September is Sickle Cell Awareness Month and National Pulmonary Fibrosis Awareness Month, an important time to recognize the connection between associated conditions and pulmonary hypertension. An associated condition is a disease that increases a person’s risk of developing PH, such as scleroderma, sickle cell disease or interstitial lung disease.

When someone has an associated condition and develops PH, it can add another layer to an already complicated health journey. Recognizing PH and beginning treatment earlier can give patients and their care teams more time to manage the condition before it progresses and to address contributing health conditions.

To help patients navigate this complex journey, Oksana Shlobin, director of the pulmonary hypertension program at Inova Fairfax Hospital and chair of PHA’s PHCC Oversight Committee, discusses the connection between associated conditions and PH, early detection and the importance of effective communication. Inova Fairfax is a Pulmonary Hypertension Association-accredited PH Care Center.

How can people living with an associated condition distinguish between its symptoms and emerging PH symptoms?

Each case is different, and PH symptoms can present differently depending on the type of associated condition someone already has. People with interstitial lung disease may already experience shortness of breath and fatigue, so if they develop PH, those symptoms may worsen.

When someone with a connective tissue disease such as lupus or scleroderma develops PH, they might experience more symptoms related to their autoimmune disease rather than respiratory symptoms. These symptoms can include swelling in their joints and muscle pain. They might also experience fatigue, but it may be caused more by the preexisting condition than by PH.

There is no clear-cut pattern that doctors can follow for every patient. That’s why it’s important to tell your care team about new or worsening symptoms so they can evaluate the changes and determine what may be causing them.

Why is PH more difficult to diagnose when someone has an associated condition?

If someone has an associated condition that already affects their lungs, it can be difficult to distinguish between symptoms of that condition and symptoms of PH. Often, symptoms of associated conditions and PH overlap, so doctors look for new or worsening symptoms and other changes over time.

Patients might also experience new symptoms caused by medication side effects rather than PH. Providing details about when symptoms began and how they have changed over time can help your care team identify potential causes and assess whether the condition is progressing. It’s like putting together a puzzle, with each new symptom or change providing another clue.

Additionally, people with some associated conditions have been underrepresented in PH clinical trials, so there is still a lot to learn about how treatments affect these patients.

Why is early detection of PH important?

The sooner PH is diagnosed, the sooner you and your care team can develop a treatment plan. When a patient experiences new or worsening symptoms, they may gradually adjust their daily routine without realizing how much their health has changed over time.

For example, you might have been able to exercise or stay active six months ago, but now you may avoid physical activity because you are frequently short of breath. Changes like these are important to discuss with your care team, even if you have become accustomed to them.

As activity levels decrease, it can become harder to maintain or build endurance. You may notice that you can’t walk as far as you once could or that everyday activities leave you more fatigued. These changes can also affect measurements your care team uses to monitor your health, such as the distance you can walk during a six-minute walk test or your oxygen saturation levels.

Communication is important between you and your doctor, as well as among all members of your care team. Keeping everyone informed about changes in your symptoms, activity, levels, and treatment can help your care team coordinate your care and develop a clear long-term plan.

Once diagnosed, you should seek care from an experienced PH specialist or accredited PH care center when possible. Treatment looks different for each person, but the earlier you tell your care team about new or worsening symptoms, the sooner they can evaluate those changes and determine whether your care plan needs to be adjusted.