Noah's Story

Early morning, August 26, 2009 I was awakened and knew it was time to go

to the hospital.  We made arrangements to get the girls off to school and

we packed and headed off to St. Vincents Hospital to meet our son, Noah.

He was originally due September 22 but I knew he was at a decent weight because I had been to the doctor the previous day.  I was a little worried, but not overly concerned.

Noah arrived in the world the morning of August 26, 2009. He did not make one

sound whatsoever when he was delivered, which worried me.  I continually

asked the doctor if he was ok and she assured me that he was fine.  There

was a small nagging doubt that kept telling me something was wrong.  After awhile, Chris left to check the girls out of school and I settled in to hold

Noah and rest.  The nurse brought him to me fresh from his bath.  As I

was holding him, I noticed a small blue tinge around his lips.  The

nurse came to check on us and I showed her.  She immediately took him

and checked his vitals, reporting that his oxygen was a little low.  She

gave him some o2 and worked with him for awhile.  After about 15

minutes, she took him to the nurse’s station so she could keep an eye on

him.  It wasn't long before she returned (without Noah) and informed me

that he had to go to the NICU because his oxygen kept dropping and there

was no way for him to be monitored in my room.  She asked if the girls

were close to coming and stated that if they were 5 minutes out they

would wait for them, otherwise he was going on to the NICU.  In a panic,

I called Chris to find out that they were in the parking deck.  So the

nurses wheeled Noah in the room in an incubator.  I held him while the

girls talked to him, touched him and kissed him.  They only let them see

him for 5 minutes then he was wheeled to the NICU.  It all happened so

fast, we had no time to process it.  The girls kept asking where he went

and why couldn't they see him.  I was extremely upset and it was so hard

to keep composed in front of them. 

There were no first family pictures, no pictures of Noah with his grandparents, nothing but tears and fear. This was the year of the swine flu and the NICU did not allow anyone except parents to visit.  Because of this, nobody except Chris and me got to see Noah until he was 4 months old.

We were first told that he would be able to go home with me.  But the day came and they said, he would stay another week so I packed up and walked out of the hospital without my baby.  It was the hardest thing I have ever had to do.   We visited daily.  It was extremely difficult having a child in the NICU and 2 children at home.  After the girls left for school, I would drive

to the hospital and stay with Noah until it was time to leave to meet

the girls at the bus stop.  We would do the regular after school things

of homework and dinner.  When the girls had gone to bed, Chris would go

to the hospital to see Noah.  On the weekends, we would get a sitter and

stay with him a few hours on Saturday and Sunday. 

Noah had many different tests (MRIs, Blood Tests, Genetic Tests, etc).  Everything came back normal.  He would desat down to the 80s and then before the nurses could even come check on him, he was back up to the upper 90s.  This was constant.  He would do this all day and all night and they couldn't figure out why.  He was eating fairly well. He was fine other than the many desats.

Then, a week after he was born he hit rock bottom.  Chris had

gone to see him that evening and he called me when he got to the

hospital.  His voice was shaking and he said they had closed the NICU.

I asked if it was Noah and he said he didn't know because they weren't

letting anyone back there.  I hung up the phone with him promising to

call me soon.  I immediately knew it was Noah.  I just had this horrible

feeling and a deep blackness overcame me.  I began crying hysterically,

trying to keep quiet so as not to frighten the girls.  Not too much

later, Chris called me and I have never heard his voice sound so sad and

scared.  He informed me that Noah had a seizure and they had to intubate

him.  We were terrified.  They said his CO2 was too high and they were

giving him phenobarbital to prevent more seizures (in retrospect this

was not good because it was depressing his breathing even more but at

the time we didn't know his diagnosis).  The next day, they asked

permission to give him a blood transfusion.  They had taken so much

blood from him for tests that he needed more.  Seeing him laying there

with a tube down his throat, tape all over his face, iv in his head,

wires all over and blood on the bed was the most terrifying thing I had

ever seen.  The depression hit full force and I feared for his life.

