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 --> 00:00:47.049 fast. Welcome to the emergency room. Welcome 00:00:47.049 --> 00:00:50.329 back to the deep dive today. Today is a big one. 00:00:50.329 --> 00:00:53.600 I'm sitting here looking at this. stack of materials 00:00:53.600 --> 00:00:55.719 we've uploaded. It's a mountain. It really is. 00:00:55.799 --> 00:00:58.939 We've got textbook chapters on cellular regulation 00:00:58.939 --> 00:01:01.840 that seem to go on forever, drug monographs that 00:01:01.840 --> 00:01:03.320 look like they were written in ancient Greek, 00:01:03.960 --> 00:01:07.730 nursing care guides, lecture notes. Honestly, 00:01:07.870 --> 00:01:11.409 it's intimidating. It is. We are diving headfirst 00:01:11.409 --> 00:01:15.109 into pediatric hematology and oncology. And it's 00:01:15.109 --> 00:01:17.989 a massive topic. I won't sugarcoat it. It's one 00:01:17.989 --> 00:01:20.709 of those areas in nursing school and in practice 00:01:20.709 --> 00:01:23.849 where the stakes feel incredibly high. We're 00:01:23.849 --> 00:01:25.950 dealing with children, often very sick children, 00:01:26.170 --> 00:01:29.430 and the biology is complex. Yeah. But, you know, 00:01:29.709 --> 00:01:31.370 despite the volume of information you're looking 00:01:31.370 --> 00:01:34.189 at, there is a very clear structure to it. OK. 00:01:34.329 --> 00:01:35.969 if you know how to read them out. And that is 00:01:35.969 --> 00:01:37.810 exactly our mission today. We are going to take 00:01:37.810 --> 00:01:40.010 this mountain of medical literature, and we are 00:01:40.010 --> 00:01:42.409 going to apply the Pareto principle. We are hunting 00:01:42.409 --> 00:01:45.469 for the 20 % of the information that answers 00:01:45.469 --> 00:01:48.909 80 % of the exam questions and, more importantly, 00:01:49.189 --> 00:01:51.250 keeps these kids safe when you're actually at 00:01:51.250 --> 00:01:54.390 the bedside. That's the goal. And I want to frame 00:01:54.390 --> 00:01:56.629 this entire deep dive from the perspective of 00:01:56.629 --> 00:01:59.329 an exam coach. OK, I like that. I'm not just 00:01:59.329 --> 00:02:01.250 going to read the textbook definitions to you. 00:02:01.469 --> 00:02:05.390 That's useless. We're going to deconstruct why 00:02:05.390 --> 00:02:08.969 exam writers test certain things, what the specific 00:02:08.969 --> 00:02:11.789 safety traps are, and how to spot the patterns 00:02:11.789 --> 00:02:14.229 that repeat over and over again. I love that 00:02:14.229 --> 00:02:16.110 approach, because when I was reading through 00:02:16.110 --> 00:02:18.870 the notes on leukemia or sickle cell, I found 00:02:18.870 --> 00:02:21.050 myself just getting lost in the weeds of cell 00:02:21.050 --> 00:02:23.810 counts and protocol. It's so easy to do. So. 00:02:24.000 --> 00:02:26.259 We need a roadmap. You're saying we're going 00:02:26.259 --> 00:02:28.240 to start with what you call the master 80 -20 00:02:28.240 --> 00:02:31.680 map to find the big patterns. Exactly. Find the 00:02:31.680 --> 00:02:34.060 patterns first. Then we'll go system by system 00:02:34.060 --> 00:02:36.699 anemia, bleeding disorders, and then the big 00:02:36.699 --> 00:02:39.319 one, cancer. And we have a dedicated segment 00:02:39.319 --> 00:02:41.580 on pharmacology called The Chemo Man, which I'm 00:02:41.580 --> 00:02:43.599 actually really curious about. Oh, yeah. We'll 00:02:43.599 --> 00:02:45.840 get to him. And then we'll wrap up with oncologic 00:02:45.840 --> 00:02:48.759 emergencies. And throughout all of this, we have 00:02:48.759 --> 00:02:51.900 to keep one thread running through every single 00:02:51.900 --> 00:02:56.000 topic, development. You cannot treat a child 00:02:56.000 --> 00:02:59.680 like a small adult. The sources are so emphatic 00:02:59.680 --> 00:03:03.580 about this. The disease impacts the child's development, 00:03:04.319 --> 00:03:07.319 and where the child developmentally impacts how 00:03:07.319 --> 00:03:10.360 we treat the disease. So let's unpack this. Before 00:03:10.360 --> 00:03:12.840 we get into the nitty gritty of specific diseases, 00:03:13.439 --> 00:03:16.419 you mentioned there are four mega patterns or 00:03:16.419 --> 00:03:18.819 buckets that pretty much everything in this stack 00:03:18.819 --> 00:03:21.719 fits into. Right. If I'm a student walking onto 00:03:21.719 --> 00:03:24.400 the unit, What are these buckets? OK, so if you 00:03:24.400 --> 00:03:26.500 step back from the hundreds of pages, you're 00:03:26.500 --> 00:03:28.340 really only looking at four fundamental problems. 00:03:28.919 --> 00:03:31.819 The first pattern is the something is low pattern. 00:03:31.979 --> 00:03:34.719 This is anemia. OK. Red blood cells or hemoglobin 00:03:34.719 --> 00:03:37.080 are down. Simple enough. On the surface, yes. 00:03:37.340 --> 00:03:39.659 But the exam decision point, the thing the test 00:03:39.659 --> 00:03:42.639 wants you to figure out, is why is it low? OK. 00:03:42.939 --> 00:03:45.229 Is it a production problem? Is the bone marrow 00:03:45.229 --> 00:03:47.150 factory just not making enough? That's your iron 00:03:47.150 --> 00:03:50.129 deficiency or your plastic anemia. Right. Or 00:03:50.129 --> 00:03:52.270 is it a destruction problem? Is the body making 00:03:52.270 --> 00:03:54.789 them but then, you know, chewing them up? That's 00:03:54.789 --> 00:03:57.909 sickle cell or thalassemia. And clinically, if 00:03:57.909 --> 00:04:01.009 I walk into a room, what is the first thing I'm 00:04:01.009 --> 00:04:03.330 going to notice in a kid with this something 00:04:03.330 --> 00:04:07.370 is low pattern? Crackicardia. That is your priority 00:04:07.370 --> 00:04:09.830 assessment. The heart rate. The heart is the 00:04:09.830 --> 00:04:12.659 engine. If the blood can't carry enough oxygen, 00:04:12.860 --> 00:04:15.159 the heart has to pump faster and harder to compensate. 00:04:15.800 --> 00:04:18.079 It's working overtime. Okay, so that's the first 00:04:18.079 --> 00:04:20.019 vital sign I'm looking at. Absolutely. You'll 00:04:20.019 --> 00:04:22.079 probably also hear a murmur because the blood 00:04:22.079 --> 00:04:24.759 is thin and turbulent. The kid will be exhausted, 00:04:25.220 --> 00:04:28.680 pale. But tachycardia is the body's primary distress 00:04:28.680 --> 00:04:31.439 signal. Got it. Pattern number two. The bleeding 00:04:31.439 --> 00:04:33.480 pattern. This covers all your clotting disorders. 00:04:34.040 --> 00:04:36.480 The basic mechanism to stop bleeding is broken. 00:04:36.639 --> 00:04:39.100 And the decision point for the exam. The decision 00:04:39.100 --> 00:04:41.439 point here is distinguishing which part of the 00:04:41.439 --> 00:04:44.339 mechanism failed. Is it the platelets? You know, 00:04:44.379 --> 00:04:46.139 the little first responder cells? That's usually 00:04:46.139 --> 00:04:49.139 ITP. If it's platelets, you see superficial bleeding, 00:04:49.160 --> 00:04:51.660 petechiae, those little red pinpoints, or purpura. 00:04:51.920 --> 00:04:55.279 Okay, so skin level stuff. Exactly. Or is it 00:04:55.279 --> 00:04:57.560 the clotting factors? The proteins that build 00:04:57.560 --> 00:05:00.259 the strong clot? That's hemophilia. And does 00:05:00.259 --> 00:05:02.079 that look different to the naked eye? Oh, completely 00:05:02.079 --> 00:05:05.019 different. Factor issues cause deep bleeding. 00:05:05.230 --> 00:05:07.290 We're talking bleeding into the joints, into 00:05:07.290 --> 00:05:09.949 muscles, deep tissues. It's internal. You might 00:05:09.949 --> 00:05:12.310 not see anything on the skin at first. Oh. And 00:05:12.310 --> 00:05:14.810 the safety can't miss rule for this entire bucket 00:05:14.810 --> 00:05:17.689 is simple. Do not traumatize the child. Meaning 00:05:17.689 --> 00:05:20.870 what exactly? No rectal temperatures ever. No 00:05:20.870 --> 00:05:23.790 IM injections unless it's an absolute life or 00:05:23.790 --> 00:05:26.689 death emergency. And strict fall precautions, 00:05:27.230 --> 00:05:29.750 padded bed rails, grippy socks, the whole nine 00:05:29.750 --> 00:05:32.509 yards. OK. Pattern three involves the starey 00:05:32.509 --> 00:05:35.980 stuff, the C word. Yes. The mass or cellular 00:05:35.980 --> 00:05:39.800 chaos pattern, neoplasms, uncontrolled growth. 00:05:39.939 --> 00:05:42.740 And the first signs are subtle. So subtle. The 00:05:42.740 --> 00:05:44.600 priority cues are often things that could be 00:05:44.600 --> 00:05:47.420 anything else. Weight loss, pallor, maybe some 00:05:47.420 --> 00:05:50.019 swollen lymph nodes that don't hurt, or the silent 00:05:50.019 --> 00:05:52.120 abdominal mass. And the safety rule here, and 00:05:52.120 --> 00:05:54.040 I cannot stress this enough, it was everywhere 00:05:54.040 --> 00:05:56.800 in the notes, relates to a specific tumor called 00:05:56.800 --> 00:05:59.339 Wilms tumor. I saw that in the notes in all caps. 00:05:59.639 --> 00:06:02.259 Do not palpate. Exactly. We'll get into the why 00:06:02.259 --> 00:06:04.899 later, but if you palpate that abdomen, you could 00:06:04.899 --> 00:06:07.740 kill the child. It is a never event. Wow. Okay. 00:06:07.939 --> 00:06:10.420 And the fourth and final pattern. The neutropenic 00:06:10.420 --> 00:06:12.959 pattern. This is immunosuppression, whether it's 00:06:12.959 --> 00:06:14.959 from the leukemia itself crowding out the good 00:06:14.959 --> 00:06:17.939 white blood cells or the chemo just wiking them 00:06:17.939 --> 00:06:21.379 out. Right. The decision point is that fever 00:06:21.379 --> 00:06:24.759 in a cancer kid is not Just a fever. It's an 00:06:24.759 --> 00:06:27.379 emergency. It is a full -blown medical emergency. 