Living Well with a Bad Diagnosis - Lung Disease

Friday, February 28, 2014

Mom and Michael

I always check my phone after I leave the rehab class just incase Michael or William phoned while it was locked away. It was a shock to see a message from Michael and it was a photo. A photo of my mom with a hamburger in her hand!

Well, I giggled all the way home then phoned her. I had to hear the story. She decided to get a McDonald's hamburger for lunch (with only catsup and onions, please) and was standing in line when she felt a tap on her shoulder. She was floored when she looked up to see Michael grinning at her! He told me later that she greeted him like a returning soldier from the war. Everyone in line was smiling. There was a lady between them, mom offered for her to go ahead in line but she declined. She, too, was laughing and let Michael join my mom. They sat and chatted and Michael said there was not a moment of silence. Mom said it was so much fun, such a surprise and a real treat to spend some time with him. She really didn't want him to go back to work!

He told me that he was thinking later in the afternoon about how very blessed he was to have a mother-in-law who really loves him. I know that if I died suddenly, Michael would be in charge of my mom. He would just assume the role and would be so good with her.

I will be spending the day with her today after working out at the other rehab. She will probably cream me in card again, try to buy my something, chat during lunch and just enjoy being together. A perfect day.

Thursday, February 27, 2014

Weight Loss Revealed!

What a day. The big storms are now rolling into Northern California and the beginning was yesterday morning, just as I was leaving for the long drive deep into the city. It was a surprisingly easy drive and I arrived anxious about finding parking. Street parking. Within a block of the hospital, there were three buildings being constructed and one area was closed for road work. It was a mess. My chances were slim. There were garages but they are EXPENSIVE! And I am frugal. So, I gave up and was heading into a garage when I spotted a blue curb! It was right next to the entrance to the garage. It was right in front of the blood draw area where I needed to have some blood tests for the liver clinic. The miracle happened!

This blood draw lab is usually very crowded but when I arrived at 8AM, I was the only one there! Another miracle! I was out within ten minutes, ate my breakfast in the lobby, walked through the garage to the next building and arrived to my 9AM appointment with the nutritionist at 8:15. Mr. B. took me in early - another miracle - and I hopped on the scale. It was exciting. I had been on the liquid diet for 26 days.

Last time I was on this diet three years ago, I bought myself something to wear for each monthly weigh in. New shoes, earrings or a new top. Nothing expensive, just new. So, I continued that tradition by buying a silver stacking ring, which I twisted while standing on the scale. I was wearing my smallest jeans and a heavy sweater (it was cold) yet I was thrilled when the scale revealed that I had lost fourteen pounds! In twenty-six days!

I now want to lose another eight pounds before the road trip in May, which will put me at my lightest since 1987. The re-feeding will begin a week from Saturday when Michael and I will share dinner together. A dinner featuring real food: rib-eye steak, mushrooms, cabbage. I must say that I will be having a small piece of Michael's steak. From that point on, I will replace one liquid supplement with dinner. A low fat/low carb dinner. That will be my diet for a week. Then, I will be adding lunch into the mix the following week and finally breakfast will be added the week after. No more liquid diet. During the transition last time, I lost five pounds. If that happens again, I will need to lose just four pounds in six weeks. I think I can do that!

The goal will be to eat 1,200 calories per day to lose weight or 1,400 calories to maintain my weight. Not a lot of difference there! And exercise. I believe that was how I lost so much weight in such a short period of time. I really kicked it up a notch.

I am feeling great. Nine more days before real food. Let the countdown begin!

Wednesday, February 26, 2014


BIG NEWS! I received the below from the people at the Coalition for Pulmonary Fibrosis:

The CPF would like to inform you that InterMune, Inc. announced positive results related to its Ascend Trial, a phase 3 trial of pirfenidone.  According to the company's press release, the trial met its primary endpoint as well as both of its secondary endpoints. 

Here is the report from interMune:

InterMune Reports Phase 3 ASCEND Trial Results of Pirfenidone in Idiopathic Pulmonary Fibrosis (IPF)

