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.
Monday, January 21, 2013
Trach Myths
1. If your child has a trach they cannot talk--This is not necessarily true. The trach can interfere with speech but there are some things that can help with this. The PMV (speaking valve) allows the air to be breathed in through the trach but back out of the nose. The cap completely closes off the trach and the person is able to breathe the natural way. It took Noah 2 years (yes Years) to get use to the PMV. He hated it. We would put it on and he would scream. One day while at the beach we popped it on to show everyone how he screamed while wearing it and miraculously he did nothing. He tolerated it and even began making noises. I found that the PMV dried him out terribly and we had to be really careful to prevent plugs (this is where the trach becomes plugged and no air gets in or out. It is dangerous for trach patients). Shortly thereafter we tried capping him. The first try was disastorous. He also hated it. But a few months later he didn't even realize we had put it on him. Now he wears it any time he is not hooked up to the vent. It is a miraculous little invention. Who would have thought such a little piece of plastic could be so important and amazing. Noah did learn how to make sounds and even could say a few words without using the PMV or the cap but his voice was very soft and he only said a few words. So, even if one doesn't tolerate the PMV or cap, it is still possible to vocalize around the trach.
2. Your child can never take a bath or shower. They must be spongebathed--We have always given Noah baths. As a baby, we used the baby bathtub. Now he sits in the big tub. Before being capped, he always wore his HME in the tub. Now we use the cap. I keep the water shallow and let him splash to his heart's content. I have him hold his head back and rinse his hair.
3. Your child cannot swallow with a trach--Depending on the diagnosis, this is not necessarily true. A lot of trach babies have problems with aspiration but no trouble swallowing. In Noah's case, he cold swallow but after doing a swallow study, we found that the possibility of his aspirating was high. Because of this, we had to thicken all of his liquids. He had many swallow studies and we did vital stim therapy for about a year (this is where electrodes are placed on his throat and a small current is used to stimulate the throat). Finally, after 2 years he passed his first swallow study and showed no signs of aspiration.
4. Your child cannot swim with a trach--We live in Alabama. The summers are brutal and humid. It is impossible to do anything outside for any length of time without water being involved. We go to the beach every year for vacation and sometimes go several times throughout the year when it's not hot. Swimming is a huge part of our lives. Because of the trach, there is an open hole that leads directly to the lungs. We have to be very careful to keep water out. At first, we used HME's then graduated to the cap. When Noah was a baby, I would put him in his baby float and keep the suction handy. He was content to sit in that and float around. Now that he's older, it's becoming more of a challenge to keep him safe in the water. This past summer we went to an indoor water park for a week. His favorite ride was the lazy river. We were able to sit him in one of their double floats and he was able to enjoy the water without being submerged. They had a huge kid area that was only about 2 inches deep. There was a huge climbing structure that squirted and dumped water. They also had a big regular pool. We just carried him around in this one. He actually did fall face first a few times and scared me to death, but he came up laughing and since he was capped there were no issues. I made sure to keep the suction machine close at all times. We also purchased a small life vest, which provided extra protection. The trach was hidden behind it and I think that even when he fell face first, the trach never got in the water, just his face. I also kept a swim shirt on him and tucked his trach (he has the longer bivona flex) underneath. I remember once seeing a picture of another CCHS child in the pool and he even had his ventilator. They just had the ventilator sitting by the side of the pool and he was in the shallow end, circuits and all. This inspired me and I vowed to allow Noah to have the same experiences as his sisters. Every child is different and doctor's should be consulted before trying any of this. I am in no way advocating that every child with a trach should swim. I'm just stating what has worked for us.
5. You can never travel in the car by yourself if your child has a trach--We have never had nursing so all of Noah's care is my responsibility 24/7. With two older children, we are always on the go. Plus, Noah was constantly required to be at many appointments when he was younger. I really had no other choice than to travel alone with him. It was definitely a pain when he was hooked up to the vent because he always seemed to pull his circuits off. I made sure to keep him attached to his pulse ox and placed the ventilator so I could see the numbers. I also had a mirror that I used when he was backwards facing. Every time he pulled the circuit off, he was awake and the alarms were always false. I just found a good spot to pull off the road and reconnect him. Of course, traveling alone depends on the child's specific health issues. Some children need a lot of suctioning and some children are dependent on the ventilator and cannot breathe if it gets disconnected. In these cases, I would not travel alone. Again, consult your doctor on traveling tips.
