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1 TRANSCRIPT Detection of Suspected Neuroleptic Malignant Syndrome: Three Case Examples The following transcript does not include graphics that appear on-screen throughout the program. 2 INTRODUCTION NARRATOR: Worldwide, a significant number of people are taking neuroleptic medications as part of treatment regimens for schizophrenia, schizoaffective disorder, bi-polar disorder, and clinical depression. In addition, large numbers of the developmentally disabled and elderly are also taking neuroleptics. DSM4 reports that .07% to 1.4% of those taking neuroleptics will suffer a toxic reaction to neuroleptic medication, referred to as neuroleptic malignant syndrome, or NMS. NMS may be fatal for some patients, while other patients who survive may suffer permanent harm. However, with early detection, fatalities can be prevented and the incidence of permanent harm can be reduced. In Case Example 1, the patient died approximately 27 hours after administration of neuroleptic medication. In Case Example 2, the patient died about 52 hours after receiving neuroleptic medication. In Case Example 3, the patient recovered after a severe course of the syndrome. DSM IV reports that the presentation and course of neuroleptic malignant syndrome are quite variable. To detect suspected NMS, some clinicians consider that there are four categories of signs and symptoms to closely monitor. Unstable Vital Signs Rigidity Confusion Lab Abnormalities It is thought that when NMS occurs, the hypothalamus (the organ regulating vital signs) is destabilized by neuroleptic medication, triggering dysregulation of the autonomic system; blood pressure, respiration, pulse and temperature. Profuse sweating or diaphoreses is a common and easily identifiable sign. Rigidity occurs on a continuum from some rigidity to lead pipe rigidity. Though in some cases, rigidity is not reported. Significant confusion, or altered or impaired consciousness is another of the common early presenting signs. Abnormal laboratory findings constitute a secondary level of signs, indicating more severe stages of NMS. One key laboratory abnormality occurs with creatine phosphokinate or CPK. A blood test for increased CPK measures muscle damage caused by rigidity. There are other significant laboratory abnormalities that can also occur. CASE EXAMPLE 1 In case example one, Christine Patchen, 23, had been diagnosed with schizophrenia three years earlier and had been under a neuroleptic medication regimen. She had been successfully working and going to school and had completed a secretarial course shortly before she was hospitalized. CHRISTINE’S MOM Christine had worked for YWCA for about a year, doing a lot of computer work for them. She would sign up all the new entries coming into the YWCA. Christine also worked for the animal 3 shelter in the City of Moreno Valley. She wrote all their bylaws and when they first opened and she worked for City of Moreno Valley for the Emergency Operation Unit. NARRATOR: Prior to hospitalization, Christine had not been taking her regular neuroleptic. She had a loud argument with her ex-boyfriend and the police were called. The police reported that Christine and disruptive and confused about events. She was placed on a 72-hour involuntary hold while in an acutely agitated state and then admitted to a county psychiatric unit. After admission, Christine received a physical. Her blood pressure of 120 over 80 was within normal range. Her pulse was elevated at 116. Her temperature was at the high end of normal ranges for her age group, 99.9. After receiving intramuscular Haloperidol, Christine developed signs and symptoms typical of NMS. Unstable vital signs, including elevated blood pressure of 142 over 112, and an unusually elevated pulse of 152, profuse sweating or diaphoreses, and the appearance of significant confusion or impaired consciousness. Christine died approximately 27 hours after the first administration of Haloperidol. The county coroner’s report indicated cause of death was cardiopulmonary arrest associated with haloperidol therapy for schizophrenia while the hospitals own emergency department report indicated that death was caused by cardiac arrest and possible neuroleptic malignant syndrome. Much can be learned about prevention of fatalities from NMS by reviewing the details of cases such as Christine Patchen’s. Christine’s medication history is important to consider. Two years before this hospitalization, Christine had an extremely severe adverse reaction to oral Haloperidol. CHRISTINE’S MOM: She was uncapable of her body movement. It was completely rigid. Her arms she couldn’t put down to her side. She had to keep them up bent up to her side. She was unable to actually bathe herself. I had to bathe her. She was uncapable of brushing her teeth, and I brushed her teeth for her. At that time I knew the medication was too strong. NARRATOR: The adverse reaction with extreme rigidity described by Christine’s mother may have involved parkinsonian symptoms, dystonia, other EPS, or possible the early onset of NMS. At that time Christine’s psychiatrist withdrew her