Living with Pulmonary Fibrosis

My husband Bill is in the late stages of pulmonary fibrosis, a lung disease with no known cure. This is our story of our journey through the disease and how it has affected one family's life.

Monday, October 31, 2005

Medicare is the Problem
I talked with Dr. D's nurse today about the lack of physical therapy and the need for oxygen or some help with Bill's breathing. She said that Medicare will not allow him to have oxygen until his resting blood oxygen level is 88%. She says she can do nothing to obtain oxygen for him at this point. We may be too far past the broken back to qualify for physical therapy, so we still seek answers.

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Thursday, October 27, 2005

October 27, 2005 - Today we were contacted by someone from the Duke University Medical Center. They are conducting a research study to identify a genetic link for pulmonary fibrosis. They are sending a kit that we must take to the doctor for a blood draw and we must release medical records to them. We must also contact family members and find out if they are willing to be tested. If we can help in finding a cure, we are more than happy to participate.

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Wednesday, October 26, 2005

October 26, 2005 - Bathing has become a challenge. Bill showers once a week now. He enters the shower, turns on the water and leans against the side of the shower. He rapidly soaps and rinses and it is over in a couple of minutes. Then he is out of breath for several minutes and has to sit down before he can dry off and begin dressing. It won't be long before he must take sponge baths.

About a month ago Bill cut his hair very short. It is easier to care for and looks much less messy. He doesn't think he can walk into the barber shop, so our son Jim is going to start cutting his hair for him. We gave Jim a barber kit several years ago and he clips his own hair, so Dad is next.

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Monday, October 24, 2005

My husband Bill is in the end stages of pulmonary fibrosis, a lung disease in which the air sacs slowly turn to fibrous tissue and lose the ability to take in oxygen, eventually causing death by suffocation. This is our story of our journey through the disease. We are telling it in the hope that others may learn from our experiences. There is encouragement in knowing that one is not alone in such a journey. By sharing experiences, we can support one another and perhaps lift some of the burdens of confusion and frustration that come with living with a terminal illness.

Our journey "officially" began in August 2000. Looking back, we can point to numerous instances that were related to the pulmonary fibrosis but were misdiagnosed or ignored by the medical community. One such instance occurred in March 2000 when Bill had what appeared to be a viral infection accompanied by chest pains and shortness of breath. The emergency room doctors diagnosed it as bacterial pneumonia. They could find no signs of pneumonia in his blood, but the cloudy lung x-ray led to the conclusion that he must have pneumonia. To their credit, he did improve after receiving intravenous antibiotics.

Doctors have been unwilling or unable to tell us what Bill's prognosis may be. All they can tell us is that he has outlived the expectations for someone with PF. Most people survive 3 to 5 years after diagnosis and they spend a portion of that time on oxygen. It is now 5 years since Bill's official diagnosis and he is still not on oxygen.

Bill is 90 years old, which in itself, doctors find remarkable. He is a highly decorated veteran of World War II where he served as a bomber pilot in the European theater. After the war he was assigned as a test pilot to work on the first jet aircraft. One of his doctors theorizes that prolonged exposure to pure oxygen may have caused the PF. Others believe that his woodworking hobby may be the culprit. For over 20 years he breathed fine sanding dust and the fumes from paints, lacquers, and stains, often without the benefit of a mask.

Bill's younger sister Pauline died of PF in July 1997 at the age of 77. Two of his younger brothers now have it. Clearly there is a family link, though many doctors are not yet willing to recognize that such a link may exist.

August 2000 - Bill took a friend to the airport. As they walked from the parking lot to the terminal with her suitcases, he suddenly was unable to breathe and he felt a tightness in his chest. After resting in the terminal, he drove home. Thinking that he was experiencing a problem with his heart, I immediately called his cardiologist who said to take him to the emergency room.

