Living Well with a Bad Diagnosis - Lung Disease

Saturday, May 31, 2014

Recipes for the Week

Philly Cheese Steak in a Bowl
It is wet here. Thank goodness the yards really don't need me so I am shaking up my Saturday schedule. Banking will happen then I will wait a bit so it is not quite so wet, strap on my oxygen backpack then head to the ped walk right along the cliffs of the ocean. We are almost in the middle of a large ocean bay and today I want to walk north to the harbor. It may take hours! During this part of the trail, it is not unusual to see dolphins or huge pelicans or sea lions. On very rare occasions, I have even seen whales.

Afterwards, I am going to nap, do some prep work for the food we are taking to the surprise birthday party for Randy tomorrow and I have three good books waiting for me.

We have some interesting new food recipes for this week. Remember, it is important for those of us on longtime prednisone therapy to avoid sugar or foods that process into sugar because our pancreas just can't handle it well. It makes us fat. So, the goal is 3-4 ounces of lean protein and non-starchy vegetables.

Here we go:

Breakfast:
Pizza-Bellas - Portobello mushroom pizzas.  http://www.hungry-girl.com/newsletters/raw/1492-hgs-veggie-pizza-party-2-kooky-recipes
Low Carb Breakfast Casserole - I make 1/2 recipe.  http://lowcarbfamily.com/archives/506

Lunch:
Tuna Melt - To make the Tuna Salad for the Melt - http://www.genaw.com/lowcarb/tuna_salad.html now to make the Melt: http://www.genaw.com/lowcarb/tuna_melts.html
Hot and Spicy Buffalo Shrimp Dip - I will make 1/2 recipe -  http://www.skinnytaste.com/2013/01/hot-and-spicy-buffalo-shrimp-dip.html

Dinner:
Cauliflower Bake - http://mylifewellloved.com/cauliflower-casserole/
Mediterranean Shrimp over Spinach - http://www.kalynskitchen.com/2014/02/mediterranean-shrimp-spinach.html
Asian Grilled Beef with Steamed Eggplant and Mushrooms in a Peanut Sauce - I use 1 pound of meat for 4 servings and do not use the brown sugar or rice with the eggplant - http://heartmindandseoul.com/asian-grilled-beef-steak/
and
http://www.marthastewart.com/316445/steamed-eggplant-and-mushrooms-with-pean?czone=food%2Fvegetarian-cnt%2Fvegetarian-ethnic-recipes&gallery=365724&slide=316445&center=852566
Primal Philly Cheesesteak in a Bowl - http://www.health-bent.com/beef/philly-cheesesteak-in-a-bowl

Friday, May 30, 2014

Goodbye to Barbara

When William started Kindergarten at the little school way up in the hills, there was a woman hired by the donated money from the community to be a paid aide. Barbara was brilliant with conflict resolution while on playground duty. She should have worked for the State Department! I used to watch how she dealt with problems with the children, allowed the children to maintain their dignity but was clear about the issues and reached agreements of expectations of future behavior. I learned so much from her that years later, I gained a positive reputation for understanding children when working for the school where I got sick.

Winnie is now finishing first grade at the school and through Natalie, I have been invited to Barbara's retirement party at a local restaurant this afternoon. When the students graduate from the little school, they come down here to the coast for Middle and High schools. I love that the party is being held from 3-6 so the alums currently at those schools have the opportunity to walk to the restaurant downtown to say goodbye to Barbara. 

Rehab: My numbers were not quite as good yesterday but it still felt fantastic. And I wore my new sweater! Sherman was in fine form and we laughed the entire time. 

Weather: June Gloom has arrived: morning fog/clouds which clear for late afternoon sunshine, hopefully. There will soon be so many tourists on the coast when schools are out and they stick out from the masses with their shorts and goosebumps. Summers in the Bay Area are so much cooler than in Southern California. Our best weather is September after the kids go back to school and January, oddly. 

Thursday, May 29, 2014

Stain Magnet

What to wear to the weigh in yesterday? I pulled on the very first pair of white trousers I have owned in decades. I should not be allowed to wear white. Ever. What was I thinking?

The first time I ever wore them was on the road trip. We were in Raleigh and it was perfect weather for white trousers with gold sandals and a gold striped t-shirt. While walking through a very cool Asian Market, I somehow got purple ink on them in two places.

The second time I ever wore them was yesterday. Newly washed and ironed, I wore them with my Ralph Lauren black and white polka dot lightweight pullover sweater with white slingback short heals. Perfect for the weather, to see the nutritionist and to have lunch with mom.

Could I get through the day without getting anything on them? That was the goal.

