ADSHG Medical lectures and seminars - Addison's Disease Self ...

Addison’s Disease
Self-Help Group
Medical lectures
and seminars
A compendium 2004 - 2010
2010 medical lecture
Pages 2-5
Should everyone
with Addison’s be
taking DHEA?
Professor
Krishna Chatterjee
2009 medical lecture
Pages 6-9
Replacement therapy
in Addison’s disease:
challenges and
opportunities
Dr Kristian Lovas
ADDISON’S
S e lf H elp G ro u p
2008 medical lecture
Pages 10-13
The causes of Addison’s
Professor Simon Pearce
2007 medical lecture
Pages 14-17
Trends in the
management of
Addison’s disease
Professor John Monson
2006 medical lecture
Pages 18-22
Sex and Addison’s: how
adrenal insufficiency
affects men and women
differently
Professor Wiebke Arlt
2006 Cambridge
advanced endocrine
course
Pages 23-25
Addison’s disease
in the 21st century
Professor John Wass
2006 medical seminar
Pages 26-29
The ABC of Addison’s
Dr Simon Pearce
2005 medical lecture
Pages 30-32
You and your General
Practitioner
Professor John Wass
2005 Addenbrooke’s
endocrine seminar
Pages 32-33
Why are Addison’s
patients “difficult”
Katherine White
2004 medical lecture
Pages 33-38
Managing your steroid
medication
Dr Trevor Howlett
©ADSHG 2010

