LIVING WITH ADDISON'S DISEASE An owner's manual for individuals with the disease

24 Pages·2001·0.22 MB·English

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LIVING
WITH
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ADDISON’S
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DISEASE An owner’s
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manual for
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individuals with W
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Ethe disease D
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S replicate the necessary amounts of the missing hormones - O
cortisol and aldosterone - that the individual can no longer w
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produce from the adrenal glands. With the right balance of e
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Idaily medication, most people with Addison’s disease are able ’s M
to continue life much as it was before their illness. a
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This guide has been co-written by Sarah Baker and u
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Katherine White, members of the UK Addison’s Disease Self- l
D
Help Group, in collaboration with endocrinologist, Professor
I
J A H Wass. It aims to provide you with sufficient information
to allow you to work well with your doctors in fine-tuning
steroid replacement therapy, and in making informed choices
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about some of the practicalities of managing the condition.
A glossary of the terms and references used in the manual
starts on page 21.
In common with fellow Addisonians, you will learn to
manage your own health day-to-day. At some stage, you may
experience illnesses or other situations requiring significant,
short-term adjustments in medication. You will need to take
extra medication if you get a serious infection or injury. Without
this extra medication, you could experience potentially life-
threatening symptoms known as an Addisonian crisis. This
guide aims to help with both the everyday and the more
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unusual challenges that Addisonians can experience.
This guide is addressed principally to UK residents
and reflects UK medical practices. Please remember that the
information it provides is intended as a supplement to, not a
substitute for, the advice of your doctor who knows the details
of your medical history. Individual responses to the condition
can vary widely and, in some instances, individuals may have
experienced symptoms needing differing treatment to that
described here.
Many of the specific issues addressed in this guide
A were raised in the most recent UK survey of the Addison’s
Disease Self-Help Group (ADSHG), conducted in 1996/7.
The full results of that survey can be found on the ADSHG
website listed in section 11.
Sarah Baker &Katherine White
SUMMARY OF 1 HOW ‘NORMAL’ CAN LIFE BE
WITH ADDISON’S DISEASE?
CONTENTS
a) Managing your lifestyle 3 5 DIET
b) You and your doctors 3
i. Plenty of fluids, protein,
2 THE CAUSES OF ADDISON’S AND vitamins and minerals 13
RELATED AUTOIMMUNE DISEASES ii. Tastes and cravings 13
iii. Multi-vitamins and other supplements 13
a) Primary and secondary adrenal disease 4
b) Related autoimmune conditions 4
6 EXERCISE
3 MEDICATION
a) Exercise is good for us
a) What is available and what it does 5 (so why are we couch potatoes?) 14
(cid:2)Cortisone replacement 5 b)Adjusting your medication
(cid:2)Aldosterone replacement 6 to your fitness regime 14
b)The natural daily cycle of
adrenal hormone production 6 7 PREGNANCY
c) How do I know what is the right
al dose for me? 6 a) Becoming pregnant 15
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an d)Timing your divided dose 7 b)Managing your pregnancy 15
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e) Monitoring your medication: blood tests 7 c) Childbirth 15
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r’ f) Drug interactions 8 d)Caring for a newborn baby 15
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n g)Medication and food 8 e) Inheritance 15
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O h) Replacement of other adrenal hormones? 8
n i) Alternative and complementary therapies 9 8 BEING THE PARENT OF A CHILD
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E 2 4j) SStOoMrinEg C yOoMurM mOeNd iCcaOtiNoCnERNS 9 a) WMIoTnHit oArDinDgI SyOoNur’ Schild’s condition 16
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A b)Supporting your teenager 16
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S a) Experiences common to many Addisonians 9
