MOTHER'S NAME: Kim
AREA: San Antonio, TX
DIAGNOSED: Sep. 3, 2003
BD INVOLVEMENT: Arthritis, rashes/skin lesions, mouth/throat & genital ulcers, bladder & GI involvement, fevers, fatigue, Eye involvement?
POSTED: November 30, 2003
Hannah was just diagnosed (tentatively) with Behcet’s about two months ago, four days after her 6th birthday. She has "always" had allergies, rashes, tummy aches, etc., but was a fairly healthy, typical kid until she had her first arthritis flare at 3 1/2. It was awful; she couldn't even move she was in so much pain, in so many different joints. She was quickly diagnosed with ANA+ polyarticular JRA then, but Vioxx got it under control within 4 months, & we thought she was in remission for the next 2 yrs., except that she did seem to be stiff in the morning & after sitting for long periods.
Then, just before this summer, after a bout of strep. that took several months to kick (we almost removed her tonsils), Hannah developed a new, weird, psoriasis-looking rash on her legs that no one could get rid of. The only thing that helped was covering her legs (sun made it worse), so she wore long jeans in the Texas heat for a while, and it finally went away. She also started complaining of heartburn and joint pain occasionally, but neither was ever severe & they never lasted long.
Right after that, we saw an allergist because I wanted us all to start shots. (The whole family is terribly allergic & I was growing tired of the pharmacy in our kitchen. I had no idea about pharmacies yet; Hannah was only on Allegra & Nasonex at that time.) The allergist didn't want to give Hannah shots until she was cleared by a rheumatologist because of Hannah's history and because she suspected that the new rash was rheumatic, not allergic, in nature. I thought it was just a formality & that the new rheumatologist would blow us off (our old one did, since Hannah had never had warmth, redness or swelling), but, when we got in his office, a month later, Hannah started telling him about all sorts of achy, sore joints that I had no idea about, and, on exam, he found that she had lost some range of motion in her shoulders, hips, and back, plus had a little warmth and swelling in her knees. He tested her for spondyloarththropies (all her tests were negative this time except for makers for allergies and general autoimmune activity) & put her back on the Vioxx, Hannah started PT instead of allergy shots, and I felt like a real dolt!
It was shortly after that the BD-specific symptoms started. At the end of July, Hannah called me into the bathroom to show me that her genitals were red & sore & had a goopy discharge. I took her to the ER, thinking that it was only a bacterial infection, but I really wanted to get the visit over with right away so that I could stay home the next day. It wasn't until I wiped her for a clean-catch urine (and Hannah hit the roof!) that I discovered the dozen or so open ulcers under the discharge. Behcet's was mentioned but dismissed, & Hannah was tested for every STD under the sun & sent to the Child Advocacy Center the next day (where she was already being seen because her dermatologist had also just misdiagnosed molluscum in her groin area as HPV). With that history, it really seemed like she had been molested, so they did some tests of their own but did nothing to treat her symptoms since they didn't want to mask any evidence. By the time we got the results back ten days later (everything was negative, except that she was severely dehydrated, presumably because she had stopped drinking so that she wouldn't have to pee), the ulcers had gone away on their own.
Unfortunately, the ulcers came back with a vengeance after just a few days, so I made an appt. with Hannah's pediatrician. That next AM she started having stomach pain with vomiting & diarrhea. All her labs were normal again, except that she was still dehydrated, so we started making a huge deal about drinking plenty of water, watching her down a 16 oz. water bottle every time she took her meds. (The pedi. assumed that the dehydration was from being sick, but Hannah admitted that she still wasn't drinking.) We treated for infection (Betadine sitz-baths AM & PM, and holding Hannah down to apply antibiotic & antifungal ointments to open genital ulcers), got sent back to her dermatologist who changed the regimen (Aveeno sitz-baths AM & PM, & holding Hannah down to apply *different* antibiotic & antifungal ointments to open genital ulcers, plus Desitin after every trip to the bathroom), and were referred to assorted urologists and gynecologists who wouldn't see us. (None of the GYNs would see Hannah because they didn't see children, and none of the urologists would see her because they either didn't see children or didn't treat genital ulcers, saying that's a GYN's job.) Then the bottom fell out. It was all very fast.
First, Hannah developed light sensitivity, which made me panic, but her opthal. exam was normal. (She did have a corneal abrasion just before the BD symptoms started, and perhaps it was just related to that.) Then she started going to the bathroom every 20-30 min, so we started her on Ditropan, which did help for a while, but then her urethral area started bleeding & swelled up to about 5x its normal size, her bladder started screaming at her when she peed (which was all the time, since the Ditropan wasn't helping anymore, even though we kept increasing it), and she finally lost bladder control nearly altogether. Her belly started hurting & swelling up after every meal, & she had diarrhea as often as not. Her eyes started burning & got red & watery, and, again, I panicked, but, again, the opthal. exam was normal. (With her history of allergies, the opthal. added Patanol & Refresh eye drops to her regimen.) Then she developed mouth sores, twice: just a few each time, but she had never had one before, and after the reading I had done on Behcet's, it was enough to make me throw a fit. (Now we're switching to a new pedi. since, once he became thoroughly stumped, our old one did nothing to help us figure out what was wrong with Hannah and even stopped returning our calls.)
