Living Well with a Bad Diagnosis - Lung Disease

Monday, October 31, 2011

Halloween

Rocky Road Popcorn Balls
Happy Halloween.

Our plans? Rehab, of course. Bills and banks and errands. Michael is planning to be home early tonight. Winnie, her brother Oliver and her parents Natalie and Ethan are coming to show us their costumes around 5:30. I am going to serve some cider (some good Oktoberfest beer for the adults), pumpkin pie with whipped cream and cookies.

Also, Melanie's birthday is today. She is the sophomore in high school who lives down the street and recently spent the night with us while her parents were out of town. Every year since she was born, she and her parents come down early in the evening for some sweets and a present.

Yesterday, I made Rocky Road popcorn balls with marshmallows and chocolate chips. Too good! Everyone will be going home with a goodie bag of these sweet morsels of goodness along with Halloween candy.

All the kids on our street and their parents will also get a goodie bag with the popcorn balls in them. Along with candy, of course.

We love seeing the costumes on all the little ones who visit us through the evening. Hope you have visits from lots of ghosts and goblins, too!

Sunday, October 30, 2011

Pain Management and Cardinals

My 83-year old mom had the procedure on her back again, this after rehab for her broken pelvis hurt something in her broken back. Lots of broken bones in that woman!

We returned to the same doctor and hospital where she was relieved of all her back pain earlier this year. The doctor was able to “zap” the nerves once again and she walked out of the surgery center pain free. A miracle.

It was good to spend some time with her. We never stopped talking. I am so grateful that she is still alive and active and living life to the fullest. She is a role model to me.

It was Friday night so the traffic was heavy driving home. It was also the final game of the World's Series. As a member of a family of generational St. Louis Cardinal fans, we were glued to the first several innings until Michael notice how beautiful the evening was outside. So, we lit the fire pots, turned on the fountain, he made himself a cocktail and we sat outside to get caught up with each other's day.

It wasn't until the 7th inning that we make it back into the house. Then the excitement began. My poor brother was still recovering from the Thursday night game when he said he was rolling on the floor as the pain and torture watching it was driving him crazy. During the game on Friday, he was so amazed that his team was even in the World Series but he was dying with every out. Six outs to a win. Three outs. We texted each other. When they won, I phoned him and he was in total shock. It was so great to share the moment with him.

So, in a way, both my mom and brother were relieved of their pain on Friday.

Saturday, October 29, 2011

Nothing is Impossible

That is the title of a book I just finished reading written by Christopher Reeve. Superman. It is about living as a quadriplegic after a horse riding accident. Its subtitle is “Reflections of a New Life.” My friend Barbara sent it for me to read. We have very different problems, Christopher and I, but really not so different outlooks or aspects on dealing with a new life. A changed life.

The chapters are broken down to subjects of Humor, Parenting, Religion, Advocacy and Hope among others. These are all subjects that have been written here in this blog from the beginning.

There were other correlations. He mentioned several times that he did not except failure well. He also did not handle criticism well. He clearly was an Alpha male and a Type A personality. After reading about his determination to walk again, I realized what I suspected all along - that I really am an Alpha female and a Type A personality though my edges have been softened living with a decidedly Type B non-Alpha male. This non-Alpha male does not let me dominate or control him which I have been trying to do for 38 years!

One reference was also very similar and made me laugh out loud was the philosophy of his physical therapist:

“Show a Type A personally a display of his achievement (or lack thereof) and you can harness his competitive nature to motivate improvement.”

Boy, did that ring true to me. If the tech can show me the computer screen while I am doing the Pulmonary Function Tests, I always do better. I want to do the best ever. Every time. 

What I came away with was a story of a very strong man who was very vain and hated his situation but loved his life, family and friends enough to stay in the world of the living and tried to make sense of it all.

It is inspirational. It is mind-boggling. It is worth your time.

Friday, October 28, 2011

Pulmonary Fibrosis Fundraising Walks in Southern California

I received an e-mail from the Coalition for Pulmonary Fibrosis regarding several fundraisers. If you are in the Southern California area, you may wish to participate in the first of many Walk for Their Breath fundraiser. The information is below:

Please help support our local fundraisers in their upcoming Pulmonary Fibrosis walks – it’s not too late to register!  If you can’t attend but would like to support them in their efforts to raise awareness and funds for research/patient support, please visit their online pages below. 

Saturday November 5, 2011
1st Annual – Walk for Their Next Breath Hosted by Liz McDonald
Santa Monica, CA
Clover Park

Saturday November 12, 2011
1st Annual – Walk for Their Next Breath Hosted by Wilma Vijandre
Ontario, CA
Cucamonga - Guasti Park

Su Hwang
Director, Special Events
Associate Director, Development

Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd #343
Culver City, CA 90232

888-222-8541 x704

Thursday, October 27, 2011

Results of Recent PFTs and HRCT Scan

HRCT Scan of Hypersensitivity Pneumonitis with "ground glass"
On Tuesday afternoon, I met with Dr. K. to review all my tests. The results were amazing.

I have gained 200ccs in vital lung capacity and my DLCO (diffusion) is the highest level ever – just under 14 after an adjustment for hemoglobin. It was 11 during the last test.

She also said that my HRCT Scan has not changed since 2008, that is almost 4 years. That alone is remarkable.

What was even more interesting is that she feels that I can be weaned off of supplemental oxygen. She asked that when I am off of oxygen during rehab, that the RNs contact her so she can arrange to have my saturation levels checked while I am sleeping.

