Adakveo is a brand-name prescription medication. It’s FDA-approved to prevent vaso-occlusive crisis (VOC) in adults and in children ages 16 years and older with sickle cell disease. VOC is also called pain crisis.

Sickle cell disease is a genetic condition that affects the shape of your red blood cells. In most cases, people get a diagnosis of sickle cell disease when they’re a baby.

Pain crisis is a condition that affects some people with sickle cell disease. Pain crisis usually causes very sudden, severe pain. It may also cause a blood clot, which increases the risk of stroke or organ damage.

Drug details

Adakveo comes as a liquid solution that’s given as an intravenous (IV) infusion into your arm. (An infusion is an injection into your vein that’s slowly dripped in over time). The drug comes in one strength: 100 milligrams of the drug per 10 milliliters of solution.

You may get your Adakveo doses at an infusion suite, which is a healthcare facility specifically used to give infusions. You may also receive Adakveo at your doctor’s office or at the hospital. In some cases, you may be able to receive the infusion in your home, given by a nurse.

Adakveo belongs to a group of medications called selectin blockers. The active drug in Adakveo is crizanlizumab.

FDA approval

Adakveo was approved by the Food and Drug Administration (FDA) in November 2019. Adakveo is the first targeted treatment for pain crisis. This means it works differently than other medications for pain crisis. Instead of treating the pain, Adakveo targets blood cells and prevents blood clots.

Effectiveness

For information about the effectiveness of Adakveo, see the “Adakveo uses” section below.

Adakveo is a biologic drug that’s available only as a brand-name medication. It doesn’t come in a biosimilar form.

A biologic drug is made from living cells, while other drugs are made from chemicals. Drugs made from chemicals can have generics, which are exact copies of the active drug in the brand-name medication. Biologics, on the other hand, can’t be copied exactly. So, instead of a generic, biologics have biosimilars. Biosimilars are “similar” to the parent drug, and they’re considered to be just as effective and safe.

Like generics, biosimilars are often less expensive than brand-name medications.

Adakveo contains the active drug crizanlizumab.

The Food and Drug Administration (FDA) approves prescription drugs such as Adakveo to treat certain conditions. Adakveo may also be used off-label for other conditions. Off-label use is when a drug that’s approved to treat one condition is used to treat a different condition.

Adakveo is FDA-approved to prevent vaso-occlusive crisis (VOC) in adults and in children ages 16 years and older with sickle cell disease. VOC is also called pain crisis.

What happens with sickle cell disease?

Sickle cell disease is a genetic (inherited) condition that affects the shape of your red blood cells.

Red blood cells are usually round. But in people with sickle cell disease, the red blood cells are shaped are C shaped (like a sickle). Because of their shape, the cells can get stuck to the inside of your blood vessels, causing pain. When this occurs, it’s called vaso-occlusive crisis (VOC) or a pain crisis.

Pain crisis usually causes very sudden, severe pain. It may also cause a blood clot, which increases the risk of stroke or organ damage.

In people with sickle cell disease, pain crisis may occur multiple times per year. When pain crisis occurs, it often leads to a hospital stay. It’s not known which people with sickle cell disease have a higher risk for pain crisis. But infection, dehydration, and cold weather may increase the risk.

Effectiveness for pain crisis in sickle cell disease

In a clinical study, Adakveo was found to be effective for treating pain crisis in sickle cell disease. It was studied over the course of 1 year in people who had at least two to 10 pain crisis episodes in the year before the study.

The study showed that over a 1-year period, pain crisis did not occur in:

  • 36% of people using Adakveo
  • 17% of people using a placebo (a treatment with no active drug)

Pain crisis also took longer to occur in people using Adakveo compared with people taking a placebo. The clinical study showed that pain crisis occurred within:

  • 4.1 months after starting Adakveo
  • 1.4 months after starting a placebo

The study also showed that pain crisis occurred at a rate of:

  • 1.63 per year in people using Adakveo
  • 2.98 per year in people using a placebo

Overall, this study showed that Adakveo reduced the number of pain crises that people with sickle cell disease had per year. It also reduced the number of days per year that people spent in a hospital for complications of sickle cell disease, including pain crisis.

Adakveo and children

The clinical study of Adakveo included children ages 16 years and older. It showed that Adakveo was safe and effective for children in this age group.

For more information on the clinical study of Adakveo, see the “Effectiveness for pain crisis in sickle cell disease” section above.

Adakveo hasn’t been studied in children younger than 16 years old, so it’s not known if it would be safe or effective in this age group.

