Updated May 2022
The Coronavirus (COVID-19) pandemic continues to pose a significant risk to the population worldwide with new variants of SARS-CoV-2 virus are emerging. The International Clinical Council (ICC) on FOP recommends that people living with FOP to continue to follow strict precautionary measures to prevent infection from SARS-CoV-2, the virus that causes the COVID-19 illness. The ICC is providing this update to the prior statement in March 2021. This document focuses on updated information reporting on COVID-19 infection and vaccination in FOP patients, approval on COVID-19 vaccination in children aged 5 and above, boosters and treatment.
The recommendations are changing rapidly, are country specific and based on COVID-19 vaccine availability:
The recommendations are changing rapidly, are country specific and based on COVID-19 vaccine availability:
- UNCHANGED! The ICC does not provide recommendations on whether a patient with FOP should or should not receive a COVID vaccine.
- UNCHANGED! The decision to take a vaccine is a personal one and based on the balance of risks and benefits, and this should be discussed with your medical team. ICC continues to recommend that COVID-19 vaccines be administered that same route that it was approved (ie intramuscular).
UPDATED! Additional information about COVID-19 and COVID vaccination in patients with FOP was published on March 4, 2022 in the Orphanet Journal of Rare Diseases. Read Social and clinical impact of COVID-19 on patients with fibrodysplasia ossificans progressiva
- Amongst 15 FOP patients who received intramuscular COVID-19 vaccination. Most common symptoms were pain/soreness, tiredness and swelling. 1 out of 15 developed a flare up and none were hospitalized.
- Amongst 10 FOP patients with COVID-19 infection. Most common symptoms were fatigue, loss of taste or smell and cough. 2 out 10 FOP patient developed flare ups and 1 patient was hospitalized.
UNCHANGED! Vaccines are now generally available for children age 5 or over. ICC does not provide recommendations on whether a patient with FOP should or should not receive a COVID vaccine
- UPDATED! ICC does not provide recommendations for or against the booster vaccination, but boosters should be considered if you completed vaccinations previously and are in a high risk area. Please consult with your medical team prior to receiving the booster to discuss if a booster is appropriate and safe.
UPDATED! Patients with FOP are at high risk of complications with COVID-19 infection and should discuss with their medical team if use of monoclonal antibodies or anti-retroviral medications would be beneficial, in the event of a SARS-CoV2 infection.
- Monoclonal antibodies are given intravenously and are approved for adults and pediatric patients (≥12 years of age weighing ≥40 kg). Those innervations should be started as early as possible and before 10 days of symptoms onset.
- Anti-retrovirals are pills that have been approved. These should generally be administered within 5 days of symptoms onset.
- Availability and recommendations of the use of these treatments are rapidly changing and country specific. Some of these therapies may not work against strains prevalent in a particular region. Please consult with your local medical team for recommendations
- Discuss with your doctors to make sure there are no medication interactions
- UPDATED! Thank you to all of our prior participants who have contributed clinical data about FOP and COVID/SARS-CoV2 infection. The studies at the University of California, San Francisco (UCSF) have now concluded and were published in the Orphanet Journal of Rare Diseases on March 4, 2022: Social and clinical impact of COVID-19 on patients with fibrodysplasia ossificans progressiva
- NEW! Masking continues to be an important component of controlling the spread of SARS-CoV-2. The ICC strongly recommends the use of tight fitting N95, KN95, or KF94 masks whenever possible to protect the wearer from infection by SARS-CoV-2. If these masks are not available or uncomfortable, then wearing a 3-layer surgical mask would be the next best choice.
NEW! The ICC is aware of a recent publication suggesting that the use of subcutaneous needles could still induce adequate vaccine response. However, this study likely delivered the vaccine via a shallow intramuscular route. Furthermore, there are multiple reports in the literature of severe reactions to subcutaneous injection of the COVID vaccine. Efficacy of subcutaneous delivery of a COVID vaccine remains unproven. Therefore, the ICC continues to recommend following the manufacturers' directions for vaccination and NOT taking intramuscular COVID vaccines by the subcutaneous route.
IMPORTANT! If you decide to take the COVID vaccine or booster, we recommend:
- Discuss your plans with your doctor. Review any potential allergies or prior reactions like anaphylaxis that you should consider before taking the vaccine.
- Take the vaccine via the recommended route and dose (ie intramuscular (IM) for the currently available vaccines). Safety and efficacy of taking an IM vaccine through the subcutaneous route is not known, and could cause a more unexpected inflammatory responses or poor immune reactions, and is currently not recommended.
- If possible, take the vaccine in a location that is already fused, as the vaccines all appear to induce some local site reaction (arm pain and swelling). For example, if your left hip or right shoulder are fused, you should use the muscle around those sites.
