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Pregnancy Bliss | Reproductive Health Hub

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Pregnancy with Ehlers-Danlos syndrome (EDS)

Question:  I’ve found out I’m pregnant for the first time and I’m thrilled. I was disappointed to find no mention of this condition on your site. Im seeing my OB in two weeks? What can I expect? I.E. (Canada)


Answer: Congratulations.  You know; I am going to sit on the fence on this one and for a good reason: The condition you have is a complex one. In fact, it describes what can be logically described as different conditions sharing a name. What binds them in one group is that they are all due to an abnormality of collagen, the principle connective tissue in the body. However, even though they are all genetic conditions, mode of inheritance vary significantly. In addition, their manifestations also vary a lot, ranging from moderate to quite severe with effect on life expectancy for some types.


Because of all those differences, it is not possible to give uniform advice for all affected women who become pregnant. There are six main types of Ehlers-Danlos Syndrome. You did not say which type you have. I will therefore briefly discuss pregnancy issues associated with the three commonest types:


Hypermobility Ehlers-Danlos Syndrome: This is also known as Type III. Ehlers Danlos Syndrome Type IIIIt is the commonest as it affects 1 in 10 -15 thousand people. Mode of inheritance is Autosomal dominant. This means, only one parent need to have the condition to potentially pass on to the offspring. As the name suggests, joint hypermobility is the main feature of this sub-type. The joints have an increased and unusual range of movements. Diagnosis is by clinical features only. There is no specific test. In pregnancy, the main problems encountered with hypermobility Ehlers Danlos Syndrome are an increased risk of preterm labour and postpartum haemorrhage.




Classical Ehlers-Danlos Syndrome: This is the second most common typeEhlers Danlos Syndrome (Classical) and groups together what were formerly known as Type I and II. This is also Autosomal dominant and is roughly half as common as the hypermobility type. The commonest features are skin hyperextensibility and redundant skin folds.  In pregnancy the concerns are restricted fetal growth in the womb, preterm delivery I.e. Increased risk of prematurity and postpartum haemorrhage. At caesarean section, this can be so serious as to lead to an emergency hysterectomy.




Vascular Ehlers-Danlos Syndrome: This was previously known as Type IV. It is arguably the most serious and there is shortened life expectancy. It is not as common as the two types above, affecting 1 in 100 - 200 thousand people. It is also Autosomal dominant. There are hundreds of gene mutations that have been identified in this EDS sub-type. The affected gene function is in the creation of procollagen for strength in the conEhlers-Danlos Type IVnective tissues of skin, lungs, intestinal walls, and blood vessel walls. The main risk to affected individual is arterial rupture leading to massive haemorrhage and sudden death typically in the third or fourth decade of life. Clinical features include absence or deficiency of subcutaneous fat and collagen in the hands and feet, a condition known as acrogeria (pictured, right), the skin is thin and almost translucent over the chest and abdomen, there may be distinctive facial features and affected individuals tend to be short. In pregnancy, there are significant risks: There is real risk of uterine rupture because of its inherent fragility; severe intra-operative haemorrhage (arterial) during a caesarean section can occur. If a vaginal delivery is attempted and achieved, there are usually vaginal tears that are tricky to repair and healing is poor and delayed. After a caesarean section, poor healing and wound dehiscence is common. Maternal mortality in the vascular type of Ehlers Danlos Syndrome is as high as 25%.



OK, this is probably a longer answer than I set out to give. It may also sound rather grim in parts but I thought you will be best served by a factual discourse and since I do not know which type of EDS you have, there is inevitably material that does not apply to you in here. Best wishes.


























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