We went in for Isaac's surgery and everything went very well but we may have more difficult procedures ahead depending on the results of the procedures. 
          We should have results of the CT scan in a couple weeks regarding his lungs.  At the last minute they decided not to check his heart with the CT scan as they want to do a night study that will check his heart in a better way.  I don’t know exactly what they are checking with his heart right now as I believe there are several things they are looking at, but his heart rate keeps dipping into the 50’s, 3 to 5 times a night and the doctors ( as well as my husband and I ) are concerned about it.  During the night study they will also check his oxygen levels to see where they’re at while he sleeps.  The date has not been set for the night study but it will happen soon. 

           As for his ears, the tubes (tympanostomy tubes) were put in successfully but not without much cleaning first.  Isaac’s ear drums were very inflamed.  The E.N.T. said that one’s ear drums should be paper thin, but Isaac’s were very thick and full of pus.  After releasing the pressure and cleaning both ear drums the E.N.T. put in antibiotics and the tubes.  In one month the E.N.T. wants to see Isaac again to check his ears.  He said there is a possibility that the ear drums will fill up with fluids and mass from the same or another ear infection and if this is the case Isaac will have to go in for surgery again to re-do the tubes in his ears.  We are in strong hopes that in a month Isaac’s tubes will be clean and his ears will be better.  Then we can move forward with the hearing test. 

            The eye procedure that was done on Isaac yesterday is called stenting or intubation.  This process simply puts a small tube into his tear duct to keep it open and allow his tear ducts to drain correctly.  Unfortunately they have realized that the bone in Isaac’s nose is very wide and is pinching his tear ducts closed.  Because of this the stenting procedure may not work and Isaac will then have to go through a procedure called Dacryocystorhinostomy. This procedure is very painful as they have to remove part of the bone to make way for his tear ducts.  For this reason I am praying his procedure done yesterday will work. We will see the Ophthalmologist in a month as well to assess the results of his procedure. 

This road has been a long one for our whole family, but through it all we have grown and it's all because of our Little Isaac.

 