Thinking that the problem was a blockage of an artery or vein in the heart, the cardiologist ordered an angiogram. An angiogram is major surgery. It involves inserting a tube into an incision made in an artery in the groin. The tube is then fed up through the body to the heart as the process is filmed. After the procedure, the patient must lay flat without moving for 6 hours while the artery seals over (no stitches are used). The procedure is hard on the body and can possibly result in major bleeding. It is also very expensive. Our 6-hour hospital visit cost over $3,200.

Bill's angiogram showed not problem with his heart. The cardiologist sent him home with the recommendation that he see his family doctor. Bill had been telling the family doctor for years that he had a problem with his lungs, but the doctor dismissed his concerns. He would listen to Bill's lungs with a stethoscope and say that they sounded fine. When Bill mentioned the lung problem to his cardiologist, the cardiologist would say "Your heart is fine." No one was interested in pursuing a lung problem.

This time, Bill insisted on having a lung x-ray. When the results came back, the general practitioner was totally surprised to find that the lower half of Bill's lungs were black. He then recommended that we visit a pulmonologist and he sent the x-rays to one.

In a week or so we received a notice from the pulmonary clinic that the recommended doctor was not taking new patients and their first opening with another doctor would be in February 2001 - 5 months into the future. When I relayed the information to the general practitioner, he contacted another pulmonologist who was willing to see us in a couple of weeks.

We picked up the doctor's report and the x-rays to take to the new doctor. In looking at the radiologist's interpretation of the x-rays we learned that Bill probably had pulmonary fibrosis. We were not totally surprised by that since Bill's sister Pauline had died of pulmonary fibrosis 3 years earlier.

October 2000 - We visited Dr. O for the first time. We liked him immediately. He was an older man who seemed very thorough in his examination and in the questions that he asked. We spent 1 1/2 hours with him. He was not willing to immediately accept the diagnosis of pulmonary fibrosis without more information. He ordered a blood test, an echocardiogram, and a lung function test, all of which substantiated the fact that Bill did indeed have pulmonary fibrosis. Bill's resting blood oxygen level was 96, which is very good.

November 2000 - The recommended treatment for pulmonary fibrosis is Prednisone, a drug that has many side effects, including causing ulcers, bone loss, kidney problems, and cataracts. Prednisone is a cortisone-based anti-inflammatory. It is supposed to slow down the inflammation that causes the lung scarring associated with the disease. (For more information on pulmonary fibrosis, visit Before Bill could begin taking Prednisone, he had to first be checked for the presence of the H. Pylori bacteria, which causes ulcers, and have a bone density scan.

Bill tested positive for H. Pylori. For two weeks he had to take two pills a day at precise times. He also had to restrict his diet during that time. He then had another blood test that showed that the treatment had been effective. For most of his adult life Bill had been plagued with what he called "sour stomach" or heart burn. He has not had it since completing the treatment.

The bone scan showed that Bill already had the start of osteoporosis in his lower spine. Therefore, Fosimax was added to his list of drugs as was Guaifenesin, a drug that adds moisture to the mucus in the lungs and helps control the dry, hacking couch that accompanies PF. Suddenly Bill had gone from taking one drug, to lower cholesterol, to taking a handful of them. Thankfully, we have an excellent prescription drug plan through Bill's former employer.

February 2001 - Bill's life as a drug-dependent individual begins. He also starts seeing his ophthalmologist every 4 months to monitor for cataracts.

February 2003 - Bill has been relatively stable in his illness for two years. Dr. O, the pulmonologist, is ready to retire. He transfers Bill to an internal medicine doctor, Dr. M. Dr. M is nice, but he is not versed in the care of someone with a lung disease. Basically, for nearly two years, he does little monitoring of Bill's progress other than to write occasional refills for medications.

March 2004 - Our son Bill (not a junior), who lives in Wisconsin, asked his father to come spend spring break with him (he teaches at a college) and help him build a buffet for their dining room. Because of his shortness of breath, Bill was a bit concerned about making the flight by himself and particularly about changing planes in Minneapolis. He would have to traverse from one end of the terminal to the other, a distance of nearly a half mile, which would need to be accomplished in about 40 minutes. However, he made the trip with no problems and had a great time getting to know our then one-year old grandson Liam. Bill and Liam, whose birthdays are 4 days apart, celebrated their birthdays together in a St. Patrick's birthday party, which is Bill's actual birthday.

