Skylar is getting some good rest right now... I am not too far behind :) Kyle stayed here for a few hours after lunch and let me go home to shower and take in some fresh air. He spoke with some of the doctors while I was gone and everyone seems to be pleased with her progress. We MIGHT be able to go home as early as tomorrow if she can tolerate her full feedings (we're still at 20 mL right now) but if not, then probably the next day. We still have to learn how to feed her through the g-tube with the pump (shouldn't be too different than what we were doing with the ng tube) so things are looking good. We'll try her first full feed in the morning and see how she does.
When I got back to the hospital after going home, and grabbed her little hand, I was greeted by the first smile of our hospital stay... It was comforting to see her give a few smiles throughout the evening, because when we came at the beginning of the month for her ng tube, she didn't smile at all the entire time! I almost got a little giggle out of her today, but not quite. She hasn't had too much pain medicine today and is such a trooper! She's adjusting to the c-pap at night and doing really well. I'll update you with our schedule tomorrow...
Wednesday, March 31, 2010
New Room :)
So we moved to the floor today out of the PICU - Skylar's hardly on any pain medication and seems to be doing well. She's awake and content, not really fussing, and loves the new pink Superwoman balloon Grandma brought :) We decorated her crib since she's alert and just looks around. We just had her first feeding through the new g-tube to test it out... a whopping 10 mL! We'll be testing out the feeds in really small doses for a while and then we'll bump it up to the amount she was on pre-op. Once she's handling that, hopefully we'll be able to go home. It still may be a couple of days, but I'm hopeful that we'll be out by Saturday, possibly sooner. She just closed her eyes for a nap.
I've been really blessed to have been put in contact with a woman whose little boy has SMA type 1 and he is doing really well - she had some great information to pass on to us that will hopefully help Skylar in the long run. Their blog is www.hopeformax.com if you're interested. She was able to share about issues with feeding and typical formula vs. a simple protein mix to save muscle strength in digestion. We're going to meet with a nutritionist here to discuss things further to make sure Skylar gets exactly what she needs. We'll keep you posted. Hope everyone is having a great week so far - thanks for checking in on us!
I've been really blessed to have been put in contact with a woman whose little boy has SMA type 1 and he is doing really well - she had some great information to pass on to us that will hopefully help Skylar in the long run. Their blog is www.hopeformax.com if you're interested. She was able to share about issues with feeding and typical formula vs. a simple protein mix to save muscle strength in digestion. We're going to meet with a nutritionist here to discuss things further to make sure Skylar gets exactly what she needs. We'll keep you posted. Hope everyone is having a great week so far - thanks for checking in on us!
Tuesday, March 30, 2010
Recovery Goal: Decrease Pain
Lots of thanks and praise going on right now! Skylar has been on room air since the procedure (I guess it's been 9 hours or so) and is having a great recovery thus far. We were told prior to surgery that Skylar would most likely be on some breathing assistance coming out of surgery and that part of the recovery time would be weaning her off the machines (either ventilator or bi-pap) and she hasn't needed anything! I am so proud of her!!! Like I said before, she's a champ :) she's got 4 little incisions across her belly in an arc and they went through her belly button too so it's a bit bigger than I remember... then she's got the plastic port for the g-tube so I am sure her whole middle section is pretty sore. There's a board in our room with all of the nurse info on it and at the bottom is a goal for the day: decrease pain. I like it.
Today she has been on IV fluids as far as feeding goes and has been on heavy pain medication, but she's handling it really well. Tonight she will be on the c-pap (if they can find one that fits her) just to help open her lungs some, which she does at home too, and then tomorrow afternoon or evening will be her first feeding through the new g-tube. She's had some pretty thick mucous in her mouth that we've been suctioning out, but that's about it. So far so good :) Once we get her feedings on the g-tube regulated and up to the amount we were doing through the ng tube, we should be good to go home. Hopefully that will just be three days now.
I can't tell you how amazed I am by her current state. I honestly didn't know what to expect. I know that some babies don't come out of surgery well, so I just thank you for your prayers and praise God for Skylar and great doctors. Even the nurse said we had some of the best doctors working with Skylar (knowing the surgeon and the neurologist). Our nurses so far in the PICU have been great too. We feel extremely blessed. Just taking things one day at a time... today was a good day and hopefully tomorrow will be too! I'll keep you posted.
Today she has been on IV fluids as far as feeding goes and has been on heavy pain medication, but she's handling it really well. Tonight she will be on the c-pap (if they can find one that fits her) just to help open her lungs some, which she does at home too, and then tomorrow afternoon or evening will be her first feeding through the new g-tube. She's had some pretty thick mucous in her mouth that we've been suctioning out, but that's about it. So far so good :) Once we get her feedings on the g-tube regulated and up to the amount we were doing through the ng tube, we should be good to go home. Hopefully that will just be three days now.
I can't tell you how amazed I am by her current state. I honestly didn't know what to expect. I know that some babies don't come out of surgery well, so I just thank you for your prayers and praise God for Skylar and great doctors. Even the nurse said we had some of the best doctors working with Skylar (knowing the surgeon and the neurologist). Our nurses so far in the PICU have been great too. We feel extremely blessed. Just taking things one day at a time... today was a good day and hopefully tomorrow will be too! I'll keep you posted.
What A Champ!
Skylar just got out of surgery and she's such a champ! The surgery was just under two hours - she has a few incisions on her belly where they did the fundo and the g-tube is in place. The doctors thought she might have to be on assisted breathing coming out of surgery because of her SMA weakness, but she's able to breathe on her own just fine! Even though there's no respirator or bi-pap machine, they're keeping her in the PICU (pediatric intensive care unit) for the night just to be safe. We just saw her as she was wheeled by and she looks great - a little out of it because of the drugs obviously - but you would never know she just had a serious procedure done by looking at her face. Hopefully the pain medicine keeps her comfortable and she's able to handle fluids well with the new g-tube. Once she gets back up to the schedule we had her on at home, we'll be able to be released... I'll keep you posted. Thanks for the prayers!!!
Monday, March 29, 2010
Life and Death.
Being told your child is going to die, well, it makes you think.
