Start to a new year....

January 2016 – March 2016

January was a busy month of appts.  We started to notice a weird honking noise when Landrie was running (see video below).  The winter months had been rough with sicknesses already and the chronic cough and congestion has not improved.  She was also having episodes of respiratory distress for no apparent reason.  I had made an appt with Pulmonary doctor a few months ago but took until January to get in to see him.  He watched the videos I had taken of Landrie and he said it sounded like her trachea was still collapsing.  He could also hear some wheezing when he listened to her lungs with a stethoscope.  He recommended putting her under and doing a flexible bronchoscopy and lung wash.  He sent a note to her ENT to let them know and they decided that they wanted to be involved in the scope also and they decided to make it a triple scope….flexible and rigid bronchoscopy and endoscopy and they would remove her adenoids if they were swelled.  We also had an appt with Genetics to see if we could find a genetic disorder causing her issues…especially the unexplained autonomic nervous system issues.  Despite her issues, she did not meet certain markers that the insurance company would cover the tests and they are expensive.  So we passed on doing any testing at this time.  If anything comes up later, we will revisit.  I started a new job with a company I had worked at a few years prior.  It was a good change for me and they are very understanding about my daughters health issues.  At the end of the month, Landrie turned 18 months old and hit the 7 percentile for weight….slowly increasing.  Which is funny, because she eats a ton now….the girl loves food. 

We weaned Landrie off the Erythromycin and she was doing great off of it…no reoccurrence of gastroparesis.  Her GI doctor said we could start to wean her off the Nexium.  No meds for first time in 15 months….

She had a bad sickness in February that got her admitted overnight for observation.  Her breathing was crappy and oxygen levels were low.  Thankfully, she bounced back quickly and we waited for her scopes in March.  

The rest of 2015

April 2015 - December 2015

Landrie recovered well from surgery.  She started to army crawl at the end of April just before turning 9 months old.  She continued to have a chronic cough and congestion but her breathing sounded good.  This time period is full of a lot of doctor appts and tests to figure out some weird non-breathing related issues.

May – She met with ENT for her follow-up after surgery/scopes.  He recommended another swallow study and evaluation with feeding team to make sure she wasn’t aspirating….she sounds more congested after drinking bottles which can be a sign.  Her swallow study showed penetration, which means the liquids go into the top of airway but doesn’t go down.  She did this more and more as she drank.  They didn’t think they saw it aspirate but wasn’t 100% sure.  They were concerned about it and said she was definitely at risk to aspirate so they recommended we continue her on nectar thick thickener.  She also hadn’t gained any weight since the surgery.  Every time I would increase her food intake she would vomit.  And was struggling to eat finger foods.  She went from the 21^st percentile before her surgery to the 5^th percentile 3 months after surgery.  The feeding team suggested an appointment with GI doc for reflux and to check for Gastroparesis (slow emptying stomach).  They also recommended having her evaluated by Early Intervention.  

June – We made an appt with her cardiothoracic surgeon for an echocardiogram.  Her hands and feet had been swelling and turning colors (red splotchy or blue/purple) since her surgery….didnt happen all the time, but would randomly happen.    Docs were concerned something could have gotten crimped in her chest when they moved things around.  They echo looked good but she drank a bottle while they did the echo to keep her still and it showed her heart rate getting elevated while drinking again.  The CT surgeon was not sure what was going on with the swelling and discoloration, but was concerned.  He referred us to a diagnostic doctor.  She also had an evaluation with Early Intervention…they said that she was behind in her feeding skills.  Her lip, mouth and tongue muscles were very weak causing her to not chew and move food around properly causing her to cough and gag to the point of vomiting.  So they recommended occupational therapy (OT).  She had an appt with ENT at the end of the month to discuss swallow study, feeding team and OT findings.  I also mentioned that she was still having pauses in her breathing when she sleeps and was that normal for her age.  She wasn’t having a ton of them but enough that I was concerned by it.  Its very scary when her Snuza monitor goes off in the middle of the night and she is not breathing when I check on her.  He did a laryngoscopy in the office to check on her tonsils, adenoids and larynx to see if there was anything that could be causing sleep apnea.  He said all looked good.  e recommended a sleep study. 

July – The GI doctor did believe she had Gastroparesis so she was put on Erythromycin which is an antibiotic.  So that sounds weird, but it is prescribed at a low dose and it helps by increasing the contracting of the stomach muscles which move the food.  Doc kept her reflux meds the same.  Landrie also started her OT…it was very interesting watching how they get a little one so young to exercise their mouth muscles.  We met with the Diagnostic Group Doctor to discuss the hand/feet swelling and discoloration issues.  He said there could be something going on with her autonomic nervous system which could explain the hand/feet thing along with a few other things like the Gastroparesis, heart rate and temp control issues.  He asked me to keep a detailed log of anything I notice and we would follow up in a few months.  If symptoms worsen, than they will do an MRI which they would have to put her under for.   One week before Landrie’s 1^st birthday, she had a sleep study done.  That was interesting!   All sorts of wires were hooked up to her.  She wasn’t thrilled by this but did surprisingly well.  She went to sleep pretty quickly and slept well.  I, however, did not sleep.  We had to wait a couple weeks to have an appt with ENT to discuss the results.  Landrie turned 1 year old on July 30^th!  

