It is a good idea to change an immobile person's posture at least once every hour, day and night, not just to ease monotony, but to keep the body supple. Some different suggestions as to fun ways to position them are listed below:
Tilt in space dining chair: If you can get funding a tilt in space dining chair such as Lekkey Advance, will be useful for mealtimes, and a nice change from the wheelchair.
http://www.leckey.com/template.asp?parent=48&pid=218&webArea=1
Feeder seat and positioning wedge: particularly for children, the feeder seat allows the person to play with friends and siblngs at floor level.
http://www.sammonspreston.com/content/PDF/spr/Pediatrics/PediatricForms/FeederSeat_09.pdf
Hammock: Use only with supervision - a lying down alternative to the swing, relaxing and giving a sense of motion. They can be bought with stands as well, which allows them to be used indoors too.
http://www.hammocks.co.uk/hammock-stands.htm
Swing: Also provides a sense of motion. For the UK:
http://uk.specialneedstoys.com/swings/1201-full-support-swing-seats.html
For the US:
http://www.backyardcity.com/swingsets/accessories/JennSwing.htm
Wagon: If a car seat is used, this can be an alternative to a wheelchair. Or the person can lie supine like a mobile bed. It allows comfortable reclining position whilst someone uses the handle to pull the wagon, meaning the person gets an easy change of scene.
http://www.wagonworld.com
In the US:
www.westcoastwagons.com
Fold-out Chair-bed: This can be used in the living room, and folded away to make extra room. Provides comfortable, floor-level positioning for the person.
http://www.nextag.co.uk/fold-out-chair-bed/zzukzB1z38--products-html
Other Useful equipment includes:
-A neck pillow
-A draught excluder, which can be used to prop a person up on their side
-A small cushion for the person's feet
- A V-shaped pillow for propping a person up in bed
- A wedge-shaped pillow for keeping a person's head propped up when lying flat
- A bath/shower seat
http://www.smirthwaite.co.uk/bathing/index.html
- A Car seat
http://www.adaptivemall.com/colorseat.html
Tuesday, 27 October 2009
Rice Cushions - Great for Aromatherapy and Fluctuating Temperatures
Many people in the late stages of leukodystrophy experience repeated infections that cause fever, but also temperature fluctuations due to damaged myelin in areas of the brain that regulate temperature. This is a little trick that may help.
1. Find four cotton cushion covers that are tightly woven, and fill two of them with dry, uncooked rice, corn or buckweat. Don't stuff them full, let there be some room for the filling to flow and the cushions to mould to desired shapes.
2. If you want, add some scent such as dried lavender, cammomile and lemon peel, and put these in the filled cushion covers too.
3. Sew the filled cushion covers firmly shut, place each in an empty cushion cover and sew these shut too. Now you have what look like two cushions.
4. If you want to use them you can either microwave them for a couple of minutes, or put them in the freezer for 45 minutes, depending on whether the person's temperature is too high or low. If the person's temperature is swinging between extremes, one can be microwaved and one can be put in the freezer, and as the temperature fluctuates, you can switch between the two.
WARNING: Make sure the rice does not overheat by heating it for a maximum of 20 seconds at a time in the microwave, then testing the temperature. Place a non-metal glass of water in with the sock to prevent a fire starting. Always test the temperature of the heated/chilled rice cushion on your own cheek or arm first, to make sure it is not uncomfortably hot or cold.
1. Find four cotton cushion covers that are tightly woven, and fill two of them with dry, uncooked rice, corn or buckweat. Don't stuff them full, let there be some room for the filling to flow and the cushions to mould to desired shapes.
2. If you want, add some scent such as dried lavender, cammomile and lemon peel, and put these in the filled cushion covers too.
3. Sew the filled cushion covers firmly shut, place each in an empty cushion cover and sew these shut too. Now you have what look like two cushions.
4. If you want to use them you can either microwave them for a couple of minutes, or put them in the freezer for 45 minutes, depending on whether the person's temperature is too high or low. If the person's temperature is swinging between extremes, one can be microwaved and one can be put in the freezer, and as the temperature fluctuates, you can switch between the two.
WARNING: Make sure the rice does not overheat by heating it for a maximum of 20 seconds at a time in the microwave, then testing the temperature. Place a non-metal glass of water in with the sock to prevent a fire starting. Always test the temperature of the heated/chilled rice cushion on your own cheek or arm first, to make sure it is not uncomfortably hot or cold.
