Kai and bottling

After about a week of trying to bottle Kai, last night I finally got him to bottle a little. He took about 5-10 mls. I was so excited! We had such good progress with bottling before he get sick mid-December and ever since then, he doesn't bottle much. Now since he has been battling a nasty cold, it has been really hard for him to bottle. It's like he doesn't know what to do with it. It makes me sad. I don't want him to loose interest in any oral feeding. We also do some oral stimulation with him as well.

Kai has some new things

Kai has some new things to work with. PT from early intervention brought a tumble chair for Kai to use and a wedge for Kai to do tummy time. She also brought a few toys for him to work with. He seems to enjoy both. He can only tolerate tummy time for a short period of time, but does well with it. He really needs to work with head control. This is going to be a little challenging since he has low muscle tone. He will be starting weekly therapy at Pediatric Therapy Services in Mankato. I'm excited for him to learn to do new things. Today, he discovered his voice and was really talking up a storm! It was so nice to listen to him.

4 months old

Kai is now 4 months old. I can't believe how fast time is going. Kai has his 4 month check up tomorrow. Right now he is battling a nasty cold and his feedings aren't going the greatest. He just a little under 13 lbs. Kai has just recently really taken an interest in his hand. He likes looking at it. Kai has therapy at Pediatric Therapy on Wednesday. I'm hoping that we will come up with a set therapy plan.

Here is a tidbit about DeBarsy Syndrome...Debarsy syndrome was first described in 1967, since that time 28 further cases from 22 different families have been reported WORLD WIDE, making Kai the the 30Th DeBarsy case on earth, That's pretty darn rare !

The latest on Kai

The genetics doctor is going to clinically diagnose Kai with DeBarsy Syndrome. There is no blood test to confirm this. She is confident that he has this by ruling out other syndromes with similar features and that all the features that he does have, fall under the features of a child with DeBarsy Syndrome. DeBarsy Syndrome "is a rare, autosomal recessive syndrome characterized by a progeria-like appearance with distinctive facial features and cutis laxa. Ophthalmological, orthopedic and neurological abnormalities are also typically present. " (De Barsy syndrome: a review of the phenotype; Clinical Dysmorphology 2008). So this doesn't really tell us a whole lot since this is so rare and there isn't that much information on it. I'm hoping to connect with some others that have children with DeBarsy Syndrome. I have been in touch with one woman from Nevada that has a 19 year old with DeBarsy. This will hopefully give us a little more insight about this syndrome.

Today he saw the orthopedic doctor for his hips. His hips are still dislocated and the only way to correct this would be by surgery. Since this can be a complicated surgery and may not benefit him, we are waiting awhile to decide what to do. He is to go back in 6 months. The good thing is that he has no limitations right now. At this appointment he also got fitted for MicKie splints for his hands. He is to wear this during the day. He also had a swallow study done. That went well and he did not aspirate. They also tried a little bit of baby cereal and he did good with that (even though it was just a little bit). This is the latest on Kai. The next major thing so far is for him to get fitted for his craniocap (at the beginning of March).

Call from the genetics doctor

Today I received a call from the genetics doctor. She believes Kai has Debarsy Syndrome. I am meeting up with them on Monday to discuss it more.

Pictures

Levi and I

Gavin, Jace and Kai with their cousins

Gavin

Jace

Kai

Update on Kai

It's been a little while since I have posted an update about Kai. Kai is doing good. Kai is weighing in at 11 lbs 13.4 ozs and is 22 1/2 inches long. Last week Kai had an echo done on his heart and a scope procedure done. We have not heard the results on those yet. On Monday, Kai had another ABR hearing test done and that came back the same as the previous time. We are waiting to hear back from his ENT doctor to see what she wants to do with him. There has been some talk about possible hearing aides, but we won't know until we talk to the ENT doctor. Today Kai had appointments up in the cities. His first visit was with the pain doctor. They upped his Tylenol (as needed basis) dose. We talked about the plan with the tramadol and going to try the same dose the next time it is needed and see how he does with it. At this point, the pain medicine is only going to be used on a needed basis. Next he visited the developmental doctor. I think this doctor was a little surprise on how well Kai is doing. Cognitively, Kai seems to be pretty much on track with a normal 3 month old. This is a huge relief! This doctor would like us to work on have Kai more propped up (in like almost a sitting position) and work with build his head control. The last and longest appointment was the feeding clinic. There Kai worked with a nurse, dietitian, occupational therapy and speech therapy. They would like us to work back into the every 3 hour feedings and start decreasing his overnight drip feeding. We are to continue to work with bottling him. He is having a swallow study done on Monday. We are to do gentle range of motion with his hands so that he can use them to start grasping. They are suggesting for him to get McKie splints for his hands. I have to talk to his orthopedic doctor about this on Monday. We will be working with a few different oral stimulation. We also need to work with positioning with him. On Monday, he goes to the orthopedic doctor for his hips. I am very anxious for this appointment. I am eager to find out what the plan is for his hips. We have also added another person to his team, the deaf and hard of hearing early intervention teacher. Otherwise right now Kai is doing good. Majority of the time, he is a happy content baby. He smiles a lot, coos and loves to be talked to.

