Medications

Riluzole – prescribed specifically to treat ALS. It works by reducing excess glutamate which is believed to be a mechanism in motor neuron death. Available since 1997, it is said to only prolong life (or time before tracheotomy/ventilator is needed) by 3 months. However, more recent studies show that that time frame is actually longer by many months in many cases. It can cause liver damage so routine blood tests are required to assess liver function. If you are debating about taking Riluzole, my neurologist always says this: * if you have insurance, you should take it but if your insurance doesn't cover it then don't take it(financial burden) * if you feel fine when taking it continue taking it but if you have several side effects then don't take it(severe side effects include upset stomach, strange tastes, and feelings around the lips and mouth

Nuedexta - - prescribed to relieve/reduce the PBA (pseudobulbar affect) symptoms of excessive laughing and/or crying at inappropriate times. In countries where Nuedexta is not commercially available or for people that are unable to afford the Nuedexta name brand pills, it can be made at a compounding pharmacy since it is a combination of two other medications, dextromethorphan and quinine. There are PALS who report just taking the dextromethorphan, but if you are not adding the quinine component, then you're not getting the extended availability of the dextromethorphan to the brain, which is what makes Nuedexta effective. Also, if you take too much dextromethorphan, or take your own mix of dextromethorphan/quinine that are not at proven therapeutic levels as demonstrated in the Nuedexta trials, then you can actually have no improvement of the PBA symptoms and increase stomach and bowel issues and other problems. Part of the Nuedexta clinical trials included much higher doses of dextromethorphan but it was found to not be as effective. In addition to relieving PBA symptoms, many patients taking Nuedexta experience improvement in speech and swallowing. There are clinical trials underway to explore this effect. The pharmaceutical company Avanir has a program to reduce the cost of Nuedexta by signing up for a limited use discount card. NOTE: when taking Nuedexta, be sure you do not also take any other medications (especially cold medicines) that contain dextromethorphan. Read labels carefully. Baclofen – prescribed to reduce muscle symptoms, such as spasms and spasticity (stiffness). It is initiated on a schedule until a therapeutic dose is achieved for patient:

**If you reach 60 mg per day in total, you should call your doctor before going any further. If you find a dosage level that is satisfactory, there is no reason to increase the medication. If, after you have increased the medication, you find that the previous dose was just as effective, it would be better to use the lower dose.

Zanaflex – prescribed for muscle cramps. It will make you sleepy, so it’s best taken at night. Alternatives are Flexoril or drinking tonic water with quinine.

Radicava – prescribed specifically for ALS. It is given via IV infusion, requiring either a CVC (Central Venous Catheter, aka “a port”) or PICC line (Peripherally Inserted Central Catheter). It can be administered at an infusion center, doctor’s office, hospital, or at home, preferably through a home health provider. It must be administered on a schedule: Initial cycle: one 60 mg-60 minute infusion per day for 14 days, followed by 14 days off (no infusions). Subsequent cycles: 10 60 mg-60 minute infusions in 14 days, followed by 14 days off (no infusions).