Hospital stay updates

These are the updates that I have been putting on facebook.  I decided to get them together in one spot in case anyone missed any of them.

5/30/13:
For those of you that don't know Riley ha her tonsils out yesterday. She is in pain and not her normally smiley self as to be expected. We will be in the hospital until at least tomorrow. Please continue to keep her in your thoughts and prayers

6/1/13:
On Wednesday Riley went in to have her tonsils taken out, the surgery went fine. Thursday night she developed a fever and vomiting, she was running a fever all day on Friday. Friday late afternoon, she took a turn for the worst. Her oxygen levels dropped very low and the doctors and RTs tried everything they could to bring them back up. It was decided that Riley needed to be sedated and put on a ventilator. We are currently in the Pediatric ICU at John Hopkins, and will most likely be here for at least a week.

Riley is doing better today but she is a very sick little girl. Right now the only thing that we really need to good thoughts and prayers sent Riley's way. She is a strong little girl and we and the doctors are confident that she will recover from this without any lasting effects.

6/2/13:
Not much change with Riley today. She is still sedated and on the ventilator. Katie and I are giving her lots of love, sent from so many people. Thanks for all the prayers and kind words. We are staying positive and are thankful that the doctors think that when she recovers there will be no lasting effects. Thank goodness for that

6/3/13:
We are still in the PICU and still on the vent. They have been able to lower some of her setting, but it has been slow process. Riley has developed a fever and her heart rate has been high, but the doctors are trying different things to get the fever under control and when her fever is down so is her heart rate. They may be switching her to a different vent either today or tomorrow. Small steps is all we can ask for.

I wanted to thank everything for your thoughts and prayers. They are greatly appreciated and needed. At this point there is not really anything that we need, other then for you to keep us in your thoughts

6/5/13 (8:38 am):

Riley had a pretty good night overnight. Her heart rate, blood pressure, & oxygen levels are good. They did decide to give her another unit of blood as some of her levels were down. She seems to have liked it as her stats seems to be stable.

She is still on the vent and requiring about 60% oxygen to keep her happy. The doctors still have her heavily sedated, but she is moving in response to being touched. When she gets over stimulated she will pull away from you. So it is a constant debate whether to be standing at her bedside holding her hand or just sitting quietly next to her.

Riley's chest x-ray from yesterday did look a little better, but we still have a ways to go. I have not seen the x-ray from today yet as the doctors have not yet rounded.

We have been talking with the RTs and doctors and will probable be in the hospital for about a month. They are also saying that she may have asthma after this is all done, as any time you blow air into the lungs it does some damage and your lungs are never prefect again.

The RT also told us that he thought would probable come home on a biPAP machine. Which is like a cPAP, but helps you when to breath in AND out.

6/5/13 (12:40):
Her latest chest x-ray was worst, the bottom of her lungs has collapsed.  They started her percussion chest PT every 4 hours when she gets her albuterol.

6/6/13:
We had a good news / bad news day today. She is still on the vent and requiring about 50% oxygen to keep her happy. The doctors still have her heavily sedated, but she is moving in response to being touched. 

Good News: Her chest x-ray was better this morning. Good News: The doctors have been able to identify the virus (Human metapneumovirus) that they believe Riley had before surgery, but was not showing symptoms of until after surgery. So now we know why crashed so hard, so quickly on Friday. Bad news: The virus just has to run it's course. Good news: We are already about half way through the virus' course.

Bad news Riley has picked up a bacteria while in the hospital (an unfortunate consequence of being in the hospital alot of the time). Good news: The antibiotic she is on seems to be help to fight it.

Bad news:The medication that Riley has been on for sedation and pain is a narcotic so she has to slowly have it replaced with another medication then we will have to ween off the new medication too. It will be a bit of a long process.

Riley is still in for a long recovery. We will be in the PICU for a while still and the hospital even longer. Please continue to keep us in your thoughts and prayers.

6/8/13:
Riley had a pretty calm day today.  

We are still on the vent, but they have been able to lower a several of the settings.  They are planning on lowering even more tonight.  

The doctors have started to ween her off some of her sedation so she is moving around more.  In typical Riley (aka Houdini Bodenhorn) fashion she was actually able to get out of one of her restrains today during rounds with the doctors.  Luckily we caught her before she could do any damage.

Riley's coloring in looking better, her heart rate, blood pressure, and oxygen levels have all been good so far today.


Please continue to keep us in your thoughts and prayers.

6/13/13:
Riley Update:
The last 60 hours for Riley have been very good. Her fever has broken, her heart rate and blood pressures have been good. How oxygen levels have been pretty good. In the last 24 hours the doctors have been making good changes to her vent settings. 

Yesterday's chest x-ray look better, there had not been much change to it for the days prior. I have not seen today's x-ray or heard the plan for today yet as the doctors have not been on their rounds yet. 

But we are moving in a good direction. Please continue to keep us in your thoughts and prayers.

6/17/13:
It has been a really good 72 hours.  

The doctors had planned on taking Riley off the vent on Saturday morning...  Well Riley decided the she was done with it on Friday night.  When she woke up from the heavy meds she had been on and started fighting the vent.  The doctor made the decision to pull the tube early instead of medicating her even heavier.  So at 10:30 pm on Friday night just over two weeks (by about 4 hours) since the tube had gone in it came out.

