The SF-36 Health Status Questionnaire in Assessing Patients with Epistaxis Secondary to Hereditary Hemorrhagic Telangiectasia

Lennox, P.; Hitchings, Anne; Lund, Valerie J.; Howard, David

doi:10.1177/194589240501900112

Cited by 48 publications

(58 citation statements)

References 11 publications

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“…Some research assessed health-related quality of life (QoL) quantitatively among individuals with HHT with surveys such as the 36-Item Short Form Health Survey (SF-36) (Geirdal et al 2012;Geisthoff et al 2007). Compared to the general population in various countries, patients with HHT scored significantly lower on all domains of the SF-36 except for bodily pain (Geisthoff et al 2007; Lennox et al 2005;Pasculli et al 2004;Geirdal et al 2012). Within the HHT patient population, patients with severe epistaxis scored significantly lower than patients with mild epistaxis on all physical and mental dimensions of the SF-36 (Merlo et al 2014).…”

Section: Introductionmentioning

confidence: 93%

Life experiences of individuals with hereditary hemorrhagic telangiectasia and disclosing outside the family: a qualitative analysis

et al. 2015

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Hereditary hemorrhagic telangiectasia (HHT; OMIM 187300) is a disorder that affects 1:5000-1:10,000 people worldwide, with an estimated 60,000 affected individuals in the USA. Approximately 50 % of patients with HHT experience potentially life-threatening health complications such as stroke, brain abscess, or heart failure. However, the most common symptom is spontaneous and frequent nosebleeding. HHT is a hereditary condition with significant health consequences, but little is known about how individuals cope with HHT on a daily basis and how individuals share information about the disorder with social groups outside of the family. The objectives of this study were to improve understanding of the daily experiences of patients with diagnosed HHT and to investigate how they disclose their diagnosis to various social groups (friends, dating partners, employers, and coworkers) outside of their biological family. Adult patients seen at a university HHT clinic and who had been diagnosed with HHT for at least 6 months were recruited by mail. Participants completed semi-structured telephone interviews (n=19). A qualitative content analysis of interview transcripts identified four major categories: (1) the emotional impact of HHT, (2) the social impact of HHT, (3) concerns for current and future health related to HHT, and (4) social context drives disclosure of HHT. Participants reported that although HHT was a manageable hereditary disorder, the symptoms negatively affected their daily life. It is important for health care providers to understand how individuals with rare genetic disorders are managing.

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Section: Introductionmentioning

confidence: 93%

Life experiences of individuals with hereditary hemorrhagic telangiectasia and disclosing outside the family: a qualitative analysis

et al. 2015

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“…in HHT (Fig 2b, 2c), and the symptom most frequently associated with impaired quality of life in HHT, 111,112 the assumption of age-related deterioration is less clear. Classical studies 103 , mirrored in our population (Fig 2a), indicate that the proportion of individuals who have experienced nosebleeds increases with age.…”

Section: 1a) Histopathologymentioning

confidence: 99%

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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Hereditary haemorrhagic telangiectasia, inherited as an autosomal dominant trait, affects approximately 1 in 5,000 people. The abnormal vascular structures in HHT result from mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence TGF-ß superfamily signalling in vascular endothelial cells. The cellular mechanisms underlying the generation of HHT telangiectasia and arteriovenous malformations are being unravelled, with recent data focussing on a defective response to angiogenic stimuli in particular settings. For affected individuals, there is often substantial morbidity due to sustained and repeated haemorrhages from telangiectasia in the nose and gut. Particular haematological clinical challenges include the management of severe iron deficiency anaemia; handling the intricate balance of antiplatelet or anticoagulants for HHT patients in whom there are often compelling clinical reasons to use such agents; and evaluation of apparently attractive experimental therapies promoted in high profile publications when guidelines and reviews are quickly superseded. There is also a need for sound screening programmes for silent arteriovenous malformations. These occur commonly in the pulmonary, cerebral, and hepatic circulations, may haemorrhage, but predominantly result in more complex pathophysiology due to consequences of defective endothelium, or shunts that bypass specific capillary beds. This review will focus on the new evidence and concepts in this complex and fascinating condition, placing these in context for both clinicians and scientists, with a particular emphasis on haematological settings. Blood Reviews _ HHT 2010_ Shovlin 3 Overview of HHTHHT, also known as Osler Weber Rendu syndrome [1][2][3] , is one of the most common disorders to be inherited as an autosomal dominant trait. Careful epidemiological studies reveal that it affects approximately 1 in 5,000 individuals, 4,5 with regional differences, 6 and isolated communities displaying higher prevalences due to founder effects. 7 8 HHT was first described as a familial disease characterised by severe recurrent nasal and gastrointestinal bleeding with associated anaemia, and visible dilated blood vessels (telangiectasia) on the lips and finger tips. The majority of HHT patients are also affected by larger arteriovenous malformations (AVMs) in the pulmonary, hepatic, cerebral, pancreatic, spinal and other circulations. 1,2,9 These features, presented in more detail within Table 1, are used as criteria to diagnose HHT. 2 The spectrum of disease within the HHT umbrella has extended beyond the telangiectatic/AVM HHT pathology delineated by the Curaçao criteria. 2 More recently recognised features include pulmonary arterial hypertension 10 ; juvenile polyposis 11 ; pulmonary hypertension in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [12][13][14][15][16] ; a prothrombotic state associated with elevated plasma levels of factor VIII 17 , and poten...

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“…Lennox et al [39] looked at the HR-QoL according to epistaxis and blood transfusions in 38 HHT-patients (20 male, 18 female, mean age 60, range 18-78) and compared it to the population norm in the UK. The SF-36 showed no significant difference for bodily pain, all other dimensions were significantly reduced in HHT-patients.…”

Section: Introductionmentioning

confidence: 99%

“…For the international HR-QoL the SF-36 has been proven to be suitable especially in surveys of general and specific populations for differentiating health benefits caused by various treatments and for comparing the relative burden of diseases and it is the most widely proven instrument in patient-assessed health measure [45]. The HR-QoL was evaluated in 50 Italian [40], 38 British [39] and 171 (94 + 77) [43,44] German HHT-patients by using the SF-36. The German group investigated as well the impact of the Genotypes HHT1 and HHT 2 on HR-QoL.…”

Section: Introductionmentioning

confidence: 99%

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Impact of Hereditary Hemorrhagic Teleangiectasia on Quality of Life

Holderried¹,

Baur²,

Pfister³

2010

TOOTORJ

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Abstract:Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominantly inherited disorder of the fibrovascular tissue. The most common symptoms are recurrent nose bleeds and multiple mucocutaneous telangiectases. Arteriovenous malformations (AVMs) are less obvious. Because there is no permanent cure available, patients often suffer from mental stress. Several studies have been performed to assess the quality of life (QoL) of HHT patients. These studies showed that HHT patients rated bodily pain lower than the general population but altogether they had lower health-related QoL.

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The SF-36 Health Status Questionnaire in Assessing Patients with Epistaxis Secondary to Hereditary Hemorrhagic Telangiectasia

Abstract: The SF-36 reflects the reduced health status of patients with epistaxis due to HHT. Changes in the SF-36 score could be used as an outcome measure in assessing efficacy of treatment of this condition.

Cited by 48 publications

References 11 publications

Life experiences of individuals with hereditary hemorrhagic telangiectasia and disclosing outside the family: a qualitative analysis

Life experiences of individuals with hereditary hemorrhagic telangiectasia and disclosing outside the family: a qualitative analysis

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Impact of Hereditary Hemorrhagic Teleangiectasia on Quality of Life

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