First and foremost, you should consult with your healthcare provider for the best advice tailored to the patient’s condition. As a caregiver of an ADA-SCID patient, it is normal to experience feelings of anxiety and uncertainty. You may feel overwhelmed or nervous about day-to-day care, such as giving an injection or managing multiple health care appointments, but proper planning can help relieve some of the anxiety.
As a caregiver, you are an important part of the healthcare team for someone affected by ADA-SCID. You are the primary advocate. You may be interacting with several different doctors, so your ability to organize test results, keep healthcare records, and observe the day-to-day health of someone affected by ADA-SCID is critical. Having all, relevant information in one place such as a binder, notebook, folder, etc. is helpful to keep an organized record for management of the ADA-SCID condition.
Here are some steps that as either a patient or a caregiver you can take to make the most of your visits with a healthcare provider:
Tips for Effective Communication with Your Healthcare Providers
Communication is key in building good relationships with your healthcare providers and getting the best possible care. Here are a few tips to help you communicate effectively with your doctors and other healthcare providers.
Lists are your friend.
Before your appointment, make lists to keep track of medicines and dosages, symptoms, allergies, supplements, etc. It is most helpful to have an updated list of ALL medications, including over the counter medications to bring to the healthcare provider visit. It will help to have them in front of you, so you don’t forget to report anything to your doctor. Also, keep an up-to-date list of your doctors’ contact information, including phone numbers, email addresses, and postal addresses. Be certain to update the healthcare provider office with any changes to your contact information.
Keep track of your questions.
Even if you think you will remember all of your questions, it’s always a good idea to write them down. Bring your notebook to your appointments so that you can write down the doctor’s answers, too.
It is helpful to take an active role in your healthcare management, which means bringing up questions to your healthcare provider and addressing issues as soon as they come up whenever possible. You may have concerns that the doctor may not be aware of so it is critical to address those in person whenever possible.
It is important to take precautions so that people with immune deficiencies can avoid germs whenever possible. Practice good hygiene when visiting the doctor and follow your doctor’s recommendations to help prevent the spread of infections. Be cautious about touching surfaces and maintain space around you to avoid others with active symptoms (cough, sneezing, runny nose, etc.).
Bring your full medical history to appointments.
Ask for copies of all test results and lab work and keep them organized in a binder. Bring test results, names of current and past medications, details of hospital visits, and treatments to all of your doctor’s appointments. Often, you will be the only complete source of information about you or your child’s medical history. Thorough records from all doctors and specialists that treat you or your child will help ensure that you or your child are receiving the appropriate treatments for your condition. Ask about a patient portal (a way to electronically communicate with the doctor’s office), even if it is for your child so that you can access electronic information including lab tests, reports and also have an opportunity to reconcile medications and ask questions.
Report any side effects to your doctor immediately.
Always inform your doctor about new symptoms, reactions to medications, etc., so that they can help you manage any reactions and provide appropriate resources and treatments. You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/Safety/MedWatch/ or call 1-800-FDA-1088
Keep your doctor’s appointments.
It is important that you keep your scheduled doctor’s appointments so that your doctor is able to monitor your condition. Missed or cancelled appointments should be rescheduled as soon as possible.
Having all of this information organized, up to date, and in one place will make it easier when you are visiting a new physician for the first time. It may also help you save time in the doctor’s office, allowing more time for discussions with busy doctors. In addition, there may be times when someone else who is less familiar with your child’s medical history will have to take them to their office visit, and the binder will be a valuable resource for the doctor.
Questions to ask your healthcare provider about living with ADA-SCID
Communication is key to building good relationships with your healthcare providers and getting the best possible care. Here are some questions to consider asking your doctor.
- How long will treatment be required?
- Why is it important to not have any interruption in treatment?
- What are the potential side effects with the prescribed treatment?
- Who will be part of my healthcare team? Who will be my main point of contact?
- What can I do to help prevent getting infections?
- Are there any restrictions on my activity level or diet?
- Is there a specific medical diet or are there nutritional guidelines that you would recommend?
- Can I get all of the recommended vaccinations?
- What are the long-term health consequences of ADA-SCID?
- Is there an ADA-SCID support group I can join?
- Do I need to see any other specialists?
- Who should I call if I get sick?
- How often will I need to have blood testing?
- How will I know if the treatment is working/not working?
Revcovi (elapegademase-lvlr) is indicated for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients.
Important safety information
Warnings and precautions:
- Injection site bleeding in patients with thrombocytopenia: Increased risk of local bleeding in patients with thrombocytopenia; should not be used if thrombocytopenia is severe.
- Delay in improvement of immune function: Protect immune deficient patients from infections until improvement in immune function.
The most commonly reported adverse reactions were cough and vomiting.
In addition, the following post-marketing reports for the same class of enzyme replacement therapy used in the treatment of ADA-SCID, may also be seen with Revcovi treatment:
- Hematologic events: hemolytic anemia, autoimmune hemolytic anemia, thrombocythemia, thrombocytopenia and autoimmune thrombocytopenia
- Dermatological events: injection site erythema, urticaria
Important monitoring information:
Treatment with Revcovi should be monitored by measuring trough plasma ADA activity and trough dAXP levels for maintenance of therapeutic targets. If a persistent decline in plasma ADA activity occurs, immune function and clinical status should be monitored closely, and precautions should be taken to minimize the risk of infection.
Please refer to Revcovi’s Full Prescribing Information.