I have just discovered a new type of monitor manufactured by an Australian company called EC international. I am not sure if it is available in the UK but I'll post about it in case it helps someone. You may be able to contact them and have them supply it - there are no specifications on the website. The monitor is called My Angel iMonitor. The monitor and sensors clips onto a nappy or underwear, and there is a hand held wireless receiver that the carer takes with them that bleeps when an alarm in the monitor goes off. It has multiple functions:
- It can sense abnormal movements and muscles and bleep the carer - so muscle spasms, exercises, pain and seizures can be detected
- It can sense somebody falling and bleep
- It can bleep the carer when it is time to give feeds or medications
- It can sense multiple urinations and defecations and it bleeps when these occur. It can also display the time since the last urinations and defecations (it counts them separately from each other).
- It can allow the person wearing it to call a carer by pressing a button
- It can detect room temperature and bleep the carer when it gets too hot or too cold
- It can read the position of the person when they are lying in bed, which may prevent migration across the bed and falls.
- It can sense if the person wanders off or is abducted.
It doesn't touch the person's skin except possibly for a small piece of the clip that clips onto the nappy or underwear, and the urine and feces sensors are not placed inside the nappy or underwear; they are stuck onto the outside of it.
Here is the link for more information:
http://www.myangelmonitor.com/index.html
Wednesday, 2 December 2009
Monday, 9 November 2009
Effective Infection Control
With winter and swine flu, I thought I'd post a general blog about controlling infections which, aside from apnea, are one of the main causes of death in leukodystrophy patients. You don't need to follow all of these unless there is a very vigorous need to control infections (such as immune system compromise after a bone marrow transplant). I am not a doctor, nurse or transplant advisor or anything - these were just ideas I collected from multiple sources and brought together.
General
- Sterilise all feeding equipment daily
- Give an antibacterial-antiviral such as sambucol, olive leaf extract or eccinacea at the first hint of any infection.
- Wash hands regularly throughout the day, using hot water and an antibacterial soap.
- Wash hands before handling the feeding tube, doing breathing treatments or doing anything near the person's mouth
- Use surgical gloves when dealing with breathing treatments or feeding tube, and wipe the feeding tube site with an antiseptic when changing it.
- Wear a facemask when giving breathing treatments or when dealing with the feeding tube
- Vaccume the floor and disinfect surfaces daily
- Have the person wear a facemask when outside in a crowded area. Give the person a full body scrub and put their clothes for laundry after being in a crowded area. Do the same for yourself.
- Do not allow anyone into your house or near you or the person, who has had an infection in the last week, or who has been in contact with someone who has had an infection in the last week.
- Get the person and yourself seasonal, and any other, flu vaccines
- Clean gastrostomy feeding tube sites in the morning and evening with warm water and TCP.
- If someone who was infected within a week ago, or was associated with someone with an infection in the last week has been in the house, vaccume all carpets, wash all carpetless floors with hot water and soap, wash and dry all curtains and seat covers/throws, disinfect all surfaces and allow the room to air for a day before allowing the person with leukodystrophy back into it.
Urinary Tract Infections
- When changing the nappy, ensure that the entire area covered by the nappy is wiped with baby wipes, and allowed to thoroughly dry before the new nappy is put on.
- Try to induce a bowel movement in the morning by giving a rectal wash out with warm water and glycerine, so that bowel movements come into minimal contact with the front area. This will also help prevent constipation.
- Try to catheterise the bladder four times a day, as this will ensure all the urine drains from the bladder and does not stagnate. However an indwelling catheter may increase the chance of infection by making it easier for infections to enter into the body from outside.
- Give adequate water through the tube to ensure proper hydration and flushing of the kidneys. The person's doctor should be able to recommend a good total volume to aim for.
Eye Infections
- Wash hands thoroughly and wear a face mask and surgical gloves when doing anything with the eyes.
- Use artificial tears to keep eyes moist and prevent fluid stagnating in them.
Ear, nose and throat infections
- Have a look in the ears, nose and throat each day with a small torch. If there is any redness or swelling in the throat, or any waxy build-up in the ears, see a doctor for antibiotics and/or to get ears syringed. Suck out mucus from the nose and throat, if there is any.
Gastrointestinal Infections
- Clean and disinfect cooking surfaces thoroughly before use
- Feed a probiotic such as yakkult or actimel.
- Wear an apron when cooking to prevent germs from getting from your clothes onto your hands and thus into food. Wash and dry apron after every cooking session.
