They Called.

....And the news was not surprising. I'd say Ainsley is now officially a medical enigma (my words not theirs).

Some of the things they thought may be wrong were not. Last week there was mention of Esptein-Barr virus, a possible lymphatic problem, aspiration, a laryngeal cleft. Why they tell you things until they're sure I don't know but the one thing I've learned over this last year and a half is you don't worry until you know for sure you have something to worry about.

Here it is blow by blow, all the results for those of you who like details. (Some info is a repeat of previous posts.)

Chest CT - Revealed Bronchiectasis (Irreversible dilatation of part of the bronchial tree. Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions). She seems to be doing well breathing despite having this at level 2 on a scale of 1-10. It's possible it could be congenital or caused by past trauma. Her doctors here will get the report and follow her for this condition but there is no treatment and we have been advised there is nothing we should be doing differently. We will be watching for signs of increased work of breathing which could indicate the condition has worsened. I don't expect that to happen.

The laryngoscopy and bronchoscopy showed she still has massive swelling of the false vocal cords/folds and cobblestoning (bumpiness) on her epiglottis. They also found some irregularities (bumpiness) of the tissue in her bronchi. All the tissue biopsies were negative so that ruled out Epstein Barr or a lymphatic problem. They checked for a cleft in her larynx, a very rare condition but one photo from the Seattle ENT seemed to show she had one (it would have explained some things) but there was NOT one. They saw true vocal cords. It's tough to see movement because of the massive swelling of the false cords but there is no suspicion of paralysis.

PMV use- They don't really think she should use the PMV because she has to force the air out to exhale. That makes sense to me but at the same time we want her to be able to vocalize. Perhaps we'll use the modified one for awhile then try the non-modified one when she seems to be having an easier time. How she does really does seem to vary from day to day. We have always taken it off when it seems that she's having a hard time.

The endoscopy showed her GI system is beautiful. No problems other than a small hernia which could cause/allow reflux. They would not treat it as it is right now. The gastroenterologist did recommend staying on the reflux meds. (Although previous PH probe results while she was NOT on meds showed no increase in PH).

The lung wash was unenlightening, no bugs our critters to suggest aspiration or other problems.

The FEES (dye swallow study with fiber-optic camera placed through the nose to view actual swallows) surprisingly looked pretty good. It showed that she has normal sensation at the back of her throat (really good) and that she does what she is supposed to. There is a small amount of fluids getting to the airway when they should not (it seems she's allowing some to slip back in her throat before she swallows, hopefully this will improve with practice) but because of the swelling she is not actually aspirating (the swelling is essentially protecting her airway). She does better with thick than thin liquids. I've often wondered if her swallow function might improve if the swelling was reduced. They said probably not, that her swallow is actually pretty good, it's more learning how to control the food/liquid. They want me to continue feeding her just a few spoonfuls of food to keep her interested in eating but not to try to give her meals by mouth. I think that makes sense. I've said all along that I don't want to try to get her to eat until I know it's really safe (until a swallow study shows no fluid in that part of the airway.) They didn't really see a substantial amount of saliva above the airway that would indicate aspiration of saliva.

Feeding Consult- The feeding team was impressed with how well she did (remember she was hungry so she ate.) They were really glad that we are doing the home-blended formula (food) and would have suggested the same if I had not already started it. They suggested making the formula even thicker and giving it with a syringe. I may well try that, they are going to give me a recipe. I'm still in the process of tinkering with her feedings anyway. They want her to gain a little weight. I have to say she looks a little chubbier just in the last few days. It helps not to be NPO for anesthesia.

I had some questions about the impedance probe results and what that means for the reflux, so they're going to get back to me on that.

They assured me that I've done a great job and that despite how "bad" things look for Ainsley on paper she's really doing remarkably well and that is owed to the care she's receiving. They said that there is nothing at all that we should do differently. They would be happy to see Ainsley again in 6-12 months if we want to bring her there.

Unfortunately even though no news is good news (in a way) it does leave us at a loss for how to get this darn swelling to go away! At this point we have to just be satisfied that we've done everything we can and just enjoy Ainsley for who she is. (Of course those of you who know me know I won't give up and will always be thinking of what we could do that might help her.)