LIFE IS GOOD! Life is oh so good, even when it isn't exactly what you expected. Twists and turns in your life can make things hard or challenging. We have learned that hard doesn't have to be mean things are bad. It just means that we have been given the opportunity to grow and learn from an experience. We will strive to make this a good one for our family! Life is beautiful!
Friday, November 7, 2014
The muscle biopsy is back!
We have wonderful news! The muscle biopsy is back and it was negative for the many muscle diseases tested for! We are so happy! We ruled out a lot of terrible disease and are grateful he doesn't have them. The muscle biopsy did show "non specific myopathic changes." This means an "unknown muscle disease." The doctor called and we chatted for awhile. Her and the other panel of doctors that reviewed his biopsy don't agree. They said it was minimal and insignificant. I hope they are right. She said we wait and watch. She also said a possible future biopsy may be needed Years down the road, When his muscle has developed more. She said he needs to meet back up with genetics now. I hope they are right. I understand they see these all the time but it worries me that we are ignoring something that could us get an answer for him. I do know he is getting the care he needs today and that is what matters. And we are thrilled to have ruled out the terrible things they have been worried about with him! He is doing well and happy, so all is well. Next step, cardiology. Love this little man of ours!
Tuesday, November 4, 2014
The last year in a nutshell
WOW, what a year it has been! I cannot believe a year ago tomorrow we admitted our sweet little angel into the hospital, our lives changed a lot that day. 5 hospitalizations, 7 times under anesthesia, countless appointments, trips to SLC, ng tube, gtube, oxygen, suctioning, vest treatments, Physical Therapy, hearing appointments, ABR, Sleep studies, Feeding therapy, Swallow studies and a very resistant little kid are some words that come to mind. We know more than we have ever wanted to know about our bodies but what we need to know to take care of this sweet little man and we are still learning. Here is a very long, version of the past year if you want to read. :0)
I remember a year ago feeling like something was so very wrong with our little boy and no one would listen. I remember hearing everyone tell me that kids all grow and develop at their own pace and that Easton would grow out of everything. It was so nice to hear but my mother heart didn't agree. I felt like I needed to fight for my little boy and get him the help he needed. When he was born he was very floppy. His head especially. He had really low muscle tone and a croupy cough. No one was worried, except us and a few family members. Doctors said he was fine. At two months Dr G said she thought he had a small air way and unless he got sick it wasn't a big deal. She sent home with a monitor and we watched him. He was all over the place with oxygen and heart rate. When he got sick a few days later they admitted him and watch him. His oxygen would drop to the high 70's, but because he would recover on his own they said not to worry and sent us home. Dr G referred us to his pulmanologist dr P who we met with when he was 4 months old. She took one look at him and told me he had Dysphasia. She gave us the rundown of him aspirating. She sent us to primaries the next day for a swallow study with his speech therapist M R and confirmed her diagnosis. We started on thickened formula, oxygen and over the next few days he got worse. We had met with Dr M the day of his swallow study and he ordered a bronchoscopy to look at his air way and clean out his lungs. All went well and they found out he had trechobrocheomalacia. He was concerned with the amount of fluid in there and decided to admit him. Over the course of the following 4 days we ran a lot of tests and blood work including an MRI. All went well and still no answers, which was good news! Many prayers were answered. After doing an X-ray they found more fluid in his lungs and they decided to place an ng tube. They told us to get in touch with our early intervention program called up to 3, and to continue with oxygen and sent us on our way. The next while was exhausting, but so rewarding. We started to see Easton learn to hold us his head up more, he started coughing less and became the happiest little guy. (He had been really colicky.) It was a challenge, but worth every effort to see him doing so much better!
I remember a year ago feeling like something was so very wrong with our little boy and no one would listen. I remember hearing everyone tell me that kids all grow and develop at their own pace and that Easton would grow out of everything. It was so nice to hear but my mother heart didn't agree. I felt like I needed to fight for my little boy and get him the help he needed. When he was born he was very floppy. His head especially. He had really low muscle tone and a croupy cough. No one was worried, except us and a few family members. Doctors said he was fine. At two months Dr G said she thought he had a small air way and unless he got sick it wasn't a big deal. She sent home with a monitor and we watched him. He was all over the place with oxygen and heart rate. When he got sick a few days later they admitted him and watch him. His oxygen would drop to the high 70's, but because he would recover on his own they said not to worry and sent us home. Dr G referred us to his pulmanologist dr P who we met with when he was 4 months old. She took one look at him and told me he had Dysphasia. She gave us the rundown of him aspirating. She sent us to primaries the next day for a swallow study with his speech therapist M R and confirmed her diagnosis. We started on thickened formula, oxygen and over the next few days he got worse. We had met with Dr M the day of his swallow study and he ordered a bronchoscopy to look at his air way and clean out his lungs. All went well and they found out he had trechobrocheomalacia. He was concerned with the amount of fluid in there and decided to admit him. Over the course of the following 4 days we ran a lot of tests and blood work including an MRI. All went well and still no answers, which was good news! Many prayers were answered. After doing an X-ray they found more fluid in his lungs and they decided to place an ng tube. They told us to get in touch with our early intervention program called up to 3, and to continue with oxygen and sent us on our way. The next while was exhausting, but so rewarding. We started to see Easton learn to hold us his head up more, he started coughing less and became the happiest little guy. (He had been really colicky.) It was a challenge, but worth every effort to see him doing so much better!