Monday, 17 August 2009
Useful Items of Clothing
This is not meant to be a means of advertising, just providing links so the things that I mention can be easily found and seen.
- Tummy Tunnels - For children. Iron on patches for clothing that allow easy access to gastrostomy tubes.
http://www.tummytunnels.com/
- Special needs all-in-one body vests. Prevent draughty gaps. These vests have options that allow access to gastrostomy tubes, and are good for older children who use nappies.
http://www.tanniandanni.com/kidsclothing/poppervests.htm
- Slipper socks. These are good for people who may want shoes off in the house or when in wheelchair, and need footwear that will be both comfortable and warm. The trouser legs can be tucked into them to avoid draughty gaps.
Adult slipper socks: http://www.sockshop.co.uk/by_type/shop-socks/bed_slipper_sogcks/index.html
Child slipper socks: https://www.socksolutions.co.uk/sess/utn;jsessionid=154a892e63b2979/shopdata/0040_Bed+Socks+=26amp=3B+Slipper+Socks/product_details.shopscript?article=0030_FALKE%2B%3D27Catspads%3D27%2BSlipper%2BSocks%2B%3D28Young%2BChildren%3D29%2B%3D28FCATC%3D29
- Muslin squares - good if the person drools saliva. They avoid getting clothes too wet, and getting chaffed skin from too much wiping of the mouth. They are also good if there is a tendency to vomit
http://www.amazon.co.uk/Muslin-squares-pack-of-10/dp/B000Z7BJ8K
- Special needs swim nappy - for incontinent children, teens and adults
http://www.incywincy.net/acatalog/Swimwear_for_Older_Children_Special_Needs__.html
- A crewsaver with a neck support, and a couple of pairs of armbands (one pair for the arms, one for the legs) should enable the person to float unaided in a swimming pool, even if they can't move
- All-in-one UV resistant swim suit. May keep the person warmer in water. This site is American, but the suits are sold in Britain too. Also they are aimed at children but go up to adult size
http://www.funsunwear.com.au/index.php
- Tummy Tunnels - For children. Iron on patches for clothing that allow easy access to gastrostomy tubes.
http://www.tummytunnels.com/
- Special needs all-in-one body vests. Prevent draughty gaps. These vests have options that allow access to gastrostomy tubes, and are good for older children who use nappies.
http://www.tanniandanni.com/kidsclothing/poppervests.htm
- Slipper socks. These are good for people who may want shoes off in the house or when in wheelchair, and need footwear that will be both comfortable and warm. The trouser legs can be tucked into them to avoid draughty gaps.
Adult slipper socks: http://www.sockshop.co.uk/by_type/shop-socks/bed_slipper_sogcks/index.html
Child slipper socks: https://www.socksolutions.co.uk/sess/utn;jsessionid=154a892e63b2979/shopdata/0040_Bed+Socks+=26amp=3B+Slipper+Socks/product_details.shopscript?article=0030_FALKE%2B%3D27Catspads%3D27%2BSlipper%2BSocks%2B%3D28Young%2BChildren%3D29%2B%3D28FCATC%3D29
- Muslin squares - good if the person drools saliva. They avoid getting clothes too wet, and getting chaffed skin from too much wiping of the mouth. They are also good if there is a tendency to vomit
http://www.amazon.co.uk/Muslin-squares-pack-of-10/dp/B000Z7BJ8K
- Special needs swim nappy - for incontinent children, teens and adults
http://www.incywincy.net/acatalog/Swimwear_for_Older_Children_Special_Needs__.html
- A crewsaver with a neck support, and a couple of pairs of armbands (one pair for the arms, one for the legs) should enable the person to float unaided in a swimming pool, even if they can't move
- All-in-one UV resistant swim suit. May keep the person warmer in water. This site is American, but the suits are sold in Britain too. Also they are aimed at children but go up to adult size
http://www.funsunwear.com.au/index.php
Saturday, 15 August 2009
Potential Curative and Palliative Treatments, Scientific and Non-Scientific
I am not actively advocating any of these, and you should definitely talk to a doctor (or even more than one - it is your right to be able to do this), and/or homeopathic expert, and do more research before considering pursuing any of these. The purpose of these being here is to give parents, carers and in some cases patients, the widest range of choices. Leukodystrophy and the secondary problems arisinf from it are tricky to treat, and quality rather than quantity is the most important thing - but that does not always have to mean just taking a person home and loving them.
