New born baby with osteogenesis imperfecta (brittle bones)

Introduction

Babies diagnosed with osteogenesis imperfecta (OI) may have fractures which appear to have little or no apparent cause. It is important to realise that with the best care in the world, babies with brittle bones may still have fractures.

Babies with brittle bones enjoy the same things as other babies. When handling a baby with OI use slow, gentle movements. Plan ahead: avoid startling baby as this could cause a sudden movement that could result in fracture. Babies with brittle bones also like to be spoken or sung to, kissed, cuddled, rocked and stroked as with any other baby, but just with a little more care.

Handling

Always check that baby’s arms and legs are not caught in a blanket or any other objects as you lift, paying special attention to fingers and toes. There could be an increased risk of causing rib fractures if a baby is lifted under the arms or around the ribs. Lift with both hands wide and slide one hand under the buttocks and lower back, with the other hand behind the head, neck and upper back.

If your baby does have a fractured arm or leg, seek advice from your local physiotherapist or occupational therapist.

Nappy changing

Slide your hand under their buttocks to lift your baby to replace or remove the nappy. It may be easier to slide a clean nappy under them before removing the dirty nappy, as this helps to avoid having to move baby twice.

When you change the baby’s nappy, do not hold or lift the baby by the ankles or legs.

Rolling baby to the side can also be helpful when cleaning.

Feeding

When breast feeding, avoid putting baby’s arm around your back. Take care of their legs, which may lie against your body at an awkward angle.

If bottle-feeding it is helpful to change the side from which you feed (left or right) in order to prevent one-sided head turning and altered head shape. Feed slightly inclined on the pillow if necessary.

Babies may get tired quite easily when feeding. It is helpful to be aware that some babies take very small amounts of food, little and often, as they cannot manage very much at one time.

Traditional methods of getting wind up may be too much for babies with more severe OI. You should also consider rubbing their back instead of patting. If winding becomes a problem, then speak with your GP about colic drops like Infacol or similar medicines. Please ask the specialist team for further advice if this becomes a struggle.

Bathing

Be well prepared before you start and get out everything you need. You can use a bath support sponge which supports baby’s body and limbs. Take care to clean skin folds such as the armpits and neck creases — cotton buds can be helpful. Make sure the creases are dried thoroughly.

Dressing

Choose cotton rich, lightweight clothing, as some babies with brittle bones can sweat quite heavily. Choose clothes that are easy to put on to reduce the need to bend baby’s limbs when dressing. Choose a garment that is a size or two larger, or look for clothing that opens wide. This will allow you to place baby onto the garment and then fasten it. Avoid ribbons, frills and knitted fabrics with holes that may trap fingers.

When dressing your baby, always slip your hand up the sleeve and gently bring baby’s arm back down. This helps avoid accidentally catching baby’s arm in the garment. At times of fracture, dressing may be tricky and it is likely your baby will have to be only partially dressed.

Clothing can be adapted by cutting down the main seams and adding Velcro or fasteners, allowing baby to be laid onto the clothes before fastening, rather than having to pull clothes over baby’s head and limbs.

Positioning

The skull of a young baby may be quite soft. As a baby tends to spend time on their back, a flattened area may develop on the head, often at the back. Depending on the severity of their condition this is more likely to happen in a baby with OI.

In order to promote good head shape you should encourage regular position change. A physiotherapist can teach you this activity. Side-lying can also be used to improve head shape, but must be done with advice from the specialist team or local therapist.

Car seats

Always use a car safety seat that is appropriate for the baby. You should always follow the manufacturer’s instructions when using and installing your child’s car seat and harness.

Your baby should not remain in a car seat for long periods (over 2 hours). If you are going on a long journey, take frequent breaks when you can remove your baby from the car seat.

Fracture management

Recognising that a fracture has occurred can be very difficult. Crying, swelling or refusing to use a limb may be the first indication that a fracture has happened. A baby may not always cry.

It is rarely anyone’s fault and may happen even with the best care given. Sometimes X-rays do not initially show up a fracture, so if there is any doubt, you should treat your baby as if one had occurred. Try to move your baby as little as possible and seek medical advice as soon as you can.