We had an appointment in the Genetics clinic a earlier this week. I've been mulling things over for a while now, unsure of how to post what we learned.
For a long time some of Ricky's other doctors and I have been wondering if there might be something else going on with him... Some other genetic disorder. You may have seen me post about this here before. Among other things, he has these signs of "something else":
-hyperflexible joints
-high, narrow palate
-heterotopias (undeveloped gray matter) in the brain
-soft, thin skin
-thin veins
-issues with teeth
-issues with vision
We saw the geneticist back in 2006 and were supposed to go back a year later. Unfortunately, with Misty being born, time slipped by and I just finally got him another appointment. This time around, the geneticist did a lot of diagnostics, including examining Ricky's fingers and toes, measuring his eyes, his armspan, etc.
Finally, the geneticist and genetic counselor explained what they planned to do. First of all, blood was drawn for a full chromosome analysis. They did this when we were there in 2006, but as time goes on there end up being more and more genes identified. As the geneticist says, "In genetics, time is on our side." My blood was also taken as a control for this test. Secondly, he had blood drawn for another test that is for one of the connective tissue disorders that relates to the heterotopias -- it's more common in females . One of these tests will take 6 weeks (done at Stanford) and the other one will take 8 weeks (sent to Boston). If they are both negative, he will then have testing for Ehlers-Danlos Syndrome. Based on what I see on that page... Well, it really sounds like Ricky.
Here's something else I learned: When Ricky had his EKG (heart ultrasound) in April of last year, when he was in the hospital for his CF and was also having his dizziness investigated, the result was a finding of an enlarged aorta. Now, that would have been a useful thing to know, wouldn't it? I was pretty upset to find this out in via an offhand remark by the geneticist! The genetic counselor subsequently emailed Ricky's pulmonologist, who out of all of the specialists basically manages his care, to ask about a visit to a cardiologist. The pulmonologist agreed that Ricky should see a cardiologist to find out if there is really a problem. (Heart issues are also common in some connective tissue disorders.) So we are waiting for a call on that.
I have mixed feelings. If Ricky does have Ehlers-Danlos or some other connective tissue disorder, it would be good to know. It would be nice to have some answers. On the other hand -- jeez, how much does one kid have to deal with!?
In CF news, today I got a call back from the CF nurse in regard to Ricky's visit last week. His DEXA (bone density) test was normal (yay). His sputum culture showed pseudomonas aeruginosa and stenotrophamonas maltophilia, both bugs he has had before, though the second one is fairly new. We'll start up his TOBI (inhaled antibiotic) tomorrow.
The rest of us are doing okay... Misty apparently wants some health attention of her own (well, okay, I know she doesn't WANT it) and has a virus that's made her wheezy and miserable. She was seen by her pediatrician this week and her chest x-rays were a bit iffy for pneumonia. So we are monitoring things. It's probably just a virus and hopefully she improves soon. She has been having breathing treatments regularly. The coughing spells are pretty brutal on her. :(
Guess that is it for now!
Showing posts with label stenotrophomonas maltophilia. Show all posts
Showing posts with label stenotrophomonas maltophilia. Show all posts
Thursday, March 4, 2010
Wednesday, November 19, 2008
Ricky news
I will get back to reporting on our San Francisco trip tomorrow, but for now I wanted to give a couple of updates on Ricky.
Today I was actually home with Andrew, who was sick with a sore throat and cough (which hopefully the other two kids and I do not catch). I hated to miss work, but it was also nice to get a few things done around the house and run some errands.
