KEVEYIS (pronounced keh-VAY-iss) (dichlorphenamide) is the first and only FDA-approved treatment for Primary Periodic Paralysis (PPP)1. It is a prescription drug used to treat primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and other similar diseases. Keveyis has been shown to reduce the number, severity, and duration of PPP attacks.1,2
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.3,4
PPP episodes (muscle weakness or temporary paralysis) can vary in their frequency, severity, and duration. A study found that most episodes range from 30 minutes to several hours. For some patients, episodes can last for days.5
Episodes of weakness or paralysis usually begin in childhood, before age 20. Some people begin having attacks as early as age 2 or even younger5. PPP is usually inherited, but you can still have PPP (detectable through its signs and symptoms) even without one of the genetic mutations.4,5
Important Safety Information
- Hypersensitivity to dichlorphenamide or other sulfonamides
- Concomitant use of KEVEYIS and high-dose aspirin
- Severe pulmonary disease, limiting compensation to metabolic acidosis caused by KEVEYIS
- Hepatic insufficiency: KEVEYIS may aggravate hepatic encephalopathy
WARNINGS AND PRECAUTIONS
Hypersensitivity and Other Life-Threatening Reactions
- Fatalities associated with the administration of sulfonamides have occurred because of adverse reactions, including Stevens-Johnson syndrome, toxic epidermal necrolysis, fulminant hepatic necrosis, agranulocytosis, aplastic anemia, and other blood dyscrasias
- Pulmonary involvement can occur in isolation or as part of a systemic reaction.
- Discontinue KEVEYIS at the first appearance of skin rash or any sign of immune-mediated or other life-threatening adverse reaction.
Concomitant Use of Aspirin or Other Salicylates
- Carbonic anhydrous inhibitors, including KEVEYIS, can cause metabolic acidosis, which can increase the risk of salicylate toxicity.
- Anorexia, tachypnea, lethargy, and coma have been reported with concomitant use of dichlorphenamide and highdose aspirin.
- Concomitant use of KEVEYIS and high-dose aspirin is contraindicated. Use with caution and carefully monitor in patients receiving low-dose aspirin.
- KEVEYIS increases potassium excretion and can cause hypokalemia.
- The risk of hypokalemia is greater when KEVEYIS is used in patients with conditions associated with hypokalemia (e.g., adrenocortical excess, renal tubular acidosis types 1 and 2), and in patients receiving other drugs that may cause hypokalemia (e.g., loop diuretics, thiazide diuretics, laxatives, antifungals, penicillin, and theophylline).
- Baseline and periodic measurements of serum potassium are recommended.
- If hypokalemia develops or persists, consider reducing the dose or discontinuing KEVEYIS and correcting potassium levels.
- KEVEYIS can cause hyperchloremic non-anion gap metabolic acidosis.
- Concomitant use of KEVEYIS with other drugs that cause metabolic acidosis may increase the severity of acidosis.
- Concomitant use of KEVEYIS in compensated patients with respiratory acidosis, such as in advanced lung diseases, may lead to respiratory decompensation.
- Baseline and periodic measurements of serum bicarbonate during KEVEYIS 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 at higher doses.
- Consider dose reduction or discontinuation of KEVEYIS in patients who experience falls while treated with KEVEYIS.
Pregnancy and Lactation
Use during pregnancy only if the potential benefit justifies the potential risk to the fetus. It is not known in humans whether dichlorphenamide is excreted in human milk; exercise caution when administered to a nursing woman.
The most common adverse reactions seen in clinical trials (incidence ≥10% and greater than placebo) include paresthesias, cognitive disorder, dysgeusia, and confusional state.
Please see Full Prescribing Information.
1 KEVEYIS [package insert]. Feasterville-Trevose, PA: Strongbridge Biopharma; 2019.
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 Statland JM, Fontaine B, Hanna MG, et al. Review of the diagnosis and treatment of periodic paralysis. Muscle Nerve. 2018; 57:522–530.
4 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.
5 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.