Addison’s Disease
Patient Advocate
Equity Press - HealthScouter.com
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Edited By: Katrina Robinson
Includes Addison’s Disease from Wikipedia http://en.wikipedia.org/wiki/Addison’sDisease
HealthScouter Addison’s Disease: Addison Disease Symptoms and Addison’s Disease Treatment (HealthScouter Addison’s Disease)
ISBN: 978-1-60332-263-8
Smashwords Edition
Edited Components are Copyright (c) 2009 Equity Press
Permission is granted to copy, distribute and/or modify this document under the terms of the GNU Free Documentation License, Version 1.2 or any later version published by the Free Software Foundation; with no Invariant Sections, no Front-Cover Texts, and no Back-Cover Texts. A copy of the license is included in the section entitled "GNU Free Documentation License".
HealthScouter and Equity Press do not provide medical advice. The contents of this book are for informational purposes only and are not intended to substitute for professional medical advice, diagnosis or treatment. Always seek advice from a qualified physician or health care professional about any medical concern, and do not disregard professional medical advice because of anything you may read in this book or on a HealthScouter web site. The views of individuals quoted in this book are not necessarily those of HealthScouter or Equity Press.
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Introduction to Addison’s Disease
Adrenocorticotropic Hormone Stimulation Test
Adrenocorticotropic hormone plasma test plus cortisol stimulation
Other hormones and chemicals that will rise in the adrenocorticotropic hormone stimulation test
Relatives of an Affected Patient
Factors affecting cortisol levels
Factors generally reducing cortisol levels
Factors generally increasing cortisol levels
References: Adrenal Insufficiency
References: Adrenocorticotropic Hormone Stimulation Test
References: Adrenoleukydystrophy
References: Adrenomyeloneuropathy
References: Autoimmune Adrenalitis
GNU Free Documentation License
Dear Reader,
I like to think of myself as a polite, well-reasoned person. I rarely speak out or complain. When a waitress spills something on me, or if my meal is cold—or if I’m overcharged—I generally try to be as polite as possible. I don’t like to make very many waves. I often secretly hope that the manager will hear about my predicament and come out and offer me a free meal, or something similar. I generally hope that my polite and respectful demeanor pays off. And it does happen from time to time. You know, I think many people are brought up to believe that this is just good manners. It’s how you’re supposed to behave. And if you knew me personally, I think you’d agree that I’m generally pretty reserved. Of course my wife may raise an objection or two (!), but I really believe that it’s important to treat others as you would like to be treated. We’re talking about the golden rule here—it works well and it applies to almost every life circumstance.
But I have to admit that when it comes to my health, or the health of someone I care about—all bets are off. I want to know what’s going on—when, why, where, and how. And I make these feelings known. I tend to get downright assertive. It’s just something I feel very strongly about. And I feel that when you are in a hospital, or if you’re brushing up against the healthcare system, that you should feel the same way. It’s unfamiliar turf, and the professionals who work in this system often take advantage of their positions. They may use some jargon to hide the whole truth— or they may say something without checking to make sure you understand completely. They may presentthe options that are best for them, perhaps the most profitable or convenient. Now I’m not saying this goes on everywhere. There are many professionals in thebusiness of health who go out of their way to make sure you have the best care. And I’m not suggesting that you should become a bully, or purposefully annoying—absolutely not. But I am suggesting that I think it’s OK for you to step outside of your typical comfort zone, and put on your patient advocate hat. Because you, the patient or patient advocate, care the most about your care—not the medical system or healthcare providers.
HealthScouter was created to help patients become better advocates for their own medical care. Because when it comes to your healthcare, the stakes are high. There are none higher. And healthcare is one area where consumers (us, the sick people) are notoriously unaware of their options. And that’s why I’m publishing these books. To help you understand your options, and to help you get the best care possible. I want to help you become a better advocate for yourself and for your loved ones.
It’s my sincere hope that you can take this book with you to the hospital, to be read in the waiting room or by the bedside—and when you see a relevant patient comment you can use this book to ask questions of your health care providers. My advice: Ask lots of questions! Providers are busy people who generally go about their business with little questioning, delivering care as they see fit—making quick decisions—and again, nobody is going to care as much about your health as you. So now, more than ever, you need tools at your disposal to get the best care possible. One of the tools at your disposal is this HealthScouter book and the material within. You need to be armed with questions, and you need to ask questions all of the time. And so the difficult part is now to understand the right questions to ask.
