Well, No Body’s Perfect

On a lazy Friday afternoon last year my doctor informed me that I was missing a major internal organ.

“Everything is normal except for the absent left kidney,” he said, referring to a recent ultrasound examination.

I was stunned -- then downright disbelieving. It seemed ridiculous that I could have lived 43 years without knowing this.

The doctor quickly allayed some fears. Although the remaining kidney was extra vulnerable and should be protected from injury -- rugby, never a serious consideration, was now out -- in other respects life could go on as usual. There was every expectation of a normal lifespan. The body can get by with half a kidney or less.

“If you’re going to be missing a body organ, this is the one to miss,” another doctor said.

As it turns out, a surprising number of us do lack a kidney -- roughly 1 in 1,000, according to autopsies, medical dissections and one exhaustive 1989 study of 132,686 Taiwanese schoolchildren. And that is just the beginning. Many thousands are walking around with other oddball configurations: extra vertebrae, duplicated or oddly placed blood vessels, absent or multiple muscles, little supernumerary spleens, bladders shaped like hourglasses, even 12-lobed livers.

Of course, many oddities tossed up by nature are serious, even deadly, birth defects. But other differences are benign or just inconvenient, sometimes undetected for years and fortuitously found by radiologist, surgeon, medical student or coroner. Often, they are never discovered -- secrets that crumble to dust with their owners.

It is a lesson all good surgeons take to heart: None of us are possessed of Gray’s Anatomy perfection -- we are all variations on a theme. But it is still hard to accept when you are the anomaly.

“We are all amazed when we discover that no two humans are exactly the same. Sometimes we have extra parts and sometimes we were shortchanged,” said Ronald Bergman, professor emeritus of anatomy at the University of Iowa, who has been diligently cataloging human anatomical variations for decades. “No two faces are alike -- it’s the same inside.”

A quick survey of friends and co-workers revealed that my lone kidney was only the tip of the iceberg. People admitted to (among other things) an extra rib sticking out from a neck bone, a double ankle bone, an extra finger, extra and fused vertebrae, missing toe bones, absent or extra sinus passages and extra ureters. Of special interest was a miniature third kidney “piggybacking on an existing kidney,” a trait that was presumably inherited, because a father and niece have the same oddity.

It was reassuring to discover I was surrounded by nonstandard humans, but it only made me more determined to find out how I had gotten this way.

In the 19th and early 20th centuries, anatomists and medical museums would eagerly amass such curious specimens. But the fascination with human anatomy was eventually overshadowed by the marvels of modern molecular biology.

Even Philadelphia’s Mutter Museum, keeper of such items as the “megacolon” of a young man with a congenital bowel disease and the shared liver of conjoined twins Chang and Eng, has sent many specimens to the trash can.

The double bladder: gone. The kidney with the giant drainage duct: long gone.

“They even threw out the two-headed chicken,” sighed museum director Gretchen Worden. “What were they thinking?”

There are plenty of potential specimens out there should she wish to restock.

One of them is 29-year-old David Knowles of Springfield, Ill., who discovered his abnormality last year by chance. He had been suffering from mild discomfort in his right side and went for an ultrasound.

“Everything’s fine except there’s something about your left kidney,” his doctor’s office told him. It didn’t exist.

“I was shocked,” Knowles said. “This is a major organ.”

Knowles later learned of a cousin with the same oddity, and now accepts that in the grand scheme of things, missing a kidney isn’t so bad.

He gave up his motorcycle on a doctor’s advice but is glad, in a way, of his ignorance all these years. In high school he played football, which is discouraged, sometimes prohibited, for people with single kidneys.

Ruth Santiago of Long Beach is relieved that doctors discovered her single-kidney status when they did.

In 1964 she suffered excruciating pain, and doctors discovered that her right kidney was enlarged. They suspected it was cancerous and were considering taking it out.

Santiago’s husband wrapped her in a blanket and took her for a second opinion. She soon learned that her enlarged kidney was her only kidney (a small nubbin is all that exists on the left) and that the pain had most likely been a kidney stone.

“Imagine if they had taken it out without checking the other end,” said Santiago, 76. “I would have been up the creek without any kind of paddle.”

Since then, she has spent her life with few restrictions (driving tractor-trailers, flying twin-engine planes), while tending to her kidney’s well-being (drinking lots of water and avoiding jalapenos and alcohol).

As they told her at the clinic: Some people have three kidneys; you’re going to live your life with one. Go and be happy.

My own lone kidney might also be a nice fit for the museum, perhaps somewhere near the megacolon. A solitary kidney often grows to compensate for the lack of a partner. While most human kidneys are nine to 13 centimeters in length (13 for especially chunky folks), my right kidney is a hulking 14.

The size of my right kidney was deemed especially hilarious by friends, who happily traded kidney jokes once they knew death was not imminent. Still, one nagging thought remained -- that the condition could be passed on to my daughter.

I contacted my parents and siblings to find out how many kidneys they have, and discovered that most of them have no idea.

As inner organs go, kidneys are particularly variable, said Dr. Carmine Clemente, a white-haired UCLA anatomy professor and editor of the U.S. edition of Gray’s Anatomy, the field’s bible.

