Hereditary hemorrhagic telangiectasia: Documentation of treatment and the course of disease in an “Osler-Calendar”

Introduction:

Hereditary hemorrhagic telangiectasia (HHT; Morbus Osler) is a disease affecting capillary vessels, leading to arteriovenous shunts. Predominantly these shunts can be found in the nasal mucosa, in the intestine, lung, liver and cerebrum. Severe epistaxis causes complaints in everyday life and is the leading symptom. The aim of an “Osler-Calendar” is to inform patients and attending physicians about HHT and additionally to update them on the extent of the systemic disease and the state of the treatment.

Methods:

Being part of the center of orphan diseases our ENT clinic at the University of Regensburg provides treatment for more than 100 HHT patients suffering from epistaxis per year. A multistage concept includes Nd:YAG laser therapy, surgery as well as drug therapies. In addition, a screening to determine the affection of internal organs is carried out. Lesions are treated in interdisciplinary cooperation with the corresponding specialist department.

Results:

A calendar, including an emergency card, information on HHT, advise about self-treatment of epistaxis and antibiotic prophylaxis, was created. Furthermore, organ-screenings are recorded and the ongoing treatment is documented. A questionnaire records treatment success. This includes among others patient satisfaction, frequency of hemorrhage and current Hb value.

Conclusions:

The “Osler-Calendar” provides information about the rare disease HHT and offers answers to frequent questions. It records the individual intensity of the disease and facilitates the communication between attending physicians. The “Osler-Calendar” is a tool for ENT specialists to review treatment strategies and it enhances the patients' comprehension of their condition beyond the main symptom of epistaxis.

Publication History

Publication Date:
18 April 2018 (online)