Sunday, June 24th

I have felt like we never receive good news, but Sunday morning was the worse news we have yet to receive. I always wondered how doctors break the news that there isn't anything more they can do for your child, Sunday morning I found out. The doctor was extremely calm and basically told us that within the next few days we would have to make some extremely hard decisions, ones that no parents should ever have to make. We were told that they believed Garak's liver was failing and on top of that, they believed Garak had suffered severe brain damage due to lack of oxygen. We knew that his liver was having issues. The liver enzyme levels were elevated to 500, normal is 100. We also knew that his platelets continued to drop, the lowest was in the 30's after two bags of plasma he went up to the high 60's. After two additional bags it was in the 90's. His coags and ffp also continue to drop as well. The coag (coagulation) is blood clotting, the ffp is the time it takes the blood to clott. The brain damage came by surprise to us. Since the MRI came back normal the day before we did not think he suffered any brain damage. Unfortunately, throughout the night Garak continued to do neuro posturing. Small movements with his arms and legs that are not normal, especially being as sedated as he was. After receiving this type of news, your mind races to things you should have done, things you didn't do, things you want to do. To say the least, this was the worst day of my life and it was just going to get harder. We decided we needed to tell the boys so that it did not come as a shock to them. This was horrible.... all the boys digested this information in their own way, but they all cried a lot.

Saturday, June 23rd

Saturday morning a little after 6am I was awoken by Garak sounding like he was gagging or trying to throw up. With the neesan, I knew this was a bad sound. Immediately, I realized I had laid him down in his crib 5 1/2 hours earlier, since he has never slept for more than two hours on his own, I knew something was wrong. When I took him out of his crib his eyes were rolled back in his head, he was lethargic, very limp, not breathing well and his skin was on fire. Since he had been running the high fever for basically 3 weeks I knew his temperature was extremely high. I took his temperature rectally, 105, 106, 107, 108, at that point I did not even wait for the temperature to stop, we grabbed the oxygen tank, hooked Garak up to the oxygen and speed to the Riverton's Hospital ER. Within a minute of arriving in the ER at least a dozen people were hooking him up to every type of medical equipment in the room. When we arrived his oxygen had de-satted to high 40's to low 50's, and his blood pressure was 57/22. The respiratory technician pulled a oxygen mask and tried to get is oxygen stat up, but no matter how high they turned the oxygen up they could not get is oxygen stat above 60 (normal is 90-100). At this point they decided to put an oral intubation in him, which means they placed a tube into his lung to help him breathe. They also tried to put in an IV to help give him fluids and meds, but they could not, so they put in an I/O (IV through his bone marrow). They drew a lot of blood and started the works on him. It was clear that after a half hour whatever was happening to him was extremely life threatening, they decided to life flight him to Primary Children's PICU (Pediatric ICU) in Salt Lake. Once he arrived at Primary's everyone realized he was in a very long seizure. At this point we knew it had started at 6am (possibly earlier). By this time it was 9:30am so it had been going on for at least 3 1/2 hours. They gave him Versed which also has an amnesia property to it to help him forget about everything. They also gave him Ativan which is another sedating medicine. Unfortunately, neither of these meds stopped the seizure so the gave him a bolus of Keppra which did not stop it either. They then took him down for CT scan on his brain, an MRI and EEG. By 12:30pm the seizure finally stopped. The CT scan and MRI both came back normal, the EEG showed the seizure. The doctors at Primary's also did a LOT of blood work, but they are not sure what is causing the high fever. That evening the EEG looked good, the fever was down to 103.6 and he remained sedated as they looked for an infection.

ER Visit/Pediatrician's Visit

On June 19th, five days after we returned home from the hospital for the G-tube, Garak was a little feisty and pulled his G-Tube out.  The pediatric surgeons told us that this was a possibility, but said very rarely does this ever happen.  Even more rare, was the balloon inside his stomach was fully inflated, so not only did the button come out, the 4ml balloon also came out.  We took him to Primary Children's ER where they had to replace the tube.  Because the tube had only been in for a week they had to x-ray the positioning of the tube to make sure the balloon was actually inside the stomach and not between the stomach and his outer wall.

While in the ER, we also brought up Garak's fever situation.  Since Sunday, June17th his fever stayed between 102.8° and 103.6°.  They drew his blood twice to do tests, but unfortunately the blood kept clotting so they were not able to do all the tests they wanted to.  They were able to pull a CRP test to measure inflammation which tells them if he has some type of infection, but this test came back normal.  Since the CRP test came back normal they felt comfortable to let us leave.


