Crazy Times!

Kaitlyn's been a little "off" for two weeks now! Two Sundays ago Kaitlyn had what can only be described as a plug. A plug is when secretions pool in the lungs and form a ball of mucus. This happens when there is decreased airflow to the lungs and inefficient secretion removal (ie. coughing). The plug blocks the airways into the lung causing atelectasis or even collapse of the lung.

Since the plugging Kaitlyn has had little spells of desatting, mostly through the night and first thing in the morning. Almost like she still has some areas of minor collapse...atelectasis. She has normal lung sounds and no fever, but we have been watching her close.

A few days ago she started with watery stools...literally! We took her back to a base formula diluted with extra pedialyte and lots of water. We took out all of her juices and fruit. After a couple of days she seemed to improve so we began very slowly adding back in her juices and fruit. Then she went to the other extreme and no stools at all for four days! CRAZINESS!

The last three or four nights she has been awake several times throughout the night. This is tough on Tim and I as he has to get up and work the next day, and I have to get up and be mentally alert and physically able to handle all of Kaitlyn's and the boy's needs. Tough, tough, tough! Yawn!!! Oh and Alexander has taken to late night strolls in the dark and needs to be taken back to bed!! I know....any other time I'd be LMAO too!

Today Owen is running a fever. Tonight it was 39.1 poor little guy! His voice sounds a little off but he is only complaining of a sore stomach from time to time. No other symptoms other than the fever. He put himself to bed tonight at 7:30pm. However he was up at 11:45pm to sit on the couch with Tim and I for a few minutes. Pathetic!!! Says his throat is really sore tonight, and sneezed all over me several times! We gave him some tylenol and he went back to bed without a fight, but the poor little guy looks pretty sick.

Now we just have to hope that whatever he has Kaitlyn already had!!

Kaitlyn thought MaMa was pretty funny the other night. As I recall she was just laughing at me for no reason. I think this was the last night before she started getting fussy day and night.





Owen and Alexander got new dolphin and shark flashlights from NaNa. They were having fun in the big box Daddy gave them that came with his new exercise bicycle.

The end of what little sleep we get!!

Owen was so proud the other day! I watched Alexander climb into his crib as I was making the spare bed in his room. I groaned because this meant that he was going to proceed to fuss the entire time that I was making the bed (a job that I hate in the first place). As I finished pulling up a blanket I heard Alexander feet running on the carpet...I turned and thought that I was seeing things. At first I thought that my brain was playing tricks on me and that Alex really hadn't climbed into the crib. Then I asked, "Owen did you get him out?" Owen said, "no....I teached him how to climb out....see!!!!"

He was sooo proud that he had done this....liberated his brother! I groaned as I new it was the end of what little sleep we do get. Last night Alexander did well, no crying out through the night, but this morning Tim asked me, "did you get Alex up?" I said, "no"....then he asked my Mother (who had spent the night) "did you get Alex up?" Followed by a no. Then Tim asked Owen, "did you help Alex out his his crib this morning?" To which Owen replied, "no....he helped himself!"

Round two of serial casts

We went to see an orthopaedic surgeon yesterday about Kaitlyn's ankle contractures. Not knowing SMA he was pleasantly surprised at what we accomplished with only one casting so far, and even more pleased that we have been able to maintain what we gained for the past two weeks without the use of splints or AFO's. This he found encouraging and has decided to support our request to do serial casting instead of tendon release surgery. Although he did make his big speech about how the casting may have to be repeated and depending on how often, then we may still have to consider surgery at some point to cut the tendons. For now we are happy to avoid anything related to more surgery, more anesthetic (even if it is "only" a local), more scalpels, more stitches, more pain and discomfort!! Enough is enough, and this Kid has been through the wringer enough for the past two years of her life. It's time for a break and to minimize as much as possible everything that we have to do for her to keep her comfortable. It's time she started enjoying some of the life we have worked so hard to give her.

On a lighter note the surgeon said that he thought Kaitlyn's heels were healed enough to recast, so we had round two of serial casts placed yesterday. That's what I love about our children's hospital they will bend over backwards for us. If they can squeeze us in for a casting and save us a trip back in another day then they will! Even when it was a spur of the moment decision. We didn't want to overdo this casting as Kaitlyn had skin breakdown from the last casts. So they decided to take each foot only as far as they could without a lot of force. They still managed to get minus 5 on each foot. When we started with the last casts we had minus 30 one foot and minus 25 the other. So I thought minus 5 after a two week break was pretty good.

