(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.
Thursday, May 23, 2013
Tuesday, April 30, 2013
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.
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.
Monday, April 22, 2013
More than a Mom
From the moment our babies are born, we go in to "mommy mode". Instincts kick in and we somehow know how to care for these tiny creatures who come into the world with no instruction manuals. We learn how to feed them, dress them, bathe them, hold them, and nurture them. For most women, this is something they do easily from instinct. But what happens when you are required to be more than a mom?
Special needs moms begin their journey like every other mom but then something happens. We are required to become more than a mom. We have no college degrees, yet we are required to know how to work a ventilator. We have no medical training but are required to know how to use the ambu bag on our child and perform life saving CPR. We are not nurses but are required to know how to do trach care and change a trach. We are required, with little or no training to perform the following tasks:
1. work a ventilator, even though we are not respiratory therapists.
2. clean the trach site and change trachs, even though we are not nurses.
3. monitor sats, co2, heart rates
4. order medical supplies
5. become therapists--feeding therapists, speech therapists, occupational therapists
6. become a secretary--we have to make numerous phone calls to the doctors, equipment companies and insurance companies. Our daily life includes juggling many appointments, sometimes several in one day.
7. become a medical researcher--we scour the internet looking for information and then try to understand the medical jargon in the medical journals that we read.
I am sure I probably left a few things out, but you get the general idea. We are required to do so many more things than just being a mom. We have no formal training in these areas and get no pay. All of this is in addition to our "normal" mom duties like cooking, cleaning, laundry, grocery shopping, chauffeuring everyone around to different activities, etc. There is really too much to do in just one day. So, inevitably, something suffers. Our social life becomes almost non existent, our houses are not clean, we do not schedule doctors appointments for ourselves because we are so busy and exhausted. There is no humanly way to do all of this without letting some things go.
We sometimes forget about the normal mommy things. The other night Noah got sick. I immediately went to nurse/doctor/medical mode and began checking his sats, CO2, vitals, monitoring heart rate, checking breaths. I was very concerned because he was shaking, which he had never done before. Immediately I worried that he was having a seizure, which can be common in CCHS kids. Finally it occurred to me to do the "normal" mommy thing and check his temperature. He did, indeed, have a fever, which is not as common with CCHS. Once I gave him medicine, his fever began to recede and he stopped shaking. Most moms would have grabbed the thermometer first. Not me. I grabbed the sat machine, then the CO2 machine and began checking vitals.
Because our children have such complex medical issues, we often overlook the normal childhood illnesses. We expect the worst and have prepared ourselves to do whatever it takes to keep our children alive. We become doctors, nurses and specialists with no formal training. Many times we learn from trial and error. This is our life. Being a mom is not enough for our children. We have to be so much more. The responsibility is overwhelming at times. The choices we make can impact our children's lives forever. We are so much more than a "mom".
Tuesday, April 16, 2013
Guest blog
Several weeks ago I was approached by a man named Cameron Von St. James. He had read my blog and wanted to share his story. His wife has a rare form of cancer called mesothelioma and he wanted to share his experiences as a caregiver. Even though this is not related to CCHS, I still feel that it is important to share. Rare diseases have a huge impact on families. Many of us with a rare diagnosis have the same experiences and feelings. I think that it is important for us to share our stories.
Here is Cameron's story.
Learning to Cope with a Cancer Diagnosis
I know that my wife, Heather, has often wondered how her mesothelioma diagnosis affected me as I cared for her. I hope that this will give her more clarity and provide some level of help for those currently struggling through similar situations.
Our only child, Lily was born about three months before my wife was diagnosed with mesothelioma. While this should have been a joyful time in our lives, our joy was tempered with grief and deep concern as we heard the doctors’ diagnosis of mesothelioma. At that point, I had no idea how we would get through this seemingly impossible dilemma.
Immediately, I was asked to help make healthcare decisions with my wife. Although I felt completely inadequate for this task, I knew that I had to face reality because it was vital for me to be there for my wife and help make these decisions.
