Some Harsh (Potential) Realities

I wrote this "downer" post a couple of days ago. I don't want to go back and edit it, so I'm posting as-is and then will update with today's status.

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I've been blogging about Andrew's daily process for so long that I think I may have neglected the bigger picture. When we had a family conference with the doctor and social worker last week, we talked about some of the longer-term issues and timetables. I've also been doing a lot of research about the problems and recovery times that face children with diaphragmatic hernias, and I want to share some of them with you. This is not going to be a rosy picture, but I think preparing ourselves emotionally for some of the challenges he could face is a worthwhile task. If you don't want to listen to me being a downer, then skip this post.

First, let's talk about his time in the hospital. If we hear the phrase "Every baby is different" one more time, I think we may scream....but at the same time, it's absolutely true. As such, they can't really use his current progress to accurately predict when he'll be able to come home or how he'll do with the remaining tasks on his checklist. However, from what we've seen of his progress and what I've heard/read about the process in general, I think a realistic estimate would be between four and seven months (total) in the hospital. There is a (very tiny) chance that it could be sooner, and a (less tiny) chance that it could be longer. CDH (congenital diaphragmatic hernia) patients have been known to spend over a year in the hospital, especially those with cases as severe as Andrew's. His almost total lack of left-side diaphragm and his time on ECMO suggest it won't be a short stay, though he has fortunately shown no signs of any of the common defects that often go hand-in-hand with CDH. He has a healthy heart, healthy kidneys, and no chromosomal abnormalities that they know of...those all increase his likelihood of a quicker recovery. If Andrew is able to come home by Christmas, I'll be happy. If it's by my birthday, I'll be thrilled. If it's by his aunt's wedding, I'll be shocked. Unfortunately, if it's not until D's birthday, I wouldn't be completely surprised.

Going home from a case of CDH is really a checklist-style process. Here are the major points that I've come up with, though this is definitely an unofficial list:

1. Survive the first few days and stabilize for further care (may require ECMO).
2. Successful surgery to repair the hernia and reposition his digestive organs.
3. Successful removal from ECMO with no need for it to return.
4. Recovery from surgeries (CDH and ECMO).
5. Weaning from high settings on an oscillating vent to lower settings.
6. Switching from a high-frequency ventilator to a conventional ventilator.
7. Weaning to minimal settings on a conventional ventilator (standard oxygen levels, minimal machine-induced breaths)
8. Weaning from high levels of nitric oxide to lower levels, and eventually to none.
9. Extubation from the ventilator.
10. Demonstration of continued stability without advanced life support methods (ventilator).
    
11. Successful feedings of small amounts of milk through a nasogastric tube.
12. Successful feedings of larger amounts using NG tube.
13. Successful oral feeding of milk through a bottle.
14. Successful surgeries (if required) to prevent gastric reflux and/or insert a gastric tube for direct feedings.
15. Recovery from surgery and acclimation to new stomach valve and/or feeding method.
    
16. Discharge from hospital.
    

As you know, Andrew has completed the first several items on the list...six down, ten to go. Unfortunately, I've found that feeding issues often take much more time than the actual CDH recovery period. So even after he is off the ventilator, it could be months before his feedings are sorted out. Fortunately, at that point we will be able to handle him, hold him, and treat him more like a normal child than a fragile, sedated baby in a NICU bed. On the down side, all of you still won't be able to visit, and even our designated visitors can't come in during cold and flu season.

Next, let's talk even longer-term. Andrew had CDH, he was on ECMO, his lungs are underdeveloped and hypertensive, and he will have been in the NICU for quite a while before he comes home. This combination of problems lends itself to some continuing, often life-long issues and side effects. Before I get into them, let me say that ALL of these are just possibilities. It is very possible that he could have NONE of these issues. However, they are common enough that they are worth mentioning...I won't go into the far less common (sometimes more serious) possibilities. I'm also not including anything that could be caused by complications that happen along the way.

The first and most common challenge will be feeding issues. Ninety-five percent of CDH patients have some feeding challenges, and I'm not vain enough to think my child will be an exception. Not only does this prolong the actual hospital stay, it also continues after coming home. He may have a gastric tube, requiring us to feed him by injecting food directly into his stomach. He will likely always have reflux issues, meaning he'll bring food back up more than most, and may potentially need medication as well. Alternatively, he may need surgery to create a valve in his stomach (numbers 14 and 15 above).

Andrew will also probably always have lung issues of one form or another. First, we're dealing with pulmonary hypertension that makes it more difficult to oxygenate blood. His saturations will never match those of a normal child (or even adult), as his nubbin will never completely fill his chest cavity and even the right lung isn't perfectly formed. We may have to take him home with an oxygen tank, giving him additional O2 through the nose. That oxygen may or may not need to be mixed with nitric oxide....if so, that would be an additional tank. We'll probably have to give him breathing treatments, and we'll have to worry every time he decides to get mad at us by holding his breath.

Andrew will be more susceptible to additional respiratory issues than most normal babies. The likelihood of asthma, pneumonia, or RSV (upper respiratory infection) will be both higher and more dangerous for him, as he won't have the reserves necessary to maintain his oxygenation. We'll actually worry about any viruses or infections, including staph infections and other blood-based versions. Allergies are a real possibility too, though I really hope he's not allergic to Willow.

We'll certainly be cautious with Andrew, especially for the first few years, to prevent any of the above respiratory issues. He'll have to stay away from crowds, avoid anyone that's sick, and generally dread cold and flu season. When people come over to visit, a long hand wash will be a requirement...pretend you're on ER and Dr. Kovac just told you to "scrub in." We'll probably buy stock in Purell hand sanitizer.

Next up are potential developmental issues. Any baby that's in the NICU for an extended period of time will likely be significantly behind in developmental milestones. We will go back to the hospital every six months to meet with a developmental specialist, test Andrew's abilities, and discuss his progress. His normal timetable won't be the same as any other kids his age, so we shouldn't expect the standard "walk at one, talk at two" scenarios. Other less obvious milestones also won't happen on schedule, so we'll learn to develop a whole new schedule for him. Generally, kids that are behind in the first couple of years can catch up and be in-line with their ages by the time they're teenagers, or even before. We hope that Andrew's size and genetics will be a positive influence on his physical and mental abilities, and we look forward to him winning the sixth grade spelling bee.

Now it's time for the scary stuff. I feel I have to mention these only because they're somewhat common in CDH patients, especially those that have been 1) without oxygen for any period of time, and 2) on ECMO. Hearing impairment is one possibility. I've read a "success story" where a CDH kid was completely normal after recovery, aside from being deaf. Most deficits are not this severe...mild hearing loss is much more common...and again, this is only a possibility. In one less-than-scientific survey, only 23 of 461 CDH patients had any hearing loss at all. The other scary possibility is cerebral palsy. This generally refers to any motor defect caused by damage to the brain. We don't know of ANY damage to Andrew's brain and there will be additional tests (CAT scan) before he is released. If there were damage, it would likely be minor and non-progressive, meaning that it wouldn't get worse over time. If CP reared its ugly head, the worst I would expect would be leg braces, mild spasms, or something similar. In the same survey I just mentioned, 24 of 461 CDH patients had some form of cerebral palsy.

Sorry to be such a downer, and I realize this post was FAR too long and perhaps too sad, but I wanted to get it out there. Probably the most important part is realizing that he'll be in the hospital for a while...I don't want anyone to expect a huge party in August/September. First, he's not supposed to be around groups. Second, I think a late autumn discharge is definitely more realistic.