Pain has many forms.
For Leland Smith, 16, who has severe hemophilia A with an inhibitor, sometimes it feels like an enormous force is crushing his joints. That’s how he knows he’s having a bleed—usually in a target joint, though he sometimes gets spontaneous bleeds, too.
Other times, the Shrewsbury, Massachusetts, high schooler says it’s a phantom—pain caused by nerve stimulation in a joint that regularly bleeds. Something will trigger the nerve, and he’ll experience a body memory of crushing bleeds from his past.
“It feels like the pain of a bleed, but a little different,” Leland says. “It can get pretty depressing, because it sometimes feels like there’s no end in sight.”
[Steps for Living: The Pain Facts]
For Christian Brothers, 46, with severe hemophilia A, the pain is usually a constant, throbbing ache that can keep him home. Since he grew up before the advent of prophylactic clotting factor, he’s had two bleeds a week for most of his life, typically into target joints such as his knees. Now his joints are damaged and arthritic.
Brothers, a stay-at-home dad from Dearborn Heights, Michigan, prefers the pain of acute bleeds because he knows it will subside when he treats with factor. In contrast, the chronic arthritis in his knees, hips, ankles and elbows is more stubborn. On most days, it takes him an hour to get out of bed because of his arthritis. Those are the good days, when Brothers is able to grocery shop and watch his stepson at karate practice.
“I’m one of those people who has so many things I want to do,” says Brothers. “If I can’t do something, that weighs heavily on me and I feel more isolated.”
If you have a bleeding disorder, you know that chronic pain can permeate every part of your life. “The pain gets so overwhelming that it affects people physically and emotionally, as well as financially and socially,” says Angela Lambing, MSN, NP-C, nurse practitioner for the Henry Ford Health Systems Hemophilia and Thrombophilia Center in Detroit. But if you fight back using every technique you can—pharmacological, holistic, psychological and behavioral—you can live a content, full life. “People don’t realize if they treat the pain, a lot of problems can be managed or resolved.”
Confronting the Pain
“Pain can be the primary problem people with hemophilia have with their disorders, more than finding time to infuse or anything else,” says psychologist Kathy Parish, PhD, who is the Southern California staff member for the Hemophilia Council of California. “That’s especially true for children with inhibitors, in which the bleeding is poorly controlled, or the older guys, who grew up without early prophylaxis.”
The pain, she says, has far-reaching effects.
Physically, pain is exhausting, preventing people who live with it daily from accomplishing all that they can. Further, it can cause you to lose focus at work or school. It can also disturb sleep, preventing the restorative properties of complete rest that can ease suffering, leaving you groggy and unfocused the next day. Chronic bleeds can mean limited mobility or even confinement to your home.
For Leland, who sometimes has to go to the hospital to cope with the level of pain he experiences, nighttime and school are the most trying times. He can’t use distraction to ignore the pain. After school, when he wants to have energy, he often finds it’s sapped.
“My friends will be so energetic and want to do something, and I’m exhausted from fighting the pain all day,” says Leland.
Socially, pain disrupts consistency when it comes to obligations, scheduling social events or committing to volunteer work. Also, the more time you spend by yourself, the more time you have to focus on your pain, which can exacerbate it.
When Brothers makes plans for dinner and a show with his wife, it becomes a logistical act of timing his medications perfectly so that he’s lucid, energetic and pain-free enough to go out. He doesn’t always make it. Joint pain can prevent him from doing many of the things he loves: coaching sports for his stepson, working and, sometimes, taking care of basic family responsibilities.
“My parents are older, and I haven’t been able to get over to see them for a couple of weeks,” Brothers says. “You start feeling like you’re not doing your fair share for your family.”
There can be a financial cost to chronic pain. Long-term absenteeism at work because of bleeds can translate into financial hardship when you lose your job. “Especially in this economy, the financial impact of pain can hit people really hard,” says Peg Geary, MPH, LCSW, social worker at the New England Hemophilia Center in Worcester, Massachusetts. “With a lot of pain and associated physical limitations, people end up not being able to work or are in and out of work. That can mean losing insurance and some degree of income. It’s a big problem.”
Brothers managed a bakery and deli until 16 years ago, when complications from transfusion-contracted HIV, as well as his chronic pain, made it difficult to keep working. When he retired, he went through an arduous, two-and-a-half-year journey through the Social Security system.
“It became almost like a battle of wills, with them holding all the cards,” Brothers recalls. “My application was denied the first three or four times, and I had to appeal. And the doctors they sent me to didn’t know the first thing about hemophilia or HIV. They seemed to figure that if you could walk in the door, you could probably go to work.” He has toyed with the idea of finding another job, but the prospect of losing his government insurance is enough to keep him at home.
For students whose classwork is constantly interrupted and who fall behind in school, the pain forces them to defer graduation or plans for higher education.
Emotionally, pain is associated with an increased incidence of depression, anxiety, irritability, anger and even grief. For Brothers, the overwhelming feeling is frustration: “You get to feeling sort of stuck in your home and isolated,” he says. “Especially when it’s icy out, and I can’t get around, I feel like I can’t do what I’m supposed to do as a husband and father, and I feel bad about myself.”
