Down but not out

Members of the online Behcet's support groups know that this hasn't been an easy year for me. Breast cancer is no fun, but it was caught early and I was able to get away with surgery and radiation treatments over the summer -- no chemo.

My cancer treatments stopped at the end of September, and the oncology staff threw confetti at me on the last day. I went back to work full-time, and I ran an online survey of BDers' experiences with different meds, for an upcoming book on BD treatments. Everything was fine, except for an occasional nagging, dry cough that I couldn't shake. By the end of October, I started having low-grade fevers and occasional chills... but those problems were listed as possible side effects of the Arimidex that I need to take for 5 years (it's an anti-cancer hormone treatment, like Tamoxifen). Then came the first week in November: My cough got a lot worse, I had trouble breathing at night, and a just-taken xray of my right lung looked like a white, fluffy cloud. After taking antibiotics for three days with no improvement, my PCP threw me in the local hospital.

They figured I had some kind of bacterial or viral pneumonia, and put me on IVs, different antibiotics and full-time oxygen. Four days later, nothing had changed, except that I needed more oxygen than when I was first admitted, I was coughing non-stop, and just getting up to go to the bathroom made it hard to breathe.

Enter the hospital's "Pulmonary Specialist," the doctor who was finally going to make it all better. He said he was going to perform a bronchoscopy to see if anything else was going on in my lung(s). A bronchoscopy involves sending a "thin" tube through your nose, between your vocal cords, and down into your lung; there's a light and a camera on the end of the cable. If you're especially "lucky," the tube also sends some saline solution into your lung and withdraws the fluid to see if there are any nasty cells lurking down there. The specialist (and his accompanying respiratory therapist) said he'd be able to do this test quickly, in my room, with little to no sedation needed. Mistake #1: Believing him. Having no experience with the test, and wanting to believe that a doctor in this hospital actually knew what s/he was doing, I allowed him to do the procedure sedation-free. They numbed my vocal cords with lidocaine and got to it. It took two assistants to hold me down. I've since read up on the procedure, and anti-anxiety meds and/or conscious sedation is standard for a bronchoscopy. I will never have this procedure done without sedation again.

The results of the bronchoscopy were interesting, though: My airway and lung looked perfectly healthy. The next diagnosis turned out to be the correct one: radiation pneumonitis. It's a form of pneumonia that can happen to a very small percentage of people who have radiation treatments for lung or breast cancer. Since my cancer was on the right side, my right lung was the one that was affected. A dry cough, low-grade fever and chills all fit the pattern, and the specialist put me on IV steroids, 60 mg four times/day. Finally, a drug that made sense to my BD brain: zap the inflammation with steroids, get better, the hell with side effects.

That's the point when any hope of meaningful doctor/patient communication went downhill (not that there was much hope at that point anyway). Yes, the steroids made an immediate difference in my breathing and coughing. I was concerned, though, that this dose might not be enough if BD was throwing some extra lung inflammation into the mix -- I'd developed a pathergy response within 30 hours of the withdrawal of two IVs, so I thought BD might be an added issue. It was "discussion time" with the pulmonary specialist. I explained more about my BD issues, and asked him to speak with my BD specialist in Boston (who had already agreed with my assessment, and wanted to suggest high-dose IV methylprednisolone over a 3-day period).

The following day? The pulmonary specialist returned, fresh from talking with my BD doc, and from a short amount of time looking up Behcet's online. He announced (incorrectly) that "Behcet's never affects the lungs, and therefore it's a non-issue." He also said that my BD doc's treatment suggestion was unnecessary overkill, and that it was time to continue the established treatment for radiation pneumonitis. My IV steroids would be cut in half that day, and in half again the next day. I would hopefully be going home on the third day with an oral prednisone taper: 60 mg the first day, 60 the second, then 50, 50, 40, 40, 30, 30, 20, 20, 10, 10, and totally off pred on the 12th day. Trying to explain Behcet's, or my BD specialist's expertise in the subject, was totally lost on the pulmonary doc. If I'd had a copy of the "Essential Guide" with me, I'd have thrown it at him and told him to read the section on pulmonary and respiratory complications.

After he left, I was visited by two other doctors who said that they were in agreement with the pulmonary doc, that Behcet's isn't a lung issue, and that there was no disagreement (which was later found to be untrue) between my BD expert and the pulmonary doctor.

Egotistical and ignorant doctors are dangerous people. By noon of that day I was determined:

• To get out of that hospital as soon as possible, on whatever prednisone dose I was allowed, whether I was discharged on oxygen or not
  
• To get out of that hospital before I came down with swine flu (which was starting to thin the ranks of the staff) or some other dangerous respiratory infection
• To have no future contact with any of the doctors who ignored and/or belittled the advice of my BD specialist

I was finally discharged two days later (the 8th day of my hospitalization), without oxygen, and with a 12-day 60 mg tapered plan of prednisone. On the 4th day at home, on 40 mg of prednisone, my cough and breathing issues roared back again. I went online and started to use those old research skills. Gee, guess what? The "established" (rapidly tapered) treatment protocol for radiation pneumonitis that was prescribed by the pulmonary specialist was correct about dosages, but dangerously INcorrect about the timeframe. Standard treatment is to maintain a patient on 60 mg of prednisone for weeks, with the goal of totally tapering off pred over 6 months to a year.... to prevent the exact kind of relapse that I was facing after 4 days. Radiation pneumonitis that is undertreated has the potential to become incurable pulmonary fibrosis within 6 months to a year, causing a lifetime of possibly-dangerous breathing issues. Who wants that?

I immediately called my PCP and explained what was happening. She supported my reasoning, agreed to make sure that I had enough prednisone to taper at a proper rate, and will see me on Friday. At which point I'll also discuss using inhaled steroids as an alternative to the nasty side effects of oral prednisone (yes, I found an article recommending it as a valid option). It should allow me to taper a little more quickly, but still maintain my recovery.

I've spent my life advocating for Behcet's patients. And I work full-time as a medical writer for a company that helps to change peoples' health-related behaviors. I like to think that I can handle my own medical issues and problems, and I'm floored that my medical situation (and patient/doctor communication) went downhill so fast in spite of it. Being so sick didn't help, but this was a strong wakeup call.

On the plus side:

• I know that my Boston Behcet's expert has my back. In the event that I can't get relief from this pneumonia through standard tapering and the use of inhaled steroids, I know that he'd provide the 3-day IV methylprednisolone treatment that would cut through all the crap -- quickly and without any argument. Why not do it now? I've been through the treatment before and don't want the side effects unless it's absolutely necessary.
  
• In the event that I have a relapse, my local hospital is no longer an option. Boston hospitals are an hour away, but I have a much better chance of coming out in better shape than I went in.
• And finally, I do have to say that not all interactions with the hospital staff were bad. Several nurses and other personnel spent time on my behcetsdisease.com website, learning about BD. One doctor brought her medical students into my room for an impromptu lesson on Behcet's. I didn't mind any of it, and thanked everyone for being willing to learn about a rare illness.
  

In the end, though, I'm angry and I'm frustrated. I thought Behcet's sucked all by itself, but add breast cancer and radiation pneumonitis, and it's been the year from hell. Short of an absolute meltdown, though, I'll be at the American Behcet's Disease Association medical conference at the end of April, speaking about Behcet's and pregnancy, and selling my books (conference registration is now open at the ABDA site).

I may be down, but I'm definitely not out. Happy holidays, and see you in April.
P.S.: And for people suggesting that I file a formal complaint against the pulmonary specialist to the hospital and the Mass. Board of Registration in Medicine, yes, all options are on the table.