Muscular dystrophy has caused a curvature of your spine called
scoliosis. Scoliosis and other back problems are treated by a bone
and joint specialist called an "orthopaedic surgeon". The purpose of
this booklet is to give you some information concerning scoliosis and
possible treatments. We hope that knowing a little bit about
scoliosis treatment will help you and your parents be better prepared
for your visit with the orthopaedic doctor.
What Is Muscular Dystrophy?
You have a muscle disease called Muscular Dystrophy (MD). MD is an
inherited disorder that produces a progressive weakening of your
muscles, making it difficult for you to control how you move and hold
What is Scoliosis?
Scoliosis is a problem with your backbone, or spine, causing it to
bend sideways and twist. Scoliosis can happen for many different
Neuromuscular diseases, like MD, can cause scoliosis because the
muscles that support the spine start to weaken and can no longer hold
it straight up and down. Scoliosis can occur in either the upper back
(thoracic), lower back (lumbar) or, very rarely, in the neck
(cervical region). Scoliosis can develop slowly or quickly depending
on its cause. Patients with Duchenne Muscular Dystrophy (DMD) usually
have faster progressing curves than people with other kinds of
Why Worry About Scoliosis?
Up to 90% of people with DMD will develop a severe scoliosis. The
curve generally begins shortly after a person can no longer easily
walk and needs to use a wheelchair. This usually happens at about 10
years of age. Due to muscle weakness in the back and chest, a person
with DMD may no longer keep the spine in an upright, straight
position. As the curve gets bigger, it changes the way you sit in
your chair and where the pressure points are underneath you. If you
use a wheelchair these changes require frequent mod)fications of your
chair to keep you well supported, prevent skin problems, and to keep
you as independent as possible. While your chair can help support
your spine, it cannot stop the progression of the curve. We also
worry about scoliosis because if the curve becomes too large it can
crowd your heart and lungs, making it hard for you to breathe
properly. This may cause lung problems like pneumonia.
Bracing is not a treatment option for DMD. The treatment of choice
for DMD is surgical correction of the curvature. Surgery is done to
straighten your spine and to prevent the curve from getting any
worse. The doctors decide it's time for surgery when the curve gets
to a certain size, which is usually around 25 degrees in patients
with DMD. We do surgery earlier in children with DMD than in other
conditions, because we know from research and experience that if a
curve reaches 25 degrees in a person with DMD, it will almost
certainly continue to get bigger. Deciding to have surgery is a big
decision and can be very frightening for you and your family. This
booklet was prepared to answer your questions about surgery if you
and your doctor decide it is necessary. If you have any further
questions, please write them down so you will remember to ask your
Advantages of Early Sugery
We know that people with scoliosis and muscular dystrophy can develop
problems with their lungs, problems with sitting balance and with
back pain. Having your spine straightened before these conditions
develop may save you lots of problems later on. If you are already
having lung problems, having surgery can improve your breathing and
help prevent problems like pneumonia. Also, straightening your spine
will help you keep your balance better, help prevent back pain, and
pain while sitting, allowing you to sit comfortably for longer
periods of time. All of this can improve your quality of life.
Changes and Risks Related to Surgery
As a result of your surgery, you might be taller because your spine
will be straighter. Being taller may make it harder for you to fit
into your car or van. Being taller may also interfere with eating
because your arms must move a longer way to get to your mouth. There
are always risks involved with the surgery itself and with being put
to sleep for surgery. Some of these risks include the possibility of
infection or a problem with the metal rod the doctor will attach to
your spine. The chance that these complications will occur is very
low, and we do everything we can to prevent them. The doctors and
nurses will make sure you know what the risks are and will answer all
your questions before your surgery.
How the Surgery Works
The goal of surgery is to straighten your spine to prevent
breathing and sitting problems. To do this, the surgeon will use a
long curved stainless steel rod, called a Luque (loo-key) rod. The
doctors attach the rod to your spine. In most patients, the rod is
attached to the back of the pelvis. This is done if the hips are
slanted or tipped. We look at the x-rays we took before your surgery
to determine where your curve starts and where it ends, and also what
shape the rod should be. That way we know where on your spine to
attach the rods. Bone from the bone "bank" is placed along the side
of the rod. This bone will grow into the spaces between your
backbones (vertebrae) and will hold them straight. This is called a
"spinal fusion." Until these bones heal together, they need to be
supported and kept from curving again. This is the purpose of the
Luque rod. The rod is attached to your backbone and holds everything
straight until the bones are fused together.
