Short summary

This research project involves a small patient group, with major challenges in the Danish hospital system and the patient's quality of life. The project has combined two areas, each of which has proven to be challenging for the patients: Having a different ethnic background than Danish and having a rare diagnosis, which in this project is the disease hereditary angioedema (HAE).

Rationale

Hereditary angioedema (HAE) is a rare but potentially life-threatening disease characterized by episodic spontaneous swelling of skin and mucous membranes. Symptoms can be triggered by physical trauma, mental stress, hormonal factors or infections, and do often affect the face, extremities or intestinal mucosa. The swelling is not itchy but may be painful but when it is located in the airways, the condition is potentially fatal. The estimated minimum prevalence in Denmark is 1.41 / 100.000 inhabitants. The Danish national comprehensive care Centre has been established at Odense University Hospital (OUH), where the country's HAE patients are followed and offered tailor-made treatment based on international standards to optimize the effectiveness and safety of the treatment, as well as to improve individual health-related quality of life (HRQoL). 

Many patients suffer from the unpredictable nature of the acute and potentially life-threatening attacks, which reduces their quality of life, even among patients with relatively few attacks. A low HRQoL has been found in many patients with HAE, and a Danish study showed that more than half of HAE patients experienced that the disease had psychological and physical constraints in their daily lives. However, it has also been shown that patients who have access to treatment and regular visits to a specialist have the same quality of life as the general population, stressing the need for a tailored treatment program and regular monitoring of quality of life. The individual assessment of their own quality of life can be put into perspective to the patient's own background and life experience, disease perception and cultural values

Over the years, there have been more immigrants coming to Denmark suffering from HAE, and they are primarily treated as the ethnic Danish HAE patients. There is a total of 18 patients from a total of 6 different countries (Germany, Portugal, Hungary, Turkey, Syria and Pakistan). Their perceptions of illness and perception of quality of life may be different than among ethnic Danes, and it is also the opinion of the HAE experts that these patients are sometimes treated differently (read: worse) at the local emergency rooms and related functions, e.g. patient transportations. The impression is also that they are less likely to use supportive features such as the HAE App for smartphones and rarely participate in events through the HAE Patient Association.

Immigrants generally have poorer access to healthcare because of language barriers, a limited social net-work and poor understanding of health and how the body works, often due to lack of educational opportunities. How an immigrant family affected with illness, experiences the contact with health services plays a crucial role, because vulnerability of one family member can be "transmitted" to other family members. The difference between the family member's expectations and needs and the society's expectations brings the family in ethical and moral dilemmas. This divides and stresses the family sulting in difficult decision, increased stress and vulnerability. These vulnerable patients with a rare disease have double trouble.

The aim of the project is to illustrate how HAE patients with a different ethnic background experience living with HAE in Denmark. The patient group's perception of disease and quality of life must be highlighted as well as their views on treatment and how they were received in the Danish hospital system. It is expected that the results are transferable to similar groups of patients with rare diseases. Furthermore the results will identify the main problems for this group of vulnerable patients so the problems can be improved making the acces to the Danish health care system easier and making them feel better treated.

Description of the cohort

The patients are recruited form the department's national HAE center and are selected on the basis of their diagnosis (HAE) and ethnicity (other than Danish). The patients are given oral and written information about the project and are subsequently asked if they want to participate in the project.

The entire project is based on the patient's own experiences and handling of the disease in everyday life and in contact with the Danish hospital service. All the involved patients have an important share in the completed data sets and the results from the study

Data and biological material

The project is designed as a qualitative study, where semi-structured interview is chosen to ensure a deeper and broader understanding of how patients of other ethnic backgrounds experience, understand and manage HAE in their everyday lives. In addition to the interview, project participants are asked to complete two questionnaires (EQ-5D and HAE-QoL), both of which support the patient's quality of life.

Collaborating researchers and departments

Department of Dermatology, Odense University Hospital

• Professor Anette Bygum, MD
• Clinical nurse specialist Jeanette M. Hansen, SD, MKS

Migrant Health Clinic, Odense University Hospital

• Associate Professor Dorthe S. Nielsen, Docent