One of the criteria for the diagnosis of SMA Type 2 is that the patient has reached the milestone of sitting unassisted. Ella fits that criteria well. In fact, almost her entire day is spent sitting, either independently or strapped in a wheelchair.
One of the consequences of SMA is, of course, the atrophy and weakening of muscles. When you combine muscle atrophy with non-weight bearing a host of problems can and do occur. One of those problems has to do with the hips.
The weak muscles cannot hold the hips in the proper place and over time they begin to dislocate. We've been keeping an eye on them alongside Ella's orthopedic surgeon for the past year now.
Historically speaking kids with SMA Type 2 wouldn't have had hip surgery for the simple reason that if there was no pain associated with the dislocation the call for surgery was unwarranted. Doctors simply "kept an eye on them".
So what's changed?
Survival.
Kids with Type 2 SMA are surviving longer these days with improved care. And while kids before were not experiencing pain, many of them weren't living long enough to get to the point when there would be pain. Not so true anymore.
If Ella's hips go untreated they will continue to dislocate; producing a pronounced pelvic tilt, contributing to scoliosis, and causing pain...much pain. Not to mention thwarting her ability to sit independently. In fact, she has "tipped" over a few times over the past year---just sitting there and "boom", down she goes.
Not only are we looking to the future in terms of pain, scoliosis, and independence...we are also looking at helping her to be "mechanically sound" for when the treatments and/or cure become available...we want her in the "best position" at that time.
Not to mention the surgery itself. Right now, Ella is strong. Her respiratory system has been doing well, and her ability to recover quickly exists. Her doctor says that waiting until the hips are worse and pain is present would make for a much more complicated surgery.
So what's involved? Well here's why it's taken us so long to come to this decision:
- The surgery is 7 hours long for both hips.
- The surgeon will cut her femurs and re-position them properly.
- The femur will be held in place in the hip socket with metal brackets screwed into surrounding bone.
- Depending on the condition of the socket he may have to "create" a socket. This is because when the femur is not in the socket the socket itself tends to "flatten" out.
- The possibility of a blood transfusion exists so we will prepare for that.
- She will be intubated. This can pose a problem for kids with SMA for a variety of reasons. She's strong now and her doctors think and feel that she can be extubated successfully. This is one of our big concern areas, though.
- She will spend 4-5 days in the hospital.
- She will be immobile for at least two weeks...then we will slowly get her back to sitting.
- Regression will occur...so intense PT will commence when she's ready.
We know that there are different schools of thought when it comes to hip surgery and kids with SMA. We have--through prayer, research, discussion, and thought--come to the realization that we must do what's best for Ella's future.
We have yet to tell Ella of the upcoming surgery...that will be a discussion for the time just preceding the day of surgery.
Surgery is set for July 9th (2015).
We will share, once we know, the details of the hospital stay (4-5 days) so that those who would like to visit or send a card/something special can do so. We know that Ella would also love to have visitors at home while she recovers...play dates, movie dates, coloring dates, play-doh dates, etc!!
Ella's a fortunate girl. She is surrounded by so many people who care for her. She just turned 5 years old and has been through so much in such a short time...she is really going to need you for this one...it's a big one.
We've spent much time with her doctors...all of them...and we are confident in their opinions, amazed by their professionalism, grateful for their compassion, and trusting in their abilities to care for our Ella.