We came home and got connected with early intervention and they did an ABR. That test showed severe to profound hearing loss in his left ear. It is called auditory Neurpathy and is a problem with the nerves firing synchronously. We started to see dr P his audiologist on a regular basis and she has been a huge part of his development and care. We continue to see her on average of 2 times a month. In the following weeks we started in home therapy with K his PT, J his OT, and soon to follow was M for his hearing. They all have played an important role of where Easton is today. He is now crawling a saying quite a few words. He wears a hearing piece called a Baha on a headband. It transfers the sound from the bad side to the good ear, through bone conduction. We notice a huge difference when he has it on all the time!
We continued to meet with Easton's primary Dr, Dr G and she didn't seamed worried about Easton. I felt we were missing some things. B R, Easton's care coordinator listened and got him an apt with cardiology in December. The cardiologist said the structure of his heart looked perfect but that he had pulmonary hypertension. In short, the effort he makes in breathing puts stress on his heart. They told us to continue oxygen while he was asleep and upped it to half a liter. The following days were long as we followed up with neurology, ENT for a FEES study, met with genetics, a sleep study, and met with Dr P. The sleep study showed severe secondary obstructive sleep apnea. Again, treatment is oxygen. So I sat frustrated and defeated at what to do for this sweet little fighter we just kept getting more frustrating news for. They placed tubes in his ears while were were down there and we headed back to logan for my grandmas funeral.
The following days we tried to feed Easton by mouth again, he started to get sick and fussy. We took him off his food over new years and he got better and became his happy little self. So why can't he eat by mouth if his swallow studies say he can with certain thicknesses? Well, we didn't quite know but knew we couldn't feed him, we had to listen to our gut instinct as parents. At the end of January we decided to try again and we had the same result. The beginning of February we met with a GI doc, Dr O. She and Dr M were confused why he wasn't growing out of this. They thought he would be improving and didn't understand why he wasn't. They said they felt it was time to place a gtube and possibly a nissians. Dr M said, the bigger he gets the more He worried this might be "nerve" related. Those words struck me to the core because his hearing problem was nerve related as well. I went home to research anything I could find. I found out a lot about the Vegas nerve and went to his ped with the information. She said there was no way because his diaphragm was normal. Ok, but everything else fit him to a T and it sounded just like him. I wanted to find a nerve doc! I then scheduled his follow up neurology apt with a neurologist who specialized in nerves and waited it out.
The following week Easton was admitted for a second bronch and several tests to determine if he needed a nissians. When he came out of his bronch he was chocking like never before and something wasn't right. None of the nurses would listen and I assured them this wasn't normal. After the bronch they had placed a dual ph probe that is similar to his ng tube. After about 3 hours they tried to feed him and his ng tube wouldn't work. There was resistance, so she decided to pull it out. He finally stopped throwing up/gagging like crazy and went to sleep. I still don't know what happened but the poor guy was misriable and I was happy they fixed it. Easton did great during the study and his other tests which made it so he didn't have to have a nissians! Yay! We were so happy.
Easton got his gtube at the end of February, the following week we took off to get a break from all the appointments, etc. We enjoyed a nice break at Disneyland as a family and spent the remaining part of the week in Vegas to watch some Aggie basketball. It was nice to be together and to let our lives just be normal for a minute.
About a month later Easton got sick, really sick. He was miserable and requiring a lot of suctioning and more oxygen. I finally took him in and asked them to check for pneumonia. They did a chest X-ray, and sent us home with antibiotics. After a day or two he was a different baby and feeling much better. This illness was scary and pushed for Easton to get a vest. It is a treatment that vibrates and breaks up the secretions in his lungs. After starting this Easton seamed to be improving.
At the end of April we got in to see Dr Sakonju, his new neurologist. She agreed that this could all be nerve related and ordered his EMG. We did that at the end of May and it showed that Easton has a Bulbar Neurpathy. This effects four of his major nerves and is the reason for what he is going though. They tested him for a syndrome that is treatable and it came back negative. At that point his doctors said it was time to meet together to plan his future care.
In June Easton had another swallow study and only aspirated on thin liquids. I was perplexed. Why are all of his swallow studies so different. Dr Meier gave us the go ahead to try and feed him again. we continued to give him tastes of things to keep him from gaining an aversion to food but didn't quite jump the gun yet. Soon after we met with dr Tristani his cardiologist and he got another Echo cardiogram. It showed that he had right ventricular thickening which means his heart continued to show stress. It is working too hard. He said that Easton needed to be on 24 hour oxygen so that he wasn't working so hard to keep it up, which in turn would hopefully help his heart. He told us he can have small breaks here and there but to keep it on as much as possible.
Easton started to do well and improve a lot. He was happy and seamed to be less junky and hardly ever coughing. I thought he must be growing out of his reflux and weaned him off one med. He did great so when we ran out of the other I took him off that too. He was doing great and was happy. For his 1st birthday we went to Hogle Zoo, he loved it. We had a big Mickey Mouse party and celebrated him and his life. He has been through so much and fought so hard. It was a day to celebrate for sure.
In the middle of July he went to bed with a raspy voice and woke up at 1 AM struggling to breathe. We were told if he every sounded like he had croup to take him in right away. I ended up taking him in to Logan Regional, they gave him a treatment, he improved and they decided to keep him for observation. He did great and in the end this was most likely due to him being taken off his reflux meds but we weren't sure. We were told to wait it out and see if it happened again. WHAT? Isn't it better for him to just start meds again? No... so we waited.
