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.

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!!!

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!!

A Change in Thinking

Recently I was having a conversation with another mom of a different needs child, we were discussing a possible new diagnosis that they were looking into that has the potential to shorten her child's life.  She told me that at this point they don't know if it is true or not, but I commented that it would be good to know because she might do things different if they knew they only had few years with their child.  Maybe they would go to Disney earlier than they would otherwise, or they would take a photo of their child everyday.  She went on to tell me about a friend of hers that is currently dealing with just that with not one but two of her children.  This woman walked into find her kids doing something that most parents would have started yelling about, but she couldn't bring herself to yell, so instead she laughed and took a picture.

This conversation got me thinking why should a child dying changes how we act toward them.  If you only get a few years with a child because they are sick and you then make sure to take a photo everyday or you plan a trip that you would have otherwise put off until "they were older," or you laugh off small misbehaviors.  Why not do that with a healthy child too?

This world is unpredictable.  A health child could be diagnosis with cancer, or get in a car accident.  Look at the parents of the Sandy Hook students.  I am sure they are all wishing for one more photo or one more trip or one more anything.

We should cherish EVERYDAY we have with each other.  Whether it is child, or husband, or wife, or moth, or father, or brother, or sister, or friend.  Don't wait until tomorrow to tell someone that you love them.  We live in a world where we are so focused on what we don't have that we forget to look at what we DO have.

I have recently watched two movies that drove this point home. And yes they could be a bit preachy, but sometimes preaching isn't a bad thing if it gives you a new perspective.  For anyone that is interested the movies were "Courageous" and "The Heart of Christmas."

I watch my Facebook feed all through November and everyone was posting what they were grateful for, but as of December 1st that was no more.  Are we only grateful for our lives and the little things in them on month of the year.  I am going to try to find 1 thing I am grateful for everyday for a year. Some days I may not write a full post but I will try to at least post what I am grateful for each day.

So Day 1 of the year of Thank you for my life.

I am grateful for the joy on Riley's face when I walk in the door from work.  She greets me with a big smile, a yell of happiness, and a giant hug.  It is a great way to end a good day or even better a bad day.

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.

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.

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.

Trach removal

While we sitting in the waiting room for Riley to come out of surgery to see if her trach can come out I started writing this but the day got busy and I didn't finish. So some of this was written on the day and some later.  Here we go.

We have been waiting for the this day for a long time.  When the trach as first placed her ENT thought that it would be in for 3yrs.  Well Riley proved to be the fighter that we all know her to be and is getting it out 1yr 3mo and 6days after it was placed.  So here we sit in the waiting room while the ENT has her in the OR evaluating her for the removal and possible taking it out.  Riley is surpassing our expectation of her everyday

(That is the end of what I got written on the day of the removal)

So since I am now sitting down to write this on my computer I thought I would recap the whole day.

We got up at 5:30 to be able to leave by 6:15.  I go into Riley room at about 5:45 and find Riley sitting up in her crib with her nurse.  The nurse tells me that she has been up for able 15 minutes.  So apparently just excited to this day as we are.

We arrived at the hospital at 7am as we were requested to do and headed up to the pre-op check in.  Riley recently developed a white coat fear which she extended to the nurses as well.  Sometimes I think she is more afraid of the nurses then the doctors.  Which is funny since they mostly are just taking her head measurements, temperature, or some minor thing.  So the hospital we go to is a teaching hospital so we get alot of students or residents.  The nursing student that had starts to process us for check in.  Putting on an ID bracelet became a chore and we ended up having to have them print another one so it could be off Riley's wrist and on her ankle.  (I am going to have to remember that for the next time to save us the trouble and the tears)  She listens to her heart and tries to get a blood pressure.  Riley HATES the blood pressure cuff, she has since the beginning.  The NICU use to have to put it on her and wait for her to sleep so they could get a blood pressure.  So now she is screaming and crying so of course it doesn't read the first time then the second time it is reading high (go figure if I was screaming like that my blood pressure would be high too).  Finally one of RNs comes over and says they would be fine without the reading since she looked fine and her heart rate was fine.  They need a weight now, but Riley is too big for the baby scale but can't seat on her own without falling over on the big people scale.  So Kyle  has to hold her they weigh both of them, then he hands me Riley and the weigh just him and subtracted the two to get her weight.  So now we are all checked in and have to waiting for anesthesia come in and consent us and ENT to come in. 

