Saturday, March 29, 2014

Vacation Time!

It has been 4 glorious days of vacation up on the 21st floor.  The hallways are empty and quiet, darker than the PICU, and less hustle and bustle which is exactly what we all needed. Now only if Ryan and I could be laying out at a pool and sipping frosty drinks with umbrella we would ALL be happy campers, but for now that can only be a distant dream.

Cohen, aka: Mystery Man, for the most part has been smooth sailing upstairs.  His old nurses and attending doctors welcomed him back with open arms and all took turns coming to see him.  They could believe he was back, but couldn't believe the path he took.  No one had anticipated his health and needs changing so drastically.  Most everyone was impressed with how big he was, even to us he still looks so small, there have been notable changes and that was good to hear!

The goal for our vacation upstairs was to fine tune Cohen so he would be a safe and strong engine we could eventually take home for good. The doctors wanted to work on continuing to wean him from his narcotics, increase his feeds to reach his caloric intake goals, as well as try to calculate his true oxygen needs.  Weaning off the narcotics has been a slow and steady process for Cohen.  He has really shown to be very sensitive in general and even this has shown to be true throughout this process.  Next Friday it has been three weeks since he has been off the ventilator and he is still feeling the effects of the medicines. As for now Cohen still has pretty significant symptoms of withdrawal so we are trying to space out when he receives his medication, day by day.

Last night he had his fourth sleep study.  This is right after he was finished with it and still has the icky hair to prove it!

Once again his body was covered from head to toe in wires and probes to monitor every movement, breathing, co2 levels, rapid eye movement, etc.  Cohen held his own for about 15 minutes with no oxygen requirements, but then dipped his oxygen sats to the 80's so they slowly increased his oxygen and was very content on 1.5 liters.  This seemed to be his "sweet spot" and he was able to maintain 100% oxygen saturation, while breathing comfortably and having lowered co2 levels.  Even though this is a slight increase to his oxygen needs from our 2nd discharge from the hospital (only needing 1/8 of a liter while sleeping), it was a HUGE improvement from the previous discharge (needing 4 liters of high flow 24/7).  They are still planning to work out whether or not he truly needs to be on oxygen during the day as well, but we are happy with not needing to travel home with HIGH FLOW.  No more humidified water rushing through his nasal cannula for him to choke on.  He will now only be hooked up to a pulsometer, feeding pump, and oxygen concentrator.  I'm also working with the doctors and our insurance company to hopefully get approval for another oxygen concentrator for downstairs, so he can venture down to his room for the first time one day.

Besides from all of that, even in the confines of his small hospital room on his vacation Cohen is developing remarkably for everything he has already endured.  He now has a state-of-the-art toy string that hangs across his hospital crib where he can work on batting, swatting, grabbing, and everything else a 4 month old should be doing.

He is now sticking out his tongue and smiling on a regular basis (even while dreaming)

 and has recently found his mouth with his hands.  These are such huge accomplishments for him!

Friday, March 21, 2014

Vacay to the 21st floor postponed!

Plan B was set in motion and Cohen was supposed to travel in his baby throne upstairs to The Floor.  In hospital terms when ICU nurses or docs talk about "the floor" they are talking about the less intensive hospital floor where the ratio of patients to nurses is 5 to 1.  Everyone was very excited last Friday and expecting him to vacay on the 21st floor on Monday for a few weeks until he was able to gain weight and be more stable and comfortable for transporting back home. 

Well as we all know by now, Cohen doesn't always exactly like to follow THE PLAN.  Over the weekend he became increasingly irritable, upset, and every other synonym you could possibly think of for this. He wasn't sleeping, was unable to do any type of physical therapy and even his regular nurses were commenting on how different of a kid he was.  We had been increasing his feeds and changing his formula, as well as still trying to wean him from Methadone and Ativan.  He was dry-heaving every few hours even though we were no longer feeding him from his stomach and he was unable to be alert and happy at the same time. It was hard to say what was causing this change but we continued to bring up this drastic change during rounds each morning and doctors were hesitant to look deeper.  Finally on Tuesday morning, they agreed maybe we should get an x-ray of his abdomen since it seemed to be GI related.

The x-ray showed that he definitely had some stool in his bowl and possible air (Pneumatosis).  This was quite worrisome to us but also at least would help answer why he had been so upset for the past few days.  GI and Pediatric Surgery came to consult and decided to go NPO (no food) for 48 hours.  They would need to monitor his bowel with many x-rays and antibiotics to make sure it did not persist and improve or it could lead to further complications for Cohen.Pneumatosis was common in kids with feeding issues, jtubes, but also could be a sign of something else that was wrong and we needed to rule this out.