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Should everyone
with Addison’s
be taking DHEA?
ADSHG medical lecture 2010
Professor Krishna Chatterjee
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Should everyone with Addison’s be taking DHEA?
ADSHG medical lecture 2010 Professor Krishna Chatterjee (continued)
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Replacement therapy in Addison’s disease:
challenges and opportunities
ADSHG medical lecture 2009 Dr Kristian Lovas
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Replacement therapy in Addison’s disease:
challenges and opportunities
ADSHG medical lecture 2009
Dr Kristian Lovas (continued)
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The causes of Addison’s:
medical lecture report
ADSHG medical lecture 2008 Professor Simon Pearce
The adrenal glands are often referred to as the “fight or flight”
glands. This is mostly a reference to their function in producing
adrenaline, although this is only one of the four main hormones
produced by the adrenals and is not essential for life.
In Addison’s, the outer layers of the adrenal glands (the cortex)
are no longer able to produce three main hormones: cortisol,
aldosterone and DHEA. In secondary adrenal failure, aldosterone
production keeps going.
Adrenaline production by the inner part of the adrenals is
unaffected, both in Addison’s and in secondary adrenal failure. Even
people whose adrenal glands have been surgically removed still have
adrenaline, as it is also produced by nerve endings throughout the
body.
The adrenal cortex actually produces these important hormones
using cholesterol as its raw material, converting it into the active
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hormones through a series of pathways. So some cholesterol
is necessary for good health, although too much is a common
problem.
These powerful little glands have four layers, each responsible
for production of producing adrenaline, DHEA, cortisol, with
aldosterone produced by the outermost shell. There are two
separate feedback loops controlling cortisol and aldosterone, via
ACTH from the pituitary and renin from the kidneys.
Dr Thomas Addison was working in inner city London in the 1840s
and 1850s when he identified adrenal failure as a fatal condition.
Then, it was mostly triggered by tuberculosis. But some cases were
autoimmune, with associated vitiligo. Dr Addison was actually a
Geordie, born in Longbenton, then a village just outside Newcastle.
Back in the 1850s, hormones were unknown, so Dr Addison
couldn’t tell why adrenal failure led to death. It was not until the
early 1900s that the concept of a hormone was suggested.
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What causes the adrenal glands to stop working? Autoimmune
attack is the most common reason in today’s world, where
the body’s immune defences attack other cells. We don’t fully
understand what causes this to happen, or why very specific cells
are the target of attack. This is one of the things my genetic research
is trying to understand.
To give you a crash course in biology, there are two main arms to
the immune defence system, T lymphocytes and B lymphocytes.
The T lymphocytes do the serious work of attacking and destroying
infected cells, while the B lymphocytes make antibodies that stick
to bacteria and flag them as targets for the T cells. The challenge
for the immune system is that it has to know which cells to attack,
and which to protect. How does the immune system go wrong
in Addison’s? Something causes T cells and B cells to invade the
adrenal gland and start destroying the body’s own tissues. They
usually target a specific sort of protein in the adrenal cells, known as
a steroidogenic enzyme. One of the main adrenal enzymes has the
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scientific name of 21 Hydroxylase (also known as P450C21). More
than 90% of people with autoimmune Addison’s have antibodies
to this enzyme. The test for 21 Hydroxylase antibodies is the only
really reliable antibody test for autoimmune Addison’s, although,
frustratingly, it is not widely available within the NHS. A very similar
enzyme, P450C17, is associated with premature ovarian failure;
this is another autoimmune condition that commonly occurs
alongside Addison’s.
The process of adrenal destruction in autoimmune attack is a slow,
gradual one, taking many years. You might not be aware of the
symptoms until it is quite advanced and nearly all your cortisol
production is lost. In people with Addison’s, other endocrine glands
are quite often the target of a similar process of autoimmune
destruction, as the 2003 international Addison’s survey showed.
Most frequently, it is the thyroid gland that is also attacked,
resulting in hypothyroidism.
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The causes of Addison’s:
medical lecture report
ADSHG medical lecture 2008
Professor Simon Pearce (continued)
We know that autoimmune diseases run in families. In Graves’
disease, for example, around 30% of people have an immediate
family member who is affected, in diabetes this is around 6%.
Some of the medical researchers who have been collaborating
with me in our genetic analysis have looked at blood samples from
Norwegian Addison’s patients, just as I have been busy collecting
samples from as many of you as I can here in the UK. Across the
UK and Norway, we have found around 2.3% of patients have a
second family member with Addison’s. Since there is an inherited
component to these conditions, it seems logical that genetic analysis
should help to understand why things go wrong.
Genetic analysis can be a daunting challenge. The Human Genome
Project identified 30,000 functional genes, all of them made up
from 4 different DNA bases, encoding the proteins. (Proteins do
everything in your body). The major histocompatibility complex,
which contains tissue typing (transplantation) genes, is found
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on the short arm of chromosome 6. This is where much work
in autoimmune disease has focused, looking at specific variants
which seem to turn up more often in people with Addison’s. There
is one variant of HLA DR3, for example, which appears in 40% of
people with Addison’s and only 15% of health controls. Another
variant on chromosome 1, that was first associated with diabetes, is
known as PTPN22 620W. We found that this is also more common
in autoimmune Addison’s, occurring in 12% of you but just 7.8%
of healthy people. Interestingly, PTPN22 is more common in
northern European populations and less common in Mediterranean
populations, and has the highest incidence in Finland, where Type
1 autoimmune Addison’s also occurs at higher rates than other
countries.
It takes a lot of number crunching to identify the odds ratios for
the association between different genetic variants and different
autoimmune diseases, to work out which are statistically significant
and which are just random. So far, there seem to be nine different
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genetic variants associated with Addison’s and similar autoimmune
