Today is Rare Disease Day.  Noah has the rare disease of Congenital Central Hypoventilation Syndrome (CCHS).  A rare disease is defined as "any disease or condition that affects less than 200,000 persons in the US or about 1 in 1,500 people"  (Rare Disease Act of 2002).  Rare diseases can be difficult to diagnose and because so few people have the disease, research for a cure is not widely available.  Approximately 50% of people affected by rare diseases are children.  30% of children with rare diseases will not live to see their 5th birthday.  Rare diseases are responsible for 35% of deaths in the first year of life.  According to the Kakkis EveryLife Foundation, 95% of rare diseases have not one single FDA approved drug treatment.  Approximately 50% of rare diseases do not have a disease specific foundation  supporting  or researching their rare disease. (From globalgenes.org). 

CCHS is NOT a lung disease.  Noah's lungs are perfectly normal, even though he must use a ventilator while sleeping or sick.  CCHS is a disease of the autonomic nervous system.  In addition to problems with breathing, CCHS patients can also have problems with regulating heart rate and blood pressure, have abnormalities in the nerves that control the digestive tract (Hirshprungs Disease), have an increased risk of developing tumors of the nervous system (neuroblastomas), have eye abnormalities (decreased response of the pupils to light), decreased perception of pain, low body temperature and sometimes profuse sweating.  They can also have learning difficulties and other neurological problems.  Currently, there are only approximately 1,000 people worldwide with CCHS.  More than 90% of cases occur from new mutations of the PHOX2B gene and there is no history of the disorder in the family. 

In Noah's case, he has a mild mutation of 20/25.  We have not noticed any issues with his heart rate and blood pressure as of yet.  He gets an annual holtor monitor to check for heart pauses.  He does have some eye issues and had to have his eye patched when he was younger.  I have also seen his decreased perception to pain.  We call him our tough guy because he rarely cries when he gets hurt.  He does have issues with being hot most of the time, especially in the summer.  We also have to be careful in the winter because he can get cold very quickly.  As for learning difficulties, we have not noticed any as of yet except for his speech, which I think has to do with being in the hospital for 4 months and having a trach at an early age.

It is amazing to think how one small change in one gene can create such widespread issues throughout the whole body.  This is a lifelong condition for Noah.  He will never outgrow it and will never get worse, but also will never get better unless there is a cure.  We have to be vigilant every day of his health and make sure he is well ventilated to prevent serious complications. 

My hope for him is that there is a cure.  My goal is to help raise awareness of CCHS in the hopes that it will become recognized by the medical community and that more research is done to help find a cure.  I am so thankful for our small, but close knit CCHS family and our CCHS Network.  We have never met face to face and some of us are in different countries but together we have our amazing children who fight a battle every day.  We can learn so much from them and from each other.  

Please take a moment to think of those of us who are faced with rare diseases every day.  Help us spread the word and find a cure. 

(Special thanks to Jo Cramer Isley, a special mommy to our CCHS friend Lacy) 

Trach Myths

1.  If your child has a trach they cannot talk--This is not necessarily true.  The trach can interfere with speech but there are some things that can help with this.  The PMV (speaking valve) allows the air to be breathed in through the trach but back out of the nose. The cap completely closes off the trach and the person is able to breathe the natural way.  It took Noah 2 years (yes Years) to get use to the PMV.  He hated it.  We would put it on and he would scream.  One day while at the beach we popped it on to show everyone how he screamed while wearing it and miraculously he did nothing.  He tolerated it and even began making noises.  I found that the PMV dried him out terribly and we had to be really careful to prevent plugs  (this is where the trach becomes plugged and no air gets in or out.  It is dangerous for trach patients).  Shortly thereafter we tried capping him.  The first try was disastorous.  He also hated it.  But a few months later he didn't even realize we had put it on him.  Now he wears it any time he is not hooked up to the vent.  It is a miraculous little invention.  Who would have thought such a little piece of plastic could be so important and amazing.  Noah did learn how to make sounds and even could say a few words without using the PMV or the cap but his voice was very soft and he only said a few words.  So, even if one doesn't tolerate the PMV or cap, it is still possible to vocalize around the trach.

