Wilson's Disease: Treatment Goals (Nursing)

00:00 Now, let's look at our lifetime treatment goals because remember the prognosis for this is very good if you get treatment.

00:06 So we've got two goals.

00:08 Get rid of the copper that's accumulated and the second, stop you from accumulating more copper in the future.

00:15 That's it.

00:16 That's what we have to do.

00:18 Remove the copper that's accumulated and then prevent copper from accumulating.

00:23 So you see why early diagnosis is life-changing for people and that's our role as nurses, we're part of the team that puts that all together.

00:33 Now, you might be interested in how do we get rid of the copper that's accumulated. Right? That seems kind of weird.

00:39 Well, we use these drugs called chelator.

00:42 Now chelator are chemical compounds and they react with metal ions, and then they make a stable water soluble complex.

00:51 I'm sorry, but I just think that is so cool.

00:54 You can give them these drugs it reacts with metal ions, and now it's stable and its water soluble.

01:01 Well now that its water soluble I can just simply what? Pee it out.

01:06 That is the bomb.

01:08 You got a body full of copper.

01:10 We give you these medications, they combined, now it becomes water soluble and you wash that extra copper right out of their body.

01:19 Now some examples are D-penicillamine or the secondary one is trientine.

01:25 Don't really worry about the names right now.

01:28 There's a lot of other drugs you have to know but you do need to know what a chelator is, that chemical compound it reacts with metal ions, if forms stable, that's important, so it'll stick around long enough that you can pee it out in a water-soluble complex.

01:45 So how do we get rid of the copper that they have? You give them the chelator drugs.

01:50 Now, how do we stop future copper accumulation? Will we continue to give them chelator but at one-third of the dose we gave them on the initial round.

02:00 We keep the standing chelator for the rest of their life because those pathways don't start working again.

02:05 They just don't instantly redevelop.

02:08 So they're going to need chelator for the rest of their life.

02:10 They can also take zinc salts and zinc salts will prevent their bodies from absorbing copper.

02:17 The last part is everyone's least favorite.

02:21 They can maintain a low copper diet.

02:25 Well, I'm going to introduce you to the food that have copper but anytime you start messing with people diet, it is real personal, right? You're getting up in their business.

02:36 So that takes a lot of patient.

02:39 I mean you being patient that takes you being very patient with your clients and helping them understand what these foods are and helping them find recipes and ideas and how they can still eat foods they enjoy and limit themselves in the copper that they take in.

02:57 Why do we do all this? Because if we can help them stick to in a treatment plan, they have an excellent prognosis.

03:04 Without treatment it's fatal, Wilson's disease is fatal.

03:08 Most of the patients would die from cirrhosis or acute liver failure if they didn't receive treatment, so that's why you and I can make a phenomenal difference in patients life as we talk to them, as we gather information, and then as we help them follow the best treatment in their daily life.

03:28 So here we go, we're getting personal.

03:30 These are foods that are high in Copper and you'll see them appear on your screen.

03:35 Shellfish, nuts, Say it isn't so chocolate, mushrooms and organ meats.

03:47 Now that's a pretty wide range.

03:49 So we're going to have to walk them through this.

03:53 I would collaborate with a dietitian and we've got several meal plans out there for patients but work together with the team to help a patient stick to this low copper diet because in the beginning it's extremely restrictive like zero, none of this.

04:10 Then as it's gotten under control, we can gradually We add levels of copper that are acceptable to that patients copper levels.

04:18 So when you're first diagnosed with Wilson's, it's a super strict diet.

04:22 There's just no margin for error.

04:24 Afterwards, chelator have kicked in, we've got things under control, then you'll work with the physician and the dietitian to find out what's the appropriate level of foods with copper for each individual patient.

04:36 Now I don't even like talking about this but this is the worst case scenario.

04:40 If we weren't able to get treatment to them quick enough and they end up needing a liver transplant.

04:45 Because if we weren't able to address the liver failure didn't respond to the drug therapy.

04:51 They may need to have just the organ replaced.

04:54 So during that time while they're waiting for an organ, they might have plasmapheresis, they might have an exchange transfusion, try and filter that blood or dialysis.

05:04 We're just trying to do everything we can to bring that copper level down while they're waiting for an organ transplant.

05:11 Now the good news.

05:12 This is kind of a dark scenario, but this is a good news if they are able to get a transplant then they don't need any further treatment for Wilson's disease.

05:21 Now I'll have to stay on immunosuppressive treatment for the liver, but they don't need any more treatment for Wilson's disease.

05:27 So that's a bright spot in a pretty tough solution.