The Smith's

Wednesday, August 29, 2012

About Tubie Friends

About Us Tubie Friends a non-profit group that is administered by two mothers whose children have feeding tubes and want to use their experience to make life easier for the thousands of children relying on feeding tubes for their nutrition. Tubie Friend Surgeons (TFS) are volunteers who care for or love tube feeders and donate their time and resources. Many of us are still in the trenches with you, which means that sometimes our kids have surgeries, procedures, tests, or hospital stays that takes our time away from Tubie Friends applications and processing. Our administrators monitor the e-mail account and websites. Co-Founders Our children's Tubie Friends have been a teaching tool for family, friends, school nurses and caregivers as well as something that has provided comfort for our children. When our sons met for the first time, they instantly bonded and told "war" stories about all the medical interventions they have undergone. It was an amazing thing to see our boys brag about their feeding tubes rather than feel isolated. Unfortunately we live in different states, but when they are apart each has their own Tubie Friend to provide comfort and friendship. Cheryl When we found out Brayden needed a feeding tube, I was blessed with friends who had already mastered the art of tube feeding. They encouraged us to create a Tubie Friend to help teach him what about the feeding tube so the process would not be as scary. He and his monkey "Sam" became instant friends. He immediately gave Sam medications and becoming an expert at connecting the extension prior to surgery. I believe it helped our entire family be more prepared for our new life as a tubie family. Unfortunately my son's Mitochondrial Disease has caused us to spend more and more time in the hospital. Over the years Sam has continued to have many surgeries to change to a GJ tube, add a PICC line and now a Broviac for TPN in addition to his tube feeds. Brayden is now 7 and still holds Sam tight anytime he is scared about a medical procedure. I have a full time paying job in Human Resources and spend a lot of time taking care of my son's medical needs. However Tubie Friends provides a great distraction and helps me feel good that we can take what we are learning with my son's journey and help smooth the way for families that follow in our footsteps. Shannon Tubie Friends is my habit, hobby & labor of love. When I'm not working on Tubie Friends, I teach 6th grade students and invite them to join us in helping others. I also serve on the board for the Seafair Pirate Charity Foundation as Director of Tubie Friends. My son, Caleb, is almost 7 years old and currently receives most of his nutrition through his GJ tube. He was a toddler when he got his feeding tube and we had no connection to any kind of support group. Our family helped where they could, but we had no one who could walk us through the process. The idea to make his first Tubie Friend was more to help his older sister, who wanted to mother him and feed him, than it was to help Caleb. Immediately after making a Tubie Friend for both kids, we were pleased to see that he finally began to accept tube feeding without the tears and battles and his sister was content to just feed her Tubie Friend. Over the years, when he has changed to different types of tubes, we have made new Tubie Friends to familiarize him with what the new tube will look like before it is placed in his body. We have also used it as a teaching tool for school nurses and caregivers as well as sharing it with the kids in his class to satisfy their curiosity. My hope is that no family will go through this without the support of others who know what it is like and without the love of a Tubie Friend.

5 Month Photo Shoot

Guess Who's 5 Months!

RILEY IS!!!! Cant believe she is 5months now! My little worm is growing! Update on her Gtube. We are home now finally after spending 7 days in the hospital. Just took her some time to recover. She isn't gagging anymore or having any reflux issues YAY!!! I say that now and watch next week it comes back. hope not lol. She is also tolerating her feeds quite well :) Theres a website called Tubiefriends.com and you can order a build a bear and they put trachs or Gtubes in whatever kind your kid has and its free all you pay is the shipping! which is only 8bucks but even that if you cant pay for the shipping they will for you. So i got one for the kids because i know they will interested on how there little sis eats now. Then when Riley gets bigger she will be able to play with her Gtube bear too :) I signed up for early intervention, that helps with kids with special needs! They have PT, OT, and Speech come to your house and work on therapy sessions. It will also help with SSI they said. So im excited to start that. Everything with lil missy and going great. I see her pcp next month for a 6motnh check up and shots :( and re due of a sleep study to see if she needs her NP tube anymore. I also think she will be ready for her infant car seat now. No more car bed which I hope!!! Maddie and caleb start school in a few weeks. Cant wait for that :)They both are excited to get back in school mood. :D Oh and im going to be a auntie :) Found out what she is having today but cant say bc she wants to surprise everyone with cupcakes and the middle of them they are going to reveal what it is :D alright her are some pics from her gtube surgery and 5month :)Love you all have a good afternoon!