2.  Your child can never take a bath or shower.  They must be spongebathed--We have always given Noah baths.  As a baby, we used the baby bathtub.  Now he sits in the big tub. Before being capped, he always wore his HME in the tub.  Now we use the cap.  I keep the water shallow and let him splash to his heart's content.   I have him hold his head back and rinse his hair. 

3.  Your child cannot swallow with a trach--Depending on the diagnosis, this is not necessarily true.  A lot of trach babies have problems with aspiration but no trouble swallowing. In Noah's case, he cold swallow but after doing a swallow study, we found that the possibility of his aspirating was high.  Because of this, we had to thicken all of his liquids.  He had many swallow studies and we did vital stim therapy for about a year (this is where electrodes are placed on his throat and a small current is used to stimulate the throat).  Finally, after 2 years he passed his first swallow study and showed no signs of aspiration. 

4.  Your child cannot swim with a trach--We live in Alabama.  The summers are brutal and humid.  It is impossible to do anything outside for any length of time without water being involved.  We go to the beach every year for vacation and sometimes go several times throughout the year when it's not hot.  Swimming is a huge part of our lives.  Because of the trach, there is an open hole that leads directly to the lungs.  We have to be very careful to keep water out.  At first, we used HME's then graduated to the cap.  When Noah was a baby, I would put him in his baby float and keep the suction handy.  He was content to sit in that and float around.  Now that he's older, it's becoming more of a challenge to keep him safe in the water.  This past summer we went to an indoor water park for a week.  His favorite ride was the lazy river.  We were able to sit him in one of their double floats and he was able to enjoy the water without being submerged.  They had a huge kid area that was only about 2 inches deep.  There was a huge climbing structure that squirted and dumped water.  They also had a big regular pool.  We just carried him around in this one.  He actually did fall face first a few times and scared me to death, but he came up laughing and since he was capped there were no issues.  I made sure to keep the suction machine close at all times.  We also purchased a small life vest, which provided extra protection.  The trach was hidden behind it and I think that even when he fell face first, the trach never got in the water, just his face.  I also kept a swim shirt on him and tucked his trach (he has the longer bivona flex) underneath.   I remember once seeing a picture of another CCHS child in the pool and he even had his ventilator.  They just had the ventilator sitting by the side of the pool and he was in the shallow end, circuits and all.  This inspired me and I vowed to allow Noah to have the same experiences as his sisters.  Every child is different and doctor's should be consulted before trying any of this.  I am in no way advocating that every child with a trach should swim.  I'm just stating what has worked for us. 

5.  You can never travel in the car by yourself if your child has a trach--We have never had nursing so all of Noah's care is my responsibility 24/7.  With two older children, we are always on the go.  Plus, Noah was constantly required to be at many appointments when he was younger.  I really had no other choice than to travel alone with him.  It was definitely a pain when he was hooked up to the vent because he always seemed to pull his circuits off.  I made sure to keep him attached to his pulse ox and placed the ventilator so I could see the numbers.  I also had a mirror that I used when he was backwards facing.  Every time he pulled the circuit off, he was awake and the alarms were always false.  I just found a good spot to pull off the road and reconnect him.  Of course, traveling alone depends on the child's specific health issues.  Some children need a lot of suctioning and some children are dependent on the ventilator and cannot breathe if it gets disconnected.  In these cases, I would not travel alone.  Again, consult your doctor on traveling tips.

6.  You can't travel too far from home or your hospital--For us, this was never the case.  Our first trip was to the beach when Noah was 7 months old.   (3 months after being released from the hospital).  Since then, we have been to Disney, the beach every year, the Wilderness at the Smokies Water park,  and several other places.  I always pack a lot of supplies, several extra of everything.  Traveling long distances can be done, it just takes a lot of planning.  The hardest part is figuring out how to set the vent up in these different hotel/condos. We make sure to take our long extension cords and our power strip. 