After watching Gran Torino (a fabulous movie I highly recommend), I realized that I don't know much about death. Unlike Clint Eastwood's character, Walt, I have never been to war, never killed anyone, never had an immediate family member die, never watched anyone die, etc. My first experience with death that I can remember was when I was around 7 or 8 years old and I went to an open casket funeral for a lady who lived next door to my grandparents. I'm not gonna lie, it freaked me out! My dad and I watched too many scary movies together while I was growing up and I seriously was waiting for her to pop up and grab me, even though she was a super sweet old lady. It makes me laugh thinking about it now, and how different things can influence your thoughts and beliefs... Anyway, not knowing a lot about death isn't a bad thing, but I think it leaves room for a lot of fear. I've had quite a bit of time to think on the subject recently, and since we're going to be faced with it if Skylar isn't healed miraculously by God, I thought I would ramble along some of my thoughts here...
My "postpartum depression" as it's called, was interesting. I had some uncontrollable sobbing, but only because I was thinking about death so much. It's funny what bringing a life into this world will do to you. I don't know if God was preparing me to deal with what lies ahead, but I just remember being extremely challenged with the purpose of life. I knew that one day, I would have to explain to my little girl that I brought her into this world (through birth, not that I created her), she has a purpose, but she will have to die some day too. I had no idea at the time of course that it may be so soon... I was thinking about how I would answer her questions about God, about life, and about death. I had to really examine what I believe and why I believe it because I couldn't just make something up to tell my child, or for that matter, just tell her what sounds "right" according to society. I was being brought back to the fundamentals of Christianity. Looking back on it, I think God was asking me if I truly believed in Him and used that time of questioning to strengthen my faith for the present.
I really struggled with death growing up. I used to cry myself to sleep thinking about losing my dog. It's something I really never understood. I knew that everyone was going to die at some point, but I just assumed it was going to be of old age - nothing horrible would happen to me or my family because I loved them too much, right? Wrong. My aunt passed away when I was in college - she battled cancer for a long time. I saw her faith and how many people she impacted through her life and it blew my mind. I hated that she had to die, but she went out smiling to be with the Lord. I realized then, and am still learning, that as Christians, we shouldn't be afraid of death because we have the promise of eternal life in Christ. I guess my faith was never solid enough to stand on that truth until now. I can honestly say, after everything we've been through, that I am not afraid of death anymore. I am afraid of how I am going to die, how family members and friends will die, because I love them so much and don't want to see anyone suffer or go through horrible pain... but once I am gone, I have faith that there is a heaven and I will be there celebrating life hopefully with everyone that I love. It's frightening to me how fragile we are and I am much more aware of my mortal flesh, but that's no reason to live in fear and not experience life. My dad and I have discussed recently how death makes us sad just because we have so much love for someone and miss the relationship we had with that person. A good friend was just over today for lunch and we talked about the same thing and how we feel a little left behind, or are scared to leave others behind. She mentioned that there's nothing natural about losing a child though and that death is a little harder to deal with. I have to agree.
There is a book called The Mystery of Marriage that is absolutely incredible and has a chapter dedicated to death. I hope you can check out the book sometime because it is everything I have ever thought about death, but put so eloquently and has so many great metaphors I'm not even going to try to summarize. It's a great book about marriage and life as well :)
I learned last week that 80% of marriages that go through the loss of a child end in divorce. I think that stat should be changed to any marriage that deals with a tragedy of some sort because it puts a lot of stress on people as individuals, and in turn, the marriage. I think that going through anything tragic will put incredible strain on a relationship, but Kyle and I are committed to each other and in our faith - we're confident that we can beat the odds, especially with the love and support from friends and family and your continued prayers. We're hoping that Skylar can beat the odds too and look forward to her first birthday.
Just recently, Kyle asked me why I tend to avoid the subject of death (as far as watching movies, reading books, etc.). Kyle is very pensive and enjoys thinking about a lot of different subjects and then discussing them... Not that I am not a thoughtful person, but I tend to be an extreme romanticist, while he's more of a realist. I don't mind a serious movie or book every now and then (I just finished Gilead, which was a great book!) but I like to be entertained to give my mind a break since I think about serious things quite often - I just don't talk about it much because it makes me sad. With movies, and really life too, I love the happy ending, the warm fuzzy feeling you get when things go "the way they should" or when people get what they deserve, or better. I loved the thought that we are going through this season of lent with a heavy burden and lots of suffering, the way lent is supposed to be, and that God would heal Skylar on Easter or during the Easter season because it's a time of celebration and would be incredible symbolism of Christ's resurrection. I then quickly realized that my plan isn't always the best plan, even though it sounds good to me, lol. God may have other plans, as much as I would love to see my little girl grow up. I know that God allows things to happen for a reason and has perfect timing and perfect planning. Don't get me wrong, I will be asking for a miracle until she takes her last breath, and then I'll still hope that there's a chance. Already, I have been so blessed by Skylar and how she's allowed me to grow as a person. To see how her life has already impacted others has been incredible and would not have been the case if she were a "normal" baby. Kyle and I have been able to meet some awesome people and continue to be surprised by the crazy connections and fun stories we're able to take part in because of this - not to mention the love we've felt from others.
Last week on monday, we had a meeting with the neurologist, who is kind of like an angel of death. What I mean is that he knows what is to come for SMA babies (he had a family member go through it) so he is doing his best to look out for us and prepare us for the road ahead the best he possibly can. He is a super nice guy, compassionate, and wants to make sure that Kyle and I are taking care of ourselves, and our marriage, in addition to Skylar. He asked me about any decisions we've made for Skylar and her medical care, support groups, etc. He said I looked tired (which was an understatement at the time) and wanted to make sure we got Skylar on a more manageable feeding schedule as soon as possible. He was encouraging - it was a good meeting and I let him know that we were preparing for the surgery and hopefully it would be sooner than later. That's typically the motto with SMA - the sooner the better...
