Building Your Pulmonary Arterial Hypertension Care Team

• Pulmonary arterial hypertension (PAH) can impact many parts of the body, so you may need a health care team with specialists for your various symptoms.
• You are the most important person on your PAH team. Learn as much as you can about the condition, and identify your treatment goals to get the best care.

Pulmonary hypertension (PH) refers to high blood pressure in the arteries that deliver blood from the right side of the heart to the lungs. There are several types of PH associated with different causes. Pulmonary arterial hypertension is a type of PH caused by the narrowing of the pulmonary arteries that bring blood from the heart to the lungs. PAH can be caused by genetic factors, exposure to certain drugs or toxins, or another disease.

The symptoms of PH, including PAH, include trouble breathing, chest pain, discomfort, fatigue, “brain fog,” swelling, and changes in skin color. Though PAH is a lung disease, it can also cause some types of heart disease and heart failure.

Various medical conditions and factors can cause the narrowing of arteries in the lungs, leading to PAH. These include:

• Exposure to toxins
• History of drug use
• HIV and other infections
• Connective tissue disorders
• Lupus
• Liver disease
• Congenital heart disease
• Genetic causes

PAH can also be idiopathic in some people, meaning there is no known cause for it.

Since PAH can be caused by different systems in the body and has wide-reaching impacts on many aspects of life, it’s important to have a team of multidisciplinary medical professionals to effectively manage your PAH.

PAH can also impact your mental health and social life. Your care team should include people who can help you navigate the emotional and social struggles of living with a chronic health condition.

Consider talking with the professionals below to create a team that provides the most informed care for your PAH.

Medical Specialists

Some doctors specialize in treating PAH and other types of PH. A doctor who is knowledgeable about PAH can teach you more about your condition, prioritize helpful tests, and work with other specialists to manage your symptoms. Doctors who specialize in PAH come from a variety of specialty backgrounds, including pulmonology (lung specialty) and cardiology (heart specialty).

Medical doctors specialize in specific systems of the body and can help inform your care. Here are some of the doctors who might be on your team:

• Cardiologists specialize in treating the cardiovascular system, or the heart and blood vessels.
• Pulmonologists study the respiratory system, including the lungs.
• Rheumatologists treat issues with the joints and muscles. If your PAH stems from connective tissue disease, it is especially important to have a rheumatologist on your team.
• Hepatologists manage liver problems. If liver disease caused your PAH, a doctor specializing in liver care is a helpful asset.
• Infectious disease specialists treat diseases caused by pathogens like viruses and bacteria. Infection with HIV, the virus that causes AIDS, can also cause PAH and needs to be managed with specific care.
• Hematologists diagnose and treat issues with blood, such as blood clots lodged in the pulmonary arteries that can cause PAH.
• Transplant physicians manage the medical care, tests, and medications for people awaiting transplant surgery, such as those receiving a lung transplant to treat their PAH.

Which specialists you include on your care team will depend on the causes of your PAH and any other health concerns you may have. The primary doctor managing your PAH can refer you to specialists.

Palliative Care Professionals

Palliative care focuses on relieving the symptoms caused by chronic illnesses like PAH. The goal is to improve quality of life, as opposed to treatments intended to cure a condition. Palliative care is not only for people at the end of life. In fact, palliative treatments can help most when they are started early in the course of PAH. Palliative care can occur alongside other types of treatments.

Palliative care options for PAH can include surgeries to treat the symptoms of PAH or noninvasive care like the management of pain, nausea, and shortness of breath. PAH also takes an emotional toll on people it affects and their loved ones. Palliative care specialists include social workers and pastoral care workers, as well as referrals to support groups and counseling to help you manage the anxiety and depression associated with PH.

Occupational Therapists

Practitioners of occupational therapy help people modify their habits and environments so they can be as independent as possible. For people with PAH, everyday activities can feel difficult and lead to fatigue, shortness of breath, chest pain, or dizziness.

Occupational therapists help people develop and maintain the skills they need for everyday work and life. Occupational therapists often focus on simplifying daily activities, like getting dressed, or helping modify a home environment so tasks like cooking are easier.

Physical Therapists

Physical therapists have expertise in physical activity. They often help people with health conditions or injuries perform exercises that will help improve their physical functioning. Though physical activity may feel overwhelming when you have PAH, exercise can also help improve symptoms and quality of life.

A review of more than 20 studies on people with PAH found that regular exercise correlated with less right ventricular hypertrophy (increased size of right ventricle) and a higher quality of life. Another review of 17 studies also found that regular exercise for people with well-managed PH was safe and improved quality of life.

Always ask your doctor before beginning or changing an exercise routine. Your doctor can refer you to a physical therapist who can help you get on track to improve your fitness and manage PAH.

Dietitians and Nutritionists

People with PAH tend to have a higher incidence of some nutrient deficiencies, including vitamin D, vitamin C, and iron. Salt and sodium consumption can also worsen hypertension, so people with PAH must carefully manage their diet. Dietitians and nutritionists can create meal and nutrition plans to address your dietary needs for managing PAH.

Pregnancy Specialists

People with PAH have a high risk of mortality related to pregnancy. The World Health Organization (WHO) recommends discussing terminating a pregnancy if the mother has PAH. People with more severe PAH have a higher risk of mortality from pregnancy than those with lower disease severity.

Though PAH complicates pregnancy, people with PAH may still decide to have a baby. For these people, it is important to have a care team, including an OB-GYN and high-risk pregnancy specialist, who understands the risks of PAH during pregnancy.

Your Role in Your Care

Effectively managing PAH and achieving your goals requires you to be engaged and active in your care. People who are active in their care:

• Learn more about their health condition
• Develop better communication skills to talk with their doctors
• Improve their self-care
• Are involved in decisions about their PAH treatment

Working with the professionals on your PAH team and joining support groups can help you learn about PAH, ask important questions, make informed decisions about treatment plans, and work toward your goals.

One observational study of more than 40,000 people across 44 clinics in Minnesota found that more people who were more active in the management of their health care had better health outcomes and lower health care costs. Though this study was not specific to people with PAH, it highlights the importance of being active in your own care.

If you’re having trouble communicating with your doctor, learn more about how to connect with your doctor about PAH.

Building your PAH care team may feel overwhelming, but reading this article means you are already taking steps to learn about PAH. Your doctor can give you referrals to specialists for any specific issues you have. You can also find pulmonary hypertension care centers with multidisciplinary teams that specialize in treating PAH.

The Pulmonary Hypertension Association (PHA) offers accreditation to pulmonary hypertension centers that meet high standards for the quality of care they provide. A PHA-accredited Center of Comprehensive Care must have a multidisciplinary team of specialists prepared to tackle all aspects of PH care. More information about these centers can be found on the PHA website.

Talk To People Who Understand

Joining myPHteam is a great way to learn about PAH and find support as you build your PAH team. This social network for people with pulmonary hypertension and their loved ones has more than 41,000 members who come together to ask questions, give advice, share their stories, and support others.

Share your experience with building your pulmonary hypertension team in the comments below, or share your story on myPHteam.