Wouldn’t it be marvelous not to need oxygen when we travel? Or to sleep? Or to exercise? I never thought I would be without it again. Fingers crossed that it actually happens.


My medical biologist friend Lois just sent this in an e-mail: 
All your hard work with rehab and exercise and losing the weight is really paying off.  Recently, I read a review article in Science on autophagy, a process of recycling damaged materials, proteins and lipids and DNA, from mitochondria. This is greatly stimulated by exercise and caloric reduction.  So what you have been doing has a mechanistic explanation.

I feel great. Everything is going well but this time of year can be off putting. Staying healthy is the goal so I don’t lose any of the recent gains.

Wednesday, October 26, 2011

News from Pulmonary FIbrosis Foundation (PFF) Regarding New Research Fund


Below is exciting news from the good people at The Pulmonary Fibrosis Foundation regarding a new research fund. This fund’s goal is finding a cure for pulmonary fibrosis.
Pulmonary Fibrosis Foundation Launches Research Fund to Cure Pulmonary Fibrosis
POSTED BY MATT DERDA ON 10/17/11

New Awards Totaling $200,000 to be Offered in 2012;
Deadline for Letters of Intent is November 17, 2011

CHICAGO, October 17, 2011 /PRNewswire/ -- The Pulmonary Fibrosis Foundation (PFF) announced today that it has established the PFF Research Fund to Cure Pulmonary Fibrosis, a fund created to support research that will assist in the development of successful therapies for pulmonary fibrosis (PF). PF is a progressive and ultimately fatal lung disease that affects more than 200,000 Americans, and takes an estimated 40,000 lives each year. The primary goal of the Fund is to help identify new treatments, and ultimately a cure for PF
There are currently no FDA-approved therapies to treat PF, and there is no known cure. The Research Fund to Cure Pulmonary Fibrosis has been established to support projects in the areas of basic science, clinical research, or translational research.
"The Foundation is pleased to be able to establish the Research Fund, which will be a critical part of our mission to cure pulmonary fibrosis for years to come," said Daniel M. Rose, MD, President and Chief Executive Officer of the Pulmonary Fibrosis Foundation. "We sincerely hope that these new Awards, and future Awards, will help provide the necessary research that will advance our understanding of PF and related conditions, and eventually lead to a cure."
In 2012, four recipients will receive Awards totaling $200,000:
Young Investigator Awards - $100,000 to be given over two years. This award category was established to encourage young investigators (researchers within 5 years of completion of their formal training) to maintain and enhance their interest in PF research during the early stages of their academic career.
Established Investigator Awards - $100,000 to be given over two years for established investigators to explore preliminary innovative areas of research that may not yet be eligible for an NIH grant.
Further details for each Award category, including specific criteria for investigator eligibility and an annual calendar of deadlines, are available on the PFF's website at www.pulmonaryfibrosis.org.
The Chairman of the Research Advisory Committee is Jesse Roman, MD, Chairman of the Department of Medicine at the University of Louisville (Louisville, KY). The Research Advisory Committee is comprised of respected PF researchers who are recognized leaders in the field. Applications will be scored based on their scientific merit, novelty, and responsiveness to the specific purpose of each Award category.
"Once again the PFF has stepped up to the plate to address a very important need - support for innovative research in lung fibrosis and the recruitment/retention of talented investigators into this field. Investing in research and future leaders in pulmonary fibrosis research is critical for promoting advancements in the diagnosis and treatment of lung fibrosis. It is an honor to support this important endeavor," said Dr. Roman.
For more information on the Research Fund to Cure Pulmonary Fibrosis, or to learn more about the other programs and services offered by the PFF for the PF community, please visit www.pulmonaryfibrosis.org or call 888-733-6741.
About Pulmonary Fibrosis
According to the National Institutes of Health, pulmonary fibrosis is a condition in which over a period of time the lung tissue becomes thickened, stiff, and scarred. The development of the scar tissue is called fibrosis. As the lung tissue becomes scarred and thicker, the lungs lose their ability to transfer oxygen into the bloodstream. As a result, the brain and other organs don't receive the oxygen they need. In some cases, doctors can determine the cause of the fibrosis, but in most cases, there is no known cause. When there is no known etiology for the fibrosis (and certain pathologic criteria are met) the disease is called idiopathic pulmonary fibrosis or IPF. IPF affects approximately 200,000 individuals in the US, and the annual mortality is estimated to be 40,000. At present there are no FDA-approved medical therapies.
About the Pulmonary Fibrosis Foundation
The mission of the Pulmonary Fibrosis Foundation is to help find a cure for idiopathic pulmonary fibrosis (IPF), advocate for the pulmonary fibrosis community both locally and in Washington D.C., promote disease awareness, and provide a compassionate environment for patients and their families. The PFF collaborates with physicians, organizations, patients, and caregivers worldwide. From December 1-3, 2011, the PFF will host its first international scientific conference, IPF Summit 2011: From Bench to Bedside, in Chicago. With more than 60,000 constituents worldwide, the Pulmonary Fibrosis Foundation is the international resource for the pulmonary fibrosis community. For more information visit www.pulmonaryfibrosis.org or call 888-733-6741.

Tuesday, October 25, 2011

Goodbye Weight Management Clinic

Gyro Burger with Greek Salad
Yesterday was a sad but glorious day. I graduated. They threw me out. After seeing the nutritionist, Mr. B. on December 23, 2010 and Dr. M-J shortly afterwards and working together for 10-months, we said our goodbyes. With a smile and a hug.

