Ninlaro: Uses, Mechanism, Side Effects, Dosage, and Cost

Published on by Dr. Saba Rassouli, PharmD

Ninlaro is an oral prescription drug used with other medications to treat a type of bone marrow cancer (multiple myeloma) in adults.

Get Financial Assistance

(877) 778-0318

Before Taking Ninlaro

Before receiving your first dose, inform your doctor if you:

  • Have a history of allergy to this or other medications or any product components
  • Take medicines to treat seizures, HIV infection, type II diabetes, or tuberculosis (TB)
  • Take any supplements, specifically St. John’s wort
  • Have or ever had liver or kidney disease

Ninlaro Introduction and Uses

Ninlaro is a brand-name product. It contains the active ingredient ixazomib, a member of a family of anticancer drugs known as proteasome inhibitors. 

Ninlaro is not chemotherapy. Chemotherapy works by killing rapidly multiplying cells in the body. Unfortunately, it kills some healthy cells as well. Thus, chemotherapy can cause very severe side effects.

On the other hand, Ninlaro is a type of targeted therapy for cancer treatment. Targeted therapies identify features specific to cancer cells. As a result, they usually have less severe side effects compared to standard chemotherapy. 

Ninlaro is used with lenalidomide (Revlimid) and dexamethasone to treat recurring or treatment-resistant multiple myeloma in adults who did not benefit from at least one prior therapy. 

It is NOT intended for use in newly diagnosed cases or those on maintenance treatment. 

Ninlaro Mechanism of Action

Ninlaro works by causing a buildup of unneeded proteins in cancer cells. When cancer cells cannot recycle damaged proteins, they die. 

Ninlaro Dosage

Ninlaro comes as 4 mg, 3 mg, and 2.3 mg oral capsules. 

The usual starting dose is 4 mg orally once weekly on Days 1, 8, and 15 of a 28-day treatment cycle. However, a doctor may reduce the starting dose to 3 mg in people with severe liver or kidney issues. 

Likewise, if you experience severe side effects when starting at 4 mg, your doctor will likely ask you to take 3 mg. If your problems persist, the amount is further reduced to 2.3 mg. Lastly, your doctor will stop treatment if you cannot tolerate the 2.3 mg dose. 

Your doctor may also give you medication with Ninlaro to prevent herpes zoster (chicken pox virus) reactivation. They may also order specific blood tests before each new cycle. 

Proper Use, Storage, and Disposal 

  • Before using this medication, read the instructions on the prescription label carefully. Take your doses exactly as directed. 
  • If you have any questions, talk to your doctor or pharmacist. 
  • Never stop taking this or any medicine without talking to your doctor.
  • Take the capsules on an empty stomach, at least 1 hour before or 2 hours after a meal. 
  • Swallow the entire capsule; avoid crushing, chewing, or breaking it. 
  • If you have to use Ninlaro and dexamethasone on the same day, do not take them simultaneously. Dexamethasone should be taken with food. 
  • If your skin comes in contact with the capsule contents, wash the exposed area with soap and water. 
  • If your eye is exposed, rinse it well with water. 
  • If you vomit after swallowing a capsule, do not take it again. Instead, take your next dose as scheduled. 
  • Store Ninlaro capsules at room temperature in their original packaging.
  • Do not store above 86°F. 
  • Do not freeze.
  • Dispose of unused or expired medicines following the local requirements. 

Speak to a Specialist

About Copay Assistance
Schedule a Consultation

Ninlaro Side Effects

Side effects can be mild or severe.

Common Side Effects

Man suffering from back pain as a side effect of taking Ninlaro
  • Diarrhea
  • Constipation
  • Nausea
  • Vomiting
  • Back pain
  • Swelling
  • Neutropenia (low white blood cell counts)
  • Upper respiratory tract infections or bronchitis (swelling of a tube-like structure that carries air to your lungs)

Talk to your doctor or pharmacist if any side effect worsens or persists. 

Severe Side Effects

Call your doctor immediately or seek emergency care if you have signs and symptoms of:

  • Low platelet counts, such as unexplained bleeding or bruising
  • Nerve problems (tingling, numbness, burning sensation in your hands or feet, or weakness in arms or legs)
  • Skin reactions, such as rashes, mouth sores, or severe peeling of the skin
  • Swelling, such as unusual weight gain or swollen arms, hands, legs, ankles, or feet
  • Blood clots in small blood vessels, such as fever, bruising, tiredness, nosebleeds, or decreased urination
  • Liver problems, such as yellow skin or eyes or pain in the upper right region of your stomach 
  • Vision problems, such as blurred vision
  • Chickenpox virus reactivation, such as skin rash and pain (shingles)

Use in Pregnancy and Lactation

Using Ninlaro during pregnancy can harm the baby. Please check with your provider if you are pregnant before starting treatment. Use effective contraception during treatment and for 90 days after the final dose. 

Men with partners who might become pregnant should use effective contraception during treatment and for 90 days after the final dose. 

Avoid breastfeeding during treatment and for 90 days after the final dose.

Missed Dose and Overdose

Take the missed dose only if the next scheduled dose is at least 72 hours away. Never take a double dose to compensate for the missed one. 

Overdosage can lead to death. Seek emergency medical care if you have severe nausea, vomiting, and diarrhea. 

Get Copay Assistance Now

(877) 778-0318

Ninlaro Cost

Cost can vary depending on your insurance plan, location, and pharmacy. Ninlaro is a specialty medication, which means it is available to fill through specialty pharmacies.  

Ask your insurance provider if your plan covers this medication or if you need prior authorization. 
Takeda Pharmaceuticals, which makes Ninlaro, offers a program called Takeda Oncology Here2Assist to help you access Ninlaro treatment. Contact us if you are interested in exploring financial assistance options for Ninlaro.

IVIG for Bone Marrow Transplant: Is IVIG Therapy Actually Effective?

Published on by Dr. Samantha Kaeberlein, PharmD

Though not recommended for routine use, IVIG for bone marrow transplant may be an option for specific individuals with severe immunodeficiency and recurrent infections. Learn about the benefits of IVIG for specific populations, common side effects, and more. 

Consult an IVIG Specialist

(877) 778-0318

A Quick Overview of Bone Marrow Transplant

A bone marrow transplant is an important treatment option for certain cancers. 

In this procedure, healthy cells (which will eventually become blood cells) are administered into the body. These cells — stem cells — replace the diseased or damaged bone marrow. Several factors can damage or weaken the bone marrow, such as immune disorders, radiation, or chemotherapy.  

Other names for this procedure are:

  • Hematopoietic stem cell transplant
  • Peripheral blood stem cell transplant 
  • Umbilical cord blood transplant

There are two sources of stem cells:

  1. Your own body (autologous transplantation) 
  2. A donor (allogenic transplantation)

Your hematologist may recommend a bone marrow transplant to:

  • Help your body produce enough healthy blood cells. 
  • Treat cancer along with high-dose chemotherapy or radiation. 
  • Increase the levels of stem cells, which can become immune cells that destroy cancer cells. 

A bone marrow transplant can also benefit people with noncancerous diseases like immune deficiency disorders and certain inherited blood disorders.

According to the Center for International Blood and Marrow Transplant Research (CIBMTR), over 8,000 individuals received stem cells from donors in the U.S. in 2016 [1].

What Are the Potential Benefits of IVIG for Bone Marrow Transplant? 

IVIG may benefit select patients undergoing a bone marrow transplant. Early studies suggest IVIG may help prevent infections, manage post-transplant autoimmune disorders, and reduce the risk of graft-versus-host disease (GVHD). 

GVHD happens when the donor stem cells attack and destroy healthy cells in the recipient (host).

While older studies found a promising role of IVIG for bone marrow transplant, newer studies and extensive reviews have failed to find any clear benefit of IVIG, especially with routine use for the general transplant patients. 

For example, a 2008 review of 30 trials including over 4,000 individuals undergoing bone marrow transplantation found no benefits of IVIG in improving survival or preventing infections [2].

IVIG for Bone Marrow Transplant: Potential Benefits for Specific Populations

Though convincing data are unavailable, IVIG may be considered in specific populations. 

