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Penicillamine challenge

The penicillamine challenge is a 48 hour test that is done to check for Wilson’s disease. Wilson’s disease is a rare, inherited condition in which the body cannot handle copper correctly, and needs medication to remove the excess copper from the body.

Your consultant will discuss the test results with you once they are available.

If you have been asked to do this test at home, it would be best to do it over a weekend as it is very important that all of your child’s wee can be collected.

To do the test you will need

  • 2 large wee collection containers
  • 2 lab forms for urinary penicillamine challenge
  • 2 doses of Penicillamine 500mg

All of these will be provided to you by the Sheffield Children’s Hospital.

Illustration of 2 large jugs and 4 tablets

Day 1

Day 1 uses wee bottle number 1. Do not collect your child’s first wee (for example 8am on Saturday).

When you child needs their second wee of the day, collect you child’s wee in bottle number 1. Write start time and date on the label.

Illustration of large jug with wee in it

You must now collect all of the wee that your child passes for the next 24 hours in this same bottle.

Day 2

Collect your child’s first wee of the day in wee bottle number 1 (for example 8am on Sunday). Write the end time and date on the label.

The rest of day 2 uses bottle number 2.

Illustration of large jug with wee in it

Give your child the first dose of 500mgs of Penicillamine (for example at 9am).

Illustration of giving child tablet

For the next 24 hours, collect your child’s wee in bottle number 2. Write the start time and date on the label.

Give the second dose of 500mgs of Penicillamine 12 hours after the first dose (for example at 9pm).

Illustration of giving child tablet

Continue to collect wee in sample bottle 2.

Day 3

Collect your child’s first wee of the day in wee bottle number 2 (for example 7am on Monday). Write the end time and date on the label.

Make sure both wee bottles are clearly labelled with your child’s details, and the start and finish time of the wee collections for each bottle. Both wee bottles and lab forms should be taken straight to Sheffield Children’s Hospital and handed in at Clinical Chemistry (open 9am till 5pm, Monday till Friday).

Illustration of parent dropping off wee samples to Chemical Chemistry

If you have any questions regarding the Penicillamine challenge, please speak to the clinical team that asked your child to complete this test.

Contact us

If you need any help or advice with the information in this resource, you can contact the gastroenterology nurses by telephoning 0114 271 7111 or 0114 271 7162 or by emailing: scn-tr.gastronurses@nhs.net

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Please note: this is a generic information sheet relating to care at Sheffield Children’s NHS FT. These details may not reflect treatment at other hospitals. This information is not intended as a substitute for professional medical care. Always follow your healthcare professionals’ instructions. If this resource relates to medicines, please read it alongside the medicine manufacturer’s patient information leaflet. If this information has been translated into another language from English, efforts have been made to maintain accuracy, but there may still be some translation errors. If you are unsure about any of the guidance in this resource or have specific questions about how it relates to your child, always ask your healthcare professional for further advice.

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