Πληροφορίες για την Ατελή Οστεογένεση > Πληροφορίες για την Ατελή Οστεογένεση από το OIF > Φυσιοθεραπεία > GMD
Matthew Hunt, PT
Stephanie Gould, PT
© Shriners Hospital for Children, Canada
The gross motor development of children proceeds in a sequence of different steps known as motor milestones. Though not every child achieves these steps in exactly the same order, the pattern is generally consistent. Variations in the sequencing of these milestones for children with OI is often attributable to fractures and immobilization. As well, developmental stages are often achieved more slowly but can be facilitated by carefully chosen activities and stimulation.
This booklet is designed to present different activities to promote the motor development of children with OI. This booklet should be used under the direction of a Physical Therapist. Not all of the exercises and activities will be pertinent or appropriate for every child. As well, the order in which activities are selected will be critical and an individualized program should be created to reflect the needs of each specific child.
Before addressing specific activities and exercises a brief overview of OI is pertinent.
Osteogenesis Imperfecta is a genetic disorder characterized by bone fragility and low bone mass. OI is grouped into several different types based both on clinical and microscopic variances. Type I, the mildest form of OI, is known for mild bone fragility, minimal secondary deformities and normal, or near normal, stature. Persons with type III OI have multiple fractures, progressive limb and spine deformities and short stature. The Type IV phenotype may present with some fractures, mild to moderate deformities and somewhat short stature (but less severe than for Type III). Type V was recently described and demonstrates hypertrophic bone calluses, ossification of the membranes between bones of the forearm and leg, and certain microscopic aspects. It is important to remember that no two children with OI are exactly alike: the picture of OI varies greatly, not only between different types, but also within each grouping.
Most, but not all, forms of OI are associated with mutations in genes encoding type I collagen. As a result the structure of the bone is abnormal and bone mass is decreased leading to bone fragility and recurrent fractures. As noted above, some (but not all) children develop secondary skeletal deformities secondary to OI – these can include bowing of the long bones, flattening of the skull, chest deformities or scoliosis (lateral curvature of the spine). Hypotonia (decreased muscle tone), muscle weakness and laxity (looseness) of the ligaments are also often present. These factors, coupled with repeated immobilizations following fractures, can result in delays of grass motor development in children with OI.
Global motor development can also be slowed if the child is overprotected by family and caregivers and not given adequate opportunities to move in and explore their environment. The beneficial effect of muscle activity on bone growth is also diminished when motor activity is limited. As such, it is very important to find a balance between providing adequate safety and allowing the child to develop gross motor abilities through play and movement.
Frequently young babies with OI spend most of their time lying on their backs. It is important to begin varying the child’s positioning as soon as appropriate to minimize flattening of the back of the skull and shortening of the hip muscles. Contractures and skull reshaping tend to develop in a child who is always on his or her back. The side lying and tummy lying positions will help greatly in this regard.
Initially, the use of pillows, towels or rolls may assist in maintaining this position. Commercially available wedges with Velcro are often very useful. It is important to promote symmetry by placing the child on alternate sides.
Lying in this position is initiated once partial head control is achieved. The suggested steps to follow to acquire head control are discussed in subsequent sections. Once possible for the child, this is an invaluable position for motor skill acquisition.
For a child with a pointed chest lying directly on their tummy will be not comfortable. Try propping them slightly to one side with a folded towel along one side of the body. Frequently what is best tolerated is to lie on your back with your child in prone on top of you.
Generally, prone lying is not begun until the child is at least 3 months old. Babies are encouraged to be on their tummies during waking hours only. Follow your doctor’s instructions regarding sleep positions on back or in side lying.
When your child is lying on his or her back use the following technique to promote good alignment:
Purpose:
a) to facilitate the use of the arms and legs, and encourage bringing hands together
b) to avoid contractures (shortening) of the hip muscles which keep the legs in a widespread and outwardly rotated position.
Technique: Position your child on his or her back with rolled receiving blankets on either side of the body and slightly propped under the shoulders and hips. In so doing you will bring the shoulders forward and keep the legs in a neutral position (i.e. thighs in parallel with each other and the knees pointing upwards).
Progression: Decrease the thickness of the rolls as you see that your baby is able to move more freely.
Early Intervention Some babies with OI tend to develop motor skills more slowly than other children their age. Several activities can be done to assist in motor skill development even when the child is quite young or has restricted mobility.
Purpose: Encourage your child to reach for his or her legs and/or feet. This will help develop the stomach muscles and improve control of the leg muscles.
