For US Residents

Understanding
Carnitine Deficiency

Carnitine deficiency is a metabolic disorder that can affect you or your loved ones.

Carnitine deficiency is a medical condition defined by low levels of carnitine.

When levels of carnitine are too low, cells may not be able to produce the energy they need to perform their tasks, which can cause symptoms such as tiredness, muscle weakness, heart problems and others.1

Carnitine deficiency can also lead to other unhealthy changes in the body, including low blood sugar, liver enlargement and elevated levels of ammonia and elevated levels of toxic byproduct of metabolism in the blood.2

There are two major forms of carnitine deficiency.

Primary carnitine deficiency is caused by a gene mutation that directly affects the synthesis or transport of carnitine.

Genes carry instructions for making proteins in the body. A gene mutation is an error in the instructions for making a specific protein. Primary carnitine deficiency occurs when a mutation is present in a gene that carries the code for making a protein that shuttles carnitine into cells. As a result, production of this important protein is inadequate or absent altogether, which means that carnitine is unable to enter cells and perform its key role in energy production.3

Secondary carnitine deficiency has several potential causes, including:

A genetic mutation in the metabolic process that indirectly affects carnitine levels.

Hemodialysis for end-stage renal disease and for other forms of kidney failure.

People undergoing hemodialysis are at risk for secondary carnitine deficiency because4:

  • carnitine loss due to hemodialysis
  • impaired carnitine production due to kidney disease
  • patients are often restricted from eating carnitine-rich foods

If you are on hemodialysis, talk to your doctor about getting tested and treated for carnitine deficiency.

Signs and Symptoms of carnitine deficiency and how it can affect the body

A person with carnitine deficiency may or may not have any signs or symptoms, though this condition can cause serious health problems. The signs and symptoms experienced by a person with carnitine deficiency and the age when they first appear will depend on its cause and how severe the deficiency is. It is important to work closely with your doctor to develop a personalized management plan. Carnitine deficiency may cause the following:3,5
  • A condition called encephalopathy that is a result of impaired brain function and may cause confusion, memory loss and other symptoms
  • A failing heart, a condition called cardiomyopathy
  • Abdominal pain
  • Muscle weakness, aches and cramps
  • Fatigue
  • Vomiting
  • Diarrhea
  • Low blood sugar
  • Anemia (low red blood cells) which can cause some fatigue and malaise

Severe carnitine deficiency also increases the risk of serious health issues.

A simple blood test can diagnose carnitine deficiency.

Newborns are screened for primary carnitine deficiency at birth. To learn more, visit Baby’s First Test.

Talk to your healthcare team to find out if CARNITOR® may be an appropriate treatment for your carnitine deficiency.

LEARN MORE

Sources

  1. Longo N, Frigeni M, Pasquali M. Carnitine Transport and Fatty Acid Oxidation. Biochim Biophys Acta. 2016;1863(10):2422-2435. doi:10.1016/j.bbamcr.2016.01.023
  2.  Carnitine: Fact Sheet for Health Professionals. National Institutes of Health. Updated April 17, 2023. Accessed September 20, 2023. https://ods.od.nih.gov/factsheets/Carnitine-HealthProfessional/.
  3.  Primary Carnitine Deficiency: MedlinePlus Genetics. MedlinePlus. Updated November 27, 2023. Accessed September 20, 2023. https://medlineplus.gov/genetics/condition/primary-carnitine-deficiency/#causes.
  4.  Evans AM, Faull R, Fornasini G, et al. Pharmacokinetics of L-carnitine in patients with end-stage renal disease undergoing long-term hemodialysis. Clin Pharmacol Ther. 2000;68(3):238-249. doi:10.1067/mcp.2000.108850
  5.  Eknoyan G, Latos DL, Lindberg J. Practice Recommendations for the Use of L-Carnitine in Dialysis-Related Carnitine Disorder. National Kidney Foundation. Carnitine Consensus Conference. American Journal of Kidney Diseases. 2003;41(4):868-876. doi:10.1016/s0272-6386(03)00110-0

INDICATIONS AND IMPORTANT SAFETY INFORMATION 

Indications  
CARNITOR® (levocarnitine) Tablets, Oral Solution, and Sugar-Free Oral Solution are indicated for the treatment of primary systemic carnitine deficiency.
CARNITOR® (levocarnitine) Injection is indicated for the prevention and treatment of carnitine deficiency in patients with end stage renal disease who are undergoing dialysis.
CARNITOR® (levocarnitine) Injection, Tablets, Oral Solution, and Sugar-Free Oral Solution are also indicated for the acute and chronic treatment of patients with an inborn error of metabolism which results in secondary carnitine deficiency.

IMPORTANT SAFETY INFORMATION

Contraindications 

None known.

