Before reading any further it is important to understand the following:
If you have been referred to the National Amyloidosis Centre (NAC) because of a suspected periodic fever syndrome, this does NOT mean that you have amyloidosis.
Very understandably, some patients and family members may become concerned for the following reasons:
- The Periodic Fever service is based at the National Amyloidosis Centre.
- The consultants are specialists in amyloidosis as well as fever syndromes.
It is true that a few patients with longstanding, untreated periodic fever syndromes may eventually develop amyloidosis.
However for the vast majority of patients:
- Referral to the Periodic Fever clinic at the NAC is for the purposes of diagnosis and treatment of a fever syndrome.
- Amyloidosis is not suspected at any time in the course of their illness.
- Amyloidosis can be easily prevented by appropriate treatment.
Since fever syndromes are very rare genetic diseases, patients are often ill for a long time before they are referred and a diagnosis is made. Many patients are referred after undergoing extensive investigations elsewhere. A definite diagnosis of a fever syndrome is made in up to half of the patients referred.
Treatment is now available for many types of inherited periodic fever syndromes. Since the therapy varies according to the precise syndrome, accurate diagnosis is essential. For many patients daily medication is necessary to prevent disease attacks.
When a fever syndrome is diagnosed, the Periodic Fever clinic consultants then follow the patients and recommend appropriate treatments. In most of the cases when a hereditary fever syndrome is not diagnosed, follow up visits after the initial assessment are not recommended.
Inherited Periodic Fever Service
The Inherited Periodic Fever Service is the only centre in the UK dedicated to the needs of patients with inherited fever syndromes. It is based at the National Amyloidosis Centre, with “state-of-the-art” clinical and research facilities and a team of highly qualified clinical, research and support staff. Funding comes from the Department of Health to provide a diagnostic and management advice service for the UK’s national caseload of patients with inherited periodic fevers and related disorders. The clinical service includes:
- Detailed clinical assessment.
- Characterisation and exclusion of hereditary periodic fevers by DNA testing.
- Genetic counselling.
- Recommendations for treatment and monitoring response.
- Measurement and monitoring of specialised biochemical (blood) tests for C-reactive protein (CRP) and serum amyloid A protein (SAA).
- Three-twelve monthly follow-up to assess response and further treatment requirements.
- Providing information and support to fever patients, their families and health providers.
- NHS funded national CAPS treatment service – discussed below.
- Systematic evaluation of existing and new treatments.
The approach to each patient is tailored individually to the type of fever suspected and to the patient’s particular problems. A careful balance is required between administering sufficiently ‘strong’ treatment, and minimising adverse effects. Wherever possible, patients are discussed with the referring physician. After this the consultants at the Periodic Fever clinic may request that blood samples are sent to them for genetic testing. This testing is often performed before the patient’s appointment, so that the results can be discussed in person.
Most patients with inherited fever syndromes need long-term surveillance, with at least six-monthly or annual specialist follow up.
When practical, the consultants provide on-going direct management at the Royal Free Hospital for adults, and at Great Ormond Street Hospital for Children.
When patients live far away, treatment may be administered at their local hospitals or at other regional centres. In these cases, the Periodic Fever clinic consultants can help by providing:
- regular advice to patients and doctors involved in their treatment
- regular reviews at the Royal Free Hospital or Great Ormond Street Hospital for Children
- arrangements for hotel accommodation during visits
The Periodic Fever clinic consultants at the NAC share a specialist paediatric fever clinic with consultants at the Great Ormond Street Hospital for Children. The atmosphere and environment there is child-friendly, with fun activities to occupy children while waiting for their appointment. The specialist paediatric nurses are expert at taking blood from children and putting them at ease. Therefore, most young children and parents prefer to attend this clinic.
Children who require continued follow up over many years may transition to continued care at the Royal Free Hospital clinic. The timing of this transition is usually determined by the doctors after discussion with child and parents. It is usually appropriate sometime between ages 13 and 18, depending on individual preferences.
Occasionally children are seen at the Royal Free Hospital. This may be appropriate for older children if this location is more convenient, if the child prefers a quicker visit and is prepared to undergo drawing of blood without need for specialist paediatric staff. This option should be discussed with the consultants before making the first appointment.