Initially, I could only feel angry and scared, and I did not know how to deal with these emotions. Many times, I found myself lashing out with profanities because I did not know how else to respond. However, for my wife’s sake, I gradually overcame these feelings since I knew she needed me to be there for her. I learned to mask many fears so that she would feel cared for and protected.
After Heather’s mesothelioma diagnosis, I found myself incredibly busy caring for her as well as taking care of daily tasks including childcare, work and travel. In the beginning, I had trouble finding the time for all these tasks, but I soon learned a system of dealing with the most important tasks first, and taking the list on one thing at a time. In addition, our family was blessed with many offers of help from family and friends. Without these people, I am not sure how we would have managed throughout these days.
One of the hardest periods was shortly after Heather’s surgery. For about two months following this, both Heather and Lily stayed at her parents’ home in South Dakota. Heather used this period to recuperate from her surgery and to prepare for her next round of mesothelioma treatment. However, during these two months, I only saw my wife and daughter once.
To make this visit, I had to travel 11 hours on a Friday night in a snowstorm. Upon my arrival, I was exceptionally tired, and only got to spend a little time with them. On Sunday afternoon, I had to turn around and repeat the 11 hour drive back home. It was exhausting and short lived, but seeing my family was worth every second of travel.
These two months were quite difficult, but I realize that they were necessary. Making the decision to be apart was the best decision for our circumstances because it allowed me to work while knowing that my wife and daughter were receiving necessary care. This was just one of the hard choices that I found myself making during this time.
Even though these were difficult times, I learned several things. First, I learned to accept help from others. Second, I found that the ability to make difficult decisions is actually a blessing because it gave us a sense of control over this difficult situation. Through all of our struggles, Heather is still here and still healthy over six years later. I hope that our story can be a source of hope and help to those currently battling cancer.
I know that my wife, Heather, has often wondered how her mesothelioma diagnosis affected me as I cared for her. I hope that this will give her more clarity and provide some level of help for those currently struggling through similar situations.
Our only child, Lily was born about three months before my wife was diagnosed with mesothelioma. While this should have been a joyful time in our lives, our joy was tempered with grief and deep concern as we heard the doctors’ diagnosis of mesothelioma. At that point, I had no idea how we would get through this seemingly impossible dilemma.
Immediately, I was asked to help make healthcare decisions with my wife. Although I felt completely inadequate for this task, I knew that I had to face reality because it was vital for me to be there for my wife and help make these decisions.
Initially, I could only feel angry and scared, and I did not know how to deal with these emotions. Many times, I found myself lashing out with profanities because I did not know how else to respond. However, for my wife’s sake, I gradually overcame these feelings since I knew she needed me to be there for her. I learned to mask many fears so that she would feel cared for and protected.
After Heather’s mesothelioma diagnosis, I found myself incredibly busy caring for her as well as taking care of daily tasks including childcare, work and travel. In the beginning, I had trouble finding the time for all these tasks, but I soon learned a system of dealing with the most important tasks first, and taking the list on one thing at a time. In addition, our family was blessed with many offers of help from family and friends. Without these people, I am not sure how we would have managed throughout these days.
One of the hardest periods was shortly after Heather’s surgery. For about two months following this, both Heather and Lily stayed at her parents’ home in South Dakota. Heather used this period to recuperate from her surgery and to prepare for her next round of mesothelioma treatment. However, during these two months, I only saw my wife and daughter once.
To make this visit, I had to travel 11 hours on a Friday night in a snowstorm. Upon my arrival, I was exceptionally tired, and only got to spend a little time with them. On Sunday afternoon, I had to turn around and repeat the 11 hour drive back home. It was exhausting and short lived, but seeing my family was worth every second of travel.
These two months were quite difficult, but I realize that they were necessary. Making the decision to be apart was the best decision for our circumstances because it allowed me to work while knowing that my wife and daughter were receiving necessary care. This was just one of the hard choices that I found myself making during this time.
Even though these were difficult times, I learned several things. First, I learned to accept help from others. Second, I found that the ability to make difficult decisions is actually a blessing because it gave us a sense of control over this difficult situation. Through all of our struggles, Heather is still here and still healthy over six years later. I hope that our story can be a source of hope and help to those currently battling cancer.
