Living Well with a Bad Diagnosis - Lung Disease

Monday, April 30, 2012

Maybe, Just Maybe

Sunday's concert venue
Yesterday, I noticed when I took a long, steep set of outside stairs through a garden at a fast pace that maybe, must maybe my breathing is getting better. I realized it when I was standing at the top without being short of breath.

This means that maybe, just maybe, the large dosage of prednisone is reducing the inflammation in my lungs and there just might be a chance that I am getting some lung capacity back.

I am thrilled.

This event happened after the concert with the little violin kids at a beautiful venue but difficult to carry my large, heavy string bass up and down odd garden steps. Claudine’s husband was gracious enough to do the hauling round trip. I was running up the stairs trying to keep up with him after the concert. I am still so surprised that I was able to do it so easily.

The concert went really well, several of the students performed as soloists playing songs so familiar to me with all my performances at the school still in my brain. It was joyful to watch and listen to children play music again.

So, the final rehearsal until September is Friday afternoon in preparation for the group who is scheduled to perform two songs as a prelude to my orchestra concert next Saturday night. I am feeling good about that concert and that will be my final performance until the fall. The summer off. Time to have some work done on my bass during the break.

Mom and I are getting together after my rehab this morning and heading to Big Lots. I have never been to one so that should be an adventure followed by a nice lunch thrown in somewhere.

Monday. A rather quiet week ahead. No doctors. Just rehab and two rehearsals.

Sunday, April 29, 2012

New Dress All Weekend!

The new dress. I wore it for the first time to Natalie's 50th birthday party yesterday afternoon. It was fun to feel so girly in a sweet, cotton dress and tall spiky sandals. Threw on some nice jewelry and I was feeling great.

Photo of the new spring dress made by
Presley Skye from their site.
Their house was packed with people and food and wine and gifts and conversation. We had a ball. Yesterday, my Yahoo horoscope yesterday said to listen more than talk. I made sure to do that and really enjoyed myself. Interesting people and conversations abounded. I ate very little because I made sure to eat a large-ish lunch earlier. It worked. I did not eat any of the pasta and just had a forkful of cake. Thank you for sharing, Michael!

So many of their relatives and friends whom we have met through the years had not seen us since my big weight loss. Lots of questions. One even wanted more details about my illness and how we are handling it. As a rule, I only offer so much unless specifically asked for more. She really wanted to delve. I tried to keep my answers short and eventually, she got the bigger picture.

Earlier in the day, I worked in the gardens while Michael worked on the Mustang. It was fun to have him home with me on a Saturday. Such a rare treat.

Today, the new dress goes on again before I head north to sit in Dream Machine traffic to play a concert with the little violin students. Afterwards, Michael is going to haul out the BBQ and cook a beautiful rib eye steak while I cook crimini mushrooms on the stove top. A steak. Another rare treat.

Saturday, April 28, 2012

Not a Normal Saturday


Friday, I made my way into the city for an appointment with Dr. M, my endocrinologist. We met to review my latest thyroid and cholesterol tests. All are perfect! I will slowly be adding two more fish oil pills a day for a total of six!

What was really nice was Dr. M. told me I looked fantastic but was sadden to hear that I had the major downturn with my lungs. I did wear my new black and white summer top over a black pencil skirt with green apple colored sandals with a small heal. I felt that I looked put together and stayed in the outfit to show Michael, who arrived home at 3:00. I had to take off at 3:30 for the final rehearsal with the little violin kids for our concert on Sunday. I changed into a shirt, jeans and flat sandals. Comfortable.

It is sunny and beautiful on the coast today. Should be in the 70’s and thousands of people are descending upon us for the Dream Machines, a fundraiser for the local senior center. Antique planes, fancy and classic cars and lots of food booths bring the crowds into the small airport north of us. We are staying home.

Yard work and bedroom wall painting are on the list of things to do this morning then, at 3:00 we are heading down the street for a 50th birthday party for Natalie, of Rick and Natalie. It is an Italian party and, as Michael is a HUGE Godfather fan, we offered to “bring the cannolis!”

My garden is bursting into spring. Here are some photos.


Enjoy your Saturday!




Friday, April 27, 2012

Blog About Blog Statistics


When I look at the statistics of this blog and the audience it attracts, I am humbled that most of the countries from around the world – including China – have checked in. It is remarkable.

This month, someone or a few people from Russia have found me. Because they are reading so many past blogs, I have the largest number of hits ever for a month. April has been a good month. So, thank you to everyone but especially to the readers in Russia!

I was also shocked to read that this blog has been noted as being an excellent site for medical information. Again, I am so grateful that I have been able to pass along everything I continue to learn about living with a lung disease.

