Ear Pillows

About a year ago now Kaitlyn started having continuous problems with breakdown on both ears from having to constantly lay on them. Even off-loading by rolling her from side to side didn't work. We would get raw skin that would lead to infections even though we kept the skin as clean and dry as humanly possible! At one point our infections were so bad we had to see plastic surgery as we thought that the infection might be in the cartilage of the ear!!

When our pediatrician told us how serious the infection could be if it were in the cartilage of the ear we knew we had to find a way to get Kaitlyn's ears up off of the surface she was laying on! I thought that if Kaitlyn were to lay on a pillow with a hole in the center then she could still be sidelying while having her ear completely suspended (zero pressure)! (Sidelying for a child with SMA type 1 is essential as they cannot swallow. It allows them to pool secretions in their cheek and even spit out secretions so that they do not aspirate.)

I like to use flannel fabrics when making my pillows. I find flannel absorbs moisture quickly and washes really well. The flannel is also soft and I find it irritates Kaitlyn's skin less. I started with a circle of fabric slightly larger than Kaitlyn's head. For us at that time a dinner plate worked great as a template for marking my fabric. Any circle shape the right size will work to make a pattern for cutting out your fabric. When cutting out my circle I always fold my fabric in half with the right side in. This way I cut out both sides of the pillow at the same time. I then take these two circles with the right side still in, and I sew around the outside with a sewing machine.

I then fold the circle shape in half and cut out a small semi-circle in the middle. The hole in the center of the pillow does not need to be any larger than your child's ear! The smaller the hole the easier it will be to position your child on the pillow when it is finished. I always find that if I cut out the hole to fit Kaitlyn's ear then the finished product will be slightly larger than her ear.

I then turn the fabric right side out and begin sewing up the ear hole. For Kaitlyn's pillows I like to turn the fabric in and sew a nice smooth edge (seamless). This way there is no edging to rub against her ears. Once I have the hole about two-thirds closed I then stuff the pillow with a hypoallergenic polyfoam stuffing. I like to use a proper pillow stuffing as it holds it's shape quite nicely through continuous washings! Once the pillow is stuffed full I sew up the rest of the ear hole. I stuff my pillows full but not too firm!

I have had several families ask me recently how I make Kaitlyn's ear pillows so I thought that I should just post how I make them :) I hope this helps any families interested in making their own pillows! I know for us Kaitlyn is on an ear pillow 24/7 now!!

PICU and Another Lengthy Hospital Stay

Remember when Kaitlyn was sick with a pnuemonia back in September?? Well it would seem that we never really ever got that cleared up!!

Kaitlyn was on ciprofloxacin (broad spectrum antibiotic) back in September for an illness that seemed bacterial in nature. We know Kaitlyn colonizes pseudomonas in her lungs so cipro is always our drug of choice when she gets an infection. Well back in September cipro didn't work to get rid of the infection. Kaitlyn ended up hospitalised on IV antibiotics. The IV antibiotics were given for two weeks (one week at home) and then another eight days on two broad spectrum oral antibiotics!

After about a week Kaitlyn started with increased secretions again. So we put her back on oral ciprofloxacin. She seemed to respond nicely to the antibiotic this time and we breathed a sigh of relief! However we did run cultures also and the cultures grew heavy growth psuedomonas and heavy growth Staph. Aureus among other bugs. However since Kaitlyn seemed to be getting better we assumed that the buggies weren't infectious but rather just colonized in the lung :(

We finished a ten day course of antibiotic at home. After which Kaitlyn was healthy for two whole days! She went from healthy and happy sitting up and acting normal to wet and junky lungs in twelve hours!! We chose not to expose Kaitlyn to all the sick kids flooding the ER late at night. I stayed up and did therapies every three hours all night last Monday night. In the morning we took Kaitlyn to ER. It would seem that she didn't appreciate being moved! She crashed down into the seventies a couple of times in ER and was having a rough time coming back up! We increased her vent settings and bled some O2 through her bi-pap (it took both to bring her up to 90% O2). Typically we don't like the O2 through the bi-pap, but Kaitlyn's PCO2 level was fine and our goal was to discontinue the O2 as soon as possible!! They accessed Kaitlyn's port and began IV antibiotics right away. I'm glad that they did. She was running a high fever and we believed that she probably had something bacterial as nobody else in our house was sick.

