Sunday, July 1, 2012
Connor's Pierre Robin story ....in pictures
I feel a responsibility to put informatin out there on what Connor/we have been through since his diagnosis. For one, I hope to help someone find information when you're frantically searching for information after a new diagnosis and secondly, I hope to find other people dealing with our same issues. ....so for my friends and family, this may be a boring post. also.....it's a ton of pics.
This is me and Connor, March 30th, 2012. He weighted 7lbs, 14 ozs.
Our newborn baby. Swollen as all newborn's sort of look but something off with his chin. I got a lot of feelings at this time where I felt people were judging me for thinking I was calling my baby imperfect....what I know now is to trust your gut.
Shortly after he was born, I fed him a bottle and swore he was turning blue around his lips and nose. He'd of course recover by the time a nurse came in but eventually, his pediatrician came and said he believed he should spend his first night in the NICU. We were informed about 4 hours after his birth that he had a cleft palate. We were devestated because at that point we were having a real difficult tmie distiguishing what happened with Ireland (CDH, congenital diphragmatic hernia and her passing away 11 days later...story at www.lovingourirelandrose.blogspot.com) and what was going on with Connor. It is safe to say that I was hysterical the first night after Connor was born. I knew something else was going on.
Connor had a few times when he'd choke, turn blue, and basically have a tough time breathing.
He was not eating well and was placed on an NG tube his second or third day in the NICU. Because I had a c-section and an awesome OBGYN (Dr. Willam Hughes. Rocky Mountain in Davis Hospital), I ended up staying in the hospital for about 5 days so I could be with Connor for atleast part of his NICU stay. ....what we thought would be a couple of nights in the NICU for Connor, ended up being 2 entire weeks and there was alot of stress, trying to do right by a teen, a 14 month old, my husband's job, etc. Not easy at all.
In order for Connor to get home, he had to have no destats for 72 hours straight. (destat meaning that he had a 'spell' of not breathing, chocking, or needing oxygen). He got off his NG tube after 5 or 6 days and then started taking a bottle from the Haberman nipple. The Haberman had a special valve that allowed for you to help squeeze formula into his mouth since he really didn't have suction without a palate in his upper mouth. ...we were instructed to squeeze hard because he was truly not sucking but he would swallow it. He began to grow about an ounce a day with this (after having lost a little over a pound...which is considered normal).
We were still in a state of not knowing why Connor was having his 'spells' and little by little the doctors started saying that they thought his chin was more of a problem then his cleft palate. We had no answers and were extremely frustrated. We had noticed a smaller than normal tongue as well and after a week, one of his doctor's finally said the words, Pierre Robin. We were given the number of a craniofacial plastic surgeon (Dr. Faizi Siddiqi) at Primary Children's and the rules for him having 'spells' and not leaving for 72 hours were relaxed somewhat because he wasn't needing oxygen but definitely having little choking issues. At some point during his NICU stay it was decided not to leave him on his back, to always have him elevated and after 2 weeks he was sent home with an apnea monitor that would go off if his heart stopped or his lungs went too fast or too slow. It was a battle to get the apnea monitor but alot of that had to do with us having lost a daughter only 2 and a half years before and us believing we would never sleep worrying about Connor. Fortunately, his pediatrician agreed and insurance covered the monitor.
This is Connor at home in his bassinett. This shows his profile pretty well.
14 month old brother, Sawyer....styling during his bath.
yep....cute little Mohawk
Connor's first visit to Primary Children's Hospital....a great children's hospital that serves no less than 7 states. Our first visit with Dr. Siddiqi, craniofacial plastic surgeon, was fairly quick. He gave us basics that his palate would be fixed at around a year old, that he would schedule a sleep study, we were to schedule a "team" visit with the craniofacial team, we'd schedule a visit with an ENT (ear, nose and throat doc), and he'd see us again soon. .....also, can not stress enough what a wonderful doctor this is!!!! He makes his own house calls and during his hospital stay, was there daily to check on him.
