I purposely left out a lot of the medical stuff in my last post, I wanted to shine the spotlight on some other areas of our life. Our main doc has been on vacation for the past two weeks. Before he left he put in a script for two weeks of antifungals to cover the reoccurring yeast that our girl just can't seem to shake. Our hope was to have some time away from DuPont while he was away…really we were!
In June we had a conversation with our main doc about keeping Lucy out of the hospital for the summer, or really for as long as we possibly can. We have had some very serious discussions about how exactly we were going to set out to achieve this. With every decision, we talked about whether or not doing this or that was going to give her “quality of life”. In all honestly, we’re not sure, but we certainly hope the decisions we are making for Lucy, and all of our children for that matter, give then “quality of life”. Looking back on the last two months it would appear that our decisions have kept our girl from having any sleepovers at the hospital, and for this we are so grateful. Spending time out of the hospital and at home as a family is quality of life! However, I will admit that I am scarred for the future, of what our decisions now are going to mean for our girl down the road. In order to keep Lucy home this summer we have her on some pretty heavy duty antibiotics and antifungals to keep the infections at bay. We are messing with the bug kingdom by choosing this route for our girl, knowing that one day we will most likely be confronted with a bacteria that we can not get rid of, when that happens we will need to realize that we did all that we could to give our girly, and our entire family for that matter, quality of life.
Last week we had a real fear that Lucy had a urinary tact infection brewing, when we dipped her urine at home it showed signs of infection, even more obvious was her discomfort. We took our girl and enough urine for several cultures over to DuPont Monday afternoon to meet with our urology group. The overwhelming fear expressed by the group was what antibiotic would we put Lucy on to cover the infection. Most everything they thought of, she would likely be resistant to because she is currently on several pretty heavy duty antibiotics. We discussed using prophylactic antibiotic washes in Lucy’s bladder, but the med that we would use to do that is a med that we routinely use for small bowel bacterial overgrowth. Fortunately, Lucy did not have a UTI, but rather we discovered that she has developed a reaction to the lubricant in the closed cathing system that we use on her, her urethra was swollen and bleeding. Here was one of those situations that if this was the only problem we had to deal with, then we might feel overwhelmed by it. However, I was relieved to learn that this was our issue and not an infection. This is not to say that what Lucy is experiencing is good, but it is better than the alternative, that she is growing resistant bacteria. As five women were standing examining our little ones very sensitive urethra, we could all relate in some way to her discomfort and focused on trying to improve her quality of life when it comes to cathing. Our urologist walked over to outpatient pharmacy to personally put in a script for Lidocaine for our girl. She did this for us knowing that it would require some persuasion on her behalf to get this med. It worked and so is the med. Needless to say, we are trialing different cathing systems.
Last Friday we had an urgent need to have Lucy’s gj-tube looked at in IR. Ugh…if it’s not one thing it’s her gj-tube! I called Friday morning at 9:00 am to schedule a time to have it looked at, they told me the schedule was packed and the only time they had was in an hour. I was on a tight schedule on Friday, we had to be at the U of De by noon for Jack’s final presentation, but I also knew that I had to have her tube looked at before the weekend. Getting the very potent yet small volumes of antibiotics into Lucy’s jejunum is how we are keeping the bacteria from translocating into her blood stream and making her sick. Without j-tube access we fear that the meds would not come in contact with the bacteria in her intestines. I sprung into action, I called Helen to rearrange our schedule, dressed and fed the girls, gathered all of the medical supplies I would need to be out of the house for a few hours(this in itself is impressive), loaded up the troops and Helen, grabbed a power bar for breakfast and we were on our way. Let me just state for the record that I was only 15 minutes late, not too bad if I do say so myself. Lucy’s tube had indeed migrated, but not out all of the way, they did a dye study to check for placement and all agreed that it was in far enough that nobody wanted to risk replacing it. We have all been witness to Lucy’s bowels push back a j-tube during a placement, and we all agreed that just barely in position was better than not being able to position it at all. We left IR half joking that we would see them next week.
On Thursday, we had a two hour therapy evaluation at DuPont with an OT who specializes in hand strength. Lucy’s hands, just like her entire body, are weak. She suffers with hypotonia, and has loose joints. As a result she has some physical limitations with what she can do with her hands. Mr. Joe was amazing with our girl, he had all kinds of ideas and devices for us to trial from things as simple as a foam cylinder to poke her crayon through to help improve her grip, to custom made hand splits that secure her wrists giving her hands a stronger grip. I am amazed by what splinting her wrists have done for her hand strength.
I wish there was something we could do to improve the strength of Lucy’s GI system. Our weekly labs on a weekly basis are telling us that Lucy’s TPN cholestasis is trending the wrong way. Her liver numbers are over 300 and climbing, as well as her GGT and alkaline phosphate, all indicating worsening liver disease. We are scheduled to see Dr. R this week, at that time we are going to have to make some decisions as to what to do about the lipids in Lucy’s TPN. Lipids are the fat source in Lucy’s diet that are processed through the liver, we are only running a minimal amount of lipids one day a week. Lipids provide the essential fatty acids that the body needs. I am not sure what the next steps will be, but will let you know when we do.
Overall Lucy looks puffy, her edema is dramatic. Almost everyone who has seen our girl has commented on how much bigger she looks compared to what she used to look like. There are a lot of theories out there in the medical world as to why Lucy’s body is third spacing, obviously there is a vascular leak somewhere, but why we really don’t know. Her kidney numbers continue to indicate that she is in need of more fluids, which would be the opposite of what you would think she needs when looking at her. Her fluid in compared to her fluid out are indicating this as well. Cardiology is concerned about her edema and the impact that will have on her heart. We use Lasix twice a day pulling off at least two pounds of fluid daily. We have been told that the body responds in all sorts of ways when it is in distress, we will continue to treat the symptoms.
I stopped by urology on Thursday to pick up some more cathing samples. I was talking with the very wonderful PRN who takes such good care of us. She very sweetly said, “We did it, we made it almost two weeks without the good doc!”, referring of course to our beloved Dr. R. Laughing we said very seriously, we can’t wait until he is back!