Addisons disease

Addison's disease  Illnesses and conditions Symptoms and self-help Tests and treatments Healthy living Care, support and rights Scotlands Service Directory 0 Home Illnesses and conditions Glands Addisons disease Addisons disease About Addisons disease Symptoms of Addisons disease Causes of Addisons disease Diagnosing Addisons disease Treating Addisons disease About Addisons disease Addisons disease (also known as primary adrenal insufficiency or hypoadrenalism) is a rare disorder of the adrenal glands. The adrenal glands are 2 small glands that sit on top of the kidneys. They produceessential hormones: cortisol,aldosterone and adrenaline. In Addisons disease, the adrenal gland is damaged, and not enough cortisol and aldosterone are produced. About 8,400 people in the UK have Addisons disease. It can affect people of any age. Its most common between the ages of 30 and 50.Its also more common in women thanmen. Early-stage symptoms of Addisons disease are similar to other more common health conditions, such as depression or flu .You may experience: fatigue (lack of energy or motivation) muscle weakness low mood loss of appetite and unintentional weight loss increased thirst Over time, theseproblems may become more severe and you may experience further symptoms, such as dizziness, fainting,crampsand exhaustion. You may also develop small areas of darkened skin, or darkened lips or gums. Although these symptoms arent always caused by Addisons disease, you shouldsee your GP, so they can be investigated. Read more about the symptoms of Addisons disease and diagnosing Addisons disease Why it happens The conditionisusually the result of a problem with the immune system, which causes it to attack the outer layer of the adrenal gland (the adrenal cortex), disrupting the production of steroid hormones aldosterone and cortisol. Its not clear why this happens, but its responsible for 70% to 90% of cases in the UK. Other potential causes include conditions that can damage the adrenal glands, such as tuberculosis (TB) , although this is uncommon in the UK. Read more about the causes of Addisons disease Treating Addisons disease Addisons disease is treated with medication to replace the missing hormones. Youll need to take the medication for the rest of your life. With treatment, symptoms of Addisons disease can largely be controlled. Most people with the condition live a normal lifespan andare able tolive an active life, with few limitations. However,many people with Addisons disease also find they must learn to managebouts of fatigue and there may be associated health conditions, such as diabetes or an underactive thyroid . People with Addisons disease must be awareof the risk of a sudden worsening of symptoms, called an adrenal crisis. Thiscan happenwhen the levels of cortisolin your body fallsignificantly due to not taking your medicines, or during another illness. An adrenal crisis is a medical emergency and warning signs include severe nausea, confusion, fever, headache and weakness. If left untreated, it can be fatal. If you or someone you know has Addisons disease and is experiencing severe symptoms, phone 999 for an ambulance. Read more about treating Addisons disease Information about you If you have Addisons disease, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS). This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time. Find out more about the register Symptoms of Addisons disease Addisons disease can be difficult to detect at first, because early symptoms are similar to symptoms of many other health conditions. Initial symptoms of Addisons disease caninclude: fatigue (lack of energy or motivation) lethargy (abnormal drowsiness or tiredness) muscle weakness low mood (mild depression ) or irritability loss of appetite and unintentional weight loss the need to urinate frequently increased thirst craving for salty foods Dehydration can also be an early sign of Addisons disease. Its caused by lack of the hormone aldosterone in your body, which is used to regulate the balance of salt and water. Later symptoms Furthersymptoms of Addisons disease tend to develop gradually over months or years. However,additional stress, caused by another illness or an accident, for example, may cause your symptoms to suddenly get worse. You may go on to develop: low blood pressure when you stand up, which can cause dizziness and fainting feeling sick (nausea) vomiting diarrhoea abdominal,joint or back pain muscle cramps chronic exhaustion, which may cause depression brownish discolouration of the skin, lipsand gums (hyperpigmentation), particularly in the creases on your palms, on scars or on pressure points, such as your knuckles or knees a reduced libido (lack of interest in sex) , particularly in women Some women may also have irregular periodsor miss some periods completely.Children with Addisons disease may experience pubertylater than usual. Some people with Addisons disease also develop low blood sugar (hypoglycaemia) . This can cause