Two days later on June 21st, Garak had a temperature of 104.8°.  At this point I felt like something was wrong, so I took him into his pediatrician.  His pediatrician looked him over, and despite the high fever felt that he looked good.  He diagnosed him with have Roseola (aka sixth disease).  This is a viral illness in young kids, most commonly affecting those between 6 months and 2 years old.  It usually is accompanied by several days of high fever, followed by a rash.  At this point he did not have a rash, but that was the best conclusion they could come up with at this point.  Knowing what I know now, I should have pushed for some testing and more answers, but thinking doctors know what they are doing (which I've now know most do not) I left the office.  

G-Tube/Feeding Tube

Since the time we had to add Simply Thick to Garak's formula, Garak lost a lot of weight.  In 3 weeks he lost just over a pound.  Since he was already low in weight, losing the pound dropped him off their chart from three percentile to "zero".  He was eating approx 20-24 ounces each day, once the Simply Thick was added that amount dropped down to 10-15 ounces.  It was so thick for him to suck through the bottle, he just could not get enough volume.  After 6 months of physicians inferring putting in a feeding tube we finally agreed.  It was probably the best thing for him.  At this time, we needed to figure out exactly what type of tube we wanted to Garak, we had the following two options.

1. G-Tube with a Nissen Fundoplication:  A gastric feeding tube (G-Tube) is a tube inserted through a small incision in the abdomen into the stomach and is used for long-term enternal nutrition.  In the Nissen Fundoplication, the Gastric Fundus (upper part of the stomach) is wrapped around the lower end of the esophagus and stitched in place.  This reinforces the closing function of the lower esophageal sphincter.  The nissen is to help people with severe reflux, it prohibits anything from go back up.  This will prohibit him from throwing up, burping and also having reflux (acid) go up.
2. GJ-Tube: A Gastrojejunostomy tube is similar to the G-Tube, but once in the stomach is then threaded into the jejunum (the jejunum is the middle section of the small intestine)

Both type of tubes are reversible, meaning we can take them out any time we feel like he doesn't need them. However, the Nissen Fundoplication is not reversible so we will never be able to undo this.  We opted to go with option 1.  Since we will continue to try to feed him portions by mouth, he would continue to have the bad reflux.  With doing option 1 we hope to eliminate his reflux to make him feel better.

On June 11th we went in to place the G-Tube and to have the Nissen done.  We were told we would be in the hospital for 2 days, but right after surgery he came out with an extremely high fever again and lasted four days.  Garak was also inconsolable, regardless of touching is hand/foot or rubbed his cheek or held him he would cry and cry.  The nurses continued to give him morphine thinking he was in extreme pain.  After 2 days they had to take him off morphine since they do not want infants on a hard drug for more than 48 hours.  Once the morphine got out of his system, his demeanor improved tremendously.  Since the only thing they changed was the morphine, the doctors believe he had a bad reaction to the morphine.  They say some side effects small children and infants can experience on morphine is severe pain throughout their entire body and hallucinations. On June 14th we left the hospital and returned home.  We were hoping with the feeding tube and the Nissen we would have a happy baby, but unfortunately that was not the case.  Garak seemed to get fussier each day and his fever came back.  

Since his bronchoscopy, his fever has remained very constant, the highest being at 104.6°.  Despite the meds we gave him, we had not been able to drop it less than 100°.    

Bronchoscopy and pH Probe

Due to the aspiration, they performed a bronchoscopy on May 29th.   The had to sedate him in order to put the scope down into his lungs, they also wanted to check out his airway and stomach area during this time.  His left lung was perfect, his right lung had a small amount of scar tissue, but it was very little compared to what they thought they would see.  This was very good news.

Since he was getting sedated and had to be at the hospital anyway, they also did a pH Probe.  The probe study was to measure the amount of reflux he was having.  The results of the pH probe was very surprising.  In 24 hours he had 126 episodes of level 3 reflux.  Level 3 is the worst level of reflux anyone can have.  The episodes lasted an average of 7.5 minutes, the smallest one lasting just over 5 minutes.  From these results they determined that in those 24 hours he refluxed at least 15-18 hours.  