Kaitlyn hasn't minded round two of serial casting any more than she minded the first round. This is good, I'm glad that she is not in any discomfort! She still moves her legs about and swings them back and forth in her slings. She can even lift the casts off of the bed to squeeze her legs open and shut. She should not be able to do this without casts let alone with! SHHHH don't tell her!

We decided to leave off the proper casting sock that is usually the first line of defense in any casting. The socks are ribbed and very thin. But we think that the ribbing may have irritated Kaitlyn's skin instead of aiding skin breakdown. It may have increased the friction associated with the normal sheering force inside the cast. This time we went with an extra thick layer of fluffy cotton wrap, followed by a much thinner layer to hold this all together, and then lastly the casting medium itself. Her casts haven't got the nice smooth edge that they had the last time, as they roll the sock back and make a nice edge with a normal casting. But hopefully we have traded our nice smooth edge for not as much or no breakdown!

A pic of Kaitlyn sporting her nice new casts. Boy her legs look really chubby in this photo!

More Screaming and Camera Hogging!!

Kaitlyn loves to scream! She has recently taken it up as a hobby!! She also is a BIG camera hog lately. Notice how she fusses, looks ugly, then brightens when I turn the camera back to her!!! Too funny...too cute! She's one smart, manipulative cookie. Even with her disabilities she still has spunk!!!

Something's A-Foot

Let's start with Sunday. Kaitlyn started her day like any other day. Absolutely nothing was wrong....besides our obvious battle. When I got her up from her afternoon nap I decided to give her a saline neb and some coughs as I hadn't done an afternoon treatment yet. I gave her the neb but I didn't do it in-line with the bi-pap, which is the way that we usually do nebs (however we have done nebs without bi-pap in the past). I was suctioning her frequently as the saline makes her drool and we never want her to choke and aspirate secretions. One time I went to suction her and she got a funny look on her face and started to sort of look like she was choking. I grabbed the neb mask off of her face an started suctioning some more, but her sats were dropping and fast!! Suctioning had NO impact. So I turned on the cough-assist and tried to cough her. As I delivered a breath it was making no impact on her sats and she couldn't produce a cough! I personnally have never had this happen before!! I tried coughing her once more but again no cough. It was like she had no air in her lungs to produce the cough. So then I turned off the exsufflation and just did several breaths of 2 second insufflation (we currently do not have an ambu-bag in the house. Something that I think we may have to remedy!). The whole time her sats were still dropping. She hit 70's....dropped into 60's....and then we had O2 hooked up to cough assist. I kept giving her breaths with the cough-assist for another minute and watched as her sats very slowly creeped up closer to 90's!!! We then put her on Bi-pap with 3 litres of O2 and over the next hour, with a few semi-successful coughs, her sats were back to normal. The really odd thing is that she was pretty dry. We weren't getting much out of her. Very odd!!! There wasn't any junk in her lungs, the only thing that I noticed was very reduced air flow in both lungs.

The really odd thing is that 1 1/2 hours after putting her on Bi-pap she was back to room air laying on the master bed begging to taste some supper!! I'm thinking this is CRAZY! Sunday night she was fine and her sats were normal and her lung sounds were perfect! We figured that she must have plugged (mucus in the lung blocking some of the larger airways and collapsing the lung, or a portion of the lung). We thought that we must have gotten the plug out, even though we didn't suction anything much out. Sunday night she slept through the night. Monday morning she got up like nothing had happened! She did dip a little with the first round of coughs and I did get a fair amount of stuff out of her. Then she did fine through physio and more coughs and stared her day the same as normal. She spent the whole morning out with the boys, off of bi-pap sats 98-100. I was careful to do all her treatments really well to get out any risidual junk. She did have a three hour nap in the afternoon. Something that she hasn't done for a few months now. But other than that she was "normal", no lung sounds, no increased secretions and no decreased air flow that I could detect.

Monday night she went to bed and she was a little junky (wet sounding in the lungs). I had to cough her at 10pm at 1am and again at 6am. After the 6am coughs I didn't hear any more junk!?! It's like it disappeared with the sunshine! I thought that whatever had been in there that I must have gotten out. Then she had her morning breathing treatment at 8am and started her day just as normal as always! Sats 98-100 all day...room air...no Bi-pap! Tonight we put her to bed again sounding junky. Am going to bed soon and will cough her again and then throughout the night as needed. Tomorrow we will keep up with the treatments and hope that whatever she has goes away without a hitch!!! We may end up going for x-rays at the very least before the end of the week. But we will avoid a hospital stay at all costs!!!