00:06:27.560 --> 00:06:29.579 You have to treat it like sepsis until proven 00:06:29.579 --> 00:06:31.839 otherwise. We have a specific number for the 00:06:31.839 --> 00:06:34.180 danger zone, right? We do. An absolute neutrophil 00:06:34.180 --> 00:06:38.639 count, or ANC, of less than 500. 500. That is 00:06:38.639 --> 00:06:41.579 the definition of severe neutropenia. If you 00:06:41.579 --> 00:06:43.579 see that number on a lab report, your radar needs 00:06:43.579 --> 00:06:45.860 to be on high alert. OK, so we have our four 00:06:45.860 --> 00:06:49.839 pillars. Anemia, bleeding, mass, and neutropenia. 00:06:50.410 --> 00:06:52.110 That actually makes it feel manageable. It's 00:06:52.110 --> 00:06:54.069 the framework for everything. So let's zoom in 00:06:54.069 --> 00:06:56.970 on pattern one, anemia. And I want to start with 00:06:56.970 --> 00:06:58.930 a concept that I found fascinating because it 00:06:58.930 --> 00:07:00.930 sounds so innocent, but it's actually dangerous. 00:07:01.670 --> 00:07:07.069 The milk baby. Ah, the milk baby. This is a classic, 00:07:07.069 --> 00:07:09.790 classic presentation for iron deficiency anemia 00:07:09.790 --> 00:07:12.449 or IDA. So paint the picture for me. What does 00:07:12.449 --> 00:07:15.009 this kid look like? The paradox here is that 00:07:15.009 --> 00:07:18.389 the child, usually a poddler, looks chubby. They 00:07:18.389 --> 00:07:21.350 look well -fed. They might even be in a higher 00:07:21.350 --> 00:07:23.430 weight percentile. So they look healthy. They 00:07:23.430 --> 00:07:26.029 look like the picture of health. But they are 00:07:26.029 --> 00:07:28.829 pale, almost porcelain -like, and completely 00:07:28.829 --> 00:07:31.449 exhausted. Listless. They don't want to play. 00:07:31.790 --> 00:07:34.110 It feels counterintuitive, though. We're raised 00:07:34.110 --> 00:07:36.889 to think milk does a body good. You see a chubby, 00:07:36.970 --> 00:07:38.810 bouncing toddler, you think, that's a healthy 00:07:38.810 --> 00:07:41.790 kid. Why is that chubby appearance actually a 00:07:41.790 --> 00:07:44.230 warning sign here? It is the classic deception. 00:07:44.560 --> 00:07:46.259 And this is where you had to think like a nurse 00:07:46.259 --> 00:07:49.000 and separate calories from nutrients. OK. Cow's 00:07:49.000 --> 00:07:50.959 milk is high in calories and fat, which is why 00:07:50.959 --> 00:07:54.879 they bulk up. But it is virtually devoid of bioavailable 00:07:54.879 --> 00:07:57.899 iron. So you have a child who is calorically 00:07:57.899 --> 00:08:01.860 replete, but nutritionally starving for one specific 00:08:01.860 --> 00:08:04.259 critical nutrient. They're full, but they're 00:08:04.259 --> 00:08:06.959 running on empty. Exactly. And there's a mechanical 00:08:06.959 --> 00:08:09.120 issue here too. Think about a toddler's stomach. 00:08:09.339 --> 00:08:11.680 It's small, right? It's the size of their fist. 00:08:11.959 --> 00:08:15.420 Tiny. So if you fill that tank with 30, 40, even 00:08:15.420 --> 00:08:18.939 50 ounces of milk a day, which is so easy to 00:08:18.939 --> 00:08:20.860 do with a sippy cup they carry around like a 00:08:20.860 --> 00:08:23.959 security blanket. There is physically no room 00:08:23.959 --> 00:08:27.920 for broccoli or steak or iron -fortified cereal. 00:08:28.160 --> 00:08:30.459 So it's a displacement issue. The milk is pushing 00:08:30.459 --> 00:08:33.059 the iron right off the plate. That's factor one. 00:08:33.480 --> 00:08:35.419 But factor two is where the exam writers will 00:08:35.419 --> 00:08:37.539 trap you. It's not just that they aren't eating 00:08:37.539 --> 00:08:40.240 iron. Wait. They're actively losing blood. Wait. 00:08:40.519 --> 00:08:43.840 From the milk. How? Yes. And this is distinct 00:08:43.840 --> 00:08:46.679 to cow's milk in young intestines. The proteins 00:08:46.679 --> 00:08:49.240 in cow's milk can actually irritate the lining 00:08:49.240 --> 00:08:52.460 of a toddler's gut. It causes a low -grade chronic 00:08:52.460 --> 00:08:54.419 inflammatory response. Chronic allergy. You can 00:08:54.419 --> 00:08:56.399 think of it like that, yeah. A condition called 00:08:56.399 --> 00:08:58.480 allergic colitis. It's not a massive hemorrhage 00:08:58.480 --> 00:09:00.039 where you see bright red blood in the diaper 00:09:00.039 --> 00:09:02.759 every time. It's microscopic, occult bleeding. 00:09:02.940 --> 00:09:05.340 So the gut is just weeping blood. And slowly, 00:09:05.679 --> 00:09:08.399 drip by drip, day after day, you're losing hemoglobin 00:09:08.399 --> 00:09:10.700 and iron in the stool. and it's invisible to 00:09:10.700 --> 00:09:13.100 the naked eye. So you have zero intake plus active 00:09:13.100 --> 00:09:15.779 loss. That is why these kits crash so hard and 00:09:15.779 --> 00:09:18.860 so fast. That makes so much more sense now. So 00:09:18.860 --> 00:09:21.159 let's talk about the path, though, in plain language. 00:09:21.659 --> 00:09:24.500 Why does iron matter so much? I know it's for 00:09:24.500 --> 00:09:27.519 blood, but what is it actually doing? Think of 00:09:27.519 --> 00:09:30.940 iron as the magnet or the glue. It's the core 00:09:30.940 --> 00:09:33.879 component of the hemoglobin molecule that physically 00:09:33.879 --> 00:09:36.580 binds to oxygen. So it's the seat on the bus 00:09:36.580 --> 00:09:39.779 where oxygen sits. Perfect analogy. Without iron, 00:09:39.820 --> 00:09:42.580 you can make the bus the red blood cell, but 00:09:42.580 --> 00:09:45.480 there are no seats. The factory still works, 00:09:45.919 --> 00:09:48.860 but the cells it produces are useless. On a lab 00:09:48.860 --> 00:09:51.100 report, they're described as microcytic, meaning 00:09:51.100 --> 00:09:53.820 small, and hypochromic, meaning pale. Little 00:09:53.820 --> 00:09:56.000 pale cells. They can't carry the payload. It's 00:09:56.000 --> 00:09:58.080 like sending out a fleet of delivery trucks with 00:09:58.080 --> 00:10:00.460 no cargo space. And this change is based on age, 00:10:00.620 --> 00:10:02.580 right? A newborn usually has iron. They don't 00:10:02.580 --> 00:10:05.200 come out anemic. Correct. A full -term newborn 00:10:05.200 --> 00:10:07.980 has the maternal iron stores that they packed 00:10:07.980 --> 00:10:09.799 away in the third trimester. Those last about 00:10:09.799 --> 00:10:12.269 four to six months. And for a preemie. They miss 00:10:12.269 --> 00:10:14.509 that third trimester packing party. So their 00:10:14.509 --> 00:10:16.210 stores are not much faster, maybe around two 00:10:16.210 --> 00:10:18.629 months. That's why the age of presentation for 00:10:18.629 --> 00:10:20.730 IDA is usually later infancy or toddlerhood, 00:10:21.070 --> 00:10:23.269 once those stores are gone and the diet transitions 00:10:23.269 --> 00:10:26.870 to cow's milk. So for the need to know the 80 00:10:26.870 --> 00:10:29.909 -20 core for the exam, what are we looking for? 00:10:29.970 --> 00:10:32.710 What are the top findings? Top findings, tachycardia 00:10:32.710 --> 00:10:35.509 and power, obviously. But look for the weird 00:10:35.509 --> 00:10:38.470 stuff, the things that make you go, huh, spoon 00:10:38.470 --> 00:10:40.899 -shaped fingernails. The medical term is koala 00:10:40.899 --> 00:10:44.299 nikia. The nail becomes concave. And pica craving 00:10:44.299 --> 00:10:47.379 and eating non -food items. Like what? Ice is 00:10:47.379 --> 00:10:51.360 a classic one. But also dirt, clay, paint chips, 00:10:51.720 --> 00:10:54.080 laundry starch. If a parent says their toddler 00:10:54.080 --> 00:10:56.539 is eating the potted plant soil, your first thought 00:10:56.539 --> 00:10:58.840 should be to check a hemoglobin. And the priority 00:10:58.840 --> 00:11:00.480 intervention is not a blood transfusion right 00:11:00.480 --> 00:11:02.519 away, is it? Not usually. You fix the underlying 00:11:02.519 --> 00:11:05.679 problem. So diet first. You have to educate the 00:11:05.679 --> 00:11:07.799 parents. Limit that milk to 24 ounces a day. 00:11:07.840 --> 00:11:09.799 That is a hard number you need to know for your 00:11:09.799 --> 00:11:12.720 exams. 24 ounces. Got it. Introduce iron fortified 00:11:12.720 --> 00:11:16.279 cereals, pureed red meats, beans, spinach. But 00:11:16.279 --> 00:11:18.720 usually, diet alone isn't enough to replete their 00:11:18.720 --> 00:11:21.580 stores. They need oral iron supplements. And 00:11:21.580 --> 00:11:23.580 I notice in the drug guides, there are some very 00:11:23.580 --> 00:11:26.899 specific kind of fussy rules for giving oral 00:11:26.899 --> 00:11:29.379 iron. It is fussy, but it's all about chemistry. 00:11:29.559 --> 00:11:31.399 And exam writers love this stuff. So what are 00:11:31.399 --> 00:11:33.940 the rules? Rule one. Give it with vitamin C. 00:11:34.110 --> 00:11:36.509 a little bit of orange juice or some citrus fruit. 00:11:37.090 --> 00:11:39.289 An acidic environment converts the iron into 00:11:39.289 --> 00:11:42.149 a form that is much easier for the gut to absorb. 00:11:42.370 --> 00:11:44.950 Okay, so OJ with the iron drops, what's rule 00:11:44.950 --> 00:11:48.509 two? Rule two. Do not give it with milk or antacids. 