Tuesday, 28 June 2011

More Equipment Links

Sorry it has been a while - I wanted to update with some new information. I have discovered the names of a couple of pieces of equipment I had heard of in passing, but could not investigate due to knowing nothing about it and therefore having no search terms. Most of my information is serendipitous in this way.

Farrell Bag
This is used to prevent reflux when a person is being tube fed. Reflux is when acid, stomach gas and contents flow back up into the throat, often causing pain and a risk of aspiration (food entering the lungs), which in turn can be a pneumonia risk. Think of it a bit like a full carton of juice, with the straw stuck in the top. Reflux is like what happens when you squeeze the carton and juice comes up the straw. Farrell bags help reduce this, by providing an place other than the throat for the contents to flow into, i.e. like providing a more desirable straw for the juice to flow into.

The bag is connected to a tube, which has several valves which clip onto the person's feeding tube extension. During a feeding, air and stomach contents that would have refluxed end up going into this bag. The air is released through a valve in the top of the bag, and when the http://www.blogger.com/img/blank.gifstomach is empty enough, the refluxed food flows slowly back into the stomach, there to be digested. Because it is a closed system it is hygeinic, and one bag can be used, unrefridgerated, for twenty four hours. They can be used with G-tubes (tubes going straight into the stomach), or GJ tubes (tubes with one part in the stomach and one part in the small intestine. They can be used with IV-pole style tube feeding, or pump-in-a-backpack style feeding.

If your loved one with leukodystrophy is having trouble with reflux or vomiting, it might be worth asking your consultant about using farrell bags to help with this. If these are cleared with your doctor you will probably be provided with them, and tuition in using them. If you need a reminder, having gone down this route, there is a very good youtube video at: http://www.youtube.com/watch?v=2yi9K0ENrIM

Hensinger Head Support

I have been trying to find out more about this head support for some time now, and only recently stumbled on the name of it. It provides full head support when sitting up, using points on the chin and jaw to prop the head up. Unlike travel neck pillows, it is fastened at the front, making it much harder for it to slip or for the head to flop forwards when it is being worn. It is also thicker than most travel horse-shoe pillows, meaning that better support is provided if the person has a longer neck. They don't seem to be available in the UK without being imported from the US, but I will include a US link:


E-Z-on harness

This is an essential piece of equipment if your loved one with leukodystrophy is child-sized and travels lying down across the back seat of a car. It is a harness specially adapted to this purpose.http://www.blogger.com/img/blank.gif

This is a link to a UK page which describes the harness more fully and shows a cartoon drawing of the harness in use:


For illustrative purposes here is a link to a photo of one of the E-Z-on harness types on the E-Z-on website:

If your doctor or physiotherapist thinks your loved one with leukodystrophy may breath better (less stomach compression and improved drainage of saliva and mucus out the mouth onto a towel or muslin square), have better posture (less pressure on the back so possibly less likelihood of aggravating scoliosis) or have better bladder and bowel function (less compression which might possibly make emptying easier), they might recommend your loved one with leukodystrophy is kept horizontal. If so, you may choose to use a harness and have them lie on a mattress across the back seat of the car.

If your loved one with leukodystrophy needs to be kept horizontal, the physiotherapist might well decide a wheelchair with tilt and recline function might be required too, possibly with a reversible seat so that you can keep your eye on their facial expressions and movements, and hear any sounds they make to communicate better. If your wheelchair services can't provide or recommend such a chair then it may be worth doing some research yourself, and then pushing for the funding (and/or assistance) to obtain one. To name drop, some suitable strollers for children that might - and I only say might as I am not a physiotherapist or specialist - be suitable: JazzeasyS, Tom Cross Country Stroller, Ormesa New Bug.

Here is a UK link where you can see photos, read about features and gain an estimated cost of each of these strollers: http://www.livingmadeeasy.org.uk/featurescompare.php?groupid=2776

Wednesday, 1 September 2010

September - Leukodystrophy Awareness Month

The colour for raising leukodystrophy awareness is apparently navy blue so if we could all wear something of that colour that might encourage people to ask questions, thereby giving us a way to broach the subject. Find out if any places in the area e.g. schools, hospitals etc. will let you speak publicly about the leukodystrophies or put out information. Of course, these are just two ideas!