A few weeks later we did a care conference with Neurology, Genetics, Pulmonary, Audiology and his speech Therapist was there for ENT. They were able to talk about his EMG and the results and what the future can hold for him. But, no one had any answers. Just more, lets wait and watch. They talked about how his dyphasia was the primary problem. Dr Sakonju explained that yes he can have good moments with swallowing and bad ones, which explains his inconsistent swallow studies. It was remeasuring for me, because I never know what to do with his eating and it is so frustrating. It was so nice to get them all on the same page though! That afternoon we saw Dr Meier Eastons ENT and he scoped him. His throat looked terrible, and was full of secretions. I fed Easton while we were there and a few minutes later he coughed (a sign of aspiration) Dr Meier then prescribed the reflux meds, sent us on our way and told us to keep trying to feed him. WHAT? I get so frustrated. We want this kid to eat. MORE THAT ANYONE ELSE! It would be so much easier to sit him up to the table with the rest of our family, cut up his food and let him feed himself. It would be so nice to not have meal times be such a sad time for this sweet guy! BUT... every time we try HE GETS SICK. We have had several people not understand this. It is so hard as parents. Clear back when he was eating, they determined he was having micro aspirations. That means some/most of his food goes down. But, he still aspirates. How are we supposed to know at any given moment if he will aspirate or not. We were frustrated and stumped. (More on this later) We started him back up on his reflux meds and he was doing great.
In September we went back down for another routine sleep study. Easton was a trooper as always and performed like a champ. Dr Pfeffer was happy to tell me that his obstructive sleep apnea has drastically improved. This was great news! This says that he is aspirating less, and his airway isn't clasping as much. The frustrating news is that his Central Sleep Apnea was worse. This is the neurological side of things. Dr Pfeffer had no explanation, just lets wait and see what his next one looks like. (Oh how I hate "Lets wait and watch!") As parents, we are just praying it isn't a sign that this is degenerative. We met with ENT again while we were down there since Easton was showing hearing loss in his good ear and his Audiologist believes it is due to fluid behind his ear. He was starting an ear infection, one tube was plugged, one tube was coming out and he said it is time for a new ones. He said he would like to do a bronch at the same time.
A week later Easton saw his Neurologist and she was so happy to see his progress! He had just learned to crawl and was rolling all over the place. She said that was encouraging and said, "Ok, now we wait and watch." :( I was hoping to get her to test his nerve again but she said it was too early. I asked what the next step is. She had previously mentioned after we redid the previous test (We did and it was normal) that we would do a muscle biopsy. I asked about it and she didn't know. She sat perplexed as what to do. She left the room to talk to the Neuromuscular Doctors in her office what they thought. She came back and said they said "Absolutely" do the muscle biopsy. So, we got that scheduled with the tubes and bronch.
That morning Easton had woke up sounding junky, over the next week he got worse. We kept thinking he was getting sick. Nothing, just a lot of extra secretions. It was getting very hard for him to be around food and we started giving him suckers more often to keep him happy while we ate. He continued to sound worse all the time and on October 13th I took him in to see Dr Garg. I explained that I thought he was aspirating and I was hoping he wasn't getting pneumonia. He was chocking a lot in the mornings and sounded like he had deep secretions settling. She listened and he sounded great. (HE ALWAYS DOES!) She looked at his ears, said he had a blaring ear infection and that we needed to put him on an antibiotic for that. If there was anything in his lungs it would get better if it was bacterial and sent us on our way. I was very frustrated by this because his lungs always sound clear. ALWAYS! I had expressed concern about him being put out the following week and asked if he needed an xray. NO, He sounds great. Of course, he always does. So, I left and didn't worry about it. He started the antibiotic and it seamed to help in both areas, but didn't take away his junky sounds and cough.
Over the course of the next week Easton was fine. Just junky and coughing here and there. Kenadee got croup on Monday and we watched him close, he was fine so we went in for his procedure as scheduled. Before the procedure I talked with Dr Pfeffer and explained my concerns. She said she didn't like the sound of his junky cough but thought it was fine to proceed. I felt like this cough was due to a ton of secretions and aspirating on his own saliva. He hadn't had a day where he was visibly "SICK" just his normal signs of aspirating. They told me it would take an hour for all three of them. They normally come out after their individual procedures and no one was coming. After 50 minutes Dr Pfeffer came out. Her first words were, "He is so sick Amber!" She sat down and talked with me for the following 20 minutes. "He is such a fighter." "He scared us several times in there." "We thought he was going to go into cardiac arrest." "We are missing a piece to his puzzle and he needs us to figure it out, and he needs us to figure it out now." "I am worried about his heart, his heart should not have done that in there." "He needs a heart cath done right away." "He had a profuse amount of secretions in there, it was so dark I couldn't see a thing." "His lungs are permanently damaged." "I touched the side of his lung and it bruised immediately, that is not ok. His lungs are not ok." Are among the words she spoke to me. Now, to back up for a minute, the night before this procedure Larry gave Easton a blessing. Jaxson was very upset after and cried begging me not to take Easton because he felt like he was going to die. We reassured Jaxson that Easton would be okay and that when we have fear we need replace that with faith in our Father in Heaven. As I sat and listened to Dr Pfeffer Jaxsons words hit me. I was scared and I didn't have my baby in my arms yet. He was still in the OR getting the other stuff done. I prayed and continued to talk with her and understand what she was trying to tell me. Basically. when they started to intubate him he started using every muscle to breathe. His whole upper body was contracting with every breath. They bagged him to force air in, it didn't work. He started having brochospasums, the air wouldn't go in. Then his heart plummeted. It was 80-90 and dropped to 50. They tried Albuteral to open his airway and it didn't work. They tried a second steroid and it didn't work either. She said this happened several times and they gave him a second dose of that steroid and they were able to get him settled. After he was stable they proceeded. She couldn't believe the amount of secretions in there. I asked her if we shouldn't have had this done that day. She said "Now that I know he is ok, I can say That was really good for us to see. I think I can say that now, because this kid needs help." He always looks and sounds so good and yet he struggles so much inside. Easton came out and struggled with his airway for little bit, he recovered and did great after that. They kept him for 24 hours to monitor him since we live so far away from primaries. He did great and we headed home.
