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.

http://www.angelflight.com/

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.

http://www.songsoflove.org/

Caringbridge:

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.

http://www.caringbridge.org/

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.

http://www.thhn.co.uk/

Starlight:
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).

http://www.starlight.org.uk/

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.

http://www.rmhc.org.uk/

Songdrops:

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

www.songdrops.com

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:

http://www.samuelsformula.com

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.

***

BASIC OVERVIEW OF HOW TO MAKE SAMUEL'S FORMULA

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
Fybogel
Organic butter
Organic vinegar
Spring or tap water
Acerola berry juice

BEFOREHAND

1. Cook Broth

Ingredients:
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

Ingredients:
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

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:

www.asonginthisworld.com

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):

http://www.youtube.com/watch?v=KKxbexuV804&feature=related

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.

http://www.asonginthisworld.com/images/client_pics/Beckman%20Motor%20Oral%20Therapy.PDF

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.

http://www.asonginthisworld.com/caring_for_sma.php

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.

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".

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.

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/

Getting Help

Caring for a person with leukodystrophy will most likely be, in one way or another, a twenty-four hour job. As such, nobody reasonable could expect one or even two parents/relatives to be physically and mentally able to carry out the entire care of their loved one with leukodystrophy. Because of budgets and stringent rules about who gets help and when, the hours of home nursing provided by the council will probably not come anywhere close to what you actually need. You’ll probably get only a few hours a week, and possibly a night aid.

Because of this lack of help versus need for help, you as parents may be pressured by doctors, council or even family members to put your child with leukodystrophy into a hospice hospital, home or other institution. There is nothing at all wrong with this if you and the person with leukodystrophy are happy with it, but it should be your decision and must not be forced upon you.

For this reason, if your wish is for your loved one with leukodystrophy to be cared for at home, I think it’s important to advertise a need for volunteers. This can be done in a local paper, by circulated email or a website, advertised in a doctor’s surgery, school (whether special needs or not) or in the window of the post office. Several volunteers may reply, but a proportion of volunteers will probably not turn up. Sadly one can’t simply assume everyone who says they will help actually will. Some people just volunteer to make themselves feel good and are too apprehensive or (pre)occupied to actually follow it through. But of those who do follow through, it may be possible for a rota to be drawn up. Some people may even volunteer/be willing to help you with cleaning, gardening or cooking, which I am sure would be invaluable, especially as a treat!

If your loved one with leukodystrophy is a child, then whether they go to a mainstream or special needs school, class-mates will probably be fascinated by how your child is cared for, and eager to learn and help. If you let them help at weekends or after school, this is not exploitation! It is merely eagerness to be a friend and to learn – classmates can potentially be an enormous help, and a great entertainment for your child.

You may be thinking that you just couldn’t blatantly ask for people to give up their time to work for nothing. This is an understandable worry, but I think it is probably unfounded in most communities. If people are at all reasonable they will see that you didn’t want to be in this situation, and would give anything for your loved one to return to full health and not need all this extra care. If they are fully informed of the nature of leukodystrophy, they should be able to see that your child being disabled is something that you could not have prevented, and that you are simply being creative in finding ways to make a good quality of life for them. They will probably be glad of an opportunity to do something to help, even if it’s much smaller than the sample rota implies. Furthermore, if they have ever met your loved one, many of them will have been deeply moved by their personality and ‘plight.’

In the case of a child, ‘plight’ probably need not be the way they see their situation. For a child - even one who struggles daily, their physical condition will likely become to them in time just their way of life. Surrounded by cheerful, honest, determined people who show compassion but do not over-pity them, they may well learn to just take the rough with the smooth.

Another great source of help is a hospice association (I have provided a link in the 'Useful Organisations' post. These provide volunteer helpers who can come out on home visits. They also provide hospice facilities with activities aimed specially for people of all ages and abilities (including relatives of the loved one with leukodystrophy), and helpers specifically trained to work with profoundly disabled people. Going into a hospice does not mean waiting for the end – rather, a stabilisation of symptoms and/or a break for parents and carers.

Certain family holidays are of a nature such that the loved one with leukodystrophy might not get any fun out of them. Arranging a week at a hospice or care home for the loved while you go off is not abandoning them. With research into an appropriate place to spend that time, they will be in capable, caring, experienced hands, and they will almost certainly get a lot of pleasure and probably make several friends. Furthermore, it allows you to come back from your family holiday much less frayed and well rested, ready to take up the challenges of caring for the loved one with leukodystrophy again.

Similarly to a hospice, there are respite care centres for people with disabilities, that can take your loved one with leukodystrophy in for just the day, or for a short stay. Again, these provide one-to-one care and stimulation, with highly trained and experienced carers.

The final help option, particularly for children, is partial fostering. This may sound like admitting defeat at first glance, or as though I’m implying you should give the loved one up, but this is far from the truth. The loved one simply spends stretches of time at (a) trained foster parent/parents’ house, and is made a full and valued part of the family while they are there. The period when your loved one with leukodystrophy is at the foster parents’ house gives you a chance to devote yourself to your other children, your house, your friends, your pets, your relatives or your spouse. This option is obviously much more personal than a hospice or respite care centre can be, so it’s essential that both you as a carer/relative, and the loved one with leukodystrophy, get on well with the foster parent(s), and that he and/or she is/are committed and devoted.

http://www.thefca.co.uk/placement.php