different rates. Sometimes a lung biopsy helps us understand how quickly you might get sicker. This information might be helpful (for example in deciding when to seek a lung transplant evaluation), but is probably not enough of a reason to undergo a lung biopsy in most cases. 

    Tuesday, August 27, 2013

    The Alphabet Soup of Pulmonary Fibrosis

    The Alphabet Soup of PF
    It is not surprising that there's a lot of confusion out there about the different kinds of PF. Here's why...
    • There are over 100 different kinds of PF that all look very much alike
    • There is an alphabet soup of confusing and sometimes poorly defined abbreviations for many types of PF (IPF, PF, ILD, NSIP, RB-ILD.... the list goes on and on).
    • Even experts will often disagree about which kind of PF you have.
    Experts typically do agree, however, that it is important to try to sort out which kind of PF you have, since different kinds of PF behave differently both in terms of how quickly the disease might progress as well as whether it might respond to medical treatments (such as prednisone). I'll talk about how we sort out which kind of PF is affecting the lungs in future posts. In this post, I'll try to clear up the confusion about the different terms used to describe different kinds (and families) of PF

    Friday, August 23, 2013

    What is Pulmonary Fibrosis?

    The word "pulmonary" means lungs and the word "fibrosis" means scar tissue -- just like the scars you might have on your skin from previous injuries or surgeries. So, in its simplest sense, pulmonary fibrosis (sometimes called PF) means scarring in the lungs. But many people use the term PF to describe a family of over 100 diseases that affect the walls of the air sacs* of the lung. While in many cases, scar tissue is present in the air sacs, there can also be inflammation or other changes to this delicate lung tissue. The details depend on which kind of PF you have (more on this later).

    There are four consequences to the build-up of scar tissue or inflammation in the lungs. Doctors use these consequences to recognize that someone has PF.

    • Stiff lungs. Scar tissue and inflammation make your lungs too stiff. Stiff lungs are hard to stretch,

    Thursday, August 22, 2013

    Why a blog about pulmonary fibrosis?

    Why a blog about pulmonary fibrosis? You already understand why.

    There is a world of confusion out there about PF. What is it? What's the difference between IPF, PF, and ILD? What causes it? Will my kids get it? Which treatments work? Should I try stem cells? prednisone? NAC? Do I need a lung transplant?

    Top that off with the nearly universal problem that in the months and years before you are finally told that you have PF, you were probably told that your cough or difficulty breathing was due to asthma, COPD (chronic obstructive pulmonary disease), emphysema, heart disease, or sleep apnea, or that "you just need to lose weight and exercise." 

    It's no surprise that just about every single one of my patients with PF is frustrated and confused when he or she walks in the door. 

    I created this blog to try to clear up the confusion about PF. Here we go...