Monday, January 28, 2013

Learning About PFFD

If you would like to learn more about Kelli's condition, and the surgeries she will face in the future, you can check out the following websites:

Dr. Paley is the leading doctor in this field.  He has created the hip surgery that she will have this summer.  In the coming years, she will need at least 3 lengthenings.  The links under "Congenital Femoral Deficiency" are the ones that pertain to Kelli.  Congenital Lower Limb Problems

This is where Kelli is being treated.  These doctors were part of Dr. Paley's team before he moved to Florida.  International Center for Limb Lengthening

This site has some great articles and testimonies of others who have PFFD.  A Guide to PFFD

Somewhere I have a PDF file that explains the superhip surgery.  If/when I find it, I will post it as well.  The site I originally downloaded it from no longer has the file available. 



Upcoming Appointments

Please keep the upcoming appointments in your prayers...

This week Kelli will be going back to Hershey for two tests concerning her cough that she's had off and on since June.  One will be a barium swallow to check for acid reflux (a problem she had as a baby).  The other is a sweat test to check for cystic fibrosis.  The allergy medicine that the pulmonologist gave her does seem to be helping quite a bit though.

Also this week, Kelli will be meeting with a counselor for the first time.  She doesn't talk with people she doesn't know, so please pray that she will feel comfortable with this counselor.   We're hoping to help her with some of the self-esteem issues that have come up as well as help her control the intense emotions that she has.

Next week she will see the dentist, and in a few weeks she will have her 4 year check up.

We see the pulmonologist again in April, unless this week's test results show the need for an earlier appointment.

Her hip surgery has been changed from June 18 to June 11 because her surgeon will be out of the country on the originally scheduled date.

Saturday, January 19, 2013

Thank You!

I just wanted to say thank you to everyone who has sent cards of encouragement and care packages to Kelli.  You have been such a blessing and source of joy in Kelli's day.  She has really enjoyed receiving mail this week, and keeps asking why people are sending her things.  We've told her that many people care about her, are praying for her, and want to show her God's love.  My camera has officially died, but before it did, I was able to take a few pictures of Kelli opening her mail...
opening her cards

I wonder what is in here...

new hair accessories

I love receiving mail!

wrapped in a prayer shawl






Saturday, January 12, 2013

Wheelchair

For the past year, Kelli has been complaining of pain in her legs.  When asked, she usually says the pain is in her right leg (the longer one) near the knee.  However, sometimes she says both legs hurt.  In December we took her to the Bone and Joint Institute in Hershey, and the doctor said that the x-rays seem to show avascular necrosis in her right hip.  (death of the bone cells due to a lack of blood flow)  She said this could cause pain in the upper part of the leg from the hip to the knee.  Her recommendation was to limit Kelli's activity and to keep an eye on this condition for awhile.

She is beginning to out grow her stroller, so we began a search for a wheelchair.  Today, my aunt and uncle brought one out for her.   She loved riding around the house in it and is planning to use it tomorrow when we take a walk through the neighborhood.



 

PFFD Doesn't Stop Me!

A three inch leg difference doesn't stop Kelli from accomplishing the things she wants to do!

hiking

dancing at church


carrying heavy things

walking in the pumpkin patch

playing with animals

making new friends


riding a bike

riding a horse

playing Wii games

trying to figure out a jump rope



leading the pledge

climbing

running

going up the climbing wall


bouncing

jumping

jumping high

posing for the camera











Friday, January 11, 2013

Beautiful

Wednesday night my husband and I had a little talk with Kelli about her behavior as of late.  She has been having lots of temper tantrums and has been having a difficult time obeying in general.  When we asked her why she behaves this way, she said she feels sad and angry.  Then we asked her why she feels this way.  She told us she hates herself and does not like herself.  We kept asking questions and finally she came out and said she doesn't like her legs. She wants to have them taken off and doesn't want to walk anymore.  That just broke our hearts! Please pray that God will lift her spirits and will show her just how precious she is!

Treatment Plan

Kelli is current seeing the doctors at the International Center for Limb Lengthening in Baltimore, MD.   Dr. Herzenberg said he could treat her condition with a superhip surgery at age 2 and lengthenings at age 4, 8, and 12.  In his estimation, without treatment, her left leg would always be 2/3 the size of her right leg.  At her current age, that means she has a 3 inch leg difference.  As an adult it would be closer to a 6-8 inch difference. 

We've opted to try the hip surgery during the summer when I am able to stay home and take care of Kelli while she is in the spica cast for six weeks.   The surgery was scheduled for June 2011.  However, the night before we were to go there, the doctor called and said he didn't think he could do the surgery because of her size.  He planned to evaluate her the next day, but wanted to give us some advanced notice that the surgery might not take place.  After examining her, the doctor decided that she was just big enough, but because of her tiny size, it would make the surgery more risky.  He advised us to wait a bit more to allow her to grow, but ultimately left the decision up to us.  We decided to wait.