Warnings

Carnitor®(levocarnitine) Tablets, Oral Solution, and Sugar-Free Oral Solution

Hypersensitivity Reactions

Serious hypersensitivity reactions, including rash, urticaria, and facial edema have been reported with oral CARNITOR®. Other serious hypersensitivity reactions, including anaphylaxis, laryngeal edema, and bronchospasm have been reported following intravenous levocarnitine administration, mostly in patients with end stage renal disease undergoing dialysis.

Discontinue use of CARNITOR® and instruct patients to seek medical attention if they experience symptoms suggestive of a hypersensitivity reaction.

Carnitor® (levocarnitine) Injection

Hypersensitivity Reactions

Serious hypersensitivity reactions, including anaphylaxis, laryngeal edema, and bronchospasm have been reported following CARNITOR® administration, mostly in patients with end stage renal disease who are undergoing dialysis. Some reactions occurred within minutes after intravenous administration of CARNITOR® .

If a severe hypersensitivity reaction occurs, discontinue CARNITOR® treatment and initiate appropriate medical treatment. Consider the risks and benefits of re-administering CARNITOR® to individual patients following a severe reaction. If the decision is made to re-administer the product, monitor patients for a reoccurrence of signs and symptoms of a severe hypersensitivity reaction.

Precautions

General

The safety and efficacy of oral levocarnitine has not been evaluated in patients with renal insufficiency. Chronic administration of high doses of oral levocarnitine in patients with severely compromised renal function or in ESRD patients on dialysis may result in accumulation of the potentially toxic metabolites, trimethylamine (TMA) and trimethylamine-N-oxide (TMAO), since these metabolites are normally excreted in the urine.

Carnitor® (levocarnitine) Tablets, Oral Solution, and Sugar-Free Oral Solution

CARNITOR® (levocarnitine) Oral Solution and CARNITOR® SF (levocarnitine) Sugar-Free Oral Solution are for oral/internal use only.  Not for parenteral use

Gastrointestinal reactions may result from a too rapid consumption of carnitine. CARNITOR® (levocarnitine) Oral Solution and CARNITOR® SF (levocarnitine) Sugar-Free Oral Solution may be consumed alone, or dissolved in drinks or other liquid foods to reduce taste fatigue. They should be consumed slowly and doses should be spaced evenly throughout the day to maximize tolerance.

Drug Interactions

Reports of INR increase with the use of warfarin have been observed. It is recommended that INR levels be monitored in patients on warfarin therapy after the initiation of treatment with levocarnitine or after dose adjustments.

Adverse Reactions

The following adverse reactions associated with the use of levocarnitine were identified in clinical trials or postmarketing reports. Because these reactions were reported voluntarily from a population of uncertain size, it is not always possible to estimate their frequency, reliability, or to establish a causal relationship to drug exposure.

Neurologic Reactions: Seizures have been reported to occur in patients, with or without pre-existing seizure activity, receiving either oral or intravenous levocarnitine. In patients with pre-existing seizure activity, an increase in seizure frequency and/or severity has been reported.

Carnitor® (levocarnitine) Tablets, Oral Solution, and Sugar-Free Oral Solution

Gastrointestinal Reactions: Various mild gastrointestinal complaints have been reported during the long-term administration of oral L- or D,L-carnitine; these include transient nausea and vomiting, abdominal cramps, and diarrhea. Gastrointestinal adverse reactions with CARNITOR® (levocarnitine) Oral Solution or CARNITOR® SF (levocarnitine) Sugar-Free Oral Solution dissolved in liquids might be avoided by a slow consumption of the solution or by a greater dilution. Decreasing the dosage often diminishes or eliminates drug-related patient body odor or gastrointestinal symptoms when present. Tolerance should be monitored very closely during the first week of administration, and after any dosage increases.

Musculoskeletal Reactions: Mild myasthenia has been described only in uremic patients receiving D,L-carnitine.

Hypersensitivity Reactions: Rash, urticaria, and facial edema have been reported with oral CARNITOR® (see WARNINGS).

Carnitor® (levocarnitine) Injection

Gastrointestinal Reactions: Transient nausea and vomiting have been observed. Less frequent adverse reactions are body odor, nausea, and gastritis. An incidence for these reactions is difficult to estimate due to the confounding effects of the underlying pathology.

Hypersensitivity reactions: Anaphylaxis, laryngeal edema and bronchospasm (see WARNINGS).

To report SUSPECTED ADVERSE REACTIONS, contact Leadiant Biosciences, Inc. at 1-888-393-4584 or by email at drugsafety@leadiant.com or contact the FDA at 1-800-FDA-1088 or www.fda.gov/safety/medwatch.

The risk information provided here is not comprehensive. Refer to the full FDA-approved product labeling.

© 2024, Leadiant Biosciences, Inc.

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