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.

http://www.dentisan.co.uk/index.html

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.

http://www.rmsupply.co.uk/Virkon_lab_products.htm

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.

http://www.amazon.co.uk/s/ref=nb_sb_noss?url=search-alias%3Dstripbooks&field-keywords=myelin&x=13&y=18

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
http://www.amazon.co.uk/Leukodystrophy-Dictionary-Bibliography-Annotated-References/dp/0497006545/ref=sr_1_1?ie=UTF8&s=books&qid=1270037258&sr=1-1

The Official Parent's Sourcebook on Leukodystrophy: A Revised and Updated Directory for the Internet Age
http://www.amazon.co.uk/Official-Parents-Sourcebook-Leukodystrophy-Directory/dp/0497009943/ref=sr_1_2?ie=UTF8&s=books&qid=1270037258&sr=1-2

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)
http://www.amazon.co.uk/Official-Parents-Sourcebook-Leukodystrophy-Directory/dp/0497009943/ref=sr_1_2?ie=UTF8&s=books&qid=1270037258&sr=1-2

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
http://www.amazon.co.uk/How-Doctors-Think-Jerome-Groopman/dp/0547053649/ref=sr_1_1?ie=UTF8&s=books&qid=1270038126&sr=8-1

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
http://www.jillk.org/author/without-a-word/

"Eyes That See" - Christina Levasheff - parent of Judson Levasheff who had Krabbes leukodystrophy
http://www.storyofjudson.com/eyesthatsee

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

http://www.child-disability.co.uk/Index.html

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.

Thursday 11 March 2010

Some More Links

Waterless ltd. is an online store where you can buy hair and body personal hygeine products that do not require water. For example, soap that you just rub on and it cleans and then evaporates. Hand sanitiser gel that you just rub on and it disinfects and then evaporates after a minute or so. Shampoo and conditioner that is massaged in and then towel-dried as normal, with no rinsing required. These could be very useful in hospital or bedridden at home, as well as when travelling. Here is the link:

http://www.waterlessltd.co.uk

I have added a link for cpaps and bipaps because if you can't get funding to buy a bipap machine it can be extremely difficult to find them, and even more difficult to get one at even a semi-affordable price. So here is a link to a website that sells second-hand cpap and bipap machines. A person with leukodystrophy will probably benefit best from a bipap machine because they assist with breathing out as well as breathing in. The link is American, but it should be possible to order it - though you may need to contact the seller for importation tax rates and delivery costs.

http://www.secondwindcpap.com/Used_CPAP.html

When using a bipap giving the person dry air can cause a dry nose and mouth, and sometimes bleeding from the nose. To get around this problem a heater-humidifier can be bought which delivers heated, humidified air. Some bipap machines come with a built in heater-humidifier. Distilled, deionised water should be used rather than tap water, otherwise you'll get problems like limescale. Another problem is rainout - where water collects in the mask and pools on the face. This can be overcome by making sure the hose tubing through which the air flows into the mask doesn't get so cold that condensation can form on it. This can be done by purchasing a bipap hose insulator, which is reasonably cheap and they can be attractive looking. They are easy enough to find on ebay and amazon among other places, but here is a link to start with:

http://www.eu-pap.co.uk/6ft-fleece-tube-wrap-white.html

My last link is to an oxygen concentrator. If the person with leukodystrophy is suffering from a respiratory infection or any other thing that makes their oxygen levels drop despite using a bipap, a relatively cheap and portable way of providing that oxygen is by using a concentrator, rather than a cylinder. This takes air from the room and modifies it in such a way that the oxygen content is increased. Here is the link. Again it is American, rather than British, but it may help nevertheless.

http://www.oxygenconcentrators.org/info/eclipse/