from Haldol and informed her mother that Haldol and informed her mother that Haldol could be life threatening. He prescribed another neuroleptic that was well tolerated by Christine. A medical record stated, “Stop the Haldol and congentin. Take the new meds as prescribed. Return if you have any problems.” This aftercare instruction sheet had become part of Christine’s medical records in the county where she was hospitalized. Concerned about her daughter’s welfare, Helen Patchen telephoned the hospital and told the head nurse and physician on-call about Christine’s prior adverse reaction to Haloperidol. CHRISTINE’S MOM: I called on the phone and let them know that she had complications with Haldol, and I did not want her on Haldol, and I read off the bottle what type of medication she was on, and the 4 milligrams that were on that bottle. NARRATOR: The medical records did not show that the staff had followed up on the report of an adverse reaction to Haldol, by reviewing past medical records or contacting Christine’s outpatient psychiatrist. BRUCE VICTOR, MD: The fact that Helen Patchen called the hospital and informed some of the personnel that Christine had had terrible reactions to Haldol should have been taken seriously by the medical personnel there. NARRATOR: The FDA approved product insert sheet warning provides a significant guideline: Haldol is contraindicated for individuals who are hypersensitive to this drug. Without a medication history, a prudent course might have been to administer a small dose of Haldol and test for sensitivity. However, at 9:15 AM on Saturday, Christine was given 10 mg of an intramuscular injection of haliperidol, placing her at increased risk for NMS. By 8:00 PM that evening, approximately 11 hours after the first injection of haliperidol, Christine’s pulse was 114, down from 116 and her blood pressure changed from 120 over 80, to 132 over 68. Her temperature had risen slightly to 101.1, but a blood test had previously ruled out other medical conditions. Apparently, to treat the signs and symptoms of psychosis, Christine was given another 10 mg of haliperidol at 10 PM. By 11 PM that evening, Christine’s temperature had risen to 101, but the staff failed to report her blood pressure, pulse, or respiration. Frequent monitoring of all vital signs is crucial for tracking the possible development of NMS. SHIRLEY WATKINS, JD: The issue for me, as the lawyer for the family, was that it wasn’t as important as whether or not she had NMS as whether or not the hospital recognized that she had symptoms which reflected that she might possibly have NMS… and take steps to treat the symptoms. NARRATOR: By 6:30 AM the next morning the progress notes state: “Christine had slept the night with her entire body and head covered, was selectively mute, was extremely bizarre, hiding under bed, seems very frightened.” T these observations suggest significant confusion. However, there was no indication that the staff evaluated whether or not Christine might have been suffering from altered consciousness. 5 BRUCE VICTOR MD: There are various diagnostic clues to the assessment of altered consciousness. So, if in fact somebody asked Christine Patchen where she was, who she was, what day it was, was she hiding under her bed because the voices were getting too loud, was she hiding under her bed because she didn’t think it was a bed at all.” NARRATOR: By 6:30 AM Christine’s acute agitation had subsided, but her blood pressure had risen alarmingly, from 132 over 68 to 142 over 112. Christine’s pulse had risen from 114 to a dangerously high 152 for a resting patient. With a pulse over 150 and unstable vital signs a doctor should have seen Christine promptly. A hospital internal policy required that once a patient has a sustained pulse of over 150 the patient should be moved to the emergency room. However, a doctor did not see Christine at that time. Christine was not moved to emergency. The 6:30 AM progress notes had also stated, “Unable to obtain temperature,” without further explanation. SHIRLEY WATKINS, JD: The doctor for the family had some substantial criticism of the inability, according to the nurse, to take the temperature. It just doesn’t make sense that they could take the blood pressure but not take the temperature. And obviously there was a lack of understanding of how important it was to take the temperature. NARRATOR: Some patients resist having their temperature taken with an oral thermometer. However, it might have been possible to take the temperature with a tympanic thermometer, inserted in the ear. Especially since in produces a temperature reading within a second and tracks core body temperature more accurately than an oral thermometer. Christine was examined by a psychiatrist at 8 AM, 90 minutes after her pulse of 152 was recorded. However, no vital signs were taken at the 8 AM visit. The record does show: “patient is lethargic… confused… mumbling to self” -- suggesting significant confusion. The examining psychiatrist placed Christine on a 14-day hold and then left. Two-and-a-half hours later Christine was seen an internist from emergency. SHIRLEY WATKINS, JD What’s