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When It’s More Than Just Routine Fatigue


Sites on vacation in Miami, enjoying a return to traveling following treatment for PH

So many of us have been there: working long hours and feeling exhausted! But how do you know if it's just the body’s normal reaction to being overworked or something more serious? For Tiffany Sites of Mechanicsburg, Pa., who was putting in long hours at her banking job, it was persistent and worsening shortness of breath that ultimately led her to start seeking some answers from her local primary care doctor. Sites was diagnosed with pulmonary arterial hypertension in 2010.

Pulmonary hypertension, also known as PH, is high blood pressure in the lungs. Compared with regular hypertension, where arteries throughout the body are constricted, PH affects only the blood vessels in the lungs. If left untreated, PH can lead to right heart failure. According to the Pulmonary Hypertension Association (PHA), it can often be difficult to diagnose, since the most common symptoms are breathlessness, fatigue and dizziness, which could be associated with a variety of other conditions.

Sites, now age 42, was feeling tired and short of breath before she was diagnosed with PH, but chalked it up to working long hours — she had no idea it could be something more serious. Once she started treatment, which initially included using an inhaler every two hours, Sites began to feel better, but not enough to avoid cutting back her work hours to part-time. 

In 2013, while undergoing treatment at Penn — recently named by the PHA as one of just seventeen fully accredited Pulmonary Hypertension Care Centers in the country — Sites was put on a continuous infusion that delivered a powerful medication (called a prostanoid) subcutaneously via a pump attached to a small catheter that resides under the skin. “It was really painful,” says Sites, who had to master re-loading the pump and periodically changing the catheter during the year and a half she was using that medication delivery. She was even forced to stop working altogether. Sites credits her doctors and nurses with helping her through the sometimes scary and painful experience of using the pump, especially Christine Archer-Chicko, MSN, CRNP, a nurse practitioner at Penn Medicine. “She gave me so much encouragement and support,” Sites says.

Then in February of this year, Sites started taking a newly available oral form of the medication, the very first oral prostanoid available for PH in the U.S. The drug’s U.S. Food and Drug Administration’s (FDA) approval in December 2013, then availability to patients in mid-2014, was “a big deal in the PH field,” according to her doctor, Jason Fritz, MD, a pulmonologist at Penn Presbyterian Medical Center and an assistant professor of clinical medicine at Penn. Now, Sites just takes the pill three times a day. This has freed her to get back to activities she loves, like traveling and even swimming (which she wasn’t able to do with the pump attached). 


Sites’ dog, Vinnie

She’s also able to do everyday things, like walk her dog and take out the trash, which were a struggle before treatment. “I used to get tired just walking my dog in front of my house,” says Sites. “But now I can actually take him for a real walk.”

The root cause of Sites’ PH dates back to infancy. She was born with a ventricular septal defect — a hole in the wall separating the two main pumping chambers of the heart. This can cause excessive blood flow and pressure within the lung circulation, leading to PH, and in some cases this can persist despite having surgery to correct the defect, as Sites did when she was two years old. However, if left untreated, over time, the high pressure can strain the right side of the heart, leading to a form of congestive heart failure.

PH can complicate a variety of conditions, including more common types of primary heart and lung problems as well as chronic liver disease and autoimmune disorders, such as scleroderma. Infrequently, people who have suffered a blood clot in the lungs (pulmonary embolism, or PE) can develop PH.

Luckily, Sites took her adult symptoms seriously and got proper diagnosis and treatment, and is finally feeling good and looking forward to going back to work. “I’m very excited to get back to work,” says Sites, who has just recently started applying for jobs.

Plus, Fritz says the FDA is currently reviewing yet another oral prostanoid-type medication that requires only twice-daily dosing, which could make PH treatment even more convenient for patients. It’s promising news for patients like Sites and so many others, who may not yet even realize that their symptoms indicate PH.

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