So, I arrived deep into the city with unusually light traffic and the nutritionist saw me a half hour early, again. It is good to be early. I was nervous. I kept reviewing all the foods I ate the previous month. The moment arrive, I stepped on the scale and...I gained less than a pound. A true miracle. Total relief flooded my body.

I flew down the Peninsula to mom's, we chatted then headed to Nordstroms for lunch (and a bit of shopping). I found a lightweight lime green with large white squares sweater to wear with shorts or my new white trousers. It was on sale $25.00. It was my reward for a good weigh in. We went up to the Cafe, had a lovely small lunch and talked and talked and talked. I just mentioned that I had made it through the entire lunch without dropping any food onto my new white trousers! Moments later, mom was gesturing with her hands, knocked over her diet coke which immediately flowed onto my white trousers. She felt awful about it but I was laughing. Day two and I now had a huge stain all over one leg.

They will be washed and ironed again and prepared for the third wearing. I know that somehow, somewhere, I will get something on them. Some people should not be allowed to buy white. We are stain magnets.

Wednesday, May 28, 2014

The Post Vacation Weigh-In

This morning, I will be facing the scale after our road trip. I kept dreaming about it last night and it was not good. Visions of fried pickles, one slice of pizza in the car for dinner on day two, bites of french fries, green chili cheeseburgers, bites of desserts, one quarter of a muffaletta and a few more bad things danced across my brain. But, I also have to remember all the great meals, which featured foods that were good for me. All in all, I think I did okay as my smallest clothes are not tight.

The three days of heavy yard work all holiday weekend should help, too!

I find I need a goal (like the road trip) to keep on target and focused when it comes to my diet. This Sunday, we have been invited to a surprise birthday party for our friend, Randy the cop. Well, he is now a 57-year old retired Sheriff who drives a 57 Chevy. Go figure. His wife has put it all together, I am bringing an appetizer for 70 people and we had to write something to put into a memory book. Since we have known him for almost 40 years, it was hard to keep it to one page!

Back to the goal: The twin will be there. I want to look good. During the road trip, I tried to remember that I would not be able to get any weight off in time for this party. It helped.

Once Sunday's goal is past, our dear friend's son is getting married in October and I have the perfect dress for the event. I really need to not gain any weight so I can wear it. The next goal.

So, I am good with the goals through October.

A special shout out to Jeff on the first day of his 51st year. Happy 50 years!

Tuesday, May 27, 2014

Don't Miss the Conference Call This Morning re: Pirfenidone

DON'T FORGET THE CONFERENCE CALL 11AM EASTERN TIME.  The NEW number is 1-855-210-9373 and for international callers is 1-817-382-4419. The information to complete the call is below. If you have IPF, this is for you!


email header
 

A quick reminder of this morning's conference call at 11 a.m. Eastern on the data from three important Idiopathic Pulmonary Fibrosis (IPF) clinical trials. 
Due to high demand for space on the important Idiopathic Pulmonary Fibrosis (IPF) conference call we have a new larger capacity number that can handle the volume of participants.  
The NEW number is: Toll-Free 1-(855) 210-9373.  For international callers, the number is 817-382-4419.
Join an Important Coalition for Pulmonary Fibrosis Conference Call  
Tuesday May 27, 11 A.M. Eastern

Learn About Data on Three Important Drug Trial Outcomes from
Expert Andy Tager, M.D.

Dr. Tager will discuss data from from the InterMune, Boehringer Ingelheim and NIH Trials.  Mary Tagliaferri, MD from InterMune, Inc. will also be on the call to answer questions regarding the Expanded Access Program for pirfenidone.

Last week, medical experts including researchers and physicians have been discussing the positive data reported by two drug trials in idiopathic pulmonary fibrosis (IPF) and negative results of a third, and discussing the long term meaning of the data at the American Thoracic Society (ATS) meeting in San Diego.  Today, patients will have an opportunity to learn about the data and ask their own questions.

The Coalition for Pulmonary Fibrosis (CPF) is hosting a conference call for patients and families affected by IPF.  Andrew Tager, M.D., an expert in IPF from Massachusetts General Hospital in Boston, MA, will be discussing the data reported on all three trials at the ATS conference.

The CPF represents thousands of patients suffering from the disease in the US that claims as many lives each year as breast cancer yet has limited awareness and recognition. 

Who should join the call?  Pulmonary Fibrosis Patients, Family Members and Caregivers

What are the trials?  Boehringer Ingelheim’s Nintedanib, InterMune’s Pirfenidone and the National Institutes of Health’s PANTHER trial of N-acetylcysteine (NAC)

How to call in? Dial in number:  NOTE THESE ARE NEW, UPDATED NUMBERS: Toll-Free 1-(855) 210-9373.  For international callers, the number is 817-382-4419.  You may be asked for the Conference ID and it is: 52075135.  

