....and life goes on!

February 10, 2012 has just marked the 3rd anniversary of Cruz's kidney transplant and he has been so busy since then.

Shortly after his surgery, Cruz and his family went on a Disney Cruise for a week courtesy of the Make a Wish Foundation.  Cruz mentioned that while he was on this trip he forgot all about being sick.  He is so brave.

Cruz is currently in 5th grade and is an A-B student.  He plays the drums and is in the school band.  His dwarfism has not slowed him down one bit.  He enjoys riding his 4-wheeler, hunting with his dad, and shooting his compound bow.  He has many friends and has sleep-overs all the time.

Cruz has only encountered a few incidences of bullying since he's been getting older and his height difference is more noticeable to the other kids.  Cruz has a strong spirit and he says it doesn't bother him.

Cruz continues to take several medications everyday and he also gets blood work done every Monday to keep a close eye on his creatinine level.  His levels have been up and down forcing him to have 3 kidney biopsies within a 2 month timeframe.  He tolerated the biopsies well.

Cruz does suffer from joint pain, achey legs, and occasional pain in his hands.

His facial features are different in comparison to an achondraplasia dwarf.  Notice that his forehead isn't enlarged, the bridge of his nose isn't flattened compared to the picture of the girl below his picture.  Cruz does have slightly bowed, short legs and short arms.

For the rest of Cruz's life he will be put in situations where his height will be a disadvantage.  So far, he has no problem asking his friends to reach the paper towel dispenser in the bathroom.  He also has no problem climbing on the kitchen counter to get a snack.  His mom just loves that.

I am so blessed to have Cruz in my life.  He has taught me to be more compassionate and patient in my own life.  He has been through more in his 10 years than most people have their whole lives and he stays so positive and happy through it all.  We could all stand to learn something from him.  He is one tough cookie and I love him dearly.

Here are some pictures of famous little people. 

Celebrate World Kidney Day on March 8, 2012 and sign up for a kidney walk to raise money for the National Kidney Foundation today.
http://www.kidney.org/

Visit the Donate Life website to see how you can save a life and donate today.
http://donatelife.net/

Visit the Ronald McDonald House charities website and see how you can help today.
http://rmhc.org/

Visit the Hershey Medical Center website today.
http://pennstatehershey.org/web/guest/home

Visit the Make a Wish Foundation website to find out how you can help grant a wish to a child in need today.
http://www.wish.org/

Visit the Camp Kydnie website to make a donation or to get information on becoming a camp counselor today.
http://www.campkydnie.org/

Cruz Rocks!

Ok, I can't take it any longer.  Who is my mystery friend?  Drum roll please!  It's my 10 year old nephew, Cruz.

Cruz was born on May 21, 2001 to my middle brother, Mike and Tina.  Mike is 6 ft. 2 in. and Tina is 5 ft. 1 in.  Tina's pregnancy was typical, nothing major that would indicate any problems with Cruz.  When Cruz was born he looked like any other baby being born that day.  It wasn't until a few years into his life that we noticed that there may be a problem.  Cruz was falling off the growth chart with every well-child check up. We also began to notice that his hands were very small.  No one in our family wanted to believe that anything was wrong with him.  We kept hoping he would soon have a growth spurt and everything would be fine.

It wasn't until Cruz was 5 years old that he was diagnosed with a form of dwarfism.  Shortly after that he was started on growth hormone injections to help him grow taller.  He tolerated the injections very well.  He also had tubes put in his ears due to recurrent ear infections and fluid.  Recurrent ear infections are common among little people.

When Cruz was 7 years old he was diagnosed with severe kidney disease.  Kidney disease isn't typically a characteristic of dwarfism so this baffled his doctors.  He had extremely high blood pressure and only 20-25% kidney function at that time.  Our whole family was devastated with this news.

Here is a timeline of Cruz's life up until his kidney transplant in 2009.

9/12/08:  Cruz's creatinine levels were 3.8.  His doctors were in close contact with the transplant team at Hershey Medical Center (HMC).

9/19/08:  Admitted to HMC for high blood pressure and to rule out pneumonia. 

9/21/08:  Home from HMC.  Blood pressure was stabilized.

9/22/08:  Back to HMC with extremely high blood pressure.  Cruz was sent home the next day.

10/9/08:  To HMC to have port placed in his belly to start peritoneal dialysis.

10/12/08:  Cruz's family held a benefit for him at Naylor’s Winery to help raise money for his medical expenses.  Cruz was still in the hospital that day but his doctors gave Cruz's mom permission to take him to his benefit for a short appearance.  Then it was back to HMC.  Cruz was discharged on 10/17/08.

10/21/08:  Cruz had his tonsils and adenoids removed.

Shortly after that, Cruz returned to school and kept very busy.  Cruz remained on in-home peritoneal dialysis for 10 hours each night.  Mike and Tina were both tested for kidney donation and Tina was chosen to give one of her kidneys to Cruz.  The picture below shows Mike hooking Cruz up for his nightly dialysis.

