Hemostasis and Thrombosis Center

The Hemostasis and Thrombosis Center at Children’s Hospital Los Angeles cares for young patients with bleeding and clotting disorders. With over 200 new patient referrals each year, the Hemostasis and Thrombosis Center is the largest coagulation center in the western United States. We serve as an international referral center for children with hemostasis and thrombosis disorders, providing excellent clinical care and research.

We see patients who have been admitted or newly diagnosed with a rare condition and may not have the resources available for treatment. Our multidisciplinary team of experts will continue to follow each child or adult through the outpatient clinic services, with 20 percent of our patients being adults.

Comprehensive Care

Our patients are evaluated and cared for by a multidisciplinary team of experts:

  • Clinical Neuropsychologist 
  • Nurse
  • Physician (Hematologist)
  • Physical Therapist
  • Social Worker

Our team works together to ensure that the whole patient is treated, including physical, mental and emotional needs of a child and their family. Once the patient is assessed, our team works together to create a patient-centered treatment plan. If located outside the Los Angeles area, we will work closely with the patient's primary care physician team for proper follow up care.

  • 340B Program
  • Clinic Schedule
  • Information for Health Professionals
  • Hemophilia Treatment Center
  • Insurance
  • Joint or Muscular Problems
  • Medical Alert ID
  • Research

The Hemostasis and Thrombosis Center’s 340B program is known as Hemophilia Outpatient Made Easy (HOME).

A 340B program is a federal program for the care and service of factor medicine needs for our hemophilia patients. Also known as the Public Health Service (PHS) Program, this program lets the Center purchase clotting factor and other specialized medicine for our patients at a discounted price. The income from the 340B program is used to provide and expand services to all our patients.

To learn more or to join HOME call 310-361-4141 or email HTCinfo@chla.usc.edu.

  • New patients:
    Call: 323-361-4100
     
  • Returning patients:
    Call: 323-361-4151

For general questions:
Call: 323-361-4141
Email: HTCinfo@chla.usc.edu

Night or weekend questions:
Call: 323-660-2450
Ask for the Hematologist on call.

 

Hemophilia and Bleeding Disorder Clinic

For all patients, please arrive 1 hour before your appointment and bring your insurance card.

Tuesday, 9 a.m. – 12 p.m.; 1 p.m. – 4:30 p.m.

Thursday, 1 p.m. – 4:30 p.m.


Hemophilia Orthopaedic Clinic

Located on the 4th Floor of the Outpatient Tower

2nd Tuesday in the months of February, April, June, August, October and December
1 p.m. – 4 p.m.

Need a referral from a Hemostasis and Thrombosis Center physician to attend this clinic.

For all patients, please arrive 1 hour before your appointment and bring your insurance card.


Thrombosis Clinic

Thursday, 1 p.m. – 4:30 p.m.

The Hemostasis & Thrombosis Center offers basic information for primary care providers regarding bleeding and clotting disorders:

Bleeding Disorders

  • Genetic (hemophilia or von Willebrand Disease)
  • Acquired (vitamin K deficiency or liver disease)

In the primary care setting, the vast majority of bleeding disorders are genetic and most (including all serious bleeding disorders) will present during childhood from as early as birth to the late teen years. Some mild bleeding disorders will present in adulthood usually following a hemostatic challenge such as surgery or trauma.

Important details every primary care provider should know about bleeding disorders:

  • Bleeding disorders are not uncommon and it is very likely that primary care providers will encounter such patients at least annually in their practice
  • The presenting symptoms can be simply stated as bleeding that is either:
    • Unusual (intracranial hemorrhage)
    • Excessive
      • Numerous bruises particularly in unusual places or that are large and/or palpable
      • Excessive bleeding following circumcision or dental work
  • The most serious conditions can often be detected by simple laboratory assays such as the PT and PTT. It should be noted that some serious conditions (factor XIII deficiency) and some mild though common conditions (von Willebrand Disease) may not be diagnosed by these tests
  • Be wary of accusations of child abuse merely for finding the excessive bruising. Unless other signs of abuse are clear, it is prudent to have the child evaluated for a bleeding disorder prior to making reports to the authorities
  • In any case where a bleeding disorder is suspected, refer to the Children’s Hospital Los Angeles Hemostasis and Thrombosis Center


Clotting Disorders

Clotting disorders can be either acquired or inherited.

Acquired

In children, the vast majority of blood clots (thrombosis) occur when a child has a serious medical condition such as cancer, heart disease, chronic gastrointestinal disorders and autoimmune disorders. Children will also develop thrombosis secondary to the use of a central venous catheter and most will present when hospitalized. Most of the children with thrombosis will not have a genetic component to their thrombosis and the treatment for the clot will be finite. These types of patients will present first to the hematologist rather than the primary care provider.

On rare occasions, children who are otherwise healthy will develop a clot and present to their primary care provider. This is a very serious and potentially life-threatening situation and immediate consultation with the Children’s Hospital Los Angeles Hemostasis and Thrombosis Center or referral to the Children’s Hospital emergency room is recommended.