- Ensure everything you use is within its best before, display until and use-by dates, and also smell/view it to ensure it has not gone bad prematurely.
- Do not re-freeze food after defrosting it once. Do not freeze cooked meat at all. In the fridge ensure raw meats are placed on the lowest shelf, so they cannot drip onto raw foods like cheese, pudding and vegetables.
- Use a clean dish towel every time you dry dishes - put each dish towel for the laundry after . Preferably allow them to drip dry.
- When washing up use water that is the hottest it can be, and an antibacterial washing up liquid. Alternatively use a dishwasher but check the dishes are clean and dry upon coming out.
- Do not sneeze, touch your face, cough or yawn near food
- Wash hands in hot water with soap and dry thoroughly before handling food or dishes, or after putting things in the bin.
- Tie back long hair before cooking.
- Wipe up any spillages immediately. After washing up or wiping up, place the dish cloth or sponge in the laundry and use a new one for next time.
Intravenous Sites
- Change the tape/dressing on these every three days at least. Wash hands in hot water and soap, dry them thoroughly and use a face mask and surgical gloves when dealing with an open IV site. Do not touch face. Always wipe site with an antiseptic swab to disinfect before immediately replacing tape/dressing. Keep the IV away from water, or the tape will peel off and site may become contaminated.
Cuts, Grazes and Open Sores
- Wear a surgical mask and gloves when cleaning these sites
- Wash hands thoroughly first with hot water and soap and dry thoroughly. Do not touch face in the process.
- Wash the site with TCP diluted in warm water, using cotton wool.
- Put antiseptic cream on the site, such as savlon
- Cover site with a waterproof sticking plaster, or sterile non-fluffy dressing if needed/preferred.
Lung Infections
- Carry out normal breathing treatments at an increased frequency throughout the day.
General
- Sterilise all feeding equipment daily
- Give an antibacterial-antiviral such as sambucol, olive leaf extract or eccinacea at the first hint of any infection.
- Wash hands regularly throughout the day, using hot water and an antibacterial soap.
- Wash hands before handling the feeding tube, doing breathing treatments or doing anything near the person's mouth
- Use surgical gloves when dealing with breathing treatments or feeding tube, and wipe the feeding tube site with an antiseptic when changing it.
- Wear a facemask when giving breathing treatments or when dealing with the feeding tube
- Vaccume the floor and disinfect surfaces daily
- Have the person wear a facemask when outside in a crowded area. Give the person a full body scrub and put their clothes for laundry after being in a crowded area. Do the same for yourself.
- Do not allow anyone into your house or near you or the person, who has had an infection in the last week, or who has been in contact with someone who has had an infection in the last week.
- Get the person and yourself seasonal, and any other, flu vaccines
- Clean gastrostomy feeding tube sites in the morning and evening with warm water and TCP.
- If someone who was infected within a week ago, or was associated with someone with an infection in the last week has been in the house, vaccume all carpets, wash all carpetless floors with hot water and soap, wash and dry all curtains and seat covers/throws, disinfect all surfaces and allow the room to air for a day before allowing the person with leukodystrophy back into it.
Urinary Tract Infections
- When changing the nappy, ensure that the entire area covered by the nappy is wiped with baby wipes, and allowed to thoroughly dry before the new nappy is put on.
- Try to induce a bowel movement in the morning by giving a rectal wash out with warm water and glycerine, so that bowel movements come into minimal contact with the front area. This will also help prevent constipation.
- Try to catheterise the bladder four times a day, as this will ensure all the urine drains from the bladder and does not stagnate. However an indwelling catheter may increase the chance of infection by making it easier for infections to enter into the body from outside.
- Give adequate water through the tube to ensure proper hydration and flushing of the kidneys. The person's doctor should be able to recommend a good total volume to aim for.
Eye Infections
- Wash hands thoroughly and wear a face mask and surgical gloves when doing anything with the eyes.
- Use artificial tears to keep eyes moist and prevent fluid stagnating in them.
Ear, nose and throat infections
- Have a look in the ears, nose and throat each day with a small torch. If there is any redness or swelling in the throat, or any waxy build-up in the ears, see a doctor for antibiotics and/or to get ears syringed. Suck out mucus from the nose and throat, if there is any.
Gastrointestinal Infections
- Clean and disinfect cooking surfaces thoroughly before use
- Feed a probiotic such as yakkult or actimel.