2/10/09:  Cruz's kidney transplant!!  This picture is of Cruz a few hours after transplantation.

These are pictures of him getting better everyday.

 This is Cruz's dad, Mike, and his mom, Tina.  She looks great after donating a kidney!

 This is my buddy, Cruz!  Notice his small hands and his scar of  life.  What a brave soul!

 This is Cruz, 10 years old, with his sister Zoe and my 6 year old son, Gavin. 

Kidney disease

The kidneys are a wonderful organ.  They do many things to sustain life.  Basically, the kidneys cleanse your blood by removing waste by-products after digestion and exposure to medications and chemicals.  The kidneys also produce the enzyme renin which helps to regulate blood pressure by maintaining a healthy salt and mineral balance.   The kidneys produce erythropoietin which stimulates RBC production, and they produce an active form of vitamin D needed for bone health.

Diabetes and high blood pressure are the most common causes of kidney failure.  Inflammation, congenital defects, long-term exposure to drugs, and immune system conditions can also play a major role in kidney failure.

With kidney disease that has progressed over time, dialysis or a kidney transplant is needed.  Dialysis is needed when the waste products in failing kidneys get too high and the patient begins to get sick from it.  There are 2 different types of dialysis: hemodialysis and peritoneal dialysis.  I am only focusing on peritoneal dialysis. 

Ok, so what is it?  Peritoneal dialysis does the job of the kidneys when they are failing and not able to work properly.  Peritoneal dialysis uses a fluid that is placed into the patient’s abdominal cavity byway of a port inserted in the stomach.  The fluid helps remove excess waste products and other toxins from the body.

During dialysis and after kidney transplantation, the patient will routinely get blood work done to check the creatinine level and the blood urea nitrogen (BUN).  These levels are checked very closely to determine if the kidneys are working properly.  A rise in these levels indicates decreased kidney function.

If you want more information, check out my links on kidney disease and dialysis.


http://www.webmd.com/a-to-z-guides/understanding-kidney-disease-basic-information

http://www.medicinenet.com/dialysis/page2.htm

Achondraplasia dwarfism

There are 200 types of dwarfism.  Achondraplasia dwarfism is the most common form of dwarfism.  It affects 1 in every 15,000-40,000 births.  It is caused by a new mutation in the fibroblast growth receptor 3 gene or FGFR3.  Researchers do not know why this mutation occurs.  There is a protein within this gene that limits formation of bone from cartilage, especially in the long bones of the arms and legs.  So it is safe to say that achondraplasia dwarfism is a bone-growth disorder.

There are a few prominent features to someone who has achondraplasia dwarfism.  They will obviously have a short stature with an average adult male being 4 ft. 4 in. and an average adult female being 4 ft. 1 in.  They will have a large head with prominent forehead.  Their hands will be small with stubby fingers.  They will have an average size trunk with short arms and legs.

Most dwarves are born to average sized parents.  For this to happen, there had to be a new mutation of the FGFR3 gene. 

Breathing disorders, obesity, recurrent ear infections, crowded teeth, and decreased muscle strength are some common health issues associated with achondraplasia dwarfism.  Even though there is no cure for achondraplasia dwarfism, some health issues can be treated on an individual basis. 

Two to five percent of newborns being born with achondraplasia dwarfism do not survive longer than 12 months of age.  This would be due to both parents being an achondraplasia dwarf and the newborn inheriting both parental mutated genes. This would be considered the most severe case.  However, most dwarves live a normal lifespan with normal intelligence.


http://bones.emedtv.com/achondroplasia/achondroplasia-dwarfism-p3.html

Introduction

Midget, dwarf, shrimp, shorty, elf..........I'm sure everyone has used those words to describe someone that isn't as tall as you.  I know.  I have too.  Well, up until several years ago, those words took on a whole new meaning.  I am so in tune to those descriptive words when I hear them now.  The term "midget" is now considered offensive and derogatory.  It is politically correct to refer to them as "little people."

My blog is going to take you on a journey in the life of a dwarf.  I will give you characteristics and stats on the most common form of dwarfism.  I will touch on kidney disease and in-home peritoneal dialysis that my mystery friend had to endure.   I will provide a detailed timeline of procedures and complications leading up to his kidney transplantation.  I will take you to the Hershey Medical Center to show many pictures of his time spent there after his kidney transplantation in 2009. 

My blog will also give a comparison to this young mans dwarfism, which has been undiagnosed at this time, with that of the most common form of dwarfism and other forms of dwarfism.  You will find that he is one of a kind.

Stay tuned to see who this miraculous young man is and what he means to me.  I will give you up to date information on how he is doing now and what his future holds.  I will provide the reader with several links to wonderful organizations that have helped this young man, such as, organ donation, Ronald McDonald House, Make a Wish Foundation, and Camp Kydnie.

I am hoping my blog gives you a better understanding of what it’s like “Livin’ Life Little.”

http://rarediseases.about.com/od/dwarfism/f/dwarfismtypes.htm