Well, let me tell you…It has not been a carefree summer by any means. Things with Lucy are stressful at the moment. She is experiencing what Dr. R describes as an autonomic storm. We have seen this in her before, with each episode we see similarities but the presentation is different as is the outcome. Lucy’s heart rate, blood pressure, body temperature, balance, over all coloring-she looks gray at times, oxygen saturation levels, energy level, attention span, motility, and bladder are all areas of concern.
Several weeks ago Lucy started with elevated temperatures which always has us on edge. Her heart rates have been swinging from the mid 50’s to 180’s. I wonder what mine have been? Lucy has been walking a hypothetical tight rope, balancing between fever and no fever, she has been sitting anywhere between 96.7 and 100.4, anything at or above 100.4 is considered a fever and thus and automatic admission. Because we have Lucy on continual antibiotics it can be difficult to determine if an infection is brewing. We did labs and cultures a few weeks ago on Lucy’s stomach and intestinal bile, they were seriously smelling like rotten eggs, to try and figure out what if anything obvious could be the cause of her issues. We switched up antibiotics to cover some of the bugs growing in Lucy’s GI tract and put Lucy on a two week course of IV antifungals in hopes of seeing some improvements in our girl. The antifungals helped to clear the yeast from her mouth which has been a problem for the past few months, so much so that Lucy developed some odd licking and sucking behaviors that I am happy to say have ceased since clearing the yeast from her mouth, poor baby. The antibiotics have helped to kill the
rotten eggs some of the new bugs that grew in Lucy’s cultures. Despite our efforts to control the bacteria and yeast, our girl has not shown us signs of improvement.
We had an appointment with Lucy’s cardiologist a few weeks ago. Lucy’s hypertension and tachycardia are being controlled with the use of the clonidine patch, this is a good thing. However, Lucy’s blood pressures are low, this is a side effect of the clonidine patch. If we make any more changes in her clonidine dosing we are all fearful that we will see a rebound effect from the medication and we will be right back to the problems with hypertension. We all agreed that clonidine is a medication that is keeping things under control for our girl, and so we are keeping the dosing where it is at. In addition to being diagnosed with sinus tachycardia, POTS, and hypertension, Lucy was also diagnosed with an irregular heart rate. Lucy's cardiac issues are not related to her heart muscle, but rather her autonomic dysfunction. We have serious concerns for Lucy’s heart, but at the present time our options for treating Lucy’s symptoms come with greater risks than benefits.
Most noticeably Lucy’s coloring is a few shades whiter than white, with a tinge of gray and purple thrown in there for coloring. A few weeks ago she herself requested the need for oxygen, this coming from a girl who at her sickest just a few months ago fought wearing a nasal cannula while heavily sedated. She has pretty much been 100% oxygen dependent for the past few weeks, without it she gets anxious and fidgety and starts looking gray. We are getting used to the constant hum of the oxygen compressor in our upstairs hallway, Jack even said the other night that he likes the noise of Lucy’s oxygen compressor, “It makes enough noise that we don’t have to be as quiet in the morning.”.
Lucy’s belly pain is constant and concerning, she continues to drain dark green and golden yellow bile from both her g and j tubes. I have brought her to DuPont almost weekly to have her gj-tube checked for placement, almost hoping that they are out of position which could be the reason for her belly pain. Surprisingly, it has been in good position which means that we have managed to keep a gj-tube in Luc for over a month! Our IR team has been just as surprised as I have to see it still in her small bowel. We started Lucy back amitriptyline, a medication that helps to calm an irritable intestinal track, at double the dosing she used to be on before she became sick in April. We are hoping that this will provide her with much needed pain control as our options for pain control are not options that I want for our little one
just yet ever.
Lucy’s bladder issues have really come to light in the last month. Since introducing straight cathing, Lucy has amazed us all at her willingness to have it done! As with all things we have to to do for our girly, once she realizes the benefit she tolerates whatever it is that needs to be done. She is so amazing! She began realizing how much better she felt when her bladder was emptied, however we also realized how severe her bladder spasms were. We started Lucy on the Oxytrol patch in an effort to alleviate the intense bladder spasms that she was experiencing every time her bladder was full and we went to cath her. This medication has made a huge difference in Lucy’s quality of life. Lucy is no longer able to empty her bladder without cathing which means we are cathing her every one to three hours or as she requests. This gets complicated by the fact that Lucy is on Lasix twice a day to help with the third spacing in her body.
With all that Lucy has going on we have decided on Friday to schedule Lucy for a blood transfusion tomorrow. We are hoping and praying that we will see some improvement in our girl.