Hello and welcome. My name is Kaitlyn Anne and I have Spinal Muscular Atrophy Type 1. Children born with SMA 1 gradually lose strength in their nervous systems and die, usually by the age of two. There is no known cure or long-term treatment. SMA not only affects the muscles that help me move, but it also affects those that allow me to breathe, swallow and clear my airway.
Everything's been pretty quiet around here. Kaitlyn is back to her normal baseline!! Still having slightly slower digestion, but nothing serious. The rest of the family is finally healthy again. I like when everything is "normal" around here. Now we just have to try and keep the kids healthy until the end of the cold and flu season, which should be soon!! Come on summer! We have had people enter our home and tell us at the end of their visit that they have had illness in their home!! Our own families don't enter our house when there is a sick family member living under their roof or if they know that they have been exposed to illness!! This is just an example of how people don't understand what it means for us when Kaitlyn gets sick. It disrupts our whole family dynamic!! Not to mention the fear, pain and suffering that poor Kaitlyn is put through everytime she gets sick. Especially when she has to endure a visit to the hospital. Don't get me wrong, our children's hospital is hands down a wonderful place and Kaitlyn would not be alive without them, but some visits are definitely easier than others.
We've been taking our normal walks down to the lobby in our apartment building to get the mail. It's a family event and one the kids enjoy, a lot of weeks it is their only outing outside of our apartment....no kidding! Some pics of the outing.
Kaitlyn waiting to go for our walk.
Getting ready to go out the door.
The door opens...Kaitlyn gets excited!
Silly boys all grins too!
New favourite thing to do...crawl through the table in the lobby!
We're home! We got back home Thursday afternoon. Kaitlyn is doing really well. Spent the last two days off of bi-pap except to sleep. She is pretty tired. Still taking 2 1/2 to 3 hour naps in the day. This morning she decided 6AM was wakey, wakey time for MaMa!! I made her watch Dora until 7AM when I started her normal morning treatment. I know bad MaMa, but I really want...no need...for her to get back into her normal daily schedule!!!
Kaitlyn is tolerating her feeds pretty well, but she's not back to normal yet! When I feed her with her pump she does pretty well. Today I tried to bous her 30 mLs and she cried when she saw me coming with the syringe!?! I'm soo confused. Hopefully she'll start tolerating full feeds at regular concentration pretty soon.
Kaitlyn's left arm is pretty tender. It's the arm her surgeon had to make the cut downs in to place the peripheral PICC line that then blew!!! Hopefully her arm will feel better in a few more days, especially once the incisions heal fully!
Sorry for no post for days 11-12. I sort of got caught up in my own illness!! So what happened Monday....we went down on Kaitlyn's Bi-pap settings. She had been holding 98% and above the entire night Sunday night. Only one jump down to home settings! We moved Kaitlyn's treatments from Q3 to Q4 and she has tolerated this just fine. She is still getting the odd pluggy thing up but overall is doing fantastic! Our goal was to also up her feeds and see how she tolerated the change. We went from 10mLs an hour up to 12mLs then fifteen, then up by 5 mLs every four hours.
So far Kaitlyn has tolerated rate increases without any issues. She seems to be digesting everything just fine now. I really think that she just needed the break from eating. Kaitlyn lost her PICC line in her arm Tuesday morning. Her arm was very sore and swollen. I cannot believe that she blew four lines this hospital stay!! Craziness! I guess we'll be having a lot of discussions now about ports (central veinous access). It would seem with Miss Kaitlyn that we haven't much choice anymore! We have tried just about every type of line they can place, and she chooses to blow them all except the central line in the groin. This is the line we requested upon our arrival in hospital the very first day. They didn't place the line....for whatever reason!!! And then a week later when they decided to try she had been undernourished and under hydrated for a whole week.....so no wonder they couldn't place the line! They won't do a jugular poke in this hospital without a secure airway (intubation)....so this was of course never an option for us! Our pediatrician has included in Kaitlyn's protocol...MUST FIND CENTRAL ACCESS STRAIGHT AWAY! Do you think next time they'll listen to us!?! I sure hope so.
Not much to report about day ten. I had been fighting a stomach bug and finally had to call Tim and tell him to come in. There was no way that I was going to be able to stand and do cough-assist when I was running to throw up!! So I went home early Sunday morning and Tim stayed with Kaitlyn. I stayed in bed almost the entire day Sunday. Tim got sick through the night Sunday. I got back to the hospital this morning early(Monday). I'm NOT 100% but at least I'm not throwing up anymore, and poor Tim needed a break. Hopefully he'll go home and spend the day in bed!!