When?  Tuesday, May 27, 11 a.m. Eastern, 10 a.m. Central, 8 a.m. Pacific

Why call?  To gain understand the meaning of the results of the drug trials and to ask questions

The call will be recorded and will be made available later in the day for those who miss the live call.  You may call the replay three or more hours after the call by calling the number below and using the Conference ID: 52075135.  The replay of the call will be available until July 27, 2014.

  
 REPLAY dial-in #: Toll-free (855) 859-2056 or (404) 537-3406
***In addition to the toll-free number listed above, participants can also dial (800) 585-8367 to access Encore***  the replay information will also be posted at http://www.coalitionforpf.org/category/news-events/pf-news/ within 3 or more hours of the call.
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 501C(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 CPF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 28,000 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 www.coalitionforpf.org or call (888) 222-8541
.
 
 
 
Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd. #343
Culver City, CA 90232
(888) 222-8541
info@coalitionforpf.org
 

Monday, May 26, 2014

Memorial Day Memories

Happy Memorial Day. A day to remember. A day to thank the past generation of soldiers for our freedoms. A day to thank the current soldiers for volunteering their service.

It is so important that families pass down the stories of brothers or sisters or fathers or mothers or uncles or aunts who served or died in previous wars. My dad told stories about some of his time in Europe but it wasn't until I was in college that he told me about being with Patton for a bit and seeing the survivors in the concentration camps. He talked about the endlessness of the war. There was no time off. He was there for four years. He was cold and skinny and miserable. In fact, we never camped as a family. He said he camped enough during the war.

His brother was in the Pacific theatre. I wrote about his terrifying experience here: http://livingwellwithabaddiagnosis.blogspot.com/2010/08/uncle-bill.html  He was injured and walked with a limp most of his life.

My mom's brother had volunteered on a run over Bremen, Germany. He was a navigator on a "Flying Fortress," they developed engine problems after the point of no return and were picked off by shrapnel as they lagged behind. He was 19-years old. My grandmother was never the same and died of a broken heart. Here is more of his story: http://livingwellwithabaddiagnosis.blogspot.com/2012/05/recovery-time.html

We knew of these stories growing up. We knew to think of them each Memorial Day and give thanks.

Sunday, May 25, 2014

Road Trip Wrap Up - With Photos!


Just one more blog about the big road trip. With photos! We compiled our favorite state, moments and food.

State:  Michael's was Northern Louisiana. He loved the people, the feeling, everyone we ran across were happy and there was a certain calmness. I chose Kentucky, which surprised even me! The joy of watching Michael at the distilleries and the beautiful drives on the tiny back roads are we drove miles and miles through the Kentucky hillside was so memorable.

Moments: The top two have to be meeting and spending time alone with the famous Jimmy Russell at Wild Turkey, master distiller celebrating 60-years at the distillery and the other at Buffalo Trace with Michael sticking his head into a vat of 23-year old Pappy Van Winkle and inhaling.

But other favorites moments include dropping in on Michael's aunt and uncle in Decatur, Ill. and meeting Joanne and Jeff, dipping a Maker's Mark bottle in the red wax, spending time with Betty and Chip, having the Waffle House waitress sing to us, dropping in on the Duck Dynasty guys, and finally finding our horrible honeymoon hotel after 40 years.

Food: Ah, the food. The best? Hands down - The Singing Pig, Williams, Az. But runner up - Manny's Buckhorn in San Antonio, NM for their green chili cheeseburgers.



We are already planning our next trip, God willing. The only thing that gives us a pause are my lungs. I know I am creeping towards transplants and maybe, just maybe I will be well enough for just one more road trip.

Saturday, May 24, 2014

NEW CONFERENCE CALL NUMBER

NEW CONFERENCE CALL PHONE NUMBER for the discussion of the new treatment available for IPF patients.


email header

Due to high demand for space on the important Idiopathic Pulmonary Fibrosis (IPF) conference call coming up on Tuesday regarding the data on three important IPF trials, we have a new larger capacity number that can handle the volume of participants.  Please make note of the NEW number for the call on Tuesday, May 27th, 11 a.m. Eastern.  
The NEW number is: Toll-Free 1-(855) 210-9373.  For international callers, the number is 817-382-4419.
Join an Important Coalition for Pulmonary Fibrosis Conference Call  
Tuesday May 27, 11 A.M. Eastern

Learn About Data on Three Important Drug Trial Outcomes from
Expert Andy Tager, M.D.

Dr. Tager will discuss data from from the InterMune, Boehringer Ingelheim and NIH Trials.  Mary Tagliaferri, MD from InterMune, Inc. will also be on the call to answer questions regarding the Expanded Access Program for pirfenidone.

