The Challenge: Can you figure out what is wrong with a 43-year-old woman who suddenly develops abdominal pain so powerful that it wakes her up from sleep?
Every month the Diagnosis column of The New York Times Magazine asks Well readers to sift through a difficult real-life medical case and solve a diagnostic riddle. This month’s case concerns a previously healthy middle-age woman who goes to doctor after doctor with abdominal pain so terrible that she can barely eat or sleep.
I will give you the story and the records from the doctors who took on this challenging case. Can you figure out what is causing this woman’s pain? As usual, the first person to crack the case gets a copy of my book, “Every Patient Tells a Story,” and the satisfaction of solving a case that puzzled many doctors.
The Patient’s Story
“You can’t wait,” the man said to his 43-year-old wife. She lay on her back with her legs drawn up, crying quietly. We’ve got to get help, he told her. He rubbed her shoulders gently, feeling the tension from the terrible pain that had been her near-constant companion these last few weeks.
The pain had come on around Thanksgiving. At first it was a vague discomfort. Something wasn’t right in her stomach, but it was hard to locate and not so bad. But a few weeks later, it felt as if all the pain had concentrated itself in a single spot, maybe the size of a half dollar, just below her rib cage, a little to the right of the middle. She could press on the spot with three fingers, and that sometimes made it feel a little better.
At first the pain came and went, but then it came and stayed. It was sometimes worse after she ate, but not always.
Thinner and Weaker
By mid-December the woman was exhausted. They usually traveled to Florida for the winter break to spend time with the grandparents and celebrate Hanukkah. Her three kids were expecting their usual holiday and her husband was doing his best to make it happen, but it was tough. She wanted to help more but just couldn’t. Most days, she could barely get out of bed.
She hadn’t been eating much because she knew that if she ate there was a good chance the pain would get worse. Eating made her nauseated and sometimes she’d vomit. But the really bad pain came a couple of hours later.
So she’d lost weight. She’d always been small – just under 5 feet tall, she usually weighed only 90 pounds. Now she was down to the low 80s.
And she hadn’t slept a whole night through for weeks. Many nights, and she could never predict which, the pain would rip her from sleep and for hours she would be unable to move, barely able to breathe. And even on those nights when there was no pain, it was hard to sleep. She’d lie in bed and worry — was this going to be one of those terrible nights?
A Slew of Doctors, a Slew of Tests
Just before Hanukkah, she went to a gastroenterologist she’d seen a couple of years earlier. He scoped her stomach, looking for an ulcer. He didn’t find it. Then he got a CT scan. Nothing there either. A round of blood tests was also unremarkable.
A few days later, she called him. Her pain was out of control. He sent her to the emergency room of their local hospital in St. Louis. She was admitted, and the doctors there got another CT scan, of her chest and her abdomen. They drew many blood tests and got an ultrasound of her belly. All the test results were normal. They had her drink some barium and took pictures as the visible liquid made its way into her stomach, through her intestines then out. Normal.
The only clue to a possible cause was the fact that one medication that they had given her, called Levbid, seemed to help — at least a bit. This medicine is used to help the smooth muscles of the GI system relax and is sometimes used to treat irritable bowel syndrome or esophageal spasm.
The hospital doc thought it might be her esophagus that was causing the trouble and gave her a powerful antacid. Reducing the acid would give the esophagus time to heal, and once healed the spasms would stop. Give it time, he told her.
You can view the discharge summary for that hospital stay here.
Too Sick to Wait
But time was the one thing the patient worried she didn’t have. At home, the pain was unchanged. By the end of December her weight had dropped to 78 pounds. The very thought of eating scared her. Her gastroenterologist had told her to come back in a month. That’s how long he thought it would take for the medicines — the antacid and muscle relaxer — to really work. But after a week, the patient was no better. Her husband had clung to the promise of recovery if only his wife could give the medicines time to work. But after a week he too was in despair. They could wait no longer.
He quickly set up an appointment for his wife with a gastroenterologist at the well-respected Washington University. That doctor, a young woman, just a few years out of training, listened to the patient’s story. She reviewed the results of the studies done. She examined the patient carefully.