A couple days later, we had a few more appointments with the pediatrician and the GI doctor. The first two weeks she was feeding through her ng tube, she didn't gain hardly any weight... we had her 4 month check up Friday morning and then a GI check up that afternoon. Both agreed that we needed to change something to help her gain weight. Her head circumference and length are both on track, but her weight isn't even close to the curve. At 4 months, she weighed only 9 lbs., but since the other measurements were on track, the pediatrician knew it was just a matter of calories to increase her weight. The GI doctor decided to bump her up from 20 cal to 24 cal and that made all the difference. She has gained 8 ounces in the past week and got the green light for surgery. We had her pre-op appointment this past Friday and were told that the surgeon is going on spring break April 2 so her surgery may be before then, but if not, it would be at least two more weeks.
We got a phone call today that her surgery is scheduled for tomorrow morning. Short notice, I know! It's a two hour surgery and will more than likely be a 4-5 day recovery. As soon as I finish posting this, I will be packing a bag, getting Skylar her last bit of food for a long time, and hopefully getting some rest. I am praying obviously for a great surgery and easy/quick recovery, but it would mean so much to me to be able to go to the Easter service at our church. I don't know that Easter has ever meant more to me than it does now. I truly am looking forward to the celebration, knowing the sacrifice that God had to make watching His only Son suffer and die on the cross, and knowing that we have hope and life to the fullest. I feel like we've been able to get a little bit more insight into the character of God through our experience and am thankful for that. I'll try to update the blog this week to keep you informed of Skylar's progress and recovery. Skylar's a little fighter and I have no doubt that her determined little spirit will help her get back to her smiley self after a not so fun surgery. Thanks for reading - my next one will probably be a short update... hopefully a lighter note - no heavy thoughts :) Have a great week!!!
Peace.
After watching Gran Torino (a fabulous movie I highly recommend), I realized that I don't know much about death. Unlike Clint Eastwood's character, Walt, I have never been to war, never killed anyone, never had an immediate family member die, never watched anyone die, etc. My first experience with death that I can remember was when I was around 7 or 8 years old and I went to an open casket funeral for a lady who lived next door to my grandparents. I'm not gonna lie, it freaked me out! My dad and I watched too many scary movies together while I was growing up and I seriously was waiting for her to pop up and grab me, even though she was a super sweet old lady. It makes me laugh thinking about it now, and how different things can influence your thoughts and beliefs... Anyway, not knowing a lot about death isn't a bad thing, but I think it leaves room for a lot of fear. I've had quite a bit of time to think on the subject recently, and since we're going to be faced with it if Skylar isn't healed miraculously by God, I thought I would ramble along some of my thoughts here...
My "postpartum depression" as it's called, was interesting. I had some uncontrollable sobbing, but only because I was thinking about death so much. It's funny what bringing a life into this world will do to you. I don't know if God was preparing me to deal with what lies ahead, but I just remember being extremely challenged with the purpose of life. I knew that one day, I would have to explain to my little girl that I brought her into this world (through birth, not that I created her), she has a purpose, but she will have to die some day too. I had no idea at the time of course that it may be so soon... I was thinking about how I would answer her questions about God, about life, and about death. I had to really examine what I believe and why I believe it because I couldn't just make something up to tell my child, or for that matter, just tell her what sounds "right" according to society. I was being brought back to the fundamentals of Christianity. Looking back on it, I think God was asking me if I truly believed in Him and used that time of questioning to strengthen my faith for the present.
I really struggled with death growing up. I used to cry myself to sleep thinking about losing my dog. It's something I really never understood. I knew that everyone was going to die at some point, but I just assumed it was going to be of old age - nothing horrible would happen to me or my family because I loved them too much, right? Wrong. My aunt passed away when I was in college - she battled cancer for a long time. I saw her faith and how many people she impacted through her life and it blew my mind. I hated that she had to die, but she went out smiling to be with the Lord. I realized then, and am still learning, that as Christians, we shouldn't be afraid of death because we have the promise of eternal life in Christ. I guess my faith was never solid enough to stand on that truth until now. I can honestly say, after everything we've been through, that I am not afraid of death anymore. I am afraid of how I am going to die, how family members and friends will die, because I love them so much and don't want to see anyone suffer or go through horrible pain... but once I am gone, I have faith that there is a heaven and I will be there celebrating life hopefully with everyone that I love. It's frightening to me how fragile we are and I am much more aware of my mortal flesh, but that's no reason to live in fear and not experience life. My dad and I have discussed recently how death makes us sad just because we have so much love for someone and miss the relationship we had with that person. A good friend was just over today for lunch and we talked about the same thing and how we feel a little left behind, or are scared to leave others behind. She mentioned that there's nothing natural about losing a child though and that death is a little harder to deal with. I have to agree.
There is a book called The Mystery of Marriage that is absolutely incredible and has a chapter dedicated to death. I hope you can check out the book sometime because it is everything I have ever thought about death, but put so eloquently and has so many great metaphors I'm not even going to try to summarize. It's a great book about marriage and life as well :)
I learned last week that 80% of marriages that go through the loss of a child end in divorce. I think that stat should be changed to any marriage that deals with a tragedy of some sort because it puts a lot of stress on people as individuals, and in turn, the marriage. I think that going through anything tragic will put incredible strain on a relationship, but Kyle and I are committed to each other and in our faith - we're confident that we can beat the odds, especially with the love and support from friends and family and your continued prayers. We're hoping that Skylar can beat the odds too and look forward to her first birthday.
Just recently, Kyle asked me why I tend to avoid the subject of death (as far as watching movies, reading books, etc.). Kyle is very pensive and enjoys thinking about a lot of different subjects and then discussing them... Not that I am not a thoughtful person, but I tend to be an extreme romanticist, while he's more of a realist. I don't mind a serious movie or book every now and then (I just finished Gilead, which was a great book!) but I like to be entertained to give my mind a break since I think about serious things quite often - I just don't talk about it much because it makes me sad. With movies, and really life too, I love the happy ending, the warm fuzzy feeling you get when things go "the way they should" or when people get what they deserve, or better. I loved the thought that we are going through this season of lent with a heavy burden and lots of suffering, the way lent is supposed to be, and that God would heal Skylar on Easter or during the Easter season because it's a time of celebration and would be incredible symbolism of Christ's resurrection. I then quickly realized that my plan isn't always the best plan, even though it sounds good to me, lol. God may have other plans, as much as I would love to see my little girl grow up. I know that God allows things to happen for a reason and has perfect timing and perfect planning. Don't get me wrong, I will be asking for a miracle until she takes her last breath, and then I'll still hope that there's a chance. Already, I have been so blessed by Skylar and how she's allowed me to grow as a person. To see how her life has already impacted others has been incredible and would not have been the case if she were a "normal" baby. Kyle and I have been able to meet some awesome people and continue to be surprised by the crazy connections and fun stories we're able to take part in because of this - not to mention the love we've felt from others.
