NTD, ETC

OK, so musculoskeletal system. I put an optional PowerPoint in there for you in case you need it.

2:40

like a quick anatomy restructure on like what tendons, ligaments, bones, all that stuff is not required, but just go down there if you need to um review it. Um, I skeletal system. This makes up our muscles and our bones and everything that connects them to each other. Um, so I think this is like your body's protection, basically.

3:11

Um, kids are more likely to suffer from conditions of the musculoskeletal system compared to adults because their musculoskeletal system is still growing and developing, OK? Um, keep in mind fractures do heal much quicker in kids, but it is also much harder to break a bump in children. Um, so these are some things that you want to keep in mind, um, that might prompt further assessment being needed literally in like an emergency room or a PCP's office. There are some fractures that that um

3:16

don't happen naturally or accidentally. Does that make sense? OK.

3:47

So variations in pediatric anatomy and physiology. Um, just to kind of show you the major differences. This is the hand X-ray of a 2 year old. You'll see the bones don't really connect and cartilage does not show up on X-ray. That's why there's all these like spaces in between their hands, their wrists, they really don't have wrist bumps all that much here versus whenever um they're they're 53 years old. This is what a hand X-ray looks like. So the bones are very well, well defined, well-formed. You

4:17

can see each of the individual bones that make up um the lower hand and the wrist so you can see kind of as time develops, um, what, um how that, how that changes. If you've ever heard of a student or my, um, a patient who has gotten, um, there's special X-ray that can be done on a patient's hand and wrist. They give basically in an estimated developmental age um based on their hand X-ray.

4:49

So, other things that changed, I know Brandon's spinal cord develops, um, at 3 to 4 weeks gestation, so he doesn't amount this is before, most women know that they are pregnant, um, but at 3 to 4 weeks gestation, the no tube differentiates between the spine and the brain. Um, things like, uh, maternal infection or trauma, malnutrition specifically folic acid, and we'll talk about that a little bit more. And um teratogen exposure can also the development specifically of in 2.

5:19

Patigen literally that word means monstermaker. Really, that just means uh any kind of um anything that doesn't develop appropriately. Um, myelination, so remember back to our growth and development lecture when we start talking about the myelination of the spine that is complete by the age of 2, which is why we were talking about growth and development, um, that's why potty training and sinter control starts to become uh possible around this stage.

5:37

Um, muscular development, um, female infant muscles are more relaxed than male infant muscles. And so because of that, they are at increased risk for hip dysplasia. We will talk about hip dysplasia further um along. But just so you know, females are more at risk than males are.

5:52

And then as far as skeletal development, we talked about the growth plate. Uh, there's more cartilage at birth and then ossified, basically the cartilage turns into bone, that's what ossifyings. Um, damage to the growth plates can be overall growth. That's why in pediatrics

6:21

we will mention um filter note made specifically to the growth plates were affected during any kind of injury, whether that be a fracture or sprain, strain, um, and then, yeah, just keep in my children's bones do not break as easily as adult bones do. These are some of the most common mushro resal conditions, and I apologize for talking about all of them. Hence why we're making a study guiding class today because there's no way y'all are gonna walk out of here and know 20 diseases inside now, right?

6:39

OK Um, and I severely noted down quite a few of these. So we're not talking about everything to do with each of these offices. I didn't want to read about it. I figured you didn't want to read about it either. So these are some of the other um disorders that we're gonna talk about.

7:06

So we're gonna talk about myrtle tube defects first. So remember this is the one that I said the gnarl tulos by 3 to 4 weeksation. Um, there are risk factors that affect the closure of the neural tube, and that is, uh, either drug exposure. This can be things like uh prescription drugs that a woman might be taking, um, and not know that she's pregnant yet. This doesn't necessarily mean like illicit drug use.

7:29

Uh, malnutrition, chemical exposure, and then just a genetic predisposition to. Um, so this is why it is recommended that any female who is capable of getting pregnant, take a boho supplement of 400 mcg per day. Um, we don't see girls to keep that quite have the same um rate that we used to. And a lot of this goes back to, I believe it's

7:53

in the 50s. Um, that was whenever we started enriching our bread if you've ever like seen like enriched flour. We started enriching flower products and bread and adding folic acid to bread. And as a result of that, we actually start seeing fewer incidents of neural tu defects because we were addressing that uh nutrition gap that women were having.

8:10

But you'll see there's 3 different kinds that we're going to talk about. Spina bifida occulta is the most mild version, and that's why there was just an opening right here. So you see how there's space right here. So just an opening, but the nerves aren't um

8:30

intruding through that. Does that make sense And then we have the meningocele, which is where there is a um sack that does protrude outside of the spine, but there are no nerves in it. And then the myelomeningocele is the most severe version, and that is where the sat also has nerves inside of it.

9:12

So spina bisaoula, what you may see with these infants, you'll see it um at delivery. Sometimes it's found on ultrasound, but sometimes at times. Um, but this is one of the things that if you were kind of like labor and delivery or postpartum. This is gonna be one of the first things that you're gonna check for um for these patients. So this right here is sacral dimples. So right there is where um like the base of their spine is, and there's this dimple right here, OK? There may also be um the skin discoloration here at the base of the spine, and sometimes in the shape of the, the skin discoloration is, there will be a patch of fit, usually dark hair.

10:07

Um, so sometimes that's there instead of or alongside that discoloration. Or you may see a combination of all three. If you see any of these things, you need to notify the provider and very likely that uh newborn is going to get an MRI almost immediately to verify if there is a neural tube defect and of some kind Um, it's very important though with these, uh, with this diagnosis that you clarify what this diagnosis means for um patients because usually we get patients and their families, usually whenever we hear spina bifida, um, a lot of people immediately go to like worst-case scenario of what that could be because we've used that term to kind of umbrella cover um multiple disease processes, spina bifida occulta is the most mild version of this. So it's important to clarify that um for our, um, patients with their families.

10:28

Um, there's something called spina bifida cystica, which is the most um severe version and often what people think of looking for the term spot is a, um, this may require surgery in the future, but it doesn't always, sometimes you just have a watch and wait approach to it to keep an eye on it.