Wednesday, 28 July 2010

More Useful Charity Links

Angel Flights:

This is a small American charity that, in some parts of the USA (mostly the heartland), gives free private flights for seriously ill patients, usually children. This would be in a situation where, for instance, the patient needs to travel long distances for medical treatment, and it would be too slow/uncomfortable for them to travel on land, and public air flight is not practical/possible/feasible. It is run entirely by volunteers.


Songs of love:

This is run by a group of professional singer/song writers. They write special, personalised, loving songs for children of up to 21 years old facing life limiting or life threatening physical, emotional or medical problems. This is to help the child recover/cope or just to give an emotional lift and some encouragement. The style, vocals, tune and lyrics are all personal to the child in question.



This is a website where people facing serious/complex medical challenges can easily keep select friends and family up to date about their condition. You can create your own web page for free, giving it its own design, and name. You can upload about 60 photos, and you have a guest book that visitors to the page can sign. There is a space for you to write about your life/hobbies/personality/ etc. and the process leading up to diagnosis, along with medical history. There is also a space where you can post links/resources, and another space where you can post medical/life updates/thoughts in the form of a blog.


Torbay Holiday Helpers Network:

This is a British network of hotels and resorts that offer free holiday stays to families with children who are seriously/terminally ill or who have been recently bereaved.


You may have heard of Make-A-Wish in America. This is the British version. The charity grants wishes to seriously or terminally ill children. Such wishes might include objects of desire (e.g. swing set), holiday, extra special activity (e.g. swimming with dolphins), meeting a revered celebrity, or fulfilling an ambition (e.g. to join the fire fighters).


Ronald McDonald House:
This is free accommodation near hospitals throughout the UK, for families with children facing hospital treatment. It aims to create a home from home environment, and as such part of this involves parents of children staying there sharing small ‘normal’ household tasks.



An archive (frequently updated) of cheerful, innocent, fun, loving and uplifting songs that can be loved by adults and children alike.


Tuesday, 13 July 2010

Another Link - Samuel's Formula

This is a link about tube feeding formula. It is about a family with a kid called Sam, who was fed through a G-tube due to heart problems. His parents were very uneasy about feeding Sam commercial formula as it is basically just water, corn syrup and fortified milk powder. Therefore they came up with their own formula which tastes good, runs smoothly through tube feeding equipment, and is nutirionally complete and optimal. They have a website:


On the website is a detailed, and I mean detailed(!), video narrated by Sam's father about how, and why, they came up with this formula, as well as how to make your own variations of this. He also talks about why each ingredient is used and Samuel's own story, and, most importantly, he teaches you how to experiment safely until you find the nutritious and tasty formula that works for your loved one with leukodystrophy.

If green goo or power juice (both described earlier in this blog) do not work well for your loved one with leukodystrophy, or you want something to supplement them, then, as always, talk to a nutritionist or other specialist (as I'm not one!) and mabye this formula or a variation will be suitable.



Full List of Ingredients
Organic pork chops
Organic pork liver
Organic banana
Organic eggs
Organic rewer’s yeast
NOW full spectrum minerals
Organic coconut oil
Organic wheat germ oil
Organic flax seed oil
Organic strawberries
Organic honey
Organic cod liver oil
Grape seed antioxidant
Optibac probiotic
Organic cranberries
Organic bio yoghurt natural flavour
Organic butter
Organic vinegar
Spring or tap water
Acerola berry juice


1. Cook Broth

Pork chops (low in thiols - sulphur containing chemicals that may aggravate some forms of leukodystrophy)
2 pints (4 cups) natural mineral water
2 tablespoons organic vinegar

Cut off the wings and remove the neck, fat glands and the gizzards from the cavity. Cut chicken parts into several pieces, especially neck and chicken wings. Place chicken or chicken pieces in a large stainless steel pot with water, let stand 30 minutes to 1 hour. Bring to a boil, and remove scum that rises to the top. Reduce heat, cover and simmer for up to 36 hours. Remove carcass. Let cool and remove chicken meat from the carcass, saving for other purposes. Strain the stock into a large bowl and reserve in your refrigerator until the fat rises to the top and congeals. Skim off this fat and reserve the stock.