Sunday, August 19, 2012

Bye Bye NG Tube! Hello Gtube!

Went in for Riley's Gtube surgery Thursday. Everything went well until they took her breathing tube out and gave her some Fentanyl. She was really working herself to breath. So they gave her some Narcan that refursed the narco and she finally woke up and started to act like her self. So they sent her over to the PICU over night just to keep a eye on her. Around midnight she started breathing harder and seemed like she was in alot of pain. They were only giving her tyalnol around the clock because they didnt want to give her morphine and have her start breathing weird. So they gave her a little bit at a time and she did very well with it but still was working herself to breath. They then put her on 8L of oxygen and 100% high flow. Since she was working so hard to breath she started gitting alot of secrections. So then we had to suction every hour seemed like. They then noticed that she looked like she was coming down with a cold. That seemed to make since, since i was coming down with one as well. So they took a siliva test and we should know Monday what it is so we can start treatment. So today (Sunday) She looks a like a whole different baby! She is finally herself. more awake, and aware of things, not breathing so hard anymore. She is also down to 3L of O2 and no more high flow. They started her feeds with 15ML every hour and going to increase it every hour now. So tomorrow we should be out of the picu and into a regular room :D Photos To come!!! XOXO Love you all

Saturday, August 4, 2012

Update!

So Miss Riley is now at 12lbs and 2oz and 22in long. She is still not taking anything by mouth. Her reflux has been getting a little better though. She is now able to put fingers and pacie in her mouth with out gagging all the time. I went to her feeding clinic for a update and they want me to dip her pacie in her formula three times a day to get her started on oral feeds. She will be getting her Gtube placed August 16th 2012. This will be her first surgery. They are a little concerned about her breathing but they said everything should be just fine. We are expected to be in the hospital for a couple of days. Had a follow up apt with her ENT doctor and they want to redo another sleep study to see how she does with out her NP tube placed. Now a days Ive been leaving it out all day except when she is in the car or sleeping. During the day when she is asleep i leave it out, and her stats have been staying at 98-100. Ive noticed when it is in though she has been gagging ALOT, then she has a hard time throwing up then her stats lower. Im curious to see what the sleep study says. Ortho- Wants to see her again in a few months. Give her time to grow, ect. Nothing new there. I do though want to get a second opinion on her spine and neck though. Because something tells me we need too.... Anyway besides that we are all doing well! Love you all! Xoxo

About Help to promote awareness of PIERRE ROBIN SEQUENCE!

To make people more aware of this rare sequence of event's Pierre Robin is a sequence of event's resulting in life threatening breathing and feeding problem's. The sequence consists of a large u-shaped cleft pallet, a small jaw which means that the tongue fall's back blocking the airway. Baby's with PRS always require surgery to close their pallet and sometimes to bring jaw forward. Nearly all have feeding difficulties and have to be tube fed or with special squeezy bottle's as they have no suction! Often the child requires help with speech as they get older, due to having a cleft pallet! Midwives sometimes miss PRS straight after birth leaving baby unable to breath and having to be ventilated. We need to make people more aware as it is rare but does happen! PLEASE JOIN THE CAUSE. 1. 1 in 30'000 baby's are born with some type of PRS 2. Alway's needing surgery to close their pallet

Thursday, July 26, 2012

Love This!