Last week on monday, we had a meeting with the neurologist, who is kind of like an angel of death. What I mean is that he knows what is to come for SMA babies (he had a family member go through it) so he is doing his best to look out for us and prepare us for the road ahead the best he possibly can. He is a super nice guy, compassionate, and wants to make sure that Kyle and I are taking care of ourselves, and our marriage, in addition to Skylar. He asked me about any decisions we've made for Skylar and her medical care, support groups, etc. He said I looked tired (which was an understatement at the time) and wanted to make sure we got Skylar on a more manageable feeding schedule as soon as possible. He was encouraging - it was a good meeting and I let him know that we were preparing for the surgery and hopefully it would be sooner than later. That's typically the motto with SMA - the sooner the better...
A couple days later, we had a few more appointments with the pediatrician and the GI doctor. The first two weeks she was feeding through her ng tube, she didn't gain hardly any weight... we had her 4 month check up Friday morning and then a GI check up that afternoon. Both agreed that we needed to change something to help her gain weight. Her head circumference and length are both on track, but her weight isn't even close to the curve. At 4 months, she weighed only 9 lbs., but since the other measurements were on track, the pediatrician knew it was just a matter of calories to increase her weight. The GI doctor decided to bump her up from 20 cal to 24 cal and that made all the difference. She has gained 8 ounces in the past week and got the green light for surgery. We had her pre-op appointment this past Friday and were told that the surgeon is going on spring break April 2 so her surgery may be before then, but if not, it would be at least two more weeks.
We got a phone call today that her surgery is scheduled for tomorrow morning. Short notice, I know! It's a two hour surgery and will more than likely be a 4-5 day recovery. As soon as I finish posting this, I will be packing a bag, getting Skylar her last bit of food for a long time, and hopefully getting some rest. I am praying obviously for a great surgery and easy/quick recovery, but it would mean so much to me to be able to go to the Easter service at our church. I don't know that Easter has ever meant more to me than it does now. I truly am looking forward to the celebration, knowing the sacrifice that God had to make watching His only Son suffer and die on the cross, and knowing that we have hope and life to the fullest. I feel like we've been able to get a little bit more insight into the character of God through our experience and am thankful for that. I'll try to update the blog this week to keep you informed of Skylar's progress and recovery. Skylar's a little fighter and I have no doubt that her determined little spirit will help her get back to her smiley self after a not so fun surgery. Thanks for reading - my next one will probably be a short update... hopefully a lighter note - no heavy thoughts :) Have a great week!!!
Peace.
Tuesday, March 16, 2010
The Night Shift
This is probably the longest post yet because there is so much to say and some of you have not been updated in a while... I apologize if you're a friend and just learning about Skylar through this and not from us personally - we've had a lot on our plate and we've lost track of who knows what. I finally had some time to get her website together so I thought I would update the blog as well to keep everyone on the same page.
Ever since high school, I thought God might give me a special child. It started when I did the "peer mentorship" program where I sat in on a class with 5 severely handicapped children (2 cases of severe cerebral palsy and three other disabling diseases) and assisted the teacher interacting with the kids. It was tough. I remember feeling a tug on my heart and I thought, "Dear God, why are these kids the way they are and how in the world do their parents do it all day, every day?! Please don't let that be me..." Then I went to N. Ireland on a mission trip and there was a child with down syndrome in the mix of kids we worked with. I felt God saying, "Ashley, get ready. That could be your child." Again, my thoughts were, please no - I don't understand. Then in college, I attended a church where a guest speaker had a sermon entitled "The Night Shift," which was based on the speaker's own life of having a severely handicapped child after one healthy child. I don't remember the speaker's name, but the entire message stuck with me, and again, I heard God saying, "Ashley, I am preparing you... get ready."
The sermon in a nutshell was that you get put in situations that you don't want to be in, and no one else would really want them either, which is why he called it the night shift: no one wants it, it's draining, not the normal routine, and you have to adjust or you won't survive. Just last month, I was flipping through my Bible, and out of nowhere, the sermon notes from that message fell out onto my lap. I hadn't seen them since the day I wrote them and wasn't even aware I still had them. I was shocked, but smiled a little bit, "Are you joking me? Thanks God..." Here is a short summary: The night shift = times in life where circumstances we can't control get out of control. It was a typical three point sermon. First you need to calm down - you can't see into the future so don't create anxiety over the "what ifs" and he quoted Phillipians 4:6-7 about praying. Second, you need to wise up. Asking why will get you no where and nobody promised life would be fair so don't go down that road either. If you compare your life to others, you'll always find someone you want to be more like, but that's not your life to live. For whatever reason, God made you unique and gave you the life that you have. Last, you need to press on. Be honest with where you are every second of the day. Pursue your relationship with God and find the joy and humor in life - even through trials.
That is where Kyle and I find ourselves right now; the night shift. Kyle too shared with me later that he felt like he was going to have a special child as well, but we had never shared that with each other until we found out about Skylar.
At Skylar's one month check up, she had low muscle tone and was really low for her weight percentile. I pointed out to her doctor that Skylar's right arm looked like a chicken wing - it was always bent at the elbow and wrist (we called it her lucky fin and she was our little nemo). Our pediatrician sent us to a brachial plexus specialist thinking it might have been a shoulder injury during birth so we went to see the doctor on the 8th of January. It was a snow day and we braved our way to Children's hospital to see the physical therapist and doctor. The PT evaluated Skylar and said both of her arms were affected in different ways. The doctor came in later and noticed that all limbs were affected because he couldn't get a reflex out of her knees. When I saw that, I knew we were in trouble. As I was leaving the office, I noticed him watching me from down the hall as I carried Skylar out of the room. The hall was dark and I couldn't see his face extremely well because I just glanced at him, but I just got this overwhelming feeling of pity - as if he knew something horrible and felt so sorry for me. It was extremely unsettling and I just wanted to scream, "what are you not telling me?!" He wanted to send us to see a neurologist and since we had a newborn, he made some phone calls so we didn't have to wait 3 months to get in. The neurologist's office was going to call me to schedule the appointment.