- Study Meets Primary and Both Key Secondary Endpoints -
- Company to Conduct Conference Call and Webcast Today at 8:00 a.m. EST -
BRISBANE, Calif.Feb. 25, 2014 /PRNewswire/ -- InterMune, Inc. (NASDAQ: ITMN) today announced that top-line data from ASCEND, a Phase 3 trial evaluating pirfenidone in patients with idiopathic pulmonary fibrosis (IPF), demonstrated that pirfenidone significantly reduced IPF disease progression as measured by change in percent predicted forced vital capacity (FVC) from Baseline to Week 52 (rank ANCOVA p<0 .000001="" additionally="" and="" both="" change="" demonstrated="" distance="" effects="" endpoints="" key="" nbsp="" of="" on="" p="0.0001).   </p" progression-free="" secondary="" significant="" six-minute="" survival="" test="" the="" treatment="" walk="" were="">
"We are pleased to report these top-line ASCEND Phase 3 results," said Dan Welch, Chairman, Chief Executive Officer and President of InterMune.  "Based on the strength of the ASCEND results, InterMune is preparing a resubmission of our New Drug Application for pirfenidone to the U.S. Food and Drug Administration (FDA), which we expect to submit by early third quarter of this year.  We would like to thank our collaborators, patients and their families for their participation in ASCEND and their contributions to IPF research."
Primary Endpoint
The magnitude of the treatment effect of pirfenidone was measured by comparing the proportion of patients in the pirfenidone and placebo groups experiencing either a clinically meaningful change in FVC, or death.  A 10% decline in FVC in an individual IPF patient is considered clinically meaningful and strongly predicts mortality.  At Week 52, 16.5% of patients in the pirfenidone group experienced an FVC decline of 10% or more or death, compared with 31.8% in the placebo group, representing a 47.9% reduction in the proportion of patients who experienced a meaningful change in FVC or death.  Additionally, at Week 52 the data demonstrated that 22.7% of patients in the pirfenidone group experienced no decline in FVC, compared with 9.7% in the placebo group, representing a 132.5% increase in the proportion of patients whose FVC did not decrease between Baseline and Week 52.
Dr. Talmadge King, Chair, Department of MedicineUniversity of California San Francisco and Co-chair of the ASCEND protocol steering committee, said, "IPF is an unpredictable, debilitating and ultimately fatal disease, and safe and effective treatments are desperately needed to alter the course of this challenging and complex condition.  The ASCEND data demonstrated that pirfenidone significantly reduced decline in lung function and significantly increased the proportion of patients who had no decline, which is an important advance in the field.  The results for 6MWT distance, PFS and mortality provide important supportive evidence of pirfenidone's efficacy." 
Key Secondary Endpoints
The ASCEND protocol pre-specified 6MWD and PFS as the two key secondary endpoints.  Change from Baseline to Week 52 in 6MWD is a measure of exercise tolerance.  A 50-meter decrement in walk distance is considered an independent predictor of mortality in an individual patient with IPF.  In ASCEND, pirfenidone reduced by 27.5% the proportion of patients who experienced a decline in 6MWD of 50 meters or greater (p=0.0360). 
PFS is a measure of time before death or a disease-progression event.  A PFS event was defined in the protocol as any of the following: death, percent predicted FVC decrement of 10% or greater or 6MWD decrement of 50 meters or greater.  In ASCEND, pirfenidone reduced the risk of death or disease progression by 43% compared to placebo (Hazard Ratio [HR]=0.57; 95% confidence interval, 0.43-0.77; p=0.0001).
Additional Secondary Endpoints
Three additional secondary endpoints were pre-specified in the ASCEND protocol: all-cause mortality, treatment-emergent IPF-related mortality and change from Baseline to Week 52 in dyspnea (shortness of breath).  The two mortality analyses were pre-specified for both the ASCEND study and the pooled population of the ASCEND study and the previous Phase 3 CAPACITY studies through 52 weeks.  Due to the relatively low overall mortality rate in patient populations in the time frames studied in a single IPF study such as ASCEND, pooled analyses of ASCEND and CAPACITY data provide more statistical power and a more precise estimate of the treatment effect of pirfenidone on mortality.
In the pre-specified mortality analysis of the ASCEND study alone, there were fewer events of all-cause mortality (HR=0.55, log rank p=0.1045) and of treatment-emergent IPF-related mortality (HR=0.44, log rank p=0.2258) in the pirfenidone group compared to the placebo group.  ASCEND was not powered to show a difference on these endpoints.  The relationship of death to IPF was determined in ASCEND by a blinded adjudication committee. 
The pre-specified analyses of the pooled population (N=1,247) across ASCEND and the two Phase 3 CAPACITY studies (taking CAPACITY mortality data through Week 52) showed that the risk of all-cause mortality was reduced by 48% in the pirfenidone group compared to the placebo group (HR=0.52, log rank p=0.0107).  Additionally, in the pooled population the risk of treatment-emergent IPF-related death in the pirfenidone group compared to placebo was reduced by 68% (HR=0.32, log rank p=0.0061).
The secondary endpoint of dyspnea, measured by the UCSD SOBQ questionnaire, was not achieved (p=0.1577).
Safety and Tolerability
In ASCEND, pirfenidone showed a favorable safety profile and was generally well tolerated. 
A total of 93.5% and 94.6% of patients completed the study, died or had a lung transplant by study day 365 in the pirfenidone and placebo groups, respectively.  The percentage of patients discontinuing treatment due to an adverse event was 14.4% in the pirfenidone group and 10.8% in the placebo group.  Serious adverse events (SAEs) were reported in 19.8% of patients in the pirfenidone group and 24.9% in the placebo group.  Hospitalizations due to respiratory, thoracic and mediastinal SAEs were reported in 3.6% of patients in the pirfenidone group and 11.2% in the placebo group.
The most common AEs with higher incidence in the pirfenidone group were primarily gastrointestinal (e.g., nausea and dyspepsia) and skin-related (e.g., rash).  The GI and rash AEs were generally mild to moderate in severity, manageable, reversible and only infrequently led to treatment discontinuations. 
Elevations of aminotransferase levels at least 3 times the upper limit of normal occurred in 2.9% of pirfenidone patients (including one case associated with a bilirubin increase) vs. 0.7% of placebo patients.  In general, these elevations occurred early, were manageable and reversible, and were similar to those observed in previous pirfenidone studies.
The safety and tolerability profile of pirfenidone was generally consistent with observations from the previous Phase 3 CAPACITY studies, open-label extension studies and post-marketing experience.
"These results from the ASCEND trial provide compelling evidence of a clinically meaningful treatment effect of pirfenidone with generally favorable safety and tolerability findings, which is very encouraging for patients suffering from this fatal and relentless disease," said Paul W. Noble, Chair, Department of MedicineCedars-Sinai Medical CenterLos Angeles, Calif. and Co-chair of the ASCEND protocol steering committee.  "Importantly, the overall safety observations from ASCEND complement and corroborate the robust safety database that already exists from the InterMune-sponsored clinical studies of pirfenidone and extensive post-marketing experience outside the United States."
InterMune intends to present additional data from the ASCEND study at the 2014 American Thoracic Society International Conference in May.
ASCEND (Assessment of Pirfenidone to Confirm Efficacy and Safety in IPF) is a multinational, randomized, double-blind, placebo-controlled Phase 3 trial designed to evaluate the safety and efficacy of pirfenidone in patients with IPF.  Patients (N=555) were randomly assigned 1:1 to receive oral pirfenidone (2403 mg/day) or placebo and were enrolled at 127 centers in the United States,AustraliaBrazilCroatiaIsraelMexicoNew ZealandPeru and Singapore. 
More than 95 percent of eligible patients (those patients who remained on blinded pirfenidone or placebo therapy) who completed the ASCEND study decided to enter the open-label RECAP extension study.  RECAP is a study in which all patients receive pirfenidone.  RECAP also includes patients rolled over from the company's prior CAPACITY studies which completed in late 2008 and enrolled 779 patients in two Phase 3 studies.  RECAP provides valuable long-term safety data that further expands the already large safety database for pirfenidone in patients with IPF.
