a) WHAT IS AVAILABLE
AND WHAT IT DOES1
1 In the UK, people with Addison’s
disease are entitled to free NHS
The adrenal hormones, cortisol and aldosterone, are
essential for life. Their technical description is steroid
prescription medication, by asking
hormones. Your steroid medication aims to replace
their GP to authorise a Medical
Exemption Certificate for them. them in a manner that approximates the natural
rhythms of the body.
HYDROCORTISONE is Most Addisonians are prescribed a 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 increase their fludrocortisone dose.
and loss of concentration enough aldosterone. Getting it right depends on Cortisone acetate is an older version of
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 liver into a form the body can use.
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. are shown in the table below.
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 20mg
aldosterone steroid normally identify the symptoms of being over-medicated, you *Prednisone 5mg
produced by your adrenal will need to review this with your doctor. *Dexamethasone 0.75mg**
glands. Lack of aldosterone *Cortisone acetate 25mg
* 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 of the potency of dexamethasone.
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 Hydrocortisone is the preferred drug
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% Hydrocortisone has a further advantage
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
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
steroid. medication is stable. taken salt tablets when I have
In the UK, the 1996 survey found that 85% of Addisonians who took part in the ”
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
Medical prescribing patterns do vary by people were taking a standard dose of 0.1mg per my sodium retention and
country. In the US, the 1997 survey found that 65% day (equal to 100mcg). keeps my blood pressure up.
of Addisonians were taking hydrocortisone. The Too much fludrocortisone can lead to a 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
dexamethasone. 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 You will need to monitor your own
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 People with secondary adrenal failure
now I need less on cortisone acetate. KELLY
” usually keep their adrenal production of aldosterone
so, in almost all cases, do not need to take
fludrocortisone. Similarly, individuals who are
experiencing steroid-induced adrenal suppression
do 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 hours before going to bed, is best.
gradually taper off during the day. That is why you Aldosterone production follows a similar
will be advised to take your largest divided dose first pattern to cortisol, with the highest levels naturally
thing in the morning. 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 medication.
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
of hydrocortisone for both men and women as taking a larger daily dose.
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
believe this is inappropriate. hydrocortisone suitable for your symptoms.
“When I was first diagnosed Most women do not need as much as 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 I 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 I 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
as low as possible.
2 Some petite women may, in fact, be able to start on a dose of
15mg hydrocortisone per day, particularly if they are also taking
contraceptives or other female hormone replacement therapy.
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 in three divided doses:
benefits. For example, if you commonly experience I 20mg on waking
an energy-lag in the afternoon, it may help to switch I 10mg with lunch
from taking your medication twice a day to taking it I 10mg at 6pm
three times a day.
“I find it important to split It prevents the energy lag that many 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
am able to continue with my while others describe it as feeling low on blood hydrocortisone dose to 25mg:
physical and usually stressful sugar, faintly dizzy, or just irritable. I 12.5mg on waking
work. Don’t be afraid to take For people taking prednisone or I 7.5mg with lunch
your medication in smaller, dexamethasone, the timing of divided doses is I 5mg at 6pm
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 I 7.5mg hydrocortisone on waking
take on time. But I can tell I 1mg prednisone at 6pm
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 10mg on waking
I start my day I need my I 5mg with lunch
medication. ALAN I 5mg with dinner
e) MONITORING YOUR
MEDICATION: BLOOD TESTS
Thorough blood tests are available in the UK to help
you determine the right daily dose of medication for
you. Remember that these are tests to monitor your
ongoing medication, not tests to determine whether
you still have the disease. Some of these tests can be
done by your GP while others need to be conducted If you cannot travel to a major hospital for a day
at a hospital laboratory. curve, it is still possible for your GP to monitor
the 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
by a GP.
The most comprehensive test of
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
The most widely used test of how well 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- dose adjusted as necessary.
afternoon blood samples recording cortisol and
“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 Following a day curve analysis, your 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 been stabilised.
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 your treatment.
to adjust their steroid medication. Anti-epileptic medications (such as
Contraceptive pills and Hormone 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 of cortisol-binding globulin available.
woman taking the pill or HRT is usually comfortable If you should need ulcer-healing drugs
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
bones and on the back of the neck. 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
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
(pharmacological doses). 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 20mg
hydrocortisone or less do not need to be taken
h) REPLACEMENT OF OTHER
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 Anyone who is considering taking DHEA is 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
are already doing so. ”
to more horse riding. JANE
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 the same fate. ”
three days later. JAMES
predict the strength or consistency of the product. Complementary therapies such as
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
contain real liquorice. day. Massage and yoga can help with the joint and
muscle aches which a number of Addisonians
experience. Regular physiotherapy can also bring
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). Travel and Crisis Management.
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. Many
medications 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.
months’ supply at home.
A variety of blood tests are available to help you and
At temperatures higher than 30˚ Celsius
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 that
you 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 an
injection 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.