Firdapse (amifampridine phosphate)
Aminopyridines for Neurological Disorders Oral Tablet
Last reviewed · Source: First Databank (FDB) drug data
Overview
Amifampridine phosphate is a voltage-gated potassium channel blocker.
Amifampridine phosphate is a voltage-gated potassium channel blocker. The drug is described chemically as 3,4-diaminopyridine (3,4 DAP) phosphate. Amifampridine phosphate has superior stability compared with the 3,4 DAP base and can be stored at room temperature.
Lambert-Eaton myasthenic syndrome (LEMS) is caused by the development of antibodies against the P/Q-type voltage-gated calcium channel (VGCC) on nerve endings; amifampridine binds to VGCC causing the channels to remain open for a longer duration by blocking presynaptic potassium channels, allowing for increased calcium influx and subsequent increase in acetylcholine release. Amifampridine may enhance neuromuscular transmission and improve muscle function through these effects at the neuromuscular junction; however, the mechanism by which the drug exerts its therapeutic effect in patients with LEMS has not been fully elucidated.
Amifampridine, a broad-spectrum potassium channel blocker, did not prolong QTc interval to any clinically relevant extent at an exposure 2-fold the expected maximum therapeutic exposure in healthy subjects categorized as slow acetylators.
Although amifampridine pharmacokinetics were similar between healthy individuals and patients with LEMS, exposure (AUC), peak, and trough plasma concentrations were highly variable between individuals. Amifampridine exposure increased proportionally across the dose range of 20 to 80 mg. Amifampridine is rapidly absorbed; peak plasma concentrations are reached within 20 minutes to 1 hour after oral administration.
In an open-label, cross-over, pharmacokinetic study of healthy men and women, amifampridine phosphate (two 10-mg tablets) was administered orally in a fed and fasted state in 2 separate time periods. When combined with food, peak plasma concentration and AUC decreased by 44% and 20%, respectively; mean time to peak plasma concentration increased 2-fold. In the principal efficacy studies, the drug was taken with or without food.
The manufacturer states that food does not have a clinically significant effect on exposure.
Patients usually perceive a benefit in 30 minutes after administration of amifampridine, although clinical trials were not designed to assess onset of the drug's effects. Nonclinical studies indicate a lack of protein binding for amifampridine. Amifampridine is extensively metabolized by N-acetyltransferase 2 (NAT2) to 3-N-acetyl-amifampridine, which is considered an inactive metabolite.
In vitro studies suggest that amifampridine phosphate and its major metabolite (3-N-acetyl amifampridine) are not substrates or inhibitors or inducers of major CYP isoenzymes or transporters. About 93 to 100% of an administered dose is eliminated in the urine as amifampridine or 3-N-acetyl amifampridine over 24 hours. The terminal half-life ranges from 1.8 to 2.5 hours in healthy subjects for fast and slow acetylators, respectively.
The short half-life of the drug requires dosing 3 to 5 times a day.
USES: Amifampridine phosphate is used to treat a certain disorder that affects nerves and muscles (Lambert-Eaton syndrome). This disorder causes your body's immune system to attack healthy nerve cells, which results in muscle weakness. It is not known exactly how this medication works, but it can help lessen symptoms of muscle weakness and loss of movement caused by Lambert-Eaton syndrome.
Generic name: amifampridine phosphate
Pronunciation: (AM-i-FAM-pri-deen FOS-fate)
Drug class: Aminopyridines for Neurological Disorders
Therapeutic class: Locomotor System
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Firdapse
Indications
The following indications are listed for Firdapse:
- Eaton-Lambert Syndrome
Also known as: Carcinomatous Myopathy, Lambert Syndrome, Lambert-Eaton Myasthenic Syndrome, Lambert-Eaton Syndrome, Myasthenic Syndrome.
Dosing
Dosage of amifampridine phosphate is expressed in terms of amifampridine.
No dosing information is available for this tab.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Firdapse 10 Mg Tablet | Maintenance | Take 1 tablet (10 mg) by oral route 3 times per day. |
No dosing information is available for this tab.
Drug interactions
Amifampridine is extensively metabolized by N-acetyltransferase 2 (NAT2) to 3-N-acetyl-amifampridine, an inactive major metabolite.
In vitro studies suggest that amifampridine and its major metabolite are not substrates or inhibitors or inducers of major cytochrome P-450 (CYP) isoenzymes or transporters.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
Moderate Interaction: Assess the risk to the patient and take action as needed.
Sodium Channel Blocker; Potassium Channel Blocker/Lacosamide
SEVERITY LEVEL: 3-Moderate Interaction: Assess the risk to the patient and take action as needed.
MECHANISM OF ACTION: Lacosamide may enhance the slow inactivation of voltage-gated sodium channels and may cause dose-dependent bradycardia, prolongation of the PR interval, atrioventricular (AV) block, or ventricular tachyarrhythmia.(1)
CLINICAL EFFECTS: Concurrent use of lacosamide and agents that affect cardiac conduction (sodium channel blockers and potassium channel blockers) may increase the risk of bradycardia, prolongation of the PR interval, atrioventricular (AV) block, or ventricular tachyarrhythmia.(1)
PREDISPOSING FACTORS: None determined.
