Important Safety Information

Prescribe Keveyis

Help patients manage Primary Periodic Paralysis today — and for the future

Discover strategies that can help patients with Primary Periodic Paralysis (PPP) better manage their attacks.

Managing Primary Periodic Paralysis

Primary Periodic Paralysis (PPP) is a complex genetic disorder, but it can be managed with a straightforward approach focused on:

  1. Long-term attack prevention through treatment
  2. Trigger management and lifestyle changes 


PPP can progress to Permanent Muscle Weakness (PMW) — constant weakness that can occur independently of attacks and becomes more likely in the fifth and sixth decades of life.1,2


As a provider, you’re in a unique position to improve your patient’s PPP by helping to guide them through the following approaches.

PROVIDER TESTIMONIAL

“Frequent attacks can have a substantial impact on a patient’s ability to work and perform daily activities, so it makes sense to do whatever possible to minimize the frequency and severity of attacks.”

Long-term attack prevention through treatment

Preventing PPP attacks is crucial for optimal management. It can help reduce the risk of PPP negatively impacting your patient’s work, school and family life, as well as their physical and mental health.3

Certain medications can help patients both prevent and manage PPP episodes.

KEVEYIS® is proven to reduce the frequency, severity and duration of PPP attacks.4,5

PPP can lead to progressive Permanent Muscle Weakness (PMW).1 Up to 60% of patients with a history of PPP experience PMW. After years of paralytic attacks, PMW may be irreversible.

Trigger management and lifestyle changes

In addition to long-term medication, helping your patient identify and manage their triggers is crucial to reducing them. Common triggers include3,6:

  • Food or beverages high in salt, carbohydrates or potassium
  • Stress or fatigue
  • Exposure to cold temperatures or cold air
  • Periods of inactivity
  • Resting after exercise

MANAGING PPP ATTACKS

Help patients make diet and lifestyle changes to manage PPP attacks based on their subtypes:

Hypokalemic Primary Periodic Paralysis (HypoPPP):

PPP episodes may involve a decrease in potassium levels.6,7

Take potassium supplements as recommended by a healthcare provider.6,7 Eat a low sodium, low carbohydrate diet.

Hyperkalemic Primary Periodic Paralysis (HyperPPP):

PPP episodes may involve an increase in potassium levels.6,7 In some cases, potassium levels are normal during an episode.6

Eat frequent carbohydrate-heavy meals.6,7 Avoid potassium-rich foods.

Paramyotonia Congenita (PMC):

Attacks of muscle stiffness that occur during activity such as exercise.9

Avoid cold temperatures and strenuous exercise.7

Andersen-Tawil syndrome (ATS):

Characterized by muscle weakness, changes in heartbeat, and distinct skeletal features.6,8 It is associated with low, normal, or high potassium levels during an episode.

Light exercise may help decrease the severity of an episode.8

COMPLIMENTARY SUPPORT

Redefining patient and provider support with Xeris CareConnectionTM

Since 2016, we’ve been helping patients with PPP identify and manage their triggers and reduce PPP attacks with KEVEYIS. Through
Xeris CareConnection™, get access to a treatment support program specifically for patients with PPP and their providers.

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References

  1. 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.
  2. Jurkat-Rott K, Weber M-A, Fauler M, et al. K+-dependent paradoxical membrane depolarization and Na+ overload, major and reversible contributors to weakness by ion chanel leaks. Proc Natl Acad Sci. 2009;106:4036-4041.
  3. 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.
  4. KEVEYIS [package insert]. Chicago, IL; Xeris Pharmaceuticals.
  5. 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.
  6. Statland JM, Fontaine B, Hanna MG, et al. Review of the diagnosis and treatment of periodic paralysis. Muscle Nerve. 2018;57:522-530.
  7. Ralph J, Ptacek L. Muscle channelopathies: periodic paralyses and nondystrophic myotonias. In: Rosenberg RN, Pascual JM, eds. Rosenberg’s Molecular and Genetic Basis of Neurological and Psychiatric Disease. 5th ed. Elsevier; 2015:1177- 1189.
  8. Veerapandiyan A, Statland JM, Tawil R. Andersen-Tawil Syndrome. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews®. University of Washington, Seattle; 1993-2019. Updated June 7, 2018. Accessed March 22, 2024. https://www.ncbi.nlm.nih.gov/books/NBK1264/.
  9. Paramyotonia congenita. MedlinePlus. Updated August 1, 2015. Accessed June 7, 2024.https://medlineplus.gov/genetics/condition/paramyotonia-congenita/

INDICATION AND IMPORTANT SAFETY INFORMATION — READ MORE

Indication and Important Safety Information

 

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.

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