Adakveo is FDA-approved to prevent vaso-occlusive crisis (VOC) in adults and in children ages 16 years and older with sickle cell disease. VOC is also called pain crisis.

What happens with sickle cell disease?

Sickle cell disease is a genetic (inherited) disease that affects the shape of your red blood cells. These cells carry oxygen throughout your body.

Red blood cells are normally round. But in people with sickle cell disease, these cells are C shaped (like a sickle). Because of their shape, they can get stuck together and also to the inside of your blood vessels, which causes very sudden, severe pain. When this occurs, it’s called vaso-occlusive crisis (VOC). Another term for VOC is pain crisis.

Pain crisis occurs because the cells that are stuck together block blood and oxygen from moving through your blood vessels properly. Pain crisis usually causes very sudden, severe pain. It may also cause a blood clot, which increases the risk of stroke or organ damage.

What Adakveo does

Adakveo is a selectin inhibitor that prevents pain crisis. This means Adakveo sticks to and blocks a protein in your blood called P-selectin. This protein is what causes your cells to stick together.

By blocking P-selectin, Adakveo prevents your blood cells from sticking together. This lowers your risk for pain crisis and other severe complications of sickle cell disease, such as stroke and organ damage. This can also help you avoid a hospital stay to treat pain crisis.

The manufacturer of Adakveo created a video explaining how the drug works, which you can view to learn more.

How long does it take to work?

Adakveo begins working right away to prevent pain crisis. Because Adakveo works to prevent pain crisis, you may not notice any changes right away. But you may notice that, over time, you’re experiencing fewer (if any) pain crises.

To learn about how Adakveo worked in a clinical study, see the effectiveness information in the “Adakveo for pain crisis in sickle cell disease” section above.

Adakveo can cause mild or serious side effects. The following lists contain some of the key side effects that may occur while using Adakveo. These lists do not include all possible side effects.

For more information about the possible side effects of Adakveo, talk with your doctor or pharmacist. They can give you tips on how to deal with any side effects that may be bothersome.

Note: The Food and Drug Administration (FDA) tracks side effects of drugs it has approved. If you would like to notify the FDA about a side effect you’ve had with Adakveo, you can do so through MedWatch.

Mild side effects

Mild side effects of Adakveo can include:*

  • joint or back pain†
  • fever†
  • nausea, vomiting, or diarrhea
  • abdominal (belly) pain
  • itching

Most of these side effects may go away within a few days or a couple of weeks. But if they become more severe or don’t go away, talk with your doctor or pharmacist.

* This is a partial list of mild side effects from Adakveo. To learn about other mild side effects, talk with your doctor or pharmacist or check Adakveo’s patient information.
† For more information on this side effect, see “Side effect details” below.

Serious side effects

Serious side effects from Adakveo aren’t common, but they can occur. Call your doctor right away if you have serious side effects. Call 911 or your local emergency number if your symptoms feel life threatening or you think you’re having a medical emergency.

Serious side effects, described below in “Side effect details,” can include:

Side effects in children

Adakveo has been studied in a clinical study that included both adults and children ages 16 years and older. In the study, side effects seen in children were similar to those seen in adults. To learn more, see the “Mild side effects” and “Serious side effects” sections above.

Adakveo hasn’t been studied in children younger than 16 years old, so it’s not known if it would be safe or effective in this age group.

Side effect details

You may wonder how often certain side effects occur with this drug. Here’s some detail on certain side effects this drug may cause.

Allergic reaction

As with most drugs, some people can have an allergic reaction after using Adakveo. Symptoms of a mild allergic reaction can include:

  • a rash
  • itchiness
  • flushing (warmth, swelling, or redness in your skin)

A more severe allergic reaction is rare but possible. Symptoms of a severe allergic reaction can include:

  • swelling under your skin, typically in your eyelids, lips, hands, or feet
  • swelling of your tongue, mouth, or throat
  • trouble breathing

It’s not known how many people may have experienced an allergic reaction in the clinical study of Adakveo.

Call your doctor right away if you have an allergic reaction to Adakveo, as the reaction could become severe. Call 911 or your local emergency number if your symptoms feel life threatening or you think you’re having a medical emergency.

Infusion reactions

Adakveo is given as an intravenous (IV) infusion into your arm. (An infusion is an injection into your vein that’s slowly dripped in over time). Some people may have a reaction to the Adakveo infusion. Infusion reactions typically occur within 24 hours of getting your dose.

