It's Complicated....

There's just no way around it. Having a child with a permanent IV is complicated. Lately, I've had to remind myself on more than one occasion that Parker's IV, which administers a very potent vasodilator called Remodulin, 24 hours a day, is very necessary and probably what's keeping his PH stable.

Parker's IV pump

Last Friday, March 11th, we had an "incident" with Parker's IV. As I was getting him ready for bed, I noticed his backpack, which holds his IV pump, was wet. I proceeded to pull the pump out of his backpack and discovered that the cassette of medication had somehow unhooked from his IV tubing. Meaning, the pump was still giving the medication, but because it was disconnected, Parker was not receiving it. I had no idea how long it had been disconnected and stopping Remodulin cold turkey can be life threatening. Thankfully, there's about a 4 hour window before the patient starts to have problems, but because I had no idea how many hours the medication had been disconnected, I was a little panicked! I immediately stopped the pump and clamped off 

Parker's broviac catheter, called Accredo Therapuetics to make sure I was doing everything right and then proceeded to put on a new line and restart his medication. I then called his cardiologist and the on-call doctor basically told me to watch him for any signs of distress that night and infection over the next few days (anytime a line is open to air, there is a possibility of infection), but since he was acting completely normal, we felt reassured. Having the tube become disconnected has never happened before for us, so that was a whole new experience, and one I don't particularly wish to repeat. It all turned out fine, but it was a very stressful 10 minutes!!! I spent the remainder of the evening second guessing myself and hoping I did everything right as I thought through the incident over and over again.

Parker showing off his muscles a few months ago.

Well, I should have known that Parker would get a fever a few days later. It's just our luck. On Thursday he woke up without an appetite (which isn't completely unusual) and little energy (which is VERY unusual). His nurse was here with him and all his vital signs were fine, but he was definitely not his normal self. After she left at 1:00, he laid down on the couch and said he needed to rest. He had only eaten a couple of cheese cubes all day and he seemed exhausted. He felt a bit warm so I checked his temperature, even though it was normal a few hours before. It was a 101 degrees. Great. Because of the incident the Friday before, my first thought was a central line infection. The chances of a line infection were probably small, but I couldn't just ignore that. So, I called Accredo once again and they recommended I take him to the doctor just to be sure there wasn't an infection. This is where having a permanent IV makes things complicated. When a normal child who has the same symptoms as Parker had, you probably wouldn't feel inclined to rush to the doctor. You would probably wait a day or maybe even two and see what happens, right? When the diarrhea starts, you would probably think, it's just a stomach bug. You would probably make them stay in their room and rest and make sure they drink fluids so they don't get dehydrated. We just can't do that with Parker. Having a permanent IV adds another layer of concern and worry.

The pediatrician checked him over and thought he seemed fine. He checked for strep throat because his throat was a little red (which was negative) but he didn't think we needed to do any blood work since he had only had a fever for a few hours. So, we agreed to wait and see before doing anything else.

Yesterday, a nurse from Accredo called me to check up on Parker. They were surprised that our pediatrician didn't do any blood work. She recommended that I talk to Parker's cardiologist and let him know about the fever. His cardiologist was off, but the on-call doctor agreed with the nurse from Accredo and wanted him to come in for blood cultures to rule out a central line infection. However, they apparently don't have a normal clinic on Fridays and so we had to go to the emergency room. This is not what I was expecting, but knowing how serious a line infection can be, I knew we had to go. So, we spent the next 4 hours in the ER. Taking Parker to the ER is not very fun. Basically, he scares them. Many doctors and nurses are unfamiliar with PH and the drugs that he is on. Especially, his IV medication, Remodulin.  There are very strict guidelines regarding his Remodulin and his central line, which we always have to discuss with the doctors and nurses. The biggest issue (and it comes up EVERY time we are in the ER) is that blood can NOT be drawn from his central line. But, because we were dealing with a possible line infection, they had to take blood directly from his central line to be cultured. Well, this was completely new territory for us and the nurses. Thankfully, the nurse was willing to listen to us and call Accredo at our request for specific instructions on how to go about getting the blood. To make a long story short, they got the blood that they needed and we were able to restart Parker's Remodulin without any problems. It was very nervewracking throughout the whole process, though! The nurse even said that in her 14 years as a nurse, this was the first time anything like Parker's situation had come up and it was just as scary for her as it was for us. She was grateful that we knew enough about Parker's medication and condition to make sure everything was done right.

Now it's just a waiting game. If any infection shows up, the ER will call us and we'll have to go back in. In the meantime, we just wait. Parker seems a little better today, and I would almost bet that he just has a stomach bug. But better safe than sorry....