IVIG for Cytomegalovirus (CMV) Pneumonia After a Bone Marrow Transplant

The recurrence of a CMV infection is a potentially life-threatening complication in which the dormant CMV becomes active following a transplant, often an allogenic transplant. Complications of CMV reactivation can include pneumonia, inflammation in the digestive tract, and vision problems.

According to a 2014 study, IVIG 0.5 g/kg weekly for the first 3 months after a bone marrow transplant from a donor may help reduce [3]:

  • Cytomegalovirus (CMV) load
  • The need for anti-CMV therapy

There’s no strong evidence to support the use of IVIG with standard antiviral therapy for CMV pneumonia after bone marrow transplantation. 

Nevertheless, some experts recommend combining IVIG with anti-CMV medication. In such cases, the dose of IVIG is 150 mg/kg every other day for 14 days. This is followed by IVIG 150 mg/kg every week for the next 4 weeks, administered with anti-CMV medication [4].

Get IVIG Copay Assistance

Learn More

IVIG in Patients With Hypogammaglobulinemia

IVIG for bone marrow transplant may also be an option in patients with extremely low antibody levels (severe hypogammaglobulinemia; IgG <4 g/L) [5]. Hypogammaglobulinemia affects one in four patients after a bone marrow transplant involving a donor [6]. 

IVIG for Bone Marrow Transplant: What Do Current Medical Guidelines Say?

Patient receiving IVIG for bone marrow transplant

Current guidelines don’t recommend the use of IVIG to prevent infections in all transplant patients. Nonetheless, IVIG therapy may be considered in specific populations or situations, after assessing the risks, benefits, and costs. 

For example, according to the Centers for Medicare and Medicaid Services (CMS), IVIG for bone marrow transplant may be an option in individuals 20 years or older during the first 100 days following a transplant. Notably, the CMS doesn’t recommend IVIG in individuals younger than 20 years and those undergoing autologous transplants [7].

IVIG for Bone Marrow Transplant: What Are the Side Effects?

IVIG products are generally well tolerated. Occasionally, severe side effects can occur, including:

  • Swelling of the lining of the brain 
  • Blood clots in the lungs
  • Fluid accumulation in the lungs 
  • Severe, potentially fatal allergic reactions 
  • Kidney failure or reduced kidney function
  • Severe infections 
  • Early breakdown of red blood cells

Common and mild side effects can include nausea, fever, chills, fatigue, joint aches, headache, and muscle pain.

Frequently Asked Questions

Why is IVIG given to transplant patients?

IVIG is more commonly administered after a bone marrow transplant.

If given before a transplant, IVIG is used in specific cases, such as when a patient is receiving a transplant from someone who is CMV positive, when the patient is CMV negative. CMV-specific IVIG in this instance helps prepare a patient for transplantation by reducing the antibody level in their body. As a result, the recipient’s immune system is less likely to reject the transplanted bone marrow. 

When given after a transplant, IVIG can help reduce the risk of infections. 

What are the advantages of IVIG treatment after a stem cell transplant?

IVIG after a bone marrow or stem cell transplant can help prevent infections and lower the risk of post-transplant autoimmune conditions. 

What is the success rate of IVIG infusions?

IVIG success rates can range from 60% to 80%, depending on the condition being treated, individual response, and the specific IVIG brand. 

How does IVIG work in rejection?

IVIG therapy reduces the immune system’s ability to identify and attack the transplanted bone marrow. 

Does IVIG therapy improve survival in bone marrow transplant patients?

According to a 2024 study, IVIG use was associated with a decrease in sepsis (an extreme and life-threatening immune response to an infection). However, the researchers failed to find any positive effect of IVIG on survival [8].

Ifosfamide: Usage, Dosage, Adverse Effects, and Precautionary Measures

Published on by Dr. Mark Alfonso, PharmD, BCMTMS

Ifosfamide injection (brand name: Ifex) is an FDA-approved drug used with other cancer medications to treat certain cancers of the testicles. Learn about its dosage, side effects, mechanism of action, and more. 

Get Financial Assistance

Get Started

Important Ifosfamide Warnings

This medication suppresses your immune system. In severe cases, it can lead to life-threatening infections or bleeding.

Call your healthcare provider immediately if you experience:

  • Fever
  • Chills 
  • Sore throat
  • Persistent cough and congestion
  • Unusual bleeding or bruising
  • Bloody or black stools
  • Bloody, coffee-ground-like vomit

Ifosfamide may cause brain damage, which can be severe or fatal. Call your healthcare provider immediately if you experience:

  • Confusion
  • Drowsiness
  • Blurred vision
  • Loss of balance
  • Problems with understanding or speaking
  • Hallucinations
  • Burning, numbness, or tingling in the hands or feet
  • Seizures
  • Coma

This medication may cause severe or potentially fatal kidney problems (during or several months after stopping treatment). Call your healthcare provider immediately if you experience:

  • Decreased urination
  • Swollen face, arms, hands, feet, ankles, or lower legs
  • Excessive tiredness or weakness

Before Taking Ifosfamide Injection

Before you receive the first dose of ifosfamide, inform your provider if you:

  • Are allergic to this medication or any component of it
  • Take a herbal product called St. John’s wort
  • Have or ever had heart, liver, or kidney disease
  • Have been treated with radiation therapy or other chemotherapy drugs
  • Are having surgery
  • Drink alcohol

Ifosfamide Introduction and Uses

Ifosfamide injection is an FDA-approved drug used with other cancer medications to treat certain cancers of the testicles. This medication may also be used with a detoxifying agent (mesna) to prevent inflammation of the urinary bladder lining. 

Ifosfamide belongs to a medicine class called alkylating agents. Other drugs in this class are cyclophosphamide, chlorambucil, and busulfan. 

Ifosfamide Mechanism of Action

Your liver enzymes convert ifosfamide into active substances. These substances work in two ways. First, they cause cancer cell damage; the damaged cells are removed by the body. Second, they permanently damage DNA and block protein formation in cancer cells. 

Get Copay Assistance Now

(877) 778-0318

Ifosfamide Dosage

Ifosfamide comes in single-dose vials in the following strengths:

  • 1 g/20 ml single-dose vial of ifosfamide
  • 3 g/60 ml single-dose vial of ifosfamide

A healthcare provider administers ifosfamide injection slowly (lasting at least 30 minutes) into your vein (intravenously; IV). 

The usual dose (in combination with other cancer medicines) is 1.2g/m2 IV daily on days 1 to 5, repeated every 21 days. In simple terms, one cycle of treatment lasts 21 days, but you will receive medication only during the first 5 days of the 21-day cycle. Your provider will determine how many cycles you need. 

Your provider will closely monitor you or change doses if you have liver or kidney problems. Treatment may be delayed if you experience severe side effects. 

If you are a female of childbearing age, your provider will order a pregnancy test to determine if you are pregnant. 

You should drink plenty of fluids during treatment to lower the risk of bladder toxicity. 

Ifosfamide Side Effects

Common Side Effects

Patient experiencing nausea, a side effect of ifosfaminde

Call your healthcare provider if the following symptoms worsen or don’t go away after taking an ifosfamide injection:

  • Nausea and vomiting
  • Confusion, vision problems, thinking problems
  • Numbness, tingling, burning pain
  • Infections
  • Urination problems
  • Hair loss

Allergic Reactions

Seek emergency medical care if you experience:

  • Hives
  • Difficult breathing
  • Swelling in your face or throat 

Severe Skin Reactions

Seek emergency medical care if you experience:

  • Fever
  • Sore throat
  • Burning eyes
  • Skin pain
  • Red or purple skin rash with blistering and peeling

Severe or Fatal Infections

Call your healthcare provider immediately if you experience:

  • Fever, chills, or flu symptoms
  • Mouth and throat ulcers
  • Skin sores, pale skin, cold hands and feet
  • Easy bruising, unusual bleeding
  • Increased heart rate
  • Shallow breathing
  • Lightheadedness 
  • Difficulty breathing

Severe Side Effects

Call your healthcare provider immediately if you experience:

  • Confusion
  • Bizarre thoughts or behavior
  • Hallucinations
  • Seizures
  • Little or no urination
  • Painful or difficult urination
  • Blood in your urine
  • Loss of bladder control
  • Uncontrollable muscle movement
  • Difficulty hearing or seeing
  • Ringing in the ears
  • Sudden chest pain
  • Wheezing and dry cough
  • Dark urine
  • Yellowish skin or eyes (jaundice)
  • Delayed wound healing

Speak to a Specialist

About Copay Assistance
Schedule a Consultation

Use During Pregnancy and Lactation

If you are a woman of childbearing age, your provider will order a test to check if you are pregnant. Ifosfamide can harm your unborn baby. Women should avoid getting pregnant, and men should not father a child during treatment with this medication. 