So to you, dear readers, thank you for hanging in there with me. I hope my story has helped you with your life and what you are facing. I hope that the medical information I am able to pass along improves your knowledge of your disease.

When I get a posted comment that something I have written has helped, it makes my day. Thanks to you all!

Thursday, April 26, 2012

Information from the Coalition for Pulmonary Fibrosis


Below is news from the wonderful people from The Coalition for Pulmonary Fibrosis. Check out their newsletter full of information as well as information about the American Thoracic Society's (ATS) conference in May. This is the conference that I have been asked to give a formal speech on my disease, Hypersensitivity Pneumonitis.

Check out all the sites below:

Also, please join us for these these UPCOMING EVENTS: 
San Francisco, CA–Friday May 18th The ATS Public Advisory Roundtable and the CPF are pleased to invite you to the Patient & Family “Meet the Expert” Forum and “Quality of Life” Presentations. For more info. and to rsvp, please click here:http://www.coalitionforpf.org/cpf_events_detailes.php?id=552.


Daughters of PF Member Meetings A chance to meet and talk with CPF staff and other daughters about the program, your ideas, and how the CPF can help you.
New York, NY - Saturday May12th http://www.coalitionforpf.org/cpf_events_detailes.php?id=550
San Francisco, CA - Friday May 18th http://www.coalitionforpf.org/cpf_events_detailes.php?id=551

Save the Date: September proclaimed National Pulmonary Fibrosis Awareness Month:http://www.coalitionforpf.org/cpf_news_detailes.php?id=549 Watch for news of how you can be a part of this month of activity around PF.


Click here for other Upcoming Events: http://www.coalitionforpf.org/cpf_events.php

Wednesday, April 25, 2012

A Wake Up Call

I had a whole other blog planned for today. It can wait. In past blogs, I have written about the other blogs I read that give me inspiration and strength. One that I never miss is written by Stephanie Nielson who, along with her beloved husband Mr. Nielson, was in small plane crash. Each had injuries but she was burned over 80% of her body including her face. She just recently had a baby and had to have surgery immediately afterwards to repair her burned skin on her belly. Very painful. Her other four children welcomed the new baby, Charlotte into this world. Stephanie is just 30-years old. 

Here is her site:    http://nieniedialogues.com/


This morning, I was feeling a bit down. I am exhausted but not sleeping. The world is quiet and dark from 12:30-5:00. I know. I watched. Is it the large prednisone dosage? Am I so overtired that I can't settle in to sleep? I so miss my former good health just months ago before the bronchitis. 


Then I read Stephanie's column this morning. I have nothing to complain about. It was a wake up call. What especially called to me: 
I pray for strength and peace.I know we each have individual trials specific to us, and this is mine. 


So, I will head into the world this morning and do my best. I will get home and take a nice nap this afternoon and go to orchestra rehearsal tonight. My life is a cakewalk compared to what Stephanie is going through now and the rest of her life. The pain must be incredibly horrible. 

Here is her blog for today:

I am home from the hospital after another long and painful surgery.

I walk hunched over with 2 diffrent drains coming out of my body.
I was so anxious and frustrated having to leave
Charlotte and go in for surgery,
but this procedure was time sensitive and had to be done.

Walking up from anesthesia is always the worst.
Getting the pain under control is hard and takes time.
When I woke up it was 11:00 p.m. I went in for surgery at 11:00 a.m.
Mr. Nielson stayed with Charlotte as long as he could and then left my room
with my pumped supply of milk and
I was alone. I felt alone too.

Mr. Nielson came the next morning with Nicholas and Lottie.
Nicholas walked into my hospital room and wouldn't look at me.
He walked close to Mr. Nielson holding his hand and looking at the floor.
I thought about the time when the children came to
see me the first time since the accident.
How I felt, how they acted, how I acted, how the looked and showed emotions.
Then I remembered I was fitted with a neck brace and had oxygen tubes in my nose.
It was a bit frighting.

{Lottie asleep in the hospital baby bed}

Nursing Charlotte at first proved to be extremely painful
but as days go by, it has been much easier.
I am always amazed at how hard these episodes are.
Even though I have been through numerous surgeries and
hospital stays it never gets easier.

The children hate to see me in pain.
Oliver cried when he saw me. He has such a tender little heart,
and he told me he hated to see me sad and hurt.
I had a good cry right along with him.

I thought being home from the hospital would be easier.
Better food, better views, my bed, my toilet and so on and so forth.
But instead it is constant reminder that I can't do the things I freely did just a week
earlier, and that bothers me.

Mr. Nielson keeps telling me that this is just temporary and I will be back soon.
I know that is true, and I know it wont always be so hard.
But it is.
I try to live hour by hour not thinking too far ahead.