With respect to adults, thrombosis is much more common and more often presents in the ambulatory setting to primary care providers or emergency rooms. The incidence of thrombosis increases steadily with age and is relatively rare prior to the age of 50 and increases dramatically after the age of 60. Similar to children, the presentation of a potential thrombosis to a primary care provider should prompt immediate consultation or referral to the emergency room (Children’s Hospital only if the patient is under age 21).

Inherited

A common scenario for a primary care provider is a question from a parent regarding the identification of a family member with an inherited predisposition to thrombosis (sometimes referred to as thrombophilia). Some of these disorders are very common.

Factor V Leiden

Factor V Leiden occurs in 2-10% of Caucasians depending on ancestry with the highest rates in the Middle East, tapering to lower rates in those of Southern European ancestry to even lower rates in those of Northern European ancestry. Patients who are genetically of pure African, Asia, Native American or Pacific Islander descent do not carry this genetic mutation. However, in the United States we have many patients of mixed ancestry/ethnicity and patients who among the groups above may still be found to have factor V Leiden.

Prothrombin Mutation

The prothrombin mutation occurs in 2-3% of Caucasians and has its highest incidence in people of Southern European descent with those of Spanish Descent seeming to have the highest incidence. The same logic regarding those of African and Asian descent applies to this mutation as for factor V Leiden.

Protein C Deficiency, Protein S Deficiency and Antithrombin

Protein C deficiency, protein S deficiency and antithrombin are less common clotting disorders and affect all races relatively equally. In total, between 5-10% of Caucasians and 1-2% of people of African, Asian, Native American or Pacific Islander descent will have a genetic predisposition to thrombosis.

Questions?

For patients and/or families that either have questions regarding clotting disorders or are requesting testing for the putative abnormality, we recommend a referral to the Children’s Hospital Los Angeles Hemostasis and Thrombosis Center.

At the Hemostasis and Thrombosis Center we can see both children and adults in our thrombosis outpatient clinic. We recommend referral to the Center prior to embarking on expensive laboratory test which often raise more questions than provide answers. Our expert staff will provide a thorough and detailed consultation and determine with the patient/family if testing should or should not be performed.

The following are important details every primary care provider should know about clotting disorders:

  • Most children with a blood clot will present while they are an inpatient and the vast majority will have a serious underlying condition
  • When children do present to a primary care provider with symptoms of a blood clot, it will manifest as pain and swelling in the leg (rarely in arm)
  • Girls will present signs more often than boys due to the use of oral contraception
  • For any child that presents symptoms which may be due to a blood clot, immediate referral to the Children’s Hospital Hemostasis and Thrombosis Center or Emergency Room is high recommended
  • For adults who present symptoms suggestive of a blood clot, immediate referral to an emergency room is highly recommended
  • In the primary care setting and increasing common scenario is the request for genetic testing for clotting disorders. We recommend that such patients/families be referred to the Children’s Hospital Hemostasis and Thrombosis Center prior to ordering expensive laboratory tests.

Hemophilia Treatment Center
We are home to a Hemophilia Treatment Center (HTC). HTCs provide comprehensive care for patients with hemophilia and other blood disorders. An HTC is a facility recognized by the Centers for Disease Control and Prevention (CDC) and Health Resources and Services Administration. We are one of about 130 HTC's in the United States.

Bleeding and clotting disorders in children are rare; therefore, most doctors including pediatricians, family doctors, emergency room doctors and other specialists may have never treated a child with hemophilia or a blood clot during their years of practice.

The Hemostasis and Thrombosis Center cares for patients with different types of insurance. This includes commercial, state and federal insurance. Learn more about insurance agreements that are accepted at Children’s Hospital Los Angeles.

  • Healthy Families
  • Medi-Cal
  • California Children’s Services (CCS)
  • Genetically Handicapped Persons Program (GHPP)

A child’s diagnosis and family income is one of the qualifications for CCS.  An adult's diagnosis and personal income is one of the qualifications for GHPP.

When a patient has a primary and secondary (supplemental) insurance usually most, if not all, of their medical costs are covered. But sometimes a patient or their family will have to pay part of the medical costs based on how much money they make.

For patients who cannot get state or federal insurance, the Healthcare Reform Act allows people with pre-existing conditions to apply to get commercial insurance.

Under Age 21
Many of our patients under age of 21 have Medi-Cal or Healthy Families insurance as well as California Children’s Services (CCS). For these patients, Medi-Cal or Healthy Families act as their main insurance. CCS is used as a secondary (supplemental insurance) for a specific health condition, such as hemophilia or von Willebrand Disease.

Age 21 and Older
Patients who are 21 and older need to apply for the adult version of CCS, Genetically Handicapped Persons Program (GHPP).

We conduct a special clinic with a surgeon from the Children's Orthopaedic Center for patients who have joint or muscular problems. 

The Center's comprehensive team is available during clinic hours and also 24/7 to answer questions and consult on medical issues.

If your child is in an accident, and wearing medical alert identification, this will let medical workers know if the child has a medical condition. It is important for anyone with bleeding or clotting disorders to wear a medical alert ID so that medical and emergency workers know what medications they can give and what injuries to look for.

Medical and emergency workers (doctors, paramedics, etc.) look for a medical alert ID tag indicating any health conditions or allergies when a child is hurt.