- Patients with FOP should be flare free for at least 2 weeks prior to receiving the vaccine.
- Have the injection done by an experienced nurse, physician, or pharmacist.
- Use the shortest needle available (this varies with site). The clinician should be aware that patients with FOP may have hidden HO and thinned muscle at the site of the injection. Avoid injecting directly next to existing HO bone if possible.
- Prior to the vaccination, have ibuprofen or acetaminophen available. Also, have a course of prednisone for flares available.
- Make sure your physician is familiar with the ICC FOP Treatment Guidelines, specifically on vaccinations and flare management. Notify your physician you plan to do the vaccine, and when.
- On the day of the injection:
- Your local team may not allow you to take ibuprofen or acetaminophen prior to the injection (this is because they may screen for COVID symptoms first).
- After you receive your injection, there may be a brief observation period.
- After that is completed, take ibuprofen (2 to 3 times/day) or acetaminophen (2-3 times/day) following the label instructions, for the next 48 hrs, regardless of your symptoms.
- Rest and stay hydrated.
- In the event of a flare, contact your physician for guidance. You may need to do a short course of prednisone, but this needs to be balanced with the immunosuppressive effects of steroids. The usual flare dosing is prednisone 2 mg/kg/day up to 100 mg, for 4 days; your physician may recommend starting at a lower dose, depending on your symptoms.
- Even if you take the vaccine, you still need to continue physical distancing, wearing masks, and appropriate hand washing
- The ICC can’t guarantee that these steps will “work” to prevent complications. All medications and treatments have risk, so it is important to discuss your specific situation with your doctor as you decide whether to take the vaccine or not.
- Make sure that you complete the full immunization regimen recommended (ie do both doses if the vaccine recommends 2 doses)
- Discuss with your physician if you should do a booster and if that is appropriate for you, such as to cover local SARS-CoV2 variants. This is an area of active investigation so will need to be updated as the ICC receives more information.
- Recent developments of a vaccine provide long term hope. However, the impact of the vaccine on the pandemic will take quite some time to manifest. Many types of vaccines are being tested and new variants of SARS-CoV-2 virus are emerging and so this is a rapidly changing field.
- Vaccines are currently not available for children younger than 5 years or with subcutaneous delivery. These are actively being studied, and will be added to the guidelines once available.
- At this time, the ICC does not recommend vaccination for children younger than 5 years due to the lack of safety and efficacy data.
- The exact duration of immunity conferred by the vaccines is unknown but does not seem to be lifelong.
- At this time, ICC does not provide recommendations for or against vaccination for patients with FOP older than 5 years of age. The specific risks and benefits should be discussed with your physician.
- The ICC recommends that FOP family members and caregivers be fully vaccinated for SARSCoV2 if safely available for them.
- Vaccinations can take 2+ weeks to show any efficacy, so there is no protection immediately after vaccination. In addition, vaccines do not confer absolute immunity to the SARS-CoV-2 virus, and may not have activity against all forms of the SARS-CoV-2 virus. Anyone who receives a vaccine should still continue with masking, hand hygiene, and physical distancing.
- This information is rapidly evolving. Please discuss with your local care providers regarding benefits and risk of any locally approved vaccines and boosters.
- It’s very important to maintain social distancing and wearing a mask when around members outside your household
- Additional updates will be shared as new information becomes available.
- Notify your primary care physician to help coordinate care.
- Follow your local guidelines for isolation/quarantine and the needed durations and procedures.
- Everyone, including the person with the positive SARS-CoV2, should wear a mask at all times to avoid transmission.
- Patients who are negative for SARS-CoV2 but have similar symptoms should be tested for influenza.
NEW! Patients with FOP are at high risk of complications with COVID-19 infection and should discuss with their medical team if use of monoclonal antibodies or anti-retroviral medications would be beneficial, in the event of a SARS-CoV-2 infection. The main reason for treatment would be to reduce respiratory complications, as patients with FOP are at high risk of breathing complications and are difficult to intubate. However, access to these medications may be limited in your area. Please discuss with your physician if these medications are an option and appropriate for you.
- Monoclonal antibodies are given intravenously and are approved for adults and pediatric patients (≥12 years of age weighing ≥40 kg). Those interventions should be started as early as possible and before 10 days of symptoms onset.
- Anti-retrovirals are pills that have been approved for treatment of COVID-19. These should generally be administered within 5 days of symptoms onset.
- Availability and recommendations of the use of these treatments are rapidly changing and country specific. Please consult with your local medical team
- Discuss with your physician about any potential medication interactions prior to starting anti-viral therapies
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