        I've never written a blog before and Isaac's history is so lengthy that I hope I don't lose you in all of this, but I will not write EVERYTHING and I am writing this blog to help people understand what has happened in the last 20 months from the time Isaac was born to now.  I do not wish for people to feel sorry for us, but I know people want to know what is going on and in writing this I not only inform you, but I create a journal entry that hopefully Isaac can keep for the rest of his life; learning how amazing he is and how much our Heavenly Father loves him.
            From the day Isaac was born he began to struggle. But in all of God's wisdom, Isaac was given the strength to live.  During the first hour after Isaac came into this world, nurses were scrambling while 9 other babies were born at almost the exact same time.  Isaac was one of the last infants to be seen, and since we were both so exhausted, I asked the doctor and nurses if I could just hold him on my chest while we waited.  All I wanted was to be close to him. Little did I know that having him on his stomach was saving his life. It wasn't until a nurse came to take his vitals and put him on his back that she realized he stopped breathing.  Without a word the nurse took him out.   An hour later I was informed that Isaac had stopped breathing three different times and had to be resuscitated. He was not allowed to be put on his back as this would cause him to stop breathing and turn blue.  I went to him immediately and was told he would stay in the NICU the remainder of his stay at the hospital.  Little did I know this was only the beginning of Isaac's struggles.  Within a week and with some accomplished goals we were finally able to bring Isaac home. The very next day I began the first of many doctors appointments.
             The doctors thought he had Pierre Robin Syndrome but this was ruled out quickly as Isaac began to grow into his jaw.  A cranial specialist sent us to an E.N.T. as Isaac began making a short high pitched squeak.  A lot of people thought he had the hiccups but we knew better and soon learned that this was what doctors call stridor.  Stridor meant that Isaac was using every ounce of strength he had to breath.  Already on a monitor at night we fought to get him on oxygen at all times as the stridor got worse.  The E.N.T.  diagnosed Isaac with having Laryngomalacia.  This is caused when a baby has immature cartilage in the upper larynx and it flaps down cutting off air supply.  After other attempts to stop the strider with prevacid Isaac got sick and breathing became harder.  At 2 months old the E.N.T. said he had no other choice but to do a surgery called Supraglottoplasty.  This would open up Isaac's Larynx and therefor give Isaac the open airway he so needed to breathe.  It took him a while to recover as his body was very weak and in a lot of pain, due to the surgery.  He didn't want to eat for several days and we had several scary moments where he would choke and gasp when trying to eat but he eventually began to eat again and came off the oxygen within a few weeks of the surgery.  This whole time I cried and prayed often that my little boy would survive and as many people prayed too I could feel angels watching over Isaac. This helped me to sleep a little more at night during this difficult time.  After about a month Isaac began smiling.  I was so excited to see him smile. I continued to take Isaac to a couple of doctors and he gained a little bit of weight, but he still had what doctors call low muscle tone so he couldn't lift his head up nor was he even trying to move at all. Something didn't seem right but we kept moving forward with hope that Isaac would be completely better now that he had surgery.
            Isaac had several good months of progression but in February of 2014 Isaac got sick with RSV and was put back on oxygen.  Week after week for several months I went to our primary care doctor to see if Isaac was well enough to come off of the oxygen but to our horror Isaac got pneumonia as well.  His oxygen levels became worse, but his primary doctor could not figure out what was wrong, so he got us into a pulmonary and a GI doctor. Both of these doctors were from Rocky Mountain Hospital for Children.  The pulmonary doctor said Isaac was by far his worst patient and put him on 6 different medications in one day.  He also wanted to do a bronchoscopy and a biopsy of Isaac's throat.The GI doctor diagnosed Isaac with chronic constipation and said that he needed to do an endoscopy to find out what was happening inside Isaac as well. Both the pulmonary and the GI doctor set up a day to put Isaac under anesthesia and find out what was going on.
           Isaac, at 12 months old, weighed 14 lbs 10 ounces, and he was still fitting in 6 to 9 month old clothes.  He could roll over but he could not sit up or crawl in any way.  I began to see many differences between other babies around Isaac's age as the other babies were starting to walk and my son just barely started holding his head up.  I got Isaac into physical therapy at Children's Hospital with the primary care doctors help. For Isaac’s first birthday he stuffed his face with cake, being my first child to do this.  I took lots of pictures.  We were all so excited for him to make it to a year.  All of his extended family, that lived close by, came to celebrate with us in this joyous occasion.  We celebrated his birthday a couple days early as he would be going through the procedures that the Pulmonary and GI doctors set up one day before his birthday.  On the day of the procedure they put him under anesthesia and we found out that, he had candida (a yeast infection) all over his throat. His larynx was inflamed even more than when he was born and the pulmonary Doctor couldn’t understand how Isaac could breathe at all. The GI Doctor put him on medication and we were told Isaac could not eat anything thinner than a honey like consistency as he was aspirating all liquids. Isaac also had severe acid reflux, but we never new it because it was silent, which meant Isaac never spit up /threw up and he never cried. Isaac's temperament had always been amazing as he smiled a lot but never cried.  He also never laughed but he was my happiest baby. This was wonderful but it made it hard to figure out when and what was wrong with Isaac. I came to understand what a blessing it was to hear my babies cry when something was wrong or they needed something.  Our ward at church was amazing as they brought in meals for our family, set up babysitters for our daughters and gave us emotional support. I took him in to see his primary care doctor for his follow up appointment and one year old check up.  His Doctor diagnosed him with failure to thrive. He told me he was going to send us to a pediatrician at Children's Hospital where a team of doctors would all wrap their head around Isaac's condition of health and figure out what was wrong.  This was all a shock to me as I thrived on consistency and didn't want to lose the only doctor Isaac had had from birth, but it also gave me comfort amongst all the emotional stress of what was happening to Isaac.
