The title of my blog is My AGS Son and Me, not because I always refer to my son as an alagille syndrome kid. It's so when another AGS parent goes to google their child's newly found disease, they will find me and be able to read my story and relate. You have no idea how much knowing that you aren't alone in this helps.
Now more about my son, he obviously has a disease called Alagille Syndrome. It affects his heart, kidneys, and liver mostly. He has stenosis of the aortic, mitral and pulmonary valves in heart. Which means those valves are extremely narrow, causing extreme amounts of pressure and obstruction. Which is very serious and they don't even know if they can do anything about it. His liver is HUGE and is full of billiruben, which makes his skin very yellow and his poop very dark. He has little to no bile ducts so the bile can not pass into the poop as well and fast as it should. He has a hypoplastic kidney and bladder reflux, so his pee splashes into his kidney which is small, so he is on an anti-biotic at all times to prevent infection. He is on countless medications. Actigall, By-cytra, Phenabarbatol, a multi-vitamin, Vitamin D, Iron, Atenolol, an anti-biotic, and right now he is on a temporary laxative and of course medication to make him asleep all the time because of the intubation.
When I was pregnant with him, on the ultrasound, they saw a few of these things but failed to tell me until the day I delivered. Yeah, I was pissed. My delivery was very hard on me. It was unorganized and rushed. Thanks to my nightmare of an OB/GYN. Just a few hours after Maddox was delivered, the doctor came in and said they wanted to do an ultrasound on his kidney because it had been smaller in the ultrasound while he was in utero. I thought nothing of it because I and everyone in my family have always had perfect health and I guess I just took that for granted. I figured he'd be fine because nothing like that ever happens to us. They came back in and said he was going to be taken to Rockford because of his kidney, liver, and heart. All I could to was cry and cry. I had no idea anything was going to be wrong with him. It never crossed my mind. Luckily my doctor discharged me from the hospital just hours after giving birth so that I could drive to Rockford and be with my newborn son.
After getting the news that my son had problems, I immediately started blaming myself for smoking that one cigarette in my second trimester, or soaking in the hot tub for 5 minutes when I was 14 weeks pregnant. Little did I know, all these problems were possibly genetic. GENETIC. (We now know it was not genetic, but this what written when we thought it was) That word sunk deep in my mind. No one in mine or my son's father's family have any problems like this. and to think it could be MY fault. MY genes that is making my son suffer makes me sick. I would have never brought him here and made him suffer if I would of known I had bad genes. I would have been more careful. We still don't know if it is me or his dad Ron's genes. But when we find out, we won't be having anymore children. If its me, I will get my tubes tied, and vice verse. I would NEVER put another child through this...
So we left KSB hospital, the hospital I delivered at, to go to Rockford hospital to meet my son there. I could barely walk, but I was eager to see my brand new son. We got there and he was in a little plastic bassinet. He was only hooked up to the monitor at this time. He didn't need oxygen or anything. He was doing great on his own. The only problem he had was eating. He couldn't drink from a bottle and I had yet to produce milk. So he had an ng tube in for a while, and then my milk came in after a week. I was SOO excited. Breastfeeding just seemed so natural and motherly. It was all I wanted and I tried damn hard to get that milk to come in! I would pump for 45 minutes at a time with just DROPS coming out. But it payed off. At first he could not latch because my breasts were so full and big. But after a few days they went down, and he latched PERFECTLY. Every time I breastfed from that point on, it melted my heart. I was feeding MY baby from a milk made just for him inside ME. I miss it so badly. Right now he can not be breastfed obviously because he is in a drug induced coma. He can't even get my pumped breast milk because it is too much work for his gut to digest. But hopefully he will be well enough to breast feed again. So I am only pumping twice a day now. I miss breastfeeding SO bad. When I am alone and just get to thinking, I start thinking and imagining the sensation of breastfeeding. His little suckling. It sometimes tickled but I love the feeling. It didn't hurt. It felt natural. It gave us a connection again like we were when he was inside me. I miss it. Extremely bad. It makes me feel like my milk is worthless because he can't have it. Today I asked the nurse for more bottle to store my milk in, and she said, "you might want to ask if he will ever even be able to have breast milk again because if he can't, you might as well dry up and that might make you feel better." ARE YOU KIDDING ME!
