How Do You Find Normalcy with a Chronically Sick Child? When Does Life With a Periodic Fever Syndrome Become Normal?

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“365 Days of Anakinra Shots.” Virginia was born sick. At just a few days old she had already needed a blood transfusion and surgery, and had unexplained rashes and joint swelling. By 6 months old she got the diagnosis of NOMID and was able to start treatment. Normal for her includes a daily shot, which lets her live a healthier life. Photo by Katherine Buster Whitworth.

Having a chronically sick child means your world gets turned upside down and inside out. You no longer get to live the normal life you once had or imagined for your child and your family. Routines change, friends may change, finances change, and you can feel like life is just chaos.

Nothing seems normal anymore as you spend more of your time with doctors and nurses instead of family and friends; your craft table may now be covered with notebooks and photos documenting symptoms instead of piles of designer scrapbook paper, vacation photos, and glitter pens; you may become more concerned with blood test numbers rather than report card grades, and you read the latest medical literature instead of the latest best seller.

For families dealing with a periodic fever syndrome (aka autoinflammatory disease), watching their child battle recurrent fevers, rashes, joint swelling, and pain becomes part of every day. These symptoms are due to a malfunction of the innate immune system and are not contagious to others. But because these symptoms are debilitating and can appear at any time without warning, it’s hard to make and commit to plans which makes a sense of normalcy seem out of reach. (Learn more about periodic fever syndromes here.)

Koen (who has uSAID), spends a lot of time in hospitals and with doctors. But he still has much to celebrate like being able to attend the first day of high school. When chronic illness works it’s way into normal everyday life, you learn that everyday moments like going to school or attending a birthday party are moments to cherish. Photo by Cat Lichtenbelt.

An invitation to a birthday party, a normal childhood activity, becomes an agonizing decision for a parent. Not because there’s a soccer game at the same time which would be a normal conflict, but because their child may suffer later with flares of fever and joint swelling. Or the excitement of the upcoming party may have already triggered a flare before the party even starts. Do you let them go and do normal kid activities and try to explain to others at the party that they are not contagious, or turn down the invitation and protect them?

This loss of normalcy can make parents and kids feel isolated and grieve the life they knew and had before the illness took over their world.

So how do parents and families find normalcy with a chronically sick child? When does life with a periodic fever syndrome become normal?

Here’s how the experts, who are the patients and parents of patients in our autoinflammatory community, answered the questions.

Our normal is not others’ normal. For years of my kids’ childhoods, and entire lives of previous generations, it was normal to be in pain, covered in rash, and exhausted at the end of the day. It was what it was, and they all assumed there was no treatment – because there wasn’t one until relatively recently. In fact, they didn’t even have a proper name for it. My husband grew up feeling like a weakling because he thought everyone else experienced what he did in cool temperatures.

We’ve had to adjust expectations for our kids from, “You can do anything,” to “Do what you can.”

My 13-year-old asked me the other day, “You know when you come inside from the cold and your skin feels like it’s on fire?” “Uh, nooo.” That’s her normal, and she’s never known anything else – that is until she got proper treatment.

My kids’ current normal is daily injections that prevent most, but not all symptoms. My husband’s normal is nearly daily symptoms even though he doesn’t work anymore. Our overall normal is tired. So tired.

We’ve had to adjust expectations for our kids from, “You can do anything,” to “Do what you can.”

On the exterior, my family looks normal, and that’s the hardest part. You can’t see their pain and fatigue and anxiety. You can’t tell that their short fuse is due to exhaustion and pain…

They look normal, but have limitations. We went to the Texas State Fair last year and I rented a wheelchair for when they get tired. Since they look normal and were intermittently active, there was actually a guy who said, “Now, I KNOW that girl doesn’t need that wheelchair. I just saw her at the tractor pull.” I screamed back at him that she has arthritis because that’s something people can understand. He didn’t even turn around. I spent the rest of the afternoon in a red haze wishing I had walked after him and made him look me in the face and say it again, and then tell him to apologize to my daughter.

Her normal is not my normal – is not her sister’s – nor her dad’s. Even amongst them with the same diagnosis, they have individual normals.

On the exterior, my family looks normal, and that’s the hardest part. You can’t see their pain and fatigue and anxiety. You can’t tell that their short fuse is due to exhaustion and pain, so they just seem like jerks. If my kid doesn’t want to look you in the eye to speak, it’s so she can actually speak in that moment. Her normal is not my normal – is not her sister’s – nor her dad’s. Even amongst them with the same diagnosis, they have individual normals.

