Procysbi
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.
What is Procysbi (cysteamine)?
What is Procysbi (cysteamine) used for?
- Buildup of cystine in the kidneys (nephropathic cystinosis) in adults and children (ages 1 year and older)
How Procysbi (cysteamine) works
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.
Drug facts
Side effects of Procysbi (cysteamine)
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.
Common Side Effects
- Vomiting (77%)
- Gastroenteritis (53%)
- Diarrhea (35%)
- Bad breath (24%)
- Nausea (18%)
- Stomach pain (14%)
- Electrolyte levels out of balance (12%)
- Headache (12%)
Note: People who've already taken Cystagon (cysteamine) are less likely to experience side effects.
Less Common Side Effects
- Dehydration
- Eye infection
- Ear infection
- Respiratory infection
- Tiredness
- Cough
- Pain in the arms and legs
Procysbi (cysteamine) serious side effects
Contact your healthcare provider immediately if you experience any of the following.
- Severe skin reactions: fever, hives, rash, skin reddening, blisters, peeling skin, swelling of the lips or tongue, difficulty breathing
- Stomach bleeding: vomiting blood, bloody or dark stools, severe stomach pain or discomfort
- Brain and mental health side effects: depression, seizures, severe dizziness and sleepiness, confusion
- High blood pressure in the brain: headache, ringing in the ears, dizziness, nausea, double or blurry vision, vision loss, pain behind eye(s) or with eye movement
The following Procysbi (cysteamine) side effects have also been reported
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.
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Pros and cons of Procysbi (cysteamine)
Pros
- Works well to lower cystine levels to protect the kidney
- Taken twice per day (compared to 4 times per day with Cystagon)
- Capsules can be opened and sprinkled onto food for people who have trouble swallowing pills
Cons
- Requires routine lab tests (to check white blood cell cystine level)
- Recommended to not eat for at least 2 hours before and 30 minutes after a dose
- Avoid during pregnancy
Pharmacist tips for Procysbi (cysteamine)
- Mark your appointments in your calendar to see your provider to be examined and get any blood tests done, such as WBC cystine levels. This will help make sure you're being properly monitored while on Procysbi (cysteamine).
- Your provider will order blood tests that measure your cystine levels about every 3 months during treatment to see how well Procysbi (cysteamine) is working for you. They'll also monitor your liver function tests and blood cell counts to make sure they're within normal range.
- Take Procysbi (cysteamine) at least 30 minutes before or 2 hours after meals. Food can lower the amount of Procysbi (cysteamine) your body absorbs.
- If you're unable to take Procysbi (cysteamine) on an empty stomach, you can have up to 4 ounces (half a cup) of food 1 hour before and 1 hour after taking the dose. Avoid high-fat foods, such as cheeses, peanut butter, or avocado. Whether you take Procysbi (cysteamine) with or without food, it's important to be consistent.
- Swallow the Procysbi (cysteamine) capsules or granules whole with water or any fruit juice except grapefruit juice. Don't crush or chew the capsules or granules.
- Avoid taking Procysbi (cysteamine) within 1 hour before or 1 hour after taking medications containing bicarbonate or carbonate, such as Alka Seltzer (aspirin / citric acid / sodium bicarbonate) or calcium carbonate (Tums). These medications can make Procysbi (cysteamine) not work as well.
- Procysbi (cysteamine) can make you sleepy and dizzy, which can affect your ability to focus. It can also cause weakness and confusion. Don't drive or do anything that requires concentration until you know how this medication makes you feel.
- Avoid drinking alcohol because it can worsen side effects from Procysbi (cysteamine), such as upset stomach, sleepiness, depression, and dizziness. It can also affect how well the medication works in your body.
- Tell your provider if you notice a skin rash while taking Procysbi (cysteamine). They'll make sure the rash isn't a sign of a more severe skin problem. You'll likely need to stop the medication until the rash clears.
- Seek medical help if you experience sudden vomiting, stomach pain, dark-colored stools, or blood in your vomit or stool. These could be signs of stomach bleeding from Procysbi (cysteamine).
- If you miss a dose of Procysbi (cysteamine), take it as soon as you remember. But if it's within 4 hours of your next dose, skip the missed dose and take your next dose as you would regularly. Don't double up the dose to make up for the missed dose.
- Don't take Procysbi (cysteamine) if you're pregnant because this medication can cause serious harm to a growing baby. You're recommended to avoid Procysbi (cysteamine) while breastfeeding because it isn't clear how it might affect a nursing baby.
Common questions about Procysbi (cysteamine)
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 (cysteamine) 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 (cysteamine) doesn't cure cystinosis. But it can protect your kidneys from any further damage by lowering the cystine levels in your body. Stopping Procysbi (cysteamine) can cause cystine to build up in your organs and cause problems. Because of this, your provider will likely have you continue taking Procysbi (cysteamine) 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 (cysteamine) 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 (cysteamine) 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 (cysteamine) based on your body weight.
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Risks and warnings for Procysbi (cysteamine)
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.
Severe skin reactions
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.
Stomach bleeding and ulcers
- Risk factors: Older age | History of stomach bleeding or ulcers | Bleeding problems | Liver problems | Taking other medications that raise your risk of bleeds | Drinking alcohol | Smoking
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).
Intestine problems
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.
Brain and mental health side effects
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).
High blood pressure in the brain (intracranial hypertension)
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).
Procysbi (cysteamine) dosage
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 |
| 60 packets of oral granules of 300mg | 1 carton | $6,122.22 | $6,122.22 |
| 120 packets of oral granules of 300mg | 1 carton | $48,933.99 | $48,933.99 |
Typical dosage for Procysbi (cysteamine)
People who weigh 112 lbs or more:
- The typical starting dose ranges from 175 mg to 250 mg by mouth every 12 hours. Your provider will raise your dose over the course of 4 to 6 weeks. The typical maintenance dose is 1000 mg every 12 hours. People who weigh less than 112 lbs: Your child's provider will calculate their dose based on body surface area (BSA), which takes into account your child's height and weight.
- The typical starting dose ranges from 25 mg to 225 mg by mouth every 12 hours. The provider will raise the dose over 4 to 6 weeks (more slowly for children ages 6 years and under). The typical maintenance dose ranges from 200 mg to 900 mg every 12 hours.
Your provider will adjust your dose based on how well the medication is lowering your cystine levels and if you're having side effects.
Interactions between Procysbi (cysteamine) and other drugs
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.
How much does Procysbi (cysteamine) cost?
$6,122.22
Procysbi (cysteamine) contraindications
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.
- Allergic reaction to penicillamine (Depen) or Cystagon (cysteamine bitartrate)
What are alternatives to Procysbi (cysteamine)?