Anyway, after a few days in Rockford, Max started having apnea spells. The scariest thing in the world. Apnea spells are where he stops breathing and turns grey and you have to either stimulate him by rubbing his back and lifting him up or by giving him oxygen. It got to the point where he was having them every 10 minutes. It's the worst feeling in the world. I was constantly on my toes, watching his stats on the monitor. If they even dropped to 90 I was up and already rubbing him. He had those for a week and then one day he didn't get one, and then another day passed without one, and then all of a sudden they disappeared. THANK GOD. The doctor said it was just a phase. But we went hope on an apnea monitor just to be safe, after four weeks at RMH. Going home was AWESOME. I loved being home with my baby, alone, doing what I want with him, without anyone watching or nurses coming to do rounds on him every couple of hours. He was ALL mine and I LOVED taking care of him. I didn't mind getting up in the middle of the night. It made me feel like he needed me, and I wanted him to need me so I could be there for him. I loved being in our own home, knowing I was his only care-giver and that he was doing just fine in my care only. We were taught how to put down the ng tube and did that every night for meds. It made me feel just as good and smart as the nurses.
After a few amazing weeks at home, Max started to have trouble breathing. I would count his respiratory rate to be 80 and you could see his chest contract and his entire diaphragm move. I took him to the hospital, thinking it was just constipation pressing up on his chest and that he would be fine. Little did I know, his condition had worsened. His weak heart could barely do its job. He needed to go back to Rockford. Where he did good for a week and then back to the respiratory distress. So they sent us to Hope Children's Hospital in Oak Lawn, Illinois, two hours from home. The night we got here we got an awesome nurse and so I was happy with that. He was doing okay on the oxygen and seemed fine. Then the next day, the most horrible thing of all happened. I was just going to go play with him, when all of a sudden his heart rate went from 100 to 70 so fast, and kept going lower and lower and lower. The alarms sounded loudly and one of the top cardiologists happened to be by his room and came rushing in, yelling for help. He immediately started doing compressions and giving him oxygen. They called code blue on him and everyone came rushing in frantically. I automatically assumed he was dying when I saw what they were doing to him. I ran out and started bawling and panicking. I kept asking them why is this happening and they didn't know at first. His heart was failing and I didn't know if he was gonna make it. I sat in a small, enclosed room, where the Chaplin was called to stay with me since none of my family was here at the time. I surely thought he was dead and I could not come to terms with it. Finally the doctor came in and told me he was in critical condition but stable for the moment being. They told me had had a tube in his mouth that helped him breath. I didn't want to go in there right away. I was afraid of seeing him like that. They had put a large amount of drugs into his system really fast so they could intubate him so he looked very out of it, limp and lifeless. When I finally decided to come and see him, they were lifting him up to change his sheets. His eyes were glazed over, his pupils were dilated, and his body was limp. He didn't look alive and it scared me so I immediately left the room bawling. I came back and finally got used to seeing him like this. His eyes look normal now and he is more alert. He will be asleep most of the day but open his eyes for ten minute periods all through out the day. He has not coded since that time last week thank God. He was blessed by the Mormon missionaries and many people are saying prayers for him. Whether they will do good I don't know. I just want my little boy back. The cardiologists always seem very concerned. They always use very blunt terms with us and it can be overwhelming. For example today, one of the best cardiac surgeons in the world said to me, "we don't want to perform such a big surgery on his heart, if he is only going to live for three months to do his liver." Just as an example. I'm sure he meant that as the worst case, but still. Anyway, that pretty much sums up our story until now. So I will post more later. There are so many more things I would like to cover. But I guess this is all for now.
Now more about my son, he obviously has a disease called Alagille Syndrome. It affects his heart, kidneys, and liver mostly. He has stenosis of the aortic, mitral and pulmonary valves in heart. Which means those valves are extremely narrow, causing extreme amounts of pressure and obstruction. Which is very serious and they don't even know if they can do anything about it. His liver is HUGE and is full of billiruben, which makes his skin very yellow and his poop very dark. He has little to no bile ducts so the bile can not pass into the poop as well and fast as it should. He has a hypoplastic kidney and bladder reflux, so his pee splashes into his kidney which is small, so he is on an anti-biotic at all times to prevent infection. He is on countless medications. Actigall, By-cytra, Phenabarbatol, a multi-vitamin, Vitamin D, Iron, Atenolol, an anti-biotic, and right now he is on a temporary laxative and of course medication to make him asleep all the time because of the intubation.
