What is Addison's? - Addison's Disease Self Help Group

What isADSHG EducationALMedicine/Science/social HistoryAddison’sAddison’s is a medical condition named after DrThomas Addison, the doctor who first identified it.Thomas Addison worked in London in the 1840s and1850s at an inner-city hospital called Guy’s Hospital. Thiswas the time when Queen Victoria was on the throne andmodern science and technology were only just beginning.Electric lights and flush toilets had not yet been inventedand people rode around in horse-drawn carriages…

1. How Addison’s got its nameAddison’s is a medical condition named after Dr ThomasAddison, the doctor who first identified it. Thomas Addisonworked in London in the 1840s and 1850s at an inner-cityhospital called Guy’s Hospital. This was the time whenQueen Victoria was on the throne and modern science andtechnology were only just beginning. Electric lights and flushtoilets had not yet been invented and people rode around inhorse-drawn carriages.Addison and the adrenalsMost of Thomas Addison’s patientswere poor people and a lot of them gotsick with a kind of chest infection calledtuberculosis (TB). This used to kill manypeople, including the well-known writer,Charlotte Bronte. Tuberculosis wasdeadly because modern medicines, likeantibiotics, had not been invented, sodoctors could not do a lot to treat peoplein those days. Thomas Addison observedthat some of his patients had othersymptoms such as darker skin and a lotof vomiting. They went on to become very thin and so weakthey could hardly get out of bed.Then after a few years they would die. By studying thebodies, Dr Addison realised that this was becausetheir adrenal glands had stopped working and hadshrivelled up. In those days, nobody knew what theadrenal glands actually did, but his work showed thatthey were essential for life.Charlotte Bronte, who died of tuberculosis in 1855, may have had Addison’sDr Addison’s scientific discoveriesIn 1855, Dr Addison published the first scientificpaper about this newly-discovered condition,called ‘Disease of the Supra-renal Capsules’. Hegave the adrenal glands this name becausethey are found just above the kidneys,sitting on top of them like two little hats. Healso observed that some of these patientsdeveloped an additional condition calledanaemia or a skin condition with patches of very pale skin,known as vitiligo. Thomas Addison was a dedicated doctorand we still remember him today.2. Understanding what the adrenal glands doDoctors and scientists kept trying to understand what theadrenal glands do and why they are so important. By thetime Queen Victoria died in 1902 and her son, Prince Edward,became King of England, they had identified that the adrenalsare hormone factories that squirt out special juices intothe blood.

So you can think of the adrenal glands like two gherkin hatssitting on top of the kidneys, squirting out special picklejuices – hormones – that help to keep our bodies going.The adrenals don’t just squirt out one kind of juice.Even though they are tiny glands, the adrenals haveseveral different layers, each producing a differentkind of hormone that helps to keep the body going.Diagramshowing thedifferent layersin an adrenalglandIt took doctors and scientists a long time to identifythe different hormones and what they do. Overtime, they realized that there are other glandsaround the body, like the pituitary, that also actas hormone factories. The study of these glandswas named endocrinology.3. The early discoveriesabout adrenal hormonesBy 1905, doctors and chemists in Poland,Japan and Germany had managed to createextracts in the laboratory for one of the main adrenalhormones, called adrenaline. To their disappointment,when doctors gave adrenaline extracts to patients withAddison’s, this made them feel better but did not keepthem alive for much longer. We now know that adrenalineis produced by the inner layer of the adrenals, called themedulla. Adrenaline injections proved to be a good wayof treating allergic reactions and adrenaline is now themain ingredient in Epi-Pens, which are used to treat severeallergies, for example to wasp stings.In the 1930s,doctors in the UnitedStates, Canada and theUK were able to create extractsof other important adrenal hormones,called corticosteroids. These hormones areproduced by the outer layers of the adrenal gland,called the cortex. At first they made these extracts by buyinganimal adrenal glands from meat works and grinding themup. Doctors were excited to discover that when they gavedaily injections of these cortex extracts to patientswith Addison’s, it kept them alive. This was the firstsuccessful, modern treatment for Addison’s. Onefamous patient whose life was saved in this way wasan American painter called Andrew Dasburg. He wasdiagnosed in 1937, when he was 49, and lived on tothe age of 92.Andrew Dasburg, a leading American artist and earlypatient whose life was saved by steroid treatment forhis Addison’s, photographed in 1930

