Pazopanib (Votrient): Uses, How it Works, Dosage, Side Effects, and More

Pazopanib, sold under the brand name Votrient, is a targeted cancer medication frequently prescribed to treat specific types of cancers. It belongs to the class of potent kinase inhibitors that work to slow down tumor growth and spread to other parts of the body. 

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Pazopanib was initially approved by the FDA in 2009 for the treatment of kidney cancer. Later, its approval was expanded to include the treatment of sarcoma as well. 

This cancer drug comes in the form of a capsule-shaped tablet that is taken orally, as prescribed. It is available only with a prescription from a physician. Read on to learn how Pazopanib works, how it is used, possible side effects, and what to expect during treatment. 

Pazopanib (Votrient): Uses

Pazopanib is an FDA-approved drug and is typically prescribed to adult patients with the following types of cancers:

  • Kidney cancer, also known as advanced renal cell carcinoma (RCC), is a type of cancer that has advanced or has spread to other organs.
  • Soft-tissue sarcoma (a cancer that occurs in bone and connective tissues, such as fat, muscle, and blood vessels). 

Pazopanib may be prescribed for other purposes as determined by your healthcare provider. 

Important Safety Information (Boxed Warning)

Pazopanib may cause severe or life-threatening liver damage (hepatotoxicity). Therefore, it is recommended that liver function be monitored during treatment with this drug. Call your doctor immediately if you experience any of the following symptoms: yellowing of the skin or eyes, dark urine, extreme tiredness, nausea, vomiting, loss of appetite, pain in the upper right part of the stomach, or unusual bleeding or bruising. 

Pazopanib: Working Mechanism

Pazopanib is a type of targeted cancer medicine that attacks cancer in a more specific way than traditional chemotherapy. Typically, cancer cells need oxygen and nutrients to grow and spread. To achieve this, they release specific proteins (tyrosine kinases) that signal the body to form new blood vessels (a process called angiogenesis). 

Pazopanib, which is a potent tyrosine kinase inhibitor (TKI), blocks these proteins (tyrosine kinases) and helps:

  • Stop the growth of new blood vessels that feed the tumor
  • Reduce the supply of oxygen and nutrients to cancer cells
  • Slow down tumor growth and spread

In short, it starves the cancer cells by cutting off their blood supply and growth signals that tumors need to grow.

Pazopanib: Drug Form

Pazopanib is available as a capsule-shaped oral tablet in strengths of 200 mg and 400 mg. 

Recommended Dosage and Administration

The recommended dose of pazopanib for patients with advanced kidney cancer and soft-tissue sarcoma is 800 mg once a day. 

Pazopanib tablets are taken on an empty stomach with a glass of water, at least 1 hour before or 2 hours after a meal. You should swallow the tablet whole; do not crush, chew, or break it. 

Also, do not eat grapefruit or drink grapefruit juice during treatment with pazopanib. Grapefruit products may increase the amount of pazopanib in your body.

If you miss a dose, take it as soon as you remember. Do not take it if it is close (within 12 hours) to your next dose. Just take the next dose at your regular time. 

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Possible Side Effects

Patient suffering from side effects of pazopanib

Pazopanib may cause some side effects. You might have one or more of them. The commonly reported side effects in individuals with kidney cancers after taking pazopanib are:

  • Diarrhea
  • Hypertension
  • Hair color changes (depigmentation)
  • Nausea
  • Anorexia
  • Vomiting

People with soft-tissue sarcoma may experience the following side effects after taking Pazopanib:

  • Fatigue
  • Diarrhea
  • Nausea
  • Decreased weight
  • Hypertension
  • Decreased appetite
  • Vomiting
  • Tumor pain
  • Hair color changes
  • Musculoskeletal pain
  • Headache
  • Dysgeusia (taste changes)
  • Dyspnea (shortness of breath)
  • Skin hypopigmentation

Adverse Side Effects

Pazopanib may cause serious side effects, including: 

  • Liver problems (yellowing of skin or eyes, dark urine, severe tiredness)
  • Severe high blood pressure (hypertension)
  • Irregular or fast heartbeat or fainting
  • Heart failure
  • Heart attack or stroke
  • Bleeding problems (unusual bruising, coughing up blood, black stools)
  • Blood clots (sudden leg pain or swelling, trouble breathing)
  • Severe stomach or bowel problems (tear in your stomach or intestinal wall [perforation] or an abnormal connection between two parts of your gastrointestinal tract [fistula])
  • Lung problems (inflammation in the lungs)
  • Reversible Posterior Leukoencephalopathy Syndrome (a serious brain condition in which blood vessels in your brain swell)
  • Hypothyroidism (low thyroid hormones)
  • Proteinuria (high amount of protein in the urine)
  • Serious infections

Although less common, these require immediate medical attention. Always tell your doctor if you notice new or worsening symptoms.

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Pazopanib: Precautionary Measures 

Before taking Pazopanib, tell your doctor if you:

  • Have or had liver problems
  • Have high blood pressure 
  • Have heart problems or an irregular heartbeat, including QT prolongation 
  • Have a history of a stroke 
  • Have headaches, seizures, or vision problems
  • Have coughed up blood in the last 6 months
  • Had bleeding in your stomach or intestines in the last 6 months
  • Have a history of a tear (perforation) in your stomach or intestine, or an abnormal connection between two parts of your gastrointestinal tract (fistula) 
  • Have had blood clots in a vein or in the lung 
  • Have thyroid problems 
  • Had recent surgery (within the last 7 days) or are going to have surgery 
  • Have any other medical conditions 
  • Are allergic to pazopanib or any of the other ingredients of this medicine
  • Are pregnant or plan to become pregnant. Pazopanib can harm your unborn baby. 
  • Are breastfeeding or plan to breastfeed. It is not known if pazopanib passes into your breast milk. You and your healthcare provider should decide if you will take Pazopanib or breastfeed. You should not do both. 
  • Are taking any prescription or non-prescription medicines, vitamins, and herbal supplements. Pazopanib may affect the way other medicines work, and other medicines may affect how pazopanib works.

Medicines To Avoid 

You should avoid taking the following medications, as they may alter the blood level of pazopanib, may affect how Votrient works, or make it more likely that you’ll have side effects. These include:

  • A proton pump inhibitor (PPI), such as esomeprazole (Nexium)
  • Any medicine that can cause QT prolongation, which is a potentially dangerous heart rhythm problem
  • Dextromethorphan (a medicine used to treat a cough caused by the common cold or other illnesses)
  • Ketoconazole (a medicine used for fungal infections)
  • Lapatinib (Tykerb) (a medicine used to treat certain types of breast cancer)
  • Midazolam (a medicine used to relieve anxiety before surgery)
  • Medicines that contain simvastatin to treat high cholesterol levels
  • Herbal supplements containing St. John’s wort

Pazopanib Cost

Pazopanib (Votrient) can be very expensive without insurance because it is a branded medication. The cost may reach several thousand dollars per month, depending on the tablet strength and how many tablets are prescribed. 

For example, a 120-tablet supply of 200 mg pazopanib may cost between $9,495 and $22,701, whereas a 30-tablet supply of 400 mg may cost approximately $3,749 or more before discounts, insurance, or assistance programs.

Many patients do not pay the full price because insurance coverage, copay assistance, or pharmacy discount cards can help reduce the cost. Contact us if you are interested in exploring financial assistance opportunities for Pazopanib. 

Medicare and TPN: What You Need To Know

Total parenteral nutrition (TPN) is a vital therapy for some patients who cannot obtain essential nutrients from food due to an impaired digestive function. However, it can be quite expensive, regardless of whether it is needed for short-term or long-term use.

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On average, the TPN nutrient solution and the necessary supplies cost more than $200 per day without insurance. If TPN is provided during a hospital stay, the total cost of the treatment can rise into the thousands of dollars, sometimes reaching $3,000 or more.

Fortunately, some insurance programs, such as Medicare, may partially or fully cover the cost of TPN when it is considered medically necessary.

This article addresses most of your financial concerns related to this treatment, including what Medicare covers, who qualifies for TPN under the Medicare plan, how reimbursement works, and what you might expect to pay. 

Total Parenteral Nutrition (TPN): Basic Understanding

TPN is a medical therapy that delivers essential nutrients like a solution of glucose (sugar), fats, proteins, vitamins, and minerals through an IV line into a patient’s bloodstream. Unlike oral or tube feeding, TPN completely bypasses the digestive system.