You’ll find a groove and accommodations to make your own normal.

– J.E., FCAS family. Learn more about FCAS, a form of CAPS here.

 

It’s a journey.

You will have a new norm. Hopefully you’ll get a diagnosis and appropriate treatment that will make it easier to create your normal. During the time it will take to get to that point, (and have perhaps time to have meds adjusted for best efficacy) you will grow to accept your new norm and before you realize it, you won’t remember life being so different “before.” Good luck to you. It’s a journey.

-S.S.H., mom of a child with CAPS. Learn more about CAPS here.

 

It’s very draining when you’re the main caretaker. For me, what has helped is to have a plan in place for when my son flares. I’ve started taking people up on their offers to help. When my son is sick, my daughter hangs out at the neighbor’s for a break. I’ll drop her off at a friend’s house, or I’ll take my neighbor up on that offer to pick some things up at the store for me. My husband works a lot and travels a lot, so if he’s here, I get out when I can (or I go crazy spending days as a human cushion). He’s understanding as he has an autoimmune condition, so I’m often doing double duty.

For me, what has helped is to have a plan in place for when my son flares. Now I’m finally accepting my “new normal,” like everyone has said, and just planning for it.

It seems like it’s a process. At first, I didn’t know what was happening and dealt with it, then searched for answers and did a daily calendar countdown, then got depressed and was afraid to schedule ANYthing. Now I’m finally accepting my “new normal,” like everyone has said, and just planning for it.

-M.K., mom of a child with PFAPA. Learn more about PFAPA here.

 

Many times, my sons are too sick during uncontrolled flares to participate in much. However, if they insist on not missing out, especially now that they thankfully respond fairly well to treatment, then I listen to them. Their quality of life was so poor when I cared about what others thought (I’m over that now lol) and when I followed normal rules of staying home when unwell. I think I pushed past that notion because hanging out by yourself when chronically unwell stinks. Now we let our boys decide what is best for them, and if they choose to go we explain to family and friends that they are not sick with something contagious.

Their quality of life was so poor when I cared about what others thought (I’m over that now lol) and when I followed normal rules of staying home when unwell. Now we let our boys decide what is best for them, and if they choose to go we explain to family and friends that they are not sick with something contagious.

We are currently on a weekend vacation at the beach. Yesterday, one of my sons (not flaring) asked to stay at the beach house instead of going to the beach. He knows his body best, so I said of course. He enjoyed playing quietly with Legos and reading books during the morning instead of sun and swimming. He slept that afternoon and was able to join us for swimming after dinner. His brother was untreated this time last year and was flaring very badly at our beach trip last summer. He still insisted on participating anytime possible. Both of those were the right decisions for them. My sons’ disease will be lifelong, so knowing their body and setting their own limitations is important.

-K.F.B., mom to twins with uSAID/IFIH1 mutation. Learn more about uSAID here.

 

We learned a long time ago to let my daughter decide if she feels well enough to participate in activities. Honestly, if we held her back from everything, she would have no enjoyment in life.

-S.T.L, mom of a child with HIDS. Learn more about HIDS here.

 

We had absolutely NO help for eight years of the hardest times, and even had to downsize to a car from our van, and live in a two-bedroom apartment (with three kids) and it was really bad mentally for me. No treatment, no help, 3 kids flaring all on top of each other. I honestly spent a few years in what I call, “robot mode.” I just did what I had to do to get through it, but didn’t feel like I was living.

My husband is the sole breadwinner, and a firefighter, and also was not aware he had CAPS. So, during that time, he would come home and be exhausted, and had these rashes he was hiding, and having a harder time hearing. All his sick leave was used for the kids. My parents would come to visit, but every single time the kids would end up flaring or getting sick as soon as they left, so it would stress me out more as they would come and ‘have fun’ and then we would be left to pick up the pieces. I think being cooped up in a small apartment is what made it all worse!

I don’t let it get in the way of our fun, but it’s still there, as I know at any moment the kids could get really sick or we could have an issue with the medicine. I know not to take for granted their current health status.

I had a lot of anger over feeling like we lost years of our life due to having no answers. Now that the kids are finally on a treatment and we now are renting a house in an area with access to lots of free fun things, I am trying to take advantage of the good times. I think the biggest fear in the back of my head is what if this ‘good period’ comes to an end. I don’t let it get in the way of our fun, but it’s still there, as I know at any moment the kids could get really sick or we could have an issue with the medicine. I know not to take for granted their current health status.