Unfortunately, the cold and dampness of Wisconsin caused Bill to get sick, but a trip to the walk-in clinic for antibiotics was all that was needed.

August 14, 2004 - Bill tipped over the riding lawn mower this morning and tore the rotator cuff in his right shoulder. That evening as he was moving a sprinkler in the back yard he fell, hitting his head on the concrete patio. A trip to the emergency room the following day revealed two broken ribs. This slowed him down for the remainder of the summer.

Up until this time, Bill had been using the riding lawn mower in the back yard (we have 1/3 acre) and mowing the smaller front yard with the self-propelled lawn mower, giving himself some much needed exercise. After his fall, he doesn't mow again. Our son Jim takes over.

December 2004 - We decide to go to Wisconsin for a post-Thanksgiving, early Christmas visit. Bill made the trip well and he was able to go out Christmas tree shopping. When we went shopping for Liam's Christmas present, Bill tried out the scooter in the store for the first time. He found it easy to use and he had fun checking out the huge store.

The week after we came home Bill had a cataract removed from one eye. Prednisone causes cataracts, so this was not a surprise.

January 2005 - The cardiologist is concerned that the PF is progressing and Bill's shortness of breath is not being monitored as closely as it should be. He sends Bill to another pulmonologist, Dr. D.

March 2005 - We see Dr. D. for the first time. He is associated with the clinic that wasn't taking new patients back in October 2001. He is young and personable, but seems to know little about pulmonary fibrosis. He is certain that Bill's breathing problems are caused by a heart valve that is slowly closing and not by the PF.

April 2005 - Back to the cardiologist to find out if the heart valve is the cause of the shortness of breath. The cardiologist assures us that it is normal in older patients and is not the cause of Bill's labored breathing. This back-and-forth issue of heart valve or PF continues even today.

July 5, 2005 - Today I am taking our 11-year old granddaughter Caitlyn to Wisconsin to visit Bill, Tricia, Liam, and new baby Cole. I was concerned about leaving Bill home by himself for 5 days, but he found a solution without my need to voice my concerns. He suggested that he go to visit his brother Denny, who lives near Eugene, Oregon. Denny will be 93 the end of August. He is a widower, having lost his wife to cancer 4 years ago. He owns a 40-acre farm, part in filbert trees and part in rye grass. He still puts in a full day of work.

Bill asked our daughter Marci and our 7-year old granddaughter Camille (Caitlyn's sister) to accompany him. This is the perfect solution. Not only does Camille get to take a trip, but Marci can keep an eye on Bill, and they will all get to visit with Denny. Plus, they went over to the coast for two days as an added bonus. They came home the same day as Caitlyn and I.

July 29, 2005 - Bill got up early to meet a friend for morning coffee. As he left the house, he stopped to turn on the sprinkler at the spigot on the front of the house. Somehow he fell, landing on his back in a flower bed. He picked himself up and went on to coffee. By the time he came home, he was in pain.

July 31, 2005 - After two days of pain, Bill decided that we needed to go to the emergency room. He was diagnosed with two broken vertebrae in the lumbar (lower back) area. He was given pain medication and told to wait 10 days then call a spine clinic if he wasn't better. He is in great pain. Bill never takes medicine unless he has to, and he can't get enough of the pain medication.

August 15, 2005 - We are finally able to see an orthopedic doctor at the spine clinic. He orders a brace to lift and separate the vertebrae and take the pressure off the injured area. The brace fits from Bill's collar bone to his pelvis. There is an oval metal tube in the front and the back. The sides are made of rigid plastic. It is all held together with 4 Velcro fasteners. Bill even has to sleep in it.

