Next is our expert on feeding in nutrition, Dr. Ajay Kaul is the director of the Neuro- Gastroenterology and motility disorders program at the Cincinnati children’s hospital Medical Center Welcome Dr..Kaul Thank you, I Am the only gastroenterologist in the team today and as you can see I’m going to go solo so Really this has been a huge learning process for me of the past two days of learned so much about congenital myotonic dystrophy I mean I was Focusing more on the nutrition part of it but was really good to learn from the pulmonologist the orthopedic surgeons the neurologists about you know kind of complete my own learning process and the other side of the story is Learning from you guys that has talked to a lot of the parents And I learned so much about what it is like you know nutritionally and all the issues with G tubes and j tubes, so I thought I’d talk about that a little Bit, I In no, way can I completely cover everything but I’m going to give you a little in a nutshell and then we could discuss have an open discussion and have questions and answers both for me and for you So we’ll be mutual learning process here So one of the major issues that a lot of our kids and adults with congenital myotonic dystrophy are the Chewing process so as you know The muscular dystrophies involve all of the skeletal muscles of the body this is skeletal muscle That’s outside of your body, and you have voluntary control over it there are two other kinds of muscles in the body. What are those? smooth muscle and the Cardiac muscle So you’ve heard a lot about cardiac Cardiac involvement in CMD as well and all the evaluations They do and the echos and all of that the third muscle Which I like to call the forgotten muscle is the smooth muscle, and where do you find smooth muscle in? my part of the body the gastrointestinal tract the skeletal muscle, really Ends in the beginning of the esophagus the swallowing Tube the rest of the swallowing Tube and all the way down to the rectum is all smooth muscle Not only that smooth muscle is also present where else in the body gall Bladder urinary Bladder the ureters the Tubes that take urine from the kidneys down to the bladder all that is smooth muscle as well in addition Smooth Muscles are also around blood vessels so around not the cardiac But the Aorta and the rest of the blood vessels you have smooth muscle so why is it that these muscular dystrophies you know primarily seem to affect skeletal muscle and in some of the Cases Cardiac muscle nobody really talks about the smooth muscle. Is it involved is it not involved there really isn’t a whole lot of of Literature out there or publications or studies or research about whether or not smooth muscle is involved in Congenital muscular dystrophies. I mean if you look do a literature search you’re really not going to find much there, so That’s one of the reasons. I guess why I got involved is to kind of you know I like challenges, and I you know this is the population like any chronic illness physicians Don’t like to take care of Chronic illnesses because there’s no cure They’re like you know we don’t have a cure for this and and they like to do things that they can fix and move on well there’s something to be said about making the quality of life or soft children with Chronic illnesses better and talking generally about all special-needs kids not specifically CMD So the first thing the swallowing, I was talking about the skeletal muscle as you can see over here in this picture When you first swallow there’s an oral phase followed by the Pharyngeal phase the blue here is showing you the food bolus But whether its water or food whatever you take in your mouth? what is the first thing you do you chew it and In that process you have to have all other muscles your facial muscles strong enough to be able to chew it well You should have a strong muscle movement where it can manipulate the bones in the mouth and then when the chewing process is over push it towards the back of the Mouth which is the Pharynx the Pharynx then takes over the process of squeezing Really hard to push push. The food bolus down into the esophagus you see right there. That’s the opening into the esophagus now Several things can go wrong in myotonic dystrophy, so you can’t chew you can’t masticate as well Also, the Pharynx is weak the back of your throat is weak because that’s all skeletal muscle So it cannot squeeze hard enough to push the food bolus down into the esophagus Thirdly This is the airway this is the larynx or the windpipe and this shuts down you see this epiglottis It shuts down as the food bolus is going down because you don’t want food going into your airways as you’re swallowing And that’s a protective mechanism guess what all of this movement of the LArynx is controlled by skeletal Muscles so if that’s effective you obviously will get Aspirations and that’s one of the things we try to avoid so then there’s the pharyngeal phase and the Pharynx is squeezing here to push the food bullets down into the esophagus and Then here the food has reached into the esophagus after which the esophagus has peristalsis or series of contractions or milks the Bolus down into the stomach So that’s how the food bolus goes reaches the stomach So like I was telling you about all the muscles on the on your facial muscles are all skeletal muscles and they aid in mastication So those that they’re involved depending on the degree of involvement The process of mastication may be mild later severely affected and you can’t really chew Also one of the consequent of a poor facial muscle Tone is poor development of your jaw because the jaw and the occlusion is All dependent upon the pull and the tone of your facial muscles when you don’t have good strong facial muscles your jaw does not develop and a lot of our kids will have what we call an open bite and Malocclusion so here’s a picture of the airway this is the esophagus is right there as you can tell they’re right next to each other the swallowing to your esophagus is at the back and This wide-open thing here is the airway you do not want food going down here when you’re swallowing when you’re breathing Yes, that should be open so you see the vocal cords when you swallow these vocal cords they shut down The epiglottis comes back down and shuts off the opening into the airway This is what I was referring to these are all of the muscles. This is a lateral view you’re now you’re looking at the Neck from the side this is the Tube their trachea And these are all the muscles that help shut the airway as you’re swallowing and again these are all Skeletal Muscles which are affected and in dystrophy and can give rise to depending on how severe the involvement is can give rise to aspiration So whenever there is a nutritional issue or feeding disorder. We have an interdisciplinary feeding team which is part of the bigger dystrophy team at