Overview

Epidermolysis bullosa (EB) is the name for a group of rare, inherited skin disorders. They cause the skin to become very fragile.

If you have EB, the care you will need during pregnancy and childbirth will depend on what type of EB you have, and how it affects you. Contact your EB team if you are planning to, or have become pregnant so you can get advice and support.

Passing EB on to your child

Passing EB on to your unborn child depends on the type of EB you have. If you are not sure if your type of EB will affect your child, ask your EB team.

Dominantly inherited EB

Dominantly inherited forms of EB usually mean there is a long family history of EB. In this case, there

is a 1 in 2 chance of each child you have being affected by EB.

Examples of dominantly inherited EB are EB simplex (EBS) and dominant dystrophic EB (DDEB).

Recessively inherited EB

Recessively inherited forms of EB mean the risk of your child being affected is fairly small. There is a 1 in 4 chance that your child will be affected if both parents are carriers of EB. There is a 1 in 4 chance that your child will be unaffected.

There is also a 1 in 2 chance of your child being a carrier of EB, but having no symptoms (asymptomatic).

Testing for EB before pregnancy

Testing to see if your child will get EB before getting pregnant is not routinely offered. Some specialist centres might be able to offer this service. Talk to your EB nurse or doctor about the risk of your child having EB, and the options for testing.

Diagnosing EB during pregnancy

Testing for EB is not routine during pregnancy, but might be recommended to see if your child has inherited EB.

Your baby is more likely to be tested during pregnancy if you have:

• EB simplex (EBS)
• generalised severe EB (formerly known as EBS Dowling-Meara)
• dominant dystrophic EB (DDEB)

If you have any type of EB, talk to your EB team about the risk of your child having EB and the options that are available to you.

Care during pregnancy

Morning sickness

Morning sickness can be a problem in early pregnancy. In some types of EB, being sick (vomiting) a lot can cause blistering in the food pipe (oesophagus). If you have any soreness after being sick, speak to your EB nurse for advice.

Weight gain

Weight gain is normal during pregnancy. Some people with more severe forms of EB might be underweight and will need specialist dietary advice and support during pregnancy. A specialist EB dietitian is available at many EB centres.

Ultrasound scans

Ultrasound scans are a routine part of your care during pregnancy. If EB does not affect the skin on your tummy, there should be no problem with having an ultrasound scan. Tell the person doing the scan to be careful when wiping off the gel, so that they do not irritate your skin.

If you have a more severe form of EB, an ultrasound scan might be more difficult. The pressure needed on your tummy during the scan might cause blistering to your skin. If you are worried about having a scan, speak to your EB nurse. They will be able to support you and offer advice to the person doing the scan.

Urine samples

You will be asked to bring a pee (urine) sample to every maternity appointment. If you have any open wounds in the area between your vagina and bottom, the midwife might find blood in your urine.

There can also be pregnancy-related reasons for the blood, which should be investigated. Tell your midwife that the blood might be related to your EB.

Vaginal examinations  

Vaginal examinations can be done, but you need to ask the midwife to be very careful, especially if EB affects the skin around your vagina and bottom. Make sure the midwife uses well-lubricated gloves.

Planning your delivery

The way your baby will be delivered will depend on your health during pregnancy, rather than the type of EB you have. Your choice should be made after talking with your obstetrician, midwife and the EB team.

Some people with relatively severe EB have had vaginal deliveries with few problems. However, many people choose to have a planned caesarean section (C-section).

An EB pregnancy information and dressings pack can be provided by your EB nurse. This will include guidelines for your obstetrician and midwife, contact details for your EB nurse, and some useful dressings. You should keep this with your ‘labour bag’ and make sure you share it with whoever is looking after you during your delivery.

Vaginal delivery

You understand your EB and your health best. Your midwife’s priority will be to provide the best care for you and your baby, but they might have limited understanding of EB.

Try to make sure you’re well prepared for labour. It is also a good idea if your birth partner has a clear understanding of how to manage delivery with EB, as they might need to speak up on your behalf during labour.

If you have a more severe form of EB, discuss and plan your delivery in advance with your obstetrician, midwife and EB nurse. Your EB nurse will be able to help organise this.

Caesarean section (C-section)

After talking with your obstetrician, midwife, and EB team, you might decide to have a caesarean section.

With most types of EB, managing a caesarean section will be very similar to any surgery and should be straightforward. Ideally, the section will be planned, but it is always worth thinking ahead about what to do if you need an emergency procedure.

Surgical wounds in all types of EB generally heal well, and stitches or clips can be used to close your wound.

If you have a more severe form of EB, your pregnancy and delivery will need to be more closely monitored.

As pregnancy in women with RDEB is rare, information about the best way to deliver your baby is limited. It is very important that you have thorough assessments, preparation and good coordination of your care. Your EB team will support you during your pregnancy to make sure this happens.

After giving birth

Feeding

Most people with more severe forms of EB choose to bottle feed because the nipple area is fragile. If you do choose to breast feed, your midwife will need to work closely with you to help your baby to latch on properly. If you get pain and blistering of the nipple, nipple shields might be helpful.

A new baby with EB

If you think your baby has inherited EB, you should contact the paediatric EB team at either of the national centres for children:

• Birmingham Children’s Hospital
• Great Ormond Street Hospital

National centres for EB

There are 4 national centres for EB in the UK:

• Guy’s and St Thomas’ Hospital
• Heartlands Hospital
• Birmingham Children’s Hospital
• Great Ormond Street Hospital

The EB service at St Thomas’ Hospital runs in collaboration with the charity DEBRA, which is a national charity supporting people affected by, and working with, EB.