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About KEVEYIS

KEVEYIS has been shown to reduce the number, severity, and duration of Primary Periodic Paralysis (PPP) attacks1,2

Keveyis is the Proven Leader for PPP treatment1
  • Efficacy:
    • The efficacy of KEVEYIS has been proven in 2 clinical studies1
  • Tolerability:
    • KEVEYIS has a demonstrated safety profile1
  • Flexible oral dosing:
    • KEVEYIS offers patients a range of dosing for an individualized treatment approach1
  • Diagnostic & coding information:
    • ICD-10 Codes: G72.3 (Periodic Paralysis) or G71.19 (Other specified myotonic disorders)
    • ICD-9 Codes: 359.3 (Periodic Paralysis) or 359.29 (Other specified myotonic disorders)
    • KEVEYIS NDC: 71090-001-01

Case studies: actual patients with PPP who started taking KEVEYIS*

Matt W. 23-year-old patient with hypokalemic PPP Initial treatment: acetazolamide  
Typical attack:
  • Common symptoms leading up to attack include calf pain or muscle cramps
  • Weakness in leg and hip muscles—resulting in difficulty walking, tripping, and falling
  • Weakness in upper extremities during more severe attacks
  • Muscles related to breathing, swallowing, or speaking are never involved
  • Typical duration of attacks ranges from 2 to 72 hours
  • Typical severity varies from mild (limping when walking) to severe (unable to walk or get up from the floor)
Attack history:
  • Attacks began occurring at 3 years of age with a frequency of 2 to 3 a month
  • Attacks were often triggered by high-carbohydrate meals or intense physical activity
  • Weakness was worse in the mornings and improved throughout the day
Treatment history:
  • Potassium tablets and acetazolamide prescribed
  • Acetazolamide became ineffective in the past 2 years despite escalating dose
After starting on KEVEYIS (50 mg BID):
  • Patient was assessed every 6 months and has demonstrated a decrease in attack frequency and severity
Helen R. 28-year-old patient with paramyotonia congenita Initial treatment: mexiletine  
Typical attack:
  • Cramps and myotonia in the hands
  • Fatigue in the lower extremities
  • Typical duration of attacks is 5 to 30 minutes
  • Typical severity of attacks is moderate
Attack history:
  • Patient experienced attacks daily in cold weather months and 3 to 5 times per month in the summer
  • Common symptoms leading up to attacks included cramping of the hands and reduced exercise tolerance
  • Lingering symptoms included stiffness of the hands
Treatment history:
  • Mexiletine (150 mg TID) prescribed
  • After 4 months, discontinued due to lack of response, flu-like symptoms, and gastroesophageal reflux disease (GERD)
After starting on KEVEYIS (50 mg BID):
  • Patient has been on KEVEYIS and receiving no other concomitant medications for PPP
  • Patient initially reported a 4-week period of intermittent confusion which resolved spontaneously
  • * Case studies are real patients and were shared by practicing physicians specializing in PPP. Due to HIPAA and privacy laws, first names and initials have been changed to protect patients’ identities.

KEVEYIS helped control the number, severity, and duration of PPP attacks in 2 clinical trials1,2

Study design1:

Study 1 was a randomized, double-blind, placebo-controlled, parallel-group, multi-center, 9-week study that consisted of 2 substudies—hyperkalemic periodic paralysis (n=21) and hypokalemic periodic paralysis (n=44). The primary endpoint was the average number of self-reported attacks of muscle weakness per week during the last 8 weeks of the study. Patients already on KEVEYIS prior to the study continued on the same dose. In patients taking acetazolamide prior to the study, the dose of KEVEYIS was 20% of the acetazolamide dose. Treatment-naive patients received 50 mg of KEVEYIS BID. Dose reduction for tolerability was permitted.

Dr. Griggs, a PPP expert, discusses 2 clinical trials in PPP

Please see Important Safety Information and full Prescribing Information available on this website.
Select a tab below to view the results of the trial.

From baseline to week 9, treatment with KEVEYIS decreased weekly attack rates while treatment with placebo resulted in increases1,2†

  • †Treatment effect equals KEVEYIS attack rate per week minus placebo attack rate per week; adjusted for missing diary days. Attacks per week (defined as weekly attack rate) was the average number of self-reported attacks of muscle weakness per week. The posttreatment attacks were calculated as the average number of attacks per week over the final 8 weeks of the 9-week double-blind phase. The first week of diary data was excluded to avoid contamination of the efficacy assessment by the participant’s previous treatment, if any.2
  • ‡Treatment effect is computed as the median of the bootstrap distribution of treatment group difference in median response. The 95% confidence interval (CI) is computed using the 2.5 and 97.5 percentiles of this bootstrap distribution.2

Treatment with KEVEYIS demonstrated a reduction in attack severity vs placebo§

Self-reported measure of attack severity, with 10 being the most severe.§

  • §The severity-weighted attack rate was calculated from self-reported scores of attack severity (scored as 1-10 with increasing severity) defined as the sum of average attack severity across all distinct attacks over the final 8 weeks (weeks 2-9) divided by the number of weeks that the patient was followed. Severity-weighted attack data were adjusted for missing diary days.2

Treatment with KEVEYIS shortened weekly attack duration vs placebo||

Self-reported attack duration across all distinct attacks (weeks 2-9), represented in total hours.||

  • ||The weekly attack duration was calculated from self-reported attack duration across all distinct attacks over the final 8 weeks (weeks 2-9) divided by the number of weeks that the patient was followed. Weekly attack duration was adjusted for missing diary days.2
Get your patient started on KEVEYIS. Find the Prescription Form here.

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Indication

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

Important Safety Information

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 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.

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 (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.

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]. 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.
  3. Tawil R, McDermott MP, Brown R, et al. Randomized trials of dichlorphenamide in the periodic paralyses. Ann Neurol. 2000;47:46-53.