Technique: Sit on the floor or on a bed and lay your child on his or her back in front of you. Place your hands under the child’s bottom to stimulate the child to lift his or her legs. Encourage the child to lift and look at his or her legs by playing with the child’s feet, touching them with toys or playing Peek-a-boo. Socks with small bells on them can be a fun stimulatory tool.
N.B. this activity should be discontinued if it appears to cause discomfort.
Purpose: To take advantages of the buoyancy of water in order to have your baby begin moving his or her arms and legs more easily. This is also the preferred environment to recover movement following any period of immobilization.
Technique: Place the child on his or her back in a comfortably warm bath, in water deep enough to cover the lower body (just enough to reach the ears when lying on the back). This is a great environment in which to play and to stimulate independent movement. Try to encourage movement of the arms and legs through play.
One of the most basic components of a baby’s gross motor development is the establishment of control of the head and trunk against gravity. Children require this core stability in order to initiate other voluntary movements and to move within their environment.
Technique: Sit with your feet well supported, holding your child in a chest to chest position. Begin by learning back slightly (make sure you have pillows to support your own back). You can lightly stroke the muscles at the back of the baby’s neck to stimulate them, all the while encouraging your baby to lift his or her head.
Talk with your child, sing, or make faces to encourage him or her to look up. Some form of stimulation (mirror, book, toys) placed behind your shoulder could be useful in enticing the child to lift his or her head. Gradually lower yourself to more reclined angles – progressing only so far as your child is still able to lift his or her head to an upright position.
Technique: Sit on the floor with your child facing towards you. Hold your child with your fingers supporting the back of the head and neck, and your thumbs on the upper chest. Place pillows behind your child for support. Begin in the vertical position and slowly lower your child so far as he or she is able to lift the head up against gravity. Talk to your child, sing, or make faces in order to maintain his or her interest.
Technique: Position your child lying on his or her stomach with a folded towel underneath his or her tummy. Gently stroke the muscles at the back of the neck with your fingers. Use some incentive to encourage lifting of the head such as noisy or brightly colored toys. Try to get down low so you can make eye contact with your child.
Technique: Hold your child facing away from you (with child’s back to your chest) and inclined to one side. Place one arm between the child’s legs to provide support at the level of the pelvis.
a) Initially you can place your second arm in front of the shoulder and across the child’s trunk to grasp your other wrist. In this way you will provide partial support to the head and full trunk support.
b) Once your child has lateral neck control you can change the position of your second hand. Instead of crossing in front of the shoulder, place your arm under the child’s lower arm and across his or her chest to grasp your other wrist. The slight tilt in the child’s position will trigger a reflex action of the neck muscles, helping to strengthen the muscles that control side flexion. Try this on both sides. It is important to promote symmetry!
Technique: Position your child on his or her elbows and knees lying across your thigh. Your leg will provide stability and support much of the child’s weight. You can also have your child lie over a half inflated beach ball.
Once your child is able to comfortably maintain this position encourage reaching up with one hand to play with a toy. N.B.: this position is not appropriate for a child who has repeated rib fractures.
Technique: Position your child kneeling on elbows and knees with a semi-firm cushion under his or her tummy. The height of the cushion should be the distance from the underarm to the elbow. Provide toys so that your child may play while maintaining this position.
N.B. This is not advised for an infant who has a pointed chest, who has frequent rib fractures in the past, or who has extremely fragile arms.
Typically the first displacements that children are able to do are accomplished by rolling. Most children first roll from their stomachs to their backs. However some children with OI will initiate rolling from their backs to their fronts first. Often children with OI may be fearful of rolling over top of their arm and may need assistance to position their arm either at their side or up above their head.
1) For a child who is starting to roll by pushing into extension try not to provide more assistance than necessary to assist movement. Rather than pulling on the arm it is preferable to aid the child achieve the transfer by simply guiding the movement with a gentle assistance at the level of pelvis. Let your child perform the movement as independently as possible.
2) You can use a toy to encourage your child to reach one arm across his or her body (while extending the opposite leg). The child may accidentally roll into sidelying – and once having experienced the movement may reproduce it on his or her own.
N.B.: Some children with OI snake on their backs instead of rolling as form of mobility. It is important that all active movements be encouraged.
To promote independent sitting the following activities can be used:
1) Lie on your back with your legs partially elevated on a couple of pillows (i.e., knees and hips somewhat bent). Place your child on his back against your thighs, facing you. This way he is partially inclined. As time goes on and your child grows to tolerate this incline, increase the height of pillows under your knees so the child is more and more upright. Eventually, he will be sitting straight up with your thighs behind his back.
2) Sit on the ground with your legs straight and widely spread. Sit your child in the triangle formed by your legs and with his or her back towards you. Play with your child in this position to increase tolerance of the seated position and improve stability.