This was our life for another week.  The doctors would come talk to us

in hushed tones.  The nurses would give us sympathetic looks and pat our

backs.  We watched many babies come and go from the NICU but there we

sat with no end in site.  I began to dread going to the hospital but I

felt so guilty when I wasn't there.  It was killing me to see him like

that.  He was never awake and we did not get to hold him.  They stopped

feeding him by mouth and gave him an IV.  The lowest day was when the

doctor told us they could not care for him anymore.  She informed us

that he was being transferred to our local children's hospital, which in

retrospect was the best thing that could have happened.  With deep pity

in her eyes, she informed us that they suspected that Noah had Ondine's

Curse or Congenital Central Hypoventilation Syndrome, a very rare disease.  She said that he would be on a ventilator the rest of his life and would most

likely have to be institutionalized.  She said that she had only seen

this extremely rare disease in a child one other time.  She handed us a

short 5 paragraph medical paper and told us she would let us "process the information" and then she walked off.  We were devastated.  How is a parent suppose to process that information?  They gave us no hope, no information and it seemed as if they had just washed their hands of him. 

Shortly thereafter, Noah was transported by ambulance to children's hospital.  I did not get to go because I was at the doctor with Hannah, who had the flu.  Chris made sure he got settled in and took some pictures for me.  Unfortunately, I got the flu also and could not visit Noah for 2 weeks.  It was truly the worst nightmare. 

Once I finally got to visit, the whole atmosphere was different.  The nurses were encouraging.  Noah was awake and the nurse asked if we wanted to hold him.  In shock, we asked if we really could and were told that they encouraged it.  I finally got to hold him after 2 weeks.  The pulmonologist did a sleep study, which Noah failed miserably. 

I remember the day we finally got a diagnosis.  We were visiting Noah together (Chris was on his lunch break) and all of the doctors and nurses gathered around Noah's bed.  I immediately became nervous.  The pulmonologist informed us that the test had came back positive for Congenital Central Hypoventilation Syndrome (CCHS).  I immediately broke down.  The doctor kept talking but for a minute there all I heard was a roaring in my ears.  The fear was overwhelming.  I had visions of having to send him out of state to some institution and never being able to see or care for him myself.  I slowly began listening to the doctor.  He told us that the diagnosis was scary but that Noah would be up in no time running around like every other child.  He told us that he would be able to go to school, play ball and do everything else (except maybe scuba dive).  We were told that we would have to have extensive training before bringing him home and it was still going to be awhile before he was released from the hospital, months not days.  I was horrified and elated at the same time.  We went from being told that he

would live in an institution to being told that he would not only be going home but that he would live life like any other child.  It was an extraordinary amount of information to process.  We went home and got on the computer, which was a mistake.  There wasn't a lot of information out there and we really felt so alone and scared. 

That evening, we got a call telling us that they wanted to do trach surgery the next day.  We consented and went to the hospital after getting the girls off to school.  The surgery  did not last long and we were able to see him soon after.  That first glimpse of him was amazing.  For the first time since he had been born, we saw his little face.  It was a little red from the tape, but he was sleeping so peacefully.  The trach looked scary but he looked so

much more comfortable.  The thing that scared us was that his breath

rate on the vent was up to 55 bpm and he was not taking any breaths on

his own.  The ventilator was taking every breath for him.  They informed

us this was because of the sedation. 

The next day, he was awake and alert.  His breath rate was down.  After about a week, they told us that he would be moved to a floor as soon as they had a bed for him.  We were so excited.  This was one more step closer to coming home and the girls could finally come visit him.  The day finally arrived and he went to the pulmonary floor.  What an amazing day.  The girls got to hold and snuggle with him and we finally got some pictures of them together.  He

was 2 1/2 months old.  We still could not stay there with him because the room he was in had no bed for us but at least we could let the girls visit whenever we wanted and stay as long as we wanted. 

