Christopher Reeve’s death this past October from cardiac complications after infection resulting from pressure ulcers is a reminder that patients with spinal cord injury (SCI) are more than their motility impairments. According to the Annual Statistical Report of the National Spinal Cord Injury Statistical Center (NSCISC), published last June, of 3312 patients for whom the cause of death was known, nearly 22% died of respiratory system diseases, 9% of infective and parasitic diseases, 8% of hypertensive and ischemic heart diseases, and 13% of other heart disease.
Christopher Reeve's death this past October from cardiac complications after infection resulting from pressure ulcers is a reminder that patients with spinal cord injury (SCI) are more than their motility impairments. According to the Annual Statistical Report of the National Spinal Cord Injury Statistical Center (NSCISC), published last June, of 3312 patients for whom the cause of death was known, nearly 22% died of respiratory system diseases, 9% of infective and parasitic diseases, 8% of hypertensive and ischemic heart diseases, and 13% of other heart disease.1 Suzanne Groah, MD, MSPH, director of the National Rehabilitation Hospital's Rehabilitation Research and Training Center on Secondary Conditions After Spinal Cord Injury in Washington, DC, noted that the NSCISC database represents only 12% to 15% of all persons with SCI in the United States; however, she said, "It's the best database we have." Secondary health problems depend on the level of the spinal cord that sustained the injury and the degree of injury. The patient's age and the time since the injury occurred also have an impact. Among the secondary health effects of the immobility resulting from SCI are pressure sores and their sequelae; respiratory complications; cardiovascular disease; hyperlipidemia; metabolic syndrome; chronic pain; osteoporosis; joint and muscular injury; urinary tract infections; bowel control problems; muscle spasticity; and emotional problems, such as depression, alcoholism, drug abuse, and suicide.2-7 Groah pointed out that as medical care for patients with SCI improves, "people are living to older ages, and we're beginning to see [increasing] chronic diseases." These patients, she explained, show accelerated aging, with diseases of aging appearing in them at younger ages than in the rest of the population. It's possible that the person would never have had any of these diseases without the SCI, but when diseases such as coronary artery disease and diabetes occur, they are more aggressive or may be at a more advanced stage at diagnosis than they usually are in patients without SCI. Part of the problem in diagnosing secondary diseases is that a quadriplegic person may not perceive chest pain, so he cannot report the signs of cardiovascular disease to his physician. Thus, the patient with SCI must be screened more often than other patients for signs of cardiovascular disease; this screening is usually done by the primary care physician. Alberto Martinez-Arizala, MD, chief of the Spinal Cord Injury Service at the Miami Veterans Administration Hospital, warned that physicians "must be aware that the typical sensory signals . . . in these patients are al- tered." This, he added, means that symptoms of myocardial infarction are different, as are symptoms of a pathologic process in the GI tract. A patient with SCI who also has a serious physical disorder may experience dystonia, sweating spells, or symptoms that are out of the ordinary but are not the same as the kinds of symptoms a physician would see in a patient with an intact spinal cord.2 Furthermore, noted Groah, immobility results in a loss of muscle mass and an increase in fat mass, leading the body to act as if it's obese. This puts the patient at greater risk for metabolic syndrome, which includes insulin resistance and may result in diabetes and heart disease. EXAMINATIONS MORE COMPLICATED Groah explained that a standard physical examination is more difficult to carry out on a patient who is immobile. "Checking someone's weight doesn't get done with the same frequency." This is because physicians don't usually have scales that can handle wheelchairbound patients. "Colonoscopies don't get done; mammograms don't get done like they should; Pap smears don't get done like they should," she continued. One of the most prevalent complications--in some studies, reported to affect nearly 40% of