The Nobelprize medal4. The search for medical treatmentGrinding up animal extracts from the meat works to makeinjections was slow and expensive and not many patientscould be treated in this way. So doctors and chemists kepttrying to find a better treatment. A team at the Mayo Clinicin the United States, led by Professor Edward Kendall, werethe world leaders in this research.During the 1940s, they worked with a major drug company,Merck, to try and produce a simpler, cheaper medicinethat could save the lives of people with Addison’s. It was in1949, a few years after the end of World War 2, that theyhad their big breakthrough. Merck was able to massproducea chemical version of the natural adrenalhormone, called cortisone. Professor Edward Kendallwon a Nobel prize in 1950 for his research on thedevelopment of cortisone.President Kennedy and Addison’sOne of the earliest patients to receive the newwonder drug, cortisone, was a young politician calledJohn F Kennedy. He had been awarded a medal forbravery in World War 2, for saving the lives of his crew whentheir patrol boat was rammed and sank. He was diagnosedwith Addison’s in 1949 and went on to be elected Presidentof the United States in 1960. Sadly, he was assassinated in1963, when he was just 46 years old. President Kennedykept his condition a secret because he worried that peoplemight say it made him too unhealthy to lead the country. Infact, he stayed well and only had one day off work during histhree years as President.5. What is life like todayfor people with Addison’s?Since then, there have been further advances in the kinds ofdrugs that are available to treat Addison’s. Most people withthe condition now take a medication called hydrocortisone.This is a fast-acting drug that has to be taken two or threetimes a day. Most people with Addison’s also take anothermedication called fludrocortisone. It is sensible for them tosee a hospital specialist, called an endocrinologist, onceor twice a year for a check-up to make sure they have theirmedication balance right.Doing a marathonProviding they keep taking their tabletsevery day, at the right time of day, peoplewith Addison’s can stay healthy. A fewpeople even compete in endurancesports like cycling or running. In April2010, three people with Addison’s, allin their 40s, successfully completedmarathons to raise funds for thePresidentKennedy, whowas diagnosedwith Addison’sat the age of 32,some 11 yearsbefore he waselected leader ofthe United States.Debra Chase completing the Londonmarathon in 2010

Singer Helen Reddy,now retired, in 2009The Addison’semergency cardAddison’s Disease Self-HelpGroup. Debra Chase, Greg Davisand Peter Raynard each ran 26miles and were awarded medalsfor taking part in the London andBrighton marathons.A famous singerHelen Reddy was a famouspop singer in the 1970s whenshe became very sick and wasdiagnosed with Addison’s. Sherecovered quickly from her illness toresume her show business career andcontinue touring the world. She finallyretired in her 60s to return to her nativeAustralia, where she is a guest lecturer andqualified hypnotherapist.6. How many peoplehave Addison’s?Addison’s is a rare condition, thought toaffect less than 8,500 people in the UK,or about 140 people in every million. Thismakes it roughly three hundred timesmore rare than diabetes, another medicalcondition that sometimes occurs alongsideAddison’s. Nowadays, very few cases aretriggered by tuberculosis and it is often notclear what causes people to develop thecondition.7. Whatcan go wrong?If people with Addison’s don’t take their medication, or don’ttake enough extra medication when they get an infectionlike a flu virus, they can quickly become very unwell. Theywill become weak and dizzy and vomit a lot; this is known asan adrenal crisis. Then they need an emergency injectionof extra hydrocortisone and may need to go into hospitalstraight away for medical care. So people with Addison’smust always carry spare medication with them and, ideally,a medical card and their injection kit. They should wearidentification like a MedicAlert bracelet.People with Addison’s often get other health complications,as the good Dr Addison identified so long ago. Sometimesthey have to take medication foranaemia (iron supplements) or forvitamin B12 deficiency. Sometimesthey can develop further conditionslike diabetes, thyroid deficiency orhyperthyroidism. And sometimesthey need to follow a special dietthat avoids gluten (found in bread andwheat-based foods) because they havean allergy to this.Pat Beeching,aged 74, afterliving withAddison’s for58 years