When I was pregnant with him, on the ultrasound, they saw a few of these things but failed to tell me until the day I delivered. Yeah, I was pissed. My delivery was very hard on me. It was unorganized and rushed. Thanks to my nightmare of an OB/GYN. Just a few hours after Maddox was delivered, the doctor came in and said they wanted to do an ultrasound on his kidney because it had been smaller in the ultrasound while he was in utero. I thought nothing of it because I and everyone in my family have always had perfect health and I guess I just took that for granted. I figured he'd be fine because nothing like that ever happens to us. They came back in and said he was going to be taken to Rockford because of his kidney, liver, and heart. All I could to was cry and cry. I had no idea anything was going to be wrong with him. It never crossed my mind. Luckily my doctor discharged me from the hospital just hours after giving birth so that I could drive to Rockford and be with my newborn son.
After getting the news that my son had problems, I immediately started blaming myself for smoking that one cigarette in my second trimester, or soaking in the hot tub for 5 minutes when I was 14 weeks pregnant. Little did I know, all these problems were possibly genetic. GENETIC. (We now know it was not genetic, but this what written when we thought it was) That word sunk deep in my mind. No one in mine or my son's father's family have any problems like this. and to think it could be MY fault. MY genes that is making my son suffer makes me sick. I would have never brought him here and made him suffer if I would of known I had bad genes. I would have been more careful. We still don't know if it is me or his dad Ron's genes. But when we find out, we won't be having anymore children. If its me, I will get my tubes tied, and vice verse. I would NEVER put another child through this...
So we left KSB hospital, the hospital I delivered at, to go to Rockford hospital to meet my son there. I could barely walk, but I was eager to see my brand new son. We got there and he was in a little plastic bassinet. He was only hooked up to the monitor at this time. He didn't need oxygen or anything. He was doing great on his own. The only problem he had was eating. He couldn't drink from a bottle and I had yet to produce milk. So he had an ng tube in for a while, and then my milk came in after a week. I was SOO excited. Breastfeeding just seemed so natural and motherly. It was all I wanted and I tried damn hard to get that milk to come in! I would pump for 45 minutes at a time with just DROPS coming out. But it payed off. At first he could not latch because my breasts were so full and big. But after a few days they went down, and he latched PERFECTLY. Every time I breastfed from that point on, it melted my heart. I was feeding MY baby from a milk made just for him inside ME. I miss it so badly. Right now he can not be breastfed obviously because he is in a drug induced coma. He can't even get my pumped breast milk because it is too much work for his gut to digest. But hopefully he will be well enough to breast feed again. So I am only pumping twice a day now. I miss breastfeeding SO bad. When I am alone and just get to thinking, I start thinking and imagining the sensation of breastfeeding. His little suckling. It sometimes tickled but I love the feeling. It didn't hurt. It felt natural. It gave us a connection again like we were when he was inside me. I miss it. Extremely bad. It makes me feel like my milk is worthless because he can't have it. Today I asked the nurse for more bottle to store my milk in, and she said, "you might want to ask if he will ever even be able to have breast milk again because if he can't, you might as well dry up and that might make you feel better." ARE YOU KIDDING ME!
Anyway, after a few days in Rockford, Max started having apnea spells. The scariest thing in the world. Apnea spells are where he stops breathing and turns grey and you have to either stimulate him by rubbing his back and lifting him up or by giving him oxygen. It got to the point where he was having them every 10 minutes. It's the worst feeling in the world. I was constantly on my toes, watching his stats on the monitor. If they even dropped to 90 I was up and already rubbing him. He had those for a week and then one day he didn't get one, and then another day passed without one, and then all of a sudden they disappeared. THANK GOD. The doctor said it was just a phase. But we went hope on an apnea monitor just to be safe, after four weeks at RMH. Going home was AWESOME. I loved being home with my baby, alone, doing what I want with him, without anyone watching or nurses coming to do rounds on him every couple of hours. He was ALL mine and I LOVED taking care of him. I didn't mind getting up in the middle of the night. It made me feel like he needed me, and I wanted him to need me so I could be there for him. I loved being in our own home, knowing I was his only care-giver and that he was doing just fine in my care only. We were taught how to put down the ng tube and did that every night for meds. It made me feel just as good and smart as the nurses.