00:11:48.889 --> 00:11:51.929 Calcium is the enemy of iron absorption. It binds 00:11:51.929 --> 00:11:54.070 to it and blocks it. Which is so ironic given 00:11:54.070 --> 00:11:56.470 the cause is often milk. Exactly. You have to 00:11:56.470 --> 00:11:58.899 separate them by at least an hour or two. And 00:11:58.899 --> 00:12:00.500 you have to warn the parents about the side effects, 00:12:00.639 --> 00:12:02.500 or they will stop giving it within a week. What 00:12:02.500 --> 00:12:06.100 are they? It causes GI upset nausea, constipation. 00:12:06.440 --> 00:12:08.559 It can stain teeth. So you teach them to give 00:12:08.559 --> 00:12:10.500 it through a straw or a syringe and at the back 00:12:10.500 --> 00:12:14.039 of the cheek. And the big one, it turns the stool 00:12:14.039 --> 00:12:17.159 a tarry green or black color. That's the good 00:12:17.159 --> 00:12:18.620 side effect, right? You want to see that. It 00:12:18.620 --> 00:12:20.679 is. If the poop is black, it means the iron is 00:12:20.679 --> 00:12:22.559 being absorbed and processed. If the parents 00:12:22.559 --> 00:12:24.440 call into panic saying there's blood in the stool, 00:12:24.860 --> 00:12:27.340 your first question is, what color is it? Black 00:12:27.340 --> 00:12:30.220 and terry is expected with iron. Bright red is 00:12:30.220 --> 00:12:33.159 not. Before we move on, let's circle back to 00:12:33.159 --> 00:12:36.080 development. How does this affect a growing brain? 00:12:36.320 --> 00:12:39.759 Oh, it's huge. The brain is the most oxygen -hungry 00:12:39.759 --> 00:12:42.980 organ in the body. Chronic hypoxia in a rapidly 00:12:42.980 --> 00:12:46.120 developing brain leads to measurable cognitive 00:12:46.120 --> 00:12:48.320 delays and behavioral changes. For how long? 00:12:48.720 --> 00:12:51.179 Is it permanent? There are studies showing long 00:12:51.179 --> 00:12:53.980 -term impacts on math scores, reading comprehension, 00:12:54.379 --> 00:12:57.179 and even attention span if severe idea isn't 00:12:57.179 --> 00:12:59.500 caught and treated early. And then there's the 00:12:59.500 --> 00:13:02.360 physical aspect, the fatigue. Right. If a toddler 00:13:02.360 --> 00:13:05.019 is too tired to pull to stand to cruise to run 00:13:05.019 --> 00:13:08.259 and explore their environment, their gross motor 00:13:08.259 --> 00:13:10.940 development just stalls. They don't play. They 00:13:10.940 --> 00:13:12.659 don't learn from their environment. It has a 00:13:12.659 --> 00:13:16.100 domino effect. Wow. Okay, let's shift gears from 00:13:16.100 --> 00:13:18.620 a production problem to a destruction problem. 00:13:19.390 --> 00:13:22.529 Sickle cell disease. This feels like one of the 00:13:22.529 --> 00:13:25.309 absolute pillars of pediatric nursing exams. 00:13:25.470 --> 00:13:28.409 It's a major topic for sure. Sickle cell disease 00:13:28.409 --> 00:13:31.590 or SCD is an autosomal recessive genetic disorder. 00:13:31.909 --> 00:13:34.129 Meaning both parents must be carriers. Right. 00:13:34.309 --> 00:13:36.889 Both parents carry the trait and there's a 25 00:13:36.889 --> 00:13:38.929 % chance with each pregnancy that the child will 00:13:38.929 --> 00:13:41.009 have the full disease. And the one -liner here, 00:13:41.149 --> 00:13:43.909 the core concept is the traffic jam. That's the 00:13:43.909 --> 00:13:46.629 perfect visual. Normal hemoglobin, hemoglobin 00:13:46.629 --> 00:13:49.110 A, is in a red blood cell that's round, squishy, 00:13:49.330 --> 00:13:51.629 and flexible. It can squeeze through the tiniest 00:13:51.629 --> 00:13:53.490 capillaries with no problem. Like a water balloon. 00:13:53.870 --> 00:13:56.850 Exactly. In SCD, the person has hemoglobin S. 00:13:57.149 --> 00:13:58.850 And under certain conditions of stress, when 00:13:58.850 --> 00:14:02.850 oxygen levels drop, HGVS polymerizes. It forms 00:14:02.850 --> 00:14:05.509 these long, rigid rods inside the cell. And that's 00:14:05.509 --> 00:14:08.269 what stretches the cell into that classic sickle 00:14:08.269 --> 00:14:10.980 or crescent shape. Right. And a rigid, pointy, 00:14:11.120 --> 00:14:13.139 crescent -shaped cell doesn't flow. It's sticky. 00:14:13.419 --> 00:14:15.539 It hooks onto other sickled cells. They clump, 00:14:15.779 --> 00:14:17.639 and they create a log jam that blocks the blood 00:14:17.639 --> 00:14:20.480 vessels. This is vaso -occlusion. I want to pause 00:14:20.480 --> 00:14:23.460 on the word crisis, because in medical textbooks, 00:14:23.480 --> 00:14:25.519 we use that word a lot. Hypertensive crisis, 00:14:25.740 --> 00:14:29.240 thyrotoxic crisis. But in sickle cell, vaso -occlusive 00:14:29.240 --> 00:14:32.679 crisis feels like a sanitized, almost sterile 00:14:32.679 --> 00:14:34.759 way of describing something horrific. It is. 00:14:34.840 --> 00:14:36.919 What is actually happening inside the body to 00:14:36.919 --> 00:14:39.399 cause that level of pain? It's a traffic jam. 00:14:39.519 --> 00:14:42.120 But that metaphor is almost too soft. Imagine 00:14:42.120 --> 00:14:44.759 your blood vessels are a highway, and suddenly 00:14:44.759 --> 00:14:48.070 the road is blocked. Not with cars. but with 00:14:48.070 --> 00:14:50.950 concrete barriers. The blood flow stops completely. 00:14:51.190 --> 00:14:53.769 The tissue beyond that roadblock, whether it's 00:14:53.769 --> 00:14:56.250 bone marrow, muscle, or organ tissue, starts 00:14:56.250 --> 00:14:58.730 to starve for oxygen. It's ischemia. The cells 00:14:58.730 --> 00:15:00.730 are screaming for oxygen, and they start to die. 00:15:00.809 --> 00:15:03.210 Ischemia is tissue death. Essentially. It's the 00:15:03.210 --> 00:15:05.129 exact same mechanism as a heart attack, but it's 00:15:05.129 --> 00:15:07.370 happening in your femur, or your spine, or your 00:15:07.370 --> 00:15:11.330 spleen. The pain isn't a simple ouch. It is bone 00:15:11.330 --> 00:15:14.500 crushing. Patients describe it as deeper and 00:15:14.500 --> 00:15:16.919 more intense than almost any other pain imaginable. 00:15:17.059 --> 00:15:19.460 Which explains why our notes emphasize pain management 00:15:19.460 --> 00:15:21.899 so heavily. I was struck by the dosage recommendations 00:15:21.899 --> 00:15:24.879 here. We're not talking about Tylenol. No. And 00:15:24.879 --> 00:15:27.840 this is a major advocacy point for nurses. There 00:15:27.840 --> 00:15:30.320 is, unfortunately, a stigma with sickle cell 00:15:30.320 --> 00:15:32.700 patients seeking opioid medications. You hear 00:15:32.700 --> 00:15:35.360 about drug seeking. Right. And you have to throw 00:15:35.360 --> 00:15:36.899 that out the window. You have to understand, 00:15:37.259 --> 00:15:39.320 this child is having a heart attack in their 00:15:39.320 --> 00:15:42.240 skeleton. We are talking high dose IV opioids, 00:15:42.460 --> 00:15:46.269 morphine. Hydromorphone, which is deloaded, usually 00:15:46.269 --> 00:15:50.129 via a PCA, a patient -controlled analgesia pump. 00:15:51.389 --> 00:15:54.230 The absolute key for the exam and for real life 00:15:54.230 --> 00:15:56.590 is the schedule. You don't wait for them to ask. 00:15:56.769 --> 00:15:59.830 Never. Never. If you see an order that says PRN 00:15:59.830 --> 00:16:02.470 as needed for a sickle cell crisis, you need 00:16:02.470 --> 00:16:04.710 to question that order. You are always chasing 00:16:04.710 --> 00:16:07.070 the pain. You need to be ahead of it. Scheduled 00:16:07.070 --> 00:16:09.169 around the clock dosing is the standard of care. 00:16:09.350 --> 00:16:11.769 What about the triggers? The exam usually asks 00:16:11.769 --> 00:16:14.350 what happened right before the crisis? What pushes 00:16:14.350 --> 00:16:16.529 them over the edge? We call them stressors. If 00:16:16.529 --> 00:16:18.590 it's anything that increases the body's demand 00:16:18.590 --> 00:16:22.009 for oxygen or alters their fluid status, epoxy 00:16:22.009 --> 00:16:24.029 is the obvious one. Dehydration is a massive 00:16:24.029 --> 00:16:26.549 one. Why dehydration? Because it makes the blood 00:16:26.549 --> 00:16:30.289 more viscous. It gets sludgy. It's thicker, which 00:16:30.289 --> 00:16:32.470 makes it even easier for those sticky cells to 00:16:32.470 --> 00:16:36.009 jam up. Fever or any kind of infection is a huge 00:16:36.009 --> 00:16:38.190 trigger. And cold? Yes. Cold temperatures are 00:16:38.190 --> 00:16:41.029 a big one because cold causes vasoconstriction, 00:16:41.169 --> 00:16:43.789 which narrows the vessels and creates a bottleneck. 00:16:44.169 --> 00:16:47.419 So for the 8020 core, We have a kid who comes 00:16:47.419 --> 00:16:51.039 into the ER in a vaso occlusive crisis. They 00:16:51.039 --> 00:16:54.919 are screaming in pain. What are our priority 00:16:54.919 --> 00:16:57.220 assessments? Pain score is vital, of course, 00:16:57.279 --> 00:16:59.720 but you need to assess their respiratory status 00:16:59.720 --> 00:17:02.899 immediately. Why? We are terrified of a complication 00:17:02.899 --> 00:17:05.460 called acute chest syndrome. OK. It looks just 00:17:05.460 --> 00:17:07.920 like pneumonia. They get chest pain, fever, a 00:17:07.920 --> 00:17:10.920 cough. They become hypoxic. It's sickling in 00:17:10.920 --> 00:17:13.380 the vasculature of the lungs, and it is the leading 00:17:13.380 --> 00:17:15.660 cause of death and sickle cell disease. Wow. 00:17:15.740 --> 00:17:17.619 What else are you assessing? You also need to 00:17:17.619 --> 00:17:19.980 palpate the abdomen, specifically over the spleen 00:17:19.980 --> 00:17:23.180 in the left upper quadrant. The spleen. Splenic 00:17:23.180 --> 00:17:25.539 sequestration. This is another emergency. The 00:17:25.539 --> 00:17:27.660 sickled cells can get trapped in the spleen causing 00:17:27.660 --> 00:17:30.039 it to swell up rapidly like a sponge. And that's 00:17:30.039 --> 00:17:32.759 bad. Because it pulls a huge volume of blood 00:17:32.759 --> 00:17:35.180 out of the general circulation. The child can 00:17:35.180 --> 00:17:37.980 go into hypovolemic shock in a matter of minutes. 