10:41

So then we have our meningocele. And so this is what that sack might look like, OK? So here at the base of the spine and um this baby and both of these babies have hydrocephalus, so there's an excess fluid on the brain.

11:24

Um, so on your assessment, you'll find a visible external sac from the spinal area. Um, almost always in the Lumar region, it can be other places, but it's almost exclusively in the lumbar region. Um, you need to make sure you do a thorough of a neuro assessment on these infants, um, because we need to know what is getting adequate um innovation from the nerves versus where paralysis might be, or they might have um difficulty differentiating like temperature, that kind of thing. Um, are they moving all four limbs or, um, it's like one of their legs or both legs kind of floppy or kind of spastic with its movement. So those are the things you want to be watching for um during that assessment.

11:30

Typically, um, surgery is for these infants. Um, we don't want to lay them on their back. Um.

11:47

so it can get kind of difficult and this is with the men still and the mylo meningocele. It can get kind of difficult whenever you consider um putting a diaper on them, wear that diaper is going to sit. We don't want to lay them on their backs. They need to be prone. So on their stomachs.

11:56

Um, what kind of difficulties do you think that might cause with like savings?

12:54

How easy is it to lay on your stomach and eat something for that something not very easy. So there's a lot of things that we need to be very um supportive of like physically supporting the infant for uh feeding, but also for the families as well, cause oftentimes, um, the family either knew about this, um, prior to birth or sometimes it was missed, not everything is found on ultrasound or through a testing. Um, so, when surgery is done for these infants, there is an increased risk for hydro ce ph al us So it is very, very, very important that you monitor your patient's test circumference and measure that. We measure that in centimeters, um, and you also want to monitor for signs of um intracranial to increased intracranial pressure. So you would likely see the increased intracranial pressure and then see a change in head circumference, OK? So usually there's built up pressure before the head starts to expand. So keep that in mind.

13:19

so. Um, the other thing that I need to keep in mind is if there is any kind of cerebrospinal fluid that is leaking outside of the sac or around the sap. This job needs to be taken in for surgery immediately, OK? So if you see that or suspect it at all, uh, you need to notify the care team and very likely that incident is being made to start the surgery.

14:15

So our mylo meningocele, this is um the most severe that, that we say it's the most severe neural tube defect. It is seen in 1 in 4000 live birds, um, and again, maybe be seen in utero and via ultrasound, these infants have an increased risk for meningitis, hypoxia, and courage. uh, and keep in mind those spinal cord ends where the defect is. So you need to be, you need to monitor where on their spine the defect is because beyond that, they don't have a spinal cord, OK? So think about, you don't have to memorize this, but um like T1, T2 with the uh or I'm sorry, like L1, L2, the lowest portion of your spine. That's what starts to uh regulate like bladder and bowel control, um, as you get further down

15:07

the leg movement sensation, but also, um, that is also partially what regulates blood pressure control and body temperature. So that's why it's very important to monitor to know exactly where the smalllo meningocele is because that tells you what kind of special considerations for this specific patient is going to need. Um, health history, you may see um those are cost risk for this for those who had a lack of prenatal hair. No folic acid supplementation, um, other children with al defect or maternal abuse of carbamazepine or phenobarbital. Those are very commonly used for seizure disorders, OK? So females who um are taking these medications, part of their, um, treatment plan is likely going to include some form of birth control just

15:31

because they would, if they were to get pregnant, they would have to come off of these medications and seek alternatives, uh, and that depending on the severity of their seizure disorder in all ways and option. OK. Um, so we want to monitor our patient's mobility status, their bladder function, and any history of UTIs is let's take it back to last semester, um, a little bit. What,

15:54

um, as far as like UTIs go, if your patient can't fully empty their bladder where they had increased risk for UT yeah and so if they have an issue with Mptying and sometimes they don't have muscle control over their bladder to actually empty it. So there's constantly dribbling urine, but it means that they're never fully empty. There's always some urine there that is really like help.

16:16

Um, and then your patients may also have a history of hydrocephalus in a shunt. Um, a shunt is basically a um surgically implanted tube, um, that shunts accessory for spinal fluid, um, into the abdominal cavity so that your body kind of reabsorbs it. Um but that way, that fluid doesn't stay inside their head.

16:46

Um, physical exam after birth, you will see that visible spinal sac or the spine, you need to check their uh neuro status, limb movement, and their anal reflex. The way we check the anal reflex and um infants, um, particularly newborns that usually for the first year of life is you check um a rectal um temperature. And the um anal reflux, it will actually push against the thermometer. So you're, whenever you're checking your baby's temperature, it's also low. You're also

17:04

assessing that reflex. And then like I said, you may notice that constant urine dribble. Um, management for this, we want to promote urinary elimination. So these infants may need to see nephrology, the kidney doctor or urology, um, for their bladder.

17:23

Um, they may have issues with uh bowel movements. So to promote bowel elimination, sometimes we'll do what's called bowel training, um, and they'll give more enemas to empty out um what is in the colon. Um, but think about this is the child gets older.

17:38

How could this potentially affect uh the child's self-esteem. They think about it, they start to attend school, uh, and they're in a school setting and likely can't do this. There's probably an aid that's with them that helps them with this in the bathroom. Um,

18:26

kids are very, um they, they see a lot and it's very normal for them to ask a question. So think about how we might talk to this child or talk to their family, um, and teach them to help like to answer those questions that this child or their family is likely going to get. We want to make make sure that we are promoting an adequate nutrition. Parents may not be able to hold their baby prior to surgery because of um how that sac is and the back of the spinal cord um ends within that sac. Um, if the mother was planning to breastfeed. Um, it's very important that you can uh teach um how to count and then save milk storage. But really the most important thing with this is like a large part of bonding with them is through that feeding process, however, parents choose to feed.

18:42

um, so involving the parents as much as possible in that process. Um, even involving them in the safe storage out there, the bottle preparation part of it. It gives them a job which kind of which lets them feel like they have an active role and something to do.

