The procedure that Abby underwent for her complex spinal deformities
is called VEPTR, Vertical Expandable Prosthetic Titanium Rib. This procedure was developed
at Christus Santa Rosa Childrens Hospital in San Antonio by Dr. Robert Campbell and his coworkers, and it is a relatively
new procedure (approx. 15yrs). This procedure has recently received Humanitarian Device Exemption approval from the FDA and
has been approved as a Humanitarian Use Device (HUD). Being considered as a HUD means that the VEPTR procedure is expected
to be used in less than 4,000 cases a year. Furthermore, it is not to be used to make a profit for the institutions that
perform the procedure. Along with this type of FDA approval comes some very strict guidelines for VEPTR's use. Specifically,
for a child to be considered a candidate for the procedure s/he must have a chest wall deformity or congential scoliosis with
fused ribs, and there must be bony deformities. If the doctors evaluating a child feel that s/he could benefit from the procedure
but s/he does not meet the criteria, then the insitutional review board could be petitioned and a "compassionate use" approval
could received; however, this is not an easy process and would only be requested and approved in extreme cases. The good
news about receiving FDA approval as mentioned above is that now more doctors at more insitutions can be trained to perform
the VEPTR procedure (after undergoing appropriate training), making it more accessible for children and their families.
We found the article "Vertical Rib Expansion For
Thoracic Insufficiency Syndrome Indications and Technique" by Lewandrowskim, Campbell, and Emans, a great article
to familiarize ourselves with the rationale behind the procedures invention and what the procedure actually entails.
Shriners Hospitals for Children in Philadelphia has been participating
in the investigational study of VEPTR implantation since May 2002, and is continuing to peform the procedure under the new
FDA approval guidelines. They have a great article about their first successful titanium rib implantation with accompanying
pictures in their own publication "Between Us" Volume 16 No. 1 April 2003.
Although the staff at Shriners did an excellent job at preparing
us for Abigail's procedure and were very open to answer any of our questions, there were small facts about the procedure that
came up along the way that we never thought about to ask. Hopefully some of the tidbits we bring up here will help enlighten
you and act as a starting point for conversation between you and your doctor. All of these tidbits were told to us in regards
to our specific child's condition; therefore, not all of this information may be exactly the same for your child. We bring
these points up for no other reason than to act as a starting point for you to think of questions you may have for your specific
child. This is not intended to replace the medical advice of your child's doctor!
*Cut through or migration of the titanium rib refers to the portion
of the "rib" that hooks onto the bone beginning to go through the bone it is attached to (because childrens bones are not
fully solidified at this young age). Dr. Campbell explained it to us as a slow sailing ship drifting through the water, the
rib drifts through the bone. Although we were aware of the possibility of cut through, we originally were not aware that the
likelihood of this occurring is at least partially based on a child's activity level. The more active young children (as opposed
to those confined to a bed or a wheelchair) tend to experience cut through more often than less active children. As explained
to us, if this occurs (which is identified either in x-ray, during an actual follow-up procedure through visual inspection,
or a "bump" under the skin in the location of the rod that was not previously present), the hook portion of the titanium rib
is moved to a different section of the same bone or on another bone altogether (determined strictly by the doctor and your
child's own individual condition).
*Most children do not need to be in a brace after surgery we were
told approximately 90% of the children do not need any stabilization after the procedure.
*Typically the hook of the device is not stabilized above the
T2 level of the child because of increased risk of damage to the nerves called the brachial plexus. These nerves are responsible
for movement in a child's arms.
*Follow-up surgeries to expand the device occur every 4 to 6 months
initially, but as the child's growth begins to slow down the expectation can be an expansion every year(this usually is between
6-8 years of age). Once the child outgrows the implanted device, a new device will be placed - this occurs approximately every
2 to 3 years (depends strictly on the individual child)
*The severity of the expansion surgeries (every 4 -6 months) was
described to be only 1/10 as involved as the original surgery (if no complications), the replacement of the rib once it has
been expanded as far as it can and needs to be replaced is about ½ as involved. The replacement surgery does not necessarily
mean the entire device is removed and replaced, but may only entail having ½ of the device replaced
*A fusion of the spine may still be required at a later time in
your child's life (around 13-14 yrs. potentially)
*Follow-up surgeries usually involve cutting on the same scar
line that already exists so no additional scars are formed post the original surgery