I was upset, outraged, and worried.  This little guy could never catch a break!  His only job right now was to sit tight and pack on the lbs and he couldn't even do that.  If it wasn't for an amazing nutritionist, Laura, who follows Cohen, I would have been riding solo arguing with the doctors over whether or not we could start TPN (Total Parenteral Nutrition).  For even a few hours it was sabotage to Cohen to hold his food.  If we had to hold off on feeding him through his stomach or intestines, at least he could get something through his veins to help combat the lack of food.

Yesterday they did a J-tube study to make sure that his jtube was properly positioned and not causing any of this new discomfort.  They had found a kink in his jtube causing the formula to be pushed back into his stomach slightly.  They switched it out for a new one and are hopeful this was partially the cause to his grumpiness.

Fast forward a day and Cohen is back to his happy self.  He is smiling daily.  With an empty belly he seems to have stopped the dry heaving, is less fussy, and doing what babies should be doing: smiling, cooing, and flailing appendages.  The doctors took close note of these improved changes.  Today during rounds he has passed the 48 hour mark and was able to start chowing down on some fortified formula :)  We agreed as a team that we only change one thing at a time to rule out the cause to his fussiness.  We would start him back on the same formula but at a very slow rate and work him back up to his normal goal.  We also would hold off from weaning him of any meds until tomorrow to make sure he was tolerating the food better and it wasn't another variable added in. 

So for now we are content.  Although he was unable to go vacation for a few weeks on The Floor, we are happy he is having a content Stay-cation on the 16th!

Friday, March 14, 2014

I'm Back!

Over these past 12 weeks that Cohen has been fighting for his life in and out of the hospital, we have been amazed by his strength, resiliency, and especially his attitude.  He is one happy baby!!  Even with having a tube in his mouth and being on crazy amounts of drugs, this little boy is one tough cookie!

To back up a few days ago, Cohen was making great strides on his very cool Heliox set-up in the hospital.  His cardiologist came by to see him and could not believe how amazing he sounded.  This proved to the doctors that he definitely had an unidentifiable obstruction.  Because the heliox was able to travel into small passageways, his breathing was slower and much quieter!  After a few days on his trusty sidekick tanks, the doctors had proved their point and felt comfortable taking him off of the Heliox.  They wanted him to return to his original home set-up but the plan was to get him down to less flow if tolerated.

Today Cohen is breathing easy on only 2 liters of regular flow, not even high flow!  He is doing so well and the doctors are so impressed with how he has improved.  If we could get him home on regular oxygen and not even high flow it would be a world of difference to his quality of life and our sanity.  We could venture outdoors more with a travel oxygen tank and not be restricted to the amount of hours he would have to be detached from his high flow system.

Dr. Harris, the amazing and caring doctor we have had all week is convinced it is reflux that has exacerbated all of his issues and she may even have me convinced at this point as well.  Talking on rounds today, we discussed how the pulmonary team is so impressed with the difference this week has made and how they were also now believers that this may not be a rare lung disease. As frustrating as it is to watch these doctors teeter totter their motives, theories, and beliefs I feel happy and confident if they are happy and confident.  Having so many specialists involved often is a negative thing because fingers are pointed and someone wants to finally be able to claim they have found the source of Cohen's struggles; however, often times theories and ideas only confuse the situation more and often scare the hell out of us!

Dr. Harris, Ryan and I have chosen Plan B for him at this point.  We want him to be the most stable and in the best place possible before taking him home for the fourth time.  We want it to be successful and memorable for positive reasons, not scary memories.  We want him to gain weight, breathe easier, and hopefully one day outgrow all the wires, cords, and monitors that are attached to him.  Our three month old has grown accustomed to being weighted down by his hospital shackles as have we with holding him and trying to ignore them.  For the meantime he will continue to be hooked up to these shackles as he grows and gains weight..hopefully quickly...but at least he will do it all while smiling :)

Tuesday, March 11, 2014

Heliox and Withdrawls

Cohen made a smooth adjustment off the ventilator and was enjoying having full access to his hands again. Usually sleeping with his hands straight in the air, like he was riding a roller coaster, is the way he enjoyed sleeping.  But being on the ventilator, the adults had strict policies about keeping their hands off the tube.  This was understandable because Sir Cohen tended to not be trusted with his hands (ng tube pulling).  So at last he could sleep with his hands free from any blankets and was comfy.

