Introduction

Pregnancy can take a huge toll on the body, so it is natural for people with pre-existing health conditions to have concerns about how their bodies will cope. Ehlers-Danlos Syndromes (EDS) are a group of disorders that affect the body’s connective tissues.¹ While many people with EDS have healthy pregnancies, understanding potential complications can help patients and clinicians to create an effective care plan.^2,3

This article provides a brief overview of EDS and its types, how they can affect the body at every stage of the pregnancy journey, and highlights the support available for patients and their families.

What is EDS?

EDS refers to a collection of conditions which weaken the connective tissues in the body. It targets a specific protein called collagen, which affects the skin, joints and blood vessels.¹

There are 13 types of EDS.⁴ Most types can be identified through genetic testing, except for hypermobile EDS (hEDS), as the genetic basis of this type remains unknown.^1,5 Each type has its own distinctive features, but common symptoms across most types include hypermobile (overly flexible) joints, hyperextensible (overly stretchy) skin, and frequent joint dislocations.¹

Types of EDS

This section outlines the most common types of EDS. Information on other EDS types can be found here.

Hypermobile EDS (hEDS)^3,6

• The most common type of EDS, generally low risk. Symptoms include:
  • Joint hypermobility and dislocations
  • Muscle or joint pain
  • Soft, velvety skin

Classical EDS (cEDS)⁷

• The second most common type. Symptoms include:
  • Joint hypermobility
  • Skin hyperextensibility
  • Poor wound healing
  •  Atrophic (sunken-looking) scars

Vascular EDS (vEDS)⁸

• Affects blood vessels and can be very dangerous. Symptoms include:
  • Thin, translucent skin with visible veins
  • Easy bruising
  • Blood vessel or organ rupture

If you notice possible symptoms of any type of EDS, talk to a doctor. The many different types of EDS can look similar, so it can be difficult to self-diagnose.⁹ Consulting a medical professional can help you get an accurate diagnosis and the right treatment.

How does EDS affect the pregnancy journey?

EDS can affect all stages of pregnancy, from family planning to postpartum recovery. While some risks apply to all EDS types, complications that arise often depend on the specific type of EDS.¹⁰ 

Pre-pregnancy planning

EDS patients are advised to see their GP before trying to conceive.¹⁰ They will review your medical history, any current medications, and may refer you to obstetricians, geneticists and fertility specialists to create a tailored care plan.^2,10,11

Having a multidisciplinary care team is especially important for high-risk patients, including those with vEDS, who may also need a cardiovascular specialist.¹⁰ Due to the risks involved, some medical associations advise people with vEDS against pregnancy altogether.¹² While many vEDS patients do still choose to become pregnant, alternatives like adoption or surrogacy may be considered.¹³

Genetic counselling

Pregnant EDS patients are often referred to a genetic counsellor to discuss the likelihood of passing on their condition to a future child.^10,14 Genetic counsellors can also arrange prenatal tests during pregnancy or embryo screening during in vitro fertilisation to check for inheritance of EDS.¹⁵ Not every patient will want these tests, but they are available to those who do.⁵

Testing positive

Once you become pregnant, make an appointment with a GP. This allows an appropriate care plan can be put in place quickly.

Pregnancy can be exciting news, but carrying a pregnancy while managing a long-term health condition like EDS is a big decision, especially if this condition might be passed down to the baby. Patients should always be informed of all their options, including abortion care, and make the choice that is right for them. Whatever choice is made, support from healthcare professionals is crucial.

Complications during pregnancy – monitoring and management

For EDS patients who continue with their pregnancy, regular monitoring is key to safeguarding both the patient and the baby.¹¹ Below, we will outline both general EDS and type-specific complications that can affect the muscles and joints (musculoskeletal), the blood vessels and heart (cardiovascular), the digestive system (gastrointestinal), and pregnancy outcomes, and how these risks can be monitored and managed.

Musculoskeletal

A common complication in pregnant EDS patients is increased joint hypermobility and pain in the pelvis, likely related to hormones that loosen the joints and muscles in preparation for birth.^16,17 This can be uncomfortable and may require a referral to a GP or a physiotherapist.¹¹ Certain positions and supportive aids like pelvic belts or joint braces can also help pain management.^11,18

Cardiovascular

• Postural orthostatic tachycardia syndrome (POTS), common in hEDS patients, can worsen during pregnancy.³ Wearing compression socks, avoiding high temperatures, and limiting intense exercise can help relieve symptoms like dizziness and heart palpitations
• In vEDS, pregnancy increases the risk of blood vessels bursting, including the aorta.¹⁰ This can be very dangerous, so a cardiologist will monitor the aorta by echocardiogram throughout pregnancy and up to six months post-birth to ensure the safety of the patient.¹⁹ Medications called beta blockers may also be recommended to reduce the risk of aortic rupture¹⁰

Gastrointestinal              

Heartburn is common in pregnancy and may worsen in hEDS due to increased elasticity of oesophogeal tissues.¹¹ It can often be managed by limiting spicy foods, eating smaller meals and taking antacids.