Pirfenidone has been studied in multiple Phase 3 clinical trials in patients with IPF, including the two Phase 3 CAPACITY trials sponsored by InterMune.
The CAPACITY program consisted of two concurrent 72-week trials which enrolled a total of 779 patients.  Both trials were multinational, randomized, double-blind, and placebo-controlled. The studies were designed to evaluate the safety and efficacy of pirfenidone in IPF patients with mild to moderate impairment in lung function.  The primary endpoint in both studies was the change from Baseline to Week 72 in percent predicted FVC.  This endpoint was met with statistical significance in CAPACITY 2 (p=0.001).  The secondary endpoints of PFS and categorical change in FVC also achieved statistical significance (p<0 .05="" 1="" 24="" 36="" 48="" 6mwd.="" a="" although="" and="" at="" capacity="" effect="" endpoint="" evidence="" fvc="" in="" including="" measures="" met="" nbsp="" not="" number="" observed="" of="" on="" p="" percent="" pirfenidone="" predicted="" primary="" supportive="" the="" treatment="" was="" weeks="">
Pirfenidone demonstrated a favorable safety profile and was generally well tolerated in both CAPACITY studies.  The most frequent side effects reported were photosensitivity rash, gastrointestinal symptoms such as nausea and dyspepsia, and dizziness.
About Esbriet® (pirfenidone)
Pirfenidone is an orally active, anti-fibrotic agent that inhibits the synthesis of TGF-beta, a chemical mediator that controls many cell functions including proliferation and differentiation, and plays a key role in fibrosis.  Pirfenidone also inhibits the synthesis of TNF-alpha, a cytokine that is known to have an active role in inflammation.
On February 28, 2011, the European Commission (EC) granted marketing authorization for Esbriet® (pirfenidone) for the treatment of adults with mild to moderate IPF.  The approval authorized marketing of Esbriet in all 28 EU member states.  Esbriet has since been approved for marketing in Norway and Iceland.  In 2011, InterMune launched commercial sales of pirfenidone in Germanyunder the trade name Esbriet, and Esbriet is now also commercially available in various European countries, including key markets such as FranceItaly and the UK.
On October 1, 2012Health Canada approved Esbriet for the treatment of mild to moderate IPF in adult patients.  Health Canadadesignated Esbriet for Priority Review and completed the accelerated review according to target guidelines of 180 days.  InterMunelaunched Esbriet in Canada in January 2013.
Pirfenidone has been marketed as Pirespa® since 2008 in Japan and since 2012 in South Korea by Shionogi & Co. Ltd.  Under different trade names, pirfenidone is also approved for the treatment of IPF in ChinaIndiaArgentina and Mexico.
Pirfenidone is not approved for sale in the United States.
About IPF
Idiopathic pulmonary fibrosis (IPF) is an irreversible and ultimately fatal disease characterized by progressive loss of lung function due to fibrosis (scarring) in the lungs, which hinders the ability of lungs to absorb oxygen.  IPF inevitably causes shortness of breath, and a deterioration in lung function and exercise tolerance.  IPF patients follow different and unpredictable clinical courses and it is not possible to predict if a patient will progress slowly or rapidly, or when the rate of decline may change.  Periods of transient clinical stability in IPF, when they occur, inevitably give way to continued disease progression.  The median survival time from diagnosis is two to five years, with a five-year survival rate of approximately 20-40 percent, which makes IPF more rapidly lethal than many malignancies, including breast, ovarian and colorectal cancers.  IPF typically occurs in patients over the age of 45, and tends to affect slightly more men than women.
About InterMune
InterMune is a biotechnology company focused on the research, development and commercialization of innovative therapies in pulmonology and orphan fibrotic diseases.  In pulmonology, the company is focused on therapies for the treatment of idiopathic pulmonary fibrosis (IPF), a progressive, irreversible, unpredictable and ultimately fatal lung disease.  Pirfenidone, the only medicine approved for IPF anywhere in the world, is approved for marketing by InterMune in the EU and Canada under the trade name Esbriet®.  Pirfenidone is not approved for sale in the United States but has completed three Phase 3 clinical trials to support regulatory registration in the United States.  InterMune's research programs are focused on the discovery of targeted, small-molecule therapeutics and biomarkers to treat and monitor serious pulmonary and fibrotic diseases.  For additional information about InterMune and its R&D pipeline, please visit
Conference Call and Webcast Details
InterMune will host a live webcast of a conference call today at 8:00 a.m. EST to discuss the top-line ASCEND Phase 3 results.  Interested investors and others may participate in the conference call by dialing 800-738-1032 (U.S.) or +1-212-231-2905 (international), conference ID# 21709573.  A replay of the webcast and teleconference will be available approximately three hours after the call.
To access the webcast, please log on to the company's website at at least 15 minutes prior to the start of the call to ensure adequate time for any software downloads that may be required.
A telephonic replay will be available for 10 business days following the call and can be accessed by dialing 800-633-8284 (U.S.) or +1 402-977-9140 (international), and entering conference ID# 21709573. 
Forward-Looking Statements
This news release contains forward-looking statements within the meaning of section 21E of the Securities Exchange Act of 1934, as amended, that reflect InterMune's judgment and involve risks and uncertainties as of the date of this release, including without limitation InterMune's expectations regarding the timing for resubmission of its new drug application with the FDA for pirfenidone; the potential to make pirfenidone available as a medicine to IPF patients in the United States and InterMune's intention to present additional data on the ASCEND trial at the American Thoracic Society meeting in May 2014.  All forward-looking statements and other information included in this press release are based on information available to InterMune as of the date hereof, andInterMune assumes no obligation to update any such forward-looking statements or information. InterMune's actual results could differ materially from those described in InterMune's forward-looking statements.
Other factors that could cause or contribute to such differences include, but are not limited to, those discussed in detail under the heading "Risk Factors" in InterMune's most recent annual report on Form 10-K filed with the Securities and Exchange Commission(SEC) on February 21, 2014 (the "Form 10-K") and other periodic reports filed with the SEC, including but not limited to the following: (i) the risks related to the uncertain, lengthy and expensive clinical development process for the company's product candidates, including having no unexpected safety, toxicology, clinical or other issues and having no unexpected clinical trial results such as unexpected new clinical data and unexpected additional analysis of existing clinical data; (ii) risks related to the regulatory process for the company's product candidates, including the possibility that the results of the new 52-week Phase 3 clinical trial (ASCEND) having an FVC endpoint may not be satisfactory to the FDA for InterMune to receive regulatory approval for pirfenidone in the United States; (iii) risks related to unexpected regulatory actions or delays, in particular in connection with our planned resubmission of a Class 2 NDA with the FDA seeking approval of pirfenidone or other government regulation generally; (iv) risks related to our ability to successfully launch and commercialize pirfenidone in the United States, if approved by the FDA and (v) InterMune's ability to obtain or maintain patent or other proprietary intellectual property protections.  The risks and other factors discussed above should be considered only in connection with the fully discussed risks and other factors discussed in detail in the Form 10-K andInterMune's other periodic reports filed with the SEC, all of which are available via InterMune's web site at
SOURCE InterMune, Inc.
Investors: Jim Goff, InterMune, Inc., 415-466-2228,, orMedia: Geoff Curtis, Edelman, 312-550-8138,