He was also enjoying his sleep because he was still on some sedation medications.  When on the ventilator, he needed some form of sedation to keep him calm.  Because these sedation medications were fairly strong, he would need to be weaned from them.  As a mother, I never thought I would have to witness my child going through withdrawals from drugs, let alone at such a young age.  Having spent the past week on a ventilator, he had also been on those medications for the same amount of time keeping him calm and distracted from the tube in his mouth.  Treating his like a heroin addict, he began to receive small doses of Ativan and another medication.  Also to bridge him between doses of these meds, he was occasionally given morphine.  Watching Cohen tremble in his sleep in between doses, sweat, and be very irritable was heartbreaking to see.  I was watching my 3 month old son go through something he shouldn't have to go through.  He was experiencing something that people do to kick something they have had a habit for for a long time.  He needed to be on the ventilator to keep him stable and this was the risk.  Everything in medicine has risks and benefits and this was one of those things where the benefits of having the breathing tube put in at the time outweighed the risks.

Over the next few days he has improved and is still on low doses of these medications but is getting better each day.  His cry is not as loud as before because he is still rather swollen...but honestly I don't mind the quiet cry!  He can keep it if he wishes.

 Ever since we had started feeding him strictly through his jtube to his small intestines, many things had improved for him. He was no longer coughing and gagging up phlegm, and he was breathing more easy.  Maybe after all the kid had some reflux going on after all.  Dr. Harris, the current attending, was making it her mission to treat him like a chronic reflux baby in hopes of improving the swollen airway.  His airway was swollen due to the tube being in but also possibly due to repeated aspiration.  Over the weekend he still sounded terrible when he cried, but was no longer showing any signs of reflux.  All of the nurses were on strict orders to deliver all medications as well through the jtube or via IV.

Yesterday on rounds Dr. Harris proclaimed her views to the 7 residents taking notes in my room, that she truly did not believe this was a case of interstitial lung disease but could quite possibly be chronic aspiration.  They suggested trying Heliox as a method for further determining whether or not he did have an invisible obstruction.  Heliox is a mixture of helium and oxygen.  Because helium is a lighter gas it can travel with less resistance down the airway and into small openings of the lungs than oxygen can do.  Their reasoning behind this was not to start us on another new therapy to take home, but to validate whether or not this airway obstruction did exist.  Now we sounded like we were treating an alien with some crazy rare illness. Where did they come up with this stuff? If not for Cohen, I would've lived my entire life and never have experienced Heliox.

Heliox is no joke.  They showed up with these two huge tanks filled with the Heliox solution.  After being hooked up for only 10 minutes, Dr. Harris called me at home.  After getting over the initial shock of seeing the scary hospital numbers come up on my caller id of my phone, I listened to her words as she uttered, "he sounds the best he has ever sounded.  He is breathing calmly and quietly."  Like a normal baby, I witnesses what most parents get to witness when they take home their baby, a peaceful, quiet breather.  As I held him in my arms, I couldn't get over the fact that he was silent.  I was so used to hearing his striderous sound it was amazing to hear him so calm.  Dr. Harris suggested we talk to discuss plans for Cohen.  At this point, they do not have helium therapy that is available in the house.  I was very happy about this because after seeing this set-up you an only imagine how much more challenging it would be to add this to the decor of my living room.

Her plan was an A or B plan.  Plan A we give him steroids to help with the swelling of his airway and send him home again hoping he grows and his airway expands.  Plan B would be to hold tight for another few weeks to a month to help him grow on the Heliox and hoping that his airway expands.  I disliked both plans for valid reasons, but ultimately the goal would be to get home and STAY home so I chose Plan B.

We will see what tomorrow for now we rest :)

Saturday, March 8, 2014

A is for Aspiration!

Cohen was finally extubated (breathing tube came out) yesterday and it was a huge celebration among us and also the hospital staff.  Nurses and doctors were coming around to see his handsome face and seemed genuinely excited for us and for him.  I stayed at home yesterday with Isla for the first time in a few weeks and it was refreshing to get away from the hospital and the medical life I had grown accustomed to seeing for the past 4 months.  Chicago had finally decided to have a "heatwave" of 40 degrees.  Isla and I took full advantage of it and played with chalk outside for a bit, took the dog for a long walk (Isla took the dog for a walk), and went to our neighborhood park for the first time since October.  It was amazing to have no cares or worries in my mind for a few hours and to spend some quality time with Isla.

