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Created in conjunction with Lilias Huxham (paediatrician and mother of a Type I baby) and other Type I families.
Day to day care of SMA babies is much the same as for other babies which is good because the more you can keep things normal the more enjoyment you will have together. As they have less movement than other babies they need more entertainment to avoid boredom. However they reward you with being quicker to watch you, smile and to babble than other babies.
The following suggestions may seem obvious, but as your baby's health changes these few things can make a significant difference in the day to day happiness of your baby.
All babies are different. Some of these suggestions may work for your baby and some may not be suitable. Trial and error is the only answer, and often your instinct as a parent will provide the right answer.
General Comfort
As your baby's strength changes you will find that he/she cannot sit propped up and needs to find another comfortable position. The comments below may help. See also the section on seating.
Try laying your baby on a feather filled pillow. Have more than one pillow as they can become damp and uncomfortable. Keep your baby's head to the side to allow their saliva and secretions to drain easily out of the mouth, and to prevent choking.
Put a facecloth or muslin square under your baby's head, which can easily be changed with a clean one when wet with saliva. A breast pad may also be used in this way to soak up saliva. Facecloths are also useful for laying your baby's head on at night, as some babies' heads become sweaty.
Regularly turn your baby from side to side to prevent soreness and stiffness. If lying on his/her side, make sure that the arm, shoulder or ear are not trapped and ensure that the ears are flat.
A sheepskin can be very comfortable for your baby to lie on and can be used in the pram too.
Because your baby may develop weak neck muscles, a small neck roll may help your baby hold his/her neck in a more comfortable position and help with breathing. Such a roll can be purchased in baby shops - often marketed for in-car travel.
If your baby is lying on his/her back, it may be useful to use either a small wedge or rolled blanket on either side of the hips, to support the legs, so they do not flop outwards all the time. Your occupational therapist or physiotherapist may be able to advise or provide appropriate hip support.
Clothes
Try to find easy ways to put on clothes so you do not have to struggle with your baby.
Make sure vests have large neck-holes and are large enough not to dig into the skin.
Cuffs on garments should be very loose.
Clothes, which button up at the back, are more difficult, as some babies with SMA dislike lying on their tummies. Front fastening are best.
Thin loose clothes are most useful as you can remove one layer to reduce heat without too much distress to your baby.
Often SMA babies' legs, feet, arms and hands are quite cold, so you could try a pair of tights, booties and mittens if necessary. Some SMA babies have very warm sweaty hands and feet, so it is best to make sure they are washed and dried regularly.
Make sure that tights and trousers are loose around the waistband so as to not impede diaphragmatic breathing (tummy going up and down with breathing).
Positioning
During the day your baby needs to change position frequently (at least hourly) to avoid stiffness and boredom.
You need to find a compromise between providing a firm level surface to aid deep breathing and a more upright position that will be better for looking around.
Most able bodied babies lie flat for the first month or so and then sit for short periods in bouncing chairs or car seats. At about 3 months they usually sit propped up with cushions for longer periods.
SMA babies will begin by lying flat and depending upon the ease of their breathing and general strength, may progress to sitting or lying in a more propped up position. Later they may again need to lie flat. If they are propped up at too steep an incline or lie on anything that sags or is curved in the trunk area, their chest will concertina and prevent them from taking deep breaths. Deep breathing is necessary to keep the lungs clear from excess mucus secretions.
You will know if the position is okay for your baby's chest if he/she is breathing in the same way as when lying down, looks comfortable and is not dribbling more than normal. If the chin is tucked in too much, it is difficult to swallow any saliva that has collected in the mouth, so it dribbles out. Sometimes a position is tolerated for a while, but if your baby becomes upset in one position or seat and all others are eliminated, then it could be that the position is causing breathing problems.
As with other needs (food, sleep etc.) babies develop characteristic ways of showing they have difficulty breathing. (See Chest Care - Recognising breathing difficulties)
Sweating
Many babies with SMA sweat, and some sweat profoundly during the night. There are a number of reasons for this which include: need for turning; too hot or too cold; need for suctioning; constipation; general discomfort. It is a good idea to lie your baby on a small muslin square as a head sheet as this can easily be changed, without disturbing your baby.
Temperature control
Babies with SMA can be very sweaty, have flushed faces, and hot or cold hands, and it can be very difficult to judge if your child's temperature is all right. This can be particularly difficult at night time.