19:00

their child Um, you also want to promote um our patient's skin integrity. So think about that constant dribbling that could be happening from the bladder or even sometimes there's constant dribbling from the anal sphincter. What are some things we can do to help promote skin integrity for these patients.

19:11

Hm Zincboide definitely an option. It's like a barrier crane, yup.

19:37

Really they're just gonna be very copperoli possibility. Um, they're going to need a like constant um pad or diaper changes, whatever it is that they are on. Um, something that you may find into these patients are extremely increased risk for a latex allergy. That's because, uh, probably from day one, especially if they're myelomeningo cell was high

20:11

enough. they have been catheterized, um. probably since day one. So as a result of that, most catheters for whatever reason they're using in the NICU or latex catheters. And so these babies because they're getting exposed to latex over and over again. Typically will develop an allergy to that. We don't use latex very often anymore. Um, people aren't really born with an allergy. You may be predisposed to one, but your immune system's not working enough to be born with an allergy, um, but they are at increased risk of

20:24

developing a latex allergy Questions on any of that? That's it for now. OK, so next is Pectus excavatum.

20:33

Does that help? OK. It's the white tening from the window. It's so bright. OK. So you see how

20:50

there has caved in right here. Um so with these patients, uh, they have what's called a flannel-shaped chest. Um, it does make up for 90% of chest wall deformities, and it is the most common in males.

21:11

Um, with these patients, you would want to monitor specifically for shortness of breath, um, exercise intolerance and chest pain. Why do y'all think that is? I But that's snarrower? Yeah, um, oftentimes they can't take as deep of a breath, um, because, um, there is

21:25

cartilage right here at your sternum that helps everything expand and sometimes they don't have that much here or it's uh very tight. The campsite does deep breath. So what would you expect if you're on the basis of the lungs?

22:00

You hear air movement, but do you think you would hear as much, uh, at the top as you know at the bottom. No, be diminished for sure. Um, so treatment for this is observation and physical therapy is also an option, and then surgery can also correct this and a still rod actually is place in here to push everything back out, um, but it is preferred that this be done before puberty. That is because, um, with puberty also comes that that not exal growth, but that's significant um

22:49

grow. And so they'll basically the issue will worsen, um, especially if they haven't had surgery, uh, puberty is just gonna make a pre-existing issue even worse than what it already was. Um, so for patients who do have um to correct this postoperatively, we want to do a very thorough pain assessment. We want to protect the surgical site and of course, manage their pain. Um, specifically to protect the surgical site. You do not want them rolling, laying on their side or rotating or flexing their spine to either side for 4 weeks after surgery. So you want them pretty flat um after surgery. That's because any kind of raising of the head is going to affect that surgical cyma incision.

23:07

and could potentially that still blight that was put in, it could pop it out and then they move too much. Um, you wanna make sure you were atrating lung cells because we want to hear that they are able to take nice deep breaths and that they're aerating the faces of their moms. What do you think of something we can do to make sure they're er writing places with their lungs.

23:13

They love crap. It's an inspirometry. There you go.

23:38

Um, but once they are cleared by the surgeon, we actually want to encourage um aerobic activity, whatever that looks like, whatever the child wants to participate in that is within um their limitations, um, for recovery from surgery. encourage it. If they want to go outside and play tag and that's well within their limits, have at it. If they want to go around their bike and that's within their limits, have that.

23:53

So really it's just let the child do whatever it is that they want me that's going to get that aerobic activity just within the um safety recognition recommendations that was given to the by their service.

24:36

So next we're gonna talk about limb deficiencies. This talks about um safe limbs altogether or if a limb doesn't fully develop. OK Uh, whenever you are assessing a child with a limb deficiency. You want to make sure that you are documenting the extent of the deficiency. So what that means is like for this so right here, I can't see his whole arm, but the way he's holding his arm wrap here, I would estimate that likely he does not have an elbow. So, um, I would describe that it essentially is the lower aspect of the arm, but without an elbow. So there's like a shoulder joint and then I'll describe that the forearm

24:45

wrist, and hand are present. Does that make sense? So you want to describe what is there, but also describe what is not there, OK?

25:21

And so accurate description, that's the most important part whenever you are documenting this. Um, as far as management goes, you want to provide activities to the child has the ability to do. Um, there are many things that either of these children would be fully capable of doing. So the focus is on what they are able to do, not what they are not able to do. Um, early intervention is one of the best things you can do, and you can have a considerable effect on their ability to make their developmental milestones, uh, because very oftentimes, they just need um some basic adaptive devices and that gets

25:34

them caught up to where it's typically developing child would be. Um, we don't see this. quite as often as we used to. Have you ever heard of the medication thalidomide.

25:52

OK, Hulu did a TV show about this no last year or so, um, but there was this medication that hit the market like the 1950s, 1960s called thalidomide. Um, and it was fantastic. It was the only thing that could touch um morning sickness and completely took away nausea.

26:05

Um, but then we learned that um just about every woman who took the little. the baby was then born with some form of limb deficiency. or um other missing like organs and things like that.

26:22

Um, it led to some very, very, very severe birth defects. Look it up, the medication doesn't even exist anymore like for other uses, if there, you can't buy it anywhere. Um, but and I've never seen a TV show, but from what I hear, they did a really good job representing it.

26:44

Um, but there are other things that can cause this. Um it's unknown specifically what it is that causes it, but it is associated with exposure to certain chemicals, some viruses, um, certain medications and then maternal tobacco use or exposure. It's also um associated with that.

27:23

Question OK So next we have polydactyle and sydactyle. So, polydactylene meaning extra, poly means extra.an means without. And so polydactyle having an extra digit, whether that be fingers or toes, send back typically is not a, an altogether missing toe or finger. Typically, it's that there's webbing between two big fingers or toes. So it doesn't mean that um sometimes it does completely delete one of the the advertis but typically there's webbing between here.

