What techniques or tips can be introduced into someone’s day-to-day routine, from a young age, to manage living a normal life with a rare autoinflammatory condition?

Daily monitoring of fevers or attacks

When attacks or fevers occur regularly, they can become less difficult to anticipate. It is important to monitor your attacks in order to help identify and recognise triggers and initial symptoms – one way of doing this is to create a calendar/diary that is completed on a daily basis with activities and habits as well as well-being and health.

This type of written information on the burden of Systemic Autoinflammatory Diseases (SAID) is really important for documentation and very useful for helping healthcare professionals and treating physicians provide the best care.

Also, introducing small measures like this daily calendar/diary allows people and parents /caregivers to learn more about how to deal with attacks, including different options for management such as medications and complementary care; relaxation, hypnosis etc. For parents, monitoring attacks will help to improve the way they deal with personal emotions too, such as guilt and stress.

Patients should always carry with them an emergency protocol, written by their treating physician, like this one for FMF: https://www.orpha.net/data/patho/Emg/Int/fr/MaladiePeriodique_FR_fr_EMG_ORPHA342.pdf

Making special arrangements
Arrangements can be made at school between families, teachers and the treating physician to explain why some days may be left from school, adapt daily activities, support medication intake (if necessary) and to have an accurate care plan in place in case of emergency.

Considerations for travelling

  • Breaking up long journeys and choosing to fly during the day can help you to avoid jet lag and fatigue for long haul flights.
  • Talk to your physician before you travel to see if any change of dosage of any medication is required.
  • It’s also important to travel with warm clothes to avoid instances of excessive exposure to cold environments.
  • Particularly for FMF, during long haul flights, requesting an aisle seat or the first row will help you to stand up and walk around regularly to avoid limb pain and swelling. If you are travelling with an injectable substance, ask your doctor to provide you with a certificate indicating the reason why, the quantity and any specific needs for refrigeration etc.
  • Another tip when travelling is to carry a dedicated box that contains your medicines and all your medical documents so that you have everything together to access easily.

Finally, remember to check the destination hotel and accommodation details before making any bookings; you can complete a quick search online to make sure your hotel has all the necessary comforts and requirements that you need.

How can people living with rare autoinflammatory conditions best prepare for routine appointments with the doctor?

Preparing for your Physicians appointment

  • A great way to ensure you are prepared is to have all your questions outlined before you go to your medical appointments. You can do this by preparing all your documents; including biological and radiological documents and any certificates required for medical referral.
  • One way to keep track of your symptoms ahead of your appointment with your doctors could be to use a dedicated tool, whether it is a paper diary or an electronic one. Ask your physician about the AIDAI grid.
  • Keeping a diary of your symptoms is an efficient way to explain to your physician what symptoms you have experienced and the type of activity you were doing before you experienced them.

What are the common challenges faced by health professionals when communicating with young people and their families affected by autoinflammatory conditions? What can be done to help?

Common challenges for young people and their HCPs
A common challenge faced by health professionals is the episodic character of rare autoinflammatory diseases. People with rare autoinflammatory conditions need to be able to effectively communicate their attacks to their physician or healthcare professional for accurate assessment and diagnosis.

Understanding patient needs
Physicians understand that it is crucial for patients to have time with their physician or healthcare professional, so they are properly understood and subsequently offered the right care options.

If you are facing a challenging time, you don't have to go it alone. Support groups can offer help by putting you in contact with people facing similar challenges. They also offer an opportunity to share experiences and advice, and can be easily accessed online. RareConnect is a great resource which connects patients globally, more information about RareConnect here https://www.rareconnect.org/

For people living with rare autoinflammatory conditions, what does it mean to get “lost in transition” when changing from paediatric care to adult rheumatology care?

Transitioning from child care to adult care

  • Adult specialist physicians need to receive specific information on autoinflammatory conditions to help people living with them. Transition of care from childhood to adulthood can be a challenging process.
  • To ensure that care and treatment is continuous and the transition is smooth when young people become adults, paediatricians must start conversations about transitioning as early as possible. It is recommended this occurs between 12-14 years of age. In addition to open communication between the physicians, the person living with the autoinflammatory condition and their families/ caregivers can build dedicated programmes to guide this process; speak to your physician about specific programme options.
  • Paediatricians can also help to identify suitable adult specialists who can be considered when the person living with an autoinflammatory condition is ready to transition into adult care.
  • Staying informed: It is also very important to keep parents and caregivers informed and supported during the transition process which is also stressful for them. Parents are encouraged to progressively accept that one part of the consult must be realized with the teen alone.
  • Paediatricians and adult specialists must be aware of frequent adolescent at-risk behaviors like low adherence to treatment, addictions and sexual wandering.
  • Families and people affected by autoinflammatory conditions can be provided with evaluation and education tools to enable healthcare professionals to effectively manage their transition plan.

What types of problems could teenagers with rare autoinflammatory conditions encounter when transitioning from paediatric care to adult rheumatology care?

What to expect when transitioning from child to adult care
It is important to ask your physician (paediatrician) to help identify an adult doctor, one that has knowledge of autoinflammatory diseases and is local to where the affected person lives. Another way to ensure a smooth transition into adult care is to find a joint (paediatric and adult) consultant, this will allow for adhesion within the transition of care programme.

When managing the transition into adult care, it is recommended that adult physicians monitor the situation carefully and allow frequent availability and support.

Understanding adolescent care needs
Teenagers can often leave their transition of care plan to the last minute, when a true emergency takes place. Adult doctors need to be flexible and, where possible, allow more time during appointments to enable opportunities of increased exchanges with teens and young adults.

The physicians should also make sure that all information is passed over to a person’s new physician so they have the full medical history on file and can offer a person the right treatment and advice during the transition into adult care.

For a teenager or carer of a teenager with a rare autoinflammatory disease, what can be done to make the “transition” of care as comfortable and easy as possible?

Comfortable care
Physicians do understand that any information given to people living with an autoinflammatory condition especially around their transition from child to adult care must be part of their educational program, which can be helped along with dedicated tools. For young children and teenagers, parents should ask the question of future adult care as early on as they can – a recommended age to start asking about adult care is anywhere between 12 and 14 years.

Differences between paediatric care and adult care
Knowing and understanding the differences between paediatric care and adult care is important and should always be carefully explained to teenagers. A transition plan should be set up and regularly evaluated by a physician, before transition, during transition and after transition, until at least 2 years after the transition has taken place.

We know that even when an accurate transition plan has been set up, a number of people can disappear from the healthcare system during variable and often lengthy periods. There are a number of reasons for this, for instance: they may have changed their way of life, moved home to another city etc. In this instance, people with an autoinflammatory condition need to have information available to them on experts in their new area and a succinct medical report of their Systemic Autoinflammatory Disease, in case they are admitted to emergency care. This should include the name of, and information about, their last expert physician (often this will be their paediatrician).

It is difficult to connect with teenagers especially if the chronic disease is the main reason for wanting this connection. Individual levels of care are required to form a positive relationship between the physician and the person living with an autoinflammatory condition, to understand the care they require, and to be empathetic to their symptoms.

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