Home Away from Home!

Things were going well at home.  We were getting into the rthythm of being home with two children and trying to meet each of their own unique needs.  Isla was beginning to have a hard time adjusting to less attention and trying to understand Cohen’s needs and special equipment.  Cohen was doing great with his new and improved high-flow system and was breathing so much easier and QUIETER!!  Last Thursday we had his pediatrician check-up and he was gaining good weight as well, a little under 9 pounds.  His pediatrician said once he gained to being 9 pounds, he would be able to increase his rate of his feeding tube by 1 ml!! It’s the little things we celebrate these days.

On Thursday afternoon we noticed his gtube was very loose and looked to be almost falling out. Sometimes this happens with them because the balloon inside the stomach tends to get deflated.  But because he was due for a gtube switch anyway, we took him into the surgery clinic on Friday and replaced that. 

Over the weekend things began to change again.  Saturday marked his 9^th day at home and we were eagerly anticipating it but also dreading it because this was the day that always marked our return to the ER, return to the hospital, and return to our other lifestyle.  On Saturday night he had three desaturations while on his high flow oxygen.  Usually we aren’t too worried when the alarm sounds on his monitor, but because he was dead asleep we knew that these were true desaturations.  He only went into the low 90’s but this seemed like something had changed in his body because he was on the high flow oxygen support so that he would not desaturate.  The rest of the night was peaceful and he slept without sending off the monitors. 

On Monday morning he was very frustrated around 4am and we could not get him to calm down. He ended up spitting up a few times and began choking on his spit up.  As most newborns spit up he was performing like any newborn….but because he was on high flow he had air pushing his spit up very quickly back down. I rushed to turn down his oxygen until he was finished but he sounded very flemy afterwards.  I stopped his feed and called his pediatrician.  She suggested we give him some time to calm his breathing down, since it had raised into the 80’s and then try to start his food again.  I did this and he was fine all morning until the afternoon when he spit up again. 

Again on Tuesday morning he spit up.  We had three doctor appointments scheduled for the day, a renal ultrasound to check the fluid in his kidneys, a genetic appointment to ask questions about his diagnosis and a swallow study to see if he was ready to take food by a bottle.  Even though he seemed fussy we had to get this kid ready and out the door by 7:45am.  As it is always a process getting any child out the door, Cohen just comes with some extra equipment and extra steps.  We were in the car and ready to leave, having only driven about 100 feet from our driveway and his monitor began going off in the car.  His oxygen was showing 85% and his heart rate was down to around 90.  Both of these numbers were extremely low for him.  Ryan pulled over and I jumped out of the car to reach him better in the backseat. He was spitting up again but seemed like he was choking on it.  The air was pushing it down and he was trying to cough it up.  His eyes were looking at me screaming, "HELP!!"  We turned the car around because it was so cold and running with him down the street was not an option.  We raced him, his oxygen tank, and monitor into the house but trying to act as calm as possible in front of Isla, Caroline (another little girl who comes over often, and our nanny Kelli.) They were all sitting down to their breakfast when we rushed in the door. I called my nanny over to see what she thought and her face showed me exactly how she felt.  He was blue and his numbers were not looking good.  I looked at Ryan and calmly said, "Call 911" as if I was asking him for a glass of water, but really my eyes were screaming at him, trying to mask my worry from Isla.  

The ambulance ride was all a blur but by the time we got to the hospital he was looking a lot better.  They did their usual routine of sauntering into our ER room and asking questions, checking medical history, taking vitals...etc.  For some reason the moment we stepped foot into the ER, with the waxed tile floors, and plain white hallways, we felt at ease...at home.  We know this is not our home, but it has practically become our second home since November and because he has spent more time here than at our actual home, we feel safe here.  He has everyone watching out for him, a personal nurse to care for him, and we have time to breathe!  Ryan and I joke about how we shouldn't even have to pay our mortgage at this point because we only sleep there..only if that were possible!!

He had a chest xray ordered and a blood gas to check his usual areas of concern.  The blood gas came back great, showing relatively normal amounts of CO2 which has always been a big concern for him.  The chest xray showed to be a bit hazy and his stomach looked very enlarged, probably due to the high flow he had been receiving.  They also performed a gtube study because my thoughts were that ever since his gtube was changed out he was having desaturations at night and spit ups, though I guess it could be a coincidence.  The gtube showed regular placement, of course and we were admitted back up to the PICU with our blue wristbands.  