Women should avoid breastfeeding during treatment with this cancer medication. 

Effects on Fertility

Men

Men treated with this medication may have little or no sperm in their semen. Male children treated with ifosfamide before the onset of puberty may not develop characteristic features of male puberty, such as voice changes, body hair, and growth spurts. 

Fertility problems may be reversible in some cases. However, it might take years after stopping treatment. 

Ifosfamide does not seem to affect sex drive and function in most men. 

Women

Women treated with this medication may stop having periods, temporarily or permanently. Higher age at the time of starting treatment is associated with an increased risk of permanent cessation of monthly periods. 

Female children treated with ifosfamide before the onset of puberty may have problems with conception. Those with intact ovarian function may reach menopause earlier than usual. Menopause is when a woman does not have periods for 12 months. 

What To Do If You Miss a Dose

Call your provider immediately and follow instructions. 

What To Do If You Overdose

Call the poison control helpline at 1-800-222-1222. Signs of an overdose can include:

  • Blurred vision
  • Hallucinations
  • Fever, sore throat, chills, or other signs of infection
  • Unusual bleeding or bruising
  • Black and tarry stools
  • Bloody vomit
  • Decreased urination
  • Swollen face, throat, tongue, lips, eyes, hands, feet, ankles, or lower legs
  • Sores in the mouth and throat
  • Seizures
  • Confusion
  • Coma

Methylene blue (MB) is often used as an antidote to treat ifosfamide-induced brain damage. 

Things to Avoid During Treatment With Ifosfamide

  • Activities that require quick reactions and concentration, such as driving 
  • Drinking alcohol 
  • Grapefruit products 
  • Close contact with sick or infected people 
  • Receiving “live” vaccines, such as measles, mumps, rubella (MMR), rotavirus, typhoid, yellow fever, varicella (chickenpox), zoster (shingles), and nasal flu (influenza)

IVIG for Opsoclonus-Myoclonus Syndrome (OMS)

Published on by Dr. Saba Rassouli, PharmD

If your child suddenly begins having unusual, rapid eye movements or starts showing jerky, uncontrollable muscle movements, these could be signs of a condition called Opsoclonus-Myoclonus Syndrome (OMS). 

Get IVIG Copay Assistance

(877) 778-0318

OMS is a neurological disorder that mostly affects young children, but sometimes adults can also be affected. OMS can be a challenging condition to manage for both patients and their families. But it can be treated if caught early. 

Research shows that adding IVIG early to standard OMS treatment (i.e., corticosteroids) can double the response rate and improve neurologic recovery in children diagnosed with OMS. Read on to learn what OMS is, why it happens, and how IVIG can help you or your child get back to living a healthy life. 

What Is Opsoclonus-Myoclonus Syndrome (OMS)?

Opsoclonus-Myoclonus Syndrome is a rare and complex neurological disorder that can result from an immune system attack on the nervous system. Less than 1,000 people in the U.S. have OMS. 

It is slightly more common in girls than boys. The OMS symptoms often begin suddenly and progress quickly. In most cases, especially in children, symptoms start to appear between 6 months and 3 years of age. 

Common signs and symptoms of OMS include:

  • Rapid involuntary eye movements in all directions (opsoclonus)
  • Quick, involuntary jerking or twitching of muscles (myoclonus)
  • Impaired balance (ataxia)
  • Speech problems
  • Behavioral difficulties and sleep disturbances
  • Cognitive impairment (especially in children)

OMS is also known by other names, such as dancing eye-dancing feet syndrome, Kinsbourne syndrome, opsoclonic encephalopathy, or opsoclonus-myoclonus-ataxia syndrome (OMA). 

What Causes Opsoclonus-Myoclonus Syndrome (OMS)?

Although the exact cause of OMS is still being studied, scientists believe it is autoimmune in nature, which means the body’s immune system mistakenly attacks healthy nerve cells in the brain. 

Illustration of B cells and T cells

In about 40% to 80% of pediatric cases, OMS develops due to an underlying disease like neuroblastoma tumor (a cancer that develops in nerve tissues). When the body’s defense mechanism (immune system) initiates an immune response against the tumor cells, it also ends up affecting the nervous system. The activation of B and T cells releases autoantibodies and inflammatory molecules (cytokines) within the brain, which damage nerve cells and cause inflammation in the brain stem and cerebellum (a part of the brain that controls eye movement, speech, and muscle coordination). 

Furthermore, around 60% of OMS cases in adults are linked to small cell lung cancer, breast cancer, or ovarian cancer, while others may result from viral CNS infections or other unknown immune triggers.

Speak to a Specialist

About Copay Assistance
Get Started

How Effective Is IVIG for Patients With Opsoclonus-Myoclonus Syndrome?

Clinical trials to determine the efficacy of IVIG when used alone or in combination with other therapies are limited due to the rarity of this disease. However, some case reports have shown that IVIG therapy is effective when used alone. 

Conversely, some research studies show that IVIG tends to yield better results when combined with standard treatment like steroids (or ACTH) and often rituximab. 

  • IVIG as a Monotherapy: Clinical Evidence

IVIG monotherapy may be considered in selected situations, typically when adults with post-infectious OMS do not respond to steroids, and when other immunosuppressants are contraindicated. 

For instance, a case report described a 63-year-old woman who tested positive for West Nile virus and developed progressive opsoclonus-myoclonus. She was treated with a 2-day course of IVIG alone — without steroids or other immunosuppressants — and achieved complete neurological remission by the 8-week follow-up.

Similarly, in a reported case of SARS-CoV-2 infection, a patient developed opsoclonus-myoclonus syndrome characterized by psychosis, chaotic eye movements, and multifocal myoclonus. The patient was successfully treated with IVIG therapy.

  • IVIG in Combination With Standard Therapies: Clinical Evidence

In most pediatric OMS and many adult paraneoplastic cases, IVIG is used as part of a multimodal regimen to prevent relapses and improve outcomes. A 2025 cross-sectional study published in the Italian Journal of Pediatrics demonstrated that IVIG combined with dexamethasone and rituximab reduced relapses and permanent neurological sequelae in OMS children. 

Likewise, a systematic pediatric review demonstrated that OMS patients treated with IVIG, corticosteroids, and rituximab/cyclophosphamide had significantly better motor, language, and IQ outcomes, as well as lower relapse rates, compared to those treated with corticosteroids/ACTH alone. 

In short, IVIG alone can be effective, especially in adults with post-infectious OMS, but when combined with steroids, ACTH, or rituximab, it gives the best results in children. This multi-pronged approach attacks the autoimmune response from different angles.

IVIG for Opsoclonus-Myoclonus Syndrome: How It Works

The exact working mechanism of IVIG in OMS patients is unclear. However, research shows that IVIG dampens the immune response and reduces neuroinflammation in the following ways: 

  • It Blocks Harmful Autoantibodies

IVIG contains healthy antibodies that bind to and neutralize the harmful autoantibodies that attack the nervous system in OMS.

  • It Modulates Immune Cell Activity

IVIG affects immune cells, primarily B cells (which make autoantibodies) and T cells. It reduces their overactivity, leading to a decrease in the production of autoantibodies and cytokines. This helps restore the immune system to balance.

  • It Regulates Cytokines and Reduces Neuroinflammation

In OMS, the central nervous system shows evidence of immune activation, including elevated levels of inflammatory cytokines — molecules produced by activated B-cells, T-cells, and other immune cells. IVIG helps reduce neuroinflammation by decreasing the production of these pro-inflammatory cytokines. 

