Laughing, Weeping, Living

Life happens. You laugh about it or cry about it, sometimes both.

Agnes Makes Progress

on July 9, 2013

Agnes with a Broviac I.V. in her head, and the Vapotherm nasal cannula.

The past five or six days have been very eventful for Agnes. She got off the ventilator and on to Vapotherm, a C-PAP like machine that supplies air pressure and oxygen as the baby requires. She started out on Friday at 6 LPM of pressure, and this morning she was down to 2 LPM. She was struggling a little bit when I saw her this morning, but they had cranked her down only an hour prior to my visit and she was still getting used to the new settings. I think she’ll be doing better by the time I go back to visit later today. The hope is to get her off all breathing assistance by the end of the week. I think she’ll make it, but only Agnes knows the truth! We’ll see how she handles each change in her treatment.

The other major change in Agnes’ care is her feeding is increasing pretty quickly. She started out on all I.V. nutrition while she was recovering from surgeries, and they finally introduced milk on a continuous drip through a feeding tube early last week. She is up to 11 ml/hr of milk, which means the I.V. fluid is reduced to 5 ml/hr. I think the goal is to get the milk up to 15 or 16 ml/hr so they can discontinue the I.V. altogether. That should also be accomplished by the end of the week. Once Agnes is up to the full amount of milk, they will start phasing her away from a continuous drip and more towards a feeding schedule. They will give 3 hours worth of milk over a period of 2 hours, with a 1 hour break. If that goes well, they will shorten the amount of time it takes to dispense 3 hours worth of milk, until Agnes is getting an entire feeding in a very brief amount of time.

When Agnes is finally free of all breathing assistance, she can start trying to feed by mouth. We will try her at the breast, and also see if she will take a bottle or suck on a pacifier. This is the part that is totally unknown right now. She has tongue tie and the recessive jaw, both of which may hamper her ability to suck effectively. Personally, I’m not at all sure she will be able to nurse at the breast, but I am confident she will be able to drink from a bottle. The alternative is she will be sent home with a feeding tube, either the kind that threads through the nose and down the esophagus, or the surgically installed kind that goes directly to the stomach. I really really hope that is not necessary!

Agnes is still gaining weight; she weighs almost 5 1/2 pounds now. She is maintaining her body temperature well, so they are moving her out of the isolette today and putting her in a crib! She can even start wearing shirts!

Yesterday Agnes got some cute little hand splints. These will help train her wrists and fingers into a more relaxed, straight position. Right now her wrists curl in toward her forearms, and her fingers are like little claws. She will open them if you work with her hands, but the splints will help get them into a straight resting position so you don’t have to work at it. The therapist said it’s good that Agnes can open her fingers enough to grasp something–she does have movement with her hands and fingers. Agnes will be able to open her hands with therapy, but it will probably take a few years with splints and stretching exercises.

The doctor told us yesterday that she did some more research about Agnes’ genetic condition, and she couldn’t find any cases in the literature where the children lived longer than 11 years. She was quick to say that this doesn’t necessarily mean Agnes will not live past 11, just that no cases where the kids lived longer were ever written about and published. The doctor also said people are living longer and longer with conditions that used to have early mortality, so we shouldn’t be discouraged. It’s hard not to be discouraged when the doctor tells you your kid will probably die as a teen or sooner, and I’m still not quite to the point where I can accept that news. The truth is Agnes has a super rare anomaly with her chromosomes, and no one is quite sure what it will mean for her long term. I’ll go ahead and tell you what it is. I hesitated before because genetics are kind of personal, but Agnes has the opportunity to educate others and help shed light on a condition that currently has very few known facts. Agnes has a partial trisomy of chromosome 16. Chromosome 16 has two arms, a short arm (p) and a long arm (q). Agnes has a third 16q, which is attached to her chromosome 15. It doesn’t appear that any material from 15 is missing. A full trisomy 16 would result in miscarriage or death shortly after birth, so that’s lucky for Agnes that she only has half of the third 16. There is still a lot of extra genetic material which is why she will probably die young. The body can’t function well with all that extra information; there will be problems, we just don’t know what they will be. I hope the doctors that are caring for Agnes do keep track of her and maybe write about her case for publication. Her life could be a helpful example for someone else who may be born with a similar condition.

There is such a wide range of possible outcomes. We can only hope and pray that Agnes’ outcome will prove the literature wrong and that she will be one of the cases where the kid lives longer or responds especially well to therapy.

3 responses to “Agnes Makes Progress

  1. Daphne says:

    I am so happy to hear progress! My baby sister had the most severe form of spina bifida when she was born and my parents had no clue anything was wrong until she was born. She had a shunt, a permanent colostomy, a permanent catheter, one working kidney, has had many corrective surgeries. She was not supposed to live past 12. She is 26! She is fully functioning mentally. She has some spatial and memory problems, but minor. She is paralyzed from the ribs down and everything internally is a reconstructed mess, but she is a grown up! Every minute we have her is a treasure. She had taught our entire family a lot, but mostly to not sweat the small stuff. She is rarely ever negative or unhappy and she is stuck in a chair and mostly helpless to get anywhere on her own. She gives us all perspective. Love that baby every chance you get and let Stephen too. She needs her big brother’s love and hugs and sloppy kisses.

    • judy says:

      Daphne, thank you so much for writing this! It is true that expected life spans for people with serious conditions are not always written in stone and your sister is proof of that. Agnes has the best possible care here and I shouldn’t let a little prediction get me down about her long-term outcome. I can tell already that Agnes will be a joy in our life: she is already smiling!

  2. woodra01 says:

    Hi! I really enjoy reading about Agnes. Congrats on the progress! The pictures of her are beautiful. I applaud that you are using a very difficult experience to help others. Your family is in my thoughts. BTW, one of the moms that helped me the most during our NICU stay was the mom of a baby with a chromosomal anomaly. Through my conversations with her, I was able to realize no matter what challenges we faced… We would somehow find a way to be OK.

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