 Magandang araw sa inyong lahat, good day to all. I am Dr. Carmencita Padilia, one of the proponents of the newborn screening program in the Philippines. Join me in uncovering the wonderful story of newborn screening in our country. Together, let's zoom in on what makes MBS, a comprehensive program for every Filipino here at newborn screening in focus. To ensure that newborns are truly healthy, they must undergo newborn screening, a public health program that helps determine if a baby is born with one of the more than 20 congenital disorders. Its importance cannot be overemphasized. If any of the congenital disorders is left undetected and not managed immediately, it can lead to mental retardation and even death. It was integrated into the public health delivery system with the enactment of Republic Act 9-88 or newborn screening act of 2004. Now part of PhilHealth's newborn care package, newborn screening is being offered in more than 7,000 hospitals and birthing centers nationwide. It has also saved thousands of children. This educational series is intended for health professionals who deliver services of the newborn screening program. Whether you are online or offline, this program aims to further enrich your knowledge in newborn screening and be able to apply the highest quality of service to Filipinos, especially during the challenging times. We will discuss the very process of newborn screening from the moment the baby is born and into the continuing care available for newborns found positive. We will also zero in on the features and management in each of the conditions included in the newborn screening panel. We will also interview patients as well as their parents. And in keeping up to the challenges, talk over how facilities and centers managed to give quality service despite the limits brought about by the COVID-19 pandemic. This program is the newest educational platform for our newborn screening coordinators, one in every 7,200 health facilities throughout the country. We also hope that this series will also benefit the health professionals, physicians, nurses, midwives, medtechs, nutritionists, as well as students in the health professions. So take a seat, get comfortable as you're in for quite an adventure here at Newborn Screening in Focals. I'm Miss Christine Joy Conicolas, Mother of Martin Nicolas. I'm 33 years old, working as a back employee and assistant manager. My husband is working as OFW. Challenges kasi pagdating sa food, di kasi normal nung pag-take nila ng food, for example hindi sila pwede sa beef, chicken, pork, mga seafood, limited lang yung protein na kailangan nila iintake. So we have a dietician na ilan lang yung per grams na pwede ng itake ni Martin per day. Yung siguro yung pinaka-struggles talaga na mahirap yung gatas. Kasi yung gatas kasi abroad namin binibilay. May free kami nakukuha sa NIH which is yung donation. Ang anong kasi, minsan kasi dumarating yung point na nakaubusan ng gatas. So yung mga nagdodunit, hindi agad na kakapagpadala dito sa Philippines. So wala kami nang choice, need namin bumali abroad. Yung gatas niya MSUD para talaga siya sa maple syrup urine disease. Dun sa mga bagong mamis na may MSUD, kung makikita niyo kasi si Martin as in normal talaga siya, nakakapaglakad siya, nakakapagsalita, pumapasok sa school. Siguro yung ano na lang, yung control, yung control talaga sa mga kinakainong bata. Saka yung disciplina, yung number one talaga yun na, ikaw talaga yung magtuturo dun sa anak mo kung ano talaga yung pwede at bawal ka-inin. Talaga disciplina, pagsunod sa doctor, yun talaga importante para maging magandang risulta na may batang may MSUD. Nutrition therapy plays an essential role in restoring and maintaining metabolic homeostasis in MSUD. The goals of medical nutrition therapy in MSUD are to rapidly reduce toxic metabolites by restricting dietary, branched chain amino acids or BCAA to amounts allowing the individuals to achieve and maintain plasma, BCAA, amino acid concentrations within the targeted treatment ranges. Reduce catabolism, promote anabolism, monitor nutritional status and alter intake to promote normal growth, development and health maintenance. In this episode, we will learn strategies in feeding our MSUD patients with nutritious low protein food and products locally available in the country. In addition, we will also share some food preparations using low protein recipes. So today, we have four special guests, Dr. Leah Hamoy, a clinical geneticist and a metabolic medicine specialist from the University of the Philippines, Manila. Dr. Maveh Declaro, a medical specialist at the newborn screening continuity clinic in Beacoll Regional Training and Teaching Hospital. Dr. Calibo, a pediatrician and deputy director of DOST, Food and Nutrition Research. And Miss Jean Basas, a metabolic dietitian at the Institute of Human Genetics National Institutes of Health University of the Philippines, Manila. Welcome to newborn screening in focus. Dr. Leah, Dr. Anthony, Dr. Maveh and of course Jean are nutritionists. So in the last episode, we had an extensive discussion of what MSUD is all about. We discussed the short and the long term share of these patients and many times we discussed the importance of nutritional management. So for this episode, we're