That brings me to an explanation of how these books are structured. HealthScouter books include a number of what we call patient comments. These patient comments are summaries of what people have experienced. They’re first hand accounts of what you may expect. These experiences effectively help you “catch up,” and understand what outcomes are possible. They expose you to the treatments are available, and provide insight as to potential outcomes. They help you understand what other people are doing. So if you find yourself stuck feeling like you’re receiving substandard medical care—or if you need a push to broach the subject, you can take this book to your provider and say, “Hey, I read here that another patient had this treatment—is that an option for me? If not, Why?” I believe that other peoples’ experience is the most valuable way for you to formulate and build a list of good questions for your healthcare providers.
That notion is at the core of the HealthScouter philosophy.
So HealthScouter, by providing patient comments about a particular medical condition, will help expose you to what other people have experienced about a particular medical problem. If you know what other people have experienced, you can better understand what your options are. You’ll be better informed and you’ll have some questions to ask—it’ll be like you’ve had access to dozens of other people who have gone through the same thing you’re going through. And so armed, maybe you’ll be able to move through your condition and get back on the road to health, and maybe you’ll be able to do this with more grace than I have. And that is my sincere wish.
It’s also my wish that perhaps when a doctor or nurse sees this little book, that they’ll think twice about the care they’re about to provide—knowing that the owner is a little bit better prepared, a little bit better armed—and yes, maybe even downright assertive.
I hope this book helps.
Yours truly,
Jim Stewart
San Diego, California
Introduction to Addison’s Disease
Addison's disease (also known as chronic adrenal insufficiency, hypocortisolism or hypocorticism) is a rare endocrine disorder in which the adrenal gland does not produce enough steroid hormones (glucocorticoids and often mineralocorticoids).[1] It may develop in children, adults or some species of animals, and may occur as the result of many underlying causes.
The condition is named after Dr Thomas Addison, the British physician who first described the condition in his 1849 publication On the Constitutional and Local Effects of Disease of the Suprarenal Capsules.[2] The adjective "Addisonian" is used for features of the condition, as well as patients with Addison's disease.[3]
The condition is generally diagnosed with blood tests, medical imaging and additional investigations.[3] Treatment involves replacement of the hormones (oral hydrocortisone and fludrocortisone). If the disease is caused by an underlying problem, it may be possible to address that. Regular follow-up and monitoring for other health problems is necessary.[3]
While Addison's six patients in 1855 all had adrenal tuberculosis, [4] the term "Addison's disease" does not imply an underlying disease.
Patient 1:
In June, the doctors diagnosed me with Addison's disease. Now the doctors have told me that they believe I may be suffering with Lyme’s disease as well. My condition has gotten much worse since they put me on these steroids for Addison's. Now it has effected my nervous system and the doctors are baffled. They are doing a Lyme’s.
Does anyone if it’s true that steroids can make Lyme’s disease twice as bad? This scares me, as I cannot come off the steroids because of the Addison’s disease. Does anyone know how hard it is to get rid of Lyme’s disease once it has started to hit the nervous system? Is it curable?
Patient 2:
A person can get better if they have Lyme. Not cured but better. I have heard once you have gotten the bacteria in you, you will always have it. The trick is, is to raise the immune system.
I was told I had Addison at one point along my journey. I was told I had many conditions...but I had Lyme. I didn't do treatment for Addison's. I did have a doctor put me on steroids to ease the inflammation and it was the worst thing any doctor could do to a patient. They are NOT good for a person’s system. If the doctors suspect Lyme, they need to treat you for Lyme. They need to put you on antibiotics until your symptoms disappear.
The symptoms of Addison's disease develop insidiously, and it may take some time to be recognized. The most common symptoms are fatigue, dizziness, muscle weakness, weight loss, difficulty in standing up, vomiting, anxiety, diarrhea, headache, sweating, changes in mood and personality, and joint and muscle pains. Some have marked cravings for salt or salty foods due to the urinary losses of sodium. [3] Adrenal insufficiency is manifested in the skin primarily by hyperpigmentation.[5]
Patient 1:
A few months ago I learned I have Addison's. Does anyone have problems with pain in their legs, and is it because of Addison’s? I am extremely tired and get pains day and night.
Patient 2:
I am also new to Addison’s and have pain in my legs, along with lots of fluid, and they feel really weak too. I have been sick with some other pain problems for many years, and have been very inactive for all that time, so I did not have a lot of strength even before I got Addison’s. But the leg swelling and the pain came when I started the medicine. I would say, but without knowing much about it, that to me it sounds like you might not get enough medicine if you are so tired.
Patient 3:
I have been diagnosed with Addison’s. I have been taking hydrocortisone, but it only helps a little. I have to say the pain in my legs and hands started after taking this medicine.