Over the last several days, medical experts including researchers and physicians have been discussing the positive data reported by two drug trials in idiopathic pulmonary fibrosis (IPF) and negative results of a third, and discussing the long term meaning of the data at the American Thoracic Society (ATS) meeting in San Diego.  On Tuesday, patients will have an opportunity to learn about the data and ask their own questions.

The Coalition for Pulmonary Fibrosis (CPF) is hosting a conference call for patients and families affected by IPF.  Andrew Tager, M.D., an expert in IPF from Massachusetts General Hospital in Boston, MA, will be discussing the data reported on all three trials at the ATS conference.

The CPF represents thousands of patients suffering from the disease in the US that claims as many lives each year as breast cancer yet has limited awareness and recognition.

Who should join the call?  Pulmonary Fibrosis Patients, Family Members and Caregivers

What are the trials?  Boehringer Ingelheim’s Nintedanib, InterMune’s Pirfenidone and the National Institutes of Health’s PANTHER trial of N-acetylcysteine (NAC)

How to call in? Dial in number:  NOTE THESE ARE NEW, UPDATED NUMBERS: Toll-Free 1-(855) 210-9373.  For international callers, the number is 817-382-4419.  You may be asked for the Conference ID and it is: 52075135.  The replay of the call will be available until July 27, 2014.

When?  Tuesday, May 27, 11 a.m. Eastern, 10 a.m. Central, 8 a.m. Pacific

Why call?  To gain understand the meaning of the results of the drug trials and to ask questions

The call will be recorded and will be made available later in the day for those who miss the live call.  You may call the replay three or more hours after the call by calling the number below and using the Conference ID: 52075135.  


Encore dial-in #: Toll-free (855) 859-2056 or (404) 537-3406
***In addition to the toll-free number listed above, participants can also dial (800) 585-8367 to access Encore***  the replay information will also be posted at http://www.coalitionforpf.org/category/news-events/pf-news/ within 3 or more hours of the call.


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 501C(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 CPF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 28,000 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 www.coalitionforpf.org or call (888) 222-8541
.
 
Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd. #343
Culver City, CA 90232
(888) 222-8541
info@coalitionforpf.org

Friday, May 23, 2014

ATTENTION IPF PATIENTS: Conference Call re: Pirfenidone


email header

Join an Important Coalition for Pulmonary Fibrosis Conference Call  
Tuesday May 27, 11 A.M. Eastern

Learn About Data on Three Important Drug Trial Outcomes from
Expert Andy Tager, M.D.

Dr. Tager will discuss data from from the InterMune, Boehringer Ingelheim and NIH Trials

Over the last few days, medical experts including researchers and physicians have been discussing the positive data reported by two drug trials in idiopathic pulmonary fibrosis (IPF) and negative results of a third, and discussing the long term meaning of the data at the American Thoracic Society (ATS) meeting in San Diego.  On Tuesday, patients will have an opportunity to learn about the data and ask their own questions.

The Coalition for Pulmonary Fibrosis (CPF) is hosting a conference call for patients and families affected by IPF.  Andrew Tager, M.D., an expert in IPF from Massachusetts General Hospital in Boston, MA, will be discussing the data reported on all three trials at the ATS conference.

The CPF represents thousands of patients suffering from the disease in the US that claims as many lives each year as breast cancer yet has limited awareness and recognition.

Who should join the call?  Pulmonary Fibrosis Patients, Family Members and Caregivers

What are the trials?  Boehringer Ingelheim’s Nintedanib, InterMune’s Pirfenidone and the National Institutes of Health’s PANTHER trial of N-acetylcysteine (NAC)

How to call in? Dial in number:  1-866-551-6201, login 303-521-4080.

When?  Tuesday, May 27, 11 a.m. Eastern, 10 a.m. Central, 8 a.m. Pacific

Why call?  To gain understand the meaning of the results of the drug trials and to ask questions

The call will be recorded and will be made available later in the day for those who miss the live call.  http://www.coalitionforpf.org/category/news-events/pf-news/

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 501C(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 CPF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 28,000 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 www.coalitionforpf.org or call (888) 222-8541.
 
Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd. #343
Culver City, CA 90232
(888) 222-8541
info@coalitionforpf.org

Thursday, May 22, 2014

What I Learned While on the Road

Just want to sing,
"Which one of these is not like the others?"
It was an amazing trip. Twenty states and 6,861 miles. We never hit any storms or tornadoes or snow. There was a bit of rain while we dropped by the Duck Dynasty headquarters in West Monroe, LA but that was it. So much happened. The wild three 12-hour days push along Highway 80 was exhausting. The elevation really affected my breathing and I experienced saturation rates in the low 80s while just sitting in the car. We will never drive over the Rockies again.