6. You can't travel too far from home or your hospital--For us, this was never the case. Our first trip was to the beach when Noah was 7 months old. (3 months after being released from the hospital). Since then, we have been to Disney, the beach every year, the Wilderness at the Smokies Water park, and several other places. I always pack a lot of supplies, several extra of everything. Traveling long distances can be done, it just takes a lot of planning. The hardest part is figuring out how to set the vent up in these different hotel/condos. We make sure to take our long extension cords and our power strip.
7. You must have a full time nurse--We've never had nursing. Our insurance does not cover it. I'm not going to go into a lot of detail here because I've already blogged about this but it is definitely possible to take care of your trached child with no nurses. (See my blog on Nursing).
8. Your child will never have a normal life--Again, I'm not going into a lot of detail here because I've already blogged about this. (See my blog entitled Normal). I must say that Noah is as normal as you or I. He just needs help breathing at night and when he's sick. He goes everywhere we go and has all of the experiences that my girls had at his age.
9. Your child will have to be institutionalized--Unfortunately, this is what we were told when Noah was in the NICU. The doctors were baffled. They ran every test they could think of and everything came back normal. He had EEGs, EKGs, and tons of Xrays. Everything was normal. He had so much blood taken from him that he had to have 2 blood transfusions. There is nothing more horrifying to a parent to come in the NICU and see an IV in your child's head. Everyone around us was going home. Day after day beds were being emptied and there we sat, waiting for answers which would not come. "I don't know" became the doctors standard answer. Finally, after he had a seizure and was put on the ventilator, they were going to transfer him to Children's Hospital. The doctor came over to us, handed us a piece of paper and said that they thought that he had Ondine's Curse. She said in all her 40 years of being a doctor, she had only seen one case. She then went on to say that he would more than likely be institutionalized the rest of his life. Her parting words were for us to read the paper she gave us and she was going to leave us to process. I was in complete shock. I looked at the paper and it was one paragraph on CCHS. It was so complicated and very negative. At this point, I lost hope. I lived in a deep dark fog until he was finally moved to Children's and they were so encouraging. The day we got the diagnosis, all the doctors gathered around (which is never a good sign) and told us they had the test results back and it was CCHS. I immediately broke down because I had been reading things on the internet and after being told by that other doctor, I was thinking that he would not be able to live with us. Through my tears, I heard his new doctors say that everything would be ok. They told me he would live a normal life and be running around like a normal toddler. They said he could do anything he wanted to do (except maybe deep sea dive). I began to feel hope again. They were very honest and said it would be a long process to get him home. I remember saying "home?". They probably thought I was hard of hearing or something because he kept repeating the fact that Noah would come home. In our case, the doctor was wrong. They did not understand the disease and were not very educated. I think they underestimated us. For some, an institution is the only option. Some care is so involved that one person or even a whole family cannot handle doing it alone. In Noah's case, it just did not make any sense.
Living with a trach is certainly different. The main thing is know what to do in an emergency situation. (trach plug or decannulation-when the trach comes out accidentally). It is important to know how to clean the trach site and prevent infections. Other than that, it is not complicated at all. My main goal is for people to see that the trach is no big deal. It's really no different than someone using an inhaler or a shot for diabetes. Don't be scared of it just because it's something you don't see every day.
2. Your child can never take a bath or shower. They must be spongebathed--We have always given Noah baths. As a baby, we used the baby bathtub. Now he sits in the big tub. Before being capped, he always wore his HME in the tub. Now we use the cap. I keep the water shallow and let him splash to his heart's content. I have him hold his head back and rinse his hair.
3. Your child cannot swallow with a trach--Depending on the diagnosis, this is not necessarily true. A lot of trach babies have problems with aspiration but no trouble swallowing. In Noah's case, he cold swallow but after doing a swallow study, we found that the possibility of his aspirating was high. Because of this, we had to thicken all of his liquids. He had many swallow studies and we did vital stim therapy for about a year (this is where electrodes are placed on his throat and a small current is used to stimulate the throat). Finally, after 2 years he passed his first swallow study and showed no signs of aspiration.