Connor had his sleep study in the middle of April...he wasn't yet one month old.
He was connected from the top of his head to the bottom of his feet...literally. It was beyond uncomfortable looking and I didn't know how he'd ever sleep.
This was my bed. Yeah...didn't sleep there. I laid down next to Connor and pretty much was up hourly with him and also had to feed him.
This was the morning after the sleep study when they unhooked him from everything. He actually did amazing and got SOME sleep. I felt certain that they wouldn't figure out a thing because he was up so much.
Sawyer having a little nutella. (Tristan avoids my phone picture taking...so don't expect too many!)
Connor's cleft palate.
A break for the babies to go to the park....Sawyer did alot of running with the ducks this day!
It took some time for Sawyer to learn to just love on Connor but honestly, even to this day, Sawyer kisses Connor more than anything. There are the occassional moments when Sawyer wants total attention and he may feel the need to hit...but this has been rare...thank God!
A visit with Dr. Siddiqi soon after his sleep study. (this is daddy, Mikey) ...on this visit we discovered that Connor showed severe obstructive sleep apnea so the doctor wanted to go ahead with a jaw distraction surgery that would push Connor's chin forward. The chin back meant his airway was severely obstructed. This day Connor got a quick cat scan and we were given a date of May 9th for Connor's surgery. We were told to expect 3 or 4 days for a hospital stay and that we'd turn the screws from his distractors 4 days after his surgery. We'd end up turning the screws twice a day for just over a week.
This was May 9th, right before his surgery This a pretty good picture of how far back his jaw and chin are at this point in time. This was a scary Wednesday morning. Luckily, Connor was out so handing him to the doctor wasn't a horrible teary production though Mikey and I had a tough time. ....he was slotted to be in surgery for 4 and a half hours. He had an anasthesiologist, his ENT doctor (whom we had not met because his surgery was so soon), and of course, Dr. Siddiqi and some nurses and residents.
This was one of our first views of Connor after surgery.
He was on a ventilator to help with breathing after anasthesia. This is pretty common and remember he's just over a month old here. If you look closely, you can see the distractors behind his ears and the cut that was made on each side of his chin. They basically scrape past tissue and muscle to expose the bone, cut the bone, place the screws and soon after, we will begin turning the distractors behind his ears to push his jaw forward.
This was beyond a horrible experience for us. Once again we had a baby hooked up to a ventilator and machines. We had a horrible time again of seperating Connor's experience with what we had been through with Ireland. Ireland and Connor just happened to look very much alike swollen and attached to machines. ....Connor was having a tough time getting off of the ventilator. He was on morphine, other pain meds and what we ended up calling the evil Precedex. The precedex did something to slow his diaphragm down and Connor was getting in a state of just wanting the machines to do all of the work for him.
This is a close up of the distractor. We started turning it 3 times, twice a day. After 8 days of doing this we were done. This pic also shows alot of his swelling.
Connor as on a ventilator, too many meds, and scaring us horribly. We discovered the precedex was the medicine that really through Connor into a lazy state but because he was having difficult time with pain and all of a sudden his lungs were sounding horrible, they kept him on this med.
As Connor slowly was waking up from his drug induced sleep, he barely had a voice or cry. I was staying every night even though during his PICU (pediatric intensive care unit) stay he had 24 hour nurse care where his nurse literally stayed by his side. If a nurse would take a break she/he ould get a replacement before leaving.
Connor ended up being in the NICU for a week before his intubaton tube (conected to he ventilator and between his lung) was pulled. ....what actually happened was, I had finally decided to come home and within a couple of hours, the doctors hd decided to do an x-ray to check tube placement and they noticed it wasn't where it should have been so they called us and long story short, the tube was pulled out before we even made it back to the hospital. This involved us getting a sitter, Mikey having to leave work (yet again for a mini crisis), and us making the phone calls to family and close friends. I repacked to stay the night because we'd be watching how he did off the tube.
