For years, the recommended treatment for patients whose chronic low back pain stemmed from degenerative disc disease has been fusion surgerythe use of bone grafts to solidify the disc and spine. On the plus side, the procedure provides relief in 90 percent of cases, on the minus, it requires a huge incision and removal of a rib to reach the spine. As a result, many patients go on to experience more back pain and fatiguenot because of the disc, but from tissue damage and muscle weakness from the surgery.
Now, miraculously, an outpatient procedure, called Intradiscal Electrothermal Annuloplasty, or IDET, is making it possible for some to reap the benefits of fusion surgery without undergoing the huge operation. In other words, disc pressure and pain are being treated without an incision and without destroying tissue.
Orthopedic surgeon Khaled Kebaish who performs the IDET, explains how the technique works anatomically: Spinal degenerative disease occurs when tiny nerves in the annulus, or outer wall, of the disc begin to bulge and tear. The body attempts to heal the area by sending in small blood vessels carrying nerve fibers. But like an open sore, these nerves themselves become a source of pain. With IDET, Kebaish guides a wire through a catheter into the disc space and coils it. He then heats the wire for about 15 minutes and burns away the small, pain-inducing nerves in the annulus. The result, studies show, is that disc pressure and pain decrease dramatically in about 80 percent of patients.
But not everyone with degenerative disc disease is a candidate for the procedure. Ideal cases are those with limited damage to the annulus and only one or two damaged discs. Additionally, the treatment is not recommended for patients with herniated discs or those who already have had fusion surgery.
Still, Kebaish says, in the correct patient IDEP is a very attractive option. "It gives a surgeon more room to do something that is not irreversible, and it doesn't involve sacrificing the disc."
Three different neurosurgeons told Marcy Lash the same thingthe only way for her to get relief from the ruptured disc that was pressing on a nerve was major back surgery. Not eager to undergo such an ordeal plus a long, painful recovery, the 38-year-old credit analyst from Olney, Md., tried for two months to tolerate the shooting pains in her right leg. Then Lash went to see a neurosurgeon she'd heard might offer her another optionAllan Belzberg.
Instead of open surgery, Belzberg does a procedure called microdiscectomy. He starts by making a small cut rather than a large incision in the skin and inserts a tubelike catheter into the cut. Then, using surgical instruments and a small video camera at the end of the catheter, he begins to separate muscle fibers covering the disc so he can access the damage. As in the conventional approach, Belzberg removes herniated disc fragments. But because he splits muscles rather than cutting and stripping them off the bone, there's less dissection, and so less tissue damage, scarring and pain.
"It's an attempt to minimize the amount of discomfort a patient suffers after surgery," Belzberg says.
And that, Lash says, is just what he accomplished with her. "The second I woke up from anesthesia I said, 'Oh my God, the pain is gone.' It was miraculous."
Monitoring intracranial pressure is key to preventing brain injury.
Nine-year-old Ryan Kupfer was properly seatbelted in the back of a Volvo station wagon last summer when a fast-moving SUV side-swiped the car. Within seconds, the child's skull was fractured on both sides, both mastoid bones were crushed and his ear was severed. Doctors found multiple broad contusions in the brain and an intraventricular hemorrhage. The boy spent two months in a coma.
To say, five months later, that Ryan is out playing ball would be a lie. But, according to pediatric neurosurgeon Tony Avellino, he's doing "extraordinarily well." He talks, walks with help, is relearning to read and his sense of humor is great. That Ryan was rushed to the Pediatric Trauma Center here, Avellino says, surely contributed to his outcome. The surgeon was able quickly to perform a hemicraniectomy for brain-swelling. But Avellino also credits the boy's unusual progress to details like aggressive intracranial monitoring.
"You need to keep intracranial pressure low," Avellino says, "to lessen the chance of secondary injury." That sort of damage appears as insulted neurons and glia secrete a wash of excitatory neurotransmitters or other toxic substances in brain tissues. The result can be diffuse swelling of the braina response more common in children than adultsand it can come fast.
