Living Well with a Bad Diagnosis - Lung Disease

Saturday, April 30, 2011

Hotel Rooms for the Disabled

As we didn’t have a lot of time to prepare for this trip, I called Hampton Inns and made almost all the reservations at one time. It was easier. The only problem was in my brother’s town. We had to stay at a Best Western. To our surprise, we were given a room for the disabled. The disability parking was right out front within feet of the room, the bathroom was huge to accommodate a wheelchair, the room was spacious and we loved the location – not up an elevator and down a long hallway.

It never occurred to me to ask for a hotel room for the disabled. In Oklahoma City, we were talking about it with the Hampton Inn desk clerk. She changed my profile and requested a disability room or a room on the first floor so we would not have to haul all of the luggage so far. It kicked in a few hotels later and really made a difference.

A new lesson learned this trip – one can request hotel rooms for the disabled.

Friday, April 29, 2011

Portable Oxygen System (POS)

I loved the portable oxygen system we used this trip. It was the Eclipse on it’s own rolling case. It was continues flow, which is what I must have for my disease. I needed it only at night but really should have used it walking in high altitude. A big lesson learned.

The last trip we took, the POS stopped working about half way through our trip. I had not been told that these are very sensitive machines, which must been protected from the sun and heat in the car. This trip, we kept a constant cover on it in the back seat to keep it out of direct sunlight and we also made sure we kept it cool. We had no problems this trip.

Every stop, we rolled it into the hotel room and placed it beside the bed. At night, I turned it on, it would get warmed up then begin to produce 2.5 liters of oxygen. It also was not so loud as to disturb our sleep.

I will returned it to the oxygen people this week along with a bottle of good bourbon from the Jim Beam Distillery in Kentucky as a thank you.  

Thursday, April 28, 2011

Liquid Diet Begins Today

I visited my nutritionist Mr. B on Wednesday morning. I weighed in and lost just 5 pounds. I was disappointed. I felt I had lost more….The new challenge will begin today.

For the next three months, I will eat nothing other than protein shakes and bars. I am now close to the 30 BMI needed to begin the lung transplant qualifying process. I don’t want my weight to become an issue throughout the process, so I wanted to be well under the stated maximum BMI.

The goal – Forty pounds in three months.

The process – Four 200-calorie “meals.” The morning with start with a meal supplement shake. Next will be another type of shake or pudding followed by one of the following: potato soup, chicken soup, or oats. The final meal of the day is a protein bar.

I must also drink 12 cups of water a day.

The downside:  I will be cold, have bad breath, very dry skin, lose lots of hair, have constipation, brittle nails, muscle cramps, and will feel tired, weakness and maybe some nausea. Sounds like a lot of fun, doesn’t it?

But, I will be finished. I will be ready to begin the qualifying process. That was my goal this year.

Well, maybe I am not all the way done. I think I want to lose another 10 pounds after the fast. We’ll see if the doctor will let me go for it. I will be very close to the minimum BMI of 25 to begin the lung transplant process. Ha! 

Wednesday, April 27, 2011

Vacation, Diet and Confessions

I think others were very worried about me sticking to my diet on the road. I felt I had it nailed. In the end, I did really well. We found great restaurants, I ordered what I wanted or didn’t want, we split most dinners, I ate ribs without any sauce (they were all great!), we had some sort of eggs for breakfast every morning, I watched Michael eat all the things I couldn’t eat and I was okay with it.

Did I take a bite of forbidden food? Yes, I did. I think it was six times during the three weeks and it was only a bite. Not a helping. A bite.

We ate at Paula Dean’s place in Savannah, Georgia. We had to try her Fried Green Tomatoes (Sin #1) so I had two with a small dot of red pepper sauce on top along with julienne sliced cucumbers and onions. Delicious. Michael had the crab stew that was primarily a vessel for butter and cream. He also had some amazing fried cornmeal pancakes and garlic and cheese biscuits along with a crab cake burger. Oh my. I had the grilled shrimp and cole slaw and gave Michael my potatoes, which were simply double-fried chunks of heaven. Wonderful. It was just fun to be there.

We also had a great experience at Gladys Knight’s Chicken and Waffles in Atlanta. We loved that it was opened until 4:00AM. Can you image what a wild place that must be after everyone swings by for food after the bars close? We also noticed a sign on the window that said, “No Weapons Allowed.” Wow. Michael ordered the Midnight Train, which was the famous four huge fried chicken wings and a perfect waffle with maple syrup. I had a forkful (Sin #2). Just a taste. It was amazing. Savory and sweet. I ate a huge baked chicken breast with vegetables. I couldn’t finish it.