Beyond kidneys, the body has tiers of variation, Clemente said. Superficial veins are super-variable, barely noteworthy. Arteries vary, but less so than veins. Next come nerves and maybe muscles, followed by skeletons.

A one-kidneyed person is on the rare side and would certainly be of interest, he said, but not even close to the cadaver he encountered in 1993.

A student approached Clemente one day and told him something was wrong with the body she was dissecting. “The heart isn’t in the right place,” the student said.

Clemente took a look. The heart was in the right chest, not the left. The lungs had three left lobes and two right ones, instead of the other way round. Everything in the chest was flipped.

As the students crowded close, Clemente pressed the body with his hands, feeling for organs that had not yet been exposed by the knife. The large lobe of the liver, normally on the right, was absent. He felt on the left side -- and there, where it shouldn’t be, was the liver.

“When we finally opened the abdomen, all of the abdominal organs were inverted,” Clemente said.

It was a perfect case of situs inversus, a 1-in-10,000 anomaly, the first case he’d seen in five decades of teaching. “It really was quite a remarkable cadaver,” Clemente reminisced.

Such mistakes seem understandable considering the millions of details involved in growing a single organ. Take, for example, the kidney, which is so complex and convoluted that it is astonishing that one manages to pull itself together. Millions of ducts, branches, tubes and blood vessels have to form and then link up correctly, guided by rafts of chemical messengers that tell cells which genes to use, what shapes to become, when to divide and when to self-destruct. The finished product must be able to crisply filter toxins from blood and adjust salts and fluid.

Our adult kidneys are really our third set of kidneys, said Robert Trelease, UCLA associate professor of pathology and laboratory medicine. The first two come and go quickly. The permanent ones start growing in the fifth week of embryo development, down near the embryo’s tail, and start filtering blood long before we are born. Amniotic fluid, which we need for our lungs to develop, consists largely of fetal urine.

Only by week nine do the kidneys take up their final position high in the abdomen -- except sometimes they don’t rise, or rise too far. Roughly 1 in 3,000 people has a kidney skulking in the pelvis, and 1 in 13,000 has a kidney in the chest.

Sometimes the two organs fuse. Actor Mel Gibson’s body houses one chiseled kidney shaped like a horseshoe (a 1-in-500 peculiarity) instead of two regular, bean-shaped organs.

There are myriad ways for kidney development to go wrong, said Trelease as he thumbed through a textbook chapter brimming with kidney oddities. “That could have been you,” he noted, pointing to a case labeled “unilateral renal agenesis,” the technical term for missing a kidney.

Studying kidneys is hot these days. Developmental biologists have spent years trying to figure out matters such as how embryos discern front from back and top from bottom. Many are moving on to the next issue: organ formation.

“I view it as the next frontier,” said Andrew McMahon, chair of the department of molecular and cellular biology at Harvard University.

Scores of genes have already been implicated in kidney formation, many of which also are involved in starkly different tasks, be it helping build the eye of a fly or directing the growth of a nerve in a brain. Many more genes -- guiding kidney creation from the first tiny bud to the last, twisted tubule -- remain to be uncovered. When kidneys don’t form, a genetic mistake can sometimes be the culprit, McMahon said. Some strains of mice and rats produce one-kidneyed offspring in exceedingly high numbers, as well as some offspring with no kidneys at all. The same thing is true for certain human families.

I might, McMahon offered, be abnormal for one of a growing list of genes needed for the very first steps in kidney formation.

But it is also possible that my kidney started to grow nicely and then fell off the straight and narrow because of some later mess-up, said Dr. Adrian Woolf, a kidney researcher and pediatric nephrologist at the Institute of Child Health in London. Ultrasounds of fetuses sometimes show kidneys that later cannot be found.

Some kidneys start growing in a malformed way, becoming abnormally large before disappearing without trace, “like a supernova,” Woolf said.

I had taken my quest for understanding as far as it could go -- and in many ways was left unsatisfied. I had become obsessed. Even the garden hose, splayed across the yard one afternoon, evoked images of the kidney’s miles of tubing.

For completeness’ sake, I was tempted to pursue a full-body scan or an injection of radioactive dye to light up my urinary tract like a Christmas tree -- two methods that could easily determine if my missing kidney was simply hiding somewhere in some unexpected spot.

Doctors said to just drop it. Unlike ultrasound, these forms of imaging are mildly invasive, and it is not a good idea to probe your insides unless you suspect a problem.

But the specter of genetics still lurked. Only my father knew for sure that he had two kidneys. A doctor refused to test my brother merely to tally body parts. No one else in the family seemed to care how many kidneys they had -- as long as it was more than none.

There was one thing left to do. One day I took my 13-year-old daughter -- lover of sports, scary rides and all things high and hazardous -- to an imaging center. We watched as an ultrasound scanned her depths.

Bewildering blizzards of gray morphed in and out of focus on the screen as the right kidney swirled into view: north pole, south pole, center.

Then the ultrasound moved to the left, plunging through spleen, swooping across liver.

Finally, out of the mists, up it rose: a second kidney, in all its blurry perfection.