Addisons.org.uk

MEDICATION
A few people with autoimmune Addison’s develop platelets), Sjogren’s syndrome (dry eyes and mouth) a combination of related autoimmune conditions, which are known as a polyglandular autoimmune directly inherited condition. But a tendency to Non-endocrine autoimmune diseases
autoimmune diseases does seem to run in some families. Where autoimmune Addison’s occurs on autoimmune Addison’s. Although these are seen less its own, some kind of family association with the frequently than the endocrine conditions mentioned condition can be traced in about one-third of cases.
above, a very small number of people find they Where it occurs as part of a polyglandular syndrome, some kind of family history of related autoimmune autoimmune conditions related to their Addison’s. diseases can usually be found in about half the cases. of individuals with autoimmune Addison’s develop endocrine disorders is inherited, it is often not the pernicious anaemia (vitamin B12 deficiency). Much same condition as the parent/grandparent but some LIVING WITH ADDISON’S DISEASE An Owner’ smaller proportions are estimated to develop other related autoimmune condition, which appears conditions such as vitiligo (loss of pigmentation from in the next generation. For example, a grandmother parts of the skin), coeliac disease (gluten allergy), with Addison’s disease may see one of her alopecia (hair loss), myasthenia gravis (muscle grandchildren develop vitiligo or a thyroid condition.
wasting), thrombocytopenia purpura (loss of blood a) WHAT IS AVAILABLE
AND WHAT IT DOES1
1 In the UK, people with Addison’s The adrenal hormones, cortisol and aldosterone, are 3 MEDICATION
essential for life. Their technical description is steroid prescription medication, by asking hormones. Your steroid medication aims to replace them in a manner that approximates the natural HYDROCORTISONE is
Prednisone and dexamethasone are longer-acting prescribed to replace the combination of hydrocortisone and fludrocortisone
steroids and take longer to become active in the cortisol steroid normally to replace the cortisol and aldosterone hormones.
bloodstream after being swallowed. Prednisone and produced by your adrenal The exact dose depends on the size and metabolism
dexamethasone are normally taken less often and in glands. Lack of cortisol is of the individual, as well as how advanced their
larger single doses. Individuals who switch to these what causes the muscle condition is. In the early stages of the disease many
drugs from hydrocortisone may find they need to weakness, low blood sugar individuals are still able to produce some cortisol and
and loss of concentration enough aldosterone. Getting it right depends on
commonly experienced by collaboration between you and your medical
hydrocortisone and less readily absorbed. It is also Addisonians.There are several practitioners; they must monitor your blood tests
slower acting, as it must first be processed by the substitutes for hydrocortisone, and you must monitor your well-being in response
the most common ones being to different levels of medication. In the end, a large
Each of these other drugs has a different prednisone, dexamethasone part of your mutual success will be due to a
strength to hydrocortisone; roughly equivalent doses and cortisone acetate. considered process of experimentation.
Over time, an individual’s need for each FLUDROCORTISONE is medication may alter slightly. If you feel a return of
ROUGHLY EQUIVALENT DOSES OF STEROIDS*
prescribed to replace the any of your former Addison’s symptoms, or start to
*Hydrocortisone
aldosterone steroid normally identify the symptoms of being over-medicated, you
*Prednisone
produced by your adrenal will need to review this with your doctor.
*Dexamethasone
0.75mg**
glands. Lack of aldosterone
*Cortisone acetate
* Each of these drugs has slightly different properties, causes the electrolyte balance
Cortisol replacement
which should be discussed with your endocrinologist. in the body to become Hydrocortisone is usually taken in three or two small
** See Glossary for a more thorough discussion destabilised as the kidneys doses over the course of the day. You may find it
retain potassium, the body helpful to adjust the timing and amount of each
loses salt and fluids, leading divided dose to match changes in your lifestyle.
to low blood pressure and
dehydration. because it is quickly absorbed and the closest
mimic of what the body would naturally produce.
“I was on cortisone acetate A hydrocortisone tablet has been almost totally
for a few years and didn’t absorbed by the stomach and is active in the
think too much about it. bloodstream within 30 minutes of being swallowed
When I switched to on an empty stomach.
hydrocortisone I felt 100%
better. It made me feel more over other types of steroid: the amount of it in your
‘normal’ also when my bloodstream can be accurately measured. This
endocrinologist added makes it easier to monitor your dosage and adjust
fludrocortisone. I got rid of it up or down if necessary.
the lower back pain I had had
for so many years.” SUE
MEDICATION
Why is hydrocortisone
Aldosterone replacement
the preferred drug?
Fludrocortisone is usually taken as a single daily “I recently started taking
Although it is a synthetic drug, hydrocortisone dose, first thing in the morning. It is slower acting fludrocortisone after my latest