Symptoms of an infusion reaction to watch for after your infusion include:

In a clinical study, infusion reactions occurred in 3% of people using Adakveo. It’s not known how many people taking a placebo (a treatment with no active drug) may have experienced an infusion reaction.

If you develop any infusion reaction symptoms while taking Adakveo, talk with your doctor right away. In some cases, such as if you’re having trouble breathing, you may need to go to the hospital and be seen by a doctor immediately.

If you have an infusion reaction, your doctor may have you stop using Adakveo and switch to a different medication to treat your condition.

Joint or back pain

Some people may experience joint or back pain while using Adakveo. In the clinical study of Adakveo, joint pain occurred in:

  • 18% of people using Adakveo
  • 8% of people using a placebo (a treatment with no active drug)

In the study, back pain occurred in:

  • 15% of people using Adakveo
  • 11% of people using a placebo

If you develop back or joint pain while using Adakveo, talk with your doctor. They may be able to recommend ways to relieve this side effect. If your pain is severe or bothersome to you, they may recommend a different medication to treat your condition.

Fever

Fever may occur during Adakveo treatment. Symptoms of fever include chills, sweating, and fatigue (lack of energy).

In the clinical study, fever occurred in:

  • 11% of people using Adakveo
  • 7% of people using a placebo (a treatment with no active drug)

If you develop a fever while using Adakveo, talk with your doctor right away. They may want to see you to determine the cause of your fever. In some cases, they may recommend a different medication to treat your condition.

The Adakveo dosage your doctor prescribes will depend on several factors. These include:

  • your body weight
  • other medical conditions you may have

The following information describes dosages that are commonly used or recommended. But your doctor will determine the best dosage to fit your needs.

Drug forms and strengths

Adakveo comes as a liquid solution that’s given as an intravenous (IV) infusion into your arm. (An infusion is an injection into your vein that’s slowly dripped in over time). Adakveo comes in a strength of 100 milligrams (mg) of the drug per 10 milliliters of solution.

Dosage for pain crisis in sickle cell disease

The dosage to prevent pain crisis in sickle cell disease is based on your body weight. The drug is given as 5 mg per kilogram (kg) of your body weight. For example, if you weigh 165 pounds (75 kg), you’ll get a dose of 375 mg.

When you start treatment with Adakveo, you’ll get one dose, followed by a second dose 2 weeks later. After that, you’ll get a dose once every 4 weeks.

You may get your Adakveo doses at an infusion suite, which is a healthcare facility specifically used to give infusions. You may also receive Adakveo at your doctor’s office or at the hospital. In some cases, you may be able to receive the infusion in your home, given by a nurse.

Children’s dosage

Adakveo is approved for use in children ages 16 years and older. The Adakveo dosage for children is determined in the same way as it is for adults, based on body weight. The drug is also given on the same schedule for adults and children. To learn more, see the “Dosage for pain crisis in sickle cell disease” section directly above.

What if I miss a dose?

If you miss your appointment for a dose of Adakveo, call your doctor as soon as possible to reschedule. They’ll determine whether you can continue with your current doing schedule or will need to move any appointments because of the missed dose.

To help make sure you don’t miss an appointment for your Adakveo infusion, try setting a reminder on your phone.

Will I need to use this drug long term?

Adakveo is meant to be used as a long-term treatment. If you and your doctor determine that Adakveo is safe and effective for you, you’ll likely use it long term.

Other drugs are available that can treat your condition. Some may be a better fit for you than others. If you’re interested in finding an alternative to Adakveo, talk with your doctor. They can tell you about other medications that may work well for you.

Note: Some of the drugs listed here are used off-label to treat these specific conditions. Off-label use is when a drug that’s approved to treat one condition is used to treat a different condition.

Alternatives for pain crisis in sickle cell disease

Examples of other drugs that may be used to treat pain crisis include:

Adakveo may be used with other treatments to prevent vaso-occlusive crisis (VOC). VOC is also called pain crisis.

For example, some people may take hydroxyurea (Droxia) with Adakveo. Like Adakveo, hydroxyurea is a medication used to prevent pain crisis in people with sickle cell disease. In the clinical study, 64% of people using Adakveo were also taking hydroxyurea.

In some cases, Adakveo may also be given along with pain medications. Examples of pain medications that you may take along with Adakveo include ibuprofen (Advil, Motrin), diclofenac (Voltaren), and morphine (MS Contin).

Adakveo may also be given along with blood transfusions, if needed.

If you have questions about other treatments you’ll need to use with Adakveo, talk with your doctor.