interesting is that the internist who saw her at 10:30 in the morning, about an hour and a half before she was found, testified that he believed that her sweating her sweating, the diaphoreses, and that her elevated pulse and her blood pressure which had been elevated earlier, were autonomic responses due to the fact that she was hallucinating and hearing voices. BRUCE VICTOR, MD: Now, at 10:30, in the face of all of these medial symptoms, Dr. A comes in… decides that this is all attributable to schizophrenia, and in essence writes it off as psychiatric symptomotology, rather than as real medical disease, induced by a very potent medication… 6 SHIRLEY WATKINS, JD The hospital’s doctor testified that you must have a temperature in excess of 103, in order for there to be a diagnosis of NMS. The doctor for the family testified that the temperature’s only one thing you look at in the constellation of symptoms that turn into a diagnosis of NMS. The temperature does not have to be that high, or that elevated, in order for there to be a diagnosis. The point is that the hospital staff is trained what the symptoms are of NMS. Once they see a pattern developing, of abnormal symptoms, diaphoreses, temperature, pulse, respiration, that can alert them to the fact that treatment is necessary, supportive treatment is necessary, in order to prevent the temperature from getting elevated at a later time. NARRATOR: One of the hallmark signs of suspected NMS was reported at 10:30 AM when the chief of emergency visited Christine and found her diaphoretic… a condition that commonly reflects increased temperature… yet no one took her temperature, pulse or other vital signs at that time. The autopsy report also confirmed, “Diaphoretic, Pre-Terminal.” During hospitalization, Christine was observable on a TV monitor in a nearby nurse’s station close to her room. However, when someone went in to wake Christine for lunch it was discovered that she had no blood pressure, pulse, or respiration. A crash cart was required on this unit but no crash cart was obtainable. HELEN PATCHEN: On the morning that I was called… they asked if I was a parent of Christine Patchen and I said yes… and I was told to come on into the conference room… and at the conference room there was two nurses that were there… that talked to husband and myself… and… they said that they had found Christine, laying face down in a pillow… and they turned her over and tried to resuscitate her.. and found her blue… and could not resuscitate her… and she died of a full cardiac arrest… SHIRLEY WATKINS, JD: The point of the family’s doctor is that she had very definite abnormal vital signs and other symptoms that showed over the course of that morning that something was going on, and that had the hospital staff recognized that there was this collection of symptoms, which showed that there was something going on, she could have been treated and would have survived. HELEN PATCHEN: I’ve thought about the effect of Christine in my life… and how things could have been and how things are now… she was every bit a part of my life… but life is now and I have to live it now… she’s not with me so I have to remember her… You take from just goin’ into the store… seeing things that you used to buy for your daughter… things that everybody takes for granted… to having holidays where other people having family around… to not having that… to friends sitting there knowing the hurt that you’re having… and continuing to tell you that they’re sorry… for asking about your daughter… she was my life… 7 NARRATOR: To review key points, the staff failed to obtain a medical history of Christine and failed to fully consider a past severe reaction to Haldol reported by her mother. However, NMS could have been caused by any neuroleptic. Careful documentation of complete vital signs should have led to appropriate medical intervention. Under the hospitals own policy, with a pulse of 152, Christine should have been transferred to emergency for a complete medical work up and appropriate treatment. In conclusion, with regular monitoring and prompt intervention, Christine Patchen’s death might have been prevented. Following Christine’s death, improvements in the detection of NMS were made at this hospital. STATEMENT FROM HOSPITAL MEDICAL DIRECTOR: We have increased staff awareness of NMS; its manifestations and risk factors. We are monitoring vital signs much more closely. We are providing much more thorough medical evaluations and ongoing care for all patients. CASE EXAMPLE 2 NARRATOR: When Neil Sherr was 8-years-old his family found he had a learning disability. Neil participated in cub scouts and boy scouts. He tried hard to emulate to the achievements of his three older brothers and was able to graduate with his high school class on time. Diagnosed with schizophrenia at age 17, and treated with medication over a period of 6 years, Neil Sherr suffered a number of adverse reactions, but he functioned appreciably better when he was taking a neuroleptic, Perphenazine, that was well tolerated. Prior to this hospitalization, however, his family reported that Neil had not been taking his neuroleptic