I learned that I can deal with heat but not humidity. In Atlanta, I walked half of a block from our car to The Porter Beer Bar and I was very short of breath. It was like the humidity was coming up through the sidewalk and encasing me. I just couldn't breathe. So, I am no longer afraid of heat. Just humidity.

I learned that family doesn't have to sharing ancestors. We met Joanne and Jeff in Ohio. We had been in contact via email for years after Joanne found this blog. Jeff had been misdiagnosed and actually has HP. I know in my heart, that Joanne and I are somewhere related down our family trees. Too many similarities. Our mothers look alike. I met her mother, we took a photo together and I was shocked to see how much we also looked alike. My heart and soul feels very connected with Joanne. Family.

I learned that I will order a portable oxygen system next time we take a long road trip. It was too much to deal with 11 concentrators to met us along the way. And, I could have used supplemental oxygen in the car, especially over the Rockies.

I learned that we are three years older since our last road trip. Three years. We just can't put in twelve hours in a car anymore. Thankfully, our trip home from North Carolina was just four to five hours each day. It was relaxing and fun instead of pushing and trying to get someplace. Better.

I learned that what I loved more than anything was being out of a routine with very few interruptions. Life did not intrude. We were together. Alone most of the time. No phone calls. No doctors. No rehab. No nothing. It was very freeing and very relaxing.

So we are home. Back to life. Michael spent yesterday handing out bourbon and hats and stuff to people. He is back to work next Monday. But until then, we are still enjoying a slower schedule. We are meeting my mom for her shot in her eye then we are going to lunch. The three of us. I haven't seen her since before we left almost a month ago. There will not be a moment of silence!!

Wednesday, May 21, 2014

MORE INFORMATION ABOUT NEW TREATMENTS FOR IPF

Thanks to Ellen for sending this article from the New York Times regarding the two new treatments for IPF (and other ILDs down the road), which I wrote about yesterday. In 2011, I met Dr. Luca Richeldi from Modena, Italy who discovered that a cancer drug stopped lung fibrosis in one of his IPF patients being treated for ovarian cancer. (My quote from the blog: The other exciting news is that he is heading a Phase 2 study for IPF patients only of oral Triple Kinase Inhibitor BIBF 1120 which has greatly slowed the progression of fibrosis in IPF patients. He told us that these enzymes have also been successful in suppressing ovarian cancer in patients for the past 10 years. He said the genes in cancer are close to IPF.) I wrote about his talk at our ILD meeting here: http://livingwellwithabaddiagnosis.blogspot.com/2011/07/latest-drug-and-treatment-guidelines.html



Continue reading the main storyShare This Page
Continue reading the main story
Continue reading the main story

 Top Stories

This article and others like it are part of our new subscription.
Learn More »
For the first time, researchers have found drugs that can slow the decline of people with a fatal lung disease, idiopathic pulmonary fibrosis.
Studies of two experimental drugs were published in The New England Journal of Medicine and presented on Sunday in San Diego at a meeting of the American Thoracic Society. The drugs did not make patients better, but slowed the rate at which lung function got worse.
The odds of death increase as lung function decreases, so researchers hope that by stabilizing it, the drugs will prolong survival. But it is too soon to tell.
The disease causes scarring of the lungs, making it harder and harder to breathe. At least 80,000 Americans have it. Half of patients die in three to five years.
An editorial accompanying the studies called the results “a major breakthrough for patients,” but also cautioned that questions remained about how long the drugs would work and whether they would help people with severe disease.
Until now, there had been no treatments that could do anything other than relieve symptoms like coughing.
“There’s been nothing,” said Dr. Gary M. Hunninghake, the author of the editorial and a lung specialist at Brigham and Women’s Hospital in Boston. “That’s about to change. I think for a lot of patients this is going to be pretty exciting.”
Lung transplants save some patients with the disease, but many are too old or too ill for the surgery. What causes the lung scarring is unknown. Smokers and former smokers are at higher risk, but most people who smoke do not develop the disease, and many patients who do get it never smoked.
“Once you’ve been given the diagnosis, people start to plan for how long they’ll be living,” said Dr. Kevin K. Brown, an author of one of the studies and a lung specialist at National Jewish Health, a respiratory hospital in Denver. He added, “Patients hope they can get better, but pray they don’t get any worse.”
Neither of the drugs has been approved by the Food and Drug Administration. The data from the studies will be submitted to the agency as part of the process of seeking approval for the drugs.
One drug, pirfenidone, is made by InterMune, which is based in Brisbane, Calif. It has already been approved in Europe, Canada and Japan. InterMune paid for the study. The drug fights the scarring process, but how it does so is not fully understood.
The other drug, nintedanib, is made by the German company Boehringer Ingelheim, which helped pay for the study. Dr. Brown, who took part in that study, said the drug was originally being developed to treat cancer, and was tested with the lung disease when researchers learned that lung fibrosis had some biological features in common with cancer.
The drugs were studied separately and were not used in combination. Each was compared with a placebo, not with the other drug, so researchers say it is not clear whether one drug is better than the other.
Each drug was studied for a year, and one of the most important results was lung function, measured by breathing tests. Patients taking either drug had significantly smaller drops in lung function than those taking placebos. Both drugs had side effects, mostly not severe: In some patients, nintedanib caused diarrhea, and pirfenidone caused rashes and nausea.
Ed Duncan, 78, of Aurora, Colo., said he had been taking pirfenidone in various studies since 2007 or 2008. He has had idiopathic pulmonary fibrosis for 10 years, and suspects that his long survival with the disease may be due at least in part to the drug. He needs oxygen all the time, but can get about, slowly. Strenuous exercise is out of the question.
“Ten years was my bucket list,” Mr. Duncan said. “I was happy to make that. The way I feel now it could be several more years.”
InterMune announced on Friday that it would make pirfenidone available for free to some patients in the United States through an “expanded access program,” designed to help patients obtain experimental drugs that have not yet been approved. The move seems most likely to give InterMune a head start since patients will probably not have access to the other drug until it is approved.

