Seventy-four-year-old Linda Knybel of Manchester was living fairly comfortably with her pulmonary fibrosis, taking her daily walks and going out for lunch or shopping. But suddenly, in May, Linda began experiencing severe shortness of breath.
She couldn’t walk up a short flight of stairs without needing to rest for several minutes at the top, and she was afraid to take her walks because of lightheadedness that could lead to a fall.
A regular checkup with her pulmonologist and a new echocardiogram led to a referral to Dr. Raj Parikh, a pulmonologist at Hartford Hospital and its co-director of the new Pulmonary Hypertension and Pulmonary Vascular Disease Program.
Dr. Parikh did a right-heart catheterization to confirm what he suspected would be a diagnosis of pulmonary hypertension, a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. A typically underdiagnosed condition because it is often brought on by diseases like COPD, congestive heart failure, HIV and others, it can be fatal if untreated.
The bad news for patients with pulmonary hypertension is that up until a couple of years ago, there were almost no treatment options. Today, there are drugs and delivery options that make a world of difference to many patients’ quality of life.
The catheterization did confirm the diagnosis, and it also confirmed that Linda would need the most intensive treatment to manage her condition: around-the-clock intravenous delivery of a prostacyclin agent. In this scenario, the patient has a port, and is trained by a pharmacist how to mix and deliver the medication through a device contained in a fanny pack the patient wears. After about two weeks in the hospital, Linda was discharged home to her husband.
“At first, it was terrifying,” Linda said. “My whole family is trained how to do it. At the beginning, it took us forever to do it, but now it’s easy. I would recommend it to anyone who needs it. Now I have no problem going up stairs, I don’t need my oxygen very often, and I am walking farther all the time. I go to lunch, I go shopping, I go out with friends.”
This is a story Dr. Parikh loves to hear, because he’s been working hard for the last six months to raise awareness not only of pulmonary hypertension in general among practitioners, but also of Hartford Hospital’s new program that focuses on it. When he came onboard in October 2020, there were three or four identified patients. Today, there are more than 100.
Part of the challenge is the patient demographics of the condition — they range from young, fit women to the morbidly obese to the elderly to those with heart conditions or clotting disorders. Because there is no clear typical patient, and also because so many of them are living with a larger disease like COPD or congestive heart failure that mirror symptoms, pulmonary hypertension can be easy to miss.
“So my focus is on outreach to the providers, I put my name out there to cardiologists, rheumatologists, pulmonologists, to tell them about symptoms and all the new therapies there are for treatment,” Parikh said. He is also working to bring a clinical trial to Hartford Hospital for a new treatment as well.
Current treatments include pills, inhalers, subcutaneous and intravenous options. Parikh noted that with these new medications, patients can often see improvement in their day-to-day lives, as Linda did. “We’ve gone from no treatments 15 years ago to multiple options today,” he said. “It will only get better moving forward.”