To recap: I lost 60 pounds in six months with the ongoing range of 17.5 to 40 mgs of prednisone in my body for six years. Three months included a liquid and protein bar diet which accounted for 33 pounds of the total weight loss. I was in food hell. It was isolating. It was awful.

It has been oddly easy to maintain my weight loss as I have a deep rooted fear of having to return to the liquid diet. I never want to go there again. Ever.

At this point, I can eat whatever I want as long as I stay under 1,400 calories a day. I also can eat one of the formally banned foods as long as it is pared with protein but only two servings of it a week.

There is something psychological about knowing that you can eat anything to really make you not need to eat everything. I find that I don’t stray far from my regular diet though I do revel in having a piece of fresh bread with butter (shocking!) when we go out to lunch or dinner. I feel naughty. It tastes soooo good. A rare treat.

So, as one reader asked, what do you eat?

A reminder that I use low fat everything and adjust the recipes so the protein serves 4ozs per serving for 4 servings. We have leftovers for the next night’s dinner. Here is the menu for next week with the dinner recipes:

Breakfast: Crustless Quiche made with Egg Beaters, non-fat cottage cheese, low fat shredded Jack cheese, smoked salmon, scallions and dill.

Lunch:  ½ of a chicken breast that has been roasted on the bone or canned white tuna both served with chopped veggies or shredded cabbage mixed with a small amount of salad dressing.

Dinner:
Baked Meatballs served with Pasta Slim (no carbs)

Cheesy Chili Chicken with an artichoke

Gyro Burger with Greek Salad

Pecan Chicken with an artichoke

Preparation is the true secret of my maintenance success. I must have the food in the house and a menu so there is no last minute panic or choice that may not be the best for me.

My goal for years has been to lose weight and now it is to keep it off. But, it also allows me to begin to address other goals, ones that I had dismissed to focus on weight loss. It is going to be an interesting year ahead.

Monday, October 24, 2011

Scary News about N-acetylcysteine (NAC) PANTHER Study


Lots to read today. I heard such great news from several people in my ILD Support Groups who had added N-acetylcysteine to their drug therapy that I started taking it about two months ago. There was an actual study called PANTHER that was tracking IPF volunteers taking this supplement along with prednisone and azathioprine. It is available over the counter. After reading the below article, I am stopping immediately. SEE UPDATE BELOW.
Commonly Used Three-Drug Regimen for Idiopathic Pulmonary Fibrosis Found Harmful
POSTED BY CARA SCHILLINGER ON 10/21/11
NIH stops one treatment arm of trial; other two treatments to continue
The National Heart, Lung, and Blood Institute (NHLBI), part of the National Institutes of Health, has stopped one arm of a three arm multi-center, clinical trial studying treatments for the lung-scarring disease idiopathic pulmonary fibrosis (IPF) for safety concerns. The trial found that people with IPF receiving a currently used triple-drug therapy consisting of prednisone, azathioprine, and N-acetylcysteine (NAC) had worse outcomes than those who received placebos or inactive substances.
"These findings underscore why treatments must be evaluated in a rigorous manner," said Susan B. Shurin, M.D., acting director of the NHLBI. "This combination therapy is widely used in patients with IPF, but has not previously been studied in direct comparison to a placebo for all three drugs."
The interim results from this study showed that compared to placebo, those assigned to triple therapy had greater mortality (11 percent versus 1 percent), more hospitalizations (29 percent versus 8 percent), and more serious adverse events (31 percent versus 9 percent) and also had no difference in lung function test changes. Participants randomly assigned to the triple- therapy arm also remained on their assigned treatment at a much lower rate (78 percent adherence versus 98 percent adherence).
"Anyone on some combination of these medications with questions or concerns should consult with their health care provider and not simply stop taking the drugs," said Ganesh Raghu, M.D., professor of medicine at the University of Washington, Seattle and a co-chair of this IPF study. "It is important to realize that these results definitively apply only to patients with well-defined IPF and not to people taking a combination of these drugs for other lung diseases or conditions."
The other two study arms, or intervention groups, of this IPF trial comparing NAC alone to placebo alone will continue. In stopping this part of the trial, the NHLBI accepted the recommendation of the Data and Safety Monitoring Board (DSMB) – an independent advisory group of experts in lung disease, biostatistics, medical ethics, and clinical trial design. The DSMB has been monitoring the study since it began.
This study, called PANTHER-IPF (Prednisone, Azathioprine, and N-acetylcysteine: A Study that Evaluates Response in Idiopathic Pulmonary Fibrosis) was designed and conducted by the Idiopathic Pulmonary Fibrosis Clinical Research Network, funded by the NHLBI. The PANTHER-IPF study was designed to evaluate whether this commonly used triple-therapy regimen could slow disease progression and improve lung function in people with moderate IPF.
PANTHER-IPF was the first study in IPF comparing the effectiveness of this combined treatment to a placebo for all three drugs. Each participant had a one in three chance of being randomized to receive the triple drug regimen, NAC alone, or placebo for a period of up to 60 weeks.
"We will continue to analyze the data to try to understand why this particular combination may be detrimental in people with IPF," said Fernando Martinez, M.D., professor of medicine, University of Michigan, Ann Arbor and co-chair of the PANTHER-IPF study. "The results are not explained by any differences between the two groups before the treatments started."
IPF is a progressive and currently incurable disease characterized by the buildup of fibrous scar tissue within the lungs. This accumulation of scar tissue leads to breathing difficulties, coughing, chest pain, and fatigue. Approximately 200,000 people in the United States have IPF. The cause or causes of IPF remain unknown; as a result treatment options remain limited. PANTHER-IPF began enrollment in October 2009.
The study had enrolled 238 of a planned 390 participants prior to the stop announcement. Participants ranged from 48 to 85 years of age, with an average age of 68. The placebo and NAC arms will continue enrolling and following their participants, and this part of the PANTHER-IPF study is expected to be completed by late 2013.
In addition to NIH funding, the Cowlin Family Fund at Chicago Community Trust provided financial support for this study. Zambon donated the NAC and matching placebo; the prednisone, azathioprine, and their matching placebos were purchased using study funds.
Find more information about this clinical trial at http://clinicaltrials.gov/ct2/show/NCT00650091
To arrange an interview with an NHLBI spokesperson, please contact the NHLBI Communications Office at (301) 496-4236 or nhlbi_news@nhlbi.nih.gov.