When TPN Is Recommended

You may qualify for TPN treatment if you have or are suffering from the following conditions:

  • Short bowel syndrome
  • Severe Crohn’s disease
  • Bowel obstruction
  • Severe pancreatitis
  • Certain cancers affecting digestion
  • Intestinal failure
  • Severe malabsorption disorders
  • Complications after major abdominal surgery
  • Recovery after complex surgery

TPN is usually administered in a hospital setting. But it can also be administered in a skilled nursing facility or at home with supervision.

Cases Where TPN Isn’t Recommended 

TPN is not appropriate for every patient, and you may not receive it if you have:

  • A functioning digestive tract that can tolerate oral or tube feeding.
  • A severe metabolic instability that cannot be safely managed.

Estimated Price Structure of TPN Therapy

TPN is expensive because it requires specialized nutrients, sterile preparation, equipment, and monitoring. Below is the estimated price breakdown to give you an idea of the costs. 

  • Daily TPN therapy: $200 to $400 per day (or more)
  • A 16-day regimen: $2,405 to $3,169 (including supplies, lipids solution, hospital stay, and nursing)
  • Monthly costs: $6,000 to $15,000+
  • Hospital-based TPN may cost significantly more than home-based TPN

The actual costs may depend on several factors, such as your specific nutrient composition, how long you need treatment, whether it is provided in a hospital or at home, nursing services, and any complications or monitoring needs.

If you don’t have insurance, TPN can be expensive. But with Medicare coverage, you can significantly reduce your out-of-pocket expenses. 

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Medicare: Brief Overview

Medicare is federal health insurance for people 65 or older, and some people under 65 with certain disabilities or conditions like end-stage renal disease or permanent kidney failure requiring dialysis or a kidney transplant. 

In 2024, more than 66 million people in the U.S. received health coverage through Medicare.

Key Components of Medicare

Medicare coverage is offered in four different parts. Each part varies and covers different medical care, products, and services, which are as follows:

  1. Part A (hospital insurance): Covers inpatient hospital care, skilled nursing facility care, and some home health care.
  2. Part B (medical insurance): Covers doctor services, outpatient care, durable medical equipment (DME), preventive services like vaccines & screenings, and medically necessary supplies.
  3. Part C: (medicare advantage): Private insurance plans that replace Parts A and B, often offering additional benefits.
  4. Part D (prescription drug coverage): Covers the costs of prescription medications (both generic & branded drugs).

Medicare Coverage for TPN: What It Covers 

Nurse and patient setting up TPN at home

Medicare can cover TPN therapy under Part A or Part B, depending on where and how the therapy is given. Medicare coverage can include both the nutrition therapy itself and the TPN equipment needed to safely deliver it. But both of these are paid under two different benefit categories.

1. Enteral & Parenteral Nutrition (Prosthetic Device) Benefit

Medicare’s Enteral and Parenteral Nutrition benefit covers the TPN nutrient solution and necessary supplies under Part B. This benefit falls under Medicare’s legal category of Prosthetic Devices Benefit, as outlined in the Social Security Act § 1861(s)(8) and defined in the Medicare Benefit Policy Manual (CMS Pub. 100-02), Chapter 15, Section 120. 

Moreover, when patients are metabolically stable enough to be safely managed outside the hospital and require long-term nutritional support, home parenteral nutrition (HPN) may also be covered under this category if they meet the medical criteria. 

In case of an inpatient hospital stay, it is usually covered under Medicare Part A as part of the hospital’s bundled payment. In that situation, patients are not billed separately for the TPN therapy.

2. Durable Medical Equipment (DME) Benefit

TPN requires specialized equipment to deliver nutrients safely into the bloodstream. The Durable Medical Equipment benefit category, which falls under Medicare Part B, helps cover TPN equipment (such as an infusion pump and an IV pole). 

In short, the nutrition (TPN solution & supplies) is covered under the enteral and parenteral nutrition (prosthetic device) benefit, and the equipment used to administer TPN is covered under the DME benefit category.

How the Two Benefits Work Together

If you want to receive TPN at home (HPN), you may qualify for both benefits (nutrition solution and TPN equipment). This would require documentation showing that TPN is medically necessary because the patient cannot absorb nutrients through their gastrointestinal tract.

Patient Costs for TPN Under Medicare 

With Medicare Part A, which covers hospital stays, patients must pay a $1,736 deductible (2026) for each benefit period before Medicare starts covering costs. If the hospital stay is long, they may also owe extra coinsurance.

For Medicare Part B, which covers TPN at home or in outpatient settings, patients first meet the annual deductible. After that, they usually pay about 20% of the Medicare-approved cost.

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Who Qualifies for Medicare Coverage of TPN?

Medicare applies strict medical necessity standards for long-term parenteral nutrition coverage under its National Coverage Determination.

Medicare coverage is available when all of the following conditions are met:

  • The digestive tract is permanently or indefinitely impaired and cannot absorb enough nutrients to maintain health.
  • Oral and enteral feeding (tube feeding) have failed, are contraindicated, or are not sufficient.
  • TPN is medically necessary to sustain life and is reasonable and necessary for the patient’s condition.
  • The condition is long-term or of indefinite duration (generally expected to last at least 90 days or longer).

Conditions That May Qualify for Medicare Coverage of TPN

Medicare may cover TPN for patients with certain conditions, including:

  • Chronic intestinal failure
  • Short bowel syndrome
  • Severe Crohn’s disease with malabsorption
  • Malignant bowel obstruction
  • Chronic intestinal pseudo-obstruction
  • Severe motility disorders
  • Certain cancers affect nutrient absorption

To receive Medicare coverage, you typically need to provide:

  • A detailed physician order.
  • Medical records showing the inability to absorb nutrients.
  • Evidence that enteral (tube) feeding is not sufficient. 

When Medicare May Deny Coverage for TPN

There is a chance that Medicare may deny TPN coverage if certain criteria are not met. Denials most commonly occur when documentation does not clearly demonstrate long-term gastrointestinal failure or medical necessity. For instance:

  • The condition is temporary and expected to improve quickly.
  • The patient can still absorb nutrients through the gastrointestinal tract.
  • Enteral (tube) feeding is possible and adequate.
  • The medical record does not clearly explain why the GI tract cannot absorb nutrients.
  • The physician’s order is incomplete or missing required details.
  • There is no clear statement that the condition is expected to require long-term therapy.

If you have questions about Medicare coverage or want to know which benefit category you qualify for, contact our support team for assistance.

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Other Important Frequently Asked Questions (FAQs)

1. What TPN Equipment Does Medicare Cover?

For eligible patients receiving home TPN under Medicare Part B, the following equipment and supplies are typically covered:

  • Parenteral nutrient solutions
  • Daily supply kits, including:
    • IV tubing
    • Infusion bottles
    • Catheters and related care supplies
    • Dressings
    • Sterile gloves
    • Alcohol wipes
    • Saline/heparin flushes
    • Needles and syringes
  • Daily administration kits, including:
    • Infusion tubing set
    • Volumetric monitors
    • Filters
  • Infusion pump (one pump is covered)
  • IV pole hook or related components
  • Equipment maintenance

Supplies must be ordered by your physician and provided by a Medicare-approved DME supplier. 

2. Are Nutrition Services Covered by Medicare?

Yes, Medicare covers certain nutrition services, including Medical Nutrition Therapy (MNT) under Medicare Part B. This service normally provides nutrition counseling and diet planning to patients with diabetes, chronic kidney disease, or a recent kidney transplant with a doctor’s referral. 

However, TPN is not part of MNT. Instead, it is covered separately under a different Medicare benefit category. 

3. What is The Nutrition Therapy Act for Medicare?

The Nutrition Therapy Act of 2023 is a proposed federal bill intended to expand Medicare coverage for Medical Nutrition Therapy (MNT) to more people with chronic conditions.

Currently, Medicare only covers MNT for diabetes, chronic kidney disease, or a recent kidney transplant. Because the act has not yet become law, existing Medicare rules still apply.

4. How Much Does Medicare Reimburse for Medical Nutrition Therapy?

Medicare Part B generally reimburses 100% of Medical Nutrition Therapy (MNT) costs for eligible patients, covering up to 3 hours in the first year and 2 hours in subsequent years, with additional time possible if medically necessary.