symptoms such asdifficulty concentrating, confusion, anxiety and even unconsciousness (particularly in children). If youre experiencingsymptoms of Addisons disease, see your GP so they can diagnose or rule out the condition.These symptomswill usually improve withappropriate treatment. Read more about diagnosing Addisons disease and treating Addisons disease Adrenal crisis If Addisons disease is left untreated, the levels of hormones produced by the adrenal glandgradually decrease in the body. This causes your symptoms to get progressively worse and eventually lead to a life-threatening situationcalledan adrenal or Addisonian crisis. During an adrenal crisis, the symptoms of Addisons disease appear quickly andseverely. This could happen whenyoure already experiencing initial symptoms or without any symptoms at all. Signs of an adrenal crisis include: severe dehydration pale, cold, clammy skin sweating rapid, shallow breathing dizziness severe vomiting and diarrhoea severe muscle weakness headache severe drowsiness or loss of consciousness An adrenal crisis is a medical emergency. If left untreated, it can be fatal. If you think you or someone you knowwith Addisons diseaseis having an adrenal crisis, phone 999 for an ambulance. If an adrenal crisis isnt treated, it can lead to a coma and death. Theres also a risk your brain wont get enough oxygen if treatment is delayed, whichcan cause permanent disability. Causes of Addisons disease Addisons disease develops when the outer layer of your adrenal glands (your adrenal cortex) is damaged, reducing the levels of hormones it produces. Problems with the immune system In the UK, a problem with the immune system is the most common cause of Addisons disease, accounting for 70% to 90% of cases. Theimmune system is your bodys defence against infection and disease. If youre ill, your immune system produces antibodies (a special type of protein that destroys disease-carrying organisms and toxins). These antibodies attack the cause of the illness. However, if you develop a problem with your immune system, it can start to attack your own healthy tissues and organs. This is known as an autoimmune disorder. Addisons disease can develop if your immune system attacks your adrenal glands and severely damages your adrenal cortex. When 90% of the adrenal cortex is destroyed, your adrenal glands wont be able to produce enough of the steroid hormones cortisol and aldosterone. Once levels of these start decreasing, youll experience symptoms of Addisons disease . Its not clear why some people develop this problem with their immune system, although it can run in families. Genetics Research has shown that some people withcertain genesare more likely to develop autoimmune disorders. Its not clear how these genes lead to Addisons diseaseandsimilar conditions, but it does meanyour risk of developing Addisons disease is increased if you or aclose family memberhave another autoimmune condition, such as: vitiligo  a chronic (long-term) condition that causes pale, white patches to develop on the skin type 1 diabetes  a chronic condition caused by too much glucose in the blood underactive thyroid gland (hypothyroidism) Other causes Tuberculosis (TB) is the most common cause of Addisons disease worldwide, but is rare in the UK. TB is a bacterial infection that mostly affects the lungs, but can also spread to other parts of your body.It can cause Addisons disease if it damages your adrenal glands. Other possible causes of Addisons disease include: infections  such as those linked to AIDS , or fungal infections a haemorrhage  very heavy bleeding into the adrenal glands, sometimes associated with meningitis or other types of severe sepsis cancer  if cancer cells from elsewhere in your body spread to your adrenal glands amyloidosis  a disease where amyloid, a protein produced by your bone marrow cells, builds up in your adrenal glands and damages them surgical removal ofboth adrenal glands (adrenalectomy)  for example, to remove a tumour adrenoleukodystrophy (ALD)  a rare, life-limiting, inherited condition affecting the adrenal glands and nerve cells in the brain thatmost oftenaffects young boys certain treatments needed for Cushings syndrome  a collection of symptoms caused by very high levels of cortisol in the body Diagnosing Addisons disease To help diagnose Addisons disease, your GP will first ask about your symptoms and review your medical history. Theyre also likely to ask if anyone in your family hasan autoimmune disorder (a condition caused by a problem with their immune system). Your GP will examine your skin for any evidence of brownish discolouration (hyperpigmentation), particularly in certain areas, such as: where skin creases on your palm in your elbow crease on any scars your lips and gums However, hyperpigmentation doesnt occur in all cases of Addisons disease. Youll also be tested for low blood pressure (hypotension) while youre lying down and again shortly after you stand up. This is to see whether you have postural or