Understanding PAH progression and management

Pulmonary arterial hypertension (PAH) is a rare disorder that causes high blood pressure in the lungs. When a person has PAH, cells that make up the inner lining of the blood vessels in the lungs stop functioning normally. These changes cause the blood vessels to become narrow, thickened, and inelastic, making it more difficult for the heart to pump blood through the lungs.

PAH is a progressive condition, meaning it is a condition that becomes more severe over time. Early in the course of the disease, a person may not notice any symptoms, or they may feel shortness of breath during activity or have a reduced capacity for exercise.

As the disease progresses, it can lead to heart failure, it can significantly limit a person’s ability to perform everyday activities, and it can lead to potentially life-threatening complications, including right-sided heart failure.

If you or a loved one is living with PAH, here are a few things to know about disease progression.

Regular assessments are a critical part of care

Signs and symptoms of disease progression can be subtle and easy to miss for a person with PAH and caregivers. Even as changes occur in the lungs or the right-side of the heart, a person may not experience worsening symptoms or a noticeable difference in how they feel day to day.

Regular assessments by a PAH healthcare team can identify any changes as early as possible. Regular assessments also allow a healthcare team to monitor how the disease is responding to treatment and, if necessary, make adjustments to a treatment plan.

Appointments may be recommended every few months, but how often you or your loved one will need to come in for appointments is a topic to discuss with your healthcare team. Also ask what to expect at appointments and how PAH will be assessed. Physical exams, blood work, breathing tests, echocardiogram, and a six-minute walk test are commonly used.

If you do notice any changes, it’s important to report these to a healthcare team as soon as possible.

Progression is often measured with “risk stratification”

Regular assessments allow your healthcare team to collect data about PAH and how it is affecting your health. This data is then used to calculate a score and a risk category, which refers to the risk of dying or significant disease worsening within the next year. This process is known as risk stratification.

Risk status is used to guide treatment, and risk status can improve with treatment. Achieving and maintaining “low risk” status is often used as a treatment goal. Risk status is an important topic to discuss with a healthcare team, who can explain in detail what you or your loved one’s risk status means and how it was calculated.

Treatment will need to evolve to keep up with progression

There is no cure for PAH, and treatment is goal oriented, focused on reducing symptoms, preventing complications, maintaining quality of life, and aiming for a low-risk status. The therapies required to meet these goals can change as the disease progresses.

Medications are the main treatment. Early in the disease course, a person may be prescribed one or two oral medications. If symptoms and function do not improve or progress, they may need to add additional medications, take higher doses, or add medications that are administered in different ways, such as adding a medication that is given as an infusion. Some people also require diuretic medications to manage edema (fluid buildup caused by heart failure) and oxygen therapy.

In severe cases that are no longer responding to medications, a healthcare team may discuss lung or heart-lung transplants.

It’s important to remember that needing to add treatments, switch treatments, or change treatments is not a sign of personal failure. Whenever possible, it is recommended to increase treatment before the disease progresses.

Follow your treatment plan, pay attention to how you feel, ask for help when you need it, and work with your healthcare team.