Tuesday, May 20, 2014

TWO NEW ANNOUNCEMENTS REGARDING TREATMENTS FOR IPF

There were two major announcements regarding treatments for Idiopathic Pulmonary Fibrosis while I was on vacation. Those of us with other ILDs can be treated off label when both new drugs are finally approved, though it will be expensive. For some time, it will take working with the insurance companies to cover other ILD. But, there is hope.

The results of last year's Phase 3 worldwide study using an already approved drug to see if it stopped lung fibrosis produced good results. Here is the information. (The second release regarding Pirfenidone follows.)


Phase 3 INPULSIS™ Trials Published in the New England Journal of Medicine Show Investigational Nintedanib Slowed Lung Function Loss in People with Idiopathic Pulmonary Fibrosis
RIDGEFIELD, Conn., May 18, 2014 /True Blue Tribune/ – Boehringer Ingelheim announced the results of its two pivotal Phase 3 INPULSIS™ trials (INPULSIS™-1 and -2; NCT01335464 and NCT01335477), which were published online today in the New England Journal of Medicine (NEJM). The INPULSIS™ trials evaluated the efficacy and safety of nintedanib, an investigational therapy being studied in people with idiopathic pulmonary fibrosis (IPF). The INPULSIS™ data will also be presented at the 2014 American Thoracic Society (ATS) International Conference in a joint NEJM/Journal of American Medical Association (JAMA) session on May 18 and as a late-breaking oral presentation on May 20.
IPF is a rare, progressive and fatal lung disease that causes permanent scarring of the lungs, difficulty breathing and decreases the amount of oxygen the lungs can supply to the body. IPF affects as many as 132,000 Americans. There are currently no FDA-approved treatments.
INPULSIS™-1 and -2, which involved a total of 1,061 people with IPF, met the primary endpoint: reduction in the annual rate of decline in forced vital capacity (FVC) over 52 weeks. In these trials, nintedanib reduced the annual rate of FVC decline compared to placebo by 48% in INPULSIS™-1 (-114.7 vs. -239.9 mL/year, respectively [95% CI: 77.7, 172.8]) and by 55% in INPULSIS™-2 (-113.6 vs. -207.3 mL/year, respectively [95% CI: 44.8, 142.7]).
“IPF is a very serious disease with a high unmet medical need for which there are currently no FDA-approved treatments,” said study investigator, Luca Richeldi, M.D., Ph.D., Professor of Respiratory Medicine, Chair of Interstitial Lung Disease, University of Southampton, Honorary Consultant Physician, University Hospital Southampton. “We are excited by these data because the INPULSIS™ trials suggest evidence of nintedanib’s impact on lung function loss in patients with IPF.”
In both trials, the most prevalent adverse events (AE) were gastrointestinal. Diarrhea was the most frequent AE in the nintedanib groups compared to the placebo groups, and was reported in 61.5% vs. 18.6% of participants in INPULSIS™-1 and 63.2% vs.18.3% of participants in INPULSIS™-2. Most cases were mild to moderate in intensity (93.7% in INPULSIS™-1 and 95.2% in INPULSIS™-2). In both trials, about 4.5% of people who used nintedanib discontinued treatment due to diarrhea (n=28 of 638). In these clinical trials, investigators had the option of dose reduction or dose interruption to help manage diarrhea.
Nausea was the second most common adverse event among patients treated with nintedanib versus placebo, occurring in 22.7% vs. 5.9% of participants in INPULSIS™-1 and 26.1% vs. 7.3% of participants in INPULSIS™-2.
The absolute change from baseline in FVC at 52 weeks, a pre-specified secondary endpoint, for nintedanib versus placebo was 109.9 mL in INPULSIS™-1 (-95.1 mL, nintedanib vs. -205.0 mL, placebo [95% CI: 71.3, 148.6]) and 109.8 mL in INPULSIS™-2 (-95.3 mL, nintedanib vs. -205.0 mL, placebo [95% CI: 70.9, 148.6]).
The proportion of FVC responders (i.e., categorical change), a pre-specified secondary endpoint, was also measured based on the number of patients who had an absolute decline in FVC of less than 5%, as well as an absolute decline in FVC of less than 10% at 52 weeks.
Key secondary endpoints included time to first acute exacerbation over 52 weeks and change from baseline in health-related quality of life at 52 weeks, as assessed by St. George’s Respiratory Questionnaire (SGRQ).    
In INPULSIS™-2, there was a significant increase in time to first acute exacerbation in the nintedanib group compared with placebo (HR 0.38 [95% CI: 0.19, 0.77]). In addition, the proportion of patients with at least one investigator-reported acute exacerbation was lower in the nintedanib group (3.6%) compared with placebo (9.6%).
In INPULSIS™1, there was no difference between the nintedanib and placebo groups in time to first acute exacerbation (HR 1.15 [95% CI: 0.54, 2.42]), and the proportion of patients with at least one investigator-reported acute exacerbation was comparable between the nintedanib and placebo groups (6.1% vs. 5.4%, respectively).
In the pre-specified pooled analysis, there was no significant difference between nintedanib and placebo in time to first investigator-reported acute exacerbation (HR 0.64 [95% CI: 0.39, 1.05]). The proportion of patients with at least one investigator reported acute exacerbation was 4.9% in the nintedanib group versus 7.6% in the placebo group. A pre-specified sensitivity analysis based on adjudicated acute exacerbations (confirmed or suspected) in the pooled data was conducted and showed a benefit of nintedanib compared with placebo (HR 0.32 [95% CI: 0.16, 0.65]).
In INPULSIS™-2, there was a significantly smaller increase in SGRQ total score with nintedanib versus placebo at week 52, which is consistent with less deterioration in health-related quality of life (2.80 versus 5.48; difference of -2.69 [95% CI: -4.95, -0.43]). In INPULSIS™-1, there was no difference between the treatment groups in adjusted mean change from baseline at week 52 in SGRQ total score (4.34, nintedanib vs. 4.39, placebo [95% CI: -2.50, 2.40]).