28:01

Um, it's unknown but the cause is, but it, it, it is associated with tobacco exposure. um exposure to certain chemicals, viruses, and medications. Um, and these children do oftentimes have other congenital anomalies. So you'll see, um, again, newborns, uh, as part of the newborn assessment, um, very closely looking at the shape of the hands and feet and counting fingers and toes too, because if you see this on assessment, this says we need to do a very thorough physical assessment because there could be, there's an increased risk of there being

28:42

other congenital deformities that we may not know about, OK? Um, it can be genetic or it can be just a normal variation within some new warm. Um let's see, with yole is very important that whenever you are assessing this, that you are feeling between the two fingers, the two toes, and feeling, is it tissue that's connecting them? Is there any kind of bone that crosses over any kind of fusion. So you want to assess what you can feel all the way down between those two fingers. Um, this one, because I can see that it's bending, uh, both fingers are kind of bending forward. I know that there is a joint here.

28:57

a joint here and a joint here. And I'm betting this is simply webbing. Wrap you're connecting these two fingers. And if the parents so chose, um, then this could be separated surgically and then skin wrapped around the side. Yes.

29:11

So I was born with extra toes the sides of my foot. So my mom, they were like they didn't know what the cause was. They just randomly happened, but my mom did opportunity, you know, cut off.

29:42

OK. OK. And I think this, I do still have a boat that's like pull it out, but I don't know if that's like cartilage or weapon or anything. She was like, no pictures at all, like, you know, and that is, I, I like the you point that out. That's a very common reaction for some parents. Some parents do want to have that surgically removed and request that there not be any photos of that. Um, and so it's important that we respect that if parents are asking for that too. Some parents do want photo

30:09

s so just kinda keep that in mind, everyone's a little different with what they're uh reasoning is and what their thought process is, um, reasons that I have heard and I went to school with a girl who um had her thumb and then she had this part of her finger that was coming off the knuckle, so she didn't have any fingers. Fantastic basketball player that girl can shoot from anywhere on the court. She was a Um.

30:27

but also her toes didn't grow in like a typical fashion. They were very like abnormal in the way the shape was, um, and her parents actually considered because her toes were fairly long. Her parents actually considered removing her toes and having them move to her hands.

30:48

Um, ended up deciding not to do that, but there are lots of options as far as treating that nowadays. Um, some reasons for, um, removing like excess digits if it's going to affect function. Um, sometimes the extra toe or the extra finger doesn't have ner ve s running to it. So there's no way to move it. Um.

31:15

I hate to make this comparison but I don't know how else to do it. Have you ever had like huggies with like dewclaws and sometimes with that like removes those, they can't move them at all. The y'all are seeing that. So if you were like, what are you talking about? I'm getting some guesses. Um, basically, sometimes the extra digit doesn't have nerves in it, and so it can get caught on things and potentially like fracture it, increase risk for infection, that kind of thing. So sometimes parents will ought to have that removed

31:19

too.

31:30

OK, so this is called metatarsus adductus. So you adductor, um, I think that goes towards the deadline, OK? That's where you adju period is.

31:49

Your adductor muscle in your leg is on your inner leg. Um, and so this is curving in towards midline. So this is the most common congenital deformity in newborns, and it is all, it also causes what we call pigeon-toe or in towing.

32:13

Um, it happens in approximately 1 to 2 in 1000 births. Um it's most common to be found in first-born children. I have no idea why. I tried to find it, but first ones apparently are at the highest risk of getting this. Uh, you'll wanna know it on physical exam, um, and half of all cases affect both things.

32:32

So half the time it's that's go have the time and that's only one. Um, so you'll see the front half of the foot, so basically like from um the midline of the foot, um, will start to curve inward. So this one is more severe than this foot, OK?

33:02

It typically result s from abnormal position in utero and in these children means that they have a very high arch, um, of visibly curved and separated big toes. You see how there's more space, uh, from the start normal but you see how there's more space right here from this foot to this foot. and this one with the way that toe is curving, I can tell that there's a large space right here for it to be able to be in that direction, that's completely.

33:22

So management for this um is stretching, so you'll stretch and the turn foot or um we tell these parents to put their children's shoes on the wrong feet. OK? Have you ever put your sit on the wrong foot and you feel like kind of pushing on your toe. That's actually the treatment for this is to tell a kid to put their shoes on the wrong feet.

34:01

Um, if that does not work, then we do what's called serial casting, where a cake gets put on the child's foot, um, that is pushing it to the like back to midline and then another cast will push it more and another cast will push it more, some serial casting multiple tasks to try to force the foot back in the appropriate shape. Um, surgery is rarely needed to fix this, but it may be required if the child makes it to 6 years of age and it has not been corrected or if the child's experiencing pain as a result of the knee part of the way it is.

34:29

So not to be confused, Mears to add that this is just the um top half of the foot time. And then we have clubfoot, which is where there's turning that happens at the ankle, OK? Um, on assessment, you might find that there's a family history of clubfoot anomalies. This is very common in babies who are breech position for those of you who don't know, most babies are head down in utero. They're upside down. Um

34:55

but if they're sitting outside up, they're kind of, they kind of sit crisscross or with their legs kind of tucked up underneath. And so you'll see their foot develops in this club shape because it doesn't have enough room um and gravity to having their body look kind of sit down on it. Um, when you're doing your assessment, you want to assess range of motion, but you will find that you are unable to turn his foot back to midline. It is stuck in that position, OK?

35:15

Um, to treat this, we do serial casting very similar to what we did with the metatarsus Adepis. You want to make sure that you are doing neurovascular checks. So, um, is there still sensation in the toes? What's the temperature look like? Um, can you feel pulses, all of that, um, as well as providing emotional support to families.

35:31

Questions there OK. It happens in the 1 in 1000 live births, um, half of them occur bilaterally and again it is more common in males.

35:42

Here's what's Grey's Anatomy. package. Osteogenesis and perfected. This is where most people have heard of this disease from Washington Grey's Anatomy. Spoiler alert has been out there.