00:17:38.460 --> 00:17:41.400 So if you feel a massive hard spleen and the 00:17:41.400 --> 00:17:43.839 kid is hypotensive with a high heart rate, that's 00:17:43.839 --> 00:17:45.890 a surgical emergency. Let's talk interventions. 00:17:46.150 --> 00:17:48.910 There's an acronym here in the notes, HOP. HOP. 00:17:49.369 --> 00:17:51.970 It stands for hydration, oxygen, and pain management. 00:17:52.410 --> 00:17:54.769 Some people teach HOK to it. OK, so hydration 00:17:54.769 --> 00:17:57.670 first. Hydration is absolutely critical. We run 00:17:57.670 --> 00:18:00.069 IV fluids at one and a half to two times the 00:18:00.069 --> 00:18:02.190 normal maintenance rate. We need to dilute the 00:18:02.190 --> 00:18:04.630 blood, make it less viscous, and help flush those 00:18:04.630 --> 00:18:06.650 sickled cells through the logjam. What about 00:18:06.650 --> 00:18:10.640 oxygen? Only if they are hypoxic. If their oxygen 00:18:10.640 --> 00:18:13.700 saturation is low, then yes, oxygen helps to 00:18:13.700 --> 00:18:16.220 reverse the sickling process. But giving oxygen 00:18:16.220 --> 00:18:18.539 to a kid who isn't hypoxic doesn't really help 00:18:18.539 --> 00:18:21.400 and isn't the priority. Hydration and pain control 00:18:21.400 --> 00:18:23.380 are the drivers. And there is a specific drug 00:18:23.380 --> 00:18:25.859 warning here in the notes regarding pain, a big 00:18:25.859 --> 00:18:30.079 do not use E. Yes, no meparidine. The brand name 00:18:30.079 --> 00:18:33.380 is Demerol. Do not give it. It has a toxic metabolite 00:18:33.380 --> 00:18:36.460 called Normapyridine that can build up, especially 00:18:36.460 --> 00:18:39.819 with repeated doses. It's a neurotoxin. It lowers 00:18:39.819 --> 00:18:42.579 the seizure threshold and can cause tremors and 00:18:42.579 --> 00:18:44.839 seizures. And kids with SED are already at risk 00:18:44.839 --> 00:18:48.000 for strokes. Exactly. So we never use a medication 00:18:48.000 --> 00:18:49.839 that could further destabilize their central 00:18:49.839 --> 00:18:52.700 nervous system. Speaking of CNS issues, let's 00:18:52.700 --> 00:18:55.019 talk about stroke. That's a huge complication. 00:18:55.140 --> 00:18:57.660 It's a devastating complication. The sickled 00:18:57.660 --> 00:19:00.539 cells block the blood flow to the brain, causing 00:19:00.539 --> 00:19:03.880 an ischemic stroke. This is why we do routine 00:19:03.880 --> 00:19:07.119 transcranial Doppler, or TCD, ultrasound screening 00:19:07.119 --> 00:19:10.039 annually for kids with SED, usually from ages 00:19:10.039 --> 00:19:12.319 2 to 16. And what are you looking for with that? 00:19:12.619 --> 00:19:14.559 We're looking for high velocity blood flow in 00:19:14.559 --> 00:19:17.119 the cerebral arteries. If the blood is moving 00:19:17.119 --> 00:19:19.420 really fast through a certain area, it means 00:19:19.420 --> 00:19:21.680 that vessel is probably narrowed. That's a high 00:19:21.680 --> 00:19:24.160 -risk sign. And those kids might be started on 00:19:24.160 --> 00:19:26.160 chronic blood transfusion therapy to prevent 00:19:26.160 --> 00:19:28.759 a stroke. And developmentally, how does a lifetime 00:19:28.759 --> 00:19:31.519 of this impact a child? It disrupts everything. 00:19:32.119 --> 00:19:34.539 We have delayed physical growth and delayed sexual 00:19:34.539 --> 00:19:37.180 maturation because of the chronic anemia and 00:19:37.180 --> 00:19:40.019 poor oxygenation. And socially? Socially, it's 00:19:40.019 --> 00:19:42.420 incredibly isolating. These kids have frequent 00:19:42.420 --> 00:19:45.099 painful hospitalizations. They miss school. They 00:19:45.099 --> 00:19:47.500 miss birthday parties. It's hard to form and 00:19:47.500 --> 00:19:49.920 maintain peer relationships. Is there an early 00:19:49.920 --> 00:19:51.920 developmental sign we should look for in babies? 00:19:52.259 --> 00:19:56.180 Yes. In infants, the first sign of SED is often 00:19:56.180 --> 00:19:59.299 dactylitis, also called Hanfoot syndrome. What's 00:19:59.299 --> 00:20:02.440 that? It's a vaso -occlusive crisis in the small 00:20:02.440 --> 00:20:05.000 bones of the hands and feet. They get symmetrically 00:20:05.000 --> 00:20:08.490 swollen, red, and are incredibly painful. An 00:20:08.490 --> 00:20:10.750 infant who should be learning to grasp or crawl 00:20:10.750 --> 00:20:13.029 suddenly won't use their hands or bear weight 00:20:13.029 --> 00:20:16.009 on their feet. It hinders those early motor milestones 00:20:16.009 --> 00:20:18.829 right from the start. Okay, moving to our second 00:20:18.829 --> 00:20:21.450 pattern, bleeding. Let's start with hemophilia. 00:20:21.670 --> 00:20:24.549 The missing factor disorder. The classic genetics 00:20:24.549 --> 00:20:27.349 are X -linked recessive. So the mom is the carrier 00:20:27.349 --> 00:20:30.349 and the son has the disease. That's the textbook 00:20:30.349 --> 00:20:33.369 exam scenario. Yeah. Hemophilia A is a deficiency 00:20:33.369 --> 00:20:35.880 of factor 8. and that accounts for about 80 % 00:20:35.880 --> 00:20:38.700 of cases. Haemophilia B, which is sometimes called 00:20:38.700 --> 00:20:41.119 Christmas disease, is a deficiency of factor 00:20:41.119 --> 00:20:45.619 9. Can you explain the why briefly? Why does 00:20:45.619 --> 00:20:48.319 missing just one of these dozens of factors cause 00:20:48.319 --> 00:20:50.920 such a profound problem? Think of the clotting 00:20:50.920 --> 00:20:54.920 cascade as a line of dominoes. To form a stable, 00:20:55.200 --> 00:20:58.339 strong, fibrin clot, you need every single domino 00:20:58.339 --> 00:21:00.940 to knock over the next one in the correct sequence. 00:21:01.079 --> 00:21:04.200 Okay. If factor 8 is missing, the dominoes stop 00:21:04.200 --> 00:21:06.380 falling right in the middle of the chain. You 00:21:06.380 --> 00:21:08.680 might form a weak little platelet plug, which 00:21:08.680 --> 00:21:10.980 is why they might stop bleeding from a tiny paper 00:21:10.980 --> 00:21:13.759 cut. But it's not enough for a real injury. Not 00:21:13.759 --> 00:21:15.759 at all. You can't form the strong fiber mesh 00:21:15.759 --> 00:21:18.160 that's needed to seal the vessel and stop deep 00:21:18.160 --> 00:21:20.579 bleeding. So that explains the clinical picture. 00:21:20.640 --> 00:21:23.220 It's not usually superficial cuts. It's deep 00:21:23.220 --> 00:21:25.480 tissue bleeding. Exactly. The hallmark finding 00:21:25.480 --> 00:21:28.019 of hemophilia is hemarthrosis bleeding into the 00:21:28.019 --> 00:21:30.700 joint cavities. The big joints are the most common 00:21:30.700 --> 00:21:33.730 targets. knees, elbows, and ankles. And the priority 00:21:33.730 --> 00:21:36.170 action when a kid comes in with a swollen, hot, 00:21:36.190 --> 00:21:39.410 painful knee. RICE. That's the acronym you need 00:21:39.410 --> 00:21:43.250 to remember. Rest, ice, compression, and elevation. 00:21:43.690 --> 00:21:47.009 Okay, RICE. Now here is the trick question I 00:21:47.009 --> 00:21:49.710 saw in the notes. Do you perform passive range 00:21:49.710 --> 00:21:53.230 of motion or ROM exercises during an active bleed? 00:21:53.589 --> 00:21:58.410 My gut says no. If I move it, I feel like I might 00:21:58.410 --> 00:22:01.250 break whatever weak clot is trying to form. Your 00:22:01.250 --> 00:22:04.450 gut is 100 % right. Absolutely not. Why? If you 00:22:04.450 --> 00:22:06.730 move a joint that is actively bleeding, you stretch 00:22:06.730 --> 00:22:09.029 the joint capsule, which increases the space 00:22:09.029 --> 00:22:11.769 for blood to pool and can dislodge any fragile 00:22:11.769 --> 00:22:14.609 clot. You immobilize it during the acute phase. 00:22:15.109 --> 00:22:17.650 ROM comes later, after the bleeding has completely 00:22:17.650 --> 00:22:19.970 stopped, to prevent long -term joint damage and 00:22:19.970 --> 00:22:21.890 contractures. And the medical management is just 00:22:21.890 --> 00:22:24.309 giving them what they're missing. Right. Factor 00:22:24.309 --> 00:22:27.009 8 or factor 9 replacement given intravenously. 00:22:27.490 --> 00:22:29.430 The key exam tip here is to always administer 00:22:29.430 --> 00:22:32.269 the factor before any invasive procedure, even 00:22:32.269 --> 00:22:34.450 a routine dental cleaning. Prophylactically. 00:22:34.670 --> 00:22:37.509 Yes. And for mild hemophilia A, there's a drug 00:22:37.509 --> 00:22:41.150 called DDAVP or desmopressin, which can be given. 00:22:41.750 --> 00:22:43.750 It works by stimulating the body to release its 00:22:43.750 --> 00:22:45.869 own stored factor 8 from the lining of blood 00:22:45.869 --> 00:22:48.210 vessels. Safety and education seem huge here. 00:22:48.250 --> 00:22:50.970 This is a lifestyle diagnosis. It is. A huge 00:22:50.970 --> 00:22:53.900 part of the nurse's role is education. No contact 00:22:53.900 --> 00:22:57.240 sports, so no football, no hockey, no wrestling. 00:22:57.579 --> 00:23:00.160 What can they do? We encourage sports that build 00:23:00.160 --> 00:23:03.140 muscle without high impact. Swimming is perfect. 