endocrine conditions. In any one individual with the condition, there
may be three or four ‘bad’ variants, and no one variant seems to be
necessary for Addison’s to develop. So this is not a straightforward
exercise. The complexity of the problem explains why Addison’s
seems to be difficult to inherit and doesn’t show up that often even
in families carrying these genetic variants. In trying to understand
how Addison’s is acquired, we are still looking at the tip of the
iceberg.
So how do you get all these funny gene variants? From your
parents, of course, but let’s look at why. We need to go back to
someone who was studying at about the same time as Dr Addison:
Charles Darwin with his theory of natural selection. European
history is full of infectious diseases that have killed off anyone whose
immune system was weaker. The Black Death in the 1300 to 1600s
was the most devastating of these epidemics, killing nearly one third
of the population, so that only the strong survived. Later waves
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of infection were less disastrous, but still severe. So the modern
population is enriched for genetic variants that fight off infectious
diseases. But in today’s world, we are only rarely exposed to severe
infections, despite our highly active immune systems. This leads to
the immune system mis-firing in some people and their adrenal
glands fall victim to “friendly fire”.
Thus, autoimmune disease is the consequence of an overactive
immune system in a clean world, just as obesity and late-onset
diabetes are the consequences of famine-adapted genes in a foodrich
world.
Finally, I would like to say thank you to the trustees of the ADSHG
and to all those of you who have generously helped me by giving
a blood sample. Our work is still ongoing, and I will continue to be
really pleased every time I get a few more tubes of blood in the post.
Simon Pearce
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Trends in the
management of
Addison’s disease
ADSHG medical lecture 2007
Professor John Monson
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Professor Monson introduced his subject on a sombre note,
reminding the audience that there are two endocrine conditions
where the sufferer is at immediate risk of death from deficiency:
insulin-dependent diabetes and Addison’s disease. Prescription
medication is an essential part of our daily existence. He outlined
the history of the disease, from its early identification by Dr Thomas
Addison, when death inevitably occurred within a few years of
diagnosis, through to the development of synethetic steroids in
the 1940s so that, ‘with care, the expectation of life should not be
shortened’.
Professor Monson then turned to an assessment of current
practices in medicine. He suggested that an understanding of
Addison’s deserves to be part of the core knowledge of all medical
students, for two reasons. It is a deficiency state which aids in the
understanding of normal physiological function and, even though
rare, it is life-threatening but amenable to life-saving intervention.
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Professor Monson then continued with an explanation of
the changes over time in the approach to maintenance therapy.
Hydrocortisone replacement used to be prescribed twice daily, which
was optimistically claimed to ‘mimic the natural circadian rhythm’.
However this resulted in poor afternoon and evening levels. The
typical dosage has changed to three times a day to aid the late
afternoon levels in the blood and to keep us safe.
Our morning dose is the most important of the day as
it kick-starts our systems. He explained that people with healthy
adrenals have very low cortisol levels in their blood between 11pm
and 4am, so at these times our natural cortisol levels are close
to normal. However, steroid levels in ‘normal’ people start to rise
gradually during sleep from about 3.30am, whereas we have to
wait until we wake up and take a tablet.
He then explained the usefulness of day curves. These help
in two ways: firstly, to ensure that the stomach is actually absorbing
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the hydrocortisone and secondly, to ensure that the blood levels are
correct at the times of day they are most needed by the body.
Professor Monson explained that cortisol binding globulin
(CBG) is the ‘carrier protein’ which transports our medication
around the blood stream. It is highest in the evening, so serum
cortisol readings for a given dose will be slightly higher later in the
day. Oestrogens increase the amount of CBG so that you get an
artificially inflated cortisol reading in pregnancy or with HRT.
Some people feel better splitting their hydrocortisone even
further to take it four times a day. However, the overall daily dose
should probably be reduced further and the practicalities of multiple
doses can be hard to manage. The last dose of the day should be no
later than four hours before bedtime. A dose of hydrocortisone lasts
approximately five hours, so that taking a dose on waking (7am),
midday and 5pm suits most people.
Measuring renin activity helps to fine-tune the
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Trends in the management of Addison’s disease
ADSHG medical lecture 2007 Professor John Monson (continued)
fludrocortisone levels. In earlier decades, renin and aldosterone
tests were not available; this has been a big advance. Generally the
person just doesn’t feel well when the fludrocortisone is too low
and their blood pressure will be low.
Physical stress in ‘normal’ people causes cortisol to rise, so
that extra hydrocortisone is needed for endurance sports. Emotional
stress in ‘normal’ people does not increase cortisol, however this
kind of psychological stress can cause physical symptoms which
would then require an increase. Nausea in Addison’s is usually an
indicator that extra hydrocortisone is needed.
Professor Monson drew on the results from the ADSHG’s
2003 international survey. He commented that these days there are
many more diagnoses of Addison’s, and recovery after diagnosis is
generally quicker than it was in previous decades.
Although survey responses indicated that those taking their
hydrocortisone twice daily had a similar quality of life to those taking
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it three times daily, it was noticeable that those on three-times daily
were able to take a lower total daily dose without any increase in
symptoms. Professor Monson’s own studies indicate that where
patients switch from two to three times daily, they often report an
improved quality of life with fewer ongoing symptoms.
He noted that Addison’s patients have at least a 50%
chance of developing another autoimmune disease and that
diarrhoea and vomiting are the most common causes of adrenal
emergency.
However he encouraged a non-fatalistic view of Addison’s
as (reassuringly) it is unlikely to be the Addison’s which sees us
depart this world. Accidents, cancer and cardiovascular disease
are the most common causes of death in the west and we are not
