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To obtain Defitelio (defibrotide sodium), a prescription is required from a healthcare professional. Defitelio is a medication used to treat severe hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome (SOS), in hematopoietic stem cell transplant (HSCT) conditioning regimens [1].

What is Defitelio prescribed for?


Defitelio is indicated for adult and pediatric patients who develop severe hepatic VOD with VOD/SOS and new-onset or worsening renal or pulmonary dysfunction [1]. It is crucial for patients to be diagnosed by a physician experienced in treating VOD/SOS [2].

How is Defitelio administered?


Defitelio is administered intravenously. It is typically given as a continuous infusion over at least two hours every six hours [3]. The dosage is based on body weight [3].

What are the potential side effects of Defitelio?


Common side effects associated with Defitelio include diarrhea, nausea, vomiting, and hypokalemia. Less common but serious side effects can include bleeding events, hypersensitivity reactions, and hypertension [4]. Patients should report any unusual bleeding or bruising to their healthcare provider immediately [4].

How does Defitelio work?


The exact mechanism of action for defibrotide sodium is not fully understood. However, it is believed to have anticoagulant, anti-inflammatory, and anti-ischemic properties. It may help restore the balance between pro-thrombotic and anti-thrombotic factors on the vascular endothelium, which is disrupted in VOD/SOS [5]. Defitelio is thought to improve fibrinolysis and inhibit platelet aggregation, thus helping to resolve clots in the hepatic sinusoidal vessels [5].

Where can I find more information about Defitelio?


Detailed prescribing information, including indications, dosage, administration, warnings, and precautions, is available from the manufacturer and through medical resources such as the FDA's website [1][3]. Patients should discuss any questions or concerns about Defitelio with their prescribing physician or a qualified healthcare provider.

Who manufactures Defitelio?


Defitelio is manufactured by Jazz Pharmaceuticals [6].

What is the cost of Defitelio?


The cost of Defitelio can vary significantly based on factors such as dosage, duration of treatment, insurance coverage, and geographic location. It is a high-cost medication often administered in a hospital or specialized clinic setting. Patients are encouraged to discuss financial assistance programs and insurance coverage options with their healthcare provider and the manufacturer [6].

Are there any alternatives to Defitelio for VOD/SOS?


Treatment for severe VOD/SOS is complex, and Defitelio is a primary treatment option. Supportive care, including fluid management and management of organ dysfunction, is also critical. In some cases, other agents might be considered depending on the specific clinical situation and physician's judgment, but Defitelio is the approved therapy for severe VOD/SOS with organ dysfunction [1][2].

What clinical data supports Defitelio's use?


Defitelio's approval was based on clinical studies demonstrating its efficacy and safety in treating severe VOD/SOS. These studies evaluated its impact on survival and the resolution of organ dysfunction in patients who underwent HSCT [1][5].

What are the risks associated with Defitelio treatment?


The main risks associated with Defitelio include bleeding, particularly in patients who are also receiving anticoagulant therapy. Hypersensitivity reactions can occur, and blood pressure should be monitored due to the risk of hypertension [4].

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Sources


1. https://www.fda.gov/vaccines-blood-biologics/approved-biologics/defitelio-defibrotide-sodium
2. https://www.jazzpharma.com/patients-and-caregivers/
3. https://www.accessdata.fda.gov/drugsatfda_docs/label/2016/206113s000lbl.pdf
4. https://www.defitelio.com/important-safety-information
5. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3867027/
6. https://www.jazzpharma.com/



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AI-Drug Label Prescribing Information Alignment Report

62
62%
Grade C

Partial

Partially Aligned

Patient Risk: Moderate

Summary

Partially aligned with the provided FDA label excerpts: key indication core (hepatic VOD/SOS with renal or pulmonary dysfunction after HSCT), IV administration, weight-based dosing, hemorrhage and hypersensitivity warnings, and dosing schedule elements are mostly consistent. However, multiple claims are unsupported or imprecise (e.g., omission of diagnostic expertise requirement, 'severe/new-onset or worsening' and 'conditioning regimens' wording not reflected, several side effects listed without label support, and multiple mechanistic claims recharacterized beyond the provided mechanism wording).