patients with SCI--is pressure sores.2,3 These sores, according to newspaper reports, led to septicemia that eventually claimed Reeve's life. But according to Jung Ahn, MD, clinical professor at New York University's Rusk Rehabilitation Institute, pressure sores usually are preventable. He explained that there are 2 theories on how these sores develop. One is that the skin, especially over the bony prominences, is irritated by the patient's lying on a mattress or sitting all day in one position in a wheelchair. According to this theory, pressure sores begin superficially and, without proper care, go deeper, eventually affecting the underlying muscle and even the bone. The second theory is that the bone is pressing the muscle into the skin and ischemic changes in the muscle start the process, with the sore beginning internally and then spreading superficially. No matter how they begin, Ahn said, "Pressure sores in spinal cord injury are, as far as I'm concerned, preventable." The way to prevent the sores is to train patients and their families to inspect the patient's skin each morning after hours of bed rest and again in the evening, after the patient has spent the day sitting in a wheelchair. If a sore begins to develop, especially if it is caused by the wheelchair, Ahn may put a patient on bed rest until the sore is completely healed. But to prevent sores, paraplegic patients are told to do sit-ups or weight shifts in the wheelchair. Ahn stated that paraplegic persons could do sit-ups every half hour and lift their buttocks off the seat every half hour. Quadriplegic patients can learn how to shift their weight, leaning to one side of the chair to eliminate pressure. Good nutrition is essential in preventing pressure sores: a diet high in protein and vitamin C prevents skin breakdown. Once pressure sores develop, zinc sulfate is added to the patient's diet. It's important that skin remains dry. Fecal or urinary incontinence should be prevented or actively managed. If the patient does have a bowel or urinary accident, he should be cleaned up immediately. Bladder control is a major problem for patients with SCI.2 Although some patients are able to void urine on their own, others, Groah said, may require an indwelling catheter, such as a Foley or superpubic catheter; still others may use an external condom urine collector. Groah has been studying the relationship of indwelling urinary catheters to bladder cancer. She has found that even adjusting for other risk factors, a patient with SCI who has an indwelling catheter has a 5-fold greater risk of bladder cancer developing than does someone with an intact spinal cord. She explained, "The risk of mortality due to bladder cancer is 3.2/100,000 person-years in the non-SCI population. The age-adjusted mortality due to bladder cancer in those with SCI using indwelling catheters is 52/100,000 person-years." Also, Groah said, patients with SCI will have more aggressive bladder cancer, such as squamous cell carcinoma, than do persons with intact spinal cords, who tend to have transitional cell carcinoma. She continued, "The increased risk of bladder cancer occurs mainly when people are in their 40s--as opposed to after 60 in non-SCI [patients]--although a few cases in people in their early 30s have been reported." Long-term use of an indwelling catheter seems to predispose patients to kidney and bladder stones and pyelonephritis, she explained. At present, physicians do not know whether the greater risk of bladder cancer is related to the catheter itself or to the increased risk of infection and stone development from having a catheter. Nevertheless, Groah suggested that patients who have had indwelling catheters for 10 years or more should be more aggressively screened for bladder cancer. Martinez-Arizala said that kidney and urinary tract function should be monitored annually. Groah also explained that pulmonary problems, such as pneumonia, have been one of the major causes of morbidity and mortality in patients with SCI. The higher up the spinal cord the injury is, the greater the risk, and the longer the period of survival after the injury-- especially if someone was injured 10 to 15 years before and is now in his 40s or 50s--the greater the risk of pulmonary conditions. Said Martinez-Arizala, "These patients have impaired cough mechanisms so they don't clear their airways as they should." Monitoring these patients and giving them annual influenza