36:24

Um, so, on assessment, you will typically find whenever you're doing your patient's health history, that there is a family history of um OI and you will hear parents report and frequent fractures and then increased crying, um, especially with very routine here and hand. So thank simply picking up the baby and then placing baby gently on, on the Chinese new table, could potentially fracture their leg or fracture their arm from simply being placed down. The bones are extremely extremely fragile. Um, you'll see down here how sclera this should be bright white, has kind of like a blue, purple, gray tinge to it.

36:41

So if you see this in a child, this isn't um diagnostic or definitive of osteogenesis imperfecta, but if you see that, um, you need to do further investigation, OK?

37:04

Um, they will also typically have abnormal primary te or what we call baby teeth, um, those teeth may not grow in, uh, in the typical um fashion or there may be some missing that never broke in. Um, or that you want to do family teaching. Um, I'm not gonna go over all of this because there's a really great guide for it in your book. So make sure you read over this.

37:28

And things like walking, swimming, and water therapy are excellent um for these kids. There are varying degrees of osteogenesis imperfection, um, and there is a table in your book that um explains what he is. I'm not gonna talk about all that because I'm not going to test y'all on all the different types. Just in general, this is what you see with bo eye.

37:56

Um so walking, swimming, water therapy, those are really great for them to do as far as exercise goes, um, water therapy is probably one of the best things that can be done because it's very low stress on the body, um, and the risk of injury during that is also very low. Um, there's a medication called bisphosphonate. You put it on nod and say you remember it and I lectured on that and farm, uh, but that's a vacation that is often used to become.

38:23

moderate to severe versions of this disease. And then, uh, sometimes what'll happen is they'll put lightweight braces um on the outside of the legs, specifically like while they're walking, but also surgical rods may be placed in the long bones, things like femur, humerus, um, to support them that way, there's like that extra buffer in the event that there won't be a frac ture of some kind

38:38

The questions on any of them OK OK, so hip dysplasia. Uh, you'll also hear this referred to as DDH just so y'all know, um, what that is if you're ever looking at like the children you're looking at. um.

39:22

a patient's history that may just be that be in there DDH, but that is develop developmental dysplasia of the hip. So these uh abnormalities include dislocation subluxation, which is a partial dislocation or dysplasia, which is um a shallow acetabulum. Basically, your hip, uh, the way the pelvis and the femur connect. Your hip has this nice little cut to it, OK? And the ball of the femur should sit right inside of it, OK? In hip dysplasia, this cup is either very shallow, so the ball can move around too much or it's so it's so small or barely bare that there's really nothing for the ball of the fam

39:31

or you like to go to and stay connected to. Does that make sense? So, varying degrees, a lot of it just depends on what the shape of the acetabulum is.

39:47

And so basically abnormal relationship between from Waldhead and astropaulum. When we say that, that's what I mean, is that cup that should be there for it. Um, isn't necessarily there. Now, this is not mean that um

40:16

patients with this are um unable to walk or anything like that. Um, I've taken care of a um um college cheerleader who has a dysplasia, and was still able to do all the stunts and everything, so yes, she hasn't, she has very unstable hip. um, and had it pop out of my myselftown that she and her coach and several of her teammates have learned how to pop it back in. She just kept going. So

40:42

um um, complications, avascular necrosis of the femoral head. Um, if you all looked at all that the um review PowerPoint, uh, it is very, very, very difficult to get blood flow to the bottom, OK? So Davascular or blood flow, think that, OK? Because it's very difficult to get blood flow to bones. That's why bone infections are so

41:02

difficult to treat because um what we take what you're taking like amoxicillin by mouth. That's not going to be absorbed into your thumb. That's why people with bone infections end up on IV antibiotics for several weeks. It it takes a very long time for the antibiotic to actually penetrate the bone itself and treats the source of the infection.

41:25

So avascular, poor blood flow, and it can actually cause um the bone here to become necrotic. Um, it's very, very, very slow breakdown, but it, that means I'm there, basically. So the bone will start to break down, um, and sometimes they have to go in here and shave away the dead part and um these patients sometimes will actually get hip replacements.

41:42

Um, there is a loss of range of motion, unstable health. femoral nerve palsy. So sometimes there can be numbness at the site, a leg length discrepancy typically because this is popping up. Um, the affected leg is shorter. Does that make sense?

42:03

So if you're ever looking at a patient to when you're looking at their labs, if one is longer than the other, the shorter leg is very likely where that that dysplasia is taking place. Um, these patients will sometimes develop osteoarthritis, again, just because of the breakdown and and rubbing and foam on bone, and here with it then the abnormal and joint area. area.

42:09

Questions there

42:54

So within this, when I'm doing in health history, there's almost always a family history of it They are almost always female, um, large earthquake or a breach burn because again, this is very likely how baby is sitting um within in, in Europe. um, and then it shows up more often in people of Native American or Eastern European descent. And so whenever you are assessing for this, we're gonna start an inspection and observation first. So you would lay the infant flat and you would notice that there would be an asymmetry of their thigh and glute folds when they're in the proposition. So when you lay them down on their stomach, they'll actually notice the loopold um and where they're bifolds are, are

43:12

um, they're asymmetrical to one another from side to side. Uh, you'll also notice the lead length discrepancy on the effective side. And then when you palpate, you'll notice that there's limited hip abduction, abduction is going out, OK? So whenever you go to abduct their legs.

43:29

you won't be able to move that side as much, um, and there's a test called the Barlow and Molani test. You can look that up. I'll post a video of it too. Um, it's basically where you grab the baby's hips and you just very gently kind of make like like uh rolling motion.

43:46

um and you're feeling or hearing for a hit click. OK, so what you're doing, what actually some, somewhat kind of um just low partially dislocate the side that has hydysplasia. So you really do it with one. Like once you fill the head clip, you don't keep.

43:57

right? So just keep that in mind. Um, this is likely something you're not going to see or do unless you're working like labor and delivery or like freshly postpartum NICU, that kind of thing.

