Lots of Good Updates

Welcome back after a two-week hiatus. Please check back for more-- I'll have pics posted soon.

Madeleine and I visited with a number of doctor's yesterday and we got some really great news. In no particular order, we learned:

- Opthamologist: Madeleine has no serious eye problems. Children with Down's Syndrome commonly have problems with lazy eyes, crossed eyes, and other more serious issues but Madeleine doesn't display any of these issues. Furthermore, she didn't have any nearsightedness and the doctors only identified small farsightedness and a slight astigmatism. Neither issue is affecting her at this time and it is fully possible that she'll grow out of it so we don't have any plans for future eye visits for TWO YEARS! I can't describe how strange it was to hear the doctor tell me that she didn't need to see us for two years. It was pretty great.
- Weight: She weighed in at 15 lbs. This was a huge breakthrough as we had been struggling to reach 14 lbs. Honestly, we're a little skeptical that the scale was accurate but regardless, we know that Madeleine is gaining weight.

- Pulmonary: Madeleine is fighting a cold right now-- she may have gotten it from her Dad-- and was needing a slightly higher level of oxygen support. This is slightly concerning and we'll have to monitor her closely for the next few days. Still, the doctors were encouraged by her weight gain and overall demeanor in the office and agreed that we can just hold the course. We are going to meet with them again in two months and if all goes well, we'll likely consider weaning her from the ventilator during the day. Typically, patients start with two hours off the vent and then increase a half hour every week. Our goal is to get her off the ventilator during the day but back on it when she is sleeping. This would make moving her around much easier but would also ensure that we could hook her up to a ventilator easily if she was in distress and/or needed additional support.

- Cardiology: Perhaps the best news of the day came from Cardiology when the doctors confirmed that Madeleine's pulmonary hypertension is unchanged and is being medically managed. Normal right side heart (pulmonary) pressures should be 25% of the left side pressures (systemic pressure) and Madeleine's pressures were at 30%. When Madeleine was diagnosed with pulmonary hypertension, her percentage was above 80% so we've come down a long ways. Given her continued development and weight gain, our cardiologist is going to slowly start weaning one of her PH drugs. Our ultimate goal as her lungs develop is to wean from all PH drugs. We don't know if this will be possible but we're encouraged now that we are at least confident enough to try weaning.

- GI: We didn't meet with a GI doctor but our cardiologist cleared us to discuss removing the nasal feeding tube and installing a direct port through her abdomen. This is great news for a number of reasons. First: the nasal tube is a pain as we have to go back to the hospital whenever Madeleine pulls it out. Second, it poses a risk as it can easily be dislodged without us knowing and could pour food into her lungs-- unlikely but still possible. The direct port sounds bad but from a lifestyle perspective it is much easier to deal with. Eventually, Madeleine will eat by mouth like anyone else but given her low muscle tone issues coupled with her compromised lungs and we're likely a year away from seriously considering mouth feedings. Since it will be that long, the best option is the direct port. The direct port requires a surgical procedure and anathesia. We've avoided this surgery for quite some time as PH patients can have very bad reactions to anasthesia. We're very encouraged that our cardiologist feels confident enough to allow this surgery as it shows how far Madeleine has come in her development. In another post, I'll talk about the different options that the GI doctors will consider when determining what type of port to install.

- Physical development/benchmarking: This is the area where I've been concerned as it is very hard to compare Madeleine against a standard set of development. Since my last post, I've gotten a number of links to various sources of information but there isn't a link for babies that have Down Syndrome, prematurity, GI problems, and Pulmonary hypertension (among other health issues). Simply put, Madeleine is one unique little kid. I was very concerned about how Madeleine has been doing and I'm ecstatic to report that my fears are largely unfounded. In contrast, the doctors were amazed at the progress that Madeleine has made in the last three months. They were glad to see her weight gain and furthermore, were glad to see how much her core body strength has improved (I'll provide another post in the near future that lists out all of Madeleine's new "tricks"). Furthermore, they charted Madeleine' s weight gain against the baby development growth charts and drew the following conclusions:
1) On the "normal" growth chart, Madeleine is still below the 3rd percentile in development but the slope of her growth line is much steeper than the normal curve. For those of you that failed high school geometry (or its been too long to remember) that means that shes catching up to the curve. Even better, when we plotted her weight on the chart adjusted for female children with Down's Syndrome, she's at to 20% percentile! This was significant for two reasons: first, it underscored to me that girl's with down syndrome are actually much smaller than traditional babies-- I didn't really grasp this until yesterday. Additionally, it means that Madeleine is catching up extremely rapidly in the Down Syndrome category. In fact, the most outrageous comment of the day came from Dr. Bloom-- our do everything/quarterback/complex care doctor-- when she suggested that our GI doctors may choose to DECREASE Madeleine's caloric intake so that she doesn't grow too fast! Needless to say, I was near to tears a number of times yesterday.