Kaitlyn made out fine yesterday. He bi-pap settings went down. She's almost back to her home settings. Her sats are also much better. Our goal for today is to stretch her treatments from Q3 to Q4 and see how she tolerates the change. Her lungs sound much better, but she is still throwing the odd plug! We have also decided to try upping her feeds again. SLOWLY! She's getting 10mLs and hour now, we will up that to 12mLs and then up to 15mLs depending on how she tolerates. Kaitlyn's pediatrician is back today(Monday) so finally we should have some consistency to her management and hopefully get out of the hospital by the end of the week! Provided we can keep this stomach bug from her!! Keep your fingers crossed that Kaitlyn doesn't get anything new over the remainder of our stay!
Ok, day 9 sucked!! (Sorry, but it did!) So the Intensivist was supposed to come up and place a central line this morning, and in walks the resident whom we knew was working in PICU (where the intensivist is in charge). The resident tells us that Kaitlyn is scheduled in surgery, OR, at 2:00pm. We fought, of course, to get the Intensivist to come to us...but it was surgery or nothing!! So the they wanted us to transport Kaitlyn to the OR without bi-pap, as they have no way to power Kaitlyn's equipment for transport. We said..."UMMM NOPE!!!" She is currently ventilator dependant, panics and can't breathe without the vent right now! To put her through that would be insane! We told them that we had back up power for our equipment and if they would leave everything alone once we had it hooked up, then we would arrange to power Kaitlyn's equipment for the time they needed to have her in OR! I am so thankful now that we went with the more expensive equipment when we were purchasing back up battery power! She was able to have uninterrupted running of her vent for the entire time in OR!
Then they told us that we weren't allowed to go into surgery with Kaitlyn. The resident said that that just isn't done and it is NOT allowed! We told her that indeed it is allowed we have been offered in the past to go in the OR with our daughter! She just said, well it's not allowed this time. We said that she couldn't go into OR with an unprotected airway, unless somebody who knows her goes along!!! They finally agreed to let Kaitlyn's chest physiotherapist go in with her to suction and monitor her airway needs! She would also be there for moral support, a familiar face so that Kaitlyn wouldn't panic. We told them NO ANESTHETICS, freezing YES, but nothing that could compromise her respiratory system!! They agreed!
The plan was to place a femoral central line (called a cook line). This is the type of central line that Kaitlyn has always had. The intensivist couldn't get the cook into the vein, it just collapsed! If they had done it a week ago when we first asked them to they might have had more success!! Kaitlyn is under hydrated right now, making any vein tricky to access. They had to call in Kaitlyn's general surgeon to do what they call a cut down. They cut and pull the flesh away to expose veins and then place a PICC line (a really small catheter) into the vein and advance the line up the vein to a more central location within the body. This places the catheter into even larger vessels for less chance that the line will blow or cause any issues! Our surgeon did the cut down in Kaitlyn's left arm. The first cut he made exposed a vein that blew when he accessed it. Then he had to do a second cut down, also in her left arm, this vein he got threaded but could not advance the line all the way centrally. He could only get the line threaded up just past Kaitlyn's shoulder, but he told us that the line was enough central that it would meet all of our needs for this stay! He also told us that Kaitlyn's left arm is now inaccessible for future lines...not really sure why. I will have to have him clarify this for me next week when he's around!
Respiratorily Kaitlyn has been fairly stable today. Got out three BIG plugs, the most awful looking things you'd ever want to see! Great big gobs of stuff! RSV creates really thick, stringy secretions, I had no idea how yucky this virus was. Until you actually see it for yourself, it's hard to imagine! Anyway, we were getting lots of stuff up especially in the morning. Kaitlyn has a vaso-vagal response. It's when the heart rate drops suddenly when we are suctioning. It only goes down 20-30 beats per minute, but I have only ever seen her do this after surgeries when her body has been really stressed out. (Remember this was before Kaitlyn even went to the OR...and she didn't have any general anesthetics or anything that would typically have caused a vaso-vagal response).
Kaitlyn had another desat over night last night. She was bringing up lots of thick gunk during the 4:00am physio, as a result she gagged and spit up!! I suctioned as best I could, then coughed her to try and get all the formula out of her lungs. It was obvious that she had aspirated some despite my best efforts. She had to have 10 L of O2 added to her bi-pap just to keep her sats above high 80's!! We were able to wean her down to 6 L in only a few minutes with a few more rounds of cough-assist!
She had chest x-rays that showed she still had the infiltrates in the upper left lobe of her lungs, and a new area of what looked like either aspirant or incipient pneumonia in the upper right lobe of the lungs. X-rays are funny things as they lag behind the actual symptoms. They are also open to interpretation, but the main thin is that Kaitlyn doesn't seem to have any areas of pneumonia in her lungs. This is really good because it means that we have been successful in mobilizing whatever is in her lungs. We are not giving the mucus and secretions a chance to settle and become a full blown pneumonia!
