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 yourself upright.

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 reasons.

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 neuromuscular problems.

Figure 1

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.

Treatment
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 doctor.

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

Figure 2

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.

Before Surgery
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 taken.

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 hospital.

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 toenail polish.

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 chew gum.

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.

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 you're asleep.

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 removed.

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 lungs expand.

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 room.

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 and bloated.

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 mouth.

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 comfortable.

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 playroom.

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.

Going Home
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 higher).

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 scheduled appointments.

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.