is indistinguishable from the cortisol naturally than cortisol-replacement steroids, so people who renin test showed that my
produced by the adrenals. It is also fast acting and only require a small amount can take it every aldosterone output is waning.
can be measured in the blood. For these reasons, second day. There is a wide variation in how much I only have to take it every
hydrocortisone is the preferred drug for most fludrocortisone individuals need to take. A small other day so far, and
Addisonians. But it may not suit everyone. Some number of Addisonians find they do not need supplement with a little extra
individuals find they do better on a longer-acting fludrocortisone once their hydrocortisone salt here and there. I have
taken salt tablets when I have
been in the heat.” KARLA
around 89% of all Addisonians were taking 1996 UK survey were taking fludrocortisone. The hydrocortisone. Of the rest, 6% were taking amounts taken by individuals ranged from 0.025mg “I take fludrocortisone at
prednisone and 5% were taking cortisone acetate. every other day up to 0.4mg per day, while most 0.2mg a day. It helps with
people were taking a standard dose of 0.1mg per my sodium retention and
country. In the US, the 1997 survey found that 65% keeps my blood pressure up.
of Addisonians were taking hydrocortisone. The But the main reason it’s
remainder were taking either prednisone (20%) or potassium deficiency which, in severe cases, causes such a high dose is that
cortisone acetate (15%) while just 1% was taking an irregular heatbeat or other cardiac symptoms.
my potassium wants to
Too little fludrocortisone can lead to a potassium climb to near fatal levels
overload, which also causes cardiac symptoms. without it.” TRACEY
“I did terribly on hydrocortisone (roller coaster days) and
terribly on dexamethasone (too potent and I became
response to your fludrocortisone dose and inform Cushingoid). I felt pretty good on prednisone.That was the best
your medical practitioner if you experience new until my latest endocrinologist put me on cortisone acetate. I’m
symptoms, which you think may be related. It is happy to say it is the one for me! I did find I needed differing
important not to modify your fludrocortisone amounts of fludrocortisone on the different corticosteroids. I
medication before checking with your doctor.
needed more on prednisone than I did on hydrocortisone.And
now I need less on cortisone acetate.” KELLY
usually keep their adrenal production of aldosteroneso, in almost all cases, do not need to takefludrocortisone. Similarly, individuals who areexperiencing steroid-induced adrenal suppressiondo not usually need any fludrocortisone. b) THE NATURAL DAILY CYCLE OF
ADRENAL HORMONE PRODUCTION
In a person with healthy adrenals, cortisol levels start steroid medication too late in the evening can cause to rise around 4am, are at their maximum around sleeping difficulties. Around 6pm, or at least four the time you wake up in the morning and then gradually taper off during the day. That is why you LIVING WITH ADDISON’S DISEASE An Owner’ will be advised to take your largest divided dose first pattern to cortisol, with the highest levels naturally occurring around the time you wake up. That is why Cortisol levels are naturally at their lowest fludrocortisone is usually taken with the morning shortly after going to sleep, so it is best to take your last divided dose of the day by early evening. Taking c) HOW DO I KNOW WHAT
IS THE RIGHT DOSE FOR ME?
Some medical textbooks still refer to the Some Addisonians find that adjusting the timing and standard practice of prescribing a single dose amount of each divided dose can be just as helpful irrespective of size and bodyweight. In practice, IT IS IMPORTANT that you do not take any
most endocrinologists and Addisonians now than the smallest possible daily dose of
hydrocortisone suitable for your symptoms.
“When I was first diagnosed
Over a period of years too much can lead
in 1979 I was put on 15mg 30mg hydrocortisone while some large, well-built
to damaging side-effects such as glaucoma
hydrocortisone as I am quite men do need more. Some small, lightly-built women
and osteoporosis.
petite.This dosage gradually can live comfortably on a dose of less than 20mg.
As a general rule, taking more steroid than increased to 30mg over many Similarly, lightly-built men need less than 30mg.
your body strictly needs for a day or two is not years.Then, about six years ■ A rough rule of thumb is that both women and men
harmful. Taking too much steroid for longer periods ago, there were other health should start with a daily dose of 20mg hydrocortisone2. of time is harmful. So you can prudently increase
problems.After that I slowly ■ Then if you still feel noticeably unwell, gradually
your dose for a short time if you think you are reduced my dose so that, for increase your daily dose in increments of 2.5mg until
developing a serious illness. But for your everyday the past two years, it has been you feel well enough to live a normal daily life.
medication, the aim should be to keep your dose just 12.5mg a day.” SUSAN
2 Some petite women may, in fact, be able to start on a dose of15mg hydrocortisone per day, particularly if they are also takingcontraceptives or other female hormone replacement therapy. MEDICATION
d) TIMING YOUR DIVIDED DOSE
