Living with PAH: Self-Advocacy Tips for Pulmonary Hypertension Patients

A pulmonary arterial hypertension diagnosis changes everything. Suddenly, you’re navigating a complex medical condition, multiple medications, frequent appointments, and a future that looks different than you planned.

But here’s something experienced PAH patients will tell you: learning to advocate for yourself is one of the most important skills you can develop.

You know your body better than anyone. You feel the subtle changes between good days and bad days. You experience firsthand how treatment affects your daily life. That knowledge is powerful — and sharing it effectively with your medical team makes a real difference in your care.

“The patients who do best with PAH are actively engaged in their own care,” says Julia Kravtsova, PharmD, Head Patient Navigator at QuickRx Specialty Pharmacy. “They ask questions, they report symptoms, they take their medications consistently, and they don’t hesitate to speak up when something doesn’t feel right.”

Why Self-Advocacy Matters in PAH

PAH is a condition where patient input genuinely matters:

Your Symptoms Tell the Story

Unlike some conditions with obvious lab markers, PAH severity is often assessed through how you feel and function. Shortness of breath, fatigue, exercise tolerance — these subjective reports guide treatment decisions.

Treatment is Individualized

There’s no one-size-fits-all PAH treatment. Your response to medications, your tolerance of side effects, your functional abilities — all of this influences which treatments work best for you.

Early Detection of Changes Matters

Recognizing subtle changes in your condition early — and reporting them — can prompt treatment adjustments before problems become serious. You’re the first line of detection.

PAH is Complex

Managing PAH often involves multiple medications, specialists, and considerations. Being informed and engaged helps ensure nothing falls through the cracks.

Working Effectively with Your PH Specialist

Find a PH Expert

PAH is a specialized condition. Whenever possible, work with a pulmonary hypertension specialist or accredited PH care center. The Pulmonary Hypertension Association maintains a directory of accredited care centers.

Prepare for Appointments

Make the most of your time with your specialist:

• Write down symptoms: Note what you’ve experienced since your last visit, including timing and triggers
• Track your functional capacity: How far can you walk? What activities cause shortness of breath?
• List your questions: Prioritize — put the most important ones first in case time runs short
• Bring your medication list: Include everything — prescriptions, over-the-counter, supplements
• Bring a support person: They can help remember information and ask questions you might forget

Ask Questions

There are no dumb questions when it comes to your health. Consider asking:

• Why are you recommending this treatment change?
• What should I expect in terms of benefits and side effects?
• What symptoms should prompt me to call you?
• How will we know if the treatment is working?
• Are there clinical trials I might be eligible for?

Be Honest About Your Situation

• If you’ve missed doses, say so — your doctor needs accurate information
• If cost is affecting your adherence, share that concern
• If side effects are impacting your quality of life, speak up
• If you’re not following recommendations, explain why

Your medical team can only help if they know what’s really happening.

Medication Adherence: Why It’s Critical

This can’t be overstated: taking your PAH medications exactly as prescribed is crucial.

Why Adherence Matters So Much

• PAH medications work continuously to prevent disease progression
• Stopping or reducing doses can lead to rebound worsening
• Some medications require steady levels in your system
• Even when you feel well, your medications are working — feeling good doesn’t mean you no longer need them

Tips for Staying on Track

• Use pill organizers: Weekly pill boxes help you see at a glance if you’ve taken your medications
• Set alarms: Phone reminders at the same time daily build habits
• Link to routines: Take medications with consistent daily activities (breakfast, bedtime)
• Don’t run out: Reorder refills before you’re down to your last few days
• Travel smart: Bring extra medication and keep some in carry-on luggage

If You’re Struggling with Adherence

Common barriers and solutions:

• Cost: Contact your specialty pharmacy about copay assistance programs
• Side effects: Talk to your doctor — doses can often be adjusted, or alternatives explored
• Complicated regimen: Your pharmacist or nurse can help simplify schedules where possible
• Forgetting: Try different reminder strategies until you find what works

Daily Life with PAH

Activity and Exercise

Many patients worry about physical activity. Here’s what to know:

• Exercise is generally encouraged, but should be discussed with your PH team
• Supervised pulmonary rehabilitation can be beneficial
• Listen to your body — learn your limits without being afraid to move
• Avoid activities that cause severe shortness of breath, lightheadedness, or chest pain
• Heavy lifting and straining should typically be avoided

Diet and Fluid Management

• Sodium restriction is typically recommended — discuss specific limits with your team
• Fluid restriction may be necessary for some patients
• Maintain a healthy weight
• Some PAH medications have food interactions — know yours

Work and Activity Planning

• Pace yourself — spread demanding activities across the day
• Plan rest periods, especially on busy days
• Communicate with employers about reasonable accommodations if needed
• Listen to your body’s signals about when to push and when to rest

