Ten years and four diagnoses later, patient gets her life back
Rachel Hale knew her adolescence was unusual. The headaches, the nausea, the dehydration, the blood draws leaving scars on her arm — this wasn’t what most other kids her age were going through.
By the time she was 24, Hale was on her fourth diagnosis and had been on headache medication for years. Her condition prevented her from participating in sports and social events — sometimes she couldn’t even go out to eat with friends. She had bounced from physician to physician and hospital to hospital, without much relief.
Then, in November 2017, when Hale was at Stanford Hospital, she met Linda Nguyen, MD, a clinical associate professor of gastroenterology and hepatology at the Stanford School of Medicine who was assessing Hale’s gastrointestinal issues.
Nguyen had recently heard from Ian Carroll, MD, a headache and orofacial pain specialist at Stanford. Carroll had shared information with her about cerebrospinal fluid leaks: He said the leaks were characterized by chronic, intractable nausea in addition to ringing in the ears, vomiting and headaches.
When Nguyen contacted Carroll about Hale, he immediately took an interest in her case. He ran scans and imaging, and diagnosed a CSF leak. “I don’t think I’ll ever forget doing the first diagnostic test where he just had me lie flat,” recalled Hale. “It was the first time my headache has ever gone away, and it was a huge ‘Aha!’ moment for me.”
A patch made from blood
A CSF leak occurs when the meninges — a covering that protects the brain and spinal cord and holds the cerebral fluid in place — forms a tear, allowing the fluid to escape. CSF leaks can occur spontaneously, but people with Marfan and Ehlers-Danlos syndromes, both connective tissue disorders, are at the highest risk.
To treat the CSF leak, Carroll performed an epidural blood patch. In the procedure, the patient’s own blood is injected into the meninges, creating a seal over the tear in the covering.
After her first blood patch, Hale improved substantially, and Carroll was confident they were on the right path.
“We want multiple dimensions across her life to improve so she can return to function and do the things a young woman wants to do instead of seeing doctors all the time,” said Carroll, who is also an assistant professor of anesthesiology, perioperative and pain medicine at the School of Medicine.
Carroll believes that CSF leaks may be more common than anyone thought, and often misdiagnosed. He speaks from personal experience: His daughter had a CSF leak that had gone undiagnosed. That’s when he began to think about the interplay of different syndromes and symptoms, and how important it was for a major academic medical center like Stanford Health Care to collaborate across departments.
Orthostatic headache, or headache that is worse when upright, is a key feature of a CSF leak, but is also a common feature in patients with postural orthostatic tachycardia syndrome, or POTS.
Targeting patients at risk
“Patients shouldn’t have to wander from doctor to doctor until they randomly interact with one who happens to know that some people are at greater risk of developing a leak. After the personal experience I had with my daughter, I started reaching out to the Stanford Headache Clinic as well as the POTS and Marfan clinics. Now we’re all reading about these leaks and it has created a great dialogue,” said Carroll.
Hale laughed when Carroll insisted she watch three videos and read a paper about her procedure before he would even talk to her. But in the end, his advice was right: “He involved me a lot and kept me updated, which I really, really appreciated,” she said.
Hale’s headaches aren’t as common as they once were, and the sensory overload feelings are subsiding. She will continue to receive the blood patch procedures to keep her symptoms under control.
“This blood-patching thing, from a nerdy, scientific perspective, I think is so fascinating,” she said. “I love it.”