One sunny day in January, 12-year-old Megan Hinton decided to pedal her bike up and down the street for a few minutes.
Her mother, Kerry Hinton, broke into a wide smile, then braced for the familiar wave of worry that crashes in on Megan’s normal childhood play.
“I’m looking at her thinking, ‘Oh, yay, she’s riding her bike,’” Kerry recalled. “But there’s this other part of me thinking, ‘What’s the price going to be?’ And sure enough, the next day she’s in the hospital.”
Megan has Postural Orthostatic Tachycardia Syndrome, or POTS. When she stands up, her heart races, her blood pressure rises, and she almost faints. Simple acts like taking a shower or riding a bike can sap her energy and leave her bedridden for days.
Central Oregon is becoming a West Coast hub for teens suffering from this chronic illness thanks to one man: Dr. Michael Feldman. A kidney expert at Bend Memorial Clinic, Feldman stepped outside his specialty when he started treating Megan one year ago. Now, he has a dozen severe POTS patients in Central Oregon and a handful of others from as far away as Alaska.
Still, resources for these patients remain scarce. And life with POTS is a roller coaster. A good day triggers three or four bad ones. Joy is joined at the hip by that unwelcome friend, worry.
In 2002, at age 5, Megan was a promising gymnast, cartwheeling across balance beams. Her parents called her their hummingbird because she never sat still.
A few months before Megan and her twin sister, Katie, turned 6, Megan started coming home from her weekly gymnastics lessons pale, shaky and nauseated. Eventually, she had to drop out of gymnastics.
Over the next few years, Megan continued to develop these symptoms, which followed less and less strenuous physical exertion.
In 2004, she was diagnosed with low blood sugar. She treated it by eating small, high-carbohydrate meals every two hours.
Still, her health continued to decline. In 2006, her parents took her to Doernbecher Children’s Hospital at Oregon Health & Science University, where a doctor suspected liver disease but eventually ruled it out through genetic testing. The family returned home with no name for her illness and no treatment.
Long road to diagnosis
In May 2007, Megan went for a short walk with her family and returned home exhausted. Days passed, then weeks, and Megan still didn’t recover. She slept for 12 hours, then woke up tired. Some days, she couldn’t lift her head off the pillow. Her appetite disappeared.
“I was crawling to the bathroom for, like, a month,” Megan said. “I couldn’t walk at all.”
Kerry started taking Megan’s medical records to different doctors, hoping one would take an interest in her case. Several offered theories — allergies, for instance — about what ailed Megan. Then they stopped returning the Hintons’ calls when tests proved them wrong or treatments failed to work.
Meanwhile, Kerry took Megan to the pediatrician every week. She carried her to the car, then used a stroller to push her into the doctor’s office.
“I thought we were losing her,” Kerry said. “She was just slipping away.”
During one of those desperate drives to the pediatrician, Kerry looked over and saw Megan’s heart beating through her chest. When they arrived at the doctor’s office, Kerry asked the nurse to take her daughter’s heart rate and blood pressure. Her heart was racing, and her blood pressure was low.
A few days later, Megan went to Dr. Urszula Tajchman, who is Bend’s only pediatric cardiologist. Tajchman diagnosed Megan with severe POTS. The disorder affects an estimated 500,000 to 1 million Americans, with roughly five female patients for every male patient. Only a quarter of POTS cases are so severe that the patients are disabled.
Tajchman, with The Pediatric Heart Center of Central Oregon, says she diagnoses one or two mild cases of POTS each week.
Although POTS symptoms are varied, they all stem from the distribution of blood throughout the body.
A normal child has about four quarts of blood. When he or she stands up, gravity pulls one quart into the lower body. To adjust, the brain triggers a process that speeds up the heart, increases the blood pressure and constricts upward-pumping vessels to make more blood available to the brain. In a healthy person, it takes just two heartbeats to redistribute the blood upon standing.
