Tuesday Jan 5:
We initially went in for Jo's second ERT which went awesome with no complications whatsoever. After we were done with the treatment, the doctors wanted to measure how well her body expels CO2 from her body while she is sleeping. Her results weren't great but the numbers are not high enough to take any actions for breathing intervention. After a few more ERT treatments, the doctors are anticipating her numbers will improve.
Wednesday Jan 6:
After being discharged from the hospital, we went down to meet with JoJo's doctors in clinic. The Pediatrics Unit has set up an awesome clinic day so you can meet with all of the doctors in one day. You sit in one room and they all come to you! Luxury, if you ask me.
First, we met with the developmental group and assessed JoJo's physical development. We were all amazed to see her improvements from just one month and really only after one ERT. She is moving so much more and is more interested in her toys and the environment around her. She is equivalent to a 4 month old in terms of milestones, to give you a better idea.
After that assessment, we met with Jo's primary where we just spoke about normal baby stuff... poop, eating, sleeping, shots.
Next, the nutritionist came in and we started to talk about JoJo's weight gain. Jo hadn't gained in 2 months so we agreed that we had to intervene and ensure she is getting the right amount of nutrients. Metabolically, JoJo's body works a little harder than normal so any wasted or missed calories can make a huge difference. We decided that an NG feeding tube was our next step and would require us to stay in the hospital until Friday for observation.
Before heading upstairs to be admitted again, we met with the cardiologist. We took an echo cardiogram (ultrasound) of Josephine's heart. This is actually my favorite part. Modern medicine and the technology amazes me, we watched the valves and chambers all move together to pump the blood through Jo's heart. Although, the images we looked at weren't particularly normal. The wall of Jo's heart is still very thick, but we didn't expect that to change with only one treatment. The other abnormal issue is that there is some pooling of blood in her heart because of the thickness of the walls. When the walls are thick it makes the heart 'rigid'. So, when it contracts to push the blood through the chambers it isn't able to contract all the way. I think of it like comparing a water balloon to a stress ball. The walls of a water balloon are thin and pliable, you can squash the water around with ease as opposed to a stress ball where you have to really work extra hard to move the contents around. (If there are any doctors or nurses reading this and would like to correct, me please do!) Because there is pooling, there is a chance of blood clotting and then that clot being pushed into a vein which wouldn't be ideal, so just a half tablet of baby aspirin was prescribed.
Once we got up to our room, the NG tube was placed. The nurses were awesome and did the procedure quickly and accurately. Although Josephine hated it, I was absolutely intrigued! The tube goes directly down through her nose into her stomach. Currently, we do 140ml of breast milk mixed with Alimentum four times a day. Then at night, we are doing a continuous feeding of 280ml over 6 hours. We administer these feedings via feeding pump and an IV stand. This is so we can set a certain time frame for the formula to be injected. This helps with not feeding her too fast and causing her to throw-up because, remember, every little bit counts! If we need to go out, we will feed her using a syringe and be careful not to go too fast. This is a lot to get used to and is very time consuming. But, once we get a good routine down, I am sure it will get easier. Since starting this regimen, Josephine has been sleeping a lot better. When JoJo sleeps, mom and dad sleep, and that is good for everyone.
Friday Jan 8:
We were discharged and we headed home with all of our new duties. But, this was an important day because it had been a month since Josephine was diagnosed.
In college, I played soccer at The University of Alabama. We had a fitness test called the beep test. It started off slow and easy and then got progressively harder and it only stopped when you couldn't run any longer. When I would run this I would always tell myself, "One sprint at a time. Just take it one at a time." After meditating on 'one at a time', you get to the point where you can't go any longer and find yourself on level 18.2 (my personal best). I can't help but compare this to our life now. Every morning I tell myself, "One day at a time." You can get swept up in looking too far ahead into the future and it becomes too much. You psych yourself out and think, "How can I possibly do this?" But really, you can do it. You will be amazed at what your body and mind can handle. Just take it one day at a time, one sprint at a time. Because when you lift your head and look at your progress you can't help but be amazed at your own strength. This is our new normal and it is so hard but, it is also so wonderful to personally grow in ways I never would have imagined. With this new internal strength, I will share it with Josephine and I will be the best mother, friend, and role model I can possibly be!
With love and Joy,
Seguras
We initially went in for Jo's second ERT which went awesome with no complications whatsoever. After we were done with the treatment, the doctors wanted to measure how well her body expels CO2 from her body while she is sleeping. Her results weren't great but the numbers are not high enough to take any actions for breathing intervention. After a few more ERT treatments, the doctors are anticipating her numbers will improve.
