Still in Limboland

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

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

Limboland

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Then we got the most recent results….

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

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

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

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

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

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

Return of the apnea

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

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

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

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

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

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

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

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

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

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.

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/

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.