Review of Copper Disposal (Nursing)

00:01 Welcome to our video series on the liver.

00:04 Now in this one, we're going to look at a very special example of Wilson's disease.

00:08 It doesn't happen very often, but when it does occur, it can be life-threatening without treatment.

00:15 So let's start with a review of how copper is excreted in the body because that's the key to understanding Wilson's disease.

00:22 It's a buildup of copper in your body.

00:25 In a healthy body, by age one, you have these pathways worked out that involve your liver that can help you get rid of the excess copper.

00:34 The excess copper goes through the liver and it is then sent to the kidneys to be excreted.

00:39 That's how it should be working well in efficiently by age one, but in Wilson's disease it's a rare inherited recessive gene abnormality.

00:49 There's an impairment in that copper transport either the pathways weren't created or the ones that are there just don't work.

00:57 So let's talk about a real life example.

00:59 Let's take two people, they don't know each other yet, but they each carry the recessive gene for Wilson's disease.

01:07 That's why you see the little pinkish colored half of that circle, right? So we've got two people, they don't know each other yet, but they both have the recessive gene for Wilson's disease.

01:17 Now they meet and they decide to start a family together.

01:21 So what are the odds that this recessive gene is going to be passed on to their children when they decide to have children.

01:28 Well because both parents have the recessive gene for Wilson's disease.

01:32 These are the odds that their children will carry that disease.

01:35 Okay so they've got a one in four chance that the child won't have the disease and the child won't carry the gene.

01:43 Now they have a 2 in 4 chance or about a 50% chance that the child won't have the disease, but will carry the gene.

01:53 Now sadly they also have a one in four chance that the child will have Wilson's disease.

01:59 Remember only about One in 30,000 people have this disease but it is a possibility when two people who have the recessive gene marry or decide to have children.

02:10 So when you're thinking about these copper deposits, let's bring up the organs one by one on the screen to help you focus on the ones that have the most problem.

02:19 Now we know the patient is our ability to excrete copper and bile is not working, right? So normally the liver is filtering things and helping us excrete that in bile.

02:30 It's not working well in someone with Wilson's disease.

02:33 So it's building up in organs like your liver, your brain, and you'll even see a sign in your eyes.

02:41 Now we talked about the liver.

02:42 They're going to show you signs like someone whose liver is not doing well like we would see in other liver diseases when it starts impacting your brain.

02:50 We're going to see both some CNS types of changes and some mood disorders.

02:55 Now in your eyes, we're going to show you a really unique picture of a classic sign of Wilson's disease if you look at the patient's eye.

03:03 Now we talked about the brain, but I want you to be familiar with this other name hepatolenticular degeneration.

03:09 So hepato refers to your liver, lenticular refers to your brain.

03:15 Because in our brains most of the copper is deposited in the basal ganglia, particularly we're talking about very special places there together that are called, the lenticular nucleus.

03:26 Now these areas are normally involved in the coordination of movement.

03:31 Okay this is why it's starting to make a really important point for you because when you think about the lenticular area of the brain, but in the basal ganglia, now this is involves the patient's ability to move and to coordinate that movement.

03:46 It's also a big player in your neurocognitive processes think of things like processing stimuli and mood regulation.

03:54 So next to our brain there.

03:56 I want you to write two M's one for mood and one for movement.

04:02 So hepatolenticular generation is just another name for Wilson's disease, but it will kind of help you remember the big areas that it impacts: the liver and the brain.

04:14 And next to this brain, I want to make sure you've taken the time to write mood and movement, because we're going to see problems in both of those areas.

04:23 Now as we go through this video series, I'm going to show you some long lists, and you know how I feel about list if you watch videos with us for very long at all.

04:33 I'm going to give you some pointers, but the most important thing for you to remember about the impact on the brain is mood and movement because these copper excretory pathways, that sounds like a very fancy name, but that's just the way that we get excess copper out of your body.

04:50 They're either failing or they were never developed.