deferiprone


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deferiprone

(de-fer-ip-rone) ,

Ferriprox

(trade name)

Classification

Therapeutic: antidotes
Pharmacologic: chelating agents
Pregnancy Category: D

Indications

Treatment of transfusional iron overload due to thalassemia when other chelation regimens are inadequate.

Action

Bonds with ferric ions to form neutral complexes which are then eliminated.

Therapeutic effects

Decrease in iron overload as reflected in decreased ferritin levels.

Pharmacokinetics

Absorption: Well absorbed following oral administration.
Distribution: Unk.
Metabolism and Excretion: Mostly metabolized (by UGT 1A6 enzyme system), 75–90% excreted in urine as metabolites.
Half-life: 1.9 hr.

Time/action profile (blood levels)

ROUTEONSETPEAKDURATION
POwithin 5–10 min1–2 hr8–12 hr

Contraindications/Precautions

Contraindicated in: Hypersensitivity; Obstetric: Pregnancy should be avoided; Lactation: Breast feeding not recommended.
Use Cautiously in: Renal/hepatic impairment (safety and effectiveness not established); Any risk/history of QT prolongation including HF, bradycardia, diuretic use, cardiac hypertrophy, hypokalemia, hypomagnesemia; Pediatric: Safe and effective use in children has not been established.

Adverse Reactions/Side Effects

Central nervous system

  • headache

Cardiovascular

  • Torsades de Pointes (life-threatening)

Gastrointestinal

  • abdominal pain (most frequent)
  • nausea (most frequent)
  • change in appetite
  • vomiting
  • ↑ liver enzymes

Genitourinary

  • chromaturia (most frequent)

Hematologic

  • agranulocytosis (life-threatening)
  • neutropenia

Musculoskeletal

  • arthralgia
  • arthropathy
  • back pain
  • extremity pain

Miscellaneous

  • ↓ zinc levels

Interactions

Drug-Drug interaction

Concurrent use of other drugs that cause neutropenia/agranulocytosis may ↑ risk of neutropenia/agranulocytosis. May also chelate other concurrently administered polyvalent cations in mineral supplements and antacids, including iron, aluminum and zinc ; wait 4 hr between administration.

Route/Dosage

Oral (Adults) 25 mg/kg three times daily, may be adjusted up to 33 mg/kg three times daily (range 75–99 mg/kg/day in divided doses). Dose should be rounded to the nearest 250 mg (1/2 tablet).

Availability

Tablets: 500 mg

Nursing implications

Nursing assessment

  • .
  • Lab Test Considerations: Monitor serum ferritin every 2–3 mo to assess efficacy. If serum ferritin falls consistently below 500 mcg/L, consider temporarily interripting deferiprone therapy.
    • Measure ANC before starting and weekly during therapy. Interrupt deferiprone if neutropenia (ANC <1.5 X 109/L) or if infection develops. If ANC <1.5 X 109/L and >0.5 X 109/L, obtain CBC with WBC corrected for presence of nucleated red blood cells, ANC, and platelet count daily until recovery (ANC ≥1.5 X 109/L. For agranulocytosis (ANC <0.5 X 109/L), Consider hospitalization and manage as clinically appropriate. Do not resume deferiprone in patients who develop agranulocytosis or rechallenge patients who develop neutropenia, unless benefits outweigh risks.
    • Monitor serum AST and ALT monthly during therapy. Interrupt therapy if persistent ↑ in serum transaminases occurs.
    • Monitor plasma zinc levels. ↓ may occur and may require supplementation.

Potential Nursing Diagnoses

Risk for infection (Adverse Reactions)

Implementation

  • Oral: Administer first dose in the morning, second dose midday, and third dose in the evening. May be taken with meals to decrease nausea.