Kaitlyn watching TV the other morning. Before serial casting we could never have positioned her feet even close to this. Of course she was rolling her ankles about, so it's hard to position her now until we get her into AFO's!!

Daddy reading books to the boys. It's hard to read to both boys at once without battles over who gets to turn the pages or lift the flaps. Tim was doing a good job at disarming arguments!

Second Casting Appointment

We took Kaitlyn's first set of casts off Wednesday night before her bath. They are the soft form of casts...NOT plaster casts. Hers were made of fibreglass. They are very light-weight. Anyway, we were really hoping that she didn't have any breakdown, but alas! She has pretty severe bruising on both her heels. More on the right side. I'll put some pics at the bottom, but don't look if this sort of thing bothers you!!! Let me tell you that the bruising doesn't bother her at all! I can rub my finger across the breakdown and she doesn't even wince. Let me remind everyone too that Kaitlyn has perfect sensation.

She is such a trooper. She didn't mind having the casts at all. She could still lift her knees to center and squeeze them open and shut. She could still use her slings and is probably what saved her heels from completely breaking through! We have decided not to recast today, but rather wait until next week and recast then, if her heels look better.

So fa the gain from even one casting was HUGE! SOOOO worth having it done. She went from a -25 degrees on the right foot to zero, and she went from -30 degrees on the left foot to a -7!!!! Zero degrees is a 90 degree bend, the negative side of this is when the toes point away from the body. I thought this was pretty good. The other awesome part is that her feet are in better alignment and her toes are no longer curled down and to the side. I’m really happy so far!!!

We should be able to maintain what we've gained with proper physio over the next week, and hopefully we'll be able to recast again next week. If everything goes as well with the second casting then it may only take one more casting. Then we get temporary splints while we have AFO's made. (Ankle foot orthosis).

Kaitlyn's Right Foot






Kaitlyn's Left Foot

Copy Cat!

We have a serious case of the Copy Cats going on here now-a-days!

Sleep

Well we got a lot more sleep last night. No low grade fever, no waking up every hour, no fussy baby....Could it really have been that her feet were too hot!! When I thought about the socks the other morning and I checked her toes they were like little ovens. The casts are extremely well padded adding to the heat. I mean I have heard that some children have difficulty with temperature regulation....but really!! I didn't even have to give pain killers. She just slept through the night. I cannot believe how sensitive she is to just about everything! Not to mention dramatic!!

Needless to say we will be doing everything just the same tonight! Do I dare hope for two good nights in a row!!

No Sleep!

The last two nights have been really rough! Alexander has been calling out several times a night needing someone to come in and tell him there aren't any shadows on his ceiling!! His newest THING! Kaitlyn has been up pretty much on the hour needing to be rolled over, repositioned and nurtured back to sleep. She almost never does this unless she's sick...except this time she isn't sick! She has been running very low grade fevers. I say "very" because her temp. has only been 37.5 degrees Celsius, which isn't very high but it's up for Kaitlyn. HMMMM! Now to try and figure out what's up.

She recently had her first set of serial casts placed. Her lungs are crystal clear, as confirmed by her Chest Physio-therapist (who at our hospital do all respiratory treatments, including cough-assist....not the RT's). She is teething also, which is the most likely culprit, although Kaitlyn doesn't usually run fevers when cutting teeth. Perhaps the combo of both the casting and the teeth? Just keeping a close eye on her for the next few days. The other smaller factor, maybe not so small, is that Tim has been putting socks on her feet over top of the casts. We haven't been doing this through the day, but rather leaving her little toes out to air ;O) It might seem like a small thing to have warm toes, but for a little kid who cannot kick the covers off, and cannot communicate what's wrong...this could very likely be the problem. We'll see how tonight goes!!

Kaitlyn's first set of casts come off tomorrow night at home so that she can get a good full body bath and a bit of a break. Then she will be recasted Thursday. I really hate all these trips into the hospital. At least we don't have to go near the cafeteria, food courts, or anywhere near the floors!

Wish me luck that we can get a little more sleep tonight!

Serial Casts

We decided to do serial casting on Kaitlyn's legs. This was not an easy decision for us. We asked when she was only a year old for AFO's (ankle foot orthosis) to help prevent contractures in her ankles. Contractures are sometimes painful and they don't allow proper alignment of the legs. She cannot wear shoes and would never be able to stand in a stander if her ankles remain contracted.

