Keveyis®
KEVEYIS (pronounced keh-VAY-iss) (dichlorphenamide) is the first FDA-approved treatment for Primary Periodic Paralysis (PPP).1 KEVEYIS is indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants. KEVEYIS has been shown to reduce the number, severity, and duration of PPP attacks.1,2,3
PPP is a rare genetic condition that affects muscles and causes episodes of muscle weakness and/or temporary paralysis that can be progressive and debilitating.4,5
PPP episodes (muscle weakness or temporary paralysis) can vary in their frequency, severity, and duration.4,6 Most episodes range from 30 minutes to several hours. For some patients, episodes can last for days.
Symptoms of primary periodic paralysis (PPP) often begin before the age of 20, but diagnosis may take years. Because episodes of weakness or paralysis can be unpredictable and symptoms may resemble those of other conditions, identifying PPP can be challenging.4 PPP is usually inherited, but you can still have PPP (detectable through its signs and symptoms) even without confirmation of an identified genetic mutation on a genetic test.
Indication
KEVEYIS (dichlorphenamide) is indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.
Important Safety Information
- KEVEYIS is contraindicated in patients with:
- Hypersensitivity to dichlorphenamide or other sulfonamides
- Concomitant use of high-dose aspirin
- Severe pulmonary disease, limiting compensation to metabolic acidosis caused by KEVEYIS
- Hepatic insufficiency: KEVEYIS may aggravate hepatic encephalopathy
- Hypersensitivity and other life-threatening reactions have occurred with sulfonamides. Discontinue KEVEYIS at the first appearance of skin rash or any sign of immune-mediated or other life-threatening adverse reaction
- Carbonic anhydrase inhibitors, including KEVEYIS, can cause metabolic acidosis, which can increase the risk of salicylate toxicity. Use with caution and carefully monitor in patients receiving low-dose aspirin
- KEVEYIS increases potassium excretion and can cause hypokalemia. Baseline and periodic measurements of serum potassium during treatment are recommended. If hypokalemia develops or persists, consider reducing the dose or discontinuing and correcting potassium levels
- KEVEYIS can cause hyperchloremic non-anion gap metabolic acidosis. Concomitant use with other drugs that cause metabolic acidosis may increase the severity. Baseline and periodic measurements of serum bicarbonate during treatment are recommended. If metabolic acidosis develops or persists, consider reducing the dose or discontinuing KEVEYIS
- KEVEYIS increases the risk of falls; risk is greater in the elderly and with higher doses. Consider reducing the dose or discontinuing in patients experiencing falls
- The most common adverse reactions seen in clinical trials (incidence ≥10%) include paresthesias, cognitive disorder, dysgeusia, and confusional state
- There are no adequate data on the developmental risk associated with the use of KEVEYIS in pregnant women. Based on animal data, KEVEYIS may cause fetal harm
Please see full Prescribing Information for KEVEYIS.
1 KEVEYIS [prescribing information]. Chicago, IL: Xeris Pharmaceuticals, Inc.
2 Sansone VA, Burge J, McDermott MP, et al., for the Muscle Study Group. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis. Neurology. 2016; 86:1408–1416.
3 Tawil R, McDermott MP, Brown R Jr, et al. Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysis. Ann Neurol. 2000;47:46-53.
4 Statland JM, Fontaine B, Hanna MG, et al. Review of the diagnosis and treatment of periodic paralysis. Muscle Nerve. 2018; 57:522–530.
5 Cavel-Greant D, Lehmann-Horn F, Jurkat-Rott K. The impact of permanent muscle weakness on quality of life in periodic paralysis: a survey of 66 patients. Acta Myol. 2012; 31:126–133.
6 Charles G, Zheng C, Lehmann-Horn F, Jurkat-Rott K, Levitt J. Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals. J Neurol. 2013; 260:2606–2613.
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