Day 3 at the childrens hospital

 I am missing my other kids. :(  I need to get home and spend time with them.  I did sneak away during Ronan's nap time today and went with Isaac and Jadzia to a birthday party.  But Inara especially is missing me greatly and feeling insecure.  Ronan might have his PICC line placed tomorrow. If this is the case I won't be able to leave but if everything is going ok I might slip away for a little while to spend time with my other kids so they know their mother hasn't abandoned them.  On Monday my in-laws are picking up the boys so my mom will only have the girls.  

Ronan woke up with his right eye pretty swollen.  His whole face has been swollen but this morning there was a noticeable difference his right and left eye.  As the day went on the swelling went down but the lid stayed red it resembled when he had the periorbital cellulitis.  

One of the valves in his IV came loose.  The good news was the IV site itself was fine but he had been leaking fluid for awhile.  This made his sugar drop to 60 but as soon as the IV was hooked back up his sugar returned to normal.  He was acting like he wasn't feeling very well and very tired all day.  He was also not eating well.  He was acting strangely enough I asked the nurse to check his sugar early a couple of times and it was normal.  He continued to act sluggish then after barely touching his dinner he had four bad diapers then started laying on his floor mat lethargic.

The nurse checked his sugar and it was 311.  The doctors weren't worried because they prefer it high rather than low at this point.  He got a surprise visit from Grandpa and he was pretty happy about that.  As soon as Grandpa left he started putting his hand in the back of his mouth and acting lethargic just like when his sugar is low but he had just been 311 so I decided to just watch him.  Two hours after the 311 blood sugar they checked him and he was 37.  The nurse checked him again to be sure and she got the same result.

They gave him some emergency meds and his sugar climbed back quickly.  He was then pretty happy the rest of the night.  Tomorrow he might get his PICC line and he will have a GI consult to figure out what they will do with his esophagus problem while he is in the hospital since he is due for another dilation Tuesday and he will not be out by then.  At this rate I don't know when he'll be out since being off the IV meds for a very short time made his sugar dip low.  It's all wait and see at this point.  I love my brave, beautiful boy.

Figuring out Ronan

We are still in the hospital and will be for some time. Above is sleepy Ronan. His residency here is apparently extensive as they are putting in a PICC line.  This is because after so many hospital stays combined with blown out veins from too much poking has led to him having no where to draw blood.  They are also very concerned that if he pulls his IV out (an art form he's mastered)  they will not be able to get him IV meds in an emergency.

The Endocrinologist came by and talked to me today.  She said she suspects he has Hyperinsulinemia/hyperammonemia syndrome.  The two conditions of hyperinsulinemia and hyperammonemia can be conditions that indicate other metabolic problems but when they are found together with episodes of severe hypoglycemia they indicate their own syndrome.  The problem is with an amino acid called Leucine and it stimulates protein synthesis (formation).  It is found in animals (meats) and anything that is an animal product (milk, eggs cheese).   If Ronan has this disorder it means that when he digests a food containing Leucine something goes wrong with the protein synthesis and it triggers his body to produce excessive amounts of insulin.

The major problem with this is insulin then tells his body to get rid of sugar.  But his sugar levels are normal and he does not need the excessive insulin. Then when his body gets rid of the sugar he plummets into a severe hypoglycemic episode.  This makes sense because we had been increasing Ronan's protein to stabilize blood sugar.  Last night he had a large meal full of protein and then a little over two hours later he had a seizure from low blood sugar.  Today it is looking more like he does have this.  He's been put on a low protein diet and been put on the medication that treats this disorder and for the first time this week he has not had a single sugar low.


His Endocrinologist said this is extremely rare.  It's something she learned about in school but has never seen a case in all her years of practicing.  Her colleague who has been practicing even longer has never seen a case either.  She said she would never have guessed he had this but it was his labs upon being admitted that tipped her off.  His sugar level was in the low range of normal but his insulin level was extremely high as well as his ammonia level.  She put this together with the symptoms and she said it fits perfectly.  Now we wait for confirmation.  The lab work has been spent off to a specialty lab.  It will arrive sometime next week and will tell us for sure if he has this disorder.