All patients lose some blood during surgery, and sometimes you need
to get some back afterward. You will get blood either from our blood
bank, or, if you're medically able, you may donate your own blood
ahead of time. This is called "autologous donation." The doctors and
nurses will talk to you about donating and receiving blood when you
set up your surgery date.
What Happens on My "Workup" Day?
One to three days before surgery you will come to the Orthopaedic
Clinic for what we call your "pre-op workup." You can plan on being
at the hospital approximately five hours on this day. The doctor will
listen to your heart and lungs, and in general make sure you are in
good shape for surgery. If you are sick on this day, or a few days
before, notify the doctor, as he may decide to postpone your surgery
until a later day.
The nurses and doctors will ask you and your parents some
questions and answer any questions that you may have. This will
enable them to give you the best care while you are hospitalized.
Urine and blood samples will be obtained. After the blood sample
is taken, a bracelet will be applied to your wrist or sent home with
you. It is very important to keep the band clean and dry so that your
name and hospital number are legible. Remember to bring the band with
you on your surgery day, or another blood sample will need to be
You will have several x-rays taken.
A medical photographer will take some pictures of you and your
back, so we can compare how you look before and after surgery. You
may need to go to a special laboratory for an EEG
(electroencephalogram) so they can check the nerve messages going
through your spine before and during your surgery. They do this by
attaching special wires to your head and your legs. This doesn't
hurt. They'll be removed after this first test, and then put back on
the day of your operation.
You will also spend some time talking to the surgeon who will do
your operation, as well as to the doctor who will be putting you to
sleep. This doctor, the "anesthesiologist" will explain to you how
they put you to sleep, and will answer any questions you might have.
They will ask you about any medications that you take on a daily
basis. The anesthesiologist may instruct you to take these
medications with a small sip of water the morning of your operation.
You should tell the doctors or nurses about any allergies you may
have to medications, foods, tape or latex (rubber products).
The nurses will describe what will happen the day of your surgery
and give you general information about being in the hospital. You
should make plans to be in the hospital for 7 days.
We will teach you how to use a small breathing device called an
"incentive spirometer" or "voludyne." This device will assist you
with breathing deeply and coughing to clear your lungs after surgery,
which decreases the chance of pneumonia. Also after surgery, you will
roll side to side without bending or twisting your back. This is
called logrolling (turning as a unit). The nurses will help you, but
it is a good idea to practice this before surgery. The nurse will
discuss methods of pain control to be used after your surgery,
including the use of a machine called a patientcontrolled analgesia
pump (PCA). We'll show you scales for rating your pain and you will
be asked to choose one that you'd like to use while you are in the
The operating room staff will call you the day before your surgery
to tell you what time you need to be at the hospital. You don't need
to spend the night before your operation in the hospital.
The nurses will also give you a special scrub brush to scrub your
back and right hip the night before surgery. This will help remove
any germs on your skin and cut down the risk of infection during your
surgery. If you are allergic to iodine, please let us know and we can
give you some other kind of soap. You can do the scrubs in the shower
or tub, but will need help from another person to get all your back
clean. You'll also need to wash your hair and remove any finger or
Your stomach needs to be empty when you go to sleep; therefore,
you may not eat or drink anything after midnight the night before
surgery. This will help keep your stomach from getting upset
afterwards. You may want to avoid salty foods the night before
surgery to prevent waking up really thirsty. You cannot have a
sunburn, bad rash, or sores on your back at the time of surgery, as
they could lead to an infection.
Make sure all your questions are answered before you leave the
hospital on your workup day.
The Day of Surgery
Before you come to the hospital, you'll need to wash your back again
with another scrub brush for five minutes. Brushing your teeth and
rinsing out your mouth is OK, but do not swallow the water. Do not
When you get to the hospital, you'll need to check in at the
pre-surgical area on the 5th floor (by Elevator H). The nurse will
record your vital signs (temperature, pulse and blood pressure), talk
briefly with you and put on your identification and blood bands.