Travel Considerations

• Discuss air travel with your doctor — supplemental oxygen may be needed at altitude
• Carry medications in original containers with pharmacy labels
• Bring extra medication in case of delays
• Know how to reach your PH team when away from home
• Consider medical ID jewelry that notes your condition

Building Your Support System

Connect with the PAH Community

Other patients who live with PAH understand your experience in ways others can’t. Consider:

• Support groups: The Pulmonary Hypertension Association offers in-person and online support groups
• Online communities: Social media groups connect patients worldwide
• PHA events: Conferences and awareness events provide community connection
• Mentorship: Some organizations match newly diagnosed patients with experienced patients

Involve Your Family and Friends

• Educate loved ones about PAH — help them understand what you’re dealing with
• Be specific about what help you need
• Include a support person at important appointments
• Accept help when offered — this isn’t weakness

Know Your Healthcare Team

Your PAH care team may include:

• PH specialist (cardiologist or pulmonologist)
• Primary care provider
• Specialty pharmacy team
• Nurses and nurse practitioners
• Social workers
• Dietitians

Know who to contact for what — and don’t hesitate to reach out when needed.

Knowing Your Body and Warning Signs

Tracking Your Health

Consider monitoring and recording:

• Daily weight: Same time each day, before eating, after using bathroom
• Oxygen saturation: If you have a pulse oximeter
• Symptoms: Shortness of breath patterns, fatigue levels, swelling
• Activity tolerance: What you can do on good days versus bad days
• Medication taking: Note any missed doses

This information helps your medical team make better decisions at your appointments.

Emotional Wellbeing

Living with a chronic condition takes an emotional toll. This is normal and deserves attention.

Common Emotional Experiences

• Grief over lifestyle changes
• Anxiety about the future
• Frustration with limitations
• Fear of disease progression
• Isolation from others who don’t understand

Strategies That Help

• Talk about your feelings with trusted people
• Consider professional counseling — many PH centers have access to mental health support
• Connect with others who have PAH
• Focus on what you can control, not what you can’t
• Celebrate good days and accomplishments
• Maintain activities and relationships that bring you joy

Frequently Asked Questions

How often should I see my PH specialist?

This depends on your disease severity and stability. Newly diagnosed patients or those with recent treatment changes may be seen every few months. Stable patients might see their specialist every 6-12 months. Follow your doctor’s recommendations, and don’t hesitate to schedule sooner if you notice changes.

Can I exercise with PAH?

In many cases, yes — but discuss this with your PH team first. Supervised pulmonary rehabilitation or a guided exercise program is often recommended. Avoid high-intensity exercise, heavy lifting, or activities that cause severe symptoms. Listen to your body and stop if you experience chest pain, severe shortness of breath, or lightheadedness.

Should I get a flu shot and other vaccines?

Generally, yes. Respiratory infections can be particularly dangerous for PAH patients. The flu vaccine, pneumonia vaccine, and COVID-19 vaccines are typically recommended. Discuss your specific situation with your doctor.

What if I need surgery for something unrelated to PAH?

PAH patients face higher risks with anesthesia and surgery. Always inform any surgeon or anesthesiologist about your PAH. Your PH specialist should be involved in planning any surgical procedure. Elective surgeries should be performed at centers experienced with PH patients when possible.

Can I get pregnant with PAH?

Pregnancy carries significant risks for women with PAH. This is a complex decision that requires thorough discussion with your PH team. If pregnancy occurs, high-risk obstetric care and close PH monitoring are essential. Some PAH medications are contraindicated in pregnancy.

What should my family know about my condition?

Educate close family members about PAH basics, your medications, warning signs that need medical attention, and how to contact your medical team. Consider having advance directives in place and discussed with loved ones. Your family can be important advocates for you, especially during medical emergencies or hospitalizations.

How do I handle people who don’t understand my limitations?

PAH is an invisible illness — you may look fine while struggling inside. You don’t owe anyone a detailed explanation, but having a brief way to describe your condition can help. “I have a rare lung and heart condition that limits what I can do” is sufficient. Connect with others who have PAH for support when you feel misunderstood.

References

1. Pulmonary Hypertension Association. Patient and Caregiver Resources.
2. American Lung Association. Pulmonary Hypertension.
3. American Heart Association. Pulmonary Hypertension.
4. National Heart, Lung, and Blood Institute. Pulmonary Hypertension.
5. MedlinePlus. Pulmonary Hypertension.

This information is not intended to replace professional medical advice. Always consult your pulmonary hypertension specialist about your specific care plan. For questions about PAH medication copay assistance, contact QuickRx Specialty Pharmacy at (917) 830-2525.