for approximately 6 months. He had been suffering extreme difficulties in daily functioning. NEIL’S BROTHER GILBERT: I remember that after moved back into my parents’ home, that often he would stay out at night and we didn’t know where he was. When it got to be November and December we started worrying because it was getting cold and started to freeze at night. We were very worried that a calamity may happen, that he may be out and freeze to death. NARRATOR: Neil’s brother Gilbert persuaded Neil to seek medical help. They went to the psychiatric hospital where Neil had previously been an in-patient for one-year. Although Gilbert was unaware of the names of the medications that had previously caused adverse reactions, he wanted to convey his concerns about the past adverse reactions to the admitting doctor. 8 GILBERT SHERR: I asked him whatever you give someone else you have to give Neil one-tenth that amount because he’s very very sensitive to medication, he reactions very strongly to them. NEIL’S BROTHER LAURENCE: When Gilbert got back from the hospital that day one of the first things we did was to sit down together as a family. Gilbert, myself, my mother, and my father. We wanted to be sure that Neil wasn’t given the wrong kind of medication, or that he wasn’t overmedicated, because we didn’t want him to obviously suffer any needless discomfort and we didn’t want him to feel reluctance to accept treatment there. We wanted him to accept treatment and get better. So we called the Admissions Unit, Gilbert and I went to the phone, he called, I was standing there, he wrote down right there on the page the name of the hospital employee that he spoke to… He read this list that we had made to the hospital employee and asked him to be sure that the information would be relayed to those who would be taking care of Neil. He reassured Gilbert that he indeed would get the information to those people who would be treating Neil. NARRATOR: Neil arrived at Unit 5 at Friday at 8:30 PM. While the hospital policy required that a history and physical be conducted within 24 hours of admission, no physical was conducted. The chart does not indicate that the staff examined Neil’s past medical records at this hospital, specifying medication allergies and adverse reactions. Also, the chart did not reflect any conversations with the family about Neil’s past medication reactions. JAMES CHEATHAM, MD: He had been a patient in this particular hospital for about a year. There had been very ample documentation about past problems with medication. As I recall there were some eleven (11) different instances where patient had had adverse reactions to medication. His former psychiatrist had visited the hospital or had contacted the staff at the hospital to advise that he’d had reactions to Haldol, Prolixin, Stelazine. Some of the forms in the chart itself indicated quotes“ an allergy” to Stelazine and to Prolixin. He’d had one episode of “opisthotonus” which is one of the presenting symptoms of malignant syndrome, neuroleptic malignant syndrome. That had been totally overlooked. So there was a wealth of data available about this man. NARRATOR: The medication administration record indicates that Haldol was to be administered at 9:30 PM. A nursing assessment, completed at 10:30 PM, showed that Neil was experiencing EPS, severe cog wheeling and profuse sweating. Profuse sweating, or diaphoreses, is one of the key signs suggesting NMS. About 2 hours after Neil was first observed with EPS, he was cog wheeling, drooling at the mouth, sweating profusely. JAMES CHEATHAM, MD: It would have been appropriate at that time to have insisted that the doctor on duty see and examine him and move him, direct him, direct his movement to a medical/surgical hospital… a hospital equipped with the facilities and the resources to manage someone in his condition. NARRATOR: Neil’s mother spoke to a member of the staff by telephone on Saturday, to find out how Neil was doing. She was told that Neil would not be allowed visitors on Saturday. The records show that 9 Neil was “twitching, drooling and having body contortions,” suggesting that Neil was suffering from seizures. JAMES CHEATHAM, MD: There was no monitoring of temperature… there was no monitoring of his physical reactions: rigidity. No checking of his vital signs. NARRATOR: The chart indicates Neil received a 10 MG dose of Haloperidol in the evening. By Sunday morning, Neil was arching his back, making gagging noises and hyperventilating. The nurse writes: “behavior appears to be self-induced,” and gives Neil a 5 MG injection of haloperidol. However, an intramuscular injection accelerates the progression of NMS more than an oral dose. JAMES CHEATHAM, MD: What struck me most were the comments that he’s malingering, he’s attention seeking, he’s putting on an act… these are very often the observations that inexperienced staff members make, or staff members who are feeling overburdened and overextended. And they’re also the kind of observations that lead to one not really paying attention with what’s going on with the patient. What we’re seeing with Neil is behavior that was brought about by the