Sunday, October 7, 2012

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. 


  1. Melinda, Thank you so much for educating us on this disease. You and your family are so strong and your love for one another is evident.
    Love y'all,

  2. Thanks Kathy. Love you all too.

  3. So glad to see blogs again! Y'all are the best family that Noah could have. :)

  4. Thank you Sarah. We couldn't do this without your support.

  5. I have late onset CCHS which has only very recently been discovered. It explains many things in my past life including why I always stop breathing when I am concentrating hard on something. At a very early point in my teens I realized that I did not breath automatically. I do not recall if I ever mentioned it to anybody as it didn't seem all that important. Besides, I could swim the length of an olympic sized pool underwater.

    I now know what it is and how strange it is for me to have survived somehow at that time. I write this to tell you that your child is not sentenced to an early death or even any significant inconvenience as long as the "rules" are followed. I am now 64 and have two children and four grandchildren. I use oxygen to keep me breathing correctly at night. It works because of the Haldane Effect. More oxygen in the blood slows down the transport of CO2 from the brain. The increased CO2 activates the chemoreceptors in the brain and amplifies the urge to breath. For me it works perfectly. You may wish to ask your doctors about this. I should mention it wasn't my doctors that worked this out, it was me. I am a long term student of science including especially medicine.

    I can be reached at roboto at ixian dot ca

  6. I forgot to mention that I may be the only person alive with CCHS at my age.

  7. It's very nice to meet you Mr. Ng. You give me hope that Noah can live a long and happy life. Would you happen to know your mutation number? Do you have any other issues, especially cardiac issues? There is a wonderful website (The CCHS Network) that includes all kinds of helpful information. I am sure there are many CCHS parents out there that would love to benefit from your experiences (including myself). Thank you for sharing.