Spotlight on Rilutek
By Brett Gillis, PharmD Lou Gehrig, the son of German immigrants, was born in 1903 in New York City. His mother worked tirelessly to make ends meet, and his father, in poor health, struggled to find work. Gehrigs mother sent him to Columbia University for a good education where he began to play baseball seriously. Scouts quickly discovered his talent, and Gehrig became a Yankee for good in 1925. His strength and endurance earned him the nickname Iron Horse. However, in 1938 Gehrig began to lack his usual strength, and his batting average suffered. Doctors initially suspected a gall bladder problem and prescribed a bland diet. This only made him weaker. One day, a teammate noticed that he was sliding his feet along the ground as he walked. The medical team at the Mayo Clinic diagnosed him with a very rare degenerative disease, amyotrophic lateral sclerosis (ALS). Amyotrophy is the wasting of muscles, (and sclerosis is pathological hardening) of tissue. In 1941, Gehrig succumbed to the effects of ALS and passed away. ALS, also called Lou Gehrigs Disease (LGD), is a serious neurological disease that causes muscle weakness, disability, and eventual death. ALS occurs in 1 to 3 people in 100,000. Patients with ALS often present with muscle twitching or weakness in an arm or leg or slurred speech. The disease eventually diminishes the patients ability to control muscles. Patients initially struggle to perform activities of daily living and, sadly, eventually become unable to move, speak, eat, and breathe. Despite extensive research and several hypotheses, the etiology and pathogenesis of ALS are unknown. One hypothesis proposes that motor neurons, vulnerable because of genetic predisposition and/or environmental factors, are injured by glutamate, one of the amino acid transmitters in nerve cells. Researchers suggest that glutamate is not cleared from nerve cell junctions swiftly enough in patients with ALS. A mutation that affects the transportation of glutamate away from the nerve junction may contribute or be responsible for excess glutamate. This results in prolonged excitation and toxicity in nerve cells. 40% of patients with sporadic cases of ALS present with elevated glutamate levels in cerebrospinal fluid. Abundant evidence points to glutamate as a destructive factor in ALS. The first, and only FDA approved specific pharmacologic treatment for ALS to date, is Rilutek (riluzole). Riluzole is a benzothiazole drug that modulates glutamate. The exact mechanism of action that ameliorates ALS symptoms is currently unknown. The following pharmacological properties may contribute to Riluzoles effect on ALS: inhibition of glutamate release, inactivation of sodium channels, and interference with transmitter binding at excitatory amino acid receptors. Riluzole researchers performed two small randomized clinical trials to study patients with early stage disease (less than 5 years and baseline Forced Vital Capacity (FVC) of at least 60%). The studies followed these patients for at least 12 months but no longer than 18 months. Survival curves were not statistically significant when analyzed by the method called for in the clinical trial protocol. Although both studies revealed an early increase in survival (extending life by 60 to 90 days for those patients in the Riluzole group), Riluzole failed to decrease mortality by the end of the trials (maximum 18 months).
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Clinical Question: The Importance of PMH (Past Medical History) and Why We Ask
By Joelle Potts, PharmD, CGP During our new admission consults, ProCare Clinical Pharmacists will ask for the patients past medical history (PMH), or whether the patient has any other conditions or comorbidities. These other conditions may be from recent or remote history and may or may not be directly related to the patients primary/terminal diagnosis. However, even if these other conditions are not directly related to the terminal diagnosis, this information is still important for us to obtain for several reasons: allows for a more complete picture of the patients health and medical history, which can impact the patients status and symptoms as the patient declines allows us to more thoroughly assign coverage of medications as hospice-provided vs. patient-provided, both during the new admission consult and during future consults or medication updates provides valuable information for future consults, grouped in one place in the patients profile, when the information is often most readily available. This allows ProCare Clinical Pharmacists to quickly and easily see valuable information during future consults, when the complete information may not be available to the nurse calling in allows us to better assess whether a certain medication is related to the patients primary/terminal diagnosis or to a pre-existing/unrelated condition
Clinical examples of conditions/history that may be especially relevant to future medication choices while a patient is in hospice include: diabetes; cardiac arrhythmia or presence of a pacemaker; is the patient a current smoker; kidney or liver impairment; and psychiatric history, to name just a few. For example, certain antipsychotics and anti-emetics are either contraindicated or not recommended in patients with Parkinsons disease, and we would not recommend methadone (or certain other medications) as first-line options in patients with a pacemaker due to the potential for QTc interval prolongation. A coverage example is omeprazole, which could be patient-provided due to an unrelated history of GERD, or could be hospice-provided if it is more directly related to the patients terminal diagnosis or end of life comfort (e.g. due to other related medications such as steroids, or dyspepsia-induced nausea, etc.). We realize that sometimes PMH is just not available during the new admission consult. However, we hope that this explains why your ProCare Clinical Pharmacist asks for this information, and why it is important. In general, it allows us to make better recommendations for the patient while avoiding potential drug-disease interactions and adverse effects.
Riluzole is appropriate as part of the therapeutic management of early stage ALS to prolong survival and slow deterioration of muscle strength. Current guidelines for the clinical management of ALS recommend Riluzole therapy as soon as possible after a diagnosis of ALS is made. Riluzole is no longer indicated after switching from the active ALS treatment phase to end of life symptom management. References: Clarke, K. and Levine, T. Clinical Recognition and Management of Amyotrophic Lateral Sclerosis: The Nurses Role. Journal of Neuroscience Nurses 2011 Aug; 43 (4): 205-14 Rilutek [Package Insert]. Bridgewater, NJ: Sanofi-Aventis; 2009.
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Editor: Dr. Cody Midlam, PharmD Executive Editor: Dr. Raeanna Lewarne, PharmD