WEBVTT 00:00:06.059 --> 00:00:09.800 Welcome to the bed. We'll go ahead and give you 00:00:09.800 --> 00:00:12.580 the story. This is all going to happen super 00:00:12.580 --> 00:00:46.829 fast. Welcome to the emergency room. Welcome 00:00:46.829 --> 00:00:50.859 back to the deep dive. Today we are wading into 00:00:50.859 --> 00:00:53.240 waters that I think a lot of nursing students, 00:00:53.479 --> 00:00:55.539 and honestly plenty of seasoned educators, find 00:00:55.539 --> 00:00:58.640 pretty intimidating. We are tackling pediatric 00:00:58.640 --> 00:01:02.420 hematology and oncology. It's a unit that, you 00:01:02.420 --> 00:01:04.359 know, it usually comes with a heavy emotional 00:01:04.359 --> 00:01:07.230 tax, but also just... a massive cognitive load 00:01:07.230 --> 00:01:09.730 because the physiology is so specific. It is 00:01:09.730 --> 00:01:11.829 a massive topic. It really is. It's one of those 00:01:11.829 --> 00:01:13.209 units where the textbook feels like it weighs 00:01:13.209 --> 00:01:16.870 50 pounds and then the emotional weight of the 00:01:16.870 --> 00:01:19.790 content is even heavier. Right. But we aren't 00:01:19.790 --> 00:01:23.010 here to memorize every single cytogenetic subtype 00:01:23.010 --> 00:01:26.390 of leukemia. We are here to figure out what actually 00:01:26.390 --> 00:01:28.829 matters when you're standing at the bedside or 00:01:28.829 --> 00:01:31.049 sitting in that exam room. Exactly. We need to 00:01:31.049 --> 00:01:33.010 strip away the noise and just find the signal. 00:01:33.280 --> 00:01:35.640 And looking at our source stack for today, we 00:01:35.640 --> 00:01:37.920 have got some serious material to work with. 00:01:37.939 --> 00:01:41.519 We do. We've got a comprehensive pediatric hematology 00:01:41.519 --> 00:01:44.659 oncology textbook chapter that goes incredibly 00:01:44.659 --> 00:01:47.540 deep into the path of physiology. I mean, the 00:01:47.540 --> 00:01:49.920 real nitty gritty of cellular development. Then 00:01:49.920 --> 00:01:52.359 we have a rapid review exam prep guide, which 00:01:52.359 --> 00:01:54.260 is, you know, essentially the high yield cheat 00:01:54.260 --> 00:01:56.159 sheet for the boards. The Cliff Notes version. 00:01:56.519 --> 00:01:58.760 Yeah, exactly. And then we have these very, very 00:01:58.760 --> 00:02:02.840 dense drug monographs for methotrexate and prednisone. 00:02:02.920 --> 00:02:06.459 Which are two of the absolute cornerstones of 00:02:06.459 --> 00:02:09.240 treatment in this world. I mean, if you don't 00:02:09.240 --> 00:02:11.240 understand those two drugs, you just don't understand 00:02:11.240 --> 00:02:14.319 pediatric oncology. Everywhere. They appear in 00:02:14.319 --> 00:02:17.139 almost every single protocol. So here is the 00:02:17.139 --> 00:02:19.840 mission for this deep dive. We know our listeners 00:02:19.840 --> 00:02:22.060 are out there preparing students for high stakes 00:02:22.060 --> 00:02:24.740 exams. Or maybe you are a nurse looking to sharpen 00:02:24.740 --> 00:02:27.280 your clinical eye. Right. We want to take these. 00:02:27.449 --> 00:02:29.889 I don't know, thousands of pages and cross -reference 00:02:29.889 --> 00:02:33.229 them into what we're calling a Pareto 8020 map. 00:02:33.389 --> 00:02:35.610 I love that framework. We want to find the 20 00:02:35.610 --> 00:02:38.590 % of the hematologic concepts that generate 80 00:02:38.590 --> 00:02:42.270 % of the exam questions, and critically, 80 % 00:02:42.270 --> 00:02:44.330 of the safety saves at the bedside. And we're 00:02:44.330 --> 00:02:46.490 going to filter all of that through two specific 00:02:46.490 --> 00:02:50.069 lenses. The first lens is safety and exam priority, 00:02:50.229 --> 00:02:52.889 basically. What kills the patient? And what fails 00:02:52.889 --> 00:02:55.139 the student? Exactly, what fails the student. 00:02:55.699 --> 00:02:58.199 But the second lens is the one that really makes 00:02:58.199 --> 00:03:00.449 pediatrics unique, and that's development. Because 00:03:00.449 --> 00:03:02.310 you aren't just treating a small adult. That's 00:03:02.310 --> 00:03:04.870 the cardinal rule of pets, right? Right there. 00:03:05.349 --> 00:03:08.110 Never ever you are treating a developing human. 00:03:08.490 --> 00:03:11.689 So you have to ask yourself, how does this disease 00:03:11.689 --> 00:03:13.969 or this lifesaving treatment, how does it mess 00:03:13.969 --> 00:03:16.949 with their timeline? How does it affect their 00:03:16.949 --> 00:03:20.169 ability to walk, to learn, to hit puberty, to 00:03:20.169 --> 00:03:23.189 just be a kid? That tension between saving the 00:03:23.189 --> 00:03:25.750 life and preserving the childhood, that's where 00:03:25.750 --> 00:03:27.509 the real deep dive happens. It's so important. 00:03:27.590 --> 00:03:31.150 It's not enough to cure the cancer. if we destroy 00:03:31.150 --> 00:03:33.229 the child's development in the process. I love 00:03:33.229 --> 00:03:35.729 that framework. So let's zoom out before we get 00:03:35.729 --> 00:03:38.830 into the weeds of sickle cell or leukemia. When 00:03:38.830 --> 00:03:41.610 you look at this Master 8020 map of the source 00:03:41.610 --> 00:03:44.610 material, is there... Like a unifying pattern. 00:03:44.789 --> 00:03:46.830 There is. There really is. If you are taking 00:03:46.830 --> 00:03:49.650 notes, draw a big triangle. The overarching theme 00:03:49.650 --> 00:03:53.509 here is bone marrow failure. Almost every condition 00:03:53.509 --> 00:03:55.370 we're going to discuss today eventually leads 00:03:55.370 --> 00:03:58.270 back to the factory, the bone marrow, either 00:03:58.270 --> 00:04:00.710 shutting down or just malfunctioning completely. 00:04:00.909 --> 00:04:02.969 And that creates a specific set of symptoms. 00:04:03.090 --> 00:04:05.909 A very specific triad of symptoms that I have 00:04:05.909 --> 00:04:09.650 to say exam writers absolutely love to test. 00:04:09.889 --> 00:04:12.270 This is what we call the hemionc red flag pattern. 00:04:12.520 --> 00:04:16.040 And you have to know this triad cold the first 00:04:16.040 --> 00:04:19.279 point of the triangle is anemia The factory just 00:04:19.279 --> 00:04:22.120 isn't making red blood cells. So clinically, 00:04:22.240 --> 00:04:24.519 what do you see? You see pallor. You see pallor. 00:04:24.519 --> 00:04:27.180 They look like porcelain or wax sometimes. You 00:04:27.180 --> 00:04:29.759 see extreme lethargy. I mean, the kid who used 00:04:29.759 --> 00:04:31.899 to run around all day is now napping three times 00:04:31.899 --> 00:04:34.920 a day. And physiologically, you see tachycardia. 00:04:35.220 --> 00:04:38.420 The heart is just racing to pump what little 00:04:38.420 --> 00:04:40.819 oxygen it has to the vital organ. OK, so that's 00:04:40.819 --> 00:04:42.439 point one of the triangle. What's the second 00:04:42.439 --> 00:04:44.740 point? Thrombocytopenia. So the factory isn't 00:04:44.740 --> 00:04:46.899 making platelets. These are your plagues, right? 00:04:47.500 --> 00:04:49.860 You see bruising from minor bumps. We call it 00:04:49.860 --> 00:04:52.220 easy bruising. You see bleeding gums when they 00:04:52.220 --> 00:04:54.040 brush their teeth. And those little red dots. 00:04:54.500 --> 00:04:57.740 Patechia, yes. Those tiny, non -blanching red 00:04:57.740 --> 00:05:00.220 dots that look like a rash but are actually broken 00:05:00.220 --> 00:05:02.920 capillaries under the skin. A huge red flag. 00:05:03.040 --> 00:05:05.079 And the third point of the triangle. Neutropenia. 