Welcome to the Central Nervous
System Vasculitis Help Page!
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This website is intended as a resource for people with Central Nervous System Vasculitis (CNSV) and their families.
The information and references
contained within this site are presented as a guide, but should be used at your
own risk.
This site should not be used to make medical decisions without the consent of
your doctors.
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I am not a doctor.
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Doctors |
Central Nervous System Vasculitis is a disorder
involving the immune system and the brain.
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=> CNSV
- CNSV can be broadly classified as Primary or Secondary. Primary CNSV means there is no identifiable cause for the disorder. Secondary CNSV means there is a known cause for the disorder. Common causes are infection and drug use.
- CNSV is very hard to diagnose. CNSV can masquerade as other disorders. The lack of specific tests capable of distinguishing between CNSV and these other disorders makes diagnosis difficult.
- The most accurate way to diagnose CNSV is with a brain biopsy. Brain biopsy is a very risky procedure with a success rate of only 25% for diagnosis. CNSV requires a complicated and risky treatment plan with many possible side effects. It is imperative that all other disorders are ruled out before undergoing this procedure.
- My presentation of CNSV is atypical. After the brain biopsy, they confirmed CNSV but have not found any underlying cause. My vasculitis is contained exclusively to the microscopic blood vessels.
The following are suggestions on how to approach treating CNSV from a patient's perspective. These suggestions could also be applied to any disorder. Remember, it is your disorder and your body. If you are not comfortable with the doctors you are seeing it is your right to change. Luckily I have been able to find excellent doctors that are very knowledgeable and compassionate.
- Be proactive in your treatment
- Ask questions to understand the disorder. Understand the risks of treatment and possible side effects and how to mitigate these risks. Asking questions of your doctor can instill confidence that they truly understand the disorder and are providing the right treatment for your particular case. A good doctor will allow time for your questions and will not be offended by you asking them. An informed patient is a good patient.
- Get and keep good records
- Hospital and doctors offices keep records that you are entitled to have a copy of. When discharged from the hospital ask for a copy of the records and test results. This will expedite services at other offices if you can bring those records with you.
- Be organized
- CNSV is a confusing disorder. There will be many appointments,
treatments, bills, and paperwork to keep track of. I have started collecting
records, receipts, and paperwork into three-ring binders. This provides easy access to
supporting information to deal with insurance companies, employers and
for tax purposes (remember non-reimbursed medical expenses can be a tax write
off (Form P502.pdf) if they
exceed a certain amount of your income!
). - Be compliant
- Follow up with your doctors when requested. Take your medicines as ordered. Eat healthy and get plenty of rest.
- Learn
- CNSV is an auto-immune disorder. A good understanding of how the immune system works and how CNSV is affecting you can help make informed decisions about your treatment. This website may be a starting point, but there are many more resources out there to help.
CNSV is a very complicated disorder. My recommendation is that you use specialists for each component of your treatment. To make this arrangement work, it is imperative that all of the doctors cooperate and communicate to avoid complications. This puts the responsibility of managing the care on the patient. You could also use your primary care physician if they are cognizant of CNSV and its treatment. The doctors that I believe should be involved in the treatment of CNSV are listed below:
- Neurologist: Responsible for initial diagnosis. Should be involved throughout treatment to assess and track neurologic condition. Prescribes medication to control seizures, mediate anxiety and depression, and others as needed for specific presentation in each individual.
- Rheumatologist: Responsible for administering the treatment once diagnosis is made.
- Primary Care Physician: Can manage overall care. Can provide care for peripheral side effects from treatment.
- Neuropsychologist: Can provide assistance with assessment of cognitive deficits, cognitive retraining, therapy, and techniques to deal with the life changes CNSV brings.
- Psychiatrist: Can provide help with anxiety and depression management.
- Ophthalmologist: Specialist for certain side effects of long term steroid usage involving eyes.
- Ear Nose and Throat: Specialist for side effects from CNSV treatment involving immune suppression.
The treatment of CNSV involves three main phases of the disease. The acute phase is characterized by flares of the disease. The goal during this phase is to stop any further damage by reducing the inflammation that is causing the stoppage of blood flow. The subacute phase is characterized by continuation of therapy for a time sufficient to keep the condition in remission and allow healing in the brain. The consolidation phase is characterized by a tapering of treatments and monitoring activities to ensure the condition remains in remission.
- Goal is to stop the disease quickly to avoid further damage. This is done by reducing the inflammation using steroids. Typically a pulse dose of Solu-Medrol is given intravenously. Be aware that these pulses of steroids can give you a feeling of elation (Steroid Euphoria) while it is being administered, and a feeling of depression when it is discontinued. You may also experience muscle pain (myalgia), joint pain (arthalgia), and vision problems when the steroids are stopped.
- Long term treatment of inflammation will be done with oral steroids. Typically Prednisone is used.
- Cytoxan will be used to treat the disorder at the immune system level. The effect of this treatment is to suppress the immune system and effectively reset the immune system's memory so that it will not attack the vessels in the central nervous system. It does this by acting on the white blood cells that control the immune response in the body. The treatments will be administered monthly and the dosage will be monitored by blood testing to make sure the immune system is suppressed sufficiently, but not too low. A typical treatment goes something like this:
- An IV is started to inject the Cytoxan. I suggest getting an early appointment so that you have plenty of time to drink fluids after the treatment.
- Before the Cytoxan is administered, a solution of normal saline is administered. Approximately 1.5 L is used.
- Next an anti-nausea medication is administered. Zofran or Anzemet are typically used. In my case, the Cytoxan makes me sick for approximately 5 days. The nausea is then replaced by flu-like symptoms. I believe this is caused by your body trying to get rid of all of the dead blood cells that the Cytoxan has killed.
- It is imperative that you drink plenty of fluids after the treatment to keep your bladder active. This will mitigate the chances of developing bladder cancer from the Cytoxan.
- After 7-14 days, your immune function will reach its lowest point. A CBC test will be done to ensure that your blood counts do not dip to low.
- Sub-acute Therapy
- The goal in this phase is to keep the disease under control for approximately 6 months.
- During this time it may be appropriate to begin any cognitive assessment and retraining.
- The doctor may begin to taper the steroids.
- The longer the steroids are used, the greater the effects of myopathy and myalgia will be felt.
- During this time you will likely be reevaluated by the neurologist to see how the disease is progressing.
- If a disease flare occurs, the clock is reset and the treatment starts over.
- The treatment of Cytoxan and long-term steroid usage will bring on progressive weakness. It is important to continue to exercise when you are able. This will help fight some of the steroid side effects as well as prevent many opportunistic diseases from taking hold.
- Goal is to begin tapering treatment. This may involve spacing out the treatment intervals, or switching the medication to a less toxic immunosupressant. Methotrexate or Imuran are possible options.
- This can be a frightening time in the treatment plan. As the Cytoxan dosage and frequency are reduced, your body begins to start taking over the responsibility of maintaining your immune system again. It is important to notify your doctors of any significant change in your condition as soon as possible, since it may indicate a disease flare.
- The ultimate goal is to return to a normal and healthy life, free from medications.