In the afternoon as I was clipping coupons, I got a call from the nurse at the CF clinic. She said, "I'm calling you about Ricky's sputum culture results..." and I felt my heart drop. They never call unless it's bad news. I was envisioning the really bad bugs... cepacia, MRSA. But thank goodness, once I caught my breath, I found out that it was neither of those. The news is that he is still culturing the stenotrophomonas maltophilia that he grew the last time, and also that he's showing pseudomonas again. This is one he has had off and on since he was 2 (but went a number of years without culturing it). Now, however, he is culturing two strains. One of them is most prevalent, and there is a secondary strain that showed just a little spot. They are not mucoid. The good news is that all three of these bugs are sensitive to multiple drugs. The best one is Ciprofloxacin, but Ricky gets a nasty arthritic reaction to Cipro, so unfortunately we can't use it. The nurse called in a prescription for Septra (double strength -- that'll be fun for his intestines) and instructed me to put him back on the TOBI inhaled antibiotic, which I'd already called in a refill for since he was supposed to start it a while back. I'd put it off because of the constant change in insurance. After he has been off the TOBI for a week, we go back in for another sputum culture. Keeping our fingers crossed!
Later in the afternoon, when I was waiting for the boys at separate appointments at the same place, the endocrinologist finally called me back. I had called Tuesday late in the day and again this morning. Today I was told that they were not in today. Huh. I had asked the CF nurse to look at the numbers for me, and she told me that they were all within normal limits. I knew that wasn't usually the whole story so I waited to hear from the endocrinology fellow. When she called, she informed me that his thyroid levels are fine. His prolactin hormone level is fine (a high level could have meant a problem with the thyroid, I believe). His growth hormone and testosterone are on the low end of normal. This means he is definitely not in puberty. I told her I'd been unable to find the CD of the MRI images (which is making me nuts, believe me). She said they'd have no problem doing another MRI when he goes back in a few months if his levels are still not rising. She also said that they wait til boys turn 14 before they worry too much about puberty being late (that's a year from now). At that time they would consider testosterone therapy.
So, whew. That's a lot of news for one day. The good news is that he's not sick at the moment (at least not that we can see! the lungs are another story). He is doing okay in school (not the most spectacular report card last week, but he's trying harder now). And he's a happy, smart, fun kid. :)
Today I was actually home with Andrew, who was sick with a sore throat and cough (which hopefully the other two kids and I do not catch). I hated to miss work, but it was also nice to get a few things done around the house and run some errands.
In the afternoon as I was clipping coupons, I got a call from the nurse at the CF clinic. She said, "I'm calling you about Ricky's sputum culture results..." and I felt my heart drop. They never call unless it's bad news. I was envisioning the really bad bugs... cepacia, MRSA. But thank goodness, once I caught my breath, I found out that it was neither of those. The news is that he is still culturing the stenotrophomonas maltophilia that he grew the last time, and also that he's showing pseudomonas again. This is one he has had off and on since he was 2 (but went a number of years without culturing it). Now, however, he is culturing two strains. One of them is most prevalent, and there is a secondary strain that showed just a little spot. They are not mucoid. The good news is that all three of these bugs are sensitive to multiple drugs. The best one is Ciprofloxacin, but Ricky gets a nasty arthritic reaction to Cipro, so unfortunately we can't use it. The nurse called in a prescription for Septra (double strength -- that'll be fun for his intestines) and instructed me to put him back on the TOBI inhaled antibiotic, which I'd already called in a refill for since he was supposed to start it a while back. I'd put it off because of the constant change in insurance. After he has been off the TOBI for a week, we go back in for another sputum culture. Keeping our fingers crossed!
Later in the afternoon, when I was waiting for the boys at separate appointments at the same place, the endocrinologist finally called me back. I had called Tuesday late in the day and again this morning. Today I was told that they were not in today. Huh. I had asked the CF nurse to look at the numbers for me, and she told me that they were all within normal limits. I knew that wasn't usually the whole story so I waited to hear from the endocrinology fellow. When she called, she informed me that his thyroid levels are fine. His prolactin hormone level is fine (a high level could have meant a problem with the thyroid, I believe). His growth hormone and testosterone are on the low end of normal. This means he is definitely not in puberty. I told her I'd been unable to find the CD of the MRI images (which is making me nuts, believe me). She said they'd have no problem doing another MRI when he goes back in a few months if his levels are still not rising. She also said that they wait til boys turn 14 before they worry too much about puberty being late (that's a year from now). At that time they would consider testosterone therapy.
