Wednesday, November 3, 2010

Ooohhh, Sammy!

All I can say is Sam is a trouper! He has had a virus since last week which seemed to get worse in the last couple of days. I took him in to see his favorite doctor (and that is a compliment if you realize how many doctors Sam has) Dr. Steinert. Sam never tells you what is wrong. If you ask him the standard answer is "All better". But with Sam it has always been diagnosis through observation. Sam is rubbing his face, his eyes are gooey, he refuses to wear his hat with his hearing aid, he is running a low grade fever, his tongue is out more than usual, his breath is nasty and he overall sounds gunky but he's laughing, smiling, walking around and still managing to do some of his homework with me. His personality and overall demeanor is excellent. Sam is having hip pain from his Perthes and now...he has a sinus infection too.

I don't know about you but a sinus infection makes me curl up in bed and not want to relate with the world and yet this little guy...all I can say is "Wow". He's limping and he looks kinda beat up but look at that smile.

He's lounging on the hospital bed we recently moved into our parlor, as we prepare for his upcoming surgery. Look at the position of that right leg...and then I wonder why he has hip dysplasia???

I recently took part in a Perthes chat group with Dr. Shawn Standard an expert in the treatment of Perthes and hip dysplasia. Here is his bio for anyone looking for help with their child's hips or limbs:

Dr. Shawn C. Standard is Head of Pediatric Orthopedics and the ICLL (International Center for Limb Lengthening). Originally from Atlanta, Georgia, Dr. Standard came to the ICLL in 2003 from the distinguished Nemours Clinic in Jacksonville, Florida, where he started a combined general pediatric orthopedic and limb deformity clinic. He earned his medical degree in 1995 at the Medical College of Georgia in Augusta. He completed his orthopedic residency training at the Medical College of Georgia in July 2000 and a fellowship in pediatric orthopedic surgery at Nemours, concentrating on general pediatric orthopedics with a special interest in limb deformity correction and limb lengthening. At the ICLL, he has established a clinic with concentrations in pediatric limb deformity correction and limb lengthening, clubfoot and other congenital deformities, general developmental disorders of the pediatric musculoskeletal system, and pediatric orthopedic trauma care. Dr. Standard has special interest in radial clubhand, Perthes disease, and congenital leg length discrepancy. Dr. Standard is married and has three children. His after hours job is coach of his son's pee wee football team. Dr. Standard donates one week of his vacation each year as a volunteer orthopedic surgeon at the Caribbean island of Grenada and the Dominican Republic.

Pediatric Conditions Treated by Dr. Standard

Dr. Standard helps children who have a variety of orthopedic conditions:
Lower Extremity Conditions
Perthes disease and avascular necrosis of the hip
Achondroplasia and other forms of dwarfism
Fibular hemimelia (absent or short fibula)
Congenital femoral deficiency or proximal focal femoral deficiency (short femur)
Tibial hemimelia (absent or short tibia)
Limb length discrepancy (this occurs when one leg is shorter than the other)
Short femur, tibia, or fibula
Blount disease
Ambulatory cerebral palsy (deformities and contractures)
Nonunions and malunions (this occurs when a fractured bone does not heal correctly)
Hip deformities
Hip dysplasia (congenital or developmental)
Knee or patellar deformities
Knock knees (genu valgum)
Bow legs (genu varum)
Joint contractures (e.g., pterygium syndrome, arthrogryposis)
Foot and ankle deformities
Bone infection (e.g., osteomyelitis, bone defects)
Slipped capital femoral epiphysis (SCFE) and femoral acetabular impingement
Congenital pseudarthrosis of the tibia
Growth arrest
Bone tumors
Metabolic bone diseases
Fractures of the bones in the legs

Here is our chat:

Sue Mayer: Hi Dr. Standard, Sam is going to be 10 years old in December and has Down syndrome, a brain injury, apraxia, dysphagia, respiratory/immune issues, bi-lateral conductive hearing loss and now Perthes. I emailed Sam’s x-ray a week or so ago. I wanted to hear your opinion of the x-ray. Sam is currently scheduled for a femur varus derotation ostetomy and a possible dega osteotomy. Sam will be in a Newport Jr. Hip System for 2 or more months after the surgery. I am worried about a lot of things but wanted to hear your opinion and what are the options if a leg is shorter than the other after the surgery?
Dr. Standard: Sue- let me check email
Sue Mayer: Okay.
Dr. Standard: Sue- has Sam had any other hip surgery in the past
Sue Mayer: No
Dr. Standard: the xray is just of his left hip- how is his right
Sue Mayer: His right is also shallow with some of the same choppiness but no signs of Perthes
Dr. Standard: Sam has a very complex and special situation (do you hear my heart breaking yet again)- he has severe dysplasia of the hip joint with subluxation / dislocation of the femoral head and changes consistent with avascular necrosis (perthes)- this type of hip configuration and correction is very complex- I am performing a surgery just like this next week on another Down's patient
Dr. Standard: the perthes / avascular necrosis is secondary at this point to the hip position / configuration
Sue Mayer: Is the surgery you are performing the same as the doctor is doing here?
Sue Mayer: Do you agree with the surgery our doctor is proposing?
Sue Mayer: By the way, everything about Sam is special and complex...that I understand.
Dr. Standard: similar-use a different approach that we use for a complex congenital femoral disorder patients in order to reduce the hip - the bone work is similar- I also plan an open reduction of the hip joint to tighten the capsule- unfortunately, the laxity in Down's syndrome can always defeat you-
Dr. Standard: Since there is the presence of avn / perthes- I would consider a small diameter core decompression at the same setting to speed the healing process of the femoral head
Sue Mayer: Will my doctor know what that means, because I don't think I do?
Dr. Standard: concurrently- early and gentle range of motion will be needed - NO Cast
Sue Mayer: They are not casting him, he will be in the Newport Jr. Hip System.
Dr. Standard: maybe- I would be happy to discuss his case with your doctor-who are you seeing now
Sue Mayer: Dr. Thometz, Head of Pediatrics for Children's Hospital Milwaukee Wisconsin
Dr. Standard: I do not know Dr. Thometz personally- would be happy to discuss ideas if it would help- but I think at this point, I agree that the main issue is to reduce the hip and reconstruct the hip cup
Sue Mayer: We are trying to move up the surgery from December 7th due to Sam tending to get ill as we get further into winter. What about the leg length issue?
Sue Mayer: reduce the hip and reconstruct the hip cup, Is that what the two surgery's proposed will be doing?
Dr. Standard: also secondary to hip position- first save the hip and then figure out an equalization plan for the leg lengths if it becomes an issue
Dr. Standard: yes
Sue Mayer: What do you mean by an equalization plan, I heard mention of an attempt to shorten the other leg, why wouldn't you lengthen a leg?
Dr. Standard: depends on the actual amount- lengthening can be performed but much more involved than a gradual shortening- meaning a slowing of the longer leg- also if you have a hip at significant risk - then lengthening in the face of a compromised joint is asking for trouble- need to weigh the risks and benefits when you reach that point- but that issue is down the road
Sue Mayer: It's a lot to think about for a very special little boy. I hope everything goes well and we are praying alot. Thank you for taking the time to review Sam's x-ray. I appreciate your input. I am done now.
Dr. Standard: take care and tell Sam good luck :)