Ryan stayed at the hospital with Cohen and was able to hold the little guy for the first time in over a week.  We had all been through so much it was heartwarming to hold him.  His connection to us for the past week has only been through head rubs and hand holds for the most part due to being intubated, so being able to hold all 9 pounds of him sure made the difference in our moods!

Ryan attended a Care Conference that we had scheduled with a few of the pulmonary doctors who knew Cohen very well and also some of the residents and fellows on staff this week.  The reason for the conference was because it didn't seem that everyone was on the same page again.  We again had too many cooks in the kitchen and some were trying to decide upon pretty invasive procedures.  Now that Cohen was stable off the ventilator, we still had no answers.  It was like being back to ground zero and we had the same level of frustration as we have had for each hospitalization.  Why was he breathing so fast? Why was he needing more oxygen? Why was our baby boy seeming to be getting worse with age when we were told that growth was the best thing for all of his medical struggles?
All last week we were told that he may in fact have an interstitial lung disease.  There are a variety of diseases and each comes with its own prognosis and treatments.  Some are genetic while others are not and can be tested via blood or lung biopsy.  We sent out blood that will take a few weeks at least to get back results of any rare genetic lung diseases.  There was talk all week of doing a lung biopsy and at this point Ryan and I were both on board with going through with the procedure.  As much as we didn't want to put Cohen through any more pokes or sedation, we wanted answers for him!  Not having a diagnosis at this point has only made things more scary, more complicated, and worse for Cohen.  It is always true that once you receive a diagnosis your life will only improve from there because your medical treatment can be specific and applicable to the diagnosis.  Besides having DiGeorge, Cohen still had this unexplained breathing issue that has now ended having us return to the hospital again for the forth time.  The reason the tube was taken out was because he was truly doing better, not needing the support and the argument regarding whether to do the biopsy was still out on the table.

The doctors brought up at the meeting that he may possibly have an H Type Fistula.    It is an abnormal connection between the trachea and esophagus.  It is shaped like an H and usually can cause food to leave the esophagus and travel across and down into the lungs much easier than if it weren't present.

 If Cohen has this it can be identified and usually surgically repaired to improve chances of aspiration.  We were back to my favorite word again...ASPIRATION!  The doctors were thinking that chronic aspiration, even though never proven in Cohen and through multiple reflux meds and procedures, that can cause significant lung damage.  After reading about it, it was interesting to come across that babies that do have this in utero, tend to not swallow and it can lead to the mother having polyhydramnios (extra amniotic fluid because the baby isn't swallowing it in utero), which I had while pregnant.

We are waiting till next week to perform that test to see if he in fact does have this.  If he does, it would be a huge relief because it is a relatively easy procedure and could fix a lot of the difficulties Cohen is having.  In the meantime, we are trying to keep his breathing under control and keeping him calm.  He seems to have a bit of swelling due to being intubated and is also going through withdrawls from the sedatives.  He is receiving other medications to help with the withdrawls, but it is not uncommon for children that have been on those sedatives for awhile to go through them.

Right now he is sleeping comfortably and enjoying his hands being free again to spread out!  It is so nice to see him without a bunch of stuff on his face and he is so peaceful.

Monday, March 3, 2014


Sorry for the very delayed post, but the past week has been an emotional roller coaster and I haven't been able to even breathe for a minute to update the blog.  Cohen is finally stable now but it was a bumpy couple of days so let me explain...

Last Wednesday, the doctors decided we would switch Cohen's gtube to a gjtube.  What this would mean was he would be fed in his small intestines instead of his stomach since that may have been causing the spit up therefore leading to the scariness that occurred on Tuesday with Ryan and I.  He had been doing ok that day but seemed to have a slight cough and was occasionally "choking" on what seemed like saliva.  When this would happen, his oxygen would drop to the 80's which was a very new thing for him because he always tended to be in the 90's for the most part.  

