Excerpt from http://www.mgh.harvard.edu/ortho/Pediatric_Spinal_surgery.htm

If your child require surgical correction of scoliosis, you probably have many questions and concerns. Many children’s hospitals have prepared guides to help answer your questions. The following information is from the MassGeneral Hospital for Children, Department of Pediatric Orthopaedics in Boston, MA. What follows explain the common surgical treatment for scoliosis, preparing for the surgery, and what to expect afterwards. It is important to understand that every patient and every spinal deformity is different. If you have questions specific to your surgery, make sure to discuss them in advance with your orthopaedic surgeon.

Scoliosis is defined as a lateral curvature of the spine. Scoliosis can occur in either the upper back (thoracic), lower back (lumbar), or rarely, in the neck (cervical). Scoliosis is the most common spinal deformity affecting adolescents 10-16 years of age. Most cases (about 80%) of scoliosis are idiopathic, or have no known cause. Idiopathic scoliosis is much more common in females, and is usually noticed at the onset of puberty coinciding with the "growth spurt." Scoliosis is usually discovered during regular check-ups with the pediatrician, or during school screening programs. The most common signs are asymmetry of the spine, uneven shoulders or hips, waist and ribcage asymmetry. These changes are especially noticeable when the child is bending over.

If the spinal curvature progresses despite the use of conservative measures, it may be necessary to proceed with surgical intervention. The surgery most often performed for scoliosis is called a "posterior spinal fusion with instrumentation. The goal of this surgery is to create a solid "fusion" of the curved part of the spine. The fusion is created by operating on the bones of the spine (vertebrae), adding bone graft, and allowing the vertebral bones and bone chips to slowly heal together to form a solid mass of bone. The bone graft may come from your hip (iliac crest) or from the hospital's bone bank. Often, the spine is also partially straightened with internal metal rods and wires (spinal instrumentation).  The rods and wires hold the spine in place until your fusion has had a chance to heal. This spinal instrumentation is left in your back without causing any problems.

Before Surgery

Donating Blood: All patients will lose some blood during surgery, and sometimes it is necessary to get transfusions during or after the operation. There are several different options for donating blood.

Autologous Donations: You may choose to have your child donate his or her own blood for transfusions after surgery. The first unit of blood must be given within five weeks of the surgery, and the last, not less than seven days before the surgery. It is important to eat a nourishing meal 2-4 hours prior to donating blood. If patients are donating their own blood before surgery, they will often be given a prescription for iron pills. Iron may cause constipation, so it is a good idea to increase the fluids, fruits, and vegetables in your diet. Since the body replaces blood very quickly, healthy patients can donate and still be ready for surgery soon after.

If your child does not meet the minimum weight requirement (less than 90 lbs) or you do not wish to donate your own blood, family or friends may donate their blood. If their blood type matches yours and meets special standards, it can be used for donation. Properly matched blood is also available from the hospital’s blood bank.

Aspirin, bufferin, and non-steriodal anti-inflammatory medications such as Advil, Motrin, Naprosyn, or Aleve may cause extra bleeding at the time of surgery. These medications should be stopped three weeks prior to surgery. Tylenol (Acetaminophen) can safely be used for pain relief prior to surgery. If you are unsure whether you should stop medication, consult with your physician or nurse practitioner.

Pre-Admission Testing: One to two weeks before surgery, you will need to come to the hospital for pre-admission testing. Plan on being at the hospital for four to five hours on this day. You will meet with a physician or nurse practitioner who will perform a physical examination, and make sure your child is in good shape for surgery. The nurses and doctors will ask questions and answer any questions that you may have. If your child becomes sick during the week prior to surgery, notify the orthopaedic office. Urine and blood samples will be obtained. In addition, you will meet the anesthesiologist (the doctor who will be putting your child to sleep). He/She will explain how you will be put to sleep for the operation, as well as how pain will be controlled post-operatively. Be sure and tell the nurses or doctors about any allergies to medication, foods, tape, or latex (rubber products).

After your visit at the Pre-Admission Clinic, you will most likely visit with your spine surgeon who will answer any questions that you or your family may have relating to the surgery. The surgeon or nurse practitioner will complete a history and physical, obtain surgical consent, and obtain additional x-rays if necessary.

