This 17-year-old boy was transferred to the inpatient psychiatry unit for evaluation of behavioral changes and hallucinations. During the month previous to admission, he had become increasingly agitated and had suspected his mother of trying to poison him.
His medical history is remarkable for a 2-year history of fatigue and vague somatic complaints, including pharyngitis and "lowgrade" fevers. One year ago, he was found to have received two tick bites in Wisconsin. Six months before admission, he was diagnosed with chronic Lyme disease, based on serologic testing. He initially received several weeks of an oral cephalosporin, followed by azithromycin and metronidazole for 30 days. Following this, a percutaneous intravenous catheter (PIC) was placed, and he had been receiving daily intravenous ceftriaxone for 6 weeks before admission. By report, he had been previously diagnosed with both a generalized anxiety disorder and a major depressive disorder. However, his mother refused to have any of these reports released to the physicians here.
His review of systems was remarkable for multiple severe, unremitting headaches. His mother attributed all of his symptoms to chronic Lyme disease. He was seen by different physicians in at least six academic medical centers, none of whom could establish a diagnosis. He lives at home with his mother and stepfather. He regularly smokes marijuana, drinks alcohol, and is sexually active. He's missed at least two semesters of school related to his illnesses. However, before that, he was in school and was a highly competitive athlete.
On examination, he was an awake, very talkative young man. He was afebrile and the vital signs were unremarkable. Growth parameters were normal. Physical examination was totally unremarkable in detail save for a PIC in his right arm. On mental status examination, he was pacing and very moody throughout the exam. His thought processes were very guarded with tangential, derailed ideations, some paranoid. He stated that "two little people would talk to him routinely." He was oriented fully to person, place, and time.
Robert Listernick, MD, moderator: What are the clinical manifestations of "real" Lyme disease?
Ben Katz, MD, pediatric infectious disease specialist: Perhaps the easiest way to think about Lyme disease these days is that there are both acute and chronic manifestations. Approximately 50% of serologically proven cases have the characteristic rash, erythema chronicum migrans, an expanding erythematous macule with central clearing. About 20% of patients develop multiple such lesions as the Borrelia spreads hematogenously. Acute neurologic complications may include aseptic meningitis and cranioneuropathies, in particular seventh nerve palsy; in endemic areas, Lyme disease is the most common cause of seventh nerve palsy. Myocarditis, often evidenced by varying degrees of heart block, may be seen early in the disease.
Dr. Listernick: What are the chronic manifestations?
Dr. Katz: Perhaps the most commonly seen late manifestation is a monoarthritis or oligoarticular arthritis, usually involving the large joints, particularly the knees. This occurs 6 weeks to 6 months following the original infection. Chronic neurologic complications of Lyme disease, while rare in children, have been reported in adults. These have included polyneuritis and chronic demyelinating encephalitis.
Dr. Listernick: How is the diagnosis of Lyme disease confirmed?
Dr. Katz: Enzyme-linked immunosorbent assay tests are poor screening tests, particularly if done by a commercial laboratory. Serologic testing should be performed by reference laboratories, which have much higher sensitivity and specificity rates using Western blot technology. However, there still are a number of false positives with this testing. Patients from areas not endemic with Lyme disease are likely to have a high proportion of positive results that are false positives.
Even patients from endemic areas with nonspecific symptoms, such as this patient, should not have Lyme testing done; the rate of false positive tests is still very high.
Dr. Listernick: What about this patient's diagnosis of "chronic Lyme disease"?
Robert Tanz, MD, general academic pediatrician: It's pure fiction. There are a number of patients and physicians around the country who have attributed a wide array of symptoms to chronic Borrelia infections, including fatigue, myalgias, arthralgias, pharyngitis, and lowgrade fever. There is a wealth of data demonstrating the absence of such a syndrome. Yet there are a number of physicians, especially in endemic areas, who continue to make this diagnosis and to prescribe long-term antibiotic therapy, including parenteral ceftriaxone. There has been at least one death attributed to this form of therapy for chronic Lyme disease.
Dr. Katz: Obviously, many of these symptoms are seen in what has been called "chronic fatigue syndrome." During the past century, patients with these symptoms have been diagnosed as having many diseases that don't truly exist, including chronic influenza, chronic brucellosis, and chronic Epstein-Barr virus infection. No one dies from these diagnoses, and many patients get better over time, often with the help of psychological therapy.