Potentially Curative
- Bone Marrow Transplant: This may be an option for very mildly symptomatic or asymptomatic metachromatic or adrenoleukodystrophy. Involves killing all the bone marrow in the body using aggressive chemotherapy (this will cause hair loss, vomiting, diahorrea, possibly fevers and rashes, immune reactions, and severe susceptibility to infections). Some young children may be at risk from just the chemotherapy. Then the donor bone marrow is dripped in through an IV line in the chest, and encouraged to grow. Infection susceptibility, graft vs. host disease, and rejection of the donor marrow can be risks for months afterwards, and the child's appearence will be very much altered (although those constantly nearby will not notice so much). Furthermore, the disease may progress for some months afterwards until the marrow cells have grown more, and in some cases transplant will fail to stop disease progression. A specific bone marrow match must also be found, either from within the family, or in the general population. However, if the procedure works, the disease will be slowed or even halted for the time being. The long term effects are not known.
- Cord blood transplant: This is in many ways similar to bone marrow transplant and is used to treat asymptomatic Krabbes Disease. The main difference is that instead of bone marrow being donated, stem cells and blood from the umbillical cord of a newborn infant donor are used. Most children who undergo this kind of transplant have been diagnosed with Krabbes Disease prenatally.
- Lorenzo's Oil: This is used to treat asymptomatic adrenoleukodystrophy, and is a blend of purified oleic acid (found in olive oil) and erucic acid (found in rapeseed oil). It stops the body producing the fatty acids it can't break down, because the same chemical that produces them breaks down oleic and erucic acid - in effect the chemical is 'distracted'. The long term effects of the oil are not known and usually if symptoms of the disease have already begun, the oil does not work. However its efficacy in treating asymptomatic ALD has been fully proven scientifically.
Palliative
- Dimethyl Sulphoxide: Studied Scientifically and reported as somewhat protecting nerves and decreasing inflammation in Krabbes disease
- Nitrizepam: Studied Scientifically and reported as being helpful in reducing irritability and increasing comfort in Krabbes disease
- Gelsemium 30: This is a homeopathic medication sometimes used in the treatment of unspecified leukodystrophies.
- Equal volumes of tincture of myrrh and olive oil, mixed in a teaspoon, heated over a small flame, and once cooled to body temperature, rubbed immediately down the muscles either side of the spine every day. It can't be stored and must be mixed fresh every time. This a supposed treatment for neurological dysfunction, but its efficacy has in no way been proven. However myrrh has long been used as a painkiller, and olive oil rubs easily into skin, because the skin is very fatty. Therefore some insulation of nerves may occur - though the effect, if any, would be extremely slight.
Potentially Curative
- Bone Marrow Transplant: This may be an option for very mildly symptomatic or asymptomatic metachromatic or adrenoleukodystrophy. Involves killing all the bone marrow in the body using aggressive chemotherapy (this will cause hair loss, vomiting, diahorrea, possibly fevers and rashes, immune reactions, and severe susceptibility to infections). Some young children may be at risk from just the chemotherapy. Then the donor bone marrow is dripped in through an IV line in the chest, and encouraged to grow. Infection susceptibility, graft vs. host disease, and rejection of the donor marrow can be risks for months afterwards, and the child's appearence will be very much altered (although those constantly nearby will not notice so much). Furthermore, the disease may progress for some months afterwards until the marrow cells have grown more, and in some cases transplant will fail to stop disease progression. A specific bone marrow match must also be found, either from within the family, or in the general population. However, if the procedure works, the disease will be slowed or even halted for the time being. The long term effects are not known.
- Cord blood transplant: This is in many ways similar to bone marrow transplant and is used to treat asymptomatic Krabbes Disease. The main difference is that instead of bone marrow being donated, stem cells and blood from the umbillical cord of a newborn infant donor are used. Most children who undergo this kind of transplant have been diagnosed with Krabbes Disease prenatally.
