Diet and Hereditary Haemorrhagic Telangiectasia

A Questionnaire Study on Diet and Hereditary Haemorrhagic Telangiectasia

Hereditary Haemorrhagic Telangiectasia (HHT) affects 1 in 5,000 people. The purpose of this study is to provide data regarding the diet and nosebleed frequency using a questionnaire.

This will be filled in by people with HHT.

The questionnaire has been designed in paper format.

Study Overview

• Status: Completed
• Conditions: Hereditary Haemorrhagic Telangiectasia (HHT)
• Intervention / Treatment: Other: Questionnaire on dietary history; Other: Questionnaire on nosebleed severity; Other: One week food diary generated by weighing foods; Procedure: Blood tests for full blood count, albumin, and indices of nutritional status

Detailed Description

Hereditary Haemorrhagic Telangiectasia (HHT) affects 1 in 5,000 people, usually causing nosebleeds, skin blood spots, and/or anaemia as a result of bleeding from the nose or gut. The majority of people with HHT also have abnormal blood vessels (arteriovenous malformations) in internal organs such as the lungs, liver and brain. Management of this multisystem disorder is highly challenging.

The Lead Applicant has spent 20 years working on this rare disease, and identified multiple areas where more evidence is required to assist clinicians and patients with this lifelong condition. A particular issue is whether the diet influences HHT or its complications in any way.

In this study, people will fill in two questionnaires, one giving details of their diet, and another details of their nosebleeds. They will also be asked to consider participating in an accessory study arm which includes weighing food for one week and providing a food diary, in addition to having a single set of blood test.

• Study Type: Interventional
• Enrollment (Actual): 50
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United Kingdom
  • London, United Kingdom, W12 0NN
    • HHTIC London, Hammersmith Hospital, Imperial College Healthcare NHS Trust

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• A diagnosis of hereditary Haemorrhagic Telangiectasia (HHT)

Exclusion Criteria:

• Unable to provide informed consent
• Presence of another major organ disorder that may affect nutritional status, such as inflammatory bowel disease, or celiac disease.

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: Non-Randomized
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Diet and nosebleed questionnaire; Participants will only be required to fill in two paper questionnaires, one on dietary history, and one on nosebleed severity.	Other: Questionnaire on dietary history; Other: Questionnaire on nosebleed severity
Experimental: Weighed food diary arm; Participants will be required to weigh their food for one week to generate a food dairy, and have a single blood test, in addition to filling in the two paper questionnaires, one on dietary history, and one on nosebleed severity.	Other: Questionnaire on dietary history; Other: Questionnaire on nosebleed severity; Other: One week food diary generated by weighing foods; Procedure: Blood tests for full blood count, albumin, and indices of nutritional status

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Number of Participants That Achieving Personal Recommended Intake of Iron	Dietary food item iron content assessed by the European Prospective Investigation into Cancer and Nutrition (EPIC) food frequency questionnaire. This method has been validated against the gold standard for dietary assessment, a 7-day weighed food diary. Questions are asked about consumption of 130 major food items over the previous year, requiring participants to indicate the frequency of consumption, and to provide details about the methods of cooking, type of produce, and use of dietary supplements. The EPIC FFQ has been widely validated in a number of studies and is deemed an adequate assessment tool to assess dietary intake.	1 year
Nosebleed Severity	Nosebleeds were quantified using the validated Epistaxis Severity Score (ESS). The six questions provide an objective measure of nosebleeds: three relate to different characteristics of typical nosebleeds within the previous three months (frequency, duration and intensity (gushing/pouring or not)), three to medical attention, anemia and transfusion requirements. The final ESS score ranges from 0-10, where a higher score equates to greater blood losses.	3 months
Number of Participants That Achieving the Hemorrhage Adjusted Iron Requirement (HAIR)	The hemorrhage-adjusted iron requirement (HAIR) was calculated as the sum of the normal recommended dietary iron intake, and requirements to compensate for non-menstrual blood losses.	1 year

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Number of Participants That Ingested Chocolate	Chocolate can precipitate nosebleeds Food items intake assessed by 7-day weighed food diary	3 months
Number of Participants That Ingested Bread	Bread not precipitate nosebleeds Food items intake assessed by 7-day weighed food diary	3 months

Collaborators and Investigators

• Sponsor: Imperial College London
• Investigators: Principal Investigator: Claire L Shovlin, PhD FRCP, Imperial College London

Publications and helpful links

General Publications

• Finnamore H, Le Couteur J, Hickson M, Busbridge M, Whelan K, Shovlin CL. Hemorrhage-adjusted iron requirements, hematinics and hepcidin define hereditary hemorrhagic telangiectasia as a model of hemorrhagic iron deficiency. PLoS One. 2013 Oct 16;8(10):e76516. doi: 10.1371/journal.pone.0076516. eCollection 2013.
• Finnamore HE, Whelan K, Hickson M, Shovlin CL. Top dietary iron sources in the UK. Br J Gen Pract. 2014 Apr;64(621):172-3. doi: 10.3399/bjgp14X677761. No abstract available.
• Chamali B, Finnamore H, Manning R, Laffan MA, Hickson M, Whelan K, Shovlin CL. Dietary supplement use and nosebleeds in hereditary haemorrhagic telangiectasia - an observational study. Intractable Rare Dis Res. 2016 May;5(2):109-13. doi: 10.5582/irdr.2016.01019.
• Finnamore H, Silva BM, Hickson BM, Whelan K, Shovlin CL. 7-day weighed food diaries suggest patients with hereditary hemorrhagic telangiectasia may spontaneously modify their diet to avoid nosebleed precipitants. Orphanet J Rare Dis. 2017 Mar 28;12(1):60. doi: 10.1186/s13023-017-0576-6.

Study record dates

Study Major Dates

• Study Start (Actual): April 1, 2011
• Primary Completion (Actual): September 1, 2013
• Study Completion (Actual): September 1, 2016

Study Registration Dates

• First Submitted: September 20, 2012
• First Submitted That Met QC Criteria: September 24, 2012
• First Posted (Estimated): September 25, 2012

Study Record Updates

• Last Update Posted (Actual): March 29, 2024
• Last Update Submitted That Met QC Criteria: September 25, 2023
• Last Verified: September 1, 2023

More Information

Terms related to this study

• Keywords: Iron supplements; Hereditary Haemorrhagic Telangiectasia; Nose bleeding
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Congenital Abnormalities; Hematologic Diseases; Hemorrhagic Disorders; Hemostatic Disorders; Cardiovascular Abnormalities; Vascular Malformations; Telangiectasis; Telangiectasia, Hereditary Hemorrhagic
• Other Study ID Numbers: CLS21

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No