Tuesday, February 25, 2014

Anticipating the Weigh In

The anticipation is building for tomorrow morning's weigh in. It is a very long drive into the city and it will be during rush hour. And rain. A major storm is due to blow through right around the time I will be leaving home. After weaving my way to the far side of the city, I will search for street parking, make my way to the building, weigh in then walk a block for blood tests for the liver doctor. From the city, I will work my way to another freeway and drive to mom's house. It will be a very stressful couple hours but I am so excited to finally learn the amount of weight I have lost.

I have made a major decision. Regardless of the "number" on the scale on Wednesday, I am moving into the second stage of the liquid diet on Saturday, March 8. I will add one meal a day to the liquid diet for a week. Because of Michael, I have chosen dinner. So, Saturday, March 8 will find him in the garden with the BBQ and some dry aged rib eye steak (I will have a bit of his) while I cook mushrooms and make cole slaw. It will be my first food since January 31.

Dinners for the rest of the week: Paleo Chicken Lettuce Wraps, Halibut a la Puttanesca and a low fat Cheesy Creamed Spinach with just a simple 1/4 pound hamburger patty.

The following week, I will add another meal to my day. I am thinking of adding lunch for my mom. We will probably get a bowl of the wonderful Russian Cabbage soup at Max's and something "safe" for me at Nordstroms.

Finally, breakfast will be added back and all meals will now be real food as I enter Stage 3 of the diet. I am going to still visit the nutritionist all the way up to our road trip and a couple of visits after we get home. It will keep me "honest" while we are on the road. Between now and May 3 when we leave, I will be on my strict low fat/low carb diet and will lose even more weight. I am trying to get under my lowest weight.

I am excited to finally seeing the numbers on the scale, thinking about what I can lose before our May trip and dreaming of buying some new clothes.

Monday, February 24, 2014

Susan's Memorial Services

The memorial service for Susan was an amazing as the woman herself. It was at the Golden Gate Club at the Presidio in the city where the views of the Bay and the famous bridge were stunning. The room was large enough to seat the over 400 people who attended. First her husband, sons then famous people from organziations she was devoted to spoke to the crowd. It lasted over four hours and there was a beautiful array of food that followed the service. I was able to speak with her husband Mark just after we arrived. He was still so very stunned that she died so suddenly. As were we all.

The group from the ILD Support Group sat together and Sally, the head of the group, was the final speaker who spoke about the work Susan did to help others with IPF.

Our drive back down the coast was beautiful, the fog had drifted in and our thoughts were with Susan and her family. This woman left a very large footprint in her short time here on earth.

Sunday, February 23, 2014

Susan's Memorial Service

Tough day ahead. The beautiful Susan's memorial service is at 1:00 in the city. They are expecting over 100 people but I think that is a very low estimate. When Susan retired in April, she did not tell her co-workers that she was ill. They thought she was retiring and was riding off into the sunset. She died in January.

She was the gentlest, kindest person I have ever met. There was always an air of calmness surrounding her. Visiting, she was horrified when I killed a housefly that had been driving me crazy for two days. She encouraged me to practice yoga. To live in the present.

During our last lunch together, she laughed and shook her head at me when I was telling her about the plans for my memorial service. I said that I would be lucky to have ten people attend! Since producing over 100 performances a year, I wanted to my service to be my last production. She told me she had not even thought about what she wanted. Just two months later, today is the day. I know her husband and boys have put together an amazing tribute to her.

My purse will be packed with Kleenex and Michael made a date to have coffee afterwards with British Don. That was actually was brilliant. It will give me time to recover, talk about her with Don and take a deep breath before coming home. To our life. I am grateful for every moment.

Saturday, February 22, 2014

Let the Lists Begin!

Mom creamed me at cards yesterday. I was bruised! We played two games of Shanghai and she had the luck of the Irish! It was brutal. She had a ball. After working out at the other rehab, we went shopping to find a little table for her front entrance. We finally found it at Pier One Imports before having lunch together. She, who is so very careful of what she eats, had TWO large pancakes! It was pure joy watching her so happy and satisfied.

During our road trip in 2011, we spent just over $1,000 in food and $1,000 in gas. It will be cheaper this trip as we will be on the road fewer days and will not travel as many miles. We always charge the hotels and we love Hampton Inns. They have a great breakfast and fantastic locations. They have never failed us.

I worked on more of the trip details yesterday and have begun to make lists, which included rental of a portable oxygen system. I love the Eclipse as it has continuous flow, very important for my disease. I also need to make sure I have ordered and received all my medications, buy a new device to charge our cell phones in the car, stop the mail, make the hotel reservations, buy some new clothes, buy gifts for the people we are going to visit, prepay all our bills, garden and water right before we leave, arrange for Natalie and the kids to water while we are gone, have the monthly check of the house alarm, notify the housekeepers on the change of schedule and oh so much more. Let the lists begin!

But, it is all that preparation that brings the anticipation of the trip to a feverish pitch then what follows is the pure peace that overwhelms me as we back the car out the garage pre-dawn on the first day. Everything is done. All that has to happen is to enjoy the adventure ahead. No doctors. No phone calls. No rehab. No appointments. Freedom of the road. That is also the reason why I always dread the end of the trip. The closer we get to home, the more I feel we are traveling back to reality. Back to real life. Re-entry is always a challenge. The gardens will need work, the bills will be piled up, groceries will be needed but we will be home and there is nothing like our own bed. So, the trips are good for our soul but it reminds us that our home and lives are pretty good, despite the disease. Then we dream about the next road trip!