00:23:03.339 --> 00:23:07.420 Golf, jogging, tennis are usually okay. They 00:23:07.420 --> 00:23:09.940 need to use a soft bristled toothbrush to prevent 00:23:09.940 --> 00:23:12.960 gum bleeding and they must wear a medical alert 00:23:12.960 --> 00:23:15.000 bracelet. And there's a big medication class 00:23:15.000 --> 00:23:19.140 to avoid. Yes, absolutely. Avoid NSAIDs and aspirin. 00:23:19.480 --> 00:23:23.089 So no Ibuprofen, no Naproxen. Why? because those 00:23:23.089 --> 00:23:25.630 drugs inhibit platelet function. The kid already 00:23:25.630 --> 00:23:27.509 has a factor problem. The last thing you want 00:23:27.509 --> 00:23:29.369 to do is give them a drug that creates a platelet 00:23:29.369 --> 00:23:32.569 problem on top of it. Acetaminophen or Tylenol 00:23:32.569 --> 00:23:35.109 is the pain reliever of choice. Developmentally, 00:23:35.109 --> 00:23:37.349 this must be so tough for toddlers. It's a nightmare 00:23:37.349 --> 00:23:39.630 for parents of toddlers. Toddlers fall. That's 00:23:39.630 --> 00:23:41.369 their job description. So you see these little 00:23:41.369 --> 00:23:43.349 kids wearing soft helmets and knee pads just 00:23:43.349 --> 00:23:44.829 to play in their own living room. It's about 00:23:44.829 --> 00:23:47.170 creating a safe environment. And for teens. For 00:23:47.170 --> 00:23:49.970 the school -aged kid and the teen, it's all about 00:23:49.970 --> 00:23:53.490 the struggle between autonomy and safety. Teaching 00:23:53.490 --> 00:23:55.690 them how to perform their own venipuncture and 00:23:55.690 --> 00:23:59.430 self -administer their 5V factor is a huge developmental 00:23:59.430 --> 00:24:02.190 milestone. It gives them control back over their 00:24:02.190 --> 00:24:04.069 bodies and their lives. Okay, let's contrast 00:24:04.069 --> 00:24:08.250 that with our other bleeding disorder, ITP, idiopathic 00:24:08.250 --> 00:24:11.829 thrombocytopenic purpura. Or immune thrombocytopenic 00:24:11.829 --> 00:24:14.329 purpura. Or immune thrombocytopenic purpura. 00:24:14.549 --> 00:24:17.369 This is the viral aftermath. What's the story 00:24:17.369 --> 00:24:19.950 here? The story is almost always the same. A 00:24:19.950 --> 00:24:21.910 perfectly healthy kid gets a cold or some other 00:24:21.910 --> 00:24:23.869 common virus, they get better. And then about 00:24:23.869 --> 00:24:25.630 two to three weeks later, they suddenly wake 00:24:25.630 --> 00:24:28.369 up covered in bruises and little red spots. What's 00:24:28.369 --> 00:24:30.450 the mechanism? Why does that happen? It's a case 00:24:30.450 --> 00:24:33.250 of mistaken identity by the immune system. The 00:24:33.250 --> 00:24:35.369 immune system ramped up to fight the virus. But 00:24:35.369 --> 00:24:37.069 after the virus is gone, it gets confused and 00:24:37.069 --> 00:24:39.130 starts producing antibodies that attack the body's 00:24:39.130 --> 00:24:41.369 own platelets. It marks them for destruction 00:24:41.369 --> 00:24:44.250 by the spleen. So the platelet count just plummets. 00:24:44.589 --> 00:24:47.009 Yeah. It crashes. We're talking below 20 ,000. 00:24:47.150 --> 00:24:50.589 Remember, normal is 150 ,000 to 400 ,000. And 00:24:50.589 --> 00:24:53.670 you see the classic signs, petechiae, which are 00:24:53.670 --> 00:24:56.289 the little pinpoint spots, and purpura, the larger 00:24:56.289 --> 00:24:58.750 purplish spots. But how is this different from, 00:24:58.750 --> 00:25:01.710 say, leukemia, which also causes low platelets? 00:25:01.829 --> 00:25:04.029 This is a key distinguishing point for exams. 00:25:04.309 --> 00:25:08.240 In ITP, the child looks well. They're not acting 00:25:08.240 --> 00:25:10.900 sick. They're happy, running around playing, 00:25:11.380 --> 00:25:13.680 but they're covered in bruises. A child with 00:25:13.680 --> 00:25:16.920 leukemia is usually pale, febrile, lethargic, 00:25:17.299 --> 00:25:20.140 and has bone pain. They look sick. And the treatment 00:25:20.140 --> 00:25:23.420 for ITP? For many kids, we just watch and wait. 00:25:23.660 --> 00:25:26.140 It's often self -resolving. If the platelets 00:25:26.140 --> 00:25:28.319 are critically low or there's active significant 00:25:28.319 --> 00:25:31.400 bleeding, we might give IVG intravenous immunoglobulin 00:25:31.400 --> 00:25:33.579 to basically distract the immune system. Or we 00:25:33.579 --> 00:25:36.089 give steroids to calm it down. And you avoid 00:25:36.089 --> 00:25:39.069 platelet transfusion. We try to. Because the 00:25:39.069 --> 00:25:41.470 spleen is primed to destroy platelets, if you 00:25:41.470 --> 00:25:43.609 give a transfusion, the spleen will just eat 00:25:43.609 --> 00:25:45.329 those new platelets immediately. It's like pouring 00:25:45.329 --> 00:25:47.509 water into a bucket with a hole in it. We only 00:25:47.509 --> 00:25:49.190 do it for a life -threatening hemorrhage, like 00:25:49.190 --> 00:25:51.289 an intracranial bleed. It's all about trauma 00:25:51.289 --> 00:25:55.549 precautions. No contact sports. quiet activities. 00:25:56.289 --> 00:25:58.650 But you don't need seizure precautions like you 00:25:58.650 --> 00:26:00.910 write with other conditions. It's strictly about 00:26:00.910 --> 00:26:03.390 not letting them bang into things until their 00:26:03.390 --> 00:26:05.329 platelet count recovers. Great. Let's move to 00:26:05.329 --> 00:26:07.849 patterns three and four combined. This is the 00:26:07.849 --> 00:26:12.849 heavy hitter leukemia, specifically AICIO and 00:26:12.849 --> 00:26:15.349 AML. This is the most common form of childhood 00:26:15.349 --> 00:26:17.549 cancer. We absolutely need to spend some quality 00:26:17.549 --> 00:26:20.160 time here. I feel like leukemia is a word everyone 00:26:20.160 --> 00:26:22.319 knows, but very few people really understand 00:26:22.319 --> 00:26:24.759 the mechanics of it. It's cancer of the blood, 00:26:24.759 --> 00:26:26.720 but what does that actually mean? The one liner 00:26:26.720 --> 00:26:28.640 you need to remember is bone marrow failure. 00:26:29.019 --> 00:26:31.539 That's the core concept. OK, explain that. The 00:26:31.539 --> 00:26:33.700 bone marrow is the factory for all of our blood 00:26:33.700 --> 00:26:37.079 cells, red cells, white cells, platelets. In 00:26:37.079 --> 00:26:40.240 leukemia, one specific cell line, usually a lymphoid 00:26:40.240 --> 00:26:43.200 or myeloid precursor, mutates and starts producing 00:26:43.200 --> 00:26:46.240 blast cells. Blast cells. Blasts are immature, 00:26:46.539 --> 00:26:49.119 baby white blood cells. completely useless. They 00:26:49.119 --> 00:26:51.019 can't fight infection. They can't do anything. 00:26:51.819 --> 00:26:55.460 But they have one terrifying superpower. They 00:26:55.460 --> 00:26:58.099 replicate incredibly fast. I call them bully 00:26:58.099 --> 00:27:00.500 cells. My bully cells. Because they don't just 00:27:00.500 --> 00:27:02.559 sit there. They take over the factory. They grow 00:27:02.559 --> 00:27:05.460 so fast and in such numbers that they physically 00:27:05.460 --> 00:27:08.420 crowd out all the normal healthy cell production 00:27:08.420 --> 00:27:10.460 lines. So the symptoms we see aren't from the 00:27:10.460 --> 00:27:12.680 cancer cells themselves, but from what the cancer 00:27:12.680 --> 00:27:15.059 pushes out of the way. Precisely. This connects 00:27:15.059 --> 00:27:17.720 right back to our master map. The blasts crowd 00:27:17.720 --> 00:27:19.980 out the red blood cell production line, and what 00:27:19.980 --> 00:27:23.680 do you get? Anemia. So pallor, fatigue. Right. 00:27:23.799 --> 00:27:25.579 They crowd out the platelet production line, 00:27:25.799 --> 00:27:29.440 and you get? Thrombocytopenia. Bruising, bleeding, 00:27:29.660 --> 00:27:32.740 petechia. And paradoxically, they crowd out the 00:27:32.740 --> 00:27:34.880 production of mature, functional white blood 00:27:34.880 --> 00:27:37.720 cells. So you get? Neutropenia. High risk of 00:27:37.720 --> 00:27:40.259 infection. So even if the lab report comes back 00:27:40.259 --> 00:27:43.140 and says the total WBC count is 50 ,000, which 00:27:43.140 --> 00:27:46.299 is huge, that kid is profoundly immunocompromised. 00:27:46.319 --> 00:27:49.559 Because it's 49 ,000 useless blast cells. Exactly. 00:27:49.619 --> 00:27:51.759 Yeah. They are cardboard cutouts of soldiers. 00:27:52.440 --> 00:27:54.579 They look the part on the roster, but they can't 00:27:54.579 --> 00:27:57.359 fight. How do we confirm the diagnosis? The gold 00:27:57.359 --> 00:27:59.859 standard, the definitive test, is the bone marrow 00:27:59.859 --> 00:28:02.380 aspiration and biopsy. Where is that done? Usually 00:28:02.380 --> 00:28:05.460 from the posterior iliac crest, the back of the 00:28:05.460 --> 00:28:08.359 hip bone. We're looking for the percentage of 00:28:08.359 --> 00:28:11.019 blasts in the marrow. A normal, healthy marrow 00:28:11.019 --> 00:28:14.400 has less than 5 % blasts. Leukemia is defined 00:28:14.400 --> 00:28:18.259 as greater than 25 % blasts. That sounds incredibly 00:28:18.259 --> 00:28:21.519 painful. It is uncomfortable, for sure. Conscious 00:28:21.519 --> 00:28:24.640 sedation is standard practice for kids. The nurse's 00:28:24.640 --> 00:28:27.339 role is critical in positioning the child, providing 00:28:27.339 --> 00:28:29.880 comfort, and then monitoring the site for bleeding 00:28:29.880 --> 00:28:32.529 afterwards. And there's another diagnostic procedure 00:28:32.529 --> 00:28:35.809 that's critical, the lumbar puncture or LP? Yes, 00:28:36.029 --> 00:28:38.869 the LP is vital. Why do we tap the spine for 00:28:38.869 --> 00:28:41.690 a blood cancer? Two crucial reasons. One, to 00:28:41.690 --> 00:28:43.930 check if the cancer has metastasized to the central 00:28:43.930 --> 00:28:46.900 nervous system, the CNS. We look for blast cells 00:28:46.900 --> 00:28:49.400 in the spinal fluid. And the second reason? To 00:28:49.400 --> 00:28:51.359 inject chemotherapy directly into the spinal 00:28:51.359 --> 00:28:53.619 fluid. This is called intrathecal chemotherapy. 