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S i. Osteoporosis 10 9 TRAVELLING AWAY FROM HOME
N’ ii. What can I do to prevent osteoporosis? 10
O iii. Weight gain: are steroids to blame? 10 a) Take enough steroid cover
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DI iv. What helps to shed those to allow for emergencies 17
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A excess pounds? 10 b)Jet lag 17
H c) Hot weather 17
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WI b)Experiences associated with
G low levels of cortisol 10 CRISIS MANAGEMENT
N v. Why do I feel lousy at certain times
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LI of the day? 10 a) What is an Addisonian crisis? 18
vi. Low blood sugar and b)Colds and other minor illnesses 18
the insulin response 11 c) Serious illnesses requiring extra steroid cover
vii. Salt and other cravings 11 (cid:2) Flu and other fevers 18
viii. Feeling very thirsty 11 (cid:2) Diarrhoea lasting for more than a day 19
ix. Dry skin 11 d)Tapering your dose back down after
x. Extra pigmentation 11 a serious illness 19
xi. Exhaustion and ‘hitting the wall’ 11 e) Serious illnesses which may require
xii. Nausea and vertigo 11 an emergency injection
(cid:2) Stomach flu (gastric flu) and
c) Experiences associated with any illness involving vomiting 19
high levels of cortisol (cid:2) Shock and major accidents 19
xiii. Insomnia and other sleeping difficulties 12 f) Giving yourself an emergency injection 20
xiv. Anger, mood swings and depression 12 g)Surgery requiring extra steroid cover 20
xv. Fluid retention 12 h) Medic Alert and other useful information 20
xvi. Easy bruising 12
xvii. Thinning skin 12 11SELF HELP
xviii.Muscle weakness/wastage 12
xix. Puffy/moon face 12 Some useful contact addresses 21
12GLOSSARY OF TERMS AND REFERENCES 21
13ACKNOWLEDGEMENTS 23
HOW ‘NORMAL’ CAN LIFE BE WITH ADDISON’S DISEASE?
a)MANAGING YOUR LIFESTYLE
“I have had Addison’s for Individuals with Addison’s disease can expect
twelve years and have not had to have a normal life span, provided they manage
1 HOW ‘NORMAL’
a crisis needing medical their daily medication sensibly. Addisonians can
assistance since my diagnosis. bring up a family on their own, hold down a
I have raised seven children, demanding job, run marathons or even become CAN LIFE BE
run a motor vehicle repair the President of the United States.
business and drive extensively. That is not to say that life as an Addisonian WITH ADDISON’S
I am 55 years old,very active. will always feel ‘normal’. Most individuals find they
Like many others I have few have to become skilled at managing the fatigue that
DISEASE?
side effects and am happy accompanies the condition. In the last UK survey of
with my quality of life.” Addisonians (1996/7), around 40% of respondents
JAMES said they had ongoing difficulties with fatigue and
a lack of energy. There is more information on
“My body cannot be normal, managing fatigue in section 3: Medicationand in L
but I do pretty well.Our section 4: Common concerns. IVIN
limitations really are few.I A number of people find they develop about by a delayed diagnosis. Sometimes it is G
guess we just need to learn at related auto-immune conditions such as diabetes because they have multiple health conditions, W
what point we should go on or hypothyroidism, requiring extra healthcare which include adrenal failure. So not everyone IT
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or stop to give our bodies a management. There is more information on can run marathons, just as not all of us could A
break.”SANDRA related autoimmune conditions in section 2: become President. D
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The Causes of Addison’s. Most of us, however, continue to lead busy IS
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“I have had Addison’s for 15 Addisonians must always be aware of their own and rewarding lives, with the support of our family, N
years and have never had a health and ready to increase their dosage if they get friends and medical professionals. And if you do ’S
crisis.I have had a full-time sick or are seriously injured. A North American survey want to run a marathon, there is more information D
IS
job,raised three children (1997) found that three-quarters of respondents had in section 6: Exercise. E
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alone when my husband received emergency treatment at a hospital for their S
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normal schedule for a you are seriously ill in section 10: Crisis management. the experiences of others and discover that I really have an O
50 year old.”ROSEANNA A few individuals are less lucky and find excuse for fatigue - not just lazy.My doctor never mentioned w