our center And I was part of this and essentially we offers family-centered care which you’ve heard about Where we make shared decisions There’s the md or nurse practitioner the nurses the registered Dietician the therapists speech therapists occupation physical Homecare very important that will talk about nutrition g-Tube and all of those stuff later And of course a social worker to kind of help them navigate the system If there is a problem with feeding or the child’s not gaining weight Appropriately any member of the team can identify that problem with the swallowing feeding and nutrition any of them Including the social worker oftentimes we’ve had referrals from Social social workers saying hey So-and-so The kid was doing really well, but now he seems like he’s up having some problems you haven’t seen him in six months Can you evaluate it and then brings them back into the fold When you have problems with oral or pharyngeal Swallowing and biting these are some of the symptoms that should be you should be looking for coughing or choking with swallowing which is indicative of aspiration potentially difficulty initiating swallow food sticking in the throat Drooling of saliva unexplained weight loss because you’re not eating enough change in Dietary habits you start eating preferring certain kinds of foods that you know your body is telling you. Yes that’s safe that you can eat You start avoiding some like you avoid water. It’s easier to aspirate recurrent pneumonia if it gets getting 3-4 pneumonias don’t keep thinking. It’s because of poor clearance don’t blame the pulmonologist all the time it could be aspiration Change in voice or speech again because of the muscle involvement and of the airways There’s a nasal twang and that kind of indicates Hypotonia are the muscles When the bolus goes down into the esophagus, and there’s problems with esophageal motility that kind of stuff you would typically have the sensation of food sticking in the chest and Sometimes you also get the food come back into the back of your mouth and out of your nose and that’s got a regurgitation through the mouth or the Pharynx and Then you can get the rest of those symptoms including chest pain in a diner feature which means Difficulty or painful swallowing, so when you swallow it, it’s very painful. We kind of have this feel this pain in the your chest the rest are essentially the same as oral Pharyngeal Dysphagia Now I just want to take a step back to kind of review What is malnutrition a lot of times you’re told all your child is malnourished What does that mean so the world health organization, and that’s what that stands for not the musical group band malnutrition refers to deficiencies excesses or imbalances in a person’s intake of energy and/or nutrients the term malnutrition covers two broad groups of conditions One is undernutrition and the other is overnutrition And we see both of these we can see over nutrition and obesity as well as under nutrition in our population Within the under nutrition group you could have stunting where you have low height for age Meaning if the five year old the child’s really short that stunting wasting is low weight for height So the child is tall, but he’s really skinny That’s wasting underweight is low weight for age and then of course you can have all the different Micronutrient deficiencies when I say micronutrient, what does it mean? vitamins and minerals Now you also heard this thing’s failure to try the child is failing to thrive as opposed to malnutrition So failure to thrive is a significant interruption in the expected rate of growth. What is significant who decides? What is significant with weight Loss? so you Take help of an fermented Criteria What is the criteria it is the weight the height the body mass index and all of those To define and there are three different definitions if your weight is below the 3rd to the 5th percentile on the growth curve on more than one occasion Means it’s persistent Some people like to call that failure to thrive The weight measurements that fall to two major percentiles using the standard growth charts if it falls the growth is going okay And then it drops two percentiles. That’s failure to thrive Others like to use serial z-scores for weight for age or weight for height, and if it’s less than 2 standard deviations That’s failure to thrive for them now. This is all a very kind of bookish knowledge, and if you really look in Real Life I Personally it I can use these for sure But that doesn’t make me this is not the only thing that determines that this kid needs say for example a g-Tube That’s not the only thing you can use these Parameters, but you did are other things that come into play? You know you’ve got to talk to the family what the kids the goals that they were talking about You need to know what they are so yes, these are good too. Have as definitions, but Practically you may not necessarily use just these to make Decisions other measurements that we’ve used in Some our kids mid upper arm circumference essentially you measure the girth of the mid upper arm You could do triceps skinfold. There’s a caliper that you use Then pinch the back of your triceps or back of your upper arm And you determine that serially to see if there’s any loss of muscle mass then there’s the MAMC or mid arm muscle circumference, which is calculated with this formula again good to know and Sometimes good to follow a monitor and say oh well his mid arm circumference is going down or the MAMC is dropping So this is the only thing I’ve found in literature showing a growth curve of a boy with merinos deficiency congenital muscular dystrophy and ignored this Blue Line here, so what this is trying to show us the child did well until about three years of age well this died, so so the kid did well and about three years of age and then you can see those x’s Kind of flattening out and then actually falling more and more away from the Growth chart Now that is a problem I don’t care two percentiles or whatever but the question that most people will ask is would you at any point? There or and where would you have recommended a g-Tube or supplemental feeding? And we’ll talk about that some people would say well at the first Impression where the kid had started to falter and straighten out or plateau we should have put a g-Tube others would say no I would have waited and Because he was still kind of trying to go and put argument. You know go ahead Excuse me It certainly is possible absolutely so don’t just go by that so you have to take into consideration Other things do so that underscores my what I was trying to say The blue line there is what we see in a lot of our kids where they’re not where they’re not Following Their own center They’re nowhere on the Growth curve But they are following their percentile and a lot of the pediatricians will completely freak out and say all your