March 20, 2010
Since I have 5 minutes this morning and my little boy is resting comfortably in the hospital with Martin, I wanted to post a note so that everyone understands whats going on.
Drew and Lily were born last Sunday, March 14 at around 1:45 and then 3:30 am. They were really only born about 45 min apart, but we hit daylight savings time so his 2:30 birth time was recorded officially as
3:30. Delivery was a little traumatic but they both came out without a c-section. I ended up needing a blood transfusion, but we all survived and were doing well.
Apparently before a baby can leave the hospital they are supposed to have at least 2 dirty diapers in 24 hours. Lilly passed the test but Drew wasn't going. They gave him 48 hours because they said sometimes babies born a little early (they were 36wks) need a little extra time, but the extra time didn't help him.
On Monday night they did an x-ray of his belly and immediately transported him to Childrens NICU. Martin was able to go with him and I had to stay with Lily. They said that his intestine and bowel were distended, which meant that they were very large and irritated and clogged, and they needed to do something about it so that they didn't rupture. For the next two days they did enemas to try to flush out what they believed to be a clog. After 3 enimas, he seemed to be able to pass a little on his own, but his belly was still so hard and the x-rays weren't showing great progress so they decided to do surgery.
The procedure wasn't terribly difficult. They made a small incision in his belly and then through the muscle. Then they pulled out his intestine to examine them. Very luckily all the the intestine looked good so they did not need to cut any of it out, but about 15% of it was clogged with waste. They cut a hole in the intestine and were able to remove the blockage. They sewed the intestine back up, put it back in, and were able to seal up the cut in his belly with a little bit of surgical glue and tape, no stitches. The day after surgery they were able to take him off the ventilator and he seems to be doing great.
The next step was really just to wait and see how his intestines and bowels react to being clear. He hasn't been able to eat but they have him on an IV and some kind of electrolites to keep him hydrated. The procedures are rather remarkable as there is a line run through one of his veins up his arm, through his chest and right to above his heart to deliver the proper things. Its hard to see him like that but Childrens NICU in Cincinnati is nationally recognized and we couldn't ask for better people to be taking care of him.
We are still just waiting to see how hes reacting. He seems to be having a few dirty diapers and passing gas which are all good signs. His belly is soft again so thats also great. After things start to pass through him the right way they will add some food, very slowly, and see how his body reacts. We are hoping everything works the way it should and he can process everything normally. Its going to be a slow process, very closely monitored to make sure that he doesn't get blocked up again. They wouldn't let it get to the point of what it was, but depending on how his bowels work it could potentially reclog.
It has been such a whirlwind of running trips back and forth to the hospital, always someone by his side. Martin has been staying at the hospital most of the time because I need to be here to feed Lily, and unfortunately shes not allowed in the NICU. Both sets of our parents are here, taking care of Ella, and Lily when I do make it to the hospital to see Drew. I still can't drive so someone drives me back and forth while someone else watches Ella and another person Lily, while someone is doing laundry and all of the other basic household necessities - honestly bare minimum. We have been trying to post an update on Facebook as we can but when we do have a free minute we have been trying to get some sleep. We aren't really reachable on the phone, just because we can't use them in the NICU and haven't been at home. We appreciate everyones thoughts and prayers and concerns. So much help has been offered and we just don't even know what would be helpful. Meals would be great. It seems silly with all of this family in town but you have no idea how thin we are stretched.
We are hoping for continued improvement over the next several days/weeks but it looks like Drew will be in the hospital for at least a little while (best case scenario).As we know more we will try to post an update on here, and once life gets back to normal we hope to reconnect with everyone and introduce our two new beautiful babies.
March 22, 2010
We just has to kiss him goodbye and they took him back for surgery again. I had come in this morning at about 6 to be with Martin for rounds bc they were expecting to start feeding him today, however when they did their xray they found air in his belly which means that there is a perferation in his bowel somewhere. They said that for the first surgery everything looked okay, but they guess what happened is that after the surgery, since his lower bowel hadn't worked on its own yet, a few days of the pressure building up caused it to burst open. The doctor said that they won't know for sure what's going on until they get in there, but he thinks that's most likely what happened. He said that if that's what's wrong, he wants to do an ostomy where they will cut his bowel in half and poke it out his belly to drain into a bag. What this will do is all the bowel plenty of time to heal appropriately without having to work. They will be able to feed him too after a few days, which will let them know for sure everything is working. Once the insides are all working properly they will go back in and reconnect the bowel.We don't know how long the surgery will take, could be several hours. Definitely not the morning we were expecting but glad they found it before they started feeding him because he could have gotten very very sick, and he was awake and happy when we left him. We will let everyone know once he's out. He will be on a ventilator again overnight but are hopeful that this will fix everything.
March 31, 2010
For the few folks who haven't yet gotten the news, we had the twins, Drew and Lily, on March 14. As many of you are aware, Drew has been in the hospital the last few weeks working through the effects of Meconium Ileus – essentially, his bowels were obstructed with hard poop which he was unable to pass on his own. He had surgery two Thursday’s ago to clear the blockage; unfortunately, he required a follow-up surgery last Monday which resulted in a temporary ostomy. Testing has confirmed that the Meconium Ileus was the result of Drew having Cystic Fibrosis (CF).
