Rare Disease Means Constant Pain
Visiting 14-year-old Joseph Martinez in his home, it’s difficult to believe that, just six months ago, he was a healthy boy who enjoyed Little League baseball, flag football and carousing with friends.
Now, the Torrance teen lies in bed on his stomach 24 hours a day, drugged on methadone, with his head facing the bedroom door but his face obscured by a mop of black hair, moaning and writhing from a pain that touches every region of his body.
(See follow-up story from one year later: Boy’s Pain Eased by Podiatrist’s Treatment)
Joseph is stricken by a mysterious disease called complex regional pain syndrome, characterized by severe and relentless pain due to misfiring nerves, which in turn send pain signals to the brain. It’s a rare condition, afflicting between 200,000 and 1.2 million Americans, but is becoming more prevalent in children, according to the Reflex Sympathetic Dystrophy Syndrome Association.
Joseph chooses to lie on his stomach because, for some reason, lying on his back is unbearable. The pain is such that he cannot put on a shirt or take a shower without screaming.
He has been in this state, and in this bed, for five months.
In October, he dropped out of school at the K-8 St. Catherine Laboure school in Torrance, where he once thrived and where his family has been a prominent fixture for years.
“He used to weigh 169 pounds,” said his mother, Susan Martinez. “Now he’s bones.”
To their heartbreak, he has even asked them for death.
“He says it, `just kill me,’ I can’t go on,” said his father, Justin Martinez, who for years has volunteered at the school as a basketball coach. “Maybe three times, he’s asked me to hug him, but he can’t ask anymore because it hurts too much.”
His parents are frantically trying to find a treatment. Thus far, their efforts have borne little fruit. They’ve battled their insurance company and listened to flawed advice from doctors unfamiliar with the disorder. Now, the entire family is stressed out and sleep-deprived, due to Joseph’s round-the-clock wailing.
Their primary problem is that few hospitals — as few as three in the United States — are fully equipped to treat the condition, and the family’s insurance coverage prohibits them from seeking help outside their network area in the South Bay.
But they’ve reached their wit’s end, and plan to fly him to what they believe is the best of those hospitals, Cleveland Clinic Children’s Hospital, costs be damned. They plan to pay the $45,000 out of pocket, and that doesn’t include airfare or hotel expenses.
Meanwhile, moved by their friend’s plight, Joseph’s classmates at school have organized a weeklong event in honor of him. All this week, they’ve been wearing blue — Joseph’s favorite color — and holding bake sales.
The original idea was to raise a hundred or so dollars as a gesture of kindness. But Joseph’s story struck a chord with parents, and the money has been pouring in. Already, the school has raised more than $10,000.
“It speaks to the closeness of our school and parish community,” said Mary Dell’Amico, St. Catherine’s vice principal. “Many of our families struggle just to make tuition. To see the kind of donations coming in is just inspiring.”
The third of four children, Joseph’s two older siblings were standout athletes and scholars at St. Catherine, Dell’Amico said. He, too, was a decent athlete and student, and was always well liked by classmates.
“He was quiet but you knew he was there,” she said. “He wasn’t one of the boisterous kids that had to be the center of attention.”
The Martinez family is a single-income household, with Susan holding down a job as a project dispatcher at Triumph Aerostructures in Hawthorne. Justin, who himself suffers from a spinal disease, is Joseph’s primary caretaker during the day, and the home-schooler of their youngest child.
Even in the medical community, not much is known about complex regional pain syndrome. This means doctors are often at a loss about how to treat it.
This was certainly the case for the Martinez family, which dealt with one perplexed doctor after another at Torrance Memorial, Miller Children’s Hospital in Long Beach, and Children’s Hospital Los Angeles. One well-meaning doctor reasoned that the best remedy was to minimize the pain by prescribing a maximum dosage of heavy-duty painkillers, such as morphine and the fentanyl patches that are commonly used on cancer patients.
The powerful drugs left the boy in drowsy, but, to everyone’s surprise, were useless in relieving pain. Instead, they simply turned him into an unwitting addict.
Other doctors concluded that the pain must be entirely psychological. One even asked the parents to leave the boy’s hospital room and then, after they complied, ordered him to get out of bed and walk.