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that their health never recovers sufficiently to allow the exhaustion.I also get joint and back pain.Never e
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them to return to full-time work after diagnosis. incapacitating - just frustrating.But I do take good care ’s
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Sometimes this is because they experienced serious of myself and have a wonderful doctor.”TRISH
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damage to their health (such as a stroke) brought u
“I work 9 to 5 and I have two boys.When I feel bad I feel al
exhausted and aching and can’t get on with my life.People
don’t always feel great despite the medication.”GAIL
b)YOU AND YOUR DOCTORS
As an Addisonian, you are responsible for day-to-
day management of your own health. You must
take your daily medication at the right times and Your endocrinologist is generally responsible for “My GP and I work well as a
monitor your general health for any signs of reviewing your medication requirements and team and he also works well
serious illness where your medication might checking for any changes in your endocrine health. with my endocrinologist.My
need increasing. Serious illnesses requiring extra They will usually check for signs that your steroid GP has admitted honestly that
medication are discussed in section 10 of this medication may need adjusting up or down. Some he does not know enough
manual: Crisis management. Addisonians do also develop related autoimmune about the disease to care for
Most Addisonians are supported in their conditions, such as hypothyroidism or diabetes. me on his own.”SANDRA
health management by two medical professionals: Your endocrinologist will usually check for any
their General Practitioner and their endocrinologist. signs of these. In the UK, most endocrinologists “I see my endocrinologist
In the UK, your General Practitioner (GP) prefer to see their Addisonian patients every six every six months and it is
is usually responsible for prescribing your current to twelve months. amazing the changes that
medication and for checking your general health So, being a successful Addisonian means can happen even in that
status with you - including any problems unrelated being a good communicator, as there are two short time.”JOAN
to your Addison’s. medical professionals to keep informed about
In the UK, your GP will be able to authorise any important changes in your health. “My endocrinologist is great.
a Medical Exemption Certificate for you, allowing Some Addisonians find it helps to keep a I have kept a journal in the
you to receive all prescription medication free written record of their health status when there are past with symptoms matched
of NHS charges, so please remember to ask important health issues they want to review with to activities including blood
them for this. their GP or endocrinologist. At such times, a medical pressure.The doctor used
‘diary’ can be a useful guide for your doctor, this to help match trends
providing more specific information than you can in relation to medication.
recall in a general conversation. You will probably A journal makes your
want to record the timing and amount of information more accurate
medication you take, your level of energy/fatigue and credible.And you must
and any other important health factors or changes. educate yourself as much
as possible.”ALAN
CAUSES AND RELATED AUTOIMMUNE DISEASES
2 THE CAUSES OF ADDISON’S AND
RELATED AUTOIMMUNE DISEASES
a)PRIMARY AND SECONDARY
ADRENAL DISEASE
Addison’s disease is a rare condition where the immune system starts attacking the body’s own
adrenal glands gradually cease to function. The organs. Autoimmune adrenalitis now accounts for
disease is not usually apparent until over 90% of around 70% of all cases, and affects more women
the adrenal cortex has been destroyed, so that than men. The cause of autoimmune adrenalitis
very little adrenal capacity is left. Symptoms of is not known, in common with most other
the disease, once advanced, can include severe autoimmune diseases.
fatigue and weakness, loss of weight, increased Other, much rarer causes of Addison’s
pigmentation of the skin, faintness and low blood include certain fungal infections, adrenal cancer
pressure, nausea, vomiting, salt cravings, and painful and adrenal haemorrhage (for example, following
muscles and joints. a car accident). In some cases it can result from
The adrenal glands sit at the top of the the treatment needed for Cushing’s disease
ual kidneys, one on each side of the body, and have an (overproduction of adrenal hormones).