- Wear an apron when cooking to prevent germs from getting from your clothes onto your hands and thus into food. Wash and dry apron after every cooking session.
- Ensure everything you use is within its best before, display until and use-by dates, and also smell/view it to ensure it has not gone bad prematurely.
- Do not re-freeze food after defrosting it once. Do not freeze cooked meat at all. In the fridge ensure raw meats are placed on the lowest shelf, so they cannot drip onto raw foods like cheese, pudding and vegetables.
- Use a clean dish towel every time you dry dishes - put each dish towel for the laundry after . Preferably allow them to drip dry.
- When washing up use water that is the hottest it can be, and an antibacterial washing up liquid. Alternatively use a dishwasher but check the dishes are clean and dry upon coming out.
- Do not sneeze, touch your face, cough or yawn near food
- Wash hands in hot water with soap and dry thoroughly before handling food or dishes, or after putting things in the bin.
- Tie back long hair before cooking.
- Wipe up any spillages immediately. After washing up or wiping up, place the dish cloth or sponge in the laundry and use a new one for next time.
Intravenous Sites
- Change the tape/dressing on these every three days at least. Wash hands in hot water and soap, dry them thoroughly and use a face mask and surgical gloves when dealing with an open IV site. Do not touch face. Always wipe site with an antiseptic swab to disinfect before immediately replacing tape/dressing. Keep the IV away from water, or the tape will peel off and site may become contaminated.
Cuts, Grazes and Open Sores
- Wear a surgical mask and gloves when cleaning these sites
- Wash hands thoroughly first with hot water and soap and dry thoroughly. Do not touch face in the process.
- Wash the site with TCP diluted in warm water, using cotton wool.
- Put antiseptic cream on the site, such as savlon
- Cover site with a waterproof sticking plaster, or sterile non-fluffy dressing if needed/preferred.
Lung Infections
- Carry out normal breathing treatments at an increased frequency throughout the day.
Tuesday, 27 October 2009
Good Ways to Vary Posture
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
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
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.
Equipment to Keep Airways Clean, Clear and Strong
You should talk to your doctor, and other people experiencing leukodystrophy, about the benefits and costs of this equipment before you do anything suggested here.
As the leukodystrophy progresses, the person affected may begin to find it harder to cough and to clear their throat and nose of mucus. This is a problem, because coughing, clearing the throat, swallowing and blowing mucus out the nose is the only way the airway can stay bacteria free. Therefore being unable to do these things often enough can lead to pneumonia. There are four things that will provide the best quality lung care. The NHS may feel it is unnecessary to use all of these, but of the accounts of leukodystrophy I have read, the people that did survived the longest and had the fewest infections.
- Vibrating vest: Loosens mucus in the lungs, so it can easily be brought up
- Cough assist machine: Brings up the loosened mucus to where it can be sucked out
- Suction machine: Because people with leukodystrophy can't swallow well, mucus must
be brought out by suction instead
- Nebuliser and face mask: This is a machine that turns breathing medicines into a fine mist that flows into the face mask. The person breathes the mist into their lungs, where the medicine takes effect.
- Oxygen cylinder, hose and face mask: An oxygen cylinder holds liquid oxygen which is delivered as a gas through a tap at the top. You can attach a thin rubber hose to this to carry the oxygen into the face mask, which the person wears and breathes in the oxygen. This should not be used in excess, the reason being explained in the next paragraph, but it is useful if oxygen levels are low, and a top-up is needed quickly.
- Bipap machine, nasal mask with head gear, and air hose: The bipap machine is a noninvasive form of ventilation that need not completely replace breathing; the person can breathe on their own and just be assisted by the machine. Because it is noninvasive, there is no trauma from surgery, and no risk of infecting an open wound site. The machine both pushes air into the lungs and helps draw it out again. Most bipap machines have computers that can figure out how much it needs to help the person to breathe - so it doesn't do more than is needed. Extra oxygen can be used with a bipap machine, but usually it just uses air. This is because when you give extra oxygen the body thinks it doesn't need to breathe. When this happens carbon dioxide builds up and causes problems, such as difficulty sleeping. The bipap machine is very easily portable in a wheelchair and car, so does not restrict quality of life. I would recommend a nasal mask, rather than a full face mask to people with leukodystrophy, because these people tend to dribble saliva, which could pool in a mask that covers the whole face. From a lung point of view it helps avoid the lungs getting deflated and forming puddles of moisture which could grow bacteria.