Haldol that he’d been administered. NARRATOR: Neil’s brother Gilbert and his father Saul went to visit Neil on Sunday. They were told that Neil was in seclusion on the quiet side and could not be visited. GILBERT SHERR: But I said why is Neil on the quiet side and she said well, he’s disturbing the other patients, he’s acting out and he’s faking seizures. LAURENCE SHERR: I became very concerned when they told us that they had been told that Neil was faking seizures. I couldn’t reason how a person could fake seizures, didn’t understand that. What I thought of in my own mind at that time was that Neil was possibly having an adverse reaction medication and that possibly he could have exaggerated to get some attention to let people know that he was having some problems. At that time we did not know about NMS we had no idea that this could have been a life-threatening situation. NARRATOR: The 6:30 PM medical chart indicated that Neil had been having seizure-like activity most of the tour. The chart stated that he had a flushed face, and jerking of extremities lasting approximately 30 seconds. The medical record showed that a nurse called for an OD three times but got no result. JAMES CHEATHAM, MD: The doctor did come to the unit, but came to the unit to see another patient… she then refused to see Neil. 10 NARRATOR: After hospitalization for just two days, Neil appears to be reaching the final stages of NMS. JAMES CHEATHAM, MD: When we look at the end stage, we’re looking at a variety of things happening. One, prolonged hypothermia, which leads to extreme dehydration, and we have no indication that he was being adequately hydrated. We’re looking at things like breakdown of muscle tissue, which in turn leads to the release of hemoglobin from the muscles, blocking the kidneys, leading to acute to renal failure. We’re also leading to exhaustion, as we noted before, Neil was having difficulty breathing, laryngeal spasms, the twitching, the seizure-like activity, all of that further compromises his ability to breathe… so that setting the stage for respiratory failure and cardiac failure. NARRATOR: A nurse finds Neil in cardiac arrest and semi-conscious on the floor. He is seen by a doctor for the first-time since admission. No crash cart is available. Medical attempts to resuscitate Neil fail. LAURENCE SHERR: Later that night, just before two o’clock in the morning, we received a call from the hospital. My mother were simply told that Neil had taken a turn for the worse… and that they had to come down there… I waited for their call which came about half an hour later… they told me that Neil had died. NARRATOR: An autopsy report concluded that Neil died from a severe reaction to neuroleptic medication, of cardiac arrest from seizure disorder, associated with haloperidol drug therapy. SAUL SHERR, FATHER OF NEIL: I can only imagine… the anguish… the pain… the frustration he must have felt… being alone with nobody to help him… and the irony of it is that here he was in the hospital where he should have been safe… but he wasn’t. NARRATOR: In summary, with proper monitoring, early intervention and intensive treatment Neil Sherr would have had a chance to survive. After Neil’s death the hospital implemented policies for detection of suspected NMS. The Medical Superintendent for this hospital summarized the new policies. Each newly admitted patient receives a thorough physical with both routine and directed laboratory testing. Available family members and significant others are asked about the patient’s previous reactions to neuroleptics. Drug allergies are prominently displayed on the front of each patient’s chart. Special training for detection of suspected NMS is provided for staff. All reports of adverse reactions to drugs are reviewed monthly by the medical staff. 11 CASE EXAMPLE 3 NARRATOR: Valerie Van Schoick, age 33, had been diagnosed with schizoaffective disorder and had been on a continuous regimen of a neuroleptic, Stelazine, and other medications for an extended period of time. No changes in her medications had been made. This case example demonstrates how NMS can be successfully detected and treated. VALERIE’S MOM It was about 10 o’clock at night…the phone rang…I was in bed… scared me to death… always does. So I picked up the phone. It was a friend of my daughter, Valerie’s calling. Something is wrong with Valerie, I don’t know what to do, I can’t do anything with her… what should I do? I said I’ll be there right away. VALERIE: When my mother got there…I wasn’t making much sense… things were out of place…I was scared… it was like a nightmare I couldn’t get out of…. VALERIE’S MOM: Her apartment was a disaster… completely messed up… she’s so neat and clean it’s never that way… and she was acting very strangely, and stiff and at the same time sort of bouncing around and in and out of reality. I couldn’t make any sense of what she was doing… so we grabbed her, some clothes and came home. We didn’t get much sleep that night… Valerie didn’t sleep at all… she was up and down… we tried to get her to rest and she wouldn’t do it… she was getting stiffer and stiffer by the hour… When she was coherent, which