Until a friend got pulmonary fibrosis, Marcia Slater had never heard of the condition. But she soon learned after being diagnosed with it herself.
Pulmonary fibrosis is scarring of the lungs and is often mistaken for other illnesses. It’s also difficult to diagnose and can be mistaken for other maladies.
Doctors didn’t know what caused Slater’s disease. Even when they removed her lungs, the doctors said an outside environmental source had caused it, but they couldn’t tell her what that source was.
In her younger years, Slater said, she lived on a cotton gin yard, and later she worked at the Ector County Courthouse for 20 years, which she said was a “sick building.” There are a lot of other people who have retired from working there and come down with serious illnesses, she said.
“Who knows out here in the oilfield environment, it could be an issue; mold in your home. We had that checked out because I had to come back to that,” Slater said.
Sick building syndrome includes various “nonspecific symptoms that occur in the occupants of a building,” the National Center for Biotechnology Information said.
The only cure for pulmonary fibrosis is a lung transplant.
Slater’s friend Nick Williams was going through the process to get a lung transplant, but died.
Slater received her transplant in April 2017.
Having been one of the lucky ones, Slater is an advocate of organ donation and pulmonary rehabilitation, which she has undergone and still participates in at the Medical Center Hospital Center for Health & Wellness.
MCH registered respiratory therapist Sharla Free said providers take a team approach to pulmonary rehabilitation. The goal is to restore a patient’s ability to function without extreme breathlessness and allow them to exercise safely, the Pulmonary Fibrosis Information Guide from the Pulmonary Fibrosis Foundation said.
Pulmonary rehab meets twice a week, so it is a time commitment, Free said.
“We have a cardiac nurse and exercise specialist and a registered dietician, then the respiratory therapist. We just take each individual and come up with an exercise program tailored to their needs,” Free said. “We have some patients on walkers. We have a lot of patients that use oxygen, so we have to be creative. We want them to be able to exercise, but it needs to be safe.
“They wear a heart monitor. We regulate their rhythms and heart rate, oxygen level and just their exertion — how hard it is for them.”
Slater said the disease can take over your life pretty quickly.
“There are some cases where they have medications out now that can extend your life, to some degree, in some cases, but there is no cure for it,” Slater said.
Her severe symptoms started in February 2014, but it took until December of that year to be diagnosed with pulmonary fibrosis, she said.
The first time, doctors told her it was pleurisy and told her to take an Advil. She also was told it was an autoimmune disorder attacking her lungs and rheumatoid arthritis affecting her lungs, even though she had no symptoms.
“It’s kind of one of those things. It’s still in the research phase, but I’ve learned there are a lot of people that have it — a lot more than I realized. Pre-transplant, I got involved in a pulmonary fibrosis support group through UT Southwestern in Dallas, well sort of through them. One of the doctors there sent me an invitation to a meeting and I went,” Slater said.
She has to see a doctor in Dallas once a month, but it’s hard to get there for a support group on the same schedule. They started offering a live feed of the support group meeting that could be viewed on Facebook, she said.
“Which is nice because they have access to amazing doctors and amazing research that we don’t have here. But you still have the people around here that have the disease, even in the smaller areas; so I came up with this bright idea that why couldn’t we have a local support group but watch their live feeds?” Slater said.
At the time, she was going to pulmonary rehab with Free. Slater said she discussed the idea with Free and Free got Medical Center Hospital involved.
“She was kind and gracious enough to step in and say, ‘Let’s do this,’” Slater said.
Because of health privacy laws, Slater couldn’t call a doctor’s office and ask them for contact information for their pulmonary fibrosis patients, so Free talked to patients who might be interested.
“It’s helpful to compare notes with others, but you’ve got to let people know the group is out there,” Slater said.
After she was diagnosed, she met with the transplant team at UT Southwestern in Dallas and started the process of being listed for a transplant.
“It’s a strange thing because you have to be sick to be listed, but well enough to go through the surgery, so it’s a process,” Slater said.
She was listed for a double-lung transplant in December 2015. The first time she had gone to the team, she said, she wasn’t sick enough to be listed.
“I was on oxygen 24/7. I had 26 tests in a week. They literally checked everything. I had an MRI on my head, a bone scan, CAT scans, colonoscopy, upper GI. I had an echocardiogram. They (took) 24 vials of blood out of my arm. I had to do a heart cath, right and left,” Slater said.
“All of that stuff has to check out, or you cannot be listed, and if there’s a problem in any of those areas it has to be fixed. It’s a complicated thing,” Slater added. “You’ve got to be healthy, and yet you’re not healthy.”