Bloodwork still looks good. We asked again for a central line today. Nobody wants to place a central line in Kaitlyn. The surgeons are usually the ones to place the line, but they won't touch our girl, as she is too difficult a case. The Intensivist didn't want to place a central line, she wanted to see if a PICC line could be placed. A PICC line goes into a vein and is a long catheter that is threaded up into the vein until it meets the larger internal vessels. This type of line would be less likely to clot, and more likly not to blow!! There is only ONE doctor in our entire Children's Hospital that has the necessary skill to place these lines. If she's on vacation nobody gets a PICC line....seriously!! She came up to see Kaitlyn, at the request of the Intensivist. She has rendered Kaitlyn inaccessible for a PICC line. She is basically saying that there is no vein on Kaitlyn's entire body that she could access to thread a catheter to a major blood vessel. This is extremely scary!! If our child needed veinous access in an emergency...they might not be able to get it!!!!
They are of course begging us to reconsider some sort of more permanent veinous access. It seems like he older she gets the more tricky she gets to place lines, and the more fragile her veins become...so she blows the lines (this literally means that the fluid being delivered leaks out of the blood vessel into the surrounding tissues)!! This is why Kaitlyn has had two swollen feet and a swollen hand this hospital stay...from the three "blown" peripheral lines! Yes that's right Kaitlyn has blown her third peripheral line...no more TPN until tomorrow. The issue with some sort of more permanent line is that they always require care, which means that Kaitlyn requires more invasive type care! But the trade off is that she would have permanent veinous access. So...why haven't we done this??? Seems simple, place the access line..."POOF" magical veinous access!! (Sorr for the sarcasm ;oD )
There are two main forms of permanent lines, what they call a Port-a-cath or a Broviac line. The broviac line is typically the easiest to place but it is an external line. This means that wherever on Kaitlyn's body that the decided to access a vein, Kaitlyn would have extending outside of her body an external IV connection line! This line has a higher chance of being infected, and you have the worry of it getting pulled, and you have to keep it dry. This means that we would have to use a surgical dressing every time that we wanted to give our daughter a bath. To us anything we choose for Kaitlyn that further limits her "normal" activities just is NOT worth any advantage it may provide. The other option is the Port. It is like a PICC line that is inserted under the skin threaded up to a larger vessel and has a balloon that sits just under the skin. Any time we would need veinous access we just poke the balloon with a needle and BAM veinous access. The problem with a port is that it needs to be surgically placed, which in the past has never seemed worth it to put Kaitlyn through. We always had other options, but we are running out of access the older Kaitlyn gets.
The other issue is that Kaitlyn has a blood disorder and with ANY sort of permanent line she would have to go on blood thinners for the rest of her life. So now, not only are we dealing with permanent veinous access, which comes with it's own set of risks, now we have a whole new set of risks related to the blood thinners. There is relatively low risk with the heparin based drug we have had to have Kaitlyn on in the past. But with that drug it needs to be delivered via a subcutaneous injection two times a day, and the drug is not cheap. While drug costs are usually not our first concern, when we have to pay out of pocket for all of Kaitlyn's drugs that certainly has to factor into any decision we make based on whether to enter into a commitment where she needs to be placed on a preventative medication!
Since Kaitlyn has been rendered inaccessible for a PICC line the Intensivist said that she would be up to place a central line through the large blood vessel in Kaitlyn's groin. This is where she has had her central lines in the past....but it is also where she has had her blood clots (veinous and arterial!) in the past. We have had a consult with haematology and they have said that once the line is placed we will have to start a blood thinner to keep Kaitlyn's blood from forming clots around the central veinous line! The blood thinner should only need to be continued for a short time after the line is removed. The intensivist couldn't make it up today, as it turned out, but she has promised to be up tomorrow morning before noon to place the line.
Last night Kaitlyn blew her second peripheral line!!! This girl does not appreciate the additional hardware! It really sucked because there was a perfectly good bag of TPN hanging from an IV pole mocking me. So as can be expected over-night with only her reduced rate enteral feeds Kaitlyn stopped moving secretions and her sats went back down. The resident came in this morning and I actually had to argue with him about secretion management. I said that I felt that her lower sats were a direct result of her not being properly hydrated, to which he sort of shrugs his shoulders and gives me the eye roll. To which I say..no really. He still doesn't think that I am making any sense until I explain to him that if Kaitlyn is dry that the thicker secretions in her lungs are not being mobilized, her airways are compromised and her sats are therefore lower than normal!!! When she is well hydrated then the thicker mucus has something to slide up on and when we do a treatment more comes up! Therefore higher sats!