But a person with POTS cannot make those adjustments when going from sitting to standing, or, in severe cases, from lying to sitting. Being upright reduces the POTS patient’s blood flow to the brain.
Tajchman says she diagnoses POTS by taking a detailed patient history, and then checking the patient’s heart rate and blood pressure while lying down, sitting and standing. If fainting is a big problem, the patient may be strapped to a plank, called a tilt table, which is then rotated until the patient is upright.
If the patient’s heart rate goes up 30 beats per minute or more upon standing, then he or she likely has POTS, Tajchman said. Additional testing is often required to rule out any other disorders or reasons for such a rapid pulse.
After this sequence of testing, Megan finally had a diagnosis. But she still didn’t have a doctor.
Tajchman examines Megan every year to make sure her rapid heart rate hasn’t caused any damage to her heart. But like most cardiologists, she doesn’t treat POTS patients on a regular basis. That’s because with POTS, the problem lies not in the heart but in a part of the brain that regulates things like blood pressure.
Tajchman referred Megan to the Arizona Mayo Clinic. Doctors there confirmed her diagnosis and prescribed a beta blocker to slow down her heart rate. They also told her to drink more water and eat more salt. Then, they sent her home.
Calls for an expert
With no specialist to turn to, Kerry started taking Megan to a holistic family practice physician, Dr. Mary Ellen Coulter. Coulter has a medical degree but also uses alternative therapies. She suggested that Kerry contact a kidney specialist.
“She said, ‘I can’t help you. But (Megan) gets dehydrated, and dehydration (involves) kidneys. Let’s try a nephrologist,’” Kerry recalled.
The first two nephrologists that Kerry called declined to meet with Megan. The third was Dr. Feldman at Bend Memorial Clinic.
“He said, ‘This is a thick file, you’ve been to Mayo, I don’t know if there’s anything I can do,’” Kerry said.
But in May 2008, after another crash landed Megan in the hospital, Feldman invited Megan to come in.
As soon as the Hintons walked into Feldman’s office, the doctor started dissecting Megan’s treatment plan.
“He treated her like a patient right away,” Kerry said. “What we were doing for her wasn’t working. So he said, ‘OK, she can tolerate four times as much of this medication …’ It was the first glimmer of hope we’d had in years.”
“He was really determined to help me,” Megan added.
Kerry was so encouraged that she logged onto www.dynakids.org, a Web-based support group for children and families with POTS and similar disorders, and wrote about Feldman.
“The West Coast is just absolutely desperate for someone to treat POTS,” she said.
Before long, POTS patients from up and down the West Coast were flying to Bend for appointments with Feldman. He now has about 20 patients with severe POTS. Earlier this year, he formed a monthly support group for patients and their families. (See box on next page.)
One Wednesday evening a month, the teens gather at BMC to commiserate and share stories. Their parents meet in a separate room to do the same.
Until Feldman stepped in, Kerry spent her evenings Googling POTS and its symptoms, hoping to find new ideas for treatments. She says it has been a relief to have a medical expert take charge of Megan’s treatment.
“I don’t know what we would have done without Dr. Feldman,” Kerry said. “I mean, he hasn’t cured Megan; she’s still ill. But he tries his best to help her, which is a first.”
In his office at Bend Memorial Clinic one day this month, Feldman reached for a manila folder containing a 2-inch-thick stack of photocopies.
“Here it is,” he said, “the world literature on POTS.”
Half of it was penned by Dr. Blair Grubb, a cardiologist at the University of Toledo in Ohio, who specializes in POTS.
A patient who wants an appointment with Grubb faces a six-month to one-year wait. And, of course, one must fly to Ohio for that long-awaited exam.
Grubb says he’s not sure why more doctors haven’t focused on POTS. U.S. clinics that study and treat the disorder can be counted on one hand.
“It’s not a real sexy disease,” Grubb said.
For one thing, it’s not a single disease like, say, measles.