7.   You must have a full time nurse--We've never had nursing.  Our insurance does not cover it.  I'm not going to go into a lot of detail here because I've already blogged about this but it is definitely possible to take care of your trached child with no nurses.  (See my blog on Nursing).

8.  Your child will never have a normal life--Again, I'm not going into a lot of detail here because I've already blogged about this.  (See my blog entitled Normal).  I must say that Noah is as normal as you or I.  He just needs help breathing at night and when he's sick.  He goes everywhere we go and has all of the experiences that my girls had at his age. 

9.  Your child will have to be institutionalized--Unfortunately, this is what we were told when Noah was in the NICU.  The doctors were baffled.  They ran every test they could think of and everything came back normal.  He had EEGs, EKGs, and tons of Xrays.  Everything was normal.  He had so much blood taken from him that he had to have 2 blood transfusions.  There is nothing more horrifying to a parent to come in the NICU and see an IV in your child's head.  Everyone around us was going home.  Day after day beds were being emptied and there we sat, waiting for answers which would not come.  "I don't know" became the doctors standard answer.  Finally, after he had a seizure and was put on the ventilator, they were going to transfer him to Children's Hospital.  The doctor came over to us, handed us a piece of paper and said that they thought that he had Ondine's Curse.  She said in all her 40 years of being a doctor, she had only seen one case.  She then went on to say that he would more than likely be institutionalized the rest of his life.  Her parting words were for us to read the paper she gave us and she was going to leave us to process.  I was in complete shock.  I looked at the paper and it was one paragraph on CCHS.  It was so complicated and very negative.  At this point, I lost hope.  I lived in a deep dark fog until he was finally moved to Children's and they were so encouraging.  The day we got the diagnosis, all the doctors gathered around (which is never a good sign) and told us they had the test results back and it was CCHS.  I immediately broke down because I had been reading things on the internet and after being told by that other doctor, I was thinking that he would not be able to live with us.  Through my tears, I heard his new doctors say that everything would be ok.  They told me he would live a normal life and be running around like a normal toddler.  They said he could do anything he wanted to do (except maybe deep sea dive).  I began to feel hope again.  They were very honest and said it would be a long process to get him home.  I remember saying "home?".  They probably thought I was hard of hearing or something because he kept repeating the fact that Noah would come home.  In our case, the doctor was wrong.  They did not understand the disease and were not very educated.  I think they underestimated us.  For some, an institution is the only option.  Some care is so involved that one person or even a whole family cannot handle doing it alone.  In Noah's case, it just did not make any sense. 

Living with a trach is certainly different.  The main thing is know what to do in an emergency situation.  (trach plug or decannulation-when the trach comes out accidentally).  It is important to know how to clean the trach site and prevent infections.   Other than that, it is not complicated at all.   My main goal is for people to see that the trach is no big deal.  It's really no different than someone using an inhaler or a shot for diabetes.  Don't be scared of it just because it's something you don't see every day. 

The Box

People like boxes.  They help with organization.  They help with the storage of materials.  They make things neat and tidy. They really do make life easier.  That is, unless someone tries to stuff you inside one of them.

People are always trying to place Noah in boxes.  Not really literally, just figuratively.  Every medical procedure has forms containing many boxes.  I am asked to check the boxes that apply.  I never check any of them, except the "no" boxes.  Then I am asked to explain myself.  Does your child have a lung disease--no; then why is he on a ventilator?  Does your child use oxygen--sometimes; how much oxygen--depends on his sats.  Is your child allergic to any medication--no but please do not give him propofol as a sedative because it could cause heart pauses;  Does your child see a cardiologist--no but we do annual holter monitoring.   See the pattern?  We do not fit into any of their little boxes.  This just seems to confuse them.  It is very difficult to comprehend that Noah has a trach and is on a ventilator but is perfectly healthy.  Yes, we do have to monitor him carefully but he lives his life just as you and I live ours. 