Friday, February 21, 2014

It's Friday

Crawled into bed last night and I don't remember even turning over in my sleep until awaking at 4:30 this morning. I looked over to find the bed empty! There was some evidence that Michael had been in bed but I never felt him come to bed nor leave. I was extremely tired. Passed out. I did find him awake and in the family room so we are now having coffee together.

It's Friday. It is mild here so we will meet on the garden swing after dinner, light the little fire pot and talk. I love that tradition. All the stories and things we forget to share during the week flows as we relax into the weekend. It is going to be beautiful here. Susan's memorial service is at the Presidio in the city on Sunday afternoon. I am sure it will be packed as she was an amazing person. It is going to be very emotional for me.

Friday. The other rehab is calling to me this morning then I will spend the day with mom. She is so full of energy these days, thanks to the pacemaker. I am sure she has had adventures since I saw her on Monday. We have no doctor's appointments, nothing we HAVE to do so it will be a fun day. We'll probably shop (CVS or Safeway), have lunch somewhere and talk non-stop.

Friday. No hurry to get to bed tonight as we can sleep in a bit later in the morning.

Friday. One day closer to the weigh in at dawn on Wednesday. I was wearing my jean's yesterday and shocked Michael by pulling them down without opening the snap or zipper. My mind is a whirl. I sometimes think I have lost twelve pounds and other times, it feels like twenty. All will be revealed on Wednesday.

Enjoy Friday as it leads into a nice weekend ahead.

Thursday, February 20, 2014


I was a good girl by arriving to yoga right on time yesterday. I was the only one there, expect for the guy who opens up and takes the money. We chatted while two others arrived. The problem? No teacher. My regular teacher is three weeks into a 7-week program and we have been having a substitute. She never showed. We all got a ticket for a future free class. Thank you very much.

The day was suddenly changed.

I stayed home all day but I never sat down! Michael had been talking about cleaning out his closet. There were clothes in there from the 80s and honestly, he had decided he will never be a 32 waist again. I emptied all the stacks on the carpet, separated all t-shirts with either a hole or paint on them, tossed socks with holes and finally let four pairs of old Levi's go into the trash because of the huge holes in the knees. Then I arranged all the dress shirts and polo shirts in order of sleeve length, folded the rest of the t-shirts, organized his sock drawer and made the closet...perfect. That took a couple of hours. I did two loads of wash then ironed a few dress shirts for work.

Dinner. I made a killer pot of Clam Chowder early in the afternoon then cleaned every inch of the kitchen. It felt fantastic. The chowder was gently reheated for dinner and Michael went crazy. I had not made this recipe for many years. He even asked for a small amount to share with someone at work as he is a chowder hound.

Since we will be on the road trip, I will not be playing the orchestra concert in May but I am going to the rehearsals to help Gerry. It will be a great concert featuring The New World Symphony by Dvorak, one of my most favorite pieces of music to play. It sings to my soul. We are also doing a couple of his Hungarian Dances, also a joy. It felt like home. There is something called hand memory. My hand just went to the notes. No thinking about positions. No thinking about the specific notes. It just flowed. It felt so fantastic that I just could not sleep last night. My brain and heart was so stimulated.

I am going to be exhausted by the end of the day. Costco is first on my list of things to do. I must deal with the problems of my new glasses then Sherman will be waiting for me at my rehab class. Afterwards? A nap. Really.

The coming weekend is going to be devoted to my gardens. It is forecasted to be in the 70s here on the coast so I will have the pleasure of enjoying the weather while cleaning away the weeds. The real focus is the upcoming weigh in on Wednesday. I so hope I have lost as much weight as I feel. If not, I will be so disappointed!

Wednesday, February 19, 2014

Road Trip Plans - Liquid Diet Woes - Glasses Problem

We worked on the May road trip itinerary after dinner last night. I think we have it pretty well mapped out. There might be an issue with hotel rooms available in one city but otherwise, it looks good. We actually will be driving an area of the country we have never seen. I think we have taken every other east/west route until I discovered this one yesterday. We checked it out. It will take us from Raleigh, NC to my brother's home in Socorro, NM. It mostly is on Highway 20. Many cities we have not visited before this trip: Shreveport, Dallas, Brownfield, Odessa, Abilene to name a few. It will be a different and fun way home.

The liquid diet has been so remarkable that even I am stunned at the amount of weight I have lost in 19 days. People are noticing, something that I find happens after a loss of twenty pounds. I think I am close to that. The weigh in is next week. I am so sick of the lack of food and so wish for real, fresh, tasty flavors, that I am counting down the days to when I add one meal of real food into the mix. I am still stalking low fat/low carb recipes on the Internet and have fallen in love with the Pioneer Woman on the Food Network. Postings on Tastespotting are checked throughout the day. Somehow, it makes me miss food a little less. For my first real meal, I am going to choose dinner (mostly for Michael) and we have already agreed on the menu: dry-aged rib eye steak (I will take just 3ozs. of his), mushrooms and cole slaw. They warn that if your first meal is real fatty (lots of cream/butter/fat) that there could be an issue with the gall bladder. Apparently, it has been sleeping during the diet and doesn't like quick wake up calls.

I am fighting the urge to return my new glasses to Costco today. It is a long drive from home. I  have more than enough time to deal with the problem tomorrow while I am over the hill for rehab class. I so dislike problems hanging over my head. The prescription is wrong, both the distance and reading. I hate this kind of stuff....needless problems. I do love the Fendi frames and so happy I will have them for the road trip. Instead of waisting the day dealing with it, I am staying on this side of the hill, going to yoga then I might, just might, cut the grass. I promised Michael some Clam Chowder for dinner, so I have to get on that later this afternoon and finally clean myself up for orchestra rehearsal tonight.

William leaves for Australia today. He doesn't mind the long flight from Seattle. His new plan is to somehow fit his long legs into a window seat as he read somewhere that a person is less exposed to germs away from the aisle. We'll see how that works for him!

Tuesday, February 18, 2014

Pirfenidone Update for IPF

At the ILD Support Group Meeting last week, I sat near Robin. We were probably the longest members present. She is a large woman who walked in on oxygen, with a mask and the support of a walker with a seat in it.

She is a walking miracle.

