A neuromuscular condition has caused you to develop 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 the possible treatments for it. 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 surgeon.

What is Scoliosis?
Scoliosis is a problem with your backbone, or spine, where it begins to bend sideways and twist. Scoliosis can happen for many different reasons. Neuromuscular diseases can cause scoliosis because the muscles that support the spine start to weaken and can no longer hold your back in its usual straight up and down position. 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.

Why Worry About Scoliosis?
Some people with neuromuscular conditions will develop scoliosis. Due to muscle weakness in the back and chest, you may no longer keep your spine in an upright, straight position. As the curve gets bigger, it changes the way you sit and where the pressure points are underneath you when you sit. These changes require frequent modifications of your wheelchair (if you are using one) 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
If your curvature is not too large or growing too fast, the doctors may recommend a back brace. Bracing may be an option if you have a neuromuscular condition other than Duchenne Muscular Dystrophy. The use of a brace while you're young can help keep the curve from getting too big until you go through your growth spurt in your early teens. Back braces may have to be changed as you grow and your spine changes.

If the doctors feel bracing will not be helpful, or if your curve is still getting bigger even after you wear a brace, sometimes they will recommend surgery. Surgery will straighten your spine and to prevent the curve from getting any worse. The doctors decide it's time for surgery when your curve gets to a certain size, which is usually about 45-50 degrees.

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 Surgery
We know that people with scoliosis and neuromuscular conditions 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, surgery can improve your breathing and prevent problems like pneumonia. Also, straightening your spine will help you keep your balance better, help prevent back pain and pain while sitting, which will allow 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 your surgery. Some of these risks include the possibility of infection, or problems 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 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 alongside 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 Your "Work Up" 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 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. The wires will 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 about that. You'll talk 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.

You will be instructed on a small breathing device (incentive spirometer or voludyne), that you'll use after surgery. This device will assist you to breath deeply and cough to clear your lungs. Deep breathing and coughing will decrease the chance of pneumonia. 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 review leg and ankle exercises to keep blood moving through your legs. The nurse will also discuss methods of pain relief, including the use of a PCA pump (patient controlled analgesia) to give you pain medicine through a needle into your vein You will be given a booklet in the Orthopaedic Clinic on PCA pumps prior to surgery.

Scales for rating your pain will be shown to you and you will be asked to choose one to use you are in the hospital.

The operating room people will call you the day before your surgery to let you know what time you need to be at the hospital. You do not need to spend the night before your operation in the hospital.

The nurses will give you special scrub brushes to scrub your back and right hip. You'll need to do this for 5 minutes both the night before surgery and in the morning before you come to the hospital. This will help remove any germs on your skin and cut down on the risk of infection during your surgery. The scrub brush has iodine soap in it, so if you're allergic to iodine let us know so we can give you a different type of soap. You can do the scrubs in the shower or tub, but will need help from another person to make sure you get your whole back clean. You will need to wash your hair the night before surgery, and remove any finger or toenail polish.

Your stomach needs to be empty when you go to sleep, and so 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 this could be a potential source of infection.

Make sure all your questions are answered before you leave the hospital on your work up day.

The Day of Surgery
Before you come to the hospital, you 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 (Elevator H). The nurse will record your vital signs (temperature, pulse and blood pressure), talk briefly with you and apply 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 them 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 breath. 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 from your bladder so the nurses can measure it and keep track of how well your body is getting rid of fluids. This tube will be removed on Post Op Day 3 (three days after your surgery).

A drain is placed in the incision during surgery. This is called a Hemovac. It's a small tube that drains excess 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 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. Let the nurses know if you feel sick to your stomach (this isn't unusual after surgery). Medicine can be given to make you more comfortable if you have pain or are feeling sick to your stomach.

You will get 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 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 will 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, usually 4 to 5 days after surgery.

During surgery the anesthetic slows the motion of your intestines and may cause you to be nauseated or even vomit after surgery. The nurses and doctors will listen to your stomach every few hours to determine if your bowels are working.

You may not drink anything until the doctor determines 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. You're 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.

Antibiotics will also be given through your IV. The antibiotics will 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 applied to 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. The nurses will help you onto your back or your side using pillows to help keep you comfortable.

You'll be sitting up in bed on 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 stand to get into a wheelchair. If you are unable to stand, 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.

Your parents and/or caregiver will be taught 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 any questions you might have.

Going Home
You'll have some more x-rays before you go home, 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. Your own wheelchair may be used if the back reclines 60 degrees and the arms can be removed. If your chair does not do this, we can help you rent one. The rental chair will be a standard wheelchair. It will not have any pads or supports to help you sit, and so you will have to use pillows for support.

If you are unable to stand, you will be lifted into the chair by 3-6 people, depending on how much you weigh. At home, your parents or caregiver may lift you by the "fireman" method, and we'll teach them how to do this. Or, you could use a mechanical lift. You may not be lifted under your arms for 6 months or until your spine heals completely.

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.

You'll get a prescription for pain medicine for when you go home. 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 who help you may need instructions on how to lift you if you cannot stand. 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 your discharge from the hospital, 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
• increase 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 nurses can be reached at (319) 356-3844 (Orthopaedic Clinic). 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.