Ehlers-Danlos syndromes and pregnancy: planning, birth and recovery

The NHS describes the Ehlers-Danlos syndromes as a group of rare inherited conditions affecting connective tissue, which supports skin, tendons, ligaments, blood vessels, internal organs and bones. There are 13 types, and hypermobile EDS (hEDS) is the most common. The NHS also describes hypermobility spectrum disorder (HSD), in which people have problems from hypermobility without meeting criteria for a specific EDS type, and which is treated in the same way as hEDS.

Pregnancy places extra demands on connective tissue. Hormonal changes increase laxity, the growing uterus alters weight distribution, and birth involves stretching of the pelvic floor and perineum. The 2024 guideline in PLOS One, written by an international group including UK authors, says symptoms can be debilitating and may either worsen or improve during pregnancy and birth.

Because the type of EDS is the single most important factor, anyone planning a pregnancy should know which type they have. Where the type is uncertain, a genetics or specialist EDS service can clarify it; the NHS says specialist EDS diagnostic services exist in Sheffield and London.

The guideline recommends genetic counselling, noting that hEDS and HSD are genetically heterogeneous and that no molecular test is currently available, that a parent with hEDS or HSD has an increased chance of having a child with it, and that symptoms cannot be predicted because they vary even within families. The NHS says genetic counselling can help people learn how their condition is inherited and what the chances are of passing it on, and gives a 1 in 2 chance for each child for the autosomal dominant types, which include hypermobile, classical and vascular EDS.

The guideline also suggests reviewing pain management and medicines before pregnancy, noting that stopping or reducing pain and sleep medicines abruptly in pregnancy can have significant consequences and that a risk and benefit assessment should be individual. It recommends screening for common companions of hEDS such as postural orthostatic tachycardia syndrome (POTS), mast cell activation and irritable bowel symptoms, because pregnancy and birth place a higher demand on bodily systems.

Do not stop prescribed medicines without medical advice. Speak to your GP or specialist before trying to conceive so that medicines can be reviewed and folic acid and other pre-pregnancy advice can be given.

The NHS says vascular EDS affects the blood vessels and internal organs and can cause them to split open and lead to life-threatening bleeding, and that the possible problems include bowel tearing and the womb tearing in late pregnancy. The expert guideline discussed here is specifically about hEDS and HSD, not vascular EDS, so it should not be applied to vascular EDS.

Anyone with vascular, kyphoscoliotic or another rare type, or with a family history of arterial rupture, should have pre-pregnancy counselling and care from a specialist team, including obstetrics and genetics, before conceiving. The NHS notes that some rare, severe types can be life threatening.

The guideline says that management plans should be made case by case after an individual risk assessment, and that pregnancy may be unremarkable even though some symptoms are exacerbated. It anticipates increased joint laxity, pelvic girdle pain, low back and hip pain, muscle spasm and bleeding, and an increased risk of fetal growth restriction. It states that there is no known increased risk of uterine rupture, pre-eclampsia or stillbirth in hEDS and HSD, that evidence is conflicting about preterm labour and cervical insufficiency, and that there is no evidence to support routine cerclage, routine progesterone or bed rest.

Practical suggestions include referral to physiotherapy and rehabilitation specialists, bracing and supports where needed, individualised stability exercises, and caution with joint manipulation. For digestive issues such as constipation, reflux and nausea, it suggests usual management, such as small frequent meals and ginger for nausea. For POTS it describes compression hosiery and maintaining good hydration, and notes that symptoms can worsen in early pregnancy if there is hyperemesis and dehydration and may improve later in pregnancy.

The guideline recommends discussing possible complications at the booking appointment so that appropriate consultation and monitoring are in place early. Ask for a plan to be written in your maternity notes so that every professional you meet knows your diagnosis.

The guideline says to anticipate a protracted latent phase followed by a rapid active stage, with precipitous birth (3 hours or less) reported in 28 to 36% of cases, increased bleeding, increased perineal trauma risk and urinary retention. It says physiological vaginal birth is clinically preferable where there are no obstetric contraindications, that assisted vaginal birth is not contraindicated, and that caesarean should be reserved for obstetric indications, though it may be considered where extensive tearing is anticipated or joint problems prevent positioning.

It recommends positioning aids, careful positioning of the neck, hips and knees, manual perineal protection, spontaneous rather than directed pushing and caution with instruments. Anaesthetic planning should be done in advance because jaw and neck instability can make airway management harder, higher doses of local anaesthetic may be needed and there is a possibility of a spinal fluid leak after spinal anaesthesia. For caesarean birth, slow-dissolving sutures and glue for the abdominal wall are suggested.

Place of birth is a personal decision. The guideline notes that planning for a home birth as a contingency may be prudent because of the chance of a very quick labour, and advises understanding local service provision.

The guideline anticipates delayed wound healing, abnormal scarring, suture failure and pelvic organ prolapse, and recommends longer-lasting absorbable sutures and closing wounds without tension. It also anticipates delayed recovery of pelvic floor and pelvic girdle function and recommends a pelvic health professional experienced with EDS, with referral for incontinence, prolapse, constipation or painful sex.

It notes that babies of parents with hEDS or HSD may themselves show features such as bruising and low muscle tone, and says that mast cell and dysautonomia symptoms may need prolonged observation after childbirth. For headaches after an epidural or spinal, it advises considering a cerebrospinal fluid leak.

In the UK, pregnancy care is delivered through NHS maternity services, with referral to obstetric-led care where there is a higher-risk condition. Your GP or midwife can refer you to a consultant obstetrician, and to physiotherapy, anaesthetic and genetics teams as needed. Private maternity and physiotherapy services exist and can complement NHS care, but a joined-up plan between professionals is the priority.