OrphaCare: I have my life back.

When breathing is difficult.

The first symptoms of pulmonary hypertension (PH) often appear during childhood. Those affected suddenly lose consciousness and simply collapse. For others, it sometimes takes years for the symptoms to worsen: walking across the parking lot leaves them out of breath, and they can no longer make it up to the first floor on foot. Many patients have difficulty carrying their children or playing with them. Most are unable to carry on a conversation while out for a walk because they are out of breath.

Patient | Vice President of PH Austria | Vice Secretary of PHA Europe

I thought my symptoms might be psychological: stress, work. When I was diagnosed with pulmonary hypertension, it completely knocked me off my feet.

Incidental finding: pulmonary hypertension.

A diagnosis of PH or PAH is sometimes made by chance. But more often than not, it marks the end of a long and painful journey that even leads some patients to psychiatric facilities. They blame themselves, attributing their condition to unfounded fears or work-related stress.

A diagnosis of PH or PAH is sometimes made by chance. But more often than not, it marks the end of a long ordeal that even leads some patients to psychiatric facilities. They blame themselves, attributing their condition to unfounded fears or work-related stress.

Female Patient | Tyrol

The diagnosis of pulmonary hypertension was more or less a coincidence, because a doctor in Natters was doing her residency there at the time, and I was admitted to that hospital.

Danger recognized, danger averted?

A diagnosis of PH/PAH marks the first step on a long journey. There are treatments that provide relatively quick and effective relief for most patients. But some also experience setbacks because the medication may not work at all or may not be effective enough.

Patient | Carinthia

I tried many different medications, all of which had side effects and interactions. The doctors tried a lot of things, but I didn’t really feel well as a result. I wanted to stop one course of IV therapy, but a doctor talked me into sticking with it. Things got better very slowly, but I had to take 15 pills a day.

With pump therapy, the hoped-for improvement usually occurs: the heart returns to its normal size, and breathing becomes easier, even without supplemental oxygen. Quality of life improves.

A better quality of life, but...

Some patients still do not feel well even with a subcutaneous pump. They often experience pain when the subcutaneous needle is inserted or have trouble with the pump being attached outside the body. Showering or swimming is possible, but requires preparations such as waterproof bandages or waterproof cases for the pump. Patients with small children report pain when the child cuddles up to them or accidentally gets caught in the pump’s tubing.

Patient | Vienna

With the pump, I can now go for a walk for two to three hours. I'm used to the pump; I do have pain for a week after the injection, but ointment helps. I'm pretty pragmatic about it. The pump helps me a lot. The pain doesn't bother me much.

In pump therapy, there is the option of a subcutaneous pump, which is located outside the body, or an implantable pump. The latter is usually only used once patients are well-controlled and their vital signs (blood, heart) are within normal ranges. Due to the size of the implantable pump, it often cannot be used in very thin patients.

Patient | Vice President of PH Austria | Vice Secretary of PHA Europe

For many people, having the pump implanted is a blessing. And ever since I learned that the pump can be implanted, I'm no longer afraid of it.

PATIENT | LOWER AUSTRIA

After ten years, I wanted an implantable pump. At first, they said I was too thin. But I didn't give up.

And that was a whole new life for me. I could go swimming and take a shower on a whim. I wasn't constantly reminded of it anymore. As a result, my quality of life has improved immensely.

Extending life throughout one's lifetime.

Most patients feel better with pump therapy—whether subcutaneous or implanted. Many are able to gradually stop taking or at least reduce their oral medication. In any case, it’s important for patients on pump therapy to know how to use the device, what precautions to take, and who to contact if they need help.

Patient | Vienna

A specially trained nurse who takes care of us and gives us advice has been very helpful. You get training on how to give injections and what to do with an insulin pump. You don’t know that stuff at first. And now I’m already on my fourth pump. I also enjoy helping other patients because I’ve had this condition for so long. Anyone can call me anytime.

Navigating Life Together

Caregivers are especially important at the start of pump therapy. They provide detailed training, explain the procedures, and give patients the skills and confidence to manage the pump on their own and regain their independence. And they are always available to answer questions or assist in an emergency.

Self-help groups also play an important role. Talking with others who have lived through the disease and treatment—including its effects and side effects—helps many patients take back control of their lives.

Some patients find it easier when others are receiving the same treatment and can talk about it with them. It really helps alleviate anxiety. After all, they usually don’t know what to expect. It’s important to have someone to talk to. Many people feel better when they’re in touch with a support group.