wasn’t often, she was talking about death and destruction, blood all over the place, it was just awful. We got a hold of her psychiatrist, Mark Ragins, he said bring her in in the morning and that’s what we did. MARK RAGINS, MD: Valerie came to our office that morning with her parents in a very unusual state. She would times where she was very, very rigid, could hardly move… you’d help her walking… or she would drop a glass that was put in her hand… and then there would be periods where she would snap out of it suddenly and be absolutely normal. She could eat, or talk to you, or move about…and then it would suddenly snap back again, and be stuck. About a third of the time she was all right… and the other two thirds wasn’t. Sometimes, she would have a symptom called waxy flexibility, where you could take her arm and put it like this, and just leave it, and it would just stay there… suspended in mid-air for minutes… and then she would suddenly snap out of it and move around again. VALERIE’S MOM: We were in the doctor’s office that morning… for several hours… George and I with Valerie… she was getting stiffer and stiffer… I kept holding her and stroking her arm… and she’d come in and out of consciousness… it’s like she would leave for a moment… and she’d get a glassy stare… and then she’d come back and she’d say… Mom… you’re there… MARK RAGINS, MD: Since we were still faced with an acute, very dramatic presentation, we also did some tests looking for physical causes of it. We checked her pulse, her blood pressure, her temperature, we 12 also did a complete blood count and a chemistry panel which does include the CPK which is important for NMS. Nothing very dramatic was found with any of this… although Valerie did have rigidity and changes in her consciousness I did not think that this was neuroleptic malignant syndrome in the beginning because it was so variable, up and down, and there was no raised temperature with it. It was significantly different than the two cases I’ve seen previously in my life where the person was very rigid, extremely high temperatures, could hardly talk at all and was in that state very statically… they did not come and go the way Valerie did. NARRATOR: At this stage Val’s psychiatrist considered a number of possibilities in his differential diagnosis. The extent of the differential diagnosis indicates how NMS may be at first difficult to diagnosis. The severity of Val’s condition was recognized, but since Val hated hospitals a joint decision was made with Val’s family for her to spend the night at home with her parents. If her condition, her mother and father were prepared to take her immediately to the hospital. VALERIE’S MOM: When we got Valerie home I tried to get her to lie down and rest… go to bed… and she wouldn’t do it… she was up and down… getting stiffer… with that nightmare look in her eyes… and she, she would shudder and all of a sudden she began starting this terrible wailing sound… as if she was in the worse nightmare in the world… when she said anything it was always about blood, death and destruction… well that night I fixed her favorite dinner… she took one bite and she spit it out… apparently wasn’t swallowing… we didn’t realize at the time… and we couldn’t get her to take her neuroleptic because of that… and looking back on it that was a fortuitous event… VALERIE’S DAD: That night Valerie would be in her bed in her room for awhile and then she would come in to our room and just stand there and stare at us…After a while I decided that I would go into her room and lie on her bed with her and try to comfort her and make her feel better so she wouldn’t be so frightened… We tried to support her in as many ways as possible but nothing seemed to work. VALERIE’S MOM: By about one o’clock in the morning we knew that this was a desperate situation… Valerie was getting more and more rigid… now, I hadn’t taken her temperature because she didn’t feel hot… but I had noticed… I do know how to take a pulse reading and I noticed that her pulse was very rapid… and it was getting faster and faster as the hours were going by… and I’m thinking something terrible is happening with the stiffness… so I said we’ve got to get her to the hospital… and see what they can do for her… so George backed the car out of the garage… and it was all I could do to get her down the stairs… she was as stiff as board… every muscle completely rigid… to get her into the car took both of us… we put her in the front seat… I sat in the back seat… she screamed all the way to the hospital… screaming and crying in terror… she thought there was a war… the lights from the cars coming toward us were absolutely terrifying her… when we got to the hospital her pulse was even more rapid… we had to wait awhile but finally they took her up the fifth floor. MARK RAGINS, MD On Saturday, when I came to the hospital the internist had already been there… he thought she was suffering from EPS and gave her a shot of Benedryl, a treatment for that, but it had no effect. 