44:40

Um, management, you wanna make sure there's early recognition of it, which is why someone doing Marlow Bowai test, um, as well as harnesses. That's what this harness is for, and it basically is forcing the femoral head into um that ambulance so that it can form appropriately around it. Um and the hannas is called a tablet furnish. It can be worn for as little as 3 months and when it's used appropriately. Oftentimes though, it does have to be more longer than that. Um, if it's found after the age of 6 months, it will likely be surgical correction, and that's where this creepy baby kind of comes into play.

44:47

Um, this is called base, OK, the head will fall off. I'm sorry. OK, so this is called yota.

45:00

and I'll show you a picture of that, but like if I were to take a side, they'll see how like that shape. So this is also very similar to the harness up here. This is a cast that this baby we cannot move.

45:11

or move within this path. So it goes all the way up, so there is no movement at the hip right here at all, OK? So that's usually done after surgical correction.

45:18

It has jaundice from Alphaellow.

45:32

OK, questions on hip dysplasia. OK, yes, when you said, uh, it says not a symmetry of thought and gluco, so they they they're unheeded. Yes.

45:46

So think like the line where the glute meets the upper thigh instead of it being symmetrical and even. You may notice one is higher, or you may notice that one side doesn't have a fault at all. Yeah.

46:03

So next we're gonna talk about torticollis. This is one of my twins. He's Caroline. Um, so to Hollis, um, I posted this to the to see how this is one of the um

46:39

potential uh. issues that can come about if the baby has a policy. So, uh, this is basically where the muscle wrap it is right here or sometimes it's called the sternoclaidomastoid muscle. So the muscle that connects through here and goes up to the net is very tight and so their head is pulled tight to one side, OK? And what this will sometimes do is um it'll turn their head one like one direction or their head is can be tilted to one side. So it's very normal, especially in the first like couple of days or weeks.

47:04

after a baby is born, they might favor one side over the other. Usually because of the position they were in in utero, but um it should be corrected. Um, there's very limited range of motion with passive movement. So, um, whenever I went to, there was one time I was dressing her, and I noticed her head was kind of tilted over the side, and I went to turn her head and set her body.

47:18

And uh I was talking to one of the doctors I worked with about it and I was like, yeah, it's the strangest thing, and he's like, well, that's actually the diagnostic test that we do to improve Port Collis. So you made my job easy. Like those people.

47:42

um babies who have to colis, there's, they also have a history of hip dysplasia in 8 to 20% of cases. That was not her case. Uh, she is favored one side more than the other, and me being an exhausted mom of twins and a 2 year old and didn't notice that one of my twins only nursed on one side. Um, I just wasn't paying that much attention, to be honest.

48:11

Um I'll call myself out on that, but gentle neck scratches, it's very easy to correct. Um, so what you do is you immobilize the shoulder on the affected side. and then you just kind of pull their head as much as you can, very slowly, very gently, and we'll hold it for 10 to 30 seconds and repeat that about 10 to 15 pounds of tension. Um, to make this easy for parents, instead of giving them another thing uh in the day to do on top of like diaper

48:22

chain feeding, sleep, naps, all the things. Um, we did this anytime I changed her diaper. I knew I did that several times a day. So anytime her diaper was changed, we did the scratches.

48:57

Um, passive stretching treats 90% of cases when it started in the 1st 3 months of life. We found this at 6 months old. Um, I've been in for a wellness check and um this was whenever I thought my twins might still be paternal, um, because her face was a little different than her sisters, and we learned that just because she had developed plagiocephaly, um, which is where, because there are things um their soft spot, grow plates, all that within our head, um, we're still shifting. She had been turned to the side for so long

49:19

that he was no longer browned Um, if you looked at her head like top down, she kind of looks like a potato. I can say that on the Um, but anyways, so we wore this helmet for about 6 months. It was adorable. It said fixing my melon on the side. It was cute. We dressed her as a watermelon all the time, like her swimscas a watermelons, so we had a lot of fun with

49:44

it, um, but she's 5, you can't tell looking at her that she ever had this issue, um, and she moved her head and neck and shoulders, everything just fine. Um the main way to uh retrieve this though is just that passive stretching. Sometimes physical therapy is necessary. It wasn't in our case, uh, but occasionally it can be. So this baby bacteria you'll see um

49:55

this baby's got topois pretty severe, pull in their head. OK Questions on the that

50:28

So my sister has it and she still has a lot of issues with like the muscles on the side that that come a bit handy. Um, sometimes people are just more um likely to have issues with it. Um, usually it's like think about like sleep position. That's probably the one time that we can't like control what positions um that we're in and what potentially is happening is whenever he sleeps, she's pulling into that side, um, and then it has to stretch it back out the day likely what's happening.

50:42

Sometimes, um, to treat it, sometimes you can get Botox in your um traps, your tribeus muscles, um, to kind of release that too.

51:32

OK, so next is tibia vera or blout disease, um, on the test, I'm gonna call it tibia vera, but y'all may still see people call it mountain, so I wanna go see though. So the tibia is one of the bones of the lower leg, and that's this outward bowing right here, OK? Um there are 3 types, infantile, juvenile, and adolescent. Um, infantile is for those who are 1 to 3 years of age and are most common, uh, juvenile is 4 to 10 years of age and an adolescent is 11 years of age. So this is most common in an early walkers, um, African-American females, um, and typically affects both extremities. Um, so if you were doing like a, a health history with the patient that they came in

51:55

for a wellness check and the parents made a comment about um how they started walking at 9 months old. That would prompt me. I need to take a very close look at what their leg looks like when they're standing, particularly for names and angle, OK? So early Walkers is about what age should infants start like walking. I'm not saying pulling up like independently walking.

52:22

But a year old, 1215 months is about average. So anything before that, um, would be considered early walking, OK? Um, if it is untreated, it could lead to the growth plates ceasing developments, the growth flight here that face theneath and see and development which will actually cause the bowing to worsen because this part is no longer growing.

52:38

You see how this lengthened out, then it would stroke the leg. Does that make sense? So if the bone isn't taken care of and the growth plates and stops growing, then it's just gonna worsen because this inside where the growth is happening it's gonna remain this length.