In cases like Ryan's, Avellino is quick to implant continuous intracranial pressure monitors and alert the staff to keep a constant watch on the digital readings. Otherwise there can be a likelihood of fluid retention in the brain. "If that occurs, you can treat kids with hyperventilation, a better tactic than standard drug approaches for lowering intracranial pressure," Avellino says.
Nobody fully understands why intracranial pressure climbs in the first place in brain-injured children, but Avellino suspects that secondary injury somehow disables the system that keeps brain pressure on an even keel. "We can't stop the primary injury," he says. "Cut the spinal cord and it's cut. But physicians can make a dramatic difference in secondary injury if we know how brain chemistry shifts to cause a bad outcome." Avellino, who's new to Hopkins, aims to introduce the art of accurate prognosis for children with head trauma, something he says will come when the biology's clear.
In operating on 11-year-old Elizabeth Healy, Steve Yang faced more challenges than even he is used to.
For Mary Beth Healy, her deepest prayers seemed answered eight years ago. Her 3-year-old daughter Elizabeth was pronounced cancer-free after surgery for a malignant brain tumor, and then made it through an aggressive regimen of chemotherapy. But the story wasn't over.
For a while, Elizabeth appeared the picture of health. Then, gradually, she began to have problems breathing. Over the next few years, the child went steadily downhill until during the summer of 2000 on a family outing at the beach, her lips turned purple and she seemed to stop breathing. At 11, Elizabeth was diagnosed with an irreversible scarring of the lungs called pulmonary fibrosis, probably stemming from the toxic chemicals she'd taken during chemotherapy. Unless she received two new lungsand soon, doctors saidshe would die.
Specialists at a national transplant center put Elizabeth on a list for a lung, but doubted she would survive the year or two it would take to find a donor organ. Surgeons considered using parts of lungs from a family member, but that became a dead end when no one proved a good match. Then, last fall, friends of the Healys tuned into the ABC television documentary "Hopkins 24/7" and watched pulmonary surgeon Stephen Yang do a double-lung transplant on a teenage patient. Within days, Elizabeth was at Hopkins with Yang.
When Steve Yang met his deathly ill patient, he too knew that there was a good chance she wouldn't live long enough to make it to the OR for a transplant. By April, in fact, she had become so oxygen-starved she was rushed to the emergency room. Then, miraculously, two hours later, lungs became available from a child killed in an automobile accident.
But, still there were huge problems. The donor lungs were too large for Elizabeth's chest, and the upper lobe of the left lung was too bruised from the accident to use. Significant scarring in Elizabeth's lungs also meant that Yang would have to dissect the diseased tissue off her chest wall and the lymph nodes off her arteries so he could visualize the hookups for the new lungs. All this would have to be accomplished within the six-hour window called the ischemic time, in which donor lungs remain healthy enough to transplant.
But as Elizabeth lay connected to the heart lung machine, Yang says, "the surgery went like clockwork." Neatly stitched into her body, the new lungs had none of the rejection problems that so often result when the body's massive blood flow enters the new organs.
Recalling the surgery now, more than eight months later, Yang admits he faced more problems than even he is used to: "Elizabeth was much sicker than most patients," he says. "And we had to use marginal organs, but everything worked perfectly."
Today, Elizabeth Healy who once lay close to death is a normal, robust 11-year-old. She rides her bike; she rollerblades; she swims. And to her mother, Mary Beth, she is a living miracle.
Orthopedic surgeon Andrew Cosgarea recalls a 29-year-old computer programmer who injured his anterior cruciate ligament, or (ACL), while playing soccer one Saturday. The ligament, which connects the thigh- bone to the top of the shin, controls the knee's stability, so this weekend warrior quickly elected surgery to fix the damage. Months after the operation, though, the young man's knee again started to give out. This time he made an appointment with Cosgarea, a sports medicine specialist who's known for "revision reconstructions"fixing what the first operation failed to repair.
