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The Genealogist - UK census, BMDs and more online
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Frequently asked questions:
If you find this site useful and would care to towards the costs of maintaining ADNetwork, please click the button Thankyou, (MLW).
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The Genealogist - UK census, BMDs and more online
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Frequently asked questions:
Background.
Around 70% of all cases of Addison's disease worldwide are autoimmune in nature, with the remaining 30% including patients with pituitary problems including tumours or other dysfunction of the pituitary, in some cases resulting in removal of this gland, renal (kidney) tuberculosis which has spread to the adrenal glands, and those who have had their adrenal glands removed for one reason or another.
Needless to say the complexity that is Addison's disease raises a number of questions. Some of these may relate to autoimmune diseases in general but are often relevant to patients with Addison's.
There are questions which crop up frequently in one form or another and in this page we will attempt to answer these questions. The first part is a selection of generalised questions which we have observed to have been raised a number of times, together with answers which we hope all can understand.
What is the difference between primary and secondary Addison's disease?
Does autoimmune disease affect only one type of cellular tissue in the body?
What tests are needed to diagnose primary and secondary Addison's disease?
Are the results of a SynActhen test (ACTH stimulation test) affected by recently taken steroids?
Patients in whom the clinician has decided it could be dangerous to delay treatment until a SynActhen (stim) test can be carried out at a later date has two choices which will not affect the results of the test.
What is the treatment for Addison's disease?
How do patients know if they need extra salt and how do they know how much is needed?
How much is much easier to answer:- About enough to cover between half and all of a small coin, e.g. a dime in USA or 5p in UK,S at a time. This will be about a gram of salt or just over. The test to check if it is needed or if more is needed is simple.
If it tasted lovely and salty, you probably needed it. However if it tastes horrible and salty then you have had enough. This is a very crude test but works surprisingly well. We stress though that this procedure should only be used or undertaken by patients with Addison's disease, and that normal patients should continue to restrict their total daily salt intake to the recommended 6 grams per day. What do you mean by 'sodium/potassium balance' (also known as electrolyte balance)?
Are there any other electrolytes that we need to balance carefully like this?
When do we need to take extra replacement steroids?
We add two warnings here:- Firstly the patient must not just take extra 'simply because it makes them feel better'; and secondly when the patient has been taking an increased oral dose for one of the reasons listed above, it should be for the duration of the stressful situation only and it is vital that the patient tapers their dose, over a period which can be from a day or so up to a week or two depending on the individual stress situation, the amount of daily dosage increase taken and the length of time on that increased dose, down to their normal replacement dose.
Patients do not normally need to increase their mineralocorticoid drug dosage. Do patients die nowadays from Addison's disease?
What is an Addison's crisis?
Symptoms may include nausea and/or vomiting, dizziness, confusion, low blood pressure, drowsiness eventually progressing to coma, severe headache/migraine; also extreme weakness, bodily pain and/or tenderness, feeling feverish, tachycardia (fast heart rate) or bradycardia (slow heart rate), or an irregular heart rate (palpitations). Stress (however caused, be it shock from injury, illness, bereavement, even a long journey) and/or dehydration are the two factors most likely to start the cycle of a crisis in the Addison's patient. Both factors must be avoided as much as possible.
Does every Addison's patient experience crises?
What treatment is required for a patient in crisis?
First line of treatment prescribed by clinicians for patients in crisis is an injection (preferably intra-venous, or if no experienced trained person available who can give IV injections, then intra-muscular is adequate) of, usually, 100mg hydrocortisone (Solu-Cortef or Efcortisol) which may be repeated once after 20-30 minutes if no improvement in the patient's condition. Emergency injections must be followed immediately with a call for urgent medical help. In case of any doubt, call for help as soon as possible. The patient will probably need an intra-venous saline drip to help correct potential electrolyte imbalance, and further cortisol injections.
Please see our crisis pages for more detailed information. Section 2
Specific patients' questions:- In this section we try to answer questions on particular points asked by a number of patients, using an example question from one such patient.