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a inner core (known as the medulla) surrounded by the Certain rare hereditary diseases also cause
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outer shell (known as the cortex). The inner medulla adrenal insufficiency (such as adrenoleukodystrophy
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r’ produces adrenaline, the ‘fight or flight’ stress and congenital adrenal hyperplasia).
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wn hormone. The outer cortex produces the steroid All of the conditions mentioned above are
O hormones that are essential for life: cortisol and described as ‘primary adrenal insufficiency’, because
n aldosterone. It also produces sex hormones known they result from a disease process that has directly
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A In secondary adrenal condition. In the early stages of the disease many although it has a very different cause. Secondary
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DIS insufficiency the pituitary individuals are still able to produce some cortisol adrenal insufficiency mostly occurs when a pituitary
S gland no longer triggers and enough aldosterone. This is partly why tumour (such as an adenoma) forms, although
N’ the adrenals to produce individuals with the disease take varying autoimmune destruction of the pituitary gland is also
O cortisol, but other amounts of medication and why the known. Secondary adrenal insufficiency is even rarer
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DI adrenal hormones amount of medication you need may than primary Addison’s disease.
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A are mostly still alter over the years. Secondary loss of adrenal function occurs
H produced Dr Thomas Addison first identified when the messenger hormone, which stimulates the
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WI the disease in the mid 1800s while working adrenal glands into action, is no longer produced by
G at an inner-city London hospital. Then, the pituitary gland. The pituitary gland is located
N the main cause of the disease was inside the skull, just behind the eyes and tucked
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LI as a complication of tuberculosis. below the grey matter of the brain. This messenger
In Addison’s In third world countries today, hormone is called ACTHand is responsible for the
disease the tuberculosis remains an important extra pigmentation found in primary Addison’s.
adrenal cause of Addison’s. HIV (AIDS) People with secondary adrenal failure do not
glands is now becoming another experience the increased pigmentation found
gradually significant infectious disease in primary Addison’s, because their ACTH levels
cease to causing adrenal failure among are declining.
function third world populations. Long-term use of high doses of steroid
Among more drugs to treat other illnesses (such as the high doses
affluent countries, the main of prednisone which may be used to treat bowel
cause of the disease today disease or asthma) can also cause temporary or
is autoimmune adrenalitis, permanent loss of adrenal function. This is often
where an over-active referred to as secondary adrenal suppression.
b)RELATED AUTOIMMUNE
CONDITIONS
Where Addison’s disease is autoimmune, around half of
those with the condition will develop another autoimmune
disorder, usually another endocrine condition.
Kidneys
The most common endocrine disorder associated
with Addison’s is a thyroid problem, either hyperthyroidism
or hypothyroidism. Most medical studies have found that just
over one-fifth of those with autoimmune Addison’s are likely
Diagram of body
to develop a thyroid problem of some kind.
showing location of
Other recognised associations include premature
adrenal glands and
failure of the ovaries, insulin-dependent diabetes (type I
the location of the
diabetes) and parathyroid deficiency. These all occur less
pituitary gland
frequently than thyroid disorders.
in the skull
MEDICATION
A few people with autoimmune Addison’s develop platelets), Sjogren’s syndrome(dry eyes and mouth)
a combination of related autoimmune conditions, or rheumatoid arthritis.
which are known as a polyglandular autoimmune Autoimmune Addison’s is not usually a
syndrome. directly inherited condition. But a tendency to
Non-endocrine autoimmune diseases autoimmune diseases does seem to run in some
sometimes also occur in combination with 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,
develop both endocrine and non-endocrine 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.