Last night in bed I kept thinking about how my 30 year old body
feels like a 100 year old body and I still couldn't get over the fact that
this one shot at life- this beautiful mortal life has been so hard.
Yet still a gift.

I had a dream last night that I was at my families cabin in
the mountains in Utah.
I was young and my body pain-free.
I was me about 20 years ago.
I was wearing a night-gown with ruffles around the collar.
I had braids in my hair.
I went to bed upstairs in the loft and woke up in severe pain.
I couldn't walk very well and my stomach and back hurt so intensely.
I tried yelling for my Mom.
I walked down the stairs and into her bed and I cried to her and told her that
I hurt all over and she held me and tickled my back.
She told me that I had to go through this pain to have a beautiful life.
I didn't understand.

I woke up in my bed in pain and I missed my Mom.
I wondered about my dream.
My door opened and Nicholas came in.
He looked like a little angel.
His hair was messy and his jammies hugged tight to his little body.
He climbed onto my bed next to me and asked me
5 year old questions.
As I tried ticking his back with my wraps and tubes,
but I think then I understood my dream.

I have a beautiful life. The people in it are beautiful and this pain
is all worth it, I still cry a lot but pray more.
I pray for strength and peace.
I'd choose pain any day if it means I can have my family and my
knowledge of God's plan for me.

I know we each have individual trials specific to us, and this is mine.
But just like this trial is mine, so are my children and my husband.
And I am never alone.

Tuesday, April 24, 2012

Tired But Still Standing

I wish was I feeling better. It has been three weeks since I began the increase in prednisone to counteract the downturn with my lungs caused by acute bronchitis. Halfway. I still have been coughing a lot. A dry hacking cough. Happens when I am carrying boxes up to mom's apartment or vacuuming the house or hauling buckets of weeds from the garden.

In bed, I also notice that my breathing still is restricted while I am trying to go to sleep. It just doesn't feel right. In three weeks, I will be having a full run of PFTs and a high resolution CT Scan. I am rather worried about the results.

My to-do list is rather full today. I told Michael that things should calm down a bit after May 5th. The final orchestra concert is that evening and the concert with the little violin kids is this Sunday. No more evening rehearsals until September. A break. A needed break.

I guess I am just tired. Tired of feeling tired. But, my body tells me to keep up with rehab. Tells me to eat well and try to sleep as much as possible.

So, I am tired but still standing. Still moving forward.

Monday, April 23, 2012

Weekend Update

It was such a gift. Saturday, the weather here on the coast was in the high 70’s. I wore shorts and a t-shirt all day while working in the garden. A full seven hours. After cutting, trimming, watering and planting what turned out to be more than eighty plants, it looks fantastic. Ready for summer.

I dragged myself into the kitchen to make a dinner of Greek Chicken with a Greek yogurt, cucumber, dill and pistachio sauce and a Greek salad on the side for Michael. Heaven.

It was all I could do to wait until 6:30 to climb into bed. I slept until 6:00 Sunday morning. I was done. The F1 race from Bahrain was recorded on the DVR and ready to go with our morning coffee. Rather a surprise that Sebastian Vettel won but the driver I am watching this year is the new kid – Romain Grosjean. He made the podium in third place. Unbelievable. Amazing young driver. Keep your eye on him!

Good thing that the coffee was hot because it was so cold in the house. Surprise! The fog had rolled in and never left the coast Sunday. It was cool all day. Fifty-five degrees cool. What a difference a day makes.

Early to bed again, early to rise to grab mom for early rehab then back to our house. She wants to go through all of her boxes once more before we put them into the attic.

In fun news, I got a text from Kimberly on Saturday who was in the city on a tour with her band. She had never been in the city when it was so hot. We told William we would love to see her in concert if she wanted us but understood if she was just too focused or nervous her first time out with this group to want us around. Nice she sent a hello. Next time. She did write that it was going well and everyone was getting along. That is a big deal when traveling with a group of people for the first time. So happy for her!

Sunday, April 22, 2012

Medication Update


It has been awhile since I have listed my medications. It is often interesting when we share which medications we are taking in our ILD Support Group. For example, I learned about NAC during one of the meetings.  I have also listed all the over-the-counter supplements, too. I buy them online at a much reduced cost.

