WHAT IS HEMOPHILIA?
Hemophilia is a rare and typically inherited bleeding disorder in which the blood doesn't clot normally.
If you have hemophilia, you may bleed for a longer time than others after an injury. You also may bleed inside your body (internally), especially in your knees, ankles, and elbows. This bleeding can damage your organs and tissues and may be life threatening.
The two main types of hemophilia are A and B. If you have hemophilia A, you're missing or have low levels of clotting factor VIII (8). About eight out of 10 people who have hemophilia have type A. If you have hemophilia B, you're missing or have low levels of clotting factor IX (9).
People with Hemophilia A or B tend to bleed mostly in the joints for reasons that are are not understood. They can also bleed into the muscles or soft tissue, or excessive bleeding from wounds or surgery.
The mission of the Orthopaedic Hemophilia Treatment Center (OHTC) is to provide comprehensive care for individuals suffering from bleeding disorders and the complications of those disorders, including full care for the blood transfusion transmitted disease.
We accomplish our mission using a multidisciplinary treatment philosophy that brings together hematologists, orthopaedic surgeons, nurses, social workers and physical therapists to support patients and their families.
There are only a small number of hematologists and orthopaedic surgeons in the United States who focus their efforts on coagulation disorders, which puts the OHTC in a select category of treatment centers dedicated to hemophilia and related disorders.
As the largest treatment center for adults (ages 14+) in Southern California, we offer a unique depth of expertise in all disciplines required to treat these blood coagulation disorders. We are dedicated to providing excellent, multi specialty care to patients with hemophilia and other coagulation disorders of any age or origin, as well as adults and adolescents who have acquired blood-borne infections including chronic hepatitis and HIV infection, through prior treatment with blood products.
The OHTC community includes a world renowned, multidisciplinary team of doctors, nurses, social workers, physical therapists and pharmacists – all of whom are dedicated to providing individualized treatment and care to individuals suffering from hemophilia and other bleeding and clotting disorders.
OHTC has been an innovator from the very beginning and was the first comprehensive hemophilia treatment center in Southern California when it opened.
Our training credentials are superb. Since 1970, OHTC has been designated as one of the first four International Hemophilia Training Centers.
OHTC’s expertise is backed by a history of pioneering work. For example, OHTC, was the first organization to successfully perform hip replacement surgery in patients with hemophilia.
Groundbreaking research, innovative customized physical therapy and genetic counseling for patients.
Orthopaedic Institute for Children conducts all of its inpatient care at UCLA Medical Center, Santa Monica. Our staff works closely with our counterparts at the hospital to provide state-of-the-art care.
The hospital is a joint venture between Orthopaedic Institute for Children and UCLA as a result of a strategic alliance with the Regents of UCLA signed in 1998.
The Coagulation Laboratory specializes in tests for inhibitors to clotting factors, but it is also equipped to assess levels of all the plasma clotting factors as well as diagnose von Willebrand’s disease, a genetic disorder caused by missing or defective von Willebrand factor (VWF), a clotting protein.
OHTC’s pharmacy is a part of the 340B program, which means that we are able to provide medications at a special discount. Furthermore, all the revenue generated by our pharmacy is used to support the hemophilia program.
In addition to the 340B program, OHTC is known for the excellent patient care that our pharmacists are dedicated to providing. Our pharmacists go to great lengths to review all medications for safety and efficacy, monitoring potential drug interactions and adverse drug reactions.
Our pharmacy’s commitment to patient care also extends to making life easier for patients by allowing patients who use our 340B program to utilize our home delivery of clotting factors and other pharmaceuticals.
The OHTC is approved by the State of California as a Genetically Handicapped Persons Program (GHPP) – a program that OHTC was instrumental in helping create. We are also a recognized California Children’s Services provider.
Hemophilia can be a crippling disease with a range of issues causing discomfort and problems for patients including bleeding in joints or limbs and deformities caused by chronic arthritis.
For more than 50 years, the Orthopaedic Hemophilia Treatment Center (OHTC) has been at the leading edge of treatment to help patients suffering from hemophilia and bleeding disorders live healthier, happier and pain-free lives.