Some medical textbooks still refer to the standard NICK is in his mid-thirties and a keen runner. He used
practice of taking hydrocortisone twice a day, to take his 30mg hydrocortisone in two divided doses, early morning and evening. In practice, many 20mg before breakfast and 10mg at 6pm. Following a endocrinologists and Addisonians agree that taking day curve analysis he now takes 40mg hydrocortisone a smaller, divided dose more frequently has real benefits. For example, if you commonly experience an energy-lag in the afternoon, it may help to switch from taking your medication twice a day to taking it “I find it important to split
CATHARYN is in her early forties and a full-time
my medication up into three Addisonians experience in the afternoon when their
mother. She used to take 30mg hydrocortisone in daily doses to avoid those cortisone levels are at their lowest. Some people
two divided doses. After switching to three divided highs and lows. Doing this I describe this energy lag as a kind of ‘brain fog’,
doses she found she could cut down her total LIVING WITH ADDISON’S DISEASE An Owner’ am able to continue with my while others describe it as feeling low on blood
physical and usually stressful sugar, faintly dizzy, or just irritable.
■ 12.5mg on waking
work. Don’t be afraid to take
■ 7.5mg
your medication in smaller, dexamethasone, the timing of divided doses is
spread-out doses.Taking it usually less critical, as the medication stays in the
with food helps to slow the bloodstream for longer. This can be useful for
MIKE is a senior civil servant. For many years he
body’s absorption rate.” individuals whose lifestyle makes it difficult for them
took 15mg hydrocortisone in two divided doses. Since JAMES to take medication during the day. However, these
introducing a low dose of prednisone in the evening, drugs also take longer to become active after being he has been able to reduce his total daily dose to the “The morning dose is the swallowed, which can be a disadvantage at times.
equivalent of just 12mg hydrocortisone. He takes: most important for me to
■ 7.5mg
take on time. But I can tell
the difference with the
afternoon one if I am more
KAREN is in her late thirties and runs her own
than an hour late.The timing
business as well as bringing up a young family. can change with how the day
Before her day curve she was taking 20mg goes, if there are a lot of
hydrocortisone a day in two doses: 15mg on stresses. Once I got up at 4am
waking and 5mg with dinner. Following her day and by 7am I was on the way
curve, she now takes it three times a day: out. I learnt then that once
I start my day I need my
medication.” ALAN
e) MONITORING YOUR
MEDICATION: BLOOD TESTS
Thorough blood tests are available in the UK to helpyou determine the right daily dose of medication foryou. Remember that these are tests to monitor yourongoing medication, not tests to determine whetheryou still have the disease. Some of these tests can bedone by your GP while others need to be conducted If you cannot travel to a major hospital for a day curve, it is still possible for your GP to monitorthe adequacy of your hydrocortisone dose in TESTS FOR HYDROCORTISONE
TESTS FOR FLUDROCORTISONE
consultation with an endocrinologist. The 24 hour 8am plasma cortisol
Plasma renin
urine sample and the mid morning/mid afternoon 8am plasma ACTH
Electrolytes (potassium and sodium)
samples of cortisol and ACTH can be administered Day curve analysis
Blood pressure
hydrocortisone medication is known as a day curve Day curve analysis
and is usually done through the endocrinology Different hospitals have different procedures for a department of a major hospital. If you have not day curve, although all follow the same general had a day curve done, you may wish to ask your principles. Most are done on an out-patient basis. GP if they could refer you to a suitable hospital.
The most comprehensive day curve analysis involves taking blood samples over an 11 hour day the adrenals themselves are functioning is the starting early morning before you take your first ACTH stimulation test. Most Addisonians will have divided dose of medication for the day. Repeated undergone an ACTH stimulation test to confirm their blood tests during the day track the medication diagnosis with the disease. A few Addisonians are entering your blood stream when you take each asked to repeat this test over time to assess any dose and record how long it lasts. The amount of further deterioration in their adrenal function.
hydrocortisone in your bloodstream during the day Some endocrinologists do not offer a full can then be compared to the ideal for a healthy day curve analysis, preferring to use a combination individual, and the amount and timing of each of 24 hour urine analysis and mid morning/mid- afternoon blood samples recording cortisol andACTH levels. MEDICATION
“It took me a while to get the When you go for a day curve do remember to
blood levels of cortisol were significantly higher than medication into a good check if you are allowed to eat breakfast before you
those of a healthy person for long periods of time.
balance after my diagnosis. It arrive. Make sure you bring all your normal daily
Most specialists agree that Addisonians do really took me a couple of medication with you including any medication you