By reducing inflammation and stopping the immune attack on the brain, children and adults with OMS experience:

  • Improved eye movement 
  • Decreased muscle jerks
  • Return of balance and coordination
  • Recovery of speech and cognitive skills
  • Reduced risk of relapse, especially when IVIG is started early

Get Financial Assistance

(877) 778-0318

Are There Any Side Effects of IVIG?

Yes, IVIG can cause some side effects, though many are mild and temporary. Patients may experience some side effects like nausea, headache, chills, or fever during or after the IVIG infusion. However, with proper monitoring and infusion protocols, these side effects are manageable. 

In rare cases, aseptic meningitis, thrombosis, kidney problems (especially in those with pre-existing kidney disease), or allergic or anaphylactic reactions may also occur, requiring careful monitoring during the IVIG process. 

When and How Long Is IVIG Therapy Given to OMS Patients?

IVIG treatment usually begins soon after diagnosis to help calm the immune system and ease symptoms like rapid eye movements and muscle jerks. It is often used alongside steroids or other medications.

Children and adults with OMS may receive an initial dose of 1 – 2 g/kg given over two to five divided doses, followed by a maintenance dose of 0.4 – 1 g/kg administered every 4 to 6 weeks to keep symptoms under control. 

Treatment can last from several months to a couple of years, depending on the patient’s response and whether any relapses occur.

Talk to your healthcare provider if you’re considering IVIG for Opsoclonus-Myoclonus Syndrome as part of your OMS treatment plan. They can help you understand what to expect based on the severity of your symptoms and your overall health condition.

Enhertu: A Targeted Therapy for Advanced Cancers

Published on by Dr. Robert Hakim, PharmD

When diagnosed with cancer — especially if it has spread or returned after treatment — many patients urgently search for effective treatment options.

Fortunately, the continuous advancement in cancer treatment has brought new hope in the form of targeted medicine called Enhertu. 

Get Financial Assistance for Enhertu

(877) 778-0318

Enhertu (fam-trastuzumab deruxtecan-nxki) is the first and most effective cancer-targeted therapy for treating a variety of hard-to-treat cancers, particularly those that are HER2-positive, HER2-low, or HER2-ultralow. 

This article breaks down everything you need to know about Enhertu, including the types of cancers it treats, how it works, how to use it, typical side effects, and more. 

What Is Enhertu?

Enhertu (also known by its generic name, fam-trastuzumab deruxtecan-nxki) is an antibody-drug conjugate (ADC), which means it contains two active components that are linked together:

  1. A monoclonal antibody (trastuzumab) that targets a specific marker protein called human epidermal growth factor receptor 2 (HER2) present on the surface of some cancer cells.
  2. A chemotherapy medication (deruxtecan or DXd) that is attached to the antibody and delivered directly to the cancer cells.

Enhertu belongs to the HER2 inhibitors drug class and was first approved by the FDA in December 2019. 

What Type of Cancers Does Enhertu Treat?

The U.S. Food and Drug Administration (FDA) first approved Enhertu for treating adult patients with unresectable or metastatic HER2-positive breast cancer who have undergone two or more prior anti-HER2-based regimens. Since then, Enhertu has been approved globally for the treatment of several other indications, including:

  • HER2-positive breast cancer
  • HER2-low breast cancer
  • Hormone receptor (HR)-positive, HER2-low or HER2-ultralow breast cancer
  • HER2-positive advanced stomach or gastroesophageal junction cancer
  • HER2-mutant non-small cell lung cancer (NSCLC)
  • HER2-positive solid tumors with HER2 mutations

In short, Enhertu may not only help patients with traditional HER2-positive tumors but also those with HER2-low or HER2-mutated cancers that were previously difficult to treat.

How Effective is Enhertu for Patients?

Enhertu showed promising results during clinical trials. For instance, compared to chemotherapy, Enhertu reduced the risk of cancer progression or deaths by 36% in patients with HR-positive, HER2-low or HER2-ultralow metastatic breast cancer. On average, patients who took Enhertu have experienced longer progression-free survival (about 13 months), compared to those who received regular chemotherapy. 

Similarly, in studies of stomach cancer and lung cancer, Enhertu has also shown significant benefits, especially when previous treatments have failed. 

For instance, in patients with previously treated HER2-positive advanced gastric cancer, Enhertu reduced the risk of death by 41% compared to chemotherapy.

How It Works

Some breast cancer cells overexpress HER2 (short for Human Epidermal Growth Factor Receptor 2), which is a protein that behaves like an antenna on the surface of the cancer cells. When HER2 protein receives signals, it stimulates the cancer cell to grow and multiply rapidly. Patients with overexpression of the HER2 gene are considered HER2-positive. 

Some tumors have lower levels of HER2 and are classified as HER2-negative, HER2-low, or HER2-ultralow. 

  • Enhertu Working Mechanism

Enhertu contains two active parts that are linked together: a monoclonal antibody called trastuzumab and a chemotherapy drug called deruxtecan or DXd. 

Trastuzumab is designed to attach to the HER2 proteins on the cancer cells and stop these proteins from stimulating the growth of cancer cells. Trastuzumab also activates the immune cells that kill the cancer cells. 

Once the antibody attaches to the breast cancer cells, it moves inside the cells and delivers the potent chemotherapy medication (deruxtecan) that kills the cancer cells by blocking an enzyme (protein) called topoisomerase I, which is involved in making new cancer cells. 

Moreover, Enhertu also exhibits a “bystander effect,” which means it kills the neighboring cancer cells even if they don’t express the HER2 proteins. This makes the Enhertu an effective and more targeted treatment for patients with low or ultra-low HER2 breast cancers. 

Speak to a Specialist

About Copay Assistance
Get Started

How Is Enhertu Administered, and What Is Its Recommended Dosage?

Doctor holding pink ribbon for breast cancer awareness

Enhertu is available in the form of 100 mg lyophilized powder in a single-dose vial that is reconstituted with 5

ml of sterile water prior to administration. 

Enhertu is administered intravenously (into a vein) once every 3 weeks in a 21-day treatment cycle. Your first infusion will take over 90 minutes. If you don’t experience any adverse reactions to your first treatment, subsequent infusions will typically take about 30 minutes.

  • Recommended Dosage

The recommended dosage of Enhertu for adults with unresectable or metastatic HER2-positive, HR-positive, HER2-low, and HER2-ultralow cancer is 5.4 mg/kg. Similarly, patients with HER2-positive solid tumors with HER2 mutations also receive the same dose (5.4mg/kg). 

On the other hand, patients with unresectable or metastatic HER2-Mutant NSCLC receive a dose of 6.4 mg/kg once every 3 weeks as long as the treatment remains effective. 

Under What Conditions Is Enhertu Taken?

Enhertu may be considered under the following conditions:

  • You have HER2-positive or HER2-low breast cancer. 
  • Your cancer has spread to other body parts (metastatic).
  • Your cancer is not removable by surgery.
  • Your cancer has returned during or within six months of prior treatments, like HER2-targeted treatments or endocrine therapy.
  • You have advanced stomach, lung, or other HER2-positive solid tumors.
  • Previous treatments are no longer working, and your cancer still tests positive for HER2 expression or mutation.
  • You have no other satisfactory treatment options.

What are the Possible Side Effects?

The most common side effects reported during the clinical trials are as follows: 

  • Nausea
  • Vomiting
  • Diarrhea
  • Constipation
  • Low appetite
  • Tiredness
  • Low white blood cell counts (leukopenia)
  • Low red blood cell counts (anemia)
  • Low platelet counts (thrombocytopenia)
  • High liver function tests
  • Hair loss (alopecia)
  • Low levels of blood potassium
  • Muscle or bone pain

Though rare, Enhertu may also cause some serious side effects like:

  • Interstitial lung disease (a disorder causing scarring in the lungs)
  • Pneumonitis (inflammation of the lungs)
  • Left Ventricular Dysfunction
  • Neutropenia (Low neutrophil counts)

If you experience any of the above symptoms, consult your healthcare provider immediately. 

