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Safety & Dosing

KEVEYIS has a demonstrated tolerability profile

The most common adverse reactions with incidence ≥10% and rates greater than placebo in patients treated with KEVEYIS were paresthesia, cognitive disorder, dysgeusia, and confusional state.1

Adverse Reactions in Study 1

(≥5% and more common than in patients treated with placebo)

Adverse Reaction KEVEYIS (n=36) Placebo (n=29)
Nervous system disorders Paresthesia 44% 14%
Cognitive disorder* 14% 7%
Dysgeusia 14% 0%
Confusional state 11% 0%
Headache 8% 7%
Hypoesthesia 8% 0%
Lethargy 8% 0%
Dizziness 6% 0%
Gastrointestinal disorders Diarrhea 6% 3%
Nausea 6% 0%
General disorders and administration site conditions Fatigue 8% 0%
Malaise 6% 0%
Investigations Weight decreased 6% 0%
Musculoskeletal and connective tissue disorders Muscle spasms 8% 0%
Arthralgia 6% 3%
Muscle twitching 6% 0%
Respiratory Dyspnea 6% 0%
Pharyngolaryngeal pain 6% 0%
Skin Rash 8% 0%
Pruritus 6% 0%

Discontinuation rates in patients treated with KEVEYIS

Less than 10% (3/36) of all patients treated with KEVEYIS withdrew due to an adverse reaction in the double-blind phase of Study 12†

  • 17% of hyperkalemic patients (2/12) discontinued treatment2†
  • ~4% (1/24) of hypokalemic patients discontinued treatment2†
  • *Cognitive disorder combined cases with the preferred terms of cognitive disorder, disturbance in attention, and mental impairment.1
  • No patients treated with placebo withdrew due to an adverse reaction.

Flexible dosing of KEVEYIS allows for an individualized treatment approach1

Initiate 50 mg orally, once or twice daily1

Dosing can be increased or decreased at weekly intervals (or sooner in case of adverse reactions) based on individual response. The maximum recommended daily dose is 200 mg.

Monitor at weekly intervals1

Check in with your patients weekly to see if a dose adjustment is needed.

  • You can lower the dose of KEVEYIS if your patient is experiencing adverse reactions
  • You can also increase the dose of KEVEYIS to help further decrease the number, severity, and duration of your patient’s attacks

Evaluate overall response1

Continue to assess your patient’s response to KEVEYIS after 2 months of treatment to determine if additional dose titration is needed.

KEVEYIS dosing clinical study 1
KEVEYIS dosing clinical study 2

Patients already on KEVEYIS prior to entering Study 1 or Study 2 continued on the same dose. In patients taking acetazolamide prior to entering Study 1 or Study 2, the dose of KEVEYIS was 20% of the acetazolamide dose. Dose reduction for tolerability was permitted. Treatment-naive patients received 50 mg of KEVEYIS BID.




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

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 high-dose 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 type 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 correction of potassium levels.

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 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 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.


  1. KEVEYIS [package insert]. Chicago, IL; Xeris Pharmaceuticals.
  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.