This experience has taught me a lot. Most important, I have a mothers intuition for a reason. I have tried to tell them a million times, I know he sounds good, but he always does. This kid knows how to move air, even around all the crap in his lungs. WHY? HOW? Who knows, but I will say I think it has something to do with the fact that HE HAS TO. He has to, or it will kill him. I have been frustrated all along with the words "He looks so good." It isn't that I don't want him to... it is that there is something in me want to shout to the whole world and all his doctors that this poor kid puts up a fight, EVERY DAY OF HIS LIFE. Everyone is right, he does look good. I am happy about that, but For some reason I don't like hearing it because I am afraid if I believe it, he wont get the help he needs and we will loose our baby. So. why don't I want to listen to Dr Meier and feed my kid? Is it because I don't want him to eat? NO! It is because as a mother, something stops me. Something tells me he will aspirate. I used to think it was fear that was stopping me. I used to think it was my fault. I don't anymore. I believe it is a loving Heavenly Father inspiring me and my husband to do what is best for Easton. The two doctors that witnessed this needed to see that he needs them. He needs their help. We need to do what is best for this little guy so he can not only live, but thrive. Does it matter if he eats by a tube for his entire life? No, it doesn't. Is it hard? YES, for him and us. Is it heart breaking to watch him scream when you take away something that he finds when it tastes so good to him. Yes, it is. I hope we can get his lungs strong enough and that someday his neuropathy will not stop him from eating. But, for now we have to back off and just let him taste things here and there. And, no more suckers. :( I wish this wasn't the case, but it is what is best for him.
This past week and a half I have personally gone through so many emotions. It has been a week of refection on this past year. The ups and the downs. I watch this sweet boy in amazement for where he is at today and all that he has overcome to learn what he has learned. He is a strong kid! It has been a week of watching his every move he makes and pleading with my Father in Heaven to please not take him from us. I know he is not in a hospital bed fighting for his life, but he works so hard to survive. I look into his eyes and see the sweetest spirit. We need him in our lives and we would do anything to keep him here with our family. I am so scared. We will be getting the results of his biopsy soon, we are hoping for great news. But, I am scared, because this test could give us an answer. Do I want the answer? I have always said, "The test doesn't cause the problem, it just tells us it is there." I believe that, I want to know how to help him. But I really don't want to know if he has a syndrome that will take him from us at a young age. I pray that this comes back negative and we can rule out a lot of things that are life threatening. I pray that if it is positive that we will have the strength and rely on our Father in Heaven. I hope that we can get him the care HE needs either way and continue to be inspired as his parents to do what is best for him. Is it an honor to be his mom. I feel so blessed to have him in my life.
I have spent a lot of time this past week just staring at my kids. Playing with them more. Talking with them more. Appreciating each of them a little more. My heart has been so full. I am so blessed. I have watched Larry a little closer as he interacts with them. I feel so blessed to have this little family of mine. I feel so blessed to have Larry by my side. He has been such a strength to me throughout this past year. When no one else will listen and no one else understands, I always know he does. I couldn't have made it through all of this without his help. While I have been in Salt Lake with Easton to appointments, he holds down the fort at home. Not only does he do it, but he does it without complaining and does a great job while he is at it.
I have to thank our Father in Heaven for the blessings He has poured out upon our family. I feel like He is aware of each one of us and has helped Easton in more ways than we know. I am thankful for the power of the priesthood and am thankful to have a husband who lives his life so he is worthy to exercise it. I also have to give a shout out to our family and friends as well. A huge thanks to Jen Daniels, Sharli King and Nicky Crawford for taking our children for COUNTLESS hours! So many others have taken our kids, helped with meals, ran kids around for us, and just listened. Thank you! Most of all I am so thankful for the prayers that have been offered on our behalf and on the behalf of Easton. I sent out a message to family and a few friends during Eastons procedure and I think it would be fair to say within an hour his name was in several temples and many prayers wee offered. Our prayers were answered. We feel them. Thank you! We feel so blessed to be surrounded by such good, good people.
Life is hard, but life is good, oh so good!
About a month later Easton got sick, really sick. He was miserable and requiring a lot of suctioning and more oxygen. I finally took him in and asked them to check for pneumonia. They did a chest X-ray, and sent us home with antibiotics. After a day or two he was a different baby and feeling much better. This illness was scary and pushed for Easton to get a vest. It is a treatment that vibrates and breaks up the secretions in his lungs. After starting this Easton seamed to be improving.
At the end of April we got in to see Dr Sakonju, his new neurologist. She agreed that this could all be nerve related and ordered his EMG. We did that at the end of May and it showed that Easton has a Bulbar Neurpathy. This effects four of his major nerves and is the reason for what he is going though. They tested him for a syndrome that is treatable and it came back negative. At that point his doctors said it was time to meet together to plan his future care.