His Endocrinologist said if this comes back negative then she has no idea and we'd be starting at square one.  She would then have to take him of all IV medications and try and trigger a hypoglycemic episode so they could draw labs while he is in a state of hypoglycemia.  Hopefully it won't come to that and this will be it.  The prognosis isn't horrible.  Ronan will have to pretty much be a Vegan.  No animals or animal products for the rest of his life.  He can't have meats, dairy, eggs, peanuts or soy.  Dietary will be formulating a diet plan for him before he goes home.  It's a good thing Ronan is not a picky eater but I'm sad he has to give up his all time favorite food. Yogurt.


He will be put on a special formula and it will contain all essential nutrients except the Leucine.  This will replace his milk and pediasure as pediasure was his main source of Leucine.  The doctor told me she would try to get Ronan home for Thanksgiving but was making no promises.  I told her that was fine because I'd rather him stay longer and have this taken care of then for him to be sent home too soon and then crash again.  If this diagnosis is confirmed he will be sent home on a new medication that is specific to this syndrome.  He will also be sent home with an emergency medication.  This way if he ever has another seizure I can can bring his sugar up and stop the seizure.  this will be essential since when he's having a seizure since I can not give him anything through his mouth.

This will involve a lot of monitoring and more specialists.  Ronan will have heart checkups to make sure his heart isn't being harmed.  He will also make regular visits to his Endocrinologist and this is something he will never out grown.  It seems daunting but I'm looking on the bright side it is very treatable.  Avoid the wrong proteins, have preventive medications and emergency medications and Ronan should be just fine.  I keep thinking back to last week when I had no idea that in a few days we would be given yet another life altering diagnosis. 


Ronan will see a slew of specialist next week and be given a mountain of tests but with the picc line being place he will not have to be poked anymore during his hospital stay which is great.  But the biggest blessing is no sugar crashes today.  I am thanking the Lord for imparting wisdom to the doctors to decipher all of Ronan's confusing symptoms and allowing them to save his life.

On a side note you can see in the pictures Ronan has a floor mat in his room.  I'm not sure if anyone remembers Ronan's hospital stay where he was insistent the floor was the best place for him to be.  He started his floor demands here and they brought him a nice cushioned floor mat.  He has been happily playing on it all day.  The amazing thing is he's very happy despite being in the hospital.  I actually haven't seen him play this well or laugh this much in awhile and I'm wondering if it's because they finally stabilized his blood sugar.  This whole time I was thinking it was all the hospital stays making him depressed but it turns out it might have been his blood sugar.

Which is the last thing I have to mention.  His Endocrinolost thinks this has been happening a long time but never got bad enough to be noticed until recently.  She said it might even explain his long term apnea as this can cause apnea if left untreated.  She told me she feels bad because he has been her patient for almost two years and she never caught this.  But I don't think she could have until now because he never showed major problems until now.  As she left the room today she rubbed Ronan's arm slightly and told him "I'm going to fix this, Buddy. We're going to make you better."

Below is Ronan's excitement when he realized Elmo's World was playing on the TV.

Sugar problems

Ronan is back in the hospital.  Tuesday night my mom was watching him and he started stumbling around and acting funny.  She's diabetic so she checked his sugar with her monitor and it was 39.  After drinking some juice his sugar level stabilized and he seemed fine. The next morning he woke up energetic and ate his breakfast.  Around an hour and half after breakfast his sugar dropped to the 30s.  I called his Endocrinologist to inform her about the new sugar lows.

She advised me to increase his protein intake to stabilize his sugar for a longer period of time.  Since he can only have pureed foods she suggested I get him some Greek yogurt because it has a lot of protein.  She couldn't figure out why his sugar was dropping after eating especially since he was on the pediasure and they often give that to diabetic babies to keep their sugar up.


I did what she requested and increased his protein.  By Wednesday night he was having another sugar low.  I gave him juice and he laid down on the floor and his left arm began to jerk.  After it stopped I checked his sugar and it had gone back up. By this morning (Thursday) he woke up once again fine.  He ate yogurt for breakfast and I had to leave to get Xander to an appointment.  My mom was staying with Ronan.

