Procysbi (cysteamine) is a cystine depleting agent that lowers the amount of cystine in your body. It treats a rare genetic condition called nephropathic cystinosis in people ages 1 year and older. This medication is the extended-release version of cysteamine bitartrate and is taken by mouth every 12 hours, preferably on an empty stomach. Common side effects of Procysbi (cysteamine) include vomiting, nausea, and stomach pain.
Cystinosis is a genetic condition where there's a build-up of cystine (a protein) in the body. This happens because lysosomes, which are parts of the cell that are responsible for breaking down cystine, aren't working properly.
Procysbi (cysteamine) is a cystine depleting agent. It works by attaching to lysosomes to help them work better at removing cystine from your body.
| Common Brands | Procysbi |
|---|---|
| Drug Class | Cystine-depleting agent |
| Controlled Substance Classification | Not a controlled medication |
| Generic Status | No lower-cost generic available |
| Availability | Prescription only |
The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.
Note: People who've already taken Cystagon (cysteamine) are less likely to experience side effects.
Contact your healthcare provider immediately if you experience any of the following.
Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.
Check with your doctor immediately if any of the following side effects occur:
Rare
Increased thirst
seizures
unusual tiredness or weakness
Incidence not known
Blistering, peeling, or loosening of the skin
blurred or double vision
bone lesions
bulging soft spot on the head of an infant
change in the ability to see colors, especially blue or yellow
chills
continuing ringing or buzzing or other unexplained noise in the ears
hearing loss
joint or muscle pain
loss of appetite
loss of vision
pain behind the eyes
pain in the legs
pain with eye movement
red skin lesions, often with a purple center
red, irritated eyes
reddish purple lines on the arms, face, legs, trunk, or groin
severe headache
sores, ulcers, or white spots in the mouth or on the lips
Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:
Less common
Breath odor
hives or welts
redness of the skin
unusual drowsiness, dullness, tiredness, weakness, or feeling of sluggishness
Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.
Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.
Procysbi (cysteamine) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
People have reported skin rashes after starting immediate-release cysteamine bitartrate. Sometimes, these rashes are temporary and go away after the medication is stopped. But rarely, this medication can cause life-threatening skin reactions, such as toxic epidermal necrolysis (TEN). Let your provider know right away if you notice changes in your skin such as rash, blisters, skin reddening, or skin peeling. Depending on how serious your reaction is, your provider might ask you to temporarily stop Procysbi (cysteamine) and restart you at a lower dose, or stop this medication completely.
Some people have also reported purplish skin lesions after taking higher doses of immediate-release cysteamine bitartrate. Sometimes, they've also experienced other skin changes, bone pain, and higher risk of fracture along with these lesions. Your provider will examine your skin and bones regularly when you're taking Procysbi (cysteamine). If needed, they'll lower your dose of the medication.
Procysbi (cysteamine) might raise your risk for serious stomach problems, including bleeding and ulcers (sores). Stomach bleeding is a dangerous condition that requires treatment right away. Your risk for stomach bleeds is even higher if you're taking Procysbi (cysteamine) with other medications that can put you at risk, such as blood thinners (anticoagulants). Seek medical help right away if you're feeling faint, vomiting blood, have bloody or dark stools, or have severe stomach pain or discomfort that doesn't go away while you're taking Procysbi (cysteamine).
Although rare, there have been reports of Procysbi (cysteamine) causing a condition called fibrosing colonopathy. This is where parts of your large intestine has scarring or swelling. If you have fibrosing colonopathy, your provider will ask you to stop taking Procysbi (cysteamine) and consider immediate-release cysteamine bitartrate capsules instead. Contact your provider right away if you notice symptoms like stomach pain, vomiting, bloody diarrhea, or diarrhea that worsens or don't go away.
Procysbi (cysteamine) might affect your brain, which can cause symptoms, such as sleepiness, depression, fatigue, seizures, and brain swelling. Don't drive or do anything that requires you to focus until you know how this medication affects you. Let your provider know if you experience any of these symptoms because they might prescribe a lower dose of Procysbi (cysteamine).
Although very rare, there have been a few reports of immediate-release cysteamine bitartrate causing high blood pressure in the blood vessels in the brain, or intracranial hypertension. This can lead to changes in vision and cause symptoms like headache, nausea, vomiting, dizziness, and ringing in the ears. Your provider will likely perform eye examinations before and regularly throughout treatment with Procysbi (cysteamine), including looking at your eyes for papilledema (or eye swelling).
Carton| Dosage | Quantity | Price as low as | Price per unit |
|---|---|---|---|
| 60 packets of oral granules of 75mg | 2 cartons | $14,260.43 | $7,130.22 |
| 120 packets of oral granules of 75mg | 1 carton | $12,238.19 | $12,238.19 |
| 120 packets of oral granules of 300mg | 1 carton | $48,933.99 | $48,933.99 |
Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.
Contraindications are specific health conditions or situations in which a person should not take a medication due to safety concerns. If you have any of the following conditions or if any of the following apply to you, let your healthcare provider know because Procysbi (cysteamine) will not be safe for you to take.
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Cystinosis is a rare genetic condition where your body can't remove cystine (a protein) properly from cells. This causes cystine to build up and form crystals in many organs. When cystine builds up in the kidneys (nephropathic cystinosis), it can cause kidney failure. Without treatment, this condition is life-threatening. Procysbi treats nephropathic cystinosis by clearing the build up of cystine.
Both Cystagon and Procysbi are brand names of the same active ingredient, cysteamine bitartrate. Cystagon is the immediate-release version and is taken by mouth 4 times daily (with or without food). Procysbi is the delayed-release version that's taken by mouth 2 times daily (preferably on an empty stomach). A small study showed both medications work well to lower cystine levels, but Procysbi might have a lower risk of side effects.
No, Procysbi doesn't cure cystinosis. But it can protect your kidneys from any further damage by lowering the cystine levels in your body. Stopping Procysbi can cause cystine to build up in your organs and cause problems. Because of this, your provider will likely have you continue taking Procysbi as long as it's helping keep your cystine levels low and you're not having any serious side effects.
If you're having trouble swallowing the Procysbi capsules or granules, you can sprinkle their contents on a small amount (less than 4 ounces) of applesauce or berry jelly. Or, you can mix the medication into 4 ounces of any fruit juice, except for grapefruit juice. Take the medication within 30 minutes after you've mixed it into food or juice.
No, weight gain wasn't reported by people taking Procysbi during clinical trials. But some people did experience loss of appetite and weight loss. Talk to your provider if you notice changes in your body weight after starting treatment. Your provider might need to adjust your dose of Procysbi based on your body weight.
