An overview of Neurofibromatosis Type 2

Date issued: April 2013

For review: April 2015

Ref: A-215/neuro/HT/An overview of Neurofibromatosis type 2

PDF:  an overview of neurofibromatosis type 2 [pdf] 352KB

 

Overview of Neurofibromatosis Type 2 (NF2)

This leaflet is for patients and relatives who attend the Peninsula Neurofibromatosis type 2 (NF2) clinic.

NF2 is a medical condition caused by a misprint in one of our genes that is present from birth, but the signs of the disease don’t usually appear until later in life. You may be aware of other people in your family who are affected by this condition, or in some cases you may be the first person to be affected.

If you have not received a diagnosis of NF2, it is likely that you have been invited to the clinic for a full assessment by our specialist team. This is because of some of the symptoms you have experienced maybe associated with NF2.   

NF2 is characterised by non-malignant (non-cancerous) tumours that grow on certain nerves within the body – particularly the nerves responsible for our balance and hearing. These tumours are known as vestibular schwannomas or acoustic neuromas. Over time they may cause deafness and problems with balance. Other benign tumours may also grow elsewhere in the body, such as on the lining of the brain, the spine and under the skin. Some people with NF2 have problems with their vision from a young age or cataracts. NF2 is a variable condition, even amongst members of the same family.

 

About the NF2 service

Plymouth Hospitals NHS Trust has been selected to provide the specialist service to adults at risk or affected by NF2, across the South West Peninsula. Our service is part of a nationally commissioned network, delivering cohesive and efficient care for those people who are under our care.

NF2 is a rare and complex condition, the treatment and management of which involves a number of different healthcare professionals.

To provide the best care this service provides access to a team of professionals with the specialist knowledge and experience necessary to do so.

We are coordinated by the NF2 Specialist Practitioner, who as well as working closely with the NF2 Team, liaises with other relevant agencies and professionals who may be involved in your care and treatment.

Home visits may also be arranged to help support you and your family, whether this is around the time of diagnosis or as part of your on-going treatment plan.

We hold the monthly NF2 team meeting and outpatient clinic Derriford Hospital in Plymouth. This enables the team to work together to discuss the best treatment plan before seeing you in clinic on the same day. The clinic aims to make access to services easier, cutting down the number of hospital appointments to see different professionals.

 

Clinic location

The clinic is situated at Derriford Hospital and your appointment letter will give details of where your appointments will take place.  If you have any special requirements please let us know and we will endeavour to accommodate you.

Parking is available on the hospital site-we suggest that you allow at least 30 minutes for parking prior to your first and subsequent appointments, due to the other specialist services which Derriford Hospital provides for other patients.

There is also a park and ride service that runs every 10-15 minutes from The George Junction Car Park, Plymouth, for more information on this service you can visit www.targettravel.co.uk or call 01752 242000.

How long will it take?

You will receive a letter prior to the clinic giving details of your appointments over the course of the clinic day. Refreshments are available in the café on Level 6 or restaurant on Level 7. You may see professionals individually or receive a joint consultation from more than one person at a time

 

Who will you see?

The core Peninsula NF2 Clinic Team includes:

  • Team Secretary – Sarah Hunter
  • Consultant Neurologist  - Professor O Hanemann
  • Neurosurgeon – Mr J Palmer
  • ENT Surgeon – Mr M Prior
  • NF2 Specialist Practitioner (OT) – Helen Tomkins
  • Ophthalmology – Mr Thaller
  • Audiologist – Adam Beckman
  • Hearing Therapist – Carol Evans
  • Consultant Clinical Geneticist – Dr Bruce Castle
  • Genetic Nurse Counsellor – Andrea Rotchell
  • Radiologist – Dr W Adams

 

MRI Scanning

As part of your assessment we will arrange relevant MRI scans – these may be offered at your local general hospital. The MRI scan will not be on the same day as your clinic appointment because it is better for us to have the results and discuss them in our NF2 Team before we meet with you. If you have a cochlear implant or an auditory brainstem implant (ABI) the MRI department will be informed in advance and your  head will need a bandage and a piece of plastic placed over the magnet to secure it in place. Local anaesthetic can be given if necessary.

Clinical Genetics

The Clinical Geneticist and Genetic Nurse Counsellor can help you understand NF2 as an inherited condition. They will be able to explain in as much detail as you would like about how NF2 is caused. They will ask for information about your health, family history and will also compile your family tree.

Our team can discuss genetic testing and how this is done. There is no obligation to have a genetic test – our aim is to ensure that you have all the information you need to make the right decision for you and your family.