Then he talks about lowering her bi-pap settings to her home settings...to which I said, not until her sats actually improve instead of getting worse!! He agreed ;oP Then he tells me that her chest x-rays that we had done yesterday showed infiltrates mostly in the upper left lobe of Kaitlyn's lungs. This would be consistent with a virus like RSV. At least there wasn't any indication of pneumonia, so for now we are keeping things moving! Then, almost in the same breath, he tells me that we should move the treatments to every four hours instead of every three and that way we cold get more food into her (as we wouldn't have to turn the feeds off so often just to do a treatment). What could I say...I have to get food into her somehow!
Kaitlyn tolerated her first treatment this morning really well. I got a bit of gunk out of her nose, but overall not too productive. So I turn her feeds back on and the PT (chest PT) says that she will be back at two for the next physio. I'm thinking to myself that's too long between treatments, but I also need to feed my daughter. Since her chest sounded pretty good I was willing to try it and see how Kaitlyn tolerated the decrease in CPT. About a half hour before she was due for physio she started sputtering. Tim was here so he coughed her and then started physio. She was pretty crunchy sounding in the lungs, but I wasn't worried. PT would be in soon and Tim know how to manage Kaitlyn, so I headed home. I called to see how she had made out with her physio as she worried me a little with how wet she had sounded before I left compared with how she sounded this morning! Tim told me that she had crashed! She was on even more of an increase with bi-pap settings, needing 5L of O2 to maintain sats. A central line was outright refused as Kaitlyn has a blood clotting disorder. The only type of central line that was offered was a jugular line under general anesthetic (knowing full well that we would never choose this in her current weakened state and respiratory condition!!!!). So a THIRD peripheral line was placed and all we can do is hope that she doesn't blow this!
Tim called back a couple of hours later to say that Kaitlyn had been weaned off of the supplemental O2, but that the Bi-pap settings could not be reduced. Her lungs were still wet sounding but not as bad as earlier. She had her new IV and PPN had yet again been started!
Owen and I were playing on the spare bed tonight before supper. He likes to jump on me and rough-house. He smashed the back of his head into my nose...I heard what seemed like a really loud crunch, saw stars, saw black, then felt really sore with a bit of a head-ache. My nose is a bit swollen and there's a bit of a reddish bump!!
Tim calls me tonight and says that Alexander had vomited twice since he got home. I ask you...what else could go wrong!!! Please keep us in your thoughts.
So Kaitlyn woke up this morning with much better sats. After they placed the peripheral line (IV) and Kaitlyn got her PPN she was much better hydrated. We began getting more stuff up out of her lungs and upper airways. After about three more treatments her sats were in the higher 90's (95-97) almost the entire afternoon.
Kaitlyn's IV blew around 9:00am this morning and had to be removed. This meant no more PPN and no more additional hydration. Kaitlyn's dietician was in this morning and decided after several calculations that she really should get some nutritional support. The pediatrician on the floor didn't want to put Kaitlyn through another poke if the line was only going to blow that fast again, but what choice do we have? I cannot let her go a single day more without proper nutrition. The doctors agreed. Another request went out to get the best person in the hospital to place the new IV! The line was placed later this afternoon and after the second treatment today Kaitlyn began to run lower sats again!! Man! This girl likes to be hydrated!! I cannot believe that a few hours on low rate feeds alone could make such a difference!
After fluids were started through the new line Kaitlyn did a little better through her afternoon therapies. By this evening her sats were back up again and we are again getting a bit up. Without the PPN Kaitlyn's mouth gets sticky and NOTHING is mobilized!
No other significant changes! Still on Q3 treatments, only using regular saline nebs, and trying to just let her rest between treatments!!
Kaitlyn had a worse night Saturday night. This is to be expected RSV usually gets worse before it gets better. Sats were in the low 90's high 80's most of the beginning part of the night. She did level out as the night went on and climbed up to the low 90's early Sunday morning. We pushed on with the Q3 treatments. We still weren't getting a whole lot out of her. I think she was starting to get not "dehydrated" but definitely on the dry side!! She got a new diaper at 4:00am and it was still bone dry at 9:00am!!!
Sunday morning during rounds I again brought up my concerns about Kaitlyn's nutrition/hydration!! She was still getting only half, if that, of her daily requirement for feeds. For an SMA child this is unacceptable!! The SMA 1 child has about 5-10% of the normal muscle mass! When we think about how critical muscle is for storing nutrients and acting as a buffer for when the body is in need, then it makes more sense why we as parents "freak-out" when our children are even slightly starved! We have spent a great deal of time and energy researching-networking trying to perfect her diet. If she doesn't get the nutrition she needs EVERY 24 hours then her body could potentially waste muscle (break down muscle for nutrients). This makes Kaitlyn weaker and the strength once lost does not usually return.