I Have a Special Needs Child Welcome to Holland by Emily Perl Kingsley By: An EP User Written on March 13th, 2009 When you are going to have a baby, it's like planning a fabulous vacation to Italy. You buy a bunch of guide books and make all your wonderful plans: the Coliseum, Michelangelo's David, the gondolas in Venice. You may even learn some handy phrases in Italian. It's all very exciting. After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland." "Holland ?!?" you say. "What do you mean Holland? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy" But there has been a change in the flight plan. They've landed in Holland and there you must stay. The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place. So you go out and buy new guide books. And you learn a whole new language. And you meet a whole group of people you would never have met. It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there awhile and you catch your breath, you look around and begin to notice Holland has windmills- and Holland has tulips. Holland even has Rembrandts. But everyone one you know is busy coming and going from Italy...and they are all bragging about what a wonderful time they had there. And for the rest of your life, you will say, "Yes, that's where I was supposed to go. That's what I had planned." And the pain of that will never, ever go away, because the loss of that dream is a very significant loss. But if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, and very lovely things about Holland.

Monday, July 23, 2012

Ohhh Riley :D

Came home from my first night at work. Prepped Rileys bottle as i went into her room to set up the feeding tube this is what i saw. She flipped her self up side down in her bed lol haha she scared the crap outta me lol

