Wednesday 27 January 2010

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

Ketogenic Diet

Background Information
This is a diet used to control seizures that won't go away no matter what drugs/therapy/surgery have been used. You may have heard of it - but it's often labelled as maverick, unpalatable or old fashioned. Obviously there is a degree of potential truth in each of these things, but the negative connotations these descriptions conjure should not put people off as there are few disadvantages that can't be overcome with information, imagination and careful management.

The Diet
The diet centres around using extremely high fats, adequate protein and very, very low carbohydrates. The ratio of fat to carbohydrate and protein combined is usually 4:1 but can be lower or higher, depending on what the dietician recommends. Most peoples' bodies use glucose from carbohydrates to keep the brain supplied with energy. However, the ketogenic diet removes these carbohydrates and replaces them with fat, forcing the body to use this as energy instead. Because the body cannot burn fat completely when we take out the carbohydrates, it produces 'ashes' - called ketones. These can take the place of glucose in providing energy to the brain. It is thought that the use of ketones instead of glucose changes the structure of cell membranes in the brain, making them more electrically stable.

The diet sounds like the Atkins diet, and in a sense it is like that, except that the ratio of fat to carbohydrate is much higher than in the Atkins diet. Also the ketogenic diet requires much more rigorous adherence and you need to work more closely with a dietician than for Atkins.

Side Effects of the ketogenic Diet
Contrary to what one might think, this doesn't make a person fat, nor does it usually increase their chances of heart disease. That said, everything must be consumed according to a strict plan: no more, no less. It is absolutely essential that you find a dietician willing to work with you, because the diet will not work unless it is followed to the letter. There is a slightly higher risk of kidney stones, and a slightly higher risk of constipation. Some people feel extra hunger and thirst in the first few days of starting the diet, but usually these go away within a few days/weeks.

Evidence for the Diet
Most evidence for the ketogenic diet is anecdotal, but there have been some studies validating the claims that the diet works. There have been reported hugely positive effects - reports of intractable seizures being brought completely under control and sometimes even eradicated within a matter of months - to the extent that the patient was weaned off all medication. Whilst this may not be the case for every person, there was one study in which 50% of people claimed some improvements from the diet. Also the diet need not be adhered to permenantly - if it is stuck to through the vulnerable stages (e.g. growth spurts, childhood), there is a good chance that the person can come off the diet and remain seizure free.

If you think this diet could help then talk to your dietician about it. Some dieticians have not heard of it, or have heard the stereotypical view of it, or don't believe in it. Whilst they are experts and I am not, I would highly recommend getting a second opinion before dismissing the idea of the diet.

Links

A complete nutrition ketogenic oral/tube feeding formula
http://www.shs-nutrition.com/products/ketocal

A family who have a daughter following the ketogenic diet. They demonstrate how to make tasty ketogenic recipes, whilst following the strict diet.
http://www.youtube.com/user/tscottshell

A website about the use of the ketogenic diet, including recipes and testimonies
http://www.charliefoundation.org/

Green Goo

Background information
Thank you to Kim Behnken, mother of Reese (metachromatic leukodystrophy), who has given me details about Green Goo (passed on to her by Charlotte Logan, another MLD parent). Kim and many other families who care for a loved one with leukodystrophy use this recipe to keep their loved ones who have leukodystrophy a good weight and in good health. In many respects it is very similar to the previous blog entry 'power juice', but with some ingredients added and some removed.

Reese uses vivonex (just like Shira!) as an overnight feed and starts green goo in the morning three hours after her overnight feed has finished. Vivonex is a complete oral/tube feeding formula that consists of free amino acids rather than whole protein, and so is easier to digest. Patients under stress may well tolerate free amino acids better than whole protein. Again, a dietician is the person to consult before starting or changing any feeding regimes, and they can also help determine how much of each of the green goo ingredients will be needed for the individual with leukodystrophy. Here is a link to vivonex:

http://www.nestle-nutrition.com/products/Family.aspx?FamilyId=345ae4e7-5dd3-4a5e-9abb-3eca340c3c3a

Equipment Needed to make green goo
Freezable storage containers
Juicer
Blender

Ingredients for green goo
Spinach
Carrots
2 Salmon (pouches)
Organic Smoothe Peanutbutter*
Avacados
Bananas*
Kiwi
Blueberries
Squash
Cucumbers
Broccoli
(Prune puree)

How to Make
Juice all the ingredients with the exception of the bananas, peanut butter, avocadoes and salmon

Add to the blender all juiced ingredients along with chopped avocado, chopped banana, peanut butter and salmon

Blend

Strain

Freeze individual servings in separate storage containers.

Use as required/directed by a dietician




* Elvis Presley loved the combination of peanut butter and bananas, apparently!