Managing Stress During Cancer: Practical Ways to Cope and Find Strength

About 20 million people are diagnosed with cancer every year, which is expected to become 50 million by 2050 [1]. A cancer diagnosis is overwhelming news. It can change your life in an instant. Not only do you go through physical symptoms, but you also experience intense emotional stress. You may feel sadness, fear, loss of control, and uncertainty. 

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Stress is a natural response to cancer, but that does not mean you should ignore it. Managing stress during cancer is very important. This is because stress can affect your mental health, weaken your immune system, and reduce your ability to cope with treatment. In this article, we’ll explain everything you need to know about managing stress during cancer, including what stress is, how cancer increases stress, and practical ways to manage stress during cancer.

Understanding Stress in Cancer Patients

Stress is the mental tension or worry caused by a challenging or threatening situation. A little bit of stress is actually helpful because stress causes your body to release hormones like cortisol and adrenaline. These hormones prepare your body to handle danger. But long-term stress can harm both your physical and mental health [2].

Cancer is a very stressful event in anyone’s life. You already suffer from various painful physical symptoms. On top of that, you may worry about survival, treatment success, side effects, and the future of you and your family. According to one study, one in three people with cancer has anxiety or other mental health challenges [3]. Similarly, another study shows that women with ovarian cancer are three times more likely than the general public to be diagnosed with mental illnesses like anxiety and depression [4].

Stress is a common part of the cancer experience. If you ignore the stress, it can weaken your immune system, interfere with your treatment, and reduce your quality of life. That is why you need to recognize stress early and take proper steps. 

How Stress Affects the Body During Cancer

You may think that stress only affects how a person feels emotionally. In reality, stress also triggers some physical and biological changes throughout the body. During cancer, these stress-related changes can cause the following effects:

Effects on the Immune System: Long-term stress can weaken your immune system [5]. Stress increases your cortisol (stress hormone) levels. A high level of stress hormones will reduce your body’s ability to fight infections. This is particularly concerning for cancer patients because chemotherapy already suppresses their immune systems.

Effects on Mental Health: Stress can cause anxiety, depression, irritability, and hopelessness. It can also cause sleeping problems, which makes it harder for you to rest and recover.

Effects on Cancer Treatment: Severe stress can reduce your treatment tolerance. You may not want to take medicines. You may even lose the desire to live. Patients under high stress may experience more fatigue, pain, and difficulty following treatment plans. 

Signs and Symptoms of Stress in Cancer Patients

During your cancer journey, stress can appear in many different ways. It can affect your emotions, body, and everyday behavior. Therefore, you should recognize the common signs of stress before they become difficult to manage. Here are some of the common signs [6][7]:

  • Extreme fear and worry
  • Anxiety and depression
  • Irritability and anger
  • Difficulty with memory, concentration, and decision-making
  • Guilt and blame
  • Fatigue, insomnia, and loss of appetite
  • Social isolation, loss of interest, and avoidance behavior

Do not ignore these symptoms. If you have them, inform your doctor.

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Practical Ways to Manage Stress During Cancer

Living with cancer can feel overwhelming, both physically and emotionally. Your stress can be a result of treatment side effects, fear about the future, or changes in daily life. It is true that you can’t completely avoid or get rid of stress. But there are some practical ways to reduce your stress. Here are some of them [8][9][10]:

1. Mindful Breathing and Relaxation Techniques

Are you overwhelmed with anxiety and stress? Try some simple relaxation techniques that can quickly calm your body and mind. Deep breathing can lower stress hormones and help reduce tension. Here is a step-by-step, simple relaxation technique that you can follow to reduce stress:

  • Find a quiet and comfortable spot. Relax your shoulders and neck.
  • Inhale slowly through your nose for 4-5 seconds and allow your abdomen to expand.
  • Exhale slowly through your mouth for 5-6 seconds.
  • Focus on the sensation of the cool air entering and warmer air leaving.
  • Continue this cycle for 3-5 minutes to reduce stress and anxiety.

2. Physical Activity and Gentle Movement

Cancer patient walking in a park with a friend

When you engage in physical activity, your brain releases endorphins (feel-good hormones). They help reduce your stress, improve your mood, and boost your energy levels [11]. Some physical activities that are suitable for cancer patients include:

  • Walking at a pace that is comfortable
  • Yoga or gentle stretching
  • Light home-based exercise that your doctor recommends

Do not do any extensive exercise without first consulting your doctor. Always choose activities that match your energy level and medical condition.

3. Emotional Support and Communication

Do not keep your thoughts to yourself. Keeping stress inside often makes it worse. Find a friend or family member and share how you feel. Talking about your feelings can significantly reduce emotional burden. Do not think that you are alone. You can find emotional support from your family members and close friends, cancer support groups, and mental health professionals.

4. Sleep and Nutrition

You need proper sleep and nutrition to deal with the stress of cancer. If you don’t have proper sleep or nutrition, you may experience increased fatigue, irritability, and mood swings. Here are some helpful practices to reduce your stress:

  • Maintain a regular sleep schedule. Have at least 8 hours of sleep every day. Try to sleep and wake up at the same time every day.
  • Create a comfortable and quiet sleep environment.
  • Have a balanced and nutritious diet.
  • Stay well hydrated throughout the day.

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5. Mindfulness or Spiritual Practices

You may find deep comfort and emotional strength through mindfulness, prayer, or other spiritual practices. These practices allow many patients to connect with their inner self or higher power. Eventually, they find peace, hope, and acceptance. Examples of such practices include:

  • Meditation or mindfulness exercises: These activities can help you calm your mind and reduce anxiety.
  • Prayer or religious rituals: Certain rituals can help you find spiritual peace, acceptance, strength, and trust in a greater purpose.
  • Journaling personal thoughts: If you find it difficult to share your feelings and thoughts verbally, try to write them down. Expressing your feelings is very helpful to reduce emotional burden.

6. Financial Management and Planning

Many people overlook it, but financial stress is a big source of anxiety during cancer. You may feel overwhelmed by the thoughts of treatment costs, travel expenses, and time away from work. Here are some practical steps to cope with this stress:

  • Discuss treatment costs with your physician early and prepare accordingly.
  • Check insurance coverage and payment options.
  • Consult with social workers or financial counselors who can help you get financial aid.
  • Explore patient assistance programs.

Have a clear financial plan. This will reduce your anxiety and allow you to focus more on recovery and well-being.

The Bottom Line

Unfortunately, stress is a normal part of the cancer journey. But the good news is you don’t need to fully eliminate stress to live well. Just a small step towards reducing stress will make a meaningful difference in your day-to-day life. With the right support, coping strategies, and medical care, you can find hope, strength, and peace.

Jivi for Hemophilia A: How It Works, Benefits, and Possible Side Effects

Bleeding disorders can significantly impact your day-to-day life. One such condition is hemophilia A, which affects more than 400,000 males worldwide [1]. Hemophilia A is an X-linked condition, meaning males who carry the defective gene will become affected, whereas females with the affected gene are mildly affected, if at all.

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Hemophilia A occurs when your blood doesn’t clot properly. As a result, people with hemophilia A experience frequent or prolonged bleeding episodes. If you do not treat it, it may lead to serious complications such as joint damage, chronic pain, and disability. In some cases, it can even cause death.

Fortunately, there are many medications to manage hemophilia A and reduce the risk of bleeding. One such medication is Jivi (damoctocog alfa pegol). In this article, we’ll talk about everything you need to know about Jivi, including its dosage, side effects, and much more.

What Is Jivi?

Jivi (damoctocog alfa pegol) is an FDA-approved medicine used to control or prevent bleeding episodes in patients with Hemophilia A. Hemophilia A is a genetic disorder caused by a lack of clotting factor VIII (a protein needed for normal blood clotting). 

Jivi is a man-made version of clotting factor VIII that is chemically modified to last longer in the body. It was developed by Bayer, and the FDA first approved it in August 2018 for use in children and adults with Hemophilia A [2]. 

You can get this medication through an IV infusion, and it is designed to give you longer protection compared to other factor VIII products.

What Is Jivi Used To Treat?

Jivi is a factor VIII replacement medicine. Factor VIII is a protein that is needed to clot blood properly. Jivi is used to treat people with hemophilia A, a condition where the body does not have enough factor VIII. 