just going to talk about the nutritional management of MSUD. So let me ask, Dr. Leah, just exactly what do you mean by natural protein? And then maybe you can just give a very brief description or definition for what MSUD is for those who are coming in only just now. MSUD is an inborn error of protein metabolism. So because the body is unable to break down certain amino acids, particularly lusine, isolusine and valine, then these three amino acids are the ones that accumulate in the body. So ayaw po natin sumosobara yung lusine sa ating katawan kasi yun yung naggokos ng ating mga sintomas, naggokos ng neurotoxicity or damage to the brain. So in order to manage that through the diet, we therefore have to calculate the amount of protein that is being taken in. So yung po ang ating po nililimit ay yung natural protein or intact protein. Yung protein po na nakikita sa gata, sa pagkain, yung naturally nakikita sa ating everyday food. So yung po yung tinatawag nating natural protein, yung po yung ating kinaccount yung binibila. Okay, so Dr. Leah, in the last episode, you actually informed the viewers that the mothers can breastfeed. But you prefer that they express so that we can calculate the amount of protein that's being given to the baby. Is that correct, Dr. Leah? Yes, definitely po. We definitely encourage mothers to breastfeed because of the many advantages of breastfeeding. And siyempre, ayaw natin ang magkasakit ang mga baby nato dahil may MSU dina nga sila and at risk nga sila and breastfeeding will be protected. But we encourage them to measure the amount of breast milk that they give and they'll be able to do that if they express the milk and then count how many ounces or how many mls of milk that they give for that day. And they will follow what the doctors and the nutritionists have advised them to give in a 24-hour time period. Thank you, Dr. Leah. So yung mentioned the natural protein. So it's a matter of the baby with MSU, they should only receive a certain amount. So when you say natural protein, can we just give examples for a baby, what are the sources and as a baby gets older, what are the sources of natural protein? So definitely in the first six months, the main source of natural protein will be the breast milk. And there's nothing more natural than that. So that's what we get the natural protein from and we count and we tell them up to what amount they can get. So halimbawa sa sabihin natin, okay mommy, ganito lang po kadami yung ounces ng breast milk na maibibigay nyo sa baby. The rest of the energy will come from the specialized formula that is safe for them that has no leucine, isoleucine and valine. It's branch chain amino acid free. Once again, in the last episode, Dr. Leah, you were saying that the care of a baby with MSUD goes beyond the screening and the discharge of the baby from the hospital or the boarding center. So maybe at this point, I'd like to request Dr. Mavit to explain what is the role of the continuity clinic in the overall care for children with babies with MSUD. Dr. Mavit. Yes ma'am. So the continuity clinic caters to the long-term management of these patients. Once this patient is seen by the short-term clinic and a diagnosis, a final diagnosis is made, these patients are then endorsed to us and the continuation of the management is being catered by the continuity clinic. So Dr. Mavit, you're saying that in our program, when a baby undergoes newborn screening, the diagnosis is actually closed by the newborn screening center. And when the patient has been diagnosed and the patient is endorsed to the continuity clinic. So in your case, you are receiving the referral primarily for the vehicle region. Dr. Mavit. Yes ma'am. My area is the vehicle region ma'am. So those patients who already have final diagnosis from the vehicle region, it is then endorsed to us in our continuity clinic from the vehicle regional teaching and training hospital for continuation of care. Okay, so since we're talking about nutrition right now and we talked about a special formula, where are you getting your supply of special formula, Dr. Dr. Mavit? Ma'am, our special formula milk is being given by the NIH through the DOH. Okay, so once again we hear no that if you are a patient in the program, there is a provision for the milk, but you can see now the importance of the continuity clinic because of the continuity, because the milk will have to be delivered and given to the patients and they have to follow up. So Dr. Mavit, I just want to let the viewers know that you're not only dealing with MSUD, but you're actually taking care of all the patients with a positive diagnosis. Can you just give the viewers a chance, give them a list of the cases that you're following up right now before we just go back to MSUD? We cater to all the newborn screening, for all the diseases in the newborn screening program, such as the CAH, MSUD, what else, fetal ketunuria and all those metabolic disorders is being catered by the continuity clinic. So Dr. Mavit, what makes it more challenging to take care of a baby with MSUD? All of them are actually challenging ma'am, but with this MSUD patients really have to be very strict with their diets and really wanted to avoid them being sick in order for crisis not to set