Perspectives from the Narcolepsy Institute

Perspectives from the Narcolepsy Institute

PERSPECTIVES FROM THE NARCOLEPSY INSTITUTE Spring/Summer 2017 ANESTHESIA AND NARCOLEPSY Volume 23 | Number 1 We have received requests for information on the effect of anesthesia on the symptoms of narcolepsy. Below, for our patients’ benefit, is a revised version of this topic previously covered in Perspectives. Precautions to be observed before undergoing anesthesia People who have narcolepsy and are scheduled for an operation requiring general anesthesia must inform the anesthesiologist of their sleep disorder. Select a doctor who is knowledgeable about narcolepsy and the interaction of medications for narcolepsy and anesthetic agents. Let your doctor know about all the prescription medications, over-the-counter medications IN THIS ISSUE and supplements you are taking. Anesthesia and narcolepsy 1 Most of the medications for narcolepsy are metabolized by the liver, specifically by cytochrome P450-206; enzyme induction may occur. Also, the effects of atropine and Research Note: 3 ephedrine are enhanced by tricyclic antidepressants often prescribed for narcolepsy. Sodium Immunoglobulin therapy in thiopental will prolong the sedation during anesthesia, while ketamine may produce acute children with narcolepsy hypertension and cardiac dysrhythmias (Joseph 1990). Montelucast helps a 4-year-old boy 4 Should one discontinue the with obstructive sleep apnea medications for narcolepsy before anesthesia? Narcolepsy patients’ perspectives 5 on their disorder Because of the possibility of cardiovas- cular effects, some professionals suggest Did you know…“Social jet lag” 5 withdrawal of medications for narcolepsy linked to increased risk of before surgery. This may increase the heart disease symptoms of narcolepsy, but does not seem to cause any long-term effects. In Compliance with continuing 5 view of the exacerbation of symptoms positive airway pressure therapy of narcolepsy during withdrawal, other professionals advise against discontin- The Meaning of Happiness 6 uation of drugs and prefer to adjust the dosage for anesthetics. Patients may get cataplexy, hypnopompic hallucinations or sleep paralysis when they come out of an- esthesia (Joseph 1990). The professional should be supportive and understanding and explain the progression of events to the patient. CONTINUED ON PAGE 2 1 CONTINUED FROM PAGE 1 ● Recently, a study assessed post-operative RESEARCH NOTE: outcomes of having anesthesia in people with narcolepsy (Cavalcante et al. 2017). The study was IMMUNOGLOBULIN THERAPY IN CHILDREN retrospective and compared 76 narcolepsy patients WITH NARCOLEPSY with controls matched for age, gender and type and Case reports of intravenous immunoglobulin (IVIg) in children with year of surgery. Patients’ records were reviewed for narcolepsy have been described in literature. Recently, a longitudinal evidence of depression during phase 1 recovery observational study on this topic on children with narcolepsy was and other perioperative (pertaining to surgery) reported by a research team in France. The study was nonrandomized outcomes. and open-label, and data were collected retrospectively from a single source, a national pediatric center for the treatment of narcolepsy. Results of this study indicate that people with Twenty-four subjects received IVIg (three infusions administered at narcolepsy were more likely to be prescribed one-month intervals) in addition to standard care (psychostimulants central nervous system stimulants (73.7 percent; and/or anticataplectic agents). Thirty-two subjects continued on 4.0 percent, p < 0.001) and antidepressants (46.1 standard care alone (controls). percent; 27.6 percent, p = 0.007) versus the control group; people with narcolepsy were also more likely Medical files were not available in two patients in each group. All 22 to have a diagnosis of obstructive sleep apnea (40.8 IVIg patients had cerebrospinal fluid (CSF) hypocretin ≤ 110 pg/mL and percent; 19.1 percent, p < 0.001) versus the control were HLA-DQB1*06:02 positive. Twenty-nine of the 30 control patients group. There was no difference in the intraoperative were HLA-DQB1*06:02 positive; all 12 with available CSF readings had course (time during surgical procedure) between hypocretin ≤ 110 pg/mL. Compared with control patients, IVIg patients patients and controls. Episodes of respiratory had shorter disease duration and shorter latency to sleep onset, depression were similar in the two groups. It is and more of them had received H1N1 vaccinations. Mean (standard noteworthy that patients had a higher frequency deviation) follow-up length was 2.4 (1.1) years in the IVIg group and 3.9 of emergency response team activations versus (1.7) years in controls. High baseline UNS (Ullanlinna Narcolepsy Scale), controls. Most of