00:28:53.900 --> 00:28:55.359 Why not just give all the chemo through an IV? 00:28:55.519 --> 00:28:57.740 Because of the blood -brain barrier, a protective 00:28:57.740 --> 00:28:59.880 layer that keeps toxins out of the brain, which 00:28:59.880 --> 00:29:03.079 is normally a very good thing. But it also blocks 00:29:03.079 --> 00:29:06.200 most IV chemo from getting in. So the CNS becomes 00:29:06.200 --> 00:29:09.109 what we call a sanctuary site. Leukemia cells 00:29:09.109 --> 00:29:12.049 can hide out there, survive the IV chemo that's 00:29:12.049 --> 00:29:14.309 treating the rest of the body, and then reemerge 00:29:14.309 --> 00:29:16.650 later to cause a relapse. We have to go in and 00:29:16.650 --> 00:29:19.089 treat the sanctuary directly. Okay. Let's talk 00:29:19.089 --> 00:29:21.069 about the treatment journey. It's not just take 00:29:21.069 --> 00:29:23.630 a pill for a few weeks. It's broken into phases. 00:29:23.890 --> 00:29:27.369 It is a marathon, not a sprint. Phase one is 00:29:27.369 --> 00:29:30.720 induction. The goal is absolute obliteration 00:29:30.720 --> 00:29:34.579 of the cancer. We hit them with a very intense 00:29:34.579 --> 00:29:37.539 multi -drug chemotherapy regimen to get the blast 00:29:37.539 --> 00:29:40.140 count in the marrow down to zero. This is when 00:29:40.140 --> 00:29:42.539 they are at their sickest, most vulnerable, and 00:29:42.539 --> 00:29:44.480 most neutropenic. If they achieve remission, 00:29:44.680 --> 00:29:47.160 then what? Phase two. Phase two is consolidation, 00:29:47.500 --> 00:29:50.000 sometimes called intensification. This is designed 00:29:50.000 --> 00:29:52.359 to clean up any microscopic residual disease 00:29:52.359 --> 00:29:54.640 that might be left behind. And finally, you have 00:29:54.640 --> 00:29:57.930 maintenance, which is a less intense off an outpatient 00:29:57.930 --> 00:30:00.289 chemo regimen that can last for two to three 00:30:00.289 --> 00:30:02.450 years to keep the cancer from coming back. I 00:30:02.450 --> 00:30:04.029 want to talk about the side effects from all 00:30:04.029 --> 00:30:06.009 this chemo. We mentioned moon face earlier from 00:30:06.009 --> 00:30:08.869 steroids. Yes, that's from high dose steroids 00:30:08.869 --> 00:30:11.890 like prednisone or dexamethasone. They are a 00:30:11.890 --> 00:30:14.089 key part of the chemo protocol, but they have 00:30:14.089 --> 00:30:16.369 major side effects. It changes their appearance, 00:30:16.750 --> 00:30:19.170 the chubby cheeks, weight gain, a buffalo hump 00:30:19.170 --> 00:30:21.890 on their back. For a teenager, this body image 00:30:21.890 --> 00:30:24.049 alteration is just devastating. And it affects 00:30:24.049 --> 00:30:26.680 their mood too, right? Oh, absolutely. It causes 00:30:26.680 --> 00:30:30.339 irritability, mood swings, insomnia, and a voracious 00:30:30.339 --> 00:30:32.599 appetite. They call it roid rage for a reason. 00:30:33.019 --> 00:30:35.059 And infection control is the absolute number 00:30:35.059 --> 00:30:37.799 one need to know priority intervention. Without 00:30:37.799 --> 00:30:40.160 a doubt. Neutropenic precautions. This is the 00:30:40.160 --> 00:30:42.819 bubble boy concept, but modernized for the hospital. 00:30:42.920 --> 00:30:45.440 What does that entail? No fresh flowers or plants 00:30:45.440 --> 00:30:47.839 in the room. The stagnant water and soil can 00:30:47.839 --> 00:30:50.650 harbor fungus. No raw fruits or vegetables that 00:30:50.650 --> 00:30:52.890 can't be peeled. Everyone who enters the room 00:30:52.890 --> 00:30:55.049 must wear a mask and practice meticulous hand 00:30:55.049 --> 00:30:57.390 washing. And going back to our patterns, fever 00:30:57.390 --> 00:31:00.490 is an emergency. Fever is an emergency. If a 00:31:00.490 --> 00:31:03.049 neutropenic kid spikes a temp, usually defined 00:31:03.049 --> 00:31:06.650 as a single temp greater than 38 .3C or a sustained 00:31:06.650 --> 00:31:09.930 temp of 38 .0C for an hour, you assume it is 00:31:09.930 --> 00:31:11.890 life -threatening sepsis until proven otherwise. 00:31:12.269 --> 00:31:15.579 What's the protocol? You move fast. You get blood 00:31:15.579 --> 00:31:17.559 cultures from all their central line lumens and 00:31:17.559 --> 00:31:20.140 a peripheral site. You get a urine culture, chest 00:31:20.140 --> 00:31:23.460 x -ray. You pan culture them to find the source. 00:31:23.720 --> 00:31:26.700 And you start broad spectrum IV antibiotics immediately. 00:31:26.900 --> 00:31:28.779 You don't wait for the culture results to come 00:31:28.779 --> 00:31:31.539 back. You do not wait. Time is tissue. Time is 00:31:31.539 --> 00:31:34.119 life. You have a golden hour to get those antibiotics 00:31:34.119 --> 00:31:36.599 on board. You treat first and you ask questions 00:31:36.599 --> 00:31:39.339 later. Okay, that leads us perfectly into our 00:31:39.339 --> 00:31:41.759 pharmacology section. We call this the chemo 00:31:41.759 --> 00:31:44.380 man. This is a memory tool, right? A way to link 00:31:44.380 --> 00:31:47.099 drugs to their main side effects. Yes. The chemo 00:31:47.099 --> 00:31:49.799 man is a way to visually map the specific high 00:31:49.799 --> 00:31:52.980 -yield toxicities of common chemo drugs to different 00:31:52.980 --> 00:31:55.880 body parts. The uploaded drug guides give us 00:31:55.880 --> 00:31:58.160 four of five heavy hitters we absolutely need 00:31:58.160 --> 00:31:59.980 to know for exams. Let's start at the bottom 00:31:59.980 --> 00:32:02.819 with the bladder. Cyclophosphamide, brand name 00:32:02.819 --> 00:32:05.240 Cytoxin. The target organ here is the bladder. 00:32:05.420 --> 00:32:08.500 The exam trap is hemorrhagic cystitis. Bleeding 00:32:08.500 --> 00:32:11.279 from the bladder, what causes it? This drug breaks 00:32:11.279 --> 00:32:13.759 down in the body into a toxic metabolite called 00:32:13.759 --> 00:32:16.779 acrolein. This acrolein sits in the bladder waiting 00:32:16.779 --> 00:32:19.759 to be excreted and it chemically burns the bladder 00:32:19.759 --> 00:32:22.519 lining. It causes it to bleed. How do we prevent 00:32:22.519 --> 00:32:25.279 it? Prevention is key. Number one is aggressive 00:32:25.279 --> 00:32:27.579 hydration to constantly flush the bladder out. 00:32:28.299 --> 00:32:31.470 But the specific rescue drug, the antidote, is 00:32:31.470 --> 00:32:35.329 mesna. M -E -S -N -A. Mesna. It binds to that 00:32:35.329 --> 00:32:38.430 toxic acolyte metabolite in the bladder and detoxifies 00:32:38.430 --> 00:32:41.430 it, rendering it harmless. So on an exam, if 00:32:41.430 --> 00:32:43.410 you see cyclophosphamide, you should be looking 00:32:43.410 --> 00:32:46.410 for answers that include mesna and encouraging 00:32:46.410 --> 00:32:50.210 frequent voiding. OK. Moving to the nerves. Finchristine. 00:32:50.490 --> 00:32:53.180 This is a mitotic inhibitor. To understand its 00:32:53.180 --> 00:32:55.059 side effects, you have to understand how it works. 00:32:55.460 --> 00:32:57.920 Okay. I remember mitosis from biology class cell 00:32:57.920 --> 00:33:00.480 division. How does stopping cell division lead 00:33:00.480 --> 00:33:02.799 to nerve damage? This is actually fascinating. 00:33:03.299 --> 00:33:05.599 Vincristine works by freezing the skeleton of 00:33:05.599 --> 00:33:07.680 the cell, the microtubules. It prevents them 00:33:07.680 --> 00:33:09.480 from pulling the chromosomes apart so the cell 00:33:09.480 --> 00:33:11.980 can't divide. It stops the cancer from multiplying. 00:33:12.119 --> 00:33:14.089 Okay, that's great for killing cancer. It is. 00:33:14.369 --> 00:33:17.250 But guess what other system in your body relies 00:33:17.250 --> 00:33:20.369 entirely on those same microtudial tracts? Your 00:33:20.369 --> 00:33:23.450 nervous system. Ah. Your nerves use those same 00:33:23.450 --> 00:33:26.930 tracts as a railway system to send signals and 00:33:26.930 --> 00:33:29.470 transport nutrients from your spine all the way 00:33:29.470 --> 00:33:31.890 down to your toes. So the drug can't tell the 00:33:31.890 --> 00:33:33.809 difference between a dividing cancer cell and 00:33:33.809 --> 00:33:36.650 a functioning nerve cell. Precisely. It gums 00:33:36.650 --> 00:33:39.450 up the tracts. And that's why the signature side 00:33:39.450 --> 00:33:41.970 effect of incristine is peripheral neuropathy. 00:33:42.079 --> 00:33:44.920 It attacks the longest nerves first, the ones 00:33:44.920 --> 00:33:46.640 reaching down to the feet. And that's why we 00:33:46.640 --> 00:33:49.920 see foot drop. Yes. That's a classic sign. The 00:33:49.920 --> 00:33:51.559 nerve signal just doesn't get to the muscles 00:33:51.559 --> 00:33:53.900 in the foot. The kid tries to lift their toes 00:33:53.900 --> 00:33:56.599 to take a step, and they can't. They might start 00:33:56.599 --> 00:33:58.480 tripping a lot. They might complain of numbness, 00:33:58.700 --> 00:34:01.220 tingling, or burning pain. And there's a GI component, 00:34:01.460 --> 00:34:04.299 too. Yes, for the same reason. It causes a paralytic 00:34:04.299 --> 00:34:08.019 alias, severe constipation. The nerves that control 00:34:08.019 --> 00:34:11.760 gut motility stop firing. These kids need a proactive 00:34:11.760 --> 00:34:14.000 bowel regimen from day one. And there's a massive 00:34:14.000 --> 00:34:16.199 safety alert in the notes about this drug. A 00:34:16.199 --> 00:34:19.099 huge one. Vincristine is a vesicant, so you have 00:34:19.099 --> 00:34:21.900 to watch that IV line carefully for infiltration. 