in so that they can live a normal life. So how do you check when you were saying that you want to make sure that they follow the prescription? So can you share with the viewers how are you able to check that the patients are really following your instructions? Yes ma'am. Adherence to the diet of these patients is very important in the management of our MSUD patients. Our continuity clinic does monthly checkups for these patients. So during these monthly checkups, we not only check for their nutritional status, we check for the progress in their development and we check for their growth percentiles and we also do monthly news in monitoring. So with this, we can gauge that with these tools, we can gauge if the patient is really adhering to the diet of the patients. And during also this follow-ups, we take time to talk to the parents. We have open forum if they are having problems with their supplies, with regards to their formula milk or their food supplies, or if they are having feeding difficulties with their children so that we can address it properly. Na-o-boost ng ka-banan supply, Dr. Mangve? Actually, just for the past two months, we have been previously kasi ma'am these patients, they receive at least six cans of this special formula milk per month. Pero the following months, they only receive two cans per month. So we address this by referring our patients to our dietitian so that their diet can be adjusted in terms of the availability of the formula milk so that other food products can be incorporated in their diet. Okay, so just before I call in Jean, I want to go back to Dr. Lee and ask how do you monitor that the amount of protein being received by the patient is adequate? By two things po. So one is we have to get reports from the parents themselves so they have to give us a report of their diet, what they've actually been giving their children. And the other is by monitoring their losing levels. So we have to do it very regularly and it's a continuous monitoring and adjustment of their diet based on the levels of leucine that we get. So kung wari po, mataas yung leucine na makuha namin and then we will have to adjust the diet to decrease the leucine intake or the protein intake. Kung halimbawa naman masyadong mababa yung leucine na makuha natin, then we will adjust the diet to increase the protein para naman hindi po siya magprotein deficiency. Okay, so both of you mentioned the importance of the nutritionist na Dr. Lee mentioned that when you start giving your supplementary feeding you really want the nutritionist. And here we also heard Dr. Mavic telling us that when they run short of supply, they connect with the nutritionist just to find out what alternative food items can be given. So let me call our nutritionist, the future doctor, Jean. Share with us your experience with the patients with MSUD. Okay, so the tough things na namin niladok mawe and it's very important that the diets of the patients are really monitored and that is why we really develop a relationship with this family, especially their caregivers where we try to do regular follow-ups and talk about their diet. Dr. Mavic already mentioned that we have to discuss what feeling difficulties they are experiencing so that we can provide assistance. As well as we want to make the daily diet convenient for preparation for our parents or caregivers and as well as palatable for our patients. It's very challenging, but it's very fulfilling once you get to see these patients grow up to their optimal expected growth. So, Jean, give us an example na. I mean, you want to give high energy and you've got a, let's say a gram. So maybe before I go to, before you answer that question, let's just talk about a one-year-old baby, Dr. Leah, just to give our viewers a chance. When you talk about how much protein can they receive and then maybe I'm going to ask Jean to translate that into food items, assuming that we've run out of milk as she was, she will have to give it. Can you give an example, Dr. Leah? So let's say that that baby is, that the baby's tolerance of protein is one gram per kilogram. And let's say that the baby's, let's just make it easy. And let's just say that the baby's weight is 10 kilograms and that baby is tolerating one gram per kilogram of protein. So that means that that baby can only have 10 grams of protein per day. So our nutritionist will be computing then where will that baby get the 10 grams from. So nibiglain niya ngayon po yung mga options na yun doon sa ating families. Hindi naman po ito, exactong-exacto na itong araw na ito, itlog ang kaka-inim mo sa kanabukasan ganyito. Hindi naman po natin silag dinagawang very rigid, but we educate them and we have to tell them that these are their options and ito yung limit of their protein that they can have. So yun po, for example, our 10 kilogram baby, let's say we'll be able to tolerate 10 grams of protein, then that's what we will compute for. And kung ano po yung energy or calories na hulang pa galing doon sa in-date niya, pupunan na lang po yun with our special protein-free or branch-chain amino acid-free formula. So Jin, can you tell us what is 10 grams of protein? If I have a baby who's, as Dr. Yia said, 10 kilos and I can only give about 10 grams, what is its equivalent in ordinary food? Okay, so if we are to prepare a diet plan for a child that is one year old with 10 grams natural protein, usually we assume that this patient is already finishing per complementary feeding stage. So as much as we wanted to maximize the natural protein to be coming from the regular food items, we want to allocate some of it for the milk. That is why at least 70% will be coming from the regular food items and 30% will be coming from our breast milk or whatever regular milk does the child have. So for that 7 grams that will be getting from our regular food, usually a portion of that say 2 grams or 3 grams will be getting it from rice. So that's 3-4 cup of rice. So from the 7 we're down to 4 grams. 