Tag Archives: Idiopathic Pulmonary Fibrosis

01Sep/15

Oxygen

This update is difficult to write, as we’d hoped this would still be a year or down the road. A few weeks ago Mom failed a six-minute walk test which officially qualified her for rescue oxygen. Unfortunately shortly thereafter her breathing became worse and she started needing the oxygen more frequently until she was using it all the time. She went to the emergency room last Tuesday and was admitted. I waited to post this because at first the doctors thought maybe she had a secondary infection and they would be able to ween her back off the oxygen. But now they seem to all agree that she just had a quick down turn.

So, when mom is released from the hospital in a few days they will not be weening her off. Her new normal is oxygen 24/7. As usual, she’s handling it with more grace than any of the rest of us.

Growing up Mom always told us that everything in life happens for a reason. It’s really hard to stand by that belief when your mom is dying. The other night, with her best friend sitting at the end of her hospital bed, she told us that she wasn’t going out without a fight and that she still had things to do in this world.

Earlier in the day an NP covering for her cardiologist came back to see mom again to tell her something because, “she felt compelled to.” The NP is also a medical researcher who primarily studies lung diseases like cancer and COPD. She’s currently working on some kind of magnet hydrotherapy. I don’t really understand how it works because I’m not a doctor or scientist, but it has something to do with repairing cell damage. They’re not quite to clinical trial stage yet, but it can’t hurt mom to try it and realistically, we don’t have anything left to lose.

The equipment for the treatment will cost around $1000.00. And, because Mom will now be on oxygen all the time, she needs an oxygen tubing reel that will be $300-400. It won’t completely wipe out her funds, but will only leave a few hundred dollars her account, while her medical bills and needs will only grow as her disease progresses.

08Apr/15

Pneumonia

If you’ve previously donated to the GoFundMe campaign then you probably already know Mom is back in the hospital. She’s had a pretty good stretch; it’s been over a year since her last hospitalization.

It’s hard to write about Mom’s illness without painting it with my own emotions. As a child of someone with a potentially terminal illness, one of my biggest fears has been Dad calling and me missing it because I was asleep, or my phone was on silent, or any of a thousand other reasons I could probably come up with. And that’s what happened this time. It sucks.

You never forget that she’s sick but it’s easy, when she’s been relatively stable, to let your guard down…for lack of a better way to phrase it. But, you can’t do that with autoimmune disease, and you can’t do that with IPF. I saw her Sunday on Easter and now she’s in the hospital with pneumonia; it happens that quickly.

Mom probably won’t be thrilled with the details I’m about to share, but I think she’ll forgive me in the interest of spreading awareness. IPF is a disease that receives very little coverage, and it seems like even friends and family members of people with IPF don’t always understand how horribly crippling this disease really is.

I know Mom would agree that anything we can do to raise awareness is worth it.

So here goes: Mom passed out last night. After arguing with Dad (because she’s hard-headed; it runs in the family) because she never wants to go to the ER, she was packing a bag to take to the hospital. After a few minutes she still hadn’t come out of the bathroom. When Dad knocked she didn’t answer him. He found her passed out leaning over the sink, likely because she wasn’t getting enough oxygen.

She was admitted to the hospital from the ER with pneumonia. I don’t have much of an update as far as her current condition, but I’ll post updates here or on her GoFundMe as I have them.

 

29Oct/14

Mom’s Story: No More Cliff Diving, Part Four

Note: While I am posting this on Mom’s behalf, she wrote it. No one can tell her story as well as she can.

The future for me is to hopefully get on one of the only drugs that has shown to slow the progression of this disease. It has been used in other countries for several years, but is still not approved here. The FDA has said it will be, but they have said that for two years.** It’s only being used here in studies for “compassionate use”. If you go to Canada, it is several thousand dollars a month. There are other ways to get the medication that fall into sort of a gray area for the FDA, but that’s all I’m going to say about it. I’m willing to do anything I can to slow the progression of my disease.There are also some supplements many doctors and clinics suggest may help. I am using three of them which my insurance does not cover.

