Severe epidermolysis bullosa (EB): puberty in girls

Puberty is the time when your body starts developing to become an adult.

In girls, puberty usually starts somewhere between eight and 13 years old. In boys a little later – somewhere between nine and 14 years old.

Everyone’s body is different, so everyone will start puberty in their own time and will experience it slightly differently. Puberty takes place over a period of months, and you might notice some changes more than others.

The main changes you might notice when you reach puberty are:

• You grow taller and your body changes. You may notice your arms and legs getting longer, and that you need bigger shoes and clothes. Your hips might start getting wider, and you might notice your body storing fat in certain areas, such as your bottom, hips and thighs.
• Hair growing in new places. This can happen underarms and around your pubic area – the area around your vulva, and around and between the top of your legs. This hair may be soft at first and then become thicker.
• Becoming sweatier. You may notice you have a stronger body smell and feel the need to shower more frequently or use deodorant.
• The skin on your face may may become oily and you may get spots or acne.
• Breast development. Breast buds appear, usually beginning with tender lumps under the nipples. They grow gradually over several years. Some girls may develop fuller breasts very quickly and others may stay small. Both are normal.
• Feeling more emotional. Hormonal changes can make you feel more teary and stronger emotions than before. You may become more independent and want more personal space or privacy than when you were younger. You might experience mood swings, where your mood feels unpredictable.

Starting your period (menstruation). At some point during puberty, you will get your first period. During a period, blood leaves the body through the vagina, usually once a month. Periods can be irregular, light or heavy to start with – this is expected. Some females also experience some discharge – clear or whitish fluid – before they get their first period.

Girls with severe EB can experience delayed puberty – when puberty starts later than usual. This can be for various reasons:

• Nutrition - Patients with EB have very high calorie requirements. If the body doesn’t get enough nutrients, it may delay puberty. Key nutrients in the diet contribute to bone health and overall growth. If these are missing, then puberty can be delayed.
• Low body weight - If body weight is affected by EB, this may delay puberty as the body doesn’t have enough stored energy which can affect growth hormones. These growth hormones are responsible for the changes that happen during puberty.
• Chronic wounds and inflammation - Severe EB can cause constant skin damage, ongoing inflammation and recurrent skin infections. Ongoing inflammation can signal to the body that it is not ready to start puberty.
• Hormone disruptions - The body’s hormone system controls puberty. Long term illness, stress or poor nutrition can interfere with:
  • The brain signals to start puberty.
  • Hormones made by the female hormone system (the HPA axis).
  • Overall growth hormone levels.

Puberty triggers hormones that help build bone strength and muscle mass. People with severe EV may already have weaker bone strength and smaller muscle mass. If puberty happens too late, girls with EB are more likely to have weaker bones which can cause fractures and ongoing issues with pain.

Puberty also isn’t just physical – it also supports:

• Confidence
• Independence
• Emotional regulation
• Social skills and identity

When you are around 11 or 12 years old, we will start checking how your body is developing. This may start sooner if you or your parents report early changes. We will do some checks and ask you some questions, so we can make recommendations for treatment if you need it. These can take place with your parent or carer present, or on your own – whichever you prefer.

These assessments include:

• Tanner stage assessment - During your regular hospital appointment or home visit, the doctor or nurse will check your breast and pubic hair development. This is likely to be at a time when you have to take your dressings off anyway – for example before a bath or full dressing change. This will be done respectfully and privately, and we will always make sure both you and your parent/carer consent to this.
• Period history - The EB team will ask you if you have started your period. If you have started your period, they will ask how often you get it, and if your period is painful or heavy. The endocrinology team will also monitor your periods.
• Growth measurements - The EB team will regularly check your height and weight and monitor your growth on a chart.
• Blood tests - You will have regular blood tests to check your hormone levels, thyroid function, nutrient levels and markers of inflammation. These tests will help us understand more about how your body is changing and if any support is needed.
• Routine x-rays - To help tell us how your bones look and whether bone growth is delayed.
• Nutrition and health - The dietitian will monitor your diet and calorie intake, weight gain, wound healing and inflammation to assess if you have started puberty.

Everyone with EB is different, even if they have the same type of EB. The EB team will discuss with you whether treatment might be helpful to you, and what options there are. These may or may not include:

• Referral to Endocrinology - Endocrinology focuses on the endocrine system, which regulates the hormones. Endocrinology may suggest hormone therapy such as estrogen or progesterone patches. These patches can be safe to use on EB skin if they are removed with a suitable medical adhesive remover (MAR).
• Treating anaemia or nutrient deficiencies with additional supplements - Correcting these may help the body ‘switch on’ to jumpstart puberty.
• Managing chronic inflammation - The EB team may look for areas of ongoing infection/inflammation that could be managed differently to reduce pain and stress in the body.

Finding the right period products when you have EB can be challenging, and a lot of it comes down to personal choice and what works for you.

Our general advice would be to use a gentle and non-sticky pad, and to avoid anything that is too adhesive (sticky) or has to be inserted such as a tampon. The most popular options tend to have soft/natural fibers such as:

• Reusable period pants - These are like regular knickers but are super absorbent and can be used instead of a pad. They can help avoid friction or adhesion and come in lots of different styles. Look for brands that are made from soft cotton or bamboo to minimize any skin irritation. Most large supermarkets and chemists sell period pants or you can buy them online.
• Reusable sanitary pads - These are absorbent pads which go inside your regular underwear. They are so non-adhesive (non-sticky) so can help to minimize any potential skin damage. Cotton or bamboo are a good choice. Brands available include ‘Honour your flow’ and ‘Cheeky Wipes’. These can be purchased online.
• Eco-friendly sanitary pads - These are absorbent pads which go inside your regular underwear. These tend to be better for girls with EB as they contain fewer chemicals and synthetic fragrances which can potentially cause irritation to very sensitive skin. ‘Flo’ have a wide range of products made from bamboo that are available on their website and in supermarkets. ‘Weareriley’ also offer a wide range of sanitary products made from 100% organic cotton which have no harsh chemicals.

You may prefer to use standard products such as adhesive pads or tampons – this is fine as long as you are comfortable. Use whatever works and is the most comfortable option. Sometimes using a barrier cream around the edges of a pad or lubricating a tampon can also minimize skin damage or friction.

As your breasts grow, you might want to start wearing a bra for support and comfort. You might find a crop top or bralette more comfortable than a structured bra with wires or clasps.

We suggest picking underwear made from materials that your skin can already tolerate. Natural materials such as soft cotton, bamboo and silk are likely to irritate your skin less than synthetic (man-made) materials. Cotton is a good choice as it is breathable and does not retain moisture. Bamboo is also very soft, anti-bacterial, and often odor-resistant. Some silk garments may be available on prescription, such as Dermasilk, and are very effective at helping to minimize friction.

All young people wonder about puberty, and it’s important to talk to your parents or a trusted adult about any questions you have. The EB team are very happy to discuss any worries or fears about reaching puberty. Growing up can be stressful and new for everyone, but it’s important to remember puberty is natural and there is nothing to be embarrassed about.

Regardless of having EB, everyone develops at their own pace. Although having EB can naturally make puberty start later, by assessing how you’re developing we can help support you to be healthy and we can offer support or treatment early if it’s needed.

DEBRA is the national charity that supports individuals and families affected by EB.

They provide information, practical help and professional advice, and fund research into the condition. To find out more please visit debra.org.uk.