Robin has Idiopathic Pulmonary Fibrosis. A very difficult diagnosis. It usually is a 2-3 year life expectancy after diagnosis and there really are no treatment options other than lung transplants. She was able to be accepted into a drug study. She was lucky as she actually got the drug and not the placebo. Pirfenidone. The drug that has been a challenge for the FDA. The drug that the rest of the world has embraced. The drug that is not available in the US. Here are other blogs I wrote about the Pirfenidone journey:

This drug is VERY expensive but those who were in the study are given the drug for free for the rest of their lives. I asked Robin how long she has been on it. "Eight years." Clearly, it has extended her life.

Where does it stand in the approval process at this very moment? It still has not been approved by the FDA. The head of research for my hospital is going to give us the latest updates regarding this drug. If you have IPF, hang it there. Hopefully, it will be available soon.

Monday, February 17, 2014


After working out at the other rehab yesterday, I hit all the stores: butcher, Trader Joe's, organic store, Safeway. Then, I hauled it all into the house and put it away. I needed a rest after that! It was gorgeous here on the coast: sunny and 70 degrees. Tourists abounded.

Late afternoon, Michael and I sat out and chatted about life, my diet, our upcoming road trip, neighbors and relatives for an hour. I had spoken to William earlier in the day, so I wanted to share that conversation with him. We won't hear from him for three weeks when he gets back from the Australian leg of the tour.

We looked around our cul de sac and realized we and the very old couple directly across from us and the new people at the corner were the only ones home. Everyone else was gone for the holiday weekend. Six out of nine home. Empty. A burglar's dream! Thank goodness we are in a very safe area.

No doctors appointments for mom or me this week. Nothing but rehab and yoga and cooking fabulous dinners for Michael. A nice week.

Happy President's Day.

Sunday, February 16, 2014

Quiet Plans

It was a very quiet day yesterday and the weeding did happen. The rains blew through in the evening so the sun is shining at the moment. I love morning sunshine as it turns the house golden. Cozy.

I am driving over the pass with Michael this morning then working out at the other rehab. Afterwards, I am going to the butcher, Trader Joe's, Safeway and our little organic store for Michael's breakfast chicken mango sausages. I am a good wife!

For Christmas, one of the presents I bought with mom's money was a pair of black chords. Size 10 short.  They were a bit tight but after ten minutes, they were fine. I wore them yesterday and after 15 days of this liquid diet, I was able to pull them off without unsnapping or unzipping them. What a surprise. This diet is really working!

We are beginning to plan the road trip in May. Seven thousand miles of togetherness! The month of May can be a challenge through the Denver area as there may be a sudden snowstorm that closes the interstate. So, we will plan the trip on Highway 80 but also on Highway 40, in case the snows hit. We pride ourselves that we spend minimal time on the interstates and love the smaller highways. But this trip, we are going to use the interstates to scream to southern Ohio, then we can slow down and take our time. Our real trip will begin.

Saturday, February 15, 2014

Into a Quiet Weekend

We have a rather quiet weekend ahead, thankfully. Nothing we have to do. Nothing required of us. I do hear the distant calling from the weeds in the side yard, so I may answer their call this morning. It should take about three hours to clean the area. Rains are due around 6PM so we will sleep tonight with the light rains serenading our dreams.

Mom and I giggled and chatted the day away yesterday. After working out at the other rehab, I drove her to the every 6-week shot in her eye for Muscular Degeneration. From there we picked up my new contact lenses before a lunch at Carl's Jr. Mom had seen an ad for their turkey teriyaki burger. She ate every bit. We must have sat and talked for an hour.

Last week, mom asked me to buy a very small corned beef from my private butcher. Well, we bought the smallest they had but it was rather large, it was reduced by half during the 3-hour cooking process, she cooked it up, ate off of it for several meals, ground and froze some for sandwiches and sent the rest home with me yesterday. The smell almost brought me to my knees! It was divine! Michael is going to enjoy it in sandwiches and maybe with some eggs tomorrow morning.

The diet is going well. My pants are falling down, not attractive but a good sign. Eleven more days until I see the nutritionist and weigh in. Yes, I am counting the days. The liquid diet is harder this time around. Three years ago, I was healthier and younger but I am determined to stick with it. I am dreaming about food, dreaming that I made a mistake by licking my fingers, dreaming that I lost my purse. Oh, that's another dream. The Food Network is my current best friend. I am obsessed with food blogs and I am gathering lots of new low fat/low carb recipes to cook when I can eat food again. This coming week, I am cooking dinners for Michael from the 80s and 90s. He has loved my diet so far, though I think he has gained a pound or two. I am cooking foods we have not eaten in decades. The enchiladas and lasagna were huge successes. He is sure to enjoy these great dinners this week: my homemade Clam Chowder, Fideo (thin spaghetti in a light tomato sauce) and Pork Chops, Hot German Potato Salad with Kielbasa. He is never going to want me to stop my diet!

Friday, February 14, 2014

Mozart Festival

 My soul was filled back up to the brim yesterday. I met my friend Lois, who had just returned from her yearly adventure to the Mozart Festival in Salzburg, Austria via Munich, Germany. After twenty years of attending this festival, she enjoyed her favorite hotels, restaurants, gardens, museums and statues in both cities. The weather was cold but rather nice this year. They actually saw some sunshine!

This is a widely attended international festival. Buses from all over Europe were parked along the streets, women dressed in formal kimonos walk the streets before and after the concerts, different languages could be heard throughout the city. Lois went to high school in Switzerland, learned German there but with a strong Swiss accent. Her yearly hotel mates from Switzerland consider her a Swiss native because of her accent.

Over a year before the concerts, hundreds of performers throughout the world submit their suggested program, a committee then chooses who is accepted, the tickets alone for the three concerts a day for 10 days ran Lois about $1,500. These are all pre-ordered, she books her same hotel each year and has her routines. She is a member of a high level chorus and needs to keep her voice going, so she rents a rehearsal room at an elementary music school, where they great her kindly every year.

She said that this year's concerts were the highest level of performances that she had seen in twenty years of attending the festival. The Latvian Radio Chorus was superior (who knew?), she was present to watch Andres Schiff conduct, saw an excellent opera and two concerts with the Vienna Philharmonic. I was rather surprised to learn that this group of extremely high level musician have no head. No Board of Directors. The members run the orchestra. They hire a conductor every year. They decide what they want to play. But, they were the last orchestra to finally allow women as members. I asked Lois how many women were on stage during the two concerts, she replied, "Four." She attended so many concerts, I just can't remember the details of the others.

