Bones & Muscles

My friend’s 9-year-old daughter was just diagnosed with Pompe disease. I’d like to reach out to her and offer my support, but I’m not sure how. Can you tell me a little about this disease and what to expect?
– Ali

Pompe disease is a genetic condition that can be very debilitating, affecting many systems in the body. It occurs when there is too much of a certain type of sugar, called glycogen, in the body’s cells. This buildup of glycogen stops the cells — especially those in the body’s muscles, including heart muscle — from functioning normally.

Children with Pompe disease may have heart problems, muscle weakness (which can make walking very difficult), and breathing problems. The younger a child is at diagnosis, the more severe these symptoms can be. For example, infants who are diagnosed with Pompe disease develop life-threatening problems from an enlarged heart and liver, and can have trouble breathing, eating, and gaining weight.

In the past, many infants with Pompe disease died before their first birthday, but today those odds are improving. In 2006, a treatment approved by the U.S. Food and Drug Administration (FDA) called Myozyme® was found to extend the lives of babies with infant-onset Pompe disease.

Fortunately for older kids like your friend’s daughter, the odds of survival are even greater. With close monitoring and treatment from a cardiologist, neurologist, and respiratory therapist, many children with the disease live well into adulthood.

Learning that a child has been diagnosed with this complex condition is a devastating blow to any family. Your friend is probably dealing with some difficult emotions right now. Even though she may not ask for help, she needs it. Start by offering to help with practical things, like running errands, getting dinner on the table, or babysitting any other kids while she’s at a doctor’s appointment with her daughter.

Helping out with everyday tasks can make managing this new reality a little easier for your friend. And of course, lend a sympathetic ear when she needs it most.

Much like a fiberglass cast holds a broken arm or leg in place, a “halo” with vest holds a child’s head and neck in place after a spinal injury so that spinal bones, or vertebrae, can heal.

This brace lets children who have been injured or are recovering from spinal surgery walk, move around, and participate in many regular activities instead of being confined to bed rest while they recover.

What’s a Halo?

The halo gets its name from the metal ring that surrounds the head, which looks like an angel halo. The ring — which keeps the head fixed in a level, forward-facing position — is attached to the head with small pins that hold it in place. To keep the halo from moving in any direction, vertical rods connect it to a person’s shoulders, where it’s fastened to a vest. The vest (usually made of plastic or plaster, like a cast), wraps around the neck, over the shoulders, and down to the belly button. It’s lined with soft material to make it more comfortable and prevent skin irritations.

When properly fitted, a halo brace keeps a child’s head and neck completely still, even during movement.

Is a Halo Painful?

While the brace may look painful, it should not cause any pain to the head, neck, or shoulders. Some kids do experience a little forehead pain or a headache, particularly while eating, but this usually goes away soon after a child has gotten used to wearing the brace.

If the pain continues or gets more severe, the pins that secure the halo might need to be adjusted. Never attempt to adjust the pins or rods on your own, or to remove the vest. If your child is experiencing pain or discomfort, call the doctor right away. Also seek medical care if the area around the pins (or anywhere else) becomes red, swollen, or irritated.

How Long Is a Brace Worn?

Depending on the severity of a child’s injury and the duration of recovery, the brace may be worn for a couple of months. Your doctor can tell you how long your child needs to wear a halo brace.

Are Some Activities Off Limits?

Many kids with halos can continue their everyday activities, like going to school, doing schoolwork, and participating in extracurricular clubs, as long as they’re feeling up to it and the doctor says it’s OK.

Your child’s doctor will tell you what activities your child should avoid while in a halo brace. Sports will have to be put on hold for a while, and running — which can increase the chance of a fall — will have to be avoided, as well. Other activities to limit:

• any sort of quick or jerky movements, like jumping or dancing
• pulling or tugging on the halo or attached bars
• bumping the halo into other objects (when avoidable)
• getting the vest or vest liner wet

Since kids with a halo cannot look down while walking, it’s important for them to take precautions while walking and moving about. Clear the floor of anything that might cause your child to trip or fall, and offer assistive devices, if needed. For example, a cane or walker might give your child the confidence to get around on his or her own, rather than relying on you, a family member, or friend to help with walking.

How Will My Child Sleep at Night?

Let your child sleep in whatever position feels most comfortable. Usually, this is the position used prior to getting the halo brace. Kids with halo braces can sleep on their backs, stomachs, or sides. Some prefer to sleep on a slight incline, with pillows supporting the brace from underneath. You also can use pillows to support the back, stomach, or side as your child finds a comfortable sleeping position.

Can My Child Take a Shower?