kid needs a g-Tube tending to the gastroenterologist or a G2 know the Lot of our kids fall under that and they continue to do well, you know kind of maintain their own percentile And that’s perfectly normal So just some facts about CMD energy expenditure is much lower in those who are non-ambulatory that’s not necessarily true Just for CMD but for other conditions as well such as cerebral palsy ETC. Those children need less calories However having said that there are some kids that I’ve seen with CMD that needs significantly increased calories to grow Not sure why we could talk about that a little bit later the weight loss that you see may not accurately reflect failure to thrive because Muscle atrophy may be caused by the underlying disease rather than poor nutrition So the weight loss may not necessarily be because the patient’s losing weight from poor nutrition It may just be from muscle atrophy because of the condition of CMD That makes sense Then like I said the weight may track along below in parallel to the third percentile, and that’s fine. That is absolutely fine Remember the absorption capacity of the gut is normal There’s nothing wrong with the absorption of the gut as far as I know I haven’t seen yet anybody that has malabsorption So the gut malabsorption seems to be normal swallowing dysfunction may not parallel muscular degeneration, so if you’re seeing that the general skeletal muscle is Worsening or the the tone is worsening over time It doesn’t correlate well with the swallowing dysfunction you may get worsening swallow dysfunction even before the rest of the body starts – the muscle starts to Get worse go ahead so the pediatricians can honestly have given the staff of the pediatricians and Try to tell them but they just I think it’s more from Lack of knowledge rather than their fear of other We don’t want to do anything. This is a very complicated kid We don’t want to you know mess on with the nutrition and let’s let the gastro. Just take care of it Which is fine, and we kind of we’ll just reassure the parents you’re doing fine and the kids doing okay? But what we typically like to see is if you can do a real weight. We definitely do weights We do all of those things that I mentioned all the answers were metric measures You don’t just do one I don’t think there’s would be fair for me to say that’s just the mid-arm Circumference that you take and and nothing else So we kind of take the whole conglomerate of all of those measurements and put sit down together and talk about what the RD and say okay What’s happening here? So not just the BMI or not just the weight did that answer a question so So the first thing you do is you ask for a for the kid comes to us and what do we do the pediatricians are freaking out all the kids weight is down and There’s something going on with this kid you need to see it So the first thing is what has been the weight trend not the one point weight. What is it? What’s either done through the whole kind of past couple years or longer? Talk about chewing and swallowing How does the kid to ask the parents does he chew well in tongue movements Dietary adaptations a lot of the kids when they have these Swallowing problems will either tend to kind of when they swallow they put their heads back, and so those are just Natural reflexes of the body which tells them that if you do that It’s easier to swallow or they’ll flex their head or turn their head So those are some of the things that you asked for and then how long does it take for them to feed does it take Them an hour to feed to have a meal time so those are some of the questions you ask and then also about Coughing and choking does the kid vomit does he get recorded chest infections again the current chest infections could mean worsening the clearance something wrong there or aspiration or both Wet sounding cough or gurgly breathing. That’s another key thing we asked for and that sometimes Indicates that the kid is at risk for aspiration So when secretions are not cleared because the Pharynx isn’t strong enough to put to push the the bolus into the esophagus Secretions tend to collect in the basket or Pharynx and then when you breathe through it you get that web gurgly sound of breathing So I hope that sounded right, so So then you asked about meal times are they stressful are they enjoyable or you know how? Intense will say it takes an hour And we can’t handle this we have other stuff to do in and just take forever to feed the kid And then of course you have to actually assess the kid you get out of history make your opinion And then you actually see the child and you see what he does you Assess the anatomy look at the lips tongue jaw palate structures assess the function abnormal mouth Architecture look for that open bite look with the interior jaws when they eat a lot of it just falls out of the mouth Malocclusion of teeth Observe the child eating a meal and record the mealtime, so this is kind of what our speech and language pathologists do for us and Then the video swallow study I think somebody alluded to this earlier and those question is when you do them so first of all What is the videos fluoroscopic study of swallow it’s also called a modified Variant swallow different institutions all it differently So what is it? It’s a radiological or x-Ray study of contrast passing from the mouth to the esophagus it allows oral manipulation and swallowing efficiency with food of various Consistency so you first give liquid see how the child does with the liquids and then you give them semi solids and then you give them solids and you see how the Chewing movements are how the child is moving how’s the tongue movement with the bolus is he able to squeeze that Pharynx Hard enough to push the bolus or is there residual stuff staying behind the pharynx after he swallowed? So you look at this oral phase which actually is the first phase which gives information about chewing the ability to manipulate food into a bolus and Transfer it to the oropharynx to Initiate the swallowing process and then the Pharyngeal phase shows the efficiency of the bolus transferred through the Pharynx and also any Penetration of the laryngeal inlet so you can see that contrasts if it’s kind of Wanting to go into the airway and not the esophagus That’s a warning sign that’s called Penetration and sometimes you can actually see the contrast going straight down the airway, and that’s frankly aspiration So what are the abnormal stuff that you could see you see deficient buckle closure where the mouth is not tightly closed? and there’s anterior loss you can drool the child has abnormal tongue and jaw movements leaving leading to difficulties with bolus formation and They just keep the food and pocket it in their mouths they don’t swallow just keep it in their mouth and keep it Making these chewing kind of movements when they’re really not chewing it is