           With our family needing some well deserved vacation time we left to see my parents in another state with permission from 3 different doctors. They said Isaac should be fine as he was on medication, had oxygen, and we were going to a place with lower elevation so he would be able to breathe better.  While there we enjoyed being around family and not having so many doctors visits but this was short lived when Isaac aspirated some food and had to be hospitalized.  Within the same vacation he got pneumonia and had to be hospitalized again.  While at the hospital we figured out he was loosing weight very quickly.  Being as how he was one years old and had bad rashes due to Eczema, I had switched him to almond milk witch seemed to rid him of his rashes.  Because the almond milk did not have a lot of calories though we also fed him high calorie foods like avocados, but he still continued to lose weight. I tried giving him PediaSure to give him more calories than the almond milk and other food could provide but then he stopped eating all together.  I couldn't figure him out so when we got back home I took him to his new pediatrician at Children's Hospital right away.   She, being the wonderful doctor she was and still is, jumped into Isaac's life and began ordering tests, other specialty doctors, and making a plan of how to help Isaac gain weight. Two days later though, while taking Isaac in for a swallow study test at Rocky Mountain Hospital for Children with his speech therapist, I asked if we could admit him as he was lethargic and too weak to hold his head up.  It was at this point that Isaac was admitted and later received the G-tube, and a Nissen.  The G-Tube would help us to get Isaac all the nutrition he needed with the highest calorie, most hypoallergenic formula there is today and the Nissan would prevent any acid from going up his esophagus ever again. They also found and fixed a hiatal hernia while he was in surgery. 
          For the next year Isaac was placed with all sorts of different specialty doctors.  He had a dermatologist for something called Molluscum on his chin. We found out this meant he had a low immune system.  Isaac was also set up with amazing therapists for physical, occupational, and speech therapy.  They  came and still come to our home every week to help Isaac. Within the year Isaac was also placed with an Optometrist and an Ophthalmologist for his consistent teary / gunky eyes and he was diagnosed with very poor eye sight. He got glasses right away to prevent him from losing his eye sight more. He was given a new E.N.T. specialist as the other E.N.T. specialist moved and in the process Isaac underwent more procedures to try and figure out why he had and still does have so many ear infections and why he still could not come off of the oxygen while sleeping.  He got a cardiologist as recently Isaac's heart rate began dropping very low and being irregular. They also figured out he has an irregular shaped heart and there may or may not be more problems with it.  Isaac was sent to 2 different Geneticists who began checking him for a syndrome. In the process of checking for syndromes the doctors found he did not have MPS (Mucopolysaccharidoses),  BUT... For the first time in almost two years we found out last week that Isaac has a syndrome called Cornelia de Lange Syndrome.  This syndrome causes all of the many health problems Isaac has had and sometimes causes more severe health problems.  Our family is so blessed that Isaac was not missing limbs when he was born as some other children are who have this syndrome.  We are also very blessed in that this syndrome is not a progressive syndrome where things will continue to get worse and worse.  We found out that kids with this syndrome have their own growth chart because they grow at such a slow rate but they all fall within a general range.  Isaac is in the 80th percent tile. For the first time I have hope that Isaac can eventually get better and move forward.  He does tend to take two steps forward and then one step back with his health and currently he cannot eat or drink anything as he is aspirating everything but he has learned how to crawl on his hands and knees.  He is also working very hard to climb up the stairs and pull himself to standing.  He is very proud of himself when he can do something new and he absolutely adores his sisters who are always there to play with him and doctor him when he needs them.  Our family has learned so much about how much our Heavenly Father loves each one of us. We have seen miracle after miracle in Isaac's progression to be healthy and strong.  Isaac continues to get sick often as his immune system is very low but he is a fighter and never gives up.  In regards to the facial characteristics that Isaac does relate with in the Cornelia de Lange syndrome; he has two long thumbs that are set back on his hand, he has bushy eyebrows and his nose is said to be slightly flared. His skin has a purplish marble looking tone to it and his pinkie fingers are always curled.  He has very long eyelashes and he has a lot of hair in general.  Now that we know he has this syndrome he will get an Immunologist Doctor as well as a Nero-psychologist.  Our friends and family were and still are here for us constantly.  We really could never have gotten through this without them.  We have been so blessed.

           I will try to stay on top of this blog if people continue to want to know what's going on.  This coming Friday he will be having surgery to put tubes in his ears (because of all the ear infections) and he will be getting stents put in all of his tear ducts due to his eyes watering through out the day and getting gunky. He will also be getting a CT scan to check his chest, lunges, and heart.  And he will have several tests done to check his immune system again.