Friday night was a rough night with almost no sleep for either Riley or I as she was very uncomfortable (I think I would be uncomfortable too if I had a tube in my throat for 2 weeks) and would not let me out of her sight.

Saturday was the first time I was able to hold Riley since this all started and she I am both took an hour and half nap.  When my mom got here we switched places and I napped on the couch for 5 hours while Riley slept on my mom.


Sunday was more sleeping, but today PT came in and worked her for a good hour.  And by the end she was much more alert, but also very tired.  Riley has been able start taking food by mouth.  

We are ready to get off the PICU floor but are waiting for a bed. Thank you everyone for your thoughts and prayers.

6/20/13:
Riley is doing really well. We were moved to a regular floor. The doctors are slowly weaning Riley off the medications that she is on, at the rate we are going she should be off all her medications in 12 days.

Riley is still on some oxygen, but they are going to try turning in off tomorrow.

Our biggest hurtle right now is Riley's feeding. She is having a hard time with liquids, so tomorrow we are doing a swallow test to see what is going on when she is drinking.

I got my first Riley hug last night it was wonderful. She is starting to act more like herself everyday. 
.

6/24/13:
We are very happy to have Riley home. We were released from the hospital today after being there for 27 days. Riley is still requiring oxygen during the day and we came home on a feeding tube that is placed in her nose, but WE ARE HOME! Thank you to everyone that has been praying and sending us wonderful thoughts.

Still in Limboland

We had an appointment with the ENT on the 9th.  The appointment went well.  As I expected the ENT wants to do surgery to remove Riley's tonsils.  She is also going to look to see whether Riley's adenoids have grown back and if they have she is going to take them again too.  We have scheduled this for the 29th of the month, however the plans may change.

We still do not know about whether the neurosurgeon is going to want to do anything and we won't until we go an see him this Thursday.  I will do a more in depth updated after that appointment because then we should have a full plan, but I wanted to do a quick update today.

Limboland

I have been putting off writing this post for a few weeks.  First I was trying to do more research about it and then I decided that I did not want to worry everyone who follows this for an extended period of time.  But we are hopeful that we will have a few answers by the end of the week so I decided to post this now.

Riley has decided that we needed a visit to Limboland. I hate Limboland. Limboland is a place where you know something is wrong whether it is a serious something or a not so serious something. It is a place where you do not have the answer to the most important questions: "How do we fix this?" "Where do we go from here?" "What can we do RIGHT NOW?" "Has is caused damage already?" "Are we going in for another surgery?" "How long will we be in the hospital this time?" "When can I get in to see the doctors I need? No it can't wait a month; she needs to be seen now." "Does she need a trach again?"

These are not fun questions but they are the questions that have been running through my head since I talked to Riley’s pulmonologist. Riley had a follow up sleep study on the April 12th from her shunt revision back in September. And to say it did not go well would be the BIGGEST understatement I have ever made. Riley’s apnea is worst then it has EVER been.

Before I go into the results I want to define a few of the terms that I am going to use throughout the rest of this post.

Apnea: is a period of time during which breathing stops or is markedly reduced.

· Obstructive apnea (OA): is when air cannot flow into or out of the person's nose or mouth although efforts to breathe continue due, the airway collapses completely allowing no air to pass through during sleep causing the individual to snort and gasp for breath

· Hypopnea (H): is breathing that is shallower or slower than normal. This is a partial obstruction, the airway collapses partial it allows some air to pass through, however much smaller and it is accompanied by a arousal from sleep (either a complete arousal where the person is awake or an arousal where they simply come out of REM sleep) or a desaturation of oxygen for 20 seconds of more.

· Mixed apnea (MA): is a combination of central and obstructive apnea and is seen particularly in infants or young children who have abnormal control of breathing. Mixed apnea may occur when a child is awake or asleep

· Central apnea (CA): occurs when the brain fails to send the appropriate signals to the breathing muscles to initiate respirations

· Hypoxemia (Oxygen Desaturation): occur when oxygen in blood drops, meaning an abnormally low partial pressure of oxygen, content of oxygen or percent saturation of hemoglobin with oxygen, in combination with each other or individually (I refer to this as destat throughout my blog)

Polysomnography: (sleep study) is a comprehensive recording of the biophysiological changes that occur during sleep by monitoring many body functions including brain (EEG), eye movements (EOG), muscle activity or skeletal muscle activation (EMG), heart rhythm (ECG), the breathing functions respiratory airflow and respiratory effort indicators and peripheral pulse oximetry during sleep.

Respiratory Disturbance Index (RDI): is one very important measure of the severity of the sleep disorder. The RDI represents how many times per hour breathing stops or becomes very shallow. This index is important because it is often associated with disruption of sleep and dangerous drops in blood oxygen levels.

Arousal: abrupt transition from a deeper stage of sleep to a shallower stage

Continuous positive airway pressure (CPAP): is a treatment that uses mild air pressure to keep the airways open.

Hypoventalation: Abnormally slow and shallow respiration, resulting in an increased level of carbon dioxide in the blood.

Arnold Chiari malformation: is a structural defect in the cerebellum, the part of the brain that controls balance. The lower rear of the skull is smaller than normal, and thus the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination

Tracheostomy: is a surgically created opening in the neck leading directly to the trachea or the breathing tube. It is kept open with a hollow tube called a tracheostomy tube (or trach as I refer to it throughout this blog).

