This Boy Has Primordial Dwarfism | The Boy Who'll Never Grow Up | Real Families with Foxy Games

Nick Smith is 20 it has an extremely rare genetic condition called primordial dwarfism which means he'll always be the size of a three-year-old else yeah check you now okay okay Nick's condition means he suffers with serious illnesses he said multiple surgeries problems with the kidneys probably of cholesterol problems of blood pressure primordial dwarves have very short life expectancy many don't survive to the age Nikki's now you get too old at 18 Nick had life-saving surgery on three aneurysms bulges in the vessels feeding blood to his brain now the problems back Nick need surgery to remove another aneurysm that could burst and take his life at any moment you feel like you're walking around with the time bomb we don't know when it could rupture scientists are racing to understand how the gene mutation the causes primordial dwarfism works to help prolong the life of Nick and others with the condition one of the things that everybody asks themselves was where are the older kids where are the individuals that are in their 20s and 30s over the next three months we follow Nick his family and his doctors as they battle to save the life of the boy who never grew up Jefferson is a small city in the southern American state of Georgia this ordinary place is home to an extraordinary young man Nick Smith is one of only a few hundred people worldwide born with a genetic condition called primordial dwarfism measuring less than 3 feet in height and weighing under two stones Nick is the same size as a healthy three-year-old but Nick is almost 21 and is not just physically small yes sir sighs nick has learning difficulties which mean he'll forever see the world as a child spongebob mad Nick is the apple of his mom's eye nick is such a happy person he's priceless he's precious oh he's great oh it keeps everything still like you know like I had a child that never really grew up you know I look at your picture slaw you love picture gotcha Shelly realized early in her pregnancy that Nick was going to be small that's you in an incubator I'd had a an 8-pound baby before and I knew what it felt like to have a child growing and kicking and with Nicholas I never really felt like I was really getting big yeah I had to put you in a little baby doll carriage cuz you're too small and they pulled you out to do your feedings I'm told you haven't had to clean out your your bed totally couldn't hold you there yeah I mean they had to clean your diaper when he was born he was 2 pounds 4 ounces 12 inches long he had personality he was alert and I knew when I saw him that we were gonna be alright yeah yeah I finally get to hold you yo Pete I was happy I am happy yeah they're gonna eat their eggs come on they finish up when we gotta go whoa when he was born Nick was a medical mystery because there was no tests to confirm but my son had we probably didn't find out really what Nicholas had until he was about 10 years old happy birthday sweetie Nick was finally diagnosed with microcephalic osteo dysplastic primordial dwarfism or MO PD - the essential characteristics of somebody with mo PD - is that they were born small they stay small and that they have small head circumference of small heads in general their proportions that is the trunk size relative to their limbs it is pretty proportionate okay and when they're fully mature their length is equivalent to about a four-year-old in their head circumference - about a five to six month old Nick's tiny head means his vocal folds and larynx are also miniature creating a high-pitched voice hola brother Travis and younger brother Levi grew up watching Nick struggle every day the fact that Nick's middle son and he didn't turn out like we did I was after him and I came out to be normal that's always the question I'm I'm on it could happen Zach that could have been me just like him you can't get me yeah nope I always joke around and say me and my brother stole the tall jeans before he could get himself it's definitely a little ironic just cuz the whole family's you know very tall and oh I don't know how that happens honestly nice hit go get it go get it to me by avoiding the genetic fault Nick's brothers have sidestep a long list of medical problems that he's had to endure hi Nick alright Nicky ready let's see how tall you are nick has had eight major operations ranging from correction of curvature of the spine to hip surgery new issues can arise without warning so he's constantly under medical supervision yeah check you now okay okay stand up straight for me bend over touch toes all right put clothes on okay he's going I have no Nick since he came home from the hospital after he was born and it has had a lot of significant problems over the years his problems you know stem probably more from his primary orphism than anything but he's you know he's had multiple surgeries comes with kidneys problems of blood pressure he said major spine surgery his most significant problems now are probably his aneurysms Nick's last operation was the hardest so far two years ago he had life-saving surgery on three brain aneurysms an aneurysm is a bulge in the wall of an artery here feeding blood to the brain if the aneurysm bursts blood leaks into the brain which can cause a stroke and be fatal as he can exercise these days so he was doing the ketone brain right now doctors have discovered that Nick has another brain aneurysm and needs major surgery or he could die in a fortnight Nick and Shelly will travel to California for an operation they hope will save his life [Music] well you have to go out there and see some special people I'm gonna talk to us when we get out there about doing some things for us like