Skyclarys is a prescription medicine used for the treatment of Friedreich's ataxia, a rare neuromuscular disorder that causes loss of strength and coordination, eventually resulting in patients requiring a wheelchair.

Skyclarys contains omaveloxolone, an activator of the nuclear factor-erythroid 2 related factor 2 (Nrf2) pathway, which is suppressed in people with Friedreich’s ataxia.

• Take Skyclarys exactly as your healthcare provider tells you to take it.
• Take Skyclarys capsules on an empty stomach at least 1 hour before eating.
• Swallow the capsules whole. Do not open, crush, or chew.
• If you miss a dose, then you should skip the missed dose and take the next dose at the regular time the next day. Do not double your next dose or take more than the prescribed dose.

Usual Dose for Friedreich's Ataxia:

150 mg (3 capsules) taken orally once daily

Use: treatment of Friedreich’s ataxia in adults and adolescents aged 16 years and older.

Before you start treatment, tell your healthcare provider about all of your medical conditions, including if you:

• have liver problems.
• have a history of heart problems, including heart failure.
• have a high level of fat in your blood (high blood cholesterol).
• are pregnant or plan to become pregnant.
  • It is not known if Skyclarys will harm your unborn baby.
  • Women who use hormonal birth control should use another form of birth control such as a nonhormonal intrauterine system or an extra non-hormonal birth control such as condoms while using Skyclarys and for 28 days after stopping treatment.
• are breastfeeding or plan to breastfeed. It is not known if Skyclarys passes into your breast milk.
  Talk to your healthcare provider about the best way to feed your baby if you take Skyclarys.

Do not drink grapefruit juice or eat grapefruit. These may change the amount of Skyclarys in your blood.

Skyclarys may cause serious side effects, including:

• Increase in blood liver enzymes. Some people taking Skyclarys have had an increase in the level of liver enzymes in their blood. Your healthcare provider will do liver function tests
  • before you start treatment
  • every month for the first 3 months after starting your treatment
  • during certain times as needed during treatment

If your liver enzymes increase, your healthcare provider may change your dose during treatment, stop treatment for some time, or completely stop treatment.

• Increase in a blood protein called B-Type Natriuretic Peptide (BNP). BNP tells how well your heart is working. Your healthcare provider will check your BNP levels before your treatment. Tell your healthcare provider if you have signs and symptoms of your heart not working well such as too much fluid in your body (fluid overload). Signs and symptoms may include:
  • sudden weight gain (3 pounds or more of weight gain in 1 day, or 5 pounds or more of weight gain in 1 week)
  • swelling in your arms, hands, legs, or feet (peripheral edema)
  • fast heartbeat (palpitations)
  • shortness of breath

If you have symptoms of fluid overload that is considered a side effect of Skyclarys, your healthcare provider may stop treatment.

• Changes in cholesterol levels. Increases in low density lipoprotein cholesterol (LDL-C) or bad cholesterol and decreases in high density lipoprotein cholesterol (HDL-C) or good cholesterol have happened during treatment with Skyclarys.
  Your healthcare provider will check your cholesterol levels before and during your treatment.

The most common side effects include:

• increased liver enzymes (ALT/AST)
• headache
• nausea
• stomach pain
• tiredness
• diarrhea
• muscle pain

These are not all the possible side effects. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800- FDA-1088.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements such as St. John’s Wort.

Taking Skyclarys with other medicines can cause serious side effects.

Skyclarys may affect the way other medicines work, and other medicines may affect how Skyclarys works.

Skyclarys is known to interact with moderate or strong CYP3A4 inhibitors including:

• antifungal medicines (itraconazole, ketoconazole, posaconazole, voriconazole)
• macrolide antibiotics (erythromycin, clarithromycin)
• antiretrovirals (atazanavir, darunavir, indinavir, nelfinavir, ritonavir, saquinavir, cobicistat)
• heart or blood pressure medicines (diltiazem, verapamil)
• nefazodone

Know the medicines you take. Keep a list of them to show to your healthcare provider and pharmacist when you get a new medicine.