 

Neurology

The consultant neurologist in Plymouth has a specialist interest in NF2. At your clinic appointment he will ask you about your symptoms and how these affect you. He will perform a neurological examination. This involves mobilising limbs and head, looking at your balance, coordination and reflexes. These assessments are useful for clarifying diagnosis, as well as guiding decisions on treatment options and formally determining the progression of the disease.

 

NF2 Specialist Practitioner

We recognise that continuity is an important feature of the care and management of NF2. Our NF2 Practitioner is available to provide information, advice and support for you, your family and the professionals involved in your care. She is the first point of contact if you have any queries about your care or the service. Visits at home or in hospital can be arranged and she is involved in coordinating the clinic and your appointments. The specialist practitioner also coordinates the NF2 Patient and Carer Support Groups across the region. Her role also involves keeping you up-to-date about events, opportunities and developments that may be helpful.

 

Audiology

As part of your clinic appointment you will have a hearing test – this test is called a PTA (pure tone audiogram). You will also have a SDS (speech discrimination score). This enables us to assess how NF2 is affecting your hearing and how you are able to communicate with other people. They are generally carried out annually, so that they can be compared year to year for any changes. Our audiologist works closely with our hearing therapist and ENT surgeon to advise on the best management plan for your hearing needs.

 

Hearing Therapist

Our hearing therapist is able to offer advice on communication aids and communication strategies for hearing loss. She will help to ensure you are linked into the appropriate local resources. She can also advise on management of hearing aids, tinnitus and other hearing related issues.

 

Ophthalmology

Our ophthalmologist will assess the structures of your eye and your sight in detail. NF2 does not always affect your eyes, but this assessment can identify any early changes. It is important that you continue with your annual optician appointments in addition to the ophthalmology appointment you have with us.

 

Neurosurgeon & ENT (Ears, Nose & Throat)

We know that benign tumours of the head, neck and spine are a feature of NF2. You may have an appointment with our neurosurgeon or ENT surgeon.  They specialise in the medical and surgical treatments or procedures if surgery is considered the appropriate intervention for you. The surgical team may refer you on to a plastic surgeon if appropriate.

 

Administrative support

The NF2 team are supported by a secretary who will make sure that your clinic letters are sent to your GP and other healthcare professionals. The secretary will organise for your local hospital records and scans to be transferred from other hospitals. They will also send out your appointments and help collate the questionnaires that you may be asked to complete as part of your visit.

 

Radiologist

Although it is unlikely that you will meet the radiologist, he plays a key role in reviewing your scans and advising the Team on any radiological changes.


How to contact us

You can contact us by post, phone or email:

Helen Tomkins – NF2 Specialist Advisor or

Sarah Hunter -  Medical Team Secretary

NF2 Team Office, Level 4, Derriford Hospital, Plymouth, PL6 8DH

Tel – 01752 431719

Email – htomkins@nhs.net or sarah.hunter3@nhs.net

 

 

Other useful contacts

Action On Hearing Loss (RNID)

Tel: 0808 808 0123

http://www.actiononhearingloss.org.uk/

 

Benefit advice & information

www.direct.gov.uk or your local Citizens Advice Bureau

British Deaf Association

Tel – 01772 259725

Text phone – 05603 115295

www.bda.org.uk

 

British Sign Language Courses

Contact your local adult education provider or contact the NF2 Specialist Advisor

 

British Tinnitus Association

Tel - 0800 018 0527

Email – info@tinnitus.org.uk

www.tinnitus.org.uk

 

Cornwall Council

Services for People with a Sight or Hearing Loss

http://www.cornwall.gov.uk/default.aspx?page=5092

 

Deafblind UK
Tel - 01733 358 100 (Voice/Text)
Email - nfo@deafblind.org.uk

Information and Advice Line: 0800 132 320

 

Disabled Living Foundation

Tel – 0845 130 9117

www.dlf.org.uk

 

Genetic Alliance

Tel – 020 7704 3141

Email – contactus@geneticalliance.org

www.geneticalliance.org.uk

 

Hearing & Sight Centre

Tel – 01752 201766

Text phone – 01752 241087

Hearing Link

Tel - 0300 111 1113 

Email enquiries@hearinglink.org

www.hearinglink.org.uk

 

The Neuro Foundation

Tel – 020 8439 1234

Email – info@nfauk.org

www.nfauk.org

 

Sound Base

Tel. Number: 01392 424018

Fax – 01392 671595

www.devon.gov.uk/contrast/index/socialcare/sensory_disability/hearing/sound-base.htm

 

Worker’s Educational Association – Lip Reading Classes

Tel: 01872 320036

Email: cornwalloffice@wea.org.uk

http://www.wea-sw.org.uk/

 

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