00:05:05.339 --> 00:05:08.439 The factory isn't making functional white blood 00:05:08.439 --> 00:05:11.360 cells, or in the case of leukemia, it's making 00:05:11.360 --> 00:05:14.639 billions of them. But they're useless, immature 00:05:14.639 --> 00:05:17.120 weeds. So they're not actually fighting infection. 00:05:17.279 --> 00:05:19.620 Not at all. So the patient presents with fever, 00:05:20.300 --> 00:05:23.300 or specifically in leukemia, deep bone pain. 00:05:23.660 --> 00:05:26.040 Because the marrow is so packed with these bad 00:05:26.040 --> 00:05:28.680 cells, it's literally expanding inside the bone 00:05:28.680 --> 00:05:31.019 shaft. Wow. So if a student sees a test question 00:05:31.019 --> 00:05:33.439 description of a child who is pale, bruised, 00:05:33.560 --> 00:05:36.120 and complains of leg pain, that is not growing 00:05:36.120 --> 00:05:38.819 pains. That is a five alarm fire. That is the 00:05:38.819 --> 00:05:41.240 bone marrow failure triad just screaming at you. 00:05:41.339 --> 00:05:43.879 You need to be thinking malignancy or plastic 00:05:43.879 --> 00:05:46.220 anemia immediately. Okay. You cannot dismiss 00:05:46.220 --> 00:05:48.220 those three symptoms when they appear together. 00:05:48.540 --> 00:05:50.720 So within this map we also identified three. 00:05:51.069 --> 00:05:53.189 can't miss safety items. These are the things 00:05:53.189 --> 00:05:54.889 that are, you know, instant failures if you miss 00:05:54.889 --> 00:05:58.029 them on an exam or catastrophic outcomes in real 00:05:58.029 --> 00:06:00.829 life. Yes. Walk us through the top one. Sepsis 00:06:00.829 --> 00:06:03.230 in sickle cell disease. We will unpack the why 00:06:03.230 --> 00:06:06.509 a little later, but the headline is this. Fever 00:06:06.509 --> 00:06:09.490 in a sickle cell patient is a medical emergency. 00:06:09.689 --> 00:06:11.980 An emergency. not just something to watch. It 00:06:11.980 --> 00:06:15.139 is not a give them Tylenol and wait situation. 00:06:15.759 --> 00:06:18.579 Their spleen, which is our main filter for bacteria, 00:06:18.839 --> 00:06:21.259 it functionally dies very early in the disease. 00:06:21.699 --> 00:06:25.500 So they can go from a fever of 101 to dead from 00:06:25.500 --> 00:06:28.319 sepsis in a matter of hours. That's terrifying, 00:06:28.480 --> 00:06:30.680 but such a crucial distinction. It changes the 00:06:30.680 --> 00:06:33.180 triage priority completely. Completely. What's 00:06:33.180 --> 00:06:35.879 the second one? Head trauma and hemophilia. If 00:06:35.879 --> 00:06:38.810 a hemophiliac child bumps their head, you treat 00:06:38.810 --> 00:06:41.350 first, scan second. Say that again, treat first, 00:06:41.490 --> 00:06:43.310 scan second. You administer the clotting factor 00:06:43.310 --> 00:06:45.829 immediately. Do not wait for the CT scan queue. 00:06:46.089 --> 00:06:48.050 By the time you see the bleed on the image, the 00:06:48.050 --> 00:06:50.709 brain damage might already be done. Time is tissue. 00:06:50.750 --> 00:06:54.329 Got it. And the third can't miss item ties back 00:06:54.329 --> 00:06:56.290 to that neutropenia piece we mentioned in the 00:06:56.290 --> 00:06:59.670 triad. It does. It's the calculation of the ANC, 00:06:59.810 --> 00:07:01.689 the absolute neutrophil count. If that number 00:07:01.689 --> 00:07:04.949 is low, usually under 1 ,000 or even 500, depending 00:07:04.949 --> 00:07:07.430 on the protocol, the rules of engagement change 00:07:07.430 --> 00:07:10.899 completely. And the big rule is no rectal temperatures. 00:07:11.019 --> 00:07:13.019 No rectal temperatures. Why is that such a hard 00:07:13.019 --> 00:07:14.800 and fast rule? I know it sounds like a basic 00:07:14.800 --> 00:07:16.620 nursing school thing, but what's the mechanism? 00:07:16.860 --> 00:07:19.439 It's because the rectal mucosa is so fragile. 00:07:19.600 --> 00:07:22.160 If you insert a thermometer and cause a micro 00:07:22.160 --> 00:07:25.660 tear, even one you can't see, you have just introduced 00:07:25.660 --> 00:07:28.279 gut bacteria directly into the bloodstream of 00:07:28.279 --> 00:07:31.139 a child who has zero immune system to fight it. 00:07:31.180 --> 00:07:33.660 Wow. It's a direct line deception. It's a direct 00:07:33.660 --> 00:07:36.120 line. It's the same reason we say no fresh flowers. 00:07:36.819 --> 00:07:39.639 fungal spores like Aspergillus. And if they spike 00:07:39.639 --> 00:07:41.839 a fever, you start broad -spectrum antibiotics 00:07:41.839 --> 00:07:44.160 immediately. You don't pass -go. You don't wait 00:07:44.160 --> 00:07:47.639 for cultures. You act. That sets the stage perfectly. 00:07:48.000 --> 00:07:50.180 We have our safety landscape. Now let's move 00:07:50.180 --> 00:07:52.800 into the specific disease process lecture. We 00:07:52.800 --> 00:07:55.360 synthesize the sources into four main conditions. 00:07:55.939 --> 00:07:59.199 Iron deficiency anemia, sickle cell, hemophilia, 00:07:59.439 --> 00:08:01.259 and leukemia. Right. Let's start with the most 00:08:01.259 --> 00:08:04.560 common one globally. Iron deficiency anemia or 00:08:04.560 --> 00:08:07.379 idea. Often referred to as the milk anemia. Which 00:08:07.379 --> 00:08:10.500 sounds so deceptively harmless. It does, but 00:08:10.500 --> 00:08:13.740 it's a massive developmental thief. Let's strip 00:08:13.740 --> 00:08:16.639 it down to the plain language patho. Think of 00:08:16.639 --> 00:08:19.620 iron as the brick. Okay. You need bricks to build 00:08:19.620 --> 00:08:21.879 a house. In this case, the house is hemoglobin, 00:08:21.899 --> 00:08:24.139 which is the truck that carries oxygen. Right. 00:08:24.360 --> 00:08:26.660 If you have no bricks, you build fewer trucks. 00:08:26.779 --> 00:08:28.980 And the trucks you do build are small. We call 00:08:28.980 --> 00:08:32.179 that microcetic and pale. We call that hypochromic. 00:08:32.350 --> 00:08:36.610 So you have this fleet of tiny empty trucks trying 00:08:36.610 --> 00:08:40.230 to supply oxygen to a whole growing body. Precisely. 00:08:40.389 --> 00:08:42.590 And the body compensates by running the engines 00:08:42.590 --> 00:08:45.269 harder. That's why you get tachycardia. The heart 00:08:45.269 --> 00:08:47.809 is beating faster to cycle those few trucks around 00:08:47.809 --> 00:08:50.490 as many times as possible. But eventually, you 00:08:50.490 --> 00:08:52.570 know, the engine just can't keep up. Now, the 00:08:52.570 --> 00:08:54.490 milk baby presentation is something that often 00:08:54.490 --> 00:08:56.990 trips people up on exams and in the clinic, because 00:08:56.990 --> 00:08:58.870 we usually associate malnutrition with being 00:08:59.200 --> 00:09:02.159 you know, skinny or wasted. Right. But the classic 00:09:02.159 --> 00:09:05.000 milk baby is a toddler, usually between 12 and 00:09:05.000 --> 00:09:08.460 24 months, who is chubby. They look well -fed, 00:09:08.600 --> 00:09:10.539 maybe even overweight. But they're pale. But 00:09:10.539 --> 00:09:13.679 they're pale, almost waxy. And they are so, so 00:09:13.679 --> 00:09:15.879 lethargic. So what's the mechanism there? Why 00:09:15.879 --> 00:09:17.899 are they chubby if they're actually malnourished? 