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...





20 comments :

  1. I would spend some money on education. In this case, not education of the public, but education of our medical professionals. I understand this would be a huge undertaking. How do you "re-educate" the entire medical community around the U.S... and then the world? But it needs to happen. As a caregiver, I can't tell you how many times I had to argue with EMT's, respiratory therapists, nurses, and doctors that "yes, my mom is on 8 liters of oxygen at home and keeping her on 2 liters here in the hospital, while her saturation falls below 90%, is not going to work for her." "No... she does NOT have COPD. She has a disease called Pulmonary Fibrosis. It's restrictive, not obstructive." "Yes, she needs oxygen to go home from the hospital. She can't simply be without on the ride home." It is incredibly frustrating for caregivers and patients to have this argument frequently. It is demoralizing to be told "you don't have a medical degree so you know nothing." We are with our loved one 24x7. They live with this disease 24x7. It is disheartening that the patient/caregiver community currently knows and understands PF better than much of the medical community. I don't expect them to know everything. That would be impossible. But when doctors continue to deny supplemental oxygen to patients with PF, telling them that "too much oxygen is toxic", is not good. They need to understand when liter flows more than 2 or 3 liters are appropriate. How to teach their patients (and the caregivers and families) how to appropriately manage oxygen usage. What things to watch for. The other part of education is to teach the medical community that with a PF patient, it is not appropriate to take a "wait and see approach" to a possible respiratory infection. Hours can be the difference between life and death for someone with PF. Education is key.

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  2. Ditto everything that Taleena said!

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  3. Pulmonologists/respirologists who can't see past their COPD/Asthma glasses just because that's what they see 99% of the time in their local community-based practice. Let's check a few boxes. Yep, asthma or COPD. Differential diagnosis is definitely not their strong suit.

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  4. Education of the medical community is important, I agree but I live in the area of a large medical Center of Excellence (Yale). They are as frustrated as we are because there is nothing they can do for someone like my husband with multiple medical issues. He is beyond transplant. Meds in the pipeline are only for mild to moderate PF. Research and development of newer drugs that could help extend the quality of life for later stages along with current drugs would be my wish. Better oxygen equipment that is light and quiet and goes over 10L would be nice too.

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    1. Thanks, Judy. Better oxygen delivery & more research.

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  6. As a patient with ipf for 12 years and a single left lung recipient as of January 15,2014 and the former Executive Director of the Pulmonary Fibrosis Foundation, my thoughts on how the pf dollars should be spent have changed. In my opinion, it's quality of life for the pulmonary fibrosis patient and their families. Instead of focusing on what can't be done, focus on what can be done. Exercise/Pulmonary rehab has proven to be so helpful for everyone but especially those who have lung diseases. Dollars could go to rehab programs/respiratory therapists to create exercise programs for those with 1. pulmonary fibrosis, 2. pre-transplant and 3. post-transplant. Duke is a fine example of a pulmonary rehab program that works. I have not personally gone through their program but know several people who have. I'm sure there are other excellent programs, the wheel doesn't have to be recreated. Another component that I believe is recognized, but we do need more resources, is the grieving process that goes with losing bits of your life slowly and the changes that the patient and their families have to go through. Help families deal with the depression and the sadness and anger that some people go through. Support groups are very helpful here - staying connected to people is so important - especially people who are living the same disease you are. We learn so much from each other. Each and every pulmonary fibrosis patient and their families deserve a quality of life even if the options seem bleak. Research is important, however, it takes such a long time for research. I started at the PFF in 2004 and ten years later they have finally come out with two medications to slow the progression of the disease. Most people don't have the luxury of waiting ten years. Not only is it time consuming, but it takes a lot of money for research. Personally, I would truly like to see lung transplantation improved so it becomes more of an option for more people. In the meantime, how do we help those that "fall through the cracks" like Judy's husband and my friend Cathy and Nancy to name a few?

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    1. Hi Leanne. Congratulations on your recent lung transplant. It is great to have your input here. Thanks for sharing your thoughts -- I agree 100% with what you wrote.

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  7. I really like the comments above, and echo their ideas.

    We do need more education, and oxygen, and education about oxygen.

    We need much easier access to high flow portable oxygen and until lighter high flow portable units are designed, we need access to liquid oxygen.

    We need to change the way oxygen is prescribed to ILD patients. The prescription needs to address the need to titrate flow to maintain oxygen saturation at all exertion levels.

    We also need to increase the baseline for prescribing oxygen. Waiting until saturation is less than 88% is unacceptable. Earlier supplemental oxygen use could reduce the number of ILD patients who also acquire Pulm Hypertension and it would improve on quality of life.

    Improved education for the community pulmonologists would really help also.