Now that the vocabulary lesson is over let get back to what is going with Riley.

I think this may be the first time that I might have reached Red Alert status since coming home from the NICU.  I have wondered what it would take to get me to Red Alert status and the numbers from the sleep study seem to be doing it. When I first heard the new numbers I was freaked out but then calm came over me.

I told myself that we would figure it out. We would circle the wagons, gather the medical team (pulmonary, ENT, and neurology), make a plan, and fix this. So got on the phone and called the ENT to set up an appointment with her. I emailed the Neurosurgeon who we already had an appointment with for 5/23 to see if he wanted us to come in earlier or wanted any more tests before we came in. ENT can see on 5/9, and Neurology did not think that we needed to come in sooner. He also did not want an additional testing. I am on Red Alert and her doctors don’t seem as concerned.  Now I love Riley’s ENT, Neurologist and the Pulmonologist, but I wanted to get in and be seen as soon as I got the results.  But that would not be the case.  So instead I started searching online for my own answers. I did not find any, so I resigned myself to waiting.

When Riley was in the NICU she had 4 different sleep studies all with around the same results. I will these numbers with the most recent results.

12/28/09: RDI- 68.7/hr. (obstructive apnea (OA) -38.6/hr, hypopnea (H) -15.6/hr, mixed apnea (MA) -3.0/hr, & central apnea (CA) - 8.4/hr)

2/12/10: RDI- 40.5/hr. (H-35/hr, MA-4.9/hr & CA-.3/hr)

3/13/10: RDI- 59.6/hr (OA -33.4/hr, H-22.7/hr, MA-3.5/hr & CA-1.7/hr

3/16/10: RDI- 52.5/hr (OA-30.9/hr, H-17.5/hr, MA-3.1/hr & CA-.9/hr

The sleep study after the trach was placed was the best one:

5/6/11: RDI- 5.7 hour (OA-.2hr, H-2.2hr, & MA-.7hr)

Then we had a repeat test in January of last year that showed the apnea was coming back. Our ENT decided that it was time to take out Riley’s tonsils and adenoids; though when she went in Riley’s tonsils were not big so she left them in.

1/20/12: RDI- 16.6hr (OA-2.0hr, H-10.3hr, MA-.8hr, & CA-3.5hr

Then we had another follow up last summer that showed that it was still not getting better so we tried Riley on the CPAP machine. Which was not a success. Shortly afterward Riley’s shunt stopped working. It was then thought that the apnea had come back because the shunt was not function properly.

7/2/12: RDI-26.6/hr (OA-14.0/hr, H-10.0/hr, MA-1.6/hr, & CA-1.0/hr

Then we got the most recent results….

4/12/13: RDI-105.7/hr (OA-6.0/hr, H-98.2/hr, MA-1.3/hr, & CA-.2/hr.)

Yeah they are as bad as they look. When you look at these the RDI is the total number of times Riley’s breathing either stops or is shallow to the point where she rouses or has a destat. The other numbers are a breakdown of the 105.7/hr. So basically 6 times an hour Riley is not getting any air going through her airway, 98.2 times an hour her breathing is shallow enough that she is rousing from a deep sleep (33.7 times out of the 98.2) or her oxygen level is too low.

The hypopneas are more concerning then the full obstructions at this point. Every time that you are roused from a deep sleep to a shallower level of sleep your blood pressure goes up. And every time your blood pressure goes up you increase your chances of developing heart and lung problems.  So Riley’s blood pressure is INCREASING 33.7 TIMES AN HOUR. Come on…Really.  33.7 times an hour!  THIS IS A PROBLEM.

The other thing that is not expressed in these numbers is Riley’s CO² levels.  It is normal to have CO² in your blood; a normal level is between 35-45mm of Hg. Anything above 50% is considered hypoventilation. Riley is peaking at 65% and 68% of sleep study above 50%.  So this is bad.

The only good news in this study is the number of central apneas has gone down, meaning that it is not a problem with Riley’s brain telling her to breath.

So at this point we do not have a plan of action other than trying to get Riley use the CPAP machine again.  We have ordered a new CPAP mask because the one we have is not working for Riley.  I will do a separate post about the CPAP machine. And Riley masks once we get the new one in.

Wordless Wednesday

One of the other blogs that I read does this wordless Wednesday.  I thought I might try to start doing this. So this one is not completely wordless.

"Typical' Toddler

I spend alot of time thinking about how different Riley is from other children her age.  But this weekend and tonight she reminds me how the similar she is to children her age too.

On Sunday we went over to the playground that is across the street from our house because two of the other kids who are around Riley age and understand Riley's differences were out playing. After swinging on the swings and sitting and watching the other kids run around (Riley loves doing this) it was time to go in and have dinner.  So we said goodbye and the other kids were going in too.  Well Riley was not ready to go in.  She is threw a fit.  She pulled my hair and tried to bite me.  It made me realize that this was behavior that other "typical" 3 years would do.

Then tonight at bath time Riley decided that Mommy needed bath too.  She would splash around then turn and smile at me, then splash me again.

She amazes me everyday!

Mastering "In"

Riley has been receiving OT services for 3 years.  And one of the goals that they had been working on with her is to get her to put things "IN."  In December she aged out of the Infant and Toddlers Program (I&T) and started ECI (school) in January.  In her very last session with her I&T OT, that she had for 2 years, she was able to demonstrate "IN."  This was such a thrill for Maureen (the OT) that she got to see her master a skill she had worked so hard to help her with.  Here is best video (and it is not very good) of Riley showing the skill.  She of course stop doing it better when I pulled the camera out.