It is important to get these pieces of equipment as early as possible, because it allows for time to gradually get used to the breathing regime before it needs to become rigorous. It also means that there is no window in which therapy is needed but equipment is being waited on, when an infection may slip in and begin to grow. One last reason - some doctors believe that once a person's terminal illness has progressed enough, preventative treatment is 'not worth it'. Right at the very end of some terminal illnesses, such as certain types of brain tumour that cause loss of awareness and coma, this may be true: Treatment would do nothing to alter the course of illness, alleviate suffering or prolong life. But with leukodystrophy, life can be prolonged and suffering alleviated, because in many cases it is only movement that is affected - not intellect, consciousness or ability to feel pain and discomfort. The quality of life of the person with leukodystrophy can also be greatly enhanced by parents, carers, friends and family.
It is also important later on to get monitors for times such as in the car or at night.
- Baby monitor, preferably with monitoring screen as well as speaker: Lets you hear and see the person at night, and amplifies the sound of any distress or alarms.
- Apnoea alarm: This detects breathing movement, or lack of it, and sounds a loud alarm, which allows the carer or parent to come to the person's rescue. There are apnoea alarms available which will also sound if a seizure is detected
- Pulse-ox monitor: This is a device which is fitted over one finger, and displays oxygen saturation (100% in those with full breathing function) and pulse rate. It can be used all the time, or just to check breathing and heart each day, or if the person looks ill or breathing sounds bad.
LINKS
BIPAP Machine
http://bipapvision.respironics.com/ = non invasive breathing assistance machine
Vibrating Vest
The Vest® Airway Clearance System = vibrating vest to help loosen mucus
Cough Assist
Features, CoughAssist, Philips Respironics = cough assist to help bring up mucus
Suction Machine
AW Suction Pump Mini Aspirator : Beaucare Medical = suction machine to extract mucus
Child’s Nebuliser
http://www.nebulizermask.com/ = child friendly nebulisers and masks for breathing treatments when congested or tight in the chest
As the leukodystrophy progresses, the person affected may begin to find it harder to cough and to clear their throat and nose of mucus. This is a problem, because coughing, clearing the throat, swallowing and blowing mucus out the nose is the only way the airway can stay bacteria free. Therefore being unable to do these things often enough can lead to pneumonia. There are four things that will provide the best quality lung care. The NHS may feel it is unnecessary to use all of these, but of the accounts of leukodystrophy I have read, the people that did survived the longest and had the fewest infections.
- Vibrating vest: Loosens mucus in the lungs, so it can easily be brought up
- Cough assist machine: Brings up the loosened mucus to where it can be sucked out
- Suction machine: Because people with leukodystrophy can't swallow well, mucus must
be brought out by suction instead
- Nebuliser and face mask: This is a machine that turns breathing medicines into a fine mist that flows into the face mask. The person breathes the mist into their lungs, where the medicine takes effect.
- Oxygen cylinder, hose and face mask: An oxygen cylinder holds liquid oxygen which is delivered as a gas through a tap at the top. You can attach a thin rubber hose to this to carry the oxygen into the face mask, which the person wears and breathes in the oxygen. This should not be used in excess, the reason being explained in the next paragraph, but it is useful if oxygen levels are low, and a top-up is needed quickly.
- Bipap machine, nasal mask with head gear, and air hose: The bipap machine is a noninvasive form of ventilation that need not completely replace breathing; the person can breathe on their own and just be assisted by the machine. Because it is noninvasive, there is no trauma from surgery, and no risk of infecting an open wound site. The machine both pushes air into the lungs and helps draw it out again. Most bipap machines have computers that can figure out how much it needs to help the person to breathe - so it doesn't do more than is needed. Extra oxygen can be used with a bipap machine, but usually it just uses air. This is because when you give extra oxygen the body thinks it doesn't need to breathe. When this happens carbon dioxide builds up and causes problems, such as difficulty sleeping. The bipap machine is very easily portable in a wheelchair and car, so does not restrict quality of life. I would recommend a nasal mask, rather than a full face mask to people with leukodystrophy, because these people tend to dribble saliva, which could pool in a mask that covers the whole face. From a lung point of view it helps avoid the lungs getting deflated and forming puddles of moisture which could grow bacteria.