We were in to see PT a few weeks ago to have Kaitlyn's legs casted so a mold could be built to make Kaitlyn a pair of AFO's. Well they called us back and said that they didn't feel that AFO's were going to do anything for her. They also felt that her feet were too far out of neutral position (the bottoms of her feet were starting to turn up while the toes were pointing down). PT figured that she would never be able to stay in a pair of AFO's. Their suggestion was to put Kaitlyn through another surgery to have the tendons released.

Tim and I had a hard time with this decision for some reason! I think that we were both afraid to have to put her through ANYTHING else too invasive! But in the end we both felt that surgery might be a little in the extreme if serial casting could be tried first. PT is really doubtful that serial casts will give us the stretch that we need, but we just couldn't justify doing surgery before we tried. They have humoured us and the first set of casts were placed last Thursday. She will go in next Thursday to have the next set placed. We know some children with SMA that have had serial casting done in the past and have had good success with it! Sometimes it has to be redone in time as even with regular stretching the tendons can still get tight. This is a risk we would rather take than to have to put that poor girl through any more surgeries or pain!

While serial casting can be painful it hasn't been for Kaitlyn so far. Sometimes she fusses a bit at them, but more like she just wants them off or that she's tired of the new game we're playing! She is still doing all the same things she did before we had them put on. She can still swing her legs around in her slings. We still bath her, but we have to be extra careful not to get the casts wet. We tie her casts together (this makes them too heavy for her to lift or slide) then we place her legs up on a low stool in the tub. She can't make her frog legs anymore and squeeze her legs open and shut and lift her hip off of the bottom of the tub. But at least she can still get a nice warm bath, move her arms a bit and splash! The warm water is so good for her too before bed. it really helps get her lungs nice and clear before going to sleep.

On another note it would seem that taking Kaitlyn off of the Enoxaparin injections for her blood clotting condition has also gotten rid of her increased thickened secretions that she's had all Fall. I read something about Enoxaparin causing hayfever symptoms. This is SOOO nice for us. She doesn't need constant suctioning like she did. She can spit out most of the saliva now. She still requires constant observation and frequent suctioning, but not like before. When we have something to sit her in she should tolerate sitting up for longer periods now. She is back to her old games of bouncing on the edge of the bed on somebody's shoulder (this never gets old!!!), from the edge of the bed we then rock back and fall onto the bed (followed by hahahahaha), blanket pulls through the house (huge grins), and lots of other rough-housing games. She hasn't been able to partake in these activities as she would choke on saliva and we were forced to run for the cough assist, and/or more suctioning. We had to stop playing all her favorite games. The girls a rough-neck, and I have NO doubt would have been a severe tom boy!!!

After rough-housing on the bed. Kaitlyn's cheek is red from being on a shoulder. She gets red easy! I cannot believe how big Kaitlyn is getting!! It seems like she should look a lot smaller next to her big brother!

Enjoy this pic!!!! I don't often get all three still at once. This took a bit of doing to keep Alexander in one place long enough to capture a pic!

Turns out he just wanted to kiss his Sissy!! AWE! The boys can't go anywhere near her without stopping for a quick snuggle or mooch!

Sling Play with Serial Casts

Kaitlyn with her first set of serial casts. They aren't holding her back! She's still enjoying her slings, watching Dora, playing with switch operated toys and interacting with her brothers!

Owen Playing Tickle Game with Kaitlyn

Kaitlyn Laughing

Powder Fun

My boys were having some fun with powder the other day. I just had to post this. Thought everyone could use a good laugh! I didn't scold them as there were two adults in the house and they were not being watched. The picture quality is perfect, by the way. There really was powder from floor to ceiling and wall to wall!!

New Year!

Well this year started out much better than last.

Lets talk about last year. Last year we spent the New Year in hospital. Kaitlyn was having some serious digestive issues. Last January Kaitlyn had a G-J tube. This was a fantastic option for us. However, last January when I would begin her J-tube feeds after a brief fast about twenty minutes later it would start pouring out her G-tube. I can't even begin to tell you how wrong this is, thins should never move backwards through the gut! After a couple of hospital stays for digestive issues, we decided to switch her formula. We put Kaitlyn on an Amino Acid diet, Pediatric Vivonex. This has proven to be one of the most significant things we have done in caring for our daughter.