Medical studies estimate that around 5% Where a tendency to autoimmune
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
smaller proportions are estimated to develop other related autoimmune condition, which appears L
conditions such as vitiligo(loss of pigmentation from in the next generation. For example, a grandmother IV
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parts of the skin), coeliac disease(gluten allergy), with Addison’s disease may see one of her G
alopecia(hair loss), myasthenia gravis(muscle grandchildren develop vitiligo or a thyroid condition. W
wasting), thrombocytopenia purpura(loss of blood IT
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a)WHAT IS AVAILABLE D
IS
AND WHAT IT DOES1 3 MEDICATION O
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1 In the UK, people with Addison’s The adrenal hormones, cortisol and aldosterone, are ’S
disease are entitled to free NHS essential for life. Their technical description is steroid D
prescription medication, by asking hormones. Your steroid medication aims to replace IS
their GP to authorise a Medical them in a manner that approximates the natural EA
Exemption Certificate for them. S
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glands.Lack of cortisol is of the individual, as well as how advanced their larger single doses. Individuals who switch to these n
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what causes the muscle condition is. In the early stages of the disease many drugs from hydrocortisone may find they need to r’s
weakness,low blood sugar individuals are still able to produce some cortisol and increase their fludrocortisone dose. M
and loss of concentration enough aldosterone. Getting it right depends on Cortisone acetate is an older version of an
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commonly experienced by collaboration between you and your medical hydrocortisone and less readily absorbed. It is also al
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
ROUGHLY EQUIVALENT DOSES OF STEROIDS*
FLUDROCORTISONEis medication may alter slightly. If you feel a return of
*Hydrocortisone 20mg
prescribed to replace the any of your former Addison’s symptoms, or start to
*Prednisone 5mg
aldosterone steroid normally identify the symptoms of being over-medicated, you
*Dexamethasone 0.75mg**
produced by your adrenal will need to review this with your doctor.
*Cortisone acetate 25mg
glands.Lack of aldosterone
* 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
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
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
ual overload, which also causes cardiac symptoms. without it.”TRACEY
an “I did terribly on hydrocortisone (roller coaster days) and You will need to monitor your own
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terribly on dexamethasone (too potent and I became response to your fludrocortisone dose and inform
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r’ Cushingoid).I felt pretty good on prednisone.That was the best your medical practitioner if you experience new
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n until my latest endocrinologist put me on cortisone acetate.I’m symptoms, which you think may be related. It is
w
O happy to say it is the one for me! I did find I needed differing important not to modify your fludrocortisone
n amounts of fludrocortisone on the different corticosteroids.I medication before checking with your doctor.
A
E 6 nneoewd eId nmeeodr el eosns opnre dconritsiosnoen et haacne tIa tdei.”d KoEnL hLyYdrocortisone.And usosu, ainll ya lkmePeoepsot p tahlelel iwcra iastdhesr s,e endcaool nnpdoroat rdnyue acedtdior etnon aotlfa fkaaelidluorseterone
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A fludrocortisone. Similarly, individuals who are
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S experiencing steroid-induced adrenal suppression
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S do not usually need any fludrocortisone.
N’
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DI b)THE NATURAL DAILY CYCLE OF
D
A ADRENAL HORMONE PRODUCTION
H In a person with healthy adrenals, cortisol levels start steroid medication too late in the evening can cause
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WI to rise around 4am, are at their maximum around sleeping difficulties. Around 6pm, or at least four
G the time you wake up in the morning and then hours before going to bed, is best.
N gradually taper off during the day. That is why you Aldosterone production follows a similar
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LI 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 IMPORTANTthat 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 (cid:2)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 (cid:2)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.
MEDICATION
d)TIMING YOUR DIVIDED DOSE
Some medical textbooks still refer to the standard NICKis 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 (cid:2)20mg on waking
an energy-lag in the afternoon, it may help to switch (cid:2)10mg with lunch
from taking your medication twice a day to taking it (cid:2)10mg at 6pm
three times a day.