- Lorenzo's Oil: This is used to treat asymptomatic adrenoleukodystrophy, and is a blend of purified oleic acid (found in olive oil) and erucic acid (found in rapeseed oil). It stops the body producing the fatty acids it can't break down, because the same chemical that produces them breaks down oleic and erucic acid - in effect the chemical is 'distracted'. The long term effects of the oil are not known and usually if symptoms of the disease have already begun, the oil does not work. However its efficacy in treating asymptomatic ALD has been fully proven scientifically.
Palliative
- Dimethyl Sulphoxide: Studied Scientifically and reported as somewhat protecting nerves and decreasing inflammation in Krabbes disease
- Nitrizepam: Studied Scientifically and reported as being helpful in reducing irritability and increasing comfort in Krabbes disease
- Gelsemium 30: This is a homeopathic medication sometimes used in the treatment of unspecified leukodystrophies.
- Equal volumes of tincture of myrrh and olive oil, mixed in a teaspoon, heated over a small flame, and once cooled to body temperature, rubbed immediately down the muscles either side of the spine every day. It can't be stored and must be mixed fresh every time. This a supposed treatment for neurological dysfunction, but its efficacy has in no way been proven. However myrrh has long been used as a painkiller, and olive oil rubs easily into skin, because the skin is very fatty. Therefore some insulation of nerves may occur - though the effect, if any, would be extremely slight.
Useful Organisations
- Stennis Foundation: Research Metachromatic and other Leukodystrophies
http://www.stennisfoundation.org/web/
- Bethany's Hope: Research Metachromatic and other Leukodystrophies
http://www.bethanyshope.org/
- Brianne's LEAP (Leukodystrophy Education and Awareness Project) of Hope
http://www.briannesleapofhope.org/
- Hunter's Hope: Reseach Krabbes and other types, and push for newborn screening
http://www.huntershope.org/site/PageServer
- MLD Foundation: All things to do with metachromatic leukodystrophy
http://www.mldfoundation.org/
- Adrenoleukodystrophy (ALD) Foundation: All things to do with adrenoleukdystrophy
http://www.aldfoundation.org/
- The myelin project: Research cure and treatment of all de-myelinating disease
http://www.myelin.org/
- CLIMB (Children Living with Inherited MetaBolic disease)
www.climb.org.uk
- Childrens' Hospice UK
www.childhospice.org
- United Leukodystrophy Foundation
www.ulf.org
- Evanosky Foundation - fights metachromatic leukodystrophy and other leukodystrophies:
http://www.evanoskyfoundation.org/
http://www.stennisfoundation.org/web/
- Bethany's Hope: Research Metachromatic and other Leukodystrophies
http://www.bethanyshope.org/
- Brianne's LEAP (Leukodystrophy Education and Awareness Project) of Hope
http://www.briannesleapofhope.org/
- Hunter's Hope: Reseach Krabbes and other types, and push for newborn screening
http://www.huntershope.org/site/PageServer
- MLD Foundation: All things to do with metachromatic leukodystrophy
http://www.mldfoundation.org/
- Adrenoleukodystrophy (ALD) Foundation: All things to do with adrenoleukdystrophy
http://www.aldfoundation.org/
- The myelin project: Research cure and treatment of all de-myelinating disease
http://www.myelin.org/
- CLIMB (Children Living with Inherited MetaBolic disease)
www.climb.org.uk
- Childrens' Hospice UK
www.childhospice.org
- United Leukodystrophy Foundation
www.ulf.org
- Evanosky Foundation - fights metachromatic leukodystrophy and other leukodystrophies:
http://www.evanoskyfoundation.org/
Friday, 14 August 2009
The Importance of Physiotherapy and Good Posture
Physiotherapy is especially important for a person with limited movement, like someone with leukodystrophy. Without physiotherapy, muscles stiffen up and cause pain/deformity. Furthermore, when muscles stiffen up they press on vital organs like the intestines and heart. This increases the risk of problems like constipation or cardiovascular problems.
Again, you need to be in frequent contact with doctor and physiotherapist because the person with leukodystrophy will have changing needs as the disorder progresses. However, this is a basic guide of stretches that may help keep the body supple. The person with leukodystrophy is referred to as 'she', and a child in this - apologies if 'she' is actually an adult 'he'. You will need:
- Large Physiotherapy exercise ball
- Trampoline or similar bouncy suface, e.g. a mattress
This should be done once a day, prferably in the morning.