After a few amazing weeks at home, Max started to have trouble breathing. I would count his respiratory rate to be 80 and you could see his chest contract and his entire diaphragm move. I took him to the hospital, thinking it was just constipation pressing up on his chest and that he would be fine. Little did I know, his condition had worsened. His weak heart could barely do its job. He needed to go back to Rockford. Where he did good for a week and then back to the respiratory distress. So they sent us to Hope Children's Hospital in Oak Lawn, Illinois, two hours from home. The night we got here we got an awesome nurse and so I was happy with that. He was doing okay on the oxygen and seemed fine. Then the next day, the most horrible thing of all happened. I was just going to go play with him, when all of a sudden his heart rate went from 100 to 70 so fast, and kept going lower and lower and lower. The alarms sounded loudly and one of the top cardiologists happened to be by his room and came rushing in, yelling for help. He immediately started doing compressions and giving him oxygen. They called code blue on him and everyone came rushing in frantically. I automatically assumed he was dying when I saw what they were doing to him. I ran out and started bawling and panicking. I kept asking them why is this happening and they didn't know at first. His heart was failing and I didn't know if he was gonna make it. I sat in a small, enclosed room, where the Chaplin was called to stay with me since none of my family was here at the time. I surely thought he was dead and I could not come to terms with it. Finally the doctor came in and told me he was in critical condition but stable for the moment being. They told me had had a tube in his mouth that helped him breath. I didn't want to go in there right away. I was afraid of seeing him like that. They had put a large amount of drugs into his system really fast so they could intubate him so he looked very out of it, limp and lifeless. When I finally decided to come and see him, they were lifting him up to change his sheets. His eyes were glazed over, his pupils were dilated, and his body was limp. He didn't look alive and it scared me so I immediately left the room bawling. I came back and finally got used to seeing him like this. His eyes look normal now and he is more alert. He will be asleep most of the day but open his eyes for ten minute periods all through out the day. He has not coded since that time last week thank God. He was blessed by the Mormon missionaries and many people are saying prayers for him. Whether they will do good I don't know. I just want my little boy back. The cardiologists always seem very concerned. They always use very blunt terms with us and it can be overwhelming. For example today, one of the best cardiac surgeons in the world said to me, "we don't want to perform such a big surgery on his heart, if he is only going to live for three months to do his liver." Just as an example. I'm sure he meant that as the worst case, but still. Anyway, that pretty much sums up our story until now. So I will post more later. There are so many more things I would like to cover. But I guess this is all for now.
Sounds like my journey with my son. My son is now 8 years old. His liver is affected (takes actigal/ Ursodiol) and he has branch pulmonary stenosis. His genetic mutation also caused Pierre Robin Sequence and hyper Sticklers Syndrome.
ReplyDeleteYou are not alone! I will keep your little Maddox and YOU in my prayers... It's a long road... but so worth it!
Hang in there sweetie!
Hi Brooke, I'm Christa, mother of Arielle, 21 months old w/AGS. Joe posted your blog on Facebook, that's how I found it.
ReplyDeleteAGS can vary in very different ways. It's genetic, but that does not necessarily mean that's in your gene too! In 60% of AGS 'cases', neither of the parents carry the gene, but it got spontaneously altered. A chance of 1 in 100.000, our doctors said.
So don't be hard on yourself; you did not know, and you could not do anything to prevent it from happening. It was out of your hands.
What's in your hands is how you take care of your son. And you're doing a terrific job! You're there for him, you're breastfeeding/pumping! I'm completely w/you on the fact that in a situation in which you can do so little, making milk for your baby feels like doing something worthwhile! It felt the same way for me!
I've breastfed Arielle for 18 months, and only stopped because I got pregnant again. I've pumped, i've "creamed" my milk, to make it more nutritious etc. So if you have ANY questions on breastfeeding, pumping etc etc, please ask and I'll be happy to help you where I can!
You're a strong woman and a world class mom! Never forget or doubt that!
Wishing you all the best in these times and I'll pray for you and your son too... And remember you're not alone in this!
Sincerely,
Christa Wiersma,
The Netherlands