2 grams of that will be getting from our vegetables. So we use vegetable exchanges that are modified for our patients. So now that we're down to 2 grams, 1 gram will be getting it from the food exchanges. This is aside from the free foods that we have. So the counted foods are banana, avocado, melon, those are the things that we get 1 gram from. And then the remaining 1 gram is for the child to experience snacks. So these low protein snacks that are available in commercial. So that is how we break it down if the patient has an increased appetite but with a limited protein we provide high energy drinks or 0 gram protein food sources to suffice the need of the child. Can you give us examples of 0 protein energy drinks? Okay. So for the 0 gram protein energy drinks we make use of our free foods book. So our free foods are apple, pear, watermelon, pine apple, mango. So these food items, they can be prepared as a juice or as a blended or as a shake to provide to the child. It can also be eaten raw at it. So these are some of our 0 gram. Jean, I need you to give me something that's more affordable. Okay. Erumba? Usually po, let's say 0 gram protein also comes from our certain nudas. So yung mga mura po nudas like vermicelli or sotanghon that is also 0 gram. So they can offer it as a substitute for a child instead of rice which has actual protein. So they can have that. In terms of yung mga mura po kasi na mga putas like banana these are also high in protein. That's why we need the help of a dietician to actually provide and indicate a certain amount of these food items if that's what the patient wants or the family can afford. Okay. So the message I'm getting from so far from my team is that you cannot guess. You've got to work with a team who will actually calculate the protein content of everything that the baby is receiving. Hindi siya pa pwede yung parabang I think low protein to so I'm going to take it. This is a fruit so it's low protein. So indispensable talaga ang role ng nutrition is when you start this the supplementary feeding. Now the biggest challenge really in the Philippines is that we don't have this low protein food items because there are commercial ones overseas and maybe before I call in Dr. Kali but to tell us what the government is planning to do can you just share Jean what are the other food items that are commercially available overseas that can be the protein free items that are being sold? Abroad they have available low protein pasta and noodles which are usually at around 5 grams per serving here in the Philippines. They also have low protein rice but the good news is we also have modified rice low protein rice here provided by one Japanese manufacturer that actually has .3 grams of protein per serving. In other countries they also have biscuits and even snacks like bread, pizza pasta sauces even chowders they have that soups that they can provide to our patients specifically made for IEM patients. So we're really hoping that soon these products will be made available here for our patients. That is the reason why I invited Dr. Anthony Kalibo to this panel because I am aware that the FNRI has some plans for our patients so may I now call in Dr. Kalibo I just want to mention to the viewers that Dr. Kalibo has been working for more than a decade at the Department of Health he was actually one of our partners and he has been part of the history of new functioning so now that he is at FNRI the Food and Nutrition Research Institute we'd like to hear what is in store for us from the side of the government Dr. Anthony Good afternoon Chancellor and good day to all our viewers all over the world for this virtual recording of our session and of course happy to be with the UP Manila team especially po with the newborn screening NIH, IHG The U.S. Food and Nutrition Research Institute is also very interested in pursuing a lot of R&Ds to benefit our infants affected with newborn errors of metabolism I think ever since we have been encountering MSUD patients and of course with enactment of the rare disease law we have received a lot of interest in developing products that will be geared towards the management of these groups of patients based on our last meeting last year I think that was sometime August that we explored the possibility of having a low protein floor and at the same time the type of milk that can be also locally produced under the Nutrition and Food Research and Development Division headed by the division chief chief science research specialist engineer Rosemary Garcia we have