00:09:18.259 --> 00:09:21.139 They are filling up on cow's milk. Some of these 00:09:21.139 --> 00:09:24.200 kids drink 30, 40 ounces a day. And cow's milk 00:09:24.200 --> 00:09:26.740 is high in calories, hence the weight game. But 00:09:26.740 --> 00:09:30.320 it has... effectively zero iron. So it's empty 00:09:30.320 --> 00:09:33.039 calories, in a way. Totally. It displaces solid 00:09:33.039 --> 00:09:36.179 foods that contain iron. And even worse, in young 00:09:36.179 --> 00:09:38.980 codlers, the cow's milk protein can irritate 00:09:38.980 --> 00:09:41.539 the lining of the gut, causing microscopic bleeding, 00:09:41.960 --> 00:09:44.240 so they aren't consuming iron, and they are losing 00:09:44.240 --> 00:09:46.840 blood in their stool. It's a double whammy of 00:09:46.840 --> 00:09:49.299 deficiency. It is. A complete double whammer. 00:09:49.360 --> 00:09:51.500 Then you have the other peak age group, the adolescents. 00:09:51.899 --> 00:09:54.100 It presents differently there, doesn't it? Different 00:09:54.100 --> 00:09:56.879 mechanism, same result. You have a teenager hitting 00:09:56.879 --> 00:09:59.820 a massive growth spurt, so there's a high demand 00:09:59.820 --> 00:10:01.820 for bricks to build muscle and blood volume. 00:10:01.879 --> 00:10:04.419 Right. They have a poor diet, likely high in 00:10:04.419 --> 00:10:07.519 junk food and low in leafy greens. And for females, 00:10:07.679 --> 00:10:11.059 they start menstruation. So they are losing blood 00:10:11.059 --> 00:10:13.899 monthly. And this kid presents differently. Very 00:10:13.899 --> 00:10:16.200 differently. It's the student falling asleep 00:10:16.200 --> 00:10:19.799 in class. the lazy teenager who is actually hypoxic 00:10:19.799 --> 00:10:22.279 or the athlete who suddenly can't keep up with 00:10:22.279 --> 00:10:25.639 the team. It's fatigue and poor concentration. 00:10:26.100 --> 00:10:27.899 Let's hit the need to know data points for the 00:10:27.899 --> 00:10:30.399 exam. What are the top expected findings you're 00:10:30.399 --> 00:10:33.740 looking for? Tachycardia is a big one. Pallor. 00:10:34.120 --> 00:10:36.100 But you have to know where to look. Don't just 00:10:36.100 --> 00:10:38.519 look at the face, especially in darker skinned 00:10:38.519 --> 00:10:40.340 children. Where do you look? You look at the 00:10:40.340 --> 00:10:42.720 conjunctiva of the eyes and the palms of the 00:10:42.720 --> 00:10:45.620 hands and often a systolic heart murmur. And 00:10:45.620 --> 00:10:48.169 what causes the murmur? That's simply the physics 00:10:48.169 --> 00:10:50.970 of thinner, less viscous blood rushing through 00:10:50.970 --> 00:10:53.289 the valves. It's turbulent, and that turbulence 00:10:53.289 --> 00:10:55.730 makes noise. It's not a structural heart problem. 00:10:55.750 --> 00:10:58.549 It's a flow problem. And the labs, what are the 00:10:58.549 --> 00:11:01.309 key indicators? Low hemoglobin, low hematocrit, 00:11:01.350 --> 00:11:04.250 that's a given. But then low MCV, that's the 00:11:04.250 --> 00:11:07.649 microcytic or small cell size. And low ferritin. 00:11:07.769 --> 00:11:09.950 What's ferritin again? Ferritin is your iron 00:11:09.950 --> 00:11:12.029 storage protein. It's the bricks in the warehouse. 00:11:12.190 --> 00:11:14.029 That's actually the first thing to drop, even 00:11:14.029 --> 00:11:16.210 before the hemoglobin itself starts to fall. 00:11:16.169 --> 00:11:19.090 Now, interventions. Iron supplementation seems 00:11:19.090 --> 00:11:21.809 simple, but the pharmacology has some really 00:11:21.809 --> 00:11:24.309 specific traps that examiners love. Oh, they 00:11:24.309 --> 00:11:26.909 love them. The biggest trap involves absorption. 00:11:27.769 --> 00:11:30.690 Iron is finicky. It needs an acidic environment 00:11:30.690 --> 00:11:33.309 to be absorbed effectively in the duodenum. So 00:11:33.309 --> 00:11:35.730 you give it with something acidic. Exactly. The 00:11:35.730 --> 00:11:38.710 golden rule is give it with vitamin C, orange 00:11:38.710 --> 00:11:41.250 juice, tomato juice, or just a citrus fruit. 00:11:41.429 --> 00:11:44.070 It boosts absorption significantly. And the inverse. 00:11:44.250 --> 00:11:47.600 What kills the absorption? Calcium. Calcium neutralizes 00:11:47.600 --> 00:11:50.379 the acid. So the exam trap is the parent who 00:11:50.379 --> 00:11:52.419 says, oh, I mix the iron drops in his morning 00:11:52.419 --> 00:11:54.379 bottle of milk so he takes it. You have to stop 00:11:54.379 --> 00:11:56.299 that. You have to stop that immediately. They 00:11:56.299 --> 00:11:59.299 are completely neutralizing the medication. So 00:11:59.299 --> 00:12:02.179 no milk, no antacids within at least an hour 00:12:02.179 --> 00:12:04.299 of taking the iron. What about the side effects? 00:12:04.700 --> 00:12:06.639 This is where patient adherence usually fails, 00:12:06.840 --> 00:12:09.259 because let's be honest, iron is rough on the 00:12:09.259 --> 00:12:11.929 stomach. It is. It causes constipation, so we 00:12:11.929 --> 00:12:14.850 need to push fluids and fiber. But the scary 00:12:14.850 --> 00:12:17.769 one for parents is the stool color. Iron turns 00:12:17.769 --> 00:12:21.950 the stool Atari dark green or black. If you don't 00:12:21.950 --> 00:12:24.269 warn them, they will think it's a GI bleed and 00:12:24.269 --> 00:12:26.970 rush to the ER. Every time. You have to normalize 00:12:26.970 --> 00:12:29.049 that. You say your tile's poop will turn black. 00:12:29.129 --> 00:12:31.490 That means the medicine is working. If it doesn't 00:12:31.490 --> 00:12:33.590 turn black, they probably aren't taking it. And 00:12:33.590 --> 00:12:35.379 what about the teeth staining? That's a real 00:12:35.379 --> 00:12:37.440 issue. It is a real issue with liquid iron for 00:12:37.440 --> 00:12:39.860 toddlers. It stains their teeth black so you 00:12:39.860 --> 00:12:42.440 teach them to use a straw or a dropper placed 00:12:42.440 --> 00:12:44.379 at the back of the throat to bypass the teeth 00:12:44.379 --> 00:12:47.419 and then brush immediately after. It's temporary 00:12:47.419 --> 00:12:49.720 but it looks very alarming. Let's apply that 00:12:49.720 --> 00:12:52.639 developmental lens we talked about. Why is ID 00:12:52.639 --> 00:12:55.850 such a big deal for a toddler? I mean, why not 00:12:55.850 --> 00:12:57.509 just wait until they eat better? This is the 00:12:57.509 --> 00:12:59.690 part that I think keeps me up at night. Iron 00:12:59.690 --> 00:13:02.409 isn't just for hemoglobin. It is a necessary 00:13:02.409 --> 00:13:05.090 co -factor for neurotransmitter synthesis in 00:13:05.090 --> 00:13:07.769 the brain. Oh, wow. Chronic hypoxia and iron 00:13:07.769 --> 00:13:11.230 deficiency in infancy causes long -term, sometimes 00:13:11.230 --> 00:13:14.110 irreversible cognitive deficits. We're talking 00:13:14.110 --> 00:13:17.049 about lower IQ scores, behavioral issues, and 00:13:17.049 --> 00:13:19.149 learning disabilities that persist even after 00:13:19.149 --> 00:13:21.309 you fix the anemia. So you're not just fixing 00:13:21.309 --> 00:13:23.289 a lab value. You're not just fixing lab value. 