pocketing it because the body is telling them you cannot you cannot Swallow if you swallow you’re going to aspirate so that’s kind of the reflex mechanism underlying that This delayed onset of swallowing so again holding it there because their nose they swallow they may aspirate Nasal Pharyngeal Penetration reduced Pharyngeal peristalsis or reduced contractions at the back of the throat and Then failure of Pharyngeal emptying this is the residue that I was talking about after the kid had swallowed There’s still Persistent food bolus in the back of the throat means he hasn’t cleared completely And what does that mean when he starts to breathe that might just plop right into the airway? Contrast penetrating the laryngeal inlet and then the Franck aspiration with the contrast following Following below the vocal Cords into the trachea another important thing is you need to make sure when that happens is the kid is aspirating is he coughing or not a Lot of our kids after a while will won’t cough Cough is a natural reflex right when you ask for it your body tries to get it out of your system So you are coughing and you throw that thing right back out? But they don’t have that ability to cough because of their muscles their chest muscles their violence your muscles. It’s not strong enough to Generate enough pressure to push it back so quietly just keeps going into their lungs slowly over time and getting worse That’s why that’s called silent aspiration when you hear the word silent aspiration. It’s a little bit scary okay, so and why is it silent it’s because the deficiency of those muscles in their inability to kind of throw the thing back out So aspiration when you look at aspiration It can occur at the thymus Before the time of swallowing so here the kid has the food is still in the mouth You see the blue thing it’s still in the mouth And it’s already trickled and aspirated into the into his lungs even before the swallow occurred This is as the child is trying to swallow it goes both in the esophagus and into the Airways, and this is after the kids swallowed some fine and but the aspirant is going into the into the airway and esophagus So it can occur at any time. This is the worst obviously So this is a picture of a video fluoroscopic study, obviously it’s a video And I didn’t get a chance to download the whole video, but here what you’re seeing Is this is the airway that in the this is the contrast the black and it’s going into the esophagus But as you can see there’s some black in the airway as well And this is the little amount that that patient has aspirated into his airway So when you have dysphagia which means difficulty swallowing you can aspirate and then you have poor cough reflex where you’re not able to cough and it’s a silent aspiration you get pneumonia a Word to be said about oral care if you don’t have good oral care if you have gum disease you have gingivitis and you have Infection there that can contaminate your saliva and your secretions, which can go into your lungs and cause a bad bacterial pneumonia You can also reflex secretions from your stomach and then that can go into your lungs as well So that’s called acid reflux with pneumonia So you can have pneumonia from aspiration from above or from reflux from below So now you are told your child needs to gain weight alright. So what do we do? Well, we have the formulas Formulas formula can be taken orally there are several formulas out there that have different flavors strawberry vanilla Et cetera that can be taken orally, so if they can do that thickened they will take that by mouth and hopefully gain weight typically volume per volume Formula have more calories than does any other thing that you eat other than of course you can make a thick Smoothie with you know fat and stuff like that but typically you get more calories that way now a lot of the formulas can also obviously be given through the g or the GJ tube and we’ll talk about that later The former could be classified as cow’s milk protein based soy based With no cow’s milk protein for kids that are allergic to Cow’s milk protein lactose free for those that are In lactose intolerant if you’re allergic you could do either elemental formula or amino acid based formula There’s some organic formulas available then you can stick in them using different kinds of thickening agents that are commercially available Or you can get you could do pureed diet through the g-Tube not the GJ But the g-Tube and that you can make at home under the supervision of a dietitian She’ll tell you what to put in that pureed you use a blender at home put all the stuff then blenderize it and give it To the g-Tube as a bolus, and then there’s commercially available Pureed diets as well so the formula There’s the regular supplement regular formula is like one calorie for a one Ml. That’s the regular formula, then there are concentrated formula which are 1.52 up to two calories per Ml But remember you have to be careful when you’re giving concentrated formula because you need to determine Free fluid intake and need for supplementation because a lot of these kids are not drinking enough just by giving them extra calories Yes, you’re giving them the extra calories they need but you’re not giving them the very important free fluid that they need with it so be careful about that and the dietitians can calculate how much free fluid that your child needs, so And often times I tell you a lot of our kids with CMD are not drinking enough fluid Also, there are high protein containing formulas available because oh well. It’s the muscle degenerating or getting dystrophic You need more proteins to get to prevent that or reduce that or whatever But remember when you have a high protein load that can affect your kidneys if your kidneys, take a hit You don’t want an addition a problem there? so you have to be careful about not giving excessive proteins either if The child is exclusively formula fed You don’t really need to supplement vitamins, but if you’re not on a formula Which have which are pretty complete in their vitamin contents, you can look in the ingredients, then you do need a supplement so now you’re told Your child is having inadequate weight gain Despite the mother saying I keep giving them all the things you tell ever the kids weight is going nowhere so what are the reasons so even the supplemental calories may be inadequate for the child or There may be some absorption issues from the gut which we haven’t diagnosed or checked or looked for There could be some vomiting and/or diarrhea that where they could be losing those calories Usually not seen in our Cmd kids at least not that I’m aware of let me know if you do Increased Calorie needs and that’s probably one of the major ones in a lot of our kids with CMD Because any chronic medical conditions are just going to have a heart disease or chronic pulmonary Disease which I’m