The surgery was rescheduled for June 2012.  A week before the surgery, Kelli developed a really bad cough.  Her pediatrician didn't give her any medication for it.  Hoping for something to help her get over this cough, I took her to an urgent care center.  The doctor there gave her Dimetapp cough syrup, which didn't help.   When Dr. Herzenberg examined her the day before the scheduled surgery, he said that the anesthesiologist might not want her to have the surgery due to the cough, but we would have to wait until the next day to find out.  We arrived at the hospital early the next day and had Kelli ready in the pre-op room.
being silly with mommy

covering Jenny with stickers

waiting in pre-op

wondering if I'll have surgery













However, when the anesthesiologist arrived and heard the cough, he said it would not be wise to proceed.  The surgery was cancelled again.

It is now scheduled for June 11, 2013.  She still has the cough and we just came back from the pulmonologist today.  He's going to try to figure out what is causing this cough so that it will not interfere with the surgery. 

Shoe Lifts

Kelli got her first shoe lift when she was 10 months old.  (December 14, 2009)  It was 2 1/4 inches high. 


 Since then, we have gotten a new lift every six months as she continues to grow.  Her shoe lifts now are 2 3/4 inches high.
These boots were her favorite!
Kelli's "pretty" shoes

trying to tie her shoes
the shoes she has now
Our insurance covers the cost for a lift to be added to one pair of shoes by a orthotist.  We take her sneakers to Lawall Orthotics and Prosthetics to have a lift added. 

After calling around to a few local shoe repair shops, we found a place which will add a lift for only $30.  (Most other places charge between $65-$100 to have a lift added!)  As we are able, we have lifts added to winter boots and dress shoes. 