Pregnancy outcomes

It should be noted that there is a higher risk of miscarriage among EDS patients.² Miscarriage can be heartbreaking, but support is available for patients and their loved ones.

Delivery Considerations

Preterm birth

A preterm birth (delivery before 37 weeks) carries risks for both parent and baby, and is more common in cEDS and vEDS (evidence in hEDS is mixed).^10,20,21

This can be caused by early opening of the cervix. Any movement of the cervix can be monitored by internal ultrasound.^10,11 If the cervix is opening too much, a clinician may recommend placing a few stitches (cervical cerclage) to keep the cervix closed until delivery.¹⁰ EDS may also weaken the amniotic sac surrounding the baby, increasing the risk of it breaking early; bed rest may be recommended to reduce the risk of preterm birth if this happens.^10,22

Vaginal delivery vs. c-section

Vaginal delivery is preferred for most cEDS and hEDS patients, with c-section being a secondary option.^10,23 Positioning during labour should be carefully considered in hEDS patients, as movements that over-extend the limbs could lead to dislocations.¹¹ Tearing of the perineum (the tissue between the vagina and anus) is more likely in all EDS patients,² though management strategies depend on EDS type. In cEDS, a clinician may perform an episiotomy to prevent excessive tearing during delivery, but in hEDS an episiotomy is not recommended as it increases the risk of pelvic prolapse.¹⁰  

For vEDS patients, a scheduled c-section before 37 weeks is highly recommended.¹⁹ This is because contractions and pushing can put stress on an already weakened uterus, which can increase the risk of life-threatening complications like uterine rupture.¹⁰

Pain relief

EDS patients should discuss anaesthesia (pain relief) with a specialist before delivery, but especially those with vEDS or hEDS and POTS.¹⁰

Epidural or spinal anaesthesia (which numbs only the lower half of the body) is common for vaginal and c-section delivery in hEDS and cEDS, while general anaesthesia (which numbs the entire body and induces unconsciousness) is recommended for vEDS patients undergoing c-section.²⁴ Importantly, local anaesthetics can be less effective in EDS patients, so using methods that allow anaesthesia to be topped up regularly, like a combined spinal epidural, is important.³

Postpartum recovery

Recovery from childbirth is challenging and takes time, and for people with EDS it can bring extra complications.

hEDS patients are at greater risk of postpartum haemorrhage (excessive bleeding after giving birth).¹⁶ To manage this, patients can be given medications like tranexamic acid to help the blood to clot (administered before delivery) and oxytocin to help the uterus contract (given post-birth).¹⁰ Close monitoring in the period after birth is highly recommended to ensure any bleeding can be treated quickly, which often means a longer hospital stay.²¹

In cEDS and hEDS, fragile skin can slow healing after birth, so perineal tears and c-section incisions may take longer to mend.^3,10,11 Repairs should be done by trained specialists due to the high risk of scarring, and pain relief should be carefully planned as local anaesthetics may be less effective.^10,11

Just as the body needs time to recover from birth, so does the mind. Anxiety and depression are common in the postpartum period,²⁵ and the additional stress EDS places on the body can intensify these feelings.¹¹ Looking after your mental health is just as important as your physical health, and seeking support from loved ones, peer support groups, or medical professionals is a sign of strength, not something to feel ashamed of.

Summary

• EDS is a condition that affects the connective tissues, with symptoms varying by type
• Like many long-term health conditions, EDS can influence every stage of the pregnancy journey
• Before getting pregnant, a GP, obstetric specialists and genetic counsellors should be consulted to help create a tailored care plan
• During pregnancy, EDS can increase the risk of joint pain, vascular issues, digestive discomfort, and miscarriage, so regular monitoring is important to manage these risks
• EDS type can influence the likelihood of pre-term birth, whether vaginal or c-section delivery is preferred, and the type of anaesthesia recommended
• After birth, recovery may be slower and come with additional complications, e.g. bleeding and scarring, but with support from loved ones and healthcare professionals, most patients can navigate this journey safely