Gavin and Jace have both started ECFE. They each have their own night to go. Gavin just loves any kind of school. He is still going to Sunday school as well. He is all registered for preschool for the fall. He will be going to Little Lambs Preschool that is at our church. Gavin loves to ask a lot of questions. Right now, he is really into the days of the week and feelings. Jace...ahhh, where do we begin with him ;) He is definitely our wild child. He is a very busy boy and keeps us on our toes at all times. We have been concerned about a few things with Jace, so we have reached out for a little help. Jace just had a sensory assessment done on him. We don't know the results of that yet. It seems that Jace doesn't know how to burn off the energy that he has. One suggestion is for him to jump on a mini trampoline. So the other day, we went and got one. He loves jumping on it. This could also be an alternative to his hard core rocking that he does. Today he had visited his regular doctor for the concerns that we have. Autism was brought up as a possibility. So we are going to be looking into having an assement done on Jace for this. Otherwise, Jace is doing good. He is learning more and more words. I think his speech has really improved (thanks Becky!).

As far as me and Levi.....well, it's never a dull moment at our house and we are wiped out!

Kai is 3 months old

Kai is 3 months old today! He is just a little over 10 lbs.

Kai's surgery

Kai and his MicKey button
Kai excited about not having a tube hang from him all the time

Kai's surgery went good yesterday. We are still working him back to his normal feedings. This MicKey button is going to be much nicer. We have to go back in 3 months to change to a new one. That's a very simple procedure and I'm assumming a very quick on as well.

Pictures from today--Me and my boys

Kai smiling!

Look at his loose skin on his back side...it kinda reminds me of a puppy

Bath time...and legs stretched out (it's not very often that he has his legs uncrossed)

How Kai is doing

Kai is doing okay. He is now almost 10lbs. He smiles, coos, looks around and loves to be talked to. After the last time he was in the hospital, we have been trying to get him back on track with his feedings. We have come to the conclusion that right now he cannot handle large amounts of feedings. So he has a feeding about every 2 hours instead of every 3. He still does his continuous feeding throughout the night.

On Friday we had an appointment with the upper extremities doctor at Gillette. His right elbow is dislocated. Also his radius bone in his right arm is shifted down towards his elbow, so it does not line up with his ulna bone. At this point, they aren't going to do anything about it. She does not think Kai needs to do range of motion with his arms because he is moving his arms fine on his own. This doctor does not believe Kai is in pain because of it and will be seeing him again in 6 months.

Tomorrow Kai is having surgery up at Children's to switch from the G-tube to the Mickey button. This will be much comfortable for Kai. So we pray that everything goes good.

On Thursday, Kai will be seeing the ENT doctor. The will be doing a procedure called a scope (I think that's what it is called). Friday he has an assessment for physical and occupational therapy at the Pediatric Therapy Services. He continues to have early intervention in home every week along with his home care nurse. She now comes twice a week and helps out with some of cares.

I will try to keep more on top with blogging. It got so crazy busy at the end of the year. It is never dull at the Selly house. Both Gavin and Jace are doing good. I think they are a little stressed by everything that has been going on. They both start ECFE at the end of January.

Until next time!

Our visit with the genetics doctor

On December 18th we visited the genetics doctor. We have recently received the dictation from that visit. It is about 4 pages long, so I will just sum up some of the main things. The genetics doctor had listed some of Kai's medical problems;

1.Cutis laxa
2.Feeding intolerance, failure to thrive
3.Status post G-tube placement
4.Multiple joint dislocations including elbow and both hips
5.Cryptorchidism
6.Hearing loss
7.Concern regarding disproportion
8.Concern regarding pain

We did visit the pain team when we were there last (I can't remember if I had written that in a a previous post). Kai is taking tylenol right now for his pain. In the rest of the genetics doctor's dictation, she wrote that she is "concerned that Kai may have a disorder that is not compatible with typical lifespans. " She thinks that Kai is a tenuous baby and we are encouraged to avoid any exposures to illnesses for Kai. She continues to believe that Kai may have some form of cutis laxa or a connective tissue disorder. They are doing a fibrillin 4 and 5 testing on Kai. Other diagnostics that they are considering would be a progeria type syndrome.

