For any person diagnosed with leukodystrophy, some of the main problems they will probably eventually experience are:
- Inability to swallow
- Pneumonia
- Choking on saliva
- Vomiting
Inability to swallow and choking are linked because kids with leukodystrophy, as they lose their ability to swallow, may choke on saliva, or on vomit. Vomiting, choking and pneumonia are all linked also, due to the fact that saliva or vomit going into the lungs can increase the risk of bacteria growing. For this reason it's important to minimise the risk of vomiting and choking. I am not a specialist: All information is information I have come across from personal experience, as well as parents and carers of people with leukodystrophy and similar disorders.
Feeding, or at least the majority of it, can be done by giving liquid food through a nasogastric tube or a gastrostomy tube.
http://en.wikipedia.org/wiki/Feeding_tube
A nasogastric tube is a long thin hollow silicon tube that is posted up a nostril, down the throat and into the stomach, and held in place with a piece of tape on the cheek or the nose. A gastrostomy tube is a hollow flexible tube that is placed surgically, and connects from outside to inside the stomach. It is usually held in place by a balloon on the inside and a plastic stopper on the outside. Both kinds of feeding tube have to be changed regularly - Mum, Dad or carer can learn to do this for nasogastric tubes, and some kinds of gastrostomy tube. Liquid food is usually placed in a hanging bag, and dripped or pumped through an extension tube into the stomach via the feeding tube. Suitable liquid food may be brands such as ensure, pediasure. Tube feeding reduces risk of vomiting because foods don't have to go into the mouth, where there is a risk they'll be breathed in. Foods can be tube fed in bolus amounts (this means in seperate feeds, like breakfast, lunch and supper), or they can be dripped slowly in all through the day and/or night.
If a gastrostomy tube is placed, there is the option of also performing surgery called a nissen fundoplication - which again reduces the risk of vomiting. This is where a part of the stomach is wrapped around the base of the oesophagus (the passage leading to the entrance to the stomach) and held in place with a stitch. This makes it harder for stomach contents to come back up.
http://en.wikipedia.org/wiki/Nissen_fundoplication
Both gastrostomy and nissen can be done at the same time, although both are major surgery (even though they are performed routinely). You should discuss the benefits and disadvantages with a doctor. Usually it is better to do these surgeries early on, when the child still has good movement, breathes strongly, is not choking or vomiting much, and can still cough. If vomiting still carries on, there are medications that can be put through the tube, such as gaviscon, which is available over the counter in the UK, US and Canada. Powdered ginger is also known to target and block the nausea centres of the brain, preventing queasiness. There are also prescribed medicines that prevent nausea and vomiting, and decrease the amount of acid produced in the stomach. But it is always best to talk to the person's doctor first.
http://www.patient-pharmacy.co.uk/details.asp?productid=GACL30
If the person still vomits, even after a nissen and medication, it might be worth changing the kind of liquid food they are taking. There is a brand available called peptamen, or peptamen junior for children. This is very, very easy to digest as it is specially designed for people who have gastric problems. Some people with leukodystrophy find this easier to deal with than the more commonly used brands such as ensure plus or pediasure.
http://www.nestlenutrition.co.uk/healthcare/gb/news/Pages/PeptamenHN.aspx
http://www.nestlenutrition.co.uk/healthcare/gb/news/Pages/PeptamenJrLiquid.aspx
There is also a formula called vivonex, which is low fat and comprised of free amino acids instead of whole protein. This can also be easier to digest, and is apparently well tolerated if the person being fed is experiencing a lot of physical stresses. The link for vivonex is below:
http://www.nestle-nutrition.com/products/Family.aspx?FamilyId=345ae4e7-5dd3-4a5e-9abb-3eca340c3c3a
Both are manufactured by nestle nutrition. A dietician should ideally be consulted before changing the person's diet or adding anything to it.
If the person still vomits despite a nissen, medication, changed liquid feed, changing from bolus to continual very slow drip feed may help, or, if this is already how the person is done, reducing the speed of flow still further. If all of this fails, there is another type of tube called a gastrojejunal tube. This goes through the same hole made for the stomach tube, but is threaded down into the small bowel. Food goes into the bowel, and medicine goes into the stomach. That means the stomach is always pretty much empty, so there is nothing to vomit up. Feeding with this tube is always continuous drip feeding.
http://www.bcchildrens.ca/Services/SurgeryAndSurgSuites/GeneralSurgery/TubeFeeding/TypesOfTubes/GJFeedingTube.htm
The next blog will be about care of a gastrostomy tube and how to place a nasogastric tube, and then we will move onto another topic of importance: Preventing pneumonia by making sure mucus doesn't linger in the lungs.
No comments:
Post a Comment