sure that Dr. Hemant can tell you they have and that increases their need for more calories But you’re not giving them enough, so therefore. It’s a relative deficiency of calories there So what do you do for management? So there’s three categories, so you put your child into one of those categories failure to thrive Or poor weight gain with a normal swallow first thing you do is you try to I? Lost my pointer the first thing you do is Supplemental High-Calorie formula and beverages like we talked about increase calories and their solids by adding up butter and oil and you can only do this if your swallow is normal do not be adding oil and butter to your child solids if there is even the risk of aspiration because if you aspirate fat oil greasy stuff you get lipoid pneumonia, which is Extremely dangerous and potentially fatal So now if the child has failure to thrive and has an abnormal swallow But no aspiration detected then you can do the speech and language pathologists will talk about seating and positioning their heads And adaptive skills and devices to assist with feeding Viscosity thickness modification pacing namely don’t swallow too fast go slow or a motor therapy with a therapist And then if it’s a third category where you there is aspiration or a very high risk for aspiration on a video fluoroscopic study Then you do all of the stuff in second in number two, and then you only allow Safe Foods by mouth that could be just solid certain textures and Then that’s the point where depending on other things Consider a g or Gj Tube and I put both of those together because it all depends And we’ll talk about what are the what are the things you think about before you come to make that decision? Timing when do you that’s kind of a very? Question always ask when should my child get atG Tube It’s a difficult question to answer and like I said earlier you have to put a lot of things into consideration Before you come to that decision, so the child is having persistent weight Loss Despite you all your efforts to increase oral calories and nutrition. You’ve done your best and the kid is not going anywhere his weights still going down Persistent dehydration calories going to be fine, but the child’s getting dehydrated because they’re not drinking enough fluid aspiration recurrent pneumonias Worsening respiratory strat status worsening muscle strength prolonged mealtimes child’s family readiness we have to look into that and can the child tolerate anesthesia now if You think oh while I want to get a G2 because we’ve checked on all the rest of the stuff and the Anesthesiologist says hell heck No
We can’t do this because his rested his muscle and his respiratory are so poor he cannot withstand Anesthesia because it has to be done on a general anesthesia, so that’s out. So you have to kind of make that decision taking into consideration all of that, so I personally and I’m just saying if this is just my personal beliefs I like to put g tube earlier right at the beginning of any Abnormal swallows that I start seeing or the child’s getting more pneumonia the the wasting has started and the pulmonologist is saying you need to get the nutrition better and Parents are saying we are trying our best to get all the nutrition. You’re telling us but it’s really not getting so all of those you consider, and then say yes, let’s go ahead and do a g-Tube so Improving your nutrition and some fluid status why do we keep saying? Improve nutrition improve nutrition get the fluid status under control and that’s because if you have good nutrition it Delays the muscle atrophy that’s been shown it improves therefore your mobility it Slows the worsening of your respiratory status because your muscle is not Going atrophying as quickly so the strength is preserved So your lung function is preserved it delays the type of scoliosis Because your muscle strength is weak your kyphosis and scoliosis tend to get worse it induces Puberty a lot of times we’ve seen a lot of our kids because they are so Poorly nourished that they don’t go into Puberty until 16 17 18 19 Sometimes so once the nutrition status gets better. They go into Puberty and that’s a huge psychosocial Thing for them it reduces osteoporosis better nutrition. You’re getting enough vitamins. You’re getting enough calcium. You’re getting enough You know the rest of the nutrition? So it reduces a proper the osteoporosis as well Improves cognition and mood if your nutrition is better you’re hydrated and everything is good. You feel better your moods better You’re not depressed children that have under nutrition are depressed, and you really don’t want that in addition to everything else That’s happening and of course because of all of that it improves quality of life So the need to increase calories like I said you could do. I think we’ve already got gone over this let me just skip it So just a word about Vitamin D and Bone health The lack of Weight-bearing is a major contributor of bone loss so if you don’t wear bare weight You tend to lose bone body mass okay, and that’s been shown in all cerebral palsy kids that don’t walk and non-ambulatory kids and what is osteoporosis? It’s a systemic skeletal disease systemic meaning. It’s not just one single bone is in all bones it’s characterized by reduced Bone, Mass, or bone mineral density referred to as BMD and Structural deterioration of bone tissue leading to thin and porous bones with a subsequent increased risk for fractures And what’s scary is the next point? There’s a strong inverse meaning opposite association between bone mass by Density and fracture risk there’s a two to three fold increased risk of fractures for just one standard deviation Decrease in your bone marrow density, that’s pretty scary In addition a lot of our kids is on are on chronic steroids and other medications that can potentiate that reduction in bone mass density so puts them at high risk for fractures so there is this very interesting study and looking at BMD and Vitamin D Levels, but this was a non-ambulatory children with cerebral palsy and they found that the main predictor of low BMD scores was the inability to walk that was the one thing that affected it majorly and then the Vitamin D status Really did not correlate with the BMD z score that was a very interesting finding there because we would always think well if the vitamin D is low that’s why you get osteoporosis? but there really wasn’t any relationship between the blood levels of Vitamin D and the z-scores on the Bone Mass Density Bone Mineral density There’s another study Looking at effective interventions to improve low Bone Mineral Density in cerebral palsy again I’m using cerebral palsy there’s really no data for us So they said for the use of bisphosphonates so you guys know what bisphosphonates improves mood improves Bone density it’s a medication the Efficacy was probable it probably helps nothing