In Primary Periodic Paralysis

Helping to turn limitation
into activation

KEVEYIS can help patients gain greater control over the disease by decreasing the number, severity, and duration of attacks.1,2

KEVEYIS (dichlorphenamide) is the first and only FDA-approved treatment for hyperkalemic, hypokalemic, and related variants of Primary Periodic Paralysis (PPP).

Learn more about KEVEYIS

INDICATION AND IMPORTANT SAFETY INFORMATION

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KEVEYIS is indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.

Contraindications

  • 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/Anaphylaxis/Idiosyncratic Reactions
    • Fatalities associated with the administration of sulfonamides have occurred due to 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 idiosyncratic adverse reaction
  • Concomitant Use of Aspirin
    • Anorexia, tachypnea, lethargy, and coma have been reported with concomitant use of dichlorphenamide and high-dose aspirin
    • The concomitant use of KEVEYIS and high-dose aspirin is contraindicated
    • Use with caution in patients receiving low-dose aspirin
  • Hypokalemia
    • 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 (eg, adrenocortical insufficiency, hyperchloremic metabolic acidosis, or respiratory acidosis), and in patients receiving other drugs that may cause hypokalemia (eg, 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
  • Metabolic Acidosis
    • 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 metabolic acidosis
    • Baseline and periodic measurements of serum bicarbonate are recommended
    • If metabolic acidosis develops or persists, consider reducing the dose or discontinuing KEVEYIS
  • Falls
    • KEVEYIS increases the risk of falls; risk is greater in the elderly and with 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.

Adverse Reactions

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.

References

  1. KEVEYIS [package insert]. Feasterville-Trevose, PA: Strongbridge Biopharma; 2017.
  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.