Jivi can be used in adults and children aged 7 or older who have taken factor VIII replacement therapy before. It is FDA-approved for [3]:

  • Treating bleeding episodes when they happen
  • Controlling bleeding during and after a surgery
  • Regular preventive treatment to reduce the frequency of bleeding episodes

Please note that Jivi is not recommended for children under 7 years of age, people who never received factor VIII therapy, or people who have Von Willebrand disease.

How Does Jivi Work?

Factor VIII is a protein that your body needs to form blood clots properly. In people with hemophilia A, the body does not produce enough functional factor VIII. As a result, they experience prolonged or frequent bleeding episodes. 

Jivi is a laboratory-made factor VIII medicine. It works by replacing the missing clotting factor VIII in people with hemophilia A. The notable feature of Jivi is that it has been modified by attaching it to a substance called PEG (polyethylene glycol). This modification slows down the rate at which Jivi is removed from the body.

As a result, Jivi stays in your bloodstream for a longer time. Thus, you get longer-lasting protection against bleeding.

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Jivi Side Effects

Like any other medicine, Jivi can also cause side effects. Some are mild while others may be serious and require immediate medical attention. Here are some common and serious side effects [3][4]:

Common Side Effects

Boy experiencing cough, a side effect of Jivi

Here are the most common side effects of Jivi:

  • Fever
  • Cough
  • Headache
  • Abdominal pain

These side effects are usually mild and resolve within a few days. But if they persist or worsen, consult your doctor.

Serious Side Effects

Although rare, Jivi can cause serious side effects. Here are some of them:

Severe Allergic Reactions: Jivi can cause allergic reactions, which can be severe. Symptoms include rash, itching, swelling of the face or throat, difficulty breathing, nausea or vomiting, and stomach cramps. If an allergic reaction occurs, stop using Jivi immediately and contact your doctor.

Development of Factor VIII Inhibitors: You may develop inhibitors (antibodies) that reduce the effectiveness of Jivi. If bleeding does not stop as expected, your doctor may conduct a blood test to check the factor VIII levels in the blood to confirm whether they are lower than they should be.

Immune Reaction to PEG: The immune system of some patients may react to PEG (a substance in Jivi). This can lead to sudden allergic reactions or make Jivi less effective. 

Please note that this is not a complete list of all possible side effects. If you experience any unusual or severe symptoms, contact your doctor immediately.

Dosing

Jivi is available as a lyophilized powder in single-dose vials. Your doctor will reconstitute it before using. You will receive this drug through intravenous infusion. The dosage and frequency of Jivi infusion depend on your weight, the severity of your disease, and your treatment goal. Here is the usual recommended dosage of Jivi [4]:

Treatment of Bleeding Episodes and Surgical Use: The dosing is based on how much the patient’s factor VIII level needs to increase. The required dose is calculated using your body weight and the desired increase in factor VIII activity. Your doctor will determine the right dose for you.

Prevention of Bleeding: Here is the usual dosage that you must take regularly to prevent bleeding:

  • For adults and children aged 12 years or older, the usual starting dose is 30-40 IU/kg twice weekly. If bleeding continues, the dosage can be adjusted to 45-60 IU/kg every 5 days.
  • For children aged 7-11 years, the recommended starting dose is 60 IU/kg. Your doctor will adjust the dose based on clinical response and factor VIII recovery.

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Before Starting Jivi

Before you start your treatment with Jivi, you need to discuss your full medical history with your doctor. This will ensure that the medicine is safe and effective for you and reduce the risk of complications during treatment. You should tell your doctor if you: 

  • Have ever had an allergic reaction to factor VIII products or polyethylene glycol (PEG)
  • Are allergic to rodents (such as mice or hamsters)
  • Have developed factor VIII inhibitors (antibodies) in the past
  • Have any other bleeding disorder besides hemophilia A
  • Have a history of immune system disorders, as this may increase the risk of inhibitor formation
  • Are pregnant, planning to become pregnant, or breastfeeding

Make sure to always follow your doctor’s instructions carefully and never change your dose or treatment schedule without medical advice.

Jivi Cost

Jivi is available as a powder in a single-dose vial for injection. Depending on the strength, a single vial can cost several thousand dollars. Actual out-of-pocket costs vary based on dose, insurance coverage, frequency, and assistance programs. Contact us if you are interested in exploring financial assistance options for Jivi.  

Vasculitis: What You Need to Know

Vasculitis refers to a group of disorders characterized by inflammation of blood vessels. Many types are autoimmune, though some are triggered by infections, medications, or other conditions. While some types are extremely uncommon, overall prevalence varies depending on the specific subtype. In this article, you will learn about its causes, symptoms, diagnosis, and treatment options. Also, we will answer some of the most common questions about this condition.  

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What Is Vasculitis?

Synonyms: Angiitis and arteritis

Vasculitis is not a single disease. It is a group of disorders that involve swelling and irritation of blood vessels. When the blood vessels are inflamed and narrowed, they cannot supply enough blood to the organs and tissues. As a result, these organs and tissues can become damaged. 

Vasculitis can affect the following types of blood vessels in the body:

  • Arteries (Arteries deliver oxygen-rich blood to different organs and tissues)
  • Veins (Veins take oxygen-poor blood to the heart)
  • Capillaries (Tiny vessels that carry oxygen and nutrients to cells in the body)

While many types of vasculitis exist, most are rare. Depending on the type of blood vessel affected, this condition may affect one or several organs. 

What Causes Vasculitis?

Researchers have yet to determine the exact cause of vasculitis. Your genes might be responsible for some types, while others might result from your immune system mistakenly attacking the blood vessels. 

Several factors may be responsible for such immune reactions:

  • Infections (Hepatitis B and hepatitis C)
  • Blood cancers
  • Immune disorders like rheumatoid arthritis, lupus, and scleroderma
  • Reactions to certain drugs, including quinolones, clarithromycin, furosemide, NSAIDs, and TNF-α inhibitors

Risk Factors

Anyone can get this condition. However, some factors may increase the risk, such as:

  • Age: Giant cell arteritis typically affects people older than 50. Kawasaki disease is most common in children below 5 years old.
  • Lifestyle factors: Use of tobacco and cocaine may increase the risk of certain types of vasculitis, especially in men younger than 45. 
  • Infections and autoimmune conditions: Certain infections and autoimmune disorders can trigger your immune system to attack healthy blood vessels. 

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What Are the Symptoms of Vasculitis?

Patient experiencing headache, a symptom of vasculitis

Symptoms common to most types of vasculitis are:

  • Fever
  • Headache
  • Tiredness
  • Weight loss
  • Aches and pains

Symptoms specific to the part of the body affected can include:

Digestive system

  • Pain after eating
  • Ulcers
  • Blood in stool 

Ear

  • Dizziness
  • Ringing in the ears
  • Sudden hearing loss

Eyes

  • Itching, burning, and redness in the eyes 
  • Double vision
  • Temporary or ongoing blindness

Hands or feet

  • Numbness or weakness in hands or feet
  • Swollen palms or soles 

Lungs

  • Shortness of breath 
  • Blood in cough 

Skin

  • Red spots due to bleeding under the skin 
  • Open sores or lumps 

How Is Vasculitis Diagnosed?

Diagnosis involves a medical history and physical exam. 

Your healthcare provider may order tests to diagnose vasculitis or exclude other conditions with overlapping symptoms. Tests can include:

  • Blood tests: They help detect inflammatory markers and certain antibodies (proteins) in your body. 
  • Imaging tests: Tests like X-rays, ultrasounds, CT scans, MRIs, and PET scans can help determine the location of the affected blood vessels and organs. 
  • Angiography: This test helps your healthcare provider observe early inflammatory changes in your blood vessels. 
  • Biopsy: During a biopsy, a healthcare professional removes a small piece of tissue from the affected area. The tissue is further tested in a lab to look for signs of vasculitis. 

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What Are the Treatments for Vasculitis?

No cure for vasculitis is currently available. However, appropriate treatments can help you enter symptom-free periods (remission). Remission can last for years or months. 

Treatments help manage your symptoms and prevent damage to your blood vessels and organs. They can include:

Corticosteroids

Prednisone, a corticosteroid, is the most commonly used medication to reduce inflammation. Corticosteroids are typically used during active disease to achieve remission. 

Corticosteroids, especially when used for prolonged periods, can cause side effects, such as:

  • Weight gain 
  • Elevated blood sugar
  • Weakened bones 

Immunosuppressants

These medications help prevent your immune system from attacking the blood vessels. They are used to keep you in remission for as long as possible. Examples include azathioprine, methotrexate, and mycophenolate.