We sit there for about an hour before we are all consented and ready to go.  When the ENT comes in she explains that she is going to go in and sedate Riley.  Then they are going to put a camera in her airway and take a look both above and below the trach to make sure that their is not any granulomas (A growth of inflammatory tissue, which is caused by the irritation of the airway by the tracheotomy tube) that would pose an issue with breathing when the trach is removed.  If there are any and they would cause a issue the ENT will try to remove them and hopefully still be able to take the trach out.  She was also going to check Riley's ears because the audiologist thought that she  might have some fluid in her ears.  She lets us know that after the procedure they will take Riley back tot he PICU and it will be about 20-30 minutes before we will be able to go back.  And that once we are back if everything is good to go we will take the trach out and put a bandaid on it and that's it.  Yes removal of a trach ends with a bandaid.  It was hard decision to put it in and alot of work to take care of it and it all ends with a bandaid... kinda anticlimactic isn't.

Riley has fallen back to sleep in the stroller and when they come back in and says we are ready to go.  I get gowned up to carry her in to the OR.  Riley wakes up when I picked her and we follow to the 2 residents into the OR. She was fine as we walked into the OR, she was looking around at all the activity and the lights,  then I went to lay her down on the table. That was the end she started screaming again, I felt terrible she was so scared.  I just stood there and tried to calm her down as the put her under.  It didn't take long before she was out.  I headed out to the waiting room.

The procedure took about 45 minutes total.  The ENT comes in and tells us that everything went well.  Riley's vocal cords looked good.  there are a small granuloma above the trach but she had left it alone because it was small enough that she did not think that it would cause any problems.  The ENT said that we looked good to take the trach out and that we would do it in PICU.  She leads us to the PICU waiting area and goes to check on Riley.  She is back in like 2 minutes and say come on back.  We were surprised.  We get back there and there are like 10 people around Riley's bed, she is awake and screaming again.  My poor baby.  I got my camera out video taped Kyle taking the trach out and the ENT placing a bandaid on it. 

Riley was really upset for alot of the morning, which I think had alot to do with the IV in her hand and that she was hungry since she could not eat after midnight the night before.  She had to wait a bit to eat but once she did she was a bit happier.  They were suppose to keep us overnight but the ENT said that if she was good all day that we could go home that the same day.  So I had to scramble a bit to get things in place at home (ie nursing, oxygen supplies) so that could happen.

She did good the whole day, cranky but good.  Though I would be cranky in I had a big IV and was stuck in the hospital too.  By the time we got everything in place with the oxygen it was 6:00 at night but we did get to go home the same day.  She did great overnight with the oxygen and no trach.  She is using a nasal cannula now and that is going to take some getting use to.  She screamed every time I tried to put it on her.  i was finally able to get it on by using the stickers that hold it on but she was not a happy camper.  Tonight was a bit better but it will take time for her to get use to it.

If you have any questions about decannualization let me know.

I have started my trach blog all about our decision to place the trach, the care required for one, and the process of working toward decannualization.  I hope to have it up soon.  If it get way too long I may break it into several blogs.

Until next time,
Katie

Learning to eat

Riley's feeding has never been normal.  When she was first born she was on IVs and getting all her nutrition that way.  This was because she has surgery that say after she was born to close her spine.  Afterwards when we attempted to feed her by mouth her oxygen level would drop.  She could not figure out the the whole suck/swallow/breath combo.  I was only able to tried to breast feed her twice, but I did pump and give her breast milk solely until 4 and 1/2 months then I had to supplement because I could not keep up with her.

The NICU put in a feeding tube through her mouth.  Normally, they would do this through her nose, but she has small nasal passages and a deviated septum.  She would pull the tube out constantly.  She developed vomiting after her feeds.  They tired lots of different things to stop it, reflux medications, motility medications, moving the tube so that it was in her upper intestines instead of her stomach.  None of them worked then they started her on continuous feeds.  Where she was slowly getting food in through her tube all day and night.  They did a reflux test and found she has serve reflux..which we could have told them.  They continued to try different medications.

At one point they thought that maybe she has a milk allergy and out her on soy milk.  It didn't help so she went back to breast milk.  Through all of this her weight gain was slow as to be expected.