00:13:23.549 --> 00:13:26.029 You are protecting the entire architecture of 00:13:26.029 --> 00:13:28.070 their developing brain. It's that serious. That 00:13:28.070 --> 00:13:31.389 is a powerful why. One more quick nice to know 00:13:31.389 --> 00:13:34.929 before we switch diseases. Lead poisoning. Ah, 00:13:34.929 --> 00:13:37.629 yes. Peeking out the craving to eat non -food 00:13:37.629 --> 00:13:40.429 items like dirt or paint chips is really common 00:13:40.429 --> 00:13:42.870 in iron deficiency. The body is just screaming 00:13:42.870 --> 00:13:45.009 for minerals. And if they eat old paint chips? 00:13:45.190 --> 00:13:48.389 They get lead poisoning. And lead actually displaces 00:13:48.389 --> 00:13:50.990 iron in the hemoglobin molecule. So if you see 00:13:50.990 --> 00:13:53.049 a kid with anemia, you should always scream for 00:13:53.049 --> 00:13:55.730 lead and vice versa. They are absolute partners 00:13:55.730 --> 00:13:59.070 in crime. OK, that's great. Moving on to the 00:13:59.070 --> 00:14:01.370 heavyweight champion of pediatric hematology. 00:14:02.230 --> 00:14:04.529 Sickle cell disease. The traffic jam disorder. 00:14:04.769 --> 00:14:07.730 I love that visual. The patherol here is fascinating, 00:14:09.090 --> 00:14:10.830 but it's pretty complex. Walk us through the 00:14:10.830 --> 00:14:14.019 shape shifting. So, normal red blood cells are 00:14:14.019 --> 00:14:17.539 these round, squishy donuts. They can slide through 00:14:17.539 --> 00:14:20.519 tiny capillaries, single file, no problem. In 00:14:20.519 --> 00:14:22.700 sickle cell, the child has a genetic mutation 00:14:22.700 --> 00:14:25.139 where they produce hemoglobin S instead of the 00:14:25.139 --> 00:14:28.320 normal hemoglobin A. And when these cells get 00:14:28.320 --> 00:14:31.419 stressed by dehydration, cold, infection, or 00:14:31.419 --> 00:14:34.279 low oxygen, they polymerize. And what does that 00:14:34.279 --> 00:14:36.919 mean? It means they turn into rigid, sticky, 00:14:37.039 --> 00:14:39.960 crescent moons. Or sickles. And square pegs don't 00:14:39.960 --> 00:14:42.809 fit in round holes. Exactly. They hook together 00:14:42.809 --> 00:14:45.429 and create a physical blockage in the blood vessel. 00:14:45.789 --> 00:14:48.289 That's a vaso -occlusive crisis. The traffic 00:14:48.289 --> 00:14:50.789 jam. The traffic jam. Blood can't get past it, 00:14:50.830 --> 00:14:53.690 so the tissue downstream gets no oxygen. And 00:14:53.690 --> 00:14:56.929 ischemia equals pain. Severe screaming pain. 00:14:57.210 --> 00:14:59.549 Now, age presentation. A newborn with sickle 00:14:59.549 --> 00:15:01.269 cell usually looks perfectly healthy. Why is 00:15:01.269 --> 00:15:04.200 that? Fetal hemoglobin or HBF, we all have it 00:15:04.200 --> 00:15:07.379 in the womb. HBF is a superstar. It holds oxygen 00:15:07.379 --> 00:15:09.840 very tightly and it absolutely refuses to sickle. 00:15:09.960 --> 00:15:11.840 So it's protective. It's totally protective for 00:15:11.840 --> 00:15:13.500 the baby for the first three to four months of 00:15:13.500 --> 00:15:16.019 life. But as it naturally fades and the adult 00:15:16.019 --> 00:15:18.600 HGBS takes over, that's when the symptoms start. 00:15:18.779 --> 00:15:21.549 And the first sign is usually dactylitis. Yes. 00:15:21.789 --> 00:15:24.649 Hand -foot syndrome. The tiny vessels in the 00:15:24.649 --> 00:15:27.090 fingers and toes are the first to get clogged, 00:15:27.149 --> 00:15:29.250 so the hands and feet swell up like sausages 00:15:29.250 --> 00:15:32.450 and are incredibly painful. If you see a six 00:15:32.450 --> 00:15:35.549 -month -old crying with swollen hands, that is 00:15:35.549 --> 00:15:37.909 sickle cell until proven otherwise. Let's talk 00:15:37.909 --> 00:15:39.549 about the complications, because this is where 00:15:39.549 --> 00:15:41.370 all the safety questions come from. We already 00:15:41.370 --> 00:15:43.649 mentioned sepsis and asplenia. Right. So the 00:15:43.649 --> 00:15:46.149 spleen gets clogged so many times by these sickled 00:15:46.149 --> 00:15:48.710 cells that it infarcts and basically shrivels 00:15:48.710 --> 00:15:52.799 up and dies. By age Most of these kids are functionally 00:15:52.799 --> 00:15:55.639 esplenic. Which means they can't filter bacteria? 00:15:55.759 --> 00:15:58.120 They can't filter encapsulated bacteria like 00:15:58.120 --> 00:16:00.919 pneumococcus. That's why they're on prophylactic 00:16:00.919 --> 00:16:03.600 penicillin every single day to act as a kind 00:16:03.600 --> 00:16:06.580 of chemical shield. What about acute chest syndrome? 00:16:07.279 --> 00:16:09.960 I see this term in every textbook. And it's always 00:16:09.960 --> 00:16:12.679 highlighted. It should be. This is the leading 00:16:12.679 --> 00:16:14.840 cause of death in adolescents and adults with 00:16:14.840 --> 00:16:18.639 SED. It looks like pneumonia fever, cough, chest 00:16:18.639 --> 00:16:21.799 pain, a new infiltrate on x -ray. But what's 00:16:21.799 --> 00:16:24.419 happening is sickling in the lung vasculature. 00:16:24.519 --> 00:16:27.960 And that creates a feedback loop. It's a vicious 00:16:27.960 --> 00:16:30.480 cycle. The pain makes them breathe shallowly, 00:16:30.480 --> 00:16:33.159 we call that splinting, which causes hypoxia, 00:16:33.179 --> 00:16:35.120 which causes more sickling in the lungs, which 00:16:35.120 --> 00:16:37.820 causes more pain. You have to intervene fast 00:16:37.820 --> 00:16:40.679 with oxygen and fluids or it just spirals into 00:16:40.679 --> 00:16:43.159 respiratory failure. And splenic sequestration. 00:16:43.399 --> 00:16:44.940 This is different from the shriveled spleen, 00:16:45.120 --> 00:16:46.820 right? Completely opposite. Usually this happens 00:16:46.820 --> 00:16:49.000 in younger kids whose spleen is still working 00:16:49.000 --> 00:16:51.799 a bit. The spleen suddenly acts like a giant 00:16:51.799 --> 00:16:54.659 sponge. It just sucks a massive amount of blood 00:16:54.659 --> 00:16:57.080 volume out of circulation. and traps it. So the 00:16:57.080 --> 00:16:58.960 spleen gets huge. The spleen becomes huge, you 00:16:58.960 --> 00:17:01.200 can feel it filling the belly, and the child 00:17:01.200 --> 00:17:03.580 goes into hypovolemic shock because there is 00:17:03.580 --> 00:17:06.140 literally no blood left in circulation. This 00:17:06.140 --> 00:17:08.519 is an immediate life threat requiring volume 00:17:08.519 --> 00:17:11.240 replacement and blood transfusion to push the 00:17:11.240 --> 00:17:13.319 blood back out. Okay, let's get to the interventions. 00:17:13.720 --> 00:17:17.769 The mnemonic is HOP. HOP to it. H is for hydration. 00:17:18.089 --> 00:17:19.990 This is your number one tool. You need to dilute 00:17:19.990 --> 00:17:21.990 the blood to break up the traffic jam and lower 00:17:21.990 --> 00:17:25.809 the viscosity. We run IV fluids at 1 .5 to 2 00:17:25.809 --> 00:17:28.009 times the maintenance rate. We want them well 00:17:28.009 --> 00:17:31.349 watered. OK. H is hydration. O. O is for oxygen. 00:17:31.789 --> 00:17:34.809 But there's a caveat here. Only if they are hypoxic. 00:17:35.210 --> 00:17:37.690 Giving oxygen won't unsickle the cells if their 00:17:37.690 --> 00:17:40.910 saturation is already 99%. But we absolutely 00:17:40.910 --> 00:17:43.170 need to prevent hypoxia to stop new sickling 00:17:43.170 --> 00:17:47.000 from happening. And P is for? P is for pain management. 