categorical Vitamin D and calcium supplication possibly helps weight-bearing activities Insufficient evidence to say one way or the other so it kind of doesn’t go along with the prior study that said that So just to kind of say you know there’s a lot of data out there that we are Extrapolating from other conditions which may not be true for our CMD But that’s kind of the more similar kind of group that we’re trying to get the data from and using it for our CMD good But again, it’s it’s not it’s not it consensus-driven There is no consensus between any of the gastroenterologist and nutritionist if you talk to them And that’s one of the things that I would strongly recommend this for coming up with a care consideration for Congenital muscular dystrophies where all the experts are done and even though we don’t have a lot of data But then from their experience that have seen a lot of kids Say this is what we should be doing in the solution, and then that other guys across the country can do We did a similar thing for DMD, and I was part of the Gi group the last There may be some rule for testosterone and improving Bone mineral density in adolescent boys now refeeding syndrome whenever if Your child is really really malnourished and you get a G2 when you start the doctors go crazy about you know starting calories and start throwing all these calories that at the child that’s when you have this problem. Which is can be potentially fatal So what is refeeding syndrome? It’s a metabolic disturbance that occurs as a result of reinstitution of nutrition to patients who are starved or severely malnourished There’s a potentially fatal shift of fluids and electrolytes that result from hormonal and metabolic changes that occur in your body as you’re getting this barrage of nutrition suddenly when your baseline malnourished and what it does is there’s a Significant drop in serum potassium Magnesium and phosphorus that leads to neuropathy vomiting ataxia ocular cranial pulses tachycardia Fluid overload and Sometimes death and to avoid this refeeding syndrome want to get a g-Tube what we always do is to increase the calories gradually don’t Don’t just start flooding in with what you think they need always Start we start with a one-third required then we go to two-thirds after a few days, and then we go to The entire amount that we need to give them and then of course you have to closely monitor for blood work For two to three days after you start increasing the calories to look for the potassium magnesium and phosphorus drop now Moving gears a little bit what are the advantages of G&G tube feeds pretty intuitive it improves nutrition and hydration? Meal types are much shorter. They are less stressful it lessens the risk for aspiration, and there’s better quality of life So these are some of the g-tubes most commonly we do endoscopic placement, and it’s called peg or percutaneous endoscopic placement of Tube there on the left side you see this camera the endoscope going down into the stomach We make a little nick in the and the anterior abdominal wall and then that’s what the G tube looks like so that you look on the left the brown part is the interior of the stomach And that’s your body wall the brown right there is the stomach wall and then the anterior body wall So you see the tube going through both the walls into the stomach This is what a g tube looks like most commonly there are different kinds of g tubes. I’m just showing you one But I’m sure the people are in the room that have this they know what? The things that you need to know is what kind of g tube your patients and your child has is it AMT Is it a mickey button? What is it and what are the sizes is what is a French Which is the diameter in Red the diameter of the Tube? What is the length of your g tube so two centimeters 2.5 centimeters? Whatever, so when you go to the emergency room if your tube has fallen off you tell them hey my child has an amt 14 French two point five centimeter balloon This is a GJ2 where you see The there is a part that opens into the stomach the blue part and then there within that there’s another longer Tube that goes past The stomach into the intestines and you’re feeding into the intestines when do you do that? We sometimes do that when a child has gastroparesis meaning the stomach is affected It’s not contracting and the foods just sitting there and causing him to vomit and sometimes Aspirate so you feed past the stomach also called Trans pyloric feedings so you’re feeling Beyond the stomach and into the Jejunum or the small intestine, so the food the formula doesn’t come back into the stomach because there’s a sphincter muscle that shut Between the stomach and the intestines it prevents that from coming back Into the stomach the the beauty of this is that you have to gastrostomy Tube That you can vent and let the stomach and air and everything out of this stomach this is what the GJ tube looks like so on the left you have a button a Gj Tube button and on the right is a GJ tube the difference being the button after you take out the Attachment for feeding it’s just a little button there But the Gj Tube has this long tube that always sticks out of the belly there’s different reasons. Why we use these So that’s a skin level device and that’s not in the GJ tube you have those three Ports one is the gastric port going into the stomach we have the general port where you feed and then of course that’s the balloon on the other This is what it looks like endoscopically So you’ll in the stomach you see the balloon coming into the stomach and then that long Tube that’s going past out of the stomach and into the intestine remember when you have a Gj tube – as opposed to a g -tube You can only give continuous feeds using a pump So you need to have a pump and you have to Continuously feed you cannot give a bolus like you do for G Tube Just take a syringe in both of the g -tube you cannot do that with a GJ tube you have to have continuous And you need a pump if the Gj Tube gets displaced meaning sometimes it kind of comes back into the stomach then you’re essentially feeding into the stomach, so I’m almost done So you have to go to a hospital to a radiologist to actually place the GJ tube you cannot do it at home you? Cannot the G tube you can but not a GJ Tube And then like I said the g port could be used to vent the stomach or give medications These are some of the problems you can get with the tube g or GJ Some leakage skin Irritation granulation issue gastric mucosal prolapse clogged Tube some gagging retching after nissen fundoplication I think I’m going to stop right here and take questions if there are any yes, sir poor nutrition we go yeah, so two questions related to your speech it’s A Calcium and Vitamin D As we have daily needs of vitamins and nutrients What is the expected? duration of this Supplement is it until you are? Teenager or or is