00:34:22.320 --> 00:34:25.679 But more importantly, it is fatal if given intrathecly. 00:34:25.820 --> 00:34:28.280 So it can never go in the spine. It is for IV 00:34:28.280 --> 00:34:31.400 use only. If you accidentally inject Vincristine 00:34:31.400 --> 00:34:34.420 into the spinal fluid, it causes ascending paralysis 00:34:34.420 --> 00:34:37.440 and destroys the central nervous system. It is 00:34:37.440 --> 00:34:40.570 an agonizing and irreversible death. Hospitals 00:34:40.570 --> 00:34:42.710 have multiple safety checks in place to prevent 00:34:42.710 --> 00:34:45.630 this. Wow. Okay. Noting that in big red letters. 00:34:46.250 --> 00:34:49.190 Moving up to the heart. Doxorubicin, often called 00:34:49.190 --> 00:34:51.909 Adriamycin. The red devil. The target organ is 00:34:51.909 --> 00:34:55.300 the heart. The toxicity is... Cardiotoxicity. 00:34:55.420 --> 00:34:57.800 How does it damage the heart? It generates free 00:34:57.800 --> 00:34:59.840 radicals that damage the cardiac muscle cells. 00:35:00.440 --> 00:35:02.840 This damage is cumulative over a lifetime. So 00:35:02.840 --> 00:35:05.880 we track the total lifetime dose a patient receives. 00:35:06.219 --> 00:35:08.380 It can lead to cardiomyopathy and congestive 00:35:08.380 --> 00:35:10.659 heart failure, sometimes years after treatment 00:35:10.659 --> 00:35:12.579 has ended. So we're monitoring for signs of heart 00:35:12.579 --> 00:35:15.079 failure. Exactly. Edema, shortness of breath, 00:35:15.480 --> 00:35:18.139 crackles in the lungs, regular echocardiograms 00:35:18.139 --> 00:35:19.559 are part of the surveillance. And it changes 00:35:19.559 --> 00:35:23.010 the color of body fluids. It does. It turns urine 00:35:23.010 --> 00:35:25.650 and sweat, a bright ruby red or orange color, 00:35:25.949 --> 00:35:28.530 for a day or two after infusion. This is not 00:35:28.530 --> 00:35:30.750 bleeding, it's just the dye from the drug being 00:35:30.750 --> 00:35:32.989 excreted. You have to warn the parents and the 00:35:32.989 --> 00:35:35.250 child so they don't panic thinking they're peeing 00:35:35.250 --> 00:35:38.750 blood. Okay, last one on the chemoman. Methotrexate. 00:35:39.130 --> 00:35:42.329 A real workhorse drug in pediatric oncology. 00:35:42.949 --> 00:35:46.030 The primary targets are the kidneys and the reproductive 00:35:46.030 --> 00:35:48.150 system. How does it work? It's an anti -folate. 00:35:48.230 --> 00:35:50.769 It starves the rapidly dividing cancer cells 00:35:50.769 --> 00:35:53.429 of folic acid, which they need to make new DNA. 00:35:53.570 --> 00:35:55.929 And there's a rescue drug for this one too. Yes. 00:35:56.610 --> 00:35:59.130 Leucovorin, which is a form of flenic acid. We 00:35:59.130 --> 00:36:01.250 give high dose methotrexate to kill the cancer. 00:36:01.570 --> 00:36:04.409 And then we rescue the normal healthy cells by 00:36:04.409 --> 00:36:06.650 giving them leucovorin a few hours later. It's 00:36:06.650 --> 00:36:09.070 a very precisely timed dance. And for nursing 00:36:09.070 --> 00:36:11.829 interventions, what's the priority? Kidney protection. 00:36:12.510 --> 00:36:15.269 Intense Favi hydration is critical, and we have 00:36:15.269 --> 00:36:17.329 to alkalinize the urine, usually with sodium 00:36:17.329 --> 00:36:19.789 bicarbonate in the IV fluids. Why do we want 00:36:19.789 --> 00:36:22.710 alkaline urine? We need the urine pH to be greater 00:36:22.710 --> 00:36:26.590 than 7 .0. If the urine is acidic, methotrexate 00:36:26.590 --> 00:36:28.809 will crystallize in the retal tubules. It literally 00:36:28.809 --> 00:36:31.730 forms tiny sharp crystals like shards of glass 00:36:31.730 --> 00:36:35.469 and causes acute renal failure. So we're constantly 00:36:35.469 --> 00:36:38.590 checking urine pH. That is a solid pharmacology 00:36:38.590 --> 00:36:41.539 review. Let's move to section D. Solid tumors. 00:36:42.599 --> 00:36:44.860 We need to distinguish between two abdominal 00:36:44.860 --> 00:36:48.139 tumors that often get confused on exams, Wilms 00:36:48.139 --> 00:36:51.079 tumor and neuroblastoma. Yes, let's set up a 00:36:51.079 --> 00:36:53.820 comparison table in our minds. First, Wilms tumor, 00:36:54.099 --> 00:36:56.239 also called nephroblastoma. So a kidney tumor. 00:36:56.340 --> 00:36:58.360 Right, it's a tumor of the kidney. The peak age 00:36:58.360 --> 00:37:01.099 is around two to five years old. And critically, 00:37:01.539 --> 00:37:03.880 it is typically unilateral. It arises in one 00:37:03.880 --> 00:37:06.119 kidney and usually does not cross the midline 00:37:06.119 --> 00:37:08.000 of the abdomen. And the non -negotiable safety 00:37:08.000 --> 00:37:09.800 rule we mentioned at the very beginning. Do not 00:37:09.800 --> 00:37:12.059 palpate the abdomen. You put a sign on the bed. 00:37:12.519 --> 00:37:15.000 You put it in the chart. You tell every single 00:37:15.000 --> 00:37:17.039 person who comes near that child, do not palpate. 00:37:17.099 --> 00:37:19.039 Let's really break down the why. Is it because 00:37:19.039 --> 00:37:21.579 it's painful? It's not primarily about pain. 00:37:21.619 --> 00:37:25.139 It's about containment. This tumor is encapsulated. 00:37:25.420 --> 00:37:29.039 It's enclosed in a thin, fragile sac. If you 00:37:29.039 --> 00:37:31.079 push on it, You can rupture that capsule. And 00:37:31.079 --> 00:37:33.599 what happens then? You seed cancer cells all 00:37:33.599 --> 00:37:36.780 over the abdominal cavity. You take a stage one 00:37:36.780 --> 00:37:39.420 easily treatable tumor and you instantly turn 00:37:39.420 --> 00:37:41.760 it into a stage three or stage four disaster 00:37:41.760 --> 00:37:44.219 that requires much more aggressive chemo and 00:37:44.219 --> 00:37:47.079 radiation. You can worsen their prognosis with 00:37:47.079 --> 00:37:50.340 one push. Now contrast that with neuroblastoma. 00:37:50.500 --> 00:37:53.579 Neuroblastoma arises from primitive cells of 00:37:53.579 --> 00:37:55.940 the sympathetic nervous system. Most commonly, 00:37:56.079 --> 00:37:57.699 it starts in the adrenal medulla, which is that 00:37:57.699 --> 00:37:59.260 little gland that sits on top of the kidney. 00:37:59.360 --> 00:38:01.340 And this one is different because? Because it 00:38:01.340 --> 00:38:03.500 is a silent tumor. It's often called that because 00:38:03.500 --> 00:38:05.980 by the time it's diagnosed, it is usually already 00:38:05.980 --> 00:38:08.019 metastasized. It's a much more aggressive cancer 00:38:08.019 --> 00:38:10.519 than Wilms. How does it look different on physical 00:38:10.519 --> 00:38:13.159 exam? The abdominal mass itself often crosses 00:38:13.159 --> 00:38:16.159 the midline. Wilms tumor usually respects the 00:38:16.159 --> 00:38:17.860 midline because it's confined to one kidney. 00:38:18.300 --> 00:38:20.320 Neuroblastoma is more diffuse and grows across 00:38:20.320 --> 00:38:22.420 the belly. And because it's metastatic, you see 00:38:22.420 --> 00:38:25.239 signs elsewhere. Exactly. You see the classic 00:38:25.239 --> 00:38:27.840 raccoon eyes, period orbital eczemosis, which 00:38:27.840 --> 00:38:30.559 is bruising around the eyes from orbital metastasis. 00:38:30.900 --> 00:38:33.139 You might also see bone pain from bone mets. 00:38:33.599 --> 00:38:36.960 And the lab test is different. Yes. The key diagnostic 00:38:36.960 --> 00:38:40.739 lab is a urine test for catecholamines, specifically 00:38:40.739 --> 00:38:44.900 VMA and HVA. Because this is a tumor of the adrenal 00:38:44.900 --> 00:38:47.460 sympathetic system, it often pumps out massive 00:38:47.460 --> 00:38:49.849 amounts of stress hormones. You'll need to do 00:38:49.849 --> 00:38:52.530 a 24 -hour urine collection to catch these metabolites. 00:38:52.769 --> 00:38:55.869 Moving up to the eye, retinoblastoma. The eye 00:38:55.869 --> 00:38:57.969 tumor. The key assessment cue you need to know 00:38:57.969 --> 00:39:00.730 is the cat's eye reflex. The medical term is 00:39:00.730 --> 00:39:03.389 leukocoria. What does that mean? Normally when 00:39:03.389 --> 00:39:05.489 you shine a light in a pupil like for a photograph 00:39:05.489 --> 00:39:09.250 with a flash, you see the red reflex. In retinoblastoma, 00:39:09.489 --> 00:39:11.329 the tumor is growing in the back of the eye and 00:39:11.329 --> 00:39:13.429 it reflects the light back as a white or yellowish 00:39:13.429 --> 00:39:15.980 glow. And the exam trap here. The trap is thinking 00:39:15.980 --> 00:39:18.619 that blindness or a painful red eye is an early 00:39:18.619 --> 00:39:21.539 sign. Those are late signs. The white glow, the 00:39:21.539 --> 00:39:23.940 leukocoria, is the early sign. Parents are often 00:39:23.940 --> 00:39:25.559 the first to notice it when looking at photos 00:39:25.559 --> 00:39:28.000 of their child. One eye is red, the other is 00:39:28.000 --> 00:39:31.019 white. OK, let's briefly touch on the two main 00:39:31.019 --> 00:39:35.219 bone tumors, osteosarcoma versus Ewing's sarcoma. 