It is worth getting a small digital thermometer (available from the chemist) to measure the temperature of your child either under the arm (or inside the side of the mouth), and then you will know for definite if your child's temperature is all right. Ear thermometers are very quick to use, but don't always give reliable results if the earpiece is dirty, or not placed properly in the ear.
Movement
Most babies spend a lot of time kicking their legs and thrashing their arms, and later, rolling over and back again. SMA babies enjoy moving too and get great pleasure having their legs moved as if peddling a bicycle, twisting at the hips and doing "hand jives" with their arms to music or songs. This also stops them from getting stiff and helps to maintain the full range of movement in every joint. If this is your first baby, look at how other babies of the same age move and try to help your baby move in the same way.
Although it is necessary to be gentle with SMA babies until you have got to know the range of movement they can achieve, you will know if you have moved a joint slightly too far, because your baby will tell you! Be gentle at first, but have fun, as they will give you feedback on how they feel.
As well as moving your baby in this passive way with you doing all the work, you can encourage active movement by moving him/her slowly enough so that he/she can participate in the movement in an active way. Active movement can also be encouraged when playing with toys by careful positioning and giving the least help necessary to enable your baby to manage and still be comfortable.
For the baby of six months or more, side lying can be useful, as your baby can more easily bring both hands together, and play with both hands.
Arm slings which hang from an A frame baby gym are available. These help support the forearm, freeing up your baby's hands to allow good hand use. These are suitable for the older child - ask the Jennifer Trust for advice.
Seating
You may find some of the following ideas useful:-
Bouncing chair when your baby is not having problems with breathing.
Pushchair with wedge in seat , set to lie-back position, when your baby is not having problems with noisy breathing.
Pram with wedge shaped firm foam insert.
The supplier may be able to cut out an insert (though it may take 2 weeks or so to obtain). Have it thick enough so that your baby can see out over the side of the pram, and the sloping part angled to 15 - 30 degrees to the horizontal, depending on the strength of your baby's trunk. To prevent him/her slipping down, have the area under the legs level.
If the foam is divided into upper and lower sections, the former can be removed and used around the house.
Your physiotherapist or occupational therapist may also be able to supply an appropriate insert for your pram.
Specialist Seating
Your physiotherapist or occupational therapist may be able to assist you to get specialist seating on loan. Ideally a seat should be able to provide a lot of support when upright or semi-upright. It should be able to go into a variety of positions, including upright and flat. It should allow rapid access to take your child out if he/she is distressed. For the older child with severe SMA the Symmetrikit Chair from the Helping Hand Company may be appropriate.
Getting Out and About
It is very good for both you and your baby to get out and about. Not only is the change of scene stimulating for your baby, but it will also be beneficial for your baby's fitness - the gentle bouncing in the pram can be excellent chest physiotherapy.
There are some excellent prams available nowadays at not too great a cost, which can easily be adapted for babies and small children with SMA Type 1. Aim to have a pram that can give a variety of positions including lying flat. Ideally if your baby can lie flat, he or she should be able to see out from a side-lying position. You should also choose a pram that allows you an easy view of your baby. Many of the 3-wheelers are less good for views out from the side - but there are a few available which are good - it is a question of looking around, and the Jennifer Trust may be able to advise also. Aim to have a pram that has a carrying basket to enable carrying a suction machine and small oxygen cylinder if these are needed. Your baby may benefit from lying in a side position. A rolled up blanket to hold the back in a steady position, a small neck roll, and soft toys can be useful to hold other parts of the body comfortably. Your physiotherapist or occupational therapist may be able to advise on wedges or foam inserts in your pram to make your baby comfortable.
Specialist Pushchairs/Buggys
If your baby is less severely affected by the condition he/she may have sufficient trunk strength to sit more upright and so benefit from a specialist pushchair/buggy by the age of 4 - 5 months. Ask your physiotherapist to refer you for a wheelchair assessment and to let you know if there is likely to be a delay. If you have any problems, contact the Jennifer Trust. We have found two types to be particularly suitable; the Nursery Snug Seat, from Tendercare and a Matrix seat fitted to a Maclaren chassis. The Matrix is individually moulded around your baby's body and therefore is slightly better at keeping the spine straight if his/her trunk is beginning to bend permanently.
Car transport
Whilst transporting your baby by car will become more difficult as he/she deteriorates, it may still be possible to take your baby out. This would be safest if someone else can drive whilst you look after your baby.