After I left, within an hour, I got a phone call from Skylar's pediatrician. He said that if they couldn't get me in within two weeks to see the neurologist, then to let him know and he would make some calls. This was very alarming to me because I didn't think it could be that serious (I was thinking some physical therapy, or at worse, surgery would do the trick). I asked him what the problem was and why the neurologist. He said that he is pretty sure Skylar has some sort of neurological muscular disease or disorder and is "extremely concerned." Shocked, I asked if there was a possibility that she could be normal and it could just be that my breast milk wasn't doing the trick or something else not so bad. He said he doubted it, but didn't want to scare me with some of the possibilities, so we would just wait for the neurologist's appointment.
When I got the call that our appointment was scheduled for Jan. 22, I called my pediatrician to let him know and he said that he would give me his cell phone and would be available all weekend if my husband and I needed him. He even said that he would keep Saturday open if we needed to meet with him and talk. In my head I am thinking, "Holy crap! What the hell is going to be wrong with my baby?!" Scared, I asked what the worst case scenario was, but I still didn't know how horrible it could be. With hesitation, he listed one disorder and then listed a few others that I had never heard of. I asked if any could be cured through surgery or treatments or medication and he said no. He said he was praying with all of his heart that our daughter didn't have any of these. He gave me his cell phone number and said if I made the really bad decision to google any of them, he would be available to answer my questions. I googled them so I would at least know what we were up against, and I was terrified. I had no idea some of these disorders even existed and they were just horrible, enough to make you sick. I couldn't eat for two days, but forced myself to at least drink protein smoothies since I was breastfeeding and Skylar needed me to eat. SMA was among the top of the list, which stands for Spinal Muscular Atrophy. It is the number one genetic cause of death in infants before the age of two. I was thinking, and sadly hoping, it could be cerebral palsy and was prepared to go for stem cell transplants in Germany, a life time of physical therapy, etc. but was reminded of SMA after stumbling on a website dedicated to a girl named Skylar who had SMA type 2 and died after 7 or 8 years. A week later, after trying to hold it together, but really being an emotional wreck, we had Skylar's 2 month appointment.... The pediatrician was extremely concerned, because he felt like her muscle tone has gotten worse and said he thought it was SMA (which was the worst case scenario) and said that SMA was the first thing out of the brachial plexus specialists' mouth as well. Of course I cried, he teared up a little, and asked to pray with me. He even said he was thinking about taking Friday off to come to the neurologist with us, but wasn't sure it was possible. At our neurologist visit, our worst fears were confirmed when the doctor said he too was pretty sure it was SMA, but a genetic blood test would tell for sure. It would take 3-4 weeks for the blood test to come back...
In the meantime, we prepared for the worst and went ahead and made an appointment with the pulmonologist to establish a baseline for Skylar's lungs since SMA affects all voluntary muscles, breathing and swallowing muscles included, which is typically the cause of death (choking, pneumonia from aspiration, lung collapse, or a respiratory infection). The first bit of good news we received: Skylar's lungs were clear and looked good thus far. We also did a swallow study which showed Skylar aspirated slightly on thin fluid from a bottle, but seemed fine breast feeding. Aspiration is a huge concern because the muscles just get weaker so more and more fluid could end up in the lungs, which could be fatal.
Four weeks later, I get the call. Skylar does in fact have SMA type 1. According to statistics, she has a 10% chance of making it to her first birthday. And if so, it's even less likely that she'll see her second. SMA is caused by a missing or defective gene that is responsible for producing a specific protein that voluntary muscles use to grow. There is no cure and very little treatment currently. Because this is genetic, every child Kyle and I might have after this would have a 25% chance of having SMA, 50% chance of being a carrier, and 25% chance that the child would be unaffected. Obviously, we have some tough decisions to make as far as family planning goes, but even more tough decisions in the near future with Skylar. We've been asked to think about whether or not we would put her on life support, which no parent ever wants to be faced with that decision.
Just last week, we had an appointment with the GI doctor because Skylar started choking while breast feeding and we were looking into the option of having a feeding tube put in. The GI doctor agreed with us and said the sooner the better. She also is struggling to gain weight - one, because her muscles aren't growing due to the lack of SMN Protein so she has no muscle mass, but two, because her little muscles have to work twice as hard for normal functions and she burns through calories and isn't getting enough to gain weight. We were at Children's hospital 3 days later to get Skylar on a feeding tube through her nose to try and fatten her up a little before the surgery.
The problem with SMA is that it affects so much of the body, we have several specialists working with us instead of just one doctor. The real problem is that it is a rare disorder and not many doctors are familiar with SMA and the best "treatments" since there is no cure. Luckily, our pediatrician has been fabulous and seems to know a lot more about SMA than most. Also, our neurologist has had first hand experience with the disease - a family member of his had a child with SMA type 1. Our pediatrician mentioned some options to me that I brought up with the GI doctor while we were in the hospital the morning she was supposed to get her surgery. He seemed a little puzzled with the suggestion, and hours before she was supposed to get her IV to get ready for the G-tube placement, the GI doctor came in a told us he had a change of plans. Apparently he had talked to several other doctors about SMA and the best options for treatment during the day and heard about another SMA child in the same hospital who already had a g-tube placed, and was now getting treated for reflux. Our GI doctor knew the feeding and swallowing was a problem, but he wasn't aware how severe the reflux could be. So instead of getting the g-tube and then having another procedure a few weeks later (like the child was having to do), he said that he wanted to postpone the surgery for 3 weeks or so and do everything at once. We're continuing to pray for wisdom for each of her doctors to do the best thing and to have the most current knowledge of procedures and treatments.
