One Port, Two Port, Red Port, Blue Port

Posted Tuesday March 29, 2011 by Melissa

Mommy and Case

A port-a-cath similar to the two ports Case has

Today, Case received his 4th dose of intrathecal idursulfase or Miracase as we’ve dubbed it. Now, this sounds like a smooth ordeal, but the potential for hangups exists all along the way. Let me explain.

The port-a-cath (the device that receives the medicine) is experimental and not approved by the FDA. It is a metal device with a reservoir in the center (where the needle to infuse the drug goes) that was placed underneath Case’s skin and attached over his ribs on one side of his chest. The tube that comes out of it (called the catheter) is snaked through his muscle tissue, under the skin, around his side and then into his spinal fluid. To do that, he needed surgery to place the device, thread the catheter, then open the dura covering his spinal fluid to thread the catheter into the fluid and up several inches.

So the most obvious complications are this –  the port could break, it could stop working, the catheter could slide out of the spinal fluid, or scar tissue could cinch it off. All of these are known potential complications for this type of port and can result in leakage of cerebrospinal fluid at the base of the spine or the port site. All I can say is that one or more of these complications have already occurred with other children and are not unexpected to happen during the time Case receives this drug. Only the grace of God keeps them away.

Case also has another port-a-cath, located on the other side of his chest. This is the port through which he receives his normal Elaprase infusions every Thursday. Of course, there exists the potential for mistaking the ports given that they are on either side of his ribcage. Were the Elaprase infused into his intrathecal port, it would most likely kill him, so keeping them properly identified is not a small thing. We are looking into a medical tattoo so that a confusion never occurs given that these infusions (both of them) will likely have to happen for the rest of Case’s life.

Getting past the port, and assuming the port works each month, the clinical trial involves drawing some of Case’s cerebrospinal fluid out from the port before the medicine is infused. The tests involve looking for increased white cells which could indicate either infection (read: bacterial meningitis) or inflammation (who knows?). Again, this is not a wholly unexpected complication to occur unfortunately.

Assuming the white count is low enough, Case is then infused with the drug. It is an experimental drug. Case is the 4th human to ever receive this drug. Prior to these boys, it was infused into mice.


But that is the only animal that has been created to have MPS II, an incredibly difficult scientific puzzle to begin with.

Then, you wait. You wait to see what the drug might do. You hope and pray that the drug does not do something terrible to this child you love. You hope and pray that it does something miraculous. I believe we are seeing something miraculous.

But there are no guarantees. Drugs that work in mice sometimes never work or stop working in humans. Drugs that work in animals sometimes do terrible things to humans.

But our hope is not in the drug, our hope is in the miracle that God can perform with this drug and the miracle He can perform with the life of this little child who does not yet understand who created him, but someday might: Blessed is the man who trusts in the LORD, And whose hope is the LORD. For he shall be like a tree planted by the waters, which spreads out its roots by the river, and will not fear when heat comes; but its leaf will be green, and will not be anxious in the year of drought, nor will cease from yielding fruit. Jer. 17:7-8.

Just to be clear, I don’t describe these things so that you can worry or feel sorry for our situation. I write it so you can see the true situation as it exists and better experience the awe and wonder at God’s hand overcoming any obstacle. I am just incredibly humbled to get to see it firsthand.

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