vaccinations and pneumococcal vaccination every 5 years can help. WIDESPREAD CHRONIC PAIN Chronic pain is a major problem for about 75% of patients with SCI, said Eva Widerstrm-Noga, DDS, PhD, research assistant professor at the Miami Project to Cure Paralysis.4-7 Paraplegic patients may suffer from nociceptive pain--true pain sensations, often in the back and shoulder, resulting from wheeling around in a wheelchair. Neuropathic pain, often described by the patient as a burning, electric, or stabbing sensation, derives from the areas of the body with absent or impaired sensation. Pain also may be referred to visceral organs. This latter type of pain is especially difficult to treat and to endure, said Widerstrm- Noga. Neuropathic pain may be treated with anticonvulsant or antidepressant medications, she said, but there have been few studies on patient response to these medications. In some patients, pain may be alleviated by massage therapy or relaxation methods, Widerstrm-Noga noted. Muscle spasticity may occur after the acute stage of the injury.2 "It could be a lifelong problem for that patient," said Martinez-Arizala. But newer drugs and drug delivery methods make it possible to control severe spasticity in some patients. The lesion itself may change character. Progressive syringomyelia or progressive post-traumatic cavitation of the spinal cord may occur with or without tethered cord syndrome, a condition in which the spinal cord builds up scar tissue that prevents the flow of cerebrospinal fluid through and around the spinal cord. The patient will present with new loss of function and new neurologic symptoms that were not present before, said Martinez-Arizala. This condition may be corrected with surgery. Patients with SCI also may have sexual dysfunction. 2,8,9 Men may have erectile dysfunction, and they are likely not to be able to ejaculate even if they can maintain an erection.2,8 Women may not be able to reach orgasm.9 There are treatments that allow sexual activity in both men and women with SCI, and with some medical intervention, men can even father children. The hope, of course, is the one Reeve maintained: that someday there will be a way for damaged spinal tissue to regenerate. A study published in the November 10, 2004, issue of the Journal of Neuroscience showed that mice lacking a molecule that guides axons in the spinal cord during development will regenerate axons and will recover spinal cord function.10 This leaves the tantalizing possibility that by blocking this molecule, damaged human spinal cord tissue can regenerate. But right now, medicine is many years away from such a cure, and patients with SCIs remain at risk for numerous secondary effects of the injury. ? REFERENCES 1. National Spinal Cord Injury Statistical Center. The 2004 Annual Statistical Report for the Model Spinal Cord Injury Care Systems. Birmingham, Ala: University of Alabama at Birmingham; June 2004. 2. Ahn JH. General considerations of rehabilitation of spinal cord injured patients. In: Errico T, Bauer RD, Waugh T, eds. Spinal Trauma. Philadelphia: JB Lippincott; 1991:627-643. 3. Garber SL, Rintala DH. Pressure ulcers in veterans with spinal cord injury: a retrospective study. J Rehabil Res Dev. 2003;40:433-442. 4. Widerstrm-Noga EG, Felipe-Cuervo E, Broton JG, et al. Perceived difficulty in dealing with consequences of spinal cord injury. Arch Phys Med Rehabil. 1999;80:580-586. 5. Widerstrm-Noga EG, Felipe-Cuervo E, Yezierski RP. Chronic pain after spinal injury: interference with sleep and daily activities. Arch Phys Med Rehabil. 2001;82:1571-1577. 6.Widerstrm-Noga EG, Felipe-Cuervo E, Yezierski RP. Relationships among clinical characteristics of chronic pain after spinal cord injury. Arch Phys Med Rehabil. 2001;82:1191-1197. 7. Widerstrm-Noga EG, Turk DC. Types and effectiveness of treatments used by people with chronic pain associated with spinal cord injuries: influence of pain and psychosocial characteristics. Spinal Cord. 2003;41:600-609. 8. Amador MJ, Lynne CM, Brackett NL. A Guide and Resource Directory to Male Fertility Following Spinal Cord Injury/dysfunction. Miami: Miami Project to Cure Paralysis; 2000. 9. Sipski ML, Alexander CJ, Rosen R. Sexual arousal and orgasm in women: effects of spinal cord injury. Ann Neurol. 2001;49:35-44. 10. Goldshmit Y, Galea MP, Wise G, et al. Axonal regeneration and lack of astrocytic gliosis in EphA4-deficient mice. J Neurosci. 2004;24:10064-10073.