2. Cook Liver

4 oz pork liver (again low in thiols)
Organic butter

Saute quickly in butter.

3. Combine Liver and Broth

Blend cooked liver with 1 pint broth and combine with the rest of the broth. Freeze into separate half pint portions until ready to use in formula.

4. Soft Boil 3 organic Eggs

5. Extract the yolks and discard whites

6. Blend the yolks with 1 banana

7. Freeze in a small container until ready to use in formula

8. Repeat until 12 portions of egg-yolk/banana are made

Making the Formula Each Day

9. Get a frozen portion of liver/broth mix and mix in a frozen portion of egg/banana mix

10. Stir in 1 tablespoon nutritional yeast, 1 tablespoon coconut oil, 1 tablespoon flax seed oil, 1 teaspoon cod liver oil, 2 teaspoons wheat germ oil, 1 teaspoon acerola juice, half a crushed NOW full spectrum mineral tablet, one grape seed antioxidant tablet, some optibac and one sachet of fybogel

11. Blend

12. Stir in bio yoghurt – 1/2 a pint

13. Cut strawberries in half and add with cranberries and 2 tablespoons of honey. Blend

Sunday, 4 July 2010

Beckman Oral Motor Therapy

I owe so much to Brad Fisher, father to five year old Shira Fisher (SMA type 1, which also causes progressive movement loss) in terms of information for this site. He and his wife Maxine are parents that had to advocate and lobby and bully the stubborn health officials to get what Shira needed, and have largely created their own (very effective) care plan for Shira. Brad has posted so many videos about how he cares for Shira, and they have also put together Shira's website with many care links:


He has posted videoes on Youtube of Shira's Beckman Oral Motor Therapy routine. Since it is difficult to describe I am going to link you first to the Youtube videos of the therapy Brad and Shira do, and then (hopefully) to the page on his site where you can read the brochure describing the exercises.

As I mentioned before, Beckman Oral Motor Therapy keeps the jaw and lips loose and supple, which is important for keeping as much movement as possible. Movement is important as it might help communication to some extent, teeth brushing, suctioning, smiling and many other functions.

Here is the link to the first Youtube video (you should be able to access the others through the suggested thumbnail videos to the right of the main one):


The second link is the link to the diagrams for the Beckman Oral Motor Therapy that are posted in PDF format on Shira's website.


I know the diagrams may be hard to follow as they aren't that clear, but printing it out should make it clearer. It is also worth looking at the care page the Fishers have compiled as there is a wealth of links and information provided about so many important areas and there are bound to be important points that I have not heard of or forgotten to mention in the blog.


And last, as always, a speech therapist, physiotherapist or GP is (or should be) able to provide more crucial information and recommendations, and it is always best to consult a specialist before adopting these kinds of regimes.

Wednesday, 31 March 2010

Superior Disinfectants and Books about Leukodystrophy

These are some links to disinfectants that are used in the lab that may be of use to people with leukodystrophy, particularly in the winter time. Many people with leukodystrophy have to avoid infections (rife in winter, as we all know!), as the outcome could be catastrophic.

The below is a website that makes a range of products called 'Biocleanse' - which is proven effective against MRSA amongst other things. Their range includes wipes, disinfectant specifically for cleaning things like cough assists, bipap machines and suction machines - but on the website under 'application guide' it tells you what each product is best used for. I think you may have to contact them to get prices and information on ordering. The website is UK based.


The next link is to a disinfectant against viruses. It is marketed towards laboratories and livestock, but is effective against 18 viruses known to man and animals and might help some people with leukodystrophy to stay infection free in the winter.


The next link is the link to some books that are sold on Amazon about myelin. As can be seen many of them are pretty expensive, but at the least the link will give you names of books you can possibly find in a hospital library. The may also be good birthday or Christmas presents, or the money could be raised by fundraising.