Because Kaitlyn was a little unstable she made the nurses and ER doc a little nervous I think. They decided to send her to PICU. We have not been to PICU for an illness in over a year and a half. As it turns out PICU was an absolute nightmare!!! H1N1 EVERYWHERE!! And me without my vaccine yet...EEEPPPPS! Needless to say I was washing my hands like crazy! As luck would have it, Kaitlyn responded well to treatments and we got rid of the O2 through the bi-pap the next day. We did cough her with O2 for a day longer. X-rays showed a pneumonia in Kaitlyn's upper right lung. She also had some infiltrates throughout the lungs (areas looking mucousy/wet).

After spending one night in PICU we were able to move Kaitlyn to regular floor. This way she could be more closely followed by doctors and specialists that know our daughter best! Also Kaitlyn's pediatrician works in our children's hospital. This is great for us, as any time we are hospitalised we are admitted under our pediatrician's name :) Not a soul knows Kaitlyn like her pediatrician, and while she is not an SMA doctor we love her as she is willing to work with us and listen to our input!! As you know this is huge for our SMA families....just having someone who will listen to and try some of our unconventional ideas :P

Since last Tuesday we have been waiting for Kaitlyn to show some signs of improvement! Her fever is gone, she is not needing O2 in any form and her bi-pap settings are back to normal. But...she is still junky! We took an x-ray yesterday that showed the pneumonia in the upper right lobe has cleared (there's no consolidation anymore). But there are infiltrates throughout the lungs and appears in particular to have spread into the lower right lung! URG!!! In a consult with Infectious Disease yesterday, they confirmed that Kaitlyn is on the right antibiotics to cover the bugs she has...so why is she still sick?? Today we have to redo cultures and they also want to redo her H1N1 test and add some plasma tests (?). You know I almost hope that they find something viral!!! Crazy how we used to wish for Kaitlyn NOT to get a virus! Throw in a couple of super bugs and all of a sudden we are praying for the viral CRAP!!

Hoping my next post is from home...

New Ramp!!

We bought our new home and moved in back in August. The first thing we knew that we had to somehow make happen was wheelchair accessibility for our new abode. For three months now Kaitlyn's Wheelchair/medical stroller has had to live outside in our van :( Banished alongside the stroller was our back-up cough-assist and true sine inverter (needed to run said device). This equipment is very expensive and difficult to procure. With snow and cold weather threatening to arrive we have been worried about the equipment being stored in our van.

Tim spoke to our Social Worker at our children's hospital about needing a ramp and asked if we could get any help building. Our social worker wanted to know if we needed help building or funding supplies. We of course said that we would be happy with whatever help we could get! Tim and I were hoping for some guidance and expertise in how to build an accessible ramp. A few short weeks later our social worker told us that we would have a couple of contractors come out to assess our needs for a ramp. We had high hopes that the pair would give us some expert advice on how/where to build our new addition.

Then two weeks ago we had a call from the contractor saying that we would be receiving supplies to build a ramp and they would be out shortly to begin the build. Before the call we had no clue that any arrangements had been made for a ramp to be built!! We knew that our social worker had been looking into getting some funding for a ramp, but we had no idea that the funding had been found!

Our supplies arrived a couple of days later and the contractors the day after! They were wonderful! They were even kind and patient with our boys, who just HAD to be out there observing their every move :) The contractors were here for three days and finished our new ramp :)

It is so hard for us to put into words how we feel about having this ramp built for us! Unless you have a child like Kaitlyn and truly understand ALL that she entails each and every day, then it would be hard for you to realize just how much a simple ramp can do to make our lives that much easier! Kaitlyn has frequent appointments at our local children's hospital, not to mention the very few family outings we get together. Taking her out of the house is a huge undertaking!