Here is the latest:

Diseases:       Hypersensitivity Pneumonitis – Interstitial Lung Disease
                        Hashimoto’s – Autoimmune Thyroid Disease
                        Glaucoma – Eye Disease

Azathioprine – 50mgs – 75 mgs 2x per day – bone marrow drug for lungs

Boniva – 150mgs 1x per month for bone loss due to prednisone

Ipratropium Bromide Nasal Solution – as needed for runny nose

Levoxyl - .15mg – 1x per day– for treatment of Hashimoto’s - Thyroid

Lipitor – 10 mgs – 1x per day for high cholesterol due to prednisone

Lumigan – 2.5 mil – 1 drop each eye 1x per day for Glaucoma

Oxygen – 2 liters at night and during exercise

Prednisone – 17.5 mgs – 1x per day – for lung disease (currently at 25mgs)

Prochlorperazine – 10 mgs – as needed for nausea due to Azathioprine

SMZ/TMP – 800/160 – 1x per day 3x a week – for Azathioprine side effects

Spiriva – inhale 1 capsule 1x per day – for lung restriction

Spironolactone – 50 mgs – 1x per day – as diuretic

OVER-THE-COUNTER
Calcium – 1500 mgs 1x per day for bones
Cranberry – 450 mgs 1x per day to fight UTI
Ginkgo Biloba – 60 mgs 1x per day for memory
Omega-3 Fatty Acids Fish Oil – 2,200 mg 2x per day for cholesterol
Hair, Skin and Nails Formula (vitamin supplement) – 2 per day
N-Acetylcysteine (NAC) – 600 mgs 1x per day for lung disease
Stool Softener – 2x per week
Vitamin B-12 – 1,000 mcg 1x per day due to my diet
Vitamin D – 2,000 IU 1x per day as my levels were very low
Vitamin C – 600 mg. 1x per day as an anti-inflammatory

Saturday, April 21, 2012

Our Excellent Adventure!

We took the day off yesterday. It was a glorious day with temperatures between 68-84. Perfect convertible weather.

We dressed perfectly in light clothing, wore our hats to protect us from the sunshine and headed north. Views were world class. The iconic orange bridge against the blue water was spectacular. We were heading into unknown territory.

We were heading to Tiburon. It is a spit of land north of the city with only one road in. Attached to this spit is an island of some of the very wealthiest people in the area and also the most stunning views. We have never been on this spit of land. Many people access it from the city via ferry.

We wove our way through the area to the very end of the spit and just fell into the closest parking lot. This is an area of tiny shops and tiny, twisting roads. Lots of trucks were parked unloading goods for the weekend. We were there very early. We walked the whole downtown area, sat near the water watching the ferries while enjoying the sunshine then decided where to eat an early lunch.

Sam's Anchor Cafe. Natalie's dad lives in Tiburon and I had told her we were heading there. When I told her we ate at Sam's, she said it is a landmark. Good choice. We ate on a pier in the sunshine looking at the city's silhouetted in the distance and we were both totally relaxed.

Lunch? Oysters. The best: Hog Island Oysters. Mine cooked, Michael's raw. I also had an artichoke while Michael had a crab sandwich, which was perfect.

Afterwards, we headed back over the bridge, though the Avenues, back to our shopping area over the hill to buy a Cheesecake Factory cheesecake for a birthday party for Michael's boys-night-out. We also bought mom a single piece and dropped it off at her place. We relaxed for an hour before I had to leave for a rehearsal with the little violin kids and Michael to his party.

Just as I was heading to bed at 8:00, I got a phone call that lasted until 10:00 when Michael arrived home.

Today? It is a more beautiful morning and I am planning to cut, trim and water the garden as well as plant over 40 plants. My summer plantings. If I can't get it all done today, I will finish it tomorrow.

Friday, April 20, 2012

Worried About Sherman


I am worried about Sherman, my 87-year old rehab class friend. The past two weeks, there has defiantly been a downturn with his breathing. He was a long haul UPS driver and smoked like a demon all day while driving. He has emphysema. Nothing else is wrong with him.

I noticed yesterday that he was so very pale. Not a lot of color in his cheeks.

We got checked in at rehab, walked to the back of the gym where he put his small oxygen tank in a locker then walked just steps to get one of the gym’s larger oxygen tanks. By that time, he had to stop and breathe in oxygen for at least five minutes to get his breath back. He struggled so hard just to breathe. I stood with him until he was able to breathe again but he had to kick it up to 6 liters, which is the highest level of supplemental oxygen ever for him.

Two worries: Is this due to an infection cooking in his lungs? Is this a permanent downturn maybe going in an even worse direction? I am not ready to lose him. He is my dearest friend. I so forget that we are not the same age.

I asked if he would let the RN listen to his lungs. No. I asked again. No, again. He is seeing his doctor in three weeks.

I worry.

Thursday, April 19, 2012

Warning: Nap Ahead


I am whipped this morning. Yesterday, Mom and I enjoyed the long drive and lunch with her dear girlfriends and the orchestra rehearsal went well, but I just couldn't sleep last night. That happens sometimes when I am too tired or too hyper from the rehearsal to settle in. So, I head into the day on only six hours of sleep. That is not good.

