National Jewish Health Is Looking For Participants In A New Pulmonary Fibrosis Study

The Participation Program for Pulmonary Fibrosis (P₃F) in association with National Jewish Health are looking for participants in a new study.

Study Title: Determining the effects of supplemental oxygen on outcomes meaningful to patients with pulmonary fibrosis


Oxygen Tank Todd Huffman FLKR CC

Oxygen Tank — Todd Huffman — FLKR CC

The medical field knows very little about whether and how supplemental oxygen affects patients with PF and their loved ones.

The P₃F team (of patients, physicians, patient advocacy group representatives, PF support group leaders and others) wants to change this.


A real-world study of the effects of supplemental oxygen on outcomes meaningful to patients with pulmonary fibrosis (PF)

By joining the P₃F team, you can help move the PF field forward by participating in this innovative research study!

  • No travel required – not even to your doctor’s office
  • Sign up is simple – just contact our study personnel to get started (click here for our contact information)
  • You do not need a computer to participate but it helps if you have access to one with internet capabilities
  • There is no cost to you or your insurance

Main objective

The main objective of this study is to find out whether supplemental oxygen makes people with PF feel and function better. To do this, we need to know how PF patients feel and function before going on supplemental oxygen and then after going on supplemental oxygen.

Who is conducting the study?

The P₃F which includes investigators at National Jewish Health in Denver, Colorado are leading this research study.

Who can participate?

  • Anyone with PF
    • Regardless of cause
    • Aged 18-105 years
    • Who speaks English
    • And meets certain requirements based on prior breathing tests
  • Any 18-105 year-old English-speaking primary supporter of a PF patient (i.e. spouse, child, friend, etc.)
  • Any English-speaking physician who prescribes oxygen to a PF patient

What happens after I enroll?

  • You may be asked to participate in one or more parts of the study
  • Regardless of how you participate, you will be given a unique identifier, so we don’t have to use your name (or other private information) and can keep identifying information private
  1. If you use supplemental oxygen during the day (all the time or just with exertion), the extent of your participation will be one, 30-minute telephone interview and filling out a one-time quality of life questionnaire
  2. If you do not use supplemental oxygen at all during the day, we will collect data from you at four time-points

        a) At time of enrollment
b) Immediately before oxygen is prescribed for use during the day
c) 1 month after oxygen is prescribed for use during the day
d) 9-12 months later

What kinds of data are collected at the four time points (a, b, c and d)?

  • Four questionnaires that ask about symptoms and quality of life
  • You will be asked to wear an activity monitor on your arm for 7 days
  • You will be asked to wear a GPS monitor on your clothing for 7 days
  • The first 40 subjects who are interested will be interviewed for 30 minutes over the phone

How are the data collected?

  • Questionnaires can be filled out online, or paper versions can be mailed to you
  • The activity monitor and GPS unit are mailed to you
  • Interviews are conducted over the phone with study personnel

There is NO cost to you!

  • Telephone interviews are conducted via the P₃F toll-free phone line
  • All mailings are paid for by the study
  • Patients use pre-addressed, postage-paid envelopes to return equipment

How will you know if I my doctor wants me to start using oxygen during the day?

  • The short answer is we will stay in touch with you:
  • You will be asked to fill out a simple, 10-minute questionnaire once a month
  • You will let us know when you have appointments with your doctors
  • You will contact us if your doctor wants you to start using oxygen during the day
  • We will provide you with an informational packet to bring to your doctor visits to remind both you and your doctor about the requirements of the study

My doctor wants me to start using oxygen during the day. What now?

We are assuming the need for oxygen has come on gradually and not because something acute has occurred. If this is the case, then we will make sure your doctor is still okay with you waiting to start using supplemental oxygen until after we collect some data from you over the next 7-10 days.  We will also collect data one month after you start oxygen and finally 9-12 months after you start oxygen.

Interested? Let us know!


Hospice and Access to Medications – New CMS Guidance

Berkshires Support Group

Hospice and Access to Medications – New CMS Guidance
by John Dominguez 
IPF Support Group

On March 10, 2014, the Centers for Medicare & Medicaid Services (CMS) issued a memorandum to Part D Plan Sponsors and Medicare Hospice Providers entitled, “Part D Payment for Drugs for Beneficiaries Enrolled in Hospice – Final 2014 Guidance” (Guidance).  The Guidance identifies a billing problem related to medications after Medicare beneficiaries elect hospice, and designs a solution effective May 1, 2014.  The Guidance also describes the solution’s “implications for beneficiaries.”  ThisAlert will discuss the problem and solution.  A second Alert will discuss the identified implications for beneficiaries.


