Saturday, January 24, 2009

Weekends at KKI

This was a few days ago. You can see how swollen Kenzie's eyes were here. Today I didnt take a picture but it was much worse.

Weekends here are pretty dead. Kenzie gets her 3 feeding sessions but no other therapies. Feeding is not going well and Jordan and I are nervous that maybe shes not old enough to understand the concepts needed to progress. They have divided her feeding sessions in 2. Each sub session are 5 bites. We do 1 sub-session with food on the Nuk and 1 with food on the spoon. She does ok with the Nuk because it pushes the food back on Kenzies tongue and sort of forces her to swallow. She is not doing well at all with the spoon. We don't know if she doesn't understand what to do with the carrots when they are placed with the spoon or she just doesn't want to. It's pretty frustrating because we feel like we are at a standstill.

Medically Kenzie is getting more and more swollen every morning. We met with Kenzies interventional radiologist yesterday (he used to be at Columbia and is now at University of Maryland). He took a look at a picture of her in the morning and agreed she is symptomatic. We are hoping there and no clots and don't expect any. He said his fear always was that the stents would remain open but that where the vein and the start of the stents meet there might be a narrowing. Since this vein is the main vein that leads to Kenzies heart its a bit worrisome. This coming Wednesday morning Dr. Haskal will enter Kenzie through her femoral vein (which is in her groin area), move all the way up part her heart, through the stents, and up her neck. If there is narrowing of the veins he will take a balloon and stretch them. If there are clots (which we really hope not) he will get those too. He uses the analogy of a plumber who needs to go in and clean out her drains. We forgot to ask the real big questions "how often will this narrowing occur?" and "will she need this procedure done everytime?" My guess is that since he had never done this on a baby before... he will say he doesnt know. At the same time Kenzie will either get a "J" tube or a "GJ" tube put in. We are still sort of in discussions with which we will go for. The "J" tube sends all of her formula past her stomach and straight into her small intestines. It should look on the outside much like the button she has now. The "GJ" tube gives us options of where we want to send her formula. Sometimes we can put it into her stomach, sometimes her intestines... The problem with it is that its a bit harder to manage and is much bigger than the button she currently has. His anesthesia team will try and do all of this under a heavy sedation but we are assuming at the end of the day they will wind up intubating and putting her fully under. Hopefully they'll also be able to get all the blood we need at the same time.

So far we've had a great weekend with Uncle Justin, Uncle Jon, Aunt Courtney, Baby BOY Urfrig and daddy. Next Uncle Cousin Markie Boy is coming so this week is going to be so fun! Sorry about all the typos in my posts but I really just don't have the time or energy to proof read before I post.

Friday, January 23, 2009


FINALLY! We haven't had Internet access in 2 days here and anyone who knows me knows that's like total torture! Since I last posted we have had a lot going on.

Feeding therapy has gone better. They introduced pears and I guess Kenzie doesn't like them. She had 2 not so great meals Wed afternoon with them and then yesterday morning Kenzie was over it. She decided she wasn't letting those pears in her little mouth. So yesterday at breakfast she had what I would consider a standoff with her feeder. They put the pears up to her mouth and she wouldn't open. Kenzie sat for a full 30 minutes (the length of her session) with her mouth closed to the pears. KKI does not force feed so they will wait a kid out and usually the kids generally get bored and give in. Kenzie has a stubborn nature I guess and literally ignored the nuk. When she moved her head the nuk with the pears followed but she literally didn't look the feeder or the pears in the eyes. She did however get a laugh out of me when 24 minutes in she decided to start waving to the data collector in the room. It was really funny but because she was not doing what she was supposed to, the collector had to ignore her. Kenzie didnt care though and went on waving. 30 minutes passed and Kenzie won the battle. That's a really long time for a 14 month old to sit and do anything for so its amazing she won. Since then the team switched back to carrots and shes not doing great but at least is taking some bites. They will reintroduce pears soon and Kenzie will have to learn to like them.

After breakfast we had our steering meeting. This is a meeting with Kenzies whole team (medical and therapy). They access Kenzies progress and discuss where they think they can take her over the next 6 weeks. As for kenzies weight they told us that a normal baby of her age gains about 9 grams a day. kenzie has been averaging 12 so shes doing terrific. The allergist here wants to treat Kenzie like a new baby and stick mostly to introducing yellow veggies, rice, and fruits until we know what she can digest. In therapy kenzie is known as a "passive refuser". What that means is that she does not hit the feeder, or throw her food off the table, bite, etc. Kenzie takes a different approach that is more manipulative. They told us that Kenzie is "learning their game and testing them at every step" Apparently my child is advanced and using manipulative measures they say most 2 and 3 year old demonstrates. Lovely right?? I guess I have a lot to look forward to when shes a teenager. They did mention though that the team loves a challenge and would rather this. Their goal for Kenzie is for her to leave here taking 25% of her needs by mouth. It is not what we were hoping for. I understand that they can't get high calorie foods into her and therefore are limited by how many calories they can give her by mouth but at the same time, its just not what we wanted to hear. Her OT and ST therapy sessions are going to work hard at getting Kenzie to take drinks by mouth and start to learn how to use her muscles to chew.