00:17:47.200 --> 00:17:49.039 And this is where we really need to have a serious 00:17:49.039 --> 00:17:51.299 culture conversation regarding nursing bias. 00:17:51.420 --> 00:17:53.799 We absolutely do. The pain of a vaso -occlusive 00:17:53.799 --> 00:17:56.400 crisis is often compared to bone fracture pain 00:17:56.400 --> 00:17:59.579 or a heart attack. It is ischemia. It is tissue 00:17:59.579 --> 00:18:01.440 death. So we treat it seriously. We treat this 00:18:01.440 --> 00:18:04.839 with opioids, morphine, deloaded, and we give 00:18:04.839 --> 00:18:07.960 it around the clock, ATC, or via a PCA pump. 00:18:08.039 --> 00:18:11.319 We do not give it PRN as needed. Why not PRN? 00:18:11.359 --> 00:18:13.299 If you wait for the child to be crying in agony 00:18:13.299 --> 00:18:15.079 before you give the next dose, you are chasing 00:18:15.079 --> 00:18:17.559 the pain and you will never ever catch it. And 00:18:17.559 --> 00:18:19.960 please check your bias. These children are not 00:18:19.960 --> 00:18:21.900 drug -seeking addicts. They are seeking relief 00:18:21.900 --> 00:18:24.740 from dying tissue. Is there a specific medication 00:18:24.740 --> 00:18:27.640 contraindication here? An exam favorite? Meparidine 00:18:27.640 --> 00:18:30.319 or Demerol. Never use it. It breaks down into 00:18:30.319 --> 00:18:32.900 a metabolite called Normaparadine, which accumulates 00:18:32.900 --> 00:18:35.579 and lowers the seizure threshold. And these kids 00:18:35.579 --> 00:18:38.380 are already at risk for stroke. Exactly. Sickle 00:18:38.380 --> 00:18:40.380 cell kids are already at high risk for strokes 00:18:40.380 --> 00:18:43.259 and CNS changes. Giving them a drug that can 00:18:43.259 --> 00:18:46.700 cause seizures is just malpractice. Do not choose 00:18:46.700 --> 00:18:49.599 Demerol on the exam. What about temperature management? 00:18:49.960 --> 00:18:52.119 You're managing pain, you're managing hydration. 00:18:52.579 --> 00:18:56.579 Good question. Heat is good. It vasodilates. 00:18:56.960 --> 00:19:01.660 Cold is bad. It vasoconstricts. Never, ever put 00:19:01.660 --> 00:19:04.500 an ice pack on a sickle cell pain crisis. You 00:19:04.500 --> 00:19:06.500 will tighten the vessels and make the traffic 00:19:06.500 --> 00:19:09.380 jam a hundred times worse. Looking at the developmental 00:19:09.380 --> 00:19:12.859 impact, this is a chronic, lifelong battle. It 00:19:12.859 --> 00:19:15.630 has to take a toll. It does. A huge toll. Growth 00:19:15.630 --> 00:19:17.609 retardation is common because their metabolic 00:19:17.609 --> 00:19:19.789 rate is so high the body is working overtime 00:19:19.789 --> 00:19:22.309 just to make blood and fight and section. They 00:19:22.309 --> 00:19:24.430 are often smaller than their peers and hit puberty 00:19:24.430 --> 00:19:26.849 later. And that's tough socially. It's so tough 00:19:26.849 --> 00:19:29.009 socially for a 14 -year -old who looks 10. And 00:19:29.009 --> 00:19:30.849 school. They miss a lot of school due to pain 00:19:30.849 --> 00:19:32.910 crises. But the shadow that's always looming 00:19:32.910 --> 00:19:35.769 over them is stroke. About 11 % of untreated 00:19:35.769 --> 00:19:38.640 kids will have a stroke by age 20. Even silent 00:19:38.640 --> 00:19:41.500 strokes can cause cognitive decline. That's why 00:19:41.500 --> 00:19:43.980 we do transcranial Doppler screenings annually 00:19:43.980 --> 00:19:47.680 from age 2 to 16. We are looking for high -velocity 00:19:47.680 --> 00:19:50.460 blood flow in the brain, which implies the vessels 00:19:50.460 --> 00:19:53.339 are narrowing. One nice -to -know therapy before 00:19:53.339 --> 00:19:56.740 we move on? Hydroxyurea. It's a chemotherapy 00:19:56.740 --> 00:20:00.019 agent, technically. But at lower doses, it stresses 00:20:00.019 --> 00:20:02.660 the marrow just enough to trick it into making 00:20:02.660 --> 00:20:05.279 fetal hemoglobin again. And we know HPF is protective. 00:20:05.559 --> 00:20:08.079 Exactly. Remember, HPF doesn't sickle. It's the 00:20:08.079 --> 00:20:10.259 only drug we have that actually modifies the 00:20:10.259 --> 00:20:12.440 course of the disease. It's a game changer. Incredible. 00:20:12.920 --> 00:20:15.640 OK, let's shift gears to number three, hemophilia. 00:20:15.880 --> 00:20:19.240 The missing glue. So in this case, the platelets, 00:20:19.359 --> 00:20:22.269 the initial plug, they work. Just fine. Correct. 00:20:22.509 --> 00:20:24.690 You get a cut, platelets rush in, they form a 00:20:24.690 --> 00:20:26.890 plug, the bleeding stops initially, but to keep 00:20:26.890 --> 00:20:29.089 that plug there, you need the fibrin mesh, the 00:20:29.089 --> 00:20:31.049 clot. That comes from the clotting cascade. And 00:20:31.049 --> 00:20:33.230 in hemophilia. One of the dominers in that cascade 00:20:33.230 --> 00:20:35.289 is missing, so the plug eventually washes away 00:20:35.289 --> 00:20:37.750 and the bleeding restarts. Can you distinguish 00:20:37.750 --> 00:20:41.730 hemophilia A versus B for us? Hemophilia A is 00:20:41.730 --> 00:20:44.630 missing factor 8. I always say A, it sounds similar, 00:20:44.710 --> 00:20:46.569 helps to remember. That's the most common, about 00:20:46.569 --> 00:20:50.150 80 % of cases. Hemophilia B is missing factor 00:20:50.150 --> 00:20:53.690 9. also called Christmas disease. And this is 00:20:53.690 --> 00:20:56.349 X -linked recessive. Which means mom is the carrier 00:20:56.349 --> 00:20:59.869 and the son gets the disease. It is overwhelmingly 00:20:59.869 --> 00:21:03.089 a male diagnosis. When do we usually spot this? 00:21:03.889 --> 00:21:06.730 Often at circumcision, that's a big one. Prolonged 00:21:06.730 --> 00:21:08.849 bleeding. If not then, it's when they become 00:21:08.849 --> 00:21:11.410 mobile. The toddler phase is so dangerous they 00:21:11.410 --> 00:21:13.670 fall, they bump their heads. A normal kid gets 00:21:13.670 --> 00:21:16.309 a bump. A hemophiliac gets a massive hematoma. 00:21:16.450 --> 00:21:18.690 Or a frenulum tear in the mouth that just won't 00:21:18.690 --> 00:21:20.869 stop bleeding because saliva breaks down clots 00:21:20.869 --> 00:21:23.150 naturally. Let's look at the need -to -know exam 00:21:23.150 --> 00:21:25.930 profile for hemophilia. Easy bruising, obviously. 00:21:26.250 --> 00:21:28.690 But the hallmark is hemarthrosis bleeding into 00:21:28.690 --> 00:21:31.769 the joint spaces. Knees, ankles, elbows. What 00:21:31.769 --> 00:21:33.720 does that look like? The joint fills with blood. 00:21:33.920 --> 00:21:36.220 It becomes hot, swollen, incredibly painful, 00:21:36.359 --> 00:21:38.579 and locked in place. And over time, that iron 00:21:38.579 --> 00:21:40.460 in the blood actually destroys the cartilage 00:21:40.460 --> 00:21:43.160 and causes permanent deformity. And the labs. 00:21:43.420 --> 00:21:46.559 This is a classic question. differentiating bleeding 00:21:46.559 --> 00:21:48.660 disorders. It is. You have to know the difference 00:21:48.660 --> 00:21:51.099 between platelets and factors. In hemophilia, 00:21:51.259 --> 00:21:53.559 the platelet count is normal. The PT is normal. 00:21:53.880 --> 00:21:57.140 The PTT partial thromboplastin time is prolonged. 00:21:57.319 --> 00:21:59.680 Why the PTT? Because PTT measures the intrinsic 00:21:59.680 --> 00:22:01.700 pathway, which is where factors eight and nine 00:22:01.700 --> 00:22:03.779 live. So that's the one that will be abnormal. 