Given the symptoms the patient described and the lack of findings on the work-up so far, this doctor was concerned that the patient might have an obstructive disorder known as superior mesenteric syndrome. In this disorder the two major blood vessels of the upper abdomen, the superior mesenteric artery and the aorta, compress the upper part of the small intestine, limiting the passage of food. This rare condition is often seen after weight loss — it’s not clear how much, but enough so that the fat pad that once protected the intestine diminishes.
She sent the patient for a special type of M.R.I. to look for this type of obstruction. It was normal, she told them over the phone. She wasn’t sure what else it could be.
You can see the notes from this doctor and the M.R.I. results here.
Now frantic with worry, the patient’s husband called the Mayo Clinic in Rochester, Minn. It was Friday afternoon. After a brief conversation the appointment was set. Could they be there first thing on Monday? They could.
A Trip to Minnesota
The doctor at the Mayo Clinic was warm in demeanor and tidy in appearance. He quickly reviewed the records they brought and then just wanted to hear their experience.
He listened patiently, interrupting only occasionally to ask a question or two. In looking at the patient he could see how much she’d been affected by this pain. She was terribly thin. And the way her clothes fit suggested that she’d lost the weight recently.
When he examined the patient, she pointed to the spot where she felt her recurring pain. She could locate it easily — a few inches above her belly button and slightly to the right. The doctor gently placed his stethoscope over the spot, listening for what’s called a bruit, the noise blood makes when its flow is turbulent. There is an unusual disorder known as median arcuate ligament syndrome, in which one of the main arteries leading to the intestines is compressed against the diaphragm by this ligament, which connects the left and right sides of the diaphragm. The compression intermittently reduces blood flow, and that could make this kind of noise. But he heard nothing.
The patient’s husband pointed to a couple of other spots where he’d felt lumps or bumps. The doctor felt the spots. The first was her aorta, the vessel that carries blood from the heart to the rest of the body. The second, a little higher up and off to the side, was her backbone. That’s how thin she was.
Another Round of Testing
After the exam the doctor shared his thinking. A rare type of growth called a gastrointestinal stromal tumor, or GIST, was a possibility. A carcinoid tumor — also rare — could present with this type of intermittent pain. A more common possibility was Crohn’s disease. Though Crohn’s usually caused chronic diarrhea, it could also present with pain alone. To look for these conditions he would order a special type of CT scan, one that could give them a good look at the structure of the bowel wall, especially in the small intestines.
He also wanted to check the levels of certain vitamins — particularly those absorbed in the small intestine. She had been given a diagnosis of a vitamin B12 deficiency some years ago and he wanted to see if she had some difficulty absorbing other vitamins as well.
Normal Tests, but Still in Pain
When those studies were all normal, the doctor had to rethink her case. In two CT scans she’d had a lot of stool in the colon. Could this be a motility problem?
The doctor ordered a gastric transit study to see if there was some problem with her gut’s ability to move food forward through the system. And finally something was abnormal. The food slowed down just a little as it passed through her intestines. She had something known as gastroparesis. Most of the time the cause is never found. And often, it never goes away.
You can see the notes from Mayo and the gastric emptying study here.
No Great Treatments
There are no great treatments for gastroparesis. Patients with this disorder are told to eat frequent small meals and take medicines to ease nausea. Meals should contain little fat and only soluble fiber. If that doesn’t work, patients are put on a liquid diet or given medicines that try to make the intestines move faster. If none of that works, patients are fed through a tube that delivers nutrients directly to the small intestines.
The patient tried all of these treatments. Nothing really worked. She took a medicine to relieve the nausea. That was helpful. But nothing seemed to ease the pain that started an hour or two after eating, no matter what she ate.
Solving the Mystery
The patient and her husband returned to Mayo twice more to try to find the best treatment. They resigned themselves to the fact that this was how their life was going to go, for as long as they could imagine. “I figured that was my next 50 years, seeing my wife suffer almost every day,” her husband told me.
But it wasn’t. The patient got a diagnosis and, by treating that disorder, was able to get rid of her gastroparesis.
Can you figure out what this woman had?
Post your answers in the comments section. As usual, the first person to make the right diagnosis gets a copy of my book. I’ll post the answer tomorrow.
Rules and Regulations: The winner will be contacted. Reader comments may also appear in a coming issue of The New York Times Magazine.