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

Effective Infection Control

With winter and swine flu, I thought I'd post a general blog about controlling infections which, aside from apnea, are one of the main causes of death in leukodystrophy patients. You don't need to follow all of these unless there is a very vigorous need to control infections (such as immune system compromise after a bone marrow transplant). I am not a doctor, nurse or transplant advisor or anything - these were just ideas I collected from multiple sources and brought together.

General
- Sterilise all feeding equipment daily
- Give an antibacterial-antiviral such as sambucol, olive leaf extract or eccinacea at the first hint of any infection.
- Wash hands regularly throughout the day, using hot water and an antibacterial soap.
- Wash hands before handling the feeding tube, doing breathing treatments or doing anything near the person's mouth
- Use surgical gloves when dealing with breathing treatments or feeding tube, and wipe the feeding tube site with an antiseptic when changing it.
- Wear a facemask when giving breathing treatments or when dealing with the feeding tube
- Vaccume the floor and disinfect surfaces daily
- Have the person wear a facemask when outside in a crowded area. Give the person a full body scrub and put their clothes for laundry after being in a crowded area. Do the same for yourself.
- Do not allow anyone into your house or near you or the person, who has had an infection in the last week, or who has been in contact with someone who has had an infection in the last week.
- Get the person and yourself seasonal, and any other, flu vaccines
- Clean gastrostomy feeding tube sites in the morning and evening with warm water and TCP.
- If someone who was infected within a week ago, or was associated with someone with an infection in the last week has been in the house, vaccume all carpets, wash all carpetless floors with hot water and soap, wash and dry all curtains and seat covers/throws, disinfect all surfaces and allow the room to air for a day before allowing the person with leukodystrophy back into it.

Urinary Tract Infections
- When changing the nappy, ensure that the entire area covered by the nappy is wiped with baby wipes, and allowed to thoroughly dry before the new nappy is put on.
- Try to induce a bowel movement in the morning by giving a rectal wash out with warm water and glycerine, so that bowel movements come into minimal contact with the front area. This will also help prevent constipation.
- Try to catheterise the bladder four times a day, as this will ensure all the urine drains from the bladder and does not stagnate. However an indwelling catheter may increase the chance of infection by making it easier for infections to enter into the body from outside.
- Give adequate water through the tube to ensure proper hydration and flushing of the kidneys. The person's doctor should be able to recommend a good total volume to aim for.

Eye Infections
- Wash hands thoroughly and wear a face mask and surgical gloves when doing anything with the eyes.
- Use artificial tears to keep eyes moist and prevent fluid stagnating in them.

Ear, nose and throat infections
- Have a look in the ears, nose and throat each day with a small torch. If there is any redness or swelling in the throat, or any waxy build-up in the ears, see a doctor for antibiotics and/or to get ears syringed. Suck out mucus from the nose and throat, if there is any.

Gastrointestinal Infections
- Clean and disinfect cooking surfaces thoroughly before use
- Feed a probiotic such as yakkult or actimel.
- Wear an apron when cooking to prevent germs from getting from your clothes onto your hands and thus into food. Wash and dry apron after every cooking session.
- Ensure everything you use is within its best before, display until and use-by dates, and also smell/view it to ensure it has not gone bad prematurely.
- Do not re-freeze food after defrosting it once. Do not freeze cooked meat at all. In the fridge ensure raw meats are placed on the lowest shelf, so they cannot drip onto raw foods like cheese, pudding and vegetables.
- Use a clean dish towel every time you dry dishes - put each dish towel for the laundry after . Preferably allow them to drip dry.
- When washing up use water that is the hottest it can be, and an antibacterial washing up liquid. Alternatively use a dishwasher but check the dishes are clean and dry upon coming out.
- Do not sneeze, touch your face, cough or yawn near food
- Wash hands in hot water with soap and dry thoroughly before handling food or dishes, or after putting things in the bin.
- Tie back long hair before cooking.
- Wipe up any spillages immediately. After washing up or wiping up, place the dish cloth or sponge in the laundry and use a new one for next time.

Intravenous Sites
- Change the tape/dressing on these every three days at least. Wash hands in hot water and soap, dry them thoroughly and use a face mask and surgical gloves when dealing with an open IV site. Do not touch face. Always wipe site with an antiseptic swab to disinfect before immediately replacing tape/dressing. Keep the IV away from water, or the tape will peel off and site may become contaminated.

Cuts, Grazes and Open Sores
- Wear a surgical mask and gloves when cleaning these sites
- Wash hands thoroughly first with hot water and soap and dry thoroughly. Do not touch face in the process.
- Wash the site with TCP diluted in warm water, using cotton wool.
- Put antiseptic cream on the site, such as savlon
- Cover site with a waterproof sticking plaster, or sterile non-fluffy dressing if needed/preferred.

Lung Infections
- Carry out normal breathing treatments at an increased frequency throughout the day.