Psychosocial quality of life in hereditary haemorrhagic telangiectasia patients

Loäec, M; Morinière, S.; Hitier, Martin; Ferrant, Ophélie; Plauchu, H; Babin, E.

doi:10.4193/rhino10.090

Cited by 27 publications

(18 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It can also lead to feelings of insecurity and loss of confidence in one’s body, which sometimes make patients feeling helpless and out of control [ 14 , 42 ]. Thus, episodes of epistaxis can significantly alter the psychosocial QOL of patients [ 43 ]. Because of these irregular, spontaneous and above all unpredictable manifestations, it is essential to pay particular attention to this area that is central to QOL of HHT patients [ 7 , 13 , 44 , 45 ].…”

Section: Discussionmentioning

confidence: 99%

Development and validation of a quality of life measurement scale specific to hereditary hemorrhagic telangiectasia: the QoL-HHT

Martinent

Dupuis‐Girod

et al. 2022

Orphanet J Rare Dis

View full text Add to dashboard Cite

Background Hereditary hemorrhagic telangiectasia (HHT) disease is a rare genetic disorder with symptoms and complications that can significantly affect patients’ daily lives. To date, no scale has been validated to assess the specific symptoms of this disease on the quality of life (QOL) of HHT patients. This makes it difficult for clinicians to accurately measure the quality of life of patients with HHT. The present study aims to develop and validate a QOL measurement tool specific to HHT disease: the QOL questionnaire in HHT (QoL-HHT). Methods A quantitative, non-interventional, multi-center study involving HHT patients in twenty French HHT expert centers was conducted. A calibration sample of 415 HHT patients and a validation sample of 228 HHT patients voluntarily participated in the study. Data were analyzed using exploratory factor analysis (EFA), confirmatory factor analysis (CFA), Exploratory Structural Equation Modeling (ESEM) analyses, reliability analyses, and correlational analyses. Results The EFA, CFA and ESEM results allowed us to provide evidence of the factorial structure of a questionnaire composed of 24 items measuring 6 domains of QOL: Physical limitations, social relationships, concern about bleeding, relationship with the medical profession, experience of symptoms, and concern about the evolution of the disease. Cronbach’s alpha coefficients (> 0.70) demonstrated reliable internal consistency of all the QoL-HHT scores (dimensions). The results of the test–retest provided further evidence of the reliability of the QOL-HHT scores over time. Correlational analyses provided evidence for the convergent validity of the QoL-HHT scores. Conclusions We developed a simple and quick self-assessment tool to measure quality of life specific to HHT disease. This study demonstrated reliability and validity of our QoL-HHT scores. It is a very promising tool to evaluate the impact of HHT disease on all aspects of the quality of life of HHT patients in order to offer them individualized medico-psycho-social support. Trial registration: ClinicalTrials, NCT03695874. Registered 04 October 2018, https://www.clinicaltrials.gov/ct2/show/NCT03695874

show abstract

Section: Discussionmentioning

confidence: 99%

Development and validation of a quality of life measurement scale specific to hereditary hemorrhagic telangiectasia: the QoL-HHT

Martinent

Dupuis‐Girod

et al. 2022

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…Similarly, in the study by Loaec et al, 115 patients were interviewed, and the authors found that frequent episodes of epistaxis and abundant bleeding decreased psychosocial QOL measures. 40 In addition, these patients expressed a ''desire to withdraw'' and ''felt different'' as compared with others.…”

Section: Quality Of Lifementioning

confidence: 96%

“…These studies of adults with epistaxis and HHT have shown severity-dependent effects on QOL and impairment on psychosocial QOL measures. 40,41 Merlo et al surveyed 604 patients with HHT using a validated survey, the Epistaxis Severity Score (ESS), and evaluated their health-related QOL. 41 The authors found that 27.6% patients had mild epistaxis (ESS \4), 47.2% moderate (4 ESS \7), and 25.2% severe (ESS 7).…”

Section: Quality Of Lifementioning

confidence: 99%

“…149,150 Nosebleed frequency often increases with age, leading to anemia (low blood counts), need for iron and blood transfusions, extensive medical expenses, and a significantly reduced QOL in patients with HHT. 28,40,[151][152][153] A review of topical medications to treat nosebleed in patients with HHT was recently published, summarizing the data supporting the long-term use of these adjuvants. 28,154 These reviews report that thalidomide can improve the severity and frequency of the epistaxis, improve hemoglobin concentrations, and decrease the need for blood transfusions.…”

Section: Supporting Textmentioning

confidence: 99%

See 1 more Smart Citation

Clinical Practice Guideline: Nosebleed (Epistaxis)

Tunkel

Anne

Payne

et al. 2020

Otolaryngol.--head neck surg.

