story

“Patients should be happy.”

Pump treatment can also improve quality of life.

Univ.-Prof. Dr. Irene Lang, MD, a medical specialist in internal medicine, cardiology and intensive care, is the head of the outpatient department for pulmonary arterial hypertension at University Hospital Vienna and talks about her experiences with pump treatment for PH and PAH.

We need a kind of profession of faith from our patients: I would like to take control of my life with my pump treatment, with my caregiver, with my technologies and with my medications.

Univ.-Prof. Dr. Irene Lang, MD

MEDICAL SPECIALIST | VIENNA GENERAL HOSPITAL – AKH WIEN

 

PAH develops insidiously

 

Pulmonary hypertension (PH) is a rare disease that is diagnosed once per 1 million inhabitants in Austria. The pure form, idiopathic pulmonary arterial hypertension (iPAH), particularly affects young women up to the age of 40 without concomitant diseases. With this disease, 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 transport blood to the lungs become thick and narrowed (= remodelling). Over time, the heart has greater and greater difficulties in pumping blood to the lungs. This leads to an enlargement of the right heart chamber (ventricle).

 

PAH is difficult to diagnose and usually only identified at a late stage because patients often do not have any complaints for decades and only have a few clinical symptoms. This rare disease is chronic and develops insidiously over the years.

What role do infusion pumps play in PAH?

 

PAH patients generally have a prostaglandin deficiency (also: prostacyclin, tissue hormone responsible for dilating the blood vessels, called vasodilation). This deficiency is balanced out during treatment, although prostacyclins are not taken in easily by the body in an oral form via tablets. This has led to the need for intravenous and subcutaneous pump systems (= parenteral treatment that is administered directly via the blood vessels).

Although tablets provide greater freedom in life, they do not offer as good protection at night as a parenteral infusion.

Univ.-Prof. Dr. Irene Lang, MD

MEDICAL SPECIALIST | VIENNA GENERAL HOSPITAL – AKH WIEN

Parenteral treatments are easy to control, are titratable (= can be measured drop by drop) without a dosage limit, and they have few interactions with other medications, which must be taken into consideration for oral treatments. Prostacyclins can also be combined with all other pulmonary hypertension medications. Experience shows that we have to use a combination of several active principles, which includes parenteral prostacyclin plus-minus three months.  

 

Pump treatment is the only feasible option for parenteral treatments, which – from a clinical perspective – have a higher degree of efficiency. The 24-hour application offers the chance for remodelling in the long term.

Pump treatment ...

 

  • Easy to control
  • Titratable, no dosage limit
  • Fewer medicinal product interactions

... and challenges

 

The biggest challenge is the parenteral application, which must take place via a subcutaneous (= under the skin) catheter line. Side effects can occur, for instance prostacyclin causes skin redness.  

 

And there is a general misunderstanding in pulmonary hypertension treatment: Many patients believe that parenteral treatment involves taking on a burden that is not actually necessary. After all, there are also tablets. However, the illness is usually so serious that people need both tablets and parenteral treatment. The tablets are not intended as a replacement.

In medicine, three months are important. Three months is the time the heart needs after an infarction to remodel itself. Remodelling can happen in three months, if it happens. If it doesn’t happen, those providing treatment need to be ready for a fight.

Univ.-Prof. Dr. Irene Lang, MD

MEDICAL SPECIALIST | VIENNA GENERAL HOSPITAL – AKH WIEN

Experience shows that, if a clinical deterioration takes place within three months after the start of treatment patients are likely to pass away earlier.

Pump therapy is teamwork

 

The first important aspect of pump treatment is: “Are my patients happy with their treatment?” Patients who are not happy turn the pump off. We usually then have to find another solution and these patients generally have poorer chances of survival. However, patients should be happy, that’s the first thing.

 

The second important aspect is regular control and self-management. Patients need to be able to operate and understand the pump. They must maintain high hygiene standards, know their dosage, order their medications, and know about their reservoirs. A management function is necessary for this. Patients have to experience a certain medical learning curve here. They need to understand what it means when they’re short of breath, go red in the face, or go pale.  

 

This sometimes isn’t possible for older patients. They are in need of a nurse or carer. And this person then also has to be there, care is everything. It involves joining a community of parenteral therapists and patients. It is a profession of faith: I would like to take control of my life with my pump treatment, with my caregivers, with my technologies, and with my medications.  Some people do that completely independently, order their things themselves and manage everything. Others need institutionalised, confidence-inspiring, stable support. Nothing is worse for this group than when their support caregivers change. This is a catastrophe that often leads to treatment being discontinued.

 

The third aspect is contact with the centre. The centre is not always synonymous with what’s known as the “pump treatment profession of faith”. The centre is superordinate to that, controls, and offers access to the latest treatments. Something will also always change here because we are constantly searching for new things.

What doctors should pay attention to

 

  • Happy patients 
  • Regular control and self-management 
  • Continuous contact with the centre 

What patients should pay attention to

 

  • Maintain hygiene standard
  • Know about dosage and reservoirs
  • Management function
Care is everything.

ALWAYS ON THE JOB AS A CAREGIVER

Read here our

Caregiver Story

For whom is pump treatment not suitable?

 

  • Patients with drug-induced PAH within the scope of drug abuse
    After all, the basic prerequisite for such a complete treatment is a well-established social structure. Sometimes it’s not possible to install this kind of treatment. This is the biggest challenge for me because this kind of treatment is often not an option at all for very young patients.  
  • Parenteral treatment is also difficult for very old patients, although they are disciplined and easy to guide by a caregiver.
We need state research funding and collaborations with industry: for all those who want to conduct research into pulmonary artery diseases with good concepts and clinical trials. We need innovative research concepts to conduct clinical trials.

Univ.-Prof. Dr. Irene Lang, MD

Medical Specialist | Vienna General Hospital – AKH Wien

Research & innovation