What tests will be carried out during my time at the National AKU Centre (NAC)?
During your time at the NAC, you will have a full health check and assessment from experienced doctors and clinicians, many of whom are experts in AKU. The tests will include blood and urine tests, in order to monitor your HGA levels. You will have cardiology, physiotherapy, rheumatology and eye consultations. X-Rays and MRI’s and bone scans are also included. You will also have an ultrasound of the abdomen, pain management, gait analysis and a psychometric assessment. All of these tests are designed to evaluate the effect AKU has had on your body.
A benefit of these thorough tests is that any medical or health issues will quickly be picked up and identified, meaning you can leave the NAC with peace of mind. You will be prescribed nitisinone on your first visit to the NAC and a dietitian will work with you to ensure you have a healthy balanced diet that may also mean reviewing the amount of protein you eat.
Will these tests hurt?
The tests are unlikely to hurt, but may be uncomfortable for some. Some patients who have difficulty lying flat have been given thin pillows to rest their backs on, due to the effect that AKU has had on their spines. During the tests, everyone at the NAC will work hard to ensure your experience is as comfortable and worry-free as possible.
Can my family come to Liverpool with me?
Yes, they can. While attending the NAC, you will be accommodated overnight in a local hotel, at no cost to you. Transport will be provided to take you to and from the hospital each day. A family member can stay with you in your room at no extra cost, all they need to pay for is their own food and drink. Additional rooms can be booked for more family members to stay with you however, this extra cost is unlikely to be funded by the NAC.
How do I spend my spare time?
During your time at the NAC, there may be gaps in the day in which you have no consultations. You will also be free in the evenings, usually from about 6pm. You are welcome to spend this free time how you wish. Family or friends can visit the ward. You can also leave the hospital, as long as you are back for any scheduled appointments, staff know where you are and have a mobile number to contact you. There is shopping, good food, the theatre and more nearby, just a short walk down the hill from the hospital and from your hotel. Taxis in Liverpool are reasonably priced.
Will food be provided throughout my stay?
An evening meal is provided in the hotel on the day you arrive and subsequent evenings. This can be ordered from the hotel menu up to a set amount. The NAC co-ordinator will liaise with you and the hotel advising on this predetermined amount and, on any special dietary requirements. Alcohol is not covered under this agreement. Breakfast will be provided in the hotel except for the morning when you having fasting blood tests. On this day breakfast will be provided in the hospital after the tests. Lunch will be provided in the hospital.
Do I need to take food with me?
As some of the tests can take a long time, we advise that you take snacks with you. Some mornings you will need to fast, so you may want to bring snacks with you to eat straight after the fasting tests are completed. Other tests can take a long time and you may feel peckish if this results in a late lunch or evening meal.
What should I bring with me?
Towels and toiletries are provided in the hotel. Each room has a TV, minibar (some items you will need to pay for), climate control and wifi.
Some people attending the NAC may need to be accommodated as an inpatient in the hospital rather than in the hotel. For example anyone who has difficulty with mobility or usually has carers at home. In this case, you will need to bring your own towels and toiletries with you. You may not have a single room nor access to a TV. Wifi is available in the hospital however, the connection can be unpredictable.
We recommend you wear loose comfortable clothing. During the physiotherapy assessment and gait analysis, you are required to wear shorts or tracksuit type leggings. We also suggest you bring things to entertain yourself when waiting for some of the tests, for example a book, newspaper, ipod.
What is supplied?
In the hotel towels and complimentary toiletries are provided. Wifi is also available.
What facilities are available in the hospital?
There is a Lloyd’s pharmacy, a WHSmith and quite a few cafes that are all accessible for patients. There are also free of charge cash machines available next to WHSmith. Across the road from the hospital are a number of small shops and cafes.
How does nitisinone work?
Nitisinone works by stopping the production of homogentisic acid (HGA), which causes the damage in AKU. Nitisinone works by acting within the metabolic pathway of tyrosine, breaking down inhibiting 4-hydrox phenyl pyruvate acid dioxygenase (HPPD), the enzyme that produces HGA. Nitisinone therefore breaks the pathway before the toxic HGA can accumulate.
What research has been done into treating AKU patients with nitisinone?
In America, the NIH conducted clinical trials into treating AKU patients with nitisinone. There were two trials: a short term study lasting only a couple of months and a longer study which lasted five years. Research showed that nitisinone reduced HGA levels by up to 95%.
The University of Liverpool conducted research into the effectiveness of nitisinone in the treatment of AKU. They found that in alkaptonuric mice, nitisinone stopped the progression of ochronosis.
Are there any potential side effects?
Like any drug, there are potential side effects with taking nitisinone. You will receive a booklet explaining nitisinone and any possible side effects before you attend the NAC. This will then be discussed with you during your visit. It is important to remember that all drugs have potential side effects however, it doesn’t mean you will experience those side effects.
What do I do if I think I may be having side effects from using nitisinone?
If you think you may be having adverse effects from using nitisinone, you should contact Prof. Ranganath immediately. You can ring him on 0151 704 4197. Alternatively, you can speak to your local GP. When you attend the NAC you will be given a Patient Alert Card which has details of who you can contact to seek further advice.
Will my GP be informed about nitisinone?
Your GP will receive information about your time at the NAC and they will be made aware that you have been prescribed nitisinone. Should you have any queries or questions about nitisinone, they will be well-equipped to answer these.
Published: 24.07.16 Next Review Date: 24.07.19
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