Tuesday 26 January 2010

Diaphragmatic Paralysis

A parent who has a child with metachromatic leukodystrophy recently raised this in her blog. Apparently people with leukodystrophy can develop something called diaphragmatic paralysis. This is when one half of the diaphragm becomes paralysed, which can lead to a lung collapsing. Needless to say this would be a serious complication during an illness for a person with leukodystrophy. To prevent this going unnoticed, if a chest X-ray is required during illness, it would be wise to tell the technician carrying out the X-ray that this is a possibility, and ask them to check for this during the procedure.

Friday 15 January 2010

POWER JUICE

Rationale

‘Power Juice’ is taken from a recipe by Brad and Maxine Fisher, whose daughter, Shira, has SMA type 1. They follow an amino acid diet using vivonex. You can discuss with a dietician and/or doctor/neurologist whether the person with leukodystrophy would benefit or not from such a diet.

This recipe is the special ‘juice’ they add to Shira’s vivonex. Again a doctor or dietician or nutritionist can recommend amounts of different ingredients. The juice provides lots of fibre, a wide range vitamins and minerals as well as beneficial fatty acids, slow energy release and a good balance of sodium and potassium. The last of these can help prevent heart disease.

Because it is (mostly) raw, rather than unpasteurised (fruit juice is pasteurised too, like milk), the nutrients aren’t degraded. The resulting juice is therefore full of natural beneficial chemicals such as active enzymes (which are destroyed by heat), which can apparently really help digestion, possibly making the person with leukodystrophy less ‘sicky’ or reducing reflux.

Aside from this a good complement of natural nutrients, as well as probiotics, may help to dramatically reduce infections.

Foods used by the Fishers (check with doctor/dietician if possible):


Apples
Pears
Celery
Spinach
Avocado
Banana
Cooked yam (not sweet potato – sweet potatoes are orange inside – yams are white)
Organic, shop bought undiluted prune juice

Equipment

Juicer – preferably electric
Blender
Medium-fine strainer/sieve (some pulp getting through will be more nutricious)
Optional – grater (may help to blend the mixture better to grate it first)
Glass jars with lids

To Make:


Cut up all fruit to be juiced into small places

Place fruit and spinach in juicer and juice. If need be blend after juicing

Pour through strainer to take out large lumps that won’t go through a feeding tube.

Some sediment will still remain but this is fibre and is good.

Get the yam, wash and scrub it clean. Peel it and cut it into slices or chunks,

Boil the yam chunks until they are soft. Allow it to cool a little so it is not piping hot.

Open the avocado, scoop out the inside, and cut into chunks.

Add the yam chunks, along with the avocado to the juice, add prune juice, some water and the probiotic

Blend and strain again

Sterilise the glass jars either by boiling in water or microwaving (with the lids off!) half full of water for one minute.

Pour the mixture in to the jars, put the caps on tightly and store in the fridge for no more than four days. This can be added to tube feed or given as a supplemental drink.

Other Suggestions for foods to add (check with doctor/dietician if possible)


Royal jelly

Kale

Quinoa

Kiwi

Spirulina

Cranberries (help reduce the likelihood of urinary tract infections)

Actimel, yakkult or another probiotic

Vitamin B12

I have just read in a personal web page that vitamin B12 is important in the formation and maintenence of myelin. This is the thing that is depleted in patients with leukodystrophy, multiple sclerosis, Charcot Marie Tooth and other diseases like these. It helps metabolise fatty acids from food to form the building blocks of myelin. It is also important for maintaining the nerves that the myelin insulates, so even if myelin is depleted, vitamin B12 is needed to make the most of what little function remains. It would certainly be worth looking for a vitamin B12 supplement to include in the person with leukodystrophy's diet (with doctor's approval preferably, of course). And don't forget to include it in your own either(!)

Thursday 14 January 2010

Beckman Oral Motor Therapy

Me being absent minded, I can't remember if I have already blogged about this but in case not...this is a therapy of oral motor exercises for people with disorders like leukodystrophy, motor neurone disease and other disorders that affect nerves and movement of the body and face. It helps prevent their jaw from stiffening - through lack of movement - into a rigid position that is uncomfortable and/or affects day to day living. I don't know much about it, but I'll keep you posted. Meanwhile, here is the link:

http://www.beckmanoralmotor.com/

A Warning About Artificial Ventilation

In a past post I talked about keeping airways clean and clear. Just to prove I am no doctor and that you should check with a pulmonary specialist about everything I say before doing it, there is a danger of over-ventilation with a bipap machine. This causes very low carbon dioxide levels which, believe it or not, can cause problems due to abnormal blood chemistry.

They are more expensive but if a doctor is refusing to treat the person with leukodystrophy, and won't point you in any direction, and you can't find any other advice (I have posted charity and organisation names in previous posts), it is probably best to seek out a bipap machine with a computer control. These machines can assess how much help the person needs with breathing, as well as monitor each breath, and deliver pressure changes accordingly. This makes it harder to over-ventilate or under-ventilate.

Philips Respironics supply such a machine. The link to their website is:

http://bipapsynchrony.respironics.com/