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.

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.

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

D-Day

We have recieved word that July 6th will be decannualization day for Riley.  She will be admitted into the hospital overnight.  We go Thursday to meet with her ENT and get all the details about how it will go.  But we are all very excited and can't wait to see Riley with our her trach.

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.

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.

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..