There is so much more I could talk about, but now I want to say something about family and friends. NO ONE gets this far or continues on without both. My daughter set up the GoFundMe site for donations. Friends and family have been so generous and I thank you all!

 

I have to thank my husband of forty years for always having my back and this long journey has been no different. He is still impatient with hospitals and sitting around as most men seem to be, but I can’t fault him for that one bit. He really is my friend, the love of my life and super dependable. Everyone who meets him loves him and he is a fantastic father. I think sometimes my family would keep him over me. Thanks honey for a wonderful life so far and I know you think the words to a Cat Stevens song was written for us, “I’m lookin’ for a hard headed woman, one who’ll make me do my best….”  Well, you know the rest.

My fantastic adult kids and their spouses, you have always made me proud and never let me down. You are kind and generous and loving and smart and resilient. The moments you hate the way the world works is almost always because an underdog is being treated so unfairly, or because stupidity has reared its ugly head again. So since song lyrics keep appearing I will just say, “You are my sunshine, my only sunshine…” keep singing you know it goes.

Mom and dad, you are the best parents on the planet. Words will never express how I feel. Mom, I am doing that thing people do where you take two fingers to my eyes the point to your eyes. I call it the “Eye Connection” and you know what I mean. We do not need words. We just get each other, even if it hurts sometimes.

Sisters, I am stuck in the middle with you… isn’t that a song too? When it counts the most we lock arms and nobody gets by us. I don’t need to say anymore because sister language is only understood by sisters and we always have something to say.

Friends and what I call my other kids, this is for you. I thank you all for so many memories and all the ones still to be. I have made it a point in my life to befriend only kind people and hope only kind people would befriend me. I like that we are different, yet the same and that what I just said you understand. We are all just a bit crazy.

Hey BFF (and you know who you are) fact of life: After Monday and Tuesday even the calendar says WTF, and we have said that a lot.

One final thought, as I carry on with an uncertain future, knowing this disease will probably be my fate since now I can no longer cliff dive. Winston Churchill once said, “I am ready to meet my maker. Whether my maker is prepared for the ordeal of meeting me is another matter.”

 

**Esbriet/Pirfenidone and another drug for the treatment of Pulmonary Fibrosis were approved by the FDA on October 15, 2014 after this was written but prior to it being published.

20Oct/14

The Cost of Dying

I had planned to publish the final installation of No More Cliff Diving (part two, part three) last week, but in light of the recent announcement of the FDA’s approval of Esbriet and Ofev I wanted to devote an entry to that. And, I needed a few days to stew contemplate.

On October 15, 2014 the FDA announced it was approving the use of Esbriet/ pirfenidone and Ofev/ nintedanib for the treatment of Idiopathic Pulmonary Fibrosis in the United States. Don’t get me wrong, I applaud the FDA for finally approving not one, but two drugs for the treatment of this devastating and terminal illness. However, I can’t help but think about all the patients for whom this news is too little too late. About all the patients that died waiting for something like pirfenidone in the U.S.

While I publicly retweeted the news from Pulmonary Fibrosis Foundation’s twitter feed, privately I scoffed at their generous use of “prompt” in relation to how long the approval took.

According to a press release on Drugs.com, Intermune first submitted a New Drug Application to the FDA on November 4, 2009. I completely understand the need for due diligence, particularly in respect to prescription drugs, but pirfenindone is not a new drug.

It has been in use since 2008 in Japan under the trade name Pirespa, and in India as Pirfenex, since 2010.  I could go on.

According to the Coalition for Pulmonary Fibrosis, approximately 40,000 people die from pulmonary fibrosis every year. So… that’s about 160,000 people dead in the four complete years since the initial drug application to the FDA. Sure, I’m biased; I’m emotionally invested. But I doubt the families of those 160,000 people (remember I’m not counting 2009 or 2014 so the estimate is on the low side)  think the FDA’s approval was all that prompt either.

An article in the NY Times published the same day as the approval stated Esbriet would cost roughly $94,000 a year.