This loss of ability and independence can lead to feelings of grief about the life, activities and self-esteem you may feel you’ve lost because of your pain. “You participate in fewer social activities, which means that there are fewer opportunities to feel good about yourself and get involved in the lives of others,” Parish says. Those feelings are considered a form of loss or grief.
The Specter of Addiction
Opioids, such as oxycodone, are often prescribed for people with hemophilia, because other medications can be a bad fit, says Lambing. Aspirin exacerbates or causes bleeding, while acetaminophen aggravates liver conditions. However, pain is often undertreated, because those with hemophilia and their providers misunderstand pain treatment and fear addiction.
“People panic when it comes to opioids,” Lambing says. She has conducted pain research with the Henry Ford Health Services team in Detroit. “Patients are worried about becoming addicted, being oversedated by the medication, and don’t want to be labeled because they’re taking OxyContin®.” Providers are concerned about misuse and lawsuits, she says. Many do not have a working knowledge of narcotics or familiarity with prescribing them properly.
Brothers tells strangers that he takes codeine for his pain (although it’s OxyContin), because he doesn’t want to be lumped in with prescription drug addicts.
“I’m still not comfortable with it because of the stigma involved,” Brothers says. “I feel stuck: If I don’t take it, I’m not able to do the things a father should do, like go to my son’s karate classes. If I do, I risk becoming addicted.”
But there are healthy ways to take painkillers. In fact, the US Food and Drug Administration has created a guide to safe pain-medicine use, which Congress is expected to pass and President Barack Obama is expected to sign. The National Pain Care Policy Act of 2009 would provide doctors with more training, give more information to people like Brothers and address pain treatment disparities by race, age and gender.
What’s more, there’s an important distinction between dependence on medication and addiction, says Lambing. With dependence, your body needs the medication to be free of pain. If you stop taking it abruptly, you will feel anxious, break out in a sweat and have the flulike symptoms of withdrawal. With addiction, you experience the withdrawal symptoms, but also a mental obsession with taking more, despite negative consequences.
One of the bigger problems for people with chronic pain and hemophilia isn’t addiction, but so-called pseudo-addiction, says Michelle Witkop, FNP-BC, a nurse practitioner at the Munson Medical Center’s Northern Regional Bleeding Disorder Center in Traverse City, Michigan. With pseudo-addiction, someone with poorly managed pain might exhibit several of the signs of addiction, but they are temporary. These symptoms include shopping for doctors, hoarding medications, changing dosages without consulting doctors and visiting different pharmacies to get multiple prescriptions for medications. (See sidebar, “Addiction and Chronic Pain.”)
“Once the pain is validated—once it’s proven that the person has an untreated pain issue and is given the medically appropriate amount and type of medication—these symptoms and behaviors usually go away,” says Witkop.
Having a Good Day
Fortunately, there are many ways to cope with pain that don’t involve medication, says Witkop. The first is a pain log, she says. To create a log, write the date, time, medication use and pain rating for each instance of pain. That way, you can discover behaviors that cause pain and change them to increase your pain-free days.
Distraction also works. For instance, one of Leland’s preferred ways to relieve pain is to settle in for an episode of his favorite TV shows, Heroes or Lost.
“He becomes absorbed in them and forgets his pain for a while,” says his mother, Jane Cavanaugh Smith. “Other times, he likes watching movies like The Dark Knight.”
Internet-enabled video games can help combat feelings of isolation and loneliness. When Leland is stuck at home and unable to go out, he can still play games with his friends virtually. It restores a sense of control that the pain can take away.
“Plus, he’s good at them, and that helps his self-esteem,” says Cavanaugh Smith.
Then, there are some emerging alternative treatments. The Henry Ford Hemophilia Treatment Center in Detroit and the Dharwad Hemophilia Treatment Center in India are conducting a study to see if acupuncture can help alleviate arthritic pain in people with hemophilia. So far, the results are promising, says Lambing.
Until the results are definitive, talk to your provider about traditional pain management techniques that might bring relief. Parish suggests visualizations, self-hypnosis, meditation or even very gentle yoga to relieve some pain.
“You can envision different feelings as different colors, with, say, purple as the color of pain,” she says. “As you relax and meditate, see if you can turn down that pain a little more every night. It’s like using meditation with specific goals.”
Listening to music, playing an instrument or singing will transport you elsewhere, says pain specialist Raffi Tachdjian, MD, MPH, of the Pediatric Pain Program at UCLA Mattel Children’s Hospital in Los Angeles. One of his patients reports that his pain drops from a 10 to a 3 when he’s playing and singing music.
Brothers relies on the twin pain management techniques of patience and faith. Patience helps him wait out the arthritis when it is really bad, and faith assures him that the pain management techniques he uses, including medication, will work and he will feel good again.
“On a good day, you forget the pain and the stress,” Brothers says. “When things get rough, you can look forward and know you’ll have another good day soon.”