Shwachman-Diamond Syndrome Medication

Updated: Aug 09, 2019
  • Author: Antoinette C Spoto-Cannons, MD, FAAP; Chief Editor: Hassan M Yaish, MD  more...
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Medication

Pancreatic enzymes

Class Summary

These agents are used to replace endogenous pancreatic enzymes.

Pancrelipase (Creon, Ku-Zyme, Pancrease, Viokase, Ultrase)

Assists in digestion of protein, carbohydrates, and fats. Dosage recommendations are only approximations for initial dosages. Actual dose depends on digestive requirement of individual patient.

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Colony-stimulating factors

Class Summary

These agents provide red cell line stimulation.

Epoetin alfa (Epogen, Procrit)

FDA approved for treatment of chronic anemia. Stimulates division and differentiation of committed erythroid progenitor cells; induces release of reticulocytes from bone marrow into bloodstream. SC route provides sustained serum levels compared to IV route. Reduce dose when hematocrit reaches target range of 30-36% or hematocrit increases >4 points over any 2-wk period. Hold dose if hematocrit >36%.

Filgrastim (Neupogen)

FDA approved for severe chronic neutropenia.

GCSF activates and stimulates production, maturation, migration, and cytotoxicity of neutrophils. If IV route is used and GCSF final concentration is < 15 mcg/mL, add 2 mg of albumin/mL to prevent drug adsorption to administration set.

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Vitamins

Class Summary

Vitamins are essential for normal DNA synthesis and cell function.

ADEK vitamins (ADEKS)

PO multinutrient specially formulated for use under medical supervision to provide nutritional supplementation in individuals with malabsorptive conditions. Each dose contains water-miscible forms of fat-soluble vitamins A, D, E, and K plus other nutrients, including vitamin C, B-complex vitamins, biotin, folic acid, and zinc. Available as chewable tab or pediatric drops.

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