Early X-Ray

My Husband and I decided that we would ask for copies of all ultrasounds/x-rays/reports...anything with information on PFFD and Elsie's leg.  We are 10.5 weeks into PFFD (how old Elsie is) and I finally picked up the image from her first xray when she was not even 24hrs old.  Below is the picture.  I've also quoted the Dr.'s notes on the x-ray report.



  Here is what's in the report:

"FINDINGS:
  BONES:  Images are limited due to the less than optimal positioning.  There is asymetric widening of the left hip with deformity of the proximal left femur which may be developmental subtle lucency about the proximal left femur with slight angulation no gross callus formation but this may represent a fracture.

  CONCLUSION:  Asymmetric widening of the left hip, lateral dislocation with developmental deformity of the proximal left femur there is a relative angulation and a subtle lucency possible fracture, age indeterminate.  Right hip appears more normal but this may also be slightly widened.  The pelvis appears to be well developed.  Clinical correlation and progress studies recommended."

We will share the Ultrasound images as well once we pick those up.  The x-ray is from Borgess and the ultrasounds were taken at Bronson (both in Kalamazoo).  Side note (The right hip has looked normal in every instance since this initial x-ray)

Harness was a Success!

On Monday we had an ultrasound to determine if the femur head was in the hip socket.  We don't find out much info at the time of the ultrasound because it's done at Bronson Hospital and the results have to be sent over to Dr. Nowicki at Kalamazoo Center for Medical Studies (KCMS).  So today (Wednesday, Nov. 30) we met with Dr. Nowicki to go over the results.  Drum roll.... success!  The femur head is in place.  Hooray!  We aren't surprised to hear this because Elsie has been moving her leg just like the right one.  However, it is nice to have the official confirmation. 

As a result, we are in the weaning process now!  We're going from 23 hours a day in harness to 12!  This will last for 2 weeks and then we'll move to 8 hours a day for 2 weeks.  After that it can be removed.  I'm so excited to have my squishy baby back!  It's amazing how much I cherished holding her sans harness that 1 hour a day.  Now I get it 12 :)  Luckily, the time she spends in the harness can be all night long, and during the day out.  This will make bath, diapers and clothing changes much easier. 

The Dr. that performed the ultrasound questioned whether Elsie had a fracture.  Dr. Nowicki explained that what she saw was probably abnormal bone, which may look like a gap in the bone on the ultrasound.  He said that even if it were a fracture, the treatment is 6 weeks in the harness, which we just completed.  The Dr. at Bronson also noted that her femur head, though present, may be small.  Although this isn't great news, we are lucky that she for sure has a femur head.  Many with PFFD are born without a femoral head.   

I'm very happy that Elsie is moving her leg and seemingly no longer experiencing the pain when her leg is in motion.  No matter the inconvenience, the harness was well worth it.  But I'm not sad to say good-bye!

Elsie stretching her legs!  She loves to wiggle freely out of the harness :)

The next step is all the way out in April (23rd to be exact).  We will see Dr. Nowicki for a follow-up and to discuss what needs to be done next.  This will most likely be an MRI.  The MRI will allow us to see exactly what is or is not present in her left leg.  This knowledge will allow us to know what treatment(s) we may be looking at in the future. 

Progress

Today, like all other days, I removed Elsie's harness for her hour of freedom.  Immediately her limbs began to move all about.  This is normal for her two arms and right leg, but usually she doesn't move her left leg too much.  Today was a different story.  Her leg was extending straight, lifted in the air.  She would bring it down and flex it, just to repeat the stretching again!  I truly got tears in my eyes.  It was encouraging to see her move so much.  Not moving her leg isn't common to PFFD, but Dr. Nowicki believes it probably is just her caution in response to the pain she experiences (which is still unknown as to the exact cause of the pain).  However, with her increase in movement, I'm hopeful that it's a sign the harness is giving her the comfort we want her to experience with possible long term benefits.  YAY.

Picture Proof!!

The Harness

Elsie cries when we move her left leg.  Right off the bat it was evident that Elsie does NOT like her leg to be manipulated.   PFFD does not normally come with associated pain so the Dr. wasn't positive as to what was causing her discomfort.  The two guesses were 1) Femur Fracture or 2) Hip Dysplasia.  After an ultrasound when she was 1 week old we discovered that she does have hip dysplasia. 

Since the chances of having hip dysplasia and PFFD independent of each other is quite unlikely, the dysplasia is probably caused by either a improperly formed femur head, or an improperly formed hip.  Either way, we won't know exactly which one is the cause (or both) until her MRI.  As a comfort measure we have Elsie in a Pavlik Harness.  She's been in since 9 days old.  She'll be wearing it for a total of 6 weeks for 23 hours a day. 