13 I was still working on my trauma theory and gave her a shot of Ativan, an anti-anxiety agent but that had no effect as well. The other thing we were worried about… could this be toxicity from the medication, so we stopped all her medications at that time so they wouldn’t worsen anything that was happening. VALERIE’S MOM: She lay on the bed… catatonic… she didn’t make eye contact… she was still in a terrified state… she wouldn’t close her eyes… turns out later we find out that she thought that if she fell asleep she would die… By Sunday morning Valerie was doing much worse. She was rigid all the time. She couldn’t talk. She couldn’t swallow. She couldn’t move at all. We had to move her down to the medical ward; we put in an IV to hydrate her. We also gave her a 24-hour nurse to keep track of her and we also kept monitoring her vital signs and blood tests to make sure no irregularities came up. NARRATOR: A psychiatric unit is not equipped or staffed to treat the major medical emergencies that occur with NMS. Val was moved to a medical/surgical unit and IV hydration was begun. Valerie’s CPK lab test results rose sharply to 1560. Her pulse rate was high at 116. Her blood pressure at 150 over 100. VALERIE’S MOM: By the time I got back to the hospital on Sunday Valerie’s condition hadn’t changed… she still was lying there catatonic… not communicating, not responding… and I looked down at the table by her bed and there was a post-it there… and on it were three words I’d never seen before… neuroleptic malignant syndrome… with a question mark… so obviously during rounds doctors and nurses and been discussing her condition… so I left it there… but I did write it down… because I thought I am going to talk to her psychiatrist about this later on… and I did… and he said, yes, that’s what we think Valerie has, neuroleptic malignant syndrome… MARK RAGINS, MD: We gave Valerie’s family the same medical papers about NMS that we’d given to the staff to understand it… we wanted them to be educated and to understand what was going on… in the long run their familiarity with NMS would help considerably. VALERIE’S MOM: Well, I was glad at least to know what was the matter with Valerie. But her condition kept getting worse and worse… and uh… they had to put a catheter in… because she had no control over her bladder… and uh… they were giving her shots… and she developed double pneumonia… and uh, and Mark, her doctor, said she might not make it… and that was a terrible realization…so that’s when we called her sister and brother and said you’d better come home… Valerie may not make it… VALERIE’S DAD: When Valerie’s brother and sister flew in to see her she didn’t even know who they were… that’s how sick she was. And though we knew there was a chance that this illness could be fatal I never thought that would happen. 14 VALERIE: I’d been taken off my Stelazine which I had been on for many years… I was on IVs… they were doing shots in my stomach of Heparin, afraid of blood clots… and on the TV I was watching the President announce that the world was coming to an end… it was all a delusion. MARK RAGINS, MD: After withdrawal of her neuroleptic medication we gave Bromocryptine, this is a medication sometimes used for NMS. The neuroleptics tend to lower your dopamine and the Bromocryptine tends to raise it. So we think it has something to do with helping with this syndrome. NARRATOR: To recap -- by this time Valerie had become totally rigid, unresponsive and had developed diaphoreses. Intravenous rehydration was begun to counteract the diaphoreses. Valerie’s CPK level rose from 1560 to 1914, indicating increasing breakdown of muscle tissue. MARK RAGINS, MD: We also gave her dantroline. Dantroline is a muscle relaxant. We were giving it to decrease the risk of muscle breakdown products affecting her kidneys. At that point, neuroleptic malignant syndrome was pretty much the only diagnostic consideration. VALERIE’S MOM One morning when I went up to the unit I ran into the nurse… and I could tell… she… something was the matter she looked very shaken up. She said your daughter had suicidal ideations last night. And I said what and she said yes she got… pulled out all of her tubes and she tried to leap out the window and now she’s in restraints and we have her on 24-hour watch. VALERIE: I just remember because people told me what I did that night… that they said I was gonna jump out the window… and before I knew it there was security guards tied me in restraints to the bed and after that I looked around and everybody… the nurses looked like devils… and I guess I just wanted to end the pain and be at peace. MARK RAGINS, MD: After about 11 days of IVs and tube feedings her physical condition was getting considerably better…her muscles were still heavy but she could walk on her own and she could swallow and eat. She also had this pins and needles feeling in all of her limbs from the muscle breakdown. However, her psychiatric condition, because she had been without medication for so long, was much worse. She was very depressed and very paranoid and delusional. NARRATOR: After the last signs of the NMS had disappeared we waited another two weeks to make sure that it didn’t recur and then slowly began giving her medication again, titrating upwards very slowly and carefully and watching her vital signs and her blood tests the whole way. Her family was of considerable support to her during that period. 