“I find it important to split It prevents the energy lag that many CATHARYNis 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: L
physical and usually stressful sugar, faintly dizzy, or just irritable. (cid:2)12.5mg on waking IVIN
work.Don’t be afraid to take For people taking prednisone or (cid:2)7.5mg with lunch G
your medication in smaller, dexamethasone, the timing of divided doses is (cid:2)5mg at 6pm W
spread-out doses.Taking it usually less critical, as the medication stays in the IT
H
with food helps to slow the bloodstream for longer. This can be useful for MIKEis a senior civil servant. For many years he A
body’s absorption rate.” individuals whose lifestyle makes it difficult for them took 15mg hydrocortisone in two divided doses. Since D
D
JAMES to take medication during the day. However, these introducing a low dose of prednisone in the evening, IS
O
drugs also take longer to become active after being he has been able to reduce his total daily dose to the N
“The morning dose is the swallowed, which can be a disadvantage at times. equivalent of just 12mg hydrocortisone. He takes: ’S
most important for me to (cid:2)7.5mg hydrocortisone on waking D
take on time.But I can tell (cid:2)1mg prednisone at 6pm ISE
A
the difference with the S
E
tchaaanfn tc eharnnan ohgooenu wro niltaeht eihf. ToIw hae m tthi mem dionargye KbBeuAfsoRinrEeeN shse iasr sidn wa hye elclr u alrasv tebe r sitnhhgeir itnwiegas s ua ptna dak i nryuognu 2sn 0hgme frag omwilny. A7
n
goes,if there are a lot of hydrocortisone a day in two doses: 15mg on O
stresses.Once I got up at 4am waking and 5mg with dinner. Following her day w
n
and by 7am I was on the way curve, she now takes it three times a day: e
r
out.I learnt then that once (cid:2)10mg on waking ’s
I start my day I need my (cid:2)5mg with lunch M
a
medication.”ALAN (cid:2)5mg with dinner nu
a
l
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
ACTH 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 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
al at which the steroids are metabolised. This means thoroughly monitored by your doctors throughout
u
n that anyone taking these drugs will probably need your treatment.
a
M to adjust their steroid medication. Anti-epileptic medications (such as
r’s Contraceptive pills and Hormone phenytoin) can also speed up the metabolism of
e
n Replacement Therapy (oral oestrogens) slow steroids. Growth hormone treatment can lower
w
O down the rate at which the body metabolises blood levels of cortisol, by reducing the amount
n hydrocortisone by around one-third. This means a of cortisol-binding globulin available.
A
E 8 woshnoe ma w ahonyu dtldraok ocinothgrte itsrhwoeni sepe i dlnl oeosere dH t.h RIfaT ts ihsis e u ocsnuoean-lttlyihn icurdoe msl etfsaoskr ittnahgbanle (ysouucrh f lausd cIrfoa yrcbooeurtn issohoxonouelol,d na ens )et heydoes uue lmcderaruy-gh nse eacelaidnn g tlo od wrreuedgrusce
AS her previous hydrocortisone dose, she may develop potassium levels. If you should need to take anti-
SE mild signs of over-medication, such as a puffy face depressants, you may need to ask your doctor to
DI and little fat deposits on the tummy, above the collar monitor your electrolytes, as some types can cause
S
N’ bones and on the back of the neck. the body to lose more sodium than usual.