Other medications that may be used based on the type of vasculitis include:

Surgery 

Surgery may be an option if you have:

  • Aneurysms (balloonlike bulges in the wall of a blood vessel)
  • Blocked arteries 

Can You Use IVIG for Vasculitis?

Your healthcare provider may prescribe IVIG for vasculitis, but only in specific cases. 

Medications such as rituximab, methotrexate, and azathioprine can lead to favorable outcomes for most patients. However,  IVIG may be an option when a patient cannot take an immunosuppressant because they already have a compromised immune system. (Note: IVIG is an immunomodulatory agent, not an immunosuppressant.)

Moreover, IVIG is generally safe during pregnancy, and it might be the better option for certain pregnant women with vasculitis. 

Lastly, IVIG may be used to counteract hypogammaglobulinemia caused by rituximab. 

Frequently Asked Questions

What to avoid if you have vasculitis?

Things to avoid if you have vasculitis:

  • Processed foods
  • Added sugars
  • Alcohol
  • Excessive salt
  • Smoking 

What’s the life expectancy of people with vasculitis?

There’s no single answer. Moreover, we don’t have data on the link between specific types and lifespan. Some types are mild, while some are more severe. Types that affect vital organs like the brain, lungs, or kidneys can be life-threatening, particularly during the active disease state. Treatments that suppress the immune system may increase the risk of infections. With early diagnosis and modern treatments, many people with vasculitis live normal or near-normal lifespans.  

Can you recover from nerve damage caused by vasculitis?

Your chances of nerve recovery are higher if the treatment is started early. Sadly, in most cases, only partial recovery is possible. 

Should you follow a specific diet for vasculitis?

It’s hard to pinpoint and recommend a particular diet for a patient without knowing their unique individual situations. In general, it’s best to follow the American Heart Association’s dietary guidelines, which include:

  • Plenty of whole grains, fruits, and vegetables
  • Limited intake of added sugars, alcohol, and ultra-processed foods.

ARALAST NP: Uses, Dosage, Side Effects, Cost, and Safety Information

ARALAST NP is used to treat adults with symptoms of emphysema caused by alpha-1 antitrypsin deficiency (AATD). It was initially approved by the U.S. FDA in 2002. Baxalta Inc. (now a subsidiary of Takeda Pharmaceutical Company) manufactures this medication. 

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In this article, you will learn about its mechanism of action, dosage, side effects, cost, and more.

Important Warnings and Precautions

Allergic Reactions

This medication may cause severe allergic reactions, which can be fatal. Seek emergency medical care if you experience:

  • Hives
  • Itching
  • ­Chest tightness
  • Shortness of breath
  • Wheezing
  • Faintness
  • ­Low blood pressure

Infection Transmission Risk

This medication is made using donated human plasma. Thus, it may carry a risk of spreading viruses or other infectious agents. Talk to your healthcare provider about any potential risks of disease transmission. 

Contraindication

Don’t use this medication if you have IgA deficiency with antibodies against IgA.

Before Taking ARALAST NP

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

  • Have or had any medical issues.
  • Are allergic to this product, any product component, or any other medications.
  • Have IgA deficiency with antibodies to IgA.
  • Have or had liver disease or chronic obstructive pulmonary disease (COPD).
  • Are pregnant or planning to get pregnant. It is unknown if this medication harms your unborn baby. 
  • Are breastfeeding. It is unknown if this medication is present in breast milk, and if it can harm the baby.

ARALAST NP Information and Uses

ARALAST NP injection is a brand-name prescription product. It contains the active ingredient “Alpha1-Proteinase Inhibitor (Human)”, which is in a medication class known as blood derivatives. 

A healthcare provider may prescribe this medication for adults with symptoms of emphysema caused by severe alpha1-antitrypsin deficiency (AATD). 

Alpha1-antitrypsin deficiency (AATD) is an inherited condition. Over time, it can damage the lungs, liver, skin, and blood vessels. People with AATD have either insufficient or faulty alpha1-antitrypsin. 

Alpha1-antitrypsin is a protein made in the liver. This protein protects the lungs from smoke, dust, or pollution. When you have insufficient or faulty alpha1-antitrypsin, your lungs are not fully protected, which may cause emphysema. 

Emphysema is a chronic lung condition. Symptoms can include:

  • Shortness of breath
  • Wheezing
  • Coughing
  • Chest tightness or heaviness
  • Excessive tiredness

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ARALAST NP Mechanism of Action

ARALAST increases the levels of alpha1-antitrypsin in the blood and lungs. By doing this, it helps prevent certain substances in the lungs from damaging the tiny air sacs (alveoli) in the lungs. 

ARALAST Dosage

This medication is supplied as a white or off-white to slightly yellow-green or yellow powder in single-dose vials. Each vial contains 0.5 g or 1 g of functional Alpha1-proteinase inhibitor. The vials are included in the kit. 

Each ARALAST NP kit contains:

  • An appropriate volume of Sterile Water for Injection
  • USP diluent (25 ml for 0.5 g vial and 50 ml for 1 g vial)
  • One sterile double-ended transfer needle
  • One sterile 20 micron filter needle 
  • One package insert

The recommended dosage is 60 mg/kg body weight once weekly by intravenous (IV) infusion. Your healthcare provider may change the infusion rate, depending on your response and level of comfort. 

They may lower the infusion rate or stop the infusion if severe reactions occur. Infusion may be resumed after the symptoms go away. 

You may receive your weekly dose in any of the following settings:

  • In the clinic by a healthcare provider 
  • At an outpatient infusion center
  • At home with a healthcare provider

ARALAST NP Side Effects

Common Side Effects

Young woman suffering from side effects from taking ARALAST
  • Cold symptoms such as a runny or stuffy nose, sneezing, cough, and sore throat
  • Headache
  • Muscle or joint pain
  • Tiredness
  • Flushing
  • Diarrhea
  • Nausea
  • Bruising or bleeding at the injection site

Severe Side Effects

Talk to your healthcare provider immediately if you experience:

  • Seizure
  • Yellowing of your skin or eyes
  • Chest pain, breathing difficulty, and wheezing
  • Sudden weakness on one side of the body
  • Blurred vision or eye pain/swelling
  • Slurred speech
  • Problems with balance or coordination

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Storage and Handling

  • Store this product at temperatures below 25°C (77°F).
  • Avoid freezing.
  • Never use after the expiration date printed on the label.
  • Store in the original carton to prevent sun exposure.

ARALAST NP Cost

Cost can vary depending on your insurance plan, location, and pharmacy. Contact your insurance provider to determine whether your plan covers this medication and whether prior authorization is required.

If you qualify, you may enroll in the Takeda Patient Support Co-Pay Assistance Program. Contact us if you are interested in exploring financial assistance opportunities for ARALAST NP. 

ARALAST vs. ARALAST NP

ARALAST NP is a newer, modified form of ARALAST. 

Both products have the same active alpha1-proteinase inhibitor components and identical formulations. Structurally, they are slightly different. However, the effects of these structural differences on drug efficacy are unknown. 

ARALAST is no longer available in the U.S. 

ARALAST NP vs. GLASSIA

Both products are prescribed for alpha1-proteinase inhibitor deficiency. However, they differ in formulations and strengths. 

GLASSIA is available as a ready-to-use liquid in a single-dose vial. Each vial contains 1 g, 4 g, or 5 g of functional Alpha1-proteinase inhibitor. 

On the other hand, ARALAST NP is supplied as a white or off-white to slightly yellow-green or yellow powder in single-dose vials. Each vial contains 0.5 g or 1 g of functional Alpha1-proteinase inhibitor. 

ARALAST must be reconstituted before administration. The reconstituted liquid is colorless or slightly yellow to yellow-green.

TPN After Bariatric Surgery: Indications and Potential Side Effects

Though not routinely used after bariatric surgery, TPN (total parenteral nutrition) may help certain patients with surgery-related complications and severe malnutrition. 

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What Is TPN? 

Total parenteral nutrition (TPN) is a feeding method that bypasses the digestive tract. It involves administering a special formula directly into your bloodstream through a vein. The formula contains fats, sugar, protein, salts, vitamins, minerals, and water. Thus, TPN provides most nutrients your body needs to function. 

What Is Bariatric Surgery?

