Dawnelle Gonzalez, 35, remembers vividly the day she developed her incurable, painful neurologic condition: October 15, 2008—the day her youngest daughter, Abigail, was born. Gonzalez received an epidural during the birth. However, when the anesthesiologist inserted the needle, a complication occurred. The anesthetic was delivered directly into the fluid bathing the spinal cord and spinal nerve roots.
The procedure triggered inflammation and extreme pain and eventually left her unable to care for herself or her children, or to return to her job as a preschool teacher after her maternity leave. She spent three years bouncing from doctor to doctor searching for an answer. Finally, Gonzalez—who lives in Simpsonville, SC—found one of the few physicians in the country who specializes in her condition: Antonio Aldrete, MD, professor emeritus of the department of anesthesiology at the University of Alabama–Birmingham. Dr. Aldrete diagnosed her with arachnoiditis, chronic inflammation of one of the three membranes (meninges) that protect the brain and spinal cord (the central nervous system) and the nerve roots, which are the part of nerves that rest within the spine. (The nerve roots exit the spine to form nerves of the peripheral nervous system, which controls movement and provides sensation to the limbs.)
Arachnoiditis is considered a rare disease, affecting an estimated 11,000 people a year in the United States, although it is likely underdiagnosed, according to the National Organization for Rare Disorders. It is nearly always caused by some kind of medical procedure, and it has no cure.
The "Spider" membrane
Three membranes cover the brain and spinal cord, one on top of the other: the pia mater, the arachnoid, and the dura mater. The arachnoid forms the "filling" of this meninges sandwich, with tiny fibers that resemble a spider's web attached to the pia mater below. The space between the pia mater and the arachnoid is called the subarachnoid space. It contains blood vessels and allows clear passage of spinal fluid—a clear, colorless liquid that bathes the spinal cord and brain, circulating nutrients and chemicals filtered from the blood and removing waste products from the brain.
If the spinal fluid becomes infected, immune cells enter the arachnoid and the spinal fluid and try to eliminate the infection through inflammation. This inflammation can affect the nerve roots, which begin clumping and sticking to the inner layer of the arachnoid as a result. The arachnoid then also thickens and scars, compressing the nerve roots and blood supply and affecting the flow of spinal fluid around the spinal cord.
Before antibiotics, severe cases of tuberculosis and syphilis were causes of arachnoiditis. Today, steroid injections, infections such as meningitis, back surgery, spinal epidurals during childbirth, and the dye used in myelograms (an X-ray test occasionally used to diagnose spinal abnormalities) are common causes. An oil-based dye used in early myelograms, Pantopaque, triggered an outbreak in the 1970s before it was removed from the market. However, even the water-based dyes used today can cause arachnoiditis.
One study of 300 patients with arachnoiditis found that about two-thirds developed the condition as a result of a myelogram. The rest developed the condition from steroid injections, spinal trauma, or spinal surgery, although some patients underwent more than one procedure.
As the inflammation continues, patients experience burning pain in their back and legs, as well as dizziness, intense headaches, and skin sensations that feel like crawling bugs or dripping water. "Other symptoms include urinary incontinence and bowel problems like those Ms. Gonzalez experienced," says Dr. Aldrete. "Most patients are unable to have sex because it causes severe pain."
"My Body Just Wasn't Right"
As soon the epidural needle entered her spine during labor, Gonzalez knew something was wrong. "I was expecting pain. I'd never had any kind of back procedure or epidural before," she says. But she wasn't expecting the intense pain that shot down her leg as the needle went deeper into her back. Finally, she screamed. She recalls the anesthesiologist yelling at her, "Why didn't you tell me I missed?" He quickly withdrew the needle and reinserted it to deliver the anesthetic. Several hours later, Gonzalez delivered a healthy, 9.5-pound baby.
Within a few hours after the epidural, however, she started shaking uncontrollably. Her blood pressure spiked, remaining high during her entire hospital stay. She also began experiencing terrible pain, including unbearable headaches every time she moved. She was in so much pain she couldn't care for her daughter, who remained in the nursery during Gonzalez's two-day stay. "I cried a lot," she says.
Gonzalez also had trouble walking. "I felt like my hips were dislocating. I had raw nerve pain every time I lifted my leg," she says. She left the hospital in a wheelchair, not because it was hospital policy, but because she couldn't walk without severe pain. She—and the nurses—blamed her symptoms on the fact that her daughter weighed nearly 10 pounds.
Over the next few months, the hip pain improved, but the back pain worsened. She developed urinary incontinence. "My body just wasn't right," she says. "Whenever I did anything physical, I felt a burning, raw, fiery nerve pain in my back."
Doctors treated her for numerous urinary tract infections and even pelvic inflammatory disease, a reproductive infection. Eventually, however, the muscles in her bladder stopped working. She retained so much urine that she developed a serious blood infection. She was hospitalized and learned to catheterize herself. By now, Gonzalez was dizzy and in constant pain; she fell often, once while picking up the baby.
Her urologist determined that the bladder problems were neurologic and sent her to a neurosurgeon. "He told me I was overweight and recommended epidural steroid injections," Gonzalez recalls. She had three, which only made the pain and other symptoms worse. In fact, it is dangerous to inject anything into the spinal area in someone with arachnoiditis because it can make the condition worse.
Another neurosurgeon suspected a herniated disc but also mentioned the possibility of arachnoiditis. That's when her mom and aunt stumbled upon Dr. Aldrete. He had been studying and treating patients with the condition for more than 15 years. He also wrote the only textbook available on the condition.
