Pregnancy, Addison's and adrenal insufficiency

Planning a Pregnancy

It is recommended that all women with Addison’s or adrenal insufficiency who are planning a pregnancy have pre-pregnancy counselling. Ideally, this discussion will involve endocrinologists who manage women with Addison’s during pregnancy. Sometimes, it takes place in a joint clinic, where endocrinologists and obstetricians work together. You will have the opportunity to ask questions about pregnancy and labour, or delivery. It is a good opportunity to make sure that your steroid replacement regimen is optimised before getting pregnant, and ensure that any other health conditions you have are under control.

Some women will be able to access specialist clinics for pre-conception counselling by discussing their plans for pregnancy with their usual endocrinologist, who can then refer them on. Others may find it hard to know who to ask for help. You can use your routine clinic appointment as an opportunity to talk to your own team in the first instance. Most patients with Addison’s will have a regular 6 or 12-month appointment with their hospital team. If you are seen less regularly, this is a good reason to request an earlier review.

Steroid Treatment in Pregnancy

You should inform your team once you find out you are pregnant so they can arrange to see you soon. If you feel well early in pregnancy, often there is no need to change your steroid replacement regimen at this stage. Once you are further on in pregnancy, typically from around 24 to 28 weeks, your team will consider whether an increase in glucocorticoid and mineralocorticoid replacement doses is needed.

Monitoring during Pregnancy

Outside of pregnancy, your clinical team consider how you are feeling, alongside measurements such as postural blood pressure changes and blood markers (sodium and electrolytes, renin level), before making changes to treatment. Clinical assessment and blood tests are still important in pregnancy, but there are some subtle changes in how these findings are interpreted. Different stages of pregnancy can influence your circulating blood volume and your blood pressure in different ways, and this evolves and changes as pregnancy progresses. Blood test results, such as a renin level, can be trickier to interpret because of changes in blood pressure and hormone levels in pregnancy. Sodium levels are still helpful, and of course, your symptoms remain a really important guide to treatment.

All pregnant women, regardless of whether or not they are under specialist hospital care, must book with a Community Midwife and see them for their routine appointments and standard scheduled care throughout pregnancy. Therefore, during a pregnancy with Addison’s, you will still see your Community Midwife for a booking appointment and regular checks in the community during and after pregnancy.

If you have Addison’s, you may also have one or more additional autoimmune conditions. Some of these conditions will need extra care in pregnancy, such as type 1 diabetes or treated hypothyroidism. Others, such as pernicious anaemia, do not tend to be affected by pregnancy. If you are under a specialist obstetric endocrinology clinic (an antenatal clinic with an endocrinologist working alongside the obstetrician), this team should manage all your conditions holistically.

Some women will develop pregnancy complications, and this is often unrelated to having Addison’s disease. Gestational diabetes (GDM) is a common condition in pregnancy and does occur in women with and without Addison’s disease. If you develop GDM, this will influence your schedule of care throughout pregnancy, including the frequency of scans and timing of delivery.

Nausea and Vomiting in Pregnancy

Many women will experience nausea and vomiting in pregnancy. This needs more consideration for people on regular steroid medication who are at risk of developing a crisis if they can’t keep down fluids and tablets. You should be clear on sick day rules before heading into a pregnancy, and the usual sick day rules still apply. If you have nausea and vomiting, there should be a low threshold for admission to hospital. Often in early pregnancy, you will be admitted under the care of the medical team, and later in pregnancy (early second trimester onwards), your local pregnancy unit can see you and arrange admission for treatment. It is worthwhile checking with your team how local arrangements work, so you are clear on the pathway for getting in touch to get help if you feel less well.

There are treatments for nausea and vomiting in pregnancy that can safely be used from early pregnancy. Other anti-sickness medications are considered less safe until later in pregnancy (late first trimester) because of an association with cleft palate in a small number of babies exposed to this treatment during pregnancy. If your symptoms are slow to improve, we can sometimes use a combination of treatments. If needed, your GP should be able to start a medication for nausea or vomiting in early pregnancy. If your symptoms get worse, or are not controlled on the initial treatment, it’s really important that your specialist team know about this and can review your ongoing care.

A small proportion of all pregnant women will develop hyperemesis gravidarum. This condition is a serious condition involving persistent nausea and vomiting with weight loss. If you develop this and have Addison’s disease, you should be managed as an inpatient. This will allow hydration with intravenous fluids, treatment with anti-emetics and administration of injected steroid (either hydrocortisone 6 hourly intramuscularly or intravenously, or a continuous infusion of hydrocortisone across 24 hours). Occasionally, high-dose prednisolone (above standard steroid replacement doses) is used in the management of hyperemesis gravidarum. When this is used in patients with Addison’s, you need a careful plan for the management of your steroid replacement and overall treatment. In this situation, you must be under the care of a multidisciplinary team that includes an endocrinologist and an obstetrician.