orthostatic hypotension (low blood pressure when you change position). Blood tests If Addisons disease is suspected, blood tests will be carried out to measure the levels ofsodium, potassium and cortisolin your body. A low sodium, high potassium or low cortisol level may indicateAddisons disease. You may need to see a hospital hormone specialist (endocrinologist) for your bloodto be tested for the following: a low level of the hormone aldosterone a high level of adrenocorticotrophic hormone (ACTH) a low level of glucose (sugar used for energy) positive adrenal antibodies (antibodies designed to attack the adrenal gland) Any of these could be a sign of Addisons disease. Synacthen stimulation test If cortisolin your bloodislow, or your symptoms strongly suggest Addisons disease, youll need to have a synacthen stimulation test to confirm the diagnosis. Your GP may refer you to an endocrinology unit (a unit that specialises in the study of hormones) for the test. How urgently youre referred depends on how severe your symptoms are. Synacthen is a man-made (synthetic) copy of the adrenocorticotrophic hormone (ACTH). ACTH is naturally produced by the pituitary gland (a pea-sized gland below the brain) to encourage the adrenal glands to release the hormones cortisol and aldosterone. When synacthen is administered, the adrenal glands should respond in the same way as they would to ACTH, and release cortisol andother steroid hormonesinto the blood. A blood sample will be taken and tested for cortisol, before an injection ofsynacthen is given into your arm. After 30 and 60 minutes, a furtherblood sample will be taken for cortisol measurement. If the ACTH level is high, but the cortisol and aldosterone levels are low, its usually confirmation of Addisons disease. Thyroid function test As well as a synacthen stimulation test, your thyroid gland may also be tested to see if its working properly. Your thyroid gland is found in your neck. It produces hormones that control your bodys growth and metabolism. People with Addisons disease often have an underactive thyroid gland (hypothyroidism) , where the thyroid gland does not produce enough hormones. By testing the levels of certain hormones in your blood, your endocrinologist (a specialist in hormone conditions) can determine whether you have hypothyroidism. Scans In some cases, yourspecialist may refer you for a scan of your adrenal glands. This could be a computerised tomography (CT) scan or a magnetic resonance imaging (MRI) scan . Diagnosis during an adrenal crisis If Addisons disease is left untreated, it eventually leads to an adrenal crisis. This is wherethe symptoms of Addisons disease appear quickly and severely. During an adrenal crisis, theres not enough time to perform a synacthen stimulation test to confirm Addisons disease. If possible, blood will be taken and tested for any of the abnormalities listed above. While youre waiting for the results, treatment may be started with steroid injections, and fluids containing salt and glucose. Read more about treatingAddisons disease Driving If youre diagnosed with Addisons disease andhave a bus, coach or lorry licence, its your legal obligation to inform the Driver and Vehicle Licensing Agency (DVLA). Readabout driving andAddisons disease on the GOV.UK website. Treating Addisons disease If you have Addisons disease, youll need to take daily medication to replace the lost hormones. This should help you to live an active life, although many people find they still need to manage their fatigue. Insome cases, the underlying causes of Addisons disease can be treated. For example, tuberculosis (TB) is treated with a course of antibiotics over a period of at least 6 months. However, most cases are caused by a problem with the immune system that cant be cured. Medication forAddisons disease Treatment usually involves corticosteroid (steroid) replacement therapy for life. Corticosteroid medication is used to replace the hormones cortisol and aldosterone thatyour body no longer produces.Its usually taken in tablet form 2 or 3 times a day. In most cases, a medication calledhydrocortisoneis used to replace the cortisol. Other possible medicines areprednisolone or dexamethasone, though these are less commonly used. Aldosterone is replaced with a medication called fludrocortisone. Your GP may also ask you to add extra salt to your daily diet, although if youre taking enough fludrocortisone medicine thismay not be necessary.Unlike most people, if you feel the urge to eat something salty, then you should eat it. In general, the medications used for Addisons disease dont have side effects, unless your dose is too high. If you take a higher dose than necessary for a long time, theres a risk of problems such as weakened bones (osteoporosis) , mood swings and difficulty sleeping (insomnia) . Living with Addisons disease Many people with Addisons disease findthat taking their medication enables them to continuewith theirnormal diet and exercise routines. However, bouts of fatigue are