JAEYA'S ORTHOPEDIC EVALUATION

Jaeya had her full exam with our trusted and long time orthopedic doctors Santora and Pond.  They have been with us since Lan came home in 2004 and treat Caden.  Dr. Santora was the one to repair his frightfully broken femur last August.  He also did Lan's amputation.  We adore him.

Jaeya had twelve x-rays and has every part of her body on film and in the Shriner's computer.  She was her typically worried self and at the repositioning of each shot had to be assured
THIS POSE would not be ouchy.  Each time a new doctor or part of the team walked in she had to also be comforted that this person would not hurt her.   So, to get a picture of her smiling like this with Dr. Pond is so telling of her relief and trust in him.

We found that Jaebird has gained 2 lbs since her adoption physical in Guangzhou on March 31.  WHOOOOWEE!  She now tips the scales at 29 lbs.  Meisyn was truly delighted to find that she is 30 lbs and bigger than Jaeya.  Older sister was not at all impressed with this.

Jaeya likely has Type III osteo-genesis imperfecta (OI) which is the most deforming and debilitating after Type II which is lethal before, during, or shortly after birth.  We will know for certain when the test results come back.  That did not come as a surprise.  We suspected that before her adoption. 

These are the markers for Type III
 
Type III

• Bones fracture easily. Fractures often present at birth, and x-rays may reveal healed fractures that occurred before birth.
• Short stature.
• Sclera have a blue, purple, or gray tint.
• Loose joints and poor muscle development in arms and legs.
• Barrel-shaped rib cage.
• Triangular face. 
• Spinal curvature.
• Respiratory problems possible.
• Bone deformity, often severe.
• Brittle teeth possible.
• Hearing loss possible.
• Collagen improperly formed.

Jaeya's knee caps are on the sides of her knees.  Uh, that is not so good for bending.  Her knees cannot extend more than 45 degrees.  Her right arm is much shorter than her left due to a previous fracture.  Her elbows do not extend.  They are permanently bent at 90 degrees.  She has scoliosis.

Jaeya has significant bowing of all her limbs. Before or during birth she appears to have broken her pelvis and it has never been repaired.  Her muscles have been contracted for so long that it has caused permanent damage.  It was our hope that through rodding surgeries and extensive PT that Jaeya would be able to walk independently.

After manipulating Jaeya's limbs and reviewing her xrays the specialists feel that putting her through all the pain of the countless surgeries that would be required for a minimal chance to be able to walk "would be folly".  The amount of pain for minimal gain is likely not worth it.  The muscles have been contracted for so long that messing with them could cause nerve damage that can result in paralysis or constant and significant pain. My husband deals with neuropathy and we don't want to see her go through that.


So, the plan for Jaeya is to have pamidronate IV treatments.  She will have titanium rods implanted only after a serious break not as a preventative measure.  She will be fitted for a power chair so that she can remain safe, comfortable, and mobile.

 

It was disheartening to hear that Jaeya will likely need a wheelchair for the rest of her life.  She has been tremendously worried about being in pain from surgery and the doctors "breaking" her.  We don't want to put her through the trauma if the results will be minimal.  I do have to smile to think of her zipping around in her power chair.  Watch out world.  Jaeya wants to experience you!

Perspective is a funny thing.  As I entered Shriners I would have been so disappointed to know the prognosis that was given to us just hours later.  But, by then we had Meisyn's tentative diagnosis.  Jaeya may not walk, but, SHE WILL LIVE.

BLING...can never have enough

Meisyn has bling.  Permanent bling.  She got her ears pierced and can now wear ear pretties.  This sealed the deal for her love of America.  She told me that in China only big people can have pierced ears.  America is good, good, GOOD!

Meisyn apparently has terrible eyesight we have learned.  She picked out purple frames with colorful butterflies on the sides.  Imagine, LOL!  As we left the office and entered the world she was full of oooooohs and whoaaaaaas as there was a world she could define!