I was also concerned about Kaitlyn's ventilation. We usually check Kaitlyn's blood CO2 levels to see that she is getting enough air exchange.....breathing in enough O2 and expelling enough CO2. We also wanted to know what her electrolytes were as this can often give us an idea of hydration. None of the normal blood work had been done!
By mid-afternoon Kaitlyn had both her blood work done and a peripheral line placed. The blood work was all normal, and the IV allowed us to begin giving Kaitlyn PPN a dilute form of TPN (an intravenous form of nutrition)! Finally! So far so good. More wet diapers, and her secretions are thinner and we are getting stuff up now!
RSV! One of the most dreaded respiratory viruses that our SMA children can have! Kaitlyn has just been diagnosed with RSV! We did EVERYTHING in our power to prevent this from happening. We are diligent hand washers, Owen has to remove all clothing at the front door when he comes home from school, we are careful when out in public to teach our children not to touch things (elevator buttons and such), etc. etc. etc. RSV is nasty. It's super contagious and is not like the typical cold virus where once you have a particular virus you can't catch it again. RSV can be contracted over and over and over again. That's what makes it particularly nasty in my books!!!
We brought Kaitlyn to hospital over issues with digestion. We have only been able to get half of her normal feeds into her for four days now. We thought that if we brought her in that she could be looked at and we could figure out how to get more feeds into her, even if it meant TPN for a few days so that she wouldn't be compromised nutritionally. They of course had NO idea what to do with her!! It's kind of funny watching medical professionals stumped, kind of scary too! They decided to have radiographs done of her abdomen, particularly the placement of her G-tube and a look at the bowel. The x-rays have shown a partial blockage of the bowel, which is why she isn't tolerating full feeds. We are currently giving her a bland mix of her regular formula and pedialyte to see if she digests it any better. It did seem to be working, but Tim thought tonight that she was reverting a bit. I think perhaps she was just too tired. Time will tell.
Back to the RSV. So far, because they tested for RSV very early in her "cold" (as we hadn't brought her to hospital for her cold symptoms) she is managing very well. She has had a few dips into the low nineties, but overall doing fairly well. But this is only day four since the onset of ANY symptoms! Typically days 5-7 are the worst, this is when the virus seems to peak. A couple of doctors today were even saying that it could be as much as ten days before we see a real spike in symptoms! We are in for a long haul regardless! This is not something that any SMA child is going to shake off in record time. Kaitlyn is the second child that I personally know of that has both received the RSV vaccination and still gotten RSV! Not too sure what's going to happen next year! She'll probably be denied the shots since she got the virus anyway.
As always (but especially now), keep our little one in your thoughts! We are trying to be optimistic that since we caught it so early that we can stay on top of things. Kaitlyn is on Q3 treatments and we are doing our best to keep those airways open!!
We are officially a sickhouse. I think it's safe to say that everyone in the house is sick to some extent. Owen and Alexander have been sick since Sunday. I came down with a sore throat and no other symptoms. I thought that it was just allergies or sensitivity to environmental factors. I get sore throats A LOT! So I didn't think much of it until yesterday when my nose started to run. Today I'm full of all sorts of thick mucous (gross huh!). Tim is sick with a bit of a cough and he says he's tired, but overall pretty good.
Alexander is the WORST! Poor little guy is absolutely miserable. He and Owen have awful crunchy coughs and runny noses. Owen says his throat is sore, and Alexander keeps holding his head and saying ow..ow! Alexander got up this morning, drank some milk, coughed up a lung and went back to sleep for three hours. He got up was awake through lunch, but wouldn't eat lunch, then went back to sleep for an hour! Alexander had a fever this morning too! 39.1 when he woke up! Since then we have been keeping Tylenol into him. Throughout the day he has been running 38.1-38.3. Alexander LOVES CPT, so we did lots of CPT today! It's good for him! By tonight he had perked up a bit, hoping tomorrow is a better day for him. We'll continue with the CPT because it seems to make him feel better.
Owen is still coughing and is still pretty sick, but at least he is eating and drinking a bit. He hasn't been as tired as Alexander, but he has still been pretty tired. Owen hasn't had a "fever" yet. He is running a little higher than normal, but not technically a fever. He should be on the mend, although he still has a nasty cough and runny nose!