Gonzalez's years-long search for a diagnosis is not unusual, according to Dr. Aldrete. "Some of my patients have seen up to 15 doctors and lived with the condition for a dozen years before they are diagnosed," he says. They are often misdiagnosed with failed back surgery syndrome, complex regional pain syndrome (a chronic pain condition that usually affects the arms or legs), fibromyalgia, slipped discs, sciatica, and other back-related issues.
Contaminated Steroids Trigger Arachnoiditis Outbreak
Arachnoiditis became national news in September 2012, when reports of fungal meningitis and other infections began surfacing. The Centers for Disease Control and Prevention (CDC) traced the infections to contaminated corticosteroid injections, used to relieve back and joint pain. The drug came from a Framingham, MA, compounding pharmacy, which has since closed. By the time the outbreak petered out a year later, the CDC determined that about 14,000 people had been exposed via injections of the steroid methylprednisolone from the Framingham pharmacy. Of those, 751 people in 20 states experienced fungal infections and 64 died.
About a fourth of those cases were in Michigan, primarily Ann Arbor, which is how the infectious diseases physicians at St. Joseph Mercy Hospital found themselves diagnosing and caring for about 40 patients with arachnoiditis. All had received injections in the epidural space in the spine to relieve back pain. The steroids, according to Carol Kauffman, MD, an infectious diseases specialist at the Veterans Affairs Ann Arbor Healthcare System and the University of Michigan, acted like fuel to the fungus, providing the perfect growth environment.
Prior to the contaminated steroid outbreak, she said, she'd only seen one case of arachnoiditis due to fungal infection in her career.
The physicians at St. Joseph Mercy Hospital treated patients with powerful antifungal medications for the arachnoiditis. "Some patients have gotten better, which is exciting, but some still have symptoms of nerve damage a year and a half later, unfortunately," Dr. Kauffman says. A few underwent surgery. "But this can be very difficult to do, and most did not," she says.
In late April 2014, the Food and Drug Administration ordered that all injectable corticosteroids carry warning labels highlighting the risk of death, paralysis, stroke, nerve injury, and meningitis, among others.
Dr. Aldrete diagnoses the condition based on the patient's symptoms, a thorough medical history, and magnetic resonance imaging (MRI).
Arachnoid Cysts
Another condition that affects the arachnoid membrane is arachnoid cysts. They can be present at birth or the result of surgery or another medical condition, such as meningitis. Arachnoid cysts usually do not cause symptoms and may only be found by accident on magnetic resonance imaging (MRI) for other conditions. If they grow, however, they can cause pain, numbness, nausea and vomiting, and dizziness, among other symptoms. Left untreated, they can result in serious neurologic damage including paralysis and severe pain. For symptomatic arachnoid cysts, surgery may be considered.
Individualized Treatment
In his book, Arachnoiditis: The Evidence Revealed, Dr. Aldrete lists more than a dozen classes of drugs and individual medications that have been used to treat the chronic pain of arachnoiditis. They include traditional drugs prescribed for chronic pain, such as opioids, non-steroidal anti-inflammatories, anticonvulsants, muscle relaxers, and antidepressants; medications such as etanercept and adalimumab, used to treat some autoimmune conditions; the insomnia medication zolpidem; and magnesium. Some physicians have reported successful surgeries to "clean out" the arachnoid space, but they are rare. Unfortunately, not much evidence from well-controlled clinical trials has been collected to support the use of these treatments.
Patients generally need multiple forms of treatment: medications to control the pain, physical therapy or exercise to restore motion and preserve function, mild massage, hot and cold compresses, and psychotherapy for the depression that often accompanies the condition.
"We are on the orphan disease list," Gonzalez says, "so we are kind of stuck hanging without a set treatment plan." Orphan diseases are those that affect fewer than 200,000 people in the U.S. With so few patients, it is often difficult to conduct the kind of clinical trials needed to identify treatments. Indeed, no clinical trials of treatments for arachnoiditis are currently taking place.
Dr. Aldrete individualizes treatment for every patient. With Gonzalez, for instance, he used a five-day infusion of low-dose steroids, among other medications. "It seemed to immediately put out the inflammation and fire," she says. "I was able to walk again after the first dose."
He then referred her to a neurologist, who treated her for a year with the opioid hydrocodone. She also had twice-weekly infusions with the anesthetic ketamine, which can be effective for certain chronic pain conditions. It worked well, Gonzalez says, but then the clinic in her area stopped offering it and her insurance company stopped paying for it. Today, she manages the pain with a fentanyl patch. Even then, however, her pain rarely goes below a seven on the 10-point pain scale. She still needs a wheelchair or walker to get around.
Once she learned about her condition, Gonzalez started an advocacy group: the Arachnoiditis Society for Awareness and Prevention (ASAP), which has more than 2,000 followers on Facebook. She also started a support group, Arachnoiditis Everyday, with more than 500 followers. They share stories about what works for them, such as topical lidocaine; transcutaneous electrical nerve stimulation (TENS), in which electricity stimulates the nerves; and physical therapy programs designed for people with spinal cord injuries.
The most important thing, Gonzalez says, is to take life one day at a time. "You have to learn to set limits and do things differently," she says. "You have to learn to accept that there is no cure."
For More Information
- For those affected by the contaminated steroid meningitis outbreak, visit the Meningitis Outbreak group on Facebook.
- Arachnoiditis Everyday, the Facebook support group founded by Dawnelle Gonzalez
- Arachnoiditis Society for Awareness and Prevention (ASAP), also founded by Gonzalez
- More coverage of arachnoiditis in Neurology Today