Q&A How to Who

A nurse is teaching a parent of a child who has hemophilia how to control a minor bleeding episode

Hemophilia (he mo FEE lee ah) is an inherited blood disorder. In hemophilia, a blood clotting factor is missing. In Hemophilia A, Factor VIII (8) is missing. In Hemophilia B, Factor IX (9) is missing.

Without these factors, blood will not clot well. People with hemophilia are born with the disorder. You cannot catch it from someone else. It lasts all of your life and it will not go away. Hemophilia occurs mainly in males but females can carry the gene that causes it and may or may not have bleeding problems. Some children with hemophilia have no family history of the disorder. There is no cure for hemophilia at this time, but there is medicine people can take. This medicine helps them stop bleeding so they can do most of the things everyone else does. By using the medicine and visiting a hemophilia treatment center regularly, the person with hemophilia can expect to live a long and happy life.

Why People with Hemophilia Bleed

Some people think a person with hemophilia can bleed to death from a small cut.This is not true. Usually small cuts and scrapes stop bleeding just fine.

A person with hemophilia has problems when a fibrin clot is needed to stop the bleeding. People with hemophilia do not have enough of either clotting factor 8 or 9. Because of this, the fibrin clot is not made or is so thin that the bleeding goes on.

The person with hemophilia does not bleed faster than someone without hemophilia. However, the person with hemophilia will bleed longer.

Treatment

The key to best outcomes is to prevent and treat bleeding. You do this by making good choices of activities and wearing good safety equipment such as seat belts, bike helmets and sports safety gear. If your child has bleeding, he may be given Factor VIII (8), IX (9), IV (into a vein). Refer to Helping Hand HH-II-17, IV Therapy. For mucosal bleeding (mouth/nose) he might be given an antifibrinolytic medicine (Amicar/Lysteda). Refer to Helping Hand HH-V-258, Tranexamic Acid (Lysteda).

Your child may need one or more doses of the clotting factor. Your child will receive the factor in the clinic, the hospital or as part of a home treatment program. Where the treatment is done depends on the extent of the bleeding and its location. For some children the nurse coordinator can arrange a home treatment program.

Ways to Help Prevent Bleeding

Here are some things you can do to help prevent bleeding:

Be sure to tell the doctor, dentist or nurse your child has hemophilia.
Be sure immunizations are given under the skin (subcutaneous) instead of into a muscle (intramuscular) and are given with a smaller needle. The staff should apply pressure and ice to the injection site for 5 to 10 minutes after the shot is given. This prevents bleeding.
Do not let your child get intramuscular (IM) injections (shots).
Never let a doctor or dentist do any procedure that involves opening the skin until you have talked with the Hemophilia team
People with bleeding disorders must talk to their doctor or nurse before taking medicine that can affect their platelets. These include aspirin, ibuprofen and naproxen. Always ask your hemophilia team before giving your child a medicine.
A toddler should wear a bicycle helmet when riding a bike (Picture 1).
Children with hemophilia should not play contact sports such as football, wrestling, boxing or hockey. If you have any questions about your child playing sports, be sure to talk with your hemophilia team.

Types of Bleeding and Treatment

Remember that a person with a bleeding disorder will not bleed faster than anyone else. However, the bleeding will last longer if not treated.

Early treatment of bleeding is especially important for people with hemophilia. They should follow these good rules for early treatment:
Take factor right away if you have an injury. This is especially important if a similar injury caused a bleed in the past.
Take factor as soon as you think you are bleeding.
Take factor as soon as you see or feel any signs of bleeding such as tingling, bubbling, warmth or swelling.
If you are not sure you are bleeding, take factor anyway.

Head or Neck Bleeding

If your child has any of the following symptoms, he should see a doctor right away. These could be signs of bleeding inside the head:

Headache with vomiting
Severe headache
Blurred vision
Sleeping a lot
Personality change or child's mood is different
Seizures

A child who gives himself factor at home should immediately give himself one dose of factor after a head injury. Then he should see the doctor.

Abdominal Bleeding

If your child has any of these symptoms of abdominal bleeding, call the doctor:

Severe abdominal (stomach) pain with no explained cause
Severe back pain
Blood in the urine or stool

Joint Bleeding

If your child has any of these complaints, he may have bleeding into a joint and will need clotting factor. Call the doctor right away.

Tingling or "bubbling" feeling in a joint
Stiffness and pain in a joint
Swollen, tender, warm and painful joint
Limited or painful movement of the joint

Soft Tissue or Muscle Bleeding

If your child has any of these signs of soft tissue or muscle bleeding, call the doctor:

A raised bump
Pain, swelling; area is warm to the touch
Trouble using the injured area

Mouth Bleeding

If your child has bleeding from the lips, gums or tongue, apply pressure and ice packs. If the bleeding does not stop, call the hemophilia team. Do not let him eat or suck a pacifier. Continue to hold ice packs over the cut until your child has been treated or until bleeding has stopped.