Monday, April 1, 2013
The Sweetest Sound
From the moment they enter the world, we eagerly anticipate the first sounds of our baby. We expect them to arrive crying. My girls both entered the world crying. Then came Noah. Complete silence, not even a peep of sound. I remember asking the doctor many times if he was ok and she assured me that he was fine. His Apgar score was good and the nurses assured me he was healthy, but deep down in my mommy gut I knew all was not well. There was a nagging doubt that something was wrong. I was correct and our long 4 month stay in the hospital began.
It took a long time for the CCHS diagnosis but once we discovered it, the doctors moved quickly. He had his trach the day after the diagnosis. I had done some research on trachs and knew that it would impact his life in certain ways but I was not prepared for the reality.
Recovery from trach surgery was relatively easy. We were so happy to get Noah extubated and we finally got to see his sweet little face without all of the tape and tubes. Then he cried. I was shocked. There was no sound whatsoever. My sweet little boy was pitching a fit and there was no sound. His little face was red and tears poured down his face and all was silent. The only sound was the beeping of the ventilator. My heart broke. I realized that having the trach was going to be harder than I thought.
Once I got over the initial shock, it occurred to me that even though he was silent, I would still be able to hear him. If he cried while on the vent, I would hear the vent beep. If he cried while off the vent, I would hear the small breathless sound of air being pushed through the HME. I learned to distinguish his little sounds and realized that I could care for him properly even without him making any noise. In fact, when people would ask me if I knew when he was crying, I got annoyed. Of course I knew when he cried.
As Noah grew, he learned to make noise around the trach. We taught him some sign language but I always made sure he verbalized as well as signed because I was determined that he not rely on sign language alone. When he made that first small sound while wearing his HME, we rejoiced and praised him. We made a huge deal out of each small sound that he made. As he grew, he began to make more sounds and began speaking. That first word was music to our ears. I knew that he was not talking as much as my girls had at that age but I was so proud of how far he had come.
Eventually he started speech therapy and between that and his normal development, he began to talk. He has now completed almost 8 months of twice weekly speech therapy at the local school. His vocabulary has exploded and now that he wears the cap all day, he is loud and constantly being vocal. It took such a long time to get to this point but he has arrived.
Last week we went to the mountains for spring break. We went to a local pottery shop. It was not very crowded so the cashier was taking her time with us. While I was paying for our purchases, she engaged in conversation with Noah. She began asking him questions like what is your name, how old are you, are you having fun on your vacation, what is your stuffed bears name, etc. As usual, I got ready to translate for her but then listened closely. I realized that he was carrying on this conversation perfectly well and he did not need my help. After conversing with him for awhile, the cashier statesz that he speaks so well for a three year old. She said that she had not heard very many three year olds speak so clearly and understandably. She had no idea what that meant to me. We had tried so hard over the years to work with him and help him overcome the speech problems associated with the trach. That sweet lady gave us such a special gift when she said that he was speaking so well. I couldn't have been prouder of him. All of the speech therapies, the trials of getting him to tolerate the PMV and then the cap had finally paid off. Now I realized that everyone could finally understand him and he could communicate his thoughts and needs.
I realized early on to never take any small sound for granted. I cherish every cry, scream, and word. The other day we were shopping and Noah told me "I being loud mommy". Instead of telling him to be quieter I simply said "yes you are" and kept shopping. Every little sound means so much. Noise does not bother me in the least, in fact it does my heart good to hear him being loud. I love nothing more than to hear his little voice raised loud and proud. Never take your children's voice for granted. Every sound and word should be cherished. The words shut up are never used in our house.
Yes, trachs can impede speech development but there are tools that can be used to overcome this. It may take awhile, years in fact, but eventually the words will come and the trach voice can be heard.
The saying Silence is Golden does not apply in our house. Enjoy every little sound and word. Embrace the noise. Your children's voice is the sweetest sound you will ever hear. Never take it for granted and always listen to them. There is no sweeter sound than the sound of Noah saying "I love you Mommy". Our home is filled with the laughter, screams, words, and sounds of Beth, Hannah and Noah. I would not have it any other way.