15 VALERIE’S MOM: It was a long process until Valerie was out of the woods medically and she’s have a good day and when I’m thinking oh this is terrific. She’d say I’m doing better mom and then the next day she’d be back in bed again. She wouldn’t communicate… wouldn’t talk… hated the world… and I think that in all the fifteen (15) years of her illness that was my lowest point… those days… she wouldn’t get out of bed again… you know, she lost 34 pounds during this whole process and I think I gained about as many… VALERIE: After all the horrible stuff that happened to me while I was in the hospital my PFC Wayne at the time had come in one day and said we could go outside. I thought that would be marvelous after being in the hospital so long with the tubes and everything in me… so he wheeled me out in the wheel chair since I wasn’t really steady yet to walk… cause of losing all the weight… so we went outside and there’s a fountain in front of the hospital and I had touched the water to my skin and it felt so refreshing… and just to breathe the air… it was like being born again. NARRATOR: Five weeks after entering the hospital Valerie was discharged. Here, Val is at her integrated service agency that provides a variety of services for its clients. By this time Val had two part-time jobs, helping care for a woman in her late 80s and assisting other consumers with their daily chores. CONCLUSION Comparing the three case examples highlights how to detect suspected NMS. Profuse sweating or diaphoreses is a frequent sign. Christine, Neil and Valerie all suffered from significant confusion or impaired consciousness. All three patients showed some unstable vital signs. Christine and Neil had prior adverse reactions to haloperidol, though all neuroleptics including the newer ones can trigger NMS. For Christine and Neil the signs and symptoms suggesting NMS occurred soon after the initial administration of a neuroleptic. Two-thirds of NMS cases develop within the first week of the introduction of a neuroleptic, according to DSM IV. Neil’s and Valerie’s charts confirmed extreme rigidity, but in Christine’s case rigidity was reportedly absent. No lab tests for NMS were conducted for Neil or Christine. Valerie’s CPK rose from 150 to 1, 914, confirming muscle damage from severe rigidity. Increased risk factors from NMS include prior adverse reactions, high potency neuroleptics, rapid increase in dosage, intramuscular injections, two neuroleptics combined, dehydration, physical exhaustion, and other organic brain disease. Some clinicians suggest that such specific risk factors should be highlighted in patients’ charts. Still, a crucial question remains, when should intervention for suspected NMS occur. Some experts emphasize that it is clinically more prudent to intervene early to prevent the development of signs and symptoms that can progress to confirmed NMS. In a large county hospital in Southern California a practice of staff training about NMS and early intervention was begun 11 years ago. Under this hospital practice, neuroleptic medication is withdrawn upon the appearance of any one of the following: Any unstable vital signs, including a temperature of 100.4 or higher. Rigidity, or significant confusion. With over 11,000 sequential psychiatric in-patient admissions at this hospital over an 11-year period, there have been no fatalities from NMS. 16 With a growing population and with the introduction of neuroleptics with improved benefits and fewer side effects the number of people treated with neuroleptics will keep increasing. But this also means that the number of patients at risk for NMS will increase. Many experts believe that the key to survival from this potentially lethal reaction to any neuroleptic is early detection, followed by rapid intervention and intensive medical care. Still, a crucial question remains, when should intervention for suspected NMS occur. Some experts emphasize that it is clinically more prudent to intervene early to prevent the development of signs and symptoms that can progress to confirmed NMS. In a large county hospital in Southern California a practice of staff training about NMS and early intervention was begun 11 years ago. Under this hospital practice, neuroleptic medication is withdrawn upon the appearance of any one of the following: Any unstable vital signs, including a temperature of 100.4 or higher. Rigidity, or significant confusion. With over 11,000 sequential psychiatric in-patient admissions at this hospital over an 11-year period, there have been no fatalities from NMS. With a growing population and with the introduction of neuroleptics with improved benefits and fewer side effects the number of people treated with neuroleptics will keep increasing. But this also means that the number of patients at risk for NMS will increase. Many experts believe that the key to survival from this potentially lethal reaction to any neuroleptic is early detection, followed by rapid intervention and intensive medical care. * * * END * * *