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/

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.

Looking back

I was going to post this yesterday but the day got away from me.

Riley came home from the NICU on 4/21/10.  She has been home for a whole year.  I was looking back at photos of her in the NICU and thought I would share some of them with you.

12/11/09- this is right after is was born

12/13/09 - This is our 1st family photo

12/17/09 - She was jaundice, she had to be treated with the lights

 2/11/11- She was on and off oxygen throughout

our 4 and half month stay in the NICU

4/10/10 - 11 days before coming home

4/21/10 - GOING HOME DAY!!!

We loved the nurses in the NICU but were soooooo happy to say goodbye to them. 

Look how far she has come.  This is from this past month.  It was the first time she rode in the cart.

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.

Why don't we have to take a test to be parents

So if you have read Riley's story you will know that she spent 4.5 months in the NICU. The NICU is a very interesting place to be for any period of time. You don't realise that you are holding your breath and how on edge of your seat you are in until you walk out of the NICU. If I never have to hear the beeping and alarming of the monitors in there it will be too soon. Riley is our only child, so one or both of us were at the NICU everyday, most days more than once. We only live 20-30 minutes away. The only days we were not there were during the terrible snow storms of 2009/2010 and even than we tried ad made it on several of days during the worst of it. The other time was when we had to go out of town for a wedding and then we made arrangements to have someone come see her every day. My mother also went most days as well.

Riley was in a hospital that had a very large NICU, it had upwards of 45 babies at a time. In comparsion a friend's son was in the NICU in a differnt state for 6 weeks that only had 10-15 babies. In the room my daugther was in had 12 babies in it. I was amazed at how many of the babies had no vistors at all. I understand that people hjave other children that may keep them away or maybe they lived far away. And we were there alot, but all day everyday. But we made friends with the nurses and know that some of the babies where never visited at all.

Now I am the first person to say that the NICU is a stressful place and I get that everone deals with stress in different ways. But there was a primee that was there before Riley was born and was still there when we left. And I never saw a parent, a grandparent, no one. This poor child jsut layed in bed all day everyday. The nurses would pick him up when they could, but they could not just sit next to him and talk to him, or hold his hand, or tell him that they loved him. I just don't get that. How selfish do you have to be to never visit your sick child? It killed me that days that I could not get into the hospital to see Riley, on those days I would cell the NICU severla times a day to check on her. But not seeing her for month... Can someone explain it to me please?

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..