not need to have a repeat day curve done unless years to get it right. For a take for other conditions.
there are major changes in their general health while I was still so exhausted
or weight. For example, if you were to develop I couldn’t even walk to the specialist may advise you to reduce or increase your
a further endocrine problem - such as a thyroid letterbox and back.” JAMES current daily dose or to try taking it in smaller, more
disorder - you should probably request a repeat frequent amounts. A specialist should advise you to day curve, once your thyroid medication had reduce your dose if the day curve showed that your f) DRUG INTERACTIONS
Anyone who is taking drugs to treat tuberculosis Most prescription and over the counter drugs will (such as Rifampicin and Rifabutin) will typically need not affect your steroid medication, although it is to increase their dose of both hydrocortisone and always wise to check this with your doctor or fludrocortisone by up to double the normal dose, as pharmacist. However, there are a few prescription anti-tuberculosis drugs speed up the rate at which drugs that influence the way hydrocortisone and the body metabolises its steroid medication. These other steroid medications are metabolised by the anti-tuberculosis drugs are highly potent, so you body, either slowing down or speeding up the rate will need to make sure your condition is being at which the steroids are metabolised. This means thoroughly monitored by your doctors throughout that anyone taking these drugs will probably need phenytoin) can also speed up the metabolism of Replacement Therapy (oral oestrogens) slow steroids. Growth hormone treatment can lower down the rate at which the body metabolises blood levels of cortisol, by reducing the amount hydrocortisone by around one-third. This means a woman taking the pill or HRT is usually comfortable on a hydrocortisone dose that is one-third less than (such as carbenoxolone) you may need to reduce she would otherwise need. If she continues taking your fludrocortisone, as these drugs can lower her previous hydrocortisone dose, she may develop potassium levels. If you should need to take anti- mild signs of over-medication, such as a puffy face depressants, you may need to ask your doctor to and little fat deposits on the tummy, above the collar monitor your electrolytes, as some types can cause the body to lose more sodium than usual.
g) MEDICATION AND FOOD
Hydrocortisone prescriptions are usually issued with Swallowing your tablets just with water makes it instructions to take this medication with food. In easier to take your first dose for the day as soon as practice, most Addisonians can take their tablets you wake up. Waiting to take your first tablet with with just water without experiencing indigestion.
breakfast means a delay in getting the steroid into LIVING WITH ADDISON’S DISEASE An Owner’ This is because the amounts of steroid we swallow your bloodstream, during which time you will feel are smaller than those taken by individuals with less well with no hydrocortisone in your blood. medical conditions needing supplementary steroids It is also advisable that, if you wear contact lenses, you should put these in before taking If you find you do experience indigestion hydrocortisone tablets, so that there is no risk of taking your steroid medication with water, a glass getting any traces of the medication in your eyes.
of milk (or milk substitutes such as soy, rice milk)is usually all that is needed to prevent indigestion.
As a general rule, individual doses of 20mghydrocortisone or less do not need to be takenwith food.
h) REPLACEMENT OF OTHER
ADRENAL HORMONES?
Addisonians are usually lacking in one other major only, and none of the trials lasted longer than four group of adrenal hormones: DHEA and its related months. A follow-up study in the UK is taking place “I have been taking part in
compounds. DHEA is not available in the UK at during 2001/2 and this will last a full 12 months.
trials of DHEA over the past
present. However, during the late 1990s several two years.The first study was
clinical trials in the UK and internationally concluded advised to discuss this with their doctor and have a double blind study taking
that there were moderate benefits for most their blood level measured before they do. Although the real McCoy for three
Addisonians from taking small quantities of DHEA.
clinical trials to date have recommended a daily dose months and a dummy for
These benefits included protection against of 25-50mg, not all Addisonians will need as much three months. I knew when I
osteoporosis, greater energy, enhanced levels of as this. A few individuals have reported that doses as was on the real tablets as I
libido and lean muscle, and relief from dry skin. Side low as 10mg brought their levels up to the normal had more energy but the side
effects noted by some individuals were acne, greasy range. Bear in mind that some endocrinologists are effects were acne on my face,
skin and moderate weight gain. Two of the three cautious about prescribing DHEA in advance of the chest and scalp. I lost weight
clinical trials involved very small numbers of women results from longer-term clinical trials, while others but that may have been down
to more horse riding.” JANE
MEDICATION
i) ALTERNATIVE AND
There are no herbal therapies available which can
“I know of another male