This video is from 12/17/12:

Riley has now been in school for alittle over three months. And I think we can truly say that she has master the skill of "IN."

This video is from 4/7/12:

What a difference three months can make!!!

Medical history brochure

When Riley came home from the NICU, we came home with a very long list of doctors, 8 diagnosis, and 7 surgery dates to keep track of.  I was not sure how I was going to keep it all straight. We went for a follow up with Riley's pulmonologist and we were talking about trying to keep everything straight.  The doctor mentioned that one of her other patient's mom had created a brochure that they updated as needed. 

I thought that this was a brilliant idea so I went home and jumped on word template and found a trifold brochure.  I added pictures of Riley, all of her doctor' names and phone numbers, a brief history of her, all her diagnosis, surgeries (including dates), medication, pulled information from the Chromosome 18 website about Tetrasomy 18p.  I pulled this information most people don't know about Tetrasomy 18p.  Here is a copy of the template for the trifold brochure with information about Tetrasomy 18p.

As Riley got old and her history got longer, we got more doctors, added more services, and surgeries.  And the trifold brochure was not big enough for us any more.  So I went back in word template and end up finding a Christmas newsletter template that I convert into a 3 page brochure.  This allowed me to add milestones Riley had meet, a longer history, more details about her oxygen requirements, vision, hearing, and sleep study results.  Here is the link for the 3 page brochure with information about Tetrasomy 18p.

All of Riley's doctors love this brochure.  I update it as need.  I add new pictures.  It is great when we meet with a new doctor since her chart is so huge they can quickly look over the brochure and get a better idea about her.  It also has the link for the Chromosome 18 website so they can educate themselves about her too.  It has come in handy in an emergency too.  When you are panicked it hard to remember the last surgery or if her kidney reflux is on the left or right side.  This way you can just hand a copy to the paramedics or ER doctor.

I keep two or three in Riley's diaper bag. I also gave a copy to Riley's teacher when she started school.

This brochure is great for kids with lots of medical needs.  It is also great for when you are taking care of parent and there is more than one person handling the care.  It is an easy way to keep everyone on the same page.

Not so fun way to spend a Friday night...

I was looking forward to spending a quiet Friday evening with Riley as so she decided not to take a nap for my mom. Instead I spent the evening riding in an ambulance and in the ER.

Riley has become a climbing, crawling machine. Which is awesome but comes with it own challenges. She is not very coordinated so she falls down a lot and when she pulls herself up on to things she will just let go and go falling backwards.

On Friday the 22nd she was crawling around on the floor while we were watching Sprout and she decided that she wanted to get closer to the TV. We have a TV cabinet that has door was glass windows on the front.

She has been climbing up it for weeks she will be in her knees in front of it and put her hands up on the glass. I have been nervous that she is going to put her hands through the glass, lucky that has not happened. However that night she was wearing velour type pants and as she climbed up her knees slipped out from underneath of her and down she went. She has done this before but unfortunately this time her head caught the corner. I ran over and picked her up she started crying.  I went and got or booboo bunny out the freezer and a binky.  We went and sat on the couch.  She was not a big fan of the ice pack, but I able to get in on for a short period of time.  She did end up calming down, unfortantely it was right around bedtime but after a fall like that I did not want to just put her down.  So we relaxed on the couch for about 15 minutes playing on the iPad and watching Sprout.  After that she seemed to be fine and started to crawl around on the couch not wanting her to fall off the couch and hit her head again I put her on the ground to play.  She started coughing, but it was not a normal cough so I was keeping a close watch in her to make sure her coloring stayed ok. 

She pulled her self up to stand make to the couch and had another cough episode expect this one ended with her vomiting a little onto the couch. I jumped up and sat her down as she threw up again.  As I got her shirt off her she threw up a third time. I had called my mom to let her know that I was going to take her to the ER.  My mom told me to just call 911.  After getting the ambulance on the way I took her upstairs and put her in the empty tub in case she got sick again.  I tried to get in touch with Kyle but of course he was in the middle of a call and didn't answer.

She sounded a bit like she was having breathing issues so I grabbed her oxygen tank but she would not let me out the nasal cannula on her. The paramedics had arrived.  Of course by now Riley is acting more like her normally self, but I still wanted to have her check out since she was vomiting and she hit her head on the same side as her shunt.

I am been calm up until the paramedics got there and I had to explain what happened.  That is when I felt like i was having a panic attack.  The wonderful paramedic kept me calm and helped me gather everything that I needed to get out the door.  They even locked my front door for me.  As we are loading into the ambulance Kyle called me back.  I told him what was going on and that he should come home.

We check into the ER and were seen relative quickly.  My parent came down and Kyle rushed home from work.  Riley was acting pretty normal at this point and had not vomited again.  The doctors order a CT scan.  These are always fun with her.  She has to sit still to get a good picture and she hates laying on that table.  So she ends up wrapped up like a burrito with my hold her head still.  It is a workout in and of it self.  The tech tells me that it should be read 20 minutes and they they read them remotely.  Which is nice for the radiologist.

It is then the waiting game for the CT to read, that was the longest 20 minutes I have ever been through.  Mostly because it was actually like 45 minutes before they told us that the scan was clean.

