Tetralogy of Fallot and Scoliosis: 1 in 10,000

I really love it when people ask me, “Is Abby done with heart surgeries?”

To be honest, the answer to that question is we hope so…at least OPEN heart surgery. Dr. Kaza at Primary Children’s was able to put a valve that technically should last Abby through her full grown body. Of course, replacement valves wear out but, he is confident that by the time Abby needs another valve replacement, it will be done through a vein in her leg in the cath lab! So, while we feel so relieved that our little warrior has fought this battle with CHD so valiantly, Abby has an additional congenital defect we have to deal with–Congenital Scoliosis.

I have learned that in children with Congenital Heart Defects, especially Tetralogy of Fallot, 23% will have congenital scoliosis as well. In the general population 1 in 10,000 children will be affected by congenital scoliosis. This makes information less common to come by, additionally, most people confuse CONGENITAL scoliosis with FUNCTIONAL scoliosis. They are different and will require radically different treatment.

FUNCTIONAL SCOLIOSIS is a much more common scoliosis. This is the type of scoliosis they test for in Jr. High and High School by having children bend down and touch their toes while their spine is examined. Functional scoliosis is caused by a factor outside the spine, meaning that a muscle on one side of the spine develops more, pulling the spine slightly to the right or left. Or, one leg may grow slower than the other leg, causing an imbalance in the hips which pushes the spine to one side or the other. In this scoliosis they attempt to treat the cause of the curve. Most often it is treated with a back brace, shoe inserts, or chiropractic care. It is easily treated and rarely causes problems–keep in mind that the spine essentially FORMED straight in the womb.

CONGENITAL SCOLIOSIS is a different type of scoliosis. It is much less common. The word congenital means “at birth”–this type of scoliosis was present at birth and was caused by a birth defect in the spine. There are two different kinds of birth defects in the spine: A Hemivertebrae (or butterfly vertebrae), and a unilateral bar. The hemivertebrae is where one side of the vertebrae forms and the other does not. The unilateral bar is when a number of the vertebrae are fused together on one side so the spine grows lopsided, so to speak. Congenital scoliosis requires surgery to correct since bracing can in no way fix the deformed bones. In a small percentage of children, congenital scoliosis may be outgrown or the curve may remain stable. In 70% of cases, the curve progresses as the child grows and will require surgery to ensure that the child can walk uprightly and has no problems with other organs in the chest cavity.

Abby has both Hemivertebrae and unilateral bar defects in her spine. The picture above looks very similar to Abby’s X-ray of her spine. She has a curve of 53% (which is considered severe) in her mid-back where she has unilateral bar and a smaller curve in the upper back area where she has Hemivertebrae. We are now looking at surgery to correct this defect around 12-years-old or so. One-and-a-half years time.

This surgery always felt far away…and for some time it sounded like it might be possible for her orthopedic surgeon to remove some growth plates on one side to correct the curve; however, at our last visit it became clear that her curve is progressing faster than he likes and is already a severe curve. Her surgery will require fusing of the vertebrae affected with bone grafts and metal rods and screws. The ICU stay is 2-4 days and the total hospital stay is 5-7 days. Children are then required to wear a brace for 6-9 months to protect the spine while it heals. Additionally, some patients require rehab therapy for 1-2 weeks after the surgery to help with walking, moving, and loosening the spine. This rehab is usually done with the child sleeping at the rehab center, although sometimes children are allowed to go home and attend therapy close to home.

Having said all that….so clinical and matter-of-factly….I want to cry when I think about this sweet girl going through ANOTHER surgery. It is true this surgery is not as “serious” as open heart surgery, although children with CHD do run a higher risk of contracting staph infection which can cause serious problems to the already repaired heart. Whether it is serious or not…it inflicts pain on her. It involves a hospital stay, which is scary to her. It requires ICU stays, arterial lines, catheters, intubation, and invasive procedures on her body. It scares her. It BREAKS MY HEART.

I would love to ask WHY? But that really doesn’t matter and I won’t get the answer answered. So, we take a deep breath. I have shed a few tears over the thought of more. I have realized that it could be worse and she could have had much more to go through like so many other heart children do. And, I remember that she is a huge ball of fire in a little body and she has a soul full of fight. She can do it and so can I. So here’s looking ahead to yet one more mountain Abby must climb…and that I will follow her to the top and over the other side.

Related posts:

1. Tetralogy of Fallot: Compatible with Life?
2. Rylee’s journey with Tetraology of Fallot
3. Reflections of a heart mom