A visit to the nursery to buy summer planting for this weekend then the rehab class is on the schedule for today. Hopefully, there will be time for a nap this afternoon!

Our focus, though, is our day off tomorrow. Playing hooky: not answering our cell phones, our convertible taking us on an adventure. No decisions on where we are going yet. The weather should be beautiful. Perfect, in fact. 

Wednesday, April 18, 2012

Prednisone and Announcement of New Lungs!

I just don't feel well. My numbers were low again during the rehab class yesterday. Today is the last day of the higher dosage of prednisone, 30mgs. Tomorrow begins 25 mgs. I wish my breathing was less labored.

Today, my job is as chauffeur and to observe the joy of my mom seeing her old friends during a luncheon party. It will make me smile. She is so loved by this group of women. It means 200 miles in the car but worth every mile.

Tonight is one of the final three rehearsals with the orchestra before our May 5th concert. I hope to be awake!

The ILD Support Group received word that one of our members just received lung transplants. We hope he will soon be well enough to come to talk with the group about the entire experience. Our collective fingers are crossed that he will stabilize and be released from the hospital soon!

Tuesday, April 17, 2012

The Year of Being Social Continues

Four recent invitations. Seven events in May and June. 2012, the Year of Being Social continues.

My niece's wedding is May19th followed by breakfast the next morning with the family. As the venue is three hours away from our home, we will scream back home, throw the fancy semi-formal clothing on with some jewels for the American Thoracic Society dinner in the city at 6:30PM. RSVP for both events? Done.

My formal 5-minute speech for the Society is May 23rd from 2:00-4:30. I will post it to this blog afterwards.

We have been invited to celebrate our neighbor Ron's daughter Marisa's graduation from law school. Guess which date? May 19. Same date as my niece's wedding. RSVP? Not yet.

A friend of Michael's who worked on the Mustang that Michael is building with William has invited us to their wedding on July 7. What is so different about their invitation is that a notice was included that they are only registered at one site, which is to gift money towards their honeymoon. RSVP? Not yet.

An announcement was sent that a friend of Michael's daughter is graduating from Arizona State. We must send a card with a check. Done yet? No.

Natalie of Rick and Natalie is going to turn 50-years old this month. The big party is April 28th. We must think of something special for her. RSVP? Today. I promise.

Social and expensive, this Year of Being Social.

Monday, April 16, 2012

Rest? Ah, Next Weekend!


Rest. It has become my mantra. Saturday, I weeded the very front yard for two hours. Relaxing for me. It felt so good. After a shower, Michael and I sat in the garden enjoying the sunshine. Sunday was lovely. I finished the final book of The Hunger Games series, organized some paperwork, cleaned out the refrigerator, washed and ironed our sheets and took a nap. A good nap.

It was a very quiet day.

I spoke with my aunt in Tennessee where we were a year ago on our road trip for her granddaughter’s wedding. She has recently moved into a lovely resort-type retirement home just a few months before my mom moved into hers! Alma is not feeling well and is beginning to go through a lot of medical tests. She is just a bit of a thing. Wears girl-sized clothing. She is well under 5’ tall and weighted under 80 pounds when we saw her. It worries me that she is not well because she doesn’t have a whole lot in reserve.

I spoke with my brother. He was worried about telling my mom that they were not going to make it to my niece’s wedding. I made sure it would all be fine and it was. I learned so much managing things at the school! His project is coming to an end just about the time of the wedding and they just don’t want to commit to that expense during these times.

Today, I am going to rehab then will meet mom for lunch and to help her hang some more things on her walls. She is settling in.

Before the rehab class tomorrow, Natalie, Winnie and Oliver are going to swing by after swimming lessons. It is Natalie’s birthday. When we were working together, it was a big deal. A really big deal. It was marked on all of our calendars months ahead. Since becoming a mother, not so important. She has grown into an outstanding mom but even more importantly, a brilliant, kind, thoughtful woman. I so want to celebrate her birthday with her. So, I will pick up a great cake and balloons for a quick celebration. Shhhhh! Don’t tell her!

It is going to be a good week. Wednesday, mom and I are driving out for a lunch with all of her girlfriends whom she misses so much. Orchestra rehearsal will happen that evening so I will be very tired.

Last night, Michael and I vowed to take next Friday off. Fancy lunch in the city? A Tyler Florence restaurant Michael has been wanting to try? A ride down the coast or a ride up the coast? Down would be a beautiful drive and a light lunch. Up the coast would be fresh oysters along the water after a beautiful drive with breathtaking iconic views. Maybe even fresh sardines!

So many decisions! So much to look forward to!

Rest? Next weekend, for sure.

Sunday, April 15, 2012

Trying To Live Well


If you have been reading any of my more personal blogs, you must know that I am a pretty strong person: dealt with what happened to me and moved forward. Kept being active and living life to the fullest.