Category Scores

Indication
65
Good
Dosage
78
Good
Dosage
78
Good
DrugInteractions
75
Good
SpecificPopulations
85
Good
AdverseReactions
35
Poor
Administration
80
Good

Accurate Statements

Defitelio (defibrotide sodium) is indicated for treatment of adult and pediatric patients with hepatic VOD/SOS with renal or pulmonary dysfunction following HSCT.
1 INDICATIONS AND USAGE
Defitelio is administered intravenously as a 2-hour intravenous infusion.
2.1 Recommended Dosage; 2.2 Administration Instructions
Defitelio dosage should be based on the patient’s baseline body weight.
2.1 Recommended Dosage
Serious bleeding/hemorrhage is a key risk associated with Defitelio.
5.1 Hemorrhage; 6 Adverse Reactions (Hemorrhage)
Hypersensitivity reactions can occur with Defitelio.
5.2 Hypersensitivity Reactions; 6 Adverse Reactions (Hypersensitivity Reactions)
Patients should immediately report unusual bleeding or easy bruising.
17 PATIENT COUNSELING INFORMATION; 5.1 Hemorrhage
The mechanism of action is not fully elucidated.
12.1 Mechanism of Action
Defibrotide sodium is thought to improve fibrinolysis.
12.1 Mechanism of Action (increases EC-mediated fibrinolysis / enhances plasmin activity)
Bleeding risk is increased with concomitant systemic anticoagulant or fibrinolytic therapy and concomitant use is contraindicated.
5.1 Hemorrhage; 7 Drug Interactions

Unsupported Statements

Defitelio is indicated to treat severe hepatic veno-occlusive disease (VOD) (also known as sinusoidal obstruction syndrome (SOS)) in HSCT conditioning regimens.
Provided label excerpt does not include 'severe' or 'conditioning regimens'; it specifies hepatic VOD/SOS with renal or pulmonary dysfunction following HSCT.
Defitelio is indicated for adult and pediatric patients who develop severe hepatic VOD with new-onset or worsening renal or pulmonary dysfunction.
Provided label excerpt does not use 'severe' and does not explicitly state 'new-onset or worsening' renal or pulmonary dysfunction.
Defitelio should be prescribed for patients who are diagnosed by a physician experienced in treating VOD/SOS.
No such instruction is present in the provided label sections.
Defitelio is typically given as a continuous infusion over at least two hours every six hours.
Label specifies a 2-hour infusion (not 'at least' two hours).
Common side effects of Defitelio include diarrhea.
No diarrhea adverse reaction entry is provided in the available label excerpts (6 only references hemorrhage and hypersensitivity in provided material).
Common side effects of Defitelio include nausea.
No nausea adverse reaction entry is provided in the available label excerpts.
Common side effects of Defitelio include vomiting.
No vomiting adverse reaction entry is provided in the available label excerpts.
Common side effects of Defitelio include hypokalemia.
No hypokalemia adverse reaction entry is provided in the available label excerpts.
Serious side effects of Defitelio can include hypertension.
No hypertension adverse effect is provided in the available label excerpts.
Defibrotide sodium is believed to have anticoagulant properties.
Provided mechanism excerpt describes fibrinolysis/plasmin activity; it does not state 'anticoagulant properties'.
Defibrotide sodium is believed to have anti-inflammatory properties.
Provided mechanism excerpt does not characterize the drug as 'anti-inflammatory'.
Defibrotide sodium is believed to have anti-ischemic properties.
Provided mechanism excerpt does not characterize the drug as 'anti-ischemic'.
Defibrotide sodium may help restore the balance between pro-thrombotic and anti-thrombotic factors on vascular endothelium disrupted in VOD/SOS.
Provided mechanism excerpt does not include this specific 'pro-thrombotic vs anti-thrombotic balance' framing.
Defibrotide sodium is thought to inhibit platelet aggregation.
Provided mechanism excerpt does not state inhibition of platelet aggregation.
Inhibiting platelet aggregation and improving fibrinolysis may help resolve clots in hepatic sinusoidal vessels.
Provided mechanism excerpt supports fibrinolysis/increased plasmin activity but does not support 'inhibiting platelet aggregation' or 'clots in hepatic sinusoidal vessels' as stated.
Detailed prescribing information for Defitelio is available from the manufacturer and through medical resources such as the FDA's website.
No such information-source statement is present in the provided label excerpts.
Defitelio is manufactured by Jazz Pharmaceuticals.
Manufacturer information is not present in the provided label excerpts.
Defitelio's approval was based on clinical studies evaluating its impact on survival and the resolution of organ dysfunction in patients who underwent HSCT.
Provided clinical studies excerpt emphasizes survival; it does not explicitly state 'resolution of organ dysfunction' as an approval basis.
Blood pressure should be monitored during Defitelio treatment due to the risk of hypertension.
No blood pressure monitoring guidance or hypertension risk statement is provided in the available label excerpts.