“Roche said the wholesale price of Esbriet would be about $7,800 a month, or about $94,000 a year — two to three times what the drug sells for in Canada and Europe.”

And that’s down from one account I read (I’ll link to it, if I can find it again) that estimated the cost to be closer to $100,000. Either way the exorbitant cost will likely prevent patients, many of whom have been disabled by this disease, from receiving the drugs that can slow the progression of their disease.

The Pulmonary Fibrosis Foundation has alluded to a possible subsidy in some of their tweets, but I haven’t been able to find any concrete information on it.

08Oct/14

Mom’s Story: No More Cliff Diving, Part Three

Note: While I am posting this on Mom’s behalf, she wrote it. No one can tell her story as well as she can.

Read part one or part two.

My next step was to file for disability. I had to acquire my gimongous (yep, a word combo, like “framily”) stack of records. The only good thing to come from this diagnosis: after my 6 month wait for benefits, they automatically gave me my disability.

I didn’t experience any of the nightmare stories of being denied and having to get an attorney. The reason is this disease is on their fast track list, meaning you are terminal and might die before you get benefits if they proceeded as usual. I had my first job at 14 and have worked all my 56 years and paid into the system, with the exception of several years when I stayed home to raise our children. Of course when I did work I made below average wages due to education and just being female (which is another issue entirely). It was only in the last 12 years where my income has been commensurate with my experience.

My disability check is less than 1/3 of what I was making. Needless to say when you get ill, the bills only increase. We still have the same ones we had before. Then add medical deductibles and things insurance doesn’t cover; you need more money not less.

In December of 2013 I went for the lung biopsy and final confirmation. I knew they would have to collapse my lung and put me on a respirator. A lot could go wrong and I was very scared. We all were. I did well, but the pain was horrible and I was in intensive care for several days. The news was not good and I had the feeling it wouldn’t be.

In February, Don and I drove to Vanderbilt in Nashville to see a doctor about a future lung transplant.  I had been having sharp stomach pains on and off for two weeks. I thought I had a kidney stone again and I was not about to let the appointment slip away as we felt lucky to get in so quickly. The night before the appointment, I was in so much pain I was afraid I could not do the 6 minute walk test as part of the exam.

The doctor at Vanderbilt was very pleased with my condition and gave me a check list of things I must do. I was never so ready to go home after walking all day in pain. Then, the doctor said so matter of factly, “Oh by the way, the doctor who read your lung scan saw a dark area in your upper stomach. He is not sure what it is and wants you to go to the emergency room.” I knew nothing is ever simple with me and decided to go home. I did not want to be in a hospital four hours away if something was seriously wrong.

The next day my primary care physician sent me for scans and it was determined I had a very large cyst on my colon. I needed emergency surgery to remove the infected cyst. My colon was very inflamed. I needed a colon resection, a surgery that could take five or six hours, which was a concern because of the condition of my lungs. They decided to put a drain in first to remove infection. I was in the hospital for six days. Then, I was at home with a drain for two weeks. I had the surgery after that. It was so painful and took weeks to feel better. I had to nurse an incision with 22 staples and even now, more than 5 months later, my stomach is tender. But my lungs held up great!

 

01Oct/14

Mom’s Story: No More Cliff Diving, Part Two

Note: While I am posting this on Mom’s behalf, she wrote it. No one can tell her story as well as she can.

You can read part one here.

I continued working but was diagnosed with neuropathy, and then started having kidney problems. It was on a CAT scan of my kidney the doctor happened to see the bottom part of my right lung. I was told I needed to have it looked at. I did and was told I had some fluid in my lung. A blood test showed an elevated white blood cell count. The doctor gave me an antibiotic, and that was that.

Finally, with all my other symptoms the pulmonary doctors decided to do a bronchoscopy in May 2013. I was told the results showed inflammation in the lungs and scarring. Many months later when I had to acquire medical records for disability I saw the pathology report and it said findings of IPF (Idiopathic Pulmonary Fibrosis). I was shocked because the doctor NEVER said IPF. I was still having problems and noticed over the next several weeks I was winded when climbing stairs. I was now having second thoughts about this doctor and his lackadaisical attitude towards my symptoms. Keep in mind, I still did not know it was IPF! He continued to treat me with prednisone.

