Early recognition of hemophilia symptoms in children and prompt treatment are essential to prevent lifelong disabilities. Symptoms can range from minor cuts to potentially life-threatening internal bleeding. 

What Is Hemophilia?

Hemophilia is a bleeding disorder that prevents blood from clotting as usual. Children born with this condition have little or no clotting factor in their blood. A clotting factor (coagulation factor) is a protein in the blood that helps blood clot. Consequently, children are more likely to have severe bleeding and other complications. 

The two main types are:

• Hemophilia A: Children either lack or have too little clotting factor VIII. 
• Hemophilia B: Children either lack or have too little clotting factor IX. 

Depending on the clotting factor levels, hemophilia may be [1]:

• Mild: 5% to 40% of clotting factor levels
• Moderate: 1% to 4% of clotting factor levels
• Severe: Less than 1% of clotting factor levels

Though incurable, hemophilia is treatable. With appropriate treatments, children with hemophilia can lead healthy lives. 

Recognizing Hemophilia Symptoms in Children

Hemophilia symptoms in children can vary depending on the severity of the condition. 

Many parents first notice hemophilia symptoms soon after their baby starts crawling or walking. Symptoms at this stage can include large bruises, swollen and warm joints, and avoiding using the affected arm or leg. Likewise, some parents may notice prolonged bleeding during vaccination, circumcision, or routine dental care. 

In mild cases, obvious symptoms may not appear unless severe bleeding occurs following surgery or injury. 

Hemophilia can cause external and internal bleeding. The signs of external bleeding can include:

• Bleeding in the child’s mouth from a cut, bite, or loss of a baby tooth
• Bleeding from a cut that stops for a short period, then continues
• Unusual bleeding from a minor cut
• Nosebleeds without any obvious cause
• Heavy periods in girls 

Internal bleeding means bleeding in the organs and muscles. When bleeding occurs in the bladder or kidneys, it can cause blood in urine (may be red or brown). Likewise, bleeding in the digestive tract can cause black, tarry stool or stool mixed with bright red blood. Large bruises may indicate bleeding into large muscles. 

Bleeding Into a Joint

Joint bleeding (hemarthrosis) is common in children with hemophilia. The most commonly affected joints are knees, ankles, and elbows. Joint bleeding may occur without a major injury. If a child with joint bleeding does not get immediate treatment, the affected joint may be permanently damaged. 

A baby with joint bleeding may be cranky for no obvious reason. Older children may describe having an “aura” or tingling in the joint before the external signs. Other signs and symptoms can include:

• Stiff, painful, swollen, and warm joints
• Inability to move or straighten an arm or leg

Bleeding in the Brain

Bleeding in the brain is the most serious complication of hemophilia. It is more common in children younger than two years. Notably, it is the major cause of death and disability in children with hemophilia. 

The signs of bleeding in the brain include:

• Seizures
• Constant vomiting
• Double vision
• Neck pain or stiffness
• Long-lasting headaches
• Sleepiness 
• Behavior changes
• Sudden weakness of arms or legs 
• Problems with balance and coordination

If your child shows any of these signs, particularly after a bump on the head, seek emergency medical care. 

Hemophilia Symptoms in Children: When To Call a Healthcare Provider

Seek immediate medical care if your child:

• Has a major injury 
• Passes out
• Vomits after a bump on the head
• Has seizures

Does Hemophilia Affect the Vaccine Schedule in Children With Hemophilia?

Children with hemophilia should receive all necessary vaccines according to the schedule, regardless of the severity of hemophilia. Your child’s doctor may administer a vaccine by injecting it under the skin (subcutaneous) instead of injecting it into the muscle tissue (intramuscular).

Sevenfact is an FDA-approved medication used to treat or control bleeding in patients with hemophilia A or B with inhibitors. In this article, you will learn about its mechanism of action, dosage, side effects, cost, and more.

What Is Sevenfact?

Sevenfact is a brand-name prescription medication. The active ingredient in this product is coagulation factor VIIa (recombinant)-jncw, also called eptacog beta. 

Sevenfact belongs to a class of medications known as hemostatics.

The term “recombinant” means this medication is made in a lab using recombinant DNA technology. The four lowercase letters “jncw” are used to distinguish Sevenfact from other similar products. 

The U.S. FDA approved Sevenfact in April 2020 to treat and control bleeding in individuals 12 years and older with hemophilia A or B with inhibitors. 

This product should not be used to treat individuals with congenital Factor VII deficiency.

What Is Sevenfact Used For?

Sevenfact is used to treat and control bleeding episodes in patients with hemophilia A or B who have developed inhibitors to standard clotting factor treatments.

Inhibitors are antibodies that develop in some patients and block standard clotting factor treatments, making bleeding more difficult to control.

Sevenfact Mechanism of Action

Recombinant coagulation Factor VIIa (rFVIIa) in Sevenfact helps the blood to clot by activating another clotting factor called Factor X. 