Medical alert tags can be worn as a bracelet, watch, neck chain or necklace. For parents with an infant or toddler with a bleeding or clotting disorder, it is important to put a medical alert tag on the child’s car seat and stroller in case the parent and child are separated. Do not have infants or toddlers wear medical alert neck chains or necklaces as they can be a choking hazard.

Medical alert tags can be purchased on their own or with a membership to a medical information organization.

The Children’s Hospital Los Angeles Hemostasis and Thrombosis Center team can assist families get a medical alert tag.

Contact us by phone at 323-361-4141, email: HTCinfo@chla.usc.edu or ask us in clinic.

The Hemostasis and Thrombosis Center actively takes part in clinical trials and research. Through clinical trials, better treatment had been found for bleeding and clotting disorders.

Our patients may be eligible for certain clinical trials. This can provide a way to get new treatments before they are approved. It is important to take part in clinical trials because it helps us discover new treatments or ways to care for patients with bleeding and clotting disorders.

Getting involved in clinical trials is voluntary. No patient or family will ever be forced to or coerced to join a trial. Before patients join a clinical trial, we make sure that the patient and their family understands the potential benefits and risks of the trial. Also, the patient and/or parent or guardian must sign an informed consent form before participating in a trial.

Talking about Your Child's Diagnosis

Talking about your child's diagnosis can be difficult. Caregivers may want to start talking to the child about their diagnosis as soon as possible and provide information appropriate for the child’s developmental level to help them accept and understand their diagnosis. ​

The Children’s Hospital Los Angeles Hemostasis and Thrombosis Center offers suggestions on providing an explaination:

  • When it comes to sharing the diagnosis, patients and families should decide whom and when to tell about their bleeding disorder. 
  • However, adults who may be responsible for the patient need to be informed about the patient’s bleeding disorder (e.g., teacher, babysitter, coach, camp counselor)

For School

  • Meet with school staff, including teacher, aide(s), principal, school nurse, other relevant school personnel
  • Know the facts and be prepared to answer common questions and/or correct misinformation
  • Consider possible reactions and how you may respond
  • It may be helpful to plan and rehearse your explanation in advance
  • Do not assume that the school nurse or other school staff knows about caring for students with hemophilia or other bleeding disorder care
  • Explain the basics of the bleeding disorder and how it affects your child (e.g., common sites of bleeding, signs or symptoms of a bleed, treatment, when to go to the emergency room)
  • Discuss the importance of exercise for healthy joints, as well as safe activities and proper protective equipment
  • Inform school staff that you will notify them if your child needs to avoid physical activities for any period of time
  • Giving teachers and staff pamphlets and fact sheets may be helpful in providing accurate information and correcting misinformation
  • Provide list of individuals and phone numbers to contact in case of an emergency
  • Request that school staff not announce the patient’s diagnosis to her or his class
    • It is important to let the patient decide what, when and to whom they wish to tell their medical information.
  • If the child self-infuses or you would like to infuse at school, request to store factor concentrate and infusion equipment in nurse’s office to be used in the event of a bleed
  • Instruct school staff not to provide aspirin for pain relief if child has a bleed.  Supply the nurse with acetaminophen or other appropriate pain medicine if needed during school hours.
  • Request a letter from Children’s Hospital Hemostasis and Thrombosis Center for school staff, explaining diagnosis, recommendations or request school consultation
  • Offer school staff the opportunity to speak to our staff

Babysitters or Caregivers

  • Know the facts and be prepared to answer common questions and/or correct misinformation
  • Consider possible reactions and how you may respond
  • It may be helpful to plan and rehearse your explanation in advance
  • Allow enough time for you to speak openly and without interruption
  • Follow-up conversations are helpful to answer additional questions and/or concerns
  • Explain the basics of the bleeding disorder and how it affects your child (e.g., common sites of bleeding, signs or symptoms of a bleed, treatment, when to go to the emergency room)
  • Sharing pamphlets and fact sheets helpful in providing accurate information and correcting misinformation
  • Provide list of individuals and phone numbers to contact in the event of an emergency
  • Instruct the person not to provide aspirin for pain relief if child has a bleed.  Supply them with acetaminophen or other appropriate pain medicine, if needed
  • Contact the Children’s Hospital Los Angeles Hemostasis and Thrombosis staff for additional recommendations or request a consultation

Telling Your Child's Friends

  • It is a child's choice as to what they wish to tell their friends 
  • Know the facts and be prepared to answer common questions (e.g., “Is it contagious?,” “Can you play like everyone else?”)
  • Keep it simple - you don’t have to tell them specific details
  • Consult with parents or the Children’s Hospital Los Angeles Hemostasis and Thrombosis Center staff of concerns about telling friends or have questions about how share the information 

Below are definitions for terms that you can use in discussing your child's condition with others.

  • Bleeding Disorders
  • Clotting Disorders
  • Fibrinolysis Disorders
  • Hemophilia and Exercise
  • Hemostasis
  • Platelet Disorders
  • Thrombosis
  • Von Willebrand Disease

Bleeding disorders are also known as disorders of hemostasis. Having a bleeding disorder means that a child’s body has problems forming blood clots to stop bleeding. As a result, the child will bleed longer than a child who does not have a bleeding disorder. Bleeding disorders can be genetic, passed down from one’s parents or acquired.