They discharge us home, but tells us that we need to wake her up every 2 HOURS.  Grrr!  It was a long night.

The Great Sippy Cup War of 2013

Our household is embroiled in The Great Sippy Cup War of 2013. And I am sad the admitted that the 3 year old is winning.

Riley was never bottle fed. She went right from her G-tube to a sippy cup. You can read more about that journey in this post. And when we picked out a Sippy for her then we went with this Munchkin cup.

It also came in one that was bigger with no handles:

Riley will drink from either one of these without a problem.  The problem now is that Munchkin doesn't make that exact cup any more.  They change it to this cup:

Which looks very similar.. I know.  But my Riley can tell the difference.

Her cups mouth pieces look like this:

And the new cup mouth piece look like this:

It is wider.  It also is more floppy.  The part that the fluid comes out of is lower on the new cups.

So she won't take that cup.  I now am the proud owner of 13 different kinds of sippy cups.  And she refuses all of them.  Some of them she chooses to throw across the room when offered them.

Our collection of sippy cups.

We have tried other soft tipped cups:

And a few cups with straws.  But she doesn't know how to suck on a straw.  And I am kinda of at a lost as to how to teach a child that doesn't understand everything I say to her had to suck on a straw.

I have even gotten a few cups that open spouts.  But I am afraid to just offer her these as she throws her cups and leaves them on their sides on the floor.  And I am sure that I will be cleaning up more milk than she drinks.

So the search continues.  If any one has an suggests on a cup to try I am all ears.  I may have lost all of the battles so far, but I will win the WAR!!!!!

Riley: 12
Mommy: 0

Appearance of a disability

I came across this blog and it is one of my fears in regards to Riley.  When people look at Riley she does not look like your typical special needs child.  When you take a closer look, or watch her behave, or find out how old she is then it becomes more clear.

We have had handicap parking since around the time Riley turned 1.  And until recently she was always in a stroller.  I was always afraid that when I used the handicap tag that we would have some idiot come up to us and say something.  Riley now has a wheelchair, a very heavy wheelchair.  But at this point she really only uses it when she is at school, at other times she is in a stroller or goes right into a shopping cart.  I am still afraid or the person who will stop me and say something or give me a dirty look.  But I can't live my life in fear, so I live day by day.  Enjoying every minute (or at least most of them) that I get to spend with my beautiful, special daughter.

Here is what a child with a disability looks like.  And she is beautiful!!

Quiet Book

As I said in my last blog I am obsessed with Pinterest.  I blame my cousin Elizabeth for introducing my to the site in the first place.  

I have been seeing these quiet books show up on my board for awhile.  And I thought to myself.. "Hey I  could do that!  Riley would love it.  She is totally into books.  I should make one!"  This coming from the girl who got a sewing machine for her birthday 4 years and has used it once with the help of my mother (Who is awesome at sewing.  The woman made me a prom dress for goodness sake.  Side note on that my Dad actually taught my Mom to sew, cool parents I have right!?!) to make curtains for Riley's bedroom.

So I started collecting ideas, and fabric, and buttons, and zippers, and felt (oh so much felt) and all the things that I thought that I would need.  Well last night I started cutting out my templates.  I am realizing that I might first my first book by the time Riley turns 5.  Goodness me is it time consuming.  First I cutting the templates out in paper, because I can;t get any of the fabric pencils that I have to write on felt GRRRRR!  Then I am pinning my paper templates onto the felt to cut it out.  I brought embroidery scissors, because A) They are small enough to cut the small templates and B) They are super sharp.  

I have not even started sewing anything yet.  Hopefully I can get into a rhythm and get a few pages done.  I will keep you all updated.

These are some of the pages taht I have found my ideas on:

http://jocelynandjason.blogspot.com/2013/03/free-butterfly-quiet-book-template.html

http://quietbookblog.blogspot.com/

http://theintentionalmomma.blogspot.com/2013/01/our-quiet-book.html

Update on Riley

I have not done a Riley post in awhile.  

Riley has made some big progress recently she is pulling up on anything and everything that she can get her hands on.  She has also started doing a really hands and knees crawl.  She has been army crawling and rolling for a long time now, but just within the last week or so she has started really crawling.  It is awesome.

She also got her wheelchair because ...SHE STARTED SCHOOL YESTERDAY!!  You heard right my baby is in school.  Riley turned 3 in December and that meant that she aged out of the Infant and Toddlers Program.  In order for her to still receive services she needed to start going to school.

We had our 1st IEP (Individual Education Program) meeting at the end of November and it went very smoothly.  Through Infant and Toddlers Riley was getting PT once a week, OT twice a month, speech twice a month, and the developmental specialist twice a month.  With her IEP she is still getting PT once a week, but she is also getting OT once a week now.  She is still getting speech twice a month.  Plus she is in school 5 days a week for 2.5 hours a day so they are working on PT, OT, speech, and developmental stuff through out her time in the classroom.  But someone is coming to work specifically with her according to the schedule above.

My husband and I dropped her off yesterday and as soon as another child walked in the room it was like we did not exist.  I think she is going to be fine.  Mommy's on the other hand we a bit sad to see her go. I was proud of myself though because I did not cry like I thought I would.  It was a bit to hectic getting out the door and into class for me to get upset.  I think school is going to be a great thing for her.  I can't want to see how much progress she makes between now and summertime. 