Medically, Kenzie is not doing great. She has been very swollen again. It is getting to the point where we are pretty concerned. Luckily, today is our appt at University of Maryland with the doctor that put in Kenzie's stents. We are assuming he is going to want to do a venagram(sp??) to study her vessels and make sure there are no other blood clots. I guess I will have more to report on that tomorrow. Also, Kenzies labs came back from the other day and her IGG level is really low again. I think we are going to tell the doctors here that regardless of whether this immunization they gave her 2 nights ago will work, we want her back on her IGG infusions. We can't afford for Kenzie to get really sick while we are here as it would set her progress back majorly. The immunologist here is wondering why she is suddenly spilling out all of her antibodies again but realistically no one has ever been able to figure that out (along with all her GI problems) and we can't afford to take risks.

I don't think I mentioned that earlier this week we got moved out of isolation and into another room! We decided not to go back into the 3 person room. Although that room is so big and has lots of room for Kenzie to play (not to mention its own bath), it is very noisy at night. One of the children cries a lot and it keeps kenzie and me up a lot. We felt it was better to have a smaller room with someone who keeps the sort of hours we do. Luckily for us a new boy started the program last week and he sleeps 12 hours every night! So, for the last few days it has really been an ideal sleeping situation. Last night I think Kenzies swelling was making her uncomfortable so she woke up a few times but for the most part this room is totally quiet from 830pm - 7am.

The program is really picking up and another little girl "L" started 2 days ago. "L" loves! Kenzie. Kenzie is totally enamored by her and wants to do whatever shes doing even though "L" is almost 4 years old. It's really so cute! In about an hour Jordan will be here for a whole week which I am so happy about. Its been a really rough week not to have him here with me. Also, tonight Aunt Courtney, Uncle Jon, and baby Urfrig #2 are coming to visit! We can't wait to see everyone and hope that we have a fun weekend. Ill hopefully get time to post tonight or tomorrow after we go see Dr. Haskal.

Hope you all have a great weekend and keep the emails coming! Even if i don't get a chance to answer them quickly I do read them and will respond. This lack of Internet thing killed me!

PS - Jordan is bringing a card reader today so Ill finally be able to upload pictures again and make these posts a little less boring!

Wednesday, January 21, 2009

Its been better... Its been worse

Yesterday Kenzie progressed in feeding with the volume they were giving to her. Instead of just dipping the Nuk brush in the carrots they now take a spoon, put the food on the spoon, level the spoon off and then take the food off the spoon with the nuk and give her that amount in her mouth using the nuk. They say that using the nuk helps take some work off kenzies plate. The nuk being pushed down on kenzies tongue helps facilitate her swallow (and maybe a gag sometimes too the way I see it). Kenzie is doing pretty well with the greater volume. She is having a little more vomiting but she isn’t refusing and is still enjoying her mealtimes. Today they changed things up even a bit more by giving kenzie pears. Previously we had only worked with carrots. Kenzie definitely made a face (almost like a sour face) everytime the pears touched her lips and had a few more spitups than normally but she didn’t refuse anything and did pretty well with them.

Medically Kenzie needs blood drawn for a variety of tests. For anyone who has followed Kenzie through her life knows that is a constant struggle. For some reason Kenzies veins are so easy to see but virtually impossible to get blood out of. They need like 17mls which is a little over half an ounce. Usually we struggle to get 2 mls (just to put it in perspective). We need the blood for immunology (Kenzie gets infusions of immunoglobins once a week because she has a weakened immune system), regular labs to test her protein level, and a few mls to test for a rare genetic disorder that a doctor in san diego just discovered. Yesterday a nurse who was supposedly a good stick tried for 40 minutes, finally got 1.5 ml and then it clotted (which means we couldn’t use it). Today we sat for another 30 minutes sticking her again and got about 4 which takes care of the immunological stuff but nothing else. I’m sure I don’t have to explain to you how Kenzie is when they take blood but shes done it so much that all she has to do is see the torniquit and she starts screaming. The screaming then turns to screaming and vomiting which then gets her all worked up and nothing we do can stop it till they are done. Its really sad. Now we have to make a decision on how we are going to get the rest. I am opting for an arterial stick (which means they go directly into the artery instead of a vein). The blood will come out much faster and we should be able to get what we need. The downside is that artieral sticks are painful. Personally I think some pain is better than the psychological torture of spending a half hour 3 days in a row being held down and stuck over and over while the blood barely comes out. I guess the final decision will be made later. To top the day off, Kenzie will be getting a Pneumococcal Vaccine. Some of you might ask us “but I thought kenzie cant get any vaccines while she gets IGG infusions I thought?” Well youre right. We are stopping her infusions for 4 weeks (which makes me VERY nervous) so she can get this one vaccine. They feel that her chances of getting pneumonia are greater than the risks of not getting her infusions for 4 weeks. We spoke to her immunologist in NY to make sure he was comfortable with this decision before we went along. So even though for 6 months we gave kenzie shots twice a day… I still hate it for her and feel badly that she doesn’t know this is coming later and that tomorrow shell probably get the arterial stick.