In July, I ended up in the hospital due to a reaction to an immunosuppressant the pulmonary doctor started me on for inflammatory problems. While there I saw another doctor from the practice. He was filling in for my regular doctor and I really liked him. He showed great concern for my long history of illness. I again returned home only to be back in the emergency room about ten days later. I thought I was having a heart attack because I could not get a deep breath and my chest felt heavy. I went down to the basement at work and could not climb the stairs back up. I finally made it and called the doctor.

I was a mess! It wasn’t a heart attack, but my heart was enlarged from extremely low potassium and hypertension from low oxygen and muscular atrophy. All of this was attributed to the high levels of prednisone I was taking to keep the inflammation at bay. Like I said, I was a mess! Oh and did I say I felt like shit, almost to the Dr. Kevorkian stage. This stay in the hospital was the first time I heard the scarring in my lungs referred to as fibrosis, and that it was not good, but no one really stressed how awful the diagnosis was. A lung biopsy was likely in my future, but I needed to get back on track first and get stronger because it would be a serious and risky procedure.

Again I went back to work for two weeks and then went on the yearly family vacation to the beach. I figured I could be sick at the beach as well as at home. I was so sick my parents, who are in their 70’s, were helping me up stairs. I knew my working days were short lived and it only added to my grief.

I went back to work and within two weeks it was a mutual decision with my wonderful employers I was doing myself no good. To this day, a year ago now, it is the one thing that brings me to tears when I talk about it. They gave me a wonderful severance package which took some immediate worries off of my husband and I. I can never say enough about their compassion and support. A call from them cheers me up on my worst days.

Read part three.

20Sep/14

Mom’s Story: No More Cliff Diving, Part One

Note: While I am posting this on Mom’s behalf, she wrote it. No one can tell her story as well as she can.

For many years I told my doctors they were missing something. Two years ago I started to really spiral down,  then a year ago my world turned upside down.

When a doctor is explaining your test results to you and concludes with, “You would have been better off with a diagnosis of lung cancer,” you know you’re in big trouble. I was told I have a terminal illness called Idiopathic Pulmonary Fibrosis (IPF). I had never heard of it until I was diagnosed. There is no cure and most indications on average, are diagnosis to death in 3-5 years. The Coalition for Pulmonary Fibrosis estimates there are 128,000 people living with PF in the United States, with 48,000 new cases diagnosed every year.

Pulmonary Fibrosis is part of a collection of diseases and disorders referred to as Interstitial Lung Disease. My diagnosis is more specific as it is Usual Interstitial Pneumonia (UIP), still it falls under the IPF family of horror! It is more than likely my UIP/IPF is caused by two issues but still not conclusive. Thus, the “I” in IPF, because Idiopathic means “still unknown”. The known part is years of inflammation in my body, and the unknown might be the years of environmental workplace exposure to cleaning chemicals.

Since the early nineties I’ve suffered from periods of severe joint pain and extreme fatigue. I was working long hours as a housekeeper in the hotel industry and was frequently exposed to chemicals. My kids were teenagers at the time and I blew off the fatigue to that. Then, I started getting unusual rashes and ended up in the emergency room. The doctors said I had a high positive ANA with elevated inflammatory factors. WHAT? It meant my body was producing antibodies that would normally protect me, but in this case they were attacking my body. This was the beginning of my relationship with rheumatologists and their fix-all steroid dose packs. I was told I had Rheumatoid Arthritis and a non-specific autoimmune disease. The steroids worked temporarily but within a few months, I would flair up again and be right back where I started.

Years later, I was on to the fifth rheumatologist and the same old thing. Test after test, but because I didn’t show all the “markers” for a specific illness they were not aggressive in treating me.

