Medical Update (2001)

Volume1/2001_SummerUNIVERSITY OF UTAH HOSPITALS & CLINICS " I love you; get in the car " Parents Must Act When Kids Need Psychiatric Help Apsychiatrist who treats children and adoles-cents might not generally see his work as a " fun" experience. Douglas D. Gray, M. D, however, is optimistic about modern treatments for his young patients. " My job is great because I see kids who haven't been able to go to school or who have been hospital-ized. They improve and I see them where they should be- in school, with their families and active with their friends," he says. Gray, an assistant professor in the University of Utah medical school's Department of Psychiatry, and adjunct assistant professor in the Department of Pediatrics, is a member of a group of academic practitioners in the Child, Adolescent, and Young Adult Specialty Clinic. His specialty is severe mood disorders, including bipolar disorder. According to Gray, many new medications have become available for these illnesses during the past several decades; if one thing doesn't work, there are many other choices. In contrast, he points out, in the " old days, there were few medications, which were somewhat effective, but if they were not, most of the time there was nothing else to turn to. New medications have very few side effects, and most patients get well using them." A young woman patient is emphatic about the importance of medication, which she takes daily. Samantha ( not her real name), now 20, explains that she has taken " every type of medication" and understands the process. " It takes a few weeks for new medications to take effect and to allow evalua- University of Utah Child, Adolescent and Young Adult Specialty Clinic 801- 585- 1212 Bonnie Flick, M. D., Douglas Gray, M. D., Janet Lainhardt, M. D., William McMahon, M. D., Anne O'Melia, M. D., Sally Ozonoff, Ph. D. Specialties include autism, mood disorders, anxiety disorders, eating disorders, Asperger's Disorder and Tourette's Syndrome. Douglas D. Gray, M. D., finds joy in his work when young patients improve, become active again. tion of their effectiveness. Finding the right medication can be a very, very long and frustrating process," she says. Because the medication regimen must be continuous and regular, Samantha wishes that drug patches or implantable time- release devices were available to ensure compliance, which can be critical in psychiatric illness. Samantha's mother was concerned that her daughter was depressed and sobbed uncontrollably for no apparent reason as a young child. Counseling for Samantha didn't help. When she finally went to an inpatient program, she was put on medication, which helped her immensely, almost immediately. As Samantha got older, bipolar disorder developed and she would have as many as six highs and lows in an hour, going from hysterical laughter to uncontrollable sobbing. In her manic state, she could not sleep and she and her dad would run laps around the block in the middle of the night. When the family moved to Utah in 1992, Samantha was referred to Gray by a pediatrician and, after trying various medications, she's now on a combination that helps her. There's no cure; just treatment, Samantha says. " It's difficult to see most people around you acting normal and having a good time, while you are feeling so sad. It's sort of an ethereal illness," she says of her depres-sion. " You can't see it, touch it, but you can feel it. You know something's there. You know there's no reason for you to be feeling bad. It's hard to tell when you'll be upset; sometimes you'll be feeling pretty good, but a little thing can set off the depres-sion." Samantha says her illness involves a lot of fear and takes away her emotional control. In a closed envi-ronment, like school, she was fearful of breaking into uncontrollable sobs. " You don't want your peers to see you out of control like that," she said. Being unable to cope with school, she had home-hospital schooling during junior and high school, and also attended night school to get her high school diploma. Both Samantha and her mother talk about the stigma attached to psychiatric illness and the " unfair-ness" of having many people- even health- care professionals- view the problem as behavioral, rather than as a medical/ biological illness. Another of Gray's patients, John Turner, 19, and his mother, Marilyn Perkins, credit Gray with keeping John well and moving forward. John's depression started in 1990, about a year after he was critically injured when a car hit him while he was bike riding. He had a closed head injury and was in a coma for two weeks. Two days after the accident, he had a stroke, which subsequently caused blindness. He went to the Utah Schools for The Deaf & The Blind to learn Braille and was mainstreamed in the third grade. Last year, John graduated with honors from Hillcrest High School, with letters in academics and drama. This past year he took a class at Salt Lake Community College. Although he has regained some sight, it is not enough for reading print; nevertheless, reading Braille is a favorite activity. For school, he uses audio- tape texts and a computer that has a Braille keyboard and prints out his assignments in regular text. John is enthusiastic about acting. He appeared as a featured extra in an episode of Touched by An Angel, and had a speaking part in Breaking Free, a Disney movie. On this occasion, he had his own