Ellen Ewer, MSc1, Hanadi Kazkaz, MD1,2, Nelly Ninis, MD3, Peter Rowe, MD4, Robby De
Pauw, PhD5,6,7, Eudora Tang, MSc1,8,
Cathy Barrett, MPhtySt1,9, Lies Rombaut, PhD10, Inge De Wandele, PhD10,*, and Jane V. Simmonds, DProf1,2,9,*
1UCL Great Ormond Street Institute of Child Health, London, United Kingdom;
2University College London Hospital NHS Trust, London, United Kingdom;
3St Mary’s Hospital, Imperial College Healthcare NHS Trust, London, United Kingdom; 4John Hopkins University, Baltimore, MD
5T!Write, Ghent, Belgium
6Ghent University, Ghent, Belgium
7Sciensano, Brussels, Belgium
8National University Hospital, Singapore,
9London Hypermobility Unit, Central Health Physiotherapy, London, United Kingdom; and 10Centre for Medical Genetics, Ghent University Hospital, Ghent, Belgium
*Contributed equally.
Joint hypermobility describes when people have extra movement around their joints. This can just affect one joint, or several joints. Some people with joint hypermobility don’t have any symptoms, but others can have a range of symptoms which affect many areas of their body. People with symptomatic joint hypermobility may be diagnosed with hypermobility spectrum disorder (HSD) or hypermobile Ehlers-Danlos syndromes (hEDS). The symptoms of these conditions can include unstable joints, pain and fatigue, but also affect other body systems causing problems with the bladder and bowel, mental health and the heart. Whilst knowledge of the variety of symptoms is improving, we know that some clinicians may not be aware these symptoms are linked to hypermobility. This can lead to people not getting the treatment they need for all their symptoms.
Over the last few years, a group of international researchers have created a new screening tool, the Spider. This is not used for diagnosis of HSD/hEDS, instead is used to measure the presence and impact of symptoms often reported by people with HSD/hEDS. The Spider questionnaire has thirty-one questions that cover eight main symptom domains. These include pain, neuromusculoskeletal (issues with joints and muscles), fatigue, urogenital (bladder), gastrointestinal (digestion and bowel habits), cardiac dysautonomia (including postural orthostatic tachycardia syndrome (POTS)), and anxiety and depression. After finishing the questionnaire, an overall score and scores for each domain are calculated and plotted on a spider graph which is easy to read (image 1). Everybody’s spider graph will look different, as symptoms can affect each person differently. The results may also change over time, either as people age or if they undergo treatment.
To make sure the Spider works as it should, the researchers ran several tests with the help of volunteers from The Ehlers-Danlos Society. The first step was to test the Spider’s ‘validity’ with adolescents (aged 13 to 18) and adults (aged 18 to 65). These studies were completed over several stages, and 11,151 adults and 1154 adolescents took part. They tested validity by comparing results from Spider questions and another questionnaire, measuring the same symptom, that is already used regularly in clinics. They did this for each of the symptom domains and the whole Spider questionnaire over several stages. They found that the Spider scores were similar to the scores of the other questionnaires, suggesting that the Spider measures the symptoms as they hoped.
The researchers also checked whether the Spider could tell the difference between people with and without hypermobility, by comparing scores of people with and without hypermobility. They found that with adults there was a significant difference in Spider scores between people with and without hypermobility in all eight domains, and in adolescents seven of the eight domains showed a significant difference. In the group of adolescents that took part in the study, the researchers found that there wasn’t a large difference in urogenital symptoms (symptoms affecting the bladder and genitals) between people with and without hypermobility. The next stage of their testing is to check the Spider’s reliability, whether the Spider can consistently measure symptoms over time, and to work out at what point a change in the Spider score actually means a noticeable change in someone’s symptoms.
The Spider is now available for clinicians to use with those aged 13 to 65. The research team hopes it will be used by many healthcare professionals, including pediatricians, rheumatologists, GPs and physiotherapists. They have published the adolescent validation in the Journal of Pediatrics: Clinical Practise, a scientific journal that is read by many clinicians. If you would like to read about this study in more detail, you can access the publication here.
The researchers are planning to publish the adult study in a scientific journal in the next few months and said, “This research would not have been possible without the support of The Ehlers-Danlos Society and its members, and we are so grateful to all those involved. Because of the excellent response to our research invitations and with several thousand people taking part, our research findings are of a high standard. We are continuing to develop the Spider and the research will continue until we are sure the Spider works well in all aspects. To find out more about the Spider, please contact researchers Ellen Ewer ([email protected]) and Jane Simmonds ([email protected]). We would also like to extend our sincerest gratitude to the Hypermobility Syndromes Association and the Ehlers-Danlos Support UK and it’s members, who also helped with recruitment and participation. This research was funded by The Ehlers-Danlos Society, Ehlers-Danlos Support UK, the Private Physiotherapy Education Fund (PPEF) and the Association of Paediatric Chartered Physiotherapists (APCP).”