Oddly enough, in some ways I feel luckier to have three kids with CAPS as they all understand each other. If one is feeling bad, the others bring them lunch, set up the tray table, and get the iPad going. Plus, they all have to get the shots, so it was easier to accept that as their normal.

-J.V., family with CAPS. Learn more about CAPS here.

 

You will find a new normal and as things change with the illness for better or worse you will find another new normal. You lean heavily at times on those who understand and help you through. It isn’t easy but you will discover that you are stronger than you ever imagined, not by choice but by need. You are an amazing person and you will figure it out.

-K.C.

 

I think sometimes it’s hard to see outside of your own distinct stage in this journey. I remember the beginning of our journey, and I did grieve the loss of healthy children. Sometimes I still slide back there even with a good support system around me. I have learned to take people up on their offers to help. It was hard for me to learn that it’s OK to be the needy one sometimes. That is not my personality…I struggle when I’m not the one giving. However, I have found that while I have the most wonderful tribe of close friends (that group changed some as I did lose certain friends over having sick kids all the time) and a supportive family, that’s been a process. There were times when some family/friends were frustrated hearing about it, like it should be going away by now or something. I learned that my circle of support system was going to be different (and smaller) than I thought, but that the few people in it were going to be absolute rocks to me. They are the ones who can laugh about the ridiculousness of certain doctor appointments with me and who are fine with my kids running fevers at their houses, who treat my kids as kids and who see them for who they are as people, regardless of a possible pretty scary diagnosis. You will figure out who these people are in your life too, but it wasn’t quick for me.

I learned that my circle of support system was going to be different (and smaller) than I thought, but that the few people in it were going to be absolute rocks to me. You will figure out who these people are in your life too.

I am different now, so I’ve reminded myself maybe that’s why my friend group has changed a bit. The ones who were enjoyed hanging out with me only because I’m fun and humorous and relaxed and spontaneous…they had more trouble with the new me who spends a great deal of time researching, worrying over making the “right” decisions, and going to appointments. I couldn’t see this other side at the stage you’re in. It’s a better side, though.

This rare, chronic world is unfamiliar territory, but it becomes a lot easier to navigate with time.

-K.F.B., mom to twins with uSAID/IFIH1 mutation. Learn more about uSAID here.

 

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Hope Lives

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My son who is now in medical remission from SJIA and his sister who has juvenile arthritis (JA) enjoying healthy times at a recent baseball game.

I remember when my son was first diagnosed with an autoinflammatory illness, systemic juvenile idiopathic arthritis (SJIA). It seemed like nothing was helping. It was very frustrating, not knowing what to expect, and not knowing why he wasn’t getting better. Surely the doctors can help him, can’t they? But nothing they did seemed to make a difference.

 

It became very difficult to hear stories of children who had been diagnosed more recently than my son, who were in medicated, or even unmedicated remission. I was jealous, and couldn’t stand to hear that someone else had what I was so desperately looking for.

 

But there is hope. I didn’t rest until the doctors found the right medicine to help him. I was a mama bear and persisted and now, he too is in medicated remission.

 

So if you’re struggling because things aren’t helping; if the treatment just doesn’t seem to be doing any good, keep pushing.  Hopefully there is is a medicine out there that will help your or your child’s symptoms. If not, keep working for more research. There is hope. New medicines are being developed all the time.

 

Researchers are looking for a cure. When they make a discovery, often a new medicine is developed from that new knowledge. New resources are continually being introduced for the person whose symptoms are resistant to current treatments. 

 

Don’t give up! There is always hope.

The Pain of Autoinflammatory Diseases: Children and Adults Tell Us What a Flare Feels Like

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“Autoinflammatory Pain” by Christina Sweet

 Pain. Hurt. Fire. Needles. Burning. Knives.

Those are the words used by children and adults who have a systemic autoinflammatory disease (SAID). From diseases with sporadic attacks of inflammation, such as PFAPA, to the more chronic and damaging autoinflammatory diseases like CANDLE, NOMID and SAVI, it’s clear these are painful diseases.

Those affected by a SAID (also called periodic fever syndromes) go through periods of time referred to as “flares” or “attacks” where their disease is highly active and debilitating.