1. Uncurl her fingers, and flex them a few times, to stretch the muscles in her hand, and move each hand in circles – ten circles clockwise and ten anti-clockwise, thus flexing the wrist muscles
2. Repeat with the ankles what you did with the hands
3. Holding the leg up with one hand at the calf (muscle behind the shin), push the foot back gently towards the shin, just enough until you feel resistance from the foot to go further. You should feel the muscle on the calf flex when this happens. Push back five times for each leg, and move each foot in a few circles afterwards to relieve tension.
4. Hold the arm with one hand on the wrist, and the other hand palm to palm with her own hand. With the palm-to-palm hand, push back gently until the hand resists and you feel the underside of the wrist flex. Do this five times for each hand, and then move each hand in a few circles afterwards to relieve tension. Re-bandage the tennis balls.
5. Hold each arm, one of your hands holding her wrist and one holding her shoulder. Bend the arm at the elbow, five times, then pause for a few seconds, and then another five times. Do this with both arms, and similarly bend each leg at the knee and hip in the same way.
6. Hold each leg at the ankle. Bend the knees simultaneously as far up towards the chest as they will easily go, stretching out the back. Do this five times, and then pause. Now do this another five times, but this time, hold in the folded position with the back stretched out. Turn the legs to the right side, then to centre, then to left and then to centre five times, keeping her back flat on the surface. This swivels the hips.
7. Hold each leg at the knee and the calf muscle. Separately lift each leg upward so the sole of the foot points toward the ceiling, keeping the leg straight at all times. This stretches out the back of the thigh and the calf. Do this five times with each leg, making sure not to elevate the leg further than it easily goes.
8. Turn the child onto her stomach. Hold the leg bent at the knee, with one hand cupping the kneecap, the other holding the ankle, and lift the thigh very slightly upwards before letting it down again. Do this five times to stretch the hamstring and the abdominal muscles. Make sure you do not force upwards if the thigh doesn’t want to go.
9. Turn the child back onto her back. Place her arms straight by her sides and take her hands. Keeping her arms reasonably straight, raise them up until her fists point towards the ceiling. Still keeping her arms straight, continue the arc of movement down until her shoulders and upper arms are parallel with her ears. She should now look as though, were she standing up, she were stretching both arms up to reach something above her. Still keeping her arms straight, follow through the arc of movement again in reverse, until her arms rest once more by her sides. Do this five times.
11. Very carefully take the sides of her head in your hands, and rotate her neck to the right, back to centre, to the left, and back to centre. Do this five times to stretch her neck muscles.
12. Lay her over the large Physio ball, and, holding her in place with one hand, gently tip the ball from side to side a few times, and back and forth. This should strengthen her back muscles. If she can tolerate without nausea, push the ball up and down a little, allowing slight bouncing movement.
13. Have fun on the trampoline/mattress! Lie her on her back on the trampoline, and bounce her slightly, taking care she doesn’t choke. She should be relaxed and supple now, and this will allow movement of her muscles, warming them down. If she likes it, also bounce her lying on each side of her body, and on her stomach.
Braces and Splints
Best not to go overboard with these if the person is quite supple and everything looks normal. However there are a few that will greatly help:
- Back brace: This can be worn when sitting upright in her wheelchair, car seat, or any other seat, and stops the person slumping sideways, as too much sideways slumping can cause a curvature of the spine that's impossible to correct without surgery.
- AFOs: This stands for ankle-foot orthoses - and are braces that keep the foot at right angles to the leg. Without these, sometimes the feet can stick into a pointed position, making stretching them, or using a stander (see below), impossible. Sometimes AFOs can help keep the knees straight as well. AFOs can be worn intermittently through the day, but are definitely worth wearing if possible, when in the wheelchair or car seat. You can also get HKAFO which stands for Hip-Knee-Ankle-Foot-Orthoses. These are like AFOs but also keep the hips and knees in correct alignment too. This may be useful if the person with leukodystrophy gets frequently dislocated hips.
- Hand splints: These may be useful to wear intermittently throughout the day, in wheelchair/car seat, or at night to stop the hands becoming stuck into an unyielding fist shape.
