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Rare Blood Disorder Threatens Huntville Newborn

By Assocaited Press

Huntsville, AL – About 10 minutes after Maddox Locke was born July 2, an innocent-looking red bump appeared on his smooth skin.

Todd Locke guessed it was just a bruise from his son's rough entry into the world. Maddox's mother, Becky May, had labored for 20 hours at Florence's Eliza Coffee Memorial Hospital before agreeing to a C-section surgical delivery.

But the tiny blemishes kept coming. Dozens of them, eventually covering Maddox's arms, legs and chest. The pediatrician didn't have an immediate answer and told first-time parents Locke and May that she needed to consult a doctor in Huntsville.

May's head was spinning: What if Maddox had leukemia?

Doctors soon discovered that Maddox was born with far too few platelets. Because of a blood compatibility problem, some normally helpful antibodies that Maddox received from his mother in the womb destroyed most of his platelets.

It's called neonatal alloimmune thrombocytopenia, and Huntsville neonatologist Dr. Meyer Dworsky said Maddox is the first case he's seen in three years.

Dworsky has been caring for Maddox at Huntsville Hospital's neonatal intensive care unit since July 3.

Platelets help the blood clot during injuries, and healthy people have 150,000 to 450,000 of them per microliter of blood. Spontaneous internal bleeding can occur if platelet levels fall below 20,000; Maddox had just 10,000 platelets at birth.

The marks on his skin were evidence of internal bleeding.

If May had given birth vaginally, as planned, Maddox might not have survived the traumatic journey through the birth canal, Dworsky said.

Figuring out what was wrong with Maddox was only half the battle; he also needed transfusions of a hard-to-find platelet type, PLA1-negative. May is a match and wanted to give her platelets to Maddox, but her veins were too weak from giving birth.

"I tried three times" to give blood, said May, a social studies teacher from Killen in Lauderdale County. "It was really scary. You don't know when he's going to get better or if he's going to get better."

Unable to find PLA1-negative platelets, the hospital gave Maddox another platelet type. They didn't help, and the renegade antibodies in Maddox's system continued to attack his platelets. By July 4, he was down to just 5,000 platelets per microliter of blood.

Locke was frantic and began trying to track down more platelets on his own. A family friend at Vanderbilt Medical Center put him in touch with an American Red Cross employee in Nashville, who found a single bag of PLA1-negative platelets at a blood bank in Philadelphia.

Meanwhile, May's sister, Amy Southerland, was speeding here from northern New Jersey because doctors felt she could be a platelet match for Maddox.

"It was a race to see whose platelets would be ready first," May said Monday.

Doctors went with the Red Cross platelets and began the transfusion about 3:45 a.m. July 9. Within hours, Maddox's platelet count rebounded from 5,000 to 179,000 per microliter back in the healthy range.

May and May's mother, Linda May, were at the Embassy Suites on Monroe Street when the hospital called with the good news. It was happy hour at the hotel bar.

"179,000! My baby's going to live!" May said she screamed.

Since then, Maddox's platelet levels have bounced between 164,000 and 301,000. He will need a booster dose of platelets about every five days until the attacking antibodies in his system go away.

"It will be weeks or months until they totally disappear," Dworsky said Wednesday.

But Maddox's natural platelet production will eventually overtake the diminishing number of bad antibodies, he said.

Once that happens, Maddox should be perfectly healthy, his parents said.

Maddox's illness highlights a need for more platelet donors.

Vicky McLain, administrative director of Huntsville Hospital's laboratory, said many blood donors are not willing to spend the extra time it takes to give platelets.

Using a process called apheresis, platelets are separated from the rest of the blood, which is then returned to the donor. It takes about two hours.

Also, because platelets cannot be refrigerated, they have a shelf life of just five days, versus 42 days for a unit of blood.

Huntsville Hospital tries to keep 10 units of platelets on hand at all times, McLain said.

The biggest users are chemotherapy patients because the tumor-killing drugs also stunt platelet production. Hospitals typically do not stock PLA1-negative platelets because they are so uncommon.

McLain said Maddox is only the third PLA1-negative patient she has seen in her 26 years at Huntsville Hospital.

Debbie Penrod, manager of the hospital's blood bank, said she is working with the Red Cross to identify a donor who can provide platelets for Maddox until his mother is strong enough to donate.

And now that May knows she has such a rare platelet type, she wants to be a donor for other sick children and adults. Most platelet donors can safely give every two weeks.

"She wants to give back," Locke said Wednesday, "so someone else will not have to go through the turmoil of trying to find this type of platelet."

Copyright 2007 Associated Press. All rights reserved.
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