the emergency episodes were indicating severe sleepiness but not IVIg treatment, was associated due to hemodynamic (related to blood circulation) with a reduction in narcolepsy symptoms. Also, among patients with instability. The study notes one episode of high baseline UNS scores, control patients achieved a UNS score < 14 excessive postoperative sedation and respiratory (indicating remission) less rapidly than IVIg patients. The length of depression in one person with narcolepsy. At the disease duration did not influence treatment response. Narcolepsy Institute, we have seen one case of In summary, narcolepsy symptoms were not significantly reduced prolonged sedation after anesthesia from which the by IVIg. The independent effect of the IVIg treatment was not patient recovered with no negative effects noted by demonstrated. However, a subset of IVIg-treated patients, among the doctors in attendance. patients with high baseline symptoms, achieved remission more rapidly References than control patients. Joyce, JA Anesthetic considerations for patients with narcolepsy. AAANA J. 1999;67(1):59-66. Reference Cavalcante AN, Hofer RE, Tippmann-Peikert M, Sprung Lecendreux M, Berthier J, Corny J, Bourdon O, Dossier C, Delclaux C. J, Weingarten TN. Perioperative risks of narcolepsy Intravenous immunoglobulin therapy in pediatric narcolepsy: a nonrandomized, in patients undergoing general anesthesia: a case- open-label, controlled, longitudinal observational study. J Clin Sleep Med. control study. J Clin Anesth. 2017 Apr 19. pii: S0952- 2017;13(3):441–453. 8180(16)31027-3. doi: 10.1016/j.jclinane.2017.04.008. [Epub ● ahead of print] 2 PERSPECTIVES from the Narcolepsy Institute MONTELUKAST HELPS A 4-YEAR-OLD BOY WITH OBSTRUCTIVE SLEEP APNEA A continuous positive airway pressure (CPAP) machine is recommended for the treatment of obstructive sleep apnea (OSA) in adults. However, children with OSA are recommended an adenotonsillectomy (surgical removal of adenoids and tonsils). In this case report, the authors describe successful management of severe OSA related to adenoid hypertrophy (enlarged adenoids) by oral administration of Montelukast. Montelukast (Singulair®), recommended for asthma and for seasonal or year-round allergies, is a leukotriene receptor antagonist (LTRA). It blocks the action of substances in the body that cause the symptoms of allergies and asthma. The subject in this case report was an otherwise normal 4-year-old boy who presented with symptoms of frequent snoring and mouth breathing. Clinically, enlarged tonsils and adenoids were noted. A polysomnogram test revealed severe OSA based on the apnea- hypopnea index (AHI), which was high at 102.5 events/hour. The boy’s parents refused surgery, so oral Montelukast (5 mg/day) was prescribed. After two months of treatment, the frequency and severity of snoring was reduced and the AHI was reduced to 2.4 events/hour, suggesting that oral Montelukast may be beneficial in some severe OSA cases related to adenoid hypertrophy. Reference It is noteworthy that the patient’s point of view is gaining Li T, Zhou G, Yang L, Tan L, Ren R, Sun Y, Li Y, Tang X. A four-year-old importance in recent research on narcolepsy. In a cross-sectional boy with unusually severe obstructive sleep apnea. J Clin Sleep Med. survey of 1,699 individuals with self-reported diagnoses of 2017;13(3):513–516. narcolepsy, researchers examined functional limitations, treatment responsiveness, symptoms and comorbidities (Maski et al. 2017). .● They also investigated the relationship between the onset of NARCOLEPSY PATIENTS’ PERSPECTIVES narcolepsy in children and the time of diagnosis. ON THEIR DISORDER Several narcolepsy groups (Wake Up Narcolepsy, Inc.; Project Sleep; and FasterCures) worked to support this survey to provide Traditionally, the definition of illness and the needs of patients data for the U.S. Food and Drug Administration (FDA) in order to are determined by medical professionals who focus on the develop better drugs. People with narcolepsy had access to the physical aspects of a disorder and its medical management. survey on a Unite Narcolepsy website, indicating that it was an Today, however, complex arrays of factors operate that make it online survey, though this is not clearly stated. Individuals who necessary to reexamine the are not computer savvy or lack access to a computer may be traditional methods of patient PATIENTS NOT underrepresented. Sociodemographic variables, such as gender, care. Patients not only want ONLY WANT TO LIVE income, education, occupation and place of dwelling, were not to live longer, but they also LONGER, BUT THEY available. desire a good quality of life ALSO DESIRE A GOOD by being productive, staying QUALITY OF LIFE BY Most of the survey respondents were between the

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