Discover KEVEYIS®: Proven to treat
Primary Periodic Paralysis1,2

Learn more about the first FDA-approved drug shown to reduce the frequency, severity and duration of PPP attacks.1,2

ABOUT KEVEYIS

KEVEYIS (dichlorphenamide) is a long-term treatment that should be taken every day. It has been proven to work — controlling the frequency, severity and duration of PPP attacks — in 2 clinical studies.1,2

Clinical trial results

From baseline to week 9, treatment with KEVEYIS decreased weekly attack rates while treatment with placebo resulted in increases.1,2†

†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.

‡Treatment effects in the HyperPP and HypoPP substudies were -4.1 and -2.2, respectively. 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.

Open-Label Extension (OLE) study

Long-term data from the 1-year study in which all patients received KEVEYIS.2

KEVEYIS for PPP: Long-term data from the 1-year, open-label extension study2

Study design2:

From the 9-week, double-blind phase of Study 1, 41 of 44 patients in the hypokalemic substudy (KEVEYIS, n=22) and 17 of 21 patients in the hyperkalemic substudy (KEVEYIS, n=9) moved into a 1-year, open-label extension (OLE) phase in which all participants received KEVEYIS.

AR=attack rate; IQR=interquartile range; Wk=week.

Average attack rate per week for patients on KEVEYIS at weeks 54-61*

STARTED AND STAYED ON KEVEYIS THROUGH BOTH PHASES:

FOR HYPERKALEMIC PATIENTS (n=9)†

0.3 attacks

AR/Wk, median (IQR)

FOR HYPOKALEMIC PATIENTS (n=17)

0 attacks

AR/Wk, median (IQR)

CROSSED OVER FROM PLACEBO TO KEVEYIS IN OLE

FOR HYPERKALEMIC PATIENTS (n=7)†

0.9 attacks

AR/Wk, median (IQR)

FOR HYPOKALEMIC PATIENTS (n=14)

0.2 attacks

AR/Wk, median (IQR)

*During the 1-year, uncontrolled extension phase, outcomes assessed included attack rate, severity-weighted attack rate, total attack duration per week (from weeks 54-61), and changes from week 9 to 61 in measures of strength and lean body mass. Adverse events and other safety outcomes were also considered.

†Of the 17 patients who were enrolled in the long-term extension of the hyperkalemic PP trial, data were excluded for 1 patient in the 52-week, uncontrolled extension phase, who was included in the 9-week phase, as this participant was randomized to acetazolamide (the acetazolamide arm of the trial had to be abandoned owing to poor recruitment).

KEVEYIS patient case studies

Learn about the experiences of real Primary Periodic Patients who take KEVEYIS.*

HELEN R.

28-year-old patient with paramyotonia congenita
Initial treatment: Mexiletine

Typical attack:
  • Typical duration: 5 to 30 minutes
  • Typical severity: Moderate
  • Cramps and myotonia in hands
  • Fatigue in lower extremities
Attack history:
  • Experienced attacks daily in cold weather months; 3 to 5 times per month in summer
  • Common symptoms include cramping of hands, reduced exercise tolerance
  • Lingering symptoms include stiffness of hands
Treatment history:
  • Mexiletine (150 mg TID): Discontinued after 4 months due to lack of response, flu-like symptoms and gastroesophageal reflux disease (GERD)
After starting KEVEYIS (50 mg BID):

Patient has been on KEVEYIS and receives no other concomitant medications for PPP. Patient initially reported a 4-week period of intermittent confusion, which resolved spontaneously.

MATT W.

23-year-old patient with hypokalemic PPP
Initial treatment: Acetazolamide

Typical attack:
  • Typical duration: 2 to 72 hours
  • Typical severity: Mild to severe
  • Common symptoms before attack: Calf pain or muscle cramps
  • Weakness in leg and hip muscles
  • Weakness in upper extremities during more severe attacks
  • Muscles related to breathing, swallowing or speaking are never involved
Attack history:
  • Attacks began at 3 years of age, 2 to 3 per month
  • Often triggered by high-carb meals or intense physical activity
  • Weakness was worse in the mornings and improved throughout the day
Treatment history:
  • Potassium tablets and acetazolamide 
  • Acetazolamide became ineffective despite escalating dose
After starting KEVEYIS (50 mg BID):

Patient was assessed every 6 months and demonstrated decrease in attack frequency and severity.

*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.

Adverse reactions  

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

Common side effects

It’s normal for patients to experience some side effects when starting KEVEYIS. In a clinical study, some of the most common side effects of KEVEYIS were1:

Tingling or prickling sensations

(pins and needles)

Confusion or trouble paying attention

Changes in taste

Brain fog

How to prescribe KEVEYIS

If you’re ready to prescribe KEVEYIS for a patient, you must start by filling out our Prescription Start Form.

Managing someone on KEVEYIS

It may take up to 9 weeks to find the right dose for your patient and to assess side effects.

PATIENT TESTIMONIALS

Hear from KEVEYIS patients

Patients share their experience of taking KEVEYIS for PPP.

Paul
On KEVEYIS since 2017
“I went from about one [attack] a week to one a month since starting KEVEYIS.”

Paul
KEVEYIS patient
Hear more about Paul ➞
Kirsten
On KEVEYIS since 2016
“After starting KEVEYIS I have no attacks of full body paralysis.”

Kirsten
KEVEYIS patient
Hear more about Kirsten ➞
Dennis
On KEVEYIS since 2016
“Before KEVEYIS, I was having a lot of episodes…it was horrible. [After starting KEVEYIS], I haven’t had a serious attack in years.”

Dennis
KEVEYIS patient
Hear more about Dennis ➞
Janine
On KEVEYIS since 2016
“Having more control over my symptoms on KEVEYIS I feel more confident, more independent.”

Janine
KEVEYIS patient
Hear more about Janine ➞

PATIENT TESTIMONIALS

Xeris CareConnection™ offers patients
the help they need — at no cost

Managing Primary Periodic Paralysis (PPP) takes more than medication alone. That’s why we created Xeris CareConnection™, a range of free support services for the PPP community.

Take the next step

Questions? We’re
here to help.

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representative will be in touch.

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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.

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By submitting this form, I understand I am giving Xeris Pharmaceuticals, Inc., its affiliates, and business partners permission to use the personal information provided in this registration form to contact me by the following methods, but not limited to: mail, email, telephone call or in-person about disease and product information, disease or product-related events, support services, market research, and to share promotional and marketing information. By submitting this form, I consent to these uses and am confirming that I have read and agree to the Xeris Pharmaceuticals® Terms of Use and Privacy Statement. I understand I can unsubscribe by clicking on the unsubscribe link in future communications or by sending a letter with my full contact information (eg, name, address, email, phone, etc) to Xeris CareConnection™ Patient Support Services, 1375 W Fulton Street, Suite 1300, Chicago, IL 60607.

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