All of this can be very frustrating.  CCHS is such a complicated disease.  Every child is different.  Even children with the same mutation number can have different issues.  That is what makes this all so challenging.  Our kids do not fit into anyone's box.  It doesn't matter how many times you try to cram them in there, they just won't fit.  We live outside the box. 

I am hoping that one day there will be a cure for CCHS.  It would be nice to know that there was a simple medication that can make Noah breathe (there is currently a study being done with one of Noah's brave CCHS sisters, Zoe, who is testing this medication).  But until then, we continue to live outside of the box and will teach Noah that he is a unique individual who has a great capacity to teach the world about living outside of the box. 

Coming Home

December 15, 2009.  The day I will remember forever.  The day we finally got to bring Noah home after almost 4 months of being in the hospital.  One of the happiest and the scariest days of my life.

Babies do not come with instructions,  unless your baby has a trach and a ventilator.  Before being allowed to bring your ventilated baby home, there are many things that have to be done.  The first is that you have to be trained on how to take care of him.  This takes several months of training.  Our training consisted of myself, my mother and my mother in law being at the hospital 3 days a week for a month.  We were required to do three each of everything (three trach changes, three circuit changes, three trach cleanings, three bolus feedings because at the time, Noah still had a g tube, and even three baths.  Yes, bathing a ventilated baby is not easy, especially when you are trying hard to keep the water away from the trach).  Another requirement for discharge is that they give you a list of things you must have then they come inspect your home to make sure it is similar to the NICU.  Finally, after the training is over you must do a 4 day trial in the hospital.  This is where you spend 24/7 at the hospital providing all of the care.  The doctors and nurses come in occasionally to check vitals but other than that, you are on your own.  Another requirement was that we go home with a CO2 monitor.  This was (and still is) unheard of.  These machines are rarely used, especially in the home.  Supposedly, the doctors and the DME (equipment company) had taken care of getting us this machine.  However, they had the wrong machine.  Instead of one that can be used continuously, they only had one that did spot checks.  It's always a bad sign when 2 doctors and the ventilator nurse comes in and tells you we need to talk.  Fortunately, they recognized that we had been taught well and were capable of keeping Noah alive at home so they signed the release papers with the promise from the DME that they would get us a better CO2 monitor.

The day finally arrived.  However, it wasn't as simple a homecoming as I would have liked. Chris had to take Hannah to the doctor and she was diagnosed with a stomach virus.   Beth had her first band concert that very night at school.  The DME was to meet us at home with all of the supplies and help us set everything up.  Somehow, we made it through the day.  My daddy stayed with Hannah.  My mother and father in law stayed with both Hannah and Noah that night so that we could go to Beth's band concert.  The DME met us before the concert to bring all of our supplies and set everything up.  In addition to our regular supplies, they brought us oxygen, which Noah had not needed, and they had to show us how to use that.  We finally made it home that night and got everyone in bed.

Looking at Noah at home in his crib, I was both elated and terrified.  At the time, he was hooked up to his ventilator, pulse oximeter, sleep apnea machine, and his feeding pump.  Our dining room had been transformed to a mini ICU.  Watching him sleep, I was overwhelmed with the thoughts of how in the world can I do this?  How can I keep my baby alive?  How am I suppose to do all of this doctor/nurse stuff when I have no medical training? Not only was I worried about all of the medical stuff, but I was also worried about the boy stuff.  Having 2 girls, I had not been exposed to things like cars (except Barbie cars), superheroes (except Wonder Woman), what was I going to do with a boy?  This was when I seriously wished that there was an instruction manual.

In addition to all of this stress, Noah decided to get sick.  I remember on Christmas Day keeping him on his vent and using the oxygen.   Christmas was a little weird that day but at least we were all together under one roof and we were home.  Because of this,  Christmas is even more special to me.  The Christmas trees in Children's Hospital's lobby always remind me of our time there. 