There are no known interactions between Adakveo and alcohol. However, alcohol is a trigger of sickle cell disease. Therefore, drinking alcohol may raise your risk for pain crisis related to sickle cell disease.

If you drink alcohol, talk with your doctor about how much is safe for you to drink with your condition.

Adakveo is not known to interact with other medications. It’s also not known to interact with any herbs, supplements, or foods. However, you should still talk with your doctor about any medications, herbs, or supplements you take before starting treatment with Adakveo.

Adakveo can affect the results of certain lab tests. See “Adakveo and lab tests” directly below to learn more.

If you have questions about any interactions that may affect you, ask your doctor or pharmacist.

Adakveo and lab tests

Adakveo may cause false results for a blood test that measures your platelets (a type of blood cell). Adakveo may cause your platelet levels to show as lower than they actually are, or it might make them not measurable at all. This is especially a risk if your blood is collected in a tube that has the chemical EDTA in it. EDTA can raise the risk of incorrect results from a platelet lab test.

Make sure that the healthcare professional performing your blood test knows you’re using Adakveo. They can collect your blood in a tube that contains citrate instead of EDTA. (Citrate won’t interact with the Adakveo.) They may also run a different type of blood test, called a peripheral blood smear, to measure your platelet levels.

As with all medications, the cost of Adakveo can vary. The actual price you’ll pay depends on your insurance plan, your location, and the pharmacy you use.

Before approving coverage for Adakveo, your insurance company may require you to get prior authorization. This means that your doctor and insurance company will need to communicate about your prescription before the insurance company will cover the drug. The insurance company will review the prior authorization request and decide if the drug will be covered.

If you’re not sure if you’ll need to get prior authorization for Adakveo, contact your insurance company.

Financial and insurance assistance

If you need financial support to pay for Adakveo, or if you need help understanding your insurance coverage, help is available.

Novartis Pharmaceuticals Corporation, the manufacturer of Adakveo, offers a program called Adakveo Support at PANO (Patient Assistance Now Oncology). For more information and to find out if you’re eligible for support, call 800-282-7630 or visit the program website.

Biosimilar version

Adakveo is a biologic drug that’s available only as a brand-name medication. It doesn’t come in a biosimilar form.

A biologic drug is made from living cells, while other drugs are made from chemicals. Drugs made from chemicals can have generics, which are exact copies of the active drug in the brand-name medication. Biologics, on the other hand, can’t be copied exactly. So, instead of a generic, biologics have biosimilars. Biosimilars are “similar” to the parent drug, and they’re considered to be just as effective and safe.

Like generics, biosimilars are often less expensive than brand-name medications.

Adakveo comes as a liquid solution that’s given as an intravenous (IV) infusion into your arm. (An infusion is an injection into your vein that’s slowly dripped in over time). You’ll receive Adakveo from a healthcare provider such as a doctor or nurse. The infusion will take about 30 minutes.

When it’s given

When you start treatment with Adakveo, you’ll get one dose followed by a second dose 2 weeks later. After that, you’ll get a dose once every 4 weeks.

You may get your Adakveo infusions at an infusion suite, which is a healthcare facility specifically used to give infusions. You may also receive Adakveo at your doctor’s office or at the hospital. In some cases, you may be able to receive the infusion in your home, given by a nurse.

To help make sure you don’t miss an appointment for your Adakveo dose, try setting a reminder on your phone.

It’s not known if Adakveo is safe to use during pregnancy. However, in animal studies, Adakveo caused an increase in both stillbirth and miscarriage. Because of this possible risk, you should only use Adakveo if the benefits are greater than the possible risk of harm to a fetus.

It’s important to note that, in rare cases, sickle cell disease may raise the risk of miscarriage.

If you’re pregnant or planning a pregnancy, talk with your doctor before starting Adakveo.

It’s not known if Adakveo is safe to take during pregnancy. If you’re sexually active and you or your partner can become pregnant, talk with your doctor about your birth control needs while you’re using Adakveo.

For more information about using Adakveo during pregnancy, see the “Adakveo and pregnancy” section above.

It’s unknown whether Adakveo is safe to use while breastfeeding. Adakveo has not been studied to see if it passes into breastmilk.

If you’re breastfeeding, you should only take Adakveo if the benefit of taking the drug is greater than the risk of harm to a fetus. Talk with your doctor about the benefits and risks of using Adakveo while breastfeeding.

Here are answers to some frequently asked questions about Adakveo.

Will Adakveo cure my sickle cell disease?