No. Showers are not permitted because water can damage the halo or vest. But kids can take a bath as long as there’s a shallow amount of water in the tub and it does not wet the vest. However, since it can be difficult to get kids in and out of the tub safely, most parents opt to manually bathe their child with a damp towel.

During bathing, have your child sit in a chair while you cleanse the arms, legs, hands, head/neck, and feet using a mild soap and damp towel. Avoid using sponges that trap water and can easily leak onto the halo brace. Protect the edges of the vest with a dry towel or plastic bag (which can also be worn over the vest; cut a slit for your child’s head and arms).

To shampoo hair, lay your child’s head over the edge of the tub or sink. Small kids can lie on the kitchen counter with their head over the sink. Wash and dry as usual, protecting the vest and liner from any moisture. If you prefer, a dry or powder shampoo (that requires no water at all) also can be used to cleanse and condition the hair.

If, at any time, the skin beneath the vest becomes wet, dry it with a hairdryer set on “cool.”

Caring for a Halo

Besides keeping the halo vest free of any moisture, it’s also important to keep the site of the pins clean to avoid infection. Your doctor will give you specific instructions for cleaning the pin areas, including what type of cleaning solution to use. Run a cotton swab along the pin and surrounding area, and be sure to use a new swab for each pin to avoid spreading any germs. If you notice that any pins have come loose, call the doctor right away.

Caring for the Vest

Some vests come with removable liners that are washable. Buying more than one liner means you can wash one while your child wears the other. If you only have one liner or if it is not washable, use a cornstarch-based baby powder around the edges of the vest to keep your child and the liner cool, dry, and comfortable.

To clean a non-washable vest liner, try using a long piece of surgical gauze dipped in witch hazel. Wring out the gauze so that it is barely damp. Then, feed the gauze under the edge of the vest and liner and, with one end in each hand, slide back and forth. This can also be done in case your child’s skin becomes itchy.

The liner should always be worn against the skin, with the vest immediately over it. Regular clothes can be worn over the vest, although they may need to be adapted to fit over the halo rods.

You will be instructed on how to switch the liner.

Helping Your Child Cope

Managing any sort of physical challenge, however temporary, is difficult for anyone — especially a child or teen who is dealing with the pressures of growing up and fitting in with peers. Kids who have to wear a halo brace may feel angry, frustrated, and even depressed, especially if they’re no longer participating in activities with their friends.

In the meantime, find ways to maintain normalcy in your child’s life. Many kids can continue going to school with a halo and, if not, they can do schoolwork at home or with a tutor. If your child does go back to school, a nurse or social worker from the doctor’s office may be able to visit your child’s classroom to talk about the halo. This might make your child feel more comfortable about wearing the brace in school.

If you’re worried about your child going out with friends, encourage them to come over to your house so that you can supervise.

In time, your child can return to his or her favorite activities. But if your child continues to feel angry or depressed during recovery, consider talking to a school psychologist or counselor, who may be able to help your child cope and look ahead to better days.

A cast is a big, hard bandage that’s usually made of material like fiberglass or plaster. Casts keep bones in place while they heal.

Kids get casts for many reasons. Usually they’re for broken bones, but kids also might get casts to help bones and surrounding tissues grow properly, or to aid healing after a bone surgery.

A cast wraps around the broken area and needs to be removed by a doctor when the bone is healed. Depending on the age of the child and type of break or healing needed, a cast can be on for as little as 4 weeks or as long as 10 weeks.

Before Getting a Cast

Sometimes splints are worn for a few days (usually between 3 to 7) before a cast is made. This allows for any swelling to ease so that the cast can provide the best fit.

Splints are usually held in place by fabric fasteners, velcro, or tape. A doctor or orthopedic technician will teach you the best way for your child to wear the splint and how to ensure a good fit.

Types of Casts

Casts usually are made of:

• Plaster of Paris: This heavy white powder forms a thick paste that hardens quickly when mixed with water. Plaster of Paris casts are heavier than fiberglass casts and don’t hold up as well in water. They’re typically used when the strongest hold is needed.
• Synthetic (fiberglass) material: These casts come in many bright colors and are lighter and cooler. The fiberglass (a kind of moldable plastic) covering is water-resistant, but the padding underneath is not. You can, however, sometimes get a waterproof liner. The doctor putting on the cast will decide whether your child should get a fiberglass cast with a waterproof lining.
• Elastic or other soft material: These casts are similar to fiberglass casts but allow for more movement of the limbs inside the cast. Also, they can be removed without using a cast saw, so they may be a good choice for a very young child. They’re not used to protect fractures, but can be used when limbs are healing after a surgery.

