As part of our focus on Women's Health for our Winter 2020 magazine, ADSHG member Rachel tells us the story of her pregnancy from fertility treatment to welcoming her son into the world - all whilst managing her adrenal insufficiency. Read to the end for Lisa Shepherd (ADSHG Trustee and Endocrinology Advanced Nurse Practitioner) perspective and professional medical insight into Rachel's story. Thank you Rachel for sharing your story.

I was diagnosed in December 2017 following a severe crisis. Before then, I had been trying to get pregnant for 3 years (almost exactly) and had had no success. My husband and I had started fertility treatment after a year.

We live in Germany, which has a bit of a different set up than the NHS. No one could explain why nothing seemed to be working, as there was no obvious reason why I shouldn't get pregnant, except my thyroid levels were all over the place. I couldn't seem to find the right L-Thyroxine dose even though my Hashimoto's disease had always been stable up to that point. With hindsight my body was in a bit of a state leading up to the crisis. 6 months after diagnosis I found out I was pregnant, only to miscarry at 8 weeks. Whilst this was not a pleasant experience, I was lucky enough that I didn't need further medical intervention and the pregnancy was confirmed and the same time as the doctors identified something was wrong. To be honest, my body wasn't really back to normal and probably wasn't up to the challenge of pregnancy.

Finding out I was pregnant

I was about to restart fertility treatment at a different place (there is a huge teaching hospital in Heidelberg where I live, with an excellent team). At our second appointment, in January 2019, when we were due to discuss a plan going forwards, we found out I was about 4 weeks pregnant. The doctor was fantastic, prescribed vaginal suppositories of progesterone and asked me to come back every week until my first scan with my gynaecologist which would be once I was 8 weeks. In Germany, pregnancies are managed by your gynaecologist. This was rather unexpected but gave us a lot of reassurance. He mostly did this due to my Addison's and the miscarriage.

Once I had seen the gynaecologist and my endo, both agreed that the gynaecologist would treat me like a regular pregnant patient and defer almost all blood work and meds etc to the endo. I breathed a huge sigh of relief as I have an excellent relationship with my endo and trust her judgment and she trusts mine. I was seen every month by both doctors to monitor me closely and I kept pretty detailed records of my BP (measured twice daily), temperature and other symptoms I was experiencing.

Managing symptoms and Addison's

Luckily I experienced very little morning sickness but the fatigue was terrible. I really struggled for the first 14-16 weeks. Otherwise, the biggest challenges were; motion sickness, a really sensitive sense of smell and difficultly controlling my body temperature. Travelling on public transport was a bit of a hassle. I always kept a supply of salty snacks and cereal bars, as these usually take the edge off the motion sickness for me. I also found that I would take naps on my day off and at weekends and would head to bed every night by about 9/9:30. I always had a bottle of homemade rehydration fluids in the fridge, rehydration ice pops in the freezer and I included multiple rehydration sachets in my emergency kits (although those live in there anyway).

Adjusting my medication doses

I started splitting my hydrocortisone into 4 doses pretty early on, taking the last dose at about 8:30pm as my sleep became even worse during pregnancy. Since my Addison's diagnosis my sleep hasn't been great anyway. Since my son was born, I actually split my hydrocortisone further and have been taking 5 doses a day:

  • 10mg at 6am
  • 5mg at 11am
  • 2.5mg at 4pm (only just managed to get this down from 5mg at about 10 months post birth)
  • 2.5mg at 8:30pm
  • 2.5mg at 1am (although this is could be anywhere from 1-3am ish depending on when I wake up/get woken up)

The night doses help with sleep and getting up to deal with night feeds. My fludrocortisone gradually increased as my pregnancy went on, but it was also the height of summer, 35°C+ temperatures are a killer before factoring in being in the third trimester! My L-Thyroxine was changed multiple times too, my thyroid readings were a little strange and dosing ended up being a discussion between the 5 endos in the practise I go to. Like many pregnant women I also ended up on a low iron dose of 50mg (100mg was rough on my stomach).