Stander Time
The importance of time in a stander is being more and more understood now. When a person with leukodystrophy never supports their own weight, they can lose calcium from their bones, making them brittle. That means lifting them into wheelchairs, bathing or doing stretches can cause bone breaks. As well as possibly using calcium and vitamin D supplements, you can use a stander, which lets a person who can't move bear weight to help retain calcium in the bones.
A supine stander - one where the person's back rather than stomach is supported, is probably best for someone with leukodystrophy, as it makes breathing easiest. The height when standing and angle of recline (how horizontal or vertical it is) can both be adjusted. It should be low at first, and nearly horizontal, with only a short time spent in the stander. As strength increases, it can be raised to (nearly) vertical and time in it can be increased. I would suggest in the evening, with a distracter activity like a film, tape, story etc. would be a good time and way to do 'stander time'.
Botox and Phenol Injections
Again, talk to your doctor about this, but if the person with leukodystrophy is really stiff, sore and uncomfortable, can't use a stander due to pointed toes, and stretching is not helping, they can undergo botox and/or phenol injections which help loosen the muscles. From what I know, these are done under a general anaesthetic, after a nerve conduction test has been performed to locate the nerves to be injected (electricity is sent down the nerve, and time it takes to travel to the end of the nerve is recorded). The botox and phenol paralyse the muscles, stopping them from becoming stiff, twisting or spasming.
Use of dummies/soothers/pacifiers
One more point: In children extended use of a dummy/soother/pacifier can have the benefit of keeping the child's jaw flexible. In some cases due to lack of movement, the jaw tightens to the point that it's very hard to get their mouth open at all, whether to suction or to brush teeth. Use of a dummy in the car, at night or when relaxing can help hold the jaw open for periods of time and prevent it stiffeing up. In adults, a mouth guard, possibly such as that used to prevent teeth-clenching/grinding at night, might well do the same thing.
Again, you need to be in frequent contact with doctor and physiotherapist because the person with leukodystrophy will have changing needs as the disorder progresses. However, this is a basic guide of stretches that may help keep the body supple. The person with leukodystrophy is referred to as 'she', and a child in this - apologies if 'she' is actually an adult 'he'. You will need:
- Large Physiotherapy exercise ball
- Trampoline or similar bouncy suface, e.g. a mattress
This should be done once a day, prferably in the morning.
1. Uncurl her fingers, and flex them a few times, to stretch the muscles in her hand, and move each hand in circles – ten circles clockwise and ten anti-clockwise, thus flexing the wrist muscles
2. Repeat with the ankles what you did with the hands
3. Holding the leg up with one hand at the calf (muscle behind the shin), push the foot back gently towards the shin, just enough until you feel resistance from the foot to go further. You should feel the muscle on the calf flex when this happens. Push back five times for each leg, and move each foot in a few circles afterwards to relieve tension.
4. Hold the arm with one hand on the wrist, and the other hand palm to palm with her own hand. With the palm-to-palm hand, push back gently until the hand resists and you feel the underside of the wrist flex. Do this five times for each hand, and then move each hand in a few circles afterwards to relieve tension. Re-bandage the tennis balls.
5. Hold each arm, one of your hands holding her wrist and one holding her shoulder. Bend the arm at the elbow, five times, then pause for a few seconds, and then another five times. Do this with both arms, and similarly bend each leg at the knee and hip in the same way.
6. Hold each leg at the ankle. Bend the knees simultaneously as far up towards the chest as they will easily go, stretching out the back. Do this five times, and then pause. Now do this another five times, but this time, hold in the folded position with the back stretched out. Turn the legs to the right side, then to centre, then to left and then to centre five times, keeping her back flat on the surface. This swivels the hips.
7. Hold each leg at the knee and the calf muscle. Separately lift each leg upward so the sole of the foot points toward the ceiling, keeping the leg straight at all times. This stretches out the back of the thigh and the calf. Do this five times with each leg, making sure not to elevate the leg further than it easily goes.
8. Turn the child onto her stomach. Hold the leg bent at the knee, with one hand cupping the kneecap, the other holding the ankle, and lift the thigh very slightly upwards before letting it down again. Do this five times to stretch the hamstring and the abdominal muscles. Make sure you do not force upwards if the thigh doesn’t want to go.