Dr. Listernick: I couldn't agree more. As I understand it, there is also a "Chronic Lyme Disease Society" made up of caregivers and patients with the disease. There is a huge lobbying effort aimed at getting insurance companies to pay for therapy of this bogus diagnosis. I've seen children who had been receiving intravenous antibiotic therapy for as long as 1 2 months.
Dr. Katz: Just so that it's clear, I've prescribed as much as 4 weeks of intravenous ceftriaxone for children with unequivocal joint or neurologic Lyme disease. Rarely, I've given a second course for intractable arthritis. Anything beyond this is not based on science.
Doug Nordli, MD, pediatric neurologist: I've seen the same type of thing in patients with intractable seizures or a chronic, severe encephalopathy. Parents are desperate for a diagnosis, particularly one that's treatable, and will latch on to any physician who claims to have a cure.
Dr. Listernick: Moving on, what should physicians be thinking when faced with an adolescent with generalized anxiety and paranoid ideations?
D. Richard Martini, MD, child and adolescent psychiatrist: First, one has to clearly establish if there's an underlying medical diagnosis.
Let's assume that in this case, as has been stated, that there isn't one. Although I have not interviewed this patient, from the history it seems that he clearly has been debilitated over the past several years by all die medical evaluations and interventions. One observation is that he may have an underlying anxiety or mood disorder and that the marijuana and alcohol are forms of selfmedication. I think that it's unlikely that he has a psychotic disorder. Such people usually have global deterioration in functioning over the months before onset of delusions and hallucinations. More likely, he has a mood or bipolar disorder.
Dr. Listernick: Schizophrenia is unlikely?
Dr. Martini: Yes. Children with schizophrenia usually have a long history of problems getting along with peers; they spend increasing periods of time alone and gradually deteriorate in their social functioning until their social misperceptions and delusions become more obvious. Symptoms may progress much faster in bipolar or mood disorders (eg, depression, dysthymia, anxiety), and these patients may exhibit a much faster onset of a thought disorder.
Dr. Listernick: Hallucinations and paranoid ideations may be part of a mood disorder?
Dr. Martini: Absolutely. The mood disorder is the first to appear; the symptoms of psychosis tend to reinforce the mood problems. After examining this child, our psychiatrist's initial diagnosis was an underlying mood disorder.
Dr. Listernick: What medical evaluation needs to be performed in a child who presents in this fashion?
Dr. Martini: First, he should have a careful neurologic examination to look for focal neurologic signs. For example, Wilson's disease may initially present with psychiatric symptoms. At a minimum, I would perform a toxicological screen for alcohol and drugs of abuse as well as thyroid function testing.
Dr. Nordli: Rarely, a few amino acid and organic acid disorders will present with psychosis in the pre-adolescent.
Dr. Martini: Interestingly, one tends to think of visual hallucinations arising often as a result of organic disease. However, as opposed to dieir frequency in adults, visual and auditory hallucinations occur in early onset psychotic disorders with equal frequency.
Dr. Listernick: All of his medical testing was normal. During the course of his evaluation, it became clear that the PIC was not necessary and could serve as a nidus of infection and was discontinued. Three days later, interventional radiology called the inpatient psychiatry floor and asked that he be sent down to have a new PIC placed. It hadn't been ordered by any of his physicians here. We still can't be sure whether the mother "ordered" the test surreptitiously in anticipation of his being discharged from me hospital or whether the outside caregiver who had been treating this child for chronic Lyme disease ordered the procedure. During the preceding days, the mother had been insisting that he undergo multiple complete blood counts (CBCs) to look for "signs of infection." The PIC was not put in. The caregiver who was treating this child for chronic Lyme disease accused his inpatient physician of malpractice for not allowing the placement of the PIC.
Dr. Listernick: When does the practice of alternate health care beliefs cross over to malpractice?
Dr. Katz: If the practice causes harm to the patient.
Dr. Listernick: Would that include the placement of a PIC?
Dr. Katz: Certainly. There's a risk of infection and causing damage to the vein.
Dr. Listernick: I absolutely agree with you, but couldn't one argue that there are many doctors around the country treating children with Lyme disease with prolonged intravenous antibiotics, thus making it a "standard practice"?