Symptoms of a Bleeding Disorder

  • Easy bruising
  • Bleeding that takes a long time to stop
  • Regular bleeds that last longer than 10 minutes
  • Frequent nose bleeds
  • Abnormal menstrual (period) bleeding

Rare Factor Deficiencies/ Rare Bleeding Disorders

The blood clotting system is made up of many different proteins that work together to help form a blood clot (a plug to stop bleeding) and prevent bleeding. 

  • Children who don’t have one of these clot forming proteins (factor) have a bleeding disorder. 
  • Common bleeding disorders are a deficiency in von Willebrand factor, a deficiency in factor VIII (8)  and a deficiency in factor IX (9).
  • Bleeding disorders caused by a lack of other clotting proteins are rare. These conditions are known as rare factor deficiencies or rare bleeding disorders.
  • All of these disorders can be diagnosed with specialized laboratory testing. There are also many ways that these disorders can be treated. It is best for a person with a rare factor deficiency to be treated at a hemophilia treatment center where doctors specialize in these conditions.

Congenital Afibrinogenemia and Hypofibrinogenemia

Fibrinogen is the protein that becomes the structure of the blood clot, a plug to stop bleeding, when bleeding occurs.

  • Congenital Afibrinogenemia
    A person with congenital afibrinogenemia has very little or no fibrinogen. Patients with this condition can have serious bleeding problems and other complications.
  • Diagnosis
    Patients with afibrinogenemia can be diagnosed shortly after birth. These patients usually have bleeding from the umbilical stump after it falls off in the first week or two of life. Otherwise patients can be diagnosed later in life with bleeding symptoms.
  • Symptoms of Congenital Afibrinogenemia:
    • Easy bruising
    • Nose and mouth bleeding
    • Excessive bleeding after minor injuries
  • Patients with congenital afibrinogenemia can have spontaneous (meaning without injury) rupture of their spleen. This can cause severe bleeding and be life-threatening if it is not treated. Women with congenital afibrinogenemia can have difficulty maintaining a pregnancy and many of these women will have miscarriages.
  • Treatment
    • Patients with congenital afibrinogenemia are treated with a product called Ristrap. This product comes from purified blood and contains fibrinogen. By replacing the missing fibrinogen a person is able to form a blood clot and stop bleeding.
    • Most patients will only need to receive this medication when they have an episode of bleeding. The medication can also be used to prevent bleeding if they are having surgery or are bleeding often.

Factor II (2) Deficiency or Prothrombin Deficiency

This disorder is very rare. There have only been about 25 documented cases in the medical literature of congenital prothrombin deficiency. A person can also become deficient in prothrombin as an acquired condition, but this is also rare.

  • Symptoms:
    • Patients with prothrombin deficiency have serious bleeding symptoms with bleeding occurring in almost any location, including bruises, nose bleeding and internal bleeding.
  • Treatment:
    • For patients with this condition they can be treated with an infusion of factor concentrate like hemophilia and congenital afibrinogenemia.
    • The type of clotting factor used to treat this condition is called a prothrombin complex concentrate or PCC. It is a purified blood product.

Factor V (5) Deficiency

Factor V deficiency is a very rare factor deficiency. There are very few reports of congenital factor V deficiency.

  • This deficiency can be acquired usually as a result of receiving topical thrombin. Topical thrombin is something that surgeons may used to control major bleeding in surgeries such as heart surgery or orthopedic surgery. Factor V deficiency can lead to any type of bleeding and can be severe.
  • Treatment:
    • The only way to correct a deficiency in factor V is with a blood product called fresh frozen plasma.
    • There are not any factor concentrates that contain factor V.
  • A platelet transfusion can also be used to boost factor V levels at the site of bleeding since platelets carry factor V.  Platelets are very small cells that are an important part of the clotting process to stop bleeding.

Factor VII (7) Deficiency

This rare factor deficiency is more common. Factor VII deficiency is found in about 1 out of 100,000 people.

  • In the severest form of factor VII deficiency patients do not have any factor VII in their body.
  • As a result they can have severe bleeding symptoms similar to a patient with hemophilia and can also result in internal bleeding.
  • Treatment:
    • Factor VII deficiency can be treated with a synthetic (not from human blood) concentrate called Novoseven or recombinant factor VIIa.

Factor X (10) Deficiency

  • This deficiency is rare and occurs in about 1 in 200,000-300,000 people.
  • Patients with the severest form of factor X deficiency, where there is no factor X in the body, can have bleeding in the brain.
    • Bleeding can also occur in other areas of the body.
  • If a patient is diagnosed with severe factor X deficiency, it is best that they receive treatment regularly as prophylaxis to prevent the possibility of bleeding in the brain.
  • Treatment:
    • Factor X deficient patients can be treated with the same prothrombin complex concentrates that treat factor II deficiency.

Factor XI (11) Deficiency

This is the most common factor deficiency, but because it is different from hemophilia, it is grouped with the rare factor deficiencies.