Medications that should be covered by the Medicare Hospice Benefit are sometimes paid for by the insurance companies that administer Medicare Part D plans.  To prevent this from happening, effective May 1, 2014, all prescribed medications for hospice patients billed to Medicare Part D will initially be denied coverage.  To get their medications, hospice patients will have to initiate and ultimately succeed at a Medicare appeal.  In other words, to protect insurance companies, dying patients will have to jump through hoops to get medically necessary, potentially life-sustaining medications.


Hospice is care for the dying.  It is palliative care, which means it is patient and family-centered, and optimizes quality of life by anticipating, preventing, and treating suffering.  Good hospice care addresses physical, intellectual, emotional, social and spiritual needs, and facilitates autonomy.

Since 1983 Medicare has paid for hospice care when beneficiaries are certified as having a life expectancy of 6 months or less.  Medicare providers are paid a per diem that is designed to cover all services necessary for the palliation and management of the terminal illness and related conditions.  Hospice coverage includes the cost of medications related to the terminal illness.  When Medicare beneficiaries elect the hospice benefit, they forego Medicare coverage for curative treatment related to their terminal illness, but are still eligible for Medicare coverage for all other covered care.  For instance, if a Medicare beneficiary with liver cancer elects the hospice benefit, Medicare will no longer pay for treatment to cure the cancer, but it will continue to pay for care related to other illnesses like diabetes and hypertension.

Medicare Part D

In 2006, Medicare began paying for prescription medications.  Rather than simply doing this through traditional Medicare, Congress added a new “part” to Medicare – Medicare Part D.  In an unnecessarily complex system, the prescription drug benefit is offered by an array of insurance companies, each offering one or multiple plans with a dizzying amount of formularies for beneficiaries to choose from.  Each year, Medicare beneficiaries are expected to review their medication needs and to choose the plan that offers the best formulary and the best price.


medicareThe CMS Guidance indicates that Part D Plans might inappropriately pay for Hospice medications.  This is a real possibility, but CMS’s solution to the problem inappropriately burdens the dying and their families.

When the Prescription drug benefit was added to Medicare, very little thought was given to how it would interface with the hospice benefit.  Guidance issued at the time appropriately indicated that hospice providers should pay for palliative medications related to the terminal illness and that the insurance companies that offered the Part D plans should pay for all other medications.  However, no process was created to inform the insurance companies when Medicare beneficiaries elected hospice.

The CMS Guidance raises the possibility that, after Medicare beneficiaries elect hospice, the insurance companies may continue to pay for medications that should properly be covered by the hospice benefit.   For instance, prior to electing hospice, a beneficiary may need and receive medications for pain and nausea related to cancer.   These medications would be covered by her Part D plan.  If the beneficiary elects the Hospice Benefit because of the cancer, the hospice provider becomes responsible for covering the pain and nausea medications.  However, if a family member brings the prescription to a local pharmacy, the pharmacy will bill the insurance company administering the beneficiary’s Part D plan.  Because the insurance company does not know the beneficiary elected hospice, it will probably pay for the prescription medications.

To solve this problem, CMS designed a process whereby Part D plans can find out via the Medicare Beneficiary Database when a beneficiary elects hospice.  CMS then advises them that, “drugs covered under Part D for hospice beneficiaries will be unusual and exceptional circumstances.”  The Guidance then directs the insurance companies to “place beneficiary-level prior authorization (PA) requirements on all drugs for beneficiaries who have elected hospice to determine whether the drugs are coverable under Part D.” (emphasis in original)

The new Guidance means that whenever the hospice patient or her family tries to fill a prescription at the pharmacy, the pharmacy will get a message stating, “Hospice Provider-Request Prior Authorization for Part D Drug Unrelated to the Terminal Illness or Related  Conditions.”  Then it will be the duty of the pharmacy to contact the beneficiary or prescriber to determine whether the medication is related to the terminal illness.  If the medication is related to the terminal illness, the pharmacy will bill the hospice for the cost of the medication.  If the medication is not related to the terminal illness, the pharmacy cannot fill the prescription.  Instead, the pharmacy will provide the standardized pharmacy notice with appeal rights (i.e., Prescription Drug Coverage and Your Rights – Form CMS- 10147).