Dr. Tanz: The question about malpractice revolves around whether an ordinary physician in similar circumstances would follow the same course of action. If the answer is no, then the therapy may represent malpractice.
Dr. Martini: There are many children with a host of complaints who bounce from physician to physician looking for "the diagnosis." At some point, it's extremely important for a physician to emphasize how the child is functioning as a consequence of the multiple medical assessments and interventions. This invariably leads to a discussion of psychological factors in the child's presentation and a move away from repeated diagnostic testing. Unfortunately, many parents can tolerate a medical diagnosis more easily than a psychiatric diagnosis.
Dr. Listernick: Turning the question around, how far should physicians go in accommodating parents' alternate healthcare beliefs?
Dr. Katz: As long as no harm is being done, I try to respect parental wishes. If they would like to use a particular alternative or complementary medicine, I try to make sure that there isn't any potential toxicity.
Dr. Listernick: This mother wanted almost daily CBCs performed? Would you have done that?
Dr. Katz: Of course not, nor would I do any testing that was clearly not necessary.
Dr. Tanz: If a parent keeps pushing for the establishment of a diagnosis such as chronic Lyme disease but has been told repeatedly that it doesn't exist, shouldn't the diagnosis of Munchausen syndrome by proxy (MSBP) be entertained?
Dr. Listernick: I'd agree, but how can we begin to entertain that diagnosis when a caregiver has actually confirmed the diagnosis of chronic Lyme disease?
Dr. Tanz: Therein lies the problem. The parent's need to have a diagnosis has led her to form a relationship with a charlatan at both great financial and psychological cost to her and the child.
Emalee Flaherty, MD, child abuse specialist: We see this problem frequently when taking care of victims of MSBP. Unwittingly, physicians collude with these families by ordering tests and unnecessary therapies without fully questioning the diagnosis. Whether well-meaning or poorly conceived, as in this case, the child is caused great physical and emotional harm. Disturbingly, victims of MSBP may develop characteristics of Munchausen syndrome as adolescents and young adults.
Dr. Listernick: How do we diagnose MSBP?
Dr. Flaherty: There must be evidence that the child has presented repeatedly for medical care, all diagnoses other than illness falsification have been eliminated, and there should be no findings that exclude illness falsification. If MSBP is suspected, it is essential to review the previous medical records to look for inconsistencies in the history, documentation of the diagnoses, and the basis for these diagnoses. Unfortunately, the complexity of the medical and psychological aspects of these cases is difficult to convey in the courtroom. Such children are rarely removed from the home except in the most egregious cases.
Dr. Listernick: What did you recommend in this case?
Dr. Flaherty: Although we did not have all of the previous medical information and could not at that point determine if this was MSBP, it was clear that the mother was causing him physical and emotional harm by her actions. At the mother's insistence, he had received a PIC, unnecessary antibiotics, and unnecessary blood tests. At the same time, he had not received the treatment that he needed for his emotional health.
Because we suspected physical and emotional abuse, we reported the case to the state child welfare system and recommended removal from the home. Child welfare took protective custody because the mother threatened to remove him from the hospital. Child welfare obtained his medical records, which provided a clearer sense of the history.
Dr. Listernick: What happened to his psychological symptoms?
Dr. Martini: He was started on risperidone and his thought processes gradually improved. Interestingly, he was most concerned about his somatic complaints and actually felt better after repeated assurance. On the other hand, his mother became more stressed with each attempt at reassurance. He was sent home to receive court-mandated outpatient psychotherapy.
Dr. Listernick: Should we report this caregiver to the appropriate authorities for potentially harming this child?
Morris Kletzel, MD, pediatric oncologist: Unfortunately, even if you wanted to file this report in Illinois, it would be impossible without the family's consent. The authorities will not file the report without the name of the patient, which you can't divulge without consent. It's the proverbial Catch 22.
Rob Garafalo, MD, adolescent medicine physician: This 17-yearold is able to give this consent even if the parent won't.
Dr. Flaherty: The state has not yet made a final plan for this child, but we will continue to work with them to make sure that they understand our concerns and the risk of continued harm for mis child.
Dr. Listernick: Thank you, everybody.