“The results of the Phase 3 INPULSIS™ trials help bring us closer to our goal of delivering an effective treatment option for people in the U.S. with this progressive and fatal lung disease,” said Tunde Otulana, M.D., senior vice president, Clinical Development and Medical Affairs at Boehringer Ingelheim. “Boehringer Ingelheim has a long-standing heritage in treating respiratory diseases, and we continue to conduct important research in the rapidly evolving respiratory space. These nintedanib data exemplify our commitment to researching therapies for a variety of chronic lung diseases such as IPF.”
A higher proportion of patients in the nintedanib groups versus placebo experienced elevations in liver enzymes. In INPULSIS™-1, 15 patients (4.9%) in the nintedanib group and 1 patient (0.5%) on placebo experienced ALT and/or AST elevations of =3x ULN. In INPULSIS™-2, 17 patients (5.2%) in the nintedanib group and 2 patients (0.9%) on placebo experienced ALT and/or AST elevations of =3x ULN.
Overall, the proportions of people experiencing serious adverse events was similar in the nintedanib and placebo groups (31.1% vs. 27.0% in INPULSIS™-1; 29.8% vs. 32.9% in INPULSIS™-2, respectively). A total of 65 nintedanib patients (21.0%) and 22 (10.8%) placebo patients in INPULSIS-1 and 58 nintedanib patients (17.6%) and 33 placebo patients (15.1%) in INPULSIS-2 discontinued study medication due to adverse events.  
About the Phase 3 INPULSIS™ trials (INPULSIS™-1 and INPULSIS™-2)   
The double-blind, randomized and placebo-controlled trials evaluated the effect of oral nintedanib, 150 mg twice daily, on annual rate of decline in forced vital capacity FVC, in people with IPF over 52 weeks. A total of 1,061 (n=638, nintedanib vs. 423, placebo) people with IPF were enrolled in the two trials, including 513 people in INPULSIS™-1 (n=309, nintedanib vs. 204, placebo) and 548 in INPULSIS™-2 (n=329, nintedanib vs. n=219, placebo). The trials had an identical design, matched dosing, inclusion criteria, and endpoints.
The primary endpoint was the annual rate of decline in FVC (expressed in mL over 52 weeks). There were two key secondary endpoints: change from baseline in health-related quality of life, as assessed by the SGRQ total score, and time to first acute exacerbation (days). Other secondary endpoints were respiratory mortality, overall survival, on-treatment survival, and time to death or lung transplant.
Included were people over 40 years of age with a diagnosis of IPF within five years before enrollment based on the most recent American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and Latin American Thoracic Association (ALAT) IPF guideline for diagnosis and management.
Diagnosis required central review and confirmation of a combination of high resolution computerized tomography pattern and surgical lung biopsy if available.
About nintedanib 
Nintedanib is an investigational small molecule tyrosine kinase inhibitor (TKI) in development by Boehringer Ingelheim for idiopathic pulmonary fibrosis (IPF). It targets growth factors, which have been shown to be potentially involved in pulmonary fibrosis — the vascular endothelial growth factor receptor (VEGFR), fibroblast growth factor receptor (FGFR), and platelet-derived growth factor receptor (PDGFR).
About idiopathic pulmonary fibrosis  
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, severely debilitating and ultimately fatal lung disease for which there are no FDA-approved treatment options in the U.S. Although lung transplantation has been shown to improve survival, the procedure is uncommon because of the limited availability of lungs for transplantation or people are either too ill or don’t survive long enough to undergo the transplant. The incidence of IPF can vary considerably and there is some evidence that the population is increasing. IPF is characterized by progressive scarring of lung tissue and loss of lung function over time. Development of scarred tissue is called fibrosis. Over time, as the tissue thickens and stiffens with scarring, the lungs lose their ability to take in and transfer oxygen into the bloodstream, and vital organs do not get enough oxygen. As a result, individuals with IPF experience shortness of breath, cough and often have difficulty participating in everyday physical activities.
About Boehringer Ingelheim Pharmaceuticals, Inc.  
Boehringer Ingelheim Pharmaceuticals, Inc., based in Ridgefield, Conn., is the largest U.S. subsidiary of Boehringer Ingelheim Corporation (Ridgefield, CT) and a member of the Boehringer Ingelheim group of companies.
The Boehringer Ingelheim group is one of the world’s 20 leading pharmaceutical companies. Headquartered in Ingelheim, Germany, it operates globally with 142 affiliates and more than 47,400 employees. Since it was founded in 1885, the family-owned company has been committed to researching, developing, manufacturing and marketing novel medications of high therapeutic value for human and veterinary medicine.
Social responsibility is a central element of Boehringer Ingelheim’s culture. Involvement in social projects, caring for employees and their families, and providing equal opportunities for all employees form the foundation of the global operations. Mutual cooperation and respect, as well as environmental protection and sustainability are intrinsic factors in all of Boehringer Ingelheim’s endeavors.
In 2013, Boehringer Ingelheim achieved net sales of about $18.7 billion (14.1 billion euro). R&D expenditure in the Prescription Medicines business corresponds to 19.5% of its net sales.
For more information please visit http://www.us.boehringer-ingelheim.com