Bariatric surgery (also called weight loss surgery or metabolic surgery) is a type of obesity treatment. It involves surgical procedures that make changes in the digestive tract. These changes can help you lose weight by:

  • Limiting the number of calories you can take in and absorb
  • Reducing hunger signals 

Bariatric surgery may be done when other weight loss treatments have failed or when you have serious obesity-related health issues. According to the American Society for Bariatric and Metabolic Surgery, over 270,000 surgeries were performed in 2023 [1]. 

Bariatric Surgery Complications 

While bariatric surgery can help you lose weight, it’s no quick fix. Besides, it comes with potential side effects, such as:

  • Malabsorption (impaired absorption of nutrients by the small intestine)
  • Malnutrition (deficiency of nutrients)
  • Gallstones (hardened deposits in the gallbladder or bile ducts)
  • Bile reflux (upward flow of bile from the small intestine into the stomach)
  • Bleeding
  • Infection
  • Blood clots
  • Small bowel obstruction (blockage in the small intestine)
  • Anastomotic leaks (leaks from the surgically reconnected region of the intestine)

When May TPN Be Used After Bariatric Surgery?

TPN isn’t generally used after bariatric surgery. That said, TPN may be an option when patients have severe surgery-related complications or malnutrition following surgery. 

TPN for Post-Surgery Malnutrition

Individuals who have had bariatric surgery may have malabsorption severe enough to cause malnutrition. They may be deficient in nutrients, including [2]:

  • Vitamins like A, D, B12, thiamine, and folic acid
  • Minerals like zinc, copper, and iron
  • Protein (often causing hair loss)
  • Fat 

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Moreover, some people who have undergone bariatric surgery may develop something called “failure to thrive” or FTT. This is a potentially life-threatening condition characterized by extensive weight loss, frequent vomiting, and malnutrition. 

Extremely low albumin (a protein) levels in the blood are another complication associated with certain types of weight-loss surgery [3].

TPN can help correct deficiencies and increase weight and serum albumin levels. In a 2016 study, researchers analyzed the data of 54 patients with a history of bariatric surgery who were given home parenteral nutrition (HPN). 

By the end of the HPN treatment, they found that participants who received HPN had significantly higher weight gain and serum albumin levels [4].

Considering these benefits, parenteral nutrition may be used to:

  • Correct nutritional deficiencies 
  • Increase serum albumin levels
  • Prepare patients for revisional bariatric surgery (surgical procedures after weight loss surgery to reduce side effects)

TPN for Surgery-Related Complications

An anastomotic leak is an early complication associated with bariatric surgery. It can cause bowel contents to leak into the abdominal cavity, potentially leading to infection. Leakage occurs from the surgically reconnected region of the intestine. 

TPN is often the most preferred feeding method in such cases because it allows anastomotic leaks to heal by providing rest to the gut. Some healthcare providers may find TPN easier to administer than other feeding methods [5].

TPN for Chronic Intestinal Failure After Bariatric Surgery

Individuals who have had weight loss surgery may be at a higher risk of developing chronic intestinal failure, which requires long-term parenteral support. 

Chronic intestinal failure is a long-term condition. Individuals with this condition cannot absorb the bare minimum of nutrients, water, and salts necessary to maintain health or growth. So, they require TPN for extended periods, until other remedial actions are taken. 

Note: Chronic intestinal failure is different from intestinal insufficiency, which doesn’t require parenteral support for health or growth. 

In these patients, TPN is well tolerated and effective regardless of the type of weight-loss surgery [6].

What Are the Side Effects of TPN After Bariatric Surgery?

Patient suffering from TPN side effects after bariatric surgery

Side effects experienced while receiving TPN after bariatric surgery are generally similar to those associated with TPN in other settings. These may include:

  • Nausea or vomiting
  • Increased urination
  • Swelling of the hands, feet, or legs
  • Stomach pain
  • Tingling in the hands or feet
  • Fever or chills
  • Mouth sores
  • Skin changes
  • Rapid weight loss or gain
  • Muscle weakness
  • Catheter obstruction 
  • Liver disease 
  • Osteoporosis (brittle bones)

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Duration of TPN After Bariatric Surgery

Due to limited data, it’s not possible to pinpoint the exact duration of TPN therapy after bariatric surgery. Moreover, several factors can affect the duration, the most important being the condition for which TPN is used. 

For example, TPN treatment may last several months in a patient with intestinal failure after bariatric surgery. On the other hand, patients with infection due to anastomotic leaks may need TPN for about a month or two. 

TPN After Bariatric Surgery: Frequently Asked Questions

What are the benefits of using TPN after bariatric surgery?

TPN after bariatric surgery can help correct nutritional deficiencies, improve albumin levels, and act as a “bridge” for revisional surgery. 

What are the dietary guidelines after bariatric surgery? 

The general guidelines recommend [7]: 

Do’s:

  • Eat a balanced diet (low in calories, fats, and sugars) with small portions.
  • Record your food portions and calorie and protein intake daily. 
  • Eat slowly and chew properly.

Don’ts:

  • Eat foods that are hard to chew. These include rice, bread, raw vegetables, fresh fruits, and meat.
  • Use straws, drink fizzy drinks, or chew ice. They can cause discomfort. 
  • Consume sugar, sugar-containing foods and beverages, concentrated sweets, and fruit juices.

During the first 60 days after surgery, maintain your daily calorie intake between 300 and 600 calories. Don’t consume more than 1,000 calories a day.

What is the most common cause of death after gastric bypass surgery?

Pneumonia and other respiratory conditions are most commonly associated with death after bypass surgery. Smoking, heart disease, and high blood pressure can increase the risk.

IVIG for Collagen-Vascular Disease-Associated Secondary Immunosuppression

Collagen-vascular disease is a group of conditions that cause chronic inflammation in the collagen (a key component of connective tissue) and surrounding joints. As there is no cure for these diseases, certain immunosuppressive therapies (e.g., corticosteroids, methotrexate, and cyclophosphamide) are commonly used to control the collagen-vascular disease activity. 

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However, prolonged use of these therapeutic agents frequently results in significant immunosuppression, also known as secondary immunosuppression, which increases susceptibility to pulmonary and systemic infections in patients.

In such cases, IVIG therapy is used as an immune-supportive therapy to reduce the risk of opportunistic infections. Read on to understand what collagen-vascular diseases are, how secondary immunosuppression develops, the evidence supporting the use of IVIG in this setting, and which patients may benefit most from IVIG therapy.

Collagen Vascular Diseases: Brief Overview

Collagen vascular diseases, also referred to as connective tissue diseases (CTDs), include a group of immune-mediated disorders such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM), mixed connective tissue disease (MCTD), rheumatoid arthritis (RA), and vasculitides. 

Most of these conditions arise from immune dysregulation, in which the body’s immune system mistakenly targets its own healthy tissues, causing inflammation. The symptoms of collagen vascular diseases can vary among patients, depending on the type of autoimmune disease they have. 

But most forms of collagen vascular disease do share some common symptoms, for instance, fatigue, muscle weakness, skin rash, body aches, fever, and joint pain. 

Understanding Secondary Immunosuppression in Collagen-Vascular Diseases

Secondary immunosuppression refers to a weakened immune system that happens not because the immune system is dysregulated, but because it is being suppressed, usually as a result of treatments used to control the autoimmune disease itself. 

Many people with collagen-vascular diseases are treated with strong immunosuppressive drugs (like steroids, cyclophosphamide, or newer biologic medicines). These treatments help reduce harmful inflammation but also make the immune system weaker to fight infections. 

Hence, in collagen-vascular diseases (or CTDs), the most common and clinically significant cause of secondary immunosuppression is long-term exposure to immunosuppressive medications. For example, high-dose corticosteroids reduce lymphocyte activity and impair antigen-presenting cell function, which increases the risk of uncontrolled growth of even weakly pathogenic bacteria, viruses, fungi, and mycobacteria.

Likewise, conventional immunosuppressive agents such as methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, and biologics (e.g., rituximab) suppress lymphocyte proliferation and antibody production, particularly immunoglobulin G (IgG), which further compromises immune defense.

Treatment-Induced Hypogammaglobulinemia

A key manifestation of secondary immunosuppression in collagen-vascular diseases is hypogammaglobulinemia (reduced IgG levels) that occurs as a result of:

  • B-cell depletion
  • Impaired plasma cell survival
  • Suppressed immunoglobulin synthesis

In fact, a retrospective study of pediatric patients with systemic lupus erythematosus (SLE) found that approximately 7% developed hypogammaglobulinemia (IgG < 500 mg/dL) during follow-up, indicating clinically relevant secondary immunosuppression.