dressing room on the set. To treat his mood disorder, John takes an anti-depressant and also a stimulant to counteract leth-argy. It's important to recognize when kids need help, Gray said. The symptoms of serious mood disorders often show up at puberty. Illness can appear in younger children, usually when such disorders run in both sides of the family. All practitioners in the Child, Adolescent and Young Adult Specialty Clinic see any child or adoles-cent with problems. Many patients are " harder cases," referred by community physicians. Patients with Utah ties come to the clinic from the east and west coasts and from as far away as South America and Japan. The most important thing is to determine whether the disorder is biological or psychosocial; that is, caused by social stress. The latter category of conditions is treated not necessarily with medication, Gray explained, but with a variety of psychotherapies. " We recommend several treatment options after a diagnosis," Gray said. " We are very parent- oriented and present options to the parents. The child is in-cluded in the consult: the parent is the decision- maker, the child is a participant." For out- of- town patients, physicians may work out a treatment plan for the child's pediatrician and then serve as consultant. It is important to distinguish between teen rebellion and psychiatric disorders, Gray said. " Rebellious kids argue with parents, slam doors, but they enjoy school and friends and do stuff- they are active. " Children with mood disorders are irritable and unhappy most of the time. They may sleep a lot, have low energy or fatigue, may binge or stop eating, and may have suicidal thoughts." Gray, who runs the Utah Youth Suicide Prevention Task Force, says that the suicide rate has doubled, tripled and quadrupled in past decades. In Utah, suicide is the leading cause of death among males 15- 44. What does a parent do if a child needs help but doesn't want to see a doctor? " Do what you'd do if Continued on page 5 3 A serious accident when he was seven led to depression for John Turner. He s enthusiastic about acting, is an avid reader. Muscular Dystrophy Research MDA Clinic Coordinates Specialty Patient Services Kevin Flanigan, M. D., whose research focuses on genetic features of FSHD, hopes to discover why muscles atrophy and weaken. The high incidence of facioscapulohumeral dystrophy ( FSHD) in Utah and Idaho has been a focus of attention at the University of Utah School of Medicine for many years. Fifty years ago, a study of a large family affected with FSHD was reported by Frank H. Tyler, M. D., and Fayette E. Stephens, Ph. D., early medical school faculty. They traced 1,249 descendants of an Englishman who came to Utah in 1847. Today that pedigree includes 2,200 people and members of this originally reported family account for half of the FSHD patients seen in the Muscular Dystrophy Associa-tion ( MDA) clinic at University of Utah Hospital, or enrolled in studies of the disease. Kevin M. Flanigan, M. D., assistant professor of neurology and adjunct assistant professor of pathology and human genetics, is studying the genetic features of the disease. FSHD is the third most com-mon form of muscular dystrophy, which refers to a set of inherited disorders that lead to varying degrees of muscle weakness and, in some cases, neurological damage. Some patients are affected markedly; other cases are so mild that it is sometimes difficult to recognize. Patients may have shoulder weakness and pain, symptoms that could mean several other things, according to Flanigan. Other symptoms are asymmetric pucker of the lips and a weakness of biceps and triceps. " There is some debate as to whether the disease becomes more severe from generation to genera-tion, and whether it is more severe if it is inherited from the mother rather than the father," said Flanigan. " If this is true, it implies novel molecular mechanisms are at work. Our ongoing studies may answer these questions." A progressive disease, FSHD typically shows up in the late teens or early 20s, but may show up as early as 10 and as late as 60. Salt Laker Janet Kennedy, now 47, was four when she was tested after her mother was diagnosed with the disease. Of the five siblings in her family, she and two brothers have FSHD. Both her son and daughter are affected. She remembers the progression of symptoms when she was the ninth grade and was on the gymnas-tics team. She complained that she couldn't do two- handed cartwheels, couldn't keep her arm up over her head and couldn't pucker her lips to whistle. Despite these hindrances, she played high school sports and in the early 80s was on the Brigham Young University ballroom dance team. " I loved to dance. Everyone said I couldn't do it, shouldn't do it, but I went ahead," Kennedy said. She could dance if she had a good leader and good support, but at one dance she fell twice doing the cha- cha and a swing dance. That experience ended her dancing. " I think it's harder when you've been able to do it all and know what you're missing," she says. Now she can't walk from one end of a mall to the other and has to be careful of others; if someone bumps her she will fall down. Although she must go up and down six stairs or so to her second-floor apartment, she loves her place because she has a balcony where she can sit and enjoy her flower boxes. She does get out, driving or using her motorized scooter to go to the