OHTC pioneered the concept of providing a comprehensive, multidisciplinary team approach to the management of children and adults with bleeding disorders, and as a result the Center was designated an International Hemophilia Training Center in 1970 – one of the first four in the world — by the World Federation of Hemophilia.
Today the physicians at OHTC are highly trained specialists who are considered leaders in their field. Our physicians are at the forefront of their field and actively involved in hemophilia–based research and treatment, with several occupying leadership positions in national and international hemophilia organizations, as well as world-renowned speakers on the subject.
Even since the 1960s, a major function of the OHTC’s work has been dedicated to research and clinical trials of more effective concentrates for clotting factor substitutes. OHTC physician, Dr. Kasper was one of the first physicians to begin using a new plasma concentrate that was developed by a Los Angeles-based company and that same year, Dr. Vernon Luck at OHTC performed the first elective orthopaedic surgery under cover of the same concentrate with outstanding results.
The Center’s commitment to active participation in clinical concentrate trials continues even today under the direction of Dr. Doris Quon with trials for new recombinant factor VII(a), factor VII and factor IX products, as well as a variety of loner acting factor products. In addition, the HTC is currently recruiting patients for a gene therapy trial for patients suffering from severe Hemophilia B.
The disorders we treat include Hemophilia A and B, von Willebrand’s disease and other inborn deficiencies of plasma clotting factors and platelets, however our staff understand that hemophilia is a serious, chronic, life-long disorder and patients need more than just medical management.
For hemophiliacs, life can be scary and unpredictable because bleeding can happen at any time, disrupting life and causing prolonged periods of pain and disability, and that’s why our approach focuses on assisting patients by providing comprehensive care for including medical, social, psychological and orthopaedic care, as well as diagnostic testing and counseling.
We also recognize that each patient is different and so we customize treatment according to each patient’s needs including the diagnosis and evaluation of adult and pediatric patients suspected of having:
Sickle Cell Syndrome
Hemophilia associated bleeding or clotting disorders
Additional assistance in the form of counseling and testing is also provided to women from families affected by hemophilia who are at risk of being carriers.
Our objective is to help individuals suffering with hemophilia and associated bleeding and clotting disorders live informed, independent and high quality lives.
Hemophilia is a life-long disease that begins at infancy. From the beginning of their lives, hemophiliacs are in danger of injury and bleeding that can cause fear, insecurity, social isolation and discrimination.
At the Orthopaedic Hemophilia Treatment Center, our goal is to do more than manage the medical condition. Through specialized care, customized treatment and medication plans, counseling and social work we aim to help patients and their families understand and independently manage their disorder. In addition, our deep roots in research help us find new and better ways of diagnosing and helping patients.
We are more dedicated than ever to improving quality of life for children and adults with bleeding disorders.
Living with a bleeding disorder like hemophilia requires vigilance and planning. An injury or accident can strike at any time, and so it is important to pre-plan for these potential crises well in advance. Planning ahead will save valuable time and help you stay focused on getting you or your relative the help they need.View Subspecialty
Hemophilic arthropathy refers to permanent joint disease occurring in hemophilia sufferers as a long-term consequence of repeated hemarthrosis (bleeding into joints). Around 50% of patients with hemophilia will develop a severe arthropathy.View Subspecialty
Blood-borne infections, such as the HIV virus and hepatitis (B and C), were a major complication of treatment for hemophilia in the 1980s. In many cases these infections were transmitted through the factor concentrates and other blood products that were used to treat hemophilia due to the use of large blood donor pools to prepare factor concentrates. In addition, a lack of proper, specific tests for infectious agents contributed to the contamination of blood products used to treat hemophilia.View Subspecialty
The most dreaded complication of hemophilia treatment is the formation of an antibody to the normal clotting factor being given as therapy. These antibodies, called “inhibitors”, arise in approximately 30% of people with severe hemophilia A at some point in their lives.View Subspecialty
A joint is an area where two bones come together and joint damage is one of the most common complications of hemophilia. That’s because people with hemophilia can bleed into a joint space after an injury or even without a specific cause.View Subspecialty
Joint reconstruction or replacement is a surgical procedure that helps people who suffer from chronic pain, which may interfere with their daily activities. During this surgical procedure, the damaged joint and bone are removed and replaced with plastic and metal components.View Subspecialty
Physical Therapy (PT) can have two main roles in the treatment of hemophilia patients.View Subspecialty
Joint bleeding is the hallmark of patients suffering from hemophilia. Even though this bleeding can occur in any joint space, the most commonly affected joints are the knees, elbows and ankles.View Subspecialty
When OHTC was founded, only one treatment — plasma — was available to replace the missing or deficient clotting factors. Every patient, regardless of the type of bleeding disorder, received it. Because plasma sometimes caused allergic reactions, patients were infused only at OHTC.View Subspecialty
Testing is a valuable tool for the early detection of hemophilia. Women from families affected by hemophilia may want to know whether they are carriers and rightfully so. The sister of a man with hemophilia has a 50% chance of being a carrier, while the daughter of a sister has a 25% chance.
