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!

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.

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(!)

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/

Multi Purpose Alarm

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