submitted proposals to the Pichard or Picard I'm not sure which among them will be funding this but it's the development of local low protein floor from local agricultural products and hopefully this gets to be funded so that the product development stage will commence middle of this year and hopefully we can implement this already by 2022 the other one is looking into the development of low protein milk and I think there are certain restrictions in terms of plasma amino acids which is I think very costly but this is already part of the research agenda and the team also of engineer Garcia is looking for external funding because the general appropriations fund may not be adequate to fund the research expenses necessary for this the other products that we are looking into is the non food related which are tools for our dieticians nutritionists and this will be looking into recipe guides or menus for in born era of metabolism patients and likewise even the food composition table that may also be customized what can be the unique needs of families with children or even young adults who have actually survived the difficult management of childhood MSU we are committed Chancellor to really pursue further technical discussions and like the technical working group between NIH, UP Manila and FNRI really concretize to have more forum for exchanges and discussions thank you for the invitation Chancellor thank you Dr. Anthony it's nice to hear that we have something that can probably materialize as you said by 2022 did I hear that right possibility that this will move by then so I guess for our viewers and this is not only for MSU the problem really is that these babies are alive they grow up and they have needs they cannot be dependent on milk forever and we really have to come up with the solid food items that they can actually protect so Dr. Leah do you have any reaction to the comments of Dr. Anthony anything else you'd like to request from him while he is here with us in this panel I was very happy to hear that for Dr. Anthony and we look forward to that and to collaboration with these okay so I think so far what we've heard from the team is that nutrition as we've heard many times in this episode and in the last episode nutritional management is the main state for metabolic conditions like the maple's European disease and that is the reason why we are trying to find ways to provide the local items to everyone I try to maybe request Dr. Leah answer this question because of course we have continuity clinics all over the country for the information of our viewers we have 14 right now and the goal really is to have one in every province you heard the role of Dr. Mave Dr. Mave explained that she was in charge of just ensuring the follow up of all the patients in the BICAL regional but then how do they connect with the specialist Dr. Leah maybe you can also explain to our viewers where will the expertise come from who will partner with them so the DOH recently released their administrative order that includes the formation of the centers for human genetic services in Luzon, Visayas and Mindanao possibly even more branches in the future and the CHTS or Center for Human Genetic Services will oversee the long term management for these patients diagnosed to the newborn screening program and will give access to the continuity clinics connect the continuity clinics to the experts in the different fields would you like just to say a gauge of where the centers for human genetic services are and then what are the plans because I want all the continuity clinics to feel that they are not alone and that the centers for human genetic services are there maybe I should tell them that Dr. Leah actually is the overall head of all the centers for human genetic services helping us now set up the different centers in the different parts of the country so I want our coordinators to appreciate that much as we have been getting the coverage that we need it's not enough that we have the coverage we have to start looking at the outcomes which is now the primary purpose the primary mission mission of the Center for Human Genetic Services so can you just mention just the so we're currently setting up or have set up the Luzon which is in Manila Visayas which is in Cebu we're setting up Mindanao which is going to be in Davao and we will be setting up soon another center in Luzon this time in Northern Luzon and another center in Mindanao so possibly Northern Mindanao and so hope to have those additional centers by 2023 Thank you So Dr. Mave, do you feel like are you getting the technical support that you need and tell us what else is needed so that we can work on it Dr. Mave? Yes, definitely ma'am being in the continuity clinic as a general pediatrician I really need to connect to this specialist especially our geneticist in order for me to in order for us in order for me to somehow manage the patient in our level So hindi ka nag-iisa Dr. Mave but I want everybody to feel here that we're trying to cover all the areas that's needed for the total care over the patient So well it's been a any final words let me start now first with Jean let me have some final words from each of our panelists before we close this episode Dr. Jean Jean Our nutritionist So