I posted this on my message board for a prayer request, and thought that those of you who only know of my son through my blog should be asked as well:
It happened so gradually (or quickly?) over the last month, that he didn't notice it was his good eye doing all the work. Two weeks ago, when going for a tech visit, we found his eye sight was deteriorating (sp?). The swelling is up, blood vessels are taking root deep into the cornea (in a normal eye, there are NO vessels there...it's very dangerous). His body is still attacking the eye, and it is falling apart at the bottom (deepest layer) of the cornea. Meds weren't working well enough, but his eye is too fragile for surgery right now. The longer we wait, however, the more likely a new transplant will be rejected/attacked...but much more quickly, and much more efficiently. It could be months, the doctor said, before Maxwell can have surgery. We don't have months, though. We were in the doctor's office from 6p.m. last Friday evening till just after 10p.m. Our long, not great, visit ended up with Maxwell having to have a shot/needle injected into his eye while awake/alert, with no more than a few minutes notice. They had to leave the room to let Maxwell regain his composure before giving him the shots. He's frustrated, angry and scared. We went through the minutia (sp) of the week, to find out what would have been "signs" that we didn't read properly (wouldn't have known or associated with this problem). We've been told if anything comes of such symptoms for the next couple of weeks, that we are to call right away. Our next appointment, which will include another needle, will be March 1. We don't know what time, as we've been told that the next two weeks are completely booked, so we'll have to show up and just stay.
As non-medically as I can get 
I'll try to explain what is happening with his eye, so that specific prayers can be said.
For those of you who aren't familiar, the cornea is the clear covering over your pupil & iris area. It is domed shaped, but gradually...like a smooth, round hill more than a peaked mountain. The cornea is fairly strong, and contains special cells that allow us to see by controlling and focusing the light around us. The health of the cornea controls about 75% of our ability to see. It is the eye's last defense against germs, dust, etc. from the outside. To my surprise, there are actually five different "layers" comprising the cornea...it's so thin!
Referring back to the 75% number, Max's interior and rear of the eye have also received damage...and while those are more complicated, and we remain on vigil for those portions...the cornea is the "biggie" right now. To see at all, the light has to be focused by the cornea and lens in order for the retina to "see" and compute back what you are looking at. Maxwell received a man made lens during the same procedure in which the corneal transplant was given. Earlier he had to have his retina re-attached, and we are now on year two of the 8 year watch for a likelihood for another detachment. The lens working properly, and the retina manually attached doesn't provide sight without a cornea. Oh, and something else, the cornea does is to protect the eye from UV rays...which are the rays that deteriorate/attack both the lens and the retina. Now, the retina is very susceptible, but I don't know (and didn't ask) if the lens, since it is man-made, is anything to worry about.
Last year, Maxwell received a transplant (among other things) and it made a marked improvement on his vision. However, his body hasn't accepted this transplant and has been attacking it as a foreign body. When our immune system attacks what it deems a foreign body or an infection, it produces a vast amount of cells to fight off bacteria, infection and the like. Because this tissue (the cornea) doesn't completely match up with my son's (no organ donation honestly does match perfectly) the immune system has targeted the cornea as harmful. This "harm" is read as a new disease. Rather than things just not "matching up," which is what I initially thought a rejection was, the body is actively trying to protect itself by destroying the new tissue. The cornea, left without anyone "on it's side" to help out, is in a loosing battle.
Now, if I can explain this clearly...think of the cornea as a hill shaped covering over your iris/pupil. Obviously the internal base of the hill is going to be attacked first...which means that while it tries to heal itself (with the aid of meds) what you are actually getting is scarring all along the bottom/internal portion of the eye...which also reduces vision (clouds it...you'll have to "see" through scaring as well as decreased vision caused by the dying cornea). The bottom layer of the cornea is called the endothelium and it is responsible for the clarity of all the rest of the cornea. There's some sort of liquid that the inner eye builds up...not sure what it is, or what it is for...but the normal, healthy eye, will push out this liquid right into the middle layer of the cornea. Well, the endothelium (the bottom layer) pulls it back in a pump like fashion to keep the eye from swelling and from glaucoma (which my son has already had and is experiencing a bit now). The cells making up the endothelium are never replaced by the body. Once they are gone, they are gone. Maxwell's transplant is being attacked in this area. Once those cells are gone, they will be gone...leaving wide open the remaining cells of a blind cornea, and who knows what other damage can be done. The only option would be to receive another transplant. Which, we could do now, if he weren't in such physical stress, and which we are hoping to advert by saving this cornea. (Honestly, you'll see after I finish explaining what is happening, that I don't know which one to pray for...if we save this cornea, I'm not sure he'll receive as good an eyesight as with a new cornea...but there's little survival options for a new cornea either.)