COMPLEMENTARY THERAPIES
mimic the effects of natural human cortisol. Until
Addisonian who lived 20
Liquorice root has stimulatory properties similar steroid medication became available in the 1950s, miles north of here, but he
to aldosterone. If you eat real liquorice in addition Addison’s disease inevitably resulted in death over was ‘cured by faith’ and threw
to your fludrocortisone medication, you risk over- a period of time. Addisonians who experiment with away his medication. He
medication. Liquorice root is not recommended as ‘natural’ alternatives to their normal medication risk dropped dead in his house
a substitute for fludrocortisone as it is not possible to three days later.” JAMES
predict the strength or consistency of the product.
Most liquorice sweets are, in fact, only liquorice- meditation, massage and yoga can offer benefits.
flavoured and can safely be eaten - but do check Meditation can be a useful way of dealing with a the list of ingredients. Some cough mixtures also build-up of fatigue, tension or irritability during the day. Massage and yoga can help with the joint andmuscle aches which a number of Addisoniansexperience. Regular physiotherapy can also bring LIVING WITH ADDISON’S DISEASE An Owner’ real relief for joint and muscle aches.
j) STORING YOUR MEDICATION
the contents become cloudy or coloured. Injection Most prescription medication is stable when stored kits are discussed more fully in sections 9 and 10: between 0˚ and 25˚ Celsius (32˚ and 77˚ Fahrenheit).
Always check the expiry date on your medications,especially your injection kit, and dispose of any MEDICATION: A SUMMARY
medication that reaches its expiry date. Manymedications have a shelf-life of around two years, A combination of hydrocortisone and fludrocortisone
so it is seldom worth keeping more than six is the most usual treatment for Addison’s disease.
A variety of blood tests are available to help you and
your doctors establish your correct baseline dosage.
(85˚ Fahrenheit), most medication will reach the end of its shelf life more rapidly than the stated You may feel better splitting your daily hydrocortisone
expiry date and may start to deteriorate (lose its medication into three divided doses.
effectiveness). For this reason, any medication thatyou carry close to the body in a pocket or bag Taking the first dose of the day on waking
should be used within a few days, unless you are is recommended.
storing it inside an insulated travelling container.
Individual doses of 20mg or less do not usually
Insulated travelling containers can be ordered need to be taken with food.
through most pharmacists. If you are carrying aninjection kit with you in this kind of hot weather, it The last dose should be taken no later than
should be discarded after 12 months, or sooner if 4 hours before bedtime.
a) EXPERIENCES COMMON
TO MANY ADDISONIANS
i. Osteoporosis
“I have had Addison’s for 26
Osteoporosis is more common among Addisonians CONCERNS
years. I used to be on 40mg
hydrocortisone and had done
In this manual, osteoporosis is used as a pretty well.Then eleven years
general term to cover the various types of bone ago I was diagnosed with
Over the course of a lifetime living with Addison’s disorder that can be detected by a bone scan insulin dependent diabetes and
disease, many people find themselves addressing (the medical terms used are often osteopenia, found that the insulin was
one or more of the following areas of concern. For osteomalacia and osteoporosis). messing up the medication for
simplicity, these have been grouped into three topic Addison’s, so they switched
areas: experiences common to many Addisonians; vulnerable, but medical studies of bone density me to prednisone and
experiences associated with low cortisol levels; among Addisonians have found some evidence of fludrocortisone. Now a month
experiences associated with high cortisol levels. bone thinning even among younger men. Low levels ago I had a hypoglycaemic
of DHEA are thought to be partly to blame. seizure and ended up with
Addisonians take several divided doses of medication Long-term use of higher doses of steroids several compression fractures
a day, it is possible to have experiences associated is also commonly associated with osteoporosis. By in my spine.After the x-ray
with both high and low cortisone levels over the taking the minimum daily dose of hydrocortisone they also found four old
course of a day. Don’t be alarmed if you do have needed, you should not be at increased risk of bone compression fractures which
any of the over or under-medication symptoms thinning from your steroid medication. But anyone had already healed.At which
mentioned below; with experience you will learn to who is consistently over-medicated is increasing the point they tell me I have
adjust for them. Talking it through with your doctor, osteoporosis and I am 40
reaching your optimum daily dose and (from time Because of these associations, every Addisonian
years old.The bone loss has
to time) self-medicating as outlined in the sections is recommended to take preventative steps
wigged me out because I
below, will all minimise the frequency and severity to reduce their risks of bone thinning.
am a landscape gardener by
It is never too early, or too late, to start
trade and do a lot of heavy
working on your bone density.
lifting.” BRIAN