9. Turn the child back onto her back. Place her arms straight by her sides and take her hands. Keeping her arms reasonably straight, raise them up until her fists point towards the ceiling. Still keeping her arms straight, continue the arc of movement down until her shoulders and upper arms are parallel with her ears. She should now look as though, were she standing up, she were stretching both arms up to reach something above her. Still keeping her arms straight, follow through the arc of movement again in reverse, until her arms rest once more by her sides. Do this five times.
11. Very carefully take the sides of her head in your hands, and rotate her neck to the right, back to centre, to the left, and back to centre. Do this five times to stretch her neck muscles.
12. Lay her over the large Physio ball, and, holding her in place with one hand, gently tip the ball from side to side a few times, and back and forth. This should strengthen her back muscles. If she can tolerate without nausea, push the ball up and down a little, allowing slight bouncing movement.
13. Have fun on the trampoline/mattress! Lie her on her back on the trampoline, and bounce her slightly, taking care she doesn’t choke. She should be relaxed and supple now, and this will allow movement of her muscles, warming them down. If she likes it, also bounce her lying on each side of her body, and on her stomach.
Braces and Splints
Best not to go overboard with these if the person is quite supple and everything looks normal. However there are a few that will greatly help:
- Back brace: This can be worn when sitting upright in her wheelchair, car seat, or any other seat, and stops the person slumping sideways, as too much sideways slumping can cause a curvature of the spine that's impossible to correct without surgery.
- AFOs: This stands for ankle-foot orthoses - and are braces that keep the foot at right angles to the leg. Without these, sometimes the feet can stick into a pointed position, making stretching them, or using a stander (see below), impossible. Sometimes AFOs can help keep the knees straight as well. AFOs can be worn intermittently through the day, but are definitely worth wearing if possible, when in the wheelchair or car seat. You can also get HKAFO which stands for Hip-Knee-Ankle-Foot-Orthoses. These are like AFOs but also keep the hips and knees in correct alignment too. This may be useful if the person with leukodystrophy gets frequently dislocated hips.
- Hand splints: These may be useful to wear intermittently throughout the day, in wheelchair/car seat, or at night to stop the hands becoming stuck into an unyielding fist shape.
Stander Time
The importance of time in a stander is being more and more understood now. When a person with leukodystrophy never supports their own weight, they can lose calcium from their bones, making them brittle. That means lifting them into wheelchairs, bathing or doing stretches can cause bone breaks. As well as possibly using calcium and vitamin D supplements, you can use a stander, which lets a person who can't move bear weight to help retain calcium in the bones.
A supine stander - one where the person's back rather than stomach is supported, is probably best for someone with leukodystrophy, as it makes breathing easiest. The height when standing and angle of recline (how horizontal or vertical it is) can both be adjusted. It should be low at first, and nearly horizontal, with only a short time spent in the stander. As strength increases, it can be raised to (nearly) vertical and time in it can be increased. I would suggest in the evening, with a distracter activity like a film, tape, story etc. would be a good time and way to do 'stander time'.
Botox and Phenol Injections
Again, talk to your doctor about this, but if the person with leukodystrophy is really stiff, sore and uncomfortable, can't use a stander due to pointed toes, and stretching is not helping, they can undergo botox and/or phenol injections which help loosen the muscles. From what I know, these are done under a general anaesthetic, after a nerve conduction test has been performed to locate the nerves to be injected (electricity is sent down the nerve, and time it takes to travel to the end of the nerve is recorded). The botox and phenol paralyse the muscles, stopping them from becoming stiff, twisting or spasming.
Use of dummies/soothers/pacifiers
One more point: In children extended use of a dummy/soother/pacifier can have the benefit of keeping the child's jaw flexible. In some cases due to lack of movement, the jaw tightens to the point that it's very hard to get their mouth open at all, whether to suction or to brush teeth. Use of a dummy in the car, at night or when relaxing can help hold the jaw open for periods of time and prevent it stiffeing up. In adults, a mouth guard, possibly such as that used to prevent teeth-clenching/grinding at night, might well do the same thing.
An Example of Breathing Therapy
To establish a therapy that is effective in keeping airways clean and clear, talk to a doctor, physiotherapist or respiratory specialist. However, this is an example - just in case it is not possible to talk to one and therapy is urgently needed, or if the doctor is being very unhelpful as is sometimes, unfortunately, the case.
When the person with leukodystrophy is well, the following treatments should be performed twice a day - in the morning and evening. When ill, this should go up to four times a day.