About Pierre Robin Sequence

Pierre Robin Sequence: What is Pierre Robin Sequence? Pierre Robin Sequence (PRS) is the name given to a condition, which includes a small jaw and a cleft palate. This condition is named for a French physician who in 1923 published an article describing a child with this specific combination of medical problems. As it turns out, Dr. Robin was not the first to describe PRS; nevertheless, his name remains linked to this condition. It is often stated that there are three separate issues with PRS: a cleft palate, small jaw and glossoptosis (a term that was coined by Dr. Robin, meaning lowering or drooping of the tongue). As it turns out, glossoptosis is not an actual problem with the tongue itself, instead is the result of a normal-sized tongue sitting in a smaller than normal lower jaw. When the lower jaw (also called the mandible) is smaller, the tongue ends up being positioned closer to the back of the throat, causing the “glossoptosis.” The cleft palate in PRS is usually different from a “typical” cleft palate in that it is more U-shaped, than V-shaped in appearance. Pierre Robin sequence has been reported to occur in about one out of every 8,500 births, but some believe that it can happen even more commonly. It can either occur as by itself as an isolated Pierre Robin Sequence, or as part of a syndrome that may have a different name. What causes PRS? For many years, doctors thought that PRS might be the result of the way the baby was positioned inside the womb. If a baby were to be positioned in the womb with the neck flexed forward, then the chin would be against the upper chest, preventing it from growing. During this time the tongue would be enlarging normally, and this would cause it to push up against the roof of the mouth, resulting in a cleft palate. While this seemed to make sense, as further progress has been made in understanding human genes, we have come to realize that this simplistic theory is probably not correct. Today, geneticists believe that isolated Pierre Robin Sequence may be partly caused by changes in genes. Most recently, it has been suggested that potential problems with two genes called SOX9 and KCNJ2 mRNA might be possibly be related to PRS. However, changes in genes named: GAD67, PVRL1, COL11A1 and COL11A2,have also been noted in children with PRS. It is not clear if the changes seen in these genes cause Pierre Robin, make it more likely to happen, or just happen to occurring at the same time that something else is causing PRS. However, up to 25% of children with PRS will have a family member with a cleft palate, which is a more common association than might be expected if there was no genetic role for this problem. This condition was referred to as Pierre Robin Syndrome until a number of years ago when many physicians began referring to this condition as Pierre Robin Sequence. The reason for this change is that the term syndrome is used when a number of problems are caused by a single issue, such as a gene mutation, whereas as sequence is a term applied when there are multiple problems that have occurred in which one problem leads to another, and then another, etc. Initially, doctors thought that the cause for PRS was that the baby’s head was flexed in the womb, leading to the small jaw and cleft palate, so it made sense to change the name from syndrome to sequence. Given the rapid developments in our understanding of genetics, someday physicians may well end up calling this condition Pierre Robin Syndrome, once again. What problems do parents need to consider with Pierre Robin Sequence? The most common associated problems with isolated PRS include: Difficulty breathing – mostly caused by the tongue falling backwards and blocking the throat. Difficulty eating – mostly related to the airway problems, but can also be the result of a delay in the neuromuscular development of coordinated swallowing. Decreased hearing – with a fluid build up behind the eardrums, or otitis media (which is easily treated, but left alone may lead to hearing loss). Teeth – rarely, there may be missing teeth. The first step in the treatment of a child with suspected PRS is to determine if the child actually does have PRS and if so, is it isolated PRS or is it associated with another syndrome. PRS features can occur as part of many different conditions and syndromes, and it is very important that a genetic evaluation and testing be performed. The most common syndrome associated with PRS is Stickler syndrome. Stickler syndrome is sometimes associated with joint problems, arthritis, and with multiple eye issues (including myopia, retinal degeneration and detachment, early cataract formation, and a higher incidence of glaucoma) and this condition needs to be accurately diagnosed so that affected individuals can be closely followed by an ophthalmologist. Less commonly associated with PRS is Velocardiofacial syndrome (also known as: VCFS, 22q11 deletion, or by the older term, Shprintzen syndrome) and Fetal Alcohol syndrome. A genetics consultation is an important part of the initial treatment of a child born with PRS. What it the treatment for Pierre Robin Sequence? Difficulty breathing. The most urgent concern for any infant born with Pierre Robin Sequence is to make sure that the baby is able to breathe and get enough oxygen. Most children are initially monitored in a Neonatal Intensive Care Unit, where their oxygen levels can be continuously monitored. Some children require intubation (placing a breathing tube through the mouth into the windpipe) right at birth, and some may need intubation after a few days of trying to breathe on their own and then just tiring out. For those children who have problems breathing on their own, what is the best treatment? Different treatment options include: re-positioning, nasopharyngeal airways, lip-tongue adhesions, tracheostomies and jaw distraction. The following is a discussion of the various possible treatments for breathing problems. Repositioning. In my opinion, too many centers needlessly rush into surgery when most babies could have been managed adequately without operating, even those who have required intubation (putting a breathing tube into the windpipe). Often, surgeons will only recommend to parents cutting the bones of their baby’s lower jaw, and placing distraction devices to lengthen the jawbones. Many parents are told that this is the single best option to prevent a tracheostomy. I would not want this treatment for my own child, and I believe that it is critically important that every effort be made to first try to treat every baby with smaller treatments, whenever possible. Large studies have shown that the majority of children with isolated Pierre Robin Sequence can be treated without any surgery, and exceedingly few ever will need tracheostomies. The first thing to try is to simply position the baby correctly in the “prone position,” which means on the baby’s stomach. This is the opposite of what the American Academy of Pediatrics recommends with their “Back To Sleep” campaign, which is aimed at reducing the incidence of SIDS. Nevertheless, this it is precisely what is necessary for a child born with PRS. It is important that the baby born with a small lower jaw be positioned so that gravity can pull the tongue forward instead of backwards, which is what happens when an infant lies on his