You'll get into a hospital gown and the nurses will help you onto a
special bed. From here you will go back to the EEG lab, where they
will put the special monitoring wires on your head. Sometimes they
will put these wires on in the operating room. The operating room
transport person will take you to the 5th Floor Presurgical Care Unit
(PSCU) when it is time for your surgery.
While you are in surgery, your family can wait for you in the
Boulware Day of Surgery Lounge (Elevator H to 6th floor). The doctors
will talk to them periodically during and after your operation and
let them know how you're doing.
A nurse will greet you as you arrive in the operating room. The
room is sometimes cool and noisy. Please let the nurses know if you
are not comfortable. Warm blankets are available. In the operating
room, you will be connected to many monitors and a needle will be put
into a vein in your arm. This is called an "IV." The anesthesiologist
will give you medications to put you to sleep. Once you are asleep,
the doctor will begin the operation. A mask may be placed over your
nose and mouth to help you breathe. After you are asleep, a tube is
placed in the back of your mouth and throat to provide air to your
lungs. This tube will be removed before you wake up. You will lie on
your stomach on the operating bed with your arms and legs supported
with pads. Your back will be scrubbed before starting the operation.
The operation generally takes 4 hours, but you will be in the
operating room a total of 4-5 hours.
A tube, called a Foley catheter, is placed in your bladder while
The Foley drains urine out of your bladder so the nurses can
measure it, and keep track of how well your body is getting rid of
fluid. This tube will be removed on Post-Op Day 3.
A drain is placed in the incision during surgery. This is called a
'hemovac'. It's a small tube that drains extra fluid from your back
into a little collection container. The doctors will remove it on
Post-Op Day 2. There is a mild burning feeling when the drain is
Post Anesthesia Care Unit (PACU or Recovery Room)
When you wake up from surgery you will be lying on your back in the
recovery room area. You'll already be in your bed. You may feel stiff
from being in one position for longer than normal. A nurse will check
you frequently and make you comfortable with warm blankets. You will
receive oxygen and be encouraged to take deep breaths to help your
The nurse may ask you to rate your pain on a scale of 1 to 10. If
you feel sick to your stomach let the nurse know. You can have
medicine to make you more comfortable if you have pain or are feeling
sick to your stomach.
You will have some more x-rays while you are in the recovery room.
You will be in the recovery room for 2-4 hours, or until the
anesthesiologist says you're awake enough and doing fine. If you are
16 or younger, your parents or 2 adults may see you in the recovery
If the doctor thinks you need to be hooked up to monitors
overnight (in case of breathing or heart problems) you will go to the
Pediatric Intensive Care Unit (PICU). Your parents may see you in the
PICU. Your doctor will decide when you are ready to come to your room
on the Orthopaedic Unit from either the PACU or PICU. You will be
taken to the Orthopaedic Unit in your bed.
Orthopaedic Unit Surgery After Surgery
After surgery the nurses will frequently take your vital signs (blood
pressure, temperature, respiration rate, and pulse). This is so
important that they even wake you up during the night to do this.
The doctors and nurses will be touching your hands and feet, and
asking if you have any numbness or sleepy sensations in your arms or
legs. Let the nurse or doctor know if your arms and legs tingle or
feel numb or just plain funny.
The IV that was started in the operating room will give you fluids
during and after surgery. The IV will remain in until you are able to
eat and drink which is usually around 4 to 5 days after surgery.
The anesthesia medicine slows the motion of your intestines and
may cause you to be nauseated or even vomit after surgery. The nurses
will listen to your stomach every few hours to hear if your bowels
are working. You may not drink anything until the we determine that
your bowels are returning to normal. This may take up to 4-5 days.
