This ‘silent killer’ has no cure, but can be controlled
Pulmonary arterial hypertension (PAH) is a progressive disease so it could be fatal. And while there’s still no cure, there are effective ways to manage the disease — if you get a timely diagnosis.
High blood pressure, otherwise known as hypertension, is so common that you likely know someone with the condition. But there’s a different kind of hypertension that’s so rare and can be much more deadly.
It’s called pulmonary arterial hypertension (PAH), a rare lung disorder in which the tiny arteries that carry blood from the heart to the lungs become narrow or blocked, making it difficult for blood to flow through the vessels. That raises the blood pressure in your lungs.
“Pulmonary hypertension is not the hypertension that we all know. It’s an umbrella term for elevated blood pressure,” notes Dr. Ivan Villespin, president of the Philippine College of Chest Physicians. “It targets the blood vessels in the lungs. And then there’s pulmonary arterial hypertension (PAH), which is a specific disorder of the blood vessels in the lungs.”
PAH is a progressive disease that is caused by the narrowing or tightening of the pulmonary arteries. When that happens, your heart has to work harder to pump blood through them. After a while the heart muscle gets weak.
“The right side of the heart becomes enlarged due to the increased strain of pumping blood through the lungs. Eventually, it can lead to heart failure,” warns Dr. Villespin.
Medical experts refer to PAH as a “silent killer” because symptoms do not usually occur until the patient’s condition has progressed. And, for the most part, they are often misdiagnosed, “simply because its symptoms mimic that of other lung diseases such as asthma, emphysema, and chronic obstructive pulmonary disease,” shares Dr. Villespin. “In fact, even that of COVID-19.”
In a diagnosed case of PAH, the most frequent or common complaint of patients is dyspnea, or “hingal” upon exertion.
“Konting lakad lang, hinihingal ka na. Fatigue is a common symptom as well. Others would complain of chest pains, swelling of the feet, fainting and dizziness,” explains Dr. Villespin.
According to Dr. Villespin, these symptoms are non-specific; that’s why PAH is so very hard to diagnose.
“The sad part is, PAH patients are asymptomatic until they reach the advanced stage. By then, the prognosis is not good. Add to that the health-seeking behavior of Filipinos. They will only consult a doctor when their health already deteriorates.”
The message is clear: Go to the doctor when you feel something isn’t right, especially if you’re at risk or have a pre-existing health condition.
“Don’t wait too long,” Villespin advises.
Pulmonary arterial hypertension is a progressive disease that is caused by the narrowing or tightening of the pulmonary arteries. When that happens, your heart has to work harder to pump blood through them. After a while the heart muscle gets weak.
THE SCARY TRUTH ABOUT PAH
Although PAH is a rare disease, true prevalence is still unknown, but it’s estimated that from 50 to 100 people per million have the condition.
According to the Philippine Heart Center’s 2009-2012 single-center study, 82 out of 104 Filipino patients were found to have PAH within this period, and it was mostly seen in those between the ages of 20 to 40.
“However, there are no exact figures that determine the current prevalence of PAH in the Philippines due to limited data,” Dr. Villespin clarifies.
PAH affects women more than men. According to the Pulmonary Hypertension Association, PAH is most often diagnosed in women between the ages of 30 and 60. It’s not known why females are more susceptible, but some theories suggest it has something to do with the role of estrogen, changes that occur during pregnancy and links to autoimmune issues.
Pulmonary arterial hypertension (PAH) is a progressive disease so it could be fatal. And while there’s still no cure, there are effective ways to manage the disease — if you get a timely diagnosis.
“Just like what I’ve said, it usually takes a year — even more — to get a correct and final diagnosis of PAH because its symptoms are non-specific,” the doctor stresses. Once the symptoms starts to manifest, patients normally consult their family physicians (general practitioners), then a specialist for asthma, rheumatism, or COPD and so on before finally seeing a pulmonologist or cardiologist who diagnoses the PAH.
“Your doctors will also need to rule out all other forms of pulmonary hypertension before giving a diagnosis of PAH,” adds Dr. Villespin. “During my time, having PAH was a death sentence. You could die in less than three years despite medications.”
The good news is that the timeline for PAH diagnosis is starting to speed up a little.
“Thanks to the availability of new drugs and the advancement of technology, we have now improved the survival rate of PAH patients,” Dr. Villespin enthuses.
And out of the 82 cases of known Filipinos who have PAH, reports showed that 69 percent survived the disease.
“This is encouraging data for me as a specialist,” he enthuses.
EARLY DETECTION & PROPER DIAGNOSIS ARE KEY
In providing patients with the right access to treatment and diagnostic resources, Dr. Villespin stresses that it is important for “us to take on a multidisciplinary lens when it comes to diagnosing and treating patients with PAH.”
“We must work together across different fields of specialization to holistically care for our patients, that we may move the needle towards prevention rather than reactive treatment,” he added at the virtual round-table discussion hosted by Johnson & Johnson Philippines on the realities of PAH in the Philippines and how we can all help prevent PAH cases from becoming critical.
Through knowledge-sharing sessions and educational roadshows, J&J aims to bring its expertise in management of PAH to Filipino healthcare practitioners and work together with local leaders and stakeholders to make diagnostic tests and proper treatment more accessible for patients.
“As a healthcare company, we recognize that we play a key role in advancing the PAH advocacy in the country,” says Dr. Erwin Benedicto, head of medical affairs at J&J.
Together with their local partners and stakeholders, J&J aims to create a future wherein PAH becomes a thing of the past.
“And wherein every Filipino will have access to the right resources and information on how they can manage and overcome their PAH symptoms,” Dr. Benedicto added.
Among the programs that J&J and the PCCP mentioned include the development of a referral system wherein specialists in the field of pulmonology, rheumatology, cardiology, and family medicine will be able to work together in ensuring treatment and proper diagnosis is made available to patients.
So if you’re experiencing symptoms of PAH such as labored breathing, chest pain, shortness of breath, coughing and fatigue, cut the chase. Visit your pulmonologist for consultation and proper diagnosis.
* * *
To know more about the PAH and the medical tests required to diagnose it, visit https://www.jnj.com/tag/pulmonary-arterial-hypertension.