Although collagen-vascular diseases are often associated with autoantibody production, total protective immunoglobulin levels may decline over time, particularly following prolonged immunosuppressive therapy. This creates a paradoxical state in which patients exhibit ongoing autoimmune activity alongside reduced effective humoral immunity, leading to an increased risk of recurrent infections.

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Clinical Consequences of Secondary Immunosuppression

Many patients with collagen-vascular diseases-associated secondary immunosuppression may experience serious clinical complications, including:

  • Hypogammaglobulinemia (a hallmark of secondary immunosuppression)
  • Recurrent respiratory infections
  • Opportunistic infections
  • Increased rates of hospitalization
  • Delayed recovery from infections
  • Limitations on the use of further immunosuppressive therapies

These complications often necessitate treatment modification or the addition of immune-supportive therapies, such as intravenous immunoglobulin (IVIG), to restore immune competence while allowing continued disease control.

Clinical Use of IVIG for Secondary Immunosuppression in Collagen Vascular Diseases

Various studies reported that IVIG therapy can help manage the clinical consequences occurring as a result of secondary immunosuppression in patients with collagen vascular diseases. 

IVIG in Hypogammaglobulinemia and Reduction of Infection Risk

Since IVIG supplies a concentrated and purified form of IgG antibodies, it can help restore serum immunoglobulin levels in patients with secondary hypogammaglobulinemia. This passive immunity prevents bacterial, viral, and opportunistic infections, which are a major cause of morbidity and mortality in collagen vascular disease patients. 

For instance, a retrospective study showed that most systemic lupus erythematosus (SLE) pediatric patients with low immunoglobulin levels received IVIG replacement to reduce infection risk or active infectious complications. 

Similarly, another retrospective cohort study of autoimmune disease patients treated with rituximab reported that many developed secondary hypogammaglobulinemia, and IVIG replacement was initiated primarily due to recurrent infections or high infection risk, rather than for autoimmune disease control. 

Hence, this and various other studies across autoimmune and inflammatory conditions demonstrate that IVIG significantly reduces the frequency and severity of infections in patients with secondary antibody deficiency, including those receiving B-cell-depleting therapies.

Immune Homeostasis Without Further Suppression

Unlike cytotoxic agents, IVIG does not increase infection risk; instead, it supports immune recovery while allowing continued disease-directed therapy when needed.

Patient Selection and Clinical Considerations

Though IVIG is not a first-line treatment for collagen-vascular diseases or for immunosuppression, it is specifically considered in patients who meet the following criteria:

  • Documented hypogammaglobulinemia
  • Recurrent or severe infections
  • Ongoing need for immunosuppressive therapy
  • Contraindications to further immune suppression

In research studies, optimal dosing for secondary immunosuppression typically follows replacement regimens (e.g., 400–600 mg/kg every 3–4 weeks), rather than high immunomodulatory doses used for autoimmune flares.

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Risks and Limitations of IVIG

Patient experiencing fever after IVIG treatment

IVIG is generally well-tolerated, but some people can have side effects, including:

  • Headaches
  • Fever
  • Fatigue
  • Nausea
  • Rarely more serious issues like allergic reactions or kidney stress

The Bottom Line

The standard treatments to control inflammation in patients also pose a unique challenge of secondary immunosuppression. While IVIG is not a cure for collagen-vascular diseases, it is being used as a supportive therapy for those with documented hypogammaglobulinemia and who are at risk of recurrent or severe infections. 

If you are considering IVIG, you should discuss the potential benefits and risks with your healthcare provider to determine if it is the right option for you.

Ixinity (Recombinant Factor IX): Uses, Dosage, Side Effects, and How It Works for Hemophilia B

Ixinity is a prescription medication that is given as an intravenous (IV) infusion to hemophilia B patients. Ixinity helps to manage and prevent prolonged bleeding caused by injuries, surgery, or even spontaneous internal bleeding in people with hemophilia B. 

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It is also known by its generic name, coagulation factor IX (recombinant), and received its first approval from the U.S. Food and Drug Administration (FDA) in April 2015 as an on-demand treatment for managing bleeds in hemophilia B patients (ages 12 years and older). Later, in March 2024, the FDA expanded Ixinity approval to include children younger than 12 years old with hemophilia B. 

If a healthcare provider has prescribed Ixinity to you or your child, then it is important to understand key information about this medication, including what it is, how it works, how it is used, and what to expect during treatment. 

Ixinity: Usage or Indication

Ixinity (coagulation factor IX) is commonly used to treat and prevent certain types of bleeding episodes in people with hemophilia B. These bleeding episodes can occur due to an injury or surgery, or without any clear cause. 

Ixinity is prescribed to both adults and children with hemophilia B under the following conditions:

  • To treat bleeding episodes when they occur
  • To manage excessive bleeding before, during, or after a surgical procedure
  • For routine prophylaxis to prevent or reduce the frequency of bleeding episodes 

You should NOT take Ixinity if you have hemophilia A with factor VIII deficiency. 

Working Mechanism 

To understand how Ixinity works, let’s first briefly review what hemophilia B is.

Hemophilia B is an inherited bleeding disorder caused by a deficiency in coagulation factor IX (a protein that causes blood clotting). This results in more frequent bleeding into joints, muscles, or internal organs, either spontaneously or as a result of accidental or surgical trauma. 

Ixinity is a lab-produced version of human coagulation factor IX that closely mimics the natural protein (factor IX) found in the body. When a person with Hemophilia B receives Ixinity, the infused recombinant factor IX enters the bloodstream and temporarily replaces the missing or low levels of natural factor IX.

Once in circulation, Ixinity helps the blood to clot more efficiently and prevents excessive blood loss in hemophilia B patients.  

Ixinity: Dosage Forms and Strengths

Ixinity comes as a lyophilized powder in single-use, color-coded glass vials that contain six different dosage strengths: 

  • Yellow vial: 250 international units (IU)
  • Blue vial: 500 IU
  • Green vial: 1,000 IU
  • Orange vial: 1,500 IU
  • Red vial: 2,000 IU
  • Brown vial: 3,000 IU

Ixinity lyophilized powder is mixed in the provided diluent (sterile water) before infusion. 

Note: IU (International Unit) tells how much clotting power a dose of factor IX provides.

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Dosage and Administration

The dose and frequency depend on the patient’s body weight (kg), severity of factor IX deficiency, extent of bleeding, and whether it is used for treatment or for surgery.

The recommended dose is first mixed with a diluent and administered through intravenous (IV) infusion (injected directly into a vein). 

1. Ixinity Dosage for Routine Prophylaxis

For routine prophylaxis, Ixinity is typically administered twice a week at 40-70 international units per kilogram of body weight (IU/kg), in people aged 12 years and older, and at 35-75 IU/kg for children younger than 12. 

Children often clear clotting factors more quickly, so their doses may be adjusted within this range to maintain good protection against bleeding. 

2. Dosage for On-Demand Treatment 

When Ixinity is used to treat active bleeding rather than for routine prevention, doses are calculated to raise the person’s factor IX level to a desired target based on the severity of the bleed. Treatment is given until healing is achieved. For:

  • Minor bleeds: Factor IX levels are raised to 30–60 IU/dl, with Ixinity given once every 24 hours for 1–3 days. 
  • Moderate bleeds: Factor IX levels are increased to 40–60 IU/dl, with dosing every 24 hours for 2–7 days.
  • Major or life-threatening bleeds: Higher factor IX levels of 60–100 IU/dl are needed, with Ixinity given every 12–24 hours for 2–14 days or longer under medical supervision.

3. Dosage To Control Bleeding Surrounding Surgery

Ixinity is also used to control bleeding before or after surgery in hemophilia B patients. For a minor surgical procedure, Ixinity is given every 24 hours after surgery to maintain factor IX activity levels at 30–80 IU/dl. 

For major surgical procedures, higher and more carefully controlled factor IX levels are required. Before surgery, levels are raised to 60–80 IU/dl. After surgery, factor IX levels are maintained at 40–60 IU/dl during the early recovery period, gradually reduced to 30–50 IU/dl, and then to 20–40 IU/dl as healing continues. 

