A flare, and a short trip to San Francisco

Posted by Karen - January 29th, 2017

My daughter Miranda, who turned 12 a couple of weeks ago, hadn’t had any flare-ups of her fibrodysplasia ossificans progressiva (FOP) for quite a while. Further, it had been about 3 years since any flare had affected her mobility. And then, all of that changed last week.

It was a Thursday night, January 19, when Miranda said, “Umm, Mom, my knee kind of hurts. And also I’m having a bit of trouble with stairs.” She said this in a low key sort of a way, but from experience, I know that these things can be anything but minor. I dropped everything and came over to take a look. It turned out to be subtle – she had a slight swelling on her left inner thigh, just above the knee, but it was only slightly warm (often flares are noticeably higher temperature) and she swore up and down that it didn’t hurt “very much”. If that had been it, I probably would have doubted it was a flare – maybe she always had a slight swelling in that location and I just never noticed? – but there was no mistaking the fact that Miranda couldn’t handle stairs as well as she had done even just a day earlier. I felt that sick, panicky feeling start to take hold, but I can’t “lose it” in front of my girl – so I  took a deep breath, and told her that I was pretty sure she had a flare-up.

The next morning we started Miranda on Prednisone, as is recommended for flare-ups on a joint. I also got in touch with the Clementia Pharmaceuticals FOP Natural History Study folks in San Francisco, because Miranda is taking part in that study and we’re supposed to keep them updated about FOP developments. After a flurry of e-mails, it was decided that we should fly down to San Francisco the following week as part of the study. By way of explanation, it’s a feature of the study that the SF team is supposed to closely monitor one flare-up per year, and that involves them seeing the FOP person at their location. Anyway, we arranged for Miranda and me to fly out on Wednesday night, stay overnight in SF, do the medical stuff on Thursday, and then fly back Thursday night. I knew Miranda would prefer the trip to be as short as possible, as she had a bunch of school projects she was working on and a couple of tests to prepare for (not to mention that I couldn’t miss much work at this time of year).

Off we went to San Francisco… I got Miranda from school on Wednesday at lunch time, and then a driver arranged by the study picked us up and delivered us to the airport. We took Miranda’s manual wheelchair with us – she hadn’t used it often up to that point, but I knew she would be slowed down by the flare-up on her leg, and besides I didn’t want her to strain it. Note to self; always take Miranda in a wheelchair when taking a plane flight, because they whisk you through to the front of all security and customs line-ups and you get through everything SUPER fast (and further, nobody in any of those line-ups even gives you the stink eye!). The plane flight itself was pretty cool as well, because they booked us in business class. Our flight was very nice…

Business class, in the lap of luxury!

Business class, in the lap of luxury!

We stayed at the same hotel which we had stayed at before, the Courtyard Mariott in downtown SF. Though they have an excellent swimming pool, we decided to skip that and just relaxed Wednesday evening in our hotel room. Then, the next morning we made a quick stop at the Starbucks in the lobby (another reason why I like that hotel, ha ha), and got picked up and delivered to the University of California at San Francisco.

On the deck just outside the UCSF building we were heading into. AMAZING view from there.

On the deck just outside the UCSF building we were heading into. AMAZING view from there.

The medical stuff all went down pretty smoothly… First, we had to fill out a bunch of forms which asked questions about movement and abilities, and then it was time for urine and blood tests. Miranda and I were both kind of apprehensive about the blood test, because experience has proven to us that she’s a difficult draw. However, big props to our skilled phlebotomist, because she successfully got it done, woo hoo! (Big victory right there, I can tell you.) After that, Miranda had an electrocardiogram, a session with a physiotherapist who measured her range of motion, and finally, a meeting with the team physician, Dr. Ed Hsiao. The medical team made all this as painless and trouble-free as possible, with everything taking place in the same clinic room.

A comment now about Dr. Hsiao – I am so, SO glad that Dr. Hsiao is an experienced and knowledgeable FOP physician who lives and works on the WEST COAST of North America, just a 2.25-hour flight directly south of our home. We still love the original FOP expert Dr. Kaplan, but hey – he’s on the other side of the continent!  I can’t tell you what a great thing it is to have someone so (relatively) local who knows about FOP. Also, to top it all off, Dr. Hsiao is a really nice guy, and is down to earth and caring. We were tremendously lucky that he developed an interest in FOP.