Kimberly had told us that her most favorite restaurant in the entire country was called Porters Beer Bar also in Atlanta. Okay. Off we went. Many miles later, it was in a section of Atlanta that reminded us of Haight Ashbury in San Francisco or Soho in New York. There was also a concert happening so it was a bit crowded with people and cops. It was a bit of a shady neighborhood. As we did not know about Porter’s, we had dressed well for the occasion. Well, we stuck out like sore thumbs.

It turned out to be an amazing place. We had the chicken and waffles earlier so we weren’t hungry for a huge dinner. We arrive to the tiny bar and Michael spotted some 15-year-old Pappy Van Winkle bourbon from the Buffalo Trace distillery we had just visited in Kentucky amongst one of the largest selection of bourbon he had seen in one place. They also offered more than 400 beers. We settled in, chatted with the bartender and others at the bar. The menu was outstanding. There were two offerings of pork belly and one of beef heart, all very sophisticated offerings for a small place in not such a good area. We ordered the fois gras terrine appetizer. It arrived piled high with tasty things including slices of crispy garlic chips. I took a bite of it on a piece of cocktail bread (Sin #3). It was rich with deep flavors all working together.

After a guy we had been talking with left, two women joined us and began to chat. They insisted that we taste of their Salt and Vinegar Popcorn (Sin #4) made with herb and garlic oil. It took our breath away. We had such a good time talking with everyone including the owner that the bartender bought us a dessert of Passion Fruit Crème Brulee. I had a spoonful (Sin #5). It was the best I have ever had.

William’s concert tour was following a week behind us. After texting him about Porters, he made his way there and had their bone marrow and shrimp and grits. He also loved the place.

And my last sin was during our search for pralines in Savannah. We found them. Oh my. We watched them being cooked and they offered us one. How could I not take a bite? At least we split one! (Sin #6) We bought three pounds and have been giving them away as gifts.

So, today we will see how well I did during our three week road trip. I see Mr. B., the nutritionist, to get weighed and to begin the university’s three-month liquid diet and protein bars tomorrow. More on that to come after I meet with him.

Tuesday, April 26, 2011

Vitamin D and Interstitial Lung Disease

In their recent new packet, the Coalition for Pulmonary Fibrosis quoted an article from Better Health Research News Desk. It stated that the researchers at the University of Cincinnati College of Medicine have found that vitamin D deficiency plays a role in the severity of ILDs.

They realized that people with ILDs “tend to have lower levels of vitamin D, which may contribute to the disorder.” They went on to state that the “study authors suggested that vitamin D supplementation may improve lung function, though they added that further research is needed to determine this.”

It was further stated that the Mayo Clinic recommends that adults should consume approximately 200 micrograms of vitamin D per day.

So, dear reader, if you have an ILD, ask your doctor to check your vitamin D levels. I had very low levels -19 - and have been taking 50,000 IU of supplements a week for several years. It is measured with a simple blood test that must be checked once a year. Simple. It may help.

Monday, April 25, 2011

Humidity, Altitude and Lung Disease

Photo of beautiful Charleston, South Carolina

I now understand why Dr. K. told me to not move from in our little coastside town. No altitude, no humidity and a Mediterranean climate, which are all perfect for a person with an Interstitial Lung Disease. This point was driven home many times during our almost 7000 mile road trip.

In Charleston, South Carolina, I really struggled with the heat and humidity. I noticed my breathing was loud and I really couldn’t move as easily or quickly as usual. In Golden, Colorado, it was nearly impossible to even walk a short distance to the restaurant for dinner. I was very short of breath in the mile-high city.

We walked the beautiful gardens at Duke University in Durham, North Carolina and I found myself having to stop often to catch my breath. It was hot, humid and hilly. I was very disappointed with their lack of trails for the disabled and they even charged for parking even with a disabled sticker on our car. Not a disabled friendly place.

We definitely noticed when we were in Flagstaff, Arizona or with my brother and his wife in New Mexico or Las Vegas or southern Utah, that the altitude really affected me much more this trip than even two years ago. I was surprised. I was disappointed.

This morning, I will return to my beloved rehab and work out for the first time in 24 days. We did walk a lot during our adventure and I did some heavy yard work on Saturday but it is just not the same as sweating in a gym. It will feel good to get back to some routines.

Sunday, April 24, 2011

Research Breakthrough for IPF

5-μm lung sections were cut from lung biopsies obtained from a patient with IPF. The section reveals marked inflammatory foci within the parenchyma (arrowheads), surrounded by dense collagen deposits (arrows)

As promised, here is the information that I received from the Coalition for Pulmonary Fibrosis regarding the guidelines about treatment decisions for people diagnosed with Idiopathic Pulmonary Fibrosis (IPF).

