Hereditary angioedema (HAE) – Relevant information helps 

July 16, 2019

With its innovative approach, intermedix provides doctors with the right information at the right time so they can detect rare diseases sooner. Based on numerous individual parameters, a digital assistant in the Physician Information System finds certain combinations or patient groups, actively helping doctors with useful scientific content. The aim is to highlight possible correlations for doctors while providing them with all relevant information in the busy surgery setting.

Together with Dr. Ingo Wolf from Shire (now part of Takeda), intermedix has developed a digital assistant like this for the condition hereditary angioedema (HAE), a rare, genetic disorder. Common signs of HAE include recurrent swellings (oedemas) of the skin in various regions of the body, the respiratory system and internal organs. The biggest challenge is that HAE’s non-specific symptoms often prevent an early diagnosis.

Mr Wolf, why is it so difficult to diagnose HAE?

Recurrent swellings affecting the arms, legs, face, gastro-intestinal tract or even in the pharynx are common signs of HAE but are not conclusive. These symptoms are often confused with an allergy, gastroenterological or gynaecological diseases, delaying the right diagnosis for years. In Germany, it still takes around nine years to diagnose an HAE sufferer. An early diagnosis is crucial for patients since swelling attacks in the laryngeal region can be fatal. Skin oedemas are not life-threatening but can be so painful and disfiguring that patients suffering frequent acute HAE attacks tend to be socially withdrawn. There is at present no cure for patients suffering from HAE attacks. However, there are effective treatments that can interrupt or prevent swelling attacks. An enormous relief for sufferers.

How can information help identify sufferers?

We’re involved in various initiatives aimed at providing information on HAE and raising awareness. These include classic communications activities such as dedicated websites, presentations and cooperation with patient associations. Providing patients and doctors with information is important but is extremely challenging. Not least because of the small number of sufferers - at present we estimate around 1,600 people have HAE in Germany. And then this information relates to large doctor groups such as general practitioners or gynaecologists treating myriad conditions and receiving vast quantities of information every day. But the non-specific symptoms make it difficult to keep this condition at the front of doctors’ minds and make the diagnosis more difficult.

Why is communication at the decisive moment so important for you?

Well, HAE has non-specific symptoms unfortunately. But correlating certain information on symptoms does provide relevant clues to a possible condition. A blood test can start the process in the surgery. But then you need a specialised centre to come up with a precise diagnosis.

Based on information from a possible suspected case of HAE, intermedix has compiled a set of triggers. These triggers are then mapped in the Physician Information System. Once the doctor opens and edits the patient file, the digital assistant responds to these specific triggers. A window opens that provides the medical practitioner with information on HAE, the correct diagnosis, specialised centres, and possible treatment options.

No other information channel is this close to the patient and doctor.

Dr. Ingo Wolf, Franchise Head Immunology/HAE Germany, Shire Deutschland GmbH, now part of the Takeda Group

What convinced you to add this channel to your communications activities?

No other information channel is this close to the patient and doctor. You can give presentations at conferences, publish articles and run advertisements in major daily newspapers. Those are important channels for raising overall awareness. Yet we never know whether this information will be available when it’s needed.

What experience have you had so far with our communication for rare diseases?

We started the project 3 years ago and managed to demonstrably identify HAE sufferers in this way. That was a huge achievement particularly for the patients and their doctors. Over the past 3 years we’ve managed to double the HAE diagnosis rate overall. While the assist certainly helped, all the other campaigns aimed at raising disease awareness were just as important.

About Dr Ingo Wolf and Shire Deutschland GmbH (now part of the Takeda Group)

Dr Ingo Wolf, Franchise Head Immunology/HAE Germany, Shire Deutschland GmbH, now part of the Takeda Group

54 years old, working in biotechnology, over 20 years’ pharmaceutical sales experience in various roles. Joined Shire almost 9 years ago and works on rare diseases such as HAE, Hunter syndrome, Gaucher disease and Fabry disease.

About Shire Deutschland GmbH, now part of the Takeda Group

Takeda is a values-based, R&D-driven global biopharmaceutical company. Together with Shire Deutschland GmbH, which became part of the Takeda Group on 8 January 2019, Takeda strives to bring Better Health and a Brighter Future to people worldwide through groundbreaking medical innovations.

Further information on the Takeda Group