1) A-T National Clinic - Nottingham
Introduction
The Clinic was launched in October 1993 as a direct result of an initiative by the A-T Society. The need to establish a Clinic where clinical expertise, knowledge of the disease and management of the condition could be built up was seen as a high priority.
The Clinic is held 3 times a year at Nottingham City Hospital and families can choose between a morning or afternoon appointment.
Aims
-
To improve clinical management of A-T.
- To provide a comprehensive written report with advice regarding appropriate management strategies (a report will be sent to the family and their GP).
- To aid research.
- To provide information on all aspects of A-T based on the needs of the individual, the family and the local care team.
The Team
The 'core' team involves a neurologist, geneticist, immunologist, ophthalmologist, physiotherapist and occupational therapist.
From left to right :Dr Colin Dunkley, Dr Susan Ritchie, Dr Gabby Chow, Dr Liz McDermott, Dr William Whitehouse, Dr Nick Davies
The Patient Hotel
It is appreciated that for many families a visit to the Clinic involves much travel. Many have made use of the Patient Hotel on the hospital campus for the night before or after the Clinic appointment. Feedback about this has been very positive. Details are available from the A-T Society.
2) New Clinic at Papworth Hospital for Young Adults with A-T
Background:
In October 2004 the Ataxia-Telangiectasia Society organised an international
meeting in Birmingham to highlight progress in A-T research and the challenges
ahead. An international consensus emerged that chest problems in A-T were
a major cause of ill health and death. Separate studies indicate a number
of factors may contribute to chest problems in A-T including difficulties
in swallowing, breathing, and neuromuscular control; infections, and a deficient
or poorly regulated immune system. Establishing a programme to assess these
factors was established as a priority in A-T. Papworth Hospital was identified
as the UK centre with the blend of expertise required. The adult Ataxia-Telangiectasia
service at Papworth Hospital will include a standardised respiratory, immunological,
neurological assessment to complement the established service at the Nottingham
Clinic.
Papworth
Hospital
The hospital first opened in 1917 as a tuberculosis hospital and when Pendrill
Varrier-Jones conceived the idea, he wanted to create not just a hospital,
but a community. This sense of community remains, but our work has grown
over the years. During the 1950s, Papworth became one of the region’s
main hospitals, developing first thoracic surgery, then cardiac surgery
and cardiology. Today, Papworth provides specialized heart and lung services
to adults including cardiology, cardiac surgery, thoracic surgery, respiratory
medicine and is the country’s main heart and lung transplant centre.
We treat over 20,000 inpatients and day cases and 20,000 outpatients each
year, have 1,300 staff, and 225 beds. Papworth Hospital is one of the foremost
hospitals in the UK and has all the latest equipment that is needed to assess
patients with chest problems and infections. It is located around 10 miles
outside Cambridge with good road access, although public transport is limited.
Referral
Adults (>16 yrs) with A-T should be referred to the clinic by their local
GP or hospital doctor. They can also be referred by Dr Susan Ritchie, Nottingham
City Hospital if they have attended the A-T Clinic based there. Referrals
should be addressed to Dr Exley (consultant immunologist) & Dr Shneerson
(consultant chest physician), Adult A-T Services, Papworth Hospital NHS
Foundation Trust, Papworth Hospital, Papworth Everard, Cambridge. CB3 8RE.
We will collate information from the referring doctor, and the Nottingham
A-T Clinic including previous immunological assessments.
Patient
Pathway
Two visits to Papworth are required for an initial complete consultation
including respiratory, immunological, and neurological assessments. The
first visit will be for 2-3 days as an NHS in-patient, and the second visit,
about 6 weeks later, will include an overnight stay and a consultation with
feedback and advice about the results to patients and their family / carer
as appropriate. The A-T Society has very kindly agreed to pay any reasonable
costs of transferring you from your home to Papworth, if necessary and back,
together with a member of your family or a carer. Patients will be offered
an annual review visit. This would probably entail an overnight stay for
some of the tests to be repeated to assess your progress and to see whether
any changes to your treatment are needed. Patients referred to the Adult
A-T Clinic at Papworth Hospital will be contacted to offer a date for admission.
