Ordering Panhematin

Ordering Panhematin

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How to order PANHEMATIN

Order through Canadian Blood Services and Héma‑Québec

How supplied

PANHEMATIN is supplied as a sterile, lyophilized black powder in single dose dispensing vials in a carton.

The vial stopper contains natural rubber latex.

Store lyophilized powder at 20-25°C (68-77°F).

Hemin for injection. PANHEMATIN.

Acute intermittent porphyria defined

Acute intermittent porphyria (AIP) is a rare inherited disease caused by a partial deficiency of the enzyme porphobilinogen (PBG) deaminase in the heme biosynthetic pathway.1

  • PBG deficiency disrupts normal heme production, which leads to overproduction of porphyrin precursors.2
  • Abdominal pain, the most common symptom, is usually severe, unremitting, and diffuse.1

How should the presence of AIP be diagnosed before starting PANHEMATIN therapy?

Before PANHEMATIN therapy is begun, the presence of acute porphyria must be diagnosed using the following criteria3:

  • Presence of clinical symptoms suggestive of acute porphyric attack.
  • Quantitative measurement of porphobilinogen (PBG) in urine. The single‑void urine sample should be refrigerated or frozen without additives and shielded from light for subsequent quantitative δ-aminolevulinic acid (ALA), PBG, and total porphyrin determinations. (Note: the classical Watson- Schwartz or Hoesch tests are considered to be less reliable).

References

  1. Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the Diagnosis and Treatment of the Acute Porphyrias. Ann Intern Med. 2005;142:439-450.
  2. Gonzalez-Arriaza HL, Bostwick JM. Am J Psych. 2003;160(3):450-458.
  3. Recordati Rare Diseases Canada Inc. PANHEMATIN® Product Monograph. July 13, 2018.