The nurses slowly began showing us how to suction and we began asking a lot of questions.  The home ventilator team met with us and explained exactly what would happen. We decided to allow him to have a g-tube so that we would get to take him home quicker.  He was eating, but not enough to sustain him.  They felt that this was because he had been IV fed for so long. 

After the g-tube and trach site had healed some, we would begin our training and train for 3 days a week for a few hours.  Our training included doing

everything 3 times each (bathing, cleaning the trach, changing the

trach, suctioning, changing the circuits, doing a bolus feed, etc).  We

decided that I would train while Chris worked, since he was our only

source of income.  My mother and mother in law agreed to be trained as

well. 

We began our training on my birthday, which was the best present

ever.  For the next month, we learned as much as we could.  We had an

excellent home vent nurse who made sure were were thoroughly trained.

We learned in one month what it took professionals years to learn.  We

took lots of notes and knew that we were not eligible for nursing care

at home so we paid close attention.    We learned quickly and they told

us they would try their best to have us home before Christmas. 

December 15, 2009 was Noah's homecoming.  It was a day we never thought we would see.  The homecoming was a little crazy because Hannah had a stomach

virus and the DME had to meet us ASAP because Beth had her first band

concert.  My mother in law stayed with Noah while we went to the

concert.  The first night home, I didn't sleep at all.  Our dining room

looked like a mini ICU.  We had him hooked to the vent, pulse ox, apnea

monitor, and feeding pump.  He looked like a little science experiment

laying there but we didn't care.  He was finally home. 

Slowly, we began to get in to a routine.  I was able to wean him off the vent.  I realized that I could do this.  Then, he got sick.  Never had he had

oxygen in the hospital but now he needed oxygen.  I was so extremely

stressed for the week he was sick.  He was ventilated 24/7 and I was

constantly suctioning and dealing with alarms.  Christmas day, he slept

a lot.  We really didn't care because we were all together.  Eventually,

he got better and our routine returned to normal.  

3 months after coming home, we took our first trip to the beach.  It was the most amazing trip we have ever had.  He loved it and we were elated to

realize that we could do this and live a relatively normal life.  The

darkness was over.

Noah is now a happy, healthy 3 year old.  He is off the vent during the

day and we trach his cap.  His speech has improved greatly after 3 years of speech therapy and he has proven to be so smart with a great sense of humor.  He is our miracle and our blessing.  We have learned so much from him.  I never tire of hearing him run around the house aggravating his sisters and being a real boy. I never thought we would get this far.  We do not know what the future holds.  Hopefully we will be able to have him decannulated and just use a bipap.  We may even do the diaphragm pacer surgery.  Attending school is our next big hurdle.

Life is never boring with our little miracle man.  We are always doing research on CCHS, constantly having struggles with the equipment company, insurance, and doctors.  Slowly, we have learned how to live with a special needs child.  It has been rough at times but worth every minute.  

Before the CCHS diagnosis

Our first pictures together

After trach surgery

Before trach surgery

Noah age 3

At the beach

At the park

Pet Peeves of a CCHS Mom

 (According to Webster's Dictionary a pet peeve is a "frequent subject of complaint").  Sorry for the negativity, but here are a few of the aggravating things that happen in the special needs community.


1.  Refusal of services--When Noah was an infant he received the RSV shot.  This shot helps prevent the spread of the breathing virus RSV.  Imagine a ventilated, trached child getting RSV.  Not pretty.  When he turned one, I called about getting the shot again.  We were denied.  They said he was too old.  Regardless of the doctors orders, or the fact that he has a trach and ventilator plus was one month premature, we were still denied.  I still can't figure out how that was logical.  Another refusal of a service was for nursing in the home.  Our insurance did not cover this even though Noah was coming home with a trach and a vent. 