During the process and afterward, she found pulmonary rehabilitation very useful.
“Every doctor should refer that. I believe it helped me tremendously with my surgery. One of the things (about) the surgery is you have to be strong enough to recover. You’re in bed and down so much that you lose every bit of your muscle mass. All that has to be built back, so you’ve got to … be in pretty good shape, which is hard to do because you’re pretty sick. I’d be on their equipment out there with oxygen turned up to maximum capacity on those bottles just to be able to breathe and to keep my heart rate,” Slater said.
Slater is also in a worldwide lung transplant support group and one of the things they talk about is the irony of having to stay in good condition while being so ill. The group is very supportive, but going into the preliminaries for the procedure is negative.
“They’ll tell you have a 50 percent chance of survival from the surgery. The average lifespan post transplant is five years, so you go through all this surgery and hard work and the average lifespan is five years. So it’s all pretty discouraging,” Slater said.
She noted that lungs also are one of the hardest organs to obtain because they are easily damaged.
She added that she will have to take anti-rejection drugs for the rest of her life and that she usually wears a surgical mask when she’s out in public.
In addition, she had to learn to breathe again.
“To some degree, I’m still recovering,” Slater said in January. “And as far as being listed, the other thing is you have to have a match. They actually listed me in December of ’15 and one of the things for rejection is antibodies. My antibodies were 98 percent, which meant I had a 2 percent window of finding a match.”
She said it was a God thing that there was a match for her.
Slater was called twice for the possibility of transplant. One was a dry run, which she said is very common, and the other was real. During the first one she was a back-up for someone else, but neither one got the transplant because the lungs were not viable.
“So it was April of ‘17 before I got called again. You have to have faith that it’s all going to work out and it’s going to be the right timing. Otherwise you would just go nuts. I did have faith. I relied on faith that it was going to happen a
nd went through my day,” Slater said.
Another thing that has been difficult for Slater was that to get lungs, they had to be harvested from someone who had just been taken off life support.
“So it’s got to be extremely difficult for that family. I can’t even begin to imagine, so I carry a lot of guilt. It’s hard. I think that’s a common feeling for anyone because it’s just a different circumstance,” Slater said.
Free said for the most part it’s unknown how someone contracts pulmonary fibrosis, but there is research showing it can be genetic and the result of autoimmune disorders, smoking and esophageal reflux. Although she’s had patients who had never smoked turn up with the disease.
She added that she has had firefighters as patients who were subject to breathing in fumes and solvents.
Symptoms can include a dry, chronic cough and increasing shortness of breath. Patients may also experience fatigue, anxiety and depression, Free and literature from the Pulmonary Fibrosis Information Guide said.
The onset is usually between age 40 and 70. Life expectancy is approximately two to three years after diagnosis, Free said.
The support group started last April. It meets once a month, currently on the third Tuesday night of the month, from 7 p.m. to 8:30 p.m. at the MCH Center for Health & Wellness. Attendance varies.
“We can have anywhere from two or three to eight to 10. It just depends,” Free said. “Caregivers come with them and we encourage that because it is definitely a disease that affects everyone, not just the patient,” Free said.
There are a couple of medications that have been proved to slow down the disease, Free said.
Things that can make it easier on the patient are staying active, which is why pulmonary rehab was looked at as a primary management for the disease.
“Most of them at some point will need oxygen,” Free said. “It’s just preventive behavior. It’s not getting out in cold and flu season, being aware of people that are sick around you and taking care to wash your hands.”
Free has been seeing more cases lately. At a summit she recently attended in Nashville, Tenn., on the disease, it was said that the medical community is becoming better equipped to diagnose it. “I’ve had some patients who were originally diagnosed with COPD, then as further evaluation went on it was discovered that it was in fact pulmonary fibrosis. With chronic lung diseases you have shortness of breath, chronic cough, a need for oxygen. All of those things go along with chronic lung disease, so it takes a while to figure out exactly.
“I think that’s why it’s hard to get that final diagnosis. It’s easy to think it’s something else …,” Free said.
She said Slater was instrumental in helping her set up the support group.
“Being at home, it’s isolating. I think that’s why pulmonary rehab has been so important because it gets them out, gets them with other people who have a similar situation. Even if they don’t have the same disease, they still have a lot of the same symptoms and can meet other people …,” Free said.
She added that there is new research taking place on stem cells, new medication trials and new ways to diagnose.
For more information about treatment and the support group, call Free at 640-1472, or email [email protected]. The MCH Center for Health & Wellness is at 8050 State Highway 191.