When I arrived back home She informed me that his blood sugar had been 21 and she had given him 4 ounces of juice and a half an hour later it was just 23.  She gave him another four ounces and a half an hour after that it was 29.  I called his Endocrinologist and told her what was going on and that we couldn't raise it.  She told me to get him to the children's hospital right away.  My mom fed him Greek yogurt while I was on the phone and when I got off he was clapping and happy.  I took him into the Children's ER and his sugar was 115.  They put his IV in and he didn't even flinch he was so tired.  They checked his sugar and it was 80 so they started him on a sugar drip since it was dropping so quick.


Tonight they informed me they think he has a rare metabolic disorder that I can't remember the name of.  They told me the Endocrinologist would be by in the morning to explain what this meant and about the testing involved in confirming this diagnosis.  The entire day he kept his sugar level stable.  He ate dinner and had pureed beef, yogurt, pediasure and carrots.

The nurse checked his levels around 9:00 and he was 109.  Around 10:00 I heard him make a squeaky sound and I looked over at him.  he was jumping with each squeak.  It looked odd enough that I called the nurse and she was in the room within seconds.  We flipped him over so she could get a pulse ox on him and she left to get the glucose monitor.  He started jerking his arms and upper body.  I tried really hard to get him to respond but got nothing from him.  His eyes were rapidly opening and shutting.

The nurse came back in and saw what he was doing.  I asked her if he was having a seizure and she said she didn't know but got the on floor doctor.  She came in and confirmed he was having a seizure.  His blood sugar was low again and causing the seizure.  The doctor told me as soon as they got his blood sugar up the seizure would stop.  They gave him a load of sugar through his IV and then ordered a more sugar concentrated IV drip.  The seizure lasted 3 minutes but it felt like longer. 

He is now on a lot of sugar through his IV and he is now on a very low protein diet.  The disorder they think he has would cause his body not to process protein properly and so protein causes the low sugar.  Which means as long as he remains on a low protein diet his sugar should stay up.  This is why he got worse because we had increased his protein intake as normally that would keep sugar up.  This is also explains why he dropped while still on the sugar IV drip because he had had a lot of protein for dinner.  Now he can no longer have pediasure or yogurt.  Before he was put on pediasure his weight was dropping and he wasn't growing.  So I'm not sure how they will compensate now.


Also since he can only have pureed foods it makes our choices very difficult but dietary is coming in the morning to help me make a plan for him.  The Endocrinologist will also stop by to tell me her plan.  I was also told the arm jerking I saw at home was probably a small seizure.  He is going to be here for awhile.  He will not be going home until he can stay off all IVs and be on his at home diet and not have a single sugar low.  I'm not sure how long that will take but we will be here until he meets all of that criteria.

I think it's sometimes easy to forget how all of this affects the other kids.  Inara watched Rona fall to the floor today when he had his sugar low and she saw how concerned Grandma was.  My dad was sitting on the couch and she crawled up into his lap and said "I'm scared.  I'm scared."  Then tonight when I came home to get my stuff she said "Mommy, MoMo" (her name for Ronan) and then she pointed to the spot Ronan had fallen to.  She is very perceptive for 17 months and I feel bad she is still trying to process everything after several hours and it still worries her.  

Hopefully tomorrow we'll have answers and they'll be able to figure Ronan out.

Ronan in the PICU

When I woke up this morning I was excited.  Tonight we were going to the art center and my teacher's art gallery for my art class.  She had promised some delicious refreshments and a lot of fun.  I felt it would be a nice change of pace to get out a little bit. I never expected to end up in the PICU.  Ronan had been acting strange since he came home from his endoscopy on Tuesday.

The first thing I noticed was that he was super red.  This wasn't completely alarming because his dad naturally has a red face and Ronan and my other kids have it from time to time.  But what was strange was his face was red everywhere like he was overheated but his temp was normal.  His hands and feet were swollen and red.  I assumed the lethargy was from being put under anesthesia and figured a nap would cure it.  By the evening he was still really tired and I thought maybe all the procedures were starting to burn him out.

 

Ronan signing "Daddy"

The next day he woke up like himself and I figured we were fine.  An hour later his home teacher and Speech therapist showed up for his session.  He kept falling asleep during the session.  Our guess was he was still tired from the endoscopy.  The rest of the day seemed to go the same.  He would get bursts of energy and then collapse in exhaustion.  But he was eating ok and I thought maybe he needed more recovery time.