exempt from this, having about the same chance as everyone else of
succumbing to ordinary medical conditions.
Patients should see their endocrinologist on roughly a
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yearly basis for maintenance checks and should be able to see
their endocrinologist in between times if the need arises. Addison’s
is a lifelong medical condition and patients should not generally
be discharged back to the care of a GP, if only because they will
then have lost access to any new developments in endocrine
management and therapy.
Professor Monson then addressed DHEA replacement for
people with adrenal failure. He explained that DHEA is an androgen
which is believed to benefit the psyche, mood and fatigue, with
most – but not all – recent medical studies reporting improved wellbeing
and sexuality.
His own research with pituitary patients (who are deficient
in DHEA) does show a measurable, statistical improvement for
female patients, with less discernible improvement for men.
Professor Monson said he generally starts his adrenal
patients on a dose of 50mg DHEA. If this is too high, there will be
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mild side-effects, usually acne, in which case the dose is reduced
to 37.5mg or 25mg. He does baseline blood tests (DHEA-S and
testosterone) before starting DHEA and then again six weeks later.
Concluding his lecture, Professor Monson referred to
research under way in Sheffield and Sweden which may bring us
either delayed-release hydrocortisone preparations or mixed tablets,
containing both rapid and slow release elements. These might
enable us to take just one tablet a day and the mixed tablet might
provide better oral treatment for emergency use. In the meantime,
he was reassuring in his encouragement that we can, with good
care plus the right support and medication, lead full and active lives
on our current three-times a day regime.
All in all, it was an amazingly informative lecture. Although
I have lived with Addison’s for 17 years, several things cropped up
that surprised me and kept me on my toes. Following the lecture
we had a good socialise; I chatted to quite a few people I have
corresponded with on the e-group. I was also delighted to make
some new friends local to me. Roll on Guildford! Claire Allen
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Sex and Addison’s: how adrenal insufficiency
affects men and women differently
ADSHG medical lecture 2006 Professor Wiebke Arlt
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Sex and Addison’s: how adrenal insufficiency
affects men and women differently
ADSHG medical lecture 2006
Professor Wiebke Arlt (continued)
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Sex and Addison’s: how adrenal insufficiency
affects men and women differently
ADSHG medical lecture 2006
Professor Wiebke Arlt (continued)
Despite pressing family commitments and a struggle with the
tube, I was determined to attend the medical lecture to see some
friendly faces and hear some juicy pointers on Addison’s and sex – I
had somehow ignored the subtitle in my excitement. Professor
Arlt opened her talk with the history of Dr Thomas Addison and
the discovery of Addison’s in the 1850s. She made the interesting
observation that four times more women than men develop
Addison’s disease, and that women are more likely to develop
a second endocrine condition, most often a thyroid condition
or ovarian failure. Asthma and psoriasis are high contenders for
accompanying (nonendocrine) autoimmune conditions, with asthma
more common in women and psoriasis in men.
Age also became a significant difference between the sexes, with
men most likely to be diagnosed with Addison’s disease between
20-29 and women between 30-50 years. Professor Arlt then
illustrated the problems with diagnosis by comparing two hospital
admissions. Typically, it is the tan that initially hinders diagnosis –
because patient looks so well – while its association with extreme
fatigue then achieves it.
Looking at replacement therapy, Professor Arlt ‘asked the audience’
and found the majority on hydrocortisone, with a few people
taking prednisolone or cortisone acetate. The question on many
lips was the usefulness of day curves. Professor Arlt explained that
day curves are useful to track how serum cortisol is used by the
body, but stressed they are only helpful if there is a record of the
time the replacement hydrocortisone is taken. She then explained
why mineralcorticoid replacement – the drug fludrocortisone - is
required. Quite simply put, it is a blood pressure assistant. If the
individual is on too little fludrocortisone, they will usually experience
dizziness when standing up, and muscle cramps are also common.
Hydrocortisone exerts a small amount of fludrocortisone activity in
the body, therefore if there is a change in the type of replacement
or the amount of hydrocortisone taken, it can affect fludrocortisone
levels. At last I understand why I get cramps in hospital when I get
put onto dexamethasone for emergency treatment!
This was further explored by looking at the extra hydrocortisone
requirements during surgery and in times of infection. When the
hydrocortisone is increased to more than 50mg for illness or surgery,
this has a fludrocortisone activity equivalent to 100mcg. So anyone
whose normal fludrocortisone dose is 100mcg can probably safely
stop taking it until they taper their hydrocortisone to less than
50mg.
Plasma renin is the most sensitive test of whether an individual is
getting enough fludrocortisone, but it is not always reliable. It is not
accurate during pregnancy, because of the additional pregnancy
hormones in circulation, and it is likely to give a misleadingly low
reading for anyone who also has diabetes or who is taking NSAIDs.
In these circumstances it is best to rely on the broader indicators:
blood pressure, sodium and potassium. Professor Arlt then
tackled the management of Addison’s during pregnancy, with the
observation that both fludrocortisone and hydrocortisone may need
to be increased as the pregnancy advances, while high doses of
intramuscular hydrocortisone are needed during delivery.
Professor Arlt closed her presentation with a look at DHEA. The
question of whether people with Addison’s need DHEA seems to
be answered with a resounding yes. Tests are continuing to study
whether immune function is improved on DHEA, and Professor
Arlt has some results in the pipeline here. Studies show that people
with Addison’s subjects report fatigue, dry skin and low libido as
significant problems while on standard steroid replacement therapy;
these symptoms are improved by DHEA. Women with Addison’s are
noticeably more affected by low libido than men and report more
improvement on DHEA replacement. In clinical trials conducted by
Professor Arlt, four months of DHEA replacement therapy restored
androgens to normal levels, which boosted sexuality. And this
improvement has been observed and reported by the spouses of
women taking part in clinical trials as well. Now that’s the juicy bit I
was waiting for!
Claire Allen

