Editor's note: While going through some old Notes on facebook, I discovered this...odd piece. Apparently, at the end of my second year of medical school, while I should have been studying for Step 1, I took up my husband's dare to create a scientific-sounding explanation for the disease "supercalifragilisticexpialidociuos," as described in the SNL sketch below. (You already know the joke about Gandhi, right?) Well, here is my report, with a minimum of editing. Don't forget: a spoonful of sugar helps the medicine go down!
|Ann Hathaway as Mary Poppins and Bill Hader as Bert on SNL|
Dear Husband asked whether I could derive the pathophysiology of this disease, most commonly associated in the popular imagination with the English governess Mary Poppins, from the word itself. I have. But first, please watch the following documentary film for some background. (It will open in a new window--then come back here to read about it!)
Supercalifragilisticexpialidocious (supercali-fragilis-tic-expiali-docious) is a very and painful infectious hepatic disease in which bile production fails. This reduces the ability of the hepatoduodenal pathway to excrete hydrophobic waste products and to digest lipids. Eventually non-alcoholic steatosis of the liver commences, which manifests as hepatomegaly and right upper quadrant pain often mistaken for "a stomach ache." While the responsible microorganism has yet to be identified, existing studies suggest the involvement of a prion. There is no treatment at this current time, and the condition is inevitably fatal in 3-6 months. It's really quite atrocious.
The pathophysiology of supercalifragilisticexpialidocious is contained in its portmanteau moniker: supercali-fragilis-tic-expiali-docious.
- Super (Latin) — prefix : over, above, in addition, from super over, above, on top of
- Cali, from Calcium (New Latin from calc-, calx lime; 1808) — adjective : of or referring to the element calcium (Ca2+, atomic number 20).
Liver failure not only disrupts the production of bile salts, bile acids, and bilirubinous salty acids, it also decreases albumin production. Albumin is the major transport protein in the blood plasma serum, such that hypoalbuminemia reduces the number of binding sites for calcium. "Supercali" therefore refers to the resulting elevated levels of free calcium, which contribute to urolithiasis, osteitis fibrosa cystica, tetany, perioral numbness, hypercoagulopathy, cardiac arrhythmia, and seizures (i.e. "stones, bones, groans, moans"). Due to the decreased albumin binding sites for warfarin, digoxin, and diazepam, the use of these pharmacological agents to treat preexisting conditions like hypercoagulopathy, cardiac arrhythmia, and status epilepticus obviously will have to be modified to take into account the drugs' increased volume of distribution and decreased clearance by phase I and II modification.
- Fragilis (Latin) — adjective : 1) easily broken or destroyed; 2) constitutionally delicate
The hepatobiliary system's malfunction results in acholic stool and fat-soluble vitamin malabsorption. Depletion of Vitamin D does not help the supercali state, while loss of Vitamin C impairs cross-linking of hydroxylated lysine and proline residues on collagen triple helices. This produces a scurvacious state of easy bleeding and bruising. Hence the "fragilis" of the extracellular matrix, ground substance, and capillary basement membranes.
- Tic (French; circa 1834) — noun : local and habitual spasmodic motion of particular muscles especially of the wrists when the hands are extended on straight arms in the "stop traffic" position
Liver failure disrupts the urea cycle, producing hyperammonemia, asterixis (the "tic" in the title), altered mental status, and eventually coma. Although asterixis is a clinic sign commonly taught to second-year medical students, its presence, absence, or severity does not actually correlate with the levels of ammonia in the plasma blood serum or with disease severity, so an actual laboratory test is needed.
- Expiali, from Expectorate (Latin expectoratus, past participle of expectorare — to banish from the mind — taken to mean literally “to expel from the chest,” from ex- + pector-, pectus breast, soul; 1601) — transitive verb : to eject from the throat or lungs by coughing or hawking and spitting
A cough productive of red- and/or green-tinged sputum is seen in many cases of Supercalifragilisticexpialidocious. Its etiology is uncertain, but autodigestion of the liver by dammed up caustic waste products may perforate the diaphragm, leading to spillage of blood and bile into the lungs. An AP chest x-ray is recommended for all patients.
- Docious, from Dioecious (ultimately from Greek di- + oikos; 1752) — adjective : 1) having male reproductive organs in one individual and female in another; 2) having staminate and pistillate flowers borne on different individuals
Finally, the root of this neologism reveals the method of transmission: "among grown ups." The first known communicable disease solely of heterosexuals, Supercalifragilisticexpialidocious is transmitted from one sexual partner to the other only when the reproductive organs of the individuals involved are "opposite." All sexually active heterosexual adults should be counseled about their risk of contracting this dread disease.