Here is the second release regarding Pirfenidone. If you have IPF, run not walk to your pulmonologist and discuss if this is appropriate for you. Here is the information:

IDIOPATHIC PULMONARY FIBROSIS PATIENTS TO HAVE EXPANDED ACCESS TO THERAPY
CPF Responds to InterMune’s Launch of an Expanded Access Program for Pirfenidone
 For the first time in history, people suffering from the deadly lung disease idiopathic pulmonary fibrosis (IPF) will have access to a therapy.  The Coalition for Pulmonary Fibrosis (CPF) commends the steps taken by the company, InterMune, Inc., to provide an Expanded Access Program (EAP) for the investigational drug, pirfenidone. The CPF represents thousands of patients suffering from the disease in the US that claims as many lives each year as breast cancer yet has limited awareness and recognition. 
InterMune, Inc. announced today that the company will implement an EAP for pirfenidone.  The EAP will be conducted under a treatment protocol limited to enrollment of IPF patients in the US who meet specific medical criteria.
To enroll in the EAP, a patient must be assessed by a physician who specializes in treating IPF and whose hospital or clinic center is participating in the EAP.  The patient must also meet specific eligibility requirements.  This means that not all physicians can provide access to pirfenidone through the EAP and not all patients who seek pirfenidone will be able to get access to it through EAP.
CPF encourages its patients with IPF to inquire about the EAP by calling InterMune Medical Information at 1-888-486-6411. 
"The CPF is grateful to InterMune for its long-term commitment to the search for a PF treatment, and its willingness to extend the opportunity to access pirfenidone to an additional cohort of those who meet the enrollment requirements," said Mishka Michon, CEO of the CPF.
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 501C(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 CPF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 28,000 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 www.coalitionforpf.org or call (888) 222-8541.