The fatigue eased a lot in the middle, but never really left. The major symptom that gradually got worse as my pregnancy went on was hot feet and a general inability to regulate my temperature. Although, I normally rarely measure above 36.5°C, in pregnancy it was more like 36.9/37°C but I felt like I was unbelievably hot. Before diagnosis, my hands and feet were always cold! My husband now jokes that you can tell when I've taken hydrocortisone in the past 30mins - 1hr as my hands and feet are warm/hot.

From about 26 weeks I was seen every 2 weeks by my endo as I developed gestational diabetes. All pregnant women are tested as standard here, but due to my endocrine history I had to do the longer test. I had issues with my fasting blood sugars but not after food. I was on insulin at night for the last trimester.

My endo gave me a detailed medication schedule for me to share with the hospital for the birth, which was very helpful. If anything I was probably over medicated, but that is definitely preferable to the alternative! This was her advice:

  • Onset or labour: x2 hydrocortisone dose, no fludrocortisone.
  • Active labour/when oral meds no longer enough/c-section: 200mg hydrocortisone bolus then 100mg hydrocortisone IV over 24hrs perfusor.
  • 1st day after vaginal birth: no fludrocortisone, reduce L-Thyroxine to 75mcg (early pregnancy dose, hydrocortisone tablets 75mg (split into normal daily dose timetable). After c-section 75mg hydrocortisone IV over 24hrs perfusor.
  • 2nd day after labour (provided feeling well): no fludrocortisone, 50mg hydrocortisone (split into normal daily dose timetable).
  • 3rd day after labour (provided feeling well): 25mg hydrocortisone (my normal hydrocortisone dose for 'work days' and return to normal dose of fludrocortisone.

With hindsight I would have also ask her to include advice about what to do if sodium levels fall following the birth. I can strongly recommend diluted fruit juice with some salt stirred in to help stay hydrated during labour.

Planning meeting with hospital ahead of labour

I had my planning meeting with the hospital a little earlier than a lot of women do, just before 30 weeks, as I had heard that it was not uncommon for babies of women with Addison's to be born early. I was able to get all the details of what needs to be done on file, meet with an anaesthetist and sign all consent forms for all eventualities just in case.

It gave both me and the doctor a chance to understand the situation. Coincidentally, the doctor I had the planning meeting with, actually was there when I delivered my son. This was great as my sodium plummeted after giving birth so he called the on call endocrinologist who gave him the advice of don't let me drink any water for 6 hours! He asked me whether this sounded right. He trusted my judgement and all was fine. I was glad I had had this meeting early on, as my son was born nearly 4 weeks early (at 36 weeks + 2 days).

My waters broke, so clearly he was ready to be born and my body was done. I wish I had asked about what would happen after the birth in the planning meeting though. I was kept on an intermediate care ward, as was still on IV hydrocortisone and they wanted to monitor my BP, heart rate etc. so my son couldn't stay with me. As it happens he had a bit of a temperature when he was born so needed antibiotics for the first 24 hours so was in a neonatal unit. With hindsight, it was obvious that he wouldn't be able to stay with me, but I didn't think to ask what would happen.

The birth 

I didn't have my heart set on a specific birth plan and was happy to just go with the flow. I wanted to avoid a caesarean, primarily as I was concerned about recovery. Luckily, labour went fairly smoothly really. My husband noticed first when I would be better switching from oral meds to IV hydrocortisone, which was quite early on (I think, it's all a bit of a blur timewise to be honest). Probably around the 15hrs after my waters broke, 2-3hours into regular contractions. I was advised to go straight to hospital when everything started to be monitored instead of waiting the usually contractions every x minutes. As my waters broke before contractions started, I had to go in earlier anyway. I decided to have an epidural at 4cm (I could still move around etc), it gave me a lovely breather which helped. It wore off pretty quickly though. I was told it would last around 2-4 hours. After almost exactly 2 hours it wore off and I was given more. I was able to have a vaginal birth, which was what I wanted in the end.