As the reflux got worse the doctors suggest something call a nissen fundoplication.  Basically the muscles at the top of Ri's stomach were not doing their job keeping her food in her stomach so she could digested it.  So what the nissen does is pulls her stomach up and wraps it around the bottom of her esophagus.  Thus created the sphincter need to keep her food in her stomach.  The standard practice at the hospital where Riley was was to place a G-tube when doing the nissen.  This a feeding tube that is placed in her belly allowing us to feed directing in her stomach.

Her reflux was thought to be playing a big part in her continued breathing issues where her oxygen levels were dropping.  The thought was that if we could avoid the reflux with the nissen and the oxygen drops during feeding by placing the G-tube that she would be good to go home.  We decided to go through with the procedure.  After she healed it worked to stop the reflux but did not help with the oxygen levels.  She ended needing a trach placed in order to get her oxygen to stay up.  I will do a separate post about our decision for the trach, what it is like to have a child with a trach, and more. 

So back to her feeding.  Our next goal was to get her off the continuous feeds to what is called a bolus feed.  This where she would get her feed all of one time.  It was a bit of a slow process.  We went from continuous to 4 hours on feeding and 4 hours off, then to feeding over 2 hours, then to feeding over an hour, and finally to over 1/2 hour every 4 hours.  Riley started to gain weight consistently after that.

After we had her home for awhile we wanted to try to start her on some oral feeds, but we had get clearance from one of her doctors.  First we tried her on milk on a spoon and that did not go well.  When we tried some stage 1 foods she did better.  The doctor had only give her one baby spoon full of food, in small bites.  Then once a week we increased it by one baby spoon fully.  It several months to get her eating a enough food that they could do a swallow test on her.  In November 2010 we went in for the swallow test, what they do in put barium in her food and they feed her and as she is eating they are x-raying her to watch how she was swallowing to make sure she was not aspirating on her food.  We were told that she was doing wonderfully and was not aspirating and was good to start oral feeds full time. We were so excited because it meant that she could have icing for her birthday.

You would never know now that she had some many issues with eating.  She does not turn her nose up at any baby food that we give.  If we don't feed her fast enough she yells at you.  We are now working on getting her to take all of her fees oral and not using the G-tube at all.  She is learning to use  a sippy cup and is doing wonderful.  Though if she sees the food before she finishes the cup you have a hard time getting her to take it again until the food is gone. 

Recently we have started her on solid foods.  She is getting good at picking up the Gerber puffs with her whole hand.  She has had some scrabbled eggs.  And I have a few other foods that I am going to try her on in the next couple of weeks (raspberries, peas, avocado).  She had some cheese today and seems to like it.  We might give a grilled cheese sandwich soon too. 

If any one has ideas for fingers food or handmade baby food let me know.  I just started today making some of her food and I am looking for ideas.

Busy week

So I have not posted anything for more than 2 weeks.  It has been a bit of a crazy 2 weeks.

Last week I was packing all week for Riley's very first trip.  We went to NC to visiting family because my Grandmother turned 90.  We had a nice trip and got spend lots of time with family and even got to see some friends too.

We did have several bumps in the trip.  The first was when we first got there.  Our medical equipment company (APRIA) did not send all of the things we needed.  More importantly they did not send both machines we needed.  So at midnight I got to call and yell at someone.  APRIA is a terrible company we have more problems with them than good experiences.  So they came a delivered a piece that could work but not the right machine.  I made it work though.  It was a really good thing that I brought alot of what they were suppose to bring.  They sent us adult trach mask for an 18 month old.  How much sense does that make...NONE.  They sent a humidification container that went with a nasal cannula not a trach, I brought the right one thank goodness.  I was ready to kill someone.  But I worked with what I had and it worked out in the end.

The scariest thing happened on Saturday.  We were at my cousin's pool, which on a side note was the first time Riley got in a pool and she loved it.  We had been in the water for about 20 minutes on so and I was sitting on the steps in the water with Riley in my lap.  She all of sudden went completely stiff in my arms and would not response when I called her name.  Then she snapped at out it and started screaming.  I rushed out of the pool and over to the shade where we were sitting and it took us 15 minutes to get her calmed down.  I thought immediately that she has a seizure.  Once we got her calmed down she was lethargic and slept for about 45 minutes.  She was not her normally self for the rest of the day.  She was crabby and sleepy and just off.  She is normally super happy all the time.  She had another episode later that night at my Grandmother's party.