It took a long time for the CCHS diagnosis but once we discovered it, the doctors moved quickly. He had his trach the day after the diagnosis. I had done some research on trachs and knew that it would impact his life in certain ways but I was not prepared for the reality.
Recovery from trach surgery was relatively easy. We were so happy to get Noah extubated and we finally got to see his sweet little face without all of the tape and tubes. Then he cried. I was shocked. There was no sound whatsoever. My sweet little boy was pitching a fit and there was no sound. His little face was red and tears poured down his face and all was silent. The only sound was the beeping of the ventilator. My heart broke. I realized that having the trach was going to be harder than I thought.
Once I got over the initial shock, it occurred to me that even though he was silent, I would still be able to hear him. If he cried while on the vent, I would hear the vent beep. If he cried while off the vent, I would hear the small breathless sound of air being pushed through the HME. I learned to distinguish his little sounds and realized that I could care for him properly even without him making any noise. In fact, when people would ask me if I knew when he was crying, I got annoyed. Of course I knew when he cried.
As Noah grew, he learned to make noise around the trach. We taught him some sign language but I always made sure he verbalized as well as signed because I was determined that he not rely on sign language alone. When he made that first small sound while wearing his HME, we rejoiced and praised him. We made a huge deal out of each small sound that he made. As he grew, he began to make more sounds and began speaking. That first word was music to our ears. I knew that he was not talking as much as my girls had at that age but I was so proud of how far he had come.
Eventually he started speech therapy and between that and his normal development, he began to talk. He has now completed almost 8 months of twice weekly speech therapy at the local school. His vocabulary has exploded and now that he wears the cap all day, he is loud and constantly being vocal. It took such a long time to get to this point but he has arrived.
Last week we went to the mountains for spring break. We went to a local pottery shop. It was not very crowded so the cashier was taking her time with us. While I was paying for our purchases, she engaged in conversation with Noah. She began asking him questions like what is your name, how old are you, are you having fun on your vacation, what is your stuffed bears name, etc. As usual, I got ready to translate for her but then listened closely. I realized that he was carrying on this conversation perfectly well and he did not need my help. After conversing with him for awhile, the cashier statesz that he speaks so well for a three year old. She said that she had not heard very many three year olds speak so clearly and understandably. She had no idea what that meant to me. We had tried so hard over the years to work with him and help him overcome the speech problems associated with the trach. That sweet lady gave us such a special gift when she said that he was speaking so well. I couldn't have been prouder of him. All of the speech therapies, the trials of getting him to tolerate the PMV and then the cap had finally paid off. Now I realized that everyone could finally understand him and he could communicate his thoughts and needs.
I realized early on to never take any small sound for granted. I cherish every cry, scream, and word. The other day we were shopping and Noah told me "I being loud mommy". Instead of telling him to be quieter I simply said "yes you are" and kept shopping. Every little sound means so much. Noise does not bother me in the least, in fact it does my heart good to hear him being loud. I love nothing more than to hear his little voice raised loud and proud. Never take your children's voice for granted. Every sound and word should be cherished. The words shut up are never used in our house.
Yes, trachs can impede speech development but there are tools that can be used to overcome this. It may take awhile, years in fact, but eventually the words will come and the trach voice can be heard.
The saying Silence is Golden does not apply in our house. Enjoy every little sound and word. Embrace the noise. Your children's voice is the sweetest sound you will ever hear. Never take it for granted and always listen to them. There is no sweeter sound than the sound of Noah saying "I love you Mommy". Our home is filled with the laughter, screams, words, and sounds of Beth, Hannah and Noah. I would not have it any other way.
Wednesday, March 13, 2013
The Fear
Fear. Such a simple word but one that is very powerful. All of us have felt fear at one time or another. It's a basic human emotion that one feels from time to time.
CCHS parents feel fear every day. We can hide it and not express it but it is there. We have a special kind of fear, the kind that most parents of children with special needs feel. There are the fears of our child's general well being--are they eating enough, is that little cough going to grow into a big sickness, are they going to be bullied in school. These are just simple little fears that even the parents of "normal" children feel.