At around 3:30 two nurses came upstairs to his room and the three of us wheeled Cohen in his little bed to the elevator. As we were getting on the elevator, he began to cough and seemed like he was having some trouble getting the spit up.  The travel monitor began to make sounds and the nurses who were with me started to get a bit nervous.  They pushed his bed quickly down the hallway and began talking about how they needed to find suction.  They kept repeating this and then one said, "Get him into a room now with suction!"  Where we were in the hospital it seemed very isolated and empty.  We were going down for a routine procedure in the imaging room, not an operating room. They pushed his bed quickly into a bright room and tried attaching the oxygen mask to his face.  I stood back with my body against the wall of the room and was paralyzed.  No thoughts were going through my mind, I couldn't talk and for a second felt like I had stopped breathing.  Another nurse came in and asked if they needed help.  The look in their eyes said it all and she pushed THE BUTTON....the CODE BLUE BUTTON!  I knew what this button meant because I have seen it before living in the hospital for the past few months but never in my room. Usually nurses, doctors, residents, fellows, whoever is around when they hear the signal going skipping down the halls to the patients room and flood in without any purpose but to be there in case they are needed.  A nurse turned to me and said, "Everything is ok, but he's needing a little extra help, so we are going to get some.  There will be a bunch of people coming into the room in a minute so I don't want to alarm you."  Alarm me?  I was for some reason speechless and had no emotions. I continued to stand there as I watched the back of Cohen's neck while they help him upright on his bed turn slightly blue and that was enough for me to leave. 

As I walked into the hall in utter shock, about 15 doctors and nurses were running past me.  A receptionist on the floor led me to a room for me to wait.  Meanwhile the doctor that was going to perform the gj procedure came to meet me apparently unaware of what was happening.  He began to speak about the procedure and what was involved.  Apparently he had no idea because as I began to cry a few nurses rushed in to let me know that he was not coming back up on his own and they would need to put in a breathing tube to help him.  This was a nightmare!  I called Ryan as quick as I could to let him know the news.  

After about 15 minutes they said they had the tube in and he wasn't quite stable but they needed to get him back up in the room where they had everything readily available.  I followed them closely walking behind and only seeing the top of Cohen's head.  The nurse was "hand-bagging" him meaning pushing a bag to keep his lungs inflating since they needed to hook up the ventilator once he returned to his room.  Once we arrived back at his room 1632, it was nothing but more chaos.  Cohen's IV had fallen out and even more nurses came to assist.  They pushed a cart right up against his room wall that had every drug imaginable and as I stood there waiting for Ryan to come meet me, they began to pull up various medicines into syringes.  There was so much activity and it happened so fast it is hard to say what exactly happened in those 20 minutes but they were finally able to get an IV into both of his feet so that they could give him some pain medicine and sedation.  He was finally somewhat stable but the doctors were very worried about how much support he needed from the ventilator at the time, it almost seemed like his lungs needed a lot of support to keep them open.  He was heavily sedated that night.

The following day he had various moments of huge desaturations where he was again needed to be hand-bagged to bring him back up.  Through Thursday and Friday his desaturations increased and it was taking the doctors more to pull him out of it.  Code Blue happened three more times!  

There was a follow-up echo done of his heart and it seemed as if he was having pulminary hypertension.  Up until this point, Cohen still had a small hole in his heart, but his heart was functioning great!  During the echo they were able to see that occasionally the blood was reversing directions which was not good.  This meant that instead of blood going from the heart to the lungs it was reversing, causing there to be a lack of oxygen in his lungs and making him have these huge desaturations.  After the second one, they decided it would be best to paralyze him.  For over 24 hours, Cohen was unable to move anything for his own safety so that he would not be breathing over the ventilator.  

On Friday they sent off usual blood work and determined that his hemoglobin was very low so he endured his first blood transfusion.  This helped his numbers increase and it seemed to help him in general with dealing with the breathing tube.  They also decided it would be best for him to still get the gj so he went downstairs again but this time with two anesthesiologists.  They made sure to keep him paralyzed for even this minor procedure because he seemed to desaturate from very small upsets.  The doctors also wanted to get a central line put in.  This is like an IV but only longer and put into a larger vein (artery) which leads to the heart. The good thing about doing this was the nurses/docs would be able to pull blood from it without poking him each time and also it would stay in!  Over the next day, he became slightly more stable, and they tried to decrease the support from the ventilator.  During most of the weekend he was doing better and after getting the gj tube put in, he was able to finally eat!

Today we discussed why he has begun to have pulminary hypertension.  THis is usually a diagnosis that is secondary and caused from something else.  There was most likely something else that was causing Cohen to have this, act this way, and need the increased support from the ventilator.  The pulminologists now think that Cohen may have a rare lung disease.  This is what I have thought all along and though I'm not a doctor, my "mom instinct" has always thought that due to his increased need for oxygen, the sound of his breathing, and the increased work of breathing.  They decided to send some more blood to help determine if he has a rare genetic lung disease (which will take weeks-month to hear back about).  But at this point they are also considering doing a lung biopsy to determine whether or not they can use the tissues collected to look for other lung issues.  

This is again another waiting game but I feel more confident now that things are starting to show and hopefully pointing the doctors in the right direction.