honey but I'm gonna be there with you no matter what okay yeah don't need me I won't leave you I'm there to take care of you you feel like you're kind of walking around with a time bomb we don't know when it could rupture and in some circumstances recovery or the outcome is a lot worse so if a ruptured aneurysm can mean that's it are they like wait you could do your exercises in ten days time Nick Smith will be travelling from his hometown in Georgia for brain surgery in California in the meantime is at the high school he's been attending for the last four years nick has no trouble getting along when he came to me his parents were very worried about him coming to the high school and being around people that were so much taller and so much bigger and how are the kids gonna treat him that is not a problem here hey well this is Miss Jennifer she's my new helper up here you like all the girls I have up here don't you don't winking at her yeah you're just kidding yeah you work out Nick does not see any difference between himself and anyone else there are times that he'll say I'm as tall as you are and we say yeah you're tall it's gonna be right here he truly doesn't see that he's any different than anybody else everybody wants to see you you're popular while Nick's at mainstream school most of his classes are designed for students with special needs Wow all right dude I would say Nick's learning group is around six or seven years old the activities that we typically do were things that you would see in a kindergarten age classroom so we've got seven green and seven red so what does that make this equal good job we work on daily stuff like colors letters numbers that kind of thing what's the first letter all right No what's this letter oh yeah oh and Nick's been with me for four years and we're still working on those same type of things know what letter comes next no see he doesn't really realize that he doesn't get them right but he likes to try and he's progressed a lot over the years but his memory just isn't the same as what a normal person's would be we have a hard time seen in them 3d glasses Nick is the only primordial dwarf in his home state of Georgia but far from feeling isolated the web has allowed parents like Shelley to find families with children just like Nick all over the world gonna see us Jackie crits ik and her daughter Hana live a thousand miles north in Minnesota the network has really become an important part of our lives you know I was just starving to find another mom that had a child like mine did she have to go through all that yeah is it painful yeah Kristy Jordan and her two primordial children Bree and Brad are in the Midwest state of Illinois for each other's support system where each other's family we treat others friends the network is more than just a support group with little known about their children's condition the parents decided to act themselves we realized that we knew nothing about our diagnosis nor did any of the experts so the physicians that we went to locally didn't know anything and so our outlook was kind of educating each other and to find those families doctors to be able to help us help ourselves for over a decade the families have attended the little peoples conference of America where the children can meet up and the mums track down medical experts it was at the conference in 2004 that Shelley and the other mums met dr. Michael boba and asked for his help one of the things that everybody asks themselves in the room was where are the older kids where are the individuals that are in their 20s and 30s and the answer to that question now seems to be that individuals were dying from strokes from aneurysms that were bursting and they were having large bleeds in their head often fatal to prevent these early deaths in people like Nick dr. Bober and his colleagues first have to understand what causes mo PD to that's something being researched on both sides of the Atlantic in the last five years we've learned that the cause of Mo PD - is due to mutations in the Perry central gene so Perry Central is really important to cell division now cells are building blocks that make us who we are so for instance in MO PD - we think most of these genes are acting to slow down cell division and because you've got less building blocks you end up with a smaller person having established why people like nikka so small the next problem is how to help them one of the important things about once we've identified a gene is to try and understand what health problems are associated with it and this is an area which I worked really closely with dr. Bober on is trying to link up the gene changes we find with the sorts of problems that we get so for instance an MFP d2 we know they get brain aneurysms now they get brain aneurysms at a very high frequency and that means to us but there must be something about the Perry central gene which is important for blood vessel formation and how blood vessels forming this discovery has led doctors to look at other vascular issues that could affect children with mo PD - the type of vascular disease that we've recognized to date have really focused on the blood vessels in and around the brain but as time has gone on we've recognized that the vessels that feed the kidneys the vessels that feed the heart can also have involvement and try some of those noodles these circulatory problems mean that young people like Nick suffer with illnesses we'd associate with old age somebody getting tired let's go get an alternate your ego bed I'll pipe in the bed so I can get you two medicines me your medicine for your high blood pressure and for your kidneys fine kidneys are fine yeah it just seemed like when he turn 18 it was the high blood pressure the kidney