Get Copay Assistance Now

(877) 778-0318

What Should I Tell My Healthcare Provider Before Taking Enhertu?

Before you take Enhrertu, tell your healthcare provider if you:

  • Have lung or breathing problems
  • Have signs or symptoms of an infection
  • Have or have had any heart problems
  • Are pregnant because Enhertu can cause embryo-fetal toxicity or severe birth defects
  • Are breastfeeding or plan to breastfeed — It is not known whether Enhertu passes into breast milk. You should avoid breastfeeding during treatment and for at least 7 months after your last dose.
  • Taking any prescription or non-prescription medications, such as over-the-counter medications, supplements, or herbal products. 

Note: ENHERTU may cause fertility problems in males, which may affect the ability to father children.

Is Enhertu a Chemotherapy or Immunotherapy?

Enhertu is not like traditional chemotherapy (which kills both healthy and cancerous cells) or immunotherapy (which helps your immune system fight cancer). Instead, it is a combination of chemotherapy and targeted therapy. 

The antibody part of Enhertu finds and attaches to cancer cells that have the HER2 protein, while the chemotherapy drug (DXd) is then delivered into the cell to kill it from the inside. 

How Much Does It Cost?

Enhertu is typically an expensive medication, and its cost can vary depending on the dosage and your insurance coverage. 

In the U.S., a single 100 mg vial can cost between $2,440 and $3,022.76. The total cost of Enhertu treatment may range from approximately $9,574 to $165,949 per year, depending on the dosage and duration of treatment. To reduce your out-of-pocket expenses, you can enroll in the ENHERTU4U program, which offers copay assistance and patient support. Contact us if you are interested in getting copay assistance for Enhertu.

IVIG for Post-Transfusion Purpura: A Comprehensive Guide to Its Use and Doses

Published on by Dr. Christine Leduc, PharmD

IVIG (intravenous immunoglobulin) is the mainstay of treatment for post-transfusion purpura. It helps increase platelet count and stop bleeding. Learn about its mechanism of action, doses, and effectiveness. 

Get Financial Assistance

(877) 778-0318

Highlights

  • Post-transfusion purpura (PTP) is a rare but potentially lethal transfusion reaction. 
  • PTP is usually self-limiting and resolves within 14 days.
  • Major bleeding occurs in about 33% of patients. 
  • High-dose IVIG is the primary treatment with high response rates. 

A Quick Overview of Post-Transfusion Purpura

Post-transfusion purpura (PTP) is a rare yet potentially fatal transfusion reaction. Researchers have yet to determine the exact incidence of this condition. However, studies estimate there’s about 1 new case of PTP per 24,000 to 100,000 transfusions [1]. 

The characteristic feature of PTP is a sudden, drastic drop in platelet count, which usually occurs within 14 days of blood or platelet transfusion. A low platelet count after a transfusion is typically self-limiting, often resolving within 2 weeks. 

However, in severe cases, death may occur due to bleeding inside the skull or brain. Approximately 1 in 3 patients has major bleeding. 

Patient receiving a blood transfusion in a hospital

Symptoms can include:

  • Post-transfusion fever
  • Unusual purpuric rashes (red or purple spots beneath the skin)
  • Bruising 
  • Nosebleeds
  • Gum bleeding 
  • Bleeding in the digestive and urinary tracts

What Causes Post-Transfusion Purpura?

PTP can occur when the antibodies (proteins) directed against transfused platelets attack and destroy the receiver’s platelets. But why this happens is unclear. 

The following factors may increase the risk of PTP [2,3]:

  • Being female: The female-to-male ratio for PTP is 5:1. 
  • Having a history of pregnancy or blood transfusion.
  • Being elderly, with a history of irregular heart rhythms, bleeding disorders, blood or bone marrow cancer, and organ transplants.
  • Having a higher volume of transfused blood. 
  • Having anemia (low number of red blood cells) due to sudden blood loss, such as from a trauma.

Post-Transfusion Treatment

IVIG is the primary treatment for post-transfusion purpura. Plasma exchange (plasmapheresis) and corticosteroids may be considered in individuals who don’t respond to IVIG. Steroids may also be used with IVIG. 

Most experts don’t recommend platelet transfusions, as these can make the symptoms worse. 

Further Reading: IVIG vs. Plasmapheresis

Speak to a Specialist

About Copay Assistance
Schedule a Consultation

IVIG for Post-Transfusion Purpura: What Do Recent Studies Suggest?

Most knowledge about IVIG for post-transfusion purpura comes from case reports and small studies. Nevertheless, IVIG is often considered the treatment of choice for PTP. 

IVIG is highly effective, with a reported response rate of 90% and a favorable response within 48 hours after starting IVIG therapy. 

Many studies and case reports have found that high-dose IVIG is effective in:

  • Rapidly increasing platelet counts
  • Stopping bleeding  

For example, according to a 2025 case report published in the Canadian Medical Association Journal, researchers successfully treated a 36-year-old woman suspected of having PTP with IVIG and a steroid. 

The patient received 1 g/kg/day of IVIG for 4 days, from days 10 to 13. She also received 40 mg/day of dexamethasone from days 11 to 14. Her platelet count increased around 72 hours after starting IVIG therapy [4].

IVIG is the mainstay of treatment for PTP, according to the authors of a 2019 review. In this review, there were two dosage regimens [5]:

  1. 400 mg to 500 mg/kg/day of IVIG for 1 to 10 days.
  2. 1 to 2 g/kg/day of IVIG for 2 to 5 days. 

According to a 2024 case report published in the American Journal of Case Reports, a 69-year-old man with PTP was successfully treated with an IVIG dose of 1 g/kg per day for 2 days and a steroid (dexamethasone) dose of 40 mg/day for 4 days [6].

IVIG for Post-Transfusion Purpura: How Does IVIG Therapy Help?

The exact mechanism by which IVIG increases platelet count in post-transfusion purpura is unclear. 

Available theories are based on the role of IVIG in immune thrombocytopenic purpura. Health experts believe IVIG may help restore platelet count by: 

  • Blocking the removal of antibody-coated platelets.
  • Neutralizing antibodies that attack the receiver’s platelets.
  • Preventing immune cells from destroying the receiver’s platelets. 
  • Accelerating antibody breakdown. 

IVIG for Post-Transfusion Purpura: Frequently Asked Questions

1. How is post-transfusion purpura treated?

High-dose IVIG is the first-line treatment for post-transfusion purpura. If symptoms don’t improve, your provider may recommend plasma exchange or corticosteroids. Platelet transfusions are rarely used and should be reserved for patients with severe bleeding. 

2. How does IVIG help with thrombocytopenia?

The exact mechanism isn’t fully understood. It is believed that IVIG helps restore platelet count by preventing the body from removing antibody-coated platelets and neutralizing antibodies. 

3. How quickly does IVIG increase platelets?

IVIG increases platelet count typically within 48 to 72 hours after starting treatment. The time it takes to increase platelet count depends on the patient’s platelet levels at the time of starting IVIG, the severity of the condition, and the doses of IVIG administered.

Understanding Ilumya: Uses, Side Effects, and Pricing

Published on by Dr. Mark Alfonso, PharmD, BCMTMS

Ilumya injection is FDA-approved to treat adults with moderate-to-severe plaque psoriasis. If you or anyone you love is living with plaque psoriasis, this article will cover everything you need to know about this medication, including its uses, mechanism, dosage, side effects, and cost. 

Get Financial Assistance

(877) 778-0318

Important Ilumya Warnings

Increased Risk of Infection

This medication may suppress your immune system, increasing the risk of infections. Before starting treatment, your healthcare provider will order tests to see if you have an infection or tuberculosis (TB). 

If you have active TB or have had it in the past, your healthcare provider may treat you for TB before starting treatment. 