The Night Before Surgery: The night before surgery, your child must not eat or drink anything after midnight. This includes water, chewing gum, and candy. The stomach needs to be empty prior to going to sleep. This will help keep the stomach from getting upset afterward. It will also help avoid aspiration which is a potentially life threatening problem.

Eat a well-balanced dinner the night prior to your surgery. You may also want to avoid salty foods the night before surgery to prevent waking up excessively thirsty.

The Day of Surgery

When you get to the hospital on the morning of the surgery, you will most likely check in at the pre-surgical area. The nurses will record vital signs (temperature, pulse, and blood pressure), and you child will change into a hospital gown. An IV line (intra-venous) will often be started while in the pre-surgical area. The IV is started using a needle that is removed once the line is in place. This leaves a small plastic tube inside the vein through which medicine and IV fluids are administered. There are topical creams that can be applied to the site of the IV to make insertion more comfortable.

In the pre-surgical area, you will meet with the anesthesiologist, and your surgeon, nurse practitioner, or resident. The anesthesiologist will review the pain medication you will be receiving after surgery. You should also meet the surgical liaison nurse. During surgery, the liaison nurse checks with the operating room intermittently for progress reports and then shares this information with you. When your child is ready, they will be taken to the operating room.

The Operating Room: The room can sometimes be cool and noisy. Soon after arriving, the anesthesiologist will administer medicine though the IV line to help your child relax and fall asleep. Your child may be asked to breath through a mask, which is also used to help him or her to fall asleep. After your child is asleep, the anesthesiologist will put a small tube in the back of the mouth and throat and into the airway. This tube will deliver oxygen to the lungs, and will usually be removed before your child wakes up after surgery. Sometimes it can cause a sore throat after surgery, but usually there are no after effects. Because the stomach is empty and asleep from the anesthesia, a  nasogastric tube (NG tube) will be inserted to prevent you nausea. The NG tube will usually stay in until the third postoperative day. The nurse will place a tube, called a Foley catheter, in the bladder. The Foley drains urine from the bladder so that it can be measured, and keeps track of how well the body is eliminating fluids. The catheter is also usually removed on postoperative day three. After the Foley is out, your child will be able to use the bathroom normally.  Remember, all of these tubes are placed after your child is asleep.

Posterior Fusion: If your child is  having a posterior fusion, the incision will run straight down the middle of the back. The incision length will depend on how much of the spine needs to be fused. As explained earlier, one or two metal rods are places along the spine with hooks, wires, or screws. Frequently, there will also be a bone graft taken from the iliac crest (hip) and placed along the spine. The graft grows together with the spine and becomes solid, preventing further curvature.

Anterior Fusion: If your child is having an anterior fusion, there will be one of two possible incisions. If the scoliosis involves only the thoracic spine, the incision will be on one side of the rib cage from back to front. When the operation is on both the thoracic and lumbar spine, the incision will be across the lower rib cage and down the front of the abdomen. Again, the spine surgeon may use rods, screws, or staples to hold the correct position.

Occasionally, it is possible for the anterior spinal fusion to be completed through thoroscopic surgery. Thoroscopic surgery of the spine utilizes microscopic cameras which magnify images for the surgeon. There are three to four tiny incisions where the camera is inserted and where the surgeon makes the repair. Thoracoscopic s urgery usually is usually less painful with a shorter recovery period. In addition, the scars are much smaller as well as more cosmetically appealing.

There will usually be a "chest tube" placed after anterior spinal surgery. This tube drains the fluid that collects outside the lungs and helps keep the lungs expanding properly. The chest tube is covered with a large dressing, and is attached to a plastic container that collects the drainage. The tube is usually left in place until postoperative day three, and will then be removed by the surgeon.

Anterior/Posterior Fusions: Sometimes, it is necessary for the surgeon to complete a two-stage operation for scoliosis. This will involve both anterior and posterior surgeries. Usually, they will be done on separate days spaced about a week apart. However, in some cases, it is possible to complete both on the same day.