Now she has her very first loose tooth.  She assures me she is leaving babyhood behind.  Don't grow up so fast my dear girl.

PIERCED

We have the summer calendar cleared.  We were ready for months of surgeries, castings, recovery and physical therapy. Suddenly we now have an eerily empty schedule and I am utterly lost and unprepared.

We took Jaeya and Meisyn to our long awaited Shriner's Hospital appointment to finally get their evaluations along with the medical plan for their futures.  It sounds so strange to be devastatingly disappointed that there will be no surgery for Meisyn to repair her dislocated hips.  There will be no spica body cast to keep those hips in place as she recovers.  There will be no leg braces to support legs for walking.  There will be no back brace to keep her crooked spine straight.  There will be none of this.  Instead there will be priority given to three things:

1.  Her comfort
2.  Her safety
3.  Her mobility

For Meisyn this will mean that she will be fitted for a motorized wheelchair.  Won't she adore having the ability to zoom herself around?  She will LOVE that chair.  It will take me some time but I will get to the point that I will love that chair too.  But right now, it symbolizes to me overwhelming sadness and loss.  

I want to plead, "Tell me she has severe scoliosis.  Please say that I will need to find a way to lug Meisyn around in her cumbersome spica cast.  Tell me that she even has an enclosed form of spina bifida.  But don't repeatedly pull her skin and let me hear you tell the other doctor that you suspect she has Collagen 6."

That is how it went.  When I heard Dr. Pond tell the other attending doctor that he believes she has Collagen 6, I blurted out, "I don't want it to be Collagen 6".  Dr. Pond looked up surprised and questioned, "You know about Collagen 6?"  I replied that another little girl from China had just been diagnosed with Collegen 6 about 4 weeks ago and I have been in communication with her mother.  Collagen 6 is a rare form of muscular dystrophy.  These are the markers:

1. Congenital muscle weakness
2. proximal joint contractures
3. hyperextensible limbs
4. scoliosis
5. hip dislocation 

6. protruded heel bones
7. Low weight and height
8. normal intelligence

Meisyn has all the markers.  Each one.  And the prognosis?

"Repiratory failure in the first and second decade of life is a common cause of death.  Most die of respiratory failure in their first decade.  No individuals with this form of muscular dystrophy have been known to reproduce."

It will take some time to have the muscle biopsies back the metabolic tests completed but in my heart I feel this is the answer.  This is what my precious baby girl has been born with and will ultimately die from.  Our Shriners doctors stated, "It is possible that Meisyn has a metabolic disorder or a myopathy but it is likely muscular dystrophy.  Unless the tests come back to conclusively prove the contrary, she is considered to have muscular dystrophy."  Makayla refuses to consider any such diagnosis until the tests prove it.

I have decided to allow myself to grieve now and if by some miracle the tests come back showing something else less devastating, then I can rejoice.  For me, I can't put myself through months of hoping, hoping, hoping it is not Collagen 6 to have my hopes shattered if it is.

We have decided to live every minute with a fun list at hand.  We spent the weekend swimming, shopping, going to her first movie, freezing our behinds off at the summer parade, and even adopted a shelter dog that I DO NOT NEED but that Meisyn adores.  Right this minute Makayla has taken Meisyn to get her ear pretties she has longed for since the minute she saw Makayla's sparkly earrings.  

Meisyn heard me crying at the computer as she approached my office.  Soon I could hear her sobbing.  She knew Mommy was sad and it made her sad too.  She doesn't understand why I am crying.  Her sweet, compassionate heart just wanted me not to cry. But if I had too she would join me.  Makayla scooped her up for me and she and Shayna have gone to get her ears pierced.  I am certain it will hurt a whole lot less than the piercing in my heart.

We know we will lose Ammon far too soon.  And now we must likely do the same with Meisyn. How much can a heart take?  I am afraid I will have to find out.