2.  Back ordered supplies--It is rare that we receive all of our supplies every month at the same time.  Something is always back ordered.  Usually, its the trachs but this month it was water bags and bacteria filters.  I can't understand how the DME does not have extras of this stuff.  Our supplies do not change.

3.  Number of supplies we receive--We are allowed 2 trachs a month yet we change the trach once a week.  That means we are suppose to sterilize the trach as best we can and reuse.  I do not like doing this but we really have no choice.  Everything we receive says "for single use only".  Noah gets one (yes one) trach cap a month.  He wears them every day all day (except for sleeping).  How can it be single use?  I realize that we are in better shape than a lot of SN moms.  I have heard that someone only got one trach every 6 months!  Another mom only gets 15 suction catheters a month.  These are also suppose to be single use.  It really doesn't make much sense.  Noah wears his pulse ox probe every time he sleeps.  We are allowed 2 a month.  These small probes secure to the finger or toe using a sticky cloth tape.  The tape is useless after two uses so we have to use medical tape to adhere the probe to the skin.  We use these until they are literally falling apart. 

4.  Appointments--As a SN mom, we have a lot of appointments.  For some reason, they want to make them all at 8 in the morning.  I have two other children who do not leave until 7:15.  I have a ventilator, suction machine, and go bag to pack up and load in the car.  Not to mention doing all of this after being up 4 times for ventilator alarms. They always have vent clinic at 1 or 2 in the afternoon because we have to have clinic in the sleep lab since the home vent team doesn't have their own area in the hospital.  This is better than 8 am, however it is right in the middle of nap.

5.  Missed appointments--Probably my biggest pet peeve is the missed appointments.  We have 3 people coming to the house every month (previously we had 6).  At first, they would call at the last minute and say that they HAD to come to the house in order to get the paper work done.  The DME even came one time unannounced.  Luckily, I had just pulled in to the driveway.  They quickly learned that this was not going to work with us.  Unlike many of their other patients, we do get out and go places and some weeks we are rarely home.  I have to make appointments to see people so why shouldn't they?  I can't tell you how many times I have been sitting at home waiting for someone to show and it doesn't happen.  I get all kinds of excuses, mostly that they got tied up doing paperwork. Sometimes they call to cancel (usually an hour or two after they were suppose to be here) but mostly they do not.  I have learned to not change my schedule.  If they are not here in their allotted time and I have to leave, then I do.  Recently, I was sitting in the auditorium watching my daughter's award ceremony at school when I got a call from my mother in law.  I sent it to voicemail.  She then texts me and says the caseworker is on her way to my house.  When they couldn't reach me on the phone, they called her.  I had spoken with them earlier and told them it would have to be after 3:00 when they made their visit.  I looked at the time and it was 1:30.  I'm not sure why they think they can just show up whenever they want.  It's so frustrating. 

6.  The phrase:  "He looks so normal"--Maybe that's because he IS NORMAL!!  He's just like everyone else, only he has a trach and a vent.  


7. The phrase:  "I'm so sorry" when someone learns that he has a trach and a vent.   I'm not sorry.  He's alive and well.  To me, that's a blessing.


8.  Know it all doctors--There has only been one time that I have had a doctor say that they are the experts.  Noah's team of specialists have always treated me with respect and we work as a team. To these doctors I say " I don't care how long you went to school or how much education you have, you do not have to deal with CCHS every day (in fact, you can probably count on one hand the number of CCHS patients you have seen).  I am sure you have not spent nearly the amount of time that I have spent researching and talking to other parents of CCHS children. You may be the expert in your field, but you are NOT an expert in CCHS. "  The refusal of these doctors to listen to us parents is ridiculous.  We live this every day and know our child better than anyone. 