During testing, the gene mutation responsible for hemophilia is identified in DNA from the male with hemophilia followed by DNA testing of the female relative to see if they have the same mutation.
The Center also offers prenatal tests and diagnoses to families with carriers or who are concerned that they may be carriers. The major reason for prenatal diagnoses is to determine whether or not the fetus has hemophilia and the tests can help guide the manner of delivery of a hemophiliac infant as well as to determine whether OHTC’s on staff social workers and counselors need to be called in to provide psychological support to expecting parents.
Nowadays, women undergo Pre-Implantation Genetic Diagnosis (PGD) to avoid having an affected fetus. In PGD, the mother’s eggs are fertilized in vitro, the embryos are diagnosed and then an unaffected embryo is chosen for implantation. Several carriers from the Center – and hundreds nationwide – have had unaffected babies thanks to PGD.
No matter the situation, OHTC also has counselors on staff to provide counseling to carriers who may be concerned about options for reproduction.
Treatment of a bleeding disorder is very expensive given the high cost of factor concentrates and even people with good health insurance can be impacted by the financial impact of managing a bleeding disorder. At OHTC we provide extensive financial counseling to individuals and families so that every patient with a bleeding disorder can make the best choices regarding their health and their health insurance.
For hemophiliacs, life can be scary and unpredictable because bleeding can happen at any time, disrupting life and causing prolonged periods of pain and disability, and that’s why our approach focuses on educating and informing patients so that they know all their options and have all the information they need to take care of themselves or their relative.
When 17-year-old Bibiana Mashamba was 10, intruders ransacked her home in Tanzania and cut off her right leg and two fingers. As horrible as the incident was, it wasn’t the young girl’s first brush with tragedy. Bibiana and her sister, Tindi, both of whom are albino, also lost their parents to AIDS.
When Isabella Lopez was born, her neonatologist referred her mother, Claudia Munoz, to an orthopaedic specialist who diagnosed her baby girl with clubfoot and arthogryposis, conditions that tighten and twist her joints and keep her from moving easily. “At the time, I knew that she would need special care,” Munoz says. “But I didn’t know much about her conditions or the implications for her life.”
Imagine the peace of mind that comes with knowing expert medical care is nearby when your child needs it. Heather needed it for her beautiful daughter, Golden. Born prematurely, Golden spent 11 days in the hospital before the family went home together for the first time. Just three days later, Golden fell ill.
Jessica Chou always knew she was different. There was no lightbulb moment or sudden realization, she just knew that her cleft palate made her unique. “I knew since childhood that I was different somehow; I always had to see doctors, I got braces when I was very young, and I had to get surgery at several points in my life,” says Jessica, now 21.
Understanding Hemophilia A & B
Understanding von Willebrands Disease
Center for Disease Control (CDC)
CDC Bleeding and Clotting Disorders Surveillance
National Hemophilia Foundation
Hemophilia Foundation of Southern California
World Federation of Hemophilia
Western States Regional Hemophilia Network