Then we have the fears that occur with a rare disease--are they ventilated adequately, will our power stay on during storms, are we seeing enough specialists, should we send our kids to school during the flu season or keep them home to try to prevent them from getting sick, do we have enough supplies for the month and what do we do when things are backordered. These are small fears that we handle on a day to day basis.
Then we come to the greatest fear of all. The fear that our child will not wake up. This is a deep, paralyzing fear that we live with every single day. Most days we can bury it and go about our day to day activities. Then something will happen and the greatest fear will return with a vengeance.
Yesterday was one of those days. Our greatest fear was realized. The CCHS community found out that a little five year old girl named Lucy had passed away. She had a trach, like many of our CCHS kids and her day started normally. Then, as her mom stated, she "crashed like a rock". We are all devastated. Many of us (me included) did not know her or her family. This doesn't really matter. She is one of us. Her parents are in the fight with us, and we feel the loss also. A picture was posted showing her beautiful, smiling face and brought us to our knees. We saw a beautiful, vibrant life cut short and in her face we saw our own child.
This is our deepest fear. We try so hard to keep our children healthy. We fight the medical community, insurance, equipment companies, and schools to make sure our children get the best possible care. We hook them up every night to electronic equipment, checking and double checking everything to make sure it works properly. We sleep lightly (if we sleep at all) waking for each little beep and exhalation of air. We do everything that is humanly possible to keep our children alive. Deep down, we know that sometimes this may not be enough. CCHS is so unpredictable. One day your child is fine and feeling great, the next they are on the vent 24/7. This is our reality.
We cannot let the fear define us. We have to stay strong and provide our special children with a life like every other child. Most days this is not so difficult but every now and then it becomes almost unbearable.
Please remember Lucy and her family in your thoughts and prayers. Never take a moment for granted. Live your life to the fullest so that you have no regrets. Enjoy every moment.
Rest in peace precious Lucy. Your CCHS community mourns you deeply.
CCHS parents feel fear every day. We can hide it and not express it but it is there. We have a special kind of fear, the kind that most parents of children with special needs feel. There are the fears of our child's general well being--are they eating enough, is that little cough going to grow into a big sickness, are they going to be bullied in school. These are just simple little fears that even the parents of "normal" children feel.
Then we have the fears that occur with a rare disease--are they ventilated adequately, will our power stay on during storms, are we seeing enough specialists, should we send our kids to school during the flu season or keep them home to try to prevent them from getting sick, do we have enough supplies for the month and what do we do when things are backordered. These are small fears that we handle on a day to day basis.
Then we come to the greatest fear of all. The fear that our child will not wake up. This is a deep, paralyzing fear that we live with every single day. Most days we can bury it and go about our day to day activities. Then something will happen and the greatest fear will return with a vengeance.
Yesterday was one of those days. Our greatest fear was realized. The CCHS community found out that a little five year old girl named Lucy had passed away. She had a trach, like many of our CCHS kids and her day started normally. Then, as her mom stated, she "crashed like a rock". We are all devastated. Many of us (me included) did not know her or her family. This doesn't really matter. She is one of us. Her parents are in the fight with us, and we feel the loss also. A picture was posted showing her beautiful, smiling face and brought us to our knees. We saw a beautiful, vibrant life cut short and in her face we saw our own child.
This is our deepest fear. We try so hard to keep our children healthy. We fight the medical community, insurance, equipment companies, and schools to make sure our children get the best possible care. We hook them up every night to electronic equipment, checking and double checking everything to make sure it works properly. We sleep lightly (if we sleep at all) waking for each little beep and exhalation of air. We do everything that is humanly possible to keep our children alive. Deep down, we know that sometimes this may not be enough. CCHS is so unpredictable. One day your child is fine and feeling great, the next they are on the vent 24/7. This is our reality.
We cannot let the fear define us. We have to stay strong and provide our special children with a life like every other child. Most days this is not so difficult but every now and then it becomes almost unbearable.
Please remember Lucy and her family in your thoughts and prayers. Never take a moment for granted. Live your life to the fullest so that you have no regrets. Enjoy every moment.
Rest in peace precious Lucy. Your CCHS community mourns you deeply.
Thursday, February 28, 2013
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)
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.
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