Call your healthcare provider right away if you have the following signs of infection:

  • Fever, sweats, or chills
  • Cough
  • Shortness of breath
  • Blood in your mucus
  • Muscle aches
  • Warm, red, or painful skin or sores that aren’t due to psoriasis
  • Weight loss
  • Diarrhea 
  • Stomach pain
  • Burning during urination 
  • Increased urine output

Before Taking Ilumya

Before you receive the first dose, inform your healthcare provider if you:

  • Have a history of allergy to this product or any product components. 
  • Have long-term (chronic) or recurrent infection.
  • Have TB, or someone close to you has been diagnosed with TB.
  • Have recently been vaccinated or are scheduled to get vaccinated. You shouldn’t receive live vaccines during the treatment period. Live vaccine examples include MMR (measles, mumps, rubella), chickenpox, and nasal spray flu vaccines. 

Ilumya Introduction and Uses

Ilumya is a brand-name prescription medication. Generic versions aren’t available. 

The active ingredient in this product is tildrakizumab-asmn, which is in a category of targeted drug therapies called monoclonal antibodies. 

Monoclonal antibodies are lab-created immune system proteins that can suppress, mimic, or enhance your immune response. 

In March 2018, the US FDA approved this product to treat moderate-to-severe plaque psoriasis. Adults who may benefit from systemic therapy (oral or injectable medicine) or light therapy are considered for Ilumya therapy. 

Plaque psoriasis is an autoimmune disorder characterized by thick, scaly patches on the elbows, back, knees, and scalp. In severe cases, the patches may affect the entire body. 

Get Copay Assistance Now

Get Started

Ilumya Mechanism of Action

Ilumya directly blocks the action of a protein, IL-23, which plays a crucial role in inflammatory and immune responses. By blocking IL-23, Ilumya:

  • Helps balance an overactive immune response. 
  • Decreases the buildup of inflammatory cells in the skin. 
  • Stops the formation of scaly patches. 

As a result, the skin becomes clearer, less inflamed, and less itchy. Clinical studies show that nearly 60% of individuals who use Ilumya have minimal or no symptoms after 3 months.

Ilumya Dosage

This medication comes as a clear to slightly yellow liquid to be injected under the skin (subcutaneous/SC). 

The following strength is available in the US: 100 mg/ml solution in a single-dose prefilled syringe.

Your healthcare provider or a nurse administers Ilumya injection into the stomach area, thigh, or upper arm. The first dose is 100 mg SC. The second dose is 100 mg SC after 4 weeks. Then, 100 mg SC is administered every 12 weeks. 

Note: You shouldn’t self-administer the Ilumya injection. 

Ilumya Side Effects

Common Side Effects

Illumya patient suffering from plaque psoriasis

Call your healthcare provider if the following symptoms worsen or don’t go away. 

  • Upper respiratory infections
  • Injection site reactions (pain, swelling, or bleeding at the injection site)
  • Diarrhea

Allergic Reactions

Seek emergency medical care if you experience:

  • A feeling like you’re going to pass out
  • Swelling of your face, eyelids, lips, mouth, tongue, or throat
  • A skin rash
  • Trouble breathing 
  • Throat or chest tightness

Use During Pregnancy and Lactation 

Due to limited data regarding Ilumya use in pregnant women, it’s impossible to say if the drug will harm the unborn baby. Also, it’s unknown if this medication makes it harder for women to get pregnant. 

Ilumya may pass from the mother to the unborn baby. However, the impact on the mother or unborn baby is unknown. 

Animal reproduction studies haven’t reported a higher risk of miscarriage or birth defects with use during pregnancy [1].

No data are available about use during breastfeeding. Because tildrakizumab is a large protein, it’s unlikely to pass into breast milk. However, the medication was detected in breast milk in animal studies. 

Missed Dose

If you miss an appointment with your healthcare provider to receive your dose of Illumya, schedule another appointment as soon as possible.

Overdose

In overdose cases, seek emergency medical attention or call the poison help line at 1-800-222-1222.

Speak to a Specialist

About Copay Assistance
(877) 778-0318

Ilumya Cost

1 unit (100 mg/ml SC solution) costs around $19,000. 

However, the cost can vary depending on your insurance plan, location, and pharmacy. Contact your insurance provider to find out if your plan covers this medication or if you need prior authorization.

Sun Pharmaceutical Industries, Inc., the manufacturer of Ilumya, provides financial support if you’re eligible. 

Those with commercial insurance may enroll in the ILUMYA SUPPORT LIGHTING THE WAY® program and pay $0 for each dose with the copay card.

For those without commercial insurance, ILUMYA SUPPORT LIGHTING THE WAY® may help by connecting them to a dedicated support specialist.

The cost of a prescription may be $0 for those who have Medicare Part B with supplemental insurance.

Contact us if you are interested in exploring financial assistance options for Ilumya. 

Ilumya vs. Skyrizi

While both Ilumya and Skyrizi are used to treat moderate-to-severe plaque psoriasis, they differ in several ways. Below are some of the differences:

IlumyaSkyrizi
FDA approval date20182019
Active drugtildrakizumabrisankizumab
Who can administer?Only a healthcare professionalSelf-administration after training
Available strength100 mg75 mg 
Approved usesModerate-to-severe plaque psoriasis in adultsModerate-to-severe plaque psoriasis, active psoriatic arthritis, Crohn’s disease, and ulcerative colitis in adults

IVIG for Toxic Epidermal Necrolysis (TEN)

Published on by Dr. Samantha Kaeberlein, PharmD

Toxic epidermal necrolysis (TEN) is a rare but serious skin condition that can be life-threatening. In the United States, it affects about 1.9 adults per million people each year. This condition typically develops as a severe allergic reaction to certain medications, causing your skin to develop rashes, sores, and blisters, then peel, similar to what happens with severe burns. 

Can IVIG help?

Free IVIG Treatment Info
Learn More

Intravenous immunoglobulin (IVIG) is considered a potential treatment for toxic epidermal necrolysis because of its ability to suppress type IV hypersensitivity reactions and inhibit cell death. In fact, a recent case report demonstrated that IVIG therapy combined with high-dose steroid therapy led to the complete resolution of pembrolizumab-induced TEN in a patient. 

In this article, we will explain what TEN is, how IVIG works, and how it helps patients recover from this potentially fatal skin condition.

Toxic Epidermal Necrolysis (TEN): Overview

Toxic epidermal necrolysis, also known as Lyell’s syndrome, is a rare and most severe form of Stevens-Johnson Syndrome (SJS). In people with SJS, TEN is diagnosed when more than 30% of the skin surface is affected and there is extensive damage to the body’s moist linings, called mucous membranes. 

Just like SJS, the condition typically starts with flu-like symptoms that appear 1 to 3 weeks after the start of a medication. Then, it is followed by a painful red or purplish rash on the skin that quickly spreads to the face, neck, trunk, and the rest of the body in an irregular pattern. Blisters form, and large portions of skin may detach, exposing raw areas underneath called erosions, similar to a severe hot-water burn.

Widespread damage to the skin and mucous membranes, such as the mouth, nose, throat, and genitals, can cause severe fluid loss and infection. In the most severe cases, patients can experience shock, sepsis, multiple organ failure, and death.

What Causes Toxic Epidermal Necrolysis (TEN)?

TEN is triggered by a particular medication (e.g., antibiotics, anti-seizure medications, anti-gout medications, NSAIDs). When your body’s immune system overreacts to certain medications, it mistakenly starts attacking the skin and mucous membranes. This reaction is a delayed type of allergy (type IV hypersensitivity reaction) involving special immune cells called cytotoxic T cells and natural killer (NK) cells.

When these cells are activated, they release substances (granulysin, perforin, and granzyme) that damage the skin’s outer layer (epidermis), causing the skin to die and peel off. One crucial part of this damage comes from a signal called Fas ligand, which tells skin cells (keratinocytes) to self-destruct. 

Efficacy of IVIG Therapy in Toxic Epidermal Necrolysis: Clinical Evidence

Studies on the effectiveness of IVIG in TEN are limited due to the rarity of the disease. However, multiple case reports; open-label, prospective studies; and retrospective case series have demonstrated positive outcomes of IVIG therapy in TEN patients if initiated early. 

For instance, a case report of a 5-year-old with lamotrigine-induced TEN when treated with IVIG along with pediatric burn unit care resulted in excellent outcomes, with faster healing and lower risk of mortality than either treatment alone. 