failure the aneurysms now it's the cholesterol and then we're facing another aneurysm soap it's almost like an 18 year old it's really going through an 80 year old body okay okay yeah I fall something wrong there didn't you fairy I think it's just time to rest not too hot oh yeah you like that you like that didn't you cuddled up good yeah totally my feet getting cold up there in that night the mood bugs bite four days before the big operation let's go do it [Music] Nick needs his vital signs measured and blood tested to check he's healthy enough to undergo surgery with his aneurysm surgery imminent Nick's previous operation is preying on Shelly's mind never do you expect to see your child come out with surgery horrible scars breathing like they couldn't catch their breath and it was heart-wrenching like I couldn't do anything for him I couldn't comfort him I couldn't hold him all right that's it oh you did it oh yeah what's back is watch yes what'd I say [Laughter] it's Nick's last day of school and his final lesson is with regular students I'm gonna come around and ask each student which one they want to plant do they want to plant forget-me-nots or parsley okay Nick which one do you want to plant forget-me-nots or parsley it's putting Nick with the regular education students it gives them a heart a lot of times teenagers are a little self-centered and don't think about things outside of themselves are those big seeds or little seeds thank you don't drop them okay and your table and so when Nick comes around it makes them stop and think and even be grateful I've heard them say that they're thankful for what they have when they start talking to Nick especially learning about all the health troubles that he's gone through Nik may not be fully aware of the risks he now faces but everyone else is we're all very worried we try not to think about it and talk about it too much because we don't want to get upset all the time especially in front of him or the kids but the reality is is that we don't know what's going to happen from the surgery or after the surgery it's just devastating to us to think about anything other than him coming back to us the same only in the exact same way [Music] today says the last day of school will be leaving the California on Saturday so we're gonna let him stay from school tomorrow he has I think some sense so that there's something going on he's had a lot of tests done lately so he he he wonders why the needles and why the poking and testing and he hears this talking about it yeah I getting a little bit nervous this trip is huge it's major medical procedure to help save his life and unfortunately we have to go through it [Applause] [Music] you know so you have a good day yeah did you tell everybody you're gonna go but I didn't see him when you come back from your trip yeah yeah yeah let's do samba simba Matata don't worry you look cute both the brothers are taking time off work to spend time with Nick before he leaves the surgery in California and definitely worries me but besides that I always try to look at it in a positive light I never really try to focus in on what could happen or that you know if it doesn't doesn't go to surgery something worse could happen so that's why I try to look at it Nick how are you feeling about going to California you know I think it's better than that visit knows what's going on just because in his own mind he's having fun and here's a Hathaway so much and be like under pressure and this lives a happy child life the night before their Li for California a video call remind Shellie that they're living on borrowed time that breaks my heart Jackie and Hannah have some devastating news about the unexpected death of one of Nick's younger primordial friends all of us in the back of our minds are because of what we're hearing our risk factors and knowing what our kids already have and then for you to be going through this with Nick now that has got to be just so heavy you know I count what's about a heaven he says in heaven hold yeah and he says no pain I think he's just gonna go to sleep and then he'll wake up and you know yeah hey you have to stay like a possum oh no we're not maybe we've already talked about this he's saying he's gonna play dead like a possum and I said we don't want to use that example [Applause] okay get your jammies in there let's go yeah yeah I've been staying so strong with the surgery and just staying positive and when you lose someone you just just really shows you this we just never know you know we know they're getting older the health issues are getting a little more severe and I guess with this surgery I know you know Eric it's coming up and it just it makes it that much harder because you know it's it's reality and I could you don't be facing the same situation feeling it today 20-year old Nick Smith and his mum Shelly are making a two-and-a-half thousand mile journey to California for an operation they hope will save his life looks like how about you find me which shoes you want to wear my new ones are most comfortable for your feet we do that you know I get a little nervous sometimes fine he doesn't seem to bother him at all I like him but you don't like to sleep on the plane do you he'll never any operation on the delicate body of a primordial is dangerous but brain surgery raises the stakes even higher it's hard to say but I think it's harder on the family than it is sometimes the patient [Music] having to say goodbye is tough for 18 year old now there's no turning back the ordeal ahead suddenly hips help hurts the painful the needles she get the needle you're okay in here yeah they're little stop though nobody nobody likes that Nick yeah I know t-shirts trailing boy aren't you strong [Music] but just