00:39:35.460 --> 00:39:37.360 Osteosarcoma is the most common. It typically 00:39:37.360 --> 00:39:39.699 occurs in the long bones, especially during the 00:39:39.699 --> 00:39:42.059 adolescent growth spurt. The most common site 00:39:42.059 --> 00:39:44.780 is the distal femur, right near the knee. On 00:39:44.780 --> 00:39:47.440 an x -ray, it has a classic sunburst appearance. 00:39:47.579 --> 00:39:50.900 And the treatment is pretty drastic. It often 00:39:50.900 --> 00:39:53.719 is. The tragic part is that it frequently requires 00:39:53.719 --> 00:39:57.059 amputation of the limb or a complex limb salvage 00:39:57.059 --> 00:39:59.880 surgery. Dealing with amputation in an adolescent... 00:39:59.929 --> 00:40:02.590 That's a massive developmental and psychosocial 00:40:02.590 --> 00:40:06.550 crisis. Huge. Body image is everything to a teenager. 00:40:06.969 --> 00:40:09.090 Your nursing care has to focus on that. You have 00:40:09.090 --> 00:40:11.349 to educate them about phantom limb pain, that 00:40:11.349 --> 00:40:13.630 it's real, it's not in their head, the severed 00:40:13.630 --> 00:40:15.829 nerves are still firing. And getting them connected 00:40:15.829 --> 00:40:18.030 with other teen amputee survivors is absolutely 00:40:18.030 --> 00:40:20.269 critical for their coping. And Ewing's sarcoma, 00:40:20.309 --> 00:40:23.309 how is it different? Ewing's sarcoma is a tumor 00:40:23.309 --> 00:40:26.619 of the marrow space within the bone. It can be 00:40:26.619 --> 00:40:29.059 in long bones but also flat bones like the pelvis 00:40:29.059 --> 00:40:32.719 or ribs. On x -ray it has an onion skin appearance. 00:40:33.500 --> 00:40:35.760 The key difference is that Ewing's is very sensitive 00:40:35.760 --> 00:40:39.139 to radiation, so limb salvage is often more feasible 00:40:39.139 --> 00:40:42.360 than with osteosarcoma. We're in the home stretch. 00:40:42.760 --> 00:40:46.829 Section E oncologic emergencies. You missed this 00:40:46.829 --> 00:40:49.650 they die list. Let's start with tumor lysis syndrome 00:40:49.650 --> 00:40:52.730 or TLS. This is a metabolic emergency that happens 00:40:52.730 --> 00:40:54.969 when we start chemotherapy, especially for fast 00:40:54.969 --> 00:40:57.769 growing, bulky cancers like leukemia or Burkitt's 00:40:57.769 --> 00:41:00.670 lymphoma. The chemo works too well, too fast. 00:41:00.789 --> 00:41:02.909 It kills a massive number of cancer cells all 00:41:02.909 --> 00:41:04.909 at once. So the cells burst open. They burst 00:41:04.909 --> 00:41:07.309 open, they lies and everything that was inside 00:41:07.309 --> 00:41:09.730 those millions of cells dumps into the bloodstream 00:41:09.730 --> 00:41:12.170 simultaneously. What's in this chemical soup 00:41:12.170 --> 00:41:14.070 that gets dumped out? Three main things you have 00:41:14.070 --> 00:41:16.489 to worry about. First, potassium. Cells are basically 00:41:16.489 --> 00:41:18.329 little bags of potassium. When they burst, you 00:41:18.329 --> 00:41:20.949 get sudden, severe hyperkalemia, which can cause 00:41:20.949 --> 00:41:22.909 fatal cardiac arrhythmias and cardiac arrest. 00:41:22.989 --> 00:41:25.130 OK, that's number one. What's two? The breakdown 00:41:25.130 --> 00:41:28.269 of the cell's DNA releases uric acid. So you 00:41:28.269 --> 00:41:31.989 get hyperuricemia. This uric acid crystallizes 00:41:31.989 --> 00:41:34.230 in the kidneys, just like with methotrexate, 00:41:34.409 --> 00:41:37.469 and causes acute renal failure. And number three? 00:41:37.969 --> 00:41:40.650 Phosphate. Cells are also full of phosphate. 00:41:41.329 --> 00:41:44.019 You get hyperphostatemia. And because phosphate 00:41:44.019 --> 00:41:47.019 and calcium exist on a seesaw in the blood? Exactly. 00:41:47.400 --> 00:41:50.260 When phosphate goes way UP, it binds with calcium 00:41:50.260 --> 00:41:52.760 and pulls it way down in. So you get profound 00:41:52.760 --> 00:41:54.820 hypocalcemia. And what does low calcium look 00:41:54.820 --> 00:41:57.900 like? Titany. Muscle cramps, twitching, positive 00:41:57.900 --> 00:42:00.320 Schwastik's or Trousseau's sign. And if it's 00:42:00.320 --> 00:42:02.559 severe, seizures. The intervention. What do we 00:42:02.559 --> 00:42:05.219 do? We anticipate it and... Try to prevent it. 00:42:05.539 --> 00:42:07.579 Aggressive hyperhydration to flush the kidneys 00:42:07.579 --> 00:42:10.179 out. And we give a drug called allopurinol, which 00:42:10.179 --> 00:42:12.340 blocks the formation of uric acid. We're on strict 00:42:12.340 --> 00:42:14.360 INOs and monitoring those electrolytes every 00:42:14.360 --> 00:42:16.539 few hours. We've already mentioned sepsis in 00:42:16.539 --> 00:42:18.519 the neutropenic child, but it bears repeating 00:42:18.519 --> 00:42:21.360 here. Fever plus cancer equals antibiotics and 00:42:21.360 --> 00:42:23.889 no W. That is the mantra. Do not pass code. Do 00:42:23.889 --> 00:42:26.530 not wait for a full workup. If their ANC is low, 00:42:26.590 --> 00:42:28.309 they have no defense system. A simple bacteria 00:42:28.309 --> 00:42:30.550 can kill them in hours. Just a couple of quick 00:42:30.550 --> 00:42:32.789 add -ins from the sources before we wrap up. 00:42:33.309 --> 00:42:36.289 Lead poisoning. I saw this tucked into the hematology 00:42:36.289 --> 00:42:39.610 section. Why? Yes, because lead causes a type 00:42:39.610 --> 00:42:42.650 of anemia. It interferes with hemoglobin synthesis 00:42:42.650 --> 00:42:45.849 and blocks iron absorption. But the real danger 00:42:45.849 --> 00:42:49.230 of lead is neurological. It's a potent neurotoxin 00:42:49.230 --> 00:42:51.530 that causes irreversible damage to a developing 00:42:51.530 --> 00:42:54.809 brain. Main sources we need to know. Paint in 00:42:54.809 --> 00:42:58.230 houses built before 1978 is the big one. Also 00:42:58.230 --> 00:43:01.389 contaminated soil, some imported pottery or toys, 00:43:01.769 --> 00:43:04.349 and the treatment, chelation therapy. A drug 00:43:04.349 --> 00:43:06.690 like succimer is given, which binds to the lead 00:43:06.690 --> 00:43:08.630 in the blood so it can be excreted in the urine. 00:43:09.309 --> 00:43:11.469 But you have to monitor kidney function closely. 00:43:11.800 --> 00:43:14.039 because the chelating agents themselves can be 00:43:14.039 --> 00:43:16.139 tough on the kidneys. And finally, testicular 00:43:16.139 --> 00:43:18.780 cancer. This is a cancer of young men. The peak 00:43:18.780 --> 00:43:22.599 age is 15 to 35. This is why a key part of adolescent 00:43:22.599 --> 00:43:25.039 health teaching is the testicular self -exam, 00:43:25.179 --> 00:43:27.659 or TSE. What's the teaching point? We teach them 00:43:27.659 --> 00:43:29.159 to do it once a month, usually in the shower. 00:43:29.739 --> 00:43:31.920 The warm water relaxes the scrotum, making it 00:43:31.920 --> 00:43:34.119 easier to feel for any hard lumps, nodules, or 00:43:34.119 --> 00:43:37.099 changes in size. Early detection is key. We have 00:43:37.099 --> 00:43:39.860 covered a marathon of content. From the milk 00:43:39.860 --> 00:43:43.780 baby to the chemo man. If we had to summarize 00:43:43.780 --> 00:43:46.280 the survival hierarchy for these exams, the things 00:43:46.280 --> 00:43:49.079 you absolutely cannot miss, what does that look 00:43:49.079 --> 00:43:51.719 like? It looks like this in this order of immediacy. 00:43:52.000 --> 00:43:54.539 One, oxygen and hydration for the sickle cell 00:43:54.539 --> 00:43:56.780 crisis. That's an immediate life threat. Okay. 00:43:56.880 --> 00:43:59.739 Two, safety and bleeding precautions for hemophilia 00:43:59.739 --> 00:44:03.780 and ITP prevent the injury. Three, infection 00:44:03.780 --> 00:44:05.980 control for the neutropenic and leukemia child. 00:44:06.460 --> 00:44:08.829 Protect them from the outside world. And four. 00:44:08.969 --> 00:44:12.230 And four. Kidney protection. That means knowing 00:44:12.230 --> 00:44:16.130 your chemotoxicities and, for Wilms, do not palpate. 00:44:16.349 --> 00:44:18.349 And bringing it all back to the human element, 00:44:18.489 --> 00:44:20.789 to the child in the bed. What's the final thought? 00:44:20.989 --> 00:44:22.889 The final provocative thought is the concept 00:44:22.889 --> 00:44:25.289 of normalcy. We fill these kids with powerful 00:44:25.289 --> 00:44:27.969 drugs. We isolate them in rooms. We poke and 00:44:27.969 --> 00:44:30.550 prod them constantly. But the nurse's biggest 00:44:30.550 --> 00:44:33.150 and maybe most important developmental job is 00:44:33.150 --> 00:44:35.289 finding a way for that child to just be a child. 00:44:35.449 --> 00:44:38.219 How do you do that? Play. is the work of childhood. 00:44:38.860 --> 00:44:41.559 It's how they learn and cope. So if they're too 00:44:41.559 --> 00:44:44.340 sick to run around, we find quiet play they can 00:44:44.340 --> 00:44:47.440 do in bed. If they're isolated, we use technology 00:44:47.440 --> 00:44:48.960 to connect them with their friends and family. 00:44:49.139 --> 00:44:51.480 We decorate their room. We maintain routines. 00:44:51.940 --> 00:44:53.940 We cannot let the disease and the treatments 00:44:53.940 --> 00:44:55.920 steal their childhood along with their health. 00:44:56.219 --> 00:44:58.480 That's our job. That is the perfect place to 00:44:58.480 --> 00:45:01.019 leave it. To our listeners, take these patterns, 00:45:01.480 --> 00:45:04.360 visualize the chemo man, and go crush those practice 00:45:04.360 --> 00:45:06.989 questions. We'll see you on the next Deep Dive. 00:45:07.210 --> 00:45:07.690 Stay curious.