National Patient Registry Created

I was delighted to see in the Pulmonary Fibrosis Foundation's March e-newsletter that a national patient registry and database has been created. It is called the PFF Patient Registry. The registry will allow tracking of those being treated for the disease, what treatments they are receiving, and other vital information. The Registry information will be de-identified (no unique patient identifiers), and will prove useful to researchers developing treatment, as well as physicians determining courses of care.

Heartwarming/Heartbreaking

I came across this article, which describes the successful lung transplants for two sisters who have suffered from Pulmonary Fibrosis for ten years. Both made it onto the list to receive a new lung, which is the only long-term treatment for this disease. Without a transplant, both sisters had little time left. This disease is marked by scarring of the lungs, decline of lung function, and often a sudden and rapid decline towards complete inability to breathe.

This disease, which has no known cause, kills more people each year than breast cancer. Yet it has no official national registry, and little coordinated research to determine a cause or develop a viable treatment or cure. Patients are often misdiagnosed as asthmatic until the disease is advanced, and often languish attached to an oxygen tank until that treatment is no longer enough.

It is heartwarming to hear of these two sisters, who at 69 and 71 were still healthy enough to receive a transplanted lung. It also breaks my heart, thinking about my mother, who at 61 wasn't even offered the chance at a transplant.

Unlike the women in the article, my mother didn't have health insurance. Her jobs didn't provide it, and she certainly didn't have the money to buy it on her own. The County of Sacramento provided basic care to those who couldn't afford it, which means that she could see a doctor, but only after waiting countless hours in line, and sometimes being told to come back another day.

In this system, designed primarily for crisis response and alleviating acute illness, my mom's shortness of breath likely didn't arouse much concern. She'd had a suspicious x-ray in 2003 showing spots on her lungs, perhaps five years before the shortness of breath set in. She was healthy, never a smoker, and very active. The clinic doctors figured she had asthma, gave her inhalers, and sent her on her way with no further thought.

Eventually, as her shortness of breath began to limit her ability to ride her bike and walk (her only means of transportation), they reluctantly agreed to send her to a specialist. Some two to three years after going in for shortness of breath, the pulmonologist confirmed that the spots on her lungs were scar tissue limiting her ability to breathe. His solution was to prescribe Advair (a maintenance corticosteroid inhaler) and prednisone (a steroid pill with horrendous side effects) on top of the "rescue" inhalers she had previously been given. That, and send her in for a lung biopsy to make sure it wasn't cancer.

This was in spring 2009. My mother was still working a job that kept her on her feet, riding her bike to get there, and walking for most of her other daily chores. She wasn't on supplemental oxygen (like the women in the article), and was otherwise in good health.

I'm not an expert, but this seems like this would have been a good time to suggest getting on a list for a lung transplant. Recently diagnosed with an incurable condition, but still healthy enough to go through such a taxing procedure.

Unfortunately, my mother didn't have health insurance. The pulmonologist was consulting for a county health system that likely discouraged costly procedures, if not outright forbid them. I'm sure some unspoken rule existed, devaluing the lives of those receiving care from this system. Transplants, it seems, were out of the question.

Though I did know of her diagnosis and the lack of treatment options, I didn't grasp how serious the condition was. The doctor I went to with my mother didn't seem overly concerned, and didn't really explain the long-term prognosis. If I'd understood, if it had been explained, perhaps I would have been able to do the research and find out about lung transplants as an option. Perhaps I could have gotten her on the list.

Heartbreakingly, my mother was never given the option of even waiting on the list for a lung transplant. She was given harsh steroids which eroded her quality of life, and did little to improve her lung function. As a low-income person without health insurance, she didn't have the option available, like the sisters in the recent article, to pursue life-saving treatment.

Just five years later, would coverage under the Affordable Care Act have made the difference?

Drug News

I just read that there is good news in the search for a treatment for Pulmonary Fibrosis. A large pharmaceutical company is acquiring a much smaller one- a company that is in the second phase of clinical trials for a drug which could prevent development of fibrosis.

It sounds like a genetic link was found a few years ago, where those lacking some kind of receptor, developed fibrosis when exposed to environmental triggers. The drug would block the development of fibrosis.

I'm a bit unclear what this drug would mean- is it for those who have had genetic testing and haven't developed the disease yet (but are prone to it)? Is it for those who have the disease, and it keeps it from spreading?

Whatever the answer to these questions, it sounds like good news. Read more here.

Photo Tour

A while ago I thought it would be nice to go around and take pictures of all the places that remind me of mom. Capitol Park, where we went after church when I was little. Osaka-Ya, where we got snow cones. Southside Park, the park we lived across from for years.

I started to think about this more last winter, but who wants to take pictures in winter? Now that it is nice out, I'm thinking about it more. I could ride my bike around and take pictures on a nice day. I started a google map with locations, so I would remember everything and be able to plan a good route.


View Special Places in a larger map

Happy Birthday Mom!

"Today is mom's birthday."

I started this post on mom's birthday, and couldn't bear to finish it. I had similar feelings on Mother's Day last weekend. 

Something to consider

This was posted on the IPF listserve, and I thought it would be good to consider.

You can shed tears that she is gone,
or you can smile because she has lived.

You can close your eyes and pray that she'll come back,
or you can open your eyes and see all she's left.
 
Your heart can be empty because you can't see her,
or you can be full of the love you shared.

You can turn your back on tomorrow and live yesterday,
or you can be happy for tomorrow because of yesterday.
 
You can remember her only that she is gone,
or you can cherish her memory and let it live on.

You can cry and close your mind,
be empty and turn your back.

Or you can do what she'd want:
smile, open your eyes, love and go on.

American River Parkway

I was out on the American River Parkway doing a twenty mile run on Sunday. I often run on the Parkway, and have quickly adopted the mindset that this is a real gem in Sacramento. It is very peaceful, and a great place to run, walk and bike.

While I was thinking of all of these things that make the Parkway so great, I immediately thought of mom. She would have loved the Parkway. Did she ever come to the Parkway? Or did she not know about it since most of the trail is in farther out neighborhoods? I would love to take her to the Parkway for a walk, and I know she would have enjoyed it.

It made me very sad not knowing if she knew about and enjoyed this gem, or if I should have shared it with her. It made me sad thinking that I couldn't share it with her now. I like to think she knew about it and came out to take walks or ride her bike, but I guess I'll never know.