Cuts and Scrapes

Apply pressure over the cut for 5 to 10 minutes.
If the bleeding does not stop in 10 minutes, your child probably needs clotting factor.
If you think the cut needs stitches, take your child to the doctor.

Remember: Before your child gets stitches, he MUST be treated with clotting factor.

Daily Care

Your child should wear a medical identification bracelet or necklace. You can get it through your treatment center or local hemophilia chapter. It states that your child has hemophilia so if an emergency occurs, the doctor will know.
Regular tooth brushing, flossing and dental care are important. When he goes to the dentist, be sure to tell the dentist your child has hemophilia.

Giving Clotting Factor at Home

You can learn how to give clotting factor at home. Talk to the Hemophilia team about this.
As your child gets older and more mature, he can learn to give the factor to himself.

The Comprehensive Hemophilia Center

When your child is diagnosed with hemophilia, you will meet with the Hemophilia Team (doctor, nurse, physical therapist, psychologist, genetic counselor and social worker) to talk about your child's care.
Your child will have regular appointments at the Comprehensive Hemophilia Center. This clinic provides specialists in hematology, dentistry, orthopedic surgery, social work, clinical nursing, research nursing and physical therapy.

Phone Numbers for Emergencies

Contact the Comprehensive Hemophilia Center at (614) 722-3250 Monday through Friday, 8 a.m. to 5 p.m.

On weekends and holidays, call the Nationwide Children’s Hospital Operator at (614) 722-2000. Ask for the hematologist on call. The doctor will return your call and tell you what to do. If you are unable to reach the hematologist on call, take your child to the closest emergency room right away.

The longer you wait, the more severe the bleeding can become. Delay can increase the amount of damage and the length of treatment.

If you have any questions, be sure to call the Hemophilia Team.

Hemophilia (PDF)

A. Neutropenic*B.Bleeding precautionsC. ContactD. Droplet

Hemophilia is an inherited bleeding disorder. Children with hemophilia can’t stop bleeding because they don’t have enough clotting factor in their blood. Clotting factors are needed for blood to clot. Blood clots to prevent excessive bleeding.

There are many blood clotting factors involved in the forming of clots to stop bleeding. Two common factors that affect blood clotting are factor VIII and factor IX.

How severe your child’s hemophilia is depends on the level of blood clotting factors in his or her blood.

The 3 main forms of hemophilia include:

Hemophilia A. This is caused by a lack of the blood clotting factor VIII. About 9 out of 10 people with hemophilia have type A disease. This is also referred to as classic hemophilia or factor VIII deficiency.
Hemophilia B. This is caused by a deficiency of factor IX. This is also called Christmas disease or factor IX deficiency.
Hemophilia C. Some doctors use this term to refer to a lack of clotting factor XI.

Dr. Elias Zambidis cares for children suffering from leukemia and other blood disorders. His laboratory studies human pluripotent stem cells, which may one day be used to treat not only blood disorders, but also heart disease, vascular disease, cancer and autoimmune diseases.

Hemophilia types A and B are inherited diseases. They are passed on from parents to children through a gene on the X chromosome. Females have two X chromosomes, while males have one X and one Y chromosome.

A female carrier has the hemophilia gene on one of her X chromosomes. When a hemophilia carrier female is pregnant, there is a 50/50 chance that the hemophilia gene will be passed on to the baby.
- If the gene is passed on to a son, he will have the disease.
- If the gene is passed on to a daughter, she will be a carrier.
If the father has hemophilia but the mother does not carry the hemophilia gene, then none of the sons will have hemophilia disease, but all of the daughters will be carriers.

In about one-third of the children with hemophilia, there is no family history of the disorder. In these cases, it’s believed that the disorder could be related to a new gene flaw.

Carriers of the hemophilia gene often have normal levels of clotting factors but may:

Bruise easily
Bleed more with surgeries and dental work
Have frequent nosebleeds
Have heavy menstrual bleeding

Hemophilia C usually doesn’t cause problems, but people may have increased bleeding after surgery.

What are the symptoms of hemophilia?

The most common symptom of this disorder is heavy, uncontrollable bleeding.

The severity of hemophilia depends on the amount of clotting factors in the blood. Those affected with hemophilia that have levels greater than 5% (100% being average for unaffected children) most often have bleeding only with major surgeries or tooth extractions. These children may not even be diagnosed until bleeding complications from a surgery occur.