Late that evening, Garak began to have a very high fever of 103°.  They continued to give him meds to get the fever down, but it didn't seem like the meds were helping.  They let Garak go home the following evening thinking the fever was not due to the scope, but perhaps his reaction to Aesthetics and morphine.  They sent us home telling us to continue a Motrin/Tylenol regimen.  He continued the fever for 7 days at which time they put him on Azithromycin, which is an antibiotic.  They were pretty sure he had pneumonia due to the bronchoscopy.  On June 5, after being on the antibiotic for 2 days the fever finally subsided.

Sleep Study, Swallow Study and Upper GI Study

With the lack of sleep Garak has been getting, the neurologist referred him to a pulmonary specialist.  We had a sleeping study done on Garak which brought to life some new issues.

Below are the highlighted points of the sleeping study:

• He has obstructive airway apnea.  When not in REM sleep, he has it 28 times an hour, during REM he jumped up to 46 times an hour.
• He takes him 120-160 mins to reach REM sleep, normal is 20-40 mins
• He is only getting approx 1-3 hours of REM sleep each day, normal is 12-15 hours.

With the results of the sleeping study they decided to do a few more tests to pinpoint what was causing the obstruction.  We then had a Swallow Study and an Upper GI Study done.

The swallow study was to see if he was aspirating his food, and if so, to what extent.  When they were readying for the swallow study they warned us that they don't always capture an aspirating event. Unfortunately, after the 3rd swallow he aspirated his food.  What he did they call a 'silent aspiration' which means he did not show any sign of aspirating (no coughing, no flaring nose, no gagging, etc).  At this point, that stopped giving him the regular liquid and they tested a nectar consistency (basically just thickening it up).  Again after 2-3 swallows he aspirated (silently).  We moved up to the honey consistency (even thicker) and at this consistency he started aspirating but caught it himself and cleared it.  They were comfortable to say that at a honey thickness he does not aspirate and that it is safe to feed orally at that thickness.

A few days later we did the upper GI study which showed a small amount of reflux, and it appeared he didn't aspirate the reflux.

At this point, we were told to only give him formula which we needed to thicken with some gel product called 'Simply Thick'.

January, February and March

It has been many many months since the last post, I've realized putting his story into words has been very hard but brings me out of my denial state.  Before I begin to go into what Garak is going through now, I'm going to backtrack to December the time of the last post.

In December, the seizure seemed to be under control and life seemed to calm down.  We had an amazing Christmas, almost as if the past few months were just a nightmare.  Life was very good and everyone was doing really well.  Toward the end of December and into January we started noticing Garak having what seemed to be Moro Reflex.  Moro reflex is a response to the loss of support and feels to an infant as if they were falling.  Most commonly, infant arms flying out to the side and legs usually go stiff.  We did not think much into this since all babies do this, but then we started noticing Garak doing them ALL the time in clusters.  

At Garak's neurology follow up which occurred on January 19th everything was normal and calm until I mentioned that Garak was doing this 'moro reflex' a lot.  Suddenly everything changed from calm and normal to an urgent need to set him up with an EEG to look at what was going on.  We were told they were worried that Garak wasn't having moro reflex but he was having Infantile Spasms. Infantile Spasm (IS) is the specific type of seizure in infants that have West Syndrome.  Infantile spasm is also known as hypsarrhythmia, which is basically chaotic brain waves.  We were told that IS is worse for Garak than the seizures since when Garak is having seizures, his brain neurons are not working only during the seizure.  With IS the neurons are never working properly.  This news was hard to comprehend since we thought things were improving, but now we know the seizures were just suppressed because of the IS.  They immediately started him on Prednisolone which is an oral steroid to cure the IS.  They were hoping he would only be on the steroid for 2-3 weeks before the IS was gone.  Unfortunately, Garak was on this steriod for almost 8 weeks.  Throughout the course of the 8 weeks they had to increase the dosage twice since the smaller dosages were not helping the IS.  The last 3 weeks he was on a higher dosage then most adults are ever put on.  After 8 weeks, the motion of his arms and legs decreased almost fully so they did another EEG the second week of March and determined that he no longer had IS.

During the time he was on the Prednisolone, Garak was EXTREMELY fussy and miserable, this is one of the side effects to the steroid.  He also slept even less than he did previously which I did not think was possible.  Insomnia is another side effect of the steroid.  The one good side effect of the steroid is that it normally makes people gain weight.  Garak has been extremely under weight and in January he was in the 7th percentile.  We were hoping that the steroid would help him eat more and put more weight on, but unfortunately, that did not happen.