it something you can do every two months and then stop one month and then two months? So what is the frequency for these of the multivitamins? Yes kind of calcium and Vitamin D. So it all depends? I mean even though the data showed. Otherwise a lot of the people are still Measuring calcium and vitamin D levels and using that to supplement, Vitamin D We typically put them and a lot of these kids have to be if you look at Vitamin D deficiency? It’s very very very common even in the normal population and on top of that if you have dystrophy, it’s even more makes the problem even more acute So you really want your vitamin D? levels depending on the lab you’re using but about 30 is what we aim for And if it’s dropping below 30 then we try to keep the supplement on however long it takes Vitamin d. A lot of our kids are on it long term calcium is a different you have to be careful with calcium because if you give additional calcium in your stomach it can cause a lot of other problems because when you’re giving other medications through the through the with the calcium it can bind some of those medications and reduce their Efficacy of the other medications, and you can get some stones and stuff like that you have to be careful that calcium I’m we don’t give calcium supplement as much as we do vitamin D. Vitamin d for sure and a lot of them are long-term Excellent and the second question is regarding a collagen there are some kind of Marine Collagen you like That you can be viewed with a water and drink it Is something that could help because of the lack of production of the Collagen VI I doubt but I Honestly have no experience using that but I seriously if you look at the Pathology of what’s happening in the collagen disorders By giving extra collagen, and it’s not going to really get to where it needs to be or help you there I don’t think but again. I might be wrong. I’d be the wrong person to ask So I don’t know Yes, ma’am. Hi um I have a four year old son with Collagen six He is four years old thirty pounds even though he’s not losing weight He is not gaining it it seems. He’s been the same way for the past year or so What is the calorie intake for his age? Considering his condition I do believe he is adequately fed with snacks and Proper nutrition I am confident in that 110 percent But I’m not seeing any positive effect on on his weight a g-Tube was Kind of mentioned to us if he considers to do so um So I’m kind of hoping for input as far as calorie wise Pediasures were recommended He is not taking those of course we would you the butter with there the peanut butter Chocolate on top of the peanut butter you know anything that you can possibly think of that normal people get fed on you know So, what is your? like I said Putting a g-Tube is not an easy question there’s a lot of things you have to not just the weight So if there’s potential for him if his oral motor skills are good, and he has no problem swallowing well We say yes, but a lot of times there is some subclinical thing going on And I cannot tell you how often not necessarily in CMD case, but in other kids that have feeding issues Have you know but those are especially kids that take long to eat? He doesn’t take long to eat? He does take long to eat. He’s never aspirated um ever That I know was um he’s never had pneumonia. So his lung Health is still still good I think it was mentioned to us more so to be proactive than anything like you mentioned earlier You’d rather put it earlier than later um but Aspiration is not the issue. It’s strictly Strictly just the weight and lung as a result so you say aspiration is not an issue are we sure he has no subclinical aspiration because We all aspirate you aspirate I aspirated and you know as a result he never has had pneumonia or been brought to our attention otherwise He is fairly Fairly healthy, so just the late swallowing like Taking time to eat it could be good. Yeah, it could be an indication that something might be going wrong So I would definitely get a swallow study before you decide on anything well swallow study for sure because that gives us a lot of information is it just purely a matter of Weakness in the mouth and that’s kind of saw a weakness in the Pharyngeal muscles Or is there more say can you see the penetration? Can you see not actual aspiration and you might be surprised there may be aspiration and he’s not do you don’t know that He’s getting at for having aspiration and the other thing is you get pneumonia? Only if you aspirate a large amount okay, so if you ask for it a little bit little bit little bit Okay, you don’t get the pneumonia, but it’s enough to worsen cause more inflammation and worse It’s worse than lung disease for your kid. I would definitely get a video swallow study, okay? I’m kind of that’s not the only thing again There’s other things so if there’s muscle waste you no more wasting and the lung function is deteriorating So that’s another indication Why is there this lung deterioration is it because of nutrition or is it because of aspiration you know silent aspiration Small not enough to cause pneumonia But enough to worsen the lung function is that contributing so we need to get a little bit more information? Okay, we appreciate it. Thank you. Yes, ma’am Okay, so with the movement because of her scoliosis and meant her surgeries with a growth Rod and then her future fusion Should we be concerned about? Her g-Tube not being in the right place you Know will she need a future surgery as she grows back her g-Tube to be replaced you know Sure, and those are excellent questions. Well, how old is she So she’s probably not going to grow much now the ones where we sometimes have to change the gastrostomy site is first of all it’s very rare and if they placed itat too high below the ribs and when they get the scoliosis and it just gets underneath the ribs So you have to be careful most cases that I’ve seen we haven’t had to change the site of the gT Because what we do is We stay away from the midline and well at least two finger Breaths down below the rib cage So there’s enough kind of space there, so and then on the inside was more important than the inside of the stomach when you have the scope in there you want to be not too close to the exit of the stomach you need to be a little bit away from it, so towards the lower body of the stomach main body of the stomach so so yeah, so honestly in all my years 20 plus years I’ve never really heard that they had to change it because of scoliosis or But I will tell you our orthopedic surgeons before they put their kids on For scoliosis surgery, there’s two things they ask us one work on the nutrition yet They just kind of ordered you know wonderful surgeons you have to improve your need the child’s nutrition. Yes, sir and two Give us a recommendation of what we should do with the constipation which we didn’t talk about today as to clean out