Also below is the article from the New England Journal of Medicine regarding the genetic connection of IPF. It is a beginning to understanding the cause of the disease and possible treatment in the future.

1. Here is a copy of an e-mail I received from the wonderful people at CPF:
The Coalition for Pulmonary Fibrosis (CPF) would like to inform you about the first evidence-based guidelines on PF that were recently published. These guidelines will help your physician(s) help you and will provide the guidance they need to make recommendations for treatment decisions with you.  Also, the CPF worked with the American Thoracic Society to create a new patient information sheet on PF (linked below), as well. 
The guide and information sheet can assist you in understanding the options available to you as well as help you make the most of your visits with your doctor by being as fully informed as possible. 
Please share the links below with your doctor and consider printing out a copy of the guidelines and a copy of the patient information sheet to provide to your doctor at your next visit. 
PF Guidelines: 
Please see the CPF's full press release regarding the guidelines below.
Mishka Michon, CPF CEO 
Pulmonary Fibrosis Applauds First Evidence-based IPF Clinical Guidelines
Guidelines Update 11-Year-Old Consensus Statement for Deadly Lung Disease
The Coalition for Pulmonary Fibrosis (CPF) applauds the joint statement issued by the American Thoracic Society for the first evidence-based clinical guidelines for the treatment of idiopathic pulmonary fibrosis (IPF).  The guidelines represent progress in understanding of the disease and a milestone for patients.  The devastating and deadly disease also called simply “Pulmonary Fibrosis” or “PF” has no FDA approved treatment and no cure.
The new guidelines replace the ATS consensus statement on PF issued in 2000 and reflect the progress in the disease that has changed medical understanding of the complex disease.
“We are pleased to see the new guidelines and hope it will not only help physicians to provide better care to PF patients, but will help them to empower their patients and family members to take an active role in their treatment decisions,” said Mishka Michon, CEO of the Coalition for Pulmonary Fibrosis.

The statement reviews current knowledge in the epidemiology, etiology, diagnosis and management of IPF, as well as available treatment options, including pharmacologic and non-pharmacologic therapies and palliative care.  It appears in the March 15, 2011, American Journal of Respiratory and Critical Care Medicine.

 “In the decade since the publication of the previous statement on IPF, studies have used the  criteria for the diagnosis of IPF and recommendations published in the previous consensus-based statement to further our understanding of the clinical manifestations and course of IPF, and there has been an increasing body of evidence pertinent to its clinical management,” said Ganesh Raghu, MD, director of the Interstitial Lung Disease/Sarcoid/Pulmonary Fibrosis Program at the University of Washington Medical Center in Seattle and chair of the collaborative committee that drafted the statement. “The accumulated data and observations made in these studies are substantial and have allowed us to provide new  guidelines for the diagnosis and management of IPF to the global pulmonary community-at-large to the patients and to all concerned based on evidence  for the very first time.”

The new guidelines represent a collaborative effort between the ATS, the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS) and the Latin American Thoracic Association (ALAT).

The recommendations in the guidelines are ranked from weak to strong to help the clinician understand the strength of the recommendations made in managing patients with IPF.

“For example, in the case of a weak recommendation clinicians are especially required to spend adequate time with patients to discuss patients’ values and preferences. This may lead a significant proportion of patients to choose an alternative approach,” Dr. Raghu, who also serves on the CPF’s scientific advisory board, said.

To assist clinicians in their interactions with IPF patients and their families, the ATS has developed a companion patient information series piece on IPF that is also published in the March 15 journal. This education piece is also posted on the ATS Web site (, as well as the Web site of the Coalition for Pulmonary Fibrosis (, an ATS Public Advisory Roundtable member organization, whose focus is on research, advocacy, support and education for the pulmonary fibrosis community.

