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Take A Deep Breath: A Patient's Perspective on the Diagnosis of CTEPH, Surgery and Life Beyond

Jean Magazzu
CTEPH Patient
PTE Operation 2012

Truly, my story begins at birth. But since that was so long ago, I won't bore you with that level of detail. However, after hearing this story most people respond by asking, "How did that happen?!" and we end up going back to 1967. But for now, I'll start in 2007...

Around the time I turned 40 in 2007, I began having intermittent periods of shortness of breath. I assumed it was caused by an allergen and ignored the symptoms that would years later lead to one of the scariest moments of my life.

In early 2011, at the very young age of 44, I experienced this same shortness of breath accompanied by a wet cough. My PCP's diagnosis was bronchitis, so she put me on antibiotics and prescribed an inhaler. After a few weeks and two more courses of antibiotics with no improvement, my shortness of breath became severe - a conversation or short walk across the room took my breath away. I felt pangs of jealousy watching people casually strolling down the street - that simple action seemed like an insurmountable and exhausting task.

I stubbornly agreed to visit the ER at a local hospital, thinking they would discover some extreme environmental allergen that I was reacting to. Instead, they told me - and I will never forget these words as long as I live - that I had "large and numerous clots" in my lungs.

Now, I had watched enough episodes of the TV show "ER" to know this was not good news. In fact, had I not had a friend just survive the discovery of PE's, I would have thought it was a death sentence. I was quickly transferred to a larger center for further evaluation and consideration of what the treatment might be. My best friend since childhood, who lives near me, called my family in Phoenix and, as gently as possible, explained the situation. They arrived the next day. That was one of the moments when I realized the severity of my condition.

However, the truly defining moment occurred at the first hospital after being transferred from the ER to their cardiac unit. The doctors and nurses were gathered outside of my room discussing my transfer to the larger center. The nurse came to my bedside and very matter-of-factly stated, "They want to take you by helicopter." Her words floated above me, what little breath I had halted, and I felt the color leave my face. All I could manage was turning to my best friend, who was thankfully by my side, as I mouthed, "Ask her why." In retrospect, the reason is obvious, but at the time it was a cold slap of reality.

I spent 12 days in the next hospital during which I was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). I was placed on blood thinners and a filter was placed in my inferior vena cava to stop any further clots from reaching my lungs. It was discovered that my pulmonary pressures were very elevated (90/60), and my right heart was quite enlarged. I had a number of tests including V/Q scans, CT scans, a right heart catheterization, echocardiogram, and a pulmonary angiogram.

As it turned out, my doctors felt that the acute clots were not the major issue; it was the chronic clots and subsequent scar tissue that clearly needed to go away! I was sent home on two new medications, Warfarin and Revatio.

During that hospital visit, I met a cardiac surgeon who explained to me that if the clots persisted after being on Warfarin, the next step would be surgery. After a few months and a repeat heart catheterization, it became apparent that my condition wasn't improving and I was scheduled for pulmonary thromboendarterectomy surgery in February 2012.

Mine is a typical American family, so naturally the severity of my condition was never discussed openly. But I could clearly see the worry on the faces of my family members and best friend. We all wore a brave face and carried on like everything was normal. From my point of view, I felt a responsibility to ensure everyone that all was going to turn out just fine - particularly my then 11-year-old daughter. Normalcy was paramount, but inside I was panicked.

I'm no doctor, but I'll do my best to explain the operation. My body was cooled to 65 degrees Fahrenheit to reduce my body's need for oxygen. They perfused my brain to avoid damage (although my smart-aleck friends would tell you it didn't work). The clots were meticulously removed in a five-hour-long surgery.

I'd like to say that immediately upon awaking from the operation I sensed an improvement, but the severity of the procedure took a day or so to recover from. However, after the grogginess passed and I was more physically active, it became apparent that my breathing was normal. (While I typically strive to be unique, this is definitely one of the times in my life when "normal" was GREAT.)

My pressures immediately returned to normal after the surgery, and within a few months the size of my right heart had also returned to normal. There were some notable bumps in the road, when I had fluid accumulation around my heart after the surgery which required drainage but this, I am told, is not uncommon. I was back to work part time in about three weeks, full-time in six weeks. My head was quite foggy for several months after the PTE surgery, and at times, this odd feeling returns (it's a cross between vertigo and a hangover).

Now let's go back to 1967 and how this all began. I have a congenital condition called Klippel-Trenaunay Syndrome (Google it if you want more details, if I tried to explain it this story would be way too long!). As a result, I have arteriovenous malformations (AVMs) in my right leg, which is believed to be the reason that my hip broke so easily when I was 34 years old. Due to complications with the AVMs, after four years of surgeries and embolization procedures I had a full hip replacement in 2005.

Tests have shown no genetic predisposition to pulmonary emboli, so the prevailing theory is that either due to the prevalence of AVMs in my leg, or from the surgeries associated with my hip fracture or both, I began "throwing clots" years ago.

The treatment I received at the first, smaller hospital was excellent; but it was when they astutely recommended I be transferred to a larger, more specialized facility that made all the difference.

Being quickly diagnosed with CTEPH and receiving the appropriate treatment surely saved my life. Knowing that my condition was treatable along with the confidence that all of my doctors exhibited gave me the peace of mind I needed during this inexplicably frightening period of my life.

The impact of this treatment on my quality of life can't be overstated - and as the single mother of a most amazing, smart, beautiful daughter I need to stick around for many more years! Increased awareness of the diagnosis of this disease and the expertise in medical and surgical treatment really changed my life. But back to today...it has been four years since my surgery and I feel great! I no longer give my breathing a second thought, or worry about not being able to go somewhere for fear that the walk will be too much. ■

Disclosure Statement: The author has no conflicts of interest to disclose.

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