Noah has been home for 3 years now.  I guess I didn't need an instruction manual after all.  We have learned so much.  Some things were taught to us by other people but most things we learned by trial and error. 

In the midst of all of the gift giving, eating, and visiting during Christmas, I always take a few minutes to reflect on where we were three years ago.  We are so truly blessed.  I always wondered if Mary felt the same overwhelming fear when she had Jesus.  I wonder if she sometimes wished for an instruction manual too.

Happy 3 year homecoming anniversary my sweet, strong Noah.  

The Things We Take for Granted

Imagine this scenerio:  You and your spouse are driving in the car.  It's a beautiful evening, you've had a busy day and your children are all snuggled in the backseat ready for a long ride home.  You look in the rearview mirror and see that your baby is asleep in his carseat.  All is right with the world.  Or is it?

Scenerio #2: You have had a busy day and a long night.  You sit down to feed your infant and he falls asleep.  You reposition yourself on the couch and lie your baby on your chest.  Both of you take a nice peaceful nap together with you holding him on your chest.  What a peaceful scene.  Or is it?

Scenerio #3:  You've spent the day at the zoo.  Your baby is in the stroller and falls asleep.  You finish your walk through the zoo while your baby sleeps peacefully in the stroller.

Scenerio #4:  You and your family have a late evening and go to a restaurant to eat.  Your baby is tired from the busy day and falls asleep in his baby carrier while you are eating.  You finish your meal while your baby sleeps beside you.


Now, imagine your baby has CCHS.  None of this is possible without a lot of planning.  In all of these scenerios, a ventilator would be a necessity.

CCHS scenerio:  You notice that your child is asleep in his carseat.  First of all, you would have probably already hooked him up to his vent before you started the car.  But, if for some reason you forgot, you would immediately pull over and hook him up.

CCHS scenerio #2:  You cannot fall asleep with your child on your chest without lugging the ventilator in the room, unhooking the heater wire and making sure the battery is full and the inline HME is in the circuit.   You hook him up while the tubes hang off of you and the couch. 

CCHS scenerio #3:  Your child can sleep at the zoo in the stroller but you must first make sure that your vent is packed for travel and hanging on the stroller somewhere.  You must secure the circuits to make sure the stroller wheels do not run over them and if necessary, your pulse ox should be charged and ready to be attached to baby's foot.

CCHS scenerio #4:  You try to plan your meals around naptimes so you don't have to carry the ventilator.  However, sometimes thats not realistic so you throw the vent over your shoulder and carry your baby into the restraurant while trying not to unhook the circuits accidentally, which would cause the vent to beep loudly.   You walk in the restraurant with people looking at you strangely and try to find a table that can accommodate you, your family and your load of equipment. 

All of the above "normal" scenerios happen all the time without anyone thinking about them.  Walking, talking, eating, swallowing and breathing are all things you do without thinking about it.  We tend to take these things for granted.  But what happens when your body doesn't breathe?  Life is so much more complicated.  The ventilator becomes a vital part of your life.  Even such simple tasks such as travelling in the car, eating out and taking a nap snuggled up with your child becomes complicated. Usually a sleeping child evokes a peaceful, calm feeling in people.  For CCHS parents, its a whole different story.  We worry that our child's machine will malfunction and we will not know it,  we worry there will be an accidental decannulation during the night and the stoma will close up, we worry that our child is getting adequate ventilation so the CO2 will not climb.  When I see a sleeping baby now the first thing I think of is that they need to be hooked up to their machines.

My point is that everyone takes the simple things in life for granted.  Take the time to slow down and enjoy the little things.  Just walking to the bathroom is a miracle for some people.  Talking is the main way of communication but some people must find alternate ways, which makes life more difficult.  Eating is usually enjoyable but imagine if you must eat through a feeding tube while everyone around you is enjoying their food.  Swallowing is something that happens without you even thinking about it but imagine a life where you can't swallow; how hard would that be?  Breathing is a vital, necessary part of living but imagine if your body forgets to breathe, which is what happens in Noah's case.  If you don't breathe, you die.  It's as simple as that.  So the next time you walk to the table, sit down and converse while you are eating and swallowing your food and breathing, just remember how blessed you are.  Count your blessings and enjoy every moment of life. 