00:22:03.900 --> 00:22:05.640 Interventions. We talked about treat first for 00:22:05.640 --> 00:22:08.240 head trauma. That's paramount. Vital. Factor 00:22:08.240 --> 00:22:10.819 replacement is the priority. If a hemophiliac 00:22:10.819 --> 00:22:13.339 falls off a swing and hits their head, you push 00:22:13.339 --> 00:22:16.240 factor eight or I. immediately. You don't wait 00:22:16.240 --> 00:22:18.799 for symptoms. You don't wait for the scan. You 00:22:18.799 --> 00:22:21.019 push the factor. What about for the joint pleats? 00:22:21.259 --> 00:22:25.900 Is it rice? Yes. Rest, ice, compression, elevation. 00:22:26.160 --> 00:22:28.940 Here, ice is your friend because the vasoconstriction 00:22:28.940 --> 00:22:31.180 slows the bleeding into the joint. But again, 00:22:31.319 --> 00:22:33.339 rice is secondary to factor replacement. You 00:22:33.339 --> 00:22:35.440 have to give the glue first. Now let's talk about 00:22:35.440 --> 00:22:38.359 pain meds. We said opioids for sickle cell. What 00:22:38.359 --> 00:22:40.480 about for hemophilia? This is a really tricky 00:22:40.480 --> 00:22:45.720 one and a huge safety point. No NSAIDs. No aspirin, 00:22:45.920 --> 00:22:49.180 no ibuprofen, no naproxen. And why is that? Because 00:22:49.180 --> 00:22:52.140 NSAIDs inhibit platelet aggregation. These kids 00:22:52.140 --> 00:22:54.920 already have a broken clotting cascade. If you 00:22:54.920 --> 00:22:57.279 knock out their platelets too, they have literally 00:22:57.279 --> 00:23:00.380 zero defense. They will bleed uncontrollably. 00:23:00.700 --> 00:23:03.440 So Tylenol acetaminophen is the king here. Correct. 00:23:03.839 --> 00:23:06.720 Unless the hematologist specifically clears a 00:23:06.720 --> 00:23:09.299 certain NSAID regimen for joint inflammation, 00:23:09.839 --> 00:23:12.539 you stick to Tylenol in opioids if needed for 00:23:12.539 --> 00:23:14.880 severe pain. The elemental impact here really 00:23:14.880 --> 00:23:17.500 changes how they play, doesn't it? It does. Contact 00:23:17.500 --> 00:23:20.019 sports are completely out. No football, no rugby, 00:23:20.119 --> 00:23:23.079 no wrestling, no hockey. One hard tackle could 00:23:23.079 --> 00:23:26.160 cause a life -threatening bleed. And that's really 00:23:26.160 --> 00:23:28.339 hard for a boy who wants to fit in with his friends. 00:23:28.420 --> 00:23:31.099 So what do we encourage? Non -contact sports. 00:23:31.619 --> 00:23:33.900 Swimming is the absolute best. It builds muscle 00:23:33.900 --> 00:23:36.839 to support the joints without any impact. Golf, 00:23:37.240 --> 00:23:39.759 fishing, cycling with a helmet and pads, things 00:23:39.759 --> 00:23:42.099 like that. And what about autonomy? This is one 00:23:42.099 --> 00:23:44.380 of the great success stories of modern nursing. 00:23:44.559 --> 00:23:46.920 We teach these kids to self -administer their 00:23:46.920 --> 00:23:49.960 5E factor prophylactic infusions at home. By 00:23:49.960 --> 00:23:52.859 age 8 to 12, many of these boys are mixing their 00:23:52.859 --> 00:23:55.460 own factor and sticking their own veins. That's 00:23:55.460 --> 00:23:57.839 incredible. It gives them control over a disease 00:23:57.839 --> 00:24:00.480 that threatens to control them. It's really empowering. 00:24:00.680 --> 00:24:03.740 A quick nice to know before we move on. DDAVP, 00:24:03.880 --> 00:24:06.539 or desmopressin, it's a synthetic hormone. For 00:24:06.539 --> 00:24:09.240 mild hemophilia A, it stimulates the body to 00:24:09.240 --> 00:24:12.289 release stored factor from the blood vessel walls. 00:24:12.710 --> 00:24:15.569 So it can stop a minor bleed. It can, without 00:24:15.569 --> 00:24:18.109 a transfusion, but it does not work for hemophilia 00:24:18.109 --> 00:24:20.329 B because they don't have a factor eight issue, 00:24:20.829 --> 00:24:22.650 or for severe hemophilia A because they have 00:24:22.650 --> 00:24:24.609 no stores of factor eight to squeeze out. Got 00:24:24.609 --> 00:24:27.990 it. Okay, fourth and final disease, leukemia. 00:24:28.630 --> 00:24:31.470 Specifically, all acute lymphoblastic leukemia. 00:24:31.730 --> 00:24:33.769 Weeds in the garden. Break that down for us. 00:24:33.900 --> 00:24:36.160 The bone marrow is a garden. It's meant to grow 00:24:36.160 --> 00:24:38.680 flowers, which are your red blood cells, vegetables, 00:24:38.839 --> 00:24:40.859 your white blood cells, and fruits, your platelets. 00:24:41.259 --> 00:24:44.539 In leukemia, a single immature cell, a blast, 00:24:44.859 --> 00:24:47.460 mutates. It's a weed. It replicates incredibly 00:24:47.460 --> 00:24:49.779 fast. It doesn't do any work. It just takes up 00:24:49.779 --> 00:24:52.160 space. And eventually, the garden is nothing 00:24:52.160 --> 00:24:54.799 but weeds. The good plants are crowded out, and 00:24:54.799 --> 00:24:57.119 they die. So the patient has these super high 00:24:57.119 --> 00:25:00.200 counts of blast cells, but they're still immunocompromised. 00:25:00.329 --> 00:25:03.390 Exactly. A child might have a WBC count of 50 00:25:03.390 --> 00:25:07.630 ,000. Normal is maybe 10K. But they're all useless 00:25:07.630 --> 00:25:10.210 blasts. They don't fight infection. So the child 00:25:10.210 --> 00:25:13.130 is functionally neutropenic, even with a high 00:25:13.130 --> 00:25:15.069 white count. Who gets this? What's the typical 00:25:15.069 --> 00:25:18.089 age? Peak onset is two to five years old. It's 00:25:18.089 --> 00:25:20.390 really a preschool disease. And the presentation 00:25:20.390 --> 00:25:23.009 ties right back to our initial triad. It does. 00:25:23.309 --> 00:25:26.190 Anemia, so they're tired. Thrombocytopenia, so 00:25:26.190 --> 00:25:28.650 they're bruising. Neutropenia, so they have a 00:25:28.650 --> 00:25:31.549 fever. But for leukemia, you really need to watch 00:25:31.549 --> 00:25:35.349 for bone pain and a limp. The marrow is expanding 00:25:35.349 --> 00:25:37.450 from the pressure of the tumor burden inside 00:25:37.450 --> 00:25:40.490 the bone. If a toddler suddenly refuses to walk, 00:25:40.750 --> 00:25:45.049 you check a CBC. A peripheral smear will show 00:25:45.049 --> 00:25:47.809 the blasts, but the definitive diagnosis is the 00:25:47.809 --> 00:25:50.410 bone marrow biopsy. You need to see greater than 00:25:50.410 --> 00:25:53.549 25 % blast cells in the marrow to confirm leukemia. 00:25:55.660 --> 00:25:57.779 Leukemia cells are smart. They like to hide in 00:25:57.779 --> 00:26:00.140 what we call sanctuary sites where 5e chemo can't 00:26:00.140 --> 00:26:02.420 reach them easily. The central nervous system 00:26:02.420 --> 00:26:04.299 is one of them because of the blood brain barrier. 00:26:04.500 --> 00:26:06.799 So you check the spinal fluid. We check the spinal 00:26:06.799 --> 00:26:09.799 fluid. If there are cells there, or even if there 00:26:09.799 --> 00:26:13.019 aren't, as prophylaxis, we have to inject chemo 00:26:13.019 --> 00:26:15.700 directly into the spine. We call that intrathecal 00:26:15.700 --> 00:26:18.400 chemotherapy. Let's talk about a major safety 00:26:18.400 --> 00:26:21.180 emergency during treatment. Tumor lysis syndrome. 00:26:21.339 --> 00:26:23.819 This happens usually during induction. That's 00:26:23.819 --> 00:26:26.279 the first phase of chemo, where we just blast 00:26:26.279 --> 00:26:29.000 the cancer. We kill millions of cells instantly. 