It is important to get these pieces of equipment as early as possible, because it allows for time to gradually get used to the breathing regime before it needs to become rigorous. It also means that there is no window in which therapy is needed but equipment is being waited on, when an infection may slip in and begin to grow. One last reason - some doctors believe that once a person's terminal illness has progressed enough, preventative treatment is 'not worth it'. Right at the very end of some terminal illnesses, such as certain types of brain tumour that cause loss of awareness and coma, this may be true: Treatment would do nothing to alter the course of illness, alleviate suffering or prolong life. But with leukodystrophy, life can be prolonged and suffering alleviated, because in many cases it is only movement that is affected - not intellect, consciousness or ability to feel pain and discomfort. The quality of life of the person with leukodystrophy can also be greatly enhanced by parents, carers, friends and family.
It is also important later on to get monitors for times such as in the car or at night.
- Baby monitor, preferably with monitoring screen as well as speaker: Lets you hear and see the person at night, and amplifies the sound of any distress or alarms.
- Apnoea alarm: This detects breathing movement, or lack of it, and sounds a loud alarm, which allows the carer or parent to come to the person's rescue. There are apnoea alarms available which will also sound if a seizure is detected
- Pulse-ox monitor: This is a device which is fitted over one finger, and displays oxygen saturation (100% in those with full breathing function) and pulse rate. It can be used all the time, or just to check breathing and heart each day, or if the person looks ill or breathing sounds bad.
LINKS
BIPAP Machine
http://bipapvision.respironics.com/ = non invasive breathing assistance machine
Vibrating Vest
The Vest® Airway Clearance System = vibrating vest to help loosen mucus
Cough Assist
Features, CoughAssist, Philips Respironics = cough assist to help bring up mucus
Suction Machine
AW Suction Pump Mini Aspirator : Beaucare Medical = suction machine to extract mucus
Child’s Nebuliser
http://www.nebulizermask.com/ = child friendly nebulisers and masks for breathing treatments when congested or tight in the chest
Thursday, 13 August 2009
How to Change a Mic-Key Button, Vent, Flush and Clean a Gastrostomy Site
Changing the Tube
A mic-key button is a gastrostomy tube that rests against the skin, and is held inside the stomach by a balloon on the inside inflated with water. It can easily be changed at home by a parent or carer.
www.mic-key.com
Again, talk to your doctor about how often to change the button, how much water to inflate the balloon with, and get a nurse to show you before you try it yourself.
A mic-key button is a gastrostomy tube that rests against the skin, and is held inside the stomach by a balloon on the inside inflated with water. It can easily be changed at home by a parent or carer.
www.mic-key.com
Again, talk to your doctor about how often to change the button, how much water to inflate the balloon with, and get a nurse to show you before you try it yourself.
- New Mic key button feeding tube and extension tube
- 2 x 10ml syringes
- 1 plastic cup
- (Surgical tape)
- Surgical gloves
- lubricant that is not poisonous when eaten
- Antiseptic wipes
1. Insert the syringe into the side-channel of the tube and inflate the balloon. Check there are no dents or leaks. Withdraw the water from the balloon again.
2. Insert the syringe into the side-channel of the tube currently in place and withdraw the water. Discard the old water into the plastic cup.
3. Gently but quickly remove the old button from the site and discard water, button and used syringe. Clean again around the area with an antiseptic wipe
4. Cover the end of the new button in lubricant. Plop the button quickly but carefully all the way into the hole in the stomach.
6. Inflate the balloon. Gently tug on the button to check for resistance – it should feel like it’s firmly in place, but do not tug more than is needed to feel a slight resistance.
7. If desired, lock the extension tube into place on the button and tape down to skin to prevent tugging. If this is not wanted, leave the tube as it is, without gauze, allowing for better breathability and less skin growth (or granulosa tissue) around the button.
8. If the extension tube is left in place, take the 10ml syringe and suck a little stomach contents up to the top of the tube to get rid of any air. Then administer water through the extension tube, clearing it of stomach contents and keeping air bubbles out. Close off the end of the extension tube.