About Pulmonary Fibrosis (PF)
Pulmonary Fibrosis (PF) is a lung disorder characterized by a progressive scarring – known as fibrosis -- and deterioration of the lungs, which slowly robs its victims of their ability to breathe. Approximately 128,000 Americans suffer from PF, and there is currently no known cause or cure. An estimated 48,000 new cases are diagnosed each year. PF is difficult to diagnose and an estimated two-thirds of patients die within five years of diagnosis.  Sometimes PF can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis (IPF). 
About the CPF
The CPF is a 501©(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis (PF), while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease. The CPF funds promising research into new approaches to treat and cure PF; provides patients and families with comprehensive education materials, resources, and hope; serves as a voice for national advocacy of PF issues; and works to improve awareness of PF in the medical community as well as the general public. The CPF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 23,000 members nationwide, the CPF is the largest nonprofit organization in the U.S. dedicated to advocating for those with PF. For more information please visit or call (888) 222-8541.
 2. Here is the article from the New England Journal of Medicine regarding the genetic connection of IPF:
Genetic Discovery Published in New England Journal of Medicine Good News/Bad News for Patients, Families with Pulmonary Fibrosis
Genetic Variant Associated with MUC5B Gene ID’s Major Risk Factor, Points Research in New Direction 
A new discovery in a deadly lung disease may change the direction of research while uncovering increased risk for many patients and families.  The Coalition for Pulmonary Fibrosis (CPF) and the Pulmonary Fibrosis Foundation (PFF) applaud the efforts of scientists that led to the discovery of a genetic variation associated with the MUC5B gene which may increase the risk of developing Pulmonary Fibrosis (PF).   The two patient organizations partner with National Jewish Health (NJH), which led the team of researchers in the study, on a genetic counseling line that provides medical information and support to patients and families. 
Results of the MUC5B study are published in the April 21, 2011 issue of the New England Journal of Medicine.  
Pulmonary Fibrosis (PF) is a devastating lung disease with no FDA approved treatment and no cure and scientists have worked for many years with more questions than answers in the quest to understand its cause. 
Researchers found in study patients with the idiopathic form of the disease (idiopathic pulmonary fibrosis or IPF), the MUC5B genetic variant was present in 67 percent of patients, and in 59 percent of patients with a known familial origin to the disease (Familial Pulmonary Fibrosis or FPF) as well as in 19 percent of healthy controls. 
The study findings regarding MUC5B and its role may alter the course of research into PF by focusing attention to mucus production in the lungs as well as the space around the air sacs in the lungs.  
Scientists also found that carrying one copy of the gene increases risk for pulmonary fibrosis 6-9 times and carrying two copies of the variation increases risk by 20-22 times. 
“We are excited about these important new findings as they will guide future investigations in new directions and more precisely identify patients at risk for developing this devastating disease” said Gregory Cosgrove, MD, Director of the Interstitial Lung Disease Program at National Jewish Health.
 “Members of families like mine with Familial Pulmonary Fibrosis know we’re at risk for the disease.    
This news demonstrates that our relative risk is dramatically higher,” said Deirdre Roney, a member of the CPF board of directors who has lost eight members of her family to PF.  “On the other hand, I am excited at learning the results of this important new finding in the genetics of Pulmonary Fibrosis.   When you see a whole generation of your family perish from a disease, as I have, and you see that future facing all of your relatives, you desperately hope that discoveries around genetics will be found.  Once found, the next hope is that gene therapy will be the family’s salvation.  
My family is deeply grateful to the scientists whose commitment has led to the success of the MUC5B research.” 
The MUC5B gene is an important finding for all patients with PF, not just people at risk for the genetic version of the disease.  The CPF ( and the PFF ( partner with National Jewish Health to provide a toll-free PF counseling line.  To find out more and to ask questions, please contact the National Jewish Health Genetic Counseling Line at 1-800-423-8891, ext. 1097. 
“This is a significant genetic finding in pulmonary fibrosis, one that may be used in the future as a tool to identify individuals at risk, used for earlier detection and more predictable prognosis.  This finding increases the genetic knowledge for pulmonary fibrosis that physicians and researchers can utilize to develop new tests and target for therapies” said Janet Talbert, MS, CGC Director or the Familial Pulmonary Fibrosis Genetic Counseling Program at National Jewish Health.

Saturday, April 23, 2011

We are Home with Stories to Tell

Home. What a great word. What a great bed. What a great pile of mail....

After 21 days, 17 states and just under 7,000 miles, we have returned to our lives. I have so much to share but I promise not to do it as a travel log but from the point of view of being on the road dealing with a disease. Everything worked well. I did not get sick. But, we had adventures.

So, dear reader, let me get a bit settled and I will begin to share.

While we were gone, I received a thrilling e-mail regarding a huge breakthrough announced in the New England Journal of Medicine about the discovery of the possible cause of IPF - Idiopathic Pulmonary Fibrosis. I will reprint the article in a day or so.

I missed you all. Welcome back to my blog.

Friday, April 1, 2011

Saying Goodbye, For Now

Dear Reader, this is a goodbye for a few weeks. We are heading into the small towns and byways of this great and beautiful country of ours. We are always a little nervous about starting such a huge trip but hopefully we will avoid all bad weather, roadwork and traffic accidents.

If you miss me, now is your chance to start to read this blog from the beginning dated October 2009. There is such good information about processes and processing all the bad news within those blogs. Read a few a day.

So off we go traveling outside our comfort zone, looking for adventure, meeting new people and living well with a bad diagnosis.

Bon Voyage!