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Overview: Harliku is used for alkaptonuria, a rare inherited condition that affects the way your body handles certain amino acids (the building blocks for proteins). Common side effects can include eye problems, skin problems, and blood disorders. These can sometimes be serious. Contact your health care provider right away if you have eye pain, vision changes, or painful thick skin on your hands or feet. Keep all appointments, including those for lab work, while you are taking this medicine. Your health care provider may use lab work to monitor for blood disorders.

Uses

What is Harliku used for?

Harliku (nitisinone) is commonly used to lower homogentisic acid levels in pee (urine) in people with alkaptonuria. Alkaptonuria is a rare genetic condition that makes it hard for the body to process the amino acids tyrosine and phenylalanine. 

Harliku may also be used for other conditions as determined by your health care provider.

How does Harliku work (mechanism of action)?

Alkaptonuria is an inherited condition where your body has trouble breaking down the amino acids tyrosine and phenylalanine properly. This can lead to high levels of homogentisic acid. Harliku blocks a chemical in your body that breaks down tyrosine into homogentisic acid. This seems to lower the amount of homogentisic acid in your blood and pee. 

How is Harliku supplied (dosage forms)?

Brands and other names

  • Harliku

Dosage forms and strengths

  • 2 mg oral tablets

How should I store Harliku?

Harliku should be stored at room temperature in the original container with the lid tightly closed, between 68 F and 77 F (20 C and 25 C). It can be exposed to temperatures between 59 F and 86 F (15 C and 30 C) for shorter periods of time, such as when transporting it. Store in a cool, dry place.

Side Effects

What are the most common side effects of Harliku?

The most common side effects of Harliku are listed below. Tell your health care provider if you have any of these side effects that bother you.

  • Increased levels of tyrosine, which is an amino acid
  • Eye problems (see below)
  • Blood disorders (see below)

There may be other side effects of Harliku that are not listed here. Contact your health care provider if you think you are having a side effect of a medicine. In the U.S., you can report side effects to the FDA at www.fda.gov/medwatch or by calling 800-FDA-1088 (800-332-1088). In Canada, you can report side effects to Health Canada at www.health.gc.ca/medeffect or by calling 866-234-2345.

What are the serious side effects of Harliku?

While less common, the most serious side effects of Harliku are described below, along with what to do if they happen.

Severe Allergic Reactions. Harliku may cause allergic reactions, which can be serious. Stop taking Harliku and get help right away if you have any of the following symptoms of a serious allergic reaction.

  • Breathing problems or wheezing
  • Racing heart
  • Fever or general ill feeling
  • Swollen lymph nodes
  • Swelling of the face, lips, mouth, tongue, or throat
  • Trouble swallowing or throat tightness
  • Itching or skin rash
  • Bumps on the skin called hives that can be red, pink, white, or brown depending on your skin tone
  • Nausea or vomiting
  • Dizziness, feeling lightheaded, or fainting
  • Stomach cramps
  • Joint pain

Severe Eye Problems.Harliku may cause eye problems, such as pink eye (conjunctivitis), inflammation of the cornea (keratitis), or a corneal ulcer. Keep all health care appointments before you start and while you are taking Harliku, including for eye exams or lab work. Contact your health care provider right away if you have any of the following symptoms.

  • Eye redness, itchiness, burning, or pain
  • Swollen or itchy eyelids 
  • Feeling like you have something in your eye
  • Eye dryness, watering, or discharge
  • Vision changes, such as blurry vision or sensitivity to light

Painful Skin Problems. Harliku may cause you to have painful, thick areas of skin on the soles of your feet or palms of your hands. Your health care provider may check lab work or give you a special diet to follow if this happens. Keep all of your appointments, including those for lab work, while you are taking Harliku. Tell your health care provider if you notice pain or thick skin on your hands or feet. 

Blood Disorders. Harliku can cause blood disorders, such as low platelet levels (thrombocytopenia) and low white blood cell levels (leukopenia). Keep all of your appointments, including for lab work to check your platelet and white blood cell levels, while you are taking Harliku. 

Warnings & Precautions

Who should not use Harliku?

Allergies to Ingredients. People who are allergic to any of the following should not take Harliku.

  • Harliku
  • Nitisinone
  • Any of the ingredients in the specific product dispensed

Your pharmacist can tell you all of the ingredients in Harliku.

What should I know about Harliku before using it?

Do not take Harliku unless it has been prescribed to you by a health care provider. Take it as prescribed.

Do not share Harliku with other people, even if they have the same condition as you. It may harm them.

Keep Harliku out of the reach of children.

Follow any instructions your health care provider gives you about limiting the amount of protein you eat or drink. Changes in your diet can sometimes be used to limit your side effects. See the Side Effects section for more details. 

What should I tell my health care provider before using Harliku?

Tell your health care provider about all of your health conditions and any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. This will help them know if Harliku is right for you.

In particular, make sure that you discuss any of the following.

Other Medicines and Supplements. Harliku may interact with other medicines and supplements. Before taking Harliku, tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. See the Interactions section for more details.

Pregnancy. It is not known if or how Harliku could affect pregnancy or harm an unborn baby. Tell your health care provider if you are pregnant or plan to become pregnant.

Breastfeeding. It is not known if Harliku passes into breast milk. Tell your health care provider if you are breastfeeding or plan to breastfeed. Your health care provider will let you know if you should take Harliku while breastfeeding.

How can I get more information about Harliku?

For more information about Harliku, you can visit the drugmaker’s website at harliku.com or call them at 888-360-8482.

Interactions

Does Harliku interact with foods or drinks?

There are no known interactions between Harliku and foods or drinks.

It is unknown if drinking alcohol will affect Harliku.

Does Harliku interact with other medicines (drug interactions)?

Always tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using.

In particular, make sure that you discuss if you are using any of the following before taking Harliku.

  • Celecoxib (Celebrex, Elyxyb), which is a medicine commonly used for pain and inflammation
  • Adefovir (Hepsera), which is a medicine used for hepatitis B
  • Methotrexate (Trexall and others), which is a medicine commonly used for rheumatoid arthritis or certain types of cancer
  • Phenytoin (Dilantin, Phenytek, and others), which is a medicine to control seizures
  • Warfarin, which is a blood thinner

Harliku may alter the blood levels of some other medicines. Tell your health care provider about all medicines that you take or have recently taken.

This may not be a complete list of medicines that can interact with Harliku. Always check with your health care provider.

Does interact with other drugs you are taking?

Enter your medication into the WebMD interaction checker

Overdose/Missed Dose

What should I do if I accidentally use too much Harliku?

If you or someone else has used too much Harliku, get medical help right away, call 911, or contact a Poison Control center at 800-222-1222.

What should I do if I miss a dose of Harliku?

If you miss a dose, skip the missed dose and only take the next dose when you normally should. Do not take double or extra doses.