A Miracle is Born


            I have wanted to be a mom for as long as I can remember.  As a child I would use my dolls and pretend I was their mother.  As I got older, I would daydream about caring for my baby and watching him or her grow.  I imagined introducing my children to things I had enjoyed as a child: running barefoot through the backyard, riding bikes, and roller skating.  So when I finally got married, I was ready to start a family.  Unfortunately, my new husband was not.  He wanted us to wait for a few years before having children. I didn’t like it, but we waited for four years.  All the while, I kept dreaming about my future children.
            Finally, after begging, pleading, and even a few arguments, my husband agreed that we could start trying to have a baby.  The months ticked by and still the pregnancy tests were negative.  We turned to a fertility specialist for help.  It took almost a year of testing before I was diagnosed with Polycystic Ovarian Syndrome. 
My doctor told me that fertility pills may improve my chances of conception, so we began with this course of action.  I started on the lowest dose, but it wasn’t enough.  A few months later I tried a higher dose without success.  Finally the doctor raised me to the highest dose.  It made me sick and dizzy but not pregnant.  I was so discouraged.  I could see my dreams of a family slipping away. 
My next option was to try a different medication.  I was nervous about it because of my experience with the last one.  But I was desperate to have a child, so I tried it.  Again, the medication only caused terrible side effect.  All of the other options my doctor offered were more expensive than we could afford at the time.  By this point, three years had past since we had first started trying to have a baby.  Depression and despair was seeping in – threatening to completely kill my dreams.  Over the next two years I prayed, cried, and prayed some more.  I was now leaning solely on God’s willingness to answer the cry of my heart. 
In June of 2008, I became really sick.  I tried to ignore it for awhile, but eventually I decided to see a doctor.  It was a Saturday, and my regular doctor’s office was closed.  I found a local walk-in clinic and decided to try it.  When I met with the doctor I told him I felt sick in my stomach, I was going to the bathroom all the time, and my back was hurting.  I thought I might have a urinary tract infection.  The doctor took a urine sample and concluded that I did not have an infection.  He said I must have some type of flu.  I was in tears on the way home because I felt so terrible and couldn’t explain why. 
The next morning, Sunday, July 6, I decided to take a pregnancy test.  Something had to be making me sick, and I was hoping that pregnancy could explain my symptoms.  Waiting those three minutes for the results was agonizing.  Do I dare cling on to hope after five years of negative results?  My eyes first rested upon the straight line in one of the windows, and I felt heavy-hearted again.  What I didn’t realize right away was this was not the results window.  Then I saw the other window had a plus sign in it.  Could it be true?  I called to my husband and showed him the test, still not believing what I was seeing.  I called my parents to tell them, and my mom convinced me to take a second pregnancy test to confirm it.  This test was a digital one which had the word “pregnant” clearly in the results window.  My dreams were instantly revived.  God had heard my prayers and had provided a miracle for us.  To say I was excited would be an understatement.
My first trimester was filled with almost constant morning sickness.  However, I rejoiced at the thought of having a baby growing inside me.  Although it was way too early to tell, I knew we were going to have a girl.  We had chosen the name Kelli Beth, meaning warrior in the house of God.  It had been a fight to conceive her, and I hoped she would have this same fighting spirit.  I would daydream about what she would look like and all the things I would do with her:  I saw my husband teaching her how to ride a bike and myself teaching her how to bake. 
Our first ultrasound was performed at my first prenatal appointment.  All I could see was a flicker on the screen indicating her heartbeat, but it was enough for me.  I was in love with my beautiful baby. 
By my third month of pregnancy, my blood pressure began to rise.  My OBGYN gave me a blood pressure medication and referred me to a specialist.  This new doctor wanted to perform ultrasounds every six weeks to monitor Kelli’s progress.  I was thrilled that I would get to have a “sneak peek” at my baby more often than most.
At my 18 week ultrasound appointment we found out that we were definitely having a girl.  We were told that everything seemed to be normal, and I was happy.  We began to put the finishing touches on the nursery we had started setting up long before Kelli was ever conceived.  We bought pink clothes and toys, all the while envisioning the arrival of our beautiful, healthy baby girl - our miracle from God.
Our next ultrasound was scheduled for December when I was 24 weeks pregnant. My husband planned to meet me at the doctor’s office which was located halfway between his job and mine – about a 10 minute walk for each of us.  We were both excited about seeing Kelli again.  The ultrasound tech began the test, and the outline of Kelli’s body appeared on the screen, bringing an instant smile to my face.  There was my miracle baby.  The tech continued to move the wand over my belly, and I realized that this was taking longer than normal.  When the tech was finished, she told us the doctor would be in shortly, and she left the room.  It took awhile for the doctor to join us which was another clue that something wasn’t quite right.  When he did, he pulled up one of the ultrasound pictures and showed us that one of Kelli’s femurs was significantly shorter than the other.  In an instant, all of the dreams I had had for years shattered into a million pieces.  What did this mean for my child?  How would she get around?  What kind of life would she have?  Would she be accepted by others or would she have to endure a life of teasing?  We could never teach her to ride a bike, use roller skates, or play tag.  I feared I would never see her all dressed up for her prom or walk down the aisle in a white dress on her wedding day.  Who would want to date a girl with a disability?  What had happened to my miracle?  So many emotions filled me, but I couldn’t find the words to speak or ask verbalize the swarm of questions crowding my head.
The doctor couldn’t have answered my questions even if I was able to ask them.  He had never seen this before and couldn’t offer any advice.  He told us to set up an appointment in four weeks to monitor Kelli’s growth.  And that was it.
I fought back the tears as I trudged alone back to my car which was several blocks away.  On the way, I called my parents to tell them the news.  It was difficult to speak even then and not break down right there in the middle of town.  My husband and I spent the next four weeks scouring the Internet for information but came up empty handed.  Without a specific diagnosis, we had little to go on.  The rest of my pregnancy was spent trying to come to terms with the fact that my child would have a disability.  Gone were my dreams of life with a “normal” child.
We met with another doctor at the practice in January of 2009 for our next ultrasound.  This doctor was more compassionate but still did not have many answers.  He seemed to want to help, but since he had not seen this before, he could do little.  He did offer to schedule another ultrasound for us in February, free of charge, to continue monitoring Kelli’s development.
Kelli Beth was born on February 13, 2009 – three days before our next ultrasound appointment and almost a month before my due date.  She was so tiny – and absolutely beautiful.  Yes, her left leg was about two inches shorter than her left, but she was healthy otherwise.  I was finally holding my miracle, and I loved her unconditionally.  It didn’t matter anymore that there was a discrepancy with her leg.  She was my daughter, my miracle child, and I was extremely thankful.
The doctors at the hospital were unable to answer any questions about her leg and told us to ask our pediatrician to refer us to an orthopedist.  We followed this advice and met with an orthopedic doctor on March 2.  He diagnosed Kelli with Proximal Femoral Focal Deficiency, or PFFD.  He said her hip socket and the ball of the femur appeared to be missing and the femur itself was shorter than the one in the right.  While it was too early for treatment options, he said the usual options are either a hip surgery with several bone lengthening surgeries to follow or an amputation and a prosthetic.  He seemed to be leaning more and more each visit toward amputation – something we wanted desperately to avoid.  I couldn’t stand the thought of Kelli losing her perfectly formed foot with those adorable little toes.
Since that visit, we have consulted with two other doctors.  Both seem to think that she will be able to have a superhip surgery followed by several bone lengthening surgeries.  However, right now we are waiting to see how her bones form over the next year or so before any surgeries are performed.  In a few more months, we will be getting a shoe lift for her to help her as she learns to walk.  While we wait, we are networking with other parents online to find out their stories and hear their advice.   We are also doing extensive research on our own.
Kelli is now almost 8 months old.  Her sweet disposition, sparkling blue eyes, and warm smile has drawn others towards her.  No one has made fun of her, rejected her, or laughed at her because of her disability.   I hope that this continues as she grows older.
She hasn’t let this condition slow her down at all.  Kelli is figuring out a way to crawl and is even taking steps when we hold her hands. 
Everyday I am amazed at what my daughter can do.  Nothing stops her.  She is my hero and my teacher.  She has taught me about determination, perseverance, and being content in all circumstances.   I love her so much, and I thank God daily for this miracle that He has given to me.  (written Oct 2009)

THANK YOU!!!

  Sitting in bed all day can get quite boring.  However, thanks to friends and family, Kelli has been receiving wonderful care packages fill...