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•  To provide 30-35% of total calories (173-175) divided intoat least four doses and administered orally diluted with foods, liquids, or formula via a silicone or polyurethane feedingtube.
• For patients receiving anothermedium-chain triglyceride product, discontinue prior to the first dose of DOJOLVI.
• Calculate the volume per dose; initiate and titrate the dosage to achieve the target; and prepare and administerDOJOLVI. 
• For patients not currently taking a MCT product:
  •  Initiate DOJOLVI at a total daily dosage of approximately 10% DCI divided into at least four times per day and increase to the recommended total daily dosage of up to 35% DCI over a period of 2 to 3 weeks.
• For patients switching from another MCT product
  • Discontinue use of MCT products before starting DOJOLVI. Initiate DOJOLVI at the last tolerated daily dosage of MCT divided into at least four times per day. Increase the total daily dosage by approximately 5% DCI every 2 to 3 days until the target dosage of up to 35% DCI is achieved.
• Tolerability:
  • If a patient has difficulty tolerating 1/4 of the total daily dosage at one time, more frequent smaller doses may be considered. Monitor patients’ total caloric intake during dosage titration, especially in patients with gastrointestinal adverse reactions, and adjust all components of the diet as needed. If a patient experiences gastrointestinal adverse reaction(s), consider dosage reduction until the gastrointestinal symptoms resolve. If a patient is unable to achieve the target daily dosage of up to 35% DCI during dosage titration, maintain the patient at the maximum tolerated dosage. 

Oral Liquid, 100% w/w of triheptanoin.

PO

Abdominal pain, diarrhea, vomiting, and nausea.

Regularly monitor the gastrostomy tube to ensure proper functioning and integrity.

Low or absent pancreatic enzymes may reduce absorption of DOJOLVI. Avoid administration of DOJOLVI in patients with pancreatic insufficiency.

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173.        Mochel F, DeLonlay P, Touati G, et al. Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapy. Mol Genet Metab. 2005;84:305-12 doi: 10.1016/j.ymgme.2004.09.007 [published Online First: 2005/03/23].

174.        Roe CR, Mochel F. Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential. J Inherit Metab Dis. 2006;29:332-40 doi: 10.1007/s10545-006-0290-3 [published Online First: 2006/06/10].

175.        Roe CR, Sweetman L, Roe DS, David F, Brunengraber H. Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride. J Clin Invest. 2002;110:259-69 doi: 10.1172/JCI15311 [published Online First: 2002/07/18].