We have switched to a new orthopedic doctor because we were unhappy with the one we were seeing before   He put Ri in a cast on her right foot to try to straighten it out more and it worked well.  So she in now in new AFOs that are higher then the ones she had before.  They now come up to just below her knees.  We are going next Friday to meet with the doctor again to discuss the surgery that he wants to do on her knees.  Riley is missing about 20 degrees of flex in both of her knees.  Think about trying to walk with your knees bent at at 20 degree angle, and how tired you would be.  That is what she is dealing with now.  I don't have more information at this point but will after next Friday.  I will post after that meeting.

Here is a picture from Riley's 1st day of school.

Return of the apnea

I am been meaning to write this up for a while.

On 1/20/12 Riley went for another sleep study. And, the results came back saying that her obstructive apnea is back. Her overall episodes per hour were up from 5.7 hour (obstructive (ob) - .2hr, hypopnea (h) - 2.2hr, & Mixed apnea (MA)- .7hr) to 16.6hr (ob - 2.0hr, h - 10.3hr, MA - .8hr, & Central (C) - 3.5hr). This falls into the severe obstructive apnea category. When I first saw these numbers I was concerned that they had gone up but not overly so until I saw that they were high enough to fall into the serve range

Now you might say "If I saw that my child stopped breathing 16.6 times in an hour while sleeping I would be freaking out. Why aren't you?" And I was concerned but the fact that Riley is on oxygen at night which helps to keep her oxygen up even if she stops breathing for a few seconds helps to keep me from freaking out. Also 16.6 was nothing compared to 68.7 which was the number of episodes she was having in December 2009. Riley's first sleep study results showed she was having 68.7/hr. (ob - 38.6/hr, h - 15.6/hr, MA - 3.0/hr, & C - 8.4/hr). So when I saw a 16.6 I was disappointed that it was up and a little worried but though we would just increase her oxygen.

Well the doctors had other ideas; Her pulmonologist felt that the obstructive apnea was back for one of two reasons: (1) Riley's tonsils and adenoids were blocking her airways and if they were removed then the obstructive apnea would go away again or (2) her Arnold Chairi could have gotten worst, as your brain stem controls breathing. Riley's pulmonologist wanted us to go see her ENT before we got in touch with her neurosurgeon.

We went and saw the ENT and she thought based on the result so the sleep study that it was a good idea to go ahead and take out her tonsils and adenoids. On 3/16/12 we went in to have Riley's tonsils and adenoids out, we also were going to have her trach site and g-tube site completely closed. During the surgery her ENT decided that her tonsils were not very big and they didn't need to come out.  

On 7/2/12 Riley had a follow-up sleep study that came back it the result of 26.6/hr (ob-14.0/hr, h-10.0/hr, MA-1.6/hr, & C-1.0/hr).  So those results were not what we were looking for.  The  pulmonologist wants Riley to wear a CPAP. HAHAHAHA...is what I say to that.  We have had the machine for 6 weeks can still can't get her to wear it with it turned on.

We also went for two MRIs on 8/28/12.  One was a cine MRI, the hope of this one was to see exactly where she is obstructing.  We went on Friday to see the ENT and were disappointment that they were not able to get her to obstruct during the test.  So we got no answers.  The second was a regular brain MRI which showed slight changes in her brain.  Her neurosurgeon has scheduled us for a Shunt Patency test this coming Thursday.

A shunt patency test is used to evaluate the proper flow of cerebral spinal fluid through the shunt system and to make sure there are not any blockages. The scan involves the injection of a radioactive tracer into the shunt reservoir by the nuclear medicine doctor. Pictures will be taken to follow the path of the tracer through the shunt.

So we hope at after we meet with the neurosurgeon on 10/2/12 we might have so more answers.

Seizures

This is a blog of wrote last summer but never posted for some reason.  I am posting it now.  Enjoy.

Last June (2011) while we were in NC, Riley had what we believed were 2 seizures.  So we took her to see a neurologist.  She decided that she wante to get an EEG (An electroencephalogram (EEG) is a test that measures and records the electrical activity of your brain.) to determine if she was having abnormal activity. 

It is similar to a sleep study with all of the pobe on your head, but that is where it ends..  She HATES getting the probes put on.  As son as the tech put on his gloves and picked the first pobe Riley started to freak out.  It only got worst from there.  She screamed at the top of her lungs as he put a;; the pobes on.  She was bright red, snot and drool running down her face.  I always feel bad for the techs, they always look at me like "Do you want me to stop?" And I always tell them that she is fine and just to keep going that she will get over it.  Then you get the "Are you sure look?"  I figure that she is already pissed so you might as just keep going and I will calm her done when it is all over. 

After he left and turn out the lights she calmed right doen, just like I know she would.  She even fell asleep.  The test lasted about an hour then at the end they wake the child up and flash light in their eyes trying to see if it will cause a seizure.

Riley did goo and had no more abnormal brain activity then they would expect from someone with a shunt placed.  This of course does not mean that what she had in June were seizures, but it also ti does not mean that she isn't having them either.  The problem with this test is gthat it can only tell you if she is having seizures during the time of the test.

The doctor did say that she doesn't believe Riley has a seizure disorder.  but seh cautioned us that she could develop one.  So her advice was to keep an eye on her and if it happened again we could do a 24 hour EEG.  She told us to watch for any sudden head dropping, if she stared out into space and we were not able to get her attention back quickly, and tighting of her limbs.

