Treating Infantile Spasms – #EpilepsyNYC Infantile Spasms Awareness Week

Infantile spasms is a potentially devastating early-onset epilepsy that affects infants, typically between three and nine months of age. Many cases of infantile spasms occur in children who are already have brain abnormalities, such as children with tuberous sclerosis, down syndrome, or brain injuries sustained as newborns. However, in about one third of affected infants, infantile spasms occur for no known reason.

There are three first-line treatments used for infantile spasms. Most pediatric neurologists select one of these three as the initial treatment.

First, adrenocorticotropic hormone (ACTH) is a naturally occurring hormone that is made by the pituitary gland. It stimulates the body to produce steroids. There are several versions of ACTH available throughout the world — in the US, physicians can prescribe a pharmaceutical grade purified ACTH for infantile spasms. ACTH must be given by injection into the infant’s thigh twice a day for two weeks, and then gradually tapered over the next several weeks. There are significant side effects. Many babies become very irritable when taking this medication. It increases their appetite, and some gain weight during treatment. ACTH can also increase blood pressure and, on occasion, lead to an increase in the size of the heart. These side effects fade after the medication is stopped.

Second, oral prednisolone is a corticosteroid, which comes as a liquid. This is the same medicine given for children with asthma attacks; although, for infantile spasms we use a higher dose for a longer period of time (usually several weeks). It can have the same side effects as ACTH, but these are usually less severe.

Third, vigabatrin is a medication that comes as a packet of powder, which can be disolved in water. The major worrisome side effect of vigabatrin is loss of peripheral vision. This is uncommon. Usually when vigabatrin is prescribed, the infant will also need to see an ophthalmologist to monitor for this potential side effect.

Current evidence suggests that ACTH has the best overall response rate. The one exception is that for children who have infantile spasms due to tuberous sclerosis, vigabatrin seems to work better.

First-line therapy works between half and three quarters of the time. If it fails, there is not good evidence to guide us on what to try next. Many physicians will switch to a different first-line agent. For example, if a child continues to have seizures after ACTH, the physician may try vigabatrin next. Rarely, a deficiency of vitamin B6 can cause infantile spasms, and many physicians will give infants this vitamin. Other physicians may try dietary therapy. For example, there is a diet called “the ketogenic diet” that avoids carbohydrates and sugars. This diet can lead to changes in how the brain makes fuel for itself, which can, in turn, reduce seizures.

In some cases, children with infantile spasms may have a subtle area of the brain which is abnormal, which is difficult to see on MRI scans. Some physicians will order other kinds of brain scans, such as a PET scan (positron emision tomography), in order to look for these kinds of abnormalities. This is important, because some infants benefit from epilepsy surgery to remove the abnormal area of brain.

Dr. Zachary Grinspan, MD
Weill Cornell

Epilepsy and CARERS Act to Reschedule Cannabis Explained

What is the Compassionate Access and Research Expansion and Respect States (CARERS) Act to reschedule cannabis/ medical marijuana, and how does it relate to epilepsy?

The Compassionate Access and Research Expansion and Respect States (CARERS) Act

Medical Marijuana has been shown to alleviate symptoms of serious medical conditions including rare pediatric epileptic and seizure disorders, cancer, AIDS, and glaucoma.

Twenty-three states and the District of Columbia have passed medical marijuana laws legalizing the use of medical marijuana for qualifying patients under state law. However, the medical use of marijuana remains illegal under federal law. Even in states where medical marijuana laws exist, patients and providers are vulnerable to arrest and interference from federal law enforcement.

The bipartisan Compassionate Access and Research Expansion and Respect States (CARERS) Act would not penalize families from accessing prescribed medicine which ease the symptoms of these life threatening diseases by doing the following:

Ends the federal prohibition of medical marijuana under the federal Controlled Substances Act (CSA)

Marijuana is still illegal under any circumstances according to federal law. Physicians who prescribe marijuana for medical treatment in accordance with state law could still face federal prosecution for prescribing marijuana. The CARERS Act amends the CSA so that states can set their own medical marijuana policies. Individuals in states that take part in medical marijuana programs – patients, providers, businesses – will no longer be in violation of federal law.

Reschedules Marijuana from Schedule I to Schedule II

The Controlled Substances Act currently lists cannabis as a Schedule I drug. Schedule I drugs are those that have a high potential for abuse and has no currently accepted medical use in the United States. Cannabis’s classification as a Schedule I drug adds considerable complexity, expense, and potential access problems for clinical research. The CARERS Act moves marijuana from Schedule I to Schedule II. Under Schedule II the drug has a currently accepted medical use in treatment in the United States and may be prescribed by doctors on a limited basis.

Allow importation of Cannabidiol (CBD)

The CARERS Act would give children and adults with epilepsy and other seizure disorders access to the oil (called CBD) for treatment by removing CBD oil from the federal definition of marijuana in the Controlled Substances Act.

Banks to be allowed to do Business with Marijuana Dispensaries

Many banks do not provide financial services for state-authorized marijuana-related businesses because of fear of criminal penalties. This bill would provide legal immunity from federal criminal prosecution to banks and credit unions, their officers and employees that provide financial services to marijuana-related businesses that engage in activities pursuant to state law.

Research

The federal government retains strict controls over the use of marijuana for research purposes. Research expansion has been hindered by a complicated federal approval process and limited availability of research-grade marijuana. The CARERS Act removes unnecessary bureaucratic hurdles for researchers to gain government approval to carry out research on marijuana.

Access to Medical Marijuana for Veterans

VA doctors are currently prohibited from aiding patients seeking medical use of marijuana.The CARERS Act allows Department of Veterans Affairs doctors to recommend medical marijuana to military veterans suffering from serious injuries or chronic conditions like post-traumatic stress disorder, in states where it is legal.

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For more information on how the Epilepsy Foundation of Metropolitan New York (EFMNY) is getting involved, please visit: http://efmny.org/events/

Take action! Urge your Senators to support the CARERS Act to facilitate research on cannabis and help the epilepsy community gain safe, legal access to this treatment option. For more information on this Epilepsy Foundation ACTION ALERT, and how you can get involved, please visit: http://capwiz.com/efa/issues/alert/?alertid=64207976