Thursday, September 30, 2010
Tuesday, September 28, 2010
Friday, September 24, 2010
Thursday, September 23, 2010
Wednesday, September 22, 2010
Monday, September 20, 2010
Thursday, September 16, 2010
Malabsorption of essential nutrients is characterized in CF by poor weight gain, growth retardation, delayed puberty, and muscle wasting. Patients with CF need to be frequently monitored because nutritional needs change with disease progression and normal growth. To maintain a desirable body weight and prevent or correct nutritional deficiencies, CF patients might have to consume twice as many calories as recommended for healthy children and adults. Increasing the intake of calories can also aid in adequate fat, vitamin, and mineral absorption.
Nutritional therapy helps prevent complications, such as glucose intolerance, intestinal obstruction, cirrhosis, and pancreatic disease. Vitamin and mineral supplements such as the fat-soluble vitamins A, D, E, and K are often used. Since these vitamins use dietary fat is necessary for absorption of these vitamins, high-fat food consumption is often encouraged. Fat is also the most concentrated source of calories and improves the palatability of foods, and with caloric needs so high in CF patients, this becomes essential. Protein deficiencies are most likely to occur during periods of growth and infection.
Tuesday, September 14, 2010
Monday, September 13, 2010
I remember taking someone to the hospital for something (Lily for her staph infection at 1 week old, Ella for a broken leg, Drew for some fit of breathing trouble, you know, one of those times) and one of the medical personnel telling me that she had 18mo old twins, a boy and a girl, like I did. I remember asking her if she liked them yet.
The past 6 months have been full of many emotions. Exhaustion, if thats an emotion, is at the top of that list. Confusion is up there too, trying to remember who ate what and when they ate it. I've been forced to become organized lest someone go without dinner. So so very much love. I didn't think I had anymore love to give once I had Ella, but apparently love grows. Stress. Happiness. Sadness. Worry. Elation. Pride.
As each day passes and I long to keep them frozen in time, I like them more and more. I can't say that its fun exactly, but I wouldn't trade it for the world.
Thursday, September 9, 2010
When Drew had his surgery on Tuesday, they did a culture of the mucus they found in his lungs and it was positive for staph. They decided that trying to eradicate all of the bacteria was the best thing we could do for it, essentially give him a fresh start. They decided that on Friday morning he would get a PICC line (a special IV) that would be used to administer the antibiotics for the 2 week course. On Thursday morning, the doctors came around for rounds and they said that overnight they were able to test the bacteria's sensitivity to a number of antibiotics and that it was not resistant to anything, therefore we could treat this orally and it would be just as affective. They want to save the really strong IV antibiotics for when they really need them. And just like that, they signed the discharge paperwork and sent us on our way! (okay well really it took a couple of hours like it always does, but you get the point).
The antibiotics need to be administered every 8 hours around the clock, pretty strictly. In about 3 weeks we will go back to the hospital for an outpatient bronchoscopy so that they can get another mucus sample and make sure that the bacteria is gone, along with the majority of the mucus. They will also take a look to see how the trachea looks a few weeks post surgery. It had opened up significantly immediately after the surgery, but they are hoping that over time as his cartilage strengthens it will grow to an even better shape.
You seriously would not even know that this kid had surgery just 4 days ago. He's happy as can be and sounds like a new person. I was looking at some pictures that I had taken just the day before the surgery and poor Drew looked so sick. His face was all red and puffy and his eyes tired from the incessant coughing day and night. Since the surgery he sounds better, looks better and is acting better. He is like a new person! I'm so very happy at what a huge success this procedure was. It was a scary one. They're all scary, but this one dealt with two major parts of the body, his trachea and his heart. I'm just so very happy that it all went the way it did and more importantly the outcome. Just so elated!
Here's what else we have going on. Ella got her cast off about a week ago and is starting to get back to walking around like she use to. She kind of looks like an old man, limping around, but missing her walker. Every day it gets better and we realize how nice it was to have her contained by a non-weight bearing cast for a few weeks :) She started preschool last week as well and just loved it. She has such an outgoing and friendly personality and she just loved meeting and playing with the other kids, and talks all about the teachers and everything she learned. I'm happy that we decided to send her this year. Its good for her to get out of the house and have some
stimulation and interaction with other kids her age two mornings a week, and its nice for me to have 2 hours of time with just the twins. While I'm still a bit of a nervous wreck about the germs shes inevitably going to bring home, I'm glad that we stuck with our decision and sent her.
Lily is a rolling fool. You can't take your eyes off of her for 2 seconds. She rolled all the way across the porch this afternoon back to the doorway inside. Her demeanor has improved dramatically, and she is no longer the screaming demon for most hours of the day. Shes got a laugh that could make you smile on your very worst day and her eyes truly twinkle when she grins her huge Lily grin. I think shes gonna be a mama's girl. I'm okay with that :)
We got a new computer. The jerk who "fixed" our sound broke our network card so our old computer was rendered useless. This is my first time with the new machine. So far I'm pleased.
