3-D facial image analysis of patients with Haemoglobinopathies
Research type
Research Study
Full title
A descriptive and longitudinal clinic observational study of facial features using 3D image analysis in patients with sickle cell anaemia and thalassaemia managed in the haematology department of Barts Health NHS Trust.
IRAS ID
125323
Contact name
Lifong Zou
Contact email
Sponsor organisation
Barts Health NHS Trust
Duration of Study in the UK
3 years, 0 months, 0 days
Research summary
Research Summary:
The study’s objectives were met:
This study has primary and secondary goals. The primary objective is to utilise a 3-D facial image analysis technique to find the facial differences in patients with either sickle cell disease or thalassaemia intermediate and major from the norm, while the secondary objective is to study the extent of the abnormal growth related to the severity of the anaemia and to answer the following two questions:
1. Can we use 3-D facial imaging as a tool in children with sickle cell disease and thalassaemia to predict the time point when medical intervention is required?
2. Whether it is possible to use the magnitude of facial deviation/changes as one of the parameters to classify the degree of the severity of the disease to assist the clinician in treatment planning.Description of study population:
Participants included two groups: a patient group and a control group. The patient group were those diagnosed with sickle cell anaemia or thalassemia major or intermediate. The patient group was then subdivided into children aged 6 months to 16 years under the care of the haematology department at Bart's Health NHS Trust. The control group was siblings of patients who had no such disease and were suitable as controls.
The study group consisted of 59 participants in total. This included:
Thalassaemia Group -1A: Transfusion Dependent Thalassaemia of 12 patients with Thalassaemia major and 3 patients with Thalassaemia intermedia. All of them receive blood transfusions every 3 to 4 weeks.
Thalassaemia Group - 1B: Non-blood transfusion Dependent Thalassaemia of 12 patients with Thalassaemia Intermedia. 2 of them receive blood transfusions only when required (averaging once to twice per year); the other 10 patients have never received a blood transfusion before.
Thalassaemia Control Group - 1: 8 volunteers; Sickle Cell Group - 2: 15 patients, on a range of treatments for their sickle cell disease; Sickle Cell Control Group - 2: 10 volunteers; Participants were further divided into sub-groups based on age: 6 months to 5 years, 6 to 10 years and 11 to 15 years for the delivery of the patient information sheet. Infants under 6 months were excluded, as they were considered too young to cooperate with the scanning process. Further exclusion criteria included: participants with an additional co-morbidity/s and participants suffering from epilepsy due to the use of flash in the scanning process.
A member of the research or haematology team gave information sheets to patients expressing an interest in the study attending their routine haematology appointments at Barts NHS Trust. Information sheets were also available for control siblings regarding their role in the study. The patient contact details were then passed directly to the 3-D facial scanning team who arranged a suitable time for the scan to take place, often on the same date as their next haematology review. For those patients and their siblings who agreed to participate, an age-related consent form was signed, and a 3-D facial scan was carried out. Children were asked to attend at 6 monthly intervals where possible to look at changes over a set time points.Results / main findings:
As the summary of the results from this study project:
The Sickle Cell Control group showed greater mean growth across facial features compared to the Sickle Cell patient group. This was statistically significant for all facial areas at 18 months (p<0.01). The greatest difference was in the nasion area at 12 months, at 1.26mm. Interestingly, frontal mean growth between the above two groups was comparable, with the smallest difference being 0.1mm at 6 months.The Thalassemia Control group had the greatest mean growth overall. The pattern and extent of growth of the Thalassemia Control group were more comparable to the TDT (Transfusion Dependent Thalassemia) group. These changes were found to be more statistically significant in NTDT (Non-Transfusion Dependent Thalassemia) vs. Controls (p<0.012). The smallest difference in mean growth between the TDT group and the Thalassemia Control group was 0.1 mm at 6 months in the right cheek area.
Conclusions:
In general, these children with haemoglobinopathies show slower facial growth; Transfusion treatment improves slower facial growth to some extent because these children are maintained on a higher haemoglobin level.
Increased average facial growth in the Sickle Cell & Sickle Cell Control vs Thalassemia may be partly explained by different ethnic background, with children of African descent generally growing more than those of South Asian descent.Conclusions:
In general, these children with haemoglobinopathies show slower facial growth; Transfusion treatment improves slowed facial growth to some extent because these children are maintained on a higher haemoglobin level.
Increased average facial growth in the Sickle Cell & Sickle Cell Control vs Thalassemia may be partly explained by different ethnic background, with children of African descent generally growing more than those of South Asian descent.Sickle Cell disease and Thalassaemia are inherited conditions. A common feature of both diseases is a reduced level of normal haemoglobin in the blood steam. This leads to chronic anaemia. To compensate this the bone marrow becomes over active and hyperplastic as a consequence prominent facial bones are evident presenting as mild changes in facial bone structure on X-ray image (Frederico Sampaio Neves et al. 2012), sometimes this is referred to as “Thalassaemic faces“. Due to the limitations of radiation, it is a challenge assessing the progression of facial bone changes. Currently recognition of these facial differences are often subjective and the extent and nature of these changes have not yet been quantified, particularly in relation to the progression of the disease. This research project will be looking at Barts Health NHS Trust patients with these two conditions attending haematology outpatient clinics. It will use a new and non-invasive 3-D facial scanning technique to accurately record and describe facial profiles in these patients. The aim of this study is to assess the feasibility of using this non-invasive and quantifiable technique to obtain information of facial changes in relation to the disease progression which may help to identify when an intervention treatment is required.
REC name
South West - Cornwall & Plymouth Research Ethics Committee
REC reference
15/SW/0301
Date of REC Opinion
25 Jan 2016
REC opinion
Further Information Favourable Opinion