It is so hard to figure this out. Whatever Kai has, it is very rare! We aren't looking for a name, but we want to know what Kai's future holds and how long of a lifespan may he have. We should be meeting up with the genetics doctor again some time in March-ish.

Playing catch up on blogging

It's been awhile since I have blogged. The last blog was about when Kai was in the hospital for his cold. He spent 3 days in the hospital. His oxygen levels got better, but would still dip at times. Levi and I had to do some infant CPR training before we could take Kai home. We also had Kai on an apnea monitor at night time for the first few days after he got home from the hospital. We don't use it on him every night, just when we feel it is necessary (like when he's not feeling good). So far, he has not set off any alarms on it.

Christmas came and gone. It was busy and we kept things low key. The boys were excited about all the presents. It didn't really seem like a holiday, it came so fast. The week after Christmas, we all got hit with stomach flu. What a way to end the year and start the new year. We tried very hard to avoid Kai getting it, but it didn't work, Kai ended up with the stomach flu! This ended up putting him back in the hospital for 3 days. Due to all the sickness, we had to reschedule 3 of Kai's appointments. The one was for his hips and that doctor is really hard to get in with. Kai is at high risk for sicknessess. When he gets sick, it takes a lot out of him and takes longer for him to get through it.

It's been awhile since I have posted an update. It has been crazy busy around here and I still don't have time to post an update. I hope to get an update posted this weekend.

Kai's chest x-ray picture

We got a picture of Kai's chest x-ray. I thought this was interesting.

Kai's in the hospital

Kai was admitted into the hospital this morning. He is on oxygen and is just being monitored at this point. He has a little cold and cough and has been working a little harder to breathe. Now that Kai has been on oxygen, he is doing better. He is on a low level of oxygen and they are going to be observing him. He has that darn nasty cold...pretty little guy. They tested him for influenza and RSV and those both came back negative--so that's good. They also did a chest x-ray and that looked good. This afternoon they gave him an antibiotic to be on the safe side. It sounds like they are going to observe him for a few days. It sounds like if he stays the way he is then they will probably send him home, but he will probably be on oxygen. If he gets worse then he probably will go up to Children's. On Thursday, Kai had several appointments in the cities. I will post more about those in the days to come. But one appointment was with the genetics doctor. She had two major concerns about Kai right now. One---if he gets sick, it could potentially be life threatening for him. Two---is his breathing. He uses a lot of energy to breathe. He's not gaining enough weight to burn off so many calories. So I'm not sure exactly what the plan is. I believe once he comes home, he will be on oxygen at home. He is a very fragile baby.

Kai is 2 months!

Side view of Kai's head...it is somewhat hard to tell in the pic how flat his head is on this side

Trying to hold Kai up in sitting position

Kai--2 months old

Well today Kai is 2 months old. He now weighs 8lbs 12 ozs. Last Thursday, the home care nurse was here and plotted out his measurements. His head was at 50%. His length and weight were at 5-10 %. So his limbs are not in proportion to the rest of his body. And you can tell this by just looking at him. Tomorrow he goes in for his 2 month check up, so we will see what they have to say. On Friday, Kai saw a doctor from Gillette for the flattening of his head. From what I understand, structurally Kai's head is normal so that is a good thing. The back right side of his head is very flat and is shifting his forehead and nose in the front. The doctor highly recommends a CranioCap for Kai. He has to wait until he is about 4 1/2 months old before he can get this CranioCap. He is too little and his head is too small right now for one. So he will be getting this when the time comes. He also thinks that Kai has torticollis (tightening of this particular neck muscle). So we have to do some specific neck stretches on a daily basis with him. The good thing is that he is starting to look his left. This Thursday we have 3 appointments in the cities. We meet with the eye doctor, neurologist and the genectics doctor. We meet with the orthopedic doctor on the 30th. Hopefully we will get a better idea of what the plan for Kai's hips are. Otherwise Kai is doing good. He is more alert during the day. He is doing the same as far as feedings. We will probably up his feedings soon. He does seem like he may have acid reflux, we will see what his doctor says about it tomorrow.

Gavin and Jace are doing good. They are excited about Christmas. Hopefully we will make it to see Santa this week.

Until next time!

Kai...the day of reaffrimation of baptism

Great Grandpa and Grandma with all 3 boys

Kai on his new positioner. This positioner is awesome...it's like playdough!