In my son's case, the trauma caused by the initial injury and subsequent surgeries, is joined together by what is basically a resilient healthy body of a young boy...meaning the immune system is better than average, replenishing what it can at a faster rate than, say, a 60/70 yo transplant recipient. To send these Killer Blood Cells to the "front line" of the battle, the body is growing new blood vessels...think of them as dandelion roots....they start out very thin, etc, and very fragile...but they spindle out a long ways. Now, there is to be Zero blood vessels in anyone's cornea. Think of it this way...not only is the eye seeing through a dying cornea, but through scaring, and now through a thin web of vessels. However, the more mature these vessels become, the thicker they grow...and the more they multiply.
To stop the rejection of the tissue, the doctor's are giving more meds (here I'll talk briefly as if we hadn't received the news that he has to receive physical shots into the eye itself). These meds work to retard the growth of these vessels and to retard the immune systems other reactions against the cornea. Sounds all well and good, except that these same meds are very dangerous for other reasons, and Maxwell has reactions against the meds themselves. Think of it like a cancer patient...in order to kill the cancer cells, the patient undertakes procedures that weaken their immune system and can cause complications and other diseases. Taking these meds is not only reducing the immune systems response to attacking the eye, but is simultaneously reducing the immune systems ability to attack what it does need to attack...infections that Maxwell is having in the internal and outer part of the eye (swelling and eye pressure are a result of this...and those who have been following this saga might remember that it is like over inflating a tire with a weak spot...eventually it balloons and then will pop. When it pops...game over. For good.)
Not taking the drugs, would do irreparable damage to the eye. Taking the drugs is risking irreparable damage. Surgery for a new transplant can not be done with the eye swelling as it is, and the longer it takes to reduce the swelling (we are told that it could take months)...the thicker those dandelion-root vessels become. The thicker they become, the less likely that any future transplant will survive. Think of it like this. You can't pluck out the dandelion roots...because they are coming from inside and beyond the eye. You can cut them out...and gardeners know that if you break a root while weeding, that the root which remains will grow again...but with all the gusto of a thicker, more mature root (because it isn't starting from scratch any longer). Think of the cornea as the soil in a pot. If the soil looses all it's nutrients, but we replace that with more nutritious soil (thinking here of a new cornea), the roots will have even more of a reason to grow...and now that they've been "trained" on one area, they, too, will no longer be starting from scratch. The new cornea won't have a chance.
Our best bet right now, is to take the chance of a risk by continuing with the meds in trying to regulate the immune system. Some of those, as I've already mentioned, Maxwell has strong reactions/side-effects, too. Unfortunately, those are the exact drugs that hold the greatest promise for stopping the rejection, keeping the swelling down, and has the fewest alternatives (meaning, there isn't any other drug out there that can do the job). He's actually taking meds to counteract what his reactions are, which of course, reduces the effectiveness (if I'm understanding) of the first drug). In addition, he's taking meds purely for the infections. So, he's got quite a mix. Currently, he's taking 9 different medications from 9a.m. to 10p.m....hourly. And each med has to wait a few minutes before another med can be administered. We've tried to play around with the timing of the dosages, so that he can go places without having to stop (like we started earlier in the day, so we could have an hour in the afternoon without meds), but we've stopped that. Everything is on a routine now, which is one of the things (the monitoring and staying glued to a timer) that Maxwell didn't like before. (one of the depression issues...he feels that doing that means that we don't trust him; it also allows him, in his mind, to see that decreasing eyesight is his fault because he overslept or forgot a med on time...which is easier to do without a chart...it also isn't any fun to have your mom on you all the time reminding you, or asking you, if you have taken all your meds). Most people receiving a transplant of this kind do not have the added complications of meds. Max is just special that way, I guess. Heck, most people having a corneal transplant (I've heard the average is 20%) never have to worry about rejection at all.
Because the doctor feels Maxwell's eye needs that bit of extra help he's decided that my son needs to receive shots of a certain med that will help in the process. This is a higher concentration, and lasts approximately 2 weeks. Maxwell has to have his eye numbed (which he says doesn't work completely) by having a stick (looks like a giant end of a Q-tip soaked in something, stuck under his eye at a certain angle. Not only does it not look comfortable (Max is warned not to move) but you also have to imagine it is on a very tender eye (and all our eyes are sensitive, but most of us don't have such a fragile eye that has undergone a lot of trauma). Then a needle is injected, again at a certain angle. The doctor decided this needed to be done immediately, during our appointment last Friday. Maxwell, who has always been so brave during this ordeal, broke down. They ended up leaving us alone for a few minutes so that Max could cry it out and regain his composure. When the shot was given, however, he was a trooper, and didn't make a peep. You could only tell by his lips pursing that he felt anything.
There is no guarantee that the meds will work sufficiently enough to cause the immune system to back off (or weaken it's attack). There is always the possibility, in any transplant, that meds will not override the body's attempt at survival. We have the type of injury, the previous surgeries, his age, his immune system, even the drugs working simultaneously against and for us.
Comments