The first guidelines to provide advice on the management of pregnancy, childbirth and aftercare for women with epidermolysis bullosa (EB) have been developed in conjunction with Australian dermatologists.
Based on a systematic literature review and expert consensus, the guidelines are intended to inform and support women with EB and their partners, and aid decision-making by clinicians managing patients with EB, according to the authors, including Professor Dedee Murrell of St George Hospital, Sydney.
The guideline authors say that most women with EB do not have an increased risk for pregnancy-related complications and normal vaginal delivery appears to be safe
“Women with EB can have successful pregnancies, even in more severe EB subtypes,” they write in the British Journal of Dermatology.
However they state that there is a need for planning and consideration of EB-related clinical needs in women considering pregnancy.
In pre-conception care, the guidelines note that fertility is not typically affected in milder forms of EB but co-morbid medical problems in severe subtypes, for example nutritional compromise and low body mass index may impact on ovulation.
During the antenatal period attention is needed on gravid distention of the abdomen for impact on EB wounds, the guidelines note.
“Although skin involvement does not routinely worsen during pregnancy in EB, there are occasional reports of wound deterioration related to abdominal distention,” they state.
The guidelines advise that vaginal birth should be offered as the preferred mode of delivery for women with all EB subtypes, but care should be individualised according to the subtype of EB.
“In some cases, there may be a strong maternal preference for caesarean section (e.g. due to fear and anxiety regarding trauma to birth canal resulting in blistering/wounds); the benefits and risks of all options should be discussed with obstetrician and dermatologist ideally, and an individualised birth plan agreed,” they advise.
Issues to consider during delivery include the use of anaesthesia, particularly in women with a known difficult airway, restricted mouth opening or extensive wounds involving lower back, which would preclude regional anaesthesia.
The guidelines also provide recommendations for skincare adaptations during labour and birth, to address the EB patient’s skin fragility. For example they note that CTG may be used but skin should be protected beneath strap with Softban/cotton padding.
Advice is also provided on perineal care and management of caesarean wounds for women with EB.
“Women with EB should be encouraged in their reproductive choices,” the authors conclude.
“With the appropriate genetic counselling, and planned approach to care, positive pregnancy experiences and outcomes for mothers with EB and their babies can be achieved,” they add emphasising that multidisciplinary team input is critical to ensuring sustained quality of care.