Monday, November 19, 2012

And the Perthes Journey Continues....

On Wednesday morning Sam and I flew to Baltimore to meet with Dr. Shawn Standard at the Rubin Institute for Advanced Orthopedics. I wasn't excited about this trip...dreading would be more accurate.  We got to the airport and I learned the skill of pushing a wheelchair while pulling a suitcase and balancing the carry on bag on the handle.  I gave Sam the full dose of his pain medication and his thyroid medication before we headed for security.  I figured euphoria would be much better than frustration and pain behavior. I was thrilled to be led to the priority area and ushered through security quickly and easily.  I gave Sam something to eat so the medications would not irritate his stomach and we headed to our gate.  Sam was excited to go on a airplane but wanted to go to Florida instead of Baltimore and I kinda felt the same.

We actually flew direct into Washington D.C. and then rented a car to drive to Baltimore.  The flight went wonderful, Sam was comfortable but I could tell he was a little stiff once we exited the plane.  We picked up our big rolling bag and headed out to the shuttle for the rental car.  I noticed Sam was moving slower as he struggled to climb the steps on the shuttle bus.  I began to pray that he would stay comfortable enough to make it on the drive to Baltimore.  We arrived at the rental place and were told "We have unusually high rentals today and your SUV is not available."  At this point Sam is beginning to show his discomfort and let the lady know that her answer was unacceptable.  She could see our situation could possibly escalate quickly and she offered us a Jeep Liberty instead.  I told her as long as the suitcase and wheelchair fit in the back and Sam can stretch out in the back seat we would be good to go.  We were quickly on our way with Sam sitting sideways so he could stretch out his legs.  Sam seemed to sense that a vacation was not in the picture and began to say continuously "Mommy, go home".  Now take a moment and picture yourself driving  in a new state, through two major cities, with a bucket load of worry and anxiety while your passenger is showing a mixture of pain, frustration and anxiety and is repeating the same phrase over and over. I quickly pulled off and reassured Sam that he was fine and I was right there with him.  I gave him a snack and a promise of some soda (which in Sam's case is green tea) when we got to our destination.  He settled in and continued to repeat his mantra...just at a lower volume and I took a few deep breaths and carried on.

We arrived at the Hackerman Patz House and Sam was thrilled when we got to our room and he could take off his shoes and lay on the bed and put himself in traction.  The house is very nice and is set up like a home away from home.  Amy greeted us and was extremely helpful throughout our stay. Since we were both tired we had an early dinner and called it a night.  The next day we headed over to Sam's appointment.  It was one of those times when you really don't want to go but you really want to get it over with.

If I'm perfectly honest I knew I wasn't going to like hearing what they had to say.  It wasn't going to be something simple...and I was going to have to make some more really touch decisions on Sam's behalf.  They ordered x-rays and Sam decided to show them he wasn't happy about it.  After about 5 minutes of yelling and hitting the table I finally convinced Sam to climb on and we managed to get through them.  As I waited for Dr. Standard to come in my mind felt like it was in auto-pilot, sort of a "been here, done this" kind of feeling. When you have gone through a major surgery with your child that didn't yield the hoped for results you become a skeptic.  It is really hard to push all those doubts away and replace them with any semblance of hope.  I want with every fiber of my being to see Sam walk with a reasonable gait and without pain.  There was a time in Sam's life when I wanted him to be able to do everything any typical child can do, actually I wanted him to do them better than any typical child.  I wanted him to dispel all the preconceived notions about Down syndrome and just be a superstar.  As I looked at Sam in that waiting room, my heart over rode my mind and there sat "MY SUPERSTAR".  Not the one I imagined but instead the one that has been formed as a result of the twists and turns on a journey that neither he or I could  have ever imagined.  Sam looked up and smiled and said "Love you Mommy, all my heart!!" I crossed the room, hugged him and said "I love you too Sam, with all my heart, forever and ever" and Sam said "Amen".  I chuckled and reassured him that no matter what...we were in this together...and we would figure it out.  I know that Sam is amazingly good at picking up on my feelings and disposition so I relaxed, opened my mind and heart and prepared it for whatever was to come.

Dr. Standard came in and immediately focused on Sam and then turned to me and shook my hand.  Just that simple act of connecting with Sam first meant a great deal to me.  He asked me about Sam's first surgery and when it was done. He asked me how Sam's current function is and I explained that Sam doesn't express pain like the rest of us he just adapts.  He wakes up in the morning and says "Well, this is what it feels like to be Sam Mayer today" and he adapts, he puts himself into traction, he crawls instead of walks, he remains in bed instead of moving around.  Dr. Standard then asked about Sam's pulmonary issues and wanted to know how he came through the last major surgery.  I explained that he spent an evening in the ICU after surgery to be monitored and then came home with oxygen.

