This article is written for both patients and healthcare and social care professionals. It applies primarily to JHS / EDS-HT and where complications due to Classic or Vascular EDS occur, these are stated as such.
Pregnancy and childbirth is often a completely normal experience for women with JHS / EDS-Hypermobility Type (EDS-HT)
It is, however, important to be aware of a number of issues that might cause problems. Being alert to these healthcare staff can reduce the risk of them arising and speed up the interventions should they occur so that complications or difficulties for both mother and child may be minimised.
The first section below out lines the sorts of medical issues that need to be considered. The section following that describes some of the more common musculoskeletal issues that arise during and after pregnancy.
Pregnancy and Medical Concerns in JHS and EDS-HT
Unlike the vascular form of the Ehlers-Danlos Syndrome (EDS), formerly EDS Type IV, the Joint Hypermobility Syndrome (JHS), and EDS–Hypermobility Type (formerly EDS III) (EDS-HT) are not associated with heart disease or major hazards during pregnancy and labour.
In 2016 an important paper was published from Sweden (Sunderlin et al). The researchers identified 314 cases of pregnancy in women with either JHS or EDS through the Swedish Patient Register and Medical Birth Register. The cases were compared with 1,247,864 controls (pregnant women without JHS/EDS). The risk of complications in JHS/EDS was assessed after adjusting for maternal age, smoking, number of pregnancies, and year of birth. JHS/EDS was not associated with any increased risk of our preterm birth, the need for a caesarean section, stillbirth, complications in the infant at delivery (a low Apgar score), or the infant being small or large for gestational age.
At the same time similar observations have been published by Hugon-Rodin et al. In this study the obstetric outcomes were similar to those of the general French population for deliveries by caesarean section (14.6 %) and premature births (6.2 %). However they also found that the risks of spontaneous abortion (28 %) and multiple spontaneous abortion (13 %) were somewhat higher. General population studies show spontaneous abortion (loss of the fetus before 20 weeks gestation) occurs in 10-20% of women (Tulandi T, Al-Fozan HM).
Interestingly, aside from pregnancy, Hugon-Rodin and colleagues also reported a high frequency of gynaecological complaints: menorrhagia (abnormally heavy bleeding) (76 %), dysmenorrhea (excessive pain during bleeding) (72 %) and dyspareunia (pain during sexual intercourse) (43 %); but endometriosis was not highly prevalent in this population. The researchers noted a subgroup of women were sensitive to hormonal fluctuations with more severe symptoms occurring during puberty, prior to menstruation, and during the postpartum (after childbirth) period. To read more about hormones click HERE.
However, several considerations during pregnancy should be borne in mind. These are:
- There may be an increase in the level of pain experienced, spinal and joint pain in particular, during the course of the pregnancy.
- You could experience a more rapid labour.
- There is no absolute indication for Caesarean section; the benefits and risks are a clinical judgement, not least wound healing and risk of bleeding.
- Due to a possible resistance to the effects of local anaesthetics you may find that you gain little or no benefit from epidural anaesthesia or other local painkillers given if you have an episiotomy or tear.
- Any tear or episiotomy wound may heal slowly or healing may be impaired.
- Any surgery required will need to take the possible effect on healing into account.
- You may find feeding the baby more difficult and caring for the baby more tiring than other mothers.
- It is important to ensure that all post-natal exercises are performed with greater care taking any individual requirements into account but pelvic floor exercises are particularly important to prevent possible problems such as a uterine prolapse in later life.
- Hypermobility has a high inheritance. This does not mean the child will necessarily develop any symptoms, either in childhood or later on in life.
In Classical EDS there may be other, albeit uncommon complications such as:
- Early loss of the fetus due to rupture of membranes or bleeding.
- A tendency to early dilatation of the cervix and to premature rupture of the membranes and hence premature labour and delivery. Cervical dilatation may respond to rest and support sutures.
Musculoskeletal Issues in Pregnancy
Joint laxity increases during pregnancy; existing problems may be more of a problem and new joint pains / instability may arise. However, some individuals report feeling much better while pregnant. It is important to remain fit and active during pregnancy, with walking, swimming or exercise in water recommended.
Low back and pelvic pain is a common symptom in pregnancy. The presence of hypermobility is one of several factors associated with developing lower back pain.
Increased instability of the sacroiliac joints may be responsible for pelvic pain in pregnancy.
Pubic symphysis dysfunction is also commonly encountered in hypermobiles. This may present with pain in the midline of the groin radiating in to the lower abdomen and hips. The pelvis can feel very unstable making walking / weight- bearing potentially very painful.
Physical rehabilitation with support from a therapist may be required early and throughout pregnancy. Low back and pelvic pain can be helped by a maternity or sacro-iliac support in conjunction with exercise and pacing of activities
Positioning during delivery should be carefully thought through to make this as comfortable and physically safe as possible. It is worth discussing this with the Midwife and trying out positioning for a normal delivery, noting what works. Try to ensure the hips/legs are supported and those with pelvic girdle pain will benefit from delivery positions that do not involve lying on their backs.