Talking high level music with a passionate person was so inspirational. I miss these conversations in my daily life. I worked with respected musician of the highest levels that I almost took it all for granted. It was normal. I still miss parts of my job!

Thursday, February 13, 2014

Between the Storms

Every holiday since I have been sick, I have decorated the table in the family room. Nothing fancy. A simple reminder of the special day. Michael came home yesterday to the table set for Valentine's Day and, to my surprise, took a photo of it to share with his co-workers. That never happened before! I was rather touched!

Also yesterday, I was watching the news when it was announced that Raleigh, NC was hit hard by the snowstorm. I quickly messaged my friend to see if she made it home from work and she phoned. What was so difficult was that it was not expected to be so much snow so quickly. Everyone was caught off guard. For her 10-minute commute, it took over 30-minutes and she was happy just to get home. She said there were scattered and crashed cars abandoned at the bottom of every small hill. Yikes!

I over did it in the garden yesterday. There was a break between the needed rain storms so I was able to cut the grass, trim some bushes and weed a bit. Over three hours later, I was done. Orchestra rehearsal was not going to happen and I was in bed by 8:00.

My brain is going to be challenged today. I hope it still works!

Wednesday, February 12, 2014

Connective Tissue Disease and Interstitial Lung Disease

A large Scleroderma Support Group joined our Interstitial Lung Disease Support Group yesterday for Dr. Deborah Assayag's presentation on Connective Tissue Disease (CTD) and Interstitial Lung Disease (ILD). Canadian Dr. Assayag has been in SF for the last year and a half to study CTD and ILDS.

CTDs are a group of inflammatory diseases affecting the structural portions of the body, form the framework, are composed of collagen and proteins. It can directly affect the joints, cartilage, skin, ligaments and blood vessels.  It is autoimmune in nature, the immune system reacts to proteins. Inflammation is a major component of these diseases.

The diseases:
Rheumatoid Arthritis - affects joints
Sjogren's - affects dry eyes and mouth, lung disease features cysts in the lungs
Lupus - affects joints, only 1% develop an ILD.
Scleroderma - affects skin, lungs, blood vessels, kidneys, largest group of CTD who develop an ILD.
Dermatomyositis (and Polymyositis) - affects muscles, skin, joints, weakness in thigh or arm muscles
Mixed CTD - This is a specific disease not just a pile to put something that doesn't fit with the other diseases. It has its own auto antibodies in the blood.

Usually, the lung clinics see these patient after they have been diagnoses and treated by a Rheumatologist for a CTD. The diagnosis of a CTD bases on the organs involved and then confirmed with blood tests. Each disease has specific auto antibodies. If the patient complains about symptoms of lung disease, they are sent for Pulmonary Function Tests : Forced Vital Capacity (the clear box) and Diffusion Capacity (DLCO). Then, they are sent for a high resolution CT Scan. Blood tests will include serology to check the auto antibodies, some general tests (ANA, RF) and some are disease specific ( SC1-70, AntiCCP, RNP). With an ILD, it is often helpful to have a lung biopsy. As the cause of the ILD is a CTD, there is no need for a lung biopsy.

Natural History of CTD-ILD:
With a diagnosis of RA or Dermatomyositis, the disease progresses very slowly. With all CTDs, the younger the patient, the better they do. There is no difference in gender. If the severity of the CTD is present at the diagnosis of ILD, these patients do worse. It all depends on the amount of fibrosis present in the lungs at the time of diagnosis.

Rehab! A formal exercise program is highly encourages.
Supplemental Oxygen - to protect against the heart damage, blood vessels issues, pulmonary hypertension and strokes.
Medications -
Prednisone is used to help kick start CTDs to help suppress the inflammation. There usually is an initial run of 6-8 weeks.
Mycophenolate (Cellcept) My old friend. This is the first line of medication used on RA, Scleroderma, Sjogren's and Mixed CTD. The side effects are nausea and bone marrow suppression.
Cyclophoshamide - This drug is mostly prescribed for more severe cases of Scleroderma or if the patient did not respond well on the other drugs. It is not used for other CTD-ILDs. The side effects are liver toxicity, bladder cancer 20-30 years later.
Azathioprine (Imuran) I have been on this drug for over 7 years. The side effect can be bone marrow suppression and liver toxicity.
Rituximab - The hot new drug. It is given by infusion followed by another infusion two weeks later then another infusion six months to a year later. It is currently being prescribed mostly for patients with Dermatomyositis. The UK is currently using this drug with other CTDs, not just Dermatomyositis.The possible side effect is lymphoma.

In my ILD Clinic, they treat 40-45% of their patients for Idiopathic Pulmonary Fibrosis (IPF) , 23% for Connective Tissue Diseases( CTD) and 12% for Hypersensitivity Pneumonitis (HP).

Tuesday, February 11, 2014

ILD Support Group Meeting Today

Shirley Temple. Mom and I were just reminiscing about her last week. They were the same age and mom so wanted curly hair like hers when she was a little girl. Apparently, most little girls wanted to be like her. Mom met Shirley Temple-Black years ago at a political women's group and just told me something I had never known: She and dad went to Shirley Temple's daughter's wedding. How did that happen? Mom can't remember the circumstances behind the invitation but she does remember the wedding. In the world of child stars, she was a nice, normal person who loved her husband of over 50 years and her three children. Rest in Peace.

Yesterday, Mom and I dropped into my favorite Costco (I go there once a year for an eye exam and to order my contact lenses) to make an appointment for an eye exam and to have mom help me pick out some new glasses. Well, the doctor took me right in, had the exam, ordered new RED glasses and a year's of contact lenses. We were starved afterwards (I was an hour late for my diet food) and I remembered a place where I used to eat - a crepe place. I loved their buckwheat crepes but since I have learned I can no longer eat such things, I have not been there for at least three years. We arrive, the owner remembered me, didn't mind that I brought my own food, I ordered an iced tea, mom enjoyed a killer steak Tex Mex crepe and we were serenaded by French music during lunch. She LOVED it. We will return.