Kaitlyn: ahhhhh Kaitlyn. Well, lets see. She woke up Tuesday night fussing. Usually when this happens she wants to be rolled over or has gas in her tummy that needs to be manually vented. (We leave her tummy vented all night long, but sometimes air still gets trapped!) So I rolled her on her back. I tried manually venting her stomach, but no air would come out. Kaitlyn gave her face like she had secretions or something she needed suctioning. So I suctioned her mouth and didn't get anything really, was reaching to turn off the suction machine when she spit-up!!! Kaitlyn has a nissen!!! How can she spit-up!
Anyway, back in with the catheter I went I suctioned until I got the worst out. Then I whipped the bi-pap off. If there were formula in the airway I didn't want it getting pushed any further into the lungs! I then aspirated her stomach.....I wasn't going to risk having any more food in there until I could figure out what caused her to spit-up. While waiting for her tummy to drain I continued suctioning. The amount of formula in her stomach was astonishing. Her sats were only 1-2 % lower than normal but she was just starting to dip a bit when I felt that her stomach was done draining. I coughed her several times and she came back up pretty fast. When I was done she was holding 96-97%. After CPT and more coughs I put her back on Bi-pap. She had 120mLs drain out of her stomach (which I had to dump), there was 10 mLs left in her stomach plus however much she spit-up!! Kaitlyn sleeps with a rate of 60mLs an hour. For some reason she stopped digesting her food!
Wednesday we had a hard time getting food into her. She wasn't tolerating a very high rate at all. I think that I got most of her day's formula into her, but I had to leave out her solids. Last night we started her out at a rate of fifty, but she wasn't digesting fast enough, so we had to bump her down to 40 mLs an hour. Today she wasn't processing very fast AT ALL. I think I might have gotten about 40 mLs an hour into her, that's about half the rate that she would typically get with solids. Now it's been two days without solid foods. Not a huge loss in calories, but still worrisome when I don't know how long this is going to continue. Also worrisome because of the loss in fluids. Today Kaitlyn also got up with increased secretions. There is some thicker stuff coming out of her nose today. Overall she doesn't look too bad, except for the digestion. Her sats are 1-2 % lower than normal. She is dipping into the low nineties during coughs and when she cries (something she only does when something's up). Tonight she is sleeping comfortably and we have her rate down to 40 mLs an hour. Hopefully she'll have a good night.
Well wishes going out to Lizzy's family and Mary's family. We hope everyone is well soon! Keep us updated folks, we'll be thinking of you all!
Well morning two is definitely worse than yesterday! The boys are both mouth breathers today and NOT happy about it. Alexander's fever is back but is still low grade. They are both coughing more, nice crunchy wet coughs!! LOVELY! HA!
Alexander can't keep his hands away from his nose. He is constantly fussing over it! He's only two and cannot figure out how to blow it effectively. I keep thinking, "how am I ever going to wash his hands enough to keep the germs down so that Kaitlyn won't catch this cold?" I'm realizing that I won't keep the hands washed!! I'm going to have to wash myself EXTREMELY often, clean surfaces like crazy, and "kill" the first boy that touches Kaitlyn while sick!! It may sound really sad to someone who isn't immersed in the SMA world, but our boys know that they cannot touch Sissy when they are sick!! Even Alex!!! They seem to understand that she is different and just accept that they cannot touch her! Life gets sooo complicated when we have to go to the hospital!
I'm really hoping that Kaitlyn doesn't catch this cold. While it isn't at this stage a chest cold, it is causing thick secretions in both boy's noses and a crunchy wet cough. Something that could easily be FAR worse in their sister! We're keeping our fingers and toes crossed that Kaitlyn doesn't catch this cold!! I guess we'll know today or tomorrow whether or not we've dodged this one or not.
Yesterday we had such a beautiful day with friends at their home. Their little guy has been quite ill! He had RSV with pneumonia, couldn't eat or drink much and lost a bit of weight. He was just starting to feel a little better, eating and drinking again, when he got a stomach bug. The poor little guy was vomiting for days. He is much better now, and has been told by his pediatrician to stay home (not go to school and stay out of crowds). His immune system probably isn't up to snuff quite yet. We went to visit them yesterday. Everybody was well and my boys were soooo excited to finally get to see their buddy!! Owen sneezed once or twice while we were there, but I thought nothing of it as Owen has allergies, he often sneezes in a strange environment.
That night the boys were sneezing a bit and Owen coughed once or twice, but again I still didn't think much of it. After the boys had their bath, they looked like they had been drug behind wild horses. They were beat!! Poor Alex could barely keep his eyes open. At 7:30pm Owen says, "I think I'll go to bed now"! He was the first one in bed, usually the last because he is the oldest. I thought HMMMMM they must be really tired! I carried Alexander down to bed next and he felt warm. Our kids almost always feel warm, they run hot! But I decided to check his temp. as we are paranoid about illness in our house! To my surprise he had a bit of a fever. Low grade, but still a fever.