Addison’s disease in the 21st century
Cambridge advanced endocrine course 2006
Professor John Wass
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Addison’s disease in the 21st century
Cambridge advanced endocrine course 2006
Professor John Wass
(continued)
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The ABC of Addison’s
ADSHG medical seminar 2006
Dr Simon Pearce
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The ABC of Addison’s
ADSHG medical seminar 2006
Dr Simon Pearce
(continued)
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The adrenals are known as the “fight or flight glands”. This
is a reference to their role in producing adrenaline. But where
patients have their adrenal glands removed, in practice they do not
experience symptoms due to lack of adrenaline. This is because
the nerve endings throughout the body produce adrenaline as
well. More importantly, the adrenals produce the steroid hormones
aldosterone and cortisol. These are essential for life, which means
you will die if they are not replaced. The adrenals also produce
DHEA. All the steroid hormones produced by the adrenals are
converted from cholesterol. Although too much of it is a bad thing,
cholesterol is an essential building block for the body. The enzymes
used to manufacture these steroid hormones from cholesterol
are the targets of the autoimmune attack which causes Addison’s
disease.
What happens when you lose these hormones?
Reduced aldosterone leads to dizziness on standing, low blood
pressure, salt craving, dehydration, thirst, frequent urination. The
kidneys start to work harder and produce more renin, in an attempt
to stimulate aldosterone.
Declining cortisol production gives you extreme lethargy,
muscle weakness and fatigue, poor concentration, stomach
discomfort or pain, vomiting, weight loss and low blood sugar. The
pituitary works harder to produce ACTH, which tries to stimulate
extra cortisol production. In many people, aldosterone and cortisol
production is lost together, over a similar period of time.
DHEA production also gradually decreases, leading to
loss of armpit and pubic hair in women, a reduction in muscle
functioning and a loss of interest in sex.
What causes adrenal failure?
The adrenals have several layers. The inner medulla produces
adrenaline. The adrenal cortex is the outer three layers. The
outermost makes aldosterone (‘natural’ fludrocortisone), the next
makes cortisol (‘natural’ hydrocortisone) and the third DHEA.
Autoimmune destruction of adrenal function tends to be slowly
progressive, with people feeling quite well until a critical amount of
the adrenal tissue is destroyed. After a certain point there is a very
gradual onset of symptoms, although any unrelated illness that
requires an increase in cortisol production may precipitate a person
with partial adrenal destruction into a “crisis”.
Addison’s plus other conditions
People with autoimmune Addison’s are likely to develop further
autoimmune conditions. The 2003 international Addison’s survey
found that 50% of people had a thyroid condition. Around 12%
also had asthma, 10% had diabetes, 7% had pernicious anaemia
(Vitamin B12 deficiency). Other, more rare conditions can also
develop. People with secondary adrenal failure (a pituitary problem)
do not usually develop these associated autoimmune conditions.
They also do not usually need fludrocortisone, because their
mineralcorticoid function stays intact. The loss of pituitary hormones
also means they do not get the high tan (excess pigmentation) that
you see in primary Addison’s.
How to stay feeling better
Hydrocortisone is the standard drug in the UK: usually 15 - 25mg a
day. The evidence favours three doses. People seem to feel better
and research suggests this is a closer match to the cortisol patterns
of a person with healthy adrenals. However, there are no hard and
fast rules. Occupations with an “extended shift”, such as open-all
hours shopkeepers or the mothers of small children, may do 10 + 5
+ 2.5+ 2.5….+2.5 every four hours until their shift ends.