In June Easton had another swallow study and only aspirated on thin liquids. I was perplexed. Why are all of his swallow studies so different. Dr Meier gave us the go ahead to try and feed him again. we continued to give him tastes of things to keep him from gaining an aversion to food but didn't quite jump the gun yet. Soon after we met with dr Tristani his cardiologist and he got another Echo cardiogram. It showed that he had right ventricular thickening which means his heart continued to show stress. It is working too hard. He said that Easton needed to be on 24 hour oxygen so that he wasn't working so hard to keep it up, which in turn would hopefully help his heart. He told us he can have small breaks here and there but to keep it on as much as possible.
Easton started to do well and improve a lot. He was happy and seamed to be less junky and hardly ever coughing. I thought he must be growing out of his reflux and weaned him off one med. He did great so when we ran out of the other I took him off that too. He was doing great and was happy. For his 1st birthday we went to Hogle Zoo, he loved it. We had a big Mickey Mouse party and celebrated him and his life. He has been through so much and fought so hard. It was a day to celebrate for sure.
In the middle of July he went to bed with a raspy voice and woke up at 1 AM struggling to breathe. We were told if he every sounded like he had croup to take him in right away. I ended up taking him in to Logan Regional, they gave him a treatment, he improved and they decided to keep him for observation. He did great and in the end this was most likely due to him being taken off his reflux meds but we weren't sure. We were told to wait it out and see if it happened again. WHAT? Isn't it better for him to just start meds again? No... so we waited.
A few weeks later we did a care conference with Neurology, Genetics, Pulmonary, Audiology and his speech Therapist was there for ENT. They were able to talk about his EMG and the results and what the future can hold for him. But, no one had any answers. Just more, lets wait and watch. They talked about how his dyphasia was the primary problem. Dr Sakonju explained that yes he can have good moments with swallowing and bad ones, which explains his inconsistent swallow studies. It was remeasuring for me, because I never know what to do with his eating and it is so frustrating. It was so nice to get them all on the same page though! That afternoon we saw Dr Meier Eastons ENT and he scoped him. His throat looked terrible, and was full of secretions. I fed Easton while we were there and a few minutes later he coughed (a sign of aspiration) Dr Meier then prescribed the reflux meds, sent us on our way and told us to keep trying to feed him. WHAT? I get so frustrated. We want this kid to eat. MORE THAT ANYONE ELSE! It would be so much easier to sit him up to the table with the rest of our family, cut up his food and let him feed himself. It would be so nice to not have meal times be such a sad time for this sweet guy! BUT... every time we try HE GETS SICK. We have had several people not understand this. It is so hard as parents. Clear back when he was eating, they determined he was having micro aspirations. That means some/most of his food goes down. But, he still aspirates. How are we supposed to know at any given moment if he will aspirate or not. We were frustrated and stumped. (More on this later) We started him back up on his reflux meds and he was doing great.
In September we went back down for another routine sleep study. Easton was a trooper as always and performed like a champ. Dr Pfeffer was happy to tell me that his obstructive sleep apnea has drastically improved. This was great news! This says that he is aspirating less, and his airway isn't clasping as much. The frustrating news is that his Central Sleep Apnea was worse. This is the neurological side of things. Dr Pfeffer had no explanation, just lets wait and see what his next one looks like. (Oh how I hate "Lets wait and watch!") As parents, we are just praying it isn't a sign that this is degenerative. We met with ENT again while we were down there since Easton was showing hearing loss in his good ear and his Audiologist believes it is due to fluid behind his ear. He was starting an ear infection, one tube was plugged, one tube was coming out and he said it is time for a new ones. He said he would like to do a bronch at the same time.
A week later Easton saw his Neurologist and she was so happy to see his progress! He had just learned to crawl and was rolling all over the place. She said that was encouraging and said, "Ok, now we wait and watch." :( I was hoping to get her to test his nerve again but she said it was too early. I asked what the next step is. She had previously mentioned after we redid the previous test (We did and it was normal) that we would do a muscle biopsy. I asked about it and she didn't know. She sat perplexed as what to do. She left the room to talk to the Neuromuscular Doctors in her office what they thought. She came back and said they said "Absolutely" do the muscle biopsy. So, we got that scheduled with the tubes and bronch.
That morning Easton had woke up sounding junky, over the next week he got worse. We kept thinking he was getting sick. Nothing, just a lot of extra secretions. It was getting very hard for him to be around food and we started giving him suckers more often to keep him happy while we ate. He continued to sound worse all the time and on October 13th I took him in to see Dr Garg. I explained that I thought he was aspirating and I was hoping he wasn't getting pneumonia. He was chocking a lot in the mornings and sounded like he had deep secretions settling. She listened and he sounded great. (HE ALWAYS DOES!) She looked at his ears, said he had a blaring ear infection and that we needed to put him on an antibiotic for that. If there was anything in his lungs it would get better if it was bacterial and sent us on our way. I was very frustrated by this because his lungs always sound clear. ALWAYS! I had expressed concern about him being put out the following week and asked if he needed an xray. NO, He sounds great. Of course, he always does. So, I left and didn't worry about it. He started the antibiotic and it seamed to help in both areas, but didn't take away his junky sounds and cough.