also common, and it cantake some time to learn how to manage these periods of low energy. Some people find that needing to take regular doses of medication is restrictive and affects their daily life or emotional health.Missing a dose of medication, or taking it late,can also lead to exhaustion or insomnia. Some people can develop associated health conditions, such as diabetes or an underactive thyroid , which require extra treatment and management. Youll usually need to have appointments with an endocrinologist every 6 to 12 months, so they can review your progress and adjust your medication dose, if necessary.Your GP can provide support and repeat prescriptions in between these visits. Failing to take your medication could lead to a serious condition called an adrenal crisis, soyou must: remember to collect your repeat prescriptions keep spare medication as necessary  for example, in the car or at work, and always carry some spare medication with you take your medication every day at the right time pack extra medication if you are going away  usually double what you would normally need, plus your injection kit (see below) carry your medication in your hand luggage if you are travelling by plane, with a note from your doctor explaining why it is necessary You could also inform close friends or colleagues of your condition. Tell them about the signs of adrenal crisis and what they should do if you experience one. Medical alert bracelets Its also a good idea to wear a medical alert bracelet or necklace that informs peopleyou have Addisons disease. After a serious accident, such as a car crash, a healthy person produces morecortisol. This helps you cope with the stressful situation and additional strain on your body that results from serious injury. As your body cannot produce cortisol, youll need a hydrocortisone injection to replace it and prevent an adrenal crisis. Wearing a medical alert bracelet will inform any medical stafftreating you about your condition, and what medication you require. Medical alert bracelets or necklaces are pieces of jewellery engraved with your medical condition and an emergency contact number. They are available from a number of retailers. Ask your GP if theres one they recommend, or go to the MedicAlert website. If you need to stay in hospital, the healthcare professionals responsible for your care will also need to know you need steroid replacement medication throughout your stay. Its important to note that this advice still applies if youre not supposed to eat or drink (nil by mouth) for any reason. Adjusting your medication At certain times, your medication may need to be adjusted to take into account any additional strain on your body. For example, you may need to increase the dosage of your medication if you experience any of the following: an illness or infection  particularly if you have a high temperature of 37.5C (99.5F) an accident, such as a car crash an operation, dental or medical procedure (such as a tooth filling or endoscopy) youre taking part in strenuous exercisenot usually part of your daily life This will help your body cope with the additional stress. Your endocrinologist will monitor your dosage and advise about any changes. Over time, as you get used to the condition and learn what can trigger your symptoms, you may learn how to adjust your medication yourself. However, always consult your GP or specialist if youre unsure. Emergency treatment You and a partner or family member may be trained to administer an injection of hydrocortisone in an emergency. This could be necessary if you go into shockafter an injury, or if you experience vomiting or diarrhoea and are unable to keep down oral medication. This may occur if youre pregnant and have morning sickness. Yourendocrinologist will discuss with you when an injection might be necessary. If you need to administer emergency hydrocortisone, always phone your GP immediately afterwards. Check what out-of-hours services are available in your local area, in case the emergency is outside normal working hours. You can alsoregister yourself with your local ambulance service , so they have a record of your requirement for a steroid injection or tablets, if you need their assistance. Treating adrenal crisis Adrenal crisis, or Addisonian crisis, needs urgent medical attention. Phone 999 for an ambulance if you or someone you know are experiencing adrenal crisis. Signs of an adrenal crisis include: severe dehydration pale, cold, clammy skin sweating rapid, shallow breathing dizziness severe vomiting and diarrhoea severe muscle weakness headache severe drowsiness or loss of consciousness In hospital, youll be given lots of fluid through a vein in your arm to rehydrate you. This will contain a mixture of salts and sugars (sodium, glucose and dextrose) to replace those that your body is lacking. Youll also be injected with hydrocortisone to replace the missing cortisol hormone. Any underlying causes of the adrenal crisis, such as an infection, will also be treated.