During this past stay at home, Ryan and I had vowed to not cut off our trusty, wrinkled blue wristbands out of superstition.  We had done so before and each time ended up back in the hospital, so why not try something new.  He has been on the PICU floor now for two days and is breathing very rapidly again, similar to how he has been coming in in the past. He is still on strictly IV fluids until they can get a better sense as to why his breathing is so fast again. I'm not a doctor, as I've said before, but lets see....the kid spit up and was choking, probably aspirated some and now his lungs need time to heal....hmmm..

It's another waiting game and I'm just hoping it won't be another three week stay and will have minimally invasive tactics necessary to get him home again.  But this place is all Cohen knows as home.

Home Again...this time for good!

We are finally home for the third time in Cohen's short life and feeling more confident that this time will actually be the permanent one..or at least for a bit!  After a few more days in the PICU the slew of doctors were able to stabilize his CO2 levels with a higher flow of oxygen.  They were thinking that the reason he ended up back at the hospital twice was because while he slept his apnea caused his CO2 levels to build in his body which is why the think he was breathing so hard toward the end of our stays at home.  By having him on a higher level of flow, it would hopefully train his body to build up oxygen levels in his body so he would have them on "reserve" for when he was awake and hopefully we would not need to return as soon.  But with this new form of treatment, would again mean more appliances to fill up our living room.  He would need a high flow system  that would supply a faster flow of oxygen but also humidified air so that he could tolerate the higher flow.

As much as we were excited that this was something they found that worked, I was also annoyed because he really didn't seem to need the oxygen as much as the flow; however, they did not quite have a high flow system for the home yet so this is what needed to happen.  

Based on this new flow, they wanted to see how it would affect his sleep apnea and to make sure nothing else would come apparent when at home, so we performed one more sleep study.  Our poor little man had already been through two and would now need to be covered in wires once again, but hopefully the risks would outweigh the benefits and it would give us extra information.  But of course...in true fashion, Cohen showed no more changes, which we were happy with.

Last Thursday (we've been home for 6 days now and trying to get past our popular 9 day home stay) we were discharged from the hospital for the third and hopefully FINAL time for a while at least!  It was again a traumatizing experience getting this fragile man to and from the hospital and then setting up the equipment that he would need for the high flow system (shown above).  But he must have felt bad for us because as we finally settled into bed around 11:30pm, he slept all night!  

The past 6 days have been so relieving and comforting, even though often chaotic!  We needed this break at home to spend time as a family, make memories together and enjoy each others' company.  We have cuddled, snuggled, kissed, hugged, cried and cried some more!  

Tears of happiness to finally start our life together, but also tears of fear and sadness of the diagnosis that has still not been given and the fate of our child.  We are technically homebound right now since he is on this new system, but have approval from his doctors to venture out of the house a few hours/day on a regular oxygen tank to go to doctor appointments.  This has been the hardest part in all of this.  Living in a new home with two stories (Ryan's dream come true) and not even able to share experiences together as a family because one of our children is hooked up to two machines upstairs is very upsetting.  We have been trying to make the best of it though and are looking into getting a second system and trying to get insurance to cover it for downstairs so we can have a bit of normalcy if we do have to stay indoors for the short term.  Trying to explain to Isla and her spunky two-year-old mind that going downstairs is not an option all day long is frustrating and upsetting.  We take turns going downstairs with her because she really does love playing down there and are working on a schedule for that.

Looking forward to his pediatrician appointment tomorrow and hoping for good weight gain!

New Wristband...Blue is the New Purple!

Of course Momma's intuition was correct and this boy passed the PH probe with flying colors!  In fact, he seems to be super NORMAL and showed no indication of having any reflux so we can finally get him off of those meds and also rule out reflux as a reason to his breathing.  

After finally putting that to rest, docs still had to dig deeper to figure out why he breaths the way he does.  The pulminology team came by and decided to try him on room air without oxygen to see if he would change at all.  Within a few minutes, he began desaturating into the 70's but was not even showing signs this was happening.  He, like always, was not turning blue or gasping for air, but then would steadily increase his breathing again and be back to normal in a few seconds.  