Normally, activation of Factor X depends on Factors VIII and IX. Sevenfact bypasses this pathway, allowing clot formation even when these factors are absent. For this reason, it is known as a “bypassing agent.”

Sevenfact Dosing

This medication comes as a white to off-white powder for solution (reconstitution) for intravenous (IV) injection in single-dose vials. 

The following strengths are available in the U.S.:

• 1 mg of coagulation factor VIIa (recombinant)-jncw
• 2 mg of coagulation factor VIIa (recombinant)-jncw
• 5 mg of coagulation factor VIIa (recombinant)-jncw

The liquid to prepare the solution comes in single-dose prefilled glass syringes. Each syringe contains 1.1 ml, 2.2 ml, or 5.2 ml of sterile water for injection. 

The dose and duration of treatment depend on:

• Location and severity of bleeding
• Need for urgent bleeding control
• Frequency of administration
• Response to bypassing agents during earlier bleeding episodes

Recommended Sevenfact Dosing:

Mild and moderate bleeding: 75 mcg/kg repeated every 3 hours until bleeding stops. Alternatively, a 225 mcg/kg initial dose may be used. If bleeding doesn’t stop within 9 hours, an extra dose of 75 mcg/kg every 3 hours may be given as needed to stop bleeding.

If bleeding doesn’t stop within 24 hours after the first dose, your healthcare provider will likely switch you to an alternative treatment. 

Severe bleeding: 225 mcg/kg initial dose. If needed after 6 hours, give 75 mcg/kg every 2 hours until bleeding stops.

The duration of treatment depends on the site and severity of bleeding. 

Sevenfact Side Effects

Common Side Effects

• Headache
• Dizziness
• Pain, redness, or swelling at the infusion site
• Fever
• Joint pain
• Mild itching 
• Nausea 
• Vomiting 

Serious Side Effects

Call your healthcare provider immediately if you experience any of the following signs:

Stroke

• Sudden numbness or weakness
• Severe headache
• Slurred speech
• Difficulty with vision or balance

Blood clot in the lung

• Chest pain
• Sudden cough or breathing difficulty
• Dizziness
• Coughing up blood

Blood clot deep in the body

• Pain, swelling, or warmth in one of your legs

Allergic Reactions

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

• Hives
• Breathing difficulty
• Swelling in the face or throat
• Chest tightness
• Fast heartbeat

Warnings and Precautions

This medication can cause severe or life-threatening reactions. Call your healthcare provider immediately if you experience:

• Hives, itching, or rash
• Chest pain or tightness
• Shortness of breath
• Wheezing
• Dizziness
• Low blood pressure
• Pain, swelling, and warmth in one leg
• Sudden numbness or weakness (particularly affecting one side of the body)
• Severe headache
• Slurred speech
• Problems with vision or balance

Before Using Sevenfact

You should not use this medication if you:

• Have a history of allergic reactions to rabbits or rabbit proteins
• Are allergic to this product or any product components

Before you receive your first dose of Sevenfact, inform your healthcare provider if you have or have ever had:

• Coronary artery disease (narrowing of the arteries that supply oxygen to the heart)
• Heart attack or stroke
• Severe injury or infection
• Allergic reactions to mouse, hamster, or cow proteins
• Liver disease

Also, inform your healthcare provider if you are:

• Pregnant or planning to get pregnant
• Breastfeeding or planning to breastfeed

Sevenfact vs. NovoSeven

Sevenfact and NovoSeven are coagulation factors. The differences are listed below:

Features	Sevenfact	NovoSeven
Approved Use	Hemophilia A or B with inhibitors in adults and adolescents 12 years and older	Adults and children with hemophilia A or B with inhibitors, congenital Factor VII deficiencyAdults with acquired hemophilia
FDA approval date	2020	1999
Active ingredient	eptacog beta	eptacog alfa
Available strengths	1 mg, 2 mg, and 5 mg  of coagulation factor VIIa (recombinant)-jncw	1 mg, 2 mg, 5 mg, or 8 mg recombinant coagulation factor VIIa (FVIIa)
Manufacturer	HEMA Biologics	Novo Nordisk Limited
Contraindications	Known allergy to rabbits or rabbit proteinsSevere allergy to this product or any product components	None

Sevenfact Cost

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.

The manufacturer of Sevenfact, HEMA Biologics, offers several support services through its “HEMA Biologics Cares” program for eligible patients. These include:

Sevenfact Co-pay Savings Program

Eligible patients may receive up to $12,000 in savings per calendar year through HEMA Biologics Cares. 

Patient Assistance Program (PAP) 

The Patient Assistance Program (PAP) provides this medication at no cost if patients meet specific income requirements. 

Quick Start Program

This program is for new users who are having an insurance coverage-related delay in getting their treatment. 

Bridge Program

This program is for current users who are having a delay in getting their treatment due to changes in their insurance coverage. 

Contact us if you are interested in exploring financial assistance opportunities or copay assistance for Sevenfact.