UPDATE:  On November 2nd, I received an e-mail from the head of my ILD Support Group regarding this issue. In the e-mail, they noted that people who are on this drug along with prednisone and azathioprine should continue taking the NAC if they are diagnosed with chronic Hypersensitivity Pneumonitis or a connective tissue related lung disease. So, I have begun taking NAC again.

Sunday, October 23, 2011

Don's Birthday Event

Tired. Okay, I did a bit too much yesterday. We had a really perfect evening celebrating Don's birthday. Appetizers were served on the back patio as the sun set with the background music provided by the sounds of the water in the fountain. It was so warm that we were in short sleeved shirts in the darkness. That is such a rare event here on the coastside.

Dinner was perfect and I sent plates full of food and cake home with Don. The only problem is that he leaves for Hawaii at 7:00 this morning. He said he was going to enjoy it all for breakfast!

The highlight of the evening was when Michael and I serenaded him with a song Michael learned from a Los Angeles TV show "Engineer Bill" in the 1960's:

"Put another candle on your birthday cake, I'm gonna bake a birthday cake.
Put another candle on your birthday cake, You're another year old today.
Happy Birthday to you. You're another year old today."

Don was laughing so hard, I thought he was going to hurt himself!

We climbed into bed at 11:00 and passed out until 6:15 this morning.

The dilemma: Michael invited me to join him over the hill. He has to do some work so I can use that time stalking sales at the mall and afterwards we will have a nice long drive home together through the Redwoods. Tempting. The other choice is to stay home in the clean house with the perfect gardens with nothing to do but sit in the garden and rest.

I tend to always want to keep moving especially when I have to weight-in at the Weight Management Clinic tomorrow morning. No decision is made yet.

Enjoy your Sunday.

Saturday, October 22, 2011

Sharing Information


In the beginning of my illness, I didn't understand anything the doctors told me. It was Greek to me. It was a lot of information. My future was unclear. I couldn't get real answers. It was both frustrating and frightening. I wanted to talk with someone who had my specific disease to see how they lived and there were so many questions I would have asked. There was no one.

When I met Jo at the ILD luncheon, I thought she was in that same place. I offered to meet her for coffee yesterday. It was a much different conversation that what I had anticipated. She really has her act together. What I perceived as not dealing with the disease, she is handling it in her own way and, in the end, very similar to me.

I really like her. She is a strong woman. She decided not to have a lung biopsy but three different hospital groups gave her the same diagnosis. It is rather clear. She has not told a lot of people that she is ill. I really didn't understand that until we spoke. She is a real estate broker and if people discovered she is ill, they wouldn't hire her because she might not be able to see the sell to the end. I get it.

We didn't just talk about being ill. We talked about our children and our lives. She was one good mother to their three kids. Great stories were exchanged.

Before leaving, we both realized that we have not let this disease control our lives. Yes, we give it respect and we do not ignore it, but we wrap it into the texture of our lives.

She is choosing to living just fine with a bad diagnosis.

Friday, October 21, 2011

Preparing

Pancetta and porcini mushroom potato gratin
Busy weekend and week ahead. I'm trying to not feel overwhelmed. One step and day at a time. Today, I am off to the other rehab within the hour, meeting someone I met at the ILD Luncheon who is at the beginning of her journey followed by a trip to the butcher and Trader Joe's. Then, the back yard needs cutting.

Our friend Don is coming for a birthday dinner on Saturday night. Rib eye steak on the grill, pancetta and porcini mushroom potato gratin and heirloom tomato salad followed by a carrot cake with candles. So, tonight the carrot cake will be finished and in the refrigerator before bedtime.

A very busy, full day.

Tomorrow, I will do a quick dust and vacuum, set the table and start cooking. We love Don and so looking forward to hearing his funny stories with his British accent and wit.

Monday and Tuesday are doctor days. It will be my final visit with Dr. M-J at the Weight Management Group on Monday and a quick check-up with Dr. K, the lung goddess, for a review of the PFT's and HRCT.

Wednesday is the late night orchestra rehearsal.

Friday, I am driving the 100 miles to my mom's for another procedure on her back. It will be a long day.

Saturday, we have been invited to crazy Ricky's house for a huge Italian dinner made by his wonderful wife, Fran. I am so looking forward to spending time with them.

The calendar is rather scary and I have to admit that I am nervous that it will be too much and I will get sick. I am going to take my vitamins, Airborne and try to get as much sleep as possible. I will remember to wash my hands a lot and be very careful with my diet.

Sunday, I plan to spend all day reading and napping. Recovery time.

Thursday, October 20, 2011

Stay Well


I am breathing so well. It is fall. Cooler. Cleaner air. Lower humidity. I find myself doing things that I have not done in years. A week ago, I did all the yard work without oxygen. About halfway through, I realized it and was even more surprised that I was working hard and not short of breath.