Similarly, another case report of an 83-year-old man with TEN who received IVIG (400 mg/kg/day for 5 days) following inadequate response to high-dose steroids showed improved skin symptoms. The patient did, however, develop thrombocytopenia (low platelet count), which resolved after the IVIG treatment. 

While IVIG shows promising results, it requires careful monitoring for potential adverse effects.

Consult an IVIG Specialist

(877) 778-0318

How IVIG Works in Toxic Epidermal Necrolysis

IVIG nurse with patient

The mechanism of action of IVIG is complex and not completely understood. However, research has found that IVIG modulates the immune response by inhibiting or blocking specific immune-mediated pathways. 

1. Blocking Fas-FasL Mediated Apoptosis

In patients with TEN, the Fas-Fas Ligand (FasL) pathway is a central mechanism that causes massive skin detachment and blistering. Keratinocytes (skin cells) have Fas receptors on their surface, which can bind to Fas ligands (FasL) released by immune cells. The Fas-FasL binding triggers apoptosis (programmed cell death) of skin cells. 

The anti-Fas immunoglobulin present in IVIG blocks the Fas receptor by binding to it, thereby preventing FasL from attaching to its receptors on skin cells. This interruption in the Fas-FasL interaction halts apoptosis, slowing or stopping further skin cell death.

2. Reducing Cytotoxic T Cell and NK Cell Activity

IVIG modulates the immune response by downregulating the function of overactive cytotoxic T cells and NK cells. This reduces the release of other harmful molecules (like granulysin and granzyme B) that contribute to tissue damage.

3. Anti-Inflammatory Effects

IVIG produces anti-inflammatory effects that help to neutralize the effects of pro-inflammatory cytokines such as TNF-α, IL-2, and IFN-γ, which are elevated in TEN and contribute to inflammation and tissue injury. 

4. Modulation of Fc Receptors

The Fc receptor plays a role in immune activation. IVIG antibodies bind to Fc receptors present on immune-activating cells, such as macrophages and dendritic cells, thereby interfering with cell activation and antigen presentation. This prevents further immune activation and recruitment of additional immune cells to the skin.

5. It  Provides Passive Immunity

Although not its primary purpose in TEN, IVIG also boosts the patient’s overall antibody levels and offers protection against secondary infections in TEN due to skin loss.

When and How Long Is IVIG Therapy Given to TEN Patients?

IVIG is initiated as early as possible, preferably within 24 hours of diagnosis. The duration of IVIG therapy in patients may vary, depending on the severity of the disease. Typically, in toxic epidermal necrolysis cases, patients receive IVIG infusions daily for 3 to 5 days.

You may receive an initial dose of IVIG, up to 3g/kg, divided over 3 days (1g/kg/day) or up to 2g/kg delivered as a single dose. 

Are There Any Side Effects of IVIG? 

A patient with toxic epidermal necrolysis may experience some side effects like nausea, headache, chills, or fever while receiving the infusion. In rare cases, thrombocytopenia (a low platelet count) or aseptic meningitis may develop, requiring careful monitoring. 

Get IVIG Prior Authorization

Get Started

How Does Toxic Epidermal Necrolysis Differ From SJS?

Although both SJS and TEN involve skin peeling and mucous membrane damage, they differ in the extent of body involvement. 

If less than 10% of the body surface is involved, it’s diagnosed as SJS. If more than 30% is involved, it’s diagnosed as TEN. Cases involving 10 – 30% of the body fall into the overlap category (SJS/TEN).

Odomzo: A Targeted Oral Medication for Basal Cell Carcinoma

Published on by Dr. Saba Rassouli, PharmD

Odomzo (sonidegib) is a medication  that treats basal cell carcinoma (BCC), which is a type of skin cancer that develops in the uppermost layer of the skin. It is a common form of skin cancer that affects around 3.6 million Americans each year. 

Get Financial Assistance

Get Started

Although many cases are easily treated with surgery or radiation, some become more complex — either returning after treatment or growing in ways that make standard therapies less effective. For these challenging cases, doctors prescribe more advanced treatments, such as Odomzo (sonidegib). 

Odomzo is a targeted oral medication designed to treat locally advanced BCC (laBBC) when surgery or radiation is no longer an option. This article explores how Odomzo works, who can use it, and what patients should know about this medication.

Odomzo: Usage or Indication

Odomzo is approved by the FDA and is only indicated for the treatment of locally advanced BCC in adult patients. This drug is also prescribed to patients whose cancer has returned after surgery, or those who cannot have surgery or radiation therapy to remove all of the cancer. 

Odomzo: Mode of Action

Basal cell carcinoma (BCC) occurs as a result of the uncontrolled growth of basal cells due to the overactivity of the Hedgehog signaling pathway. This molecular pathway normally controls cell growth and repair in the skin. 

Sonidegib, the active substance in Odomzo, is a Hedgehog pathway inhibitor that binds to a smoothened (SMO) protein. This protein mainly controls the Hedgehog signaling pathway, and by blocking this protein, sonidegib shuts down the abnormal signaling that drives the growth of cancer cells. 

By working this way, Odomzo may help to shrink the tumor. 

Dosage and Administration

Odomzo is available as 200 mg capsules. The recommended dose for adults with BCC is one capsule taken orally once a day. Take Odomzo capsules on an empty stomach, either at least 1 hour before a meal or 2 hours after eating. 

Common Side Effects

The most common side effects reported by ≥10% of patients include:

  • Muscle spasms 
  • Hair loss (alopecia)
  • Taste disturbance (dysgeusia)
  • Tiredness
  • Nausea and vomiting
  • Muscle aches and pain (myalgia)
  • Pain in a joint (arthralgia)
  • Abdominal pain
  • Headache
  • Diarrhea
  • Weight loss
  • Loss of appetite
  • Itching (pruritus)
  • Little or no urinating
  • Dark colored urine
  • Fatigue and pain

In fertile women, Odomzo may cause an absence of menstrual periods (amenorrhea). It is not known if the amenorrhea is permanent. 

Contact your healthcare provider if you experience any of the above symptoms after taking Odomzo. Your doctor may prescribe medications to help manage certain side effects, such as nausea or vomiting.

In some cases, if the muscle cramps or weakness is severe, your healthcare provider may change your dose or stop your treatment temporarily or permanently. 

Get Copay Assistance Now

(877) 778-0318

Contraindications (Who Should Not Take Odomzo)

Patient on Odomzo meeting with physician and discussing contraindications

Odomzo can cause severe birth defects or fetal death. It is strictly contraindicated in pregnant women. Women of childbearing potential must have a negative pregnancy test before starting the medication and they must use effective contraception during treatment and for 20 months after the last dose.

Men must use condoms during treatment and for 8 months after the last dose, as the drug can be present in semen.

Patients with a known allergy to sonidegib or any of its components should not take Odomzo.

Before You Start Odomzo: What To Tell Your Doctor

Always consult your healthcare provider before taking Odomzo if you:

  • Have muscle pain or spasms, or have a history of a muscle disorder called rhabdomyolysis or myopathy
  • Have any other medical conditions, like kidney disease
  • Are pregnant or plan to become pregnant (Odomzo can cause embryo-fetal death or severe birth defects)
  • Are breastfeeding or plan to breastfeed (Since it is not known whether Odomzo passes into breast milk, breastfeeding should be avoided during treatment and for up to 20 months after the last dose).
  • Taking any prescription or non-prescription medicines, such as over-the-counter medications, supplements, or herbal products

During Treatment: What To Avoid While Taking Odomzo

While taking Odomzo, it is important to avoid:

Blood or Sperm Donations

It is suggested not to donate blood during treatment and for at least 20 months after your last dose. Also, men should avoid donating semen during treatment and for 8 months afterwards.

Certain Medications

Avoid strong and moderate CYP3A inhibitors or inducers (e.g., ketoconazole, rifampin, phenytoin), as they may affect how Odomzo works or increase side effects. Always inform your doctor about all medications and supplements you are taking.

Grapefruit and Grapefruit Juice

Avoid consuming grapefruit products as they may interfere with how Odomzo is metabolized in the body.