Keep taking the tablets!Provided they are sensible about taking their tabletsevery day, at the right time of day, people withAddison’s can have long and active lives. PatBeeching was one of the earliest patients in the UKto start on the new wonder-drug, cortisone, whenshe was diagnosed in 1952 at the age of 16. Now agrandmother, she has lived with her Addison’s for58 years and is still going strong.8. Medical research and the futureThere has not been a lot of medical research intoAddison’s in recent times, because it is a rarecondition and the existing treatments are working wellenough. Around the world, there are just a few adrenalspecialists looking at the causes of Addison’s and ifthere might be better ways of treating the condition.One of the possibilities they are researching is a newtype of hydrocortisone medication that would only needto be taken once a day instead of three times a day.Some of these specialists also support the Addison’sDisease Self-Help Group by writing medical informationfor the group. You can find out more about Addison’sby reading the information on the group’s website atwww.addisons.org.uk.ADDISON’SS e lf H e l pG r o u pDefinitions(For older students)AdrenalineThis is one of the hormones producedby the adrenals. It stimulatesthe heart to beat fasterand raises blood pressure. It issometimes known as the ’fight orflight’ hormone because the bodyproduces it in large amounts inresponse to scary situations. Itis produced in the inner core ofthe adrenal gland, known as themedulla. Sometimes adrenalineis also called epinephrine.Adrenaline is not essential forlife; it can be compensated for bya similar hormone noradrenaline,which is produced in nerve endingsaround the body.AldosteroneThis is one of two adrenal hormonesthat is essential for life. Ithelps to control blood pressure,sodium and potassium levels andthe amount of fluid in body tissues.Known as a corticosteroid,it is produced in the outer layerof the adrenal gland, the cortex,along with cortisol.AnaemiaThis is a form of iron deficiencywhere the body gradually losesthe ability to absorb enough ironfrom the food that is eaten. InAddison’s, this is often causedby damage to the stomach lining(atrophic gastritis), so thatit loses the ability to secretecertain enzymes necessary forthe absorption of iron and vitaminB12. This makes the person feelweak, tired and breathless. It istreated with iron supplements.CortexThe outer layer of the adrenalglands, it produces the corticosteroidhormones which are essentialfor life. They help to controlblood sugar, blood pressure, saltand water balances in the body.CorticosteroidsThese are the adrenal hormoneswhich are essential for life. Theyare produced by the outer layerof the adrenal gland, known asthe cortex. There are two maincorticosteroids: cortisol andaldosterone.CortisolThis is one of two adrenalhormones that is essential forlife. It is sometimes called aglucocorticoid hormone, becauseit helps to control blood sugarlevels as well as blood pressure,salt and other minerals in thebloodstream. Like aldosterone, itis produced in the outer layer ofthe adrenal gland, the cortex.CortisoneThis was the name given to thefirst man-made chemical versionof the natural adrenal corticosteroidhormones. This is short for itsfull chemical name of cortisoneacetate. Cortisone is now understoodto be a weaker (inactive)form of the more important adrenalhormone, cortisol. To becomeactive in the blood stream, itmust first be converted by theliver into cortisol, so it is not asfast acting as hydrocortisone.