SMA children suffer from reflux because of their weak muscle tone so we're going to get a procedure done called a fundoplication (Skylar is too small for a J-tube). A fundo is where they wrap the top of the stomach around the base of the esophagus so when it contracts to cause reflux, it closes of the esophagus instead or allowing things to come up. This is much more invasive and very intense for a small 4 month old baby, but we're optimistic and hope it will help her feel better and get her nutrients. Currently, she is still on the nose feeding tube at home and I am learning how to become a nurse :) I have mastered the feeding machine, use a suction machine to take care of the mucous she can't swallow, and also give her treatments through a nebulizer to help her lungs. We're also awaiting a c-pap machine delivery because she needs a little help getting some oxygen at night while she sleeps according to the results of her sleep study we just completed. It just adds pressure to her breaths to help her lungs open better - it's not like a ventilator or anything that breathes for her. Our nursery is starting to look like a medical room, but I would much rather have that than no reason for a nursery at all.
I am still hoping for and believing in a miracle. Her range of motion has gotten better, even though her muscles aren't gaining mass. She loves the bath tub and finds the water is a great place to move like a normal baby since her limbs are weightless. She smiles big and attempts to make little noises here and there. I hope that one day, Skylar will be able to grab for a toy she seems to stare at so intently. I hope that she'll be able to hold her head up, sit up on her own, talk and breathe easily, enjoy the taste of food, and eventually learn to crawl and walk. I pray that God would reach His hand down and replace the gene that is missing in her body or that the SMN protein that her muscles need to grow would be produced in her body. Of course, if God chooses not to heal her, I pray for strength and peace for Kyle and I to go forward and that God would be close to both of us as we draw near to Him and each other.
Skylar squealed with joy for the first time ever almost two weeks ago when I pulled her hands up and let them run down through my hair. I realized that babies typically pull on things like hair and jewelry and since Skylar can't, I thought she might like to try with my help... It melted my heart and made me cry with joy. Playing with my hair makes her smile every time... at least for now :) She's starting to giggle too, which is the sweetest sound I've ever heard. I ask God for more moments like this to help us get through the tough times.
We're supposed to be feeding her through the ng tube every 2.5 hours with 2.5 ounces. We just stepped it up to 3 ounces every three hours to try and keep our sanity. I've already missed two of her middle of the night feeds, but she slept through them too so I didn't feel too bad about it. We had a pretty rough scare the other night and we thought we might lose her.
I was supposed to be sleeping, but I couldn't, and Kyle had just finished a feed and was trying to get Skylar to sleep in her crib. She always has trouble breathing, but she was making unusual noises as her breathing pattern became concerning. It seemed as if she was really struggling so I got up and went in there and Kyle wasn't sure what to do. He said he didn't see her nostrils flaring badly (a sign we were told to look for) and I picked her up and unbuttoned her pjs to take a look at her ribs. The skin over her ribs was clinging slightly with her breaths, but not badly (another sign we were told to watch for). I just held her, rubbed her belly, told her to take deep breaths, and then just cried my eyes out. I am not ready to lose my little girl. I know that God loves her more than I do, and we trust Him, but it doesn't make it any less painful.
Thank you to all who have prayed for Skylar and for us thus far. We definitely feel the grace of God and are surrounded with peace through this tough time. I think it's fitting that it's the season of lent right now and we are definitely suffering (besides giving up stuff on our own). I look forward to Easter, to spring time, and a hope of all things made new and a cause for celebration. Our family has been extremely supportive and helpful and we are extremely thankful to have our parents close by. Friends and church family have been amazing as well - thank you for prayer, listening to my ramblings, meals and your support. Some of Kyle's friends are fasting every Monday, the moms group at church meets every other Thursday for prayer, and we've received cards/emails from classes, small groups, and from people all over who have heard about our struggle. My friend is already working on raising money to donate to Families of SMA and I even ran a 5k at 8 AM to help the cause. Go ahead, you can laugh. I haven't run a 5k since high school and I certainly haven't done anything active for years - needless to say, my body felt like it got ran over and beat up and I slept like a bear in the winter. Back to the point, to all of you who have helped and prayed - Thank You. We are very grateful and so humbled.
Sorry this one was so long - I am going to make sure I update this more frequently now that we've told everyone about Skylar and have a lot coming up to prevent further novels on the blog. Her 4 month check up is this week along with another GI appointment to track her progress. We'll have another pulmonologist and neurologist appointment to touch base with everyone before the surgery. Her pre-op will be late next week and surgery will be scheduled after that (I am guessing the following week if all goes well).
Hope you like the new website at www.skylarmariejones.com - I thought it would make it easier for everyone to find and check the blog whenever. Thanks for making it this far. I hope this post finds you healthy and happy. Talk to you soon. Peace.
Ever since high school, I thought God might give me a special child. It started when I did the "peer mentorship" program where I sat in on a class with 5 severely handicapped children (2 cases of severe cerebral palsy and three other disabling diseases) and assisted the teacher interacting with the kids. It was tough. I remember feeling a tug on my heart and I thought, "Dear God, why are these kids the way they are and how in the world do their parents do it all day, every day?! Please don't let that be me..." Then I went to N. Ireland on a mission trip and there was a child with down syndrome in the mix of kids we worked with. I felt God saying, "Ashley, get ready. That could be your child." Again, my thoughts were, please no - I don't understand. Then in college, I attended a church where a guest speaker had a sermon entitled "The Night Shift," which was based on the speaker's own life of having a severely handicapped child after one healthy child. I don't remember the speaker's name, but the entire message stuck with me, and again, I heard God saying, "Ashley, I am preparing you... get ready."