At the end of August, I took Hannah out of school (she was rarely in class anyway, between dr. appts. & trips to the bathroom & to the nurse), and made appts. with every specialist who had ever seen her, one after the other. They all referred us to new specialists, and we saw all of them, too. By the end of the week, she had been poked & prodded & tested (mostly to rule out Crohn's), and three things had happened: First, the laxative that Hannah drank the night before her endoscopy & colonoscopy didn't take effect until that morning, and, after the tests, the gastro. accused Hannah of sneaking into the fridge the night before her tests because there was still food in her belly (strawberries, he thought). Hannah denied it, and I believe her, mostly because we had no strawberries at home that night, but she *had* eaten strawberries with her last meal before the tests, nearly 24 hrs before. (That sounded like delayed emptying to me, which might explain the belly problems.) Second, there was no evidence of ulceration anywhere in her GI tract, but there was evidence of esophogitis (apparently, the heartburn was a bigger issue than I thought, too), so Hannah started Protonix, which, so far, has done nothing for her. Third, and most importantly, Hannah's rheumatologist started her on Orapred (36 mg/day) which cleared up everything except the GI symptoms within 6 days. (I don't think I've ever seen Hannah so mobile & so *happy*!!) The rheumatologist saw her a week later and decided that "this is almost certainly Behcet's". I ordered Joanne’s books & a handful of the little blue info. cards & read everything cover to cover.
Since then, though, Hannah’s GI symptoms have just gotten worse. She was sent home from school one day with belly pain, nausea, and obvious distension, so her gastro. told us to take her right to the ER to rule out an obstruction. Of course, by the time she was seen, 6 hrs later, she was feeling better, & they dismissed it as a combination of constipation, gas, & weight gain from the steroids. Over the next week, she had the same symptoms every day, so we saw the gastro., who ordered X-rays & said that stool was backed up all through her belly & started her on Miralax every AM. That worked for a week, but now she spends about 30 min. after every meal at the nurse's office, just to recover. Her belly is so bloated all the time that her clothes don't fit right anymore, even though her arms and legs are still pencil-thin. After more tests, the gastro. decided that Hannah probably does have delayed gastric emptying--that it goes along with GERD--but he also says that's not a big problem and the Protonix should take care of it (which it doesn't). He also suggested that her bellyaches & bloating are a ruse to get out of class or just "abdominal pains of childhood" which she'll outgrow. He ordered labs to test for food allergies. But he claims that GI distress is not part of Behcet’s unless there's obvious inflammation in the GI tract. Since Hannah had no lesions on the endoscopy/colonoscopy, there's "nothing to treat", and she just needs to "learn to cope". More Miralax. Ugh.
Other than the GI issues, Hannah’s meds were holding her pretty well at first. The plan was to wean her off the Orapred & then start her on Colchicine, but when we reduced the 36 mg to 30 mg (still well above the recommended dose for her size), Hannah started having low-grade fevers & fatigue, she developed spots on her feet that looked like ant bites (but, then, maybe they were ant bites, even in October; this is Texas), ulcers cropped up way back in her throat, and she developed white blotches inside her cheeks that looked like they would erupt into ulcers too. (They never did, and the "ant bites" and true ulcers cleared up in about a week, but she's still having fevers and fatigue.) Her back pain returned about that time, but that could be from her arthritis, from her bladder, or from her belly: who can tell? We had started to taper the Ditropan, too, but had to go back to a full dose because bladder control became an issue again as well.
Hannah just saw her rheumy again last week, and we’re starting to taper the Orapred again, but soooo slowly this time. She also started taking Colchicine. If she tolerates that well, we'll increase it as we decrease the steroids.
Five months ago, Hannah seemed so healthy, and she was such a cheerful, social little thing. She was such a little doll, too! Strangers used to stop us to comment on how beautiful she was, how she should be a model. Now, her face is so round that the kid next-door calls her "Hannah the Blowfish", she's so hairy that she has a uni-brow & a moustache, she has bumps all over her face, and her bloated belly & skinny limbs make her look like a caricature. She’s so quiet & glum. Hannah hardly even seems like my baby girl anymore. It may seem shallow to focus on her appearance, but even when she's feeling good, I can't ignore what this disease is doing to her, and it just makes me so sad. And Hannah is getting so frustrated by all the stares and questions from people who knew her before BD.
Yesterday she came home from school tired, sore, & cranky, and complaining that her stomach had hurt "all day". She went straight to bed (with her wedge) and slept right through dinner; she doesn't even remember me waking her to give her her meds. When I woke her up this AM, she was still sleepy. We got her weekly work folder yesterday, too, and it was awful. Stuff I know she can do, but just didn't. She made this sad little face and sort of whispered, "Sorry, I tried." I'm starting to be able to tell how she was really feeling any given week by that work folder. The first week on Orapred, it was beautiful. Now it just seems that being present at school takes all the energy she can muster.
When I picked Hannah up from school for her last rheumy appt., she was already lying down in the nurse's office. Both her teacher and the nurse have approached me recently about what to do about her regular clinic visits (5x/day last week). She's missing so much class time. She rarely goes out to recess or to PE.
I know we're luckier than some because the steroids worked right away, and the symptoms that are left now are relatively mild. We’re luckier than most because we got a diagnosis within months of Hannah's first BD symptom, but what a roller coaster those months were! And I suspect that the ride is just beginning.