Important Safety Information

Prescribe Keveyis

Primary Periodic Paralysis: Helping to simplify a complex diagnosis.

Every patient’s journey to a Primary Periodic Paralysis (PPP) diagnosis is different. The established diagnostic protocol can help speed up the path to treatment.

On average, it can take 20+ years to receive a diagnosis2

PPP often has nonspecific and episodic symptoms that mimic those of more common diseases — like cardiovascular or mental health disorders — resulting in years of diagnostic delays and misdiagnoses.1

The average time from onset of symptoms to correct diagnosis is 26 years.2*

As a healthcare provider, you’re in a unique position to help someone with PPP get a diagnosis and start treatment.

If you think you may have a patient with PPP, follow the below protocol. The sooner you can diagnose them, the sooner they can start to gain greater control over their symptoms.

*Based on a survey of 66 self-selected patients over the age of 40 years with a clinical diagnosis of Primary Periodic Paralysis who sought support via the Internet.

The PPP diagnostic journey

Because PPP is so rare, many patients have a challenging time receiving an accurate diagnosis.3

Diagnosing a patient with PPP can be a straightforward process by following the established protocol below.

An established diagnostic protocol for PPP

In a sponsored roundtable discussion, expert clinicians in the field created a white paper that includes a detailed diagnostic algorithm for Primary Periodic Paralysis, which can help you identify PPP in patients and speed up their path to treatment.

Get the Diagnostic Journey white paper

Signs and symptoms of PPP

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 questions.

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

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

Clumsiness

Palpitations

Weakness

Extreme fatigue

Pain

Genetic Testing

The genetic connection

PPP is usually an inherited, chronic, autosomal dominant disorder. However, patients can still have PPP without a genetic mutation if they have the signs and symptoms.3
  • About 30% of people with PPP do not have one of the common genetic mutations associated with it.3
A genetic test can help shorten the journey to diagnosis and speed up a path to treatment. Learn more about a no-cost genetic testing program for patients and family members who have symptoms of PPP.
Get the Genetic Testing guide

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.

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/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 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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