Venting and Flushing
This is usually done just after a tube, six times a day if on a continual drip feed, and at least every eight hours when not in use. It prevents air being trapped in the stomach, and the tube getting clogged. You will need:
- One 60ml syringe
- About 30ml of water
- Extension tube
Pull the plunger of the 60ml syringe up to the 10ml mark - this prevents any stomach contents from remaining in the tube afterwards. Attach the extension tube and put the syringe nozzle into the valve. Draw contents of stomach slowly up the tube to about 30ml. If just loose bubbles appear, keep drawing the plunger up all the way to the top, removing the syringe, discarding the air, pushing the plunger back to the 10ml mark, reattaching it to the tube and pulling up slowly until liquid begins to appear. Once liquid appears, pull up very slowly to about 30ml. When you let go of the tube and the plunger slips back down on its own, this shows the stomach is empty. Return the liquid to the stomach, detach the syringe from the tube, fill the syringe to about 30ml of water, reattach it to the tube and flush the water down the tube into the stomach. Then detach the syringe from the extension tube, and finally, if required, detach the extension tube.
Cleaning the Tube
This should be done at least twice a day. You will need:
- a bowl
- some TCP
- some warm water
- some cotton wool balls
Wash hands.
Fill the bowl with warm water and a few ml of TCP. Soak a cotton wool ball in the water, and clean around the tube until any liquid or dirt is gone. Dispose of the water
Move the tube up and down to stop the balloon from sticking to the stomach. Rotate the tube 360 degrees to stop it sticking to the tunnel in to the stomach.
Pat dry
How to Change a Nasogastric Tube
Before you try this, get a nurse to show you as many times as you need to feel comfortable. Your doctor should tell you how often the tube needs to be changed.
You will need:
- lubricant that is not poisonous to eat
- a tube
- pH paper (paper that changes different colours, depending on how acid a liquid is)
- 10ml syringe
- a large bowl in case the person vomits
- surgical tape
Measure on the person the length of tube you need to insert. Feel for the person's ribs. At the bottom of them, just between the two, is roughly over the stomach. Get a nurse to show you. Place the end of the tube with no valve here, and hold it in place with one hand. With the other, take the rest of the tube straight up until it touches the nose. Mark this spot with a piece of tape - this is how far the tube will be inserted. Then from the nose, take the rest of the tube across the cheek and behind the ear.
Now that you have measured you can begin. Wind the tube tightly around your index finger a few times so that it has a downward curve. Then lubricate the end. So the passage of the tube is as straight as possible, tilt the person's chin upward slightly. Begin to thread the tube up the nostril, with the downward curve pointing down. When the tube reaches the throat the person may gag. This is alright - wait until they stop and then continue. But if they cough, pull the tube out about an inch and try again. Once up to the mark, stop and tape the tube to the cheek. Using the 10ml syringe draw some fluid up the tube and squeeze a drop onto the pH paper. If the pH is very low - usually red - the tube is in the right place. If it's not, you need to remove it at once, in case you've accidentally put it into a lung. Once it's in place you can start liquid feeds straight away.
You will need:
- lubricant that is not poisonous to eat
- a tube
- pH paper (paper that changes different colours, depending on how acid a liquid is)
- 10ml syringe
- a large bowl in case the person vomits
- surgical tape
Measure on the person the length of tube you need to insert. Feel for the person's ribs. At the bottom of them, just between the two, is roughly over the stomach. Get a nurse to show you. Place the end of the tube with no valve here, and hold it in place with one hand. With the other, take the rest of the tube straight up until it touches the nose. Mark this spot with a piece of tape - this is how far the tube will be inserted. Then from the nose, take the rest of the tube across the cheek and behind the ear.
Now that you have measured you can begin. Wind the tube tightly around your index finger a few times so that it has a downward curve. Then lubricate the end. So the passage of the tube is as straight as possible, tilt the person's chin upward slightly. Begin to thread the tube up the nostril, with the downward curve pointing down. When the tube reaches the throat the person may gag. This is alright - wait until they stop and then continue. But if they cough, pull the tube out about an inch and try again. Once up to the mark, stop and tape the tube to the cheek. Using the 10ml syringe draw some fluid up the tube and squeeze a drop onto the pH paper. If the pH is very low - usually red - the tube is in the right place. If it's not, you need to remove it at once, in case you've accidentally put it into a lung. Once it's in place you can start liquid feeds straight away.
Preventing Pneumonia by Avoiding Vomiting
For any person diagnosed with leukodystrophy, some of the main problems they will probably eventually experience are:
- Inability to swallow
- Pneumonia
- Choking on saliva
- Vomiting
Inability to swallow and choking are linked because kids with leukodystrophy, as they lose their ability to swallow, may choke on saliva, or on vomit. Vomiting, choking and pneumonia are all linked also, due to the fact that saliva or vomit going into the lungs can increase the risk of bacteria growing. For this reason it's important to minimise the risk of vomiting and choking. I am not a specialist: All information is information I have come across from personal experience, as well as parents and carers of people with leukodystrophy and similar disorders.
