Wikipedia Definition of NF2:
Neurofibromatosis Type II (or "MISME Syndrome", for "Multiple Inherited Schwannomas, Meningiomas, and Ependymomas") is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumours in the region of the cranial nerve VIII, which is the auditory-vestibular nerve that transmits sensory information from the inner ear to the brain. Most people with this condition also experience problems in their eyes. NF II is caused by mutations of the "Merlin" gene, which, it seems, influences the form and movement of cells. The principal treatments consist of neurosurgical removal of the tumors and surgical treatment of the eye lesions. There is no therapy for the underlying disorder of cell function caused by the genetic mutation.
OK, True definition right there, lets take it a step further. They removed four Meningiomas on August 25 so her is that definition from the same source:
Meningiomas are the most common benign tumors of the brain (95% of benign tumors). However they can also be malignant. They arise from the arachnoidal cap cells of the meninges and represent about 15% of all primary brain tumors. They are more common in females than in males (2:1) and have a peak incidence in the sixth and seventh decades. Most cases are sporadic while some are familial. There has been some evidence that persons who have undergone radiation to the scalp are more at risk for developing meningiomas. The most frequent genetic mutations involved in meningiomas are inactivation mutations in the Neurofibromatosis 2 gene (merlin) on chromosome 22q.
A number of studies have linked the slow growing Meningiomas tumor to exposure to certain weed killers, herbicides and pesticides typically used in farming. Women are at a particularly increased risk if they have been exposed to these chemicals for a number of years whereas men with exposure to these chemicals show no increased risk for brain cancer.
Another type of tumor that I have is a Vestibular Schwannoma. Here is the definition for that from Wikipedia:
A schwannoma is a kind of tumor originating from the Schwann cells. The insulating myelin sheath which covers peripheral nerves is produced by the Schwann cells. Hence, one kind of tumor originating from the Schwann cells is called a schwannoma.
Schwannomas are very homogeneous tumors consisting only of Schwann cells. The tumor cells always stay on the outside of the nerve, but the tumor itself may either push the nerve aside and/or up against a bony structure (thereby possibly causing damage). Schwannomas are relatively slow growing. For reasons not yet understood, schwannomas are mostly benign and less than 1% become malignant (degenerate into cancer). This form of cancer is known as malignant peripheral nerve sheath tumour or malignant Schwannoma or neurofibrosarcoma.
Schwannomas can arise from a genetic disorder called neurofibromatosis.
schwannomas can be removed from the nerve but can also come back.
And then you have the information that I found on the NINDS website that states:
What is Neurofibromatosis?The neurofibromatoses are genetic disorders of the nervous system that primarily affect the development and growth of neural (nerve) cell tissues. These disorders cause tumors to grow on nerves and produce other abnormalities such as skin changes and bone deformities. Although many affected persons inherit the disorder, between 30 and 50 percent of new cases arise spontaneously through mutation (change) in an individual's genes. Once this change has taken place, the mutant gene can be passed on to succeeding generations. Scientists have classified the disorders as neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2). NF1 is the more common type of the neurofibromatoses. In diagnosing NF1, a physician looks for changes in skin appearance, tumors, or bone abnormalities, and/or a parent, sibling, or child with NF1. Symptoms of NF1, particularly those on the skin, are often evident at birth or during infancy and almost always by the time a child is about 10 years old. NF2 is less common. NF2 is characterized by bilateral (occurring on both sides of the body) tumors on the eighth cranial nerve. The tumors cause pressure damage to neighboring nerves. To determine whether an individual has NF2, a physician looks for bilateral eighth nerve tumors and similar signs and symptoms in a parent, sibling, or child. Affected individuals may notice hearing loss as early as the teen years. Other early symptoms may include tinnitus (ringing noise in the ear) and poor balance. Headache, facial pain, or facial numbness, caused by pressure from the tumors, may also occur.
Is there any treatment?
Treatments for both NF1 and NF2 are presently aimed at controlling symptoms. Surgery can help some NF1 bone malformations and remove painful or disfiguring tumors; however, there is a chance that the tumors may grow back and in greater numbers. In the rare instances when tumors become malignant (3 to 5 percent of all cases), treatment may include surgery, radiation, or chemotherapy. For NF2, improved diagnostic technologies, such as MRI, can reveal tumors as small as a few millimeters in diameter, thus allowing early treatment. Surgery to remove tumors completely is one option but may result in hearing loss. Other options include partial removal of tumors, radiation, and if the tumors are not progressing rapidly, the conservative approach of watchful waiting. Genetic testing is available for families with documented cases of NF1 and NF2. New (spontaneous) mutations cannot be confirmed genetically. Prenatal diagnosis of familial NF1 or NF2 is also possible utilizing amniocentesis or chorionic villus sampling procedures.
What is the prognosis?
In most cases, symptoms of NF1 are mild, and patients live normal and productive lives. In some cases, however, NF1 can be severely debilitating. In some cases of NF2, the damage to nearby vital structures, such as other cranial nerves and the brainstem, can be life-threatening.
This last part is what scares me the most. I guess you can do all the research you want, read all of the books, study, learn and still the outcome is that. So, what do I want?
I want to be a part of a study that is going to take a closer look into this disorder and into my family. I want them to study me and to see if I passed it on. I want to help them find a cure so that no one else has to ever go through this. I want to do my part.
I need to show my boys that I gave enough of a damn to give it my all to help find a cure so they didn't have to go through this. I want to show my family how all it takes is one person to say "Hey, this isn't going to get me down".
The anniversary of my mom passing is coming up. This time of year is pretty difficult on all of us without adding the strain of all of this. The doctors don't tell you anything unless you ask the questions. I want so badly to ask is this going to be my normal? Do you foresee me living a long life?
I am researching right now my options for radiation, more surgery, gamma knife, cyber knife etc.... So many options.
First things first, I need to figure out how to pay for my medical premiums. 1100.00 and then a whopping 230.00 a month from here on. A rip off if you ask me, but I can't live without it.
May 22, 2009
8 years ago