She had frequent rapid heartbeats and shortness of breath for no apparent reason.
I didnt do anything about it because I didnt have time to go to the doctor.
Aright heart catheterizationrevealed that she hadpulmonary arterial hypertension(PAH), a rare form ofpulmonary hypertension.
Illustration by Julie Bang for Verywell Health
Pulmonary hypertensionis a general term for elevated blood pressure within the lungs.
In people with PAH, the walls of the arteries in the lungs become narrow and stiff.
Only somewhere between 15 and 50 persons per million across the U.S. and Europe have PAH.
Symptoms of Pulmonary Arterial Hypertension
PAH can be difficult to diagnose because early signs mimic other diseases.
Shed improve for a while, then decline again.
PAH is often a progressive disease with a high mortality, McLaughlin told Verywell.
Other patients do not respond as well and will have multiple hospitalizations and a limited life span.
The goal is to diagnose patients early and escalate therapy to improve them to the low-risk status.
I knew my breathing had changed by that night.
It felt like I was breathing more fresh air, she said.
They called me the next day just to see how it was doing.
I told them, I got the drug, and they said, You dont know that.
Once the study was unblinded, Kauffman received confirmation that she had been receiving sotatercept.
Kauffmann continued to improve with each dose she received.
I just kept getting better and better.
I took on a new normal, she said.
I was able to walk further.
That drug was officially approved by the Food and Drug Administration (FDA) late last month.
The FDAs approval of sotatercept was based on results from phase 3 of the STELLAR trial.
In contrast, Winrevair is a subcutaneous injection given once every three weeks.
This slows and prevents damage within the pulmonary arteries over time to help improve symptoms.
It may even undo some of the damage resulting from PAH.
Is Pulmonary Arterial Hypertension Genetic?
The full extent of this process is unclear.
If you asked me, I would have said he died of a rare lung disease, she said.
Kauffman is still taking Winrevair.
She continues to take three other PAH medications she was on before starting Winrevair.
Based on her success, she also encourages patients to look for clinical trials.
Pulmonary Hypertension Association.Types of pulmonary hypertension.
Pulmonary Hypertension Association.About pulmonary hypertension: the five groups.
Levine DJ.Pulmonary arterial hypertension: updates in epidemiology and evaluation of patients.Am J Manag Care.
2021;27(Suppl 3):S35-S41.
doi:10.37765/ajmc.2021.88609
American Lung Association.Pulmonary arterial hypertension symptoms and diagnosis.
National Library of Medicine: DailyMed.Winrevairsotatercept-csrk kit[drug label].
2023;388(16):1478-1490. doi:10.1056/NEJMoa2213558