00:26:29.839 --> 00:26:32.599 And they burst open. They lie. And cells are 00:26:32.599 --> 00:26:35.299 just bags of chemicals. Right. They are full 00:26:35.299 --> 00:26:37.440 of potassium and uric acid. When they burst, 00:26:37.619 --> 00:26:39.599 they dump all of that into the bloodstream all 00:26:39.599 --> 00:26:41.960 at once. And that causes two huge problems. Two 00:26:41.960 --> 00:26:44.779 huge problems. First, you get hyperkalemia. High 00:26:44.779 --> 00:26:47.420 potassium leads to fatal cardiac arrhythmias. 00:26:47.700 --> 00:26:50.819 Second, you get hyperuricemia. Uric acid crystals 00:26:50.819 --> 00:26:53.519 form in the kidneys and cause acute renal failure. 00:26:53.599 --> 00:26:55.599 So how do nurses prevent this from becoming a 00:26:55.599 --> 00:26:57.779 catastrophe? Hydration, hydration, hydration. 00:26:57.960 --> 00:26:59.920 We flush the kidneys like a fire hose to keep 00:26:59.920 --> 00:27:02.140 those crystals from forming. And we give a drug 00:27:02.140 --> 00:27:04.859 called allopurinol, which stops uric acid production 00:27:04.859 --> 00:27:07.180 in the first place. The developmental impact 00:27:07.180 --> 00:27:10.210 here is so visibly profound. It's heartbreaking. 00:27:10.369 --> 00:27:12.250 You have a four year old, they lose their hair, 00:27:12.390 --> 00:27:14.829 alopecia. They get a moon phase from the high 00:27:14.829 --> 00:27:17.289 dose steroids. They look different. And because 00:27:17.289 --> 00:27:19.490 of the neutropenia, they are isolated. They can't 00:27:19.490 --> 00:27:21.009 go to preschool. They can't go to preschool. 00:27:21.130 --> 00:27:23.750 They can't see friends. You often see regression 00:27:23.750 --> 00:27:26.009 potty trained kids go back to diapers or they 00:27:26.009 --> 00:27:28.470 start baby talking again. It's a totally normal 00:27:28.470 --> 00:27:31.289 response to trauma and isolation. One safety. 00:27:31.569 --> 00:27:35.289 Nice to know regarding vaccines. This seems critical. 00:27:35.450 --> 00:27:37.789 Absolutely critical. No live vaccines while on 00:27:37.789 --> 00:27:41.630 chemo. No MMR. No varicella for chickenpox. No 00:27:41.630 --> 00:27:44.769 nasal flu mist. Their immune system cannot handle 00:27:44.769 --> 00:27:47.690 the live virus. It can cause the actual disease. 00:27:48.049 --> 00:27:50.769 We have to rely on herd immunity to protect these 00:27:50.769 --> 00:27:54.130 kids. That covers the diseases. Now let's pivot 00:27:54.130 --> 00:27:57.470 to the how the medication module. We are focusing 00:27:57.470 --> 00:27:59.849 on two drugs that appeared in every protocol 00:27:59.849 --> 00:28:03.079 we reviewed. methotrexate and prednisone. Let's 00:28:03.079 --> 00:28:05.039 start with methotrexate. This is a chemo drug, 00:28:05.140 --> 00:28:07.420 but it's also used for juvenile rheumatoid arthritis. 00:28:07.539 --> 00:28:10.299 How does it work? It's an anti -metabolite, specifically 00:28:10.299 --> 00:28:12.519 a folate antagonist. It's a master of disguise. 00:28:13.359 --> 00:28:16.579 It looks chemically just like folic acid. The 00:28:16.579 --> 00:28:18.720 cancer cell, which is hungry and dividing really 00:28:18.720 --> 00:28:21.339 fast, grabs the methotrexate thinking it's food, 00:28:21.480 --> 00:28:24.400 thinking it's folate to build DNA. But it's a 00:28:24.400 --> 00:28:27.599 trap. It blocks DNA synthesis, and the cell starves 00:28:27.599 --> 00:28:30.180 and dies. But it affects healthy, rapidly dividing 00:28:30.180 --> 00:28:33.039 cells, too. It does. Any rapidly dividing cell. 00:28:33.319 --> 00:28:35.420 That includes the bone marrow, which is why you 00:28:35.420 --> 00:28:38.059 get myelo suppression, and the lining of the 00:28:38.059 --> 00:28:40.599 GI tract, which is why you get mucositis. There's 00:28:40.599 --> 00:28:43.339 a specific rescue associated with high -dose 00:28:43.339 --> 00:28:45.619 methotrexate that we have to talk about. Leucovorin 00:28:45.619 --> 00:28:48.819 rescue. Leucovorin is a form of folic acid that 00:28:48.819 --> 00:28:51.359 actually bypasses the metabolic block from the 00:28:51.359 --> 00:28:54.259 methotrexate. It effectively rescues the healthy 00:28:54.259 --> 00:28:56.319 cells from the toxic effects of the drug. And 00:28:56.319 --> 00:28:59.019 the timing is critical. The timing is everything. 00:28:59.460 --> 00:29:01.700 In high -dose protocols, if you miss the leukovorin 00:29:01.700 --> 00:29:04.119 dose or give it late, the patient can suffer 00:29:04.119 --> 00:29:07.240 fatal toxicity. It's a huge med safety issue. 00:29:07.380 --> 00:29:10.859 Well, we also have to watch the urine. Yes. Methotrexate 00:29:10.859 --> 00:29:13.640 precipitates. It crystallizes in acidic urine. 00:29:13.920 --> 00:29:16.720 It will destroy the kidneys. So we have to keep 00:29:16.720 --> 00:29:19.180 the urine pH alkaline, usually greater than 7 00:29:19.180 --> 00:29:22.880 .0. We run IV fluids with sodium bicarbonate 00:29:22.880 --> 00:29:25.059 to alkalize the urine. And you're checking it 00:29:25.059 --> 00:29:27.359 constantly. We literally check the pee with a 00:29:27.359 --> 00:29:29.279 dipstick every single time they void. If it's 00:29:29.279 --> 00:29:31.359 acidic, we don't give the drug. It's that simple. 00:29:31.779 --> 00:29:33.839 There was a specific warning in the monograph 00:29:33.839 --> 00:29:36.680 about neonates. Yes, this is so important. The 00:29:36.680 --> 00:29:39.779 formulation matters. Some methotrexate vials 00:29:39.779 --> 00:29:43.079 contain benzyl alcohol as a preservative. In 00:29:43.079 --> 00:29:45.539 neonates, their immature livers can't process 00:29:45.539 --> 00:29:49.500 this. It causes gasping syndrome, fatal respiratory 00:29:49.500 --> 00:29:52.619 distress, acidosis, and death. So you always 00:29:52.619 --> 00:29:54.980 check. Always check for preservative -free formulations 00:29:54.980 --> 00:29:58.220 for infants and for any intrathecal use. OK. 00:29:58.559 --> 00:30:01.940 Moving to prednisone. The corticosteroid. The 00:30:01.940 --> 00:30:04.000 love -hate drug of pediatrics. Oh, you say that. 00:30:04.170 --> 00:30:06.269 Because it's so effective, but the side effects 00:30:06.269 --> 00:30:08.829 can be brutal. We use it for leukemia induction. 00:30:09.009 --> 00:30:10.950 Lymph flasks are actually very sensitive to steroids. 00:30:11.089 --> 00:30:13.410 It just melts them. Asthma, nephrotic syndrome, 00:30:13.569 --> 00:30:15.910 all sorts of things. And the mechanism. It suppresses 00:30:15.910 --> 00:30:18.069 inflammation and the immune response. It's also 00:30:18.069 --> 00:30:20.230 lympholytic. It actually breaks down lymphocytes. 00:30:20.509 --> 00:30:22.809 But the side effects in children are really distinct. 00:30:23.309 --> 00:30:27.130 They are. First is growth suppression. Long -term 00:30:27.130 --> 00:30:30.009 use can literally stunt a child height. Second 00:30:30.009 --> 00:30:33.930 is mood changes. We call it roid rage. A normally 00:30:33.930 --> 00:30:36.490 sweet child can become aggressive, irritable, 00:30:36.809 --> 00:30:39.430 or severely depressed. You have to warn the parents. 00:30:39.470 --> 00:30:41.990 You have to warn the parents. This is the drug, 00:30:42.130 --> 00:30:45.329 not your child being naughty. Third is the cushion 00:30:45.329 --> 00:30:48.069 -goy appearance. The moon face, the round cheeks, 00:30:48.269 --> 00:30:51.670 the buffalo hump, the weight gain. For an adolescent 00:30:51.670 --> 00:30:54.130 focused on body image, this is just devastating. 