Westley has been diagnosed with Duchenne Muscular Dystrophy.

About

Westley Groseclose is a typical boy very much like every other kid his age. He enjoys country music, Frank Sinatra, video games, being with family and using his wheelchair as an ATV. Westley has always been physically slower than his classmates but never gives up trying to keep up with them. In September 2010 we discovered the source of his physical limitations. Westley was diagnosed with Duchenne Muscular Dystrophy. Duchenne is a form of muscular dystrophy which causes rapidly-worsening muscle weakness that starts in the legs and pelvis, and later affects the entire body including his heart and lungs. It is caused by a defective dystrophin gene (a protein in the muscles). This protein is like the chocolate chips of a cookie. Those without Duchenne are Chocolate Chip Cookies and boys like Westley are Sugar Cookies. Westley’s body is missing the “chips” that make and keep muscles strong. If you met of him in a crowd of people you wouldn’t know his chips are missing. However; over time this disease will begin to limit his activity. Our family is committed to fighting this disease along with Westley by raising money for research and giving Westley all the love, fun and confidence to help him keep the positive outlook he has on life.

IMG_4762

Duchenne Muscular Dystrophy

From Wikipedia

Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy, which results in muscle degeneration, difficulty walking, breathing, and death. The incidence is 1 in 3,000.[1] Only males are affected, though females can be carriers. The disorder is caused by a mutation in the dystrophin gene, located in humans on the X chromosome (Xp21). The dystrophin gene codes for the protein dystrophin, an important structural component within muscle tissue. Dystrophin provides structural stability to the dystroglycan complex (DGC), located on the cell membrane.

Symptoms usually appear in male children before age 5 and may be visible in early infancy. Progressive proximal muscle weakness of the legs and pelvis associated with a loss of muscle mass is observed first. Eventually this weakness spreads to the arms, neck, and other areas. Early signs may include pseudohypertrophy (enlargement of calf and deltoid muscles), low endurance, and difficulties in standing unaided or inability to ascend staircases. As the condition progresses, muscle tissue experiences wasting and is eventually replaced by fat and fibrotic tissue (fibrosis). By age 10, braces may be required to aid in walking but most patients are wheelchair dependent by age 12. Later symptoms may include abnormal bone development that lead to skeletal deformities, including curvature of the spine. Due to progressive deterioration of muscle, loss of movement occurs, eventually leading to paralysis. Intellectual impairment may or may not be present but if present, does not progressively worsen as the child ages. The average life expectancy for patients afflicted with DMD varies from late teens to early to mid 20s. There have been reports of a few DMD patients surviving to the age of 40, but this is extremely rare.

 

10 Responses

  1. Heather Clary

    You guys are part of my family and family sticks together! If I or anyone of The Clary’s can help you in any way we are a phone call away. We will be wishing the best for Westley and all of you. I am sure there will be many trials along the way but know there are people out there that are thinking of ya’ll. With love, Heather, Mike, Jaden and Ashton.

    September 24, 2010 at 1:32 am

    • jessica

      Thanks Heather! Tuff road ahead but we WILL make the best of it.

      September 24, 2010 at 2:22 am

  2. Elizabeth Gerding

    We have cookies to bake, pumpkins to carve, fish to catch, balls to throw, wrestling matches to judge, marshmallows to roast over bonfires and many more things to explore together. All my love, hopes and prayers are with all of you each and every day.

    Love,
    Grammie

    September 24, 2010 at 12:18 pm

  3. Brian Sperberg

    “The man who removes a mountain begins by carrying away small stones.”
    Chinese Proverb

    Jess,

    I wish you, Westley, Wyatt, and Dallas peace, courage, joy, and inspiration on this journey. If there any “stones” that Francesca, my family, or I can help carry along the way, we would be grateful for the opportunity to support you – in any way whatsoever!

    Sincerely,
    Brian, Francesca, and the Sperbergs

    September 24, 2010 at 3:09 pm

  4. David & I send our prayers & continued support as you march forward to fight.
    May God give Westley & each of you the strength each day need to continue the battle.
    I am a first cousin to Betty Lee……a dear cousin to me.

    Gay Lee

    September 26, 2010 at 11:46 am

  5. sherrie dickerson

    You are in my daily prayers. Keep the faith and always believe in miracles.

    October 26, 2010 at 8:52 pm

  6. Kim Branch

    My son Carter was with Westley in Mrs. Johnson’s class. I remember Carter and Wesley making plans to go to the “Big City” (Richmond) together…….Please know Westley will be in our hearts and prayers.

    February 11, 2011 at 10:03 pm

    • jessica

      Hello! I remember that! They were going to see the trains or play with trains. Thank you for checking out Westley’s blog.

      February 12, 2011 at 2:05 am

  7. Jennifer Farmer

    I could not believe it when I read the memo from school. Westley and Sean are in class this year. I help out with the class alot and Westley is such a sweet kid. My heart breaks for Westley and the rest of your family. You are all in our thoughts and prayers.

    February 13, 2011 at 1:14 am

    • jessica

      Thank you Jennifter for saying what a sweet kid Westley is. We think so too. ;0)
      And thank you for your prayers and the time spent in the classroom. I appreciate the Moms that can spend time with our kids in class. Your time is very appreciated by our family. Westley was excited to know you purchased tshirts. As soon as they order is in I will let you know.

      March 3, 2011 at 9:32 pm

Leave a Reply