We will send an information pack with a request for a preliminary blood
sample.
Respiratory Support and Sleep Centre (RSSC)
This is a specialist unit with purpose-built accommodation due to be extended
shortly because of the increasing demand on its services. It is here adults
with A-T will be staying. The RSSC treats large numbers of patients with
sleep disorders but also receives referrals from all over the country for
patients with conditions affecting their respiratory system. Many patients
have chronic disorders of the nervous system which control the pattern of
breathing or the breathing muscles themselves. RSSC staff are used to dealing
with patients with disabilities due to complex neurological disorders and
the implications of these for their activities of daily living. Their aim
is to personalise the care that is required and to pay attention to each
individual’s requirements and they will be pleased to provide any
special assistance that you may need. It is anticipated that each A-T patient
would be accommodated in a side room, each of which has a roll-away bed
for a member of your family or carer.
Respiratory
Infection, Inflammation, and Immunity (RIII) Service
This includes the adult Cystic Fibrosis unit, the Lung Defence and Immunology
clinics and inpatient beds. The RIII attracts supra-regional and national
referrals because of its expertise in the management of respiratory and
immune problems. We have expertise in national clinical databases, whilst
the Lung Defence and Immunology telephone support services deal with over
300 enquiries per quarter. A-T patients will be seen in the RIII clinics
for immunological assessment. We will identify opportunities to support
the immune system with the optimal use of standard vaccines, antibiotics,
anti-virals, and immunoglobulin replacement as appropriate.
The
Multi-Disciplinary Team
Both the RSSC and RIII work in multi-disciplinary teams with excellent support
from many other disciplines. The A-T Society is supporting the Adult A-T
service at Papworth Hospital by providing initial funding for a new A-T
nurse specialist. The A-T nurse specialist will be supported by nurses on
the RSSC and the RIII to help co-ordinate visits and assessments. This nurse
will specialise in the practical and nursing aspects of adult A-T; maintain
and generate reports from the new A-T database; provide a telephone support
service with the RIII; liaise with the Nottingham clinic and the A-T Society;
and attend the Society’s Family Days. The programme includes assessment
of swallowing/reflux by speech and language therapists by a Sips test and
Video fluoroscopy. Patients will also be offered functional assessments
with Respiratory Physiotherapists, Occupational Therapist, and Social Workers.
Radiation
Risks
A high-resolution CT chest scan and video fluoroscopy are standard investigations
for assessment of chest problems, a major cause of ill health and death
in A-T. We also know that A-T is a disorder associated with increased sensitivity
to radiation at levels associated with radiotherapy, rather than diagnostics.
Discussions at the international meeting in October 2004, at the Nottingham
Clinic, with Professor Malcolm Taylor and with the A-T Society have produced
a consensus that a high-resolution CT chest scan and video fluoroscopy are
low risk and essential to clinical assessment in A-T. The CT scan and video
fluoroscopy will be assessed by our radiologists who have considerable expertise
in chest problems. The team are sensitive to concerns about radiation risks,
and are happy to discuss this. No examinations will be carried out without
the patient’s consent.
Dr Andrew Exley - Consultant Immunologist
Dr John Shneerson - Consultant Chest
Physician
Booking
Information
If you would like to book an appointment at either of the Clinics then please contact Kay Atkins at the A-T Society:
A-T Society
IACR-Rothamsted
Harpenden
Herts
AL5 2JQ
United Kingdom
Tel: +44
(0)1582 760733 | Fax:+44 (0)1582 760162
e-mail: atsociety@btconnect.com
Should any family need assistance with travel and/or the patient hotel costs please contact the A-T Society office on 01582 760733
A-T
Clinics |