We ended calling her doctor her in MD, they told us that we didn't need to go to the ER but she needed to be seen when we got back.  They also told us that if she had another seizure, came out of it, and immediately had another one that we needed to go the nearest ER.  She was fine all day Sunday.  On Monday I called and got her an appointment with a neurologist who we went and saw on Wednesday.  We are now scheduled for a EEG on 7/12 and depending on what they find Riley might be put on anti-seizure medication.

She has not had another episode since last Sunday so hopefully it may have been a one time thing.  But only time will tell.

Sleep Study

I have been wanting to put up two post this week and just have not had the time.  So I am going to combine them into one here.

In March of 2010 Riley had a trach placed due to severe sleep apnea.  It was the only way we were able to get her to the point that we were able to get her home.  Because she had the trach meant that we had to have lots of medical equipment and a nurse at night.  We had to make sure that we had her suction machine, HMEs, suction catheters, oxygen available, saline, extra trachs, and several other things before we even left the house. Plus of course all her feeding supplies and normally baby stuff.  Needless to say it was not easy to go anywhere.  We were not able to go stay any were over night.  So we have been anxious to it is out. 

Over the last year she have slowly worked Riley up to wearing a passy muir valve from 1 minute all the way to all day.  A passy muir valve (speaking valve) is a one way valve that let Riley breath in through her trach but them she had to breath out through her nose and mouth.  This allowed the her to talk since the air would go through her vocal cord.  Once she was able to wear the passy muir valve all day we moved on a cap.  Which is actually like it sounds it is a solid cap that goes on the end of the trach.  When she is wearing it she is not using the trach at all.  She is breathing like you or me.  We worked slowly to get her to wear it all day.  Once she was able to do that it was time to repeat her sleep study and see if the trach could come out.

Last Friday (5/6) Riley went in for her capped sleep study.  This was a exhausting experience for both her and I.  I had to stay up all night due to need to suction her throughout the night.  Riley had to have tons of probes on her head and face, a nasal cannula in, pulse ox on, heart monitor on and two belts around her chest and abdomen.  How they actually expect anyone to sleep well with all of that on is beyond me.  So we get there at 8:30, Riley is normally asleep around 8-8:30.  By the time they get you check in and in the back it is 9, then they spend an hour putting all of this junk all over her.  Riley was not a happy camper, she was tired and screaming (which then meant that I need to suction her about ever 5 minutes).  By the time the tech is done hooking Ri up it in now 10.  Poor girl was DONE! So she is finally able to go to sleep but doesn't go to sleep until almost 11.  At about 12-12:30 the tech comes back in and puts Riley on some oxygen because her oxygen levels at dropping.  She sleep for awhile then at 1 she is up and doesn't go back to sleep until 3:30.  I was begging her at one point to go back to sleep, at least at this time she was not crying. So she finally goes back to sleep and then it is 5:30 and time for us to wake her up and leave.  Needless to say it was a long night. 

I was afraid that they were not going to have enough information to make a decision since she was up for most of the night.  The good news was that they didn't make me take the cap off all night.  I took that as a good sign.

We leave and go to my folks house who live 20 minutes from where we had to go for the test since my husband had to work the next day and I really really needed to sleep.  I go sleep the morning away and Riley hangs out with MomMom and Pop.

When we left they told us that it might be up to 14 days to get the results.  On Tuesday I get a call from Riley's pulmonologist that the results came in.  And Riley did great.  She was having drops in her oxygen level, but that can be treated with just having her on oxygen at night.  She goes on to say that she does not see any reason why Ri's trach can't come out.  My baby girl is going to be TRACH FREE!!!!!!!!!!!!  We are beyond the moon.  I am working with Riley's ENT to get in scheduled.  We do not have a date yet, but we are hoping that it will be soon.

In order to get the trach out Ri will need to be admitted to the hospital overnight for observation.  She will still also need oxygen, but it the long run it is going to be much easier travelling and going out with her.  We had planned to go to the beach this summer but were worried how it was going to work with trach, but now it will not be an issues.  My BIL just bought a house with a pool so Riley will be able to go swimming this summer.  This has opened so many more doors for us and we can't wait to walk through them.

I will keep you updated to when we are going.

Sign Language classes

So I am super excited about next week.  On Tuesday I start a 6 week sign language class that is offered at my work.  Ever since college I have wanted to take a sign language class.  I was never able to fit into my schedule.