3) Once your child is a little more stable in sitting, bur still needs some extra support, you can position him or her sitting in a small U shaped cushion. Such a cushion can be home made, or a breast-feeding, or commercially bought, pillow can be used. Alternatively you can sit your child in the corner of the couch.
4) To work on abdominal and neck flexor strength you may do a reverse progression of #1, while stimulating the child to reach foreward for a toy, to raise his back off your thighs and some foreward sitting. Doing this from increasingly inclined angles augments the difficulty. Never have the child do this from a completely flat lying position as it will place too large a stress on the bones of the spine. Instead, work until the child can accomplish the task from 45o (i.e. halfway to flat) or to whatever position your physiotherapist recommends.
5) To attain the sitting position independently from a lying position, children are encouraged to go through side-lying or semi-prone. Thus, teach your child to roll onto her side, (or, if that still is too difficult, partially toward her stomach) and use her arms if they are strong enough to push herself up onto her buttocks. Provide close spotting and even support until your child is able on her own.
Many children with OI adopt scooting rather than crawling as a means of moving about. Children who do not show the initiative to crawl on all fours should not be pushed to do so. Crawling puts considerable stress on the bones of the arms and your child may not be ready for this. As such, children with OI frequently scoot about in a seated position and some may never crawl. It should be remembered that all forms of movement are to be encouraged.
For a child whoa has developed full trunk control and sits independently the following can be attempted to encourage scooting:
With child in a seated position. Sit or kneel behind your child. Scoop one hand under each buttock so that the child is sitting on your hands.
a) Begin by making a gentle shifting movement from side to side so that the child can feel the weight shift. Weight shifting is needed to be able to scoot. You can play a game of rocking back and forth while singing a song.
b) Once the child is doing this lateral weight shift comfortably you can incorporate a small pivoting movement. Practice going forward and back repeatedly on one side to begin, then proceed to a reciprocal movement by bringing each hip forward to a slow, alternating pattern. The forward and back movement should not be exaggerated. Encourage the child to do as much of the movement on his or her own as possible.
For a child who has good trunk and pelvis stability, and for whom the medical team has given the go-ahead for weight-bearing activities, a potential pre-walking activity is high kneeling. This is an important position in getting from the floor to standing. Encourage your child to high kneel (with hips in a vertical position). The child may initially need to hold onto a chair or low table for support. You can provide support by holding him or her at the hips.
Place toys on a low table or sofa to encourage the child to pull up onto his or her knees.
Do not, however, allow the child to sit back on his or her heels as this may encourage bowing of the tibias and shortening of the heel cords.
It is important to discuss weight bearing with the interdisciplinary team before your child begins to pull to standing. The team will provide guidance as to whether it is appropriate to initiate standing at that particular time, and as to whether your child might require the support of braces (bracing will be addressed later in this section).
The early standing phase is a crucial time as children often sit by falling on their buttocks. The child with OI cannot risk sitting in this manner therefore extra supervision is required during this phase of development.
It is important to monitor how your child stands up. The transfer to standing should be carefully observed. Particularly when standing is assumed via a half-kneeling position (see photo below), make certain that there is not an excessive amount of twisting that occurs at the hips. In order to ensure that your child does not fall, or pivot too far, support him or her by holding at the level of the pelvis.
Many children with OI will begin standing with the aid of specially-made braces. These braces can be of various designs depending on how much support is required. Possibilities include braces that go to just below the knee (AFOs), to the upper-thigh (KAFOs), or to the waist (HKAFOs). The extent of bracing will be decided upon by the orthopedic surgeon in conjunction with the rest of the team, and will depend on several factors including amount of bowing, bone density, muscular strength and flexibility.
For children who have been given braces, they must be worn for all standing activities.
If intramedullary rodding surgery is required, standing is begin/resumed in a gradual fashion. This is usually initiated with the use of a tilt table. The process of standing, and eventually walking, is directed by your child’s Physical Therapist.
As you can appreciate from the number and variety of exercises presented at this booklet there are many different activities which can be incorporated in a program to stimulate development of gross motor development in children with OI.
Remember that the needs of every child are unique. Each child will benefit from a different combination of exercises and perhaps even different sequencing of activities depending on their particular needs and abilities. As such, this booklet is intended for use when the progression of activities is guided by a Physical Therapist who can create and progress a personalized stimulation program.
If you have any questions or concerns direct them to your health care team. You may find the readings and references that follow interesting. As well, it can be very helpful to make connections with parents of other children with OI. Feel fre to communicate with us at the Shriners Hospital at mhunt@shrinenet.org or sgould@shrinenet.org.