Surgery tomorrow

This particular bacteria is one that usually only affects people with severely suppressed immune systems.  It is so common on our skin that it is often disregarded in blood cultures as a contaminate, rather than a true infection.  Staph epi has a particular habit of clinging to plastic in the body, and once it has, it can be particularly dangerous and difficult to treat.  Generally, line infections are treated to negative cultures, but the line doesn’t have to be removed until the infection recurs three times.  This is only the second time with this line, but we will be removing the line anyway to give Keegan time to recover before we make the next round of treatment decisions.  

Between the steroids and maximum doses of anakinra, it is as if we put a lid on a boiling pot of water with this disease process.  This regimen is not sustainable for much longer, and we are not getting full control of his immune deficiency, rather just keeping it mildly in check.  Keegan’s medical team is now fairly confident that what we are dealing with is an innate immune deficiency/defect that he was born with, not that he developed as a result of immune suppression from transplant.  It may sound counterintuitive that an immune deficiency could cause an overactive immune system (flare), but what is really happening is an overcompensation by one part of the immune system for another part that should be there but isn’t.  The steroids and anakinra suppress the parts that we believe are overreacting. However, they are not strong enough and have too many drawbacks to work effectively in the long term.  Keegan is already showing symptoms of chronic steroid use (no vertical growth in over a year, bruising, wound-healing issues).  The anakinra has too short of a half-life to be used at this dose for more than short periods of time.

We are waiting on two different sets of labs to be finished in order to decide what step to take next.  One is a genetic test looking for known mutations of Severe Combined Immunodeficiency (SCID), or “bubble boy syndrome.”  If he does have one of the forms of SCID, it will most likely put us back on a bone marrow transplant track.  If not, we are also waiting on some research-level labs being run by the rheumatology group that look at the inflammatory pathways of Keegan’s immune system.  Those results may help us feel more confident in choosing the next, stronger drug to use.  In either situation (transplant or more immune suppression), moving forward with a risk of an infected central line is too risky.  We anticipate the results of these labs will be back by early September, so we have to act on the line now.  Additionally, we don’t want to risk another flare like he had in Boston that we are unable to control because of a latent line infection.

So now we proceed with treating the infection while waiting on the rest of the pieces to fall into place.  That means Keegan will go into surgery tomorrow to have his current port removed.  They will then place a temporary IV in his external jugular vein (the side of his neck).  This is longer than your typical peripheral IV but not long enough to risk the staph migrating to it too.  Protocol requires that you wait 48 hours after removing an infected line before inserting another central line to be sure that any bacteria that is showered into the blood stream is treated and won’t colonize on the new line.  We will use the EJ line for his antibiotics, a less concentrated version of his TPN, and hopefully his anakinra.  If the pharmacists decide that the anakinra is too strong for that line, we will have to inject it subcutaneously twice a day.  Anakinra is very acidic and very painful, so we want to avoid that at all costs.  Keegan’s surgeon has the OR on Friday, and we are praying that he can fit Keegan into the schedule then for a new line.  Then, we could theoretically go home on IV antibiotics for two weeks to be sure that the line is and stays clean.

While the fever has broken and his labs have slightly improved, Keegan was a wreck today.  Extremely emotional, agitated, and very, very tired.  He all but fell asleep while eating his lunch, pickle in hand.  I was able to get him to go outside for about 20 minutes in the late afternoon (I know it’s hot out there, but it’s a frozen tundra in here!), but that made him so tired that he was begging for a second nap an hour after his first.  He made it to bedtime but just barely.

We do not have an OR time for tomorrow because he is an add-on.  Tomorrow he will also have an echo to make sure the staph did not migrate to his heart at the tip of the catheter, and in the next few days he may have to have an MRI scan to check for an open vein before the new line is placed, as he is running out of access sites.  We simply cannot thank everyone enough for your continued prayers as this long journey continues.  You give us hope and strength to continue on.