Riley does still state into space sometime, which could be an absence seizure, but we can get her attention back pretty quickly  So we are just watching her at this point.

Feeding Tube is gone

I have been a terrible blogger....but I do have wonderful news.  The feeding tube that Riley had placed sbout month after she was born was removed on November 14th.  We are so excited, we have been working toward this for the last 2 years. 

 

Last year on Riley's first birthday she could not have any cake, but this weekend at her 2nd birthday party she will be about to have cake, smash it, and enjoy it. 

I always wanted to have a cake smashing session with her so yesterday I borried my sister-in-law's really nice camera and had a cake smashing session.  And Riley was all about it. When I edit them I will post some here. 

 

Until next time.

Letting the Nurses go

Now that Riley is almost 2 months out from her trach removal, Kyle and I feel secure enough in her airway to let go the nurses, that we have had since she came home from the hospital.  Our last day of nursing will be Sunday.  I thought I would be more nervous about being on our own, but I am pretty calm about it.  Monday night may be different, but right now I am good. 

We just got back from our 1st family vacation last week and we used that as a test run to be without the nurses.  We had Riley sleeping in our room in a pack-n-play (which she did not like sleeping in) and she did well and so did we.  We also have a video baby monitor, which I think are wonderful.  We can look in on her without actually having to go into her room and risk waking her up.  Plus she is still on a pulse ox monitor, which alarms if her oxygen level goes below 92 for more then 5 seconds. 

She has come so far in 18 months that she has been home.

New Milestone

I know that I have not posted in awhile.  Life has been a bit crazy.  I hope to have a longer blog written soon so I can post it.  But I had to share.....

 

 

Riley stood holding on the couch tonight for several minutes by herself.  She had done it for a few second last week, but did it for ALMOST 6 MINUTES TODAY!!!!!!!!!!!!!!!!!!!!!

 

 

 

 

Riley's road to a Tracheostomy

I promised I would do a post about Riley's trach so here it is.

Riley had her trach put in on 3/31/10. She had been in the NICU for more than 3 months. Shortly after she was born she started have oxygen saturation drops, meaning that the level of oxygen in her blood was too low.  If the oxygen in your blood is too low than the rest of your body, especially your brain, doesn't get the oxygen that it needs to function and thrive.  Your blood oxygen level  should not go below 92, Riley was having drops into the 60s, and even lower into the 40s and 30s.  Obviously this is a BIG problem.

The doctors did not know why she was having this severe of drops. Riley was put on oxygen through a nasal cannula to keep her level where they needed to be. However, she was still having apneas even when she was in the oxygen.

She had her 1st sleep study done at 17 days old.  A sleep study records lots of different things during sleep, including brain activity, eye movement, oxygen and carbon dioxide blood levels, heart rate and rhythm, breathing rate and rhythm, the flow of air through your mouth and nose, snoring, body muscle movements, and chest and belly movement.  Riley had electrodes put all over her head and chest.  She was found to be having 68.7 apneic episodes an hour.  She was having 38.6/hour obstructive apnea, 15.6/hour of hypopnea, 3.0/hour mixed apnea, and 8.4/hour central apnea. (See Medical Terms page for definitions of each type)  She was diagnosed with obstructive sleep apnea syndrome.  She also had severe hypoxemia, an abnormally low amount of oxygen in her blood. 

After the sleep study Riley was put on a sleep apnea machine, which made her look like a rhinoceros.

This was bit something that she could come home on because they do not make them for at home use; for babies. So we had to find a different solutions so she could come home. She was on the sleep apnea machine for about a week before they switched her back to a high flow nasal cannula.

On January 2010 she developed terrible reflux issues.  They tired feeding her over different periods of time which didn't help.  The doctors thought they could severely reduce or eliminate Ri's apnea if we did a nissen and placed a G-tube (See Learning to eat blog for more details about this).  About two weeks after surgery we did a repeat sleep study.  This time she was having 40.5 apneic episodes an hour.  She was having 35/hour of hypopnea, 4.9/hour mixed apnea, and .3/hour central apnea.  She still had severe obstructive apnea with severe hypoxemia.  So it had fixed her reflux issues but did not make much a difference in the apnea problem.

We went on to the next step.  Her apneas tended to be position related.  Meaning if we cradled her in our arms she would have an apnea or if she was laying flat she would have an apnea.  To combat this we would hold her more upright or on out shoulder.  And her bed was set so that her head was raised all the time.  It was not enough we needed a more permanent solution.

Riley had (and still does but it is improving greatly) low tone.  Her muscles as just not as strong as they should be and it makes it hard for her to do lots of things.  it effected pretty much her whole body.  The two most important part of her body for this topic are her throat and tongue.  She also has a small chin which in turns means that she has a small mouth.  (I have a small chin and mouth too but Ri's is much more pronounced) With this in mind the doctors nest thought that they apneas might be due to the low tone in her tongue and her mouth being too small for her tongue. The fact that she was having more apneas when she was in certain positions supported this theory.  They believed that when she was in a flatter position that her tongue was falling into the back of her throat and closing off her airway.  We could actually watch her and see this happening; The plastic surgeon suggested a procedure called a tongue/lip adhesion (or TLA). 

