Bunion-Like Toes
Bunion-Like Toes
Bunion-Like Toes
Bunion-Like Toes
Bunion-Like Toes
Tumor-Like Swelling
Tumor-Like Swelling
Tumor-Like Swelling
Tumor-Like Swelling
Tumor-Like Swelling
Faculty
Christiaan Scott, MBChB: Pediatric rheumatologist, Department of Pediatrics, University of Ottawa, Canada. Michelle Davis: Executive Director, International Fibrodysplasia Ossificans Progressiva Association (IFOPA)
Program faculty members are available for Q&A by teleconference or email. Please note that faculty will only provide educational and informational input. Please email info@p2psyncro.com to schedule. This is provided at no cost.
Takeaways
Can tolvaptan be given once daily
"We don't ever want to give tolvaptan once a day, because then there will be a period of time when vasopressin is not suppressed".
How to counsel patients with nocturia
“To manage the side effects of treatment, I focus on two areas depending on their complaints."
- If they report thirst, headaches, dizziness, or constipation, I assess their fluid intake. I will ask them to calculate exactly what they drink. More often, it's not as much as they think it is.
- If the complaints are polyuria, especially nocturia, I evaluate their diet and medication timing. I encourage them to have a low-sodium, low-protein meal for dinner. I may suggest taking the first dose of tolvaptan at 6AM, so that the second dose, eight hours later, is at 2PM. This often helps reduce nocturia episodes to 1 or 2, which is much less disruptive to sleep
Water bottle in hand or drink in boluses
“Adequate fluid intake is critical in the management of the aquaretic effect from treatment. We found that those who try to give themselves boluses of fluid struggle the most. We recommend keep a water bottle in hand and drink continuously throughout the day”.
Source: Faculty presentation.
Can FOP be diagnosed from the toe and swelling?
In FOP, muscle and connective tissues are gradually replaced by bone. There are 2 cardinal signs.
- Malformed big toes that can be short and curved are present at birth.
- Swellings that look like tumors start to appear in the first decade of life, typically in the shoulder, back, and scalp. They can be warm, painful, and appear episodically. Eventually the swellings will clear up but leave behind a new piece of mature bone.
The diagnosis of FOP is made clinically by associating rapidly appearing mass with great toe malformations.
Source: Faculty presentation and IFOPA www.ifopa.org.
Looks like sarcoma? Why to avoid biopsy?
The swellings in FOP are often misdiagnosed as soft tissue sarcoma. When one cuts into the muscle for biopsy of the "tumor", the ossification that was destined to happen over a long period of time is accelerated and now happens immediately while the body is trying to replace this injured muscle with bone. Clinicians need to check the toes and recognize FOP before doing biopsy, surgery, and intramuscular injections.
Source: Faculty presentation.
Disease Experts
FOP disease experts have been identified by the International Fibrodysplasia Ossificans Progressiva Association (IFOPA). Please click Link for the following
- Emergency medical contacts (5 physicians) and the emergency dentistry contacts (3 dentists) in the US
- A complete list of FOP consultants under the “FOP Treatment Guidelines authors and consultants”.
Patient Voices
This section of the course is developed based on true stories posted on https://www.cdc.gov/ncbddd/hemophilia/stories.html.
The characters are not actual patients.
What findings often trigger the referral to you for suspected FOP?
Should any baby with malformed big toes get a consult?
Is genetic testing needed for diagnosis?
Discussion
Christiaan Scott, MBChB, Michelle Davis
Faculty
Christiaan Scott, MBChB: Pediatric rheumatologist, Department of Pediatrics, University of Ottawa, Canada. Michelle Davis: Executive Director, International Fibrodysplasia Ossificans Progressiva Association (IFOPA)
Program faculty members are available for Q&A by teleconference or email. Please note that faculty will only provide educational and informational input. Please email info@p2psyncro.com to schedule. This is provided at no cost.
Takeaways
Can tolvaptan be given once daily
"We don't ever want to give tolvaptan once a day, because then there will be a period of time when vasopressin is not suppressed".
How to counsel patients with nocturia
“To manage the side effects of treatment, I focus on two areas depending on their complaints."
- If they report thirst, headaches, dizziness, or constipation, I assess their fluid intake. I will ask them to calculate exactly what they drink. More often, it's not as much as they think it is.
- If the complaints are polyuria, especially nocturia, I evaluate their diet and medication timing. I encourage them to have a low-sodium, low-protein meal for dinner. I may suggest taking the first dose of tolvaptan at 6AM, so that the second dose, eight hours later, is at 2PM. This often helps reduce nocturia episodes to 1 or 2, which is much less disruptive to sleep
Water bottle in hand or drink in boluses
“Adequate fluid intake is critical in the management of the aquaretic effect from treatment. We found that those who try to give themselves boluses of fluid struggle the most. We recommend keep a water bottle in hand and drink continuously throughout the day”.
Source: Faculty presentation.
Can FOP be diagnosed from the toe and swelling?
In FOP, muscle and connective tissues are gradually replaced by bone. There are 2 cardinal signs.
- Malformed big toes that can be short and curved are present at birth.
- Swellings that look like tumors start to appear in the first decade of life, typically in the shoulder, back, and scalp. They can be warm, painful, and appear episodically. Eventually the swellings will clear up but leave behind a new piece of mature bone.
The diagnosis of FOP is made clinically by associating rapidly appearing mass with great toe malformations.
Source: Faculty presentation and IFOPA www.ifopa.org.
Looks like sarcoma? Why to avoid biopsy?
The swellings in FOP are often misdiagnosed as soft tissue sarcoma. When one cuts into the muscle for biopsy of the "tumor", the ossification that was destined to happen over a long period of time is accelerated and now happens immediately while the body is trying to replace this injured muscle with bone. Clinicians need to check the toes and recognize FOP before doing biopsy, surgery, and intramuscular injections.
Source: Faculty presentation.
Disease Experts
FOP disease experts have been identified by the International Fibrodysplasia Ossificans Progressiva Association (IFOPA). Please click Link for the following
- Emergency medical contacts (5 physicians) and the emergency dentistry contacts (3 dentists) in the US
- A complete list of FOP consultants under the “FOP Treatment Guidelines authors and consultants”.