Neurofibromatosis type 2
Treatment for neurofibromatosis type 2 (NF2) focuses on regular monitoring and, if possible, treatingany problems caused by tumours.
Because of the rarity of NF2, the NHS has created four specialist centres where healthcare professionals with expertise in treating the condition are based.
The specialist centres are located at:
If you're diagnosed withNF2, you'll usually be referred to one of these centres so a treatment plan can be drawn up.
Everyone with NF2 requires regular monitoring to check for signs of any problems developing and, if necessary, treatment will be arranged.
Monitoring NF2 usually involves:
Depending on the extent and severity of your symptoms, more frequent tests may sometimes be required.
Contactyour specialist centre if any new symptoms develop in between these examinations, or if any existing symptoms get worse.
The growth of tumours is one of the main problems associated with NF2 and it's not always obvious what the best treatment is.
Many tumours are small andmay not grow large enough to cause any problems, but others can be large andhave a significant impact on your life.
You should discuss the best option for you with your care team before deciding on a particular treatment.
It's possible to surgically remove sometumours, but the risks involved can often outweigh the benefits.
For example, removing tumours from the nerve tissue next to your ears could further damage your hearing and cause paralysis of your facial muscles.
Removing tumours from the spinal cord carries a small risk of damaging the spinal cord, which could cause some degree of paralysis.
However, in some cases, surgery may be required to prevent potentially serious complications, such as a tumour growing so large that there's a risk of it damaging your brain.
For smaller tumours, a type of radiotherapy known as the gamma knife may be an option. This treatment doesn't involve the use of an actual knife it uses a tightly focused beam of gamma radiation to shrink a tumour.
As with surgical removal,this treatment carries some risks. There's a possibility that the gamma radiation could cause biological changes to occur in tissue, which could result in any new tumours becoming cancerous. The chances of this happeningare thought to bequite small, but it needs tobe considered when weighing upyour treatment options.
If you haveNF2, your hearing will probably become impaired to such an extent that you'll require treatment.
One option may be to consider a hearing implant. These are surgically implanted electrical devices used to bypass problems in the hearing mechanism. There are two types of hearing implants used in NF2, called cochlear implants and auditory brainstem implants (ABIs).
Cochlea implants and ABIs have an external microphone that receives and processes sounds. These signals are passed into an internal receiver before being carried through wires to electrodes either in the cochlea (the coiled, spiral tube inside the inner ear) or the brainstem.
If you have an ABI fitted, the surgeon will first remove any tumours from the hearing nerves. These implants only restore some degree of hearing. However, they can make lip reading easier (see below).
As with all types of surgery, there's a risk of complications. Some of these can be serious, such as infection on the outer layer of the brain ( meningitis ), which can occur in around 1 in every 60 cases. These risks need to be taken into consideration when deciding the best way to manage your hearing problems.
Anotheroption is learning to lip read. Your treatment centre should be able to recommend a hearing therapist or another healthcare professional who's qualified to teach lip reading.
NF2 can also cause several other health problems, which require different treatments. For example, NF2 can cause:
Neurofibromatosis is the name for a number of genetic conditions that cause tumours to grow along your nerves. This article is related to Neurofibromatosis type 2
Symptoms of neurofibromatosis type 2 (NF2) typically start during the late teens or early twenties, but may develop at any age.
Neurofibromatosis type 2 (NF2) is caused by a genetic mutation in a gene called the NF2. Genes are the instructions that are used to produce all human characteristics.
If you or your child develops symptoms of neurofibromatosis type 2 (NF2), a number of tests can be used to look for signs of the condition.
There is currently no cure for NF2, so management focuses on regular monitoring and, if possible, treating problems as and when they arise.