Important Safety Information

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What is
Primary Periodic Paralysis?

Learn about this rare and progressive genetic condition
— and the path to diagnosis.

PPP overview

Primary Periodic Paralysis (PPP) is a group of rare, channelopathies that manifest as recurrent attacks of muscle weakness or temporary paralysis often precipitated by triggers.1,2
  • Includes a spectrum of chronic, autosomal-dominant neuromuscular disorders caused by mutations in the skeletal muscle sodium, calcium and potassium channel genes, often associated with alterations in serum potassium levels.3
  • Muscle weakness or temporary paralysis is caused by depolarization of the muscle sarcolemma, which in turn causes sodium channel inactivation and reduced fiber excitability.3

Only 4,000 to 5,000 people in the U.S. are diagnosed with PPP.4

As a physician, you can treat and manage Primary Periodic Paralysis. While PPP is rare,
you’re in a unique position to make an impact on someone’s life.

PPP can lead to Permanent Muscle Weakness2

Over time, PPP may lead to Permanent Muscle Weakness (PMW), constant weakness that can occur independently of attacks and becomes more likely in the 5th and 6th decades of life. PMW occurs when the muscle is replaced by fibrous tissue and fat. In order to delay the fatty muscle replacement, it’s been suggested that continuous treatment may be needed.2

People with PMW often require mobility aids for daily activities, such as a cane, walker or wheelchair.

Discover the PPP diagnostic journey

PPP subtypes

There are several subtypes of PPP with varying prevalence. The subtypes can be distinguished by the different gene mutations and the channel proteins these mutations affect.3

Most common:

Hypokalemic (HypoPP) (~1 in every 100,000)3,6

  • Associated with decreased serum levels of potassium during an episode.
 
  • Flaccid muscle weakness that can last for at least several hours.

Hyperkalemic (HyperPP) (~1 in every 200,000)1,3,8

  • Associated with elevated or normal serum levels of potassium during an episode.
 
  • More focal muscle weakness in the thigh and calf muscles.1

Least common:

Paramyotonia congenita (<1 in every 100,000)1,7

  • Associated with normal or elevated serum levels of potassium during an episode.
 
  • Sustained myotonia that prevents muscles from relaxing.

Andersen-Tawil syndrome (ATS) (1 in 1 million)3*

  • Associated with low, normal or elevated serum levels of potassium during an episode.

  • Flaccid muscle weakness, abnormal skeletal features and cardiac abnormalities (ventricular arrhythmia, prolonged QT interval, prominent U waves).

*Patients with ATS were not included in clinical trials for KEVEYIS.

What triggers PPP attacks?1,3

Primary periodic paralysis (PPP) triggers can vary from one patient to the next and depend on the type of PPP. It’s important to work with your patients and consider their subtype to help identify their individual triggers.


Common triggers include:

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

PPP attacks: What to look for

Signs and symptoms (attacks) of PPP are often nonspecific and may have varying clinical presentations among patients.

Attacks range in severity1:


In a study* of patients with hyperkalemic periodic paralysis surveyed about their disease:

  • 43.3% said most of their attacks were mild (defined as “some limitations on mobility, others would notice I am in an attack”).

 

  • 15.6% said their attacks were either severe (defined as “can speak, cannot move at all, can call for help”) or very severe (defined as “cannot speak, cannot call for help”).

Attacks range in frequency2†:


  • In this survey, attack frequency was 59% weekly, 28% daily and no attacks in 11%.

 

  • As patients age, the frequency of attacks may either increase or decrease. In about two-thirds of patients in one study, there was no change in frequency.

Patients with PPP often report1:


  • Attacks often occur in the morning, which may make getting out of bed difficult.
 
  • Muscle weakness due to rest after exercise.3
 
  • Requiring support from a family member or cane to walk during an episode.
 
  • In severe cases, difficulty breathing.

*Based on a survey of 137 adults (>18 years of age) with a diagnosis of hyperkalemic PPP and a genetic diagnosis. Percentages are based on the total number of respondents who answered a given question.

†Based on a survey of 66 self-selected patients over the age of 40 years with a clinical diagnosis of PPP who sought support via the internet.

Download the PPP Disease Education brochure

Symptoms of attacks may linger after a paralytic episode and can include1:

Learn more about symptoms and triggers in the PPP Disease Education brochure.

Clumsiness

Palpitations

Weakness

Extreme fatigue

Pain

Learn more about diagnosing PPP

PATIENT IMPACT

Learn more about PPP’s impact on patient lives

PPP can have a significant physical and emotional impact on patients’ lives. Patients report experiencing lingering symptoms.1 Fear of PPP episodes can cause stress, work and personal life challenges, and social anxiety.

The burden of PPP attacks is not only physical:

Living with PPP can be stressful, and since stress can trigger attacks, it may compound the problem.1

Get helpful information on PPP, from diagnosis to treatment

The Diagnostic Journey

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Impact on a PPP Patient's Life

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Management and Treatment

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with PPP

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References

  1. 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.
  2. 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.
  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. Data on file. Chicago, IL: Xeris Pharmaceuticals, Inc.
  5. 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.
  6. Cheng C-J, Kuo E, Huang C-L. Extracellular potassium homeostasis: insights from hypokalemic periodic paralysis. Semin Nephrol. 2013;33:237-247.
  7. Paramyotonia congenita. MedlinePlus. Updated August 1, 2015. Accessed June 7, 2024.https://medlineplus.gov/genetics/condition/paramyotonia-congenita/
  8. Cannon SC. Channelopathies of skeletal muscle excitability. Compr Physiol. 2015;5:761-790.

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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