Pros/benefits of having CCHS

1.  You never have to wait when you go to the ER.
2.  You get handicapped parking because of all of the equipment you have to lug around.
3.  Even the parking attendents at the hospital know who you are.
4.  You have a whole team of doctors and specialists.
5.  You are on the emergency power list and actually get a real person when you call to          report a power outage.  Our neighbors love us.
6.  You are on the emergency list with the fire department.
7.  You never get lost at the hospital because you know where the home vent clinic is located, where to go for speech therapy, where the xray lab is located, where to find one day surgery, you know which floor and where the sleep study lab is located, the cafeteria is one of your favorite places, you know which floor to find pulmonary and the respiratory therapists.  You can even help people who are lost because you also usually know where they are going.
8.  You have a free pass to sleep during the day because you were up all night with the endless alarms.
9.  You do not panic in an emergency.
10.  When your child says a cuss word you can pretend that you don't know what he is saying because his speech is not clear sometimes. 
11.  You have friends all over the world and met them through special needs facebook pages.
12.  You get preferential treatment at Disney World.
13.  When you have to call the doctor, there is no wait.  They return your call immediately.
14.  If you are having a bad day people assume it is because of the beeping alarms keeping you up at night.  You can blame everything on the ventilator.
15.  You always have plenty of medical equipment with you in case of an emergency (gauze, plastic tubing, alcohol swabs, latex gloves and an ambu bag to resuscitate someone.
16.  In the event of an emergency (tornado, earthquake, blizzard, etc) or invasion by a foreign country you have plenty of sterile water bags stored at your house. 
17.  People think you are so smart because you know all kinds of medical terms (vent, trach, circuit, low min vol, peep, ST, OT, PT, vital stim, sats, pulseox, Shiley, Bivona, HME, Passey-Muir, etc).
18.  You are never bored because your calendar is filled with appointments.
19.  You are an expert on breathing issues, feeding issues, swallowing issues, gastrointestinal issues, speech issues, etc. 
20. You never have to buy moving boxes again because you get so many every month with your ventilator/trach supplies. 

Life lessons from CCHS

Here is a list of things that I have learned from CCHS and having a special needs child

1.  Doctors don't know everything.  This is a hard lesson to learn.  I previously assumed that having going to medical school and having a degree made one very knowledgeable in the medical field.  When dealing with someone who has CCHS, this is not necessarily true.  We found that the NICU doctors at St. Vincent's had no idea of CCHS.  There were many specialist brought in to help figure out the diagnosis.  The geneticist told us he "didn't feel good" about Noah.  The NICU doctor handed us a small paragraph out of a medical journal and informed us that Noah would have to be institutionalized the rest of his life and said "I'll leave you to digest that".  Nurse friends said that nothing about CCHS was in any of their nursing books.  Once we moved to Children's, everything began to improve.  The doctors admitted that they have not had many cases of CCHS but were very encouraging and told us that Noah would be just like any other child, except that they didn't recommend deep sea diving.  Once he was turned over to the pulmonologist at children's, we became a part of a team of specialists that were willing to work with us and listen to our concerns.  His pulmo doctor does research on CCHS.   The only negative experience we had at children's was when Noah was admitted to the hospital after being home for a year.  His regular doctor was off that weekend and the doctor on call knew nothing about CCHS.  He ordered unnecessary tests, which I declined and we spent the whole weekend in the hospital even though I could have provided the same care at home.   Although I know that they do not have all of the answers, I feel like he is now in the best possible care.  So even though the doctors and specialists are not familiar with a rare disease, they can learn. 