books to read and books would be negative you know liking like what would what you going for yeah I'm scared yeah we'll talk about it no you want me to change the subject go something else yeah what you want to talk about okay let's talk about your girlfriend Nick surgery in California will be on an aneurysm a dangerous bulge in an artery feeding blood to his brain the size of the aneurysm means it could rupture causing a fatal brain bleed at any time you see here on this vessel that is the left carotid artery we have this ballooning of the vessel wall that constitutes a brain aneurysm a weak spot on the vessel that makes this person more prone for brain bleeding surgeons will perform an endovascular coiling procedure where tiny loops of platinum are packed inside the aneurysm so the blood clots closing off the Bulge that means going in through Nick's groin through the groin would have the natural highways that are the blood vessels navigating under x-ray guidance all the way to the inside of the Animus and then we can go inside the aneurysm and feel divided with this little very soft platinum wires as you can see here very soft and create a bird's nest that will induce clogging of the aneurysm and prevent further bleeding you want your back scratch again yeah I just want to let you know there's nothing to worry about you've been such a tough cookie all the way haven't you Nick we'll see your tummy remember this one this is the first surgery they did when you're a baby just to try to put a tube in your stomach to help you eat Wow we were in a water park one day and the little boy came up to him saw the little tube said to his mom look mom he's inflatable so that was the cutest thing so listen tomorrow remember last time they had to cut in your skull Yelp will remember and they cut you you had that scar on your head yeah all right they're not gonna do that to you tomorrow okay they're not gonna do that how they're gonna do is put you to sleep and you won't even know but they're gonna fix a little spot in your head while you're sleeping so when you wake up it's gonna be all over with yeah and you can watch it all the TV you want thank you and you can eat Krabby Patties minnow macaroni all eggs as soon as you're up feeling better you get out of the hospital and we can go back home [Music] [Music] NIC's operation will be at one of the world's leading pediatric hospitals the Lucile Packard at Stanford University only a handful of surgeons have ever performed this type of procedure on someone with Nick's condition even for an experienced pediatric surgeon operating on a primordial dwarf adds another level of complexity to operating a primordial is certainly different than to operating a child of same size because the primordial have different characteristics on the blood vessels on the anatomy of the brain in changes in many of the other organs and systems that a child of the same size would not have that creates a much more fragile system that you have to be prepared to deal with so a brain aneurysm has the potential of bleeding at any second there is a real possibility Nick's aneurysm could rupture during surgery if that happens there's a 50% chance he won't make it knowing the odds leaves Shelley struggling with a decision to go ahead with Nick's operation the reality is that we don't know it's gonna happen with our children we don't know how long we have them but it's almost like you know would you walk around and wonder what have him with a time bomb or correct him knowing that coming down it's process he might not have the quality of life that he once had nor could he make it with Nick's aneurysm surgery under way his future is in the surgeons hands [Music] [Music] okay this is Nick we're breaking out of the work today come on let's get down Nik for our operation was a success the coil was inserted without his aneurysm rupturing [Music] did you make it through surgery it's all over it's amazing no more surgeries no more pokes yeah no more needles I'm following you boy and yet either way I fall this is your walk after only three days in hospital Nick's allowed home to his family after the surgery Nick you know actually jump right back to normal just like Nick always does and just lucky for us but it's great [Music] it's now a month after neck surgery Shelly and Nick are flying to Minnesota after everything that we've been through my goal is just to enjoy life and mean Nicholas we're gonna go have a lot of fun and just embrace every moment that we can together right what makes him happy is he's a funny person he understands fun and laughing and cutting up and that's the healthy part of life you know for us to be able to spend some time and share a trip together and put all that stuff behind you for a week that's why we're looking forward to they're friends from the network are coming from around the country for a surprise celebration for Nick it's the first time the group has met outside the annual little people's conference it's -5 degrees in Minnesota so Nick and the gang are starting their day out inside at the Sea Life Aquarium Nick's primordial friends a 21 year old Brad Brett's older sister brie is 23 17 year old Hannah and Trinity who's 10 for us to get together as primordial families it's one of the best experiences because the kids they are just like best friends they're almost like sisters and brothers my team regular large tough time cakes nicely seen other little people Matt things creepy cuz you're not tall like me well [Music] just make like this we do a lot things that people think they're too hard for us they think we can't do but we'll find out as a special finale to the day there's a surprise for Nick he's finally going to meet his hair [Music] one of our things is we want