Sleep study set up....someone not happy

Lots and lots of wires....

 

How can you sleep like this?!?!

Happy 1st Birthday!!!

 August – I was quite annoyed by at her ENT appt.  She was scheduled with her regular ENT but some other doc that we have never met came in.  He said that she did have some apnea moments during her study but it was within normal limits.  He also said her adenoids were a bit swelled…not enough to do surgery yet but probably would need them out in a year or 2.  He would not give me details about the sleep study which really annoyed me, so later, I requested her records.  The report said her lowest O2 level was 91%....So that’s not too bad.  She had 5 central apneas, 1 obstructive apnea and 4 obstructive hypopneas (shallow breathing) with a total apnea-hypopnea index (AHI) of 1.3 per hour.  Normal is considered 0-4 so that is pretty good but still makes me nervous when I see a pause or her monitor goes off.  At the end of August, Landrie started walking!  

October – Had several follow up appts this month.  The Diagnostic Doctor wanted us to continue to monitor the autonomic nervous system issues since they hadn’t gotten worse.  But something odd I did notice during the summer was that every time Landrie was in a pool(fresh water or chlorinated), she would develop a fever shortly afterwards that would last for 1-3 days.  Doc thinks it could be related to the autonomic stuff.  Her GI doctor recommended trying to wean her off one of her reflux meds(zantac).  Over the last few months, the vomiting had gotten much better and she finally started to gain some weight.  Still slow, but gaining.  She was also eating finger foods much better after a few months of OT.  They helped us find a sippy cup that works for her and finally got her weaned off the bottle.  She really struggled with the cups.  They recommended a strawed cup so she would keep her head down since she has the tendency to really arch her head back to drink her bottles because it opens her airway up(she does this sleeping too). But because it opens airway up…it increases risk of aspiration.  So they said a strawed cup will force her to keep her head down and lower aspiration risk.  But finding the right cup was tough…she couldn’t get anything out of the ones with a valve or had to pinch with lips because she didn’t have the mouth/lip strength and then she would get too much out of regular straws and would choke.  Plus some cups just didn’t work well with the thickened liquids.  OT had us try the Rubbermaid juice boxes…They are great…No valve or pinching needed but the straw hole is a bit smaller so not a big amount of liquid comes out but its big enough for the thickened liquids.  .The feeding team recommended we continue thickening liquids due to risk of aspiration.  They wanted us to give Simply Thick a try since she was over 1 year old.  We liked it much better than thickening with rice cereal.  They wanted to see her again in the spring to evaluate going off thickener.  

The strawed cup that Landrie could use

November – Through the Early Intervention program, Landrie start speech therapy.  She was a little behind in her speech.  It was not too bad but due to the issues with her airway and mouth muscle tone…both could cause speech delays….her docs recommended we start therapy a little early.  We had a nice Thanksgiving.  Black Friday was the anniversary of when Landrie stopped breathing.  That day will always haunt me now. 

December – Had a break from appts this month and enjoyed the holidays!

Aortopexy

March 19, 2015 – April 1, 2015

Aortopexy – a surgery where they suture the aorta to the sternum.  The innominate artery (also known as brachiocephalic artery or trunk) comes off the top of the aorta so both gets moved away from the trachea.  This is the standard surgery for severe Innominate Artery Compression(IAC) Syndrome.  Doctors usually only consider surgery if the compression is more than 40% and they are symptomatic.  

Diagram of Aorta and Innomate Artery(Brachiocephalic Artery or Trunk)

 

Aorta in relation to trachea and heart

 

Innominate Artery compressing trachea

Landrie had an Aortopexy done on March 19^th.  I was very nervous leading up to this surgery because it sounded scary… the aorta is the main artery of the body and any damage to it could be BAD.  The surgery took a few hours and went smoothly.  They said they were able to reduce the compression from 90% to 30%.  She was out of it the rest of the day but not overly fussy.  She had issues with tachycardia (high heart rate) after the surgery.  The doctors were not sure why.  They increased her pain meds thinking that was the cause even though she was not acting in pain.  It did not help.  Her heart rate was staying around 190 and spiking to 210 when drinking bottles and this was while calm and happy.  For her age, 160 should have been the max heart rate.  They did blood work and an EKG but other than it being rapid everything looked fine.  They assumed it was just that her body needed time to adjust to the aorta being moved even though it is not a common side effect of the surgery.  She came home on March 21^st with a 24 holster monitor.  The results showed everything was fine except for the heart rate being fast.  Her heart rate returned to normal after 2 weeks.  Her heart rate still increases when she drinks bottles and still has occasional episodes of it being high. No one is sure why, but the episodes usually only last a few hours.  Landrie recovered well at home.  Her surgeon said she would be back to doing normal stuff within a few days but I really didn’t believe him.  But he was right…within a few days she was doing all her normal 7 month old things including jumping in her jumper…which looked painful but she wasn’t bothered.  She had a follow-up with her cardiothoracic surgeon on April 1^st and he said her incision looked great and released her from his care.  

Top - before scope with 90% compression; Bottom - after scope with 30% compression

"And though she be but little, she is fierce!". - Shakespeare

After surgery with chest tube still in place

 

Smiling the morning after surgery