A Rare and Life-Threatening Disease with Common Symptoms: Highlighting Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Determination. Strength. Resilience. These are just a few qualities that may characterize people around the world living with pulmonary hypertension (PH), a sometimes debilitating disease of the lungs. But a particularly rare form of the disease, called chronic thromboembolic pulmonary hypertension (CTEPH), is often misinterpreted by patients and doctors alike.,
November marks PH Awareness Month, designed to shine a spotlight on the disease — and generate support for patients. On November 21, patient advocacy groups, healthcare professionals and caregivers focus specifically on CTEPH and the unique needs of this patient population.
- CTEPH may be caused by blood clots that increase blood pressure in the lungs and can lead to heart failure and death if not properly treated.,
- Each year in the U.S., between 500 and 2,500 people are diagnosed with the disease.
“Patients with CTEPH may face devastating symptoms and be left searching for answers and help for years,” says Steve Van Wormer, co-founder and president of phaware, one of the leading global pulmonary hypertension advocacy organizations. “CTEPH Awareness Day is an especially important opportunity to highlight this debilitating disease that many have never heard of before. We need to provide support and raise awareness in order to reduce the time it takes to receive a proper diagnosis and treatment.”
Recognizing the signs and symptoms
PH diagnosis is frequently delayed by several months or years. This is because the most common symptoms, including breathlessness, fatigue and dizziness, are also associated with many other conditions. It is important to note that CTEPH, a subset of PH, is treatable and potentially curable through surgery when properly identified.,
CTEPH also has a connection to a much more common condition, called pulmonary embolism (PE), a blood clot in your lungs. Many people don’t realize that as many as 1 out of every 25 of previously treated patients with PE that does not resolve after three months of using blood thinners could go on to develop CTEPH*.,,
Spreading the word for healthcare professionals
In addition to raising awareness among potential patients, specialists who treat CTEPH, including Dr. Rajeev Saggar, executive director of advanced lung disease care at Banner — University Medical Center Phoenix and the clinical associate professor of medicine at University of Arizona College of Medicine — Phoenix, hope to increase knowledge of the disease among other clinicians.
“The goal of PH treatment is to lengthen survival time, reduce symptoms and improve quality of life for patients. Early, accurate diagnosis is increasingly important, particularly because medical care is steadily improving,” said Dr. Saggar. “It is vital that clinicians detect or rule out underlying diseases that may represent the cause of PH, including various heart and vascular diseases, human immunodeficiency virus (HIV), lupus, or as is sometimes the case with CTEPH, unresolved pulmonary thromboembolism, when a clot-like mass gets stuck to the lung’s blood vessel wall and blocks blood flow.”
According to Dr. Saggar, a ventilation/perfusion (VQ) scan is the preferred and recommended screening test for CTEPH. It is important for anyone diagnosed with the disease to see an experienced CTEPH practitioner who can determine candidacy for pulmonary thromboendarterectomy (PTE) surgery. Although PTE is the recommended treatment for CTEPH, not everyone is a candidate. It’s important for patients to speak with his/her doctor about options.
Despite efforts to ensure better education about the disease, its symptoms and methods of treatment, physicians like Dr. Saggar and those throughout the CTEPH community note that there is still progress to be made. That’s why November is an especially important time for the PH community to come together and raise awareness and generate additional support for research and improve care.
“Building awareness and enhancing education will help to extend and improve the lives of patients with CTEPH and all forms of PH,” says Dr. Saggar.
Dr. Saggar and phaware partnered with Bayer to help raise awareness about PH and CTEPH during PH Awareness Month. Dr. Saggar is a paid consultant to Bayer.
*Based on a study with 223 patients in which approximately 4% were diagnosed with CTEPH within 2 years of their first episode of pulmonary embolism (blood clot in the lung). CTEPH did not develop after two years in any of the remaining patients.
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