The sermon in a nutshell was that you get put in situations that you don't want to be in, and no one else would really want them either, which is why he called it the night shift: no one wants it, it's draining, not the normal routine, and you have to adjust or you won't survive. Just last month, I was flipping through my Bible, and out of nowhere, the sermon notes from that message fell out onto my lap. I hadn't seen them since the day I wrote them and wasn't even aware I still had them. I was shocked, but smiled a little bit, "Are you joking me? Thanks God..." Here is a short summary: The night shift = times in life where circumstances we can't control get out of control. It was a typical three point sermon. First you need to calm down - you can't see into the future so don't create anxiety over the "what ifs" and he quoted Phillipians 4:6-7 about praying. Second, you need to wise up. Asking why will get you no where and nobody promised life would be fair so don't go down that road either. If you compare your life to others, you'll always find someone you want to be more like, but that's not your life to live. For whatever reason, God made you unique and gave you the life that you have. Last, you need to press on. Be honest with where you are every second of the day. Pursue your relationship with God and find the joy and humor in life - even through trials.
That is where Kyle and I find ourselves right now; the night shift. Kyle too shared with me later that he felt like he was going to have a special child as well, but we had never shared that with each other until we found out about Skylar.
At Skylar's one month check up, she had low muscle tone and was really low for her weight percentile. I pointed out to her doctor that Skylar's right arm looked like a chicken wing - it was always bent at the elbow and wrist (we called it her lucky fin and she was our little nemo). Our pediatrician sent us to a brachial plexus specialist thinking it might have been a shoulder injury during birth so we went to see the doctor on the 8th of January. It was a snow day and we braved our way to Children's hospital to see the physical therapist and doctor. The PT evaluated Skylar and said both of her arms were affected in different ways. The doctor came in later and noticed that all limbs were affected because he couldn't get a reflex out of her knees. When I saw that, I knew we were in trouble. As I was leaving the office, I noticed him watching me from down the hall as I carried Skylar out of the room. The hall was dark and I couldn't see his face extremely well because I just glanced at him, but I just got this overwhelming feeling of pity - as if he knew something horrible and felt so sorry for me. It was extremely unsettling and I just wanted to scream, "what are you not telling me?!" He wanted to send us to see a neurologist and since we had a newborn, he made some phone calls so we didn't have to wait 3 months to get in. The neurologist's office was going to call me to schedule the appointment.
After I left, within an hour, I got a phone call from Skylar's pediatrician. He said that if they couldn't get me in within two weeks to see the neurologist, then to let him know and he would make some calls. This was very alarming to me because I didn't think it could be that serious (I was thinking some physical therapy, or at worse, surgery would do the trick). I asked him what the problem was and why the neurologist. He said that he is pretty sure Skylar has some sort of neurological muscular disease or disorder and is "extremely concerned." Shocked, I asked if there was a possibility that she could be normal and it could just be that my breast milk wasn't doing the trick or something else not so bad. He said he doubted it, but didn't want to scare me with some of the possibilities, so we would just wait for the neurologist's appointment.
When I got the call that our appointment was scheduled for Jan. 22, I called my pediatrician to let him know and he said that he would give me his cell phone and would be available all weekend if my husband and I needed him. He even said that he would keep Saturday open if we needed to meet with him and talk. In my head I am thinking, "Holy crap! What the hell is going to be wrong with my baby?!" Scared, I asked what the worst case scenario was, but I still didn't know how horrible it could be. With hesitation, he listed one disorder and then listed a few others that I had never heard of. I asked if any could be cured through surgery or treatments or medication and he said no. He said he was praying with all of his heart that our daughter didn't have any of these. He gave me his cell phone number and said if I made the really bad decision to google any of them, he would be available to answer my questions. I googled them so I would at least know what we were up against, and I was terrified. I had no idea some of these disorders even existed and they were just horrible, enough to make you sick. I couldn't eat for two days, but forced myself to at least drink protein smoothies since I was breastfeeding and Skylar needed me to eat. SMA was among the top of the list, which stands for Spinal Muscular Atrophy. It is the number one genetic cause of death in infants before the age of two. I was thinking, and sadly hoping, it could be cerebral palsy and was prepared to go for stem cell transplants in Germany, a life time of physical therapy, etc. but was reminded of SMA after stumbling on a website dedicated to a girl named Skylar who had SMA type 2 and died after 7 or 8 years. A week later, after trying to hold it together, but really being an emotional wreck, we had Skylar's 2 month appointment.... The pediatrician was extremely concerned, because he felt like her muscle tone has gotten worse and said he thought it was SMA (which was the worst case scenario) and said that SMA was the first thing out of the brachial plexus specialists' mouth as well. Of course I cried, he teared up a little, and asked to pray with me. He even said he was thinking about taking Friday off to come to the neurologist with us, but wasn't sure it was possible. At our neurologist visit, our worst fears were confirmed when the doctor said he too was pretty sure it was SMA, but a genetic blood test would tell for sure. It would take 3-4 weeks for the blood test to come back...
In the meantime, we prepared for the worst and went ahead and made an appointment with the pulmonologist to establish a baseline for Skylar's lungs since SMA affects all voluntary muscles, breathing and swallowing muscles included, which is typically the cause of death (choking, pneumonia from aspiration, lung collapse, or a respiratory infection). The first bit of good news we received: Skylar's lungs were clear and looked good thus far. We also did a swallow study which showed Skylar aspirated slightly on thin fluid from a bottle, but seemed fine breast feeding. Aspiration is a huge concern because the muscles just get weaker so more and more fluid could end up in the lungs, which could be fatal.
Four weeks later, I get the call. Skylar does in fact have SMA type 1. According to statistics, she has a 10% chance of making it to her first birthday. And if so, it's even less likely that she'll see her second. SMA is caused by a missing or defective gene that is responsible for producing a specific protein that voluntary muscles use to grow. There is no cure and very little treatment currently. Because this is genetic, every child Kyle and I might have after this would have a 25% chance of having SMA, 50% chance of being a carrier, and 25% chance that the child would be unaffected. Obviously, we have some tough decisions to make as far as family planning goes, but even more tough decisions in the near future with Skylar. We've been asked to think about whether or not we would put her on life support, which no parent ever wants to be faced with that decision.
Just last week, we had an appointment with the GI doctor because Skylar started choking while breast feeding and we were looking into the option of having a feeding tube put in. The GI doctor agreed with us and said the sooner the better. She also is struggling to gain weight - one, because her muscles aren't growing due to the lack of SMN Protein so she has no muscle mass, but two, because her little muscles have to work twice as hard for normal functions and she burns through calories and isn't getting enough to gain weight. We were at Children's hospital 3 days later to get Skylar on a feeding tube through her nose to try and fatten her up a little before the surgery.