But, I have to admit that I am almost grieving over the loss of my better health with this latest downturn. Losing 10% of my forced lung capacity, I miss feeling good in the morning. The past several months, I hardly noticed my breathing because it was so easy and natural not so pushed and struggling as it is today. I miss having a high energy level, which allowed me to enjoy life more. And I did! It was a great five months.

It has bothered me that I am feeling this loss so hard. Michael said that I need to cool my jets and gather my strength again. It will come back with lots of rest and rehab and taking care of myself. And maybe some rides in the convertible!

But, I want to play upcoming concerts with the little violins students and the orchestra. I want to enjoy my niece’s wedding. I want to not be exhausted driving mom out to visit her old girlfriends on Wednesday.

I want to feel well again.

So, I am gathering that internal strength that has driven me from the very beginning of living with a fatal diagnosis. It’s there. It is a bit deep right now, but it is still there.

Saturday, April 14, 2012

NEW Minimally Invasive Surgery to Treat GERD


My friend Joanne found this new procedure for treating heartburn (GERD), something it seems all ILD patients have to address. I had the Nissen Fundoplication surgery, which required a full month recovery and a stay in ICU. Not a lot of fun but it took care of the problem for me. I am grateful I had the procedure but this appears to be so less invasive.

Pass this along to your doctor if you need to consider intervention because of GERD or microaspirations:


Minimally Invasive Thoracic Surgery Technology at BMC
Incisionless Surgery to Treat Heartburn at BMC performed by Dr. Hiran Fernando
Surgery without an incision is now a possibility. The new Transoral Incisionless Fundoplication procedure or TIF® using the innovative EsophyX® device for the treatment of gastroesophageal reflux disease (GERD) is now available from Dr. Hiran Fernando thoracic surgeon and director of Minimally Invasive Thoracic Surgery for the Department of Cardiothoracic Surgery and Dr. Michael Ebright Co-Director, Lung Cancer CyberKnife Program at BMC.

Dr. Fernando and Dr. Ebright are the first specialists in Boston to be trained on the TIF procedure. "The TIF procedure with the EsophyX device can significantly improve quality of life for our patients." said Dr. Fernando. "GERD is an anatomical problem which needs an anatomical solution. Reflux medication like PPIs (proton pump inhibitors) can help relieve patients' heartburn symptoms, by decreasing acid production from the stomach, but don't solve the underlying anatomical problems so that reflux symptoms can persist. Even on PPIs many patients are still unable to eat the foods they want or have to sleep sitting up to reduce nighttime reflux. After the EsophyX TIF procedure, clinical trials show that most patients can eat and drink foods they have avoided for many years. Reflux no longer impacts their life like it previously did."

EsophyX is based on established principles of surgical repair of the antireflux barrier, except that it is "surgery from within" performed transorally (through the mouth). The EsophyX TIF procedure can reduce a small hiatal hernia and creates a valve between the stomach and esophagus restoring the natural, physiological anatomy to prevent gastroesophageal reflux. Because the procedure is incisionless, there is reduced pain, reduced recovery and no visible scar.

The EsophyX TIF procedure represents the next step in the evolution of surgery. Laparoscopy reduced the skin incisions typical of open surgery to a minimally invasive three-to-five port holes. However, laparoscopy still involves the same internal incisions and organ dissection as open surgery. EsophyX TIF requires neither internal incisions nor dissection. EsophyX lowers the hurdle for receiving an anatomical restoration of the antireflux barrier and allows earlier intervention.
With millions of Americans diagnosed with GERD and not fully satisfied with their treatment options, EsophyX offers an excellent alternative.
"Recent studies of EsophyX have shown that the procedure can reduce patients' dependency on medications with 70% of patients remaining symptom free after two years and experiencing a dramatic improvement in their quality of life," said Dr. Hiran Fernando. "We are very excited to be able to offer our patients the same incredible benefits as more invasive procedures without incisions."

Friday, April 13, 2012

April ILD Support Group Recap #3


Observational Trials are also important to moving scientific knowledge forward. Archer Eller also discussed a study funded by a grant called ROMII to study microaspirations (GERD/Reflux) and ILD.

The study includes:
30 people with other ILDs other than IPF
30 people with IPF
60 healthy people

They are primarily using a Bravo capsule that will be clipped onto the esophagus as a transmitter and the patients will use a pager. Eventually, the transmitter will detach and be flushed through the system. This is the study I was invited to join, though I did not qualify. I had a Nissen Findoplication, which stops GERD.

There has long been the question of which came first – Interstitial lung disease or GERD. Fibrosis or inflammation.