Diabetics on insulin - the cortisol counteracts the insulin,
so it can take more care to get the right medication balance.
Hypoglycaemia can be a problem. A small extra dose late in the
day can stop blood sugar levels crashing overnight. Taking bigger
doses in the morning does not produce a proportionate increase
in the level of cortisol in the body because at a certain point the
cortisolbinding capacity in the blood is saturated and the body
simply excretes the extra; regular large morning doses (15mg or
more) are generally not necessary.
Normal cortisol production is proportionate to body surface
area. A simple but approximate way to calculate your regular daily
dose requirement is to multiply your body weight by 0.3. However,
this tends to overestimate in some people. Fludrocortisone replaces
aldosterone: usually 50–200mcg per day. It is as important to keep a
good supply of fludrocortisone in the house. Lack of fludrocortisone
can trigger an adrenal crisis even where hydrocortisone is adequate.
DHEA is optional, not all people find it helpful; usually
25–50mg per day taken in one dose on wakening. Curiously, US
and UK regulations mean it is classified as a food supplement
(“nutraceutical”) rather than a prescription medication. You can buy
it over the internet but because it is not a licensed medication, not
all brands contain what they say they do.
How to monitor cortisol replacement
Day curves can help to monitor the individual’s precise response to a
particular dose regimen of hydrocortisone. Three samples matching
the three doses is usually sufficient, but many centres conduct
more frequent samples to give an even clearer picture. When the
numbers indicate everything is okay, the patient may still experience
problems. Almost every system in the body depends on cortisol, so
other factors may mean they are not receiving enough medication
for their needs. If in doubt, I believe the patient, I do not just rely on
the numbers.
A rounded face with “hamster cheeks” can indicate
overmedication. This is known as a Cushingoid appearance, named
after Cushing’s syndrome, where the adrenals produce too much
cortisol. Cushing’s is actually more common than Addison’s.
How to monitor fludrocortisone replacement
Watch for postural blood pressure – high potassium and low
sodium, salt cravings or feeling extra thirsty may indicate a need for
more fludrocortisone. Too much fludrocortisone will
make your blood pressure go up high, your ankles may swell and
you may get low potassium. The most accurate way to assess this
is to measure plasma renin. This is a specialist test that has to be
conducted by a hospital lab.
If you find you have an excessive need for salt, the
cravings should decrease once you try a little more fludrocortisone.
Excessive tiredness could also indicate a need for salt, or that you are
dehydrated.
While the target for most of the population is a low salt
diet this does not apply to people with Addison’s. You need your
salt! If you are preparing low-salt meals for the family, you may need
to add salt for yourself at the table.
Drug interactions
Liquorice root causes hydrocortisone to act like fludrocortisone. It
acts just like a real drug; it is as if you were increasing your dose of
fludrocortisone. It is best to avoid it.
Ask your doctor to check drug interactions whenever they
prescribe something new, even just a short course of antibiotic.
People have got into trouble with low doses of anti-epilepsy
medications being prescribed for nerve pain or migraine; these act
to deplete your hydrocortisone more quickly.
If you develop high blood pressure, get your
endocrinologist to review the fludrocortisone dose. Doctors
categorise the common blood pressure medicines as A, B, C, or
Ds. The D stands for diuretics and is best avoided; patients with
Addison’s and persistent high blood pressure after adjusting (but
generally not stopping) the fludrocortisone are most safely treated
with a ‘C’ or an ‘A’ drug.
Managing illness and injury
You need to be alert to the warning signs of an approaching adrenal
crisis. These will be similar to the symptoms of your pre diagnosis
illness: severe headache, nausea, vomiting vertigo, extreme
weakness, possibly a rapid drop of blood pressure. If you feel
severely unwell, take 20mg hydrocortisone immediately.
EVERYBODY should have an injection kit and a supply
of extra medication for illness or injury. The components of an
injection kit are an injectable form of hydrocortisone (generally
Solu-cortef or Efcortesol), a small syringe and a needle. Don’t feel
bad about pestering your GP or consultant for this injectable form of
hydrocortisone.
Each vial costs the NHS less than one pound, and it could
save your life. It is sensible to take spare medication with you
everywhere.
Take charge of your illness, you may encounter medics who
do not have the experience to help you. If you are travelling, you
need to take your injection kit and extra medication with you in your
hand luggage. To get you through airport security, get a letter from
your GP (or endocrine nurse or consultant) saying you need them.
There is a suppository which can replace injections for children. The
disadvantages of suppositories are that they take longer to work
than an injection and go out of date quite quickly.
You need to make sure you have adequate medication
supplies at all times. So you need to obtain a two-three month
repeat script which includes extra to cover illness, and you need to
order your next repeat when you still have one month’s supply in
hand. Take double supply on holidays, in your hand luggage and
extra in hold luggage.
Vomiting and diarrhoea are the main causes of adrenal
emergencies: take food poisoning seriously and get it sorted quickly.
Swallow 20mg hydrocortisone immediately after vomiting and sip
electrolyte fluids (eg. Dioralyte, or one pint water plus one teaspoon
salt and one of sugar). If you vomit twice and cannot keep down
medication – don’t wait, self-inject, then call a doctor.
Serious injury: take 20mg immediately to avoid shock and
maybe another 20mg a bit later (or self-inject). Then get to hospital.
Make sure your surgical team have the surgical guidelines.
These are listed on the ADSHG website and give a risk-free level of
steroid cover for most types of surgical procedure. If your medics
argue or are unsure, tell them no one ever died from this regime,
but it is possible to die from insufficient medication.
Making the most of life
People with Addison’s can and do lead long, full lives and can do
most of the more demanding or strenuous things that others do.
You may dehydrate when you exercise, so exercise may need extra
water and medication. For exceptional challenges, double your
dose; if there is a significant risk of injury, or you are in a remote
location, a companion should be trained to give an injection. Not
everyone with Addison’s wants to run a marathon, but there is
nothing stopping you and others have done in the past.
Simon Pearce & Peter Cripps
29