The problem with SMA is that it affects so much of the body, we have several specialists working with us instead of just one doctor. The real problem is that it is a rare disorder and not many doctors are familiar with SMA and the best "treatments" since there is no cure. Luckily, our pediatrician has been fabulous and seems to know a lot more about SMA than most. Also, our neurologist has had first hand experience with the disease - a family member of his had a child with SMA type 1. Our pediatrician mentioned some options to me that I brought up with the GI doctor while we were in the hospital the morning she was supposed to get her surgery. He seemed a little puzzled with the suggestion, and hours before she was supposed to get her IV to get ready for the G-tube placement, the GI doctor came in a told us he had a change of plans. Apparently he had talked to several other doctors about SMA and the best options for treatment during the day and heard about another SMA child in the same hospital who already had a g-tube placed, and was now getting treated for reflux. Our GI doctor knew the feeding and swallowing was a problem, but he wasn't aware how severe the reflux could be. So instead of getting the g-tube and then having another procedure a few weeks later (like the child was having to do), he said that he wanted to postpone the surgery for 3 weeks or so and do everything at once. We're continuing to pray for wisdom for each of her doctors to do the best thing and to have the most current knowledge of procedures and treatments.
SMA children suffer from reflux because of their weak muscle tone so we're going to get a procedure done called a fundoplication (Skylar is too small for a J-tube). A fundo is where they wrap the top of the stomach around the base of the esophagus so when it contracts to cause reflux, it closes of the esophagus instead or allowing things to come up. This is much more invasive and very intense for a small 4 month old baby, but we're optimistic and hope it will help her feel better and get her nutrients. Currently, she is still on the nose feeding tube at home and I am learning how to become a nurse :) I have mastered the feeding machine, use a suction machine to take care of the mucous she can't swallow, and also give her treatments through a nebulizer to help her lungs. We're also awaiting a c-pap machine delivery because she needs a little help getting some oxygen at night while she sleeps according to the results of her sleep study we just completed. It just adds pressure to her breaths to help her lungs open better - it's not like a ventilator or anything that breathes for her. Our nursery is starting to look like a medical room, but I would much rather have that than no reason for a nursery at all.
I am still hoping for and believing in a miracle. Her range of motion has gotten better, even though her muscles aren't gaining mass. She loves the bath tub and finds the water is a great place to move like a normal baby since her limbs are weightless. She smiles big and attempts to make little noises here and there. I hope that one day, Skylar will be able to grab for a toy she seems to stare at so intently. I hope that she'll be able to hold her head up, sit up on her own, talk and breathe easily, enjoy the taste of food, and eventually learn to crawl and walk. I pray that God would reach His hand down and replace the gene that is missing in her body or that the SMN protein that her muscles need to grow would be produced in her body. Of course, if God chooses not to heal her, I pray for strength and peace for Kyle and I to go forward and that God would be close to both of us as we draw near to Him and each other.
Skylar squealed with joy for the first time ever almost two weeks ago when I pulled her hands up and let them run down through my hair. I realized that babies typically pull on things like hair and jewelry and since Skylar can't, I thought she might like to try with my help... It melted my heart and made me cry with joy. Playing with my hair makes her smile every time... at least for now :) She's starting to giggle too, which is the sweetest sound I've ever heard. I ask God for more moments like this to help us get through the tough times.
We're supposed to be feeding her through the ng tube every 2.5 hours with 2.5 ounces. We just stepped it up to 3 ounces every three hours to try and keep our sanity. I've already missed two of her middle of the night feeds, but she slept through them too so I didn't feel too bad about it. We had a pretty rough scare the other night and we thought we might lose her.
I was supposed to be sleeping, but I couldn't, and Kyle had just finished a feed and was trying to get Skylar to sleep in her crib. She always has trouble breathing, but she was making unusual noises as her breathing pattern became concerning. It seemed as if she was really struggling so I got up and went in there and Kyle wasn't sure what to do. He said he didn't see her nostrils flaring badly (a sign we were told to look for) and I picked her up and unbuttoned her pjs to take a look at her ribs. The skin over her ribs was clinging slightly with her breaths, but not badly (another sign we were told to watch for). I just held her, rubbed her belly, told her to take deep breaths, and then just cried my eyes out. I am not ready to lose my little girl. I know that God loves her more than I do, and we trust Him, but it doesn't make it any less painful.
Thank you to all who have prayed for Skylar and for us thus far. We definitely feel the grace of God and are surrounded with peace through this tough time. I think it's fitting that it's the season of lent right now and we are definitely suffering (besides giving up stuff on our own). I look forward to Easter, to spring time, and a hope of all things made new and a cause for celebration. Our family has been extremely supportive and helpful and we are extremely thankful to have our parents close by. Friends and church family have been amazing as well - thank you for prayer, listening to my ramblings, meals and your support. Some of Kyle's friends are fasting every Monday, the moms group at church meets every other Thursday for prayer, and we've received cards/emails from classes, small groups, and from people all over who have heard about our struggle. My friend is already working on raising money to donate to Families of SMA and I even ran a 5k at 8 AM to help the cause. Go ahead, you can laugh. I haven't run a 5k since high school and I certainly haven't done anything active for years - needless to say, my body felt like it got ran over and beat up and I slept like a bear in the winter. Back to the point, to all of you who have helped and prayed - Thank You. We are very grateful and so humbled.
Sorry this one was so long - I am going to make sure I update this more frequently now that we've told everyone about Skylar and have a lot coming up to prevent further novels on the blog. Her 4 month check up is this week along with another GI appointment to track her progress. We'll have another pulmonologist and neurologist appointment to touch base with everyone before the surgery. Her pre-op will be late next week and surgery will be scheduled after that (I am guessing the following week if all goes well).
Hope you like the new website at www.skylarmariejones.com - I thought it would make it easier for everyone to find and check the blog whenever. Thanks for making it this far. I hope this post finds you healthy and happy. Talk to you soon. Peace.
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