After the Surgery

When your child wakes up from anesthesia, he or she will be lying on their back in the recovery room or post-anesthesia care unit. Your child may not remember being in the post-anesthesia area because they will still feel sleepy and groggy from the surgery. Vital signs will be checked  frequently, and staff will also make sure that your child is comfortable. If your child is having any pain, the nurse will administer pain medicine through the IV. Your child will be receiving oxygen through the nose or mouth, and will be encouraged to cough and deep breath to help clear the lungs. There will still be the IV, nasogastric tube, and Foley catheter. A large dressing will be placed over the incision. Occasionally, there will be an X-ray to check the rods and hooks. Usually, patients will stay in the recovery room for 2-3 hours while they wake up from the surgery. It is possible for you to be with your child after you have met with the spine surgeon.

Your child will most likely spend at least one night in the intensive care unit after the spinal fusion. In the ICU, your child will still feel quite sleepy from the anesthesia and pain medications. The doctors and nurses will be touching the hands and feet, and asking if there is any numbness or weakness in the arms or legs. Blood will be checked periodically. Your child will be monitored very closely while in the ICU.  

The next step is transfer to either an adolescent area or general area of the hospital. You should be able to spend the night with your child for the entire stay. There will be specialists visiting with you to help you cope with the surgery, hospitalization, as well as separation from home, routine, and friends. Television, video games, movies, and other activities are usually available in most Children’s Hospitals.

Once your child begin to drink and eat small amounts of food, they may be able to take pain medications by mouth. They will be given a stool softener or laxative to prevent constipation which is a common side effect of pain medications.

Activity: It is common to feel quite tired following spinal surgery, and your child may need help turning from side to side initially. You will meet with a physical therapist usually on the second post-operative day. The physical therapist will assist with deep breathing, coughing, mobility, and endurance. After your surgeon says it is okay, your child may get out of bed and sit in a chair  You will then progress to walking short distances on the floor. Some patients get up the day after surgery, and others have to wait a few days. Your individual pace of recovery is unique, and depends on the type and extent of spinal surgery. You will be given additional information about specific activity restrictions at your six-week follow up visit. As a general rule, however, no contact sports are allowed for six months after a spinal fusion surgery.

Eating: Your IV will remain in place so that your child’s body receives enough fluids. The IV will be removed when IV pain medications or transfusions are no longer needed, and your child is drinking enough fluids. After the digestive system is working again and the NG [nasogastric] tube is removed, your child will be allowed to have clear fluids. Examples include water, chicken broth, and jello.

It is important to advance the diet VERY slowly to prevent nausea and vomiting! It may actually take two or three weeks for appetite to return to normal.

Showering: The large dressing on the back will usually be changed to a smaller bandage on post-operative day two. Usually, the stitches used to close your incision are under your skin. They are absorbable sutures, so they will not have to be removed. It is important to keep the incision clean and dry after surgery to avoid infection. Your child will need to take sponge baths for the first 7-10 days after surgery. After this, they can shower as long as the incision is well healed and has no drainage.

GOING HOME !!!!

Most patients will remain in the hospital for about 5-7 days after surgery. When pain is well controlled, your child is eating and drinking without nausea, and is able to walk around on the floor, you will start to think about going home. Usually your surgeon will tell you when you can expect to go home about a day in advance. During your hospital stay, you will be taught how to take care of your child. You will be instructed on what problems to look for, as well as how to manage diet and medications prior to discharge. Generally, no special equipment is needed at home following a spinal fusion.

You will be given prescriptions for pain medication before you go home. Plan to give a dose of pain medication prior to leaving the hospital, as the ride home can sometimes be uncomfortable. If your child still has pain after taking the medication, call the orthopaedic office. They may adjust the main medication.

Watch for the following problems, and immediately report any that occur to the orthopaedic office:

Your child will not be able to return to school for the first few weeks after surgery. You should arrange for a home tutor through your school system if possible, as most patients typically miss between four-six weeks of school. Before you leave the hospital, you will schedule a follow-up visit with your spine surgeon, usually held six weeks after surgery.