30
You and your
General Practitioner
ADSHG medical lecture 2005
Professor John Wass
1 5
2 6
3
4
7

8
12
9 13
10 14
11
15
31

32
You and your General Practitioner
ADSHG medical lecture 2005
Professor John Wass (continued)
16
17
18
19
Why are Addison’s
patients ‘difficult’?
Addenbrooke’s
endocrine seminar 2005
Katherine White
1
2
3

4
5 9
6 10
7 11
8
33

34
Why are Addison’s patients ‘difficult’?
Addenbrooke’s endocrine seminar 2005
Katherine White (continued)
12
13
14
Managing your
steroid medication
ADSHG medical lecture 2004
Dr Trevor Howlett,
Leicester Royal Infirmary
1
2
3
4

5
6
7
8
9 14
10
11
12
13
35

36
Managing
your steroid
medication
ADSHG medical
lecture 2004
Dr Trevor
Howlett,
Leicester Royal
Infirmary
(continued)
16
17
18
15
19 24
21
22
23
20

25
26
27
28
29
30
31
32
33
37

38
Professor
Wiebke Arlt
Wiebke Arlt is the Medical
Research Council (MRC)
Senior Clinical Fellow
and also a Professor of
Endocrinologist at the Dept
of Medicine, University
Hospital, Birmingham. She is
a Consultant Endocrinologist
at the University Hospital
Birmingham, the
Birmingham Women’s
Hospital and the
Birmingham Children’s
Hospital. She heads a
research group working
on basic and clinical
aspects of adrenal and
gonadal disorders, with a
particular focus on steroid
endocrinology.
She is a steering
committee member of
the European Network
for the Study of Adrenal
Tumours, ENS@T, and the
FP7 European Collaborative
Network on Disordered Sex
Development, EuroDSD. She
is an editorial board member
of the European Journal
of Endocrinology, Clinical
Endocrinology, Journal of
Endocrinology and Journal
of Clinical Endocrinology
& Metabolism.
Professor
Krishna Chatterjee
Krishna Chatterjee heads
the Metabolic Research
Unit at Cambridge
University’s Institute of
Medicine.
After an MRC/
NIH research fellowship in
the US and a Wellcome
Senior Clinical Fellowship
in Cambridge, Krishna
Chatterjee was appointed
Professor of Endocrinology
at Cambridge in 1998.
He is the Director of a
Supraregional Assay
Service laboratory for
unusual thyroid disorders
and the Wellcome Trust
Clinical Research Facility in
Cambridge, and Deputy
Director of the Cambridge
NIHR Biomedical Research
Centre. His Wellcome Trust
and NIHR funded research
programme has spanned
genetic disorders of the
pituitary-thyroid axis, the
role of nuclear hormone
receptors (including PPAR!)
in human disease and
hormone replacement in
adrenal insufficiency.
Dr Trevor
Howlett
Trevor Howlett has led
the development of
endocrinology services
in Leicester since 1988.
During this time he has
established a large, and still
growing, clinical practice
in the speciality. He is a
founder member of the
Clinical Committee of the
Society for Endocrinology
and UK Representative
on the European Board of
Endocrinology. His major
sub-speciality interests
include the diagnosis
and management of
adrenal insufficiency; the
management of pituitary
disease and the clinical
consequences of PCOS.
Dr Howlett has also served
as the Royal College of
Physicians Regional
Advisor and Directorate
Consultant Lead for
Clinical Governance.
Dr Kristian
Lovas
Kristian Lovas did his
PhD in Addison’s disease,
at the University of
Bergen, Norway. Based
at Haukeland University
Hospital, he has also
worked at Addenbrookes
Hospital, Cambridge, and
is part of the Euradrenal
research consortium. He
has published extensively
on Addison’s disease.