Flare symptoms can include fever, rash, mouth ulcers, joint swelling, inflammation in or around organs, and other systemic inflammatory symptoms. Depending on which autoinflammatory syndrome it is, flares may last days, weeks, or months. Some may have periods of decreased severity, but never get full relief of their symptoms. And some of the more chronic SAIDs can cause permanent damage to bones, tissues or organs if untreated. (Learn more about these diseases and symptoms here.)

It feels like my bones are all broken. -R.A., age 5, diagnosed with uSAID Click To Tweet

Although we can read and know the symptoms of a periodic fever disease from medical literature, for those of us who do not suffer from it, it’s hard to image what it actually feels like to have these flares. So we asked our community to describe what a flare physically feels like to them.

The answer most everyone gave, from kids to adults was “pain.” Flares are painful, and the entire body, inside and out, can hurt.

“What a Flare Feels Like” by PythonKitten

I feel really, really sick and don’t want to do anything but sleep. I get a headache and everything hurts. -D.C., age 4 diagnosed with uSAID/SURFS

It feels like my bones are all broken. My brain hurts. My eyes are on fire. My throat feels all krinkly. My heart beats hard and fast and I can’t breathe. -R.A., age 5, diagnosed with uSAID

It makes me feel weak and like I can’t move at all. Sometimes I feel like I can’t wait to go to heaven so my body won’t hurt anymore.  -A.V., age 8, diagnosed with TRAPS

My brain and body are at odds. I feel like I’m walking through water and my head is full of cotton wool. I want to run away from my body, but I’m too busy hiding in my bed and focusing on just breathing when the pain becomes overwhelming. -S.K., age 40, diagnosed with FMF and neuro-Behcet’s disease

I want to know what not having a flare feels like. What does it feel like to be totally healthy? -K.R., adult diagnosed with MWS Click To Tweet

If pain were noise, my body isn’t loud anymore. (Stated after getting on effective treatment.) -M.C., adult diagnosed with MWS

It feels like my eyes, my knees… it feels like my whole body is in pain. It makes me tired. It makes me weak. When my bones are crackly and the pain is starting up and stairs make me cry, that’s when I know a flare is starting. -L.J., age 5, diagnosed with variant CAPS/MWS

It feels like a dragon is spraying fire on me! -O.G., age 7, diagnosed with FMF

Badness…my whole body feels bad and it means I have to get a shot. I’m scared of my shots and don’t like them. -E.E.C., age 4, diagnosed with HIDS

My body hurts. I’m always cold. -T.T., child diagnosed with FMF

Tons of knives are poking my feet and legs. -I.B., age 5, diagnosed with uSAID Click To Tweet

It feels really bad. It feels like tons of stuff hit my head and tummy. All of my mouth hurts. My whole body hurts. Tons of knives are poking my feet and legs. -I.B., age 5, diagnosed with uSAID

My body feels very tired and I say “Owww.” My knees feel like they have bumps in them and my skin looks like a spider web. -G.S., age 5, diagnosed with uSAID

A teen with NOMID drew this picture to represent the constant pain and pressure from brain inflammation (aseptic meningitis) as a device attached to his head, spine and body, with the controls on the back and out of his reach.

When I have a headache, it’s like I have a metal ball inside my head. My throat has fireworks inside. My legs are too tired and shaky. My tummy is like rotten eggs. My muscles are too big and it hurts. -S.B, age 5, diagnosed with uSAID with IFIH1 mutation

Sore- I feel sore, more sore than normal, my throat blisters. I feel different. I don’t like it. -C.D., age 10, diagnosed with CAPS

It’s an unwanted visitor that I can’t wait for it to leave. Never the same, but familiar. Always, always stops me from living normally and robs me of special occasions, social gatherings and days and nights out. It doesn’t just leave without a footprint- it leaves fatigue and an aching. And once it’s gone, it’s the mental preparation for it returning. It is unpredictable, and as such life can’t be planned counting on it to arrive at a set time and place. Sometimes it arrives out of punishment for getting to bed late, for allowing yourself to be stressed, for getting cold. These are the worst kind because you take the blame. But it’s not really our fault- it would come anyways! -M.D., age 37, diagnosed with CAPS

“Pain” by Tanja Ackerman

It feels like multiple pains at one time. Very painful pain. The pain is everywhere. -Q.T., age 8, diagnosed with HIDS