Patient/Family Teaching

  • Instruct patient to take deferiprone 3 times/day. Take missed doses as soon as remembered, but not just before next dose. Do not double doses.
  • Advise patient to stop therapy and notify health care professional immediately if signs and symptoms of infection (fever, sore throat) or if palpitations, dizziness, syncope, or seizures occur.
  • Inform patient that reddish/brown urine may occur; common and not harmful.
  • Advise female patients to use contraception and avoid breastfeeding during therapy. If pregnancy is planned or suspected, notify health care professional promptly.

Evaluation/Desired Outcomes

  • Decrease in serum ferritin levels.
Mentioned in ?
References in periodicals archive ?
Improvement of erythropoiesis during treatment with deferiprone in a patient with myelofibrosis and transfusional hemosiderosis.
Protective effects of deferasirox and N-acetyl-L-cysteine on iron overload-injured bone marrow
sup][136] The chelator deferiprone has an advantage over desferrioxamine, a 12-month study in patients with early-stage PD revealed a meaningful reduction in iron levels and improvement in motor symptoms,[sup][137] and several Phase II clinical trials evaluating deferiprone are ongoing [Table 1].
Current Nondopaminergic Therapeutic Options for Motor Symptoms of Parkinson's Disease
Pineda, "Combined Therapy with Idebenone and Deferiprone in Patients with Friedreich's Ataxia," Cerebellum, vol.
Rol de la funcion mitocondrial en el corazon y sus implicaciones en disfunciones cardiacas
Cells were then washed twice and treated with deferiprone or deferoxamine (Ferriprox; Cat No.
Estrogen-Dependent downregulation of hepcidin synthesis induces intracellular iron efflux in cancer cells in vitro
Our previous preclinical, translational and pilot clinical studies demonstrated that novel iron chelation therapy with the prototypic drug deferiprone (DFP) (i) induces neuroprotection in cell models of PD via a powerful antioxidant effect, (ii) reduces regional siderosis of the brain, (iii) reduces motor handicap via inhibition of catechol-o-methyl transferase, and (iv) slows the progression of motor handicap in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine mouse model and in early PD patients.
FAIR-PARK-II: Conservative iron chelation as a disease-modifying strategy in Parkinsons disease: a multicentric, parallel-group, placebo-controlled, randomized clinical trial of deferiprone
Safety of oral iron chelator deferiprone in young thalassemias.
A study of clinical and haematopathological correlation of haemolytic anaemias in children
In a small pilot study, he and colleagues found that an iron-slurping chelator called deferiprone may have promise.
Role of iron in Parkinson's debated: data conflict on whether too much or too little metal raises risk
All patients had been diagnosed with either [beta]-thalassemia major (BTM) or [beta]-thalassemia intermedia (BTI), and all had received at least 1 year of treatment within the previous year with an iron chelator--either deferasirox, desferrioxamine (deferoxamine in the United States), deferiprone, or a combination of desferrioxamine and deferiprone.
Sensorineural hearing loss in [beta]-thalassemia patients treated with iron chelation
Deferiprone plus deferoxamine versus deferoxamine iron chalation in beta thalassemia major.
Comparison of blood transfusion plus chelation therapy and bone marrow transplantation in patients with [beta]-thalassemia: Application of SF-36, EQ-5D, and visual analogue scale measures
Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: new data, new questions.
Awareness among parents of children with thalassemia major from Western India
Patients who were receiving frequent blood transfusions to maintain a pre-transfusion haemoglobin level of 9 g/dL and were on chelation therapy with Desferrioxamine or Deferiprone were selected for this study.
ASSESSMENT OF SERUM CALCIUM AND PHOSPHORUS LEVELS AMONG TRANSFUSION-DEPENDENT BETA THALASSEMIA MAJOR PATIENTS ON CHELATION THERAPY
She also undergoes iron chelation therapy to remove the excess iron from her body by taking various medicines, including deferoxamine and deferiprone which cost P26,000 a month, Ana Liza said.
7-year-old girl with blood disorder needs regular transfusions

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