There are many types of casts. The most common casts are:

• Short arm casts: placed from the knuckles of the hand to just below the elbow. These types of casts are used for forearm and wrist breaks and after some surgeries.
• Long arm casts: these go from the upper arm to the knuckles of the hand. They’re generally used for upper-arm or elbow fractures, but can be used in forearm breaks.
• Short leg casts: these run from just below the knee to the bottom of the foot. They’re usually used for ankle and lower-leg breaks or surgeries.
• Long leg casts: applied from the upper thigh to down over the foot. They’re used to heal breaks or fractures of the knee, lower leg, or ankle.
• Short leg hip spica casts (or body casts): these go from the chest to the knees and are used to keep hip muscles and tendons in place after surgery.

Getting a Cast

Applying a cast requires a few simple steps. First, doctors wrap several layers of soft cotton around the injured area. Next, the plaster or fiberglass outer layer is soaked in water. The doctor wraps the plaster or fiberglass around the soft first layer. The outer layer is wet but will dry to a hard, protective covering. Doctors sometimes make tiny cuts in the sides of a cast to allow room for swelling.

Once the cast is on, the doctor will probably recommend that your child prop the cast on a pillow or stool for a few days to reduce swelling and pain in that area. A child who has a walking cast (a foot or leg cast with a special device implanted in the heel to allow for walking) shouldn’t walk on it until it’s dry (this takes about 1 hour for a fiberglass cast and 2 or 3 days for a plaster cast).

If the cast or splint is on an arm, the doctor might give your child a sling to help support it. A sling is made of cloth and has a strap that loops around the back of the neck. It acts like a special sleeve to keep the arm comfortable and in place. A child with a broken leg who is mature enough and tall enough will probably get crutches to make it a little easier to get around.

The area around any break will probably be a little sore and swollen for a few days, so the doctor may recommend acetaminophen or ibuprofen to help ease any pain.

Cast Care

For bones to heal properly, certain steps must be taken to make sure the cast can do its job. These tips can help keep a cast in good shape:

• Keep non-waterproof casts dry. Many casts are not waterproof, so keeping them dry for the whole time the cast is in place is very important. It is best for your child to take baths, not showers. The doctor will tell you to cover the cast with a plastic bag or special cast protector for baths. The cast area should be propped up on something like a milk crate during the bath to keep it completely out of the water. Children under 5 years of age should be sponge bathed.
• Keep out foreign objects or substances. At some point, the skin inside the cast will probably become itchy. Your child should not stick anything into the cast to relieve itching, as this could scrape the skin and lead to infection. You also should not pour baby powder, creams, or oils into the cast.

  To relieve itching, tapping on the outside of the cast or using a cool blow dryer to blow air in around the edges of the cast may help.

  Watch young children closely because they may place small toys or food inside their cast.

• Check for cracks. Check the cast regularly for cracks, breaks, tears, or soft spots. If you notice any of these things, contact your doctor.
• Don’t alter the cast. Decorating the cast and having friends and family sign it is OK, but things like pulling out the cotton lining or breaking off parts are not.
• Fix sharp edges. If a sharp edge develops on the cast and is irritating the skin, put tape or moleskin on the edge to protect the skin from the rough surface.

When to Call the Doctor

Casts rarely cause any problems if they are applied and cared for properly. Sometimes, sores can develop if the splint or cast is loose fitting and rubs the skin. These sores can get infected. Tight-fitting splints or casts can make fingers or toes turn bluish. Fixing the cast or splint can relieve tightness.

Call your doctor if you notice any of the following:

• pain that doesn’t get better with pain medicine and/or raising the casted part
• tightness that leads to the hand or foot feeling numb or tingly
• fingers or toes turning white, purple, or blue
• trouble moving toes or fingers
• a blister developing inside the cast
• any unusual odor or drainage coming from inside the cast
• a break in the cast or the cast becoming loose
• a wet cast
• skin around the edges of the cast that gets red or raw
• fever

Cast Removal

Once the bone is healed, the cast will be removed with a small electrical saw. The saw’s blade isn’t sharp — it has a dull, rounded edge that vibrates from side to side. This vibration is strong enough to break apart the fiberglass or plaster but won’t hurt skin. Don’t attempt to remove the cast on your own.

Once the cast is off, the injured area will probably look and feel different to your child. The skin will be pale, dry, or flaky; the hair will look darker; and the muscles in the area will look smaller or thinner. This is all temporary. The doctor or a physical therapist can recommend special exercises to help the bone and surrounding muscles get back in working order.