Pulmonary Hypertension (PH) is a rare lung disorder in which the blood vessels in the lungs narrow, and the pressure in the pulmonary arteries, which carry blood from your body to the lungs, rises far above normal levels.
Moustafa Kamel, the Medical Affairs Director of Janssen South Africa, explains the symptoms in everyday terms: “The patient will suffer from shortness of breath when they undertake even the simplest activities that most of us would think nothing of – such as, walking for ten or twelve meters. People need to pay attention to such matters, as PAH is a serious and chronic disease which, untreated, can lead to heart failure.”
PH may be the offshoot of other diseases, such as lung disease and heart disease, although medical science has yet to locate its specific causes. Genetics can play a role, with family histories that include Down syndrome, congenital heart disease, Gaucher disease, or blood clotting, increasing one’s risk, although it can strike anyone, regardless, even of age. It can, indeed, affect children, although its incidence typically increases with age.
PAH can be associated with other medical conditions, most notably connective tissue diseases and congenital heart diseases (CTD).1 In registries, around half of PAH patients have idiopathic, heritable, or drug-induced PAH. In the subgroup of associated PAH conditions (APAH), the leading cause is CTD, mainly systemic sclerosis (SSc).1
Statistics also tell us that it’s more common among women, black people, and people over 75 years of age.5 Although this disease can present itself in people who lead perfectly healthy lifestyles, unhealthy lifestyle habits such as smoking and irresponsible narcotics usage can also be a casual factor.
Kamel stresses that, as with so many diseases, PAH is progressive in nature – in other words, its burden increases with time. “If left unattended,” he cautions, “it could reach a stage where the patient may need lung transplantation. So early diagnosis is crucial. This will allow medical interventions, of which there are fortunately many options available in South Africa.”
“A better lifestyle is available to the patient, thanks to the availability of proven drugs and treatment interventions,” Kamel concludes. “Patients may well find that they go from not having been able to walk for ten meters, to being able to comfortably undertake half-hour walks.”