Our conversation then turned to how he assesses a new patient and if a hip or situation is in the salvage mode or rescue mode.  The salvage mode is the last step and consists of procedures like a hip fusion or total hip replacement.  In the treatment of Perthes the best outcome is based on 3 things. The first is containment, the second is range of motion and the third is pain relief.  Sam's first surgery was successful in keeping the hip contained but he still struggles with range of motion and pain issues.  At first glance, Dr. Standard was considering Sam's hip to be in the salvage mode but as he studied the x-rays and thought of other similar patients hip structures he felt there was a strong possibility of saving Sam's hip.  Arthrodesis or hip fusion is often used with young patients to provide a pain-free stable hip which can be later changed to a total hip replacement.  It is also used when a total hip replacement has failed.  In my research hip fusion results in a pain-free fused hip in 70% of patients.  Recovery for this surgery is about 12 weeks and male patients tend to have a better outcome than female patients.  Dr. Standard did not feel Sam was a candidate for a total hip replacement because of his age but more so because of the presenting age of his skeletal structure.  In Sam's case if he were to look at his x-ray without any other information he has the skeletal bone development of a nine year old instead of an almost 12 year old, so his potential for bone growth is still very good. With a rescue approach Dr. Standard would like to create a somewhat spherical head in a somewhat spherical cup with good movement that would potentially give Sam 30 good years of pain free movement.  In Sam's case the external fixator may need to be enhanced due to his size and strength.  I asked about the viability of his femur when his current hardware is removed and more holes are placed for the external fixator.  That femur bone is significantly compromised and I worry about him breaking it during the healing or rehab process.  Dr. Standard explained that when he assesses Sam's hip during the beginning of the surgery process if he felt there was osteoporosis or a risk of a weakened femur he would put in a prophylactic femoral rod that would provide stabilization of the femur and could be removed at a later time.  The external fixator would actually help in the process of removing Sam's current hardware in order to keep the hip in a good position and good support for the healing of the hardware holes.

Soooo....if we look at the procedure to save Sam's hip this is what it will consist of.  Prior to surgery we would have Sam's pulmonary doctor talk with an anesthesiologist that Dr. Standard would choose specifically for Sam's case.  The surgery in Sam's case will probably last 4-5 hours due to the need to remove hardware, possibly insert a femoral rod, apply the fixator and do a scar revision.  The surgery is performed under general anesthesia with an epidural catheter placed while Sam is asleep.  The epidural is essential  for post-operative pain management and allows for physical therapy to begin immediately after surgery.  Sam would be moved to the ICU for a couple of days to monitor his respiratory functions.

The surgery consists of soft tissue releases (tendon releases) in the groin and inner thigh, an arthrogram of the hip joint, a small diameter core decompression and bone stem cell graft injection of the femoral head (ball of the hip joint), and the application of an external fixation device.

The groin incision is approximately 1 inch long and would be done in the same place Sam's current scar is. This incision is for the release (tenotomy) of the iliopsoas tendon.  This is a very tight tendon that prevents the hip from fully extending in perthes disease.  The second soft tissue release is the abductor muscle tenotomy. The abductor muscles are the group of muscles in the inner thigh.  This release consists of a stab incision (1/4 inch) on the upper inner thigh.  The soft tissue releases allow full mobilization of the hip joint.  This in turn allows for the hip to be placed into the proper position and complete containment for external fixation.

After the soft tissue releases have been completed, the small diameter core decompression and bone stem cell graft injection is performed.  An arthrogram of the hip is then performed.  This is an injection of dye into the hip joint that coats the cartilage and allows for visualization of the femoral head.  This is the best technique to truly determine the shape of the femoral head.  Also, the hip is taken through a range of motion under live x-ray (fluoroscopy) to allow real time visualization of the hip movements.  The arthrogram provides Dr. Standard with information and a starting point before the distraction is performed.

Sam's surgery would include the removal of his existing hardware and the addition of the femoral rod if necessary.  After that the hip is placed into full extension and approximately 20 degrees of abduction and the external fixator is applied.  This device consists of (in Sam's case) 4 pins above and below the hip joint.  The pins are placed through the skin with very small incisions and inserted into the bone.