DiabetesThis is a condition that occurswhen another endocrine gland,the pancreas, stops workingproperly. It happens when thepancreas can’t produce enoughof another essential hormone, insulin,so that the person’s bloodsugar climbs too high, particularlywhen they eat something. Peoplewith diabetes can sometimescontrol their blood sugar by beingcareful what they eat or by takingcertain medicines. But often theyneed to inject themselves with insulinto keep their blood sugar inbalance. Diabetes is 300 timesmore common than Addison’s.Some people with Addison’s alsoget diabetes.EndocrinologistThis is a specialist doctor whohas studied the endocrine glandsand the kinds of medical conditionsthat can occur when theydon’t work properly.EndocrinologyThis is the study of hormonesand the body’s endocrine glands.It is a specialized branch of medicine.The main endocrine glandsare the pituitary, hypothalamus,thyroid, parathyroid, pancreas,ovaries or testes and adrenals.FludrocortisoneThis is a synthetic chemicalcompound that is taken toreplace the natural adrenalhormone aldosterone. It is notquite identical to aldosterone,but has the advantage that it islonger-acting. Most people withAddison’s take it once or twice aday: on waking, with a second,smaller dose sometimes taken atlunchtime or bed-time. Becausefludrocortisone is not chemicallyidentical to the natural hormonealdosterone, it would show up ina sports drug test.GlutenThis is a type of protein found inwheat, barley and other grains.Some people become allergic togluten and can’t eat foods suchas bread, pasta, or cakes madewith wheat flour without becomingill. People with Addison’sare more likely to react badly togluten. This allergy is known ascoeliac disease.HormonesThese are special secretionsthat various glands in the bodyproduce. They are pumped intothe bloodstream where they actas chemical messengers, helpingto keep various body systemswell-balanced. Hormones controlthe body’s growth, immunesystem, appetite, blood pressure,reproduction and many otherimportant functions.HydrocortisoneThis is a more modern version ofcortisone and was first developedin the 1960s. Chemically, it isidentical to the natural adrenalhormone cortisol. This is now themain glucocorticoid medicationfor people with Addison’s. It isfast acting and is usually absorbedfrom the stomach within30 minutes of being swallowed.This means it has to be takento or three times a day: the firstdose as soon as the personwakes up; the next, smaller dosefive or six hours later. Becausehydrocortisone medication isidentical to the natural hormonecortisol, it would not show up in asports drug test.MedullaThe inner core of the adrenalglands, it produces adrenaline,sometimes referred to as the‘fight or flight’ hormone.MedicAlertThis is the main form of medicalinformation jewellery which isrecommended for people with serioushealth conditions. It allowsambulance crews and doctors torecognise the kind of treatmentpeople with serious medical conditionsare likely to need inan emergency.PituitaryThis is an endocrine gland foundat the base of the brain, insidethe skull. It is sometimes knownas the master gland because itpumps out hormones that controlthe function of many other endocrineglands around the body,including the production of cortisolin the adrenals and thyroidhormones in the thyroid.TuberculosisA bacterial infection that usuallyattacks the lungs ; it can alsoinfect other parts of the body.When it damages the lungs itcauses a persistent cough, fever,night sweats and weight loss.Treatment is difficult and requireslong courses of antibiotics. Leftuntreated, tuberculosis canresult in death.ADSHG EducationALMedicine/Science/social HistorydefinitionsThyroidThis is another endocrine glandthat produces hormones whichare essential for life. Thyroidhormones control body temperatureand energy. People withAddison’s are more likely to losethyroid function and many needthyroid supplements as well asreplacement adrenal medications.Vitamin B12 deficiencySometimes known as perniciousanaemia, this is a condition thatoccurs when damage to thestomach lining (atrophic gastritis)means the body cannot absorbenough vitamin B12. It usuallyoccurs alongside iron deficiency,making the person feel weak andtired, sometimes with tingling ornumbness in the hands and feet.It is treated with vitamin B12injections or supplements.VitiligoThis is a harmless skincondition where patches ofskin gradually lose their pigmentationso that they appear whiteand get easily sunburnt. It ismore noticeable in dark-skinnedindividuals. Some people withAddison’s also get vitiligo.

What isAddison’s?www.addisons.org.ukAddison’s Disease Self-Help Group, PO Box 1083, Guildford, GU1 9HX Email: info@addisons.org.ukAuthor: Katherine White Medical reviewers: Professors Simon Pearce and John Wass Design and layout: the workshopPictures: Portrait of Charlotte Bronte, © National Portrait Gallery, London; photograph of Andrew Dasburg circa 1930, © Archives of American Art, Smithsonian Institute.© ADSHG 2010