Before the ramp we had to carry supplies out to our van and load up Kaitlyn's stroller piece by piece! This took several trips back and forth to the house lugging everything out by hand. When Kaitlyn travels out of the house we have to take ALL of her equipment with us. She is a vent dependant child with an extremely unstable airway! The last step was to carry Kaitlyn out in my arms, climb into our non-accessible mini-van, work my way around her stroller and get her settled without breaking any of her limbs! Kaitlyn is a big girl and carrying her in tight spaces is becoming increasingly difficult!!

With the ramp we can load up Kaitlyn's stroller, supplies, machinery and angel baby :) Then all we have to do is stroll her out, down the ramp and into the van! This is just so much easier. Especially in the bad weather!! It is also so much safer both for Kaitlyn's equipment and for Kaitlyn :) With the ramp, an outing of a few minutes can be done without a second thought, when without the ramp it wasn't even considered. This is a huge plus for a little girl whose first words in the morning are "go, go, go"!

Supplies for our new ramp :)

Supplies unloaded....ready for the contractors!!

First snowfall of the winter season covering what was accomplished Day 1 of the build.

Our finished ramp!!! :O)

Some recent pics of the kids:

Feeling Better Again

Kaitlyn has been feeling much better the past two days! We are back to normal bi-pap times and secretions are much more manageable. I can finally carry her around on my shoulder again and boy is she taking advantage!!!

Kaitlyn goes to get her TLSO tomorrow. A TLSO is a body brace (like a rigid corset) that hugs Kaitlyn's torso so that she will be able to sit up without slumping all over!! It should help make it easier for Kaitlyn to breathe and make positioning her easier. It will help us keep Kaitlyn's back straight to avoid or prolong any surgeries to correct displacement in the spine! Currently Kaitlyn has NO noticeable curvature of her spine :) Our OT/PT is finally convinced that she needs the additional support when they tried her in the new floor sitter they are having made for her! She just collapsed into the seating...they have never dealt with a child with such low tone before! We are all learning as we go.

We are also trialing a couple of options for drive controls for a powerchair tomorrow. One is the mini proportional joystick. Kaitlyn hasn't done extremely well with this joystick :( They have left it so long that now she has lost almost all function in her little hands :((( The second option we are trialling is fibre optics :) The fibre optics are little pinpoint sensors that can be placed anywhere that Kaitlyn has predictable movement. The biggest problem with driving a powerchair like this is that it could take a LOT longer to learn.

The biggest problem by far is not having a proper chair for Kaitlyn to trial this sensitive equipment. They are having a really hard time figuring out how to position Kaitlyn's hands to give her the best function to be able to access the drive controls! Wish us luck that we can figure this all out. I am getting really tired of listening to Kaitlyn cry everytime Alex or Owen gets up and leaves the room and she cannot follow!

Increased secretions, slow digestion....You know the drill!

I was hoping our next post would be something exciting, something nice....something "normal"!! Kaitlyn has had increased secretions for the past week and a half almost two weeks. It started out like a head cold. Increased secretions were almost entirely from the nose. She has had no fever and has not been bi-pap dependant. Her spirits have been good and she has been acting normal, just extra junky.

Well yesterday it finally happened, she stopped digesting her feeds! Friday Kaitlyn was fine, digesting fine and acting fine except for the increased secretions. Then yesterday morning I gave Kaitlyn her first morning bolus and no issues. Then I fed her again and when I went to feed her a third time she still had all of her last feed in her stomach! I knew right away what was happening! Everytime Kaitlyn gets sick now it stops or severely slows her digestion. It seems as time goes on that it is taking less and less provocation for her tummy to shut down :(

The first thing I always do when digestion slows is I take her formula back to a base mix. Kaitlyn's normal formula contains fruit juice, fruits, supplements and her powdered formula. When she is sick and is digesting poorly we take out all of her fruit juice and fruits. We then add extra pedialyte and this usually helps her food move through her system better. So yesterday I remixed Kaitlyn's food and fed that to her. She still wasn't digesting!!! After we put Kaitlyn to bed Tim went out to get more pedialyte and we gave her straight pedialyte.....finally!! The pedialyte started moving through her. At this point she was pretty dry.

We then gave her pedialyte and mixed in some of her bland formula. This seemed to work! Hopefully we can keep her gut working in the right direction. Thanks to the suggestions from some of the other SMA families I have a couple of things I can try if she stops digesting again!

