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Observational Study
. 2017 Jan 23;15(1):19.
doi: 10.1186/s12955-017-0586-z.

Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)

Roberto Zarrabeitia  1 , Concepción Fariñas-Álvarez  2 , Miguel Santibáñez  3 , Blanca Señaris  4 , Ana Fontalba  5 , Luisa María Botella  6 , José Antonio Parra  7
Affiliations
Observational Study

Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)

Roberto Zarrabeitia et al. Health Qual Life Outcomes. .

Abstract

Background: There are very few studies about general quality of life parameters, standards for the description of health status and comparison with general population data on patients with Hereditary hemorrhagic telangiectasia (HHT), a rare disease in which epistaxis is a cardinal symptom.

Purpose: To assess the quality of life in a population of Spanish patients with HHT and compare it with the general population.

Design and methods: Between January 1st 2005 and December 31st 2013, 187 adult patients diagnosed with HHT who were admitted to the HHT Unit of the Hospital Sierrallana, completed on their first visit, the EuroQol 5D-3L (five dimensions and three levels) quality of life descriptive test and the visual analog scale (VAS). The numerical social index value was also determined and the subjective effect of the nasal epistaxis on their quality of life was estimated classified as mild, moderate or severe.

Results: Patients with HHT had greater problems than the general population in the five dimensions of the EuroQol 5D-3L, particularly considering pain/discomfort and anxiety/depression. In the VAS and the social index value, patients with HHT also scored lower than the general population, particularly older patients, males, and patients with HHT2. They also had values similar to those of populations with chronic illnesses. The subjective perception of the severity of epistaxis correlated strongly with the VAS and social index values.

Conclusions: The quality of life of patients with HHT, estimated using the EuroQol 5D-3L scale, is affected across all dimensions. The scores are similar to those seen in cases of other chronic diseases. Older patients, males and the carriers of the ACVRL1 mutation generally have worse scores on these scales. The VAS and the social index value are index that correlate well with the severity of the clinical symptoms associated mainly with epistaxis.

Keywords: Euroqol; HHT; Hereditary hemorrhagic telangiectasia; Qol; Rendu Osler.

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Figures

Fig. 1
Fig. 1
Distribution of HHT patients sorted by age and sex
Fig. 2
Fig. 2
Comparison of percentages that report problems in each of the dimensions between HHT and Spanish health survey
Fig. 3
Fig. 3
Distribution of VAS score and social index regarding age
See this image and copyright information in PMC

References

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