Severe hemophilia is when the factor VIII or IX is less than 1%. Bleeding can occur in these children, even with the minimal activities of daily life. Bleeding may also occur from no known injury. Bleeding most often occurs in the joints and in the head.

Your child's symptoms may also include:

Bruising. Bruises can occur from even small accidents. This can result in a large build up of blood under the skin causing swelling (hematoma). For this reason, most children are diagnosed around 12 to 18 months of age. This is when the child is more active.
Bleeds easily. A tendency to bleed from the nose, mouth, and gums with minor injury. Bleeding while brushing teeth or having dental work often suggests hemophilia.
Bleeding into a joint. Hemarthrosis (bleeding into a joint) can cause pain, immobility, and deformity if not treated. This is the most common site of complications due to hemophilia bleeding. These joint bleeds can lead to chronic, painful, arthritis, deformity, and crippling with repeated occurrences.
Bleeding into the muscles. Bleeding into the muscles can cause swelling, pain, and redness. Swelling from excessive blood in these areas can increase pressure on tissues and nerves in the area. This can cause permanent damage and deformity.
Bleeding in the brain from injury or spontaneously. Bleeding from injury, or spontaneously in the brain, is the most common cause of death in children with hemophilia and the most serious bleeding complication. Bleeding in or around the brain can occur from even a small bump on the head or a fall. Small bleeds in the brain can result in blindness, intellectual disability, a variety of neurological deficits. It can lead to death if not spotted and treated right away.
Other sources of bleeding. Blood found in the urine or stool may also signal hemophilia.

The symptoms of hemophilia may look like other problems. Always check with your child's doctor for a diagnosis.

How is hemophilia in children diagnosed?

he diagnosis of hemophilia is based on your family history, your child's medical history, and a physical exam. Blood tests include:

Complete blood count (CBC). A complete blood count checks the red and white blood cells, blood clotting cells (platelets), and sometimes, young red blood cells (reticulocytes). It includes hemoglobin and hematocrit and more details about the red blood cells.
Clotting factors. To check the levels of each clotting factor.
Bleeding times. To test the speed that blood clots.
Genetic or DNA testing. To check for abnormal genes.

How is hemophilia treated?

Your child's healthcare provider will refer you to a hematologist, an expert in blood disorders. Your child’s health care provider will figure out the best treatment based on:

How old your child is
His or her overall health and medical history
How sick he or she is
How well your child can handle specific medications, procedures, or therapies
How long the condition is expected to last
Your opinion or preference

Treatment depends on the type and severity of the hemophilia. Treatment for hemophilia is aimed at preventing bleeding complications (mainly head and joint bleeds). Treatment may include:

Bleeding in the joint may need surgery or immobilization. Your child may need rehab of the affected joint. This may include physical therapy and exercise to strengthen the muscles around the area.
Blood transfusions may be needed if major blood loss has occurred. This is when your child gets donated blood.
Self-infused factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.

What are the complications of hemophilia?

Complications of hemophilia include:

Bleeding in the joints or muscles
Inflammation of the joint lining
Long-term joint problems
Very serious tumor-like enlargements, of the muscle and bone
Development of antibodies against clotting factors
Infections from transfusions (HIV and hepatitis B and C are no longer transmitted in donated blood)

How is hemophilia managed?

With careful management, many children with hemophilia can live relatively healthy lives with a normal lifespan.

Managing your child's hemophilia may include:

Taking part in activities and exercise, but avoiding those that may cause injury. These include soccer, rugby, wrestling, motocross, and skiing.
Receiving special care before surgery including dental work. Your child's doctor may advise factor replacement infusions. These increase the child's clotting levels before the procedures. Your child may also get the specific factor replacement infusions during and after the procedure. These maintain the clotting factor levels and to improve healing and prevention of bleeding after the procedure.
Preventing dental and gum problems with proper dental hygiene.
Getting immunizations under the skin instead of in the muscle to prevent bleeding in the muscle.
Avoiding aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs).
Wearing medical identification (ID) in the case of an emergency.

When should I call my child's healthcare provider?

When you should call will vary based on how severe your child's condition is and what treatment he or she is getting. Since hemophilia is a long-term condition, talk with your child's healthcare provider about when you should call or get medical treatment for your child.

Call your child's healthcare provider if your child:

Is injured
Has pain
Has bleeding that you cannot control
Is scheduled for surgery or another procedure

Key points about hemophilia in children

Hemophilia is an inherited bleeding disorder. It causes an affected child to have low levels of blood clotting factors.
The most common symptom of hemophilia is increased, uncontrollable bleeding.
Giving factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.

Next steps

Tips to help you get the most from a visit to your child’s health care provider:

Before your visit, write down questions you want answered.
At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.