Cysteamine

Cysteamine is a prescription medication used to treat cystinosis of the kidneys and eyes. Cystinosis is a rare condition that causes cystine, a protein building block, to build up in the cells of the body. Cysteamine belongs to a class of medications called anti-cystine agents, which help by decreasing the levels of cystine in the body.

This medication comes as immediate- and delayed-release capsules to be taken by mouth or by gastric tube (feeding tube inserted into the stomach) every 6 hours or every 12 hours.

The most common side effects with the capsules include nausea, bad breath, abdominal pain, constipation, indigestion or upset stomach, and headache. Cysteamine capsules may also cause drowsiness or dizziness. Do not drive or operate heavy machinery until you know how this medication will affect you.

The most common side effects with the eye drops include sensitivity to light, redness, eye pain/irritation, headache and visual field defects. Do not drive or operate heavy machinery until you know how this medication will affect you.

Cysteamine is a prescription medication used to treat cystinosis of the kidneys and eyes. Cystinosis is a rare condition that causes cystine, a protein building block, to build up in the cells of the body.

This medication may be prescribed for other uses. Ask your doctor or pharmacist for more information.

Cysteamine may be found in some form under the following brand names:

• Cystagon

• Procysbi

Oral:

• The most common side effects with the capsules include nausea, bad breath, abdominal pain, constipation, indigestion or upset stomach, and headache. Cysteamine capsules may also cause drowsiness or dizziness. Do not drive or operate heavy machinery until you know how this medication will affect you.

Topical:

• The most common side effects with the eye drops include sensitivity to light, redness, eye pain/irritation, headache and visual field defects. Do not drive or operate heavy machinery until you know how this medication will affect you.

This is not a complete list of cysteamine side effects. Ask your doctor or pharmacist for more information.

Serious side effects have been reported with cysteamine. See “Drug Precautions” section.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Tell your doctor about all the medicines you take including prescription and non-prescription medicines, vitamins, and herbal supplements. Especially tell your doctor if you take:

• bicarbonate, an electrolyte found in products such as Arm & Hammer Baking Soda, Soda Mint, sodium bicarbonate injection, and sodium bicarbonate tablets

This is not a complete list of cysteamine drug interactions. Ask your doctor or pharmacist for more information.

Oral:

Serious side effects have been reported with cysteamine including:

• Skin, bone, and joint problems: People treated with high doses of this medication may develop problems such as stretch marks, bone injuries (such as fractures), bone deformities, and joint problems. Check your skin while taking cysteamine. Tell your doctor if you notice any skin changes. Your doctor will check you for these problems.
• Skin rash: Skin rash is common with cysteamine and may sometimes be severe. Tell your doctor right away if you get a skin rash.
• Central nervous system symptoms: Some people who take other medicines that contain cysteamine develop seizures, depression, and become very sleepy. The medicine may affect how your brain is working (encephalopathy). Tell your doctor right away if you develop any of these symptoms.
• Stomach and bowel (intestinal) problems: Some people who take other medicines that contain cysteamine develop ulcers and bleeding in their stomach or bowel. Tell your doctor right away if you get stomach-area pain, nausea, vomiting, loss of appetite, or vomit blood.
• Low white blood cell count and certain abnormal liver function blood tests: Your doctor should check you for these problems.
• Intracranial hypertension (pseudotumor cerebri) has happened in some people who take immediate-release cysteamine. This is a condition where there is high pressure in the fluid around the brain. Your doctor should do eye examinations to find and treat this problem early.
• Tell your doctor right away if you develop any of the following symptoms while taking cysteamine: headache, buzzing or "whooshing" sound in the ear, dizziness, nausea, double vision, blurry vision, loss of vision, pain behind the eye, or pain with eye movement.

Cysteamine can cause dizziness. Do not drive or operate heavy machinery until you know how cysteamine affects you.

Do not take cysteamine if you have an allergic or hypersensitive reaction to penicillamine.

Topical:

• To minimize the risk of contamination, do not touch the dropper tip to any surface. Keep bottle tightly closed when not in use.

Medicines can interact with certain foods. In some cases, this may be harmful and your doctor may advise you to avoid certain foods. In the case of cysteamine, there are no specific foods that you must exclude from your diet when receiving cysteamine.

Oral:

In addition:

• If you take cysteamine without eating, take cysteamine at least 30 minutes before you eat or at least 2 hours after you eat.
• If you are not able to take cysteamine without eating, you can eat a small amount of food (½ cup) between 1 hour before you take cysteamine to 1 hour after you take it.
• Avoid eating foods that are high in fat close to the time that you will take a dose of cysteamine.

Before taking cysteamine, tell your doctor about all of your medical conditions. Especially tell your doctor if you:

• have a skin rash or bone problems
• have a history of seizures, exhaustion, sleepiness, depression, or other nervous system problems
• have or have had stomach or bowel (intestinal) problems including ulcers or bleeding
• have liver or blood problems
• are pregnant or are breastfeeding

Tell your doctor about all the medicines you take including prescription and non-prescription medicines, vitamins, and herbal supplements.

Tell your doctor if you are pregnant or plan to become pregnant.

The FDA categorizes medications based on safety for use during pregnancy. Five categories - A, B, C, D, and X, are used to classify the possible risks to an unborn baby when a medication is taken during pregnancy.