  • Who it Affects:
    • Factor XI deficiency occurs most commonly in people of Ashkenazi Jewish ancestry (Jews from Eastern Europe) but it can occur in people from any ethnic group.
  • Symptoms:
    • Patients with factor XI deficiency only bleed if they have trauma or surgery.
  • Treatment:
    • Patients only need to receive treatment when they have an injury or are going to have surgery. Treatment consists of a combination of fresh frozen plasma and aminocaproic acid or tranexamic acid. Factor XI concentrates are available in many parts of Europe but not in the United States.

Factor XIII (13) Deficiency

Factor XIII deficiency is extremely rare and is thought to occur in about 1 in 1 million people.

  • Symptoms: 
    • This deficiency can lead to severe bleeding in the brain and unfortunately, bleeding in the brain is often the first symptom. In some cases, babies will bleed excessively from the umbilical stump and if this occurs it is very important to check for factor XIII deficiency.
  • Bleeding can also occur in other locations as well. Bruising and mucus membrane bleeding is quite common. Due to the risk for bleeding in the brain, patients should receive medication on a regular basis as a form of prevention.
  • Treatment:
    • Treatment consists of a purified blood product called Corifact. Factor XIII lasts a long time in the blood and this treatment only needs to be given once a month. A synthetic factor XIII is being developed and should be available in the next year or two.

Clotting disorders are also called thrombophilia. Having thrombophilia means that a child is more likely to get a blood clot. Blood clots are also known as thrombosis. There are different types of blood clots:

  • Blood clots in arteries
  • Blood clots in veins
  • Pulmonary embolism

Blood clots can cause post-thrombotic syndrome.

A blood clot is a blockage in a child’s blood vessels. Blood vessels are divided into arteries and veins. Arteries carry blood full of oxygen and nutrients from a child’s heart to different parts or their body. Veins bring the blood with used up oxygen back to the heart and then to their lungs so that the blood can be re-oxygenated.

Blood clots in children are not common. For healthy children, only about 1 in 50,000-100,000 will get a blood clot. For teenagers, about 1 in 50,000 will get a blood clot and for young adults about 1 in 20,000 will get a blood clot.

In most cases, children who experience a blood clot typically have to a serious medical condition and/or a central line. A central line is a device that is inserted by a surgeon to provide direct access to the blood for giving medicines or drawing blood. Some names for central lines include Broviac or Hickman, Port or PICC line (peripherally inserted central catheter). However, there are times where blood clots occur in children who do not have a serious medical condition or a central line. These clots can be a result of an undiagnosed autoimmune disease, genetic conditions or birth control pills. Special blood tests are needed to test for genetic conditions.

The most common reasons, by age, for blood clots in children and young adults are:

Newborns

  • Central lines
  • Congenital heart disease
  • Rare and severe genetic blood clotting conditions

Infants and Toddlers

  • Central lines
  • Congenital heart disease
  • Serious infections of the blood, throat/neck, sinuses and bones
  • Cancer
  • Severe intestinal disorders

School Age Children

  • Central lines
  • Serious infections of the blood, throat/neck, sinuses and bones
  • Cancer
  • Autoimmune diseases
  • Severe intestinal disorders

Teenagers

  • Cancer
  • Central lines
  • Treatment with oral contraceptive pills (hormones or birth control pills)
  • Serious infections of the blood, throat/neck, sinuses or bones
  • Autoimmune disorders

Young Adults

  • Treatment with oral contraceptive pills (hormones or birth control pills)
  • Cancer
  • Autoimmune disorders
  • Serious infections of the blood, throat/neck, sinuses or bones


Blood Clots in Arteries

Pinky clot before

Pinky clot before treatment

pinky clot after

Pinky clot after treatment

When a blood clot happens in an artery, a part of a child’s body will not get oxygen and nutrients. The most common forms of blood clots in arteries are heart attack (myocardial infarction) and stroke. Both of these conditions can cause death and are the most common cause of death in adults.

Children can get strokes and clots in their arteries, but overall this is very rare. The Children’s Hospital Los Angeles Hemostasis and Thrombosis Center cares for children who have suffered from a stroke or other clots in arteries. We also provide ongoing care for patients to prevent any new strokes or clots.

Blood Clots in Veins

Blood clots in veins are more common for children. Most children who get blood clots in veins have other medical conditions including cancer, heart disease and autoimmune diseases. Children with severe diseases of the stomach and intestine that require special nutrition treatment, total parenteral nutrition (TPN) that is injected into a person’s veins, are at greater risk for a blood clot. Patients with these conditions usually have a central line, which enhances their risk of a blood clot.

For patients who have had several central lines, having blood clots in more than one blood vessel can make it very hard to place a new central line if the current central line stops working or gets infected. The Hemostasis and Thrombosis Center has developed effective treatment approaches for children who need long-term central lines and have had blood clots or when doctors have told them that it will be impossible to place a new central line.

Pulmonary Embolism

Blood clots in veins are an uncommon, but serious medical problem because clots can travel to the lungs causing a pulmonary embolism. A pulmonary embolism is when a blood clot clogs the main artery of the lung or one of its branches. Pulmonary embolism can be fatal among adults and children.