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  8. Excellent points John. I was having a discussion with my cohorts at Canadian Pulmonary Fibrosis Foundation and while I appreciate the efforts towards pharmaceutical treatments/cures, we the patients and caregivers live in the here and now. The service on the oxygen and pulmonary rehab frankly sucks! Yes here in Canada as well.

    You need the pulmonary rehab at the beginning of your disease for the education and to help you manage it but most people don't qualify for coverage until "they're sick enough". Most of the pulmonary rehab is minimal.

    Here in Ontario and probably the rest of Canada and probably the US, everyone gets lumped into one dollar figure for oxygen therapy. That dollar amount may allow the oxygen suppliers to service and make some money on low flow patients where they can place a home concentrator and a portable concentrator and a few E tanks for emergency and visit maybe once a month.

    This monetary treatment has driven most of the oxygen suppliers out of the liquid oxygen business. A weekly visit to fill up reservoirs, purchasing or making liquid oxygen instead of letting the concentrators make it out of the air, is all more expensive.

    I've had no problem here getting multiple LOX strollers but I've heard horror stories in the US where Medicare dictates 1 LOX stroller. Really? Like some bureaucrat read that 1 cubic foot of LOX is equivalent to 50 gaseous oxygen and that a LOX can go for 22 hours so why would you need multiple units? Cause like dummy, that's at 2 lpm and if you're mobile on 2 lpm you can use a portable concentrator.

    With PF, often the only goal we can have is improving or at least maintaining our quality of life. Governments are recognizing that it is less expensive to provide medical care at home and likewise it is less expensive to have us in our own homes and mobile and active than tied up to oxygen in an hospital.

    These bureaucrats should be forced to live our lives for a week before they get to decide what we need.

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  9. Newly diagnosed with IPF I have found that the specialist I saw was very matter of fact due to the fact that both my parents died from this disease and my brother was diagnosed a few years back.. When I went to see him I felt as if I was written off with a wait an see attitude He poopooed the new drug that is being approved soon due to the fact that it only has a 3-4 % chance of slowing the progression and remarked the side effects which were possible slight nausea, 3 doses a day and staying out of the sun was a nusance...He also said NAC was also a waste of time...having lived with the effects of this disease I am fully aware of the outcome...however amore appropriate answer could have been it wouldn't hurt just some type of encouragement to make it more comfortable....

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    1. PF runs in your family. Have you seen a genetic specialist or counselor? National Jewish has a telephonic counseling program you could look into: http://www.nationaljewish.org/programs/directory/fpf-genetic-counseling

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    2. Have not seen anyone else as the person I saw was the head of the pulmonary division at UCLA...have no clue of where to find a genetic specialist....it would be nice to have some sort of direction... I will be going back in 2 months to see what the progression is. as I guess now we have a base. My questions are...How can you curtail the cough so its not so annoying....dealing with anxiety....and trying to live a normal life as possible. The doctor pretty much said there's nothing you can take or do so go\ on your merry way
      I started taking NAC... it does help some what ...and am looking in to the Dr in Germany who can analyze blood to get to the root of the immune issue...and waiting for the new drug to come out in @6 months. Anything else LOL I can do

      Thanks!

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    3. The cough in IPF is extremely bothersome for many people with IPF. Talk to your doctor about things that might be aggravating your cough (low oxygen levels, post-nasal drip, acid reflux, etc.).

      Anxiety is also common for many with IPF. Your doctor can help you figure out if there may be ways to treat your anxiety that involve medications, oxygen, or non-drug approaches.

      I hope that some of the content on my blog might help you live as normal a life as possible. Treatments such as oxygen and pulmonary rehabilitation help some people with IPF live a better life. If lung transplantation is an option for you, that might help too.

      There is no evidence that the blood analysis strategies are helpful in anyway in IPF.

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  10. I am so excited about the FDA meeting. I signed up to go and asked to be on a panel. Everyone has made some excellent comments here about what's important. I don't think just ONE thing is important. The items mentioned here, education of medical professionals, quality of life, oxygen requirements. I'm especially interested in improving oxygen requirements. While I'm grateful that I can roll a tank around to maintain my quality of life, they are heavy and bulky. And although I'm approved to fly by my doctor, I can't because there is no POC that's available for my liter flow (I'm on 4 LPM at rest and I'm sure it should be higher in the air) so that eliminates a lot of traveling that I can do while I'm still relatively healthy.

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    1. Thanks for sharing this, Christine. I agree that we need better oxygen delivery systems for people with PF.

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  11. Would plasmapheresis help?

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    1. Some doctors are studying whether plasmapheresis may help people with IPF when they are acutely ill. Currently, there is no evidence that plasmapheresis is helpful in IPF.

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