9.  Caseworkers--This goes along with number 5 above.  The very first caseworker that we had came out to the house, looked at Noah and informed me that he might be cold with the ceiling fan blowing.  I knew then that I was in for a long, frustrating time with these people. 
Our 3rd caseworker was nice but had no clue.  She missed several appointments.  The first time I met her she asked me if Noah could go outside!!  I was seriously speechless for a moment.  Did she think we kept him in the house, never to leave?  Another time, she was getting the paperwork together for him to be reviewed for another year of services.  She asked all kinds of questions, which was fine but then made the comment that the nurses reviewing the information would know all about CCHS.  I informed her that the doctors didn't even know about CCHS and I was absolutely certain that the medicaid nurses would have no clue since it is so rare.  She seemed surprised at my answer, then proceeded to call me 5 times in one day because those nurses had questions. We are now on our 4th caseworker in 3 years.  

10.  Lack of information/research-- Having a rare disease means there is not a lot of research out there.  Doctors want case studies and journal articles to back up our reasons for doing things but there are very few or even none out there.  It makes it even more difficult because there are different mutation levels and different modes of ventilation that have to be researched. 

So Your Child Has a Trach--Now What? (our experiences with a trach and some trach tips)

The thought of your child having a trach is terrifying.  When we were first told Noah was going to have a trach, we were horrified and scared.  The trach looked like some crazy torture device. We had so many questions about trachs and trach care. Once he had the surgery and we saw his little face for the first time not covered with tape, we knew we had made the best decision for him.  He was so much more comfortable.  Upon learning that your child will have one, many thoughts run through your head.  Will my child be able to talk, eat, swim, bathe, yell, etc.  You will also wonder how on earth you raise a child with a trach.  The answer is that you will raise your child just like any other child without a trach.

I have found over the years that trach care varies from person to person, state to state and country to country.  So, for this blog entry I will be speaking from our experience and from our care as we were taught at our local Children's Hospital of Alabama.   Fortunately, we had an excellent home vent nurse who made sure we were thoroughly trained in trachs and trach care.  Depending on where you live, your trach care routine may be different. So I have decided to share our experiences with trachs and try to give some tips for new trach moms.  This is in no way expected to take the place of your doctor's/nurse's orders that they give you for your own child.

Trach Surgery--Trach surgery is a relatively simple procedure that does not take long (about 30 minutes).  Noah received his trach when he was a little over a month old.  His recovery was remarkably quick and about a week after getting his trach, we began our trach training.  

Trach brands--There are several different kinds of trachs.  Noah started out with a neonatal Shiley.  He had some issues with his HME popping off and having redness around the trach site.  So, we switched to a Bivona flex, which we have been using ever since then.  We love it!  The flex hangs down some so it is easier to clean his neck.  He has no issues with redness (except for when he is sick) and the HME/cap stays on without popping off.  He also seems to like the fact that it is not bunched up under his neck like the Shiley was previously.

Trach Ties--There are also many different kinds of trach ties.  We have tried many brands but our preferred brand is the Marpac.  We cut to fit and it comes in two pieces that velcro together. For daily cleaning, we use gauze, a soap free cleanser (Cetaphyl or Physoderm), water and Nystatin powder.  First we use the soap free cleanser and wipe around his neck and under the flanges.  Then we wipe with a wet gauze to get the soap off.  We then wipe with a dry gauze and apply the nystatin powder all around the trach site and under the flanges, being careful not to get the powder in the stoma.  We then loop the new ties through the trach holes and fasten.  Its a very simple procedure that takes less than 5 minutes. The ties are changed daily. 

Suctioning the trach--Now that he is wearing a cap on the trach, we do not have to suction as much.  When we do suction, I use sterile gloves and a new sterile catheter every time.  We use it once and throw away.  The size of the suction catheter depends on the size of the trach.  We use an 8 French and suction to the number 10 line. 

Changing the trach--When we change the trach, we make sure to clean the area like normal.  I suction him.  Then I use a lubricating jelly that I place on the trach.  We then take the old trach out and insert the new.   He coughs a little when we insert the new trach. The trach is changed once a week. 

