Hereditary hemorrhagic telangiectasia (HHT) runs in Jessica Kiser’s family, so she knew from a young age that it likely was the cause of her frequent nosebleeds. But she didn’t know that it could cause pulmonary hypertension (PH) until she was an adult.
Similarly, some PH patients don’t know that they have HHT, a condition where some blood vessels develop improperly. Frequent nosebleeds, caused by small abnormal blood vessels in the nose, are a common symptom of HHT. Abnormal blood vessels that appear as tiny red or purple spots can develop on the hands, face and within the digestive tract. Large vascular malformations only visible through imaging procedures can occur in the liver, lungs, brain and spine, leading to serious complications.
Jessica, a communications and marketing coordinator from the Baltimore, Maryland, area, sought a formal diagnosis for HHT in 2010. She wanted to make sure she didn’t have related complications, such as an abnormal connection between the arteries and veins in the brain or spine. She missed a follow-up test in 2015, which she thinks would have identified her PH sooner.
She shares her story to help the Pulmonary Hypertension Association help raise awareness about the connection between HHT and PH during HHT Awareness Month in June.
Jessica noticed what turned out to be PH symptoms after a 2016 work trip to New Orleans. She had swollen ankles, heart palpitations, shortness of breath and excessive weight gain. “I felt horrible, and I could barely make it from my front door to my car without stopping several times to catch my breath.”
After two trips to the emergency room — once to rule out blood clots because of the flight and once for pneumonia from the fluid build-up – she was admitted to Johns Hopkins Medical Center, which initially had confirmed her HHT. There, she learned she had pulmonary arterial hypertension (PAH) caused by HHT. “I left with a new normal, a ton of medications and a new appreciation for life,” she says.”
The PH diagnosis meant she had to pay more attention to her HHT. The vasodilator medication used to treat her PH causes the vessels in her nose to rupture more frequently. “The longer I take the medication, the worse my bleeds get,” she says.
She tried creams, ointments, oils, supplements, sprays and two 12-week courses of Avastin, a chemotherapy-like drug, to control her nosebleeds. Eventually, she underwent laser surgery to cauterize the vessels in her nose and inject the Avastin directly into problem areas. The surgery stopped the bleeds for about six weeks. After that, she tried a monthly dose of Avastin to lessen the bleeding.
Jessica manages her PH through diet, sodium restriction, fluid restriction, light cardio exercise and good humor. “I honestly have to laugh sometimes at how ridiculous my life is,” she says. “I started taking an erectile dysfunction medication for my lungs at the age of 26, and I have most of the same problems as grandparents.”
Her humor belies the challenges of PH: “I hurt all the time, I can’t walk more than 50 feet without a ‘breather’ and I wear a CPAP to sleep.” However, her pulmonary pressure has decreased 75%, thanks to following strict routines for the past four years.
“I’m proud of how far I’ve come in life,” she says. “I made it to 30 alive and healthy when I was told I might not. I’ve beaten so many odds, no matter how hard it was, and I’m thriving in a time when that’s a rarity. Both of my conditions make it incredibly difficult, to say the least. “
She credits virtual support groups, social media and other online forums for helping her manage her conditions. “I can read up on the newest articles, help answer questions from others newly diagnosed, or meet new virtual friends,” she says.