Bushkill Valley Motorcycle Club
About Us
CYSTIC FIBROSIS
From Wikipedia, the free encyclopedia
Cystic fibrosis (CF), also known as mucoviscidosis, is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys and intestine. Long term issues include difficulty breathing and coughing up sputum as a result of frequent lung infections. Other symptoms include sinus infections, poor growth, fatty stool, clubbing of the finger and toes, and infertility in males among others. Different people may have different degrees of symptoms.
CF is an autosomal recessive disorder. It is caused by the presence of mutations in both copies of the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR).Those with a single working copy are carriers and otherwise mostly normal. CFTR is involved in production of sweat, digestive fluids, and mucus.When not functional usually thin secretions become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth take place in some areas of the world.
There is no cure for cystic fibrosis. Lung infections are treated with antibiotics which may be given intravenously, inhaled, or by mouth. Sometimes the antibiotic azithromycin is used long term. Inhaled hypertonic saline and salbutamol may also be useful. Lung transplantation may be an option if lung function continues to worsen. Pancreatic enzyme replacement and fat soluble vitamin supplementation are important, especially in the young. While not well supported by evidence, many people use airway clearance techniques such as chest physiotherapy. The average life expectancy is between 37 and 50 years in the developed world. Lung problems are responsible for death in 80% of people.
CF is most common among people of Northern European ancestry and affects about one out of every three thousand newborns. About one in twenty five are carriers. It is least common in Africans and Asians. It was first recognized as a specific disease by Dorothy Andersen in 1938, with descriptions that fit the condition occurring at least as far back as 1595. The name cystic fibrosis refers to the characteristic fibrosis and cysts that form within the pancreas.
How the Endure Came About
The Eckerts
One of our Bushkill Valley Motorcycle Club members, Derek Eckert and his wife, Daralin, were expecting their first child. (Derek is also a AMA Pro-Hillclimber, and Amateur racer.) Preliminary testing showed there was a good chance the child may have down syndrome. After the 20 week follow-up with their Doctor, Daralin went for an amniosantesis and genetic testing to confirm. They felt relieved to learn their baby did not have down syndrome but alarmed that both parents had passed the gene of Cystic Fibrosis to the baby. Wanting to know more, the Eckerts started researching as much as they could about Cystic Fibrosis and prepared for the worst. Being scared and worried, they explained the news to a few members that are close friends.
Daralin wanted to start a walk to raise awareness for Cystic Fibrosis. She asked a group of fellow members and friends to help organize and participate in the walk. Fellow member and friend, Tim Varga, suggestively stated "We are a motorcycle club, and we dont walk, we ride". Tim decided that a ride is what should be done. Tim and a few fellow members approached the club Board of Directors and started the ball rolling to have a charity race and donate all funds raised from the race to Cystic Fibrosis in the Eckerts' babies name.
On March 9th, 2014, Daralin Eckert gave birth to Wyatt Chase Eckert. Wyatt does not have Cystic Fibrosis, but the tremendous stress and effort that went into research and the worry of the expecting parents did not go unnoticed. The club decided to still host the event and donate to the worthy cause.
Using BVMC's abandoned motocross track and Hill Climb property, just outside of Bethlehem, PA, the members made a competitive track using access trails, return trails and untamed woods. The following groups came forward with very generous donations, support, and give aways to make our first annual Endure for a Cure successful, RRP Cycle Stuff, Harmony Property Maintenance, TSI Competition Engines, Mad Brothers Cycles, Suburban EMS, Geakers Taco's, Debonaire Hair Designs and Palmer Trophy, and many others. The Endure for a Cure was a such a great hit with the members in our club that they decided to go forward and make it an annual event.
Daralin Eckert, Derek Eckert, and their Son, Wyatt Eckert
