IDEAS home Printed from https://ideas.repec.org/a/gam/jijerp/v19y2022i11p6827-d830800.html
   My bibliography  Save this article

Health-Related Quality of Life of Patients with Marfan Syndrome—Polish Study

Author

Listed:
  • Agnieszka Trawicka

    (Department of Pediatric Cardiology and Congenital Heart Diseases, Medical University of Gdansk, 80-211 Gdansk, Poland
    Department of Psychology, Gdansk University of Physical Education and Sport, 80-336 Gdansk, Poland)

  • Aleksandra Lewandowska-Walter

    (Institute of Psychology, University of Gdansk, 80-339 Gdansk, Poland)

  • Mikołaj Majkowicz

    (Institute of Health Science, Pomoranian Academy in Słupsk, 76-200 Słupsk, Poland)

  • Robert Sabiniewicz

    (Department of Pediatric Cardiology and Congenital Heart Diseases, Medical University of Gdansk, 80-211 Gdansk, Poland)

  • Lidia Woźniak-Mielczarek

    (Department of Pediatric Cardiology and Congenital Heart Diseases, Medical University of Gdansk, 80-211 Gdansk, Poland)

Abstract

Background: Despite extensive knowledge about the quality of life of people suffering from rare diseases, data on patients with Marfan syndrome (MFS) are scarce and inconsistent. Hence, the problem of assessing the quality of life (QOL) and its relationship with the assessment of which ailments are the most burdensome for these patients is still open. Aim: Comparison of the quality of life of patients with MFS and determination as to which of the reported complaints in patients with MFS are related to the QOL of patients. Methods: The study included 35 patients with MFS and 35 healthy controls, matched for gender and age. In the study, the questionnaire of quality of life assessment SF-36 was used to assess the level of health-related quality of life, as well as an interview of the most severe symptoms reported by patients with MFS. Results: The level of the physical dimension of the QOL ( p < 0.001) and limiting of roles due to physical health ( p = 0.002), as well as the level of general index of the QOL ( p < 0.001), were statistically significantly lower in MFS patients when compared to controls. People from both studied groups do not vary in the scope of pain, vitality, social functioning, limiting the roles due to emotional problems, and state of mind but also in the mental dimension of the health-related quality of life (HRQL). Additionally, there has been a correlation between HRQL and the subjective assessment of the effects of orthopedic, ophthalmic, and cardiological problems in life, as well as lower exercise tolerance in the evaluation of people with MFS and QOL in most areas. Conclusions: Patients with MFS present a reduced QOL in the areas of physical functioning, limiting roles due to physical health, general feeling of general health, the physical dimension of the HRQL, and the general index of the QOL; in these areas, they require careful evaluation, as well as medical and psychosocial assistance.

Suggested Citation

  • Agnieszka Trawicka & Aleksandra Lewandowska-Walter & Mikołaj Majkowicz & Robert Sabiniewicz & Lidia Woźniak-Mielczarek, 2022. "Health-Related Quality of Life of Patients with Marfan Syndrome—Polish Study," IJERPH, MDPI, vol. 19(11), pages 1-12, June.
  • Handle: RePEc:gam:jijerp:v:19:y:2022:i:11:p:6827-:d:830800
    as

    Download full text from publisher

    File URL: https://www.mdpi.com/1660-4601/19/11/6827/pdf
    Download Restriction: no

    File URL: https://www.mdpi.com/1660-4601/19/11/6827/
    Download Restriction: no
    ---><---

    References listed on IDEAS

    as
    1. Giuseppina Laganà & Nicolò Venza & Arianna Malara & Claudio Liguori & Paola Cozza & Calogera Pisano, 2021. "Obstructive Sleep Apnea, Palatal Morphology, and Aortic Dilatation in Marfan Syndrome Growing Subjects: A Retrospective Study," IJERPH, MDPI, vol. 18(6), pages 1-10, March.
    2. Robyn Lewis Brown & Anne E. Barrett, 2011. "Visual Impairment and Quality of Life Among Older Adults: An Examination of Explanations for the Relationship," The Journals of Gerontology: Series B, The Gerontological Society of America, vol. 66(3), pages 364-373.
    Full references (including those not matched with items on IDEAS)

    Most related items

    These are the items that most often cite the same works as this one and are cited by the same works as this one.
    1. Audun Brunes & Marianne B Hansen & Trond Heir, 2021. "General self-efficacy in individuals with visual impairment compared with the general population," PLOS ONE, Public Library of Science, vol. 16(7), pages 1-12, July.
    2. Miriam Fioravanti & Francesca Zara & Iole Vozza & Antonella Polimeni & Gian Luca Sfasciotti, 2021. "The Efficacy of Lingual Laser Frenectomy in Pediatric OSAS: A Randomized Double-Blinded and Controlled Clinical Study," IJERPH, MDPI, vol. 18(11), pages 1-13, June.
    3. Stephanie K. Y. Chu & David T. C. To & Candice C. H. Liu & Tony Wong & Kenneth K. W. Li, 2022. "Psychological Impact of Cancellation of Elective Surgeries for Ophthalmic Patients during COVID-19 Pandemic," IJERPH, MDPI, vol. 19(22), pages 1-7, November.
    4. Nicolò Venza & Giulia Alloisio & Magda Gioia & Claudio Liguori & Annarita Nappi & Carlotta Danesi & Giuseppina Laganà, 2022. "Saliva Analysis of pH and Antioxidant Capacity in Adult Obstructive Sleep Apnea Patients," IJERPH, MDPI, vol. 19(20), pages 1-7, October.

    Corrections

    All material on this site has been provided by the respective publishers and authors. You can help correct errors and omissions. When requesting a correction, please mention this item's handle: RePEc:gam:jijerp:v:19:y:2022:i:11:p:6827-:d:830800. See general information about how to correct material in RePEc.

    If you have authored this item and are not yet registered with RePEc, we encourage you to do it here. This allows to link your profile to this item. It also allows you to accept potential citations to this item that we are uncertain about.

    If CitEc recognized a bibliographic reference but did not link an item in RePEc to it, you can help with this form .

    If you know of missing items citing this one, you can help us creating those links by adding the relevant references in the same way as above, for each refering item. If you are a registered author of this item, you may also want to check the "citations" tab in your RePEc Author Service profile, as there may be some citations waiting for confirmation.

    For technical questions regarding this item, or to correct its authors, title, abstract, bibliographic or download information, contact: MDPI Indexing Manager (email available below). General contact details of provider: https://www.mdpi.com .

    Please note that corrections may take a couple of weeks to filter through the various RePEc services.

    IDEAS is a RePEc service. RePEc uses bibliographic data supplied by the respective publishers.