We haven’t updated this blog in awhile but that is a good thing! It means things have been going along fairly well for her and she’s been extremely stable. We’ve been able to focus on other areas of our life. Tess has still been blogging over at Circling Through This Life and you can read there some of the things Mantha and the rest of us have been up to. Well, in the last two months, that wall (or maybe facade is a better term) has started to break down and we’ve been reminded how very fortunate we’ve been and how very medically complicated she is.
This recent wave of medical complexity began in January, when she saw another cardiologist. (Why do her cardiologists keep moving away? Same practice and this doctor we’ve dealt with before but he’s never been “her” doctor until now.). We did have some concerns that we brought to the doctor. She had been extremely tired, more than usual, more lethargic she had no energy. She wasn’t eating as much. Great news! Chest x-ray was clear. EKG was “normal” for her and her echo showed “no changes.” Doesn’t look like we’re looking at heart failure. Bad news: Blood pressure low and lab work came back “worrisome” with an ‘extremely high’ level of Vitamin D (She has had low vitamin D in the past and one of her doctors prescribed Vitamin D supplements). Dr. M took her off the supplement, and off her diuretic and blood pressure meds. She was also very dehydrated.
In less than a week we had our girl back. She was full of energy, eating like crazy, and back to her usual contrary argumentative self. She even willing did and asked to do school. She did not complain about being too tired. We repeated labs and things were trending in the right direction.
As the days went by though we noticed that she was beginning to swell. First her feet and ankles and then up her legs and in hands and face. We did labs again and the doctor put her back on her diuretic but at a lower dose. Kidney function had returned to normal. We were told to repeat labs in one month and a doctor appointment was set up. We’ve got to be out of the woods now right? Wrong.
Her face and hands went back to normal, and the swelling reduced in her legs. While the swelling improved in her feet and ankles it did not go away completely. We called the doctor again. More labs ordered and this last go round included a stool sample (the wonderful things we get to do for our kids!) The results of this last round gave us a major shakeup. The labs indicated she has Protein Losing Enteropathy (PLE for short).
This is not an uncommon condition to pop up for someone who has had the Fontan (as she has). The causes are numerous, so treatment varies according to the cause. Bottom line though is figure out the cause and treat the cause and that should, in theory, resolve the PLE. Prognosis? It depends on the severity, and how well it can be managed using the various treatments. You Google ‘PLE after Fontan’ for an idea of the prognosis and treatments (or don’t it isn’t pleasant). The first step in determining a treatment plan is doing a cardiac catheterization, which we’ve scheduled. That will lay the groundwork for where we go from here.
A little bit about the cardiac cath: they will be taking internal pressure measurements and looking for things that may be raising her pressures and causing the PLE. Things such as occlusions and collaterals. If they find them, they will try to take care of them right then and there.
It’s been a difficult watching her struggle, while hoping that things are starting to turn around. Maybe they will, but for us, it feels like the fear and uncertainty we experienced those first few years. It’s the unknown and that is scary.
Her Cardiac Cath is scheduled for this week. We’ve set up a Facebook group for friends and family would like to stay updated both for the catherization and going forward.
(Both mom and dad contributed to this post)