them to live their lives to the fullest and have every dream or hope that they can have come true so that they live while they're alive and they live and feel important I would like to do a celebratory toast I want everybody to raise their glass and just say here here for all of the mums it's important that the children celebrate while they can because the harsh reality of their lives is never far away each parent has to make a decision of how open they are to discuss the realism of their illnesses the outcomes the treatments well for brand Brad we've always been very upfront and to me it empowers them to take care of themselves it helps them understand why they go through mundane testing why they take multiple pills why they have to go to the doctor five states away it helps them be that person to pave the way for the next little guy very worried about making a surgery yeah I think he was a praying nervous I would be nervous I had my surgery I was pretty nervous I don't like medical stuff I just don't like anything right surgeries and only thing I don't worry about that person for the children understanding that condition doesn't make accepting it any easier it's really sad because we lost one of our kids thank you for Hanna that is kind of put into a perspective for her they were my age mom and they're not with us anymore and then the tears and that has been the hardest thing and you know I would I we have to say is that you know we don't know how how long you're gonna have none of us do we're all human beings you know we have God has a plan for all of us so we're just gonna live each day like it's our last day but not just you we're all gonna be doing that we each have today we're not promised tomorrow Nick doesn't have the same level of understanding as the rest of the group and for his friends that's a good thing Mick and his friends are doing well for now but the big question remains how can their short lives be prolonged the holiday to Minnesota gives Nick and his friends a chance to hang out with people who are on their level it's probably one not a good idea for him to have the cat but it also performs a vital function as a forum for the mums to discuss medical developments and new ideas the outsider looking in may think this is something that medical experts should know but if we don't know and there's only one child in that state or one child in that country how do we expect medical professionals to know that's one thing I wanted to know without these groups in this organization there would be no research there would be no progress at all this whole field owes everything to those groups if I see one child all I can do is do my best for that one child but if I see 20 children with the same rare condition now I have a much clearer idea about how to take care of that one child and that's really what's happened I'm trying to remember right know I know it's all put on that USB the medical research pursued by these mothers means treatment for their children has gone from reactive to proactive early screening for aneurysms monitoring cholesterol and management of renal failure means these children now live longer the life expectancy for people that have mo PD too is not average it's definitely shorter but it's improving it's much better now because of the screening program for aneurysms the treatment programs that we've put in place we are seeing individuals live longer there are many more people in their 20s in their 30s with mo PD too so I think that age is being pushed up and people live longer and longer every year as this generation is living into their 20s the friends can have ambitions in life beyond childhood I mean 11 3 let's drag Roger I don't play know why I'm doing it till I get out of school yet I love entertainment stuff acting from acting or I'd be in a band I want you to play the drums I'm not having plenty drums yeah you do right yeah you do I'm a good better I am I see see make me that's all I know I'm nice and funny yeah you all right yeah I think he want to be a comedy as you like to tell a large oak back at home in Georgia Nick's about to start a new phase of his life [Music] he's about to turn 21 and that means moving on from the pediatric doctor he's always known so Nick buddy's gonna miss you you know do you understand that mommy's gonna have to find you a new doctor yeah you get too old yeah hitting 21 also means only one more year at school what is it Carl it's the Constitution can't eat people have rights right this world is not built for Nick you know this world is not accessible to him and so we try to put everything into every day just to make him have as many experiences in as many happy moments as we can possibly fit in Nick's lifespan may be limited but for the family Nick still has hopes and dreams for his future that are no different to anyone else's told you doesn't like to lose it likes to be like everybody else wants to be able to ride four-wheelers and do everything we do biggest thing is I want him to feel that he is just like everyone else and he is just like everyone else inside yes you know he has big dreams he thinks he's gonna grow up and be just like his daddy one day I'll get you out of those jackets yeah I know he still lives my thing in a beautiful place it's kind of like not reality but it's in his world he's still gonna achieve and be whoever he wants to be and and that to me is a beautiful thing [Music] you

Channel: Real Families

Views: 556,023

Rating: 4.887434 out of 5

Keywords: real families, The Boy Who'll Never Grow Up, nurture documentary, dwarf, 20-Year-Old Gets Life Changing Surgery, primordial dwarf, What Is It Like Having Primordial Dwarfism

Id: 4UxqVF9Xgl8

Length: 44min 40sec (2680 seconds)

Published: Sat Aug 17 2019