Over the course of the next week Easton was fine. Just junky and coughing here and there. Kenadee got croup on Monday and we watched him close, he was fine so we went in for his procedure as scheduled. Before the procedure I talked with Dr Pfeffer and explained my concerns. She said she didn't like the sound of his junky cough but thought it was fine to proceed. I felt like this cough was due to a ton of secretions and aspirating on his own saliva. He hadn't had a day where he was visibly "SICK" just his normal signs of aspirating. They told me it would take an hour for all three of them. They normally come out after their individual procedures and no one was coming. After 50 minutes Dr Pfeffer came out. Her first words were, "He is so sick Amber!" She sat down and talked with me for the following 20 minutes. "He is such a fighter." "He scared us several times in there." "We thought he was going to go into cardiac arrest." "We are missing a piece to his puzzle and he needs us to figure it out, and he needs us to figure it out now." "I am worried about his heart, his heart should not have done that in there." "He needs a heart cath done right away." "He had a profuse amount of secretions in there, it was so dark I couldn't see a thing." "His lungs are permanently damaged." "I touched the side of his lung and it bruised immediately, that is not ok. His lungs are not ok." Are among the words she spoke to me. Now, to back up for a minute, the night before this procedure Larry gave Easton a blessing. Jaxson was very upset after and cried begging me not to take Easton because he felt like he was going to die. We reassured Jaxson that Easton would be okay and that when we have fear we need replace that with faith in our Father in Heaven. As I sat and listened to Dr Pfeffer Jaxsons words hit me. I was scared and I didn't have my baby in my arms yet. He was still in the OR getting the other stuff done. I prayed and continued to talk with her and understand what she was trying to tell me. Basically. when they started to intubate him he started using every muscle to breathe. His whole upper body was contracting with every breath. They bagged him to force air in, it didn't work. He started having brochospasums, the air wouldn't go in. Then his heart plummeted. It was 80-90 and dropped to 50. They tried Albuteral to open his airway and it didn't work. They tried a second steroid and it didn't work either. She said this happened several times and they gave him a second dose of that steroid and they were able to get him settled. After he was stable they proceeded. She couldn't believe the amount of secretions in there. I asked her if we shouldn't have had this done that day. She said "Now that I know he is ok, I can say That was really good for us to see. I think I can say that now, because this kid needs help." He always looks and sounds so good and yet he struggles so much inside. Easton came out and struggled with his airway for little bit, he recovered and did great after that. They kept him for 24 hours to monitor him since we live so far away from primaries. He did great and we headed home.
This experience has taught me a lot. Most important, I have a mothers intuition for a reason. I have tried to tell them a million times, I know he sounds good, but he always does. This kid knows how to move air, even around all the crap in his lungs. WHY? HOW? Who knows, but I will say I think it has something to do with the fact that HE HAS TO. He has to, or it will kill him. I have been frustrated all along with the words "He looks so good." It isn't that I don't want him to... it is that there is something in me want to shout to the whole world and all his doctors that this poor kid puts up a fight, EVERY DAY OF HIS LIFE. Everyone is right, he does look good. I am happy about that, but For some reason I don't like hearing it because I am afraid if I believe it, he wont get the help he needs and we will loose our baby. So. why don't I want to listen to Dr Meier and feed my kid? Is it because I don't want him to eat? NO! It is because as a mother, something stops me. Something tells me he will aspirate. I used to think it was fear that was stopping me. I used to think it was my fault. I don't anymore. I believe it is a loving Heavenly Father inspiring me and my husband to do what is best for Easton. The two doctors that witnessed this needed to see that he needs them. He needs their help. We need to do what is best for this little guy so he can not only live, but thrive. Does it matter if he eats by a tube for his entire life? No, it doesn't. Is it hard? YES, for him and us. Is it heart breaking to watch him scream when you take away something that he finds when it tastes so good to him. Yes, it is. I hope we can get his lungs strong enough and that someday his neuropathy will not stop him from eating. But, for now we have to back off and just let him taste things here and there. And, no more suckers. :( I wish this wasn't the case, but it is what is best for him.
This past week and a half I have personally gone through so many emotions. It has been a week of refection on this past year. The ups and the downs. I watch this sweet boy in amazement for where he is at today and all that he has overcome to learn what he has learned. He is a strong kid! It has been a week of watching his every move he makes and pleading with my Father in Heaven to please not take him from us. I know he is not in a hospital bed fighting for his life, but he works so hard to survive. I look into his eyes and see the sweetest spirit. We need him in our lives and we would do anything to keep him here with our family. I am so scared. We will be getting the results of his biopsy soon, we are hoping for great news. But, I am scared, because this test could give us an answer. Do I want the answer? I have always said, "The test doesn't cause the problem, it just tells us it is there." I believe that, I want to know how to help him. But I really don't want to know if he has a syndrome that will take him from us at a young age. I pray that this comes back negative and we can rule out a lot of things that are life threatening. I pray that if it is positive that we will have the strength and rely on our Father in Heaven. I hope that we can get him the care HE needs either way and continue to be inspired as his parents to do what is best for him. Is it an honor to be his mom. I feel so blessed to have him in my life.
I have spent a lot of time this past week just staring at my kids. Playing with them more. Talking with them more. Appreciating each of them a little more. My heart has been so full. I am so blessed. I have watched Larry a little closer as he interacts with them. I feel so blessed to have this little family of mine. I feel so blessed to have Larry by my side. He has been such a strength to me throughout this past year. When no one else will listen and no one else understands, I always know he does. I couldn't have made it through all of this without his help. While I have been in Salt Lake with Easton to appointments, he holds down the fort at home. Not only does he do it, but he does it without complaining and does a great job while he is at it.