O
S
DI
D g)MEDICATION AND FOOD
A
H Hydrocortisone prescriptions are usually issued with Swallowing your tablets just with water makes it
T
WI instructions to take this medication with food. In easier to take your first dose for the day as soon as
G practice, most Addisonians can take their tablets you wake up. Waiting to take your first tablet with
N with just water without experiencing indigestion. breakfast means a delay in getting the steroid into
VI This is because the amounts of steroid we swallow your bloodstream, during which time you will feel
LI
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
with 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 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
SOME COMMON CONCERNS
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. L
IV
IN
G
j)STORING YOUR MEDICATION the contents become cloudy or coloured. Injection W
Most prescription medication is stable when stored kits are discussed more fully in sections 9 and 10: IT
H
between 0˚ and 25˚ Celsius (32˚ and 77˚ Fahrenheit). Travel and Crisis Management. A
D
Always check the expiry date on your medications,
D
especially your injection kit, and dispose of any MEDICATION:A SUMMARY IS
O
medication that reaches its expiry date. Many N
medications have a shelf-life of around two years, A combination of hydrocortisone and fludrocortisone ’S
so it is seldom worth keeping more than six is the most usual treatment for Addison’s disease. D
IS
months’ supply at home. E
A variety of blood tests are available to help you and A
At temperatures higher than 30˚ Celsius S
(ee8nx5pd˚i roFyfa dhitarset senh haeenlfid tl) i,fm em amoy sostrt eam rrtea dtpoiic ddalytei totehnra iownr ialtlth eree (asloctahste et hidtse yYmooeuudr mi cdaaotyci otfoenre siln ebtseott attbehlrri esshep ldyitoitvuiindrg ec doy ordruoersc edt sab.ialyse hliynder doocosargties.one E A9
n
effectiveness). For this reason, any medication that O
you carry close to the body in a pocket or bag Taking the first dose of the day on waking w
should be used within a few days, unless you are is recommended. ne
r
storing it inside an insulated travelling container. ’s
Individual doses of 20mg or less do not usually M
Insulated travelling containers can be ordered
need to be taken with food. an
through most pharmacists. If you are carrying an u
a
injection kit with you in this kind of hot weather, it The last dose should be taken no later than l
should be discarded after 12 months, or sooner if 4 hours before bedtime.
4 SOME
a)EXPERIENCES COMMON
TO MANY ADDISONIANS
COMMON
i. Osteoporosis
CONCERNS “I have had Addison’s for 26
Osteoporosis is more common among Addisonians
years.I used to be on 40mg
than among the population at large.
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 osteomalaciaand osteoporosis).
messing up the medication for
simplicity, these have been grouped into three topic Post-menopausal women are most
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
Please remember that, because most 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, chances of osteoporosis.
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
of these symptoms. It is never too early, or too late, to start
trade and do a lot of heavy
working on your bone density.
lifting.”BRIAN
SOME COMMON CONCERNS
ii. What can I do to iii. Weight gain: “I’ve put on 30 pounds with
prevent osteoporosis? are steroids to blame? Addison’s.I’ve become aware
(cid:2)You should make sure your diet includes plenty of Before diagnosis, many Addisonians lose a lot of of how much I was trying to
calcium and vitamin D. The UK climate makes it easy to weight. Some of this is because the disease causes self-medicate with food -
run low on vitamin D. Supplements containing calcium them to lose their appetite and they are taking in I’d nibble on stuff constantly
and vitamin D are available over the counter for those fewer calories than a healthy person. An important and lately lots of candy in an
who do not like dairy products, and are also available part of this weight loss is also a loss of normal attempt to get a pick-me-up
on prescription from your GP. body fluids, as the electrolyte balance becomes from the exhaustion I was
(cid:2)Exercise helps to build your bone density. This must destabilised. feeling.It was the only way
be weight-bearing exercise (so swimming will have Rapid weight gain in the first few weeks of I knew besides drinking tons
no effect) but walking is excellent. A daily brisk walk starting medication is usually due to a restoration of of coffee to try to fix feeling
of 20 minutes is sufficient, while a gym workout of the fluid balance. Following this, however, it is easy tired.”GLENNA
30 minutes, three times a week, is even better. to put on stored fat. This is partly because most of us
(cid:2)Remember that low-calorie diets that encourage the are cautious about taking up strenuous exercise after “When I was first diagnosed
body to burn ketones also cause calcium loss from the a period of severe illness. I was put on too high a dose
bones, and so can advance osteoporosis. A well- And steroids generally do increase the of prednisone and started
balanced diet, as discussed in section 5: Diet, is best. appetite. The difference in appetite can be subtle gaining weight quickly.