108

141

View full text Add to dashboard Cite

Objective Nosebleed, also known as epistaxis, is a common problem that occurs at some point in at least 60% of people in the United States. While the majority of nosebleeds are limited in severity and duration, about 6% of people who experience nosebleeds will seek medical attention. For the purposes of this guideline, we define the target patient with a nosebleed as a patient with bleeding from the nostril, nasal cavity, or nasopharynx that is sufficient to warrant medical advice or care. This includes bleeding that is severe, persistent, and/or recurrent, as well as bleeding that impacts a patient’s quality of life. Interventions for nosebleeds range from self-treatment and home remedies to more intensive procedural interventions in medical offices, emergency departments, hospitals, and operating rooms. Epistaxis has been estimated to account for 0.5% of all emergency department visits and up to one-third of all otolaryngology-related emergency department encounters. Inpatient hospitalization for aggressive treatment of severe nosebleeds has been reported in 0.2% of patients with nosebleeds. Purpose The primary purpose of this multidisciplinary guideline is to identify quality improvement opportunities in the management of nosebleeds and to create clear and actionable recommendations to implement these opportunities in clinical practice. Specific goals of this guideline are to promote best practices, reduce unjustified variations in care of patients with nosebleeds, improve health outcomes, and minimize the potential harms of nosebleeds or interventions to treat nosebleeds. The target patient for the guideline is any individual aged ≥3 years with a nosebleed or history of nosebleed who needs medical treatment or seeks medical advice. The target audience of this guideline is clinicians who evaluate and treat patients with nosebleed. This includes primary care providers such as family medicine physicians, internists, pediatricians, physician assistants, and nurse practitioners. It also includes specialists such as emergency medicine providers, otolaryngologists, interventional radiologists/neuroradiologists and neurointerventionalists, hematologists, and cardiologists. The setting for this guideline includes any site of evaluation and treatment for a patient with nosebleed, including ambulatory medical sites, the emergency department, the inpatient hospital, and even remote outpatient encounters with phone calls and telemedicine. Outcomes to be considered for patients with nosebleed include control of acute bleeding, prevention of recurrent episodes of nasal bleeding, complications of treatment modalities, and accuracy of diagnostic measures. This guideline addresses the diagnosis, treatment, and prevention of nosebleed. It focuses on nosebleeds that commonly present to clinicians via phone calls, office visits, and emergency room encounters. This guideline discusses first-line treatments such as nasal compression, application of vasoconstrictors, nasal packing, and nasal cautery. It also addresses more complex epistaxis management, which includes the use of endoscopic arterial ligation and interventional radiology procedures. Management options for 2 special groups of patients—patients with hereditary hemorrhagic telangiectasia syndrome and patients taking medications that inhibit coagulation and/or platelet function—are included in this guideline. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group. It is not intended to be a comprehensive, general guide for managing patients with nosebleed. In this context, the purpose is to define useful actions for clinicians, generalists, and specialists from a variety of disciplines to improve quality of care. Conversely, the statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. Action Statements The guideline development group made recommendations for the following key action statements: (1) At the time of initial contact, the clinician should distinguish the nosebleed patient who requires prompt management from the patient who does not. (2) The clinician should treat active bleeding for patients in need of prompt management with firm sustained compression to the lower third of the nose, with or without the assistance of the patient or caregiver, for 5 minutes or longer. (3a) For patients in whom bleeding precludes identification of a bleeding site despite nasal compression, the clinician should treat ongoing active bleeding with nasal packing. (3b) The clinician should use resorbable packing for patients with a suspected bleeding disorder or for patients who are using anticoagulation or antiplatelet medications. (4) The clinician should educate the patient who undergoes nasal packing about the type of packing placed, timing of and plan for removal of packing (if not resorbable), postprocedure care, and any signs or symptoms that would warrant prompt reassessment. (5) The clinician should document factors that increase the frequency or severity of bleeding for any patient with a nosebleed, including personal or family history of bleeding disorders, use of anticoagulant or antiplatelet medications, or intranasal drug use. (6) The clinician should perform anterior rhinoscopy to identify a source of bleeding after removal of any blood clot (if present) for patients with nosebleeds. (7a) The clinician should perform, or should refer to a clinician who can perform, nasal endoscopy to identify the site of bleeding and guide further management in patients with recurrent nasal bleeding, despite prior treatment with packing or cautery, or with recurrent unilateral nasal bleeding. (8) The clinician should treat patients with an identified site of bleeding with an appropriate intervention, which may include one or more of the following: topical vasoconstrictors, nasal cautery, and moisturizing or lubricating agents. (9) When nasal cautery is chosen for treatment, the clinician should anesthetize the bleeding site and restrict application of cautery only to the active or suspected site(s) of bleeding. (10) The clinician should evaluate, or refer to a clinician who can evaluate, candidacy for surgical arterial ligation or endovascular embolization for patients with persistent or recurrent bleeding not controlled by packing or nasal cauterization. (11) In the absence of life-threatening bleeding, the clinician should initiate first-line treatments prior to transfusion, reversal of anticoagulation, or withdrawal of anticoagulation/antiplatelet medications for patients using these medications. (12) The clinician should assess, or refer to a specialist who can assess, the presence of nasal telangiectasias and/or oral mucosal telangiectasias in patients who have a history of recurrent bilateral nosebleeds or a family history of recurrent nosebleeds to diagnose hereditary hemorrhagic telangiectasia syndrome. (13) The clinician should educate patients with nosebleeds and their caregivers about preventive measures for nosebleeds, home treatment for nosebleeds, and indications to seek additional medical care. (14) The clinician or designee should document the outcome of intervention within 30 days or document transition of care in patients who had a nosebleed treated with nonresorbable packing, surgery, or arterial ligation/embolization. The policy level for the following recommendation, about examination of the nasal cavity and nasopharynx using nasal endoscopy, was an option: (7b) The clinician may perform, or may refer to a clinician who can perform, nasal endoscopy to examine the nasal cavity and nasopharynx in patients with epistaxis that is difficult to control or when there is concern for unrecognized pathology contributing to epistaxis.