The team had a new concern.  What if this was something that was not controlling the CO2 and O2 levels in his body, meaning his brain.  Your autonomic nervous system (fight or flight responses) control and regulate how your body does many different things including regulation of breathing.  Sometimes, very rarely, there are people that do not have this part of their brain working properly.  They cannot regulate these things and in end can die without knowing it's happening.  The genetic disorder is called Congenital Central Hypoventilation Syndrome.  This is a very rare genetic disorder that has only 200 live cases currently in the world.  This syndrome has no cure and is life threatening if not detected because people that have it need to be on a ventilator while sleeping.  We are hopeful it is not this, but if it is we will learn to take care of him as best we can.  The blood test takes about two weeks to get back so right now it's just a waiting game. But the doctors are convinced that we are really reaching for an answer right now and they are fairly confident that he will not have this diagnosis because he is a rapid breather not a slow breather. 

At the same time we have been pushing all along to get an MRI of Cohen's brain since that is the only other place we have yet to look into and really want to rule it out.  There was one research study found online that involved a child that had 22Q and rapid breathing.  The breathing was caused by compression of the spinal cord and was associated somewhat with the brain stem.  The child underwent neurosurgery and was a slower breather after that.  Even though it is traumatizing each time Cohen has gone under anesthesia, it has been important each time to rule out things that could be causing him to breathe the way he does.  We decided it was best to do this because otherwise we would always wonder if that was the area that was causing all of these issues.  He was a trooper and again recovered rather easily from the anesthesia and the MRI was NEGATIVE!!  

With having to wait 2 weeks for the results of the other labs, the team that has been working with us finally scheduled a Care Conference on Friday for us to all get together, discuss his progress, and plan for our trip home that would hopefully be sooner rather than later.  As Ryan and I were only anticipating a few doctors, we walked into a room FULL of people that have at one point in time seen Cohen.  A social worker, parent liaison, a member of the ENT team that did the first bronchoscopy on Cohen, our nurse, the head resident checking in on us this week, the Pulminology fellow, the floor manager, a charge nurse, sleep medicine doctor, and his pediatrician. We felt LOVED!  Even though we have had our ups and downs here over the past 8 weeks on and off, this truly showed us that we were being well cared for and Cohen was on everyone's mind.  

During the meeting we discussed where we have been, the variety and extensive work up on Cohen, and future plans for him.  The pulminology team really wanted him to try the high flow (not necessarily even with oxygen) to see if the flow would make a difference in his breathing.  The only way to try this would be to transfer down to the PICU (pediatric ICU) where they were allowed to have high flow nasal cannula. They would continue to work with the doctors down below and keep close tabs on him, but this would be helpful in trying to figure out the best plan for going home.   We would be sad to leave our huge support system but wanted to make every last attempt during this hospitalization to figure out the best and safest plan to go home with at this point.  

They transferred us down there by mid-afternoon and we traded in our purple wristbands for shiny new blue ones.  Once again we were settling into a new room and answering every question possible about his medical history and previous hospitalizations.  I felt like it was Groundhog's Day once again!  They were checking his "work of breathing" and asking us what his "baseline" breathing looked like.  It just seemed like we kept going through this cycle and yet still no answers.

Over the past day or so Cohen has been doing great in the PICU...still breathing about the same but they have been experimenting with different levels of flow and oxygen to try to see what is best for his breathing...hopefully they'll figure it out soon!

Bunch of little problems create a big problem...or just time?

Yesterday resulted in both good and bad news.  Cohen had two procedures to again rule out any other crazy business he had going on in his lungs.   This was also the second time he has been put under anesthesia in his short little lifespan.  The pulminology doctors performed another bronchoscopy but this time using a more flexible scope to look deeper into his airway, even venturing into the different sections of his lungs. They also squirted in saline and then extracted it to be able to process it in labs for any bacteria or to show proof of aspiration.  Meanwhile he also had a CT lung scan.  This would help the doctors look more closely at his lung tissue and to see how the air exchange was being processed.

I waited in the waiting room for what seemed like an eternity, ideas passing through my mind continuously as to why we were doing another procedure and still about to have no answers, because I know this always happened.  The two PULM doctors came out after about an hour with smiles on their faces.  This told me that he was doing well, but also that they had probably not found anything.  And I was correct.  Once again our Mystery Man had proven us and the docs wrong.  They had found some mild bronchial malasia but nothing that would indicate this rapid and noisy breathing.  They also found that he had a anatomical duplication of two lobes in his lungs but again nothing that would cause this breathing.