To complicate matters even worse, the GI doctor here just came and spoke with me about switching Kenzies GTube (the tube in her belly) to a GJ Tube. The GJ tube has 1 portion that goes directly into her stomach (the G part) and 1 portion that leads the food straight to the small intestine (the J portion). When food is delivered into the J portion, or the small intestine it makes it very difficult, if not impossible to vomit. They feel that she vomits too easily both behaviorally and medically and that eliminating the possibility of vomiting will make her progress in the feeding program much more significant. GJ Tubes are something Jordan and I have discussed multiple times over the last year. It has pros and cons. The pro of course is really only 1 thing: no vomiting. The cons are: It is a surgery to insert and will require intubation and general anesthesia. It sometimes breaks or dislodges and then requires another surgery. You cannot bolus feed through a J tube, which means she will have to remain on continuous feedings when fed through that tube. Continuous feeds are a problem because Kenzie now moves all over and I need to follow her everywhere with either her backpack or IV pole since she can’t do it herself. The J tube also requires a lot of maintenance. It needs to be flushed every 2 – 3 hours round the clock. That’s not so bad while we are here and have nurses that can help, but for me at home it just makes things way more difficult once again. So… Jordan will talk to the GI hopefully tonight and the two of us will have to make a decision.

Speech and Occupational Therapies went a bit better today but still she is not responding well with them. She tends to cry a lot. Without getting through to Kenzie during these, we will not be able to ever progress her food texture or get her to drink. I have tried to talk to her team about switching the locations of the therapies to her feeding room (she seems to like the feeding room a lot), or putting her in the same booster seat she uses but I don’t know if they will allow it.

Tomorrow is a big day as we have our steering meeting in the morning. The steering meeting will be where the whole team meets with us (Jordan via conference) and tells us their goals for kenzies time here. We also intend on letting them know our goals as well. I’ll let you know how that goes. Today and yesterday have basically sucked not having Jordan around to be apart of these conversations and to be here with me for the rough times but luckily he will be here Friday for a whole week. Ill try and update tomorrow again… the internet keeps going down here! Sorry for the long post.

Monday, January 19, 2009

Out of Solitary

Kenzie's isolation restrictions were lifted the morning... WOOHOO! She is 100% better and we were all getting cabin fever in this room. It means we can roam about free again and have Kenzies therapies in the regular room that has an observation window.
What we have seen is that Kenzie does MUCh better in her feeding sessions when they are not in our room. Here we think she freaks out because we have to leave her with strangers as opposed to downstairs they take her to a new environment.

Today we had a new feeder and Kenzie did really well! Again it was carrots on her Nuk brush. For those who want to know what a Nuk is... that's what the picture is of above. Kenzie gets two, 5 bite sessions. What that means is that the feeder says "kenzie 1, 2, 3, enter" and puts the nuk with the carrots in kenzies mouth. If kenzie does not open right away, the feeder will put her hand on Kenzies jaw to guide it to the Nuk. Once Kenzie gets the food from the Nuk the feeder says "Good job kenzie... Good job taking your food!" Then they give Kenzie a second or two to clear her mouth and say "Kenzie open up and show me your mouth" Kenzie at first did not understand this. They showed her a bunch of times and she still didn't get it (shes very young to just understand what they want her to do). Eventually after a few sessions they did a little finger push on her lips to get her to open her mouth and within a few times she got it. Now after a few days, the second they say this to her she thinks its a game and opens her mouth so wide and proud! Sometimes she even does it without them telling her to. When she does this in the allotted time, the 2 people in the room cheer for her and then the feeder reads 15 seconds worth of Dr. Seuss books (because its her favorite and she really responds to them). She has to do 5 bites and 5 opens, then she gets a break and then another group of 5 and 5. Their ideal time is for kenzie to do each bite within 30 seconds. As of today she was doing it even faster than that. She had some gags and usually averages 1 vomit a session but shes really doing well. Also, a goal of theirs was to get her crying down a bit. I think being in the room the last few days made the crying worse... today in the feeding room she barely cried at all! She was having fun with the feeder, smiling, laughing a little and totally interacting! Her dinner session went so quick i think it was over in like 8 minutes!

Jordan and I have asked that tomorrow they change something because we really don't want her having time to just relax. We want to get the most out of this 8 weeks. So we asked that either they try a new food (we've only been using carrots and want her used to a variety of tastes), or up her bite count. We'll find out in the morning if this will happen.

Other than that, the weekend was pretty uneventful. Jordan left at 5 today and although we were so sad, at least he left on a positive feeding note and we know he will come back Friday for a whole week! Ill be here by myself for the first time for the next few days so I'm not sure how much energy ill have to post but I will do my best.

Thanks for checking in and of course for all of the great comments! :-) I wish I had done this a year ago when Kenzie was first sick. Its going to be a really nice journal for her to read one day if she feels like it.