After cycling through 18 doctors, the family in January finally found hope in Dr. Lonnie Zeltzer, the renowned director of the pediatric pain program at Mattel Children’s Hospital at UCLA.
“She was the only one who went to Joseph and said, `You are not faking it,”‘ Susan said.
Zeltzer said the family needed to send the boy to one of the three hospitals. (Zeltzer’s program does not have the in-patient component that he needs. And even if it did, the insurance wouldn’t cover it.)
Zeltzer, whose name appears frequently in national media outlets as a pain expert, said Joseph’s condition has languished so long that it has morphed into “widespread central pain syndrome.”
“I think that many doctors, if they do tests and can’t find anything in their tests, they assume it’s psychology or attention-getting,” she said. “They sort of downplay the amount of suffering.”
Adding to the misery, being bedridden has brought about secondary pain. The incessant moaning and screaming, for instance, has left Joseph with a sore throat. The inactivity has weakened his left arm to the point where he only uses his right. He will have to relearn how to walk.
Still, Zeltzer believes that, if treated correctly, Joseph will again throw a baseball, toss a football, haul his books to class and hang with friends — as early as next year.
When she treated him this winter, Zeltzer’s first order of business was to wean him off the high-octane painkillers without triggering withdrawal. She put him on methadone, which is often prescribed to patients hooked on opioid drugs, as well as recovering heroin addicts. Now, she said, he needs to attend one of the three medical centers. (The other two are in Stanford and Seattle.)
There, a team of specialists in psychiatry, psychology and occupational therapy will help him retrain his brain, which has been re-networked in such a way that the pain receptors refuse to turn off. Physical therapists will get him moving again, strengthening his muscles.
Complex regional pain syndrome tends to be triggered by a traumatic event, such as a car accident or medical procedure. Often, the severity of the pain experienced by the victim exceeds what would be expected. The age of the average sufferer is 42, but experts say it is becoming more and more prevalent in children, usually girls.
In Joseph’s case, the family believes the catalyst was an episode of food poisoning that left him with flu-like symptoms.
Zeltzer said one way to better understand CRPS is to think of the amputees who routinely suffer from what is known as phantom pain. Although the limb is gone, the receptors in the brain associated with it are still there, and oftentimes are still firing.
For amputees, the phantom pain usually subsides after a year, Zeltzer said. But sufferers such as Joseph must retrain their brain. This, she said, requires learning about how emotions and thoughts affect physical pain, and then using that knowledge to change thought patterns.
For instance, she said, signals of distress in the brain often set off a chain reaction that bathes the brain in stress hormones, which may disrupt a person’s sleep schedule, which in turn often leads to a heightened sensory of pain.
“If you don’t get good restorative sleep, your body starts to feel pain in general,” she said. “It’s like a snowball effect.”
To help Joseph, a therapist may ask him to imagine an activity he enjoys, like playing baseball.
“They want them to really be there, to really feel it: the warmth of the sun, all the sensations,” Zeltzer said. Once the muscles are relaxed, once the circuitry calms down, the patient is asked to take a moment to notice his current state of tranquility.
“In a sense, you’re replacing the pain-image circuitry with developing a new circuitry of feeling really good,” she said. “Your brain learns that bodily state of relaxation just like learning how to write the alphabet or ride a bike.”
Last week, without prompting from the family, Zeltzer tried to intervene on Joseph’s behalf. She called the medical director of their health care provider, THIPA, in an effort to get the child admitted to Stanford, with THIPA picking up the tab. They haven’t heard back.
The Martinez family would prefer Cleveland to Stanford. However, they said should the health provider cover a trip to Stanford, they will gladly go. In that event, any unused proceeds from the school fundraiser — which they did not initiate — would be returned.
As for the food poisoning that may have provoked Joseph’s condition, the family believes it was a meal of undercooked chicken in September. All six family members got sick, but for Joseph and his younger brother, the symptoms developed into something akin to the flu.
His younger brother recovered after a month, but the symptoms only worsened for Joseph.
“The virus by now, it’s undetectable,” said Justin, a 39-year-old graduate of North High School in Torrance. “It’s long gone, but the damage is still there.”
How to Help
Anyone interested in contributing to St. Catherine Laboure’s fundraiser for Joseph should call the school at 310-324-8732.