In May Kaitlyn had been having some issues with increased secretions, over and above her normal range. We put her on an antibiotic when we noticed a slight change in the color of those secretions. We were hoping to avoid any bacterial infection, secondary to whatever it was she had virally. About a week later she still had the secretions and the color was getting more and more green. Then we had a cold go through the house and one day Kaitlyn was fine and the next she was very ill. We ended up in PICU with a very sick little girl. She had caught our cold and we then found out that she also had a bad case of pseudomonas!! Pseudomonas is a particularly nasty bacterium that loves living in the lungs, but can also colonize other areas of the body as well. Kaitlyn had pseudomonas in her lungs, urine, and her g-tube stoma.

She narrowly avoided intubation, but we were able to avoid it by following Dr. Bach's NIV Protocols. We were also able to show our hospital how this protocol works. A very nice feeling to say told you so to the experts! They needed to see this protocol in action! We also taught them a thing or two about using the cough-assist, including bleeding O2 through the cough-assist while coughing to reduce stress on the patient. Another patient (paralysis) in the PICU benefited from this knowledge while we were there! Some hefty antibiotics and two weeks later we went home.

At the end of July Kaitlyn's J-tube blocked. We had to go to emergency to try to get Kaitlyn's J-tube replaced. This is supposed to be a simple take out the old tube and insert a new. It needs to be done in radiography under fluoroscopy and takes about twenty minutes! Well we got to the hospital around 1:30pm and they managed to delay EVERYTHING! It is part of Kaitlyn's emergency protocol at the hospital that they are not to withhold nutrition!!! They couldn't place the J-tube that day (still not sure about that one!). The general surgeon placing the central line couldn't get the femoral vein in Kaitlyn's right leg. They had to call an intensivist down to place a central line in her left groin. By the time that this was all done it was too late to order TPN....GRRRR! That's why we said order it when we first arrived! We had no way to feed our daughter! She would have to fast until TPN could be ordered up the next day. They promised to have a J-tube into her before she would need the TPN. We said order it ANYWAY!

That night Kaitlyn's right leg got really cold, and there were no pulses. Her nurse didn't notice, I DID! They scheduled us for early morning ultrasound, before the J-tube placement so that once the J-tube was placed we could go home! We found out the next morning that Kaitlyn had pretty severe blood clotting in her right leg. Both veinous and arterial clots. There were extensive collaterals formed, so this clotting was believed to be from the central line needed back in May. We then went to Radiography for the new J-tube.

In placing the new tube the radiographer lacerated and perforated Kaitlyn's bowel. He somehow managed to make a gash in her intestine and then poke the j-tube out through the intestine wall!! Kaitlyn had to go for immediate emergency surgery. I literally had only a few minutes to process all of this and make some pretty important decisions for my daughter's well being. Our general surgeon came up to see Kaitlyn and I before surgery. He explained what had happened and what he was going to have to do to repair the damage done. He then told me that we were going to have to consider another way to feed our daughter!!! This I was not expecting! He said that once the bowel has had this type of extensive damage that we would never want to push a tube down through the bowel ever again. We had two options; place a fixed J-tube or perform a nissen/fundiplocation.

The first thing I did was call Tim. He was at home taking care of the boys. I had to tell him over the phone what had happened. He thought that it would be several hours before the surgery would be ready to take her. I told him that they were taking her in twenty minutes and that we needed to make a decision NOW! What a thing to have to tell him when he's at home, no way to get in to see Kaitlyn, and no time to think on it! Thankfully I like research and had read quite a bit about both procedures (just in case- always trying to think ahead). I knew the pro's and con's about both procedures. I told Tim what I thought and what our surgeon had said in answer to some of my questions about both and we decided to go with the nissen/fundiplocation. The surgeries were to be attempted laporiscopically so as to avoid unnecessary pain and healing time!

Seven hours later our daughter pulled through. 36 hours later she was off the vent. In a few days she was back home and we are so glad to have her nissen. This is NEVER the way that I would have chosen to have had it done, but something I wish we had had done LONG ago! She has been so much healthier since the nissen. We discovered another clot, this time in Kaitlyn's left leg, as a result of the central line from this stay. We decided to treat the clots. She had to go home with twice a day subcutaneous injections, which we were able to stop early December.

This fall and winter so far have been really good. Other than head colds that keep going through the house. We have had two aspirations since September. These aspirations were due to secretions and happened at night while we were trying to get some sleep. But we have managed everything at home.

We are hopeful that the year 2009 will be a much better year for us. We are much better prepared to care for Kaitlyn at home this year and have learned so much from 2008! We also have much more confidence in our hospital to be able to help us should we need them. They have also come a long way. There is new research and hopeful trials being started this year. The most hopeful thing by far is the Stem Cell research ongoing.

We are thinking of all the sick children this New Year and hope that they make it home with their families very soon.