or her back. This treatment requires some specialized feeding techniques, and if this positioning works well, the baby will need to go home on a monitor. Nasopharyngeal airway. If simple re-positioning does not work, then the next step is to place a nasopharyngeal airway. This is a tube that goes into the nose, and past the tongue, in order to create an unobstructed breathing pathway. It is very important that this tube be of the correct size, because a tube that is too big may eventually cause scarring inside the nose that can permanently narrow the nasal passage (this is referred to as “stenosis”). Some doctors recommend alternating the tube from side to side, every week or so. This tube has been left in place for up to 6 months, according to some studies. Typically, these tubes are only needed for a month or two because the lower jaw grows fairly quickly in the first year of life. Lip tongue adhesion. The next step to consider, if placing a nasal tube is not a workable option, is a small operation called a lip-tongue adhesion. This procedure temporarily attaches the front tip of the tongue to the inside of the lower lip, in order to keep the tongue from falling back and blocking the airway. It is a completely reversible operation, and typically, the lip and tongue are detached at the same time that the cleft palate is repaired at 9-months of age. This operation does not prevent the baby from taking a bottle (however, there are some general feeding issues associated with PRS that are discussed below). Prior to performing this quick operation, I recommend that an ENT perform an examination in the operating room to make sure that the baby does not have tracheomalacia (a condition in which the cartilage that normally holds the airway open is immature, and is softer than it should be, so that the airway collapses easily). If an infant does have tracheomalacia, the best option may be to proceed with a tracheostomy, because all other treatments will not work well. Fortunately, most infants with PRS do not have tracheomalacia. There are no long-term complications from performing a lip-tongue adhesion. The most common complication reported after this operation is that the repair falls apart. This can be avoided by placing extra supportive stitches at the time of the repair. When should jaw distraction be considered? I do not believe that there is any indication for performing distraction for babies born with PRS. There have been studies published where surgeons have done a number of cases of jaw distraction in infants with PRS, and have concluded that this is the best operation for PRS because it prevents tracheostomies. However, there are a number of things that these studies do not tell us. To begin with, many experienced surgeons argue that overwhelming majority of patients in these studies may have done just fine without this operation (based on other studies showing that treatments like nasal airways and lip-tongue adhesions have excellent results). Perhaps the most significant problem with jaw distraction in infants with PRS, is the significant complication rate with this procedure. Complications include, but are not limited to: needing to replace the device in the operating room once it has been put on because the device fails or pins loosen, permanent scars on the child’s face, possible nerve damage, and injury to permanent teeth. This last complication is pretty much inevitable when any screw or pin is put into an infants jaw. A baby’s small jaw is basically filled up with the two rows of teeth. Anything attached to the jaw can injure these tooth buds, and parents will not notice any problems for 10 to 12 years after the operation when the permanent teeth are supposed to have come in. The routine use of jaw distraction in PRS is not yet 10 years old, so infants treated with distraction are not yet old enough to show these problems. When permanent teeth do not erupt, then dental implants (metal screws are surgically placed into the bone of the jaw, on top of which an artificial tooth is attached) may be required in teenage years, and there is no guarantee that these will last a lifetime of 80 years, or longer. Why accept these problems, when there are so many other good options? Although I recognize that many craniofacial centers recommend jaw distraction in babies with PRS, when I look at the pros and cons, it is simply not an operation that I would select for my own child. Feeding issues. Many children with PRS will have disorganized swallowing. If there are any signs for this (noisy breathing, choking, sputtering) I will recommend a swallowing study for my patients. This study is to make sure that the baby is not having any “silent aspiration” (when small amounts of formula quietly go down into the lungs, instead of the stomach) while feeding. Aspiration can lead to respiratory infections, or possibly even lung injury. The most common reason for aspiration is that the nervous system is slow in developing, and as a result, swallowing is not well coordinated. I recommend that if a swallowing study shows signs of aspiration, then the breast milk or formula may need to be thickened. If thickening does not prevent aspiration, then a small feeding tube is passed through the nose into the stomach and all food is given though the tube until the swallowing ability matures, which can take weeks to months, depending on the child. This tube does not seem to bother children, and it is effective at preventing formula, or breast milk, from entering the lungs. For those babies who require a nasal feeding tube, I will retest a baby in 12 weeks to see if the swallowing has matured to the point that the feeding tube can be removed. Rarely, children with significant problems are given a gastrostomy tube, which is a tube that goes through the skin of the abdomen directly into the stomach. Hearing problems. For any child with a cleft palate, there is an increased chance that tubes will need to be placed into the ears. This is because the inner ear does not drain in a normal fashion when there is a cleft. Fluid can build up behind the eardrum that reduces hearing, and that can lead to an increased risk of ear infections. If this fluid (called serous otitis) stays behind the eardrum for an extended period of time, hearing will be decreased, which can delay the development of normal speech. I recommend that babies with cleft palates have their hearing tested more frequently, and any time a child needs to go to the operating room, for any reason, that he or she be evaluated the day before surgery to see if tubes need to go in the ears at the same time. Appearance issues. Even though the lower jaw is smaller in PRS, the good news is that it grows at a fairly normal rate. I recommend waiting until growth is nearly complete (about 15-years old for girls, and 16-years old for boys) before considering any surgery. If anything is needed, most teenagers will only require placement of a chin implant in order to normalize appearance, and this is only recommend this if the lower jaw is small enough to bother the child; otherwise, nothing needs to be done. Less commonly, if the teeth are not aligned properly because of significantly reduced growth of the lower jaw, the lower jaw can be cut and moved forward (all done from inside the mouth, so that there are no external scars).

Our Angle With Out Wings XoXo