My dad has been here helping us out for the past week while Drew was in the hospital. He leaves tomorrow and its back to just me and the three. Its definitely nice when we have an extra set of hands in the house, just to play with a kid, feed someone a bottle, fold a load of laundry, or any of the other things that go on here in a day.
I'm looking forward to the fall and hopefully finding our grove...finally. I hope that this is the end of the nonsense with Drew for a while. It sure seems like this last week may have changed his life for the better. God willing, with all of the new treatments and therapies out there for CF, he can lead a healthy and happy life just like his sisters.
Wednesday, September 8, 2010
Drew's doctors consider the surgery a huge success!! They were able to lift the artery off of his trachea and allow it to open it a normal size. Its no longer compressed flat. While its still a little floppy from being compressed for so long, its definitely a big round opening now, like its supposed to be. We could instantly tell a difference in the way he sounded and hope that this solves many of his problems. He still has the CF, unfortunately theres no cure for that (yet), but hopefully with a normal sized and shaped trachea he will be able to clear his airways affectively and breathe a little easier.
While they were doing this procedure, they also did another bronchoscopy to look into his airways with a camera to see if the shape changed (it did, dramatically, for the better...incase you didn't understand the previous paragraph :) ). Unfortunately they also found a lot of mucus. A lot of thick, sticky, CF mucus that had been trapped down there. And there's bacteria in it. Its not terrible, none of the really bad CF infections were found, but there was some staph and they want to treat it. They want this to be a total cleansing and clearing new start for Drew, so they have decided to put him on a 2 week IV antibiotic treatment to try to eradicate all of the bacteria and mucus and give him a chance to start new. Tomorrow morning he will get a PICC line (Peripherally Inserted Central Catheter) and thats how they will administer the antibiotic (its basically a fancy IV). We will talk to the doctors in the morning about whether or not we can take him home and have Home Care nurses come so that we can administer this from home rather than having to spend another 2 weeks in the joint. Its looking like we should be able to but will have to take him back toward the end of the 2 weeks so that they can do another bronchoscopy and take out the PICC line. But bringing him home for at least a week would definitely be the preferable option, even if it means being house bound until the antibiotics are done. I guess we'll see.
I'm so happy that we did the surgery. I was nervous and less than optimistic about how effective it would be, but I can see and hear a difference and the hope is that he will just continue to improve. There is still a lot of inflammation and soreness in his airways from all of the coughing and labored breathing for the past several months, but things finally seem to be getting better, and with the antibiotics hopefully there will be even more improvement.
Our computer is back in the shop so I'm just using shared ones to post an update here and there. I'll try to give more updates complete with pictures and descriptions as I get them. Thanks so much for all of the thoughts and prayers - they have and will continue to help Drew and us get through all of this!
Tuesday, September 7, 2010
Monday, September 6, 2010
I'm sure tomorrow will go fine. Its never easy giving your baby to a stranger and kissing him "see you later". The hand off is scheduled for 7:30. They said that it will probably be about an hour before the actual surgery starts as they have to knock him out, get 2 IV's run (a regular one and one in an artery to monitor blood pressure very closely since they are working so closely with his heart), place an epidural so that his entire mid-section is numb, and put him on a ventilator. Once all of that is done they will do the procedure which they estimate will take 1-2 hours. Then, depending on where they take him, either to recovery before he goes to the floor, or to ICU if they want to monitor him a little more closely because of the breathing issues that have been going on, we should be able to see him within an hour of completion.
I'll try to post a quick note on here at some point tomorrow but its going to be a crazy day. I'll definitely update Facebook when hes out. Please send all happy thoughts and prayers and all other good wishes our way tomorrow that this is a success. Its about time something starts going Drew's way!
Friday, September 3, 2010
Drew has had noisy breathing and a cough for the past several months now. Through a bronchoscopy (camera down his throat) they were able to take a look at his trachea (windpipe) and saw that something wasn't quite right. There were two major issues. The first is the compression they see right where the aorta crosses the trachea. The second is sort of "broken" or misformed cartilage causing the trachea to look funny. I'll touch more on the second issue a little later, but right now I'll try to explain the first. Keep in mind that I'm not a doctor, so if you're reading this and you are a doctor don't criticize.