show abstract

“…Está descrito que las epistaxis, además de ser el signo más frecuente de la enfermedad, es para los pacientes la manifestación que más condiciona su calidad de vida pues afecta a más del 90% de los pacientes (Hussain, 1976;Zarrabeitia et al, 2017;Kühnel et al, 2018). El hecho de que las hemorragias sean tan frecuentes y abundantes ya en el periodo de vida activa completa, entre los 30 y los 60 años de vida, hace que perturbe su vida de manera física, emocional, social y laboral (Loaëc et al, 2011;Clark et al, 2018). Tal y como se ha descrito previamente, durante este periodo de edad es probable que los pacientes ya sufran al menos un sangrado diario y que la intensidad del sangrado sea de un grado moderado-alto (Silva et al, 2013).…”

Section: Implicación De Las Hemorragias En La Vida De Los Pacientesunclassified

Estudio del proceso de hemostasia en modelos murinos de la telangiectasia hemorrágica hereditaria

Turrión¹

View full text Add to dashboard Cite

show abstract

Psychosocial quality of life in hereditary haemorrhagic telangiectasia patients

Cited by 27 publications

References 7 publications

Development and validation of a quality of life measurement scale specific to hereditary hemorrhagic telangiectasia: the QoL-HHT

Development and validation of a quality of life measurement scale specific to hereditary hemorrhagic telangiectasia: the QoL-HHT

Clinical Practice Guideline: Nosebleed (Epistaxis)

Estudio del proceso de hemostasia en modelos murinos de la telangiectasia hemorrágica hereditaria

Contact Info

Product

Resources

About