Unbeknown to the original surgeon, this patient also had a torn meniscus, the knee's shock absorber. "Even though the original ACL reconstruction probably was done appropriately, the fact that a second ligament had been damaged made the operation fail," explains Cosgarea. But The second problem also could have occurred when the reconstructed ligament re-ruptured, stretched and broke loose at the two ends from the fixation.
Restoring stability in the knee in such situations is no easy chore. The first surgeon had drilled a tunnel into the ends of the thigh-bone and shin and grafted in tissue from the patellar tendon below the kneecap. The graft was secured by wedging a screw between the side of the bone and the tunnel.
A revision reconstruction becomes more complicated, because knee ligaments that contribute to the failure of a first operation must be reconstructed and new graft tissue harvested from the quadricep, hamstring or patella tendons. Cosgarea takes care to look at the patient's normal level of activities as well as problems that could develop from a particular graft source. In knee operations, he says, a surgeon has to do a high volume of procedures and know a variety of techniques. In this patient's case, everything worked. He's back on the soccer field.
Until recently, interferon was the only treatment for hepatitis C, a blood-borne inflammatory disease that may scar and even shut down the liver, killing about 10,000 people every year. The protein has been effective in reducing hep-C blood levels substantially within 24 hours after the first dose, but frequent dosing-three injections a week over six months to a year-is needed because interferon leaves the body fairly quickly. Also, in the long-term it works on only 20 to 30 percent of patients, and carries some serious side-effects, including chronic fatigue and depression.
But a modified form of interferon now being tested here appears significantly more effective than its predecessor, and has fewer complications. Named pegylated interferon after polyethylene glycol, a molecule that is attached to standard interferon, the treatment is designed to stay in the body longer.
"After a dose of standard interferon, the virus has a chance to recover," says infectious diseases specialist David Thomas. "But one dose of pegylated interferon stays around in the blood and keeps the virus under control the whole week-long."
In a recent two-year study of more than 500 patients, Thomas explains, pegylated interferon was nearly twice as effective as standard interferon. After treatment, the virus was undetectable in 50 percent of the patients.
"We've followed patients for years after stopping treatment and the virus does not come back," says infectious diseases specialist Mark Sulkowski. "This therapy can result in the eradication of hepatitis C."
Christine Jablonski had just finished breakfast when a dark screen dropped over her right eye. At least, that's how it felt to the 61-year-old nurse. A small blood clot had occluded Jablonski's central retinal artery, reducing her vision in seconds to a shadowy realm of light and dark.
"Most patients with 'ocular stroke' experience lasting blindness of the entire eye or most of it," says neurologist Eric Aldrich who treated Jablonski. Fortunately for her, however, an ophthalmic exam in the emergency department quickly diagnosed central retinal artery occlusion (CRAO). Jablonski had a neuro exam to rule out brain stroke and, shortly after her arrival, received a targeted injection of tPA, the famed anti-clotting agent.
Aldrich then teamed with interventional neuroradiologist Kieran Murphy who fed a catheter into Jablonski's carotid artery, switching to a spaghetti-thin microcatheter at the narrower ophthalmic artery. He released a drop of tPA solution from its tip every three minutesa continuous but low tPA exposure to lessen complications. Jablonski's vision began to return while she was on the angiography room table. Now, six months later, her sight is virtually normal.
"Curing this form of blindness is the most amazing thing," says Aldrich. "You feel like you're raising up Lazarus." In the last six months, the team has done several of the new approaches, with encouraging results in patients who've arrived during the proper "window of opportunity."
Two years ago, Mark McGhee, a 42-year-old Delaware auto mechanic, had just opened a beer at a picnic when his face suddenly became numb on one side. His wife, who thought he was having a stroke, rushed him to the nearest emergency room, where ER doctors said they thought he had Bell's Palsy, a paralysis of facial nerves that usually resolves itself. So far, no one had come up with the right diagnosis.