Adrenal Crisis in Pregnancy

Women with Addison’s are at risk of having an adrenal crisis during pregnancy, and the risk of having a crisis is higher than when you are not pregnant. Women with vomiting in pregnancy, or with hyperemesis gravidarum, are particularly at risk. It is more likely that a crisis will occur during the first trimester, but it can occur at any point during pregnancy or afterwards, during the postnatal period. Awareness and education around adrenal crisis in pregnancy is incredibly important, for both patients and the healthcare teams looking after them. This is one of the reasons you should be looked after by a team who are familiar with caring for patients with Addison’s during pregnancy.

Do not hesitate to administer hydrocortisone when pregnant. Hydrocortisone is inactivated in the placenta and does not affect the unborn baby. However, failure to treat a pregnant person with adrenal insufficiency can result in death of the pregnant person and/or loss of the child.

If you have hyperemesis or overwhelming fatigue in early pregnancy, you may find it harder to advocate for yourself than you normally would. It’s a good idea to have a partner or family member who knows when and how to get help. Urgent medical attention is essential if you are unwell during pregnancy.

Steroid cards, medic-alert jewellery and emergency kits remain an incredibly important part of your toolkit for staying well during pregnancy, and in the weeks and months after you have a baby. Your team should regularly check that you have everything you need in your kit, and a plentiful supply of steroid medication.

Planning for Delivery

Having Addison’s disease will not affect your mode of delivery, so you can have a vaginal delivery or a C-section. Sometimes other things about your pregnancy might mean one or the other option is recommended by your team. There is no reason for you to have your baby early, and most babies will be born when you are full term (37 weeks onwards).

Some studies in this area do show that women with Addison’s may be more likely than those without the condition to have a C-section. There is no reason for this to be the routine plan for delivery if you are a patient with Addison’s, so do discuss options for delivery with your team. 

You will need steroid cover with injected hydrocortisone before and during delivery. The recommendations for this are laid out in the ADSHG surgical guidelines (free to download and also available in our Hospital Folder), and in national guidelines "NICE: Adrenal insufficiency: identification and management'.

Typically, women having a vaginal delivery will have injected hydrocortisone from the point they are in active labour (usually around 4cm dilated): this can be hydrocortisone injections 6 hourly, or a continuous IV infusion of hydrocortisone. Once the baby is born and you can have something to eat and drink, you can restart oral steroids. This will be at double your usual dose initially.

Women who have a C-section will have an injected steroid or be on a steroid infusion, from just before surgery. When you are in recovery or back on the postnatal ward, you will be offered something to eat and drink. Oral steroid replacement can be resumed at this stage, usually at double the dose for 48 hours.

After you have a baby

Most women will need sick day doses of hydrocortisone for 48 hours after delivery. After this, you can go back down to your usual steroid replacement regimen. If you have complications in the days following delivery, you might need to continue higher doses of glucocorticoid (usually hydrocortisone) for longer.

Most women are back on their usual steroid replacement from around 48 hours after delivery. If these doses during pregnancy were a higher total daily dose of hydrocortisone than your pre-pregnancy regimen, you can gradually titrate this back down with advice from your endocrinologist. Your fludrocortisone dose will also be reviewed by your endocrinologist after you give birth. You will usually be seen back in the endocrine clinic a few weeks or months after having your baby.

Whilst breastfeeding your baby doesn’t always necessitate updosing, we know anecdotally that some breastfeeding mums have needed to updose, particularly when breastfeeding at night.  If you have another condition such as POI (premature ovarian insufficiency), this will also mean you need to take that into consideration. You can talk to your team in the antenatal clinic about your plans for feeding and any low cortisol symptoms that you might be experiencing.

Real-Life Experiences

ADSHG member Rachel kindly shared with us the story of her pregnancy from fertility treatment to welcoming her son into the world - all whilst managing her adrenal insufficiency. Thank you Rachel for sharing your story.

Rachel's Story

As Endocrinology Advanced Nurse Practitioner, Lisa Shepherd comments: "Rachel's story highlights the complexities of managing multiple endocrine disorders during pregnancy. With the support of all her experienced multidisciplinary team (MDT), she managed her pregnancy well and is now the proud mother of a little boy."

For further support from others experiencing similar situations, please visit our online forum. There you can speak with others on 'Women's Health: Menstruation, Pregnancy and Menopause.'