This morning I woke up to the sound of Owen coughing! My first thought, oh NO here we go again! My second thought, Oh crap we were over to our friends home the day before!! But we had no way of knowing! I feel just awful. Owen's nose is all stuffed up and I think all the coughing is from the drainage down the back of his nose. The cough is wet, but not in his chest!! His voice is also scratchy, but he says that he hasn't a sore throat. Alexander had a fever this morning 37.9 degrees, stuffy nose, wheeze, and a bit of a cough! We spent the day wiping noses, hand cleaning/sanitizing, trying to keep Alex from crying (as he gets wheezy and chokes), and sooking two sick boys.
So far Kaitlyn hasn't shown any signs of cold!! Hopefully she will not! The boys have runny noses, are sooky and tired, but at least they aren't that sick. When the cold is in the head...it's a good cold!!! We are hoping this cold blows over as fast as the last one did! And we are also REALLY hoping that he boys didn't pass on their cold to their buddy! I would feel just awful if he got sick because of my boys.
Finally!! A while back I remember having said that we were looking into getting Kaitlyn a second cough-assist. I cannot even begin to imagine what would happen if something were to go wrong with Kaitlyn's machine! We use it so much, and it's not uncommon for her to need to cough at some point through the odd night! In an emergency...it NEEDS to work!
NIV isn't very popular in Nova Scotia, and I personally know of no other person using an NIV protocol in my own province. Our hospital has ONE cough-assist which they use sporadically with limited ability and therefore limited success. They of course blame the effectiveness of the machine!! The cough-assist at the hospital is kept locked in an office of PT. So if we had an emergency and needed the machine off hours we would have to wait for the on call PT to answer a page, call back, get into the hospital, bring us the machine, and then I'm sure argue with us about how it should be employed!! I'm not sure that Kaitlyn would ever live through an aspiration without a working cough-assist used at a moments notice!
Anyway, our social worker called and told us that our request for a second cough-assist was approved. Our first cough-assist came from MDA. They have been awfully good to us!! This new machine is coming from the government. It is such a relief to know that soon we will have a back-up! We have never been taught how to use an ambubag. Something that I think we will remedy very soon, but Kaitlyn has never tolerated this very well. This could be because any time one was introduced she was already pretty sick and scared. I'm thinking that if Tim and I learn how to use an ambubag that perhaps Kaitlyn could learn to cooperate somewhat, at least until we could get to a cough-assist!
Thought that I would share the good news with the ones that support us the most! Take care everyone....Spring is coming!!
This is Kaitlyn sitting in her corner seat! I finally remembered that the supports in the back come apart and can be lengthened to add a little room for growth. The chair is at it's maximum allowance, but it will work for a short time more. She loved sitting up getting a new perspective. She tolerated this position for a half an hour before getting a little tired and chokey. I didn't push her. I thought that was really good for not sitting up for months!!
Laughing at, what else, Dora!!!
She got a kick out of the tray. She fingered a little unicorn and her disney fairy!
Kaitlyn had a 9:00am ped. appointment today. It was a regular well visit check up. I was not lovin' the early appointment, but we do love being the first appointment of the day!! We tend to miss more of the sickos this way! Kaitlyn weighs 12.6 kg and is 36 inches long. She is now 27 months. She's in the fiftieth percentile for weight which is really heavier than a child with SMA should be. Typically these children are kept in the 20th percentile or close to it. But she's between the 75th and 90th percentile for height. We put Kaitlyn on the AA diet last year in March and since then we have not changed a single thing except to add calcium, Vitamin D3, and 30mLs of prune juice to keep her regular. We have just been waiting for Kaitlyn to grow into her mix. So far no plateau! She just keeps growing and growing and growing!! We asked about reducing calories but our ped. isn't comfortable with this because she says that Kaitlyn is so healthy, and is growing like a weed and she hates to take calories away and make her hungry! Especially since she IS growing so fast, and doesn't show any signs of slowing down.