The recovery

I recovered pretty quickly really, had the usual swollen ankles, feet etc but no worse than anyone else who has just given birth and was able to reduce my meds following the recommended schedule from the endo. I had some lovely bruising from the bp cuff and IVs but that is to be expected after being on a high hydrocortisone dose.

As soon as I was back on hydrocortisone tablets I started taking them 5 times a day instead of 4, which I still do now. It really helped with managing night feeds and the lack of sleep. I'm back to my pre-pregnancy hydrocortisone dose of 22.5mg daily and fludrocortisone. My thyroid levels have taken a while to stabilise but are there now. I have recently been trialling a combination of Plenadren and hydrocortisone on the suggestion of my endo. I've now been on this for a few months and it has really helped my sleep and I have no problems getting up in the night to deal with a hungry or grumpy baby. Previously getting up before at least 30 mins after hydrocortisone was a bit iffy at best.

One of the doctors I saw after I gave birth was unsure if I should be breastfeeding due to the high hydrocortisone doses. Luckily he went away and one of his colleagues told me he searched the research (which really impressed me that he could find anything! More in Nurse Lisa's commentary below) and came back saying there was no problem due to it being a replacement dose. Other than the usual slow start (well it felt like that anyway), we settled into a feeding rhythm relatively quickly and had no problems breastfeeding.

"I had a super supportive medical team so was very lucky really and had a largely positive experience. I can't quite believe my son is now 1 year old, time has really flown!"

We asked Lisa Shepherd, ADSHG Trustee and Endocrinology Advanced Nurse Practitioner at University Hospitals Birmingham NHS Foundation Trust, to provide her perspective on Rachel's story.

Primary adrenal insufficiency is rare, therefore management of pregnancy in this patient group can be more challenging, as there is lack of prospective studies in this area. However, we do know that optimisation of replacement treatment is especially important during this time; aiding conception and reducing comorbidities. Diagnosis of adrenal insufficiency during pregnancy can be particularly challenging as symptoms can often be the same in both. Healthcare professionals should have a high level of in clinical suspicion, especially in there is a family or personal history of other autoimmune conditions (Mastorakos & Iltas, 2003, Anand & Beuschlein, 2018).

Hashimoto’s is an autoimmune condition, and people who have this are at an increased risk of developing Addison’s Disease (Boelaert et al 2010). Achievement of normal thyroid function is important when trying to conceive and also throughout pregnancy (Stagnaro-Green et al 2011).

  • Dosing during pregnancy. Little evidence exists on the exact glucocorticoid dosing required during pregnancy. The main aim of replacement therapy is to avoid under and over replacement of glucocorticoid and mineralocorticoids, whilst preventing adrenal crisis. It is recommended patients should be reviewed at least once per trimester and clinical signs and symptoms assessed. hydrocortisone doses should be increased by 20-40% from week 24 (Bornstein et al 2016, Anand & Beuschlein 2018). fludrocortisone requirement may increase during the last trimester. Clinical signs and monitoring sodium and potassium should be used to adjust. Plasma renin cannot be used to monitor as this is increased in pregnancy (Bornstein et al 2016, Anand & Beuschlein 2018).
  • Medication plan. A medication plan, liaison with other members of the MDT and in conjunction with the patient is important; optimising communication, patient care and improvement of outcomes (Epstein, 2014).
  • Management of glucocorticoids. Labour is a stressful event and additional steroid replacement is required. Guidance on dosage is available from Simpson et al 2020 and Woodcock et al 2020 which also covers the postnatal period.
  • Change in other medications. Increase in temperatures in Summer can cause salt depletion and lead to you feeling washed out. Fludrocortisone will stop you losing salt, but replenishment needs to be replaced through diet.
  • Breast feeding. Studies indicate glucocorticoid physiological replacement doses are unlikely to cause harm in breast-fed babies (Anand & Beuschlein, 2018)

During pregnancy, your care should be managed by both your endocrinologist and midwife or obstetrician. A recent multicentre survey has shown that Addison’s during pregnancy can be effectively managed with specialist care. 

“Rachels 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. ”

Do you have a story about Addison's to help or encourage others? Whatever your story or experience we would be delighted to hear from you. Tell us your story.