For individuals with osteogenesis imperfecta (OI), the pain associated with multiple fractures can lead to needless suffering and, when untreated, may result in a chronic condition. Since pain may impair a person's ability to lead a productive life, chronic pain is a serious economic as well as a major health problem.
Pain is the body's way of responding to damaged tissue. When a bone breaks, nerves send pain messages through the spinal cord to the brain, where they are interpreted. How a person responds to pain is determined by many factors, including his or her emotional outlook. For example, depression seems to increase a person's perception of pain and to decrease his or her ability to cope with it. Often, treating the depression treats the pain as well. If pain is not adequately treated, the transmission of pain impulses to the brain occurs more readily. Therefore, it is more effective to prevent pain than to treat it after it occurs.
Acute pain is usually characterized by a short duration, normal functioning of both the peripheral and central nervous systems, a predictable course and, in most cases, a good outcome. Chronic pain is pain that lasts beyond the expected time for healing and interferes with normal life. The damaged tissues have healed, but the pain continues. The pain message may be triggered by muscle tension, stiffness, weakness, or spasms. Nonverbal responses to pain include elevated heart rate and blood pressure and immobilization of an injured part in order to avoid pain from movement. Whatever the cause of chronic pain, feelings of frustration, anger, and fear make the pain more intense. Chronic pain can often diminish the quality of a person's life psychologically, socially, and physically. It is also the most frequent cause of suffering and disability in the world today.
With OI, there can be associated conditions of acute and chronic pain resulting from multiple fractures, vertebral collapse, joint deformity, osteoarthritis, contractures, deformity/malalignment of limbs, and recurrent abdominal pain. Pain management for OI, in both adults and children, requires adequate assessment and implementation of a regimen that should address the multifaceted presentation of acute and chronic pain. With the increased longevity of individuals with OI, the incidence of pain syndromes relating to degenerative changes becomes more likely. An interdisciplinary approach to pain management is best.
The following information provides an overview of different options for pain management in OI. Individuals who need help managing the chronic pain associated with OI may wish to discuss the options listed below with a physician.
Physical Methods of Pain Management
Heat and Ice. Heat in the form of warm showers or hot packs can relieve chronic pain or stiff muscles. Cold packs or ice packs provide pain relief by numbing the pain-sensing nerves in the affected area. Cold may also prevent swelling and inflammation. Heat or ice should be applied for 15 to 20 minutes at a time to the painful area, and a towel should be placed between the skin and the source of the cold or heat to protect the skin.
- Warm towels or hot packs in the microwave provide a quick source of heat.
- Frozen juice cans or bags of frozen vegetables make instant cold packs.
- A damp towel that has been completely wrung out and frozen can provide relief from pain.
- Freezing a plastic, resealable bag filled with water makes a good ice bag.
Transcutaneous Electrical Nerve Stimulation (TENS). A TENS machine is a small device that sends electrical impulses to certain parts of the body to block pain signals. Two electrodes are placed on the body where the person is experiencing the pain. The electrical current that is produced is very mild, but it can prevent pain messages from being transmitted to the brain. Pain relief can last for several hours. Some people may use a small, portable TENS unit that hooks on a belt for more continuous relief. TENS machines should be used under the supervision of a physician or physical therapist. They can be purchased or rented from hospital supply or surgical supply houses; however, a prescription is necessary for insurance reimbursement.
Other forms of electrical therapy that can be beneficial include high volt, low volt, micro stimulation, bipolar, interferential, and Russian stimulation. Each of these therapies has different properties to control not only pain but to decrease swelling and strengthen weakened muscles. Most of them, however, must be used in the physician's office.
Exercise or Physical Therapy. Exercise or physical therapy, under the supervision of a health professional who understands the nature of OI, can be effective in strengthening muscles, improving stamina, and helping an individual have a more positive outlook on life. Because exercise raises the body's level of endorphins (natural painkillers produced by the brain), pain can diminish. It is beneficial for children with OI to begin physical therapy as soon as possible. Physical therapists can teach proper positioning, posture, and exercises to strengthen muscles without injuring bones. Exercise can be as simple as moving a joint through its range of motion. Pool therapy is one of the best exercise techniques to gently improve muscle strength and reduce pain.
Acupuncture and Acupressure. Acupuncture involves the use of special needles that are inserted into the body at specific points. These needles are believed to stimulate nerve endings and cause the brain to release endorphins. It may take several acupuncture sessions before the pain is relieved. Acupressure is direct pressure over trigger areas of pain. This technique can be self-administered after training with a certified instructor.
Massage Therapy. Massage therapy can be a light, slow, circular motion with the fingertips or a deep and kneading motion that moves from the center of the body outward toward the fingers or toes. Massage relieves pain, relaxes stiff muscles, and smoothes out muscle knots by increasing the blood supply to the affected area and warming it. The person doing the massage uses oils or powder so that his/her hands slide smoothly over the skin. Massage can also include gentle pressure over affected areas or hard pressure over trigger points in muscle knots. Special care must be taken when working on individuals with OI to avoid putting too much pressure on bones that might possibly fracture.
Psychological Methods of Pain Management
Relaxation training. Relaxation involves concentration and slow, deep breathing to release tension from muscles and to relieve pain. Learning to relax takes a great deal of practice, but relaxation training can focus attention away from pain and release tension from all muscles. Relaxation audiotapes are available to help achieve the desired effects.