Post-Thrombotic Syndrome

pinky clot after

Post Thrombotic Syndrome

Blood clots in the veins can also lead to post-thrombotic syndrome (PTS).

Post-thrombotic syndrome happens when a vein is damaged by a blood clot so much that blood cannot flow back easily to the heart. This causes pain and swelling in the leg or arm where the clot was located. For a child that has had a blood clot in the neck and chest, PTS can lead to pain and swelling in the head. PTS is very hard to treat and the best way to manage PTS is to prevent it from occurring in the first place. This can be done by having the patient receive early and proper treatment for their blood clot. 

A disorder of fibrinolysis is a type of bleeding disorder. Fibrinolysis is the process of dissolving a blood clot after an injury has healed. There are several proteins that are involved in fibrinolysis and the most important protein is called plasminogen.

Plasminogen

In order to work, plasminogen needs to be activated by another protein called tissue plasminogen activator (TPA). If there is too much or too little of plasminogen or TPA, fibrinolysis will not work properly.

The best known condition that leads to problems in the fibrinolytic system is a lack of plasminogen activator inhibitor. If this protein is missing, the body cannot turn off TPA, and if there is too much TPA then blood clots formed normally to stop bleeding get dissolved too early. As a result, bleeding occurs.

This condition is very rare and has only been reported in a few families.

Treatment

  • Aminocaproic acid
  • Tranexamic acid

Both medications prevent blood clots from being dissolved by blocking the action of tissue plasminogen activator (TPA).

It is important for people with all types of hemophilia to exercise. When you are physically fit, you have enough strength, flexibility and endurance for your daily activities. Being physically fit helps prevent muscle and joint bleeds!

According to the National Center for Chronic Disease Prevention and Health Promotion, children and adolescents should have 60 minutes or more of physical activity daily. Exercising helps you keep a healthy weight and builds self esteem. Take part in simple activities like:

  • Taking the stairs instead of the elevator
  • Parking further from where you want to go 
  • Exercising as a family

These activities are a fun and easy fun way to bring exercise into you day without taking too much time and it should be FUN, FREQUENT and FAMILY FEASIBLE!

Barriers to exercising when you have hemophilia may include frequent joint or muscle bleeding. Full recovery from a joint bleed or muscle bleed should take place prior to starting or resuming exercise. Recovery may include the need for physical therapy to increase strength, flexibility and endurance.

Physical Activity, Sports and Hemophilia

For young children with all types of hemophilia, it is very important to let them try a wide range of physical activities and sports so they can find out what they like to do. Children with bleeding disorders may need to avoid certain activities and sports.

Most children with mild hemophilia participate in all kinds of activities. This includes sports like soccer or higher risk sports like surfing and skiing, but those with severe hemophilia may find that these activities lead to serious bleeding. 

Activities such as swimming, golf and hiking may be more suitable, especially if you have target joints or an inhibitor. Children with inhibitors may need a specific exercise program given by the Hemostasis and Thrombosis Center that minimizes or gets rid of frequent joint or muscle bleeds. Contact sports, such as football, rugby and ice hockey are not recommended for any type of hemophilia.

Children with bleeding disorders are just as likely to get motion injuries, especially in joints that have had previous bleeding. For example, several hours of playing active video gaming can produce injuries to the upper body.

Sports Protection

The best way to protect yourself or your child is to make sure they follow the latest safety guidelines for all children and young adults playing in sports. Children with hemophilia may need extra protective gear to allow safer participation. For example:

  • Helmets for bicycling, rollerblading, skiing and snowboarding
  • Shin guards for soccer
  • Knee pads  and ankle supports for basketball
  • Face mask for batting helmet for baseball

Contact the Children’s Hospital Los Angeles Hemostasis and Thrombosis Center to ask what type of protection a  child should wear when playing their chosen sport or activity.

Hemostasis is the process of how the body stops bleeding from a cut or injury.

Bleeding happens when a blood vessel is broken. The injury can be small, like a minor scrape, or large, like a deep cut that needs stitches. When a blood vessel is injured, the body’s hemostatic system helps to stop the bleeding. This system is made up of:

  • Blood vessels
  • Tissues under the blood vessels
  • Cells that are in the blood, such as platelets, red blood cells and white blood cells
  • Proteins that help blood clot

The four parts of the hemostatic system work together to stop bleeding by:

  • Forming a clot that closes the hole in the blood vessel
  • Repairing the opening in the blood vessel
  • Fixing the blood vessel so it works like it did before the injury

When the hemostatic system does not stop the bleeding, it may mean a person has a disorder of hemostasis, also known as a bleeding disorder.

Platelets are very small cells that are an important part of the clotting process to stop bleeding. When a blood vessel is damaged, platelets along with von Willebrand factor go to the damaged area to form a plug to stop the bleeding. When the platelets are actively forming a blood clot, they send out a chemical that calls for other platelets to help form a plug.

  • The normal amount of platelets in a person’s body is between 150,000 and 350,000.
  • When a child has less than the normal amount of platelets, they have a platelet disorder called thrombocytopenia, and it is important that consultation with a hematologist be done to figure out why the platelet number is low.
  • Sometimes, a patient can have a normal number of platelets, but the platelets don’t work well - these are called platelet function disorders or sometimes just platelet disorders.