Cysteamine falls into category C. This medication may be given to a pregnant woman if her healthcare provider believes that its benefits to the pregnant woman outweigh any possible risks to her unborn baby. It is not known if cysteamine will harm your unborn baby.

Tell your doctor if you are breastfeeding or plan to breastfeed.

It is not known if cysteamine is excreted in human breast milk or if it will harm your nursing baby. Because many drugs are present in human milk and because of the potential for serious adverse reactions in nursing infants from cysteamine, nursing is not recommended.

Oral:

IMMEDIATE-RELEASE CAPSULES

• Take cysteamine exactly as prescribed. Do not increase or decrease the amount of medicine without your doctor's approval.
• If you or your child misses a dose of medicine, it should be taken as soon as possible. If it is within 2 hours of the next dose, skip the missed dose and go back to the regular dosing schedule. Do not double dose.
• If you or your child takes too much cysteamine, call your doctor or Poison Control Center, or go to the nearest hospital emergency room right away.
• Do not give cysteamine capsules to children under six years of age. They may not be able to swallow them and may choke. For children under six years of age, the capsule may be opened and the contents sprinkled on food. Talk to your doctor for complete directions.
• Your doctor will arrange for regular blood tests to be done to measure the amount of cystine inside white blood cells. This will help to determine the correct dose of cysteamine. Regular blood and urine tests will also be done to measure the levels of the body's important electrolytes. This will help your doctor to correctly adjust the doses of these supplements.
• Your doctor may also want to do certain tests to find out if unwanted effects are occurring. The tests are very important because serious side effects, including ulcers or bleeding in the stomach and intestines (digestive tract), can occur.

DELAYED RELEASE CAPSULES

• Take cysteamine exactly as prescribed.
• Cysteamine is usually taken 2 to 4 times a day (every 6 or every 12 hours).
• Take cysteamine the same way each time, either without eating or with a small amount of food, as follows:
  • If you take cysteamine without eating, take cysteamine at least 30 minutes before you eat or at least 2 hours after you eat.
  • If you are not able to take cysteamine without eating, you can eat a small amount of food (½ cup) between 1 hour before you take cysteamine to 1 hour after you take it.
  • Avoid eating foods that are high in fat close to the time that you will take a dose of cysteamine.
• Swallow cysteamine capsules whole. Do not crush or chew the capsule or capsule contents.
• If you cannot swallow the whole capsule, you can open it and take the capsule contents with certain foods and juices. Cysteamine can also be given through a gastrostomy tube, 12 French or larger.

Taking cysteamine with certain foods: You can only use applesauce or berry jelly.

• Open the capsule.
• Sprinkle the granules on 4 ounces (½ cup) of applesauce or berry jelly. Swallow mixture within 30 minutes.

Taking cysteamine with certain juices: You can only use apple juice or orange juice.

• Open the capsule.
• Sprinkle the granules into 4 ounces (about ½ cup) of apple juice or orange juice.
• Shake gently for 5 minutes.
• Drink within 30 minutes.

Taking cysteamine through a gastrostomy tube (feeding tube inserted into the stomach), 12 French or larger: You can only use applesauce.

• Open the capsule.
• Sprinkle the granules on 4 ounces (½ cup) of applesauce. Mix the granules with the applesauce.
• Give the medicine through the gastrostomy tube into the stomach within 30 minutes.
• After giving the mixture, flush the gastrostomy tube with 8 ounces (about 1 cup) of apple juice or orange juice to clear the tube. Do not use other liquids to flush the tube.
• Take supplements if prescribed by your doctor, such as salt or mineral replacement supplements, vitamin D, or thyroid hormone

If you miss a dose, take it as soon as possible. If it is within 4 hours of the time the next dose is due, skip the missed dose. Take the next dose at your regularly scheduled time. Do not take 2 doses at one time to make up for a missed dose.

Topical:

• Instill one drop of cysteamine eye drop solution in each eye, every waking hour.
• Contact lenses should be removed prior to application of cysteamine eye drops. Contact lenses may be reinserted 15 minutes following use.
• Do not touch dropper tip to any surface, as this may contaminate the solution.
• Discard after 1 week of use.

If you take too much cysteamine call your healthcare provider or local Poison Control Center, or seek emergency medical attention right away.

If cysteamine is administered by a healthcare provider in a medical setting, it is unlikely that an overdose will occur. However, if overdose is suspected, seek emergency medical attention.

Oral:

IMMEDIATE-RELEASE TABLETS

• Store cysteamine in a dry place away from light.
• Store cysteamine between 68° to 77°F (20° to 25°C).

DELAYED-RELEASE TABLETS

• Store cystemaine at room temperature between 68° to 77°F (20° to 25°C).
• Store cystemaine in a dry place away from light.
• Keep cystemaine tightly closed in the original bottle.
• The cystemaine bottle contains a desiccant packet to help keep your medicine dry. Do not eat or throw away the desiccant packet.

Topical:

• Store bottles in the freezer in the original carton.
• Each week, one new bottle should be removed from the freezer.
• Allow the bottle to thaw completely (approximately 24 hours) prior to use.
• After the bottle is completely thawed, record the discard date on the bottle label. The discard date is seven (7) days from the day the bottle is thawed.
• Store thawed bottle at 2°C to 25°C (36°F to 77°F) for up to 1 week. The thawed bottles should not be refrozen.
• At the end of 1 week (7 days), discard the bottle. There may be medication left in the bottle; however, the bottle must be discarded because the medication is only stable for 1 week after thawing.