00:30:54.269 --> 00:30:56.529 And the safety rule for stopping. This is a big 00:30:56.529 --> 00:30:59.289 one. Taper. You can never stop steroids abruptly 00:30:59.289 --> 00:31:01.730 after long -term use. The drug suppresses the 00:31:01.730 --> 00:31:04.430 child's own adrenal glands. If you pull the drug, 00:31:04.650 --> 00:31:06.410 the adrenals are asleep and can't make cortisol. 00:31:06.450 --> 00:31:09.029 And the child goes into... Adrenal crisis. Which 00:31:09.029 --> 00:31:11.109 is shock. You have to taper slowly to wake the 00:31:11.109 --> 00:31:13.029 adrenals back up. Any tips for administration? 00:31:13.529 --> 00:31:16.309 Because it tastes terrible. It tastes horrific. 00:31:16.539 --> 00:31:18.680 incredibly bitter. You have to crush it and mix 00:31:18.680 --> 00:31:20.440 it with something strong chocolate syrup, strong 00:31:20.440 --> 00:31:23.779 jam, but mix it in a small amount, like one spoonful. 00:31:24.079 --> 00:31:25.900 If you mix it in a whole bowl of pudding and 00:31:25.900 --> 00:31:28.079 they only eat half, you have no idea how much 00:31:28.079 --> 00:31:30.220 drug they actually got. Okay, we're in the home 00:31:30.220 --> 00:31:33.240 stretch. This is section D, cross -linking and 00:31:33.240 --> 00:31:36.059 synthesis. Let's connect these dots. Let's link 00:31:36.059 --> 00:31:39.819 leukemia and methotrexate. We said methotrexate 00:31:39.819 --> 00:31:42.960 attacks rapidly dividing cells. Well, the lining 00:31:42.960 --> 00:31:46.420 of the mouth divides rapidly. This causes mucositis 00:31:46.420 --> 00:31:49.000 -severe ulcers from the lips all the way to the 00:31:49.000 --> 00:31:51.700 anus. And this links back to nutrition. Of course. 00:31:52.000 --> 00:31:54.250 If a kid's mouth feels like raw meat, They won't 00:31:54.250 --> 00:31:57.250 eat. So the nursing intervention is bland, soft 00:31:57.250 --> 00:31:59.890 foods, no orange juice because the acid burns, 00:32:00.289 --> 00:32:02.730 no potato chips because the sharp edges cut. 00:32:02.809 --> 00:32:05.089 And you use things like Magic Mouthwash. We use 00:32:05.089 --> 00:32:07.730 Magic Mouthwash, which is usually a combo of 00:32:07.730 --> 00:32:10.910 lidocaine, malox, and defenhydramine to numb 00:32:10.910 --> 00:32:12.930 the mouth so they can eat and get some nutrition 00:32:12.930 --> 00:32:15.890 in. OK, let's link leukemia and prednisone. This 00:32:15.890 --> 00:32:18.750 is the double whammy for bones. The leukemia 00:32:18.750 --> 00:32:21.920 itself causes bone infiltration and pain. Then 00:32:21.920 --> 00:32:24.640 you add prednisone, which causes osteoporosis 00:32:24.640 --> 00:32:26.720 or bone weakening. So you put them together. 00:32:26.859 --> 00:32:28.900 And you have a child with incredibly fragile 00:32:28.900 --> 00:32:32.339 bones. So the safety point is, handle them gently. 00:32:32.980 --> 00:32:35.900 No rough housing. They are at a very high risk 00:32:35.900 --> 00:32:38.420 for pathological fractures. And let's synthesize 00:32:38.420 --> 00:32:40.579 pain management across the board, because it 00:32:40.579 --> 00:32:43.160 can be confusing. It is. So let's simplify. For 00:32:43.160 --> 00:32:45.779 sickle cell, opioids are the standard of care. 00:32:46.259 --> 00:32:48.920 Do not fear addiction. Treat the crisis. OK. 00:32:49.079 --> 00:32:52.609 For hemophilia, Tylenol is safe. NSAIDs like 00:32:52.609 --> 00:32:55.809 ibuprofen or aspirin are dangerous because of 00:32:55.809 --> 00:32:58.269 the bleeding risk. And for leukemia. For leukemia, 00:32:58.490 --> 00:33:00.450 you can use Tylenol for pain, but UT, you have 00:33:00.450 --> 00:33:03.430 to be careful. A Tylenol masks fever. And in 00:33:03.430 --> 00:33:05.950 a neutropenic kid, fever is the only warning 00:33:05.950 --> 00:33:08.369 sign you might get of a deadly sepsis. So you 00:33:08.369 --> 00:33:10.650 might hold it. We often hold Tylenol until we 00:33:10.650 --> 00:33:12.789 have thoroughly assessed for infection. We need 00:33:12.789 --> 00:33:14.990 to see that fever if it's there. That is the 00:33:14.990 --> 00:33:17.920 kind of nuance that really separates. a novice 00:33:17.920 --> 00:33:20.180 from an expert. It's all about knowing the why 00:33:20.180 --> 00:33:22.660 behind the rule. So we've built the map. We've 00:33:22.660 --> 00:33:24.880 covered the diseases, the meds, the connections. 00:33:25.279 --> 00:33:26.960 What does this all mean for the listener standing 00:33:26.960 --> 00:33:29.319 at the nurses station? It means that when you 00:33:29.319 --> 00:33:31.740 review a chart, you aren't just checking boxes. 00:33:31.940 --> 00:33:34.829 You are looking for the safety patterns. Is the 00:33:34.829 --> 00:33:37.930 airway safe? Is the immune system working? What's 00:33:37.930 --> 00:33:41.109 the ANC? Is the blood clotting? What are the 00:33:41.109 --> 00:33:44.250 platelets and factors? And critically, is this 00:33:44.250 --> 00:33:47.250 child able to be a child? That brings us to our 00:33:47.250 --> 00:33:50.089 final thought. The resilience of the pediatric 00:33:50.089 --> 00:33:53.529 patient versus the fragility of their physiology. 00:33:53.720 --> 00:33:56.319 It's the great paradox of pediatrics. Kids bounce 00:33:56.319 --> 00:33:58.480 back emotionally and physically from things that 00:33:58.480 --> 00:34:01.619 would absolutely crush an adult, but their physiology 00:34:01.619 --> 00:34:04.019 has so much less reserve, they crash faster. 00:34:04.720 --> 00:34:06.119 So what's the provocative thought you want to 00:34:06.119 --> 00:34:07.880 leave everyone with? The thought I want to leave 00:34:07.880 --> 00:34:10.260 you with is this. We spent so much time treating 00:34:10.260 --> 00:34:12.440 the numbers, getting the HGB up, getting the 00:34:12.440 --> 00:34:15.639 ANC up, but the patient, the person, is trying 00:34:15.639 --> 00:34:18.500 to grow up. Every time you intervene, whether 00:34:18.500 --> 00:34:21.260 it's a hospital admission, a steroid course that 00:34:21.260 --> 00:34:23.440 changes their face, or a restriction on sports, 00:34:23.860 --> 00:34:26.139 you are altering the architecture of their childhood. 00:34:26.440 --> 00:34:28.800 So the challenges? The challenges. How do we 00:34:28.800 --> 00:34:31.559 keep them safe? That's our 80 -20 clinical focus. 00:34:31.940 --> 00:34:34.639 Well, disrupting their development as little 00:34:34.639 --> 00:34:37.119 as humanly possible. So when you review a chart, 00:34:37.179 --> 00:34:39.949 ask, Is this safe? And then immediately ask, 00:34:40.329 --> 00:34:42.230 how does this affect their next birthday party? 00:34:42.389 --> 00:34:44.989 Can they go? If you can answer both of those 00:34:44.989 --> 00:34:47.489 questions, you're doing the job right. That is 00:34:47.489 --> 00:34:49.550 powerful. Can they go to the party? That's the 00:34:49.550 --> 00:34:52.010 real goal. Thank you for joining us on this deep 00:34:52.010 --> 00:34:55.389 dive into pediatric hemionc. It's a beast of 00:34:55.389 --> 00:34:57.949 a topic, but hopefully the 80 -20 map makes a 00:34:57.949 --> 00:35:00.150 little more navigable. Keep looking for the patterns. 00:35:00.429 --> 00:35:02.449 Safety first, development always. See you on 00:35:02.449 --> 00:35:03.090 the next dive.