July 2016 Newsletter

HCC Welcomes New Executive Director
The Hemophilia Council of California’s Board of Directors is pleased to announce the appointment of Jillian Hacker as the organization’s new Executive Director

CCS Protections Bill Passes Committee
We are pleased to report that Senate Bill 586 (Hernandez) passed out of the Assembly Health Committee on June 28 on an 18-0 bipartisan vote and now moves to the Assembly Appropriations Committee to be heard in August after the July California State Legislative Summer Recess…

Findings of Inhibitor Study Published in NEJM
The detailed findings of the much anticipated SIPPET (Survey of Inhibitors in Plasma-Products Exposed Toddlers) study were published in May…

Read the latest Hemophilia Council of California Newsletter here!

Read it in Spanish!

2016 California Coastal Ride For Hemophilia

2014 california coastal ride riders

2016 marks the 15th year of the California Coastal Ride for hemophilia and other bleeding disorders. The CaliforniaCoastal Ride for Hemophilia is a fitness challenge benefiting all four California Hemophilia Chapters & the Hemophilia Council of California. Cycle over 500 miles from San Francisco to San Diego in one week for hemophilia, von Willebrand Disease and other bleeding disorders!

The ride begins with a kick-off dinner on Saturday, August 13 in San Francisco and concludes on Saturday, August 20 in San Diego. Registered riders embark on a seven-day journey along California’s majestic Highway 1 for an experience like no other. From the rugged mountains of the north to the bustling beach cities of the south, you will see it all!This ride is truly a coastal ride adventure where you will test your ability and challenge yourself each day. You will ride through the coastal towns of Santa Cruz, Big Sur, Monterey, San Simeon, Pismo Beach, Santa Barbara, and Dana Point.

The Hemophilia Council of California (HCC) is a collaboration of the four hemophilia chapters serving California. HCC’s mission is to support the four California hemophilia chapters and improve the quality of life of people with bleeding disorders. HCC coordinates the advocacy concerns of chapters and individuals with bleeding disorders, runs leadership programs and organizes the annual legislative day to give a voice in the state Capitol.

 

Single Factor Deficiency

There are ten clotting factors that are necessary in forming a blood clot. Deficiencies in factors VIII and IX are well known to most people, but what of the other factor deficiencies? Not everyone is as familiar with these conditions because they are diagnosed so rarely To date, deficiencies in eight of the lesser known coagulation factors (Factors I, II, V, VII, X, XI, XII and XIII) have been documented in the medical literature. Many of these disorders were only discovered or described within the last 40 years.
In most cases, rare factor deficiencies are not genetically sex-linked. They occur in equal frequency among men and women. By and large the gene is passed down in an autosomal recessive fashion. This means that when the factor deficiency is inherited from only one parent, the child will be a carrier of the condition, but usually not have symptoms. It is possible for people to inherit a gene from both parents, but this happens very rarely and usually means a more severe manifestation of the disease.
Not all factor deficiencies have the same severity. Not everyone with these disorders needs treatment. However for those who do, the treatments available for people with rare factor deficiencies are not optimal. Many people in the United States with rare factor deficiencies need to take fresh frozen plasma, prothrombin complex concentrates (PCCs) or cryoprecipitate.
Some of the rarer factor deficiencies (such as Factor VII and XIII) do have other treatment options. As always, consult your hemophilia treatment center physician.

Platelet Disorders

Platelets serve a critical role in coagulation. Functional platelet disorders are rare, occurring in fewer than 1 in 1 million people.
Bernard-Soulier syndrome (BSS) – An inherited disorder in which platelets are deficient in glycoproteins and are unable to bind to blood vessel walls.
Gray Platelet syndrome – The platelets of a person with gray platelet syndrome lack the ability to store proteins in alpha granules.
Delta Storage Pool Disease – Resulting from a disorder of dense granules that slows platelet activation and constriction of blood vessels
Glanzmann thrombasthenia – A disorder characterized by a deficiency of platelets to clump together in a clot.