I have to thank our Father in Heaven for the blessings He has poured out upon our family. I feel like He is aware of each one of us and has helped Easton in more ways than we know. I am thankful for the power of the priesthood and am thankful to have a husband who lives his life so he is worthy to exercise it. I also have to give a shout out to our family and friends as well. A huge thanks to Jen Daniels, Sharli King and Nicky Crawford for taking our children for COUNTLESS hours! So many others have taken our kids, helped with meals, ran kids around for us, and just listened. Thank you! Most of all I am so thankful for the prayers that have been offered on our behalf and on the behalf of Easton. I sent out a message to family and a few friends during Eastons procedure and I think it would be fair to say within an hour his name was in several temples and many prayers wee offered. Our prayers were answered. We feel them. Thank you! We feel so blessed to be surrounded by such good, good people.
Life is hard, but life is good, oh so good!
Tuesday, May 27, 2014
Update
It has been awhile since I have posted. I am on the fence about even keeping this blog, but on days like today it is a nice place to update. Today Easton had a test called an EMG. It is a test where they put tiny needles in him, shock him and watch how his nerves respond. This is the test that I wrote about after our appointment to the neurologist. They did find that he has what is called a bulbar neuropathy. It is where the nerves do not fire synchronously. If they don't fire correctly then your brain doesn't get the message that is being sent. This is the same problem he has in his ear...the auditory neuropathy. This explains the dysphasia and other problems. The 1st thing we will test for now is the syndrome she talked about a month ago. Brown vialetto van larea syndrome. They have done blood work and we should get that back in a week, if it is positive we will do some more testing on his genes to confirm what they find. If it is negative they will do a muscle biopsy and test to see if it is something else. The other few things they will be looking for are worse because there is no way to treat it. All of these things are progressive, but if it is the BVVLS then we will hopefully be able to stabilize him now. They have something that is called riboflavin, it is crazy high amounts of vitamin b2, it helps because the amino acids make it so his body can not retain them. This could help him to be able to have a better life. He will start on this tomorrow.
I cannot put into words what this experience has been like. It is very frustrating to watch your sweet little boy suffer and even just want to eat a bite of what you are eating. It is so hard to see them poke and prod him left and right. It is so hard to be the one who has to do that too. Last month he got sick...really sick. He required a lot more help, specifically a lot of suctioning, the stick it all the way down his throat kind. It was nothing short of terrible. It is hard, but it keeps him here so it is worth it. It is hard to feel like you are on this journey all alone. It is so hard to feel like no one understands. Some comments are hard to hear, yet it is hard when you feel like no one cares. It is hard when you feel like people stay away because they are scared. This journey has just been hard. BUT...we wouldn't change being Easton's parents for the world. This journey has also been so rewarding. We have had people step out of the cracks, love our little boy and have a genuine interest in his well being. It is so sweet to see the little ones who flock to Easton, our little neighbor kids love him! Those moments, whether it is a stranger, a friend or family are the moments where it doesn't feel so lonely. The days when Easton melts hearts left and right, brighten our whole family. Watching our children interact with him is so special. They love and adore him and he does them. Those moments help us to know we are going through the journey that our Heavenly Father has laid out perfectly for our family and our sweet boy. Those moments make the hard one easier.
This past weekend Larry's sister Lisa stayed with us. She and Larry were making dinner when they noticed Easton playing happily and talking to someone. They both noticed a special countenance about him, as soon as he realized Mariah was near by he started to look at her and it all changed. We feel the veil is thin, we have a different spirit in our home. We are so so blessed and even though there are times where life is challenging, we are constantly learning, growing and enjoying this sweet little man and the spirit he has about him. Our life is oh so good.
I cannot put into words what this experience has been like. It is very frustrating to watch your sweet little boy suffer and even just want to eat a bite of what you are eating. It is so hard to see them poke and prod him left and right. It is so hard to be the one who has to do that too. Last month he got sick...really sick. He required a lot more help, specifically a lot of suctioning, the stick it all the way down his throat kind. It was nothing short of terrible. It is hard, but it keeps him here so it is worth it. It is hard to feel like you are on this journey all alone. It is so hard to feel like no one understands. Some comments are hard to hear, yet it is hard when you feel like no one cares. It is hard when you feel like people stay away because they are scared. This journey has just been hard. BUT...we wouldn't change being Easton's parents for the world. This journey has also been so rewarding. We have had people step out of the cracks, love our little boy and have a genuine interest in his well being. It is so sweet to see the little ones who flock to Easton, our little neighbor kids love him! Those moments, whether it is a stranger, a friend or family are the moments where it doesn't feel so lonely. The days when Easton melts hearts left and right, brighten our whole family. Watching our children interact with him is so special. They love and adore him and he does them. Those moments help us to know we are going through the journey that our Heavenly Father has laid out perfectly for our family and our sweet boy. Those moments make the hard one easier.
This past weekend Larry's sister Lisa stayed with us. She and Larry were making dinner when they noticed Easton playing happily and talking to someone. They both noticed a special countenance about him, as soon as he realized Mariah was near by he started to look at her and it all changed. We feel the veil is thin, we have a different spirit in our home. We are so so blessed and even though there are times where life is challenging, we are constantly learning, growing and enjoying this sweet little man and the spirit he has about him. Our life is oh so good.
Monday, April 21, 2014
Feeling very humbled. Easter took on a whole new meaning for me this year. I really enjoyed our lesson in YW and really thought a lot about our sweet little boy. What a blessing it will be for all of us to be perfected someday. I am very grateful to know that Easton too will be made whole and that all of his physical struggles will someday be gone. I hope they will be gone sooner than later, but to know that someday he will not have to struggle at all physically bring such a peace to our hearts. I love to know that someday he will be able to hear just like the rest of us. I am grateful for the strength the gospel brings into our lives.