52:54

but this bow is gonna remain it's gonna remain. OK. Uh, it can lead to a symmetric growth that both needs which will cause very severe knee pain and can actually lead to degenerative arthritis, um, is less untreated.

53:28

Whenever you are looking at the patient's growth parameters, uh, obesity can potentially be um a factor in this, um, but it would have to be like very morbid obesity and most children, unless they have like certain medical condition and especially this early on, aren't going to um have those those those tractors. Um, management for this are braces that are worn 2 3 hours a day. Bass take it off to me That's essentially what that means.

53:50

Um, and they'll do it for several months or even several years. It just depends on the severity. Um, but also everyone kind of responds to treatment differently. So, um, there may be someone who is less severe who wears it shorter than sunlight or some sorry, someone who's less severe who wears it longer than someone who's more severe because the more severe case responded quicker to therapy.

54:01

Um, and then sometimes um surgical treatment and that serial casting is required for this. Questions

54:31

So next we're gonna talk about muscular dystrophy. and development wise, and I want to talk about what you may see um with each developmental stage and how that might um prevent um just based on the age that your patient is. So toddlers with muscular dystrophy typically will be late learning to walk and we'll have what's called pseudohypertrophy of their calves. They will have extremely muscular calves is what that means.

54:50

OK? So very large calf muscles. Uh, the preschool child with muscular dystrophy, and their parents may note that they fall often or refer to them as clumsy. Um, these children will have difficulty climbing stairs, running, or getting up from the floor and you may, so this is

55:18

what I call I wrote that now I'll say this in a second. Um, but this is what a child's muscular dystrophy will widely look outstanding up from the floor, OK? So instead of just standing up from a seated position, they have to get to their knees pushed to, um, basically down dog y'all do and y'all get all are familiar with that, and then they'll kind of backpedal their hands and their um the forward fold and then stand up from there.

55:38

Um, school-age children will have difficulty raising their arms. So you'll ask them to raise their arm out to the side, but unfortunately at that stage of development, these muscles become affected and are breaking down. And so they can't raise their arm out to the side the way they could before.

56:24

Um, and then my adolescents, they may lose the ability to handle it altogether. Um, treatment for this is specifically I'm already use medications, glucoorcoids, um, the steroids can slow the progression. Um, but think about your side effects to steroids, that's weight gain, um, osteoporosis, steroids effect and how the body metabolizes calcium. Um, hersidism, which is the abnormal, uh, hair growth, specifically in females, and then the pushing away the parents. Have y'all done endocrine yet and um that charge. No, not yet. OK. Um, so things like moonface, so the very rounded face, rounded abdomen with purple striae, kind like purple, and

56:44

re marks, um, those are some of the features that you may see, and you'll see this actually it's new. Um, they also will likely take a beta-blockers and ACE inhibitors to decrease the work of the heart. to keep in mind, uh, the heart is a muscle too, and it is also affected in muscular dystrophy.

57:07

Um, and then we can also use braces and orthotics um for these kids. So, um talking about m us cul ar dystrophy, I'm using very general terms for it because there are um different levels of severity. Um, but what is common in all cases is progressive, so that means it will continuously get worse.

57:30

Um, there's something called Duchene muscular dystrophy. It is the most common neuromuscular disorder, um, that's all, and it does have a shortened life expectancy as a result of it. Um, because of medications like Aticorposteroids or, and, uh, beta-blockers and ACE inhibitors. We've increased the life expectancy to about 40 years of age, 40, not 14.

58:24

Um also there's increased, um, like non-invasive mechanical ventilation that can be done as well because I think diaphragm is also a multiple your diaphragm has to be able to move, take a good deep breath. So there are some advances that we made that and extend the life expectancy, but also, um, improve the quality of life of those with the company. Um, muscular dystrophy is a gene mutation that results in the absence of something called dystrophin, which is critical for the maintenance of muscle cells. So basically there's a gene mutation that um locks the breakdown of muscle cells. And so those muscle cells will start to break down and they can't breath on you. So that's why it it's progressive. That's why suddenly they can't raise their arms out the side. Why it affects her heart, why it will affects her diaphragm.

59:01

Um, it is excellent recessive, essentially meaning um so females, ex X chromosomes now have XY chromosomes. So because it is X-linked men have a harder time um with muscular dystrophy than females do because oftentimes females might have one of their X chromosomes might have the gene for this form of muscular dystrophy, but there are other X chromosomes might not have it. So because they have like a backup X chromosome. The symptoms aren't gonna be nearly as severe as what a nail may experience. If that makes sense?

59:26

That's why, that's why we talk about like X-length recessive or autosomal recessive and why males oftentimes a harder time with these diseases than females. It's because females usually have a backup chromosome that can kind of kick in and help. Um, in all cases, you'll see generalized weakness that then progresses to progressive overall weakness, um, and it will start with the hips.

60:00

shoulders, thighs, and pelvis. and then move on to um respiratory and cardiac muscles. Um, and another thing to keep in mind is many but not all will have a larvisibility of some kind along with this just as part of um the the gene mutation, but I want to clarify that learning disability, this can include things like dyslexia, ADHD, like, things like that. It's not saying, um, like a gross learning disability where, um, like communication becomes difficult for that kind of thing. There's a lot of things that can't go along

60:18

with. Scourer sign, that's what that's called. Thank you for following my next slide. OK. This is a positive Gower sign. If you tell a child to stand up from the floor and this is the way they stand up. That is a positive Gower sign.

60:37

That's fine. So whenever you are doing an assessment on one of these patients, and you want to get a thorough history of their developmental milestones. Very likely what you are going to hear from their families is that they learn to walk and then shortly after I lost the ability to walk.

60:50

OK, those muscles start to form and then started to break down, OK? And you will hear them say, typically there is a long history of coughing or frequent respiratory illnesses. Why do you think that is?

