Pulmonary Fibrosis

PF Webinar Q&A

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The Future of Pulmonary Fibrosis Research: Fibrosis Across Organs (Webinar held Wed, Sept 26, 2012)

Listed below are post-webinar questions answered by presenters, Dr. Greg Cosgrove of National Jewish Health and Drs. Jim Kiley and Jerry Eu of the National Heart Lung and Blood Institute (NHLBI). The questions and answers provided below are specific to the webinar that was presented and is intended for educational purposes only. You should not rely on this information as a substitute for a diagnosis from your physician.

  1. Is Vanderbilt Medical Center involved as a partner in this IPF research with NIH?
  2. If I get transplanted, does this mean that I won’t have PF again?
  3. How do we get our hospital involved in the IPFNet?
  4. How can I find out if I can be involved in a clinical trial? 
  5. How can primary care physicians be better educated on early detection of IPF and appropriate referrals for care?
  6. If it is genetically related, should other family members be tested for IPF?  
  7. Is anyone doing patient testing of LPA?
  8. Is Columbia Presbyterian Hospital involved in any IPF?
  9. Is there a national sign-up registry of patient and families regarding information on family history?
  10. When is too young of an age to donate DNA for our children to the FPF Study?

Ques #1: As a well family member I am having a HRCT and bronchoscopy this Friday.  If early signs are found should I contact NIH about trials or will Vanderbilt handle this for me?  Regarding working together discussed by Dr. Eu, is Vanderbilt Medical Center involved as a partner in this IPF research with NIH? (Carolyn, NC)

Ans: NIH is sponsoring studies at Vanderbilt University and other medical centers that focus on pulmonary fibrosis patients who also have family members with this disease.  These studies are not clinical trials, however.  The doctors at Vanderbilt and their collaborators in other US medical centers (for example, National Jewish Medical Center in Denver) are trying to learn all of the genetic variants that are associated with this disease.  Doctors at Vanderbilt are also studying family members who may have early, pre-clinical stages of PF so these doctors can better understand the nature of this disease.  Please see this website for more information: http://www.mc.vanderbilt.edu/root/vumc.php?site=ipfcenter&doc=6680 .

Ques #2:  I’m 19 years old and have mild pulmonary fibrosis. I have HPS type 1, so my pulmonary fibrosis is associated with HPS. I’m planning to go to the USA to get a double lung transplant. Do you think that this will be enough for me to have a better life without PF? If I get transplanted, does this mean that I won’t have PF again? (Julie, PR)

Ans: Lung diseases rarely recur after lung transplantation.  The majority of PF patients will not have problems with PF again after a double lung transplant, but the caveat is that the number of Hermansky-Pudlak syndrome patients who have had lung transplants is still small.  However, other lung problems/complications can occur after lung transplantation, so lung transplantation is typically indicated for PF patients who have advanced disease and/or who are rapidly deteriorating.

Ques #3: How do we get our hospital involved in the IPFNet? (Anne, PA)

Ans:  The 26 medical centers across the US are regional referral centers for IPF.  IPFnet just finished enrolling IPF patients for a trial sponsored by NIH.  Many of these medical centers are now enrolling IPF patients for clinical trials sponsored by pharmaceutical companies.  They (please see (https://www.ipfnet.org/) welcome referrals from other hospitals in their regions.  Please contact a referral medical center in your region for more information.

Ques #4: How can I find out if I can be involved in a clinical trial?  I was diagnosed with IPF in 2009. Concerning genetic risk factors, I have 3 siblings with CF — two deceased and one still living. (Antoinette, MA)

Ans:  Please contact one of the IPF referral hospitals, including those in the IPFnet (www.ipfnet.org), for IPF clinical trials that are currently enrolling patients.   You may need your doctors to refer you.  In addition to patient advocacy groups’ websites, a public website (clinicaltrials.gov) can also provide information for you and your physicians to participate in IPF clinical trials.

If you have the familial form of PF and you are interested in participating in research studies, please also see our responses for questions 1 and 6.

Ques#5: How can primary care physicians be better educated on early detection of IPF and appropriate referrals for care? By the time my husband was diagnosed, he was in advanced stage and lived 3 months.  How can we raise awareness of IPF - pink ribbons everywhere, how can we gain that type of support? (Mary, MO)

Ans: More public awareness is the key.  Patients, patient advocacy groups, professional societies, academic medical centers and the NIH should continue to work together, possibly using social media, to bring more awareness to this disease.

Ques #6: If it is genetically related, should other family members be tested for IPF ?  (Mary, LA)               

Ans:  Currently these tests are available in a few research medical centers and mostly for research purposes.  For those patients and family members are participating in these studies, or for those who are recommend to have these tests done, they should also get genetic counseling from the test sites to better understand the implications of the test results.  As an example, please see this website http://www.nationaljewish.org/healthinfo/conditions/pulmonary-fibrosis/genetic-counseling/ .

Ques #7: Is anyone doing patient testing of LPA? (Len, NC)
Ans:  The measurements of LPA levels are currently for research purposes and are not yet available for clinical use.

Ques #8: Is Columbia Presbyterian Hospital involved in any IPF? (Carol, NJ)

Ans: Yes.  There are ongoing IPF trials at that hospital.  Please see this website for more info http://sklad.cumc.columbia.edu/surgery/clinicaltrials/Search_List.php?type=Pulmonary%20Fibrosis

Ques #9: Is there a national sign up registry of patient and families regarding information on family history? If not, would that help in pooling information and having contact information for a registry? (Rita, San Jose)

Ans:  PF registry is relatively complicated, as there are many other fibrotic lung diseases other than IPF.  Therefore, the clinical data going into a PF registry can be quite large and will require careful attention.  A PF registry is currently being planned by a patient advocacy group.  The planned national registry will likely be confined to a few academic medical centers initially before expanding nationwide.

Ques #10: When is too young of an age to donate DNA for our children to the FPF Study? Will this be at Vanderbiilt? (Sarah, IL)

Ans: Please contact the study coordinators for FPF studies for more detailed information (http://www.nationaljewish.org/healthinfo/conditions/pulmonary-fibrosis/research/fpf-study/  or http://www.mc.vanderbilt.edu/root/vumc.php?site=ipfcenter&doc=6680).

For further information please contact:

Courtney L. White
Director, Patient Outreach
American Thoracic Society
25 Broadway, 8th Fl.
New York, NY 10004
(212) 315-8600
cwhite@thoracic.org

Teresa Barnes
VP, Patient Outreach
Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd #343
Culver City, CA 90232
(888) 222-8541
tbarnes@coalitionforpf.org