In your chest behind your breast bone, there is more than just your heart. Your trachea is back there, some arteries, your thymus gland, your esophagus and that’s all I can remember. Anyway, it’s crowded between your breast bone and your spine is my point. Your trachea and my trachea are hard as bone. Babies’ tracheas are soft as butter. Many kids suffer from something called Malacia, which is just some floppiness of the trachea. While they are still little, their already soft cartilage is even softer than normal so when they breathe, rather than the trachea expanding and contracting normally, it doesn’t keep its form and causes rubs against itself a little bit, causing a noisy sound, like you would hear if you had a frog in your throat. Drew has a little bit of this floppiness going on, but additionally, one of the arteries that is back behind the breast bone seems to be pressing on the trachea a little bit. It’s not terribly uncommon, and because of how soft the trachea is at this age still, anything that’s pressing on the trachea will cause some level of compression.
The aorta, one of the main arteries to the heart, crosses over the trachea in all of us. In babies, because of the “crowding” behind the breast bone, sometimes where this crossing takes place there is some level of compression. This is what’s going on with Drew, and here’s why it needs to be fixed. Let me first say that this is not a CF issue. This is an anatomical abnormality that is complicated by the CF, but is not a CF issue. The compression that is being caused by this artery does not allow for proper airway clearance. Drew just coughs and coughs and coughs because this compression is causing sort of a tickle in his throat, and then all of the coughing is creating irritation which is causing more coughing, but when he coughs the trachea just essentially closes for a second and he struggles to breathe. And on and on the cycle goes. So what needs to happen is to move that artery off of the trachea to allow it to remain open the way it’s supposed to be, and the way they do this is with a procedure called an aorta pexi.
On Tuesday, September 7 Drew will have this procedure, the aorta pexi, done. They will make an incision on his left side (the aorta is on the left side of the heart) kind of under his arm and spread his ribs cage to get in there. They will cut out a part of his thalamus so that they can get to his heart. This shouldn’t be a problem because studies have shown that the thalamus’ job is mostly done prior to birth, that it really has no effectiveness once you are born. That said, he will not lose his whole thymus so any unknown purpose of the thymus will be preserved. They will cut a flap in the sack around his heart (his pericardium) and then sew that to the inside of his breast bone. By doing this, they will lift the value off of the trachea and open it up. If you are curious about the order of things in your chest, it goes (front to back) your breast bone, thymus, your heart (arteries and all), your trachea, your esophagus, your spine.
What we know for sure is that by removing part of the thymus and moving the artery closer to the breast bone it will allow a little more space for the trachea and will relieve the pressure of the aorta on the trachea. What we don’t know for sure is how this will help and we won’t know until after the surgery. The reason that we just don’t know is because of problem #2, the broken cartilage. This is the issue that no one has ever seen before and we really don’t know how it’s affecting him, and won’t know until we get problem #1 fixed.
If you take your hands and make a C with each hand and put them together to make a circle, that’s what the cartilage that supports your trachea looks like. Its one nice, big, round piece of cartilage. Now looking at that circle you made with your hands, take one hand and slide the fingers down and in under the other fingers. You make an off shape circle that sort of closes in on itself. This is what’s happening with Drew’s trachea below where the artery is compressing. Its never been seen before and its unclear as to what kind of problem it is. It may not be anything anyones ever seen before, but if its not causing a problem, who cares what it looks like. We will see how this upcoming procedure affects him. With any luck, it will help to open up his trachea so that he can breathe and cough and clear his airways just like you or I, and then our problem will be solved. After this procedure we will just wait. We will wait and see what difference this makes. We will wait months to see if that broken cartilage fixes itself, if he sort of grows out of it, of if its going to be the real problem that needs to be fixed. I hope and pray to God that it doesn’t because the thinking is that he would need tracheal reconstruction which is a major major operation, not done pretty much anywhere else in the country other than Cincinnati, but its not something that we want to have to have done.
So we’ll see what happens on Tuesday. It won’t make things worse. It’s a big deal but its what has to happen so we’re going with it. Keep us all in your prayers for a successful procedure Tuesday and future free of trips to the OR.
Thursday, September 2, 2010
Naturally, the appointment got scheduled at exactly the same time Ella was scheduled to get her cast off. We were able to get a later appointment for Ella so that everyone's needs are met. I think we will probably be scheduling a surgery before we leave there tomorrow.
Lily finally sleeps past 5am. The first day she did it we had to wake her up to go to Drew's doc appointment. The past 2 days she got up at 7:30. That would be awesome if Ella hadn't start waking up at 6am.
In other baby-related news, making my own babyfood has thus far proven to be reasonably easy. The babies only eat about 1 cube a day right now so I don't have to have a ton on hand. I've made squash, sweet potato, green beans, carrots and peaches. They've only had sweet potato and squash but seem to take them alright. After I go grocery shopping I'm going to try making some more fruits. Its not hard to do and its really not terribly time consuming.
Ill try to post an update on Drew after tomorrow's appointment. Cross your fingers that Ella's leg is healed and we don't need another cast!