You may rinse your mouth out with water and brush your teeth. If you
drink or eat before your bowels are ready, you may become nauseated
You'll have pain medicine to keep you comfortable. Your IV will be
hooked up to a PCA (patient controlled analgesia) pump that has a
tube of pain medicine inside. This syringe is attached to your IV
line and continuously gives you a small amount of pain medicine. If
you still are uncomfortable, there is a button to push to give you a
little extra pain medicine. If you are unable to push this button, a
family member or the nurse may push it for you. You may need an
occasional shot in the muscle for pain along with the PCA. This
depends on how severe your pain is. Your PCA will continue until your
stomach wakes up and you are able to drink and take pain medicine by
You'll also get antibiotics through your IV line to decrease the
chance of infection. The antibiotics will continue until one dose
after your hemovac drain and foley catheter are removed. We'll take a
sample of blood from you each morning after surgery for 3 days to
check your blood count. If you're short on red blood cells, it might
be necessary to give you a blood transfusion.
In the operating room a big bandage is put on your back. This will
be changed on Post-Op Day 2 and removed on Post-Op Day 3. The
stitches used to close your wound are under your skin. Your body will
just absorb them, so they do not have to be removed. Pieces of tape
called "steri-strips" are placed over your incision after surgery,
and will gradually fall off on their own after you go home.
The day of surgery you will be flat in bed. The nursing staff will
help you move from side to side by "logrolling" every 2 to 4 hours.
When turning, your shoulders and hips must go all at the same time,
like your back is one big log. We'll help you into a position either
on your back or your side and prop you up with pillows to keep you
You'll be sitting up in bed the first day after surgery. We'll
make sure you sit up 3 times that day to get your body used to
sitting up again. The second day after surgery you will be carefully
lifted out of bed into a wheelchair.
A Child Life Specialist will come to your room and offer you
activities to do while you are in bed. When you can be up and out of
your room, you will be invited to attend group activities in the
We'll teach your parents and/or caregiver how to take care of you.
The nursing staff will encourage them - and you - to do as much as
you are comfortable with. Please ask the nurses about anything that
you may have a question about.
Before you go home, we'll take some X-rays while you're sitting,
which is usually in 5-7 days after surgery if no problems arise. No
cast or brace is usually necessary following surgery.
After surgery you can sit at only a 60 degree angle for 6 weeks.
You can use your own wheelchair if the back reclines 60 degrees and
the arms can be removed. If not, we can help you rent one. The rental
chair will be a standard wheelchair without any pads or supports to
help you sit, and so you will have to use pillows for support.
While you're in the hospital, you will be lifted into the chair by
3-6 people, depending on how much you weigh. We'll teach your parents
or caregiver how to lift you by the "fireman" method when you get
home. Or, you could use a mechanical lift. You may not be lifted
under your arms for 6 months or until your spine heals completely.
In the days right after your surgery, the nurses or a parent will
give you a bed bath. Keep your incision clean and dry. The
steri-strips will gradually fall off. You may shower two weeks after
surgery if your back incision is well healed and has no drainage.
Direct the water stream on your front and shoulders, letting it
trickle down your back. Do not direct the water stream on your back
incision. You may not get into a tub or swimming pool for 6 months
after surgery. Your back incision may be numb for 4 to 6 months.
We'll send you home with a prescription for pain medicine. You
should also continue taking your iron as you were before surgery.
You may return to school in 2-4 weeks depending on how you feel.
The people at school may need instructions on how to lift you. They
may also need to know if there are changes in your height so
adjustments can be made (for example, your desk may need to be
Your first return appointment will be about 6 weeks after you go
home, then 4 months, 6 months, and one year after surgery, and then
every year after that. It's really important that you return for your
If any of the following problems occur before your appointment,
notify your doctor right away:
- fever, chills, redness, warmth, or foul-smelling drainage at the surgical site
- increased in pain
- numbness, tingling, or increased
- weakness in your arms or legs
- change in bowel or bladder control.
We hope you've found this booklet helpful. Please let us know if
you have any questions or concerns. The orthopaedic clinic nurses can
be reached at (319) 356-3844. The orthopaedic surgeon can be reached
at (319) 356-1872, 8 a.m. to 5 p.m., Monday through Friday. If you
have urgent questions at any other times, please call (319) 356-1616,
and ask to speak with the orthopaedic resident on call. If you need
to change or confirm your return appointment, call Orthopaedic
Scheduling at (319) 356-2223.