But, all of us who are struggling with lung disease need to be careful. We are entering cold and flu season, both of which can make us very ill or even kill us. Now is the time to wash our hands a lot, get a flu shot, begin to take extra vitamin C, have a few packages of Airborne on hand for the first signs of trouble and the secret weapon against colds - Vick's Vapor Rub. At the first tickle in the throat or sneeze, for two nights just before bedtime, rub some of it onto the bottom of the feet, encase them in socks and it seems to stop a cold from taking hold.

Spouses also need to stay healthy so they don't bring illness into the household.

In rehab, so many of the people who have an obstructive lung diseases like COPD fear this time of year. They have problems with bronchitis and even pneumonia. Several told me that every year they ends up in the hospital with one or the other.

So please take care. Stay away from large crowds of people. Take vitimins. Don't shake hands. Wash hands often. Stay well.

Wednesday, October 19, 2011

Day Off

This photo looks similar to Jay and Nancy's house in their Del Webb Community.
We moved into our home on William’s 1st birthday. Next door was a family who’s son just took off for UC Berkeley and their daughter was still in high school. This was almost 30 years ago. Jay and Nancy were very private people who had a very small circle of friends.

Nancy and I began to chat while we worked in our yards. That grew to doing big yard projects together – chopping down trees and laying sod. She taught me how to make pies and set aside part of her vegetable garden for William. They would plant, water, weed and harvest together.

It was a horribly sad day when they decided to retire hours north of us where she was raised. They had family there. Often, we would visit with them or they would come to visit us. We have kept in touch though the years.

They are in their 70's now and Jay will turn 80 next year. They moved again about four years ago to be near their grandchildren and to help their daughter-in-law with their three children. Their son travels a lot for his huge job so they help out whenever possible.

Yesterday, Michael took the day off and we drove the 90-minutes to their “Del Webb Community” home. They are very happy there and it is a safe place to leave as they still travel so much each year. It is one of the warmest areas in the Bay Area and it was 89 degrees on their back patio yet didn't feel that hot. With the fan above, it was so comfortable. When we arrived home, it was wet and foggy and cold. All within 90-minutes!

They looked fantastic - very trim and loose. Jay is addicted to the treadmill and Nancy has just begun again to wrap some time of it into her morning routine. We went to lunch at a very lovely restaurant where we could hear each other and talked non-stop. Of course.

A nice day. A playing hooky day for us. Back to rehab today. Back to doctors today. Back to life today.

Monday, October 17, 2011

Research Articles



Susan from the ILD Support Group sent this e-mail to the group. I thought they were both interesting enough to pass them along to you, dear reader. Both articles are posted at the Pulmonary Fibrosis Foundation website.
UCSD: Researchers find Progression of Lung Fibrosis Blocked in Mouse Model
Study points to a phosphorylation pathway that may contribute to the development of lung injury and fibrosis

A study by researchers at the University of California, San Diego School of Medicine may lead to a way to prevent the progression, or induce the regression, of lung injury that results from use of the anti-cancer chemotherapy drug Bleomycin. Pulmonary fibrosis caused by this drug, as well as Idiopathic Pulmonary Fibrosis (IPF) from unknown causes, affect nearly five million people worldwide. No therapy is known to improve the health or survival of patients.

Their research shows that the RSK-C/EBP£]Æ’nphosphorylation pathway may contribute to the development of lung injury and fibrosis, and that blocking this phosphorylation ¡V a regulatory mechanism in which proteins and receptors are switched on or off ¡V improved Bleomycin-induced lung fibrosis in mice. The study appears on-line October 5 in Proceedings of the Library of Science (PloS ONE).

Bleomycin is a common chemotherapy drug used to treat many forms of cancer, according to study authors Martina Buck, PhD, associate professor of medicine, and Mario Chojkier, MD, professor of medicine, both researchers at UC San Diego Moores Cancer Center and the VA San Diego Healthcare System. ¡§Unfortunately, use of Bleomycin has damaging side effects, including lung fibrosis. We are hopeful that this discovery could provide a way to stop such lung damage so that cancer patients could better tolerate this chemotherapy,¡¨ said Buck.

The downstream molecular mechanism that causes Bleomycin-induced lung fibrosis remained unknown. The scientists set out to identify the specific signaling involving a single amino acid within a specific domain of one protein that could be blocked the half the progression of such injury, in order to design effective targeted therapeutics.

They found that blocking RSK phosphorylation of a binding protein called C/EBP-Beta on the RSK macromolecule Thr217 with either a single point mutation or a blocking peptide ameliorated the progression of lung injury and fibrosis induced by Bleomycin in mice.

¡§We hypothesized that this pathway was critical given similarities between liver and lung fibrogenesis. RSK plays an important role in both the macrophage inflammatory function and survival of activated liver myofibroblasts ¡V cells that contribute to maintenance and tissue metabolism,¡¨ said Buck. ¡§Therefore, we proposed that a similar signaling mechanism is also responsible for lung injury and fibrosis.¡¨

By identifying the peptide that shuts down this process, the researchers were essentially able to sequester a small piece of an important regulatory protein, C/EBP Beta, responsible for fibrosis, thereby stopping phosphorylation. ¡§Basically, the kinase protein gets hung up, trying again and again ¡V unsuccessfully ¡V to ¡¥turn on¡¦ the fibrous growth,¡¨ Buck added.

In addition, phosphorylation of human C/EBP£] was induced inÆ’nhuman lung fibroblasts in culture and in situ in lungs of patients with severe lung fibrosis, but not in control lungs, suggesting that this signaling pathway may be also relevant in human lung injury and fibrosis.

