B

Figure

For centuries, bloodletting with leeches was believed to cure many ills. This woman in seventeenth-century Belgium applies a medicinal leech to her arm.

Figure 14C

Microsurgeons sometimes use leeches to help maintain blood flow through veins in patients after reattaching severed ears or digits. An anticoagulant in the leech's saliva keeps the blood thin enough to flow.

© Biopharm (USA) Limited 1994.

Shier-Butler-Lewis: I IV. Transport I 14. Blood I I © The McGraw-Hill

Human Anatomy and Companies, 2001

Physiology, Ninth Edition

Living with Hemophilia

Don Miller was born in 1949 and is semiretired from running the math library at the University of Pittsburgh. Today he has a sheep farm. On June 1, 1999, he was the first hemophilia patient to receive a disabled virus that delivered a functional gene for clotting factor VIII to his bloodstream. Within weeks he began to experience results. Miller is one of the first of a new breed of patients — people helped by gene therapy. Here he describes his life with hemophilia.

"The hemophilia was discovered when I was circumcised, and I almost bled to death, but the doctors weren't really sure until I was about eighteen months old. No one where I was born was familiar with it.

"When I was three, I fell out of my crib and I was black and blue from my waist to the top of my head. The only treatment then was whole blood replacement. So I learned not to play sports. A minor sprain would take a week or two to heal. One time I fell at my grandmother's house and had a one-inch-long cut on the back of my leg. It took five weeks to stop bleeding, just leaking real slowly. I didn't need whole blood replacement, but if I moved a little the wrong way, it would open and bleed again.

"I had transfusions as seldom as I could. The doctors always tried not to infuse me until it was necessary. Of course there was no AIDS then, but there were problems with transmitting hepatitis through blood transfusions, and other blood-borne diseases. All that whole blood can kill you from kidney failure. When I was nine or ten I went to the hospital for intestinal polyps. I was operated on and they told me I'd have a 10 percent chance of pulling through. I met other kids there with hemophilia who died from kidney failure due to the amount of fluid from all the transfusions. Once a year I went to the hospital for blood tests. Some years I went more often than that. Most of the time I would just lay there and bleed. My joints don't work from all the bleeding.

"By the time I got married, treatment had progressed to gamma globulin from plasma. I married at twenty, the day of my graduation from college. By then I was receiving gamma globulin from donated plasma and small volumes of cryoprecipitate, which is the factor VIII clotting protein that my body cannot produce pooled from many donors. We decided not to have children because that would end the hemophilia in the family.

"I'm one of the oldest patients at the Pittsburgh Hemophilia Center, so I knew people. I was HIV negative, and over age twenty-five, which is what they want. By that age a lot of people with hemophilia are HIV positive, because they lived through the time period when we had no choice but to use pooled cryoprecipitate before recombinant factor VIII was available. I lucked out. I took so little cryoprecipitate that I wasn't exposed to very much. And, I had the time. The gene therapy protocol involves showing up three times a week.

"The treatment is three infusions, one a day for three days, on an outpatient basis. So far there have been no side effects. Once the gene therapy is perfected, it will be a three-day treatment. A dosage study will follow this one, which is just for safety. Animal studies showed it's best given over three days. I go in once a week to be sure there is no adverse reaction. They hope it will be a one-time treatment. The virus will lodge in the liver and keep replicating.

"In the eight weeks before the infusion, I used eight doses of factor. In the fourteen weeks since then, I've used three. Incidents that used to require treatment no longer do. As long as I don't let myself feel stressed, I don't have spontaneous bleeding. I've had two nosebleeds that stopped within minutes without treatment, with only a trace of blood on the handkerchief, as opposed to hours of dripping.

"I'm somewhat more active, but fifty years of wear and tear won't be healed by this gene therapy. Two of the treatments I required started from overdoing activity, so now I'm trying to find the middle ground." ■

different genes are known to contribute to the surface features of red blood cells, which determine compatibility between blood types.

Essentials of Human Physiology

Essentials of Human Physiology

This ebook provides an introductory explanation of the workings of the human body, with an effort to draw connections between the body systems and explain their interdependencies. A framework for the book is homeostasis and how the body maintains balance within each system. This is intended as a first introduction to physiology for a college-level course.

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