Feeding, or at least the majority of it, can be done by giving liquid food through a nasogastric tube or a gastrostomy tube.
http://en.wikipedia.org/wiki/Feeding_tube
A nasogastric tube is a long thin hollow silicon tube that is posted up a nostril, down the throat and into the stomach, and held in place with a piece of tape on the cheek or the nose. A gastrostomy tube is a hollow flexible tube that is placed surgically, and connects from outside to inside the stomach. It is usually held in place by a balloon on the inside and a plastic stopper on the outside. Both kinds of feeding tube have to be changed regularly - Mum, Dad or carer can learn to do this for nasogastric tubes, and some kinds of gastrostomy tube. Liquid food is usually placed in a hanging bag, and dripped or pumped through an extension tube into the stomach via the feeding tube. Suitable liquid food may be brands such as ensure, pediasure. Tube feeding reduces risk of vomiting because foods don't have to go into the mouth, where there is a risk they'll be breathed in. Foods can be tube fed in bolus amounts (this means in seperate feeds, like breakfast, lunch and supper), or they can be dripped slowly in all through the day and/or night.
If a gastrostomy tube is placed, there is the option of also performing surgery called a nissen fundoplication - which again reduces the risk of vomiting. This is where a part of the stomach is wrapped around the base of the oesophagus (the passage leading to the entrance to the stomach) and held in place with a stitch. This makes it harder for stomach contents to come back up.
http://en.wikipedia.org/wiki/Nissen_fundoplication
Both gastrostomy and nissen can be done at the same time, although both are major surgery (even though they are performed routinely). You should discuss the benefits and disadvantages with a doctor. Usually it is better to do these surgeries early on, when the child still has good movement, breathes strongly, is not choking or vomiting much, and can still cough. If vomiting still carries on, there are medications that can be put through the tube, such as gaviscon, which is available over the counter in the UK, US and Canada. Powdered ginger is also known to target and block the nausea centres of the brain, preventing queasiness. There are also prescribed medicines that prevent nausea and vomiting, and decrease the amount of acid produced in the stomach. But it is always best to talk to the person's doctor first.
http://www.patient-pharmacy.co.uk/details.asp?productid=GACL30
If the person still vomits, even after a nissen and medication, it might be worth changing the kind of liquid food they are taking. There is a brand available called peptamen, or peptamen junior for children. This is very, very easy to digest as it is specially designed for people who have gastric problems. Some people with leukodystrophy find this easier to deal with than the more commonly used brands such as ensure plus or pediasure.
http://www.nestlenutrition.co.uk/healthcare/gb/news/Pages/PeptamenHN.aspx
http://www.nestlenutrition.co.uk/healthcare/gb/news/Pages/PeptamenJrLiquid.aspx
There is also a formula called vivonex, which is low fat and comprised of free amino acids instead of whole protein. This can also be easier to digest, and is apparently well tolerated if the person being fed is experiencing a lot of physical stresses. The link for vivonex is below:
http://www.nestle-nutrition.com/products/Family.aspx?FamilyId=345ae4e7-5dd3-4a5e-9abb-3eca340c3c3a
Both are manufactured by nestle nutrition. A dietician should ideally be consulted before changing the person's diet or adding anything to it.
If the person still vomits despite a nissen, medication, changed liquid feed, changing from bolus to continual very slow drip feed may help, or, if this is already how the person is done, reducing the speed of flow still further. If all of this fails, there is another type of tube called a gastrojejunal tube. This goes through the same hole made for the stomach tube, but is threaded down into the small bowel. Food goes into the bowel, and medicine goes into the stomach. That means the stomach is always pretty much empty, so there is nothing to vomit up. Feeding with this tube is always continuous drip feeding.
http://www.bcchildrens.ca/Services/SurgeryAndSurgSuites/GeneralSurgery/TubeFeeding/TypesOfTubes/GJFeedingTube.htm
The next blog will be about care of a gastrostomy tube and how to place a nasogastric tube, and then we will move onto another topic of importance: Preventing pneumonia by making sure mucus doesn't linger in the lungs.
