Living with a rare disease like pulmonary hypertension (PH) can be challenging in ways beyond managing the disease. Rare diseases can be isolating, and finding accurate information can be difficult. Patients may also find that their families and social networks have never heard of PH. Those who aren’t familiar with PH may think that it is the same thing as high blood pressure that your doctor measures with a cuff, or they may see an oxygen canister and assume that PH is primarily caused by smoking.
To help spread the word and reduce misconceptions about PH, here are some things everyone should know.
The symptoms of PH can look like many other illnesses.
The symptoms of PH can include shortness of breath, dizziness and fatigue. Because these symptoms can mirror more common, less-threatening illnesses, patients can go years without being accurately diagnosed, or may be misdiagnosed as having other illnesses such as asthma or COPD.
Anyone can get PH.
There are risk factors that make some people more likely to get PH than others, however the disease affects children and adults, men and women and people of all races and ethnic backgrounds. PH can exist alone or be associated with many other diseases. For example, between eight and twelve percent of scleroderma patients also have PH, and between 17-53 percent of sleep apnea patients also have PH.
Not all PH is the same.
Currently, there are five classifications, or groups, of PH, according to the World Health Organization (WHO). Within these types there are more than 30 different, specific conditions that can lead to PH. PH can be associated with lung disease, left heart disease, blood clots, connective tissue diseases and genetics – among many possible associations.
There is no cure, but there is hope.
There are FDA-approved drugs for adults with WHO Group 1 PH, pulmonary arterial hypertension (PAH), and WHO Group 4 PH, chronic thromboembolic pulmonary hypertension (CTEPH). While there is no cure for other forms of pulmonary hypertension (PH), CTEPH patients may be eligible for a pulmonary thromboendarterectomy, or PTE, a surgical procedure to remove the chronic clots that cause CTEPH. CTEPH patients who are not candidates for PTE, or those who undergo the procedure and have PH that remains after surgery, may be candidates for oral medication approved by the FDA for CTEPH. There are is only one FDA-approved treatment for children with PH. But there’s hope. New research is being conducted every day. Further, with early and accurate diagnosis, proper treatment can greatly extend and improve PH patients’ quality of life.
* People who think they might have PH should seek diagnosis and treatment from a PH specialist. For more information, visit www.PHAssociation.org.