library and out for ' walks. Because both her children live out of state, she also has learned to call on her friends when she needs help. Her main activity now is doing counted cross- stitch embroidery on linen and giving away the framed beautiful mementos for special occasions. Kennedy, who's been a Univer-sity patient since 1977, goes to the MDA clinic regularly because it gives her hope. " That's the thing about Dr. Flanigan and the re-search. I know they're doing something with the information they're accumulating that may help future patients- including my children," she said. The MDA clinic, which serves the entire Intermountain area, provides patients such as Kennedy with access to specialty care. This includes physical therapy, occupa-tional therapy, genetic testing, counseling, supportive care and updates on educational develop-ments. These coordinated services help patients minimize the medical and social complications of this chronic condition, said Flanigan. There's no treatment that's been shown to alter the disease course, so Kennedy treats herself with pain relievers and some massage therapy. According to Flanigan, there was early interest about the use of the asthma medication Albuterol to slow disease progression, but a recent trial hasn't borne out the initial enthusiasm for use of the drug for FSHD. FSHD is associated with the On the cover. . . Salt Laker Janet Kennedy is hopeful that FSHD research will help her children, who also have muscular dystrophy. deletion of chromosome 4, but unlike essentially every other human disease, the deletion doesn't result in the removal of a gene. " Currently, the favored hypothesis is that this deletion alters the DNA structure such that the function of nearby genes is altered," said Flanigan. I he Utah researchers are comparing gene expression from muscle biopsy specimens from affected and relatively unaffected muscles in FSHD patients. Some altered genes have been identified and they expect studies to show why muscles atrophy and weaken. Flanigan and his associates also are studying Duchenne's muscular dystrophy, the most common form of muscular dystrophy, which affects boys and typically leads to death by the mid- 20s. In some boys, the condition is due to a mutation causing a premature stop signal, halting normal translation of the dystrophin gene. They're working to develop a way to allow rapid, accurate and inexpensive identification of premature stop codon mutations in patients. Because the dystrophin gene is the largest known, this presents a significant challenge, Flanigan said. " It has historically been tedious, time consuming and expensive to find individual mutations, but we think we have found a better method." This fall, many forms of muscle disease will be discussed during proceedings of the 6t h International Congress of the World Muscle Society, which Flanigan will host at Snowbird, Utah, in September. © For more information on muscu-lar dystrophy research, call Kevin Flanigan, M. D., 801- 587- 9540; For clinical programs, call 801- 585- 5885. Psychiatric Help Continued from page 3 grandpa were having a heart attack and refused to go to the hospital- put him in the car and take him to get help. Say ' I love you; get in the car. Children at risk need to have someone around to monitor them, to interact with them. Parents should consider that kids with acute psychiatric disorders are no differ-ent than those with other medical illnesses. If parents work, they may need to take a leave. Public agencies serve only a small percentage of the children who need help, and insurers have dropped coverage for such assis-tance, Gray said. " That's unfortu-nate, because with good treatment these kids can become productive members of society." For insured individuals, the co-pay for an outpatient visit for a physical problem is $ 15; it can be $ 50 for a psychiatric visit, Gray said. " Insurers will not cover autism, Tourette's Syndrome, or learning disabilities. Until mental health acquires parity with physical health in insurance, the mental health crisis will continue." © For more information, call Douglas Gray, M. D, 801- 585- 1212. University of Utah Hospitals & Clinics toll- free referral and consultation numbers: in Utah 1- 800- 662- 0052 outside Utah 1- 800- 453- 0122 www. med. utah. edu 5 Surgery for GERD Reflux Procedure Proves Fast Track to Recovery The gun fired and the pack was off. Cynthia Martin helped set the pace. Earlier this summer Martin pushed her limits further than she ever has before- in a 50- mile mountain race on the trails of Utah's Provo Canyon. Although Martin, a clinical research coordinator in infectious diseases at University Hospital, is familiar with the physical demands and punishment that accompany endurance races, she now runs free of unremitting pain that has plagued her most of her life. A Bountiful, Utah, native, Martin is among 60 million Ameri-cans who suffer heartburn and acid indigestion caused by gastroesoph-ageal reflux disease ( GERD). Until a few years ago, she lived with constant stomach discomfort. " Growing up, I thought it was normal to have a stomachache every night," she said. " I couldn't run for long periods of time because I couldn't eat or drink anything without throwing up." GERD results when the lower esophageal sphincter, a group of muscles above the stomach that normally tightens and loosens to allow food to pass into the stomach, spontaneously