prior to the surgery and so those are two things that they send the kids to us for the orthopedic surgeons to Did that answer your question Thank you Yes, ma’am To place when he was around two years old And we struggled with vomiting for the longest time so we switched it to a GJ in January starting March 6. He’s been throwing up Every day, and we can’t figure out why we’ve switched formulas. We have gone back to the G because with the J He was throwing up at the same time every morning We’ve had an endoscopy done We’ve tried different medications for motility medications for reflux Zofran Nothing’s helped so the first question I asked is is the gastric emptying normal As far as we know yes, he still has wet diapers his bowel. Movements seem relatively normal So there’s a test we do for stomach emptying. It’s called a gastric emptying scan as he had that test He gives you an objective kind of number of how quickly the stomach empty is food no He hasn’t had that test. We did try erythromycin To speed that up, and it didn’t seem to make a difference, okay can I take a minute? Real quick, so when you look at the stomach let’s talk about this the stomach The stomach is shaped it is a dome of the stomach right that’s called the fundus of the stomach and then there’s the body of the stomach and then There’s the antrum which is the lower part before goes into the intestine so those three major parts Pylorus is a muscle that closed and opens up when the antrum squeezes stuff fast, so there could be problem with the fundus Because the fundus is important in Accomodation in other words when you eat food the fundus of the stomach the top part actually relaxes and becomes big sometimes you wonder how you can put a whole big huge plate of food that’s because the fundus relaxing and Accommodating the food the function of the body of the stomach which is a central part of the stomach is just squeezed Mix the food and then push it down towards the antrum which is a lower part the Antrum are the third part acts like a pump and it squeezes hard and squirts the food out of the stomach through the pyloric into intestine so you may have a problem with any of those or more that can lead to this problem of You know him vomiting. I’m assuming they’ve looked for other causes of vomiting like kidneys vomiting could be from other reasons Too kidney stones and the rest of the stuff you know brain sometimes increased intracranial pressure and stuff I’m assuming they’ve took taken a look at so you need to know if there’s any problem with the gastric emptying and if there is It doesn’t empty well then that the reason he probably got the GJ2 Now rarely some kids also have what we call pSeudo obstruction It’s like pSeudo means fake right the intestines act like they’re obstructed They just don’t squeeze and push fools they just kind of sit there and the food’s trying to go through it and This problem with that and then obviously it goes back into the stomach and you puke so is the intestinal motility normal or not So I don’t I’m not aware of any literature that says that children that have that condition of CMD have more Incidence of intestinal dismotlility again going back to the question of there’s really not been a lot of research done Nobody’s really looked at whether the muscles are affected the gut smooth muscles effected in children with CMD. So that could potentially be an issue too. So there’s a test called Android wardo manometry that we can do We simply place a tube that measures pressures, it’s got sensors built in the Tube So you put the Tube through the gJt site into the intestines, and you measure pressures and see if it’s normal or not, okay? So with the J and he was throwing up like mucousy type stuff every morning, but no formula, so Could that all be related to that as well? so sometimes Kids that have a GJ Tube And they’re small and they have a bigger GJ part the Tube going into the intestine sometimes Obstructs that pylorus muscle. That’s the outlet from the stomach and intestine because the Tube is big and it’s blocking the pylorus So whatever secretions normal secretion is the stomach is producing it can go out into the intestine and it sits in the stomach and you feel nauseous and He just throws up And we typically also recommend venting the g-Tube Before feeds and several times a day try to move the child to get the air Bubbles secretions and everything out before you actually feed and that can sometimes prevent we do Continuously vent it at night when we’re running the Jay Are you saying those secretions should maybe come out of the g and maybe that would solve the problem if it’s blocked by the g j tube So that’s you can certainly try that it’s an easy enough thing to do instead of venting it just at night when you’re feeding through the Jay and venting the G vent to the G during the day – Okay, see if that improves when does he typically throw up? Well when he was on the jay, it was every morning between like 5:30 and 7 a.m. And when we have him on the g now because he’s on a bipap, so we couldn’t risk him throwing up at night It’s hard to predict when it will happen There should be a motility issue either the stomach or the intestines or both so that’s something we need to look at he’s not constipated is he? no and the reason I say that is because constipation is very common in CMD kids and constipation can actually if your rectum And your lower colon is full of stool. It actually slows the stomach It’s called intestinal intestinal reflexes so the lower bowels talk to the upper bowels and say hey wait a minute We are full right now slow down so that kind of makes you more gagging and it can also gives you fill and reduces your respiratory movement the diaphragm Movement and stuff, so That’s what Dr.. David meant by Fos. If you were wondering what FOS was oh Yeah, I didn’t get a chance to talk about constipation, but yes some other time it’s a huge problem absolutely They all say Marilax, but honestly my recommendation is in I Honestly recommend more of stimulant laxative which is. I know this is off the topic and we’re going to the other end now but a stimulant laxative like Senna or Dulcolax Senna is an herbal preparation. I prefer Senna with was it Senna herb Senna Senna because laxative could be just a stool softener and or a stimulant stimulant is what pushes and CMD kids really need a stimulant Miralax itself to just make the stool soft. That’s not No, stimulant actually the Senna stimulates contractions in the colon and pushes it out Sorry Senna S E N N A Senna there’s different preparations as a liquid preparation there is a Chewable tablet that you can chew chocolate flavored. There’s also pills there extra-strength pills 25 milligrams per pill That you can take so for older kids you probably need those extra strength pills was that your question – All right anything else. Thank you so much for waiting guys