What is Dojolvi

Dojolvi is a medium-chain triglyceride indicated as the source of fatty acids and calories to treat children and adults with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD). 

LC-FAOD are a group of rare, genetic, life-threatening disorders caused by defects in enzymes that help your body to use stored fat for energy. The primary source of energy for your body is sugar or carbs. Once sugar is gone, your body uses stored fat as energy.

When the body cannot use the stored fats, its levels increases in the organs such as the heart and the liver.

Dojolvi is the only medicine for LC-FAOD that acts as an energy source and metabolite replacement.

The drug has been approved in Feb 2021 for treating LC-FAOD in Canada.

Let’s understand more about the medicine, including its uses, side effects, and precautions to be followed.

Uses

Label Uses

Dojolvi is a prescription medicine, which is a medium-chain triglyceride indicated to provide calories and fatty acids to treat long-chain fatty acid oxidation disorders (LC-FAOD). 

Treatment with Dojolvi in LC-FAOD patients decreases complications from 60% to about 10%.

Off-label Use

There is no off-label use of the medicine so far.

Dojolvi may be prescribed for other uses; ask your doctor or pharmacist for more information.

Direction For Use

• Dojolvi is available as an oral liquid formulation in a glass bottle. 

• For calculating the dose of Dojolvi, understand your metabolic requirements by finding your daily calorie intake (DCI). The recommended daily target dose of Dojolvi is up to 35% of the patient’s total prescribed DCI. 

• The doses should be divided into at least four doses and administered at mealtimes or with snacks. The dosage will be higher in infants as the neonatal population may have a higher fat intake. 

The total daily dose of Dojolvi is to be administered in ml using the following calculation:

• Divide the total daily dosage onto at least four approximately equal individual doses
• Calorie value of Dojolvi + 8.3kcal/ml
• Round the total daily dosage to the nearest whole number.

Your physician may start Dojolvi with approximately 10% of the total daily dosage and increase up to 35% of the total daily dosage for 2 to 3 weeks. 

Other directions

• Dojolvi is to be administered with semi-solid or liquids orally via a silicone or polyurethane feeding tube. 

• Do not administer Dojolvi alone as it may cause gastrointestinal upset. Measure the medication always with an oral syringe or measuring cup. 

• Mix Dojolvi and administer it with food items instructed by the physician or pharmacist only.  

• Mix the medication with food thoroughly. The mixture may be stored for up to 24 hours in refrigerated conditions. 

• In case you miss your dose, take the medication as soon as you remember, but skip the missed dose if it is almost the time for your next dose. Following doses should be taken every 3 to 4 hours. 
• If you cannot take all four doses in a day, skip the missed dose. Do not take two doses at once time. 

Ingredients And Benefits

Dojolvi contains triheptanoin as its main ingredient. It is a triglyceride fatty acid. It is used clinically to treat inherited metabolic disorders diseases, such as pyruvate carboxylase deficiency and carnitine palmitoyltrasnferase II deficiency. It also appears to increase the efficacy of the ketogenic diet as a treatment for epilepsy. 

As mentioned above, it is indicated as a source of calories and fatty acids for LC-FOAD treatment in children and adults.  

It works by:

• Providing an alternate source of energy
• Bypassing the enzyme deficiency causing LC-FAOD and provide a source of fatty acids and calories that can be converted to energy.

Warnings and Precautions

• Inform your health worker about other prescription and non-prescription medications that you are taking. Also, inform about vitamins, herbal and nutritional supplements that you are taking or planning to take. 
• For mixing Dojolvi, always use stainless steel, glass, high-density polyethene (HDPE), polypropylene, low-density polypropylene, polyurethane, and silicone type plastic materials. But do not use a material containing polystyrene or polyvinyl chloride. 
• You must follow a proper diet when taking Dojolvi. 
• The feeding tube should be checked regularly as its performance and functionality may degrade over time depending on the usage and environmental conditions. 

Storage and Disposal

• It should be stored at 20° to 25°C
• Opened bottles of Dojolvi can be used up to 90 days after opening, but not beyond the expiry date mentioned on the bottle, whichever is earlier. 
• The medication should be stored in the bottle in which it was dispensed at room temperature
• Always keep the bottle away from moisture and heat
• Do not freeze the medication

Contraindications

Inform your healthcare provider in case of an allergy to Dojolvi or any other medication. Ask your pharmacist or check the medication guide for a list of the ingredients. 

Do not take or inform your doctor if you have or had following conditions:

• Pancreatic insufficiency 

Besides, inform your doctor if you are pregnant or breastfeeding, or planning to be pregnant. 

Interactions

Inform your physician about all medications you are currently taking as they may have some interactions or side effects if consumed together. 

Inform your doctor if you are taking:

• Orlistat

If you are taking another medium-chain triglyceride product, you should inform your physician about the same. Your doctor will discontinue it first before the first dose of Dojolvi. 

Side Effects 

Like all the medications, Dojolvi also has side effects. It is possible that you may experience the following side effects:

Common side effects

• Stomach pain 
• Diarrhea 
• Nausea 
• Vomiting

Get emergency help if you have allergic reactions. The following are the signs of an allergic reaction: hives; swelling on the face, tongue, lips, or throat; difficulty in breathing. 

This is not a complete list of side effects. If you experience severe side effects or symptoms that are persistent, please contact your doctor immediately.

FAQs

1. What Are the Side Effects of Dojolvi?

The most common side effects observed in studies are abdominal pain and distension, vomiting, diarrhea, and nausea. The chances of adverse side effects are rare.

2. How is Dojolvi Taken?

Dojolvi can be taken with semi-solid or liquids orally via a silicone or polyurethane feeding tube. 

3. Is There A Generic Version?

No. For now, there is no generic version of Dojolvi available in Canada.

4. How Is This Medication Different from Others In The Treatment Of Ailment?

Dojolvi is the one and only medicine approved in Canada for long-chain fatty acid oxidation disorders (LC-FAOD) in adults and children.

5. Who Makes This Drug and What Is Their Manufacturing Record of Success?

Ultragenyx Pharmaceutical Inc., a biopharmaceutical company, makes Dojolvi. They specialize in the production of products to manage rare and ultra-rare products.