The researchers add that it is premature to assess whether this pathway will provide an effective therapeutic target. However, blocking progression of lung fibrosis could decrease the need for lung transplantation, since IPF is the main indication for lung transplants worldwide.
*********************************************************************
Bioengineering Lungs for Transplantation
By Dr. Daniel Dilling, FCCP

Lung transplantation, at one time, seemed like science fiction, but innovations in surgical technique and immunosuppression made it clinical reality. Limited supply of donor lungs and the vagaries of immunosuppression still limit its success.

A pair of articles published last year has created an aura of science fiction once again (Ott et al. Nat Med. 2010;16[8]:927; Petersen et al. Science. 2010;329[5991]:538). Two groups of researchers developed an engineered lung through decellularization and recellularization in a rat model. In the experiments, an explanted adult rat lung was decellularized with a detergent, creating a sort of scaffolding of the lung. It retained its ultrastructural properties with complete removal of antigenic cellular components. Preservation of lung architecture and microvasculature was seen on CT imaging. Even the alveolar septal architecture remained undisturbed.

This acellular matrix was mounted inside a biomimetic bioreactor, where fetal vascular endothelium could be seeded into the pulmonary artery and fetal pulmonary epithelium into the trachea. Inside the bioreactor, the lungs were perfused with blood and ventilated at physiologic pressures, with gas exchange comparable to native lungs under the same conditions. After 4 to 8 days of culture, the lungs were removed and successfully implanted into syngeneic rats where selective blood gas analysis demonstrated gas exchange in the engineered lungs.

Tissue-engineered lungs could alleviate donor availability and many of the allo-immunity problems, if such a concept could be developed to clinical reality. There are many hurdles to overcome, but along with other novel concepts, such as a microchip that performs gas exchange (Science. 2010 Jun 25; 328(5986):1662), we must wonder what the future holds for such technologies.

One of the lead researchers, Dr. Tom Peterson, will discuss the topic, "Tissue-Engineered Lungs for In Vivo Implantation" at the Transplant NetWork open meeting on Monday, October 24, at 7:15 am in the Honolulu Convention Center, room 318B.

Sunday, October 16, 2011

ILD and Stress?


When Susan from the ILD support group and I were talking about our diseases, she said something that gave me pause. She has a very stressful job and was dealing with a very stressful case before the interstitial lung disease invaded her body.

Hmmm. I first began the big job at the school in 2000. The doctors believe I acquired the disease late 2002 or early 2003. The job was stressful. Extremely stressful. Long hard hours.

Was that the tipping point? Was stress the pathway that opened my body up for the disease to take hold?

I would love to talk with others to see if stress was a factor for them, too.

Next time I speak with the researchers at the hospitals, I will mention the possible correlation.

Saturday, October 15, 2011

Lovely Time Out

Today is the beginning of Pumpkin Festival weekend. Too crazy. Over 300,000 people are expected to make their way to our small town. Hours and hours will be spent by most of them as they try to drive over the pass. I am staying home all day. The weather is beautiful - in the 70's - so the garden calls. There is always an area that needs attention. I also see a nap in my future!

When I was first diagnosed and trying to deal with the fact that I was facing a fatal illness, I just wanted to feel normal once in a while. Somewhere where no one wanted to talk about my illness. Somewhere to get away from it.

My friend Natalie was just home on maternity leave and lived close to my hospital in the big city. We would meet at her apartment, I would hold the baby – Winnie – and it was a reminder to me that the cycle of life continued. We never had time to talk about my disease as the focus was on the baby. It was a lovely time out.

As Winnie grew, they bought a house in my town and she and Ethan welcomed the adorable Oliver. Yesterday, they came to the house for lunch and a visit. The focus was on the kids and for a few hours, I forgot I am ill.

I served them small pizzas and a nice salad and even some Halloween cookies. The weather was beautiful at the moment so we had the kids outside most of the visit. They rode their bikes in front of the house and we even let them run through the sprinklers in front. Afterward, they sat in the sun to dry off while eating small single servings of ice cream. A perfect day.

I am exhausted from the all the energy. Kid energy. I don’t know how Natalie has enough energy to keep up with them!

But, I had a great day. I had another day away from the disease. It was simply lovely.

In other news:  Lois sent a recipe that was so good and healthful that I must pass it along.

Brussel Sprouts Slaw
Shred 1-1/2 lbs brussel sprouts, using a box grater or food processor.  
Whisk together 1/2 cup mayonnaise, 1/4 cup lime juice,  2 tbls fish sauce ( mostly extracted anchovy) and 2 tsp sugar.  
Toss with the sprouts, 1/2 cup chopped scallions, 1/2 cup roasted peanuts, and 1/4 cup chopped fresh cilantro.

Friday, October 14, 2011

Third Annual ILD Luncheon

View from the ILD Luncheon 
We had a lovely time. On Thursday, the ILD Support Group was invited to the annual ILD Luncheon with all the research doctors, the clinic doctors and staff from the transplant clinic, chest clinic and all the pulmonologist. World-famous doctors were introduced. The co-heads of the ILD Clinic spoke from the podium.

One reviewed the year and the current research studies and thanked all of us for participating.  There were six people present who have had lung transplants and one lovely man who was one-year post-op from a heart/lung transplant.

I was so impressed by last year’s luncheon; I wanted Michael to meet all these doctors. So many have become my friends. He took the day off of work to join me. We sat with Susan and her husband Mark.