This pin fixation allows for a very stable hold and anchoring point for the external fixator. Again, the hip is held in a slightly spread position with the knee cap pointing forward.  The external fixator contains an articulated hinge that allows for hip flexion and extension, the motion that occurs when one sits or rises from a seated position.  All other motions are restricted while the external fixator is in place.

The device has two adjustable distraction devices.  The first distractor is on the body of the external fixator.  This is adjusted by turning the hex screws 1/4 turn twice a day which equals a 1/2 mm per day.  This device is the actual motor to distract or gently pull the hip joint open.  This allows the hip to be free of any force or pressure which decompresses the joint cartilage.  This environment of joint distraction encourages the cartilage to heal and grow into a more spherical shape.  This is accomplished with both the distraction and range of motion of the hip.  These adjustments usually continue for the first two weeks of the treatment process. The second adjustable device is the anterior extension bar.  This adjustable bar is connected to the anterior arches that come off the main external fixator body.  This device maintains full hip extension which is essential to treatment.  When this bar is in place the hip is locked and no motion can occur.  This extension bar is designed to be removable.  This allows the patient to remove the bar during the day and for physical therapy.  The bar is placed onto the fixator once or twice during the day for approximately 30-45 minutes for a stretching session.  Also, the extension bar is placed and worn all night.  This bar is adjustable for a good reason.  Sometimes the hip does not want to fully extend due to soreness or tightness.  The extension bar can be placed onto the fixator with the hip in mild flexion and slowly adjusted by turning the adjustable nut to stretch the hip into full extension gradually which reduces the discomfort. (For some reason I am picturing Sam and I screaming through this whole procedure).

The usual hospital stay is 3-5 days but will be probably be longer in Sam's case.  The epidural catheter is used for post-operative pain control for at least the first 2-3 days.  This allows for immediate physical therapy with minimal to mild discomfort.  Another important medicine that is used is valium (can't wait to see how Sam does with that??). Valium is an excellent muscle relaxant and reduces the muscle spasms which can be as problematic as the pain. (I wonder if I get a prescription of that too!!) Sam will learn to walk with a walker with 50% weight bearing allowed on the involved side. Hmmm...that will be interesting. The physical therapist will teach Sam to transfer safely in and out of bed, wheelchair and toilet. (Do you think I could bring  one home with me for the next 5 months???)

The frame is in place for at least 4 months.  I say at least because Sam has never been one to stick to the program. On discharge Sam will continue physical therapy as an outpatient 5 times per week (YIKES). The usual physical therapy protocol is one hour of land therapy and one hour of pool therapy.  I'm guessing Sam will be 2 hours in the pool and little to no land therapy.  The physical therapy is essential since the motion of the hip joint is the secret to success in perthes disease.  Also, the daily extension of the hip is performed with the extension bar 1-2 times per day for 30-45 minutes and full hip extension with the bar in place at night. Once the distraction is complete Sam will still have therapy 2-3 times per week and the bar twice a day and at night.  Below is a picture of another of our brave Perthes warriors after getting an external fixator.  I hope Sam will be able to smile like she is.

Sooooo....doesn't this sound like fun???? I am still trying to wrap my head around the whole concept but the "what if" is driving me along.  What if Sam could walk with a reasonable gate and no pain???  I can do 6 months of hell if Sam will get some relief and he will regain his mobility.  That's not to say a hip replacement isn't in our future but maybe it is not in our near future.  Most of all my heart hurts for Sam.  Perthes has been a force to be reckoned with in our world.  His diagnosis was fast and furious with 100% collapse, hip dysplasia and in the late stages of the disease.  His first surgery tested our limits as never before and we both learned we are stronger than we thought. I wasn't ready to pursue this type of surgery 2 years ago...I'm not sure if I'm ready yet...but I do know that Sam is worth it and together we can get through anything.

I know many people have tried to contact me about the appointment in the last few days but I needed time to sort everything come to terms with what we are facing.  It is one thing to hear about it and another to think about implementing it with your child.  I wish I could say our return trip to Milwaukee was as easy as our trip out but we got stuck in security for a 1/2 hour (Sam's wheelchair was causing some threat to security....really???), we ran into people that had no compassion or common sense when it comes to children with special needs and all this while dealing with a very tired, anxious and sad mindset.  I took a lot of deep breaths, prayed and tried to stay positive for Sam.  After we got home on Friday, I let Sam relax with Netflix and a snack and I watched a couple of hours of the Hallmark Channel.  The Hallmark Channel is a guaranteed cry fest and I desperately needed one.  But then it was time to pick myself back up, focus on the positive, find and hang on to some hope and keep moving forward.