(cid:2)Post-menopausal women are recommended to use so that the weight gain can be slow but sure. In Then I changed to 20mg
hormone replacement therapy as this helps to give addition, people taking steroid medication can find it hydrocortisone daily.By
protection against osteoporosis. harder to mobilise stored fat, so weight once gained being careful to eat minimal
ual (cid:2)Bone density scans are available. You are is not so easy to lose. carbohydrates I am slowly
an recommended to request a bone density scan every five getting some of the extra
M
to ten years. Women should make sure they have a iv. What helps to shed off.”CATHY
s
r’ bone density scan at the time of menopause, if they those excess pounds?
e
n have not already had one. You may wish to request a If you feel hungry frequently and are eating in “I gained well over 10 kilos
w
O bone density scan soon after diagnosis to establish a quantities such that your weight concerns you, then while I was pregnant and
n baseline for the condition of your bones, although these gradually reducing your daily steroid dose may help breastfeeding.Then I lost it
A
1E 0 at(cid:2)hrerPo rnueogstch rn itpehtceieo sNns aHmrSiel.yd iaclawtiaoynss rweahdicilhy aacvta itloa breleb iunil dth beo UnKe yeonuer gtoy -slhaIgef d ya onsuod m alroeew oe fxb ptloheoirsid ew nseuciiggnahgrt .sw ymithpotuotm nse ocefssarily atghellet t aicnghagiil ndmr eoonvree g re o1xte 2or clmdiseeor .njIut hswts aass
S
A density are available and have been proven to have feeling hungry - and you are snacking on food high pushing the buggy and
E
S positive results for those who are already experiencing in sugars and fats to keep you going through the chasing after them in the
DI
S noticeable levels of bone thinning. day - then reducing your total daily dose may not be playground.I also realised
N’ the most helpful way to lose weight. Instead you how much dried fruit,
O “I take Fosamax daily for my osteoporosis,which is due may need to review the timing of your divided doses chocolate and crisps I had
S
DI to prednisone use.I also take calcium with vitamin D. so you can maintain your energy levels and cut back been eating to keep my energy
D
A With the help of these,my last bone density study was on your intake of snack foods. up.I started eating almonds,
H normal.Previously I had one compression fracture. Like the general population, most fresh apples and carrots as my
T
WI So Fosamax has worked for me.I just have to keep Addisonians would find it easier to maintain their snack foods instead,and broke
G taking it every day.”SALLY ideal body weight if they exercised more regularly. up my hydrocortisone dose so
N I was taking smaller amounts
VI
LI more often through the day
to keep my energy up.Then
I got rid of around one kilo
a month.”CATHARYN
b)EXPERIENCES ASSOCIATED (cid:2)Low blood sugar (hypoglycaemia) is a common
WITH LOW CORTISOL LEVELS response to low cortisol levels. This usually means
feelings of shakiness, wobbliness, irritability, faintness
v. Why do I feel lousy at and hunger. Some people simply describe it as
certain times of the day? ‘brain fog’.
The most common reason for feeling lousy at certain (cid:2)Recurrent headaches or joint aches can also
times in the day is because your cortisol levels are be a response to low cortisol levels.
lower than your body needs. Typically this happens (cid:2)Loss of appetite and feelings of mild nausea are
when the gap between one divided dose of a common response to low cortisol levels and will
hydrocortisone and the next is too long. The be familiar to many Addisonians from their illness
majority of UK Addisonians who took part in the prior to diagnosis.
1996 survey were taking their hydrocortisone in just (cid:2)Severe nausea and vertigo are usually more serious
two daily doses. More than two-thirds also said they indicators of low cortisol levels and are discussed
experienced difficulties with low blood sugar at least further below.
some of the time. The point in the day when Addisonians
If you are experiencing any of the most commonly experience low cortisol levels is
symptoms described right, it may mean you need to mid-afternoon, but some people have similar
adjust your hydrocortisone medication so you take it symptoms when they first wake up.
in smaller, more frequent amounts. If you are experiencing symptoms which
do not ease once you have taken your next dose of
hydrocortisone then you need to raise them with
your medical practitioner.