Adhesion Disorders

An adhesion disorder is the result of the platelets not being able to stick to the wall of the damaged blood vessel to form a plug and stop the bleeding.

Bernard-Soulier Syndrome

Bernard-Soulier Syndrome is a rare disorder that is inherited or passed down from a child’s parent(s). A child with this syndrome has platelets that are large and missing a chemical that helps the platelets stick to the wall of a damaged blood vessel. Symptoms with this disorder range from mild to severe:

  • Bleeding that does not stop for 10 to 20 minutes
  • Mouth, dental or nose bleeding
  • Bleeding for a long time after surgery or an injury
  • Gastro-intestinal bleeding
    • Blood in vomit or stool
  • Women may have heavy menstrual periods or bleeding after child birth

Treatment:

  • Avoid aspirin or medicines affecting platelet function
  • Avoid activities or sports where there is a higher risk of injury 
  • Tell other health care providers that you or your child has a platelet disorder
  • Girls or women may need hormonal therapy to control their heavy periods
  • Epistaxis care for a nosebleed
  • Stimate (intranasal DDAVP) may be used in certain platelet disorders

Aggregation Disorders

An aggregation disorder is when platelets do not bind with fibrinogen and other proteins in order to stick to other platelets. As a result the platelets cannot form a plug to stop the bleeding from a damaged blood vessel.

Glanzmann’s Thromboasthenia

Glanzmann’s Thromboasthenia is a disorder that is inherited or passed down from a child's parent(s). This disorder causes moderate to severe bleeding symptoms:

  • Bleeding from the mouth
  • Bleeding with dental procedures
  • Nose bleeds
  • Bruising or small purplish red dots under the skin
  • Bleeding for a long time after an injury or surgery
  • Girls or women may have heavy periods
  • Infant boys may have bleeding after circumcision

Other Aggregation Disorders

People who have other aggregation disorders have a low amount or are missing chemicals needed to help platelets stick together. If the platelets cannot stick together, then they cannot form a plug to stop the bleeding from a damaged blood vessel.

Treatment:

  • Avoid aspirin or medications affecting platelet function
  • Avoid activities or sports where there is a higher risk of injury 
  • Tell other health care providers that you or your child has a platelet disorder
  • Girls or women may need hormonal therapy to control their heavy periods
  • Epistaxis care for a nosebleed
  • Stimate (intranasal DDAVP) may be used in certain platelet disorders

Secretion Disorders

A secretion disorder is when the damaged blood vessel takes more time for the bleeding to stop due to missing chemicals that signals the platelets to stick together. As a result, it takes a lot longer for the bleeding from a damaged blood vessel to stop. This is the most common platelet disorder.

Diagnosis and Treatment:

All of these disorders require specialists in bleeding disorders to diagnose them. The diagnosis is made by specialized blood tests that can be performed at Children's Hospital Los Angeles. Treatment can be done at home for most bleeding events with special medications. If your child displays symptoms of a platelet disorder, please contact our clinic for an appointment.

Thrombosis is the process of how blood clots. When blood changes from liquid to solid, it becomes a clot. Usually when a hematologist (i.e. a doctor who studies blood diseases) refers to thrombosis or pathologic thrombosis, it is meant that the body makes blood clots in blood vessels that are not broken. This leads to a block in blood flow. Thrombosis is one of the leading causes of death in the United States.

Thrombosis or blood clots can be caused by genetic or acquired conditions.

  • Genetic conditions, such as:  
    • Protein C deficiency
    • Protein S deficiency
    • Factor V Leiden 
  • Certain medications, especially birth control pills
  • Having a catheter or port in a blood vessel - known as a central venous catheter
  • Cancer
  • Surgery or trauma
  • Prolonged travel (more than 8 hours)
  • Immobilization

When a child is diagnosed with thrombosis, they will usually have one or more factors listed above. Patients may have thrombophilia if a genetic or acquired condition results in a blood clot. Thrombophilia is a serious health condition as it may cause a heart attack, stroke or pulmonary embolism.  

Von Willebrand Disease (vWD) is a bleeding disorder named after the man who discovered it, Erik Adolf von Willebrand. A child with vWD has a low level of the clotting factor von Willebrand factor (vWF). VWF helps blood form clots to stop bleeding from cuts or injury. When a child has vWD, their body has a hard time stopping a bleed.

VWD is the most common bleeding disorder that is passed down from a child's mother or father. About 1% of the general population has vWD. There are 3 types of vWD: type 1type 2 and type 3.

VWD affects males and females equally. Many with vWD go their entire life without being diagnosed. Women are diagnosed more often because of heavy periods (menstrual bleeding).


The Role of von Willebrand Factor

Von Willebrand factor (vWF) has two major jobs. One is to carry and protect the factor VIII(8) coagulation protein. The other job is to act as a glue to help blood platelets stick to the site of injury to help create a blood clot.