Michael is not feeling well. I think he has a touch of food poisoning. He had a Polish at his favorite deli yesterday and has been up all night. He is going to stay home today while I will drive into the city to pick up some of the diet food and for the ILD Support Group meeting. It will be our first one since Susan died. Thankfully, the focus will be the speaker on Connective Tissue Diseases and ILDs. I will take notes and report back to you, dear reader.

Monday, February 10, 2014

Eat Some Nuts!

Eat some nuts! Tree nuts or peanuts. Doesn't matter. I was cleaning out the magazine basket yesterday and found this December Science News article featuring a report of a thirty-year study on the healthful properties of nuts, which reports that they can actually extend life. I discovered nuts after I got ill because I read somewhere they had anti inflammatory properties, which is helpful in fighting an inflammatory disease. Pistachios are my favorite and Michael leans towards walnuts. It is our snack. I usually have some around 3PM or after dinner. Other people snack on cookies, we snack on nuts.

Eating nuts may extend a person’s life

People who regularly ate peanuts or tree nuts were less likely to die during decades-long studies
THAT’S NUTS  The healthful effect of eating nuts is reinforced by a new analysis suggesting that peanuts and tree nuts contribute to longevity.
The already-glowing reputation of nuts is now shining even more brightly: Researchers have linked consuming nuts with longer survival. The findings nudge nut consumption a step closer to the rarified circle of activities shown to fend off the reaper.
The study, the largest to tie nut consumption to longevity, jibes with work showing that the oils in nuts can reduce bad cholesterol and possibly inflammation. “If you take the evidence in its totality, the picture being painted by science is quite strongly that nuts improve health,” says Cyril Kendall, a nutritionist at the University of Toronto.
The researchers tapped into two long-term studies tracking the diet, health and lifestyle of nearly 119,000 female nurses and male health professionals. The studies’ databases include information on deaths during 30 years of follow-up for the women and 24 years for the men. The new analysis of survival rates accounts for differences in age, sex, race, smoking status, aspirin use, physical activity and other factors.
The scientists found little difference between eating peanuts and tree nuts, with the data suggesting “the more the better” applies to both. People eating scant amounts of nuts had barely better survival rates than people eating none. But those consuming a serving of nuts — at least 28 grams — two to four times a week were 13 percent less likely to die during the studies. Compared with those who ate no nuts, people eating nuts five or six times a week were 15 percent less likely and those chomping down seven or more servings per week were 20 percent less likely to die. Epidemiologist Ying Bao of Harvard Medical School and colleagues report the findings in the Nov. 21 New England Journal of Medicine.
Longevity seems enhanced by regular physical activity, alcohol in moderation, a diet rich in fruits and vegetables and possibly calorie restriction, studies show. Kendall says that it’s too early to add nut consumption as a stand-alone entry to that list, but nuts hold their own as part of a healthy diet.
Bao and her colleagues also explored how nuts seem to affect survival, finding that nut consumption was associated with less death due to heart disease and to some extent with cancer or respiratory disease mortality. 

Sunday, February 9, 2014

Breakfast Low Fat/ Low Carb Breakfast Recipes

It could have been the rain or the diet or just need, but I slept for two hours yesterday afternoon. Earlier, I had ventured out into the world to the bank then had my toe nails done, which was a true rarity to take the time on a weekend as I am usually working very hard in the gardens on a Saturday. The rains changed that plan. Wrapped up in a cozy blanket, I settled into my chair and slept the hours away.

I found myself craving online food blogs in the evening. I found some great new recipes for my low fat/low carb diet when I am finished with this liquid diet. My biggest issue was breakfast foods. I am so tired of my crustless quiches that I really cannot eat them any longer. While searching last night, I found a wide variety of amazing recipes for deviled eggs. They seem to be the hot new thing. I also found a very cool recipe featuring a vegetable and egg base with wonderful pizza-like toppings. I have a feeling this will be a favorite. The recipes are below:

Summery Pizza Omelette - Looks fantastic. A nice breakfast.

Buffalo Stuffed Eggs - I would use low-fat mayo to cut the calories.

Three recipes for Deviled Eggs -

Shrimp Stuffed Eggs - Yum.

Breakfast Meatballs - This recipe makes a ton so I would freeze them. I also would use a turkey or chicken sausage and a low-fat cheddar cheese to cut the calories.

Saturday, February 8, 2014

Diet Lunch Out in the World

The last time I was on the liquid diet (for three months), I refused to be in any social settings like parties or dinners or even lunch out with friends. Just couldn't deal with it. This time around, there is a difference out of need: my mom. She will not eat out alone and so looked forward to our twice a week or more lunches together. We always stayed chatting over our empty plates for at least an hour. Mom has an issue with not eating enough. During our lunches, I am often amazed at how much she will eat if the conversation is good and she is enjoying herself.

Just because I cannot eat, I did not want these lunches to end. I had to adjust. To my surprise, it has been rather easy. We went to Nordstroms for lunch yesterday. I brought a chocolate pudding thing with me as part of the diet and ordered a nice unsweetened iced tea. Mom enjoyed a bowl of their tomato basil soup and a small turkey sandwich. She ate every bit. We sat and talked and waved at all the children and talked to a grandma visiting from Ireland and looked at all the pretty women wearing lovely clothing and our favorite waiters fawned over mom, as usual. I hardly missed the food.

The diet food is disgusting and I hate it while I am drinking or eating it but afterwards, I love the feeling. I am never hungry, I feel strong and I swear I have lost several pounds during the first week. I don't want to weigh myself before seeing the doctor on February 26, almost a month into the diet. I want to feel the joy of looking at the surprise number, quickly doing the math then dancing when I hear the number of pounds I have lost.

My other challenge this time around, is cooking for Michael. Last time, it took a month of staying out of the room while he would cook his own dinners before I was able to cook for him. I am having no problems this time. I did make the lasagna for him this week, something I had not made in a decade. Tomorrow, I will serve panko covered fresh Alaskan cod with a side of gnocchi with pesto for a special treat. He has requested enchiladas next week, something that I haven't made in maybe fifteen years. My goal is that if I have to go on this diet again, he will be thrilled as he will have special foods that we don't include in our diet anymore. His skinny, non-prednisone body can take it.

So, I am doing fine. I hate all the food except the protein bars, my treat I save for the last meal of the day. The results will be worth the effort.