relaxes, causing a backflow of stomach acid into the esophagus. Frequent and persistent heart-burn, recognized by a rising burning sensation behind the breastbone, is the most common symptom of GERD. Another symptom may include an acidic or sour aftertaste in the mouth. Although minor GERD symp-toms can be controlled by lifestyle, diet changes and medication, chronic symptoms are sometimes Surgery for GERD helped Cynthia Martin pursue her love for marathons and triathlons without worry about pain. best treated by surgery, said James M. McGreevy, M. D. professor of surgery at the University of Utah's School of Medicine. " If left untreated, chronic GERD can lead to a more serious condition known as erosive esophagitis, a narrowing or ulceration of the esophagus," he said. People with reoccurring reflux over many years increase their risk of cancer of the esophagus, al-though no results suggest that correcting GERD reduces these risks, he added. Martin said she took medication three times a day to control her reflux. When she heard about the surgical treatment option, she did the math. Three $ 2 pills a day for the rest of her life would be a big expense for this 32- year- old wife and mother. Martin saw the benefits. " It wasn't to improve my athletic performance," she said of her decision to have surgery. " It was to improve my quality of life. I was looking forward to not taking medication every day." GERD surgery uses a fundoplication, a procedure which wraps the upper part of the stomach around itself to create a new pressure point that functions like the lower esophageal sphincter, allowing food into the stomach and preventing acid reflux. Fundoplication is done by either opening the abdomen or by using a James M. McGreevy, M. D. says patients opt for laparoscopic surgery for quick recovery. Potential surgery patients must meet criteria in evaluation by gastroenterologist. laparoscope, allowing surgeons to guide small medical instruments and a camera through small inci-sions in the stomach. University Hospital surgeons started using the Nissen laparoscopic procedure for GERD in 1994. The procedure has become the standard surgical correction of the reflux disease throughout the country, according to McGreevy. " Laparoscopic surgery for GERD is more patient- friendly," said McGreevy. " It is less painful, allows patients to return to their normal activities quicker, and costs less than open surgery." " The big advantage is the recovery time. Normal hospital stay for the laparoscopic procedure is two days and sometimes one. Open surgery would keep a patient in for five to six days." Not only was Martin out of the hospital quickly after her surgery; she was running again within five days. " A year later I ran the St. George Marathon eight minutes faster than I ever have before," she marveled. McGreevy said many potential patients are attracted to the surgery because of the post- operative recovery time. He estimates that the hospital sees about 1 5 potential patients each month. GERD surgery shouldn't be considered a quick fix for acid indigestion, however, McGreevy cautioned, because nearly 80 percent of reflux patients can be managed with medication. " The surgery is considered a major operation," he said, " and serious complications, like esoph-ageal or gestric perforation causing peritonitis, and even death can follow." A less serious and some-times common side effect is that after a period of several years the esophagus loosens and the GERD then reoccurs. Surgery can be performed again if the patient chooses to do so. Another side effect is that, sometimes, the procedure hampers swallowing and must be loosened. Potential surgery patients must meet certain criteria, determined in an evaluation by a gastroenterolo-gist, to check esophageal motility and to determine the level of esophageal acid. If medication does not help the patient and the individual meets the criteria, surgery can be the next option. Martin said it was the best thing she has could have done. " Even if the procedure stops working in ten years, I would do it again." For this passionate runner, slowing GERD gave Martin a chance at the mountain race and several marathons including the St. George and Boston marathon, which never would have been on her running resume three years ago. It also gave her a better life. © For more information, contact James M. McGreevy, M. D., 801- 581- 4488. University of Utah School of Medicine Continuing Medical Education Oct. 5- 6: Western Intermountain Neurological Organization Salt Lake City Marriott- University Park Fee: $ 125 8 AMA Category 1 hours For conference information, call University Conference Services, 801- 587- 2980, or toll free, 877- 412- 8159 For program details, call 801- 585- 5405. Clinical Study Participants are sought for a Phase III Stress Urinary Incontinence ( SUI) Study to test the safety and effectiveness of an investigational drug. Women, 18 or older, who are in general good health and have experienced occasional leakage of small amounts of urine when they cough, sneeze, laugh or exercise for at least three months, may be eligible to join the study. Participants must be able to use a toilet independently and perform simple exercises, such as walking, bending, climbing stairs and running in place. This is a randomized clinical study with participants assigned by chance to one of two groups. One will receive the study drug; the other will r