Because primary hypocortisolism is manifested as a deficiency in glucocorticoid release from the adrenal cortex, increased adrenocorticotropic hormone will be released by the pituitary in order to trigger release of the absent glucocorticoid; it is because of this overstimulation of adrenocorticotropic hormone that bronzing of the skin occurs. In secondary or tertiary hypocortisolism, there is a deficiency of either corticotropin releasing hormone or adrenocorticotropic hormone release by the hypothalamus or pituitary gland, respectively. The former will manifest as no adrenocorticotropic hormone release while the latter will manifest as physiologic (normal) adrenocorticotropic hormone release; neither will cause an overproduction of adrenocorticotropic hormone.
On examination, the following may be noticed: [3]
Low blood pressure that falls further when standing (orthostatic hypotension)
Most people with primary Addison's have darkening (hyperpigmentation) of the skin, including areas not exposed to the sun; characteristic sites are skin creases (e.g. of the hands), nipples, and the inside of the cheek (buccal mucosa), also old scars may darken. This occurs because melanocyte-stimulating hormone (MSH) shares the same precursor molecule as adrenocorticotropic hormone (ACTH); an increase in adrenocorticotropic hormone production also increases melanocyte-stimulating hormone. In secondary and tertiary forms of Addison's, skin darkening does not occur.
Signs of conditions that often occur together with Addison's: goiter and vitiligo.
Patient 1:
My daughter just got diagnosed with Addison’s, and I am completely overwhelmed. Does anyone have any tips for my daughter? She has just started flornef and hydrocortisone tablets and feels better already.
Patient 2:
If your daughter is still in school, you should make sure the school staff knows she has Addison's. You should also ask to be notified if other students in her class develop the stomach flu so you can be cautious. Did your doctor give you a shot (for example Solo-Cortef) to carry in case of an emergency? If your daughter gets the stomach flu, she will need extra medication. For me, I have had several crises because of the stomach flu and have to go to the emergency room shortly after beginning to vomit. She will also need more medication for any type of surgery or if she is in a serious accident. She should wear a medical bracelet.
Patient 3:
With Addison's, the key is getting the right amount of medications that works for you. Once you have found the right amount you do pretty well. When you get sick, if you cannot keep your medications down, you have to go to the emergency room because you can go into Adrenal shock.
An "Addisonian crisis" or "adrenal crisis" is a constellation of symptoms that indicate severe adrenal insufficiency. This may be the result of either previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal function (such as adrenal hemorrhage), or an underlying problem (e.g. infection, trauma) in the setting of known Addison's disease. Additionally, this situation may develop in those on long-term oral glucocorticoids who have suddenly ceased taking their medication. In these people, long term use of synthetic glucocorticoids will have caused further atrophy of the adrenal glands by negative feedback. It is also a concern in the setting of myxedema coma; thyroxine given in that setting without glucocorticoids may precipitate a crisis.
Untreated, an Addisonian crisis can be fatal. It is a medical emergency, usually requiring hospitalization. Characteristic symptoms are: [6]
Sudden penetrating pain in the legs, lower back or abdomen
Severe vomiting and diarrhea, resulting in dehydration
Low blood pressure
Syncope (loss of consciousness)
Hypoglycemia
Confusion, psychosis, slurred speech
Severe lethargy
Hypocalcaemia
Convulsions
Fever
Patient 1:
I have had Addison's Disease since 1987 and was put on Cortisone maintenance. I had a very severe Addisonian Crisis in January 2009 that started with vomiting and diarrhea, which made me so weak that I could not walk. The pain in my back and legs was ignored because I had back surgery four months prior. After falling three times, the EMS was called. I went into respiratory arrest before they arrived, but thanks to my partner knowing CPR, I was revived.
The EMS did not apparently did not understand Addison’s, and the emergency room also was very slow to react to my needs. Finally after I had total kidney failure and a temp of 104, they put me in ICU to address my crisis. I was in the hospital for eight days.
Two months later, I am now experiencing moderate to severe fatigue. My doctors say that it is not Addison’s, but I beg to differ. They think that I am just depressed. I am very frustrated at this point. Is the fatigue due to Addison’s?
Patient 2:
I can attest that Addison's can be associated with severe fatigue, so I agree it’s probably associated with your problems.
Did you recently take an antibiotic? If so it may be related to an intestinal yeast infection. The yeast byproducts are released into your gut and if you are sensitive to those, you can feel great fatigue. Addison's makes it easy for yeast to grow, so it’s also important to cut back on all sugar products because those make yeast grow fast.
Zinc, selenium, vitamin D and vitamin C are known to support your immune system and I strongly recommend you start taking those along with a multi-vitamin.
Patient 1:
I have been fine since being diagnosed with Addison’s diagnosis, living life to the fullest, enjoying it, and everything was peachy...until last weekend. Something seems to have snapped in my head and I do not know what hit me or how to fix it. I have been so sick, not able to eat, not able to handle doing the things I used to do all the time. I am scared, tense and feel like there is someone else in my head that is evil. I have lost weight, have shaking and crying spells, sweating and chills. I did go to my family doctor and talked in depth about this and she feels that it is a combination of things including stress and medication dosage. I am going to talk to her again on Monday but as of right now I would not say it is getting any better. Has anyone else experienced this and if so what was it and what fixed it?
