The Foundation for Children with Atypical HUS

In the pediatric population, the initial line of choice for Hemo Dialysis is usually a double-lumen catheter placed in the I-J.  However, the newest push in the dialysis world has been a move toward fistualas which are typically placed in the forearm, but may also be in the leg as well or even the chest.  There are no protruding lines which helps minimize infection as in the case of a port. 

 

This movement has been slower to catch on in the pediatric population as the vien must be tough and have a long enough run for the dialysis needles.  The vien must develop what they call a thrill, which occurs some weeks after the surgery.  If the fistula is developing, you will feel the blood flow through the skin and the vein will protrude somewhat.  It looks like an adult's veins in their hands and forearms after hard labor.  In order to help the fistula mature, patients are encouraged to use a squeeze ball several times a day.

 

Occassionally, a patient must grow before having a placement for the surgeon to be able to find a good location.  Even when the surgeon is able to perform the surgery, the vein may still need to to grow more mature.

 

The ability to use the line for dialysis takes time, it is normally used to take blood flow only from the body at first and returned to the body through the existing catheter.  They will use it for short periods of time and unhook the fistula and rehook so that the rest of dialysis is still occurring through the catheter.

 

Even less frequently, the regular pediatric needles will be too long to run in the fistula and smaller, special-order needles will be ordered.  Of course there are extra large needles as well, but this is probably not going to be needed in our population. 

 

As with any surgery/procedure, there is the usual time it takes for placement and then there is the time an actual aHUS patient will require!  If your surgery is taking longer than the surgeon anticipated, hospitals usually have some form of communication available between the operating room and the nervous family.  It is good to know what to do ahead of time, in our case, it just requires a quick request of the nurses in the parent waiting area and they can call and get an update from the operating room.

 

There is a "Button Hole" method which may also be developed.  This will be explained in another discussion.

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Did you know...

CFH (Serum Complement Factor H) is a regulatory protein. The secreted protein product of CFH consists of 20 repetitive units named "short consensus repeats" or SCRs (each approximately 60 amino acids). In patients with aHUS the last 5 "pearls" in the twenty pearl strand protein, SCR16 - SCR20, should bind to protect cells but do not- they are defective in one or more of the last 5 SCR locations. If they cannot bind or stick to the kidney to protect that tissue, the platelets clump into clots that affect the glomeruli of the kidney -potentially causing acute renal failure.
  
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It is estimated that there are about 300 cases of aHUS in the U.S., and it is most common with young children. The condition is life threatening and either can be chronic or can recur at intervals.
  
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