September is Pulmonary Fibrosis Awareness Month, Learn More About This Deadly Disease.

Idiopathic Pulmonary Fibrosis  is a disease which involves progressive scarring of the lungs of unknown cause (hence the term idiopathic).  The median survival from the time of diagnosis is 2 to 3 years, though the course is variable and many patients deteriorate rapidly.

There is little in the way of effective treatment and no cure other than lung transplantation.

There are excellent resources available to learn about this disease.

For Patients: The Pulmonary Fibrosis Foundation and Coalition for Pulmonary Fibrosis. Both resources provide information regarding patient support groups,  fund raising, and advocacy.

For Physicians: The CHEST Foundation website includes information for patients and physicians. I have found this helpful in answering many common questions for patients with newly diagnosed IPF. The author is a pulmonologist whose father died from IPF.

In addition, the Pulmonary Fibrosis Foundation conducts numerous activities throughout the year and is a great resource for information about the disease.

End of Life Conversations are Becoming End of Life Confrontations

“How Dare You!”

Life can change in a heartbeat. Most of us believe that our lives, our loves and all the that things that make us who we are is a gift from a higher power. One that can be taken away as swiftly as it is given. But somewhere in the shuffle of taking kids to practice, catching up on emails, worrying about bills, and the search for the perfect barbecue, it’s all too easy to forget the truth of life. The one truth. The one single thing that life guarantees each and every one of us. From the moment we take our first breath,  life makes to us but one promise. The promise that our life will someday end.

“Who do you think you are?”

For some of us, death comes after a lifetime of achievement, for others all too soon. For many it will be feared, for others it will be welcomed as their bodies wither away. But for more and more of us in our increasingly sterile and safe society, it is simply not to be thought of at all. An unwelcome stepchild locked tightly away in the attics of our consciousness. Like a demon in waiting, we reshape it, remake it, remold it, until it becomes an ever distant sunset that bookends a romantic dream of a life full of love, accomplishment, achievement.

“You have no right to say that!”

Until finally, that inevitable day approaches. A man or woman in a white coat tells you the terrible news that your loved one is passing away. That yes,  they are alive and can be kept alive, but there is practically no chance that they could recover. They will never go back to the person they were before.

“Where’s my regular doctor?” Continue reading “End of Life Conversations are Becoming End of Life Confrontations”

A Simple Test Can Determine Prognosis in Idiopathic Pulmonary Fibrosis

Sometimes it’s the simplest things that can make all the difference. Unfortunately, when it comes to informing patients with a new diagnosis of pulmonary fibrosis what to expect, things are anything but simple.  For years, pulmonologist, myself included, have dreaded the moment when we inform patients of a diagnosis of pulmonary fibrosis. The discussion invariably involves informing them  that this disease (which they have probably never heard of) may either kill them or have minimal effect on their lives, or have a moderate effect. Alternatively, it may have very little effect at first, but then suddenly become much worse. How can we tell which of these courses the person can expect? Well, we’re not really sure, we’ll just have to wait and see what happens.

To this day, I remain amazed at the stoicism and strength  that the  person who is sitting across from me accepts this information.  For these people there exists neither a good prognostic tool nor an effective treatment. One would think that for such a stark disease there would be 10K runs, gala fundraising dinners, and a powerful Washington lobby.

But there isn’t.

There is no nationwide clamor for research dollars. No push “for the cure” or even for any effective treatment. No distinctive ribbon clad awareness week (ok there is, but I bet you’ve never heard of it).

So it is with a great sense of excitement that we greet any new research which hints at the possibility that we can more accurately  tell our patients with this disease what they might expect in terms of prognosis. Such was the case as I read through a recent issue of Chest.

Researchers at Inova Fairfax Hospital in Falls Church, Virginia looked at the possibility of using the red cell distribution width (RDW) as a prognostic measure in patients with pulmonary fibrosis. The RDW is a blood test which measures variability in sizes of circulating red cells. The test is inexpensive, and usually drawn (and often ignored) as part of a complete blood count. Physicians who order a CBC usually pay attention to the hemoglobin, white blood cell and platelet counts, and hardly ever give the RDW a second glance.

But perhaps we should.

As variability in the size of red blood cells as measured by the RDW can tell us about pathological inflammation. The RDW has already found some use in helping with prognosis in people with Pulmonary Hypertension and Congestive Heart Failure, so why not Pulmonary Fibrosis?

In this study, they found that people who had a RDW of less than 15 had a median survival of 43 months, whereas those who had a RDW of greater than 15 had a median survival  of only 16 months. Survival appeared to be worse in those with even higher levels of RDW, and also appeared better in those with much lower levels. They also found that survival worsened in those individuals in whom the RDW increased by more than 0.01 per month on average.

Truly the idea of using a simple method to help determine the prognosis of patients with pulmonary fibrosis is exciting. Now if only we could find an equally simple method of improving that prognosis.