Von Willebrand Disease

Von Willebrand Disease is a bleeding disorder caused by a defect or deficiency of a blood clotting protein, called von Willebrand Factor. The disease is estimated to occur in 1% to 2% of the population. The disease was first described by Erik von Willebrand, a Finnish physician who reported a new type of bleeding disorder among island people in Sweden and Finland.
Von Willebrand Factor is a protein critical to the initial stages of blood clotting. This glue-like protein, produced by the cells that line the blood vessel walls, interacts with blood cells called platelets to form a plug which prevents the blood from flowing at the site of injury. People with von Willebrand Disease are unable to make this plug because they do not have enough von Willebrand Factor or their factor is abnormal.
Most fall into the following classifications:
  • Type I: This is the most common and mildest form of von Willebrand Disease. Levels of von Willebrand factor are lower than normal, and levels of factor VIII may also be reduced.
  • Type II: In these people, the von Willebrand factor itself has an abnormality. Depending on the abnormality, they may be classified as having Type IIa or Type IIb. In Type IIa, the level of von Willebrand factor is reduced, as is the ability of platelets to clump together. In Type IIb, although the factor itself is defective, the ability of platelets to clump together is actually increased.
  • Type III: This is severe von Willebrand disease. These people may have a total absence of von Willebrand factor, and factor VIII levels are often less than 10%.
  • Pseudo (or platelet-type) von Willebrand disease: This disorder resembles Type IIb von Willebrand disease, but the defects appear to be in the platelets, rather than the von Willebrand factor.

Hemophilia

Hemophilia is caused by a deficiency of clotting factor in the blood which causes prolonged bleeding from mild injuries. The kind of bruise any child could sustain during play may trigger internal hemorrhaging. In severe cases, internal bleeding can start without apparent cause, spreading into joints and tissues. Swelling and intense pain usually result and with the repetition of such episodes, the person with hemophilia becomes crippled by his own blood.
The most common form of hemophilia is hemophilia A, which is caused by a lack of deficiency of factor VIII, a blood plasma protein. Hemophilia B, the next most common form, is caused by a lack or deficiency of the factor IX blood plasma protein.
Hemophilia is an inherited bleeding disorder, occurring most often in males. Approximately 1 out of every 4,000 American males will be born with the disease, inherited through the mother. Hemophilia can occur in any family, even one without a known history of the disease.
The treatment of hemophilia is extremely expensive. Because persons with hemophilia have a virtually normal life span, they must bear the catastrophic costs for a lifetime. The cost of blood products and other hemophilia related medical expenses will vary from person to person. Studies have shown that the average cost to an individual for blood products alone is over $150,000. On occasion, complications of hemophilia have caused annual medical expenses to exceed $500,000.
Other factor deficiencies and platelet disorders (such as vonWillebrand’s Disorder) may occur in both males or females. The prevalence of these dysfunctions may be much higher.

Covered California

Covered California’s mission is to increase the number of Californians with health insurance, improve the quality of health care for all of us, reduce health care coverage costs and make sure California’s diverse population has fair and equal access to quality health care. You will have the ability to choose the health plan that offers the best services at the greatest value for you – insurance that can’t be dropped or denied if you have a pre-existing medical condition. click here

HCC Future Leader Program

May 9-11, 2016

The Future Leader Program is a three-day program designed to teach young people how to advocate for themselves and their community. This program is open to men and women with bleeding disorders ages 14-22 and their siblings. For more information on this program, click here.

ATTENTION PATIENTS AND CLINICIANS:

This is a friendly reminder for patients to alert the Genetically Handicapped Persons Program (GHPP) 60 days prior to your 21st birthday that you would like to apply for the GHPP to ensure seamless, uninterrupted coverage for your hemophilia treatment and services. Below is the link to the GHPP which includes the new FAX numbers and other important information:

http://www.dhcs.ca.gov/services/ghpp/Pages/default.aspx

Celebrate World Hemophilia Day With Us!

April 17, 2016
On World Hemophilia Day 2016, inspire others with your story of living with or treating a bleeding disorder and show your commitment to achieving treatment for all. This year’s theme celebrates supporting positive change for people with bleeding disorders and encouraging others to do the same. By working together and inspiring each other for a brighter future, we can achieve Treatment for All.