I got a call from his GI doctor saying they were going to do one more endoscopy.  I remember the sinking feeling that settled in when I heard this.  I put up a small protest but relented when they insisted this would be the last one and the surgeon would be present to watch this time.  I looked over at Ronan who was curled up in a ball on the floor and thought "How much more can he take".

He seemed like himself for the evening and I put him to bed thinking things were ok.  This brings us to this morning.  He woke up fine with a burst of energy and as the morning progressed he grew more and more tired.  He struggled eating his lunch and I was suspicious that maybe his esophagus was closing up again after only two days.  I put him down for his nap.  He slept four and half hours.  I gave him his bottle and he struggled getting it down.  I tried his pureed foods but he refused them then fell asleep on the floor. 

I was thinking I would take him to his pediatrician in the morning. He started stumbling around the house and falling over and my instincts told me to call his GI doctor. Something inside just told me he couldn't wait until morning.  His GI doctor was afraid his esophagus had perforated during his endoscopy or that his thyroid was in shock from all the procedures since he has hypothyroidism.  He sent us to the ER and said he would call ahead so they would be ready for him.  When we got there they had all the tests ordered.

Ronan was wide awake and being ornery to all the nurses.  He wasn't allowing them to examine him which is the usual Ronan.  I heard the triage nurse tell the nurses in the pediatric ER that he didn't know why I had said Ronan was lethargic because he was acting fine.  At this point I figured they'd just send him home.  My mom was there and the nurse told me it would be fine to go to my class since I had explained everything to the doctor and signed releases.  They promised to call at the first sign of trouble but expected he'd be going home.

A half an hour into my class my phone rings.  My mom called to tell me that Ronan's blood sugar is 21 and they are putting a tube in him and he's super lethargic.  My first thought was they were intibating him and things were really bad.  Isaac drove to the hospital which was a good thing because I cried the whole way there.  Isaac kept assuring me Ronan was fine and that it probably sounded worse over the phone.  When I walked into the ER I made my way to his room and rounded the corner cautiously.  I was afriad of what I would find. 


The tube was an NG tube and I breathed a sigh of relief.  They had inserted to get sugar in him quickly.  His sugar was up to 84 and I breathed another sigh of relief.  He was still out of it.  They told me they now understood what I had meant about him being lethargic. The helicopter people were placing an IV (they can place the IV in him when no one else in the hospital can). On more than one occasion I have thanked God for the helicopter people.  Normally it takes six people to hold Ronan down but this time he wasn't resisting.  When they checked his sugar again a half an hour later it was back down to 50, it wasn't stabilizing.  They decided to send a constant stream of glucose through his IV and this seemed to work.

The next thing they noticed was his heart rate was dipping dangerously low.  The tech came in to take his temp and it was 93.  She went and got a different thermometer and tried a different spot.  It was still 93 degrees.  I was stunned "Is that his real temp".  The nurse nodded and told me it was because they had left him without clothes and blankets while they did the tests.  They brought heated blankets and his heart rate returned to normal but his temp didn't.


I asked them to call his Endocrinologist and they looked at me confused.  I told them that she handles his thyroid but also deals with blood sugar issues.  They weren't convinced at first but after me asking again they decided to call her.  His Endocrinologist felt she knew immediately what was going on.  She thought his Adrenal gland was crashing.  Ronan has been on chronic steroid use for his asthma.  She thought this coupled with all the stressful procedures shut down his adrenal gland.  Without a functioning adrenal glad you can't control sugar, body temperature or handle stress.  This would be why this occurred immediately following his last endoscopy.  It was the final draw that threw him over the edge into adrenal failure.

They decided he would be treated as though his adrenal gland shut down until proven otherwise.  He was admitted the the pediatric ICU as he could no longer stabilize his sugar or temperature and he was barely responsive. 

 Now this is where we sit.  I am typing while Ronan is sleeping under his super heated blanket.  They tried heat lamps but his temp dropped to 91.  His temp is now at 95 and it is the best they can get so far.  The blanket is streaming constant heat from a large machine on the floor and it feels like a sauna in here.  The nurse asked me how I could stand sitting in here because it is so hot but I found a cool spot in the corner so it isn't so bad.  I'm just trusting God to get Ronan through like he has always done before. 