Possible Side Effects

Patient suffering from headache from taking Ixinity

The most common side effect observed in >2% of patients in clinical trials was headache. Though it’s rare, Ixinity may also cause serious side effects, which include:

  • Allergic reactions: Signs of an allergic reaction may include swelling, hives, chest or throat tightness, low blood pressure, lethargy, nausea, vomiting, difficulty swallowing, abnormal sensations, wheezing, or difficulty breathing.
  • Kidney problems (nephrotic syndrome)
  • Blood clots (thromboembolism)

Warnings

Patients who have had a history of anaphylactic or severe hypersensitivity (allergic) reactions to hamster (CHO) protein, which is present in trace amounts in the Ixinity, should not take this drug. 

Some patients may develop neutralizing antibodies (inhibitors) to Ixinity that can reduce the effectiveness of factor IX treatments. If there is a chance of this happening, your healthcare provider may perform an assay to measure factor IX inhibitor concentration. 

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Precautionary Measures 

Always consult your healthcare provider before taking Ixinity if you:

  • Are pregnant or plan to become pregnant (because Ixinity may harm your unborn baby)
  • Are breastfeeding or plan to breastfeed
  • Have any allergies, including allergies to hamster (CHO) protein
  • Are taking any over-the-counter or prescription medications, especially  other coagulation factors like carfilzomib, efgartigimod alfa, or rozanolixizumab, supplements, or herbal products
  • Have or have had any medical problems
  • Have been told that you have inhibitors to factor IX (because Ixinity may not work for you)

Ixinity: Estimated Cost

Ixinity is generally expensive because it is a specialized biologic medication. However, some insurance plans and manufacturer programs may help reduce out-of-pocket costs for patients. For instance, Ixinity’s savings programs can provide up to $12,000 per year in support for people with commercial insurance, and there may be trial offers or assistance options available. Contact us if you are interested in exploring financial assistance opportunities for Ixinity.

IVIG for Major Burns Associated Secondary Immunosuppression

One of the most severe forms of trauma that you can experience is major burns. Beyond the visible damage to skin and tissues, major burns can deeply disrupt your immune system, causing secondary immunosuppression. As a result, you become more vulnerable to serious infections, sepsis, and multiple organ failure [1]. In fact, infections are one of the leading causes of death in major burn patients.

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Fortunately, over the years, burn care has improved a lot. Advances in surgical techniques, intensive care, and infection control have improved survival rates. Besides these advancements, intravenous immunoglobulin (IVIG) has emerged as an effective supportive therapy for managing burn-related immunosuppression. In this article, we’ll explore everything you need to know about IVIG for major burn-associated secondary immunosuppression.

Major Burns Associated Secondary Immunosuppression: Overview

In normal conditions, your skin acts as a physical barrier against bacteria, viruses, and fungi. In major burns (burns covering more than 20-30% of total body surface area), this barrier is severely compromised.

Besides this, just immediately after a burn, your body enters a strong inflammatory state. In this state, your body releases massive amounts of stress hormones and inflammatory substances. Although this response is initially protective, it does not last. Soon, your immune system becomes overwhelmed and overstimulated. As a result, immune cells such as T lymphocytes, B cells, and neutrophils either stop working properly or decrease in number [2].

This immune dysfunction is known as secondary immunosuppression. Patients in this state are at a much higher risk of developing infections. Their wound may also heal more slowly, and in severe cases, this can lead to life-threatening infections such as sepsis.

Signs and Symptoms of Burn-Related Immunosuppression

In major burn patients, secondary immunosuppression is not always apparent. However, the following warning signs may indicate its presence [1][2]:

  • Frequent or persistent wound infections
  • Frequent episodes of pneumonia or bloodstream infections
  • Delayed wound healing
  • Sepsis
  • Prolonged hospital or ICU stay
  • Increased need for antibiotics

Infections remain one of the leading causes of death in major burn patients. That’s why early immune support is very important.

Treatment Options for Major Burns Associated Secondary Immunosuppression

There is no single cure for major burn-related immunosuppression. The current standard of treatment focuses on infection prevention, treatment, and supportive care. Here are some of the standard treatment options [3][4]:

Early Wound Excision and Skin Grafting: Your doctor will promptly remove damaged tissues and perform skin grafting to reduce bacterial growth and lower the risk of infections.

Broad-Spectrum Antibiotics: Doctors use antibiotics to treat or prevent infections.

Nutritional Support: Diets rich in protein, vitamins, and micronutrients will support your immune function and promote wound healing.

Strict Infection Control Measures: Special infection control practices (sterile wound care, isolation when needed, etc.) in the burn unit help minimize infection risks.

Despite these standard measures, many patients still develop immune dysfunction. In such cases, intravenous immunoglobulin (IVIG) serves as an important supportive therapy. 

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How Does IVIG Help Patients With Major Burns Associated Secondary Immunosuppression?

A patient at home receiving IVIG for Major Burns Associated Secondary Immunosuppression

Intravenous immunoglobulin (IVIG) is a purified blood product that contains antibodies from thousands of healthy plasma donors. When given intravenously, it provides passive immune support. In major burn patients with immune dysfunction, IVIG can support the immune system in several ways:

It Restores Antibody Levels: Major burns can reduce antibody levels and weaken antibody function. IVIG provides ready-made antibodies that help neutralize bacteria and toxins [5].

It Reduces Infection Rates: Burn patients are highly vulnerable to serious infections such as sepsis. Studies suggest that IVIG can reduce the frequency and severity of infections by strengthening your immune system [6].

It Modulates Excessive Inflammation: Burn injuries trigger uncontrolled inflammatory cytokine release. IVIG helps regulate this response by reducing harmful inflammation [7].

It Improves Immune Cell Functions: IVIG enhances the function of key immune cells, such as neutrophils and macrophages [5].

It May Reduce Sepsis-Related Mortality: Some evidence suggests that IVIG may lower death rates in major burn patients by decreasing infection-related complications [6].

Overall, IVIG is not a replacement for standard burn treatment. But doctors can use it as part of a comprehensive treatment plan to significantly strengthen immune defense in patients with major burn-related secondary immunosuppression. 

Effectiveness of IVIG for Major Burns Associated Secondary Immunosuppression: What Studies Say

In recent years, many studies have been conducted on IVIG use in burn-related immunosuppression. However, the overall results remain mixed. Most of the evidence came from small clinical trials and observational studies rather than large, well-controlled trials.

Many studies have shown the benefits of using IVIG in patients with major burns. For example, one study concluded that patients treated with IVIG in addition to antibiotic therapy had a significantly lower risk of death due to infections [12]. Similarly, another study suggested that IVIG can reduce infection rates in burn patients [9]. A severe complication of major burn is sepsis. It is a life-threatening infection. According to a study, IVIG is an effective adjunct therapy in treating sepsis [8]. 

In addition to reducing infections, IVIG may help correct underlying immune deficiencies caused by major burns. Severe burns typically cause a rapid reduction in immunoglobulin (IgG) levels. A study suggested that IVIG administration can correct low IgG levels [11]. Also, some non-burn specific studies showed that IVIG can improve immune functions, such as enhancing neutrophil activity and balancing harmful inflammatory cytokines [7][10].

However, not all studies showed clear benefits. Some research found no significant difference in infection rates or mortality between patients who received IVIG and those who did not [11][13].

In summary, IVIG is not a universally effective treatment for all burn patients. However, the available evidence suggests it may be especially helpful in severely burned patients with frequent infections or signs of immune failure.

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Who Should Take IVIG for Major Burns Associated Secondary Immunosuppression?

IVIG is not suited for every burn patient. Your doctor will consider IVIG if you:

  • Have extensive burns and experience repeated infections
  • Had a history of low immunoglobulin levels
  • Have sepsis that does not respond to standard therapy
  • Show evidence of immune dysfunction

Risks and Limitations

While IVIG is generally safe, it is not without drawbacks. It can cause some side effects. Here are some of them [14]:

  • Headache
  • Nausea and vomiting
  • Chest and back pain
  • Fever
  • Muscle pain
  • Allergic or infusion-related reactions

In addition to side effects, IVIG has some limitations when used for major burn-related immune suppression. Here are some of them:

  • It is not a replacement for antibiotics
  • Benefits may vary between patients
  • Limited high-quality evidence in the burn population

The Bottom Line

Severe burns can weaken your immune system and cause life-threatening infections. IVIG may help some patients by supporting immune functions, but it is not recommended for all burn cases. When used carefully, IVIG can be a valuable addition to standard burn care.