4. Your child cannot swim with a trach--We live in Alabama. The summers are brutal and humid. It is impossible to do anything outside for any length of time without water being involved. We go to the beach every year for vacation and sometimes go several times throughout the year when it's not hot. Swimming is a huge part of our lives. Because of the trach, there is an open hole that leads directly to the lungs. We have to be very careful to keep water out. At first, we used HME's then graduated to the cap. When Noah was a baby, I would put him in his baby float and keep the suction handy. He was content to sit in that and float around. Now that he's older, it's becoming more of a challenge to keep him safe in the water. This past summer we went to an indoor water park for a week. His favorite ride was the lazy river. We were able to sit him in one of their double floats and he was able to enjoy the water without being submerged. They had a huge kid area that was only about 2 inches deep. There was a huge climbing structure that squirted and dumped water. They also had a big regular pool. We just carried him around in this one. He actually did fall face first a few times and scared me to death, but he came up laughing and since he was capped there were no issues. I made sure to keep the suction machine close at all times. We also purchased a small life vest, which provided extra protection. The trach was hidden behind it and I think that even when he fell face first, the trach never got in the water, just his face. I also kept a swim shirt on him and tucked his trach (he has the longer bivona flex) underneath. I remember once seeing a picture of another CCHS child in the pool and he even had his ventilator. They just had the ventilator sitting by the side of the pool and he was in the shallow end, circuits and all. This inspired me and I vowed to allow Noah to have the same experiences as his sisters. Every child is different and doctor's should be consulted before trying any of this. I am in no way advocating that every child with a trach should swim. I'm just stating what has worked for us.
5. You can never travel in the car by yourself if your child has a trach--We have never had nursing so all of Noah's care is my responsibility 24/7. With two older children, we are always on the go. Plus, Noah was constantly required to be at many appointments when he was younger. I really had no other choice than to travel alone with him. It was definitely a pain when he was hooked up to the vent because he always seemed to pull his circuits off. I made sure to keep him attached to his pulse ox and placed the ventilator so I could see the numbers. I also had a mirror that I used when he was backwards facing. Every time he pulled the circuit off, he was awake and the alarms were always false. I just found a good spot to pull off the road and reconnect him. Of course, traveling alone depends on the child's specific health issues. Some children need a lot of suctioning and some children are dependent on the ventilator and cannot breathe if it gets disconnected. In these cases, I would not travel alone. Again, consult your doctor on traveling tips.
6. You can't travel too far from home or your hospital--For us, this was never the case. Our first trip was to the beach when Noah was 7 months old. (3 months after being released from the hospital). Since then, we have been to Disney, the beach every year, the Wilderness at the Smokies Water park, and several other places. I always pack a lot of supplies, several extra of everything. Traveling long distances can be done, it just takes a lot of planning. The hardest part is figuring out how to set the vent up in these different hotel/condos. We make sure to take our long extension cords and our power strip.
7. You must have a full time nurse--We've never had nursing. Our insurance does not cover it. I'm not going to go into a lot of detail here because I've already blogged about this but it is definitely possible to take care of your trached child with no nurses. (See my blog on Nursing).
8. Your child will never have a normal life--Again, I'm not going into a lot of detail here because I've already blogged about this. (See my blog entitled Normal). I must say that Noah is as normal as you or I. He just needs help breathing at night and when he's sick. He goes everywhere we go and has all of the experiences that my girls had at his age.