I always planned on using baby sign language with my kids.  That has become even more important with Riley since she will be delayed in the language department and with her hearing loss.  I am really hoping that this class is going to be good, because I am looking forward to it so much. 

I will update about how it is going periodically.

Gait Trainer

My daugther amazes me everyday.

Kids with T18p don't normally start walking until two and half years old.  So because of that Riley gets PT once a week.  About a month ago her PT brought in a gait trainer.

A gait Trainer allows Riley to walk around even though she is unable to stand on her own currently.  At first she was able to move it backwards alittle bit, but it was not deliberate.  But on Sunday night we put her in it and she as moving forward all over the place.  I decided to see if I could get her to deliberately move forward.

So I grabbed her most favorite thing right now . . . bubbles and sat on floor.  And look what happened.

She moved it all the way across our KITCHEN.  I was sooo proud of her.  She is making great strides and it makes my heart soar every time I see her do something tI have never seen her do before.

Ri's hearing aids

So we have had Riley's hearing aids for about a week and a half now.  And I am amzed by how little they are and how much of a difference they seems to make.  Riley is playing with sounds more now and I think it is because she is hearing more sounds now.  They are pretty small.

She hates having them put in but once they are in she is totally fine with them.  They are not nearly as much work as I thought they would be.  She goes back on Wednesday to get her hearing tested to make sure that they are helping.  I will post an update after that appointment

Curious people

At first glance you may not realize that Riley is different.  Though when you take a second look, you would notice her trach, us suctioning her, or us giving her milk through her g-tube.  People's reaction to her some times drives me nuts.  I would rather have someone come up and ask what we are doing or whatever other question is on their mind about her then stare from across the room.  I am happy to educate someone about her or her condition, but don't stare. 

Parents are the funniest though.  kids are always so curious, so you will hear the child ash a question in a normal voice and the parent whispering an answer back.  I have no problem talking to people I don't know, I take after my dad, so I always tell the parent that is tis ok and habe them come over.  The normal question is "What is that?" while pointing at her trach.  I calmly explain that is how she breathsand the child responses with "oh, ok" and that is the end of it

It is natural to be curious about something that you have never seen before.  I would think that most parents of a special child would rather have you ask you question instead of staring at them.  That being said don't ask "What's wrong with her/him?"  There is nothing wrong with my child she just has some special needs, but I love her jsut the same as you love your "normal" child.

Hearing aids

On 2/7 Riley went for a MRI for a few reasons (1) her hydrocephalus, (2) the fact that she has a shunt, (3) to have a good look at the structures of her brain, and (4) to have a baseline if she has any issues down the line to compare too.  Since she was sedated for the procedure they did an auditory brainstem response test (ABR).  Which is a hearing test were they attach electrodes to her head and record the brain activity when different sounds are played.  We had this one done as oppose to a normal hearing test because we had tried one of those several months back but she was too young and did not understand that things made noise so she failed it.

The results we got back from the ABR were that she had moderate hearing loss.  She is hearing at a 50-60 decibels range.  To put that in perspective 20 decibels is a whisper, 120 decibels is a jet engine, normal speech is at 50 decibels. 

Since Ri is hearing just at speech level we decided to get her hearing aids.  So you may ask if she is hearing speech why get hearing aids.  For a couple of reasons: (1) if you ever get sick or get an ear infections you can loss 20 decibels in hearing so then she would not hear speech, (2) hearing aids will amplify what she is hearing and make it easier for her to understand speech, and (3) she will already have a speech delay due to her Tetrasomy 18p (T18p) so we thought that we would not put any more barriers in front of her. 

So yesterday I took her to get fitted for her behind-the-ear hearing aids (BTE).  I am amazed at how small they were and how light weight they are.  The audiologist took molds of the inside of Ri's ears and they will custom make the part that goes in her ear.  We go back in 3 weeks to pick them up and learn how to use and take care of them.  When we get them I will put up a picture of them.

Coming to terms with her diagnosis

When I found out I was pregnant I could not help but plan and dream of what my child's future was going to be. At 19 weeks we were told she had spinia bifida and we had to shift those dream alittle. So now maybe she won;t play sports and may walk a bit late, but for the most part should be able to lead a pretty normal life. We expected her to be in NICU for a couple of weeks then home. As those couple of weeks changed to a month then 4 months we realized that life would never be "normal".