So, even when it is determined that the medication is not related to the terminal illness, to get her medications the beneficiary or “appointed representative” will have to contact the insurance company and request an appeal.  If this is done, the insurance company will contact the prescriber and ask him or her to complete the prior authorization form and submit it by fax or mail.  CMS imagines that if the prescriber is unwilling to complete the prior authorization paperwork, the insurance company will call the hospice and ask it to explain whether the medication is related to the terminal illness.

If the insurance company is satisfied that the medication is not related to the terminal illness, CMS expects the Part D plan to contact the pharmacy and instruct the pharmacist regarding how to override the denial.  CMS indicates that the decision should be made within 72 hours, but that the adjudication timeframe may be extended “pending receipt of the necessary information” or “based on the facts and circumstances of the case.”

This process may prevent insurance companies from bearing the cost of hospice medications, but it does this by burdening the hospice patient and his family.  For instance:

Mr. B. was 83 years old.  He was quite healthy and independent, living an active life with the help of a few medications, including one to control his blood pressure.  After experiencing some uncomfortable indigestion, he went to his physician.  Sadly, he was diagnosed with metastatic pancreatic cancer and given a limited life expectancy.  He elected the hospice benefit.  Under this new protocol, when his wife goes to the pharmacy to re-fill his life sustaining blood pressure medication to prevent a stroke, the request for payment will be automatically denied.  This is despite the fact that Mr. B. will be at high risk for a stroke without the medication.  Mrs. B. will have to contact the insurance company to appeal the denial.  The insurance company may not speak to Mrs. B. because she is not an “appointed representative.”  Mr. B. may not feel well enough to call or to complete the required paperwork.  If Mr. B is up to negotiating the appeal process, the insurance company should then call Mr. B.’s doctor who may or may not agree to fill out the pre-authorization form.  If Mr. B’s doctor does not fill out the form, the insurance company may or may not contact the hospice provider.  In the meantime, due to a protocol designed to save the insurance company money, Mr. B. may have a stroke.


This burden-shifting to the dying patient is illogical and immoral.

CMS has erred in assuming that most hospice patients will not continue to have Part D covered medications.  Most older Americans are on medications for chronic conditions, and some of these medications, like Mr. B’s hypertension medication, are very important as they prevent premature death or devastating injury.  Further, CMS has erred in designing a system further burdening the dying and their families.  The insurance companies that administer Medicare Part D plans can easily design a system to retroactively review medications covered for hospice patients.  If appropriate, the Part D plans can seek reimbursement from hospice providers.  Such a process is already imagined in the Guidance for situations when insurance companies pay for hospice medications before determining the beneficiary has elected hospice.

Insurance companies, though not legally responsible, should continue to bear the risk of initially paying for medications for hospice patients.  In weighing the financial concerns of insurance companies against the legal rights of dying Medicare beneficiaries, clearly the rights of hospice patients and their families should prevail.



Center for Medicare Advocacy’s Alerts


CPF Wins Place in Genetic Alliance Pilot Initiative to Advance Patient-Focused Drug Development


CULVER CITY, CALIF., Nov. 5, 2013 (GLOBE NEWSWIRE) — The Coalition for Pulmonary Fibrosis (CPF) has been chosen to represent Idiopathic Pulmonary Fibrosis (IPF) patients in a new pilot initiative that will help insure patients are heard in U.S. Food and Drug Administration (FDA) drug development decisions through a technology called “Platform for Engaging Everyone Responsibly” (PEER).

In an effort to help more patients have a voice in the FDA process, Genetic Alliance and the Pharmaceutical Research and Manufacturers of America (PhRMA) developed an initiative to explore the use of a technology-enabled, crowd-sourcing approach to patient engagement as a complement to ongoing patient-focused drug development efforts under the Prescription Drug User Fee Act (PDUFA V).

“We are pleased to be given this opportunity to make sure that not only are IPF patient voices heard in the FDA drug development process, but we will be able to provide a tool to our patients to make it simple for them to do so,” said Mishka Michon, CEO of the Coalition for Pulmonary Fibrosis. “Since IPF has no FDA approved therapies, it is critical the FDA understand the patient community’s perspective.”

Background on Genetic Alliance Choice to include the CPF for IPF:

After issuing a Request for Proposals, Genetic Alliance chose advocacy organizations representing three disease areas that will be the focus of FDA patient-focused drug development public meetings in 2014 and 2015: sickle cell disease, IPF and Irritable Bowel Syndrome.  The CPF’s patient community along with the other two disease communities will pilot a crowd-sourcing, technology-enabled approach to gathering input from a diverse set of patients on key benefit-risk questions.