61:23

What muscle is involved in breathing, diaphragm diaphragm. Well, it has to be working really well for you to take a big deep breath. Background. So if you can't take a big deep breath, um, what do you think happens oftentimes to try to move all the gas that is my collecting at the base of your lungs, coughing, yeah, respiratory illnesses you're gonna have uh more difficult time with because they're not able to aerate their lungs fully. Um, so any kind of respiratory symptoms

61:48

that they may have are going to be worsened due to the weekend of diaphragm and muscles involvement. Um, you'll notice that these patients will develop tachycardia and their heart muscle weakens. Um, and as I'm sure you can imagine, this tachycardia is going to further weaken the heart muscles. That's why we give them beta blockers and ACE inhibitors to decrease the work of the heart.

62:05

Um, you'll get diminished breath sounds again as the disease progresses we talked about that and from the diaphragm involvement. there is something called um CK levels increased CK levels. This uh CK stands for Creatine kinase, not creatinine.

62:42

No, it's don't just creating kind. So you'll hear some people because they take like creatine like for um then you will see an increased pain levels in these patients because they are having muscle OK, uh, it is important though whenever you are interpreting CK levels, uh, and your patient is like a weight lifter or exercises or like play sports, that kind of thing, and they're sore, um, they may have increased DK levels. So just make sure that like you you've gotten a thorough history whenever they're drawing these labs on your patient.

63:22

Um, and then there's something called electromyography that is done to diagnose these patients because what it does, it shows that the issue is with the muscles, not the nerves. OK? So with paralysis, the nerves aren't working even like the firing mechanism is trying to get there, but there's something stopping it. Muscles respond to firing from nerves, OK? So electrobiography shows us, um, basically they'll artificially stimulate um what the nerves would stimulate, but the muscles won't respond or they won't respond unappropriately. So that's why this shows that the issue is a muscle issue, not a nerve issue.

63:48

Um, and our goal for these patients is to promote their mobility. We do that again through the work fluoroids and calcium supplements and then encouraging weight bearing activities, um, within like their um ability to promote circulation, healthier bones, and a straight spine. The more you move, the better circulation you have and the more, um, weightlifting at any time you do, the stronger that you have.

64:11

Um, you want to maintain cardiopulmonary functions. So you want to assess their respiratory rate, depth, and effort. You want them sitting upright as much as possible. That's the position that they can take the deepest breath in. Um, and you also want to assess for edema, weight gain, or cracking. What do you think any of those things being present could possibly make.

64:50

Um, you know, like a problem with the heart or a problem with the lungs, yeah. pump Um, and then we want to maximize quality of life. So avoiding long periods in bed because that will actually further worsen um muscle and bone breakdown. Um, you want to provide them developmentally appropriate activities, provide emotional support for both the patient and their families, but also provide respite care for their families. Um, it's very, very common. You'll see with children whenever they are hospitalized, and that sometimes the parents will kind of like

65:31

take a um kind of take like a hands-off approach you may notice in the hospital. Sometimes they very much still want to do everything for their child while they're in the hospital. But other times, usually the reason that they're not um being hands-on with everything is because they are physically and emotionally very, very tired. And they have the opportunity to take just a little bit of a break and trust you as the nurse or the nursing assistant or anyone else in the hospital to take care of their child while they are there in the hospital. So don't assume, um, if you're taking care of a chronic kid and the parents are kind of take like a hands-off approach to it. Sometimes they're just taking a little bit of a break and

65:42

they're saying, I trust you and they'll step in if there's something that they need to set in for. Just keep that in mind.

65:52

So the hour sign, so positiveness, we need to start doing further investigation.

66:10

OK, spinal muscular atrophy or sometimes called SMA. This is a genetic motor neuron disease that does not allow spinal nerves to communicate with the muscles. So basically, what's happening in the spine is not getting trans um transported elsewhere, OK?

66:33

Um, signals to the muscles, um, are ineffective, and this will lead to um a loss of muscle function and atrophy overcome. That's why you'll see, um, this, this baby's muscles, baby muscles already aren't very, like aren't super developed, but these are even lower developed. You see how small this baby's chest is too, um, they're diaphragm is very, very small too.

66:46

Um, costsomal muscles, so the muscles that are close to the center of the body are more affected than the distal muscles. So proximal things like close proximity, distal distance. OK.

67:27

Um, there are 4 types of SMA. I'm not really on the 4 types, nor I think we'll be endless, but if you're interested you can bring that. Um there is respiratory muscle involved in all types. So that's the important thing. Um, you'll see upper respiratory infections, aspiration, dysphagia, GERD, these babies will sometimes be handed dependent and pectus excuitum is also very common in these babies. Um, therapeutic management is simply supportive care at this point. Um, there are, I will say, new medications um that have come about, um, in the last couple of years

67:42

I um have a friend that I grew up with. Her niece has SNA, and she was a children's for a very, very, very long time. Um, but they found a medication that had just got approval like for human testing.

68:10

that she was able to get um that has been like work wonders for. She is pretty thin, but she likes sits up, she can eat. She started to communicate. Um, she's meeting some of her developmental milestones, but whenever I tell y'all, this medication in an IV bag was delivered to the PICU by the pharmacist in a sealed box that had um handcuffs to the box, to the pharmacist.

68:29

And the pharmacist and doctor and nurse had to be who hung it because of how expensive this medication was. Yeah, there was no like redrawing it or if you like still the drop like cramming your line. No, none of that. That's why all like the big guns had to be there to get it all going

68:58

Um, but thankfully, there is this advancement now that this medication does exist and it's starting to help more babies that have this. Um, that's not in your book because when the book was published, that had gotten like that type of medication has made it yet is it full circulation and the clot's not here yet. Um, you will see symmetric unexplained weakness of bilateral weakness um

69:43

that's why, um, diminished, um, tendon reflexes and a loss of motor skills. Um, with my friend's niece, what happened was they went into her 4-month checkup, and she had met all of her milestones at her two-month checkup and not her 4-month checkup had lost every milestone she'd met about 4 months, and that was how they knew that there was something that needed to be looked into. Um, you'll see the CK elevation, greatly planning, uh, genetic testing, um, muscle biopsies are sometimes done and then something called a nerve conduction velocity test, just basically is just um seeing how quickly the nerves like receive messages. Um, not testing on that.