2.  Being on a ventilator is not a death sentence.  I have watched many fictional medical shows.  On every one any time someone was on a ventilator, they were close to death.  I will admit that before Noah when I heard someone was on a ventilator, I thought it was horrible. When he was first intubated, I was horrified and scared.  So many thoughts ran through my head but the main one was that he was probably close to death because he was on a ventilator.   Now when I hear that someone is on a vent, I know that they just need some extra help breathing and it doesn't scare me at all. 

3.  My girls are extraordinary.  When Noah was in the hospital, the girls were so supportive.  I remember crying a lot and trying to hide it from them.  I was on the front porch one day when they came out.  Before Hannah saw me, Beth turned around and guided Hannah back in the house to give me some time and keep Hannah from being scared.  I will never forget the first time they saw him.  They were both so eager to hold him, regardless of all of the tubing and wires.  They were not scared at all.  They have learned so much about trach care and the ventilator.  Both of them are experts at turning it on and off, hooking him up and unhooking him.  Beth helps with trach care and Hannah delights in telling all of her friends all about CCHS and trachs and ventilators.  They have become so understanding of special needs.  Hannah has a boy at her school with a trach and he has obvious learning issues. She always waves and smiles at him.  Her teacher even let her go to the special ed class and work with him some.  Hannah has wanted to go to dance again but she informed me that if it was too hard on me traveling around with Noah that she would not take dance.  Needless to say, she is starting dance.  Beth really wanted to play regional volleyball last year but settled for playing locally.  She said it would be less stress on us because the regional team travels to other states.   This year, we have told her if she wants regional to go for it and we would manage.  I have always been careful to try to prevent Noah's health issues from interfering with the girls "normal" lives.  It is sometimes difficult juggling nap schedules with volleyball and dance but we manage.  Since birth, the girls and we have been going to the beach every year and I decided early on that we were still going, regardless of a trach and a vent.  They have been so supportive and understanding.  I am so proud of them.

4. Beeping will drive you insane.  Being a kindergarten teacher, I have always been able to tune out noises.  The kids can be running around the house making noise and it doesn't bother me.  I'm use to noise.  Then Noah comes along and the endless beeping began.  There are different beeps for different machines.  The ventilator beeps, the pulse ox has at least 2 different beeps, the CO 2 monitor has 2 beeps.  I have learned what each beep sounds like, without even having to see the machine.  Now I hear EVERY beep.  It drives me nuts when people leave their keys in the car and pump gas with the door open.  All I hear is beep, beep, beep.  The grocery store is horrible.  It's just constant beeping.  We can be sitting in a resturarant , walking in a store, etc and I hear the beeps.

5.  Kids are curious.  I already knew this from teaching but I notice it more now.  They have no problems coming up and asking me what's in Noah's neck.  Several of them have asked if it hurts him.  I explain that it is a trach and it helps him breathe.  They always nod and then smile at him. 

6. Blue is a bad, bad color.  One day without thinking, Noah ate something blue and it stained his lips.  A little while after eating it, I noticed that his lips were blue.  For a small moment, I went into a panic thinking I would need to check his sats and put him on the vent.  Then I realized his lips were just stained.  Crazy, I know but I had flashbacks of his birth when I was holding him and his lips were tinged blue.  Last week at school, Beth texted me that her hands were blue.  I asked her to send me a picture, which she did.  Chris and I were analyzing them and it was so funny because we were commenting on how her fingers were still pink, not blue and her nails were not blue.  Turns out it was just her new blue jeans getting dye on her hands.  But, it made me realize that the color blue is serious business in our house.

7.  Carbon Dioxide is deadly.  The human body is very complicated.  I never really thought about carbon dioxide before Noah came along.  I never realized how important it is for the body to not retain CO2.

8.  "Normal" is what you make it.  I'm not going to go into a lot of detail here because I wrote a blog post on this but I have learned that humans have an amazing way of adapting to any situation and we decided from the first moment of diagnosis that we were going to try to live as "normal" as possible.  Please see blog post entitled "Normal".

