Stealth flares, or bone creep

Posted by Karen - October 8th, 2017

Mystery, thy name is Fibrodysplasia Ossificans Progressiva. Indeed, there are so, so many strange and confusing things about FOP. My 12-year old daughter Miranda is experiencing one of those things right now… I call this phenomenon a “stealth flare”. It’s a flare-up which is different to the norm, in that it’s missing many of the usual symptoms and is much more low key (sneaky, one might say).

See, when we think of FOP flare-ups, there’s typically a constellation of standard symptoms – significant swelling, pain, heat, sometimes skin redness and/or a “vein-y” appearance, and of course loss of movement. Over the years, Miranda has had many of that type of flare-up. When those occur, it’s absolutely crystal clear what’s going on. These are typically on the back, neck, abdomen, or head.

Then there’s this other type of flare.

Here’s what Miranda’s got going on right now… It started with a completely normal week, no falls or traumas or anything of that sort. Then, right out of the blue, Miranda started complaining a week ago Friday evening of pain in her right shin, just below the knee. She had walked a fair bit that day, so I thought perhaps she had just overworked her leg muscles and had something like a shin splint. But the next day when Miranda awoke, the affected area was terribly painful. She said it felt like when she had broken her leg in the past, but we knew it couldn’t be a break because she had suffered no traumas, and besides, she could put weight on the leg. The pain, however, was the only symptom of anything going on – there was no trace of swelling, heat or redness, which was kind of confusing. By the next day, the pain was still just as intense, and Miranda said that moving her right knee (which was already limited, but still had some good movement) hurt. That day, last Sunday, I called it as a flare-up, and Miranda started high dose Prednisone, as is recommended for flare-ups involving joints.

As this week went on, the pain continued, though Miranda says it changed in quality from a stabbing pain to a moderate soreness. As of today, she says it’s “not too bad” – still noticeably sore, but not so intense, and she can walk more steadily. But throughout the course of this flare-up, there has been no development of any swelling, heat, nor redness. I fancy I can feel a slightly firm area of tissue tightness where the pain is located (just to the left of the shin), but this is pretty difficult to determine with certainty. The only other usual flare symptom is restricted movement – Miranda is definitely able to move the knee less than she could before, though at this point she still does have some knee movement.

Here’s a picture of Miranda’s legs. Doesn’t look like there’s anything going on with the right knee/shin/upper left calf, does there (NOTE – that’s not a bruise on her left ankle, just a shadow)?

IMG_0413

So weird. However, this isn’t the only time Miranda’s had something like this happen. At least twice before she’s had these atypical flares. The first was her inaugural below-the-waist flare-up at age 8, which coincidentally also involved the right knee. On that occasion, Miranda had no swelling, no heat, no redness, and according to her, not even very much pain – we first became aware of it when she started swinging her leg out to the side to climb up some stairs to her school, and it became apparent she had lost some range of motion. That loss of motion, accompanied by mild pain, was the sum total of flare-up symptoms.

Another time, we discovered Miranda had suffered a stealth flare AFTER it ended – one day I was helping her get dressed, and I discovered quite a good size lump of bone on her inner left tibia, just below the knee (she had had an exostose there in the past, as is common for FOP’ers, but no-where near as big as this lump). This was definitely a lump of mature bone, and I realized at about this time that it caused Miranda to be unable to fully straighten her left leg – she could almost straighten it, but not quite, and this was a definite change. I questioned Miranda, and she swore up and down that she had not experienced any swelling, pain, or anything, and the existence of this big lump was as much a surprise to her as to me.

I had heard when Miranda was younger that some older FOP’ers have described something similar to what I call stealth flares. Some have referred to it was “bone creep”, suggesting that the bone sort of just “crept” up on them without warning. I’m not going to lie; I thought this was pretty questionable… Until Miranda started to experience it.

Part of the whole constellation of FOP weirdness. If you or your child have FOP, have you ever had stealth flares/bone creep? Tell me your story.

2 Responses to “Stealth flares, or bone creep”

  1. Susanna Camussi says:

    Ciao,mio figlio Paolo ha la FOP,ora ha 17 anni,cammina ancora,anche se la coscia destra è in parte ossificata. I suoi flare up non hanno quasi mai presentato gonfiore e calore ,solo dolore e neanche troppo forte.

  2. Lara says:

    J has had this happen too. In his leg that was already affected, he lost more mobility. He also had his bone spurs grow and multiply with no swelling or redness but a fair amount of pain and like M, he tends to not fully straighten his right knee anymore due to this (he can get it exceedingly close to straight but it’s uncomfortable). Thought he had a stealth flare in his left shoulder too but the X-rays show no new bone growth so not sure if it left cartilage or nothing at all and he’s simply atrophied the muscle from the fear of moving it through pain (anxiety has not been kind to us the last 2 years …).



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