Professor
John Monson
John Monson, is Emeritus
Professor of Clinical
Endocrinology at St.
Bartholomew’s Hospital,
QMUL, United Kingdom
and Consultant Physician
at the London Clinic Centre
for Endocrinology. He was
formerly Lead Clinician
for Endocrinology at St
Bartholomew’s Hospital and
Centre Lead for Academic
Clinical Endocrinology
in the William Harvey
Research Institute at Queen
Mary, University of London.
Professor Monson
has served as a member of
the Clinical Committee and
Council of the UK Society
for Endocrinology
ADDISON’S
S e lf H elp G ro u p
Professor
Simon Pearce
Simon Pearce is a Professor
of Endocrinology at
Newcastle University and
an Honorary Consultant
Physician at the Royal
Victoria Infirmary,
Newcastle.
He trained
in endocrinology at
Hammersmith, London; in
Boston, USA and latterly in
Newcastle. Since 1993 he
has being doing research
into the molecular and
genetic causes of endocrine
conditions, with a particular
interest in autoimmune
disorders. He is a member
of the Euradrenal research
consortium.
Addison’s Disease
Self-Help Group
PO Box 1083
Guildford
GU1 9HX
email:
info@addisons.org.uk
www.addisons.org.uk
Text editor:
Katherine White
Design and layout:
the workshop
©ADSHG 2010
Professor
John Wass
John Wass has been
a consultant physician
since 1982, first at St.
Bartholomew’s Hospital in
London and then in Oxford
since 1995. He has been
Chairman of the Society
for Endocrinology from
2006-2009 and Editor of
the Oxford Textbook of
Endocrinology 1st Edition
(2002) 2nd Edition (2010).
He founded the Oxfordshire
Osteoporosis Service
in 1995. His research
interests include pituitary
tumours, acromegaly,
growth hormone
deficiency, angiogenesis
in endocrinology, and the
genetics of osteoporosis
and thyroid disease. He
was editor of Clinical
Endocrinology and is on the
Editorial Board of numerous
journals including Pituitary,
the Journal of Clinical
Endocrinology and
Metabolism and Endocrine
Reviews. He was President
of the European Federation
of Endocrine Societies
from 2001-2203 and was
Chairman of the Society
for Endocrinology
(2006-2009).
This information may
be copied for personal
use or by medical
practitioners for the
education of their
patients. Otherwise,
it should not be
reproduced without
written permission
from the ADSHG. It
is available online at
www.addisons.org.uk
Katherine
White
Katherine White is the
Chair of the Addison’s
Disease Self-Help Group.
She is also the coordinator
for the Addison’s Clinical
Advisory Panel. She led
the development of
the 2003 International
Addison’s Survey.
39

2010 medical lecture
Pages 2-5
Should everyone
with Addison’s be
taking DHEA?
Professor
Krishna Chatterjee
2009 medical lecture
Pages 6-9
Replacement therapy
in Addison’s disease:
challenges and
opportunities
Dr Kristian Lovas
ADDISON’S
S e lf H elp G ro u p
2008 medical lecture
Pages 10-13
The causes of Addison’s
Professor Simon Pearce
2007 medical lecture
Pages 14-17
Trends in the
management of
Addison’s disease
Professor John Monson
2006 medical lecture
Pages 18-22
Sex and Addison’s: how
adrenal insufficiency
affects men and women
differently
Professor Wiebke Arlt
©ADSHG 2010
2006 Cambridge
advanced endocrine
course
Pages 23-25
Addison’s disease
in the 21st century
Professor John Wass
2006 medical seminar
Pages 26-29
The ABC of Addison’s
Dr Simon Pearce
2005 medical lecture
Pages 30-32
You and your General
Practitioner
Professor John Wass
2005 Addenbrooke’s
endocrine seminar
Pages 32-33
Why are Addison’s
patients “difficult”
Katherine White
2004 medical lecture
Pages 33-38
Managing your steroid
medication
Dr Trevor Howlett
©ADSHG 2010