I was having stomach problems, degenerative disk disease in my back, and vision problems. It was an ophthalmologist who said they had never seen such a severe case of dry eyes. I had hardly any tear film at all, and based on my history I was diagnosed with an autoimmune disease called Sjogren’s Syndrome. It creates inflammation, which attacks your body and wreaks havoc. Two of the most common symptoms are dry mouth and dry eyes.

Looking back, I truly believe if all those doctors had not been so “by the book” and listened to me, they might have been more aggressive in keeping the inflammation away with an immunosuppressant instead of harmful and temporary steroids. Maybe my lungs would be in better condition, who knows? I can’t help but wonder if it would have changed anything.

In February 2012, I was back at the rheumatologist and it was the same old thing. I left the office and went back to work not feeling well. That night I was in the emergency room with a severe case of pneumonia. I was in the hospital for 5 days.

This was how it was day in and day out, I was in so much pain and the fatigue was horrible. I had also developed an ongoing dry cough. It was so bad I was going through bags and bags of cough drops, and cough syrup at work. At the time, I had been at a job I loved for over six years, working as a household manager at a private estate. I never missed work before, but was starting to miss days due to illness. One morning I was so bad my husband, Don, had to drive me to a pain management doctor.

Read part two here.

01Sep/14

September is Global Pulmonary Fibrosis Awareness Month

From the Global PF Awareness website:

 

a snapshot of the disease
• In the United States (US), IPF affects between 132,000 and 200,000 people.2, 4, 11
• In the US, an estimated 50,000 new cases are diagnosed each year.2, 4, 11
• In the European Union, IPF affects between 37,000 and 40,000 people.5, 17
• In the United Kingdom, more than 5,000 new cases of IPF are diagnosed each year.3
• PF can strike anyone, but tends to affect men more than women and usually occurs between the ages of 50 and 70.4, 6

 

In addition, according to the data on Global PF Awareness, the estimated survival rate of patients is 2-3 years after diagnosis. Please help spread the word about PF, and check their calendar to see if an event is happening near you.

29Aug/14

Pulmonary Fibrosis Symposium

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Date
September 5-6, 2014

Location
The Martin Center
960 Heritage Way
Brentwood, Tennessee 37027

Time
8:30 a.m.-3:30 p.m. each day

 

Friday, September 5, 2014

8:00                Registration Opens / Coffee / Welcome

8:30                Pulmonary Fibrosis, presented by Dr. Lisa Lancaster

9:30                Connective Tissue and Pulmonary Fibrosis, presented by Dr. Leslie Crofford

15 min break

10:45               Genetics and Pulmonary Fibrosis, presented by Dr. Jonathon Kropski and Cheryl Markin

11:45                Palliative Care, presented by Sara Martin

12:15-1pm       Lunch

1pm                 Lung Transplantation, presented by Dr. Mark Steele

2pm                 Personal Experiences by Transplant Recipient, Sally Clinard

3pm                 Dismiss Group

 

Saturday, September 6, 2014

8:00                Coffee / Welcome

8:30                Douglas Herr, Patient group discussion & Michele Peters, PFF Ambassador, Caregiver group discussion

9:30                Pulmonary Rehabilitation, presented by Jeremy McNatt

15 min break

10:45               Research Review, presented by Wendi Mason, NP

11:45                PFF Ambassadors, Jeffery & Michele Peters, share their stories

12:15-1pm       Lunch

1pm                 Pulmonary Function Testing, presented by Dr. James Sheller and Phyllis Leonard, RRT

2pm                 Oxygen Therapy, presented by Sarah Roberts from Apria and Sandi Brim from Medical Necessities

 

3pm                 Dismiss Group

Click here to register.

08Aug/14

Help Researchers Learn More About PF!

The Pulmonary Fibrosis Foundation is looking for patients and caregivers to participate in a survey.

Pulmonary fibrosis drug research and development is at a defining moment with two new therapies having achieved FDA breakthrough therapy designation. If you are living with PF, or if you are a caregiver, your voice through this survey will reach decision makers in Washington, DC and will inform health care professionals around the globe.”

You can participate by taking the survey here.