POTS is “almost like saying ‘leukemia,’ where there are nine different forms of leukemia,” Grubb said. “So it’s really a collection of different disorders, not a single entity. And the different disorders have different causes and different prognoses.”
Not to mention different treatments.
“There’s no FDA-approved (Food and Drug Administration) treatment for POTS,” Feldman added. “None. Everything is off-label.”
Doctors don’t know exactly what causes POTS. Some adult patients say their symptoms began after a serious illness, surgery or pregnancy.
Feldman directs his patients to drink copious amounts of water and eat loads of salt. That expands their blood volume, to relieve some symptoms.
Compression stockings help, too, by preventing blood from pooling in a patient’s legs.
“The problem is, nobody wants to wear them,” Feldman said. “They look funny. They’re hot in the summer.”
Many teens with POTS are very thin. So Feldman tells his patients that anytime they pass a vending machine, they should buy something and eat it. He calls this the “see-food” diet.
“Anytime you see food, eat it,” he announced at a recent support group meeting.
Still, the doctor frets that even with salt tablets and free access to junk food, none of his POTS patients consume enough salt or calories.
Feldman has no training in pediatrics but said he has learned that adolescents with chronic illness can make particularly challenging patients.
“Every step is a battle,” he said. “They are not the medical decision-makers yet, but they are the patients, and that is incredibly frustrating to them, especially because, as teens, they are trying to exert some control over their lives.”
Exercise, for example, helps relieve symptoms, including pain, nausea and fatigue. But when Feldman suggests that a patient be more active, parents sometimes laugh and say they can’t get their child off the couch.
“I am uniformly, miserably ineffective at this,” he said of his exercise prescriptions.
About 80 percent of children and teens with POTS outgrow the disorder by their mid-20s.
Meanwhile, those with severe cases suffer a decade of physical pain, social alienation, missed school and reduced physical activity. POTS patients say their friends try to be understanding but eventually drift away after too many cancellations and vague claims of feeling, well, sick.
One day this spring, POTS patient Robert Pasko, 13, struggled to explain exactly how he was feeling. His head hurt right behind his forehead.
“It’s kind of a throb,” the Red-mond teen said.
His whole body ached like he had the flu, he added. Then Robert fumbled for the words to describe his other symptoms. He looked up at the ceiling.
Dizziness? Not at the moment. Nausea? Not right now. Hunger? Hardly ever.
“I can’t really concentrate or think about anything right now,” he said, finally.
The medical term for this is “brain fog.” It’s one of the main reasons why many adolescents with severe POTS have trouble finishing school.
Tajchman said children with POTS tend to be high-performing students before they get sick.
Robert was a good student until the end of sixth grade, when he started getting migraine headaches. By the beginning of seventh grade, he felt sick every day.
“I was always tired,” he said.
A doctor who examined Robert told the boy’s mother, Cindy Pasko, that he was pretending to be ill to avoid school. When he started fainting, Cindy wondered if her son was playing a joke on her.
“You start to doubt yourself, too,” she said. “Like maybe you’re not being strict enough with your kid. Or maybe you need family therapy.”
Eventually, Robert went to Tajchman, who diagnosed him with POTS. The diagnosis was reassuring to Cindy because it confirmed that her son wasn’t misbehaving. But Robert’s problems continued.
The teen dropped out of school two months into seventh grade because he didn’t have the energy to concentrate. Like many POTS patients, Robert tends to feel better during the summer. Last fall, Robert tried to attend eighth-grade classes, but again had to drop out by late October.
A tutor assigned to him by the school district could only come to Robert’s house in the evenings, when his symptoms were at their worst.
“He used to love school,” Cindy said. “He was a good student, and now he can’t concentrate or remember things like he used to. But he knows what he’s lost.”
Megan Hinton perhaps hasn’t lost as much because she was homeschooled to begin with. And she has a social lifeline in the form of her twin sister and best friend, Katie. A few years ago, the girls started a group called Kreature Komforts that collects new stuffed animals and distributes them to kids who spend major holidays in the hospital. And recently, the girls have been sewing a quilt they will submit to the Sisters Quilt Show.