- Inability to swallow
- Pneumonia
- Choking on saliva
- Vomiting
Inability to swallow and choking are linked because kids with leukodystrophy, as they lose their ability to swallow, may choke on saliva, or on vomit. Vomiting, choking and pneumonia are all linked also, due to the fact that saliva or vomit going into the lungs can increase the risk of bacteria growing. For this reason it's important to minimise the risk of vomiting and choking. I am not a specialist: All information is information I have come across from personal experience, as well as parents and carers of people with leukodystrophy and similar disorders.
Feeding, or at least the majority of it, can be done by giving liquid food through a nasogastric tube or a gastrostomy tube.
http://en.wikipedia.org/wiki/Feeding_tube
A nasogastric tube is a long thin hollow silicon tube that is posted up a nostril, down the throat and into the stomach, and held in place with a piece of tape on the cheek or the nose. A gastrostomy tube is a hollow flexible tube that is placed surgically, and connects from outside to inside the stomach. It is usually held in place by a balloon on the inside and a plastic stopper on the outside. Both kinds of feeding tube have to be changed regularly - Mum, Dad or carer can learn to do this for nasogastric tubes, and some kinds of gastrostomy tube. Liquid food is usually placed in a hanging bag, and dripped or pumped through an extension tube into the stomach via the feeding tube. Suitable liquid food may be brands such as ensure, pediasure. Tube feeding reduces risk of vomiting because foods don't have to go into the mouth, where there is a risk they'll be breathed in. Foods can be tube fed in bolus amounts (this means in seperate feeds, like breakfast, lunch and supper), or they can be dripped slowly in all through the day and/or night.
If a gastrostomy tube is placed, there is the option of also performing surgery called a nissen fundoplication - which again reduces the risk of vomiting. This is where a part of the stomach is wrapped around the base of the oesophagus (the passage leading to the entrance to the stomach) and held in place with a stitch. This makes it harder for stomach contents to come back up.
http://en.wikipedia.org/wiki/Nissen_fundoplication
Both gastrostomy and nissen can be done at the same time, although both are major surgery (even though they are performed routinely). You should discuss the benefits and disadvantages with a doctor. Usually it is better to do these surgeries early on, when the child still has good movement, breathes strongly, is not choking or vomiting much, and can still cough. If vomiting still carries on, there are medications that can be put through the tube, such as gaviscon, which is available over the counter in the UK, US and Canada. Powdered ginger is also known to target and block the nausea centres of the brain, preventing queasiness. There are also prescribed medicines that prevent nausea and vomiting, and decrease the amount of acid produced in the stomach. But it is always best to talk to the person's doctor first.
http://www.patient-pharmacy.co.uk/details.asp?productid=GACL30
If the person still vomits, even after a nissen and medication, it might be worth changing the kind of liquid food they are taking. There is a brand available called peptamen, or peptamen junior for children. This is very, very easy to digest as it is specially designed for people who have gastric problems. Some people with leukodystrophy find this easier to deal with than the more commonly used brands such as ensure plus or pediasure.
http://www.nestlenutrition.co.uk/healthcare/gb/news/Pages/PeptamenHN.aspx
http://www.nestlenutrition.co.uk/healthcare/gb/news/Pages/PeptamenJrLiquid.aspx
There is also a formula called vivonex, which is low fat and comprised of free amino acids instead of whole protein. This can also be easier to digest, and is apparently well tolerated if the person being fed is experiencing a lot of physical stresses. The link for vivonex is below:
http://www.nestle-nutrition.com/products/Family.aspx?FamilyId=345ae4e7-5dd3-4a5e-9abb-3eca340c3c3a
Both are manufactured by nestle nutrition. A dietician should ideally be consulted before changing the person's diet or adding anything to it.
If the person still vomits despite a nissen, medication, changed liquid feed, changing from bolus to continual very slow drip feed may help, or, if this is already how the person is done, reducing the speed of flow still further. If all of this fails, there is another type of tube called a gastrojejunal tube. This goes through the same hole made for the stomach tube, but is threaded down into the small bowel. Food goes into the bowel, and medicine goes into the stomach. That means the stomach is always pretty much empty, so there is nothing to vomit up. Feeding with this tube is always continuous drip feeding.
http://www.bcchildrens.ca/Services/SurgeryAndSurgSuites/GeneralSurgery/TubeFeeding/TypesOfTubes/GJFeedingTube.htm
The next blog will be about care of a gastrostomy tube and how to place a nasogastric tube, and then we will move onto another topic of importance: Preventing pneumonia by making sure mucus doesn't linger in the lungs.
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