1 HOW ‘NORMAL’ CAN LIFE BE WITH ADDISON’S DISEASE?

2 THE CAUSES OF ADDISON’S AND RELATED AUTOIMMUNE DISEASES

a) PRIMARY AND SECONDARY ADRENAL DISEASE

b) RELATED AUTOIMMUNE CONDITIONS

ii. What can I do to prevent osteoporosis?

iii. Weight gain: are steroids to blame?

iv. What helps to shed those excess pounds?

b) EXPERIENCES ASSOCIATED WITH LOW CORTISOL LEVELS

v. Why do I feel lousy at certain times of the day?

vi. Low blood sugar and the insulin response

xi. Exhaustion and ‘hitting the wall’

c) EXPERIENCES ASSOCIATED WITH HIGH CORTISOL LEVELS

xiii. Insomnia and other sleeping difficulties

xiv. Anger, mood swings and depression

i. Plenty of fluids, protein, vitamins and minerals

iii. Multi-vitamins and other supplements

a) EXERCISE IS GOOD FOR US (SO WHY ARE WE COUCH POTATOES?

b) ADJUSTING YOUR MEDICATION TO YOUR FITNESS REGIME

8 BEING THE PARENT OF A CHILD WITH ADDISON’S

a) MONITORING YOUR CHILD'S CONDITION

a) TAKE ENOUGH STEROID COVER TO ALLOW FOR EMERGENCIES

a) WHAT IS AN ADDISONIAN CRISIS?

b) COLDS AND OTHER MINOR ILLNESSES

c) SERIOUS ILLNESSES REQUIRING EXTRA STEROID COVER

Diarrhoea lasting for more than a day

d) TAPERING YOUR DOSE BACK DOWN AFTER A SERIOUS ILLNESS

e) SERIOUS ILLNESSES WHICH MAY REQUIRE AN EMERGENCY INJECTION

Stomach flu (gastric flu) and any illness involving vomiting

g) SURGERY REQUIRING EXTRA STEROID COVER

h) MEDICALERT AND OTHER USEFUL INFORMATION

Source: https://www.addisons.org.uk/info/manual/medication.pdf