There don't seem to be many reader-friendly books on leukodystrophy, which is not surprising, even if it is disappointing. However, the closest scientific ones that may help are linked here. They are mainly internet guides to sourcing more information:

Leukodystrophy - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References

The Official Parent's Sourcebook on Leukodystrophy: A Revised and Updated Directory for the Internet Age

This one is specifically for Metachromatic leukodystrophy:
The Official Parent's Sourcebook on Metachromatic Leukodystrophy: A Revised and Updated Directory for the Internet Age (Paperback)

This next book is not about leukodystrophy but if you ever have to advocate to get your loved one with leukodystrophy the equipment, care and (legal) support they need whether in hospital or out of it, this is apparently an invaluable book:

How Doctors Think

Finally, some moving, honest and unsentimental accounts from parents of caring for children with leukodystrophy:

"Without a Word" - Jill Kelly, founder of Hunter's Hope. A memoire of her time with her son Hunter, who had Krabbes leukodystrophy

"Eyes That See" - Christina Levasheff - parent of Judson Levasheff who had Krabbes leukodystrophy

I also hear that Augusto Odone plans to write a book to commemorate his son, Lorenzo, who died from aspiration pneumonia as an indirect effect of adrenoleukodystrophy. Augusto founded the Myelin project and he and his late wife Michaela concocted "Lorenzo's Oil" - a blend of 4 parts Triglyceride monounsaturated oleic acid and 1 part Triglyceride monounsaturated erucic acid that prevents the body making the very long fats that build up in ALD. Links to the myelin project are in my blog entry "Useful Organisations".

Thursday, 18 March 2010

More Links to Equipment, and Some to Experimental Treatments


For anyone in the UK, the link above goes to a fantastic website aimed at giving a range of suggestions on how to make life easier and richer for a child with a disability. From things like fun at home to days out and holidays, as well as a brilliant page about equipment types. It gives the names, photos and sometimes links as well to different pieces of equipment. The tiltrite chair, portable hoists and moulded mattresses designed to help with a twisted posture particularly spring to mind.

Here are some links to more experimental treatments I have read about. Some of these may be dated but appeared to produce small improvements in the child. These reports were purely anecdotal, and so I can't validate or invalidate them. I am also not a doctor, and doctors would be more up to date about where this research has led, or at least point you in the right direction to somebody who would know.

Clorazepate - This drug belongs to a group of drugs called the benzodiazepines, and has been used experimentally in the treatment of metachromatic leukodystrophy. Its brand names are tranxene and novo-clopate, and it is also known as clorazepate dipotassium. It is a skeletal muscle relaxant, meaning it can make a person relaxed and supple. It is also an anxiolytic (reduces anxiety), anticonvulsant (reduces seizures) and sedative. It can't be used in people with impaired kidney or liver function. You may need to wait a few months to see the effects if you do end up using this drug. Tolerance and dependence do occur with frequent use, and withdrawal symptoms can occur if use is decreased suddenly. However, clorazepate has breakdown products that are still active in the body, and tolerance to these is slower. If the person takes any other sedatives these are likely to enhance the sedative effects of clorazepate. If the person having clorazepate is a child or takes any other medicines, special precautions should be taken.

Gamma globulin - This a blood protein, given in the form of injections normally to people who have been exposed to measles or hepatitis A, but has been used as an experimental treatment for metachromatic leukodystrophy. It can also be given through an IV drip. The injections or infusions temporarily boost immunity to disease which is very important in people with leukodystrophy, since small infections can quickly become life-threatening. Gamma globulin injections are also used to treat immune disorders including an auto-immune disease where the body's immune system attacks its own blood cells. It has been postulated that demyelination (which is what happens leading to loss of movement in leukodystrophy) may occur because abnormal accumulation of chemicals in the nerve insulation causes the person's immune system to attack and destroy it. Some leukodystrophies also appear to be due to the loss of the cells themselves that insulate nerves. Since gamma globulin also causes the spleen to ignore antibody tagged blood cells, it may be possible to prevent auto-immune attack of the cells that insulate nerves using gamma globulin injections, if they have been tagged by the immune system in the same way.

Hydrocortisone - This might be useful in some cases as a short term way of reducing inflammation in the brain. This may relieve to some degree, problems such as convulsions, fevers and nerve pain.