Still, the illness looms over Megan’s life.
“It’s just all-consuming,” she said. “I can never feel good, like, completely healthy. I always need to think, like at a friend’s house, ‘Have I had enough to drink today?’ or, ‘Can I walk up the stairs right now?’”
Strain on the family
Teens with chronic illness are especially prone to depression and even suicide. Exact rates vary by study, but many experts estimate that up to one-third of all adolescents with chronic illness experience some depression. Among U.S. teens as a whole, depression affects an estimated 8 percent.
“Sometimes, it’s difficult to tease apart what’s chronic illness and what’s a teenager just being a teenager,” Feldman said.
On days when he sees multiple POTS patients, Feldman says he comes home from work emotionally drained. The patients are often sick, frustrated and angry. Their parents are ragged.
The illness is difficult to understand, so it can be hard to muster sympathy from friends, teachers and even some relatives.
“POTS is such a pervasive disorder, with a lot less to hang your hat on (than kidney disease). You can’t tell your friends, ‘My creatinine (a substance that measures kidney function) is four, and I’m going on dialysis,’” he said, referring to mechanical filtration of bodily fluids that replicates kidney function. “It’s not easily explained.”
Cindy Pasko admits that until she read about POTS, and talked to other parents and children dealing with the disorder, she would have been skeptical, too.
“I would have thought, ‘You (kids) are kind of wimpy, and you (parents) are not teaching your kids to work through their difficulties,’” she said.
But now that Pasko finds herself living with a son who has POTS, she wishes other people were more understanding.
“There’s such tremendous judgment about this, and frustration and grief, because (the children) lose their entire life. And yet, you look at him, and it looks like there’s nothing wrong with him,” she said.
Despite his illness, Robert still enjoys video games, electronics and graphic artwork. He used to play basketball, but “it’s too tiring and I can’t win anymore,” he said with a laugh.
He also used to have a more active social life. Robert has some friends in Redmond. Most of his buddies, who live in other countries, met Robert online.
Cindy, who has been a single mom for years, says she struggles to maintain her own social life. She often has to miss work or cancel plans with friends to rush home and help Robert.
“We’re still trying,” she said, “to put a life together.”
Kerry Hinton can relate.
“I have to tell people, ‘We’re not easy to be friends with,’” she said.
And although both families have health insurance, the Hintons and Paskos say they have struggled to keep up with health care bills.
Cindy Pasko said that one month she paid $1,000 in co-pays for Robert’s doctor visits. The Hintons estimate that they’ve spent between $40,000 and $50,000 on Megan’s health care.
“When you’re buying health insurance, and the plan says it pays for 80 percent of costs, that sounds like a great deal,” Kerry said. “But 20 percent of three hospitalizations in one winter … is still a lot of out-of-pocket money for health care.”
Local POTS patients say Feldman has lowered their health care costs by giving intravenous fluids from his office at BMC, for example, so they don’t have to check into the hospital every time they need an IV.
In the end, Kerry says she is grateful Megan doesn’t have cancer or any other illness worse than POTS.
“The hopeful side of it is, 80 percent (of POTS patients) outgrow it,” Kerry said. “But then the other side of it is, even if she outgrows it, she missed her whole childhood.”
She remembers one night last year when she started crying as she helped Megan get ready for bed.
“I said, ‘Megan, I’m so sorry that you have to go through all of this,’” she recalled.
Megan told her mother, “Mom, I wouldn’t trade having POTS for anything.” She went on to explain that the illness made her find pleasure in little things, and take nothing for granted.
Megan still feels the same today — in some ways, she says, POTS has been a gift. Kerry says she will never forget that night.
“It really amazed me. I can’t say that. As a mom, I would trade (POTS),” Kerry said. “I would trade it in a heartbeat.”