About Miranda

Miranda Lena Munro Friz was born on January 15, 2005, and she has Fibrodysplasia Ossificans Progressiva, or “FOP”. Her mother is me, Karen Munro, and her father is my husband, Peter Friz. Miranda is also the younger sister of our son Owen Friz, who is almost exactly 3 years older than his sister.  This is the story of Miranda and FOP…

When Miranda was born, we were delighted. Here was the hoped-for little girl to round out our family. Miranda was healthy and strong, and the only thing unusual was that her thumbs stayed clasped into her palms, and her big toes were bent inward and had a “bunion-like” bump at the first joint. Though Miranda seemed perfectly well, the unusual thumbs and toes led our midwife to take the precaution of referring Miranda to a pediatrician for further assessment. That led to further examinations by a plastic surgeon and an orthopedic surgeon. Both experts stated immediately that the thumbs were a common and easily treatable condition, but the toes… No-one had seen toes like those. Still, in the absence of unusual health symptoms, Miranda was treated on the theory that she had some sort of abnormality restricted to the thumbs and toes.

Miranda’s thumbs were treated in the usual way, which is to say that at age 3 or 4 months, she was fitted for neoprene thumb splints (like partial gloves) which forced her thumbs away from her palms. She wore them continuously while awake, and as promised, her thumbs appeared normal within 2 or 3 months. The experts figured this treatment would work for the toes as well, so Miranda was also fitted with similar splints for her feet. However… Despite Miranda wearing the splints for about 8 or 9 months, her toes didn’t change at all. Finally we gave up on the toes, and there was talk of surgery when she was older.

Apart from the thumbs and big toes, Miranda seemed completely normal as a growing baby. She hit all her milestones at the same time as her brother had done, and developed normally, with one small exception. The one exception was that “tummy-time” was difficult for her – she couldn’t get her head up without significant propping until she was 8 or 9 months old (most babies can do this by age 2 or 3 months). I puzzled about this for a while, but when she otherwise progressed as expected, I stopped thinking about it.

The fist sign of something really strange happened when Miranda was 18 months old. She took a fall and bumped her head on concrete, and over the next several days, a huge lump swelled on her forehead. After about 4 or 5 days, it suddenly started to drain down the inside of her face, causing her face to swell up and her eyes to squeeze shut. X-rays showed no skull fracture, but doctors at the BC Children’s Hospital were concerned about the swelling and ordered blood tests for clotting disorders. Those came back negative. Anyway, the swelling finally disappeared, but the hospital doctor was interested to note that it seemed to leave behind a small amount of “calcification” on Miranda’s forehead.

Pete and I weren’t much concerned about this episode after the swelling went down, especially when the blood tests came back normal. We thought all was well until, about 3 months later, I suddenly realized that the back of Miranda’s head was hugely swollen and misshapen. She looked like one of those dinosaurs with a big bony crest on the back of her skull… This time we feared hydrocephalus, and rushed Miranda back to the hospital. Another x-ray revealed that it wasn’t hydrocephalus, but rather a soft issue swelling. This was very troubling; I couldn’t remember any trauma to the back of Miranda’s head. A new set of blood tests were ordered by our pediatrician, but again they came up negative. Also again, the swelling went down again after about a week.

After this incident, Miranda had repeat episodes of swelling on various parts of her head about once every 3 months. They would last 2 to 4 weeks each time, then recede. Unfortunately, however, our pediatrician started to lose interest in the case, and stopped believing me about the swellings. He as much as told me he thought I was just being hysterical, even though I managed to show him one such swelling. By this time I was pretty sick with worry, so I wasn’t about to accept his perspective, and I asked our GP for a referral to a new pediatrician.

The appointment with the new pediatrician was set for April 12, 2007. As fate would have it, about a month before that date, I noticed a marble sized lump on the back of Miranda’s skull where it met the neck. I tried to convince myself it was a swollen lymph gland. However, about a week before the appointment, the lump started to swell. By April 12, the entire back of Miranda’s neck was swollen. The new pediatrician was stumped, and asked us to come to the Children’s Hospital the next day for a set of expert assessments. We did so, and within a few days, our doctor called us with news of an appointment with the Medical Genetics Department.

We met with a medical genetecist on April 19, 2007. He examined Miranda, and then gave us the diagnosis of FOP. I hadn’t heard of FOP before, and was horrified, but as soon as the doctor described it, I knew in my gut that the diagnosis was absolutely correct. FOP explained all of Miranda’s peculiarities and tied everything together.

In January of 2009 as I write this, it’s been almost 2 years since the diagnosis. A number of FOP “flare-ups” have resulted in new bone formations on Miranda’s little body. Her back is now rigid except for a small amount of movement in the upper back, her neck has limited range of movement, and she can move her shoulders only slightly away from her torso. Miranda’s right elbow is almost completely fused in a bent position, and her left elbow can bend normally, but can’t fully straighten. Despite her limitations, however, Miranda is a happy, vibrant and bright little girl, who loves playing with her brother and her toys, and who is learning her letters. She copes with her restrictions, and figures out ways to do the things she wants to do. Pete, Owen and I all love her dearly, and it’s our most passionate wish for her to be happy and love her life.