The day to day lifting and handling of an infant can lead to problems for some hypermobile individuals. However, women frequently report that any pain during pregnancy has disappeared following delivery. Nevertheless mum may find breast-feeding and care of her newborn baby more difficult if they are still troubled by pain, muscle weakness or subluxations/dislocations, particularly in the arms. Some simple ‘tricks’ include:
– Using carrier straps that hold baby in the centre of gravity and at the front of chest.
– Cradle supports for bathing, clothing baby.
– During breast feeding resting baby on a well-supported structure such as small beanbag placed on mums lap, or lying down with baby cradled in front.
– Reducing the activities that require bending as far as possible, but when bending forwards remember to bend the hips and knees. Try to ensure surfaces for changing nappies etc., are at an appropriate height.
– Looking after a newborn baby can be very tiring. Taking care to pace activities and allowing some rest time each day can be beneficial.
– Aim to return to normal exercise to maintain fitness (from 6 weeks post delivery or 12 weeks post caesarean).
All these issues can be managed by physical therapies and practical solutions.
Pelvic floor weakness
The pelvic floor muscles may be sore and stretched after delivery. It is helpful to start exercising the pelvic floor muscles after a few days rest to help reduce the risk of weakness in this area later in life. Practise gently pulling in the muscles (as if stopping the flow of urine) and hold for a few seconds while you breathe. Let the muscles go and relax fully. Repeat between 10-20 times a day and incorporate into everyday activities. If there are any continence issues see a physiotherapist for advice.
It is important to note infants and children are generally more hypermobile and may lose this to some degree as they grow. It is also important to understand that even if they remain hypermobile it does not necessarily mean that they will develop symptoms. The important thing is that if they did develop any problems, their doctor appreciates that JHS / EDS-HT is present in the family and may explain their symptoms.
Dr Alan J Hakim MA FRCP
Consultant Physician and Rheumatologist
Rosemary Keer MSc MSCP SRP
Physiotherapist, Hospital of St John and St Elizabeth, London
Written June 2013. Reviewed by Prof H Bird. Version1.4 – Revised October 2016. Planned Date of Review June 2017
Charvet PY, sale B, Rebaud P et al. Ehlers-Danlos syndrome and pregnancy. Apropos of a case. Journal of Gynaecology Biology and Reproduction (Paris). 1991; 20:75-78.
De Vos M, Nuytinck L, Verellen C et al. Preterm premature rupture of membranes in a patient with the hypermobility type of the Ehlers-Danlos syndrome. A case report. Fetal Diagnosis & Therapy. 1999;14(4): 244-47.
Hay-Smith J, Morkved S, Fairbrother KA. Pelvic floor muscle training for prevention and treatment of urinary and faecal incontinence in antenatal and postnatal women. Cochrane Database System Review. 2008;8(4):CD007471
Hermanns-Le T, Pierard G, Quatresooz P. Ehlers-Danlos-like dermal abnormalities in women with recurrent preterm premature rupture of fetal membranes. American Journal of Dermatopathology. 2005; 27(5): 407-10.
Hugon-Rodin J, Lebègue G, Becourt S, Hamonet C, Gompel A. Gynecologic symptoms and the influence on reproductive life in 386 women with hypermobility type ehlers-danlos syndrome: a cohort study. Orphanet J Rare Dis. 2016 Sep 13;11(1):124. doi: 10.1186/s13023-016-0511-2.
Keer R, Grahame R. Pregnancy and Hypermobility. In Hypermobility, Fibromyalgia, and Chronic Pain. Hakim A, Keer R, Grahame R (eds). Churchill-Livingston, London 2010.
Lawrence EJ. The Clinical Presentation of Ehlers-Danlos syndrome. Adv Neonatal Care. 2005;5(6):301-314.
Lind J, Wallenburg HC. Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand. 2002;81(4):293-300.
Marnach ML, Ramin KD, Ramsey PS et al. Characterization of the relationship between joint laxity and maternal hormones in pregnancy. Obstetrics and Gynecology. 2003;101(2): 331-35.
Pepin M, Schwarze U, Superti-Furga A et al. Clinical and genetic features of Ehlers-Danlos syndrome type IV the vascular type.[erratum appears in N Engl J Med 2001 Feb 1;344(5):392] New England Journal of Medicine. 2001;342(10): 673-80.
Sundelin HE, Stephasson O, Johansson K, Ludvigsson JF. Pregnancy outcome in Joint Hypermobility Syndrome and Ehlers Danlos Syndrome. Acta Obstet Gynecol Scand. 2016 Oct 14. doi: 10.1111/aogs.13043. [Epub ahead of print]
Thornton JG, Hill J, Bird HA. Complications of pregnancy and benign familial joint hyperlaxity. Annals of Rheumatic Diseases. 1988;47(3):228-31.
Tulandi T, Al-Fozan HM. 2016. UpToDate. http://www.uptodate.com/contents/spontaneous-abortion-risk-factors-etiology-clinical-manifestations-and-diagnostic-evaluation.
van Dongen PW, de Boer M, Lemmens WA et al. Hypermobility and peripartum pelvic pain syndrome in pregnant South African women. European Journal of Obstetrics, Gynecology, and Reprodroductive Biology. 1999;84(1): 77-82.
Volkov N, Nisenblat V, Ohel G et al. Ehlers-Danlos syndrome: insights on obstetric aspects. Obstetrical & Gynecological Survey. 2007:62(1):51-7.