When I went to the very first ILD support group, I met Marty. He was the first person I ever met with my disease and I globed onto him to learn from his experiences. Through the years, we still connect with each other and both of us are doing just fine, thank you. I wanted Michael to meet him. He helped me so much during the early years of my disease. It was great to hear that he is flying to Hawaii next month. Wonderful.

It was a marvelous few hours. Interesting conversations, kind people and so many smart doctors all in one room!

Thursday, October 13, 2011

Make New Friends

Roof top dining near a fireplace at Park Chow. Our table is on the left.
Monday, I met the beautiful and smart Susan from the ILD group for lunch at her request. I was intrigued and wondered why she wanted to meet. I guess she just thought I was interesting and feisty. Love that.

So much. We talked about so much. Mostly talked about being ill, the effects it has on our work and family and how we deal with it emotionally and socially.

What I think is her biggest struggle is an unclear diagnosis. One group of doctors feels it is IPF and another group feels it is my disease, hypersensitivity pneumonitis. Sadly, the IPF diagnosis was delivered to her bluntly along with a three years prognosis. It is horrible to not have a definitive diagnosis.

Three and a half hours later, we departed. The time flew by.

Yesterday, I was hauling groceries into the house and beginning to make a raw brussel sprouts slaw when Susan phoned and we talked again for almost two hours. I swear, she is the nicest woman!

Today, we are going to meet again with our husbands in tow this time for the annual ILD Support Group Luncheon. In one room with us will be various doctors, researchers, faculty and staff of the ILD Clinic, Chest Faculty Clinic and Lung Transplant Clinic. It is heartening to hear a review of the year and what the focus of these various groups will be going forward.

And the food is usually great, too!

Monday, October 10, 2011

Pre-Holiday Drama

I called my sister on Saturday afternoon. We talked. I told her that mom had re-injured her back while doing rehab for her broken pelvis. I told her that I am going to drive her to the pain management doctor for another shot to stop the nerves in the area of her broken back later this month. I told her that mom is downhearted and so sick of being in pain for the past two years.

I told her that we talked about the Thanksgiving plans of celebrating it at our house. I told her that mom was just not up to packing up and leaving her home or her bed for a few days. I told her that mom is not up to the 2-hour drive.

I told her that after cooking Thanksgiving dinner four years ago that I was done. It required too much last minute work and that I was exhausted and became ill afterwards. I told her that when I made a non-traditional Thanksgiving dinner I could manage two years ago, they complained.

I told her that I had been looking for a local restaurant for Thanksgiving dinner but after speaking honestly with mom, I told her to make reservations at a local restaurant. Mom and I are going to split the bill. It won’t cost my sister anything. The reservations are for all of us including my sister and her husband, their daughter and her fiance.

The plan presented to my sister: We will meet at noon at mom’s house; have one cocktail and some chips and salsa then head to the restaurant. There will be full Thanksgiving dinner available but they also lamb and pork and other meats available. A great place. She will love it. We’ll eat at 2:00 and head home by 5:00. Perfect.

It fell like a lead balloon. She heard me but she really didn’t hear me. Sometimes I think she forgets that mom is 83-years old. With a broken back. With a broken pelvis.

Well, my sister was not happy. When I asked how it all sounded, she replied, “I don’t know. I have to ask everyone.” Thud.

What I expected a daughter to say was, “ Whatever we have to do for mom is fine.” That thought never entered her mind.

I replied that if it was not going to work for them to please let me know so I can cancel the additional people on the reservation. I told her that Mom so wanted everyone together.

Bets are on whether they will join us or not.

Sunday, October 9, 2011

Racing to Lunch

Sunday morning. Pre-dawn. Smells of Fall. Cool.

The house is cosy, the smell of coffee is lingering and the promise of some eggs and salsa is definite. Our secret passion? Formula One Racing. A car thing, of course. We finally gave up Comcast a few years ago and bought the small package from DirecTV which included Speed Channel. It was the reason for the change.

Almost every other Sunday during the season, the race is DVRed for Sunday morning fun. Today is the Grand Prix of Japan. Beautiful track. Sebastian Vettel will nail his championship for the season if he just finishes this race. It is a fun way to start a Sunday.

Yesterday, I met my friend Barbara at Nordstrom's to look for a dress to wear to a wedding in two weeks. Within 15-minutes, found it. Spectacular dress. Done. It left the rest of the few hours for great conversations and a nice lunch.

Today, I am meeting my friend Matt from the school where I worked, for lunch and the latest gossip. Should be fun. It is supposed to be sunny and warm today. We will be at the harbor enjoying the fresh air and a new restaurant.

Happy Sunday to you!

Saturday, October 8, 2011

New Clinical Study of Pirfenidone in Australia, New Zealand, Mexico, US and South America

Once again, the marvelous people from The Coalition for Pulmonary Fibrosis has sent an e-mail regarding the new clinical trial now enrolling people with Idiopathic Pulmonary Fibrosis. This is an international study on the drug pirfenidone. There are several people in my ILD Support Group who have been on this drug for years and say they feel it has been very positive. Remember, it is now available in Japan, Europe and India. Really, it's time for it to be made available to other countries as well.