I think the most important thing that I want to leave here is that dietitians are partners not only of the doctors but also of the families of these MSUV patients In terms of protein intake what we want to remember is you should measure to ensure that these MSUV children are guided to E-12 and UL So that's all Dr. Mave Yes ma'am I just want to tell everybody that the continuity clinic is here to help in the continuation of the management for this patient Even the little things like dadalhin namin you formula milk and deliver it to the families just for the sake of these patients ma'am Thank you Dr. Mave Dr. Leah The success of the newborn screening program particularly in the long term outcomes of our MSUV patients will definitely depend not only on the expertise of geneticists but also of the entire team and this time I don't mean just the medical team but as you've seen in this episode even our partners in the food development in DOS, the FNRI all of these contributions will help us to improve the lives of our patients Dr. Anthony baka gusto mo patagdagan ng promises mo sa amin First one to Secretary de la Pena's slogan of science for the people and I think FNRI being the premier agency of the government for food and nutrition restoration development we are morally bound to make sure that not all children regardless of their medical conditions we receive appropriate technology that can be accessible by those in need and clearly there seems to be really a gap of the availability of these products and that's where really R&D comes in what benefits are people I think should be really felt it may take some time but at least I think the realization that there's a need for these products for our children's future and welfare so I would really think the opportunity for hearing the issues and concerns of this episode and we will work together for the benefit of our children and for our future Salamat po Well thank you very much to our panelists for a very lively discussion on the nutritional management of MNU's UD our panelists reiterated at the value of nutritional management in the care of our patients and indeed this is a team effort involving so many teams the new birth screening centered team takes care of the diagnosis and the short term follow up with the help of the geneticists we've heard Dr. Mave telling as the continuity clinic team is there to handle the long term care and their assistance goes beyond the medical care we have the team of the attending physician and the families working together with the nutritionists in the preparation of the diet plans and today we're very happy to note that FNRI is truly on board as they develop a low protein flour from local agricultural products by 2020 and the team of Dr. Calibo and engineer Garcia of FNRI are exploring also the development of a low protein milk it's also happy to note that FNRI is not only looking at food products they're also looking at non food products like recipe guides and expanding the food composition table that will detail the amino acids that we need in preparation of the food products well for us maybe the message here of FNRI is that the FNRI FNRI team is here committed to concretize the plans that have been that have been put forth by Dr. Calibo in today's episode we have appreciated that there is actually so much hope because of the partnerships that are happening and this episode also show that the management of MSUD is also a whole of society approach not only among the medical and the specialist and the pediatricians, the physician but bringing in government and all other sectors of the health profession so we would like to thank Dr. Leah Dr. Maved, Dr. Gin and Dr. Anthony for making this a lively discussion and bringing hope to our patients especially those for the parents with MSUD Maraming salamat sa inyong lahat to our virtual audience please send us your comments questions or list of topics that you want us to cover in our succeeding episodes email us at info at newbornscreening.ph or you may tweet us at newbornscreenph or also include the hashtag hashtag enbsph before we end I want to again take this opportunity to present to you a new addition to our tools in learning our ENBS mobile app The ENBS mobile app is a one stop hub for all newborn screening health workers on everything they need to know about newborn screening it also features a rewards program that our health workers can use to earn points and use it to claim shop vouchers with our partners if you have already downloaded the app answer the quiz that we will send to your inbox and earn those points we continue to improve our services as deemed necessary by the emerging challenges through an open dialogue about our experiences in newborn screening it is our hope that through this video series we extend the sharing of knowledge which empower our frontliners improve connectivity with newborn screening coordinators and most importantly provide unparalleled service to every family next week will feature the newborn screening teams from different centers for health development offices this and more here in newborn screening in focus nothing is more precious than seeing a child grow healthy let's realize this through newborn screening newborn screening is a gift of life