Her cold does seem to be getting a little worse, but she is not presenting any differently than the rest of us. We'll see how she does over the next day and decide whether or not to take her and have some cultures done. She is nowhere's near needing to be admitted unless she needs fluids!

Home Again

Sorry for the long time between posts!! Kaitlyn got home last Friday the 18th of September. So what happened.....

Kaitlyn's sputum cultures (mucus from her lungs) came back from the lab positive with both Pseudomonas and Stapholococcus Aureus bacterias! Both of these buggies are serious on their own. Not to mention hard to treat. Kaitlyn colonizes pseudomonas. This is not something that anyone with a normal respiratory system would be susceptible to growing in the lungs. Kaitlyn grows two strains that are both now resistant to the oral antibiotic that we usually use to treat pseudo at home. This medcation is really the only type of oral antibiotic effective against pseudo!!! GREAT! The Staph buggy also turned out to be a fairly resistant strain. It was the Staph that Infectious Disease figures was the cause of Kaitlyn's illness this time around. I think this is the longest illness we have dealt with so far!

In hospital Kaitlyn was placed on two IV antibiotics to get rid of the buggies. After about two days I started asking about getting things in place so that we could take Kaitlyn home and administer the IV meds at home. At first they were telling me that there was no way that we could go home with IV antibiotics....I raised my eyebows and said that I knew for a fact that people DID go home on IV meds and fluids and even TPN when needed!!!! I was only asking for the same rights!

When we are in hospital we do ALL of Kaitlyn's care. The nurses are not familiar with Kaitlyn's equipment, including her cough-assist, and especially her bi-pap. These things are not extremely complicated peices of equipment, but we would have to teach their use twice a day with shift changes and then it takes TIME to get familiar with Kaitlyn specific techniques. We usually spend a good two weeks to train our home nurses to care for Kaitlyn when she is healthy!! It's a whole new ball game when Kaitlyn is sick!!! The "rules" change...she becomes a LOT trickier to deal with. That said...we find it soooo much easier to be at home when Kaitlyn is sick but relatively stable!!! It disrupts our whole family when we have to go to hospital :o(

In the end they agreed to let us home on IV antibiotics as long as our regular nursing service could arrange to deliver the meds. I did learn how to administer the meds myself, but did not take it upon myself as they took away all of our regular service. Tim and I had been working around the clock for three weeks at this point and the last thing I needed was more work!!!

IV antibiotics were finished this Wednesday and Kaitlyn has been switched to two oral antibiotics. One covers her pseudomonas somewhat and the other is knocking the stuffing out of the Staph buggy :o) Our goal in leaving her on antibiotics (hefty antibiotics) is to completely knock out the Staph buggy!!!! The last thing we need is for her to colonize such a NASTY resistant bug!!! ID (infectious disease) had a hard time finding antibiotics that would cover all of the bacteria that Kaitlyn was growing in her lungs. She was even resistant to the newer generation antibiotics that they wanted to put her on!!

So now we are trying to get back into our "normal" routine. It's tough for all involved, especially since Tim and I are still trying to catch up on lost sleep! We are just so glad to be back home! Here are some recent pictures of the kids.

Kaitlyn is REALLY enjoying these kittens! She loves it when they come lay down with her. This little black and white kitty LOVES laying beside her and doesn't even budge or wake up when we turn the suction machine on!!!

Alexander loves the kitty too! This little kitten doesn't mind when they pick him up and cuddle him....he doesn't have enough sense to run :o) But seriously the kids are soooo good to him.

My boys outside in their PJ's playing in our yard one weekend. Tim and I didn't have time to fight with them about getting dressed....so out they went. They'd live in PJ's if we let them ;)

I love my diapers!! This is how I found Alex one morning, laying on top of Kaitlyn's diapers. He looked like he was sad that he no longer needs them :o)

This is Owen on his first day of school.

In Hospital

Where to begin....