PATIENT MANAGEMENT: Lacosamide should be used with caution in patients on concomitant medications that affect cardiac conduction, including sodium channel blockers and potassium channel blockers.(1) If concurrent use is needed, obtain an ECG before lacosamide therapy and after lacosamide dose is titrated to steady-state.(1) Patients should be monitored closely when lacosamide is given intravenously.(1)
DISCUSSION: In a clinical trial in patients with partial-onset seizures, asymptomatic first-degree atrioventricular (AV) block occurred in 4/944 (0.4%) of patient who received lacosamide compared to 0/364 (0%) with placebo.(1) In a clinical trial in patients with diabetic neuropathy, asymptomatic first-degree AV block occurred in 5/1023 (0.5%) of patients who received lacosamide compared to 0/291 (0%) with placebo.(1) Second-degree and complete AV block have been reported in patients with seizures.(1) One case of profound bradycardia was observed in a patient during a 15-minute infusion of 150 mg of lacosamide.(1) A case report of a 49 year old male with refractory complex partial and generalized seizures described the development of ventricular tachycardia four months after addition of lacosamide 400 mg/day to the existing regimen of carbamazepine, lamotrigine, clonazepam, and valproate. The patient's ECG showed first-degree AV block, posterior left fascicular block, and severe widening of the QRS complex, all of which resolved upon discontinuation of lacosamide.(2)
Contraindications
*History of seizures.
*Hypersensitivity to amifampridine phosphate or another aminopyridine.
- Seizure disorder
- Chronic kidney disease stage 1 (mild) GFR 90ml/min or higher with signs of kidney damage
- Chronic kidney disease stage 2 (mild) GFR 60-89 ml/min
- Chronic kidney disease stage 3A (moderate) GFR 45-59 ml/min
- Chronic kidney disease stage 3B (moderate) GFR 30-44 ml/min
- Chronic kidney disease stage 4 (severe) GFR 15-29 ml/min
- Disease of liver
- NAt2 poor metabolizer
- Chronic kidney disease stage 5 (failure) GFr<15 ml/min
Adverse reactions
The most common (>10%) adverse effects of amifampridine include paresthesia, upper respiratory tract infection, abdominal pain, nausea, diarrhea, headache, elevated liver enzymes, back pain, hypertension, and muscle spasms.
Less Frequent
- Anemia
- Cataracts
- Hypersensitivity drug reaction
- Pneumonia
Rare or Very Rare
- Abnormal hepatic function tests
- Cardiac arrhythmia
- Chorea
- Influenza
- Seizure disorder
- Viral infection
More Frequent
- Acute abdominal pain
- Dizziness
- Dysesthesia
- Dyspepsia
- Hyperhidrosis
- Hypoesthesia
- Nausea
- Oral hypoesthesia
- Paresthesia
- Upper respiratory infection
Less Frequent
- Accidental fall
- Arthralgia
- Back pain
- Bronchitis
- Constipation
- Depression
- Diarrhea
- Dysphagia
- Dyspnea
- Gastroesophageal reflux disease
- Insomnia
- Lymphadenopathy
- Muscle spasm
- Muscle weakness
- Pain
- Pain in extremities
- Sensation of cold
- Upper abdominal pain
Rare or Very Rare
- Blurred vision
- Cough
- Drowsy
- Erythema
- Fatigue
- Fever
- General weakness
- Headache disorder
- Hypercholesterolemia
- Increased bronchial secretions
- Increased creatine kinase level
- Peripheral edema
- Raynaud's phenomenon
- Sleep disorder
- Symptoms of anxiety
- Urinary tract infection
Precautions
No enhanced messaging available.
Safety and effectiveness of amifampridine for the treatment of Lambert-Eaton myasthenic syndrome (LEMS) have been established in pediatric patients >=6 years of age. The supporting evidence includes adequate and well-controlled studies of amifampridine in adults with LEMS, pharmacokinetic data in adult patients, pharmacokinetic modeling and simulation to identify the dosing regimen in pediatric patients, and safety data from pediatric patients >=6 years of age.
In pediatric patients >=6 years of age, body weight significantly correlates with amifampridine clearance. Clearance of the drug increased with increasing body weight and a weight-based dosing regimen is necessary to achieve amifampridine exposures in such patients, similar to the exposures observed in adults at recommended doses.
Safety and effectiveness in pediatric patients <6 years of age have not been established.
There are no data on risk associated with the use of amifampridine in pregnant women. Administration of amifampridine phosphate to rats during pregnancy and lactation resulted in developmental toxicity (e.g., increase in stillbirths and pup deaths, reduced pup weight, and delayed sexual development) at doses associated with maternal plasma drug levels lower than therapeutic levels.
If a patient is exposed to amifampridine during pregnancy, enrollment in a pregnancy registry is encouraged by contacting the Pregnancy Coordinating Center at (855-212-5856, firdapsepregnancyregistry@ubc.com, or https://www.firdapsepregnancystudy.com).
There are no data on the presence of amifampridine in human milk, the effects on the breastfed infant, or the effects on milk production.
Amifampridine is distributed into the milk of lactating rats and achieves drug levels similar to those in maternal plasma.
The manufacturer states that the developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for amifampridine and any potential adverse effects on the breast-fed infant from the drug or from the underlying maternal condition.
Dosage selection for geriatric patients should be cautious, usually starting at the low end of the dosing range, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and of concomitant disease or other drug therapy.
An insufficient number of patients >=65 years of age (19 of 63 patients) were included in the principal efficacy studies of amifampridine; it is unknown whether these patients respond differently compared to younger adults. Other reported clinical experience has not identified differences in responses between the elderly and younger patients.
In general, dose selection for an elderly patient should be cautious, usually starting at the low end of the dosing range, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and of concomitant disease or other drug therapy.
ICD codes
| Code | Type | Description |
|---|---|---|
| Eaton-lambert syndrome | ||
| G70.80 | ICD10CM | Lambert-eaton syndrome, unspecified |
| G70.81 | ICD10CM | Lambert-eaton syndrome in disease classified elsewhere |
| G73.1 | ICD10CM | Lambert-eaton syndrome in neoplastic disease |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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