Headed for a feeding tube...

We last sent out a communication to you all after having just gotten the news that Nora does fit in to a genetic category after all (at least probably), although it is a rather extremely obscure one. Since that time we have continued to try to keep up with the daily needs of our family and work while coming to terms with the implications of Nora’s designation.

It cannot be said with certainty what we can expect out of Nora’s future, though the rough idea seems to be that she’s likely to develop normal mental capacity and normal motor function, and have no particularly abnormal health problems. However, it is probably inevitable (with current medical capabilities) that she will live her life without the benefit of body fat; she will also present with various other physical anomalies including bizarre hair and possibly tooth patterns, shortish stature and very slight frame, lack of normal fingernails and toenails, and wrinkled skin. Possibly there will be more that either I’m not remembering or of which we have not yet become aware.

Implications? There is considerable relief in being able to seriously hope that we will be able to have an intellectually deep and satisfying relationship with Nora. Of course we are now more than ever before sensitized to the fact that many parents need to find fulfilling ways to interact with their children when that kind of connection is not as possible…we admire those relationships and value the contribution they make to our understanding of the human condition and experience. It is still possible that Nora’s condition holds jarring surprises for us, and we remain aware of that. But so far the expected results of this condition are being borne out each day in Nora’s development and behavior. That is to say that her mind seems to be active and curious and pleasant. Whereas her body seems to be, while not suffering in any obvious way, totally neglecting to follow the normal developmental trajectory (except that motor development is pretty well normal).

She still has not hit 7 pounds, and she is now just over 5 months old. One of the other cases of Nora’s syndrome was first seen at 18 months of age, at which time she demonstrated normal cognitive and motor development while being at a weight ordinarily expected for a child of 4 months of age. Nora seems on track to hit that same mark pretty closely.

Right now she has basically plateaued on weight for a couple of weeks, and had been growing extremely slowly before that as well. Her doctors are getting pretty uneasy with that, as are we. She seems to have insufficient eating capacity to move forward in her growth. They do not seem to think there is an imminent threat to her cognitive development, but we do need to keep her growing for many reasons related to her general health. Janelle has been bending over backward 24/7 to get milk in her (while also trying to work her normal schedule), but our best estimates indicate that she’s getting less milk by mouth than she needs available to her to reach her body’s best potential.

So it seems quite likely that we’re headed for a stomach tube. That’s a supposedly surprisingly noninvasive apparatus that allows us to bypass the oral route when we feel more milk is needed than she can take in on her own. It would especially be used during sleep times with milk being introduced over several hours (by mechanical pump) to avoid overfilling. No use pumping it in only to have her spit it up.

We feel pretty ready to accept that this is a necessary step and are even feeling like there would be some pressure taken off of Janelle to convince Nora to eat enough if we could cheat a little. But for Nora’s sake (we couldn’t “stomach” installing a tube if it was possible to get the milk to her naturally) and for the sake of caution we have purposely drug our feet some, so that we could make sure we had exhausted every possibility before resorting to the apparatus. We still have some genuine reservations. Complications from the tube are supposedly rare and mild, but we’ve got a rare girl, so it’s not as safe a bet as usual. Also, there’s a chance that it won’t help to give her more milk. She may already be taking all she can process according to her genetic potential. In the womb she showed no signs of distress related to placental function, so all the indications are that she had access to perfect nutrition then and still ended up half the size that would be expected. But it seems like we have to try. It would be too sad to know she could have grown better and we didn’t do all we could to help her.

The evaluation that will probably be the last step before scheduling a stomach tube installation will likely take place within the week. It could have happened today except for some logistical snaffoos, but it may be a good thing it didn’t, since Nora spent today getting over her first cold. We had a long night last night but she didn’t get a high fever, and the fever stage hasn’t lasted too long…we’ll see about the secretion stage. We had to spend our morning with the pediatrician so he could be satisfied that she wasn’t melting away. Kali got drug along, of course, but showed her true colors all morning; she cooperated very well and was not inappropriate or disruptive. For a high-energy 4 ½ year-old, we think that’s pretty impressive (and unexpected given her recent, understandable tendency towards attention-getting).

We don’t know if this tube is it, but our family is really ready for a change in the right direction. This has been a long row to hoe…it feels like it keeps getting longer and we can never get near the end. We remain so thankful for the kind words (not to mention food) we keep receiving from many of you. It really helps.

Love, Jason for the Myers-Benners