Brooke was solely three years previous when she first realized about desmoid tumors. “My mom had six or seven,” she remembers. Brooke’s mom additionally had 28 surgical procedures associated to her desmoid tumors and a number of rounds of radiation and chemotherapy, which have been the one remedy choices out there for her on the time.
A number of years in the past, Brooke started her personal well being battles when she had a mass the dimensions of a lacrosse ball in her breast eliminated; solely after two surgical procedures did she study it was a desmoid tumor.
In March 2023, Brooke—then 40 years previous—felt a pointy ache in her stomach. Regardless of her earlier desmoid tumor analysis, she initially suspected one thing else. “I believed it was my gallbladder,” she explains.
The extraordinary ache prompted Brooke to go to the ER the place docs concluded that she had a hematoma (a pool of principally clotted blood) as a result of she has hemophilia (a uncommon genetic blood dysfunction). “Regardless that it was aggressive and saved rising, I used to be denied care as a result of they mentioned it was a hematoma,” she says.
This dismissal went on for eight months.
Ultimately, an MRI revealed that what docs beforehand thought was a hematoma was in reality a desmoid tumor the dimensions of a big grapefruit. “I appeared like I used to be eight months pregnant,” Brooke says. She even named her desmoid tumor “Large Bertha.”
Related story
4 Ways Apple Gift Cards Can Deliver Subscriptions the Whole Family Can Enjoy
What are desmoid tumors?
Desmoid tumors—generally known as aggressive fibromatosis or desmoid fibromatosis— are rare, locally aggressive tumors that kind within the connective tissues of the physique. Whereas they don’t unfold from one a part of the physique to a different, these tumors have invasive, tendril-like growths and may wrap round tissue and compress muscle mass, vessels, nerves, and organs. “These little suckers are like little octopus tentacles,” Brooke explains.
Desmoid tumors could cause severe pain, limited function and mobility, disfigurement, and could be debilitating for individuals dwelling with them. In rare cases, when vital organs are involved, desmoid tumors can be life-threatening.
Desmoid tumors are mostly recognized in individuals 20 to 44 years of age, and girls are two to three times more likely to be recognized than males.
Sadly, Brooke’s challenges receiving an correct analysis aren’t out of the strange: roughly 30 to 40% of desmoid tumors are initially misdiagnosed.
In one study, the time from onset of signs to analysis was over a yr for greater than half of the sufferers. Throughout this time, they’re left dwelling with ache and different debilitating signs, identical to Brooke.
Among the bodily symptoms of desmoid tumors can embrace:
● Ache
● Restricted vary of movement
● Fatigue
● Impaired mobility
How are desmoid tumors handled?
Quickly after studying in regards to the desmoid tumor in her stomach, one other desmoid tumor developed close to her pelvis that was compressing her bladder and inflicting incontinence.
Brooke was cautious of getting surgical procedure to take away her tumors. “I had post-op points with my earlier surgical procedures,” she says. “It was very traumatic for my physique and regardless of the surgical procedures, the tumors grew again.” Actually, as much as 77% of individuals with a desmoid tumor can expertise recurrence following surgical removing.
Immediately, specialists and remedy guidelines¹ advocate medical remedy as a first-line lively remedy possibility for desmoid tumors which might be rising and/or symptomatic in most tumor areas.
Discovering an FDA-approved remedy
When Brooke started exploring remedy choices in September 2023, there wasn’t but an FDA-approved drugs for desmoid tumors. Her healthcare group instructed her a couple of new remedy being thought of for approval by the FDA known as OGSIVEO® (nirogacestat). Within the meantime, they urged beginning chemotherapy, an off-label remedy.
“My physician requested if I wished to begin chemotherapy or await OGSIVEO,” Brooke remembers. “I selected to attend as a result of it was particularly studied for desmoid tumors.”
Brooke started taking OGSIVEO on December 30, 2023, only a month after it was authorised by the FDA for adults with progressing desmoid tumors who require systemic remedy. With this remedy, her tumors shrank, and he or she additionally skilled a lower in her ache.
Brooke additionally appreciates that OGSIVEO is a tablet that may be taken at dwelling. “I wouldn’t be capable to do it if I needed to go to the hospital each time,” she says, noting that as a single guardian, she doesn’t have that sort of time.
Brooke felt it was vital to share her story and expertise with others. “That is what my mother wished—extra consciousness for desmoids, as a result of there isn’t sufficient,” she says.
Be taught extra about this FDA-approved remedy possibility at OGSIVEO.com.
That is Brooke’s private expertise solely and particular person outcomes might differ.
Necessary Security Info
What’s OGSIVEO?
OGSIVEO is a prescription remedy used to deal with adults with progressing desmoid tumors who require a drugs by mouth or injection (systemic remedy). It isn’t identified if OGSIVEO is secure and efficient in youngsters.
Earlier than taking OGSIVEO inform your healthcare supplier about all your medical situations, together with in case you:
● Have liver issues.