The parameters of being accepted into the study are below. Also, there is a web site for further information.   Here it is:


"If you are a Pulmonary Fibrosis (PF) patient and would like to help find answers to PF, the Coalition for Pulmonary Fibrosis (CPF) urges you to consider being involved in clinical trials. No treatments or cures can be discovered without these trials.
One important trial is being conducted is the ASCEND Trial.  ASCEND is a large clinical trial currently enrolling patients in the United States, Mexico, Australia, New Zealand, and select South American countries. This trial is designed to assess the efficacy and safety of the investigational use of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF).
There is a full listing of PF clinical trials on the CPF website at: http://coalitionforpf.org/cpf_research_clinical.php
To view Frequently Asked Questions about Clinical trials, please refer to the NIH website on the subject at:http://clinicaltrials.gov/ct2/info/understand.  Please also feel free to contact the CPF with questions about the trials.
Sincerely,
Mishka Michon, CEO of the CPF


ASCEND is a large clinical trial currently enrolling patients in the United States, Mexico, Australia, New Zealand, and select South American countries. This trial is designed to assess the efficacy and safety of the investigational use of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF).
What Treatment is Being Tested?
Eligible patients will receive either pirfenidone (an investigational medication for IPF in the U.S., Mexico, Australia, New Zealand, and select South American countries) or placebo for one year (52 weeks). Patients who complete the entire 52-week study period will be offered the opportunity to continue taking pirfenidone treatment (or switch from placebo to pirfenidone) as part of an open-label rollover study. Patients may also be offered participation in the rollover study if they are able to follow their dosing regimen during the initial clinical trial.
Objectives
Clinical trials must be designed to address specific objectives. The objectives of the ASCEND Trial are to:
• Assess the effect of pirfenidone compared with placebo on change in a lung function measure (percent predicted forced vital capacity, or %FVC)
• Assess the safety of pirfenidone compared with placebo
Select Inclusion Criteria
Clinical trials must also set specific criteria for patient enrollment. To participate in the ASCEND Trial, the main requirements are:
• The diagnosis of IPF (definite or probable) must have been made within 4 years (48 months) of study randomization — the time at which patients will be randomly assigned to receive either pirfenidone or placebo
• At the time of study randomization, the patient must be between 40 and 80 years of age
• Lung function, measured by a test of the ability to exhale (forced vital capacity, or FVC) must fall within a specific range (50% to 90%)
• Another measure of lung function, called carbon monoxide diffusing capacity (DLCO), must be within the range of 30% to 90%
• The patient must be able to walk at least 150 meters (about 500 feet) within 6 minutes
Select Exclusion Criteria
In order to produce scientifically meaningful results, the ASCEND Trial cannot admit all people with IPF. Patients with any of the following criteria are not eligible to participate in the ASCEND Trial:
• At the time of study randomization, the patient is either expecting to receive a lung transplant within one year or, for patients in the United States, is on a lung transplant waiting list
• The interstitial lung disease has a known explanation
• There is a history of asthma or chronic obstructive pulmonary disease
• The patient has an active infection
• The patient is already receiving ongoing IPF treatment such as an investigational therapy, immunosuppressant, or a cytokine modulating agent
• Within the past 6 months, the patient has had unstable or deteriorating cardiac disease or a pulmonary disease other than IPF
• History of unstable or deteriorating cardiac or pulmonary disease (other than IPF) within the previous 6 months
Study Details:
• Screening period (up to 56 days)
• 52 Week Treatment Period
• All treatment-compliant patients who complete 52 weeks will be offered participation in an open-label rollover study
Learn More About the ASCEND Trial
If you would like more detailed information about the ASCEND Trial, please go to www.ascendtrial.com, or call InterMune Medical Information at 1-888-486-6411"


Friday, October 7, 2011

Gardening, Shopping and Pumpkins

It's cold this morning. It's in the 40's outside and 61 inside. I try to not turn the heater on each year until the rates drop on November 1st. This morning, Michael was cold. He made that comment while hovering around the light bulb over the stove for warmth. Ha! So cute. He was in long pj's, shirt, sweater, socks and a full blanket wrapped around his body. Maybe it is time to re-think the November 1st thing....

The good news is that the rain has stopped for a few days and I am heading into the gardens all day today. This is my usual Saturday work but I have plans for tomorrow. My friend Barbara and I have shopping and lunch on our list of things to do tomorrow morning. Nordstroms.

The town that we live in is known for its pumpkins. Lots of pumpkins. Even a pumpkin festival, which is next weekend and brings several hundred thousand people to our little town. But, for the entire month of October, traffic is slow. On the weekends, it is impossible to drive the normal 10-minute drive on the pass in under 90-minutes. There is another way to get home from the north, though it is much longer. That is how I am going to get home tomorrow afternoon.

Thursday, October 6, 2011

Grace



Grace has a son. Mom has heard stories about this son for years always spoken in hushed "God-like" tones. When I was at mom's for her birthday after her stint in the hospital last August, Grace threw a birthday party for her and I was invited. We talked a lot. I guess she liked me, too.

Grace. Last week, mom e-mailed that Grace wanted to know the name of my exact disease. She then called and told her biologist son, who has taken new drugs through the process then onto the market, and told him to find a cure for my disease. No one says no to Grace, not even her son.

Grace told mom yesterday that her son just told her that he has an idea and is going to start working on it.

I often wonder why I got this disease. Wouldn't it be delightful that because of my disease, a biologist focused his full attention on it and stumbled upon, at the very least, a treatment or maybe even a cure. That would be worth dying for. All thanks to Grace.

In other news: I just have to comment on the passing of Steve Jobs. I do not talk a lot about the parents at the school where I got sick. The very rich. The very famous. The very brilliant. Steve Jobs was one of our dads. He had a daughter who graduated from the school years earlier and he even designed the new science lab. With his second family, he returned to the school with a pre-K son and babies. I can tell you this: He was a very good dad. That is how I will remember him. My thoughts are with his children.