The emergency room doctor said that Bill was a candidate for vertebroplasty, a procedure in which a cement or "super glue" is injected into the broken area to fuse it together. However, this option was not offered by the spine doctor. Apparently, if a person's body is capable of producing new bone, which Bill's was, then vertebroplasty shouldn't be done.

In retrospect, if I were doing it again, I would not have waited to make an appointment with the spine doctor. Instead, I would have called the day after the emergency room visit.

September 2005 - Bill is out of the brace and walking slowly with a cane. We see Dr. D., who is concerned with his appearance. Bill has lost over 40 pounds since February. The brace restricted his ability to breath deeply and the injury prevented him from getting even limited exercise. He has clearly gone down hill very rapidly. Again Dr. D. mentions the heart valve as the primary reason for Bill's shortness of breath. He recommends physical therapy to try to restore some muscle function. Bill's resting blood oxygen level is 94, which is extremely good. When it decreases to 88, he will be placed on oxygen.

I think that Bill has had a bit of depression since his fall. He is beginning to feel sorry for himself and to make excuses for why he can't do the simplest thing. He has worn nothing but sweats since the visit to the emergency room 6 weeks ago. Bill has never been good at helping inside the house, but at least he would put his dishes in the dishwasher and try to clean up a bit, but no more. Now, if I am home, he expects to be waited on hand and foot. He sits in his chair holding out his glass waiting for me to see his pathetic expression. The fact that I have to go to work occasionally forces him to do something for himself.

The day after the doctor's visit, when our pastor, who just returned from a 3-month sabbatical, called to ask about having lunch, Bill actually offered to pick him up! He put on clothes and drove for the first time in six weeks. This seemed to be the catalyst that he needed to again show some interest in life.

Bill has been back to church only once since his fall. For over a year he has been concerned about having a coughing spell during the service and having to leave. When he did have a spell, one of the church members, who is a chiropractor, often followed him out and I would find the two of them sitting in the hall discussing airplanes. Jim was a great encouragement to Bill and because he was with Bill I didn't feel the need to leave early.

Late September - A physical therapist referred by Dr. D. called and then came by. She said that Bill didn't need her; he needed lung therapy. In addition, because he is able to drive and not totally house-bound, Medicare will not pay for his treatments. So far, that is the only time we have seen or heard from a therapist.

October 5 - 8, 2005 - Bill's brother and wife from Dallas plan to meet us in Jackson Hole, Wyoming for a few days. They are at their son's in eastern Wyoming. Though I question the wisdom of the trip, Bill insists that he really wants to see his brother for what could be the last time.

The day before we are to leave, the sister-in-law Billye (do you see a family trend in names here?), called to say that it is supposed to snow and they are afraid to drive through the Rocky Mountains, so they canceled. They are going home to Dallas instead. Bill decides that we are going anyway. We are taking our 11-year old granddaughter, Caitlyn and we had promised to spend a night with my sister in southern Montana.

Bill did most of the driving and he did well. We borrowed a wheel chair from Caitlyn's other grandmother. Caitlyn and I walked and wandered in Yellowstone and Grand Teton National Parks while Bill rested in the car. In Jackson Hole we put Bill in the wheel chair and pushed him around town. Everywhere we went, the people were great. They helped us unload and reload the chair into the car. They offered help wherever we went.

The last evening Bill started feeling the effects of the altitude and had some difficulty sleeping. On the trip home Bill rested in the car while Caitlyn and I hiked in the rain at Craters of the Moon National Monument. In her science class, Caitlyn is studying the formation of the Earth, including earthquakes and volcanos, so this trip was a living science project for her.

Caitlyn was a trooper. She understands Grandpa's problems and she takes them in stride. When he has coughing seizures, she doesn't get upset, and she accepts his limitations. In Jackson Hole she had to take her turn in the wheel chair, doing spins in the parking lot. She thought the chair was cool.

October 23, 2005 - This brings us to today. Bill continues to lose weight, which along with loss of appetite, is a symptom of PF. Today he weighed 136 pounds, over 40 pounds less than he did in February. What the future holds, we don't know, but we thank God for each day that he gives us.

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