The Robert Gregory Alkaptonuria Centre (NAC)

Activities at the NAC

The first visit to the NAC will be over four nights and four days followed by yearly visits of three nights and three days. Patients will need to arrive around lunchtime on the first day, and there will be a busy schedule over three and a half days. The NAC will provide nitisinone treatment with a low protein diet and physiotherapy. Any potential complications of AKU will be identified and reported to the patient’s local healthcare team so treatment between visits can be supplied by the local GP or hospital.

Nitisinone therapy:

Nitisinone is a drug not licensed for use in AKU. There is good evidence that it may work as a treatment for AKU. Through the NAC, nitisinone will be made available to patients, following the complete medical examination during your visit. When taking nitisinone, it is recommended that patients follow a low protein diet, and a dietician will meet with them to provide advice. When patients leave the NAC, they will be prescribed with a yearly supply of nitisinone.

Treatments will be monitored by regular assessments during visits and by phone calls in between annual visits. If patients have any concerns, they should contact the NAC team.

Dietary Advice:

Low protein diets by themselves are not effective to prevent progression of AKU. However, low protein diets will make nitisinone treatment safer, by reducing the availability of tyrosine, the precursor of homogentisic acid. After nitisinone use, tyrosine levels generally increase and a low protein diet will minimise the rise in tyrosine. A separate nitisinone information booklet will be provided at the NAC to explain all about the drug in more detail.

Discharge from NAC:

On completion of the programme of assessments and treatments at each visit, patients will be discharged to their local area Health Trust. If any health problems develop in between annual visits, and they cannot be dealt with locally, they will be able to attend Liverpool for additional assessment and treatment that falls within the scope of the nationally commissioned service.

Reports:

On completion of your NAC clinic, patients are sent a discharge summary letter. This is a detailed comprehensive report on the totality of all assessments and treatments and guidance for further local monitoring and treatment and will be sent 10 working days after being discharged. This report will be sent to the patient, their GP and any other named consultants or therapists actively involved in their care.