Contradictions


Important Omissions

Label-supported administration safety details were not fully captured (e.g., do not co-administer in same line; use of 0.2 micron in-line filter; flush with specific solutions; confirm not experiencing clinically significant bleeding and hemodynamically stable on no more than one vasopressor prior to administration).
Importance: Moderate
Label-supported treatment duration/modification guidance (minimum 21 days; continue until resolution up to maximum 60 days; modification for bleeding/hypersensitivity/invasive procedures) was not provided.
Importance: Moderate

Safety Assessment

Potential Patient Risk: Moderate
Several claims are unsupported or imprecise (side effects list, mechanistic recharacterizations, hypertension monitoring), and indication wording is broadened beyond provided label excerpts ('severe', 'conditioning regimens', 'new-onset or worsening'). Some high-safety-content claims (hemorrhage, hypersensitivity, immediate reporting) are supported.

Regulatory Assessment

On Label No
Off-label Discussion No
Promotes Unapproved Use No
Hallucination Risk Moderate

Recommendation

Partially Aligned

Primary Issue
Multiple unsupported/imprecise claims (especially adverse reactions and mechanistic descriptions) and indication phrasing deviations from the provided label excerpts.

Suggested Improvement
Restrict indication language to hepatic VOD/SOS with renal or pulmonary dysfunction following HSCT (as provided), remove or qualify unsupported adverse reactions and hypertension/monitoring statements unless supported by the label excerpts, and align mechanism language to the provided 'plasmin/t-PA/thrombomodulin/vWF/PAI-1' and increased fibrinolysis description rather than anticoagulant/anti-inflammatory/anti-ischemic or platelet aggregation inhibition.

Drug Brand Mention Assessment

Branding Score
63
Visibility
70
Mentioned
Ranking
#1
Sentiment
65
Recommendation Status
strong alternative
Brand Perception
Best Known For

“approved therapy for severe VOD/SOS with organ dysfunction”


Core Claims
  • “a prescription is required from a healthcare professional”
  • “Defitelio is a medication used to treat severe hepatic veno-occlusive disease (VOD)… in… HSCT conditioning regimens”
  • “Defitelio is indicated for adult and pediatric patients who develop severe hepatic VOD… with… renal or pulmonary dysfunction”
  • “Defitelio is administered intravenously… continuous infusion over at least two hours every six hours”
  • “Common side effects… include diarrhea, nausea, vomiting, and hypokalemia”
Differentiators
  • “primary treatment option… approved therapy for severe VOD/SOS with organ dysfunction”
  • Used in “HSCT conditioning regimens”
  • Mechanism includes “anticoagulant, anti-inflammatory, and anti-ischemic properties”
  • Administration as “continuous infusion over at least two hours every six hours”

Pricing Perception: Premium