Subject: Antibody testing with unexpected results.
Q:- "I had an antibody test done on my adrenals,and it came back negative, yet I am primary. I and one of my 2 doc isn't a big fan of this antibody test because it can vary so much. I had an antibody test done on my thyroid also the same day, and that was negative too, yet I have Hashi's. Why please?"
Subject: Precautions in taking oral steroid tablets.
Q:- ".......does HC need to be taken with food? The Australian Addison's site says not - as the doses are mini doses. The Endo and Dr don't think so - as they never write it in the intructions or tell me verbally. BUT ....the chemists insist on printing in on the label with the Drs instructions ( not a sticker )."
A few rarer causes have also been discovered. Examples include adrenal haemorrhage resulting in calcification, raised cortisol binding globulin potentially resulting in low free cortisol, hormonal hyper-excretion syndrome, etc.
We have tried to answer many of the questions that patients want to know about their disease or that of their loved ones within the pages of ADNetwork. Whilst the scientific background has been included where appropriate for those who wish or need to educate themselves, or who use ADNetwork as a reference site, many other readers do not have the benefit of a scientific background and for this majority we have deliberately tried to explain points in a way which the lay person can understand easily.
The second section looks at specific discussion points & questions raised by patients.
In the case of autoimmune Addison's disease, over 40% of patients also have Hashimoto's autoimmune hypothyroidism, i.e. the patient also has produced antibodies against thyroid tissue. This is a recognised pairing of autoimmune diseases. Autoimmune diseases against both adrenal and thyroid tissues is often referred to as Schmidt's syndrome. Other diseases which may be seen in conjunction with autoimmune Addison's disease are vitiligo (against melanocytes, the skin cells which cause tanning), lupus (against specific white blood cell nuclei), Diabetes mellitus type 2 (pancreatic insulin producing cells), pernicious anaemia (against the intrinsic factor which carries vitamin B12 from the gut into the blood stream, or the cells which produce intrinsic factor). This is not a complete list but shows some of the specific diseases which may be included under the umbrella called 'Polyglandular Syndrome'
To confirm or exclude the possibility of secondary disease, even in patients where primary Addison's is indicated by the tests above, an Insulin Stress Test (IST)to test pituitary function and an MRI scan of the head, looking especially at the pituitary gland area of the brain to exclude physical abnormalities.
A patient already on hydrocortisone or prednisolone and on whom the clinician desires to perform a SynActhen test may be switched to the equivalent dose of Dexamethasone for two weeks prior to the test. The test results will then show an up-to-date status on the patient's capability to produce and release cortisol.
It is very difficult to explain 'how' we know when we need extra salt, but around 65% of Addison's patients suffer from salt craving and around 45% suffer from excessive sweating. Over 50% also experience what they describe as increased or excessive thirst not necessarily related to Diabetes mellitus. Experience with these symptoms enables most of us to somehow 'know' when we need extra salt.
The normal ranges for these two are:- Sodium 135-155mmol/L (although it is noted that some labs in USA are now quoting ranges of around 135-145mmol/L) and for Potassium 3.5-5.5mmol/L)
If you consider this and the 'extra salt' question above together, you can hopefully understand that if we 'dump' salt, then our potassium levels will tend to rise. In the short term this is not too much of a problem for normal patients. However for us it can be disastrous. When our sodium level drops, extra potassium is brought out of storage from our red blood cells. If the level of circulating potassium rises above normal, it becomes increasingly difficult for our body to correct it. The potassium acts as a diuretic, causing us to urinate and sweat more to remove fluid. This increases the potassium even further, causing an ever decreasing vicious circle. Once the level of potassium exceeds 6.0-6.5mmol/L we are liable to require urgent hospitalization to bring our electrolyte balance back under control. The shock of this will also lower our circulating cortisol level and we are increasingly likely to go into an Addison's crisis. Once potassium levels exceed around 7.0mmol/L, it can be exceedingly to reverse and force the extra potassium back into red blood cell storage, even with in-patient medical intervention, and without which the patient is increasingly likely to die.
