Osteogenesis Imperfecta Treatment
At COA, we are focused on maximizing function and minimizing pain that can be associated with OI. Both of these goals are best accomplished by decreasing the number of fractures that a child with OI endures.
Strategies used to improve bone health include (but are not limited to):
- Encouragement of weight-bearing activities
- Use of braces, when necessary
- Exercise and/or physical therapy
- Taking adequate levels of daily calcium and vitamin D
- Possibly the use of medications to make bone stronger (typically bisphosphonates)
- Possibly the use of surgery to implant rods both to straighten bones and protect them from future fractures.
The COA Approach to Your Child With OI
If your child has recently been diagnosed with OI, the diagnosis can be an overwhelming one. The COA team is here to support your child and your family every step of the way, from resources on how to prepare your child to same day appointments.
Especially for the more severe types of OI, fractures are inevitable. They will happen. We obviously want to minimize these fractures as much as possible. Do not consider yourself a bad parent if your child has a fracture. At the same time, we would also not encourage your child to do risky activities to promote fractures. There needs to be a balance, and with time, you will learn to find it.
In general, there are two phases of care for OI – the “maintenance phase” in which the COA team is screening your child’s bones to evaluate for their overall quality, and then the “acute fracture phase” in which your child has suffered from a broken bone.
Since your child’s bone is weaker, all efforts need to be made to strengthen the bones as much as possible.
For children with the more moderate to severe OI, we will typically start physical therapy around the age of 3 – 6 months. Therapy can either be “land therapy” or “aqua therapy” depending on your preference and your doctors’ preference as well. Keep in mind that pools are limited in the greater Atlanta area, but are present. Kids with OI obviously have high priority for these pools. Other more creative options could be dance/ballet and/or martial arts. PT is imperative for a young child with OI. They need to learn balance skills to prevent them from falling and gain muscle strength and endurance. For an older child, he or she may have anxieties about walking and running. Working with an experienced PT may be necessary to get your kid moving and keep them moving. When you get a prescription for PT from your provider, you can discuss your options.
Your COA provider may recommend braces for your child’s ankles to provide additional support with walking and running. Many children with OI have very flexible joints and very flat feet. Thus, these braces are used to support these joints and feet. The most common brace prescribed is something called an SMO (supra-malleolar orthosis). It is a brace that can fit into most sneakers, and it comes up to just above the balls in the ankle joint (known as malleoli). If your child’s ankles are very wobbly, an SMO may be very useful. Often times, if your child benefits from an SMO when he or she is younger, they will likely just transition to a shoe insert as they get older. Braces are expensive as they are custom made.
Prior to the recommendation of braces, a thorough discussion should be made with your COA doctor.
Acute Fracture Phase
If your child has suffered from a broken bone, we will take the highest precaution in coming up with the right solution and treatment plan. Below are a couple of the options that surface when addressing fractures in children with OI.
Medical Treatment (Bisphosphonates)
Bisphosphonates are a class of medications that can be used to strengthen bones. Your bone is constantly breaking down old bone and making new bone (a process known as turnover). These bisphosphonates inhibit bone turnover which improve bone density, can benefit children with more severe forms of OI. This type of medication has revolutionized care for children with OI. It has allowed previously wheelchair-dependent kids to be able to walk. It has allowed children who previously were in chronic pain to live a life with less pain and less fractures. Biophosphonates even come in either oral or IV forms.
Biophosphonates are considered if your child has:
- Obvious crookedness to the bone
- Multiple long bone fractures (femurs, tibias, humerus, etc.) in one year
- Any pathologic back fracture
These medications are certainly not without risk, and therefore, must be discussed in detail with your physician before starting such treatment. Typically, endocrinologists are the doctors that prescribe these medications. If the decision is made by you and your COA provider to consider this type of medication, you will be referred to an endocrinologist that is specially trained in treating bone.
Surgical Treatment (Rodding surgeries)
The decision to consider surgery for your child is not taken lightly. For children with the more severe types of OI where the bones are too crooked to stand on, your COA provider will typically recommend placing rods in all 4 portions of the lower limbs. Surgery is usually performed just when your child is pulling up to stand (which averages 18-24 months in the more moderate to severe forms). The types of rods most commonly used are called Fassier-Duval rods.
The surgery is designed not only to straighten the bones, but the rods also protect the bones from bending and breaking in the future. The rods are not full proof, and therefore, should not be considered for every child with OI. Above is an example of a “before and after” rodding surgery performed at COA. This child is now able to walk and rarely suffers fractures that slow him down.
Contact COA Today
If you have OI in your family, or your child has just been diagnosed with OI, don't hesitate to request an appointment today. Come experience the COA difference.