We were back to the beginning yet again with no answers!  How could this be happening AGAIN???  The pulminologists looked stumped.  Another set of specialists that were completely mind-boggled by my son.  They explained what they felt to be his diagnosis...at this point..a little bit of everything can make a BIG problem like breathing quickly.

-He has a little hole in his heart that may not ever need surgery.
-He has a little reflux.
-He has a little bronchial malasia.
-He has a little sleep apnea.

All of these things very well could be coming together to create a rather large problem.  A problem that has been with us since day 1.  The more we looked around, the less we found and maybe because it was in front of us the entire time.  We have been trying to look for this HUGE discovery, but maybe there never would be that huge discovery because there wasn't even one to find.

After the disappointing yet happy news, the docs started talking again about discharge...though within a few hours, they had completely changed their opinions, wanting to rule a few more things out to be safe, before we went home.  They ran one more sleep study to make sure they hadn't missed anything from about a month ago and then they also decided they would consider measuring the acidity of his stomach acid by performing a PH probe study.  In order to do this though, they would need to take him off of his acid blocking medicine for 72 hours to make sure that the study was accurate without any meds affecting the outcome.

The sleep study went well, if you can say that since Cohen screamed on and off most of the night.  We are still waiting the results from that.  He had his last dose of medicine yesterday afternoon. Now I expressed my concerns to the resident because taking a kid off of reflux meds who seemed to really need them made me very nervous. I know they needed to do it for the study, but part of me was wondering if he was going to be in agony refluxing continuously with nothing to protect him.

So when I came to the hospital this morning, I was expecting there to be mayhem with this little guy.  He had been off his meds for over 12 hours so I knew I was going to see throwing up, lots of coughing/gagging sounds, painful cries and some desaturations in his oxygen.  As I'm writing this though, it is 8pm, almost 18 hours after being off all meds, and the child doesn't seem any different.  Hmmmmm..... you may be saying to yourself as I have done all day in my head.  But why?  Well as I was wondering the same thing, I decided to start looking back at my spreadsheet of Cohen's medical history.  This wonderful ideas was given to me by a co-worker (Jessica :)) who told me when things were still very uncertain, to keep a log of his daily events to try to work out solutions from that.

When I did explore back over the notes, about 10 pages worth at this point, I found the point in his stay when we had returned to the hospital the first time.  I had increased his feeds and the reason I brought him in was simply because he had been breathing harder.  He had never thrown up at home or shown any more signs of refluxing than previously before.  We started his feeds back up and he continued to have desaturations of his oxygen, over and over again.  We decided to increase his med dose and add 2 more meds as well as change his food.  I can remember back then that even after he had been on these meds for about 1 week he was still having desaturations, making docs and us think he wasn't tolerating the feeds.  The interesting thing was though that his breathing remained the same and he still hadn't been throwing up like a typical refluxing baby.  But as I read my spreadsheet something jumped off the page.  All of these changes had been made prior to us getting the sleep study which determined he had sleep apnea and thus explaining the desaturations.  Now push forward about a month.  He is being treated for his apnea via the oxygen at night and no longer has desaturations.  If my theory was correct, then this boy didn't even have that extreme of reflux as we had thought and this may be just a matter of time...for him to grow...develop...and breathe more easy.   If that is the case, I feel terrible for all that I have put him through but at the same time these past months have taught me that medicine is all about finding patterns and coming to a consensus by eliminating possible things.  We will know in another day whether or not we will even need to go on with the ph probe and maybe the point of this visit was to get off the reflux meds because he may not even need them.  I would be very excited to think that was the case but we will see.

But please slap me tomorrow if he completely changes overnight and proves me wrong.  He has done it many times before!

Anything Else?

Today was another productive and eventful day.  It was an argument between nurses and doctors over Cohen's oxygen or room-air flow.  Upon entering his room this morning we were advised that he was now going to receive 2 liters of flow continuously to help prop open his airway to treat his sleep apnea.  The talk around the hospital today was whether or not he needs the actual oxygen or just needs the airflow to keep open his airway.