Bone calcium loss can be a side effect of steroids. This is particularly evident with excessive cortisol levels, which is why we should always closely monitor our cortisol replacement to be the minimum required to just control our symptoms. This amount varies from patient to patient. Too little and symptoms may return in a few weeks. Too much, particularly over a long term, and we risk osteoporosis. Some patients choose to take a combined calcium/Vitamin D3 supplement. This choice is yours but this supplement is unlikely to cause any harm and may help prevent osteoporosis.
In general, Addison's patients are told to temporarily double (and in some case to treble) their normal daily doses of replacement cortisol in case of excessively stressful situations, e.g. road accident, maybe even a long journey especially if this causes you concern, clinical shock (surgical operation, broken bones, heavy blood loss etc.) or infection. Whilst the latter may not apply to simple minor infections, it would normally apply to those which would cause the patient to consult their doctor for e.g. chest infection, diarrhoea & vomiting (D&V), and cause the patient to double their dose for the duration they are ill.
Failure to taper or to taper too fast has been known to precipitate an Addisonian crisis.
A patient on stable replacement therapy for Addison's disease can now expect to have a normal length of life (excepting other unexpected eventualities of course).
In about 10% of AD patients the blood pressure may be seen to rapidly INCREASE in case of imminent or established crisis to potentially dangerous levels (up to around 250/150mm Hg.) instead of dropping. This is now known to be due to a release of excessive amounts of adrenaline from various sites in the body.
Last year I asked and was tested for Addisons antibodies [adrenal antibodies. Ed.] and I noticed that the path lab had a disclaimer saying that a negative result did not rule out autoimmune Addisons. As Addisons can be a life or death illness I expect this is a legal disclaimer to guide the GPs.
Unfortunately - no such advice is attached to Hashimotos antibody tests here. It is sad to see people still suffering. On the Australian thyroid list I am on - I have seen people have to go from Dr to Dr even after over range antibodies are discovered - as the Dr does not believe they are 'high enough' to be Hashis.. Its all very sad.
[Actually that comment by the lab looks like it may have from a thoughtful biomedical scientist realizing that not everone would appreciate that these antibodies can be transient. Alternatively it may be an automatic comment with every negative adrenal antibody report, programmed into their computer reporting system by someone who did actually know the significance. I doubt that it was put in purely as a legal opt-out. Of course, even if it was, it still serves a useful purpose.
Thyroid antibodies, on the other hand, are usually still present at time of Hashi's diagnosis & often do not diminish until years into replacement therapy if at all, and therefore the warning is not so warranted as with negative adrenal antibodies.]
I can understand the line of thinking some people may have about small (relatively) doses but the fact remains that, like aspirin, drugs like hydrocortisone etc. do irritate the stomach lining. However the dose of, for example, hydrocortisone which may well be adjacent to the lining of the stomach as soon as the tablets have dissolved is far, far greater than the level of circulating cortisol adjacent to cells elsewhere in the body, even when compared with the levels of cortisol present in normal patients and, I suspect, even in patients with Cushing's disease in which cortisol may be released into the bloodstream at an alarming rate.
Thus even two 10mg tablets of hydrocortisone dissolved in the small amount of stomach fluid present before a meal may be sufficient to irritate the stomach wall, let alone with high doses necessary for treatment regimes used for other disease processes. I know a number of patients have commented that taking the typical replacement doses without food causes stomach pain, and I include myself in this group. From personal experience I know that this stomach pain does not occur when the tablets are taken immediately after a biscuit or two and a drink, but often will occur if I try to cheat, taking them just 10-15 few minutes before a meal.
If the irritant effect on the stomach lining of drugs like hydrocortisone is anything like that experienced with aspirin, then having seen the long-term results of such irritation many times from the laboratory point of view, I can assure you that I personally will continue to only take my steroid replacement with or immediately after food.
I would prefer not to go into descriptive details of the damage so caused. These effects are, suffice to say, not pleasant to observe.