Doesn’t feel really good, my body hurts so bad. I wish they [the flares] would go away with a hug and kiss. -O.G., age 5, diagnosed with uSAID

It feels like I’m very burning everywhere and my head hurts so bad. -P.R., age 5, diagnosed with Majeed syndrome

All I want to do is sleep. My stomach hurts. My whole body hurts. I can’t hold down anything. -A.O., age 21, diagnosed with NOMID

It's like having a bad flu. I'm only getting half my life done. -C.M., adult diagnosed with FMF Click To Tweet

It’s like having a bad flu. I told my doctor I can see why people die from dysentery, because that’s what it’s like for me: very painful everywhere, nauseating, disgusting, and I feel like curling up into a ball and rocking. I’m only getting half of my life done. -C.M., adult diagnosed with FMF

It’s like the worst flu you’ve ever had. -N.S., adult diagnosed with HIDS

It burns when people touch me! Or when things touch me! – child diagnosed with PFAPA

So easy to sleep! Just about the only time I don’t have insomnia. Heavy eyes, SUPER fatigued, screaming legs. Sometimes I still gotta go to school. That’s the worst. I used to be very nauseous and vomit frequently. I often lose my appetite and have lost weight because of it. Stomach pains. Throat feels like needles are stabbing it. -J.J., age 13 diagnosed with uSAID

A flare feels like a really bad flu bug – rash, fever, swollen glands, sores in my mouth and throat with a pain throughout my bones. However, because it doesn’t have a name and because I can’t find a doctor who understands how to diagnose this, I am expected to continue preform my life as if I am a healthy person. Worse, as a business woman, I have to keep my illness hidden from my male colleagues so that I do not appear weak and inferior. -C.E., age 42 diagnosed with uSAID

It's the excruciating twist of pain in your gut...you know it's coming for you. -A.F., age 38, diagnosed with uSAID. Click To Tweet

A flare is fatigue so bad you can’t sleep. It’s bone pain in your arms and legs. It’s every joint screaming at you when you try to move. It’s skin so sensitive you can’t bear the thought of a shirt. It’s constant GI issues, such as debilitating nausea. It’s having a sudden bout of diarrhea in the drop-off line at your child’s school, causing you to sacrifice another pair of underwear and pants to the cause. It’s the excruciating twist of pain in your gut. It’s the realization that after 15 bouts of diarrhea in 4 hours, you’re going to have to face the ER. It’s always knowing more about your disorder than any doctor you encounter aside from your specialist. It’s knowing you’ve failed most known treatments for this, and waiting for the last one that you’re currently on to lose effectiveness. It’s being a walking time bomb because your disorder has attacked almost every organ in your body at some point EXCEPT for your heart and lungs, but you know it’s coming for you. -A.F., age 38, diagnosed with uSAID

My tummy feels like out tightens then un-tightens. I hurt all over. My head feels like it will explode. I can’t open my eyes and it feels like glass is in my throat. -J.R., age 7, diagnosed with uSAID

It feels like being attacked from within; my whole body was shaking, throbbing, in pain, and unable to walk, unable to use a knife and fork or drive; having uncontrollable temperatures; having a rash that is all over and intensely itchy at times and a sore throat and glands up so bad that your jaw can’t open properly; stomach pain and chest pain.  -A.L.M, age 42, diagnosed with Still’s disease (AOSD)

Today's rash is like poison ivy over a second degree burn. -M.S., adult diagnosed with uSAID Click To Tweet

Today’s rash is like poison ivy over a second degree burn. -M.S., adult diagnosed with uSAID

I want to know what not having a flare feels like. What does it feel like to be completely and totally healthy? What does not having fatigue, bloodshot eyes, joint pain, hearing loss, stomach and abdominal cramps, chest pain, chills, headaches, and hives feel like?! -K.R., adult diagnosed with MWS

I never knew what “normal” was supposed to feel like until I went on treatment for CAPS. I am free-I am no longer a prisoner of pain. -A.C., diagnosed in adulthood with CAPS

The feeling that a patient “never knew what normal felt like” before being on the right medication for their disease is one of the most common statements that we have heard from a number of patients with various forms of autoinflammatory diseases. For many of these syndromes there is effective treatment, but because these are rare diseases, it can take many years to get to a diagnosis. For some there is not yet a highly effective treatment. This is why the Autoinflammatory Alliance is dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases. You can help us by making a donation here.

To learn more about periodic fever syndromes, click here.

 

 

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