Wednesday Jan 6:
After being discharged from the hospital, we went down to meet with JoJo's doctors in clinic. The Pediatrics Unit has set up an awesome clinic day so you can meet with all of the doctors in one day. You sit in one room and they all come to you! Luxury, if you ask me.
First, we met with the developmental group and assessed JoJo's physical development. We were all amazed to see her improvements from just one month and really only after one ERT. She is moving so much more and is more interested in her toys and the environment around her. She is equivalent to a 4 month old in terms of milestones, to give you a better idea.
After that assessment, we met with Jo's primary where we just spoke about normal baby stuff... poop, eating, sleeping, shots.
Next, the nutritionist came in and we started to talk about JoJo's weight gain. Jo hadn't gained in 2 months so we agreed that we had to intervene and ensure she is getting the right amount of nutrients. Metabolically, JoJo's body works a little harder than normal so any wasted or missed calories can make a huge difference. We decided that an NG feeding tube was our next step and would require us to stay in the hospital until Friday for observation.
Before heading upstairs to be admitted again, we met with the cardiologist. We took an echo cardiogram (ultrasound) of Josephine's heart. This is actually my favorite part. Modern medicine and the technology amazes me, we watched the valves and chambers all move together to pump the blood through Jo's heart. Although, the images we looked at weren't particularly normal. The wall of Jo's heart is still very thick, but we didn't expect that to change with only one treatment. The other abnormal issue is that there is some pooling of blood in her heart because of the thickness of the walls. When the walls are thick it makes the heart 'rigid'. So, when it contracts to push the blood through the chambers it isn't able to contract all the way. I think of it like comparing a water balloon to a stress ball. The walls of a water balloon are thin and pliable, you can squash the water around with ease as opposed to a stress ball where you have to really work extra hard to move the contents around. (If there are any doctors or nurses reading this and would like to correct, me please do!) Because there is pooling, there is a chance of blood clotting and then that clot being pushed into a vein which wouldn't be ideal, so just a half tablet of baby aspirin was prescribed.
Once we got up to our room, the NG tube was placed. The nurses were awesome and did the procedure quickly and accurately. Although Josephine hated it, I was absolutely intrigued! The tube goes directly down through her nose into her stomach. Currently, we do 140ml of breast milk mixed with Alimentum four times a day. Then at night, we are doing a continuous feeding of 280ml over 6 hours. We administer these feedings via feeding pump and an IV stand. This is so we can set a certain time frame for the formula to be injected. This helps with not feeding her too fast and causing her to throw-up because, remember, every little bit counts! If we need to go out, we will feed her using a syringe and be careful not to go too fast. This is a lot to get used to and is very time consuming. But, once we get a good routine down, I am sure it will get easier. Since starting this regimen, Josephine has been sleeping a lot better. When JoJo sleeps, mom and dad sleep, and that is good for everyone.
 |
| new tube! |
Friday Jan 8:
We were discharged and we headed home with all of our new duties. But, this was an important day because it had been a month since Josephine was diagnosed.
In college, I played soccer at The University of Alabama. We had a fitness test called the beep test. It started off slow and easy and then got progressively harder and it only stopped when you couldn't run any longer. When I would run this I would always tell myself, "One sprint at a time. Just take it one at a time." After meditating on 'one at a time', you get to the point where you can't go any longer and find yourself on level 18.2 (my personal best). I can't help but compare this to our life now. Every morning I tell myself, "One day at a time." You can get swept up in looking too far ahead into the future and it becomes too much. You psych yourself out and think, "How can I possibly do this?" But really, you can do it. You will be amazed at what your body and mind can handle. Just take it one day at a time, one sprint at a time. Because when you lift your head and look at your progress you can't help but be amazed at your own strength. This is our new normal and it is so hard but, it is also so wonderful to personally grow in ways I never would have imagined. With this new internal strength, I will share it with Josephine and I will be the best mother, friend, and role model I can possibly be!
 |
| always a happy girl |
With love and Joy,
Seguras
You and Tony are my heroes! You are the best mother, friend and role model possible.
ReplyDeleteAlways thinking of you and your family! Love these updates. Prayers for baby girl!
ReplyDeleteThis little girl and her mom need matching SUPER GIRL outfits!!
ReplyDeleteyou are amazing Megan! Many prayers for you all!
ReplyDeleteSending you so much love! Your so strong and amazing! Praying for you guys!
ReplyDelete