Types of von Willebrand Disease

There are 3 types of von Willebrand Disease (vWD):

Type 1

  • Most common form of vWD
  • von Willebrand factor is made in smaller amounts
  • Mildest form of vWD

Many have:

  • Nose bleeding
  • Gum bleeding
  • Heavy periods (menstrual bleeding)

Type 2

  • More bleeding than in type 1
  • von Willebrand factor doesn't’t work normally
  • Has four subtypes
    • 2A
    • 2B
    • 2M
    • 2N - Can look like mild hemophilia A

Type 3

  • Most severe form of vWD and a more serious bleeding disorder
  • Bleeding more often
  • von Willebrand factor is very low or absent


How Does a Child Get von Willebrand Disease?

von Willebrand Disease (vWD) is a genetic condition that is passed down from a child’s mother or father. Everyone is made up of two chromosomes and inherits them from their parents. Boys and men have an X and a Y chromosome. They get an X from their mother and a Y from their father. Girls and women have two X. They get an X from their mother and an X from their father. The von Willebrand gene occurs equally in men and women.

A mother with the vWD gene can have a:

                                          

A father with the vWD gene can have a:

A father and mother who have the vWD gene can have a:


Common Signs of von Willebrand Disease

  • Continued bleeding after dental work, especially after a tooth is removed
  • Easy bruising
  • Nose bleeds that last longer than 10 minutes 
  • Regular nose bleeds
  • Heavy or long  periods (menses) in girls and women
  • A history of any of the above in one or more of your family members


Diagnosis of von Willebrand Disease?

There are special blood tests that will find out if a child has von Willebrand Disease (vWD). These tests are usually done by a hematologist (a doctor who specializes in blood diseases). The blood tests measure how much von Willebrand factor a child has and how well it is doing its two jobs:

  1. Carrying factor VIII (8) by measuring factor VIII in the blood
  2. Acting as the glue for platelets

The test is called Ristocetin co-factor activity.


Difficulties in Diagnosis

Sometimes it is difficult to determine if a child has von Willebrand Disease (vWD). This is because there are many things that affect von Willebrand factor and factor VIII (8) in a child's body. For example, stress can raise the factor level in a child with vWD making the test results seem normal. Hormones can also affect a child’s factor levels. For this reason, girls should be tested on the first day of their menses (period) because this is when a girl's hormone levels are the lowest.

If there is a history of vWD in a child’s family or a strong history of bleeding, doctors may have to repeat blood tests a few times to find out if a person has vWD.


Treatment of von Willebrand Disease

There are several types of von Willebrand Disease. Each one affects how well blood clots. The type of treatment depends on:

  • Type of von Willebrand Disease (vWD)
  • Severity of bleeding
  • How a child responds to different treatments

Mild types of vWD

Desmopressin (DDVAP) is usually used as treatment. DDVAP is a nose spray that raises von Willebrand Factor (vWF) for a few hours.

For some type 2 and all type 3

Infusion into a vein (Intravenous) of a special medicine called factor that contains both factor VIII and vWF is usually used as treatment. This raises vWF for less than a day.

For severe bleeding

Multiple infusions into a vein (intravenous) of a factor product that has both factor VIII and vWF may be needed for treatment.


Von Willebrand Disease and Women

Von Willebrand Disease (vWD) can be more challenging for girls and women because of the risk for heavy periods (menses) and bleeding after childbirth. Once a girl or woman is diagnosed and they are given the correct treatment, these normal life events can be controlled safely.

Exercise and von Willebrand Disease

It is important for a child with von Willebrand Disease (vWD) to be in good physical health because it will lead to less bleeds.  This is especially true for people with Type 3 vWD.

Exercise can raise the level of von Willebrand factor (vWF). vWF and factor VIII are hooked together and flow in the blood.  vWF is released in the blood during moderate to high intensity exercise. As a result there is more vWF flowing in the blood.

"Perceived exertion” can indicate the intensity of certain exercises. Perceived exertion shows how hard the body is working. It is based on the physical feelings a child has during a physical activity. This includes:

  • Raised heart rate
  • Harder to breathe (raised breathing rate or respiration)
  • More sweat
  • Muscle fatigue or tired muscles

An example of exercising at a moderate to high intensity level, is breathing so hard while hiking up a hill, that one cannot talk to a friend.

Exercising helps a child keep a healthy weight and builds self esteem. Remember simple activities like:

  • Taking the stairs rather than the elevator
  • Parking further from a destination
  • Exercising as a family

These activities are a fun and easy way to bring exercise into the day without taking too much time.  Remember, exercise needs to be FUN, FREQUENT and FAMILY FEASIBLE!

For young children with all types of von Willebrand Disease (vWD), it is very important to let them try a wide range of physical activities and sports so they can find out what they like to do.

Most children with mild vWD participate in all kinds of activities. This includes sports like soccer, or higher risk sports like surfing and skiing. Children with Type 3 VWD may find that these activities lead to serious bleeding.

A valuable resource is the National Hemophilia Foundation’s (NHF) booklet, Playing It Safe: Bleeding Disorders, Sports and Exercise

Protection

The best way to protect yourself or your child is to make sure they follow the latest safety guidelines for all children and young adults playing in sports. For example:

  • Helmets for bicycling, rollerblading, skiing and snowboarding
  • Shin guards and a helmet for soccer

Contact our clinical team to ask specifically about what physical activities children can play and the about the necessary protection.