Music is liberating for Beverly, who writes and plays songs about finding the strength to start over. “I’ve had to recreate myself,” she says, calling music her key to survival. For Beverly, nothing is more physically and emotionally healthy than creativity.
For most of her adult life, Beverly had unexplained and sometimes severe attacks of abdominal pain. “At one point I thought I was going to die,” she says. “I was picking out songs for my funeral.”
Over 7 years she saw dozens of specialists before receiving an accurate diagnosis of acute intermittent porphyria (AIP). AIP is an inherited disorder involving overproduction and buildup of natural chemicals called porphyrins in the liver. Symptoms of AIP attacks often include severe abdominal pain, vomiting, constipation, rapid heart rate, and pain or weakness in many areas of the body. Because these symptoms are common to many other diseases, AIP can be challenging to diagnose.
The roller coaster begins
Beverly first had symptoms of AIP while working for a major medical device company. She saw herself as a “right-fighter” whose mission was to ensure the safety of surgical devices. Despite long hours, she loved her job.
Over time Beverly’s symptoms got worse, and she started to miss work. After coping with pain on and off for nearly a year, Beverly had a severe attack with abdominal pain, vomiting, flu-like symptoms, and tingling sensations. When the symptoms didn’t go away, she ended up in the hospital, undergoing exploratory surgery and various diagnostic tests, and on medical leave from her job. “At that point it all just came crashing down,” she says.
Through it all, Beverly was determined to get a diagnosis. Unfortunately, none of the nearly 30 specialists she saw tested her for AIP. She began to think of her disease as a monster that was stripping away her career, friendships, relationships, and even her identity.
Just when Beverly thought things couldn’t get worse, she got a devastating—but inaccurate—diagnosis: peritoneal cancer. With her life turned upside down, she returned home to live with her mother and prepare for the worst. She struggled for two more years before returning to a hematologist she had seen previously. This time, the doctor expanded his range of tests. The results were conclusive: AIP. Beverly finally had the answer that had eluded her for so many years.
The “right fighter” returns through activism and music
Most people get upset when they are diagnosed with a rare disease, but Beverly’s not most people. Because with the diagnosis of AIP came the possibility of treatment. Beverly’s doctor told her about Panhematin® (hemin for injection), the only approved therapy to treat repeated attacks of AIP in affected women. Beverly’s decision to begin treatment changed her life. “When pain and extreme swelling would begin in my abdomen, I would rest in bed for days at a time in search of relief,” she says.
Although living with AIP has forced Beverly to redefine herself, she continues to be a “right fighter.” When an animal shelter in her hometown was exposed for serious infractions, she rallied her community online and urged local officials to take action. “Having AIP doesn’t change who I am or my core beliefs,” she says.
Spreading award-winning messages of hope and inspiration
While there are times when she misses her corporate career, Beverly has rediscovered herself through activism—and also through music. Music helps Beverly live with her AIP and connect with others in the rare disease community. She was a finalist in the 2014 International UK Songwriting Contest, and her songs have earned 11 semi-finalist spots in the 2015 competition.
Beverly wants to inspire others with her music, including people living with AIP. “It feels like I’m being heard,” she says. A real triumph after so many years of feeling like no one was listening!
Indications And Usage
PANHEMATIN is a hemin for injection prescription medication used to relieve repeated attacks of acute intermittent porphyria related to the menstrual cycle in affected women, after initial carbohydrate therapy is known or suspected to be inadequate.
Limitations of Use
- Before giving PANHEMATIN, consider an appropriate trial of carbohydrates [i.e., 400 grams of glucose (a carbohydrate or sugar) per day for 1 to 2 days].
- Attacks of porphyria may progress to a point where irreversible nerve damage has occurred. PANHEMATIN therapy is intended to prevent an attack from reaching the critical stage of nerve breakdown. PANHEMATIN is not effective in repairing nerve damage that has already occurred.
Important Safety Information
- PANHEMATIN is not for patients known to be allergic to this drug.
- Vein inflammation is possible. Use a large arm vein or a central line to administer PANHEMATIN to minimize the possibility of vein inflammation.
- Elevated iron levels may occur. Your doctor must monitor your iron levels if you receive multiple courses of PANHEMATIN.
- PANHEMATIN has a passing and mild blood thinning effect. Avoid blood thinners during PANHEMATIN therapy.
- Reversible kidney shutdown has occurred when too high a dose of PANHEMATIN was given in a single dose.
- PANHEMATIN may carry a risk of transmitting agents that can cause infections, such as viruses, and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
- Most common side effects are headache, fever, infusion site reactions, and vein inflammation.
- To report SUSPECTED SIDE EFFECTS, contact Recordati Rare Diseases Inc. at 1-888-575-8344, or FDA at 1800-FDA-1088 or www.fda.gov/medwatch.
- When taking PANHEMATIN, do not take drugs such as estrogens (e.g., oral contraceptives), barbiturates (drugs that help with sleep and used to treat epilepsy) or steroids (body hormone-like drugs), because such drugs can trigger an attack or make an attack worse.
For more information, please see Full Prescribing Information at www.recordatirarediseases.com.
Panhematin is a registered trademark of Recordati Rare Diseases Inc.
You are encouraged to report adverse events to the FDA. Visit www.fda.gov/MedWatch, or call 1-800-FDA-1088.