Sam's surgery is scheduled at Sinai Hospital in Baltimore on January 15th.  I ask that you continue to keep all of us in your prayers.  Jeff and I will travel in with Sam for the surgery and when Jeff needs to return to work I hope to have a replacement come help us.  Sam will require 24/7 care again and I want to make sure that whenever he is awake there is someone he knows by his side.  This will be a long and tiring 6 months but my main concern is getting Sam through, providing him with all the love and support I can and keeping him as comfortable as possible in between his therapy and pin care.  I ask that we all pray that he stays healthy and infection free and that this procedure be the one that restores his pain free mobility. I ask for prayers for the entire family as this will rock all of our worlds in so many different ways.  I am praying for strength, patience, the ability to cope with whatever comes our way and that this experience draws us together even closer than what we already are.

It is so hard for me to think about putting Sam through all of this, I would do anything if I could take his place and spare him the pain and the frustration.  I know that God will get us through, I have faith that he is listening, guiding and loving us beyond measure.  We will make sure the holidays are special this year.  This week we are thankful for family, friends and a plan that we hope will restore Sam's mobility. I am counting my blessings, thanking God for his comfort and support, enjoying every waking moment with a little boy that is continually teaching me life lessons that I never would have learned on my own.  I love you Sam Mayer...with all my heart...forever and ever....Amen!!!


  1. I'll be praying for you & Sam & that he is pain free after this procedure.


  2. *Hugs to you both* I am sure the trip was physically and emotionally exhausting! Sounds like at least there is a plan in action to get Sam feeling better!!
    lots of hugs, love, and prayers to all of you
    Amanda Sweeney

    1. Hugs right back at ya Amanda! Keeping you and your family in our prayers too!!

  3. Praying for your whole family.

    Glad there is a new plan with hope for pain free walking for Sam!
    Please let us know what we can do y
    To support you all through this.
    Prayers and blessings to you all.
    -Anne Calzone

    1. Lots of prayers Anne!! Still wrapping my head around all this and starting the process of planning and organizing.

  5. You will be in our constant prayers. Jeanette

  6. Hi Sue,
    Greetings from Baltimore! I read your post with a mix of tears, smiles, dread and hope. Although it will be a tough journey, I am glad to hear that Sam's hip is in a rescue mode.

    Here are some things to consider as you prepare for Sam's surgery:

    Get a back brace for yourself- you will be doing lots of bending and lifting and your back will need the extra support.

    My husband found a back scratcher at Sinai's gift shop that my son has found extremely useful in relieving the itching the pain medications cause. It extends so he can reach his legs and his back.

    Being familiar with a urinal and knowing how to use it will save Sam a lot of trips to the bathroom.

    The toilets at Hackerman Patz house are low. Ask if you can borrow a commode from the RIAO.

    Valium does work wonders with muscle cramping, and eases some of the anxiety.

    I have nothing but wonderful things to say about Dr. Standard and his team, as well as the nurses at Sinai and the staff at Hackerman Patz. They have been knowledgable, compassionate, and caring. Sam will be in good hands.

    In anticipation of my son's surgeries, there have been many sleepless nights when your gift of words have brought me comfort and hope. I am thankful that I had the opportunity to meet Sam and express to you my appreciation in person.

    Have a wonderful Thanksgiving. Please don't hesitate to contact me. I will be thinking of you and your family.

    Veronica (from Oregon)

    1. Thanks Veronica, it was so nice to meet you. When I started my blog it was my therapy, getting my thoughts and feelings out but I love hearing that others have found encouragement from it too. We had a wonderful Thanksgiving and will put all our focus on Christmas and try not to think about January too much. May God bless you and your family this holiday season!!

  7. Wow. Sam, you and your entire family will be in our prayers. Praying for a miraculous surgery and recovery. -Cass

    P.S. you are AMAZING... and so is Sam:-)

  8. Sue, I hope your Thanksgiving was wonderful. We are praying every day for Sam and your entire family. Love, Audrey

    1. Audrey, thank you my friend. Sorry for the lack of communication but as you are is a little hectic at times. Focusing on one day at a time and enjoying each and every one!!

  9. Sue, I finally got a chance to read this post. I will be praying for you and Sam. Take care.

    Jeanne S.