I have some news that makes for a wonderful post! To start off with, Easton is getting so much stronger. Lately he can sit for longer periods of time. It is so nice to see! On another happy note, Easton now longer has a cannula! He was pulling it out all the time and I was so worried he was going to choke himself with it, so they have put him on a blow by which just blows it into his face at a higher concentration. It is so nice to not have that on his face! On Friday I did face time (Thank goodness for technology... again!) with a mom of a little girl with BVVL who lives in England. Her daughter is 7 and started having symptoms with it when she was 2.5 and it took them three years to diagnose her. She told me of many stories and said Easton sounded a little different for two reasons. He was born with problems and his hearing is severe to profound in one ear with no hearing loss in the other. They usually loose it together although one can be worse... just not that big of a difference. She explained that all the cases are different though. She gave my information to a neurologist from Canada whose daughter has the same thing. He called about 10 min after I got off with her. It was so good to talk with him. He answered so many questions and put many fears at ease. His daughter is almost 3 and they found it a year and a half ago. He talked to me about the vitamin treatments they do and how effective they have been. He said any older information past 2010 we can disregard because people are doing really well with the treatment. They have only seen good since. It has helped many kids be able to improve and they haven't seen any regression since using the treatment. He said getting an early diagnosis is vital though because they cannot regain everything they have lost, like eastons hearing loss. His daughter is doing well tho. He said all the cases are so different and agreed with the hearing loss part but definitely thought it is nerve related. The treatment can help with other nerve problems to if vitamin B is the issue. It was very reassuring, even though it doesn't take away all the concerns we have. Later that day his Neurologist office called and they are in the process of getting the testing approved for him. We will hopefully be doing it within a month and are continuously praying for good news that day. Thanks again for all your continued prayers, love and support!
Monday, April 14, 2014
More testing
I don't know how to express my feelings right now. What a journey this has been. There are days where our new normal seems so normal and there are days where I am grasping at things to make our life "normal". It seams to go in waves with doctor appointments and testing. On Friday I took Easton to a new Neurologist. Dr Sakaunju at the University of Utah. She specializes in nerves. Clear back in November most of Eastons doctors thought he would gain better muscle control start to out grow some of these things he struggles with. Doctors left and right have been shocked that he hasn't been making progress on his eating. We have been struggling with this because his muscle tone has improved a lot. They told us his eating should along with it. It hasn't. We are starting to try and feed him again by mouth and are hoping that he can tolerate the pudding consistency. (the thicker the food, the easier it is supposed to go down.) Because of the decline in his ability to swallow with the increase of muscle tone, they are becoming more convinced it has to do with the nerve. So, on Friday we met with Dr Sakaunju and she agrees. She felt like his muscle tone was good and has nothing to do with his eating. She feels that this is a nerve problem. She feels like it is most likely a "Degerative Nerve Disease/disorder." There are several syndromes that go along with something like this. She gave us paperwork for one they will test for. It is called "Brown Vialetto Van Larea Syndrome." I have to make this clear that he has NOT been diagnosed with this. We are however going to start the process of testing for it. Once approved by our innsurance we will to a test where they put him out, shock him over and over and watch what his muscles and nerves do. They will then do a muscle biopsy and if they are positive they will do blood work to confirm their findings. The prognosis of this specific syndrome is not good. The most frustrating part is that this fits him better than anything we have ever seen and she seamed very confident in her findings. People who get this syndrome usually pass away from respiratory failure. We are praying this is not what Easton has. Not one doctor has been able to put the pieces together like she has tho. It scares us. No one has been able to put together his hearing loss with anything else. This does. We are praying this is not the answer. Please pray along with us. If you go to your local temple, please add his name to the prayer roll if you don't mind. I cannot explain how it feels to have the doctors testing you baby for this. There are no words. But, never in my life have I had more faith in our Heavenly Fathers plan for us. He truly has a plan for each of us and Eastons plan will be carried out the way that it needs to be for him. I know he chose this trial and I know that we chose to be his parents and love and take care of him. I am so grateful for that and I wouldn't change it for the world. This whole process has been a challenge, but the hard moments are outweighed by so much joy, the sweetest little giggle and a smile that will melt your heart. For that, we are so very thankful. Easton is such a joy to have in our home and brings a spirit that I can't explain. We are so blessed and sure do love this sweet little angel! We will update here as we get more information.
Sunday, March 30, 2014
This blog will be a place where I can update our family and friends with the challenges and accomplishments of our sweet little man, Easton. I have struggled over the past several months as there have been many people who ask us to "let them know how things go." It isn't that I don't want to update everyone. However, it is overwhelming to make sure to get everyone and it is also hard to know who is interested in getting what updates. This will hopefully solve that problem for me, anyone who wants an update can follow our little story here as little or as often as they would like. The other big reason for this blog, is that with recent testing we are learning more about our little man. There are 2 other people who might have the same thing as Easton. Yes, only 2. I cannot wait to get in touch with these families and find out more about them. Hopefully someday this can be a resource for someone who is in our shoes with lots of questions and fears. I am hoping this will be a resource that they can find peace and learn more about what to expect for the life of a loved one who may be going though something similar to our sweet boy. I am going to start on day one, 9 months ago, so bare with me as I retrace our experience.
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