My name is Phebe Kanaratnam-Roberts and my son Shane who is now 12yo has CLS. He was diagnosed 5 years ago. Shane is one of the very few Asians known to be challenged with CLS. We are originally from Malaysia. We have 2 other sons, Kyle - 10yo and Ethan - 8mo who are doing just wonderful. Reading the messages [in the online support forum]brought back memories of how desperate I felt when Shane was diagnosed and was very thankful for the existence of this foundation where I found much support, help and comfort. I even went to Australia with Shane and Kyle 5 years ago to meet with two families that I met and became friends with on the foundation.  We immediately felt the warmth of the bond we shared with each other.   It was amazing and touching simultaneously - can't truly describe in words. I am glad to be here to support and receive support.
 

Love to all,
Phebe

Columbia, MO
Parent mentor - Missouri Developmental Disabilities Resource Center, University of Missouri - Kansas City

Respite Care for Adults

If your child is 18 or older and you have legal guardianship, you may qualify for respite care through a program sponsored by Medicaid. This program pays for one or more caregivers who can assist with all aspects of personal hygiene, meals, everything except personal finances. They can even wash the windows twice a year in the parts of the house used by your child. :-)

Eva Shamp is using this service for her son, Van:

“It really is nice for Van - he has two different attendants because one of them drives him home from work and helps him clean his mess up in his room. The other one helps him with his bath and grooming. 

They are both special to him, but one of them especially is like family to us. She's been helping him for about 4 years now and she's like another grandmother, not only for Van, but I think she cares about all my kids as if she's family!  They come home from school before she gets here and she always talks to them and asks them questions about what's going on just as if she's family. 

Van feels extra special to have so many people who care for him.  It's a great thing for me because sometimes the physical care for an adult child wears me out!  But I don't ever want Van to be anywhere but here.  I could not stand for him to live anywhere else, because I have to know he's got the best of care and all the love in the world."

Eva Shamp

Merdian, MS

The first step is applying for SSI through the Social Security Administration. The programs vary by state.  In Washington State, the program is called Medicaid Personal Care Services and is administered  through the Department of Developmental Disabilities. After an initial assessment to determine how many services you qualify for, you can get as many as 184 hours a month of help from any one provider, and you can even get more than one provider. The “provider” can be through an agency, or can be a family member (over 18) or they can even pay you to take care of your own child.

Contact your state case worker or the Social Security Administration in your state for more information.

P.S. I just got  hooked up with this program in Washington State. Davis's caregiver starts on April 10th! - MCH

Introducing...

Kyle Bauer,  Age 11

My son’s name is Kyle and he was born on 3/4/95. His health history is kind of long.  He was born at 28 weeks at 2 lbs 8 oz but dropped in weight to 2 lbs, 2 oz. There were many complications. Bronchopulmonary dysplasia (BPD)/Chronic Lung Disease, hernias, tracheomalacia, severe respiratory distress syndrome (RDS), heart murmur, feeding problems, and more.

I'm basically in this alone. I have the support of family, but I'm divorced. I have been for a number of years. I have sole custody of my son.  True he has a lot of problems but he is a very happy little boy and very loved. He deserves all the chances I can give him to stay that way. He's also a very strong little guy. I came close to losing him more than once and his scoliosis was so bad it was threatening his pulmonary system. I love him more than I can say and want to help him in any way that I can.

Loren Bauer 

Oak Forest, IL

Mark and Matthew Snyder, Age 37

Please welcome Chester R. Snyder to CLSF. Chester is the father of probably the only set of identical twins with CLS in the world. Mark and Matthew are now 37 years old and doing well. Chester is the primary caregiver for his sons and his wife. 

Chester does not have internet access, but would like to be in contact with other CLS families.

Chester R. Snyder

Hamburg, PA

Thanks for Caring!

The following families or individuals have recently made generous donations to the Coffin-Lowry Syndrome Foundation:

Marcia Scowcroft

In memory of Milton Scowcroft

Karl and Olga Rassau

In memory of Andrew Anderson

Send your donations to:  

Coffin-Lowry Syndrome Foundation

c/o Mary Hoffman

3045 255th Ave SE

Sammamish, WA 98075 

(U.S. Funds, please)

Why are medical IDs critical? In an emergency, when your child might not be able to speak for himself, a medical ID bracelet or necklace speaks for him.  Symptoms of common ailments can easily be misdiagnosed. Prompt diagnosis is critical to effective treatment. A brief description of vital medical facts engraved on your medical ID ensures appropriate and timely medical care.