He did alright though his sounds were still very soft. here you can see how much his chin has been moved forward and he looks fairly different. He also looks just tired. Soon after being extubated (removing the tube from the ventilator) we moved to the ISCU (infant surgical care unit). Once there, he would be off 24 hour nursing care. And though his nurses were wonderful, we felt it best I stay because the nurses were usually tending 3 or 4 other babies and you could not hear if Connor was crying. ...so, it was a personal decision to keep watch. Plus, Connor was still hurting from a major jaw distraction surgery and now was having an unknown/undiagnosed problem.
After having cultures done to rule out Connor contracting RSV or any other virus, it was decided t see if he was aspirating (basically swallowing and instead of all the formula going to his stomach, some going to his lungs). He had a swallow study that is basically a live x-ray watching him swallow different thichnesses of formua. It was pbvious he was pooling his formula in the back of his throat and then just letting it drop...meaning, he was aspirating. ....this picture shows the bottle nipple (the dark spot at the left) and you can also see his distractors (the long angled screws below his teeth buds).
This is Connor done with his screws being turned. Major difference in his chin.
Connor still on meds but beginning to be a happier baby. Not as many tubes, not always drugged to the point of constant sleeping, and getting tons of attention.
Shortly after being done turning his screws, the long adaptor "thingies' were removed and he was left with a small screw. There is a sort of joint at the tip here and we ended up needing to keep bandaids under this all the time to keep that joint from rubbing him raw here (to this day, caring for his ears involves placing a bandaid under here...it's a tight fit under there and not something Connor looks forward to).
After deciding thickening formula and swallowing was hurting Connor more than helping him, it was decided to put an NJ tube in him for his nutritional needs. ....he was extremely swollen in his throat and down his esophogus from his surgery and most likely from reflux and aspiration from birth. ....the NJ tube differs from the NG tube in that it requires an x-ray to place the tube in the coils of his intestines.
We were finally released on Monday the 21st. A home health company would meet us at home and show us how to use his feeding equipment. Of course, drama came. The company was late, we panicked and called the hospital because this meant he wasn't getting nutrition. ....the home health van eventually came...the home health nurse eventually arrived....a grandma had been flown here to help us out....and we were on our way to caring for Connor on our own. ....and then that night, the tube came out. .........a little panic, a call to the hubby, a call to the doctor.....and I was back at Primary Children's to have another x-ray done to put the NJ tube back. Yep, same day he was released. ...........not an easy road peeps.
We learn as we go. We learned of these binkies that had little stuffed animals attached to them (wubbanub). They supposedly helped keep the binkies in babies mouths. Well, didn't really work that great for Connor. We were up constantly in the night because he wanted his binky but because he couldn't suck, we'd have to prop things against his binky. Fortunately, he sleeps with a monitor so if by any chance something went wrong, we'd be alarmed by a pretty lound beeping from his machine. (to this day, this has never happened even though the monitor did initially go off mainly because his leads would fall off...leads are the sticky things attached under his arms and to the machine)
Sawyer helping with clean up after a party we had for his brother Tristan's 17th birthday. ....hmm, where are the pics from that????
Marnee, goddess shopper, found us a bunch of beanie babies. I sewed this one to a binky and man...it's been a life saver. If your kid needs one because of a cleft palate, get a good strong embroidery needle, a book to push against as you sew through that thick binkie and then thank your mama or grandma or whoever for teaching you this basic skill. You may now sleep a little better....until the dog/beanie/baby moves.
Sawyer got his first hair cut at some point in this journey. Did great with the scissors but hell broke loose the moment the clippers came out. My boy may end up with a mullett because the experience ended with just a horrible front cut.