9.  You get preferential treatment at emergency rooms when your child is on a ventilator.  A year after Noah had been home, he got really sick and his CO2 monitor stopped working correctly, which we were not aware.  The monitor never indicated that his CO2 was too high.  He was very lethargic, which I though was because of his high fever.  CCHS kids usually do not run a fever, and so my thought was that he was lethargic because of this.  We ended up taking him to the hospital and found his CO2 to be really high.  The funny part was when we arrived at the ER.  We walked in carrying his equipment, he was hooked up to the vent, and we had tons of bags with us.  The police officer at the desk was eating when we got there.  He put his food down and said he would have to search us.  When he saw the huge amount of stuff we had, he said "never mind.  You have too much stuff" and waved us through the metal detector.  This poor lady was behind us with her daughter.  He stopped her and made her let him search her bag.  She gave us the meanest look.  We then walked up to the nurses station and they immediately gave me paperwork and began taking Noah's vitals.  Without thinking, I started spouting off all of the medical terms and numbers to them (what his sats were, CO2 numbers, medicines and times, etc).  I gave them a thorough assessment of Noah and we were taken immediately to a room, where they began breathing treatments.  After the nurses left, Chris told me that they looked at me in amazement while I was giving his symptoms and history.  I never noticed that because I was so focused on letting them know what was going on.  It was so nice not to have to wait in the ER waiting room.  Breathing problems are serious business at the hospital.

10.  Medical shows are funny.  I was a big fan of the tv show ER and I love Grey's Anatomy.  It cracks me up when these shows show people on ventilators.  I'm always finding the mistakes.  I also understand much of the termonology that they throw around in an emergency situation.   I can only imagine what the real nurses and doctors say while watching these shows.

11.  Kids are stronger than adults realize.  Noah is such a strong little boy.  He has fought from the moment he was born to survive.  He has been through so many medical procedures in his short life.   For a long, long time he did not want anyone messing with his feet, even to put on socks.  I guess this is because they stuck him numerous times in his feet getting blood and having IVs.  He also hates bandaids, which is unusual for children.  We even have cartoon character bandaids and he still hates them.

12.  My husband is my hero.  He has been so supportive thorough all of this.  It has been difficult trying to juggle our every day life with all of the appointments, therapies, doctor visits, etc.  Through it all, he has been understanding and strong.  When Noah was in the NICU, he worked himself to death.  He got up and went to work while I sent the girls off to school and then spent the day at the hospital.  He met me at the hospital on his lunch break and spent his hour there.  I was home in time for them to get off the bus.  We then had dinner, did homework and spent some time together before the girls went to bed.  After tucking them in, he left and went to the hospital to see Noah while I stayed with the girls.  Every weekend, we sent the girls to their grandparents and spent several hours at the hospital.  During this time, they were not letting anyone other than parents in the NICU because of the swine flu outbreaks.  The girls were not even allowed to see Noah until he was moved out of the NICU to a regular floor.  This was our life for months.   I will never forget the night he called and told me they had intubated Noah.  They wouldn't let him go back there for awhile and we were terrified.  I could hear how scared he was but he was trying so hard to be strong for me.  That was the scariest night of our life.  The next day was even worse when we got the call for us to give them permission to give Noah a blood transfusion.  I sent the girls off to school and went to the hospital.  Seeing him intubated for the first time was devastating.  There was blood in his bed from the transfusion.  I couldn't stop crying and Chris was there to comfort both of us.  He has always been positive and encouraging with the diagnosis.  I suppose Noah gets his strength from his daddy.  They say that there is an extremely high divorce rate for parents of children with special needs.  I can see why this is true.  It is very difficult on a spousal relationship.  The stress level is high and there is no time for spending with one another.  I am blessed that he is such a strong and understanding man who honors his commitments to his family.

I have learned so much about myself, my family and my son.  One of the most important things I have learned is to celebrate the small stuff and do not take anything for granted.  Life is difficult, but even more so with a diagnosis like CCHS.  One of my favorite quotes is "Life is like a piano; what you get out of it depends on how you play it".  Take each day as it comes and live life to the fullest.