The Therapy
Fifteen minutes or so before treatment begins, give a dose of expectorant cough syrup (see link for a child-friendly expectorant), to help loosen the mucus. Your doctor may also prescribe a nebuliser treatment to do this and/or to dilate the airway tubes in the lungs. If it seems to help, also bring the person into a steamy room, as steam can make mucus easier to loosen.
Use the vibrating vest in blocks of 3-5 minutes with a couple of minutes rest in between blocks. Two or three blocks will probably be enough. This will help fully loosen the mucus. The vest can sometimes irritate a gastrostomy site if used too often. There are two ways around this: One is to place gauze under the button or tube until the vest treatment is over. The other is to alternate between using the vest and doing something called manual percussion. This is done with gentle 'slaps' to the chest, upper back and sides of the rib cage - one hundred 'slaps' to the chest and each side, and 300-500 lighter ones on the back. It is very important that this is done with a cupped hand, or with something like a cup, because it is not the slapping that loosens the mucus, but the vibrations the action produces - called 'percussion'. It should not sound like a slap - it should sound more hollow.
Use the cough assist on blocks of 4-5 coughs each, with about thirty second pauses between blocks. Start with low pressure of air in and out, and gradually build up the pressure over time. Four or five blocks will probably be enough to bring all the mucus up if the pressure is medium-high, but it will be more if the pressure is still low.
Finally suction. The end of the hose goes into the person's throat to bring out the mucus. The doctor, or possibly respite or hospice nurse, will recommend how far in the hose should go. To stop sucking you just need to remove your finger from the small button near the end of the hose.
When it might be particularly useful for the person to use the bipap
- In the car or disability van when travelling
- At night
- When oxygen saturation is low
- When being very active and/or upbeat, it will allow them to do so without lacking energy from low oxygen, or worrying about breathing
N.B. When removing the bipap mask it is important to suction the mucus and saliva out of the person's throat first. This is because secretions like these build up in the throat while the bipap machine is on, because breathing is stronger than usual but swallowing is weak. If you take the mask off without suctioning first, you might find they struggle a little to breathe through the build-up.
When the person with leukodystrophy is well, the following treatments should be performed twice a day - in the morning and evening. When ill, this should go up to four times a day.
The Therapy
Fifteen minutes or so before treatment begins, give a dose of expectorant cough syrup (see link for a child-friendly expectorant), to help loosen the mucus. Your doctor may also prescribe a nebuliser treatment to do this and/or to dilate the airway tubes in the lungs. If it seems to help, also bring the person into a steamy room, as steam can make mucus easier to loosen.
Use the vibrating vest in blocks of 3-5 minutes with a couple of minutes rest in between blocks. Two or three blocks will probably be enough. This will help fully loosen the mucus. The vest can sometimes irritate a gastrostomy site if used too often. There are two ways around this: One is to place gauze under the button or tube until the vest treatment is over. The other is to alternate between using the vest and doing something called manual percussion. This is done with gentle 'slaps' to the chest, upper back and sides of the rib cage - one hundred 'slaps' to the chest and each side, and 300-500 lighter ones on the back. It is very important that this is done with a cupped hand, or with something like a cup, because it is not the slapping that loosens the mucus, but the vibrations the action produces - called 'percussion'. It should not sound like a slap - it should sound more hollow.
Use the cough assist on blocks of 4-5 coughs each, with about thirty second pauses between blocks. Start with low pressure of air in and out, and gradually build up the pressure over time. Four or five blocks will probably be enough to bring all the mucus up if the pressure is medium-high, but it will be more if the pressure is still low.
Finally suction. The end of the hose goes into the person's throat to bring out the mucus. The doctor, or possibly respite or hospice nurse, will recommend how far in the hose should go. To stop sucking you just need to remove your finger from the small button near the end of the hose.
When it might be particularly useful for the person to use the bipap
- In the car or disability van when travelling
- At night
- When oxygen saturation is low
- When being very active and/or upbeat, it will allow them to do so without lacking energy from low oxygen, or worrying about breathing
N.B. When removing the bipap mask it is important to suction the mucus and saliva out of the person's throat first. This is because secretions like these build up in the throat while the bipap machine is on, because breathing is stronger than usual but swallowing is weak. If you take the mask off without suctioning first, you might find they struggle a little to breathe through the build-up.
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