The sleep doctor, who reminds me of who Mr. Sandman from the song would look like if you could visualize him (thick dark beard, soft spoken, and wears a bow-tie) came to pay us another visit.  He felt very strongly that Cohen only needed the flow because he has never needed the actual oxygen to bring him out of an apneic spell.  With this being said, the only problem is that the nurses protocol did not allow them to use only room air because that is something that can not technically be managed at home.  The current machines tend to be very large and do not travel easily, though he did say that they have since come out with a machine in New Zealand that will be available in March.  Cohen could be one of the first to possibly use it!  Through the day he seemed to be so much more peaceful.  His respiratory rate was the lowest I had ever seen and he was not breathing as noisily.  It just could be that all the little guy needed was a little more flow moving into him, but I wasn't completely convinced.

They were able to override the usual protocol for this floor and kept him on the room air for the remainder of the day.  We also were able to increase his feeds to 25ml/hour to allow us for more breaks at home.

Meanwhile the new idea on the table currently was whether or not we should have another look inside his airway one more time prior to being discharged just to make sure we are not missing ANYTHING!  They will be performing another bronchoscopy but this time the pulminology team will be performing the procedure with a more flexible scope to really go down deep and look at the anatomy of Cohen's airway and lungs.  It is possible they could find nothing but also very possible they could find something that is causing all of this drama with his breathing.  They will also be performing a CT lung scan to monitor how his lungs are filtrating the oxygen.  On top of that they want to extract any liquids from the lungs and send them off to the lab to check acid levels to finally determine whether or not Cohen has aspirated in the past.  All of this will be done hopefully in the realm of a few hours under anesthesia and hopefully we will walk away with more answers after putting our little guy through more procedures.

 As much as I am worried to agree to have him go under anesthesia again, at the same time there has always been a big part of me that thinks we have been missing something, that the doctors were missing something and just treating symptoms that have continued to return.  He seemed to have reflux so we treated it with medicine and he improved.  But we have never been able to calm down his breathing or determine why he does better on his tummy or sleeping on his side.  When Cohen received his last bronchoscopy, the ENT surgeons not finding anything was exciting, but also a let down.  We thought we were going to walk away with answers and reasons to why he breathed the way he did, but we didn't.  Throughout this entire process doctors have been convinced and sometimes had us convinced that they were about to find "the answer."  But every time, there was no answer.  There has been no light at the end of the tunnel on each of our discharges and I've known deep down that we were not bringing back a completely "figured out" little man.  I'm excited about tomorrow and what they may find and hopefully it won't be anything that we are not equipped to handle.  I'm also excited that we may finally get peace of mind that there really isn't anything else we are missing and we can sleep soundly at home hopefully for good!

Please keep positive vibes or prayers (whichever you prefer) flying our way tomorrow throughout the day.  We are not sure when the procedures will take place but will try to keep everyone updated as much as possible.

Too Many Cooks in the Kitchen, but one amazing nurse!

Today was the day I finally took a stand!  I know I have said in the past that I had to get tough with the docs, ask the hard questions, and demand answers!  Well today I finally felt like I actually did that. Before I would ask questions, but do it through sobbing tears, showing my vulnerability to the "head honchos." Today that all changed...I was one PISSED MAMA, but I can only attribute that to one amazing nurse!

After last night resulted in lack of sleep for Cohen and I due to the ineffectiveness of the three medication trials, we were basically back to the beginning again and still no answers.

We have had some rather crappy nurses, but for the most part we have been blessed with some amazing nurses. These nurses are the ones that bring comfort to you even on the most eventful days or uneventful days.  They know the right things to say when you are balling your eyes out with no answers.  They also are dedicated and will do whatever it takes to make sure that your questions are answered and that you meet with the people you need to meet with and not to feel embarrassed about it.

Today we were blessed to have our favorite nurse thus far in this entire experience, Sarah.  Sarah is a 26 year old nurse who is wise past her years.  She first had Cohen way back during the beginning of his second hospitalization and I could tell based on all she said and did, that she was not only an amazing person but great at her job!  She has been with us through the ups and downs and always made us feel like we were not asking dumb questions and also explaining each step, procedure, medicine, along this crazy process.  I remember one time she called me at home to explain the salivagram procedure he was going to have because she wanted to make sure I knew about it even though I wasn't able to be at the hospital during that time.  We have vented our frustrations to her regarding doctors, other nurses, etc and she has always been very professional about it.