Kaitlyn has break down on her ear that we have to go see plastic surgery about!! Usually we get very superficial breakdown every few months and it takes a few days to heal. This current breakdown is not very big, but has never really healed up in over two months now. It looks really good most days then will puff up a bit and ooze for a day. We have off-loaded as much as possible, but Kaitlyn obviously prefers that side of her head and can still turn her head to lay on that preferred side. Our ped. gave us a stronger antibiotic cream to try in the meantime. But she wants us to see plastic surgery to be on the safe side, she wants to rule out that there isn't infection in the cartilage! I have made Kaitlyn three pillows now that have a hole in the center for her ear. This way she isn't putting any pressure on that sore ear, but can still have her way about what ear she wants to lay on ;o)
The last big thing we discussed with the ped. was Kaitlyn's Bi-pap settings. Now I know that typically this would be the job of the pulmo. but we have a little resistance in this department! We lost our original pulmo. that got us started on NIV. The pulmo. left now has the work-load of three pulmonologists, and no time for things he has to do a lot of research on. Contacting an SMA expert doesn't happen that much up here, especially when said experts are in the States!! Kaitlyn is still on the same settings she was put on when she first started using Bi-pap. While she has always done really well, she is getting bigger, and it only stands to reason that as she gets older the settings would change to allow her lungs to develop in as normal a manner as the machine can provide. What we have noticed the past few months is a general increase in her respiratory rate, while on bi-pap. Like she is trying to take in more air, but just getting it in the form of faster breaths. Kaitlyn used to spend at least a bit of every night in deep sleep riding the machine. Not anymore. Her RR on bi-pap is well into the 50's most of the night. She has NO issues with her lungs, all is clear, and she still seems well rested throughout the day. But she does tire really easy. It takes a lot out of her when we have a day of appointments.
Here are her current settings in case any REAL experts are reading (MOMS!):
Resmed VPAP III ST-A
Mode: ST IPAP: 18 EPAP: 3 RR: 30 (back-up rate) Rise Time: 150mS IPAP Max: 1.00s Max I:E- 1:1 IPAP Min: 0.4s Trigger: high Cycle: high
After the ped. appointment we headed over to the Children's Hospital for Kaitlyn's RSV shots. One in each leg. She doesn't mind this too much. A quick cry and then it's all done until the next month. Only one more round to go and then we are done until next year!!! Hopefully we will continue to get the shots! Our pediatrician is totally behind us on this front, so hopefully we will get approval again next year. These shots are really expensive, but a PICU stay for two-three weeks is WAAAYYYY more costly!!
In the hallway on our way to RSV shots we ran into our General Surgeon. He is a super wonderful man, who has gotten Kaitlyn through three surgeries so far and we really trust. We asked him if he had time to change out Kaitlyn's G-button. She's had the same button since last summer when she had her surgery. We kept asking if we shouldn't change the button, and everyone kept telling us to change it when it acts sticky or if it clogs. It never did these things. What we were noticing was blood flashing back into her extension more and more often, like there was irritation there somewhere. I suspected the G-button, but Tim and I were really hesitant to change it at home. We have two replacement buttons at home ready to use, but we KNOW Kaitlyn. She likes to be difficult!! (Drama Queen!!)
Anyway, our surgeon said to have him paged as soon as we were done with RSV shots and he would arrange to change the button out. He came to us...and he changed the button out himself...in a three piece suit!! VERY nice man!! BUT it wasn't a classic pull the old one out, put the new one in....OF COURSE!! He deflated the balloon on the g-tube, inside Kaitlyn's stomach, then he gave a pull. It budged but gave some resistance. He pulled harder and Kaitlyn really fussed. Then he gave a good tug and out the tube came with a bit of tissue, blood and yucky stuff around the portion of the tube that had been inside her stomach! The tube had been in way too long! But we didn't know any better (we do now!).
The tissue on the inside of Kaitlyn's stomach had grown around the tube a bit. The site bled for a minute after the old tube came out. But once the tube was out Kaitlyn didn't seem bothered too much by the site. Next the surgeon tried to put the new tube in! HA! Wouldn't go in! Tim and I are sooo lucky that we didn't try to change this tube out at home. Our surgeon had to run and get a guidewire and what he called a "stiffener" to help thread the new tube into her stomach! (The G-button is a very soft silicon material that I'm sure must be very comfortable once in, but not very sturdy for a tight push!) The "stiffener" didn't work, the site was too tight. He then had to stretch the opening and try again! The new tube went in the second time, but not without a pretty good push! He wants to do the next tube change in three months time. We say, "Gladly"!
Since coming home Kaitlyn has been a little fussy about feeds, but so far everything is fine. She went to bed well and seems comfortable.
Kaitlyn all ready to go to her appointments this morning! She always looks forward to trips out of the house. She LOVES going in the van. We always take all of her equiptment. Did I say before how much I LOVE this Max EasyS stroller!!!! Onboard....cough-assist, bi-pap, oximeter, feed pump with food, inverter, gel cell battery, mini DVD player, suction machine, and nestled on top....Kaitlyn!! We carry emergency O2 in the van.
This is the inconvenient way in which we have to load Kaitlyn's stroller. Fully loaded with equipment, Kaitlyn's stroller is very heavy....130-140 lbs !! It takes both Tim and I to push/pull this stroller up the ramps! Not ideal! But worth it to get Kaitlyn out of the house!