By Marilyn Hanft, Special to Everyday Health
I am a 66-year-old critical care registered nurse. I retired in November 2009, and my husband and I moved onto our 36-foot motor sailer and headed south from Portsmouth, N.H., along with our golden retriever and a ragdoll cat.
Our plan was to head down the Atlantic Intracoastal Waterway, stopping eventually in St. Mary’s, Ga., where we would join boating friends. That trip is a major story in itself, but a tale for another time and place.
We eventually arrived in St. Mary’s and soon fell in love with this lovely, small Georgia town. Planning to use it as our home port for future cruising, we joined the Yacht Club and bought a house. We spent most of 2010 traveling up and down the Eastern Seaboard, either by car or boat. We arrived for good, on the boat, on December 31, 2010, and went to work organizing our new home.
An Asthma Flare? No. A Missed Diagnosis
About mid-February 2011, I began having a lot of trouble with my allergies, which triggered my asthma. I saw a local allergist and was started on nebulizer therapy, along with antihistamines, prednisone, and various other meds. Nothing seemed to help, although I was no longer wheezing. I did have sudden bouts of coughing for anywhere from one or two episodes up to seven or eight in a single day, that made me feel as if I were going to pass out.
Mine was a classic case of the doctor not seeing the zebra among the horses. I had a 25-year history of multiple allergies and asthma, ergo I must be having an asthma flare.
On June 2, I finally gave in and went to our local ER. Once they had my oxygen levels up and my respiratory rate down a little, the doctors told me I had a large blood clot, a deep vein thrombosis (DVT), in my right calf. They didn’t do a CT, but they felt sure we were dealing with at least one pulmonary embolism, a blood clot in my lungs, and maybe more.
Unfortunately, they didn’t diagnose my pulmonary hypertension (PH).
Out of the Hospital and Back Again
I remained in the hospital for 11 days, and I was discharged when I knew I wasn’t really ready. Over the following week I got worse instead of better. My doctor had given me oxygen to use with activity, but I didn’t even think to use it all of the time, especially at night. This was an indication that my cognition was seriously impacted. I had worked with respiratory patients for 40 years, but I didn’t apply that learning to my own situation.
Exactly a week after my initial discharge, I had my husband call 911. I realized later that the rescue team thought I might go into respiratory arrest any minute. We went “lights and sirens” back to the hospital.
That is when they discovered I had many, many pulmonary emboli, not just one or two. Added to that, I now had a severe right-side pneumonia. They put me on BiPAP (a type of breathing support) and back to the intensive care unit (ICU) I went.
The rest of that day is a bit of a blank for me. The next clear memory I have is the on-call doctor saying they wanted to transfer me to the regional hospital, 35 miles away in Brunswick, Ga., because they thought I would need a ventilator. Again we went lights and sirens to the Brunswick hospital. Again I was admitted to ICU. By the next morning I seemed to have turned the corner toward recovery, so I never did need the respirator. This was when I met my current pulmonologist.
Pulmonary Hypertension — Plus a Gene Disorder
Over the next 11 days he did multiple tests — CT scans, blood work, V-Q scan (a type of scan that looks at both air flow and blood flow), and more. Unfortunately, I was still throwing clots, even on heparin and warfarin. At this point my doctor decided I needed a venous filter, which finally stopped the pulmonary emboli. On the day before discharge the doctor told me they finally knew what else I had besides pulmonary arterial hypertension (PAH).
I have Factor V Leiden, a genetic clotting disorder. That explained the problems with getting my body not to develop more clots, and why I had the DVT in the first place. I was discharged on July 1, 2011.
Over the course of the next few months I learned that I would be on oxygen and warfarin for life. My husband and I regretfully decided that cruising was out. We would sell our boat to help offset the $150,000 medical bills, most of which were not covered by our bare-bones health insurance.
In February 2012, I started taking Adcirca (tadalafil) and very quickly went from 24/7 oxygen to only using it 15 hours a day. When I finally qualified for Medicare that year, the drug company withdrew my no-cost supply of Adcirca. My pulmonologist and I decided to switch me to Viagra, as it was the cheaper choice. I had been doing very well but started a mild decline with the drug change. In January 2014, I started on Adempas. I have responded very well to it and feel the best I have felt since beginning this long journey.
In December 2013, I set up a website and blog for those with PAH and/or thrombophilia (like Factor V Leiden) called Of Bad Lungs and Blood Clots. I also discovered the Pulmonary Hypertension Association about the same time and have become very active in it, participating in the Phenomenal Mile walk, the conferences in June, joining the CTEPH (chronic thromboembolic pulmonary hypertension) Advisory Board, and participating in the Media Blitz for World PH Day.
The future looks a lot rosier than it did just a couple of years ago.
Marilyn Hanft is a retired critical care RN from Georgia, shown above at the Pulmonary Hypertension Conference fashion show in an outfit she made herself. She first shared her story on the Pulmonary Hypertension Association site.