A ray of light and an ILD Specialist

After the diagnosis of PPFE and no real options for treatment or a plan of action, life just went on for the rest of 2017 into 2018. We had no idea how quickly the disease would progress, but we knew the symptoms were getting noticeably worse once again in August 2018. After an exhausting trip up a single flight of stairs, Danny made another appointment to visit the Pulmonologist at St. Mary’s. Danny’s pulmonary function tests had dropped 10% in the last year and a half, which was far more aggressive than we had expected. At that point, our doctor felt it would benefit us to transfer to an Interstitial Lung Disease (ILD) specialist at UW-Health University Hospital in Madison.

As with most hospital experiences, we had to wait for some referrals, paperwork and insurance approvals, but we finally had our first meeting with the ILD specialist on October 17, 2018. He had already treated a PPFE patient before and knew of at least one that had undergone a transplant as well.

Prior to any appointment, we show up early so he can do a pulmonary function test (PFT) and a 6-minute walk to test his distance and check his oxygen levels. During his test at this appointment his O2 levels dropped to 89, but they don’t recommend oxygen until the patient is around 88-77 so he was right on the threshold but not consistently in need of it. He was also losing quite a bit of weight at this point, which is consistent in patients with decreased lung function. He was down from around 160lbs in 2017 to 136 lbs at this appointment.

He wanted to discuss some strategies and medication options, but it needed to be very clear that nothing we were going to do was going to cure it, stop it, improve it, or make it better. We were simply trying to delay the inevitable, and even then, nothing we did had any proven efficacy.
Option 1: Anti-scarring medication (OFEV) – OFEV is a medication commonly used to treat idiopathic pulmonary fibrosis. The problem we would encounter is that it’s not approved for PPFE patients; therefore, insurances tend to deny it. You can’t really blame them. It’s an expensive medication that doesn’t have enough of a proven benefit to PPFE patients in particular. If Danny had an auto-immune deficiency, they may approved the medication on that basis, but not solely PPFE. It makes sense in theory, but insurances don’t typically care to foot the bill simply based on your theory. The plan was to apply for it and see what happened (SPOILER: they denied it). After that, we’d try reaching out to the drug manufacturer directly to see if they’d cover the cost.

Option 2: FAM – an anti-inflammatory therapy that had the potential to slow progression. F stood for Fluticasone (Flovent), a steroid. A stood for Azithromyacine, an antibiotic. M stood for Montelukast (Singulair), an allergy medicine. This option was low risk with very low side effects. The antibiotic (Zpac) had the potential to cause an arythmia, so they had to do an electrocardiogram prior to ensure his heart could handle it. Outside of that the only other concern was with the Fluticasone and a possibility of thrush if he didn’t rinse his mouth after each use. Compared to the OFEV, this was a more reasonable option for the time being.

We chose Option 2 and were introduced to a spacer with his inhaler, which essentially helps the medicine get into your airways better. They described it as helping the medicine turn the corner down your throat rather than landing on your tongue.

The ILD specialist was surprised he was still working in his condition. They were incredibly supportive and offered to connect us with a social worker to set up disability whenever he was unable to work. Working wasn’t going to hurt him, but they wanted him to know he had support when it became too much.

The appointment closed with a flu shot and a Pneumonia 23 vaccine (both highly recommended to patients with impaired lungs like his) and a visit to the check-out desk to begin scheduling routine office visits every 6 weeks with CTs and PFTs to closely monitory the efficacy of FAM and any progression.

We may not have had the answers everyone wants when they leave a doctor’s office, but we left feeling like we had a medical support team and some options to try to improve Danny’s quality of life. Next on the list was a discussion around Lung Transplant.