The study will involve IPF patients, which is a restrictive lung disease that changes the lung to become stiff. The thought is that while it is growing stiff, it is pulling away from the connection at the end of the esophagus. They also are looking at connective tissue diseases like lupus, scleroderma and arthritis to see if they also show signs of GERD and this “pulling” away from this connection causing stomach acids or microaspirations to enter the lungs.

And finally, the question of whether everyone has some sort of aspirations.

We also learned there is another observational study looking at Biomarkers. They are looking at telomeres in patients DNA. Interesting.

Next month, a woman I met at the Lung Transplant clinic will be speaking to us regarding that process which I have detailed beginning here:

Finally, there was a woman sitting in the back of the room and was quiet until the end. We notice that when a new person joins the group, they need to tell their story. It is good for them. We are a group who can offer support and our own experiences to help a new member. She was really needing help.

She announced that she is one year away from lung transplants but is not able to move forward in the process because she just couldn’t get under the mandatory 30 BMI. I raised my hand and told her that I had help for her and that we would talk after the meeting.

She is a patient of Kaiser. I spoke with her briefly, told her to ask her doctor for a referral to the ILD Clinic. She was worried about who would pay for this change and another member of the support group talked about his experience being a Kaiser patient. She would still be a Kaiser patient even though she is being seen at the ILD Clinic. Next, I told her about my experience at the Weight Management Clinic and told her to ask for a referral from the ILD Clinic at her first appointment.

It gave her hope.

Thursday, April 12, 2012

ILD Support Group Recap #2


What is learned in clinical trials? To advance medical and scientific knowledge. Results may not help an individual participating in a clinical trial but adds to overall knowledge for the larger group. And maybe our children and grandchildren.

At the April ILD Support Group last Tuesday, Archer Eller, the Clinical Trial Manager gave a brief interesting example.

Reading this blog, you may remember that my ILD Hypersensitivity Pneumonitis is the only lung disease that does not develop in smokers. In a Phase 2 study, the HP patients and a control group inhaled carbon dioxide and it was there that it was discovered that smoking killed the antigens before they were allowed to develop into HP. Interesting.

The next clinical trial we discussed was the BIBF 1120 #1199.34 study first introduced in this blog: http://livingwellwithabaddiagnosis.blogspot.com/2011/07/latest-drug-and-treatment-guidelines.html

This study is a worldwide study involving an anti-fibrotic drug. Fibrosis does not just happen in the lungs. It can also develop in the liver and kidneys. This drug inhibits the pathways to stop fibrosis from developing.

In Phase 2, 100 people were randomly given a placebo, 100 on 50 mgs, 100 on 100 mgs and 100 on 150 mgs. They found the difference in the dose effect was 60%. A big deal.

In Phase 3, 500-600 people will be tracked. The number of qualified patients exceeded the goal and the study is now closed waiting to begin by the end of the year. It will be a 52-week trial.

We next discussed the PANTHER study first discussed in this blog here: http://livingwellwithabaddiagnosis.blogspot.com/2011/08/research-regarding-pulmonary-fibrosis.html


Here is latest on this study:
The study began in Europe with 100 IPF patients on a placebo and 100 on N-acetycysteine (NAC) which is an over the counter supplement. They were divided up into three groups:

Placebo
NAC
Prednisone, Imuran and NAC

Archer reported that of the three people from this university hospital in the third group of all three drugs, two dropped out due to side effects. They were primarily suffering from hallucinations. While this study was happening, the Data Safety Management Board discovered very serious problems in the third group of iPF patients taking prednisone, Imuran and NAC. They pulled that part of the study.

The other two groups will continue with a trial for a 52-week study, which is still being enrolled soon. After that year, the data will be reviewed and the results of this study should be published in two years.

He also briefly mentioned a new Phase 2 study of a twice a week injection in IPF patients to treat fibrosis.

The next Clinical Trial discussed was for connective tissue diseases like Scleroderma. The National Institute of Health is running the 52-week study and will follow it for two years afterwards. Scleroderma is an autoimmune disease but when it develops in the lungs, it is the most serious aspect of the disease. The only treatment now is a lung transplant.

Scleroderma Lung Study I (SLS I) involved a doctor using Cellcept as an immunosuppressant in 2 or 3 people with Scleroderma noticed that it was helping. He approached the drug company Roche then did another small study with 7 patients. They all did better.

SLS II had a group of people placed on a placebo for an entire year. After the year, half were given Cytoxan. The study was to compare Cellcept to Cytoxan for an additional year. There was no placebo-only group. They are half way in their enrollment and the results will be published approximately two years after the study. If you have Scleroderma, please ask your doctor to include you in this study.

Tomorrow’s blog will discuss observational studies and bench work as well as a story about a new person to our group.