Yeah....freaking adorable!!!.....just recently, Connor has been moved to an NG tube. He was to have the distractors removed on June 20th but his lungs sounded horrible so the doctors made the decision to postpone the surgery until July 18th when he'll also have tubes placed in his ears. The journey for discovering his lung and breathing issues is ongoing. His aspiration, reflux and feeding issues are also still being looked into. As of today, he does not know how to swallow.
This is his feeding machine and various meds. Every morning he gets a new bag. We tend to put 8 ozs of formula in the bag at a time. He gets all of his nutrition from this. He has been on Similac Sensitive formula and gets 24 ozs. a day.
This is his handy, dandy backpack. It holds his formula, feeding machine, some sterilized water with a syringe (to flush the system or give meds), a stetoscope (to listen for air bubbles when placing NG tube and also to make sure placement is where it should still be ...you'll hear bubbles when you put air in the tummy and listen with the stethoscope to the left of his belly button), this also holds his diapers, an extra outfit, and if we need to go anywhere, it holds Sawyer's extras and a wallet and cell phone for me.
This is a port on his tube to give meds and to also flush the tubes with water periodically to keep them from getting blocked. We use warm water when flushing and he needs this done atleast every 4 hours. Now that he is also getting intermittent feeds (2hours receiving 90ml/3ounces and 1 hour shut off), we can disconnect him and don't have to constantly carry him around with the back pack. Of course, whenever he is reciving a feed/formula, he has to be connected to the feeding machine and therefore, the backpack is a constant fixture.
This is a typical scene at our house. He is in his high chair, in the kitchen with his binky dog and back pack hooked to the front of his seat. He is at about a 30 to 45 degree angle as much as possible to help with his reflux that we are discovering he tends to aspirate rather than spit up (though there is a little spit up now that he's having formula in his stomach). ....once we're all done with breakfast, Connor has his diaper changed, I take care of his ears (washing around the distractors with a wet q-tip and bacetracin placed where the distractors protude from his ears and then a baindaid placed under the joint), and we care for his mouth (using a damp mouth sponge to clean out his mouth and some special chapstick that won't eat at the plastic from his tubes). I also used to suction Connor at this time with an industrial hospital suctioning device. From the time he got off the ventilator (after his surgery) until about 3 weeks ago, he has needed suctioning anywhere from 3 to 5 times a day. I used to get huge amounts of white mucous from him......this is better even though there is still an issue. We've also just recently discovered he has a case of retracted breathing ( a quick youtube search will show you this). It is something we didn't look for before and only in the past week the pediatrican noticed during a trial breathing treatment (which just exasperated his lung/breathing problems).
A typical morning with Sawyer watching me get Connor together and trying to keep Sawyer in a good mood too.
Blocks under Connor's bed ....this helps with reflux.
A snugglie to keep Connor swaddled at night. He gets an ounce an hour of formula so that we don't have to get up to turn off the feeding machine. We do get up atleast once a night to flush his tubes out. He also stays hooked up to his apnea monitor at night just in case he chokes on his reflux (he's always self recovered from these episodes and he's yet to set off the machine....but we sleep well because we know we'd be alarmed if he needs help...and alarm is as loud as a fire alrm!!)
Sawyer getting some pool time. He prefers to play outside of the pool rather than in it. ...and Tristan is with his dad for the summer. I wish I could add the pics his dad's sent me because he's having a blast in Florida.
Connor is actually looking good. He has quite a few doctors, some equipment issues that may be changing back to an NJ tube and maybe move to a G tube (more to come on that one), he has oodles of appointments, and another surgery soon (July 18th). His weight this past Friday was 11lbs, 2 ozs. and he turned 3 months on Saturday. He's got a road to go but we feel confident that he'll become a normal, responsible, caring adult. ........we'll keep ya posted as well as we can as we learn more. If by chance you have a question, email me (firstname.lastname@example.org) or send a message through this blog. We'll do our best to answer anything. ....there isn't a ton out there for Pierre Robin and I hope this helps someone in our same boat.