As you can probably pick up from our chat...Sam's diagnosis is not a good one. This is not a quick fix. Sam will have the surgery and then be in the brace pictured below for two months or more as we wait for the top of the femur to grow back. Oh, except Sam's brace extends down to his calf on his left leg and he will have to remain in bed for those two months since he has balance issues and he can not put any weight on his left leg.

Sam will have to go through PT to facilitate walking again and may have one leg longer than the other. In a year he will have a second surgery to remove all the hardware. He will then have another surgery to shorten the growth plate of the longer leg. And this is if everything goes well.

His pulmonologist, Dr. Daiva (another favorite doctor) has asked that he be admitted into the ICU after surgery so she can monitor his respiratory system. Sam will be under anesthesia for 6 to 8 hours and he doesn't come out of anesthesia well. He will require help to clear his lungs and heal his already compromised upper respiratory tract. Sam's immune system is weak and that is why we have asked that the surgery be moved up if possible. As we get further into winter he tends to get sick easier.

Sam will require 24 hour care for 2 months or more. Sure he'll sleep at night (I hope) but Sam tends to be restless, he used to bending himself in half at night. The brace will keep him from doing that and restrict his movement but Sam can be creative in his sleep as well as when he's awake and we may need to add an extension to the right leg to keep him from trying to turn over on his own or attempt to get out of bed. Sam will need to be fed, bathed and use a bedpan/urinal for the 2 months he is in bed...and that's only his basic needs. I still need to figure out how to keep him entertained, happy, healthy and continue his education. Then let's not forget there are two other children and one of those needs my help with his schooling, my daughter's activities, keeping my husband on schedule and "Oh Yeah" finding time for me???? I think I start to chuckle here!!

Remember how earlier I talked about curling up into a little ball and ignoring the world around me when I have a sinus infection...kinda feeling the same way.

But then I look at Sam.

Today we cuddled up in his hospital bed and watched movies together. I stroked his back and tummy and played with his hair and enjoyed all his smiles and laughter. Sure I had housework to do...I had lessons to prepare...but none of that mattered when Sam asked me to come sit with him. And no, I can't curl up into a little ball and let life pass me by...Sam is counting on me. I need to proceed one day at a time...sometimes one moment at a time and always remember that I'm not in this alone. I have my faith, a wonderful supportive family, I have fantastic friends, and I have a little boy that challenges me to live by his example.

Tomorrow is another day that I get to love Sam learn, enjoy his smiles and laughter, accept each and every hug he offers and remember to hug him twice as much. I have a well known routine with my children, I ask them "Have I told you lately??...." and they answer "I love you!" and I tell them "I love you more!" Our journey has built our strength and each day I challenge myself to find the blessings, to not waste time worrying and to enjoy each and every moment given to me. Life is still good...overwhelmingly good!


  1. Sue, I am glad you are getting the expert information you need. I am praying for Sam and you. I would be happy to come during the school days sometimes and sit and play with Sam, watch some TV, etc, while you run errands and/or sleep. I have during the day time. Hang in there and lots of virtual hugs. Remember Sue, Sam is a lucky child. He has you as a mom, lives in this country, and will get great medical care. Hugs again.

  2. Oh Sue, ((((hugs))))
    You and Sam are amazing! Sam see's the blessings in everything, everyday! That is where you get your strength and will continue to do so throughout this process! You have God and an amazing Angel, Sam!
    In peace and prayers to you and your family!

  3. Sue, thank you for sharing this information. Your blog was given to me by another mom of a child with Down syndrome. (I believe you also friended me on Facebook). My Joseph was just diagnosed with Perthes this past week. He is just 3 right now, I am sure I will learn much more as the years pass. I will be praying for you and Sam. God bless, and thank you so much for sharing this