Patients should be happy.

Prof. Irene Lang, MD, a specialist in internal medicine, cardiology, and critical care medicine, is the director of the Pulmonary Hypertension Outpatient Clinic at Vienna General Hospital (AKH) and discusses her experience with pump therapy for PH and PAH.

PAH Develops Gradually

Pulmonary hypertension (PH) is a rare condition diagnosed in one person per million residents in Austria. The primary form, idiopathic pulmonary arterial hypertension (iPAH), primarily affects young women up to age 40 without comorbidities. In this condition, patients have above-average blood pressure in the pulmonary arteries (≥ 25 mmHg; the normal value is below 20 mmHg). As a result, the walls of the arteries that carry blood to the lungs thicken and narrow (= remodeling). Over time, the heart has increasing difficulty pumping blood to the lungs. This results in enlargement of the right ventricle.

PAH is difficult to diagnose and is usually not detected until a late stage because patients often have no symptoms for decades and exhibit only a few clinical signs. This rare disease is chronic and develops gradually over the years.

What role do infusion pumps play in PAH?

PAH patients generally have a deficiency of prostaglandins (also known as prostacyclins; tissue hormones responsible for the widening of blood vessels, known as vasodilation). Treatment aims to compensate for this deficiency; however, prostacyclins are poorly absorbed by the body when taken orally in tablet form. This has led to the need for intravenous and subcutaneous pump systems (= parenteral therapy, which is administered directly into the bloodstream).

Parenteral therapies are easily controlled; they can be titrated (= administered drop by drop) without a dose limit, and they have few interactions with other medications—a factor that must be considered with oral therapies. Prostacyclins can also be combined with all other pulmonary hypertension medications. Experience shows that we must use a combination of several therapeutic approaches, including parenteral prostacyclin for approximately three months.

Pump therapy is the only viable option for parenteral therapies, which are perceived—clinically—to have a higher degree of efficacy. The 24-hour administration offers the long-term potential for remodeling.

Pump therapy ...

• Easily controllable

• Titrable, no dose limit

• Few drug interactions

... and challenges

The biggest challenge is parenteral administration, which must be administered via a subcutaneous (under the skin) catheter. This can lead to side effects; for example, prostacyclin causes skin redness.

And there is a common misconception regarding pulmonary hypertension therapy: Many patients believe that parenteral therapy imposes a burden they don’t necessarily have to bear. After all, there are also pills. But in most cases, the disease is so severe that patients need both pills and parenteral therapy. The pills are not intended as a substitute.

Experience shows that if clinical deterioration occurs within three months of discontinuing therapy, it is associated with a loss of survival years.

Pump Therapy Is a Team Effort

The first important aspect of pump therapy is: “Are my patients happy with their therapy?” Patients who aren’t happy turn off the pump. In most cases, we then have to find another solution, and these patients usually have poorer survival outcomes. Patients should be happy—that’s the first thing.

The second important aspect is regular monitoring and self-management. Patients must operate and understand the pump. They must adhere to hygiene standards, know their dose, order their medications, and be aware of their reservoirs. This requires a management function. Patients have to go through a certain medical learning curve. They need to understand what it means when they’re short of breath, have a flushed face, or look pale.
This is sometimes not possible for older patients. They need a nurse or a caregiver. And that person must then also be there—care is everything. It’s an entry into a community of parenteral therapists and patients. It’s a statement of faith: I want to take control of my life through my pump therapy, my caregivers, my technologies, and my medications. Some do this completely independently, ordering their supplies themselves and managing everything. Others need institutionalized, trustworthy, and stable care to do so. Nothing is worse for this group than when their care provider changes. That’s a disaster that often leads to treatment discontinuation.

The third aspect is contact with the center. The center is not always synonymous with the so-called “creed of pump therapy.” The center oversees the program, provides guidance, and grants access to the latest therapies. Things will always be changing here, because we’re constantly on the lookout for new developments.

Care is everything.

Always on the job as a caregiver.

Read our caregiver story here

Who is pump therapy not suitable for?

• Patients with drug-induced PAH resulting from substance abuse

  This is because a well-established social support system is a fundamental prerequisite for such a complex therapy. Sometimes it’s impossible to implement this kind of therapy. For me, this is the biggest challenge, because very young patients are often not even eligible for this type of treatment.

• Parenteral therapy is also difficult for very elderly patients, who, on the other hand, are disciplined and can be well managed by a caregiver.