Wednesday, April 11, 2012

April ILD Support Group Recap #1


The subject of this month's ILD Support Group was research. Archer Eller, the Clinical Trial Manager, gave a very detailed update to the group on studies and research for Idiopathic Pulmonary Fibrosis (IPF) patients. Of all ILDs, the clear majority of patients have IPF, therefore that group of people are used in studies. When a medication to treat IPF is approved, it can be prescribed off-label to patients with other ILDs.

The research goal is to insure that the research is safe, to create good data and to involve a specific population. The results are only trusted when the study is randomized, includes a placebo control and is a double-blind study.

Archer began with a short tutorial on the process of research. It begins with an idea then tried with chemicals in a petri dish. Humans are brought in during Phase 1 who are usually poor, healthy college students paid to try, for example, a pill. This phase usually involves 5-10 people.

Phase 2 involves a target population from 50 to maybe even 200 people. The question is whether the new medication is safe, see if it works and to fine-tune the dosage.

Phase 3 includes hundreds of people, usually 500-600. Specifically to our group, they usually include IPF and transplant patients. This is the final phase before FDA review and approval.

Phase 4 is after the drug is marketed by following any side effects.

We began the discussion of the saga of the research on the drug Pirfenidone, which was the first anti-fibrotic drug ever. It has been approved in India, Japan, Europe, Australia and parts of South America. At the last moment, before it was expected to get FDA approval in the US, they requested more research.

The first Pirfenidone trials began in Japan then my university hospital enrolled seventeen patients in the 004 study then there was also 006. The name of the study was CAPACITY. They took the results to the FDA who found it to be helpful in treating IPF and five people continued with the study. Six years later, it was expected to be approved to open label, which could then be used on patients with other ILD diseases. The long term effects would be studied. But, it didn't happen. The FDA wanted another one-year study.

The FDA found that the people who had a good result were in a specific sub group: people who had IPF but had specific other numbers. This Phase 3 study is called ASCEND. The specifics to be included into this study included:
Diagnosed longer than six months but not more than four years
Forced Vital Capacity of 50-90% predicted (mild or moderate)
FEV/FVC (Blow out 1 second) greater than .8 or 80%.

At my hospital, they tested fifteen people for this study but nine failed to meet the requirements.

The process began with the initial screening visits, which sometime lasted two weeks or months, enrollment was next in which the patients were randomized to insure a double-blind study then, when the study finally begins it will last 52-weeks. They anticipate that it will begin in 2013 and completed in 2014 when the data will be analyzed for 3-6 months. The FDA will also study the results so the earliest the drug will be approved in the US market will be 2015.

But, here is the problem. Once it is approved for use in the US as the first anti-fibrotic drug, there will be no attention paid to only treat this subgroup of people that showed a positive result. So, the drug itself will be VERY expensive yet probably not do anything for a huge number of IPF and ILD patients.

IPF is an orphan disease. It does not have a lot of public awareness. Apparently, there is not a lot of money to be made by the drug companies because there are not a lot of people with the disease to take their new drug. Why spend all the money on research?

We were told an interesting story of another drug that had been discarded by the developing drug company and picked up by a non-profit. It was a drug for cystic fibrosis, an autoimmune disease. The Cystic Fibrosis Foundation bought the rights to the drug, developed it and now owns it. They have made enough money from it that they are now doing other research on other drugs. A success story.

The hope is that the Coalition for Pulmonary Fibrosis or the Pulmonary Fibrosis Foundation will step up and begin to develop these drugs as a non-profit, just like the story above.

In the following blogs, I will report about the latest in the hopeful BIBF trials, PANTHER NAC trials, the National institute of Health's trial of connective tissue studies, Scleroderma Lung Study regarding cytoyan, and observational study regarding GERD, biomarkers and the suspected reason why ILD patients develop GERD.

Tuesday, April 10, 2012

ILD Support Group Meeting Today

It is dawn. Pre-dawn. Mom has a doctor appointment at 8:00AM so I will be on the road by 6:00AM. It is going to be a long day. After her appointment, we are going to my ILD Support Group this afternoon, which is going to feature an update of lung disease research around the world. I will report back tomorrow.

Mom is coming back to our house for dinner of lamb stew I made yesterday. It is made with eggplant, which breaks down to make a sauce along with lots of vegetables. A perfect meal for a long, rainy day.

I am taking tomorrow off and just resting. And a hair cut.

The sheetrock was taped and textured yesterday and is now ready for paint. Hopefully, we can get my closet remade soon. My clothes are scattered around the house. Damn water heater!

Yesterday, I discovered that I took and extra prednisone pill on Sunday. It was a total of 40 mgs instead of 30 mgs. Oops. Explains the extreme manic day.

Off I go into the darkness and rain.