The hope is that it is adrenal crisis and he'll respond to the meds.  If it is not they have no idea why this is happening or what will happen from here. The main problem with why they are still not sure it is adrenal crisis is that labs that should be off are normal and this has them worried.  It's hard to be in the dark but I know God isn't and he knows exactly what is going on with Ronan.  I'm praying he will guide the doctors to the right answer and Ronan will be back to himself soon. 

Another thing that needs prayer is what this means long term.  If Ronan is in adrenal crisis that means surgery would be very dangerous and even future dilation life threatening.  The problem now becomes when Ronan's esophagus closes again there will be no way to fix it.  How will he eat?  I'm trying to focus on the here and now but that is weighing heavy on my heart.  I feel like we may have missed the window to fix the problem.  I'm trying to fight anger about this.  Why didn't they go back and take care of the problem right away.

Dreaming of Snow

 As you may think from the title I've gone crazy.  How could I dream of snow especially so early in the year.  No, I have not been dreaming of snow but Landon has.  He informed me a couple of days ago that he had a dream and in this dream the first snowfall happened on my birthday.  It was 70 degrees out when he informed of this and I thought it was cute.  He mentioned it again last night.  He said "Tomorrow is your birthday, it's going to snow!"  Well guess what today we wake up on my birthday and the ground is white with snow.

Landon came bounding down the stair sure of himself.  He pulled the curtain open and proclaimed "I told you it was going to snow today".  Maybe I should rent him out to the weather channel?

I know it has been awhile since my last post at least compared to the past frequency of my posts.  Life has been busy but good.  Here's the Fullmer kid update.

I have received many requests for Ronan updates so we'll start with him and I'll  go as quick as possible.  He had another endoscopy and dilation yesterday.  This is is his fourth one in six weeks.  Isaac and I are about to insist something more be done or we are going to get a second opinion.  His GI doctor agrees with us (he is the one doing the dilations).  He thinks we are putting Ronan under way too much and it's getting too risky.  Not only that but after only ten days his esophagus was closed again and very hard to dilate this time.

 His GI doctor said he is about to put his foot down and tell his surgeon he's not going to dilate Ronan anymore unless it is an emergency.  He thinks this may be the only way to get the surgeon to go back in and fix it.  Ronan is scheduled for one more dilation in two weeks.  After that another plan needs to be made.  Things are getting too much for him. After the last two dilations he's been very lethargic for two to three days and acted almost depressed.  Also he is now having chronic stomach issues something that they think is a result of the food poisoning he got during his long hospital stay.

 Xander.  What is there to say about Xander?  He is really speeding along in Math.  He seems to grasp math concepts really quickly now.  I asked him what 15 minus 7 was and he instantly replied 8.  He has already buzzed through his kindergarten math book so I am debating moving him onto the first grade one.  I hope he continues to excel at math.  Math is not my strong point and if he can be great at mathematics he can do whatever he chooses.

He is still my clown and makes me laugh daily.  He is also a huge charmer.  I'm going have to watch him when he's a teenager.  Not a day goes by he doesn't tell me how beautiful I am.  He was a soldier this year and he took it upon himself to protect his sisters from all rogue gorillas.  You have to  know Xander to completely grasp that one.

 Jadzia has bumped herself up from princess to queen.  I am not sure how long this will last since she views the honor of princesshood far above that of being a queen.  The main reason for this step down is she has found it as an excuse to wear a skirt under her crown.  How this change bumps you from princess to queen is beyond me but whatever works I guess.

 This leaves us with Inara bug.  She is talking up a storm and her sentence structure is improving.  She talks more and more for Ronan and she signs to him as she is picking up sign language faster than the rest of us.  I think it will be cute to see them carry on a conversation in sign language.

She has started calling Ronan "Elmo."  This stems from Xander's nickname for Ronan which is "Mo Mo".  Inara called Ronan "Mo Mo" for quite some time but then one day she decided Elmo suits him better."  I think in reality she just likes saying Elmo because when she has her play phone Elmo is always the one calling.  She'll go "Ring Ring."  Pick up the toy phone and say "Yes."  Then she hands me the phone and says "Mama, it's Elmo." 

And because I have way too many pictures here's a random stream of photos.

 The kids Fest.  Ronan was home sick and Landon was with Grandma Fullmer.

More Randomly cute and silly photos.