McGhee's symptoms didn't go away. They got worse: he began to experience problems swallowing. Luckily, he had a good physician and this was an alert. "'Mr. McGhee, I think you have Wegener's Granulamotosis, and if we don't get you in the hospital right now you're going to die,'" McGhee says his doctor told him.
Wegener's is a rare but lethal inflammatory disease that attacks the upper respiratory track, lungs and kidneys. Although high-dose steroids and the anti-cancer drug cyclophosphamide are effective short-term remedies, they have strong side-effects like bladder damage and increased risk of cancer. Worse, there has been no known drug that would keep the disease at bay for the long haul. As patients are tapered off the traditional toxic treatments, their symptoms flare and they begin another high-dose cycle.
But, for McGhee there was promising news: Hopkins had just begun a clinical trial of a potential new treatment with fewer side-effects. The therapy, called Etanercept, works by inhibiting a protein called tumor necrosis factor, or TNF, which plays an important role in many forms of inflammation. A week later, McGhee met with rheumatologist John Stone of the Vasculitis Center.
"Etanercept seems to work by soaking up this protein, which we believe is central to the inflammation in Wegener's," Stone says. The drug has been shown to be safe in treating rheumatoid arthritis, a similar systemic inflammatory disease.
In the Etanercept study, the first randomized trial on Wegener's in the United States, all patients receive the standard therapies, which are tapered off at six months. They then receive either Etanercept or placebo. McGhee, who has completed one year of the study, is now off steroids and cyclophosphamide. Although some symptoms linger, he says, "I feel a whole lot better."
An agonizing rectal hernia is one of the most disruptive conditions a woman can experience. A bulging pouch in the rectum (rectocele), the hernia can be the result of aging or pregnancy. It traps stool and pushes it into the back wall of the vagina, causing chronic constipation and painful intercourse.
Traditional surgery has been helpful in reducing the protrusion symptoms, says gynecologist Geoffrey Cundiff, but in many women it doesn't get rid of the constipation and sexual discomfort. That's because in conventional approaches surgeons simply stitch together muscles from the sidewalls of the pelvis, creating a retainer wall at the back of the vagina to keep the rectum from bulging. But they don't repair the problem itself.
"The standard procedure doesn't really fix the support defect, the tear in the connective tissue between the vagina and the rectum that causes the hernia," Cundiff explains.
Now, Cundiff has developed a technique that goes after the tear. Through an incision in the back wall of the vagina, he separates the skin from the underlying tissue until he uncovers the actual hernia. He then pushes the pouch back into the rectum and sutures it shut. For patients with recurring hernias, Cundiff may reinforce the repaired hernia with a tissue graft, usually from a pig's intestine.
"The problem with the traditional repair is not one of durability, it's a problem of not relieving the physical problems the hernia is causing," Cundiff says. "With this repair we hope to avoid those symptoms by restoring normal anatomy."
Even with advances in mammography, breast cancer in young women is difficult to detect because their breast tissue tends to be very dense. But because most breast cancers begin in the lining of the milk ducts, a new test developed at Hopkins may do what mammography often can't do-pick up very early forms of breast cancer in these younger patients who need a close examination.
In the test, saline is injected into the ducts through a tiny catheter inserted into the nipple and then flushed out-along with duct cells. Then, like cervical cells from a Pap smear, these cells are analyzed under a microscope for signs of cancer. Researcher Saraswati Sukumar, who developed the molecular test, looks specifically for signs of methylation, a process that silences genes that normally suppress tumors.
"Methylation," Sukumar says, "essentially puts the brakes on the promoter, or driver, of the gene, so that the tumor-suppressor genes are not expressed."
In one study, Sukumar notes, 17 of 20 women with invasive breast tumors and two of seven with localized cancers had traces of methylation in their duct cells. In another experiment, methylated genes showed up in five, or 11 percent, of 45 women with no breast lumps apparent on mammogram. Two of the women went on to develop cancer.
"We were able to show that our test is extremely sensitive," Sukumar says, "even in patients shown to be tumor-free by mammography and clinical examination."