● Are pregnant or plan to turn into pregnant. OGSIVEO can hurt your unborn child. Inform your healthcare supplier in case you turn into pregnant or suppose you might be pregnant throughout remedy.
● Females who’re capable of turn into pregnant:
● Your healthcare supplier will provide you with a being pregnant check earlier than you begin remedy with OGSIVEO.
● It is best to use efficient contraception throughout remedy and for 1 week after the final dose. Speak to your healthcare supplier about strategies which may be best for you.
● Cease taking OGSIVEO and inform your healthcare supplier straight away in case you turn into pregnant.
● Males with feminine companions who’re capable of turn into pregnant ought to use efficient contraception throughout remedy with OGSIVEO and for 1 week after the final dose.
● Are breastfeeding or plan to breastfeed. It isn’t identified if OGSIVEO passes into your breast milk. Don’t breastfeed throughout remedy with OGSIVEO and for 1 week after the final dose.
Inform your healthcare supplier about all of the medicines you’re taking, together with prescription and over-the-counter medicines, nutritional vitamins, and natural dietary supplements.
It is best to keep away from taking proton pump inhibitors (PPIs) and H2 blockers throughout remedy with OGSIVEO. Ask your healthcare supplier if you’re unsure in case you take one among these medicines.
How ought to I take OGSIVEO?
● Take OGSIVEO precisely as your healthcare supplier tells you to take it.
● Your healthcare supplier might change your dose, briefly cease, or completely cease remedy with OGSIVEO in case you develop unintended effects.
● Take OGSIVEO 2 instances a day with or with out meals.
● Swallow OGSIVEO tablets complete. Don’t break, crush, or chew.
● In case you take an antacid drugs, take OGSIVEO 2 hours earlier than or 2 hours after the antacid.
● In case you vomit after taking a dose or miss a dose of OGSIVEO, take your subsequent dose at your common time. Don’t take 2 doses of OGSIVEO to make up the dose.
What ought to I keep away from whereas taking OGSIVEO?
Keep away from consuming or ingesting grapefruit merchandise, Seville oranges, and starfruit throughout remedy with OGSIVEO.
What are the attainable unintended effects of OGSIVEO?
OGSIVEO could cause severe unintended effects, together with:
● Diarrhea. Diarrhea is frequent with OGSIVEO and should generally be extreme. Your healthcare supplier might let you know to drink extra fluids or to take antidiarrheal medicines. Inform your healthcare supplier straight away when you have diarrhea that lasts longer than a number of days and doesn’t get higher after taking antidiarrheal medicines.
● Ovarian issues. Females who’re capable of turn into pregnant might have ovarian issues and adjustments of their menstrual cycle throughout remedy. OGSIVEO might have an effect on fertility which can have an effect on your means to have a baby. Inform your healthcare supplier when you have any adjustments in your menstrual cycle or sizzling flashes, evening sweats, or vaginal dryness throughout remedy.
● Liver issues. OGSIVEO can improve liver enzymes. Your healthcare supplier will do blood assessments to examine your liver operate earlier than you begin and through remedy with OGSIVEO.
● New non-melanoma pores and skin cancers. Your healthcare supplier will do pores and skin exams earlier than and through remedy with OGSIVEO if you’re in danger for pores and skin most cancers. Inform your healthcare supplier when you have any new or altering pores and skin lesions.
● Electrolyte (salt) issues. Your healthcare supplier will do blood assessments to examine your phosphate and potassium ranges throughout remedy and should provide you with medicines to deal with low phosphate or low potassium if wanted. Inform your healthcare supplier in case you develop any muscle ache or weak point.
Inform your healthcare supplier straight away when you have any facet impact that bothers you or that doesn’t go away. Your healthcare supplier might change your dose, briefly cease, or completely cease remedy with OGSIVEO.
The most typical unintended effects of OGSIVEO are:
● rash
● nausea
● tiredness
● mouth sores
● headache
● abdomen (stomach) ache
● cough
● hair loss
● higher respiratory an infection
● shortness of breath
OGSIVEO can have an effect on fertility in females and males, which can have an effect on your means to have a baby. Speak to your healthcare supplier if this can be a concern for you.
These aren’t all the attainable unintended effects of OGSIVEO. Name your physician for medical recommendation about unintended effects. Chances are you’ll report unintended effects to the FDA at 1-800-FDA-1088.
Please click here for full Prescribing Info together with Affected person Info.
OGSIVEO is a registered trademark of SpringWorks Therapeutics, Inc. CORP_OGS_US_0027 10/24
Reference
¹Referenced with permission from the NCCN Scientific Observe Pointers in Oncology (NCCN Pointers®) for Comfortable Tissue Sarcoma V.3.2024. © Nationwide Complete Most cancers Community, Inc. 2024. All rights reserved. Accessed October 24, 2024. To view the newest and full model of the rule of thumb, log on to NCCN.org. NCCN makes no warranties of any form by any means concerning their content material, use or software and disclaims any accountability for his or her software or use in any manner.