Strenuous Physical Activity

Avoid exercise, or exercise with caution, as Odomzo can cause muscle pain or weakness. Notify your doctor if you feel muscle cramps, aches, or unusual fatigue.

Speak to a Specialist

About Copay Assistance
Learn More

Cost

Odomzo (200 mg oral capsule) is an expensive medication, and it will cost approximately $15,262 for a supply of 30 capsules without an insurance plan. However, with insurance plans, manufacturer copay programs, or patient assistance foundations, you can get this medication at a low cost or even for free if you qualify. Contact us if you are interested in exploring financial assistance options for Odomzo.

Frequently Asked Questions (FAQs)

Is Odomzo chemotherapy?

Odomzo is not chemotherapy. Rather, it is an oral medicine that was approved by the FDA in 2015 for the treatment of locally advanced BCC in adult patients whose cancer has recurred after standard treatment. 

Does Odomzo work differently from chemotherapy?

Odomzo works differently from chemotherapy by blocking the molecular pathway to stop the growth of cancer cells. It has the ability to penetrate deeper into the tissue and target the tumor where it resides.

What if I forget to take a pill?

If you miss a dose, skip the missed dose. Take your next dose as scheduled. Do not take two doses to make up for a missed dose.

What are the most serious side effects?

Odomzo may cause stillbirth or severe birth defects. It commonly causes muscle pain or spasms, which can sometimes signal serious muscle problems, including rare cases of rhabdomyolysis that may lead to kidney damage.

What if I can’t tolerate the side effects?

Discuss any side effects you experience with your doctor. Let them know right away if the side effects are bothersome or do not  go away.

IVIG for Toxic Shock Syndrome

Published on by Dr. Robert Hakim, PharmD

Toxic shock syndrome (TSS) is a rare condition caused by certain types of bacteria. It can be life-threatening. Even with antibiotic therapy and supportive care, TSS can lead to rapid deterioration and multiple organ failure. If diagnosis and treatment are delayed, the death rate due to Streptococcal TSS can exceed 50% [1]. However, in recent years, intravenous immunoglobulin (IVIG) has become a new hope in the fight against TSS. Several clinical studies have shown that IVIG can lower the death rate in TSS patients [1]. In this article, we’ll discuss everything you need to know about IVIG for toxic shock syndrome (TSS).

Get IVIG Copay Assistance

(877) 778-0318

Toxic Shock Syndrome (TSS): Overview

Toxic shock syndrome (TSS) is caused by certain strains of bacteria that produce toxins (poisons). In most cases, two kinds of bacteria, Staphylococcus aureus and Streptococcus pyogenes, are the culprits. 

The toxins released by these bacteria act as “superantigens.” They cause your immune system to overreact and release a large amount of inflammatory chemicals called cytokines. This overreaction is called a cytokine storm. This can lead to serious damage to multiple organs in your body and can even cause death. This extreme immune reaction, not the bacteria itself, is what makes TSS so dangerous and life-threatening.

Woman with fever as a symptom of TSS

The signs and symptoms of TSS develop very quickly. They include [3]:

  • High fever
  • Low blood pressure
  • Diarrhea and vomiting
  • Confusion or altered mental state
  • Skin rash that feels rough
  • Difficulty breathing or breathing very fast
  • Severe pain all over your body
  • Reddening of the tongue, palms, soles of the feet, and the whites of the eyes 

In the case of TSS, you need early diagnosis and urgent treatment. The standard treatment of TSS involves:

  • Antibiotics to kill the bacteria responsible for TSS. 
  • IV Fluids to prevent dehydration and maintain blood pressure. 
  • Surgery to remove infected or dead tissue in the body. In rare cases, your healthcare provider may need to amputate the affected area to save your life.
  • Ventilation to help with breathing difficulties by administering oxygen through a mask. In more severe cases, patients might need a ventilator.
  • Dialysis if your kidney stops functioning. 

Your healthcare provider may also consider intravenous immunoglobulin (IVIG) with any of these treatments.

How Does IVIG Help?

Intravenous immunoglobulin (IVIG) is a product that contains human antibodies extracted from the plasma of thousands of healthy blood donors. It is widely used to balance and regulate the immune system in various inflammatory, autoimmune, and infectious diseases.

In the case of toxic shock syndrome (TSS), toxins released by certain bacteria cause an extreme immune reaction that damages your body’s organs. IVIG can help you with your condition by:

Neutralizing Bacterial Toxins: In TSS, the main cause of the dangerous immune overreaction is toxins (poison) released by certain bacteria. IVIG contains antibodies that can bind to and neutralize these harmful bacterial toxins [1][2].

Reducing Inflammation: IVIG can reduce the inflammation. It helps suppress the excessive immune activation that causes tissue damage and organ failure.

Helping the Body Fight Infection: IVIG may also strengthen your body’s defense system by delivering ready-made antibodies that can help your immune system fight off the bacteria that is causing the infection.

Overall, IVIG helps to calm the harmful immune reaction without weakening your body’s ability to fight the infection. This makes it a valuable addition to the treatment plan for patients with severe cases of TSS.

Consult an IVIG Specialist

Book Consultation

Who Should Take IVIG for Toxic Shock Syndrome (TSS)?

IVIG isn’t a required treatment for every TSS patient. It is only used for severe cases where traditional treatments like antibiotics and supportive care are not working well enough.

According to the UK’s National Health Service (NHS), you can take IVIG for TSS if your condition doesn’t improve within 6 hours of aggressive treatment [4]. Similarly, the Australian National Blood Authority suggests that doctors can consider IVIG early in both streptococcal TSS and staphylococcal TSS when there’s no rapid improvement with standard care [5].

Ultimately, your healthcare provider will make the final decision based on the severity, timing, and overall clinical picture of your condition. In the case of TSS, quick decision-making and fast treatment are critical for the best outcome.

Effectiveness of IVIG in Toxic Shock Syndrome (TSS) Found in Clinical Trials

IVIG is not considered a first-line treatment for TSS. However, in clinical studies, it has shown promise as an add-on therapy, especially in severe or life-threatening cases that do not respond well to traditional treatment (antibiotics and supportive care) alone.

For example, a 2003 study showed that patients with streptococcal TSS who received IVIG had a significantly lower death rate (10%) compared to those who did not (36%) [6]. Similarly, a 2014 study showed that IVIG improved survival in patients with streptococcal toxic shock syndrome (STSS) [7]. Likewise, a 2018 study showed that IVIG administration in patients with streptococcal toxic shock syndrome was associated with a reduction in death from 33.7% to 15.7% [9].

A recent case study reported the story of a 63-year-old female with streptococcal TSS who didn’t respond to standard treatment. Despite receiving multiple treatments, she failed to improve clinically. Later, she received IVIG, which significantly improved her condition [8]. This demonstrates how IVIG can be an effective option when toxic shock syndrome does not respond to conventional treatments.

However, not all research supports its effectiveness. Some meta-analyses have found no significant reduction in mortality associated with IVIG use in streptococcal TSS [10]. Therefore, even though IVIG may offer benefits in certain scenarios, further large-scale and high-quality studies are needed to confirm its overall impact on outcomes in TSS.

Ask About IVIG Home Infusion

(877) 778-0318

FAQs

Here are some frequently asked questions about IVIG for toxic shock syndrome:

1. Is IVIG FDA-approved for treating toxic shock syndrome (TSS)?

No. The use of IVIG for TSS is not FDA-approved. However, several clinical studies have shown that IVIG can be an effective add-on therapy for TSS, particularly in severe or unresponsive cases [6][7][8][9][10]. Therefore, IVIG is often used “off-label” for TSS, based on the clinical judgement of the healthcare provider.

2. Can IVIG cure TSS on its own?

No. IVIG is an add-on therapy (supportive treatment). It doesn’t treat TSS on its own. IVIG must be used with antibiotics and other supportive care.

3. How long does it take for IVIG to work in toxic shock syndrome (TSS)?

Intravenous immunoglobulin (IVIG) can lead to clinical improvements in toxic shock syndrome (TSS) within hours of administration [11]. However, the full response time may vary from days to weeks depending on the severity of your condition, how early you received IVIG, and your overall health.