Kaitlyn had her Port-a-cath surgery August 27th and like I said everything went just fine! She came home and had a slight increase in secretions which is normal after being intubated. After about 3-4 days she is usually back to her baseline. By the Monday (four days) post surgery Kaitlyn's secretions were on the rise and not getting any better. She also started with a bit of a cough on Monday. By Tuesday she was bi-pap dependant, but not because she was desatting. She would go into fits of continuous coughing every time we took her off of bi-pap. Secretions were still on the increase and we thought that perhaps she was getting a cold. By Wednesday post surgery Kaitlyn spiked a fever of forty. Considering she just had her port placed we worried about infection in her blood so we brought Kaitlyn into ER to have them run some cultures and figure out what was wrong.

The cultures from ER all came back negative and in the meantime we had taken Kaitlyn back home to care for her. So from Wednesday until the following Monday (1 week since onset of symptoms) she still had fever that would spike to forty every time we took her Tylenol away and she was still obviously junky and sick! On Monday (Labour Day) we took Kaitlyn into ER again as she obviously wasn't getting any better. She had even come down with a new issue of not digesting her feeds very well. Kaitlyn was started on Ciprofloxacin as it is the antibiotic of choice due to her colonization of a particularly nasty bacteria pseudomonas.

After starting an antibiotic Kaitlyn usually responds very well to treatment. Within a couple of days we can witness a remarkable change in her symptoms. This time after five days of antibiotic treatment in our home she still wasn't getting better. She was only digesting about 1/3 her normal volume of fluids. We also had to take Kaitlyn's normal formula down to a base mix, which means that she was getting a lot fewer calories and less of all other nutrients. We did this as Kaitlyn won't digest her feeds otherwise. So we brought Kaitlyn back into ER yesterday (Friday) as we really felt that she needed IV antibiotics and IV fluids.

Our stay in ER was really ridiculous! They kept us there for a total of six hours! SO much for her protocol!!! Five hours after waiting around ER the attending pediatrician from the floor told us that they would admit us for the night, but that they didn't want to change any of her meds. and they just wanted to observe her through the night!!! I told the physician that we've BEEN observing her or the past five days at home and she is not getting any better. I said that if I wanted to observe her for one more night then I would have kept her at home!!! There was a resident shadowing the physician and I thought his eyes were going to pop out....hehehehe!! Long story short they called Kaitlyn's pediatrician who is in charge of one of the teams at our hospital and whom Kaitlyn is always admitted under!! She told them to start the IV meds. but left the fluids up to them. So IV meds were started Friday night and fluids were not.

This morning (Saturday) Kaitlyn is about the same. Because they didn't start the fluids like we had wanted she was pretty dry...so she sounded great when the nurses listened to her. This as we know can be dangerous as if she gets too dry we may not be able to mobilise the junk in her chest to be able to get it out! When Kaitlyn's pediatrician came in to see her this morning she wanted her started on IV fluids. They don't want to give her much, just enough to keep her better hydrated so her secretions won't be so thick!

Overall I think that we needed to be in hospital and now that we are here she will be followed properly so that we can figure out how to keep her from getting sick because of pseudomonas. We may have to start some sort of preventative regimen in order to keep her healthy! Oral cipro alone doesn't seem to be working for her anymore. I'm glad that she will be followed by Infectious Disease from now on...we have been asking for this for some time now! Infectious Disease wants to leave her on IV cipro until they can figure out exactly what it is Kaitlyn seems to be culturing and then hopefully we can check for sensitivities and get her on one or more antibiotics that will knock those buggies out of her!!! Infectious Disease also wants to run a host of other tests (not the standard run of the mill). I am much happier today knowing that they are finally going to DO something to help.

I have observed today that Kaitlyn's tidal volumes are pretty low (60-80) depending on her leak!! That's pretty low for a child her weight. I have also noticed that she is paradoxical breathing while on her vent, and this shouldn't be the case. So far her blood gases are good, but we may have to increase her pressure settings to make her more comfortable so that she isn't having to fight so hard to breathe!!! No wonder she has been sleeping almost 24/7 at home! I don't know how long she has been breathing this way either...I hope not long. I always wonder how much I fail to pick up on when we are home...as I have more responsibilities at home and more distractions. When we come into hospital Kaitlyn is my number one concern as I know my boys are being WELL cared for (Thanks to Grammy and Grampy!!!).

I'll keep everyone posted on how we make out! Right now we are where we need to be!!