We're getting ready for a planned hospital stay for David. That's going to take some teamwork and we're lucky to have a very big Team David. I'm going to try keeping up a blog to let everyone know what's going on each day.
The previous blog entry tells the story of how we came to have David in our family. I posted it right around his adoption day. I thought, before the hospitalization, I'd explain a big about his medical issues and why the doctors want him in the hospital for a week or two.
David has the following diagnoses: Vitamin D Resistant Rickets, Seizure Disorder (aka Epilepsy), Global Developmental Delays, swallowing issues (so he's primarily tube fed), and undiagnosed immune system issues. These are all genetic things he was born with. While I think the doctors would like to have some sort of name for what he's got, it seems like it's just a tough genetic draw.
First, the easy (to explain) issues. Global developmental delays for David mean that he’s four years old, but he’s functioning like a one year old. As our doctor pointed out “delay” isn’t really the right word, because it implies David with “catch up” some day, and he probably never will. But we’re enjoying watching his development in slow motion appreciating each milestone all the more for the time and effort it took to reach it.
Seizure disorder is something that didn’t really show up until David was 3 ½ years old. The different-from-normal brain tissue that David has makes it not too surprising that David starting having seizures. Thankfully, the seizures have been stopped by some daily medicines David takes. Unfortunately, these medicines have to be adjust for physical growth, and each adjust means getting used to the medicines’ side effects, which means regression in development. Thankfully, again, David has bounced back pretty well from these regressions and is doing very well at present.
When David was about 18 months old his first foster mom noticed he was getting sick very often. The doctor ordered a swallow study and discovered that David was inhaling (aspirating) as much food as he was swallowing. They quickly put in a feeding tube (AKA g-tube) and took David off food/drink by mouth. About a year later David was showing signs of swallowing better, and working with his therapists, we began introducing food by mouth again. That’s continuing, slow but sure. His primary nutrition still comes from his tube feedings, but he enjoys pureed food by mouth, too!
Now the more complex issue. David has Vitamin D Resistant Rickets. You may have heard of regular old rickets, where when a person doesn’t get enough of the right vitamins, their bones gets soft and bendy, due to lack of calcium in a balanced diet. David’s form of rickets is like that, except that even on a perfect diet, David still wouldn’t get calcium in his bones. His body just can’t move calcium from his stomach to his blood stream. So, due to nutrition from his birth mother, David was born with a healthy set of bones. Once he was born, his blood stream went “Hey, where’s my calcium?” And his stomach said “Sorry, I’m not sharing.” So his brain sent a hormone messenger service to his bones saying “Help! The blood needs calcium! Please loan us some of yours!” So, his bones, givers that they are, started loaning calcium. But the blood is kind of greedy and doesn’t really repay loans, so David’s bone started having less and less calcium. He was about 18 months old when they finally figured out what was going on and gave it a name – Vitamin D Resistant Rickets.
The best treatment for David’s rickets, so far, has been an IV supply of calcium straight into his blood stream. With extra calcium on board, his blood started paying his bones back, so to speak, and he built up bones full of calcium again. Calcium is kind of rough on the veins, so, like when people get chemo, it needs an IV that taps in close to the heart, where the blood is moving fast. That’s a central line IV, which can be a port-a-cath (AKA port) or a hickman. That’s where the final issue “undiagnosed immune system issues” comes into play.
So, the port worked well for delivering IV calcium to the bloodstream. But David kept getting infections. Five infections in nine months – five different central lines. Each time he’d get an infection, he’d end up in the hospital where they’d treat him with antibiotics and remove the central line and place another one. After the fifth infection the doctors started wondering just what was going on . They pulled the last line out and decided to take a break from IV calcium. In the mean time they did some genetic and immune system work ups. They didn’t end up with any answers, but they know he’s got issues, so “undiagnosed immune system issues” is what he’s got. This is something they’ve seen in other Vitamin D Resistant Rickets patients, so it’s probably linked somehow.
So, they pulled the lines out and did some tests. In the mean time, the doctor decided to try some oral (or in David’s case g-tube) medicines that didn’t work when David was first diagnosed, but were worth a shot now. (The difference was that the first time they tried these meds, David’s bones were low on calcium with little to lend out, so they were trying the meds as a cure. This time around, we were trying them as maintenance. And it turned out they worked ok. The meds give him megadoses of calcium (something like a bottle of tums, given 3x per day) and megadoses of Vitamin D (which helps move Calcium around). The thought is that if even a tiny bit of the megadoses makes it to the bones, then it will help. They worked ok enough that from October 2008 to September 2010, David has been taking just oral meds, no IV meds. Hooray!
However, labwork is showing that, while David looks really healthy on the outside, inside the calcium loans have started again. They can see the hormone messengers in the blood stream asking the bones for calcium. They can see on x-rays that David has lost calcium in his bones. He’s ok on the outside, but it’s a downhill slide inside. We could start IV calcium again, but there are risks with that because of the immune system issues.
So now they have a new idea. What if they could use a drug to stop the hormone messenger? Then there would be no more calcium drawn out of his bones. Now, David still needs calcium in his blood stream, but right now the hormone messengers are opting on the safe side. They ask the bones for more than the blood really needs. So, if the doctors can adjust the hormone messengers, they can see if the blood could survive with just the right amount of calcium in it. That might slow down the calcium loans out of the bones enough that the megadoses of oral meds could keep up. That’s the hope anyway.
So why the hospital stay? As it turns out, the whole hormone messenger system is really important. If the blood does get too low on calcium, it’s a serious health concern that could lead to seizure and other problems. So, while the doctors are using a medicine to limit how many hormone messengers are going out, they need to monitor David’s blood calcium levels and be ready to act quickly to give him some extra calcium. That’s why he’ll be in the hospital. And to make doing blood labwork and possibly giving calcium easier, they will put in a PICC line – a temporary central line.
It’s going to be an adventure. The doctor’s will try the medicine and see what kind of results they get. Best case scenario it will work like a charm and David come home with a new oral medicine. Worst case, we start talking about placing a more permanent central line again.
So, that’s the story. I’m making my lists and organizing for the stay which is about a week and half away. This was probably more information than you needed, but it felt good for me to get it out. I’ll try to keep things up to date so you know what’s going on with David!
No comments:
Post a Comment