When Erin was pregnant with Ella, a genetic screening showed that she was a carrier of CF – basically, one of the two genes that control CF was mutated, the other was normal meaning that she does not have CF but could pass it along. After learning that she was a carrier, I was tested and discovered that I’m also a carrier. Everyone has two of the CF genes - one from their mom and one from their dad. Each child we have therefore has a 25% chance of having CF, a 25% chance of having two “normal” genes and a 50% chance of being a carrier. Ella is a carrier. Lily does not seem to have CF but we do not yet know if she is a carrier.
Obviously, our hope was that we’d beat the odds and none of our kids would have CF. That said we certainly have been aware of the odds so were not surprised to learn of Drew’s condition. We have been getting a pretty fast education about CF and wanted to pass along some of that information to you all. Our hope is that you will take some time to understand the disease – what it is and what it is not - so that you can come to realize that Drew is going to be just like any other little boy.
As I mentioned above, CF is an inherited, genetic disease that causes certain glands in the body not to work properly. Without getting into too much detail (you can go to www.cff.org for more detailed info), his glands will make a too thick, mucusy secretion which will plug various ducts and passageways. Though there are other areas where he may be impacted, the two most typical areas of concerns are in his pancreas and in his lungs.
When working normally, the pancreas secretes enzymes which are important to the proper absorption of nutrients, particularly in breaking down fat. In most people with CF, their pancreas can’t pass those enzymes into the intestine, causing mal-absorption. The good news is that there are some really effective treatments to help here – basically, Drew will take enzymes (pill form) whenever he eats that will allow him to absorb nutrients and retain fat. The goal is for Drew to grow completely normally – gaining weight just like every other kid. When the time comes, we’ll have to discourage too much grazing…but that’s probably more something that his dad will have to learn so as to lead by example.
Really, the lungs are where the more serious complications with CF tend to occur. Again, without getting into too much detail, at some point early in his life Drew will begin to culture bacteria in his lungs. Eventually, this will lead to lung disease. Currently, the treatments for his lungs are aimed at slowing the progression of the disease by reducing the amount of bacteria built up in his lungs. While there will eventually be some medicines he will need to take, his treatments will mostly center around airway clearance techniques. Basically, we will tap on certain areas of his chest several times a day to loosen up the mucus so that he can cough it up along with some bacteria (which is basically what the rest of us do as a matter of routine). Drew will likely spend a little time each year in the hospital to treat infections that his body is not able to fight off on its own.
So, with that stuff out of the way, we wanted to let you know how the disease will affect his life. Right now Drew is recovering from his surgery. Meconium Ileus is an early sign of CF in babies that occurs because the pancreas cannot secret the appropriate enzymes to break down the waste, therefore leaving the bowel clogged. We are currently in the hospital recovering from surgery, but this is not an ongoing problem that we will be dealing with. Other than the airway clearance stuff which we’ll do two or three times a day and the enzymes he’ll have to take, you really won’t be able to tell that Drew has CF. He should physically develop like other kids, the disease will have no impact on mental development and he is encouraged to be physically active (except that SCUBA diving is apparently out of the question – damn!). At some point, he will cough more than other kids. It’s important for you to understand that his cough is part of the disease which is genetic and therefore NOT contagious. His immune system will be strong; he won’t get necessarily get sick more often than other kids, however when he does get sick he will likely get sicker. For us, this means that we won’t be living in a bubble, but we will be sure to make common sense decisions to avoid unnecessary contact with germs. There will be a lot of hand washing and some avoiding kids with obvious colds or other illnesses. Otherwise we will try to live a normal life.
So now a couple of things that CF is NOT. I’ve already mentioned, but it’s important to reiterate that CF is not contagious. The cough he will have regularly is also not contagious. He can catch a cold and give a cold like anybody else, but his typical cough is different. As we mentioned, it’s not a developmental disease – he will be just as smart and well built as his dad :). CF is not Cerebral Palsy (CP) – they are completely different diseases that some books have told us people confuse. Lastly, CF is no longer a “pediatric disease”. Advances in treatments and research mean that many people with CF live well into adulthood, have careers and families of their own.
While this was a hard pill to swallow despite knowing our odds, we’ve spent the last week getting an education on the disease and are getting comfortable with what it all means for Drew and for us and for our family. I'm sure as time goes on we will have different feelings and emotions that we will go through, but we're happy that they were able to diagnose this problem at such an early age and we are confident that we will be able to manage it so that Drew can lead a happy, healthy life.
If you have any questions, please check out the CF Foundation website - www.cff.org - or ask us; nothing is out of the question. If we don’t know the answer, we’ll ask our care team and pass along the info. Please don’t speculate – there are lots of available resources to help get good answers to any question. Please also feel free to forward along this email and/or our email addresses to anyone you wish. We are open and happy to discuss it with whomever.
Thanks to everyone for all of your thoughts and prayers during this tough time. We hope that he continues to improve everyday and that we will have our family all home and together soon!
Marty & Erin