Sterilizing trachs--We only receive 2 trachs a month so I have to sterilize and reuse, which I am not fond of but insurance will only allow 2.  So to sterilize, I wash the trach and oberator with soap and water.  I then use a pyrex measuring cup and boil water in the microwave.  After removing the measuring cup from the microwave, I drop the trachs and oberators in the boiled water.  I cover it with a paper towel and let the water cool.  Once cool, I remove the trachs and place on a towel to dry.  I cover the trachs while they are drying.  After being thoroughly dried, I place each trach in its own sealed lunch baggie.  So far, he has had no infections and I have been using this method for 3 years now.

Special clothing for trach kids--Noah does not wear any special clothing because of his trach.  I do not usually put him in turtlenecks because he's not fond of them but if I do, I make sure the trach is not covered by the neck.  We even figured out a way for him to wear a tie.  I just make sure I can easily get to the ties and that the trach itself is not covered. 

Covering the trach--We always make sure to keep the trach either capped or use an HME.  

Trach Devices-- We always keep the trach covered.  At first, we used the HME's.  This device does the same thing that a nose does.  It heats, moisturizes and filters the air.  When wearing this, Noah needs suctioning several times.  The PMV (passey muir valve) is used for speaking.  Air enters the PMV but cannot escape, making the wearer breathe out of their nose.  Noah hated his PMV.  It took him 2 years to tolerate it!  We did several bronchs to make sure there was no physical reason why he wouldn't tolerate the PMV.  When he finally began to wear it, he was 2 years old.  The only problem I had with the PMV was that it dried him out very badly.  I had to remove it for awhile each day because he was in danger of getting a plugged trach.  Once he tolerated the PMV, we switched to a cap.  We love the cap!  It looks like a larger version of a toothpaste cap and works wonderfully well.  The cap completely closes the trach off and he breathes normally through his nose.  Once he began wearing his cap, his speech improved greatly. 

Eating with a trach--Noah eats normally with his trach.  He did have some aspiration issues, causing us to have to thicken his liquids with rice cereal for several years.  I would highly recommend getting swallow studies done periodically to check for aspiration. When he was a baby, I used a cloth bib to cover the trach while he ate. Now, I just make sure he has his cap on the end and he eats without a bib. 

Water play with a trach--We are very careful with him around water.  Noah bathes with no special equipment.  I just make sure he is capped or wearing his HME.  He does swim also.  I keep his head above water and make sure the suction machine is close by in case I have to suction him quickly.  We go to the beach every year and I always make sure to take the suction machine.

Trachs on the go--We always have an emergency go bag with us at all times when leaving the house.  The go bag contains extra trachs (his size and one size below), trach ties, gauze, scissors, saline bullets, trach lubricant, sterile gloves, an ambu bag, stethescope,  saline bullets and hand sanitizer.  I also have a few extra suction catheters in there.  Currenly we use a large backpack to hold everything but I am looking for a somewhat smaller backpack that's easier to carry.

Travelling with the trach-- Noah has 3 suitcases when we travel-one for clothes, one for ventilator supplies and one for trach supplies.  There are a few ways to keep track of the supplies.  One is that you can just go ahead and create little trach bags.  Use a baggie and put all of the stuff needed for one day of trach care in there.  If you are gone for more than one day, have a bag for each day.  Then, all you have to do when doing trach care is grab the bag.  Another thing you could do is to create a master list of all the supplies needed and keep it in the suitcase.  Check off each item as you place it in the suitcase.  During volleyball season, we went out of town several times.  I had extra supplies so I just packed those and left the suitcase packed all season, making sure to replenish the supplies I used during the trip.  It saved me a lot of work.  Once the season was over, I unpacked the suitcase.  I always take more supplies than we need, just to be safe. 



These are just a few tips and ideas that work for us.  Having a trach is just a little more work but really no big deal.