Biofeedback. Biofeedback is taught by a professional who uses special machines to help a person control certain functions such as heart rate and muscle tension. As the person learns to release muscle tension, the machine immediately indicates success. Biofeedback can be used to reinforce relaxation training. Once the technique is mastered, it can be practiced without the use of the machine.
Visual imagery or distraction. Imagery involves concentrating on mental pictures of pleasant scenes or events or mentally repeating positive sayings to reduce pain. Videotapes are also available to help visual imagery. Distraction techniques focus the person's attention away from negative painful images to more positive thoughts. This technique may include watching television or a favorite movie, reading a book or listening to a book on tape, listening to music, or talking to a friend.
Hypnosis. Hypnosis can be used in two ways to reduce a person's perception of pain. Some people are hypnotized by a therapist and given a post-hypnotic suggestion that reduces the pain they feel; others are taught self-hypnosis and can hypnotize themselves when pain interrupts their ability to function. Self-hypnosis is a form of relaxation training.
Individual or family therapy. A psychologist, psychiatrist, or psychiatric social worker can help people cope with feelings of depression, frustration, and anger that often accompany chronic pain.
Medications for Pain Management
To be most effective in alleviating pain, medications should be administered before pain appears and certainly before it worsens. Pain medications should be administered prior to painful procedures or before the performance of an activity that may be painful, such as deep breathing exercises after a spine fusion. As stated earlier, it is easier to prevent pain rather than to attempt to alleviate it. Pain is best prevented or treated by providing pain medication on a continuous or around-the-clock basis rather than as needed. Pain medications should be administered in an acceptable manner that is less uncomfortable than the pain itself. Ideally, they should be administered orally, intravenously, or under the tongue. Medications taken orally are generally the least expensive and most convenient alternative. Intramuscular injections are painful and may be a last resort.
Over-the-counter pain relievers. Aspirin, ibuprofen, naprosyn sodium, and acetaminophen can effectively relieve pain. While these medications are relatively safe, they can still cause side effects, such as stomach upset, and may result in complications from excessive dosing or prolonged administration. For this reason, these medications should be taken according to the doctor's or manufacturers' directions. Before increasing the frequency of use or the dosage, the person should first check with the doctor or pharmacist.
Non-steroidal anti-inflammatory medications (NSAIDs). NSAIDs may be prescribed to treat mild to severe pain. These preparations block pain and treat inflammation. There are dozens of NSAIDs on the market, and a person may have to try several different brands to find the one that works best.
Topical Pain Relievers. A variety of topical creams may also relieve pain when they are rubbed directly into the painful area. While some of these creams are available by prescription only, others may be purchased over-the-counter.
Narcotic pain medication. Narcotics are powerful pain-relieving medications derived from opium or synthetic opium. Narcotics alter a person's perception of pain and can also induce euphoria, mood changes, mental cloudiness, and deep sleep. These drugs may also cause nausea, lethargy, and constipation. People with OI must be especially careful when taking these medications. Narcotics can affect a person's balance and increase the chance of falling. After repeated and prolonged use, some people may become dependent on or addicted to these medications, which are available only by prescription.
There are new medications that appear to relieve pain without the serious side effects of narcotics. There is also a skin patch that releases small amounts of a narcotic into the body through the skin. These medications can only be prescribed by a physician.
Antidepressant medications. People who suffer from chronic pain frequently suffer from chronic depression as well. Several studies using antidepressant medications have noted that these medications may not only improve depression but may also relieve or reduce the amount of pain a person feels. Additional research is needed to determine whether antidepressants can treat the chronic pain of OI and which antidepressants produce the best results.
Nerve block. In some cases, a physician may perform a nerve block that involves the injection of pain-relieving medications into the tissues around an affected nerve. The block numbs the nerves and surrounding tissues and eliminates the sensation of pain. Pain relief may last for hours or months, depending on the medications used and the person's response to them. All of these methods of pain management, alone and in combination, are used in hospitals and clinics across the country. People who are suffering from unrelieved chronic pain may wish to consult a physician for a referral to a physical therapist or a clinic specializing in pain management.
Pain management for individuals with OI often requires a multidisciplinary approach involving specialists in medicine, psychology, and rehabilitation for adequate treatment. The goal for treatment is effective therapy that will not only reduce or remove the pain but will also achieve mental well-being and an improvement in physiological function.
This information is adapted from the "Osteoporosis Report," Winter 1996, National Osteoporosis Foundation.
This information is brought to you by the
NIH Osteoporosis and Related Bone Diseases~National Resource Center (ORBD~NRC)
and the Osteogenesis Imperfecta Foundation
National Institutes of Health
Osteoporosis and Related Bone Diseases
National Resource Center
1232 22nd St., NW
Washington, DC 20037-1292
Tel: 800/624-BONE or 202/223-0344
Fax: 202/293-2356, TYY: 202/466-4315
The National Resource Center is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases with contributions from the National Institute of Child Health and Human Development, National Institute of Dental and Craniofacial Research, National Institute of Environmental Health Sciences, NIH Office of Research on Women's Health, Office of Women's Health, PHS, and the National Institute on Aging. The Resource Center is operated by the National Osteoporosis Foundation, in collaboration with the Paget Foundation and the Osteogenesis Imperfecta Foundation.