Back to our story… After lunch at the UCSF cafeteria (which had surprisingly good food), we were picked up and delivered to a different location where Miranda had to have a couple of imaging scans carried out. First, she had a CT-scan, and then an ultrasound. Our driver had our luggage in the car, and he waited for us to be finished, and then picked us up and delivered us back to the San Francisco Airport. We again blitzed through security, and then spent a pleasant hour shopping at souvenir stores at the airport.

Until this point, everything had gone smoothly. And then – well, nothing can ever be perfect, right? We were on our airplane, ready for our 7 PM take-off, when the pilot suddenly announced that there was a problem with one of the engines and we had to sit on the runway while they checked it out. After about an hour, they finally told us to get off the plane and take all our belongings, because they were going to have to move us to a DIFFERENT plane, one which wasn’t arriving until 9:45 PM. Gah! This was upsetting and frustrating, but what can you do? And, as I told Miranda, better that they should have discovered the problem with the engine on the ground, before we took off… Anyway, to make a long story short, we finally got on the second plane, which ultimately took off at 11 PM, about a half hour after we should have been dropped off by our driver at home. Miranda slept pretty much the whole plane flight, and then we struggled our way across the airport and through customs at 1:30 AM.

Luckily our driver had stuck around to pick us up (yes, he gets paid to do so, but still). However, I had a moment of serious dismay when I saw what kind of vehicle he was driving – an extra-large SUV. All of our other drivers on this trip had had luxury sedans, which worked just fine, but I knew Miranda was going to have a hard time stepping up into this high vehicle. Here was my poor girl, who was extra-sleepy, tired, and out of sorts, and we had to figure out how to get her inside the car. We tried a few different things, to no avail. Then, just as I was about to declare this to be a failure, our driver found a luggage cart nearby which he held securely in place while Miranda stepped first onto the cart, and then into the vehicle. PHEW. We arrived home at 2:30 AM, 4 hours later than expected.

We’ve now been back at home for 2 days, and I can see that this flare-up has significantly changed Miranda’s mobility. She can still walk – which is a relief – but she needs to go more slowly. However, her ability to handle stairs has taken a major hit. Prior to this flare-up, Miranda could bend her left knee normally, but her right knee could only bend to just less than 90 degrees. This meant that to go upstairs, she would lean against the left staircase wall, bend her left leg and step up, and pull the stiffer right leg up after it. Down was the reverse; she would step down with the right leg, then bend the left leg and pull it down to join right. While this sounds awkward, it worked fine and she could do it with no help. Now, with a left leg which also can’t bend the whole way – though still can bend more than the right – Miranda steps up with the left leg, but has to put her foot considerably further out along the stair, and after drawing her right leg up, she has to then inch her left leg backward to join the right before she can take the next step. This is a slower process, and further, due to the flare-up, weakness, and pain, Miranda is more unsteady. In our crazy-ass townhouse, which has two sets of stairs, we’re having to make sure that an adult is always with Miranda directly behind (for up) or in front (for down) to brace her and make sure she doesn’t fall. Oy. In fairness, she may become more steady and regain some modest amount of movement once the flare-up ends, but I still think this is mostly a permanent change for her.

What does the future hold? We had already been planning to sell our townhouse in 2017 and move to a one-level home, but this experience has held a cattle-prod to our butts. We will be proceeding as soon as possible.

 

PS – In case anyone was wondering, Miranda doesn’t qualify right now for the FOP drug Palovarotene, which is still in clinical testing. It just happens that right now, there are a couple of types of tests ongoing, but Miranda doesn’t fit within the window of the type of subject which they need. Therefore, she was not put on Palovarotene.

One Response to “A flare, and a short trip to San Francisco”

  1. Sarah says:

    Hi there. My son Corey, whom just turned 14, was diagnosed with FOP about 1 month ago, after a long process, of course! We also see Dr. Hsiao in SF, we live in Lincoln ca. I am really lost! They say he is a mild case and he does not possess the typical malformations that accompany the disease. But non the less, he continues to have flare ups in his back for the past few months. I really don’t know how to go about clinical trials or anything else. If it is at all possible for some help in the right direction I would greatly appreciate it! Bless your family. Bless your sweet girl!



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