The desperately sad reality is that child abuse exists. The Ehlers-Danlos community is not immune to it. Equally, it is devastating to be accused of abuse when innocent.
Abuse occurs in many forms. One form is unexplained injuries. Another form is “fabricated” or “induced-illness”.
Any concern raised about possible child abuse should always be considered carefully. However, some things known to be features of the Ehlers-Danlos syndromes can look like injuries seen in physical abuse. Some things can appear very similar to symptoms or behavior that could be thought to be fabricated illness (fabricated illness). In addition it is absolutely understood that self-harm is common in children and adolescents in general and that this arises for a number of different reasons. Self-harm may be clear from the nature of the injury and a full assessment, and is not considered ‘child abuse’, but would itself identify the need for psycho-social and medical support dependent on the cause(s).
In this article we summarize the concerns that might arise in the Ehlers-Danlos syndromes, and offer general advice to parents/guardians/carers/advocates and professional colleagues alike on where to seek information and how to support the process if a Child Abuse concern has been raised.
Signs and Symptoms in Ehlers-Danlos Syndromes
Signs of physical abuse include things like unexplained bruising, soft tissue injuries, and dislocations. These can also occur naturally to varying degrees in the different types of EDS, and to differing degrees between individuals with the same form of EDS.
In conditions like osteogenesis imperfecta, osteoporosis, and some of the more rare types of EDS, fractures can occur either spontaneously or with apparently minimal trauma. However there is no evidence that fractures occur in this way in children with the hypermobile variant of Ehlers-Danlos syndrome (An Open Letter on Ehlers-Danlos Syndromes, Child Abuse, and Bone Fractures).
There is also evidence that vascular complications such as hematoma (swelling of blood in soft tissues / skin), intracranial (internal within the skull) bleeding, and dissection (split of the lining of a blood vessel) can occur, albeit it rarely, in several of the more rare forms of EDS, and not just in the vascular form of EDS.
However, there is no evidence in the literature that these severe vascular concerns arise in the hypermobile variant of EDS.
In addition there are a number of reasons why fabricated illness might be raised as a concern in children and adolescents. For example:
- symptoms or signs found on a physical examination may not be explained by any medical condition; or
- medical investigations do not explain the symptoms and signs; or
- the onset of more new symptoms after the resolution of previous ones; or
- over time there are repeated presentations with a range of signs and symptoms.
Normal daily life activities could be being affected, for example school attendance, to an extent that seems beyond what might be expected for the medical disorder the young person is known to have.
Because this is so complex, and because welfare is of fundamental concern either way (be it abuse or a medical condition), all cases should be assessed very carefully, and all the medical possibilities considered. What is irrefutable is that with over-whelming pain and multiple symptoms both a young person and their family need dedicated multidisciplinary help.
Supporting the Assessment of a Young Person with EDS
When the explanation given does not appear to match the injury or illness found, health and social/welfare staff and law enforcement staff are obliged to report their concerns if they think someone may be a victim of abuse. These staff may or may not be familiar with conditions like EDS.
There are ways to help those assessing concerns over abuse. Some key things to consider in EDS are:
- Is there a confirmed diagnosis of EDS and how has this presented over time, as an individual? It is important to have a clear picture of the “natural history”—i.e. how the condition has affected them to date. Not everyone with EDS has the same set of concerns or severity of concerns. Is what has happened normal for them or abnormal for them? If it is abnormal, why might this have happened? Are there explanations other than abuse for the change in the pattern or type of injury or behavior?
- If either or both biological parents have got EDS or a similar condition this may help to guide opinion but ultimately it is important to know whether the child or adolescent has the condition.
- Have those raising the concern consulted a professional who has experience diagnosing and treating EDS, if they do not have that expertise themselves?
- With regard to fractures, have other medical causes known to be associated with fracture been considered, and is the type of fracture a recognised or reasonably a potential complication of that condition?
- Around the world there may be differing policies and laws when dealing with child abuse. Advice should be sought from a legal representative familiar with local policy and law.
- Whenever possible work with the health, welfare, and law enforcement staff. In doing their job they are obliged by law to report their concerns. The welfare of the young person will be at the forefront of their minds.
It is inevitable in complex cases that multi-disciplinary input is needed, but in the presence or possible presence of EDS this should include expert opinion in EDS and the heritable disorders of connective tissue.
Dr. Alan Hakim and Dr. Brad Tinkle, peer reviewed by the Medical and Scientific Board of The Ehlers-Danlos Society, and the Pediatric Working Group of the EDS International Collaboration.
For more information on fractures in childhood, please see: An Open Letter on Ehlers-Danlos Syndromes, Child Abuse, and Bone Fractures.
For more information on fabricated or induced illness, please visit:
For more information on Safeguarding, please visit:
DCSF (2008) Safeguarding children in whom illness is fabricated or induced. Supplementary guidance to Working Together to Safeguard Children. http://bit.ly/2KF19Zh
HM Government (2013) Working Together to Safeguard Children: A guide to inter agency working to safeguard and promote the welfare of children. London. The Stationary Office. http://bit.ly/2Fat9kH
Dr. Michael Holick