To summarize yesterday's visit: Madeleine has good eyesight, is gaining weight, her heart conditions are stable and improving, and we now have a plan in place to rid ourselves of the nasal feeding tube and to even start weaning off the ventilator during the day. Not a bad day's work, eh?

I'm getting way ahead of myself but I'm usually over-confident (much to the chagrin of my wife and mother) but I know many have asked, "how long will Madeleine be on X medical equipment?" Here's a quick synopsis/list of goals:

- Ventilator: 24 hours/day through May. The plan-- assuming two good months of health-- is to start weaning from the ventilator during the daytime. We'll likely keep this arrangement for at least another year (Madeleine would be around 2 and a half) before considering weaning further. The vent isn't a problem at night and it ensures that her lungs are supported and also allows Madeleine to grow faster by making breathing easier thereby decreasing the amount of calories she burns.

- Trach: She'll have the trach until we're certain she won't need the ventilator. This means all surgeries need to be completed, including reattachment surgery, and that Madeleine's lungs are developed enough that they won't need ventilator support. A good goal is likely around Madeleine's 3rd birthday. When we take off the ventilator, we'll put a "nose" on the ventilator-- a small cover that humidifies and filters the air that Madeleine breathes (kinda like our own nose). It looks a little strange but nearly as strange as all of her tubes.

- Colostomy bag: At this point, the decision to get rid of the colostomy bag is mostly cosmetic. If we hated the bag and complained enough, our surgeon would agree to reattach her. However, our stance is that first, it is a major surgery and we have no reason to subject Madeleine to such a surgery given her pulmonary hypertension. We do believe the PH is under control but have no reason to tempt fate with such a large surgery. Furthermore, while Madeleine is stooling fine (yes, "stool" is still my favorite word since her birth), her poop is still watery. Over time, her body should get better at extracting liquid via the small intestines. Additionally, when she begins eating solid food, we expect that the stool will thicken up. Until that time, the colostomy bag is a lot more convenient that dealing with watery, poopy diapers. We currently don't have any issues with diaper rash or skin issues associated with the colostomy bag. Eventually, we'll have to deal with these issues but again, they shouldn't be as bad when she is larger. Our current plan is to wait until she starts crawling/walking and then re-assess what to do. The goal is that around 2.5 years old, we'll want to re-attach her.

- Feeding tube: We hope to get rid of the nasal tube in the next few months and to install a direct port for feeding. I'll list out all of the options for this direct port and the decision factors that we must consider but ultimately, we hope to stop using a continuous feed pump and instead "bolus feed" large amounts of food in short timeframes. This would be great as we wouldn't have to carry around the pump and bag all the time.

Here's where I get really confident: if all goes well (yes, a very big "IF"), there could be a day in June where we pick up Madeleine and she doesn't have any cords dangling. The ventilator could be off (albeit for a small amount of time), the feeding tube would only be necessary during bolus feedings, and the rest of the monitors aren't necessary when she's healthy and looking good. That thought brings a huge smile to my face.

Okay, this post is WAAAAY to long. If you've made it this far, thanks for your interest and persistence. I'll post some great pictures from this weekend shortly and will also plan on posting more regular life postings once again. Just a quick FYI: I've been really busy working on our rental house (we just listed it for sale) so I haven't had much free time at all and blogging fell off my priority list. I'm done remodeling for a while and I plan on posting more often. That's all-- Have a great day!