A month after her birth we got the news that she had a chromosome abnormality*. I remember sitting in a conference room near the NICU surrounded by doctors, my husband and parents on my right and my daugther's primary nurse, Lana, on my left. The genetic counselor telling us there was a problem with her genetic code and that she would have mental retardation and probable would never be able to take care of herself or live on her own. My world came crashing down around me. I burst into tears and heard nothing else from the rest of the meeting.  How could my prefect baby have much a terrible future?  She was suppose to go to college, meet a nice man, get married, and give me grandbabies.  Not being living with me for her life.  I was angry at the doctors, at the world, at God.  My friends all had this perfectly health little babies.  Why were their babies fine and mine wasn't?  I had to moron the loss of all of those dreams. 

When we first got her home some times it felt more like we were her caretakers instead of parents.  There were lots of doctor's appointment to go to, our home was invaded by nurses at night, there were all kinds of medical equpiment that now took over our cute nursery.  Now though I feel more like a parent that just happens to know alot about Riley's medical conditions.  People tell me that I could be a nurse with my knowledge, I turn in tell that I only know alot about a little.  Mind we still have lots of doctors that we see, but it is less often.  And the nurses are still here every night, but they are more like family then stangers.  The medical equipment, well that is still a big part of Riley's room but it is normal now. 

Riley is now 15 months old and I have accepted her.  There are still times when I am talk to my friends about their kids or we see a another child that is Riley age or younger doing things that she can't do yet and I get sad. Then I tell myself that I can't compare Riley to all kids.  She will reach her milestones in time and when she does it will mean even more to us then if she was like my friend's children.

It is weird how your definition of normal changes.  We recently found out that Riley has moderate hearing loss and will need hearing aids, but it in the long run of her short little live it seems like nothing.  I mean I just have to makes sure she wears them and they are batteries.  Easy Peasy.

But this smile makes it all worth it:

*PS I hate that term "chromosome abnormality" she may not be exactly the same as a "normal" child but which child is. Unless you are an identical twin no one chromosomes are exactly the same. So how can we really define normal. Riley is going to be who she is going to be. She will have her own definition of normal.

Riley's Story

When we found out that we were pregnant my husband (Kyle) and I were soo excited. We had dreams for our child and hopes for what they would be. We choose not to find out if we were having a boy of girl. And had a lot of fun tormenting our family and friends.

At 19 weeks we went for a standard ultasound and not had a not so standard appointment. We found that our little bundle of joy has spinia bifida. This is when the baby's spinal column does not close completely early in development. So this meant that we would now be followed very closely. They were also worried about the baby's size, which was about 3 week behind where it should be. So this brought on ultasounds every 3 weeks, visits to the high risk OB/GYN, a change on the deliver hospital, visit to a cardiologist when they could not see the heart well enough, and stress test every couple of days for the last month of the pregnancy. Needless to say it was not stressful 9 months.

They finally diagnosed the baby with Failure to Thrive, which required me be induced on 12/9/09. My labor failed to progress and I had a C-section. So after 46.5 hours of labor my beautiful baby girl Riley Ann made her entrance into the world.

Riley spent the first 4 and half months of her life in the NICU. Before she left the hospital she would have 7 surgeries, and 7 different diagnosis.

Surgeries:
meningomyelocele closure
VP shunt placed
VP shunt revision
G-tube and Nissen placed
Tendon release (local anesthetic)
Tongue/lip adhesion (TLA)
Trach placed and TLA released

Diagnosis:
Fetal Myelomeningocele
Arnold-Chiari Malformation
Obstructive Sleep Apnea
GERD (Gastroesophageal Reflux Disease)
Clubfoot, bilateral
Spina Bifida with Hydrocephalus
Tetrasomy 18p

Since leaving the hospital we have added 2 more surgeries and 3 more diagnosis. She wear AFOs (ankle foot orthotics) during the day and Ponsetti shoes (which are two shoes that are attached to each other with a bar) at night to correct her clubbed feet. In September 2010 we found out that she also have central apnea so now she recieved oxygen at night.

You would think that a kid that has been thorugh as much as she has in such a short time would be a cranky kid. But that is that is the exact opposite of what she is. She is the happiest, sweetiest thing you will ever meet. She wakes up almost every morning with a smile on her face and it brings joy to me heart. So our dreams for Riley may have had to change, and we may to teach the world about her just as much as we have to teach her about the world. But day by day and smile by smile she is teaching us alot too. Until next time folks..