Background on FDA decision to include IPF in its Patient-Focused Drug Development Efforts:

IPF was chosen to be a disease focus of the FDA earlier this year when the agency announced 20 diseases for which it will hold public meetings to better understand the patient experience and patient perspective on drug development including a patients’ willingness to accept risk with treatments.  The CPF and more than 3,000 of its members sent letters to the FDA last year asking that IPF be included in the FDA list of diseases, since it was not included in the draft list posted by FDA in the fall of 2013.    The FDA has announced plans to hold the IPF workshop in March 2014.

Background on IPF:

IPF is progressive and causes deadly scarring in the lungs that renders the patient breathless and debilitated.  Unlike breast cancer PF has no FDA approved therapies and no cure.  Scientists don’t know yet what causes the disease that affects 200,000 Americans.  The average lifespan, post diagnosis, is less than three years.

To learn more about PF, visit 

About Pulmonary Fibrosis (PF)

Pulmonary Fibrosis (PF) is a lung disorder characterized by a progressive scarring – known as fibrosis – and deterioration of the lungs, which slowly robs its victims of their ability to breathe. Approximately 200,000 Americans suffer from PF, and there is currently no known cause or cure. An estimated 48,000 new cases are diagnosed each year. PF is difficult to diagnose and an estimated two-thirds of patients die within five years of diagnosis.  Sometimes PF can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis (IPF).

About the CPF

The CPF is a 501(c)(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis (PF), while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease. The CPF funds promising research into new approaches to treat and cure PF; provides patients and families with comprehensive education materials, resources, and hope; serves as a voice for national advocacy of PF issues; and works to improve awareness of PF in the medical community as well as the general public.  The ‘PF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S.  With more than 27,000 members nationwide, the CPF is the largest nonprofit organization in the U.S. dedicated to advocating for those with PF.  For more information please visit or call (888) 222-8541.

 Read More: 

Finding cellular causes of lung-hardening disease

lungsby Tracey Peake

(Medical Xpress)—Idiopathic Pulmonary Fibrosis, or IPF, is an incurable lung disease that, over time, turns healthy lung tissue into inflexible scar tissue – hardening the lungs and eventually causing respiratory distress and death. Currently, there is no cure.

Phil Sannes, a professor of cell biology, studies IPF on the cellular level. In his most recent research, he’s found that in the case of IPF patients, three growth factors within different types of cells in the lung may be working together to cause the disease.

Previous research, including earlier studies from Sannes’ lab, established two signaling molecules that seemed to be involved in the development of IPF: Wnt7B and TGF-?.  In normal lungs, Wnt7B regulates the epithelial cells on the surface of the lung, and TGF-? plays a role in the development of fibroblasts in the layers underneath the epithelium.  When the lung is damaged, Wnt7B and TGF-? help cells in the lung work together to make sure that the injured area is sealed off and the epithelium can repair itself.   If the epithelium can’t recover within roughly 48 hours after the injury, then fibroblast production accelerates and the quick growing fibroblasts create a scar.  When the epithelium recovers, fibroblast production gets turned off and the scar eventually resolves itself.

In IPF, fibroblast production is stuck in overdrive.  Somehow, the fibroblasts don’t get the message to stop producing.  Sannes’ latest work, supported by the NIH, identifies a third possible player in the mix, a signaling molecule known as fibroblast growth factor nine (FGF9).  In a paper in the Journal of Histochemistry and Cytochemistry, Sannes shows that FGF9, which is normally found only in the smooth muscle tissue in the lungs, also appears in epithelial cells of IPF patients.

“FGF9 is important to lung development, but in normal lungs it’s only found in small amounts in smooth muscle tissue,” Sannes says.  “In fact, none of the three molecules are expressed a lot in normal lung – just when they’re needed in the healing process.

“Finding FGF9 out of place like this is unusual.  We don’t know why it’s there or what its role is, but our operating hypothesis is that in an IPF lung FGF9, Wnt7B and TGF-? are acting together in a coordinated way.  Our next steps will be to find out how this combination may lead to development of IPF.”

Explore further: Metabolomics key to identifying disease pathway: Research reveals lactic acid’s role in lung disease

More information:
Provided by North Carolina State University