Walking in today to see her in Cohen's room, I felt at ease but also felt like this was going to be a very productive day!  And it was!  After having discussions with her during her in/out visits into our room I was able to voice my concerns about: the lack of communication amongst the professionals seeing Cohen, the terrible night nurses we have encountered on a few nights, the terrible contacted oxygen provider we had been given through the hospital and many other issues.

Instead of many of the nurses trying to please their patients but also keep the peace in their job setting, she took action and I am forever thankful for that.  Throughout the day, people continued to filtrate in and out for various things I had addressed with her and it seemed like many more questions were answered than before and even though we are still unknowing of Cohen's condition, I felt at ease.

I met with the hospital case manager and addressed my concerns about our oxygen provider and how they have been nothing but terrible in their communication and customer service (that could be another blog in itself).

I met with the floor manager to address my concerns about the lack of diligence in some of the night nurses we have had before (really only a few but of course the negative experiences tend to stick more than the positive at this point).

I met with the pulminology team and the ENT team separately and felt completely lost afterwards.  They were both coming from two different angles and often leaving it up to the other specialty to make the call as to what the next steps would be.  We were back to blaming aspiration and reflux, even though GI had just said that they didn't believe this was due to that even happening. I was becoming more frustrated as some of the doctors were talking about not even wanting to do anything more to find out why he is breathing the way he is breathing, but yet we were still stuck here in the hospital!  I asked her what she thought about gathering a grand meeting with all of the specialists involved so that the communication could be strengthened, myths or theories could either be put to rest or tested, and we could try to come to an understanding because we were talking about an 8 week old that has only seen his home walls for 18 days of his life!  She heard me loud and clear and approached the resident who is nowcurrently working on making that meeting happen hopefully this week!

I am so thankful for her and even though there are many cooks in the kitchen I wish we could always have this one special nurse on the sidelines cheering for us!

More Meds...Guinea Pig!

Later on that evening Ryan went back into the hospital for his evening shift.  He had told his nurse about how when we took Cohen home he seemed very fussy and uncomfortable to due possible gas.  With g-tubes it is often that children can become gassy and the only way for them to relieve that gas is to "burp" them.  She suggested we do this with using a Farrel Bag.  Using gravity it looks like an IV bag that is hooked up to Cohen's gtube site and then when he needs to release gas it naturally pushes some of the formula back out causing him to burp.  He had a relatively mellow evening.

The following morning we met with the Pulminology team to get their insight into why we were back here again and why his breathing continues to be an issue.  They feel that even though we had the bronchoscopy earlier on (Dec) it seemed to show that his airway was clear; however, he could have possibly developed some bronchial malacia over time or there could've still been something that they had missed.  Since they said ENT didn't use a flexible scope, there could be a chance that the airway was just propped open, not allowing any anatomy concerns to be as visible.  They also thought that he could have this "floppy airway" farther down that where ENT had traveled.  Before putting him through another procedure and another incident of having to go under anesthesia, they decided that they would try two different types of medication they would give to asthmatic patients to see if that would stabilize his breathing more.

Later that day he received his first dose of albuterol.  In general, Ryan said he was a very ALERT baby, possibly due to the albuterol.  He was the most interactive Ryan had seen and was Mr. Smiles! We were pleasantly relieved and excited about this new plan from pulminology. If this was the reason as to why we were back in for another stay, we were happy to have more answers!!  The doctors seemed confident and we were all on the same page.  If this plan worked, then Cohen would be coming home with another med, but it would only need to be taken when his breathing was getting out of control and then we would not be having to come back to the hospital each time this happened.

Meanwhile the cardiologist has always adamantly stood by the fact that he does not need any heart medication (lasix) to help remove fluid.  He doesn't think his breathing is being affected by the small size of his VSD.  With this being said, he was pressured into trying out a trial run of lasix to rule it out, just because we are at the point now where having only spent 18 days at home is kind of ridiculous!!  We would also experiment with this to see if it had any improvement on his breathing.

So we gave it a try....and not surprisingly, they did not work! The poor little guy was WIDE AWAKE all last night to the point where he only slept for 2 hours due to the albuterol.  I understand that we are trying to figure out the cause to this breathing and how to best treat it but sometimes I wonder if our baby boy is the guinea pig to some doctor's ideas.