A TLA is exactly like it sounds.  A surgeon goes in and pulls her tongue forward and places a cut on the bottom of the tongue then they place another cut in the inside of her lip.  They then stitch the two together.  For recovery they also place a stitch that goes from the top of the tongue through the bottom of the tongue and then through the lower palate.  This is secured under the chin with a small plastic bar.  This is to keep her form bring able to move her tongue too much so that it can heal together.  Once it is healed enough that there is not a concern that she would be able to pull her tongue and lip apart the stitch through the tongue is removed.

As I write about this procedure I realize how barbaric it sounds and let me tell you she was not pretty to look at when she came out of surgery either.  It was frankly down right scary to see her like that.  There was another little girls in the NICU that was having similar breathing issues as Riley and she had under gone this procedure prior to Riley having hers.  They were kind enough to let us see their daughter before we decided to go ahead so we could get a better idea of what to expect. 

Some of the risk and effects of a TLA are that it can harder for the child to talk and eat.  Eating was not an issue for us because Ri was not safe to have food orally and she had the G-tube,  But the delay in talking was a concern for us.  T18p kids already do not normally starting talking until 2.5 years old and we could be delaying her even more.  There was also no guarantee that it would work to get rid of her apnea.

We debated for awhile and tried to find out if there were other options.  This seems like a drastic procedure.  If we decided not to do the TLA, than our options would to stay in the NICU, have a tracheotomy, or a jaw distraction.  All of these options were more drastic then the TLA.  The tracheotomy would require 24 care when we got home.  Staying in the NICU was really not an option, she could not grow up in a hospital and never see the outside world.  And a jaw distraction is another surgery where they attach a device to the patient jaw and then they are able to slowly strench the jaw, this making it larger.  That was way to drastic and even the doctors thought so.

We decided to go ahead with the TLA.  The plastic surgeon told us that if it did not work that the next step would be the tracheotomy.  After about 2 weeks they removed the stitch through her tongue.  We went about another 2 weeks before doing a third sleep study.  We wanted to give her enough time that she could heal and the swelling could go down.  The third sleep study results were not much better than before the TLA: 59.6 apneic episodes an hour including 33.4/hour obstructive apnea, 22.7/hour of hypopnea, 3.5/hour mixed apnea, and 1.7/hour central apnea.  They did a fourth sleep study 3 days later but this time they part her on oxygen to see if that made any difference and it didn't. She was still have 52.5 apneic episodes an hour including 30.9/hour obstructive apnea, 17.5/hour of hypopnea, 3.1/hour mixed apnea, and .9/hour central apnea.

So it was decided that the tracheotomy was our only option so that we could get Riley stable enough to bring home.  It would mean alot of changes in our home life and seeing as this blog is super super long already I will do a separate one on how if effected our home life and what is needed to care for a child with a trach.

Once she had the procedure she was like a different baby.  She was sleeping better, and calmer, she looked healthier, and she starting gaining weight wonderful.  Once we got her oxygen requirements fixed she do so much better.  I just felt bad that it had taken us 3 long months to get her there.  Before we could leave the hospital we had to learn how to clean, and take care of, and change the trach.  That was really kinda of scary at first.  The first time I had to change her trach I was shaking, but after I did it I realized that it was alot easier then I thought it would be.  I quickly became a pro.

I hope you have enjoy this ridiculously long post.  If you have any specific questions please let me know.  I hope to get home life and care part of this topic up by the end of the weekend.

Here are so links that we find useful. When we were trying to make our decision about the trach.

http://www.trachkids.org/

http://www.tracheostomy.com/

http://passy-muir.com/

http://www.hopkinsmedicine.org/tracheostomy/about/

Hearing aid update

I thought I would do a quick update on how Riley is doing with her hearing aids.  We have had them since March and it is amazing how much it has made a difference in her language.  She still does.  She doesn't have words and probable won't for another year, but she has more sounds now.  And plays with them more.  Now if she is on the floor by herself or in her carseat she just starts babbling.  It is quite funny.  She is more alert now.

I have been quite pleased that she had not been pulling them out like I thought she would.  She has recently being pulling them out but that is because they have not been fitting correctly.  When they are in correctly she doesn't mess with them at all.  We had them readjusted when we were in the hospital to have her trach removed.  But she has not been wearing them since because while she was in the hospital they checked to make there was no fluid in her ears.  To do this the ENT put a little cut in her ear drums.  She would let us put the hearing aids in but as soon as you turned them on seh would cry.  So I think her ears hurt.  We will give it a couple more days than put them back in.

So there is the update on the hearing aids.  If any one has questions about them let me know.

So there is the hearing aid update. If any one has questions about them let me know.

Making Baby Food

I have never been  avery good cook, but I throughly enjoying making Riley baby food.  It is so easy.  I just spent a couple of quite hours in my kitchen alone making her food.  I put her to bed, put some music on and danced around my kitchen as I made food. 

 

Here is what I made:

Peas (she LOVES peas)

Apples

Apple and Avocado (Avocado is the newest thing that she has had and she loves it)

Pear and Avocado

Pear and Apple

Banana, Raspberries, and Gram Cracker

Banana

Yellow and Green Squash

Grean Beans

 

I think this might become a new hobby for me.  I want to figure out which combination she likes best and make a bunch it so in the winter she can still have summer produce.  If anyone has good recipes that their kids like let me know.  Riley is not a picky eater.  the only food she can't have is blueberries.