Although it's a pain to maneuver around with diapers and clothing, it's been a blessing.  I can see a difference in her comfort.



Elsie in the harness at 9 days old



Change in Attitude

The news of PFFD was devastating.  So many questions with few immediate answers.  And the answers we did receive were not comforting.  The discovery of Elsie's deformed femur unfortunately stole from the joy of her arrival.  It was frustrating knowing immediately that Elsie would face surgeries and physical challenges and we couldn't do anything to make it go away.  After a few days in this mindset, I decided I needed an attitude change.  I came to the realization of a few things:

1) A leg is not life threatening.  She can survive, thrive and have a wonderful life even if worse case scenario she needs a full amputation.
2) We are fortunate eniugh to have health insurance and jobs that will give us the flexibility to do all surgeries and physical rehabilitation/therapt as necessary
3) PFFD can be bilateral and can also be accompanied with a deformed foot and lower leg.  Thankfully Elsie's lower leg and foot appear to be in great shape and her PFFD is only Unilateral.

My attitude truly did change.  I have a beautiful, healthy daughter.  God will take care of her and I'm confident of that. 

Just to be clear, I was never disappointed or devastated by Elsie.  She is a true blessing and perfect addition to our family.  I have felt that way since moment one and still do.  I was just dwelling on the negative a little too much. 

The Blame Game

One of the hardest parts initially to me as Elsie's mom was wondering what I did in my pregnancy to cause this.  I did many google searches on the cause of PFFD.  Even though I kept seeing the same answer, there is no known cause, I didn't believe it and blamed myself.  I wanted to ask Dr. Nowicki if he knew of a cause, but was afraid I would cry asking.... or even worse learn that I actually did cause it.  Eventually I did ask and he assured me that it is something that just happens.  So to any PFFD mom who may be reading this... if you're like me and you wonder if you're the cause of PFFD for your little infant and are too afraid to ask........ You're not!  It's something that just happens and isn't something that is caused. 

The Beginning

Elsie was born on Monday 10/10/2011.  Instantly I was in love.  Elsie was the perfect addition to our family.  The first hour after her birth she was on me and I gawked over her, starring at her beautiful face.  After the initial hour she left my side to be cleaned, measured, weighed and evaluated.  That's when the midwife noticed the difference in her leg lengths.  Neither my husband nor I had noticed this.  The nurse brought it to our attention as we had family visiting.  This was awkward since we were shocked and didn't know what to think, but also had family in the room so we wanted to keep up the positivity.


We almost had to put the leg difference on hold in our minds for the first few hours because we had many family members visiting.  Eventually we had the pediatrician visit (as he would with any newborn).  After he looked at Elsie, he told us that he was going to order her an xray and that we'd be seeing an orthopedic specialist to discuss her leg prior to discharge.


We had the xray done in the room and at this point we didn't have any idea what the leg being shorter meant.  On Tuesday evening, 10/11/11, we meet with a resident orthopedic doctor.  He said that he works with the doctor we would eventually be seeing.  He examined Elsie and looked at her xrays.  Also, he discussed these items with Dr. Nowicki (our Orthopedic Dr).  After talking with Dr. Nowicki, the resident said that the limb difference could be one of 3 things:  1) hip dysplacia; 2) femur fracture; 3) Proximal Femoral Focal Deficiency (PFFD)  Of course, we didn't know what any of this meant.  The resident explained each in a nutshell.  After he left we were hoping it was just a fracture, so it would eventually heal.  We were hopeful this was the cause because there was obvious discomfort when her left leg was moved.  After doing internet searches, we were really praying it wasn't PFFD.  We would find out more the next day when we met Dr. Nowicki.


On Wednesday, 10/12/2011, we met Dr. Nowicki.  He is a pediatric orthopedic specialist in the Kalamazoo area, working at the Kalamazoo Center for Medical Studies.  Immediately upon visiting, Dr. Nowicki examined Elsie.  He didn't have much hesitation in saying the she for sure had PFFD.  It was evident because of 1) the xray and 2) the skin on her thigh wasn't saggy or baggy.  It was normal, so that eliminated the fracture (if there were loose skin, that would have signified that there is supposed to be a longer leg there. 


Unfortunately, Matt and I were crushed.  We didn't want this perfect little girl to face any set backs in her whole entire life, let alone start off with one.  This was a shock.   We were of course overwhelmed at this point.  Not only is labor and delivery a long, tiring process, but we had this bomb dropped on us.  I just wanted to hold little Els and think of a way to take care of her.