Patient 2:
This is exactly how I feel before I go into a crisis. Make sure you are getting enough hydrocortisone.
Patient 3:
I also feel like this before I am going into crisis or if I have had a particularly stressful day or week. The shaking and crying happens to me and it's very frustrating. I would work to reduce whatever stressors you have in your life right now and possibly double your medications (particularly your prednisone, if you take that).
Routine investigations may show:[3]
Hypercalcemia
Hypoglycemia, low blood sugar (worse in children)
Hyponatraemia (low blood sodium levels), due to loss of production of the hormone aldosterone
Hyperkalemia (raised blood potassium levels), also due to loss of production of the hormone aldosterone
Eosinophilia and lymphocytosis (increased number of eosinophils or lymphocytes, two types of white blood cells)
Metabolic acidosis (increased blood acidity), also due to loss of the hormone aldosterone because sodium reabsorption in the distal tubule is linked with acid/hydrogen ion (H+) secretion. Low levels of aldosterone stimulation of the renal distal tubule leads to sodium wasting in the urine and H+ retention in the serum.
Patient 1:
I have just been diagnosed with Addison’s this week and was told I have complete adrenal failure. My doctor thinks I have more going on than just Addison’s disease as well, but my endocrinologist can’t see me for at least another eight weeks as he is too busy. I have been told they want me in hospital as soon as possible, but due to the lack of beds and staff, this is not possible at the moment.
Has anyone had to wait this long before seeing their endocrinologist? I was sent home from the hospital with just my medication and no information about the disease at all.
Patient 2:
I cannot really address the Addison's issue but I would suggest that you take charge of your own health and get a copy of the test results for yourself. The adrenal glands produce a variety of hormones including testosterone, aldosterone and cortisol. All of these steroids control many things in your body including your blood pressure, how you handle stress, and your gynecological functions such as ovulation, menstruation, etc. There is a large variety of problems that can occur with adrenal glands and sometimes the adrenals simply have a problem converting the precursor of these hormones into the hormones themselves. You may therefore be experiencing an imbalance of these hormones and this would be causing your problems. The steroids are the treatment of choice at the moment. However, the medication may need to be adjusted or perhaps another type of steroid tried in order to fit your specific situation. All will depend on what your endocrinologist has to say.
I would also try to find an endocrinologist a little sooner. Ask some of your friends or family if they happen to know anyone or call the association of endocrinologists in your country or area and see if they can suggest something.
Patient 3:
You have to get to an endocrinologist soon; look in your area for endocrinologists that can see you quicker. Or go to your closest emergency room for help and get monitored.
In suspected cases of Addison's disease, one needs to demonstrate that adrenal hormone levels are low even after appropriate stimulation (called the adrenocorticotropic hormone stimulation test) with synthetic pituitary adrenocorticotropic hormone tetracosactide . Two tests are performed, the short and the long test.
The short test compares blood cortisol levels before and after 250 micrograms of tetracosactide (IM/IV) is given. If, one hour later, plasma cortisol exceeds 170 nmol/L and has risen by at least 330 nmol/L to at least 690 nmol/L, adrenal failure is excluded. If the short test is abnormal, the long test is used to differentiate between primary adrenal failure and secondary adrenocortical failure.
The long test uses 1 mg tetracosactide (IM). Blood is taken 1, 4, 8, and 24 hours later. Normal plasma cortisol level should reach 1000 nmol/L by four hours. In primary Addison's disease, the cortisol level is reduced at all stages whereas in secondary corticoadrenal insufficiency, a delayed but normal response is seen.
Other tests that may be performed to distinguish between various causes of hypoadrenalism are renin and adrenocorticotropic hormone levels, as well as medical imaging - usually in the form of ultrasound, computed tomography or magnetic resonance imaging (MRI).
Adrenoleukodystrophy, and the milder form, adrenomyeloneuropathy, cause adrenal insufficiency combined with neurological symptoms. These diseases are estimated to be the cause of adrenal insufficiency in approximately 35% of male patients with idiopathic Addison’s disease and should be considered in the differential diagnosis of any male with adrenal insufficiency. Diagnosis is made by a blood test to detect very long chain fatty acids (VLCFA).[7]
Patient 1:
I was wondering if anyone here has been diagnosed with secondary or tertiary Addison's? My primary physician is convinced that this is what has been plaguing me for almost five years now. She referred me to an Endocrinologist, and I see him this afternoon. I only have my lab results from 2005, which are: