Clotting Disorders

girl in the hospitalClotting and Bleeding Disorders
“A clotting disorder can mean that your blood doesn’t clot enough, or it may clot too much. Both can cause medical emergencies. Too much clotting in a blood vessel in your head can cause a stroke, even in a young child,” says Dr. Nugent, CHOC pediatric hematology specialist. “Alternatively, not enough clotting results in a bleeding disorder, like Von Willebrand disease which is common, occurs in about 1 in 100 people, and affects boys and girls. Boys tend to get frequent nosebleeds and bruising when they are younger and girls can have a problem with heavy menstrual periods that can make them anemic.” Von Willebrand disease is the most common bleeding disorder in the U.S. affecting both men and women, while hemophilia is a well-known X-linked genetic bleeding condition affecting primarily boys.

Treatment at Home
Many children with clotting and bleeding disorders are diagnosed as babies and can be successfully treated at home. The clotting protein or factor that they are missing in their blood can be produced by genetic engineering and concentrated in a small vial, so patients can get an intravenous infusion. This treatment corrects the defect for several days so the children can participate in normal activities without fear of bleeding, Dr. Nugent says. “Family education is critical with all bleeding and clotting disorders. Families should know how to treat their child in all situations if there is a problem. We want families to know more than most doctors know about their child’s rare bleeding disorder. We want to empower patients to be independent and knowledgeable so they can regularly treat themselves at home to prevent bleeding.”

Helping the Family
Dr. Nugent says it’s common that young patients with bleeding or clotting disorders will have other family members — both other children and adults — who have a hereditary blood disorder that has never been diagnosed. Help is available, she says. “If you or someone in your family may have a blood or bleeding disorder and it’s a child, contact CHOC. Adults should call the Center for Inherited Blood Disorders next door to CHOC.
The website is c3dibd.org or they can call 714-221-1200.”

Fast Facts

  • Estimated Number of Hemophilia Patients in the U.S.: 20,000-25,000
  • Number of Americans who die each year from abnormal blood clots: 600,000+

View the full feature on Kids and Clotting Disorders

Dr. Nugent
Dr. Diane Nugent
CHOC Pediatric
Hematology Specialist

Physician Focus: Dr. Diane Nugent

Dr. Nugent is a nationally recognized expert in pediatric hematology. Her clinical and research interests include blood disorders, bone marrow failure, bleeding and clotting disorders, and white cell and immune deficiencies. Dr. Nugent is Chair of Hematology and medical director of the Blood and Donor services at CHOC Children’s, as well as the Hematology Advanced Diagnostic Lab. Dr. Nugent completed her residency and internship at Denver Affiliated Hospitals in Denver. Dr. Nugent received her fellowship training at Children’s Hospital Medical Center in Seattle and the Fred Hutchinson Cancer Research Center in Seattle.

Dr. Nugent’s philosophy of care: “I take a family-centered approach and educate families to help them understand what they have and how to care for it. The family is key for the diagnosis, treatment and education, and optimal outcomes. I want them to be empowered and independent.”

EDUCATION:
David Geffen School of Medicine at the University of California, Los Angeles

BOARD CERTIFICATIONS:
Pediatrics
Pediatric Hematology-Oncology

More about Dr. Diane Nugent

This article was featured in the Orange County Register on July 28, 2014, and was written by Amy Bentley.

Treating Hemophilia Today and Tomorrow

Much has changed in the ways of treating hemophilia – an inherited bleeding disorder in which a patient’s blood does Hemophilia_CHOCnot clot properly – and even more changes are on the horizon, says a CHOC Children’s hematologist.

“Within just the last 20 years – one generation – is when we began having factors to treat the patients with hemophilia,” says Dr. Diane J. Nugent, chair of hematology and medical director of hematology and Blood and Donor Services at CHOC Children’s. Dr. Nugent also is the medical director of the Hematology Advanced Diagnostic Lab.

Today, hemophilia is treated by replacing the missing blood clotting factor so the blood can clot properly. This is done by administering factor concentrates into a vein. Today, patients can perform these infusions themselves at home to prevent and stop bleeding episodes and enjoy a better quality of life, she says.

“Thanks to therapy, kids can play sports, attend school like any other child and live a full and complete life,” Dr. Nugent says. “Just a generation ago, by the time they were adults, patients with hemophilia were disabled. Today’s treatments are safe and lots of people can do this at home with no problems. This gives the patients more independence.”

Those with hemophilia are typically treated twice a week at home, but new treatments for some patients reduce the frequency. Some patients can now treat themselves as little as once a week because of a new long-acting factor to correct their bleeding.

A new long-acting product called Alprolix treats about one out of six patients who have a type of the condition called hemophilia B. In addition, a long-acting factor for different but common type of hemophilia will be coming out soon as well, Dr. Nugent says.

“Long-acting factors for hemophilia patients will reduce the frequency of IV pokes and will greatly improve their quality of life because now they will only need to treat themselves once a week,” she says.

Because hemophilia is a genetic disorder, specialists hope the condition will one day be corrected through gene therapy.

“Those genetic trials are ongoing at Stanford University and starting with adults,” Dr. Nugent says “We hope that will trickle down to kids in the next decade.”

According to the Centers for Disease Control, the median age in the United States for diagnosis 3 for those with mild hemophilia, 8 months for those with moderate hemophilia, and 1 month for those with severe hemophilia. In most cases, there is a family history of hemophilia.

CHOC always needs new donations of blood and platelets to help patients with hemophilia. To donate blood or platelets, call 714-509-8339 or email donatebloodforkids@choc.org to make an appointment.

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Video: Thank You, Blood Donors!

An important part of the CHOC Children’s family is Blood and Donor Services, the department that helps collect blood products for our patients.

Did you know that one pint of blood can save two lives? And your donation at CHOC goes directly to a child.

I’d donate if I could, but unfortunately, my blood type — B for bear — isn’t what the patients need. Instead, I donate hugs and offer endless praise and thanks for the generous people who give blood at CHOC.

Check out this fun video to learn more about the numbers that drive Blood and Donor Services at CHOC.

By the Numbers: Blood and Donor Services

January is National Blood Donor month, but CHOC Children’s appreciates its blood donors all year long. After all, blood products are needed each and every day to help care for patients.

Let’s look at the numbers that drive Blood and Donor Services at CHOC:DSCF8506

365 – Days a year that blood is needed at CHOC

100 – Percent of blood donations at CHOC that help kids

10 – Average number of pints of blood in an adult body

2 – Lives saved by 1 pint of blood

65 – Percent of CHOC’s total blood needs met by donations

35 – Percent of CHOC’s total platelet needs met by donations

1 million – Dollars CHOC spends annually to purchase blood products

42 – Days that red cells last after donation

5 – Days that platelet products last after donation

200 – Average number of blood donations per month at CHOC

50 – Average number of platelet donations per month at CHOC

6 – Times per year that donors can give whole blood

45 – Minutes the blood donation process lasts at CHOC

3 – Cookie choices available to donors at CHOC

Have you given blood before? Donating at CHOC is fast and painless thanks to expert and efficient staff. Even better, your donation directly helps children served by CHOC.

The department is open Monday through Friday, and same-day appointments are often available. Call 714-509-8339 or email donatebloodforkids@choc.org to schedule an appointment.

Related content:

  • Clotting Disorders
    “A clotting disorder can mean that your blood doesn’t clot enough, or it may clot too much. Both can cause medical emergencies. Too much clotting in a blood vessel in ...
  • Treating Hemophilia Today and Tomorrow
    Much has changed in the ways of treating hemophilia – an inherited bleeding disorder in which a patient’s blood does not clot properly – and even more changes are on ...
  • Video: Thank You, Blood Donors!
    An important part of the CHOC Children’s family is Blood and Donor Services, the department that helps collect blood products for our patients. Did you know that one pint of blood ...

CHOC Blood Donors: Like Father, Like Son

Since 2006, father and son Jerry and Jeremy Wilcox have had a standing date every eight weeks at CHOC Children’s:  Together, they roll up their sleeves and donate blood.

“We both have the mindset that if we can help people, then we help people,” Jeremy says.  “We may not always have the money to donate to organizations, but we do have time and we do have the ‘good veins.’ Because of that, we are just doing what we can to help those in need.”

The father-son tradition of giving life together is particularly significant with Father’s Day this weekend – as well as the nearing of summertime, which is traditionally a season of low blood donations at CHOC Children’s and blood centers nationwide. At CHOC Children’s, donation rates are typically 20 percent lower during this time.

The Wilcox men’s multi-generational tradition of donating blood extends well past 2006: Jerry’s own father was a frequent donor, which inspired Jerry to begin donating in college. Jerry had a similar influence on Jeremy.

In addition to providing a way to help others, the Wilcox men’s regular donations allow the duo an opportunity to take a break from their busy lives and catch up.

“I enjoy it,” Jerry says. “It’s a chance for us to see each other at least every eight weeks. We walk in, there’s no wait. We get to talk for an hour, and we get great cookies.”

Jerry began donating at CHOC Children’s in 2005 as a participant in the hospital’s Designated Donor Program, which allows blood donations to be directed to a specific patient.

Jeremy’s long tradition of blood donation began in high school. Now the father of two small children, he has a finer understanding of the significance of blood donations in a pediatric setting.

“If my kids got sick, they’d come to CHOC,” he says. “CHOC takes care of me, and I have all the confidence that they would take great care of my children.”

Despite the steadfast commitment from the Wilcox men and other blood donors, CHOC Children’s is in desperate need of blood donations of all types year round. Donations meet just 65 percent of the hospital’s annual need, and blood platelet donations meet just 35 percent of CHOC’s need. To supplement donations, CHOC spends more than $1 million annually to purchase necessary blood.

“Just try donating once,” Jeremy says. “It doesn’t hurt, and it gives you a warm fuzzy feeling that you could really help someone.”

Learn more about blood donation.

Related articles:

  • Clotting Disorders
    “A clotting disorder can mean that your blood doesn’t clot enough, or it may clot too much. Both can cause medical emergencies. Too much clotting in a blood vessel in ...
  • Treating Hemophilia Today and Tomorrow
    Much has changed in the ways of treating hemophilia – an inherited bleeding disorder in which a patient’s blood does not clot properly – and even more changes are on ...
  • Video: Thank You, Blood Donors!
    An important part of the CHOC Children’s family is Blood and Donor Services, the department that helps collect blood products for our patients. Did you know that one pint of blood ...

January is Blood Donor Awareness Month

January is Blood Donor Awareness Month. The Blood & Donor Services program at CHOC Children’s provides comprehensive blood therapy for children. This ranges from blood donor collection services to therapeutic procedures. The program offers designated donor and autologous donor programs for both blood and platelet products to support children undergoing surgery, cancer treatment, and various other conditions.

CHOC relies on volunteer blood donors like you, your friends and co-workers to meet these needs. Donating blood is a safe and easy process, and all blood types are needed.Volunteers who donate can leave knowing they will have a direct impact on the recovery of a child.

Check out these interesting facts from the American Red Cross:

  • 1 pint of blood can help save up to 3 lives.
  • Adults have around 10 pints of blood in their body. 1 pint is given during a donation.
  • Blood cannot be manufactured; it can only come from volunteer donors.
  • 5 million patients in the U.S. need blood every year.
  • Every 2 seconds someone needs a blood transfusion.
  • The average red blood cell transfusion is approximately 3 pints.
  •  A single car accident victim can require as many as 100 pints of blood.
  • There are four types of transfusable products that can be derived from blood: red cells, platelets, plasma and cryoprecipitate. Typically, two or three of these are produced from a pint of donated whole blood.
  • Platelets, critical for cancer patients, have a shelf life of about 5 days.

To donate blood to CHOC patients, please call 714-509-8339. For more information, please visit http://www.choc.org/programs-services/blood-donor-services.

Related articles:

  • Clotting Disorders
    “A clotting disorder can mean that your blood doesn’t clot enough, or it may clot too much. Both can cause medical emergencies. Too much clotting in a blood vessel in ...
  • Treating Hemophilia Today and Tomorrow
    Much has changed in the ways of treating hemophilia – an inherited bleeding disorder in which a patient’s blood does not clot properly – and even more changes are on ...
  • Video: Thank You, Blood Donors!
    An important part of the CHOC Children’s family is Blood and Donor Services, the department that helps collect blood products for our patients. Did you know that one pint of blood ...