Even if your baby is dependent on suction and oxygen, it can still be possible to get out and about, and visit enjoyable places such as zoos and the seaside. If your baby is dependent on suction, see if you can get a second suction machine as a back up and keep spare equipment such as suction catheters in the car.
Your baby will probably be able to use a standard car seat at first, but later this may be too upright and flexed. If you are using the Snug Seat, this can be removed from the wheelchair chassis and used as a car seat. The Matrix is not legally allowed to be sold as a car seat because the individual design for each user means that they would have to safety test every seat issued. However it is up to you to decide what you think is best for your child from an overall perspective.
If your baby is having difficulty with the car seat, try to pad out your baby's car seat and also try making head supports that can be velcroed on and adjustable. A small neck support may also be useful.
If your baby cannot use a car seat, the following may be useful:
Contact your GP for a letter/certificate enabling you to take your baby in the car without being in a car seat. Carry this document at all times in your car.
Pram reins can be fitted as a harness in a carrycot and the cot secured with a safety belt in the back seat. This is probably the best method if breathing is difficult, but the worst for providing a good view out. The foam wedge mentioned above (seating section) would help though.
If someone can drive the car for you, transport your baby in the carrycot so he/she can be flat. If you cannot strap him/her in you will have to hold or secure the carrycot as best you can. As your baby grows you may consider transporting him/her by side lying flat on a raised cot mattress within the car. Securing him/her provides a challenge to meet your own acceptable safety limits, but you can try Velcro strips and a high chair harness. Contact the Jennifer Trust about other parents' experience.
Bathing
Even children who have severe SMA Type 1 and who are very dependent on suction can really enjoy bathing. They can often move very much better once they are weightless in water, and so plenty of time in the bath provides good physiotherapy. It will also help the breathing muscles.
Bathing can be tiring for your baby. If he/she gets upset after bathing it may need to be kept brief and followed by a nap. It is probably best to feed after bathing, rather than before.
To assist bathing, and to enable you to give a bath without needing to hold your baby, it is useful to use a bath support.
There are a variety of bath supports available. Ordinary baby bath supports, available from baby-care shops, can be used to support your baby's head above the water. The rigid bath support will hold the baby safely in a slightly upright position, but will allow free movement of the arms and legs. . There are also cloth bath supports but these may allow the chest to sag and cause breathing difficulties. Another type is a soft cloth support filled with small polystyrene balls, which allow the baby to float supported on the surface - this may be useful if your baby has difficulty in the more upright positions. There are specialist bath supports, for example the "Souris Bath Support", if your baby outgrows commercially available bath supports. There are also inflatable neckbands, eg the Schwimmi Swim Collar, available from specialist companies that can support the larger baby totally in the water, allowing safe and comfortable floating on the top of the water. Your occupational therapist can advise on which to use.
Swimming
Ask your physiotherapist if a hydrotherapy pool is available in your region. This is a very warm swimming pool for people with medical needs. Babies with SMA can really enjoy this, and it can be an excellent environment for physiotherapy.
Comfort at Night
Try and keep your baby on a baby mattress or pillow, as these are far more comfortable than an adult mattress.
Have plenty of extra mattress covers and facecloths to replace regularly if they become damp in the night.
Rotate your baby as much as possible to reduce stiffness.
If your baby frequently coughs during the night try removing the bumper to allow air movement without being in a draught. Some babies do not like being in an enclosed cot i.e. Moses Basket, as air circulation is not so good.
If your baby produces a lot of saliva at night, try tilting the cot slightly (feet end up) using books etc., so that the saliva can run out of their mouths more easily. This may not be suitable for all babies however, particularly if they have problems with gastro-oesophageal reflux or are having overnight tube feeding.
Use several thin light layers of covers to vary heat temperature as some babies become cold, and some sweat a lot and require fewer covers.
Keep your baby in your own bedroom at night so you can check on him/her regularly.
It is a good idea not to overheat the room or have a room that is too dry as this may cause the secretions to become too sticky, which will make it more difficult for your baby to get them up or for you to carry out suctioning. It is worth keeping the window open a little.
There are specialist mattresses available for babies and young children made of "tempura" which moulds with the body, and can be more comfortable for a child with SMA. Your district nurse, or the Jennifer Trust may be able to advise.
If your baby needs a lot of help overnight, your local health trust may be able to provide night nursing. Discuss this with your health visitor or paediatrician. | 