No, Adakveo will not cure your sickle cell disease. However, it can help prevent vaso-occlusive crisis (VOC). VOC may also be known as pain crisis. The drug reduces the pain that you may experience from your sickle cell disease.

The only cure for sickle cell disease is a stem cell transplant. This is a high-risk procedure in which your immune system is destroyed and your bone marrow cells are replaced by a donor’s cells. Stem cell transplants are not commonly done to treat sickle cell disease.

Where can I get Adakveo infusions?

Adakveo can only be given by a healthcare provider such as a doctor or a nurse. This is because Adakveo is given as an intravenous (IV) infusion. (An infusion is an injection into your vein that’s slowly dripped in over time).

You may receive your Adakveo dose at an infusion suite, which is a healthcare facility specifically used to give infusions. You may also receive Adakveo at your doctor’s office or at the hospital. In some cases, you may be able to receive the infusion in your home, given by a nurse.

Talk with your doctor about the best place for you to receive your Adakveo dose.

Will Adakveo weaken my immune system?

No, Adakveo is not known to weaken your immune system. In the Adakveo clinical trial, a weakened immune system was not reported as a side effect of the drug.

However, other medications you may take with Adakveo can weaken your immune system. For example, hydroxyurea (Droxia) decreases the amount of blood cells your body makes, which can weaken your immune system.

Sickle cell disease itself may also weaken your immune system. This is because sickle cell disease can affect your spleen, which affects your immune system by removing bacteria from your blood. Sickle cell disease can cause blockages in the vessels that lead to your spleen, which means blood can’t flow in or out properly. This may also cause swelling and pain.

If you have questions about how your condition or treatment may affect your immune system, talk with your doctor.

Can I take other pain relievers with Adakveo?

Yes, you can take other pain relievers during your Adakveo treatment. Examples of pain relievers you may take with Adakveo include ibuprofen (Advil, Motrin), diclofenac (Voltaren), and morphine (MS Contin).

If you have any questions about which pain relievers may work best for you, talk with your doctor or pharmacist.

Will Adakveo interfere with any regular lab tests I get for sickle cell disease?

It’s possible that Adakveo may cause false results for a blood test that measures your platelets (a type of blood cell). Adakveo may cause your platelet levels to show as lower than they actually are, or it might make them not measurable at all. This is especially a risk if your blood is collected in a tube that has the chemical EDTA in it. EDTA can increase the risk of incorrect results from a platelet lab test.

Make sure that the healthcare professional performing your blood test knows you’re using Adakveo. They can collect your blood in a tube that contains citrate instead of EDTA. (Citrate won’t interact with the Adakveo.) They may also run a different type of blood test, called a peripheral blood smear, to measure your platelet levels.

Do I have to have a certain genotype to take Adakveo?

No, you can take Adakveo with any genotype. A genotype is your genetic makeup. You get one copy of each of your genes from your mother and one copy from your father. Your genotype is the combination of your two copies of each gene.

In the clinical study, all genotypes of sickle cell disease were included. Adakveo was found to be effective in preventing pain crisis in any genotype.

The most common form of sickle cell disease occurs in people who have the HbSS genotype. People with the HbSS genotype have two “S” genes. However, there are other genotypes of sickle cell disease, including HbSC and HbS beta-thalassemia.

Before using Adakveo, talk with your doctor about your health history. Adakveo may not be right for you if you have certain medical conditions or other factors affecting your health. These include:

  • Allergic reaction. If you’ve had an allergic reaction to Adakveo or any of its ingredients, you shouldn’t take Adakveo. Ask your doctor about other medications that may be better options for you.
  • Pregnancy. It’s not known if Adakveo is safe to use during pregnancy. For more information, see the “Adakveo and pregnancy” section above.
  • Breastfeeding. It’s unknown whether Adakveo is safe to take while breastfeeding. For more information, see the “Adakveo and breastfeeding” section above.

Note: For more information about the potential negative effects of Adakveo, see the “Adakveo side effects” section above.

Disclaimer: Medical News Today has made every effort to make certain that all information is factually correct, comprehensive, and up-to-date. However, this article should not be used as a substitute for the knowledge and expertise of a licensed healthcare professional. You should always consult your doctor or other healthcare professional before taking any medication. The drug information contained herein is subject to change and is not intended to cover all possible uses, directions, precautions, warnings, drug interactions, allergic reactions, or adverse effects. The absence of warnings or other information for a given drug does not indicate that the drug or drug combination is safe, effective, or appropriate for all patients or all specific uses.

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