9. Your child will have to be institutionalized--Unfortunately, this is what we were told when Noah was in the NICU. The doctors were baffled. They ran every test they could think of and everything came back normal. He had EEGs, EKGs, and tons of Xrays. Everything was normal. He had so much blood taken from him that he had to have 2 blood transfusions. There is nothing more horrifying to a parent to come in the NICU and see an IV in your child's head. Everyone around us was going home. Day after day beds were being emptied and there we sat, waiting for answers which would not come. "I don't know" became the doctors standard answer. Finally, after he had a seizure and was put on the ventilator, they were going to transfer him to Children's Hospital. The doctor came over to us, handed us a piece of paper and said that they thought that he had Ondine's Curse. She said in all her 40 years of being a doctor, she had only seen one case. She then went on to say that he would more than likely be institutionalized the rest of his life. Her parting words were for us to read the paper she gave us and she was going to leave us to process. I was in complete shock. I looked at the paper and it was one paragraph on CCHS. It was so complicated and very negative. At this point, I lost hope. I lived in a deep dark fog until he was finally moved to Children's and they were so encouraging. The day we got the diagnosis, all the doctors gathered around (which is never a good sign) and told us they had the test results back and it was CCHS. I immediately broke down because I had been reading things on the internet and after being told by that other doctor, I was thinking that he would not be able to live with us. Through my tears, I heard his new doctors say that everything would be ok. They told me he would live a normal life and be running around like a normal toddler. They said he could do anything he wanted to do (except maybe deep sea dive). I began to feel hope again. They were very honest and said it would be a long process to get him home. I remember saying "home?". They probably thought I was hard of hearing or something because he kept repeating the fact that Noah would come home. In our case, the doctor was wrong. They did not understand the disease and were not very educated. I think they underestimated us. For some, an institution is the only option. Some care is so involved that one person or even a whole family cannot handle doing it alone. In Noah's case, it just did not make any sense.
Living with a trach is certainly different. The main thing is know what to do in an emergency situation. (trach plug or decannulation-when the trach comes out accidentally). It is important to know how to clean the trach site and prevent infections. Other than that, it is not complicated at all. My main goal is for people to see that the trach is no big deal. It's really no different than someone using an inhaler or a shot for diabetes. Don't be scared of it just because it's something you don't see every day.
Friday, January 4, 2013
The Box
People like boxes. They help with organization. They help with the storage of materials. They make things neat and tidy. They really do make life easier. That is, unless someone tries to stuff you inside one of them.
People are always trying to place Noah in boxes. Not really literally, just figuratively. Every medical procedure has forms containing many boxes. I am asked to check the boxes that apply. I never check any of them, except the "no" boxes. Then I am asked to explain myself. Does your child have a lung disease--no; then why is he on a ventilator? Does your child use oxygen--sometimes; how much oxygen--depends on his sats. Is your child allergic to any medication--no but please do not give him propofol as a sedative because it could cause heart pauses; Does your child see a cardiologist--no but we do annual holter monitoring. See the pattern? We do not fit into any of their little boxes. This just seems to confuse them. It is very difficult to comprehend that Noah has a trach and is on a ventilator but is perfectly healthy. Yes, we do have to monitor him carefully but he lives his life just as you and I live ours.
All of this can be very frustrating. CCHS is such a complicated disease. Every child is different. Even children with the same mutation number can have different issues. That is what makes this all so challenging. Our kids do not fit into anyone's box. It doesn't matter how many times you try to cram them in there, they just won't fit. We live outside the box.
I am hoping that one day there will be a cure for CCHS. It would be nice to know that there was a simple medication that can make Noah breathe (there is currently a study being done with one of Noah's brave CCHS sisters, Zoe, who is testing this medication). But until then, we continue to live outside of the box and will teach Noah that he is a unique individual who has a great capacity to teach the world about living outside of the box.
People are always trying to place Noah in boxes. Not really literally, just figuratively. Every medical procedure has forms containing many boxes. I am asked to check the boxes that apply. I never check any of them, except the "no" boxes. Then I am asked to explain myself. Does your child have a lung disease--no; then why is he on a ventilator? Does your child use oxygen--sometimes; how much oxygen--depends on his sats. Is your child allergic to any medication--no but please do not give him propofol as a sedative because it could cause heart pauses; Does your child see a cardiologist--no but we do annual holter monitoring. See the pattern? We do not fit into any of their little boxes. This just seems to confuse them. It is very difficult to comprehend that Noah has a trach and is on a ventilator but is perfectly healthy. Yes, we do have to monitor him carefully but he lives his life just as you and I live ours.
All of this can be very frustrating. CCHS is such a complicated disease. Every child is different. Even children with the same mutation number can have different issues. That is what makes this all so challenging. Our kids do not fit into anyone's box. It doesn't matter how many times you try to cram them in there, they just won't fit. We live outside the box.
I am hoping that one day there will be a cure for CCHS. It would be nice to know that there was a simple medication that can make Noah breathe (there is currently a study being done with one of Noah's brave CCHS sisters, Zoe, who is testing this medication). But until then, we continue to live outside of the box and will teach Noah that he is a unique individual who has a great capacity to teach the world about living outside of the box.
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