So, whew. That's a lot of news for one day. The good news is that he's not sick at the moment (at least not that we can see! the lungs are another story). He is doing okay in school (not the most spectacular report card last week, but he's trying harder now). And he's a happy, smart, fun kid. :)
Monday, October 6, 2008
Update!
I've been getting a lot of messages from people who are curious about how Ricky is doing. He is doing okay! We got an appointment at 10:30, as previously mentioned, and headed up there.
Ricky slept most of the way there. When we got there he did not have an x-ray like last time, but he did have his vitals taken and have spirometry. His oxygen saturation was up to 97-98, which was a definite improvement, and his spirometry was actually almost up to baseline. Also, his lungs sounded not too bad. There was some consolidation in the lower lobes, which follows what has been going on with him. Ricky repeatedly complained about his upper chest and throat being congested.
I found out that his sputum culture from last week had eventually grown a bug that he hasn't cultured in many years... Stenotrophomonas Maltophilia. I just looked back in the Cystic-L archives and found that it was 2001 when he last cultured it! (I'm actually going back and transferring that message to Cystic-L over to this blog, so if you see it pop up in my feed please don't be alarmed!) The good news about this bug, though it is a medium-bad one, is that it is sensitive to a few good midrange antibiotics, and the one they chose to put Ricky on is Bactrim. He will be off the Rifampin (no more orange pee, tears, sputum, and spit!). He is also going onto a slightly longer course of Prednisone and they upped his Singulair dosage to a higher one. He has been on the 5mg chewables and he weighs enough now to go to the adult dose of 10mg non-chewable tablets.
Before we left the clinic, Ricky had blood drawn for an aspergillus panel. Looking back, I see that when he was hospitalized in 2001 for the s. maltophilia, was when he first had issues with aspergillus (which is a mold that can grow in CF lungs because of all of the antibiotics that CFers are on).
We are also going to continue the increased treatments and hope for the best. I am really hoping that he can go back to school tomorrow. Keep your fingers crossed. :) He'll be getting extra treatments still. If he gets worse we need to go back in, but at this point I'm thinking that he'll start to improve. The Prednisone is going to go on longer this time, and that should help... It was after he finished the Prednisone before that he started to feel worse.
Thursday is when they'll be doing the prizes for the magazine fundraising drive that Ricky was so excited about, so at the very least it would be good if he were back by then.
So, yay. :) Go Ricky!
Ricky slept most of the way there. When we got there he did not have an x-ray like last time, but he did have his vitals taken and have spirometry. His oxygen saturation was up to 97-98, which was a definite improvement, and his spirometry was actually almost up to baseline. Also, his lungs sounded not too bad. There was some consolidation in the lower lobes, which follows what has been going on with him. Ricky repeatedly complained about his upper chest and throat being congested.
I found out that his sputum culture from last week had eventually grown a bug that he hasn't cultured in many years... Stenotrophomonas Maltophilia. I just looked back in the Cystic-L archives and found that it was 2001 when he last cultured it! (I'm actually going back and transferring that message to Cystic-L over to this blog, so if you see it pop up in my feed please don't be alarmed!) The good news about this bug, though it is a medium-bad one, is that it is sensitive to a few good midrange antibiotics, and the one they chose to put Ricky on is Bactrim. He will be off the Rifampin (no more orange pee, tears, sputum, and spit!). He is also going onto a slightly longer course of Prednisone and they upped his Singulair dosage to a higher one. He has been on the 5mg chewables and he weighs enough now to go to the adult dose of 10mg non-chewable tablets.
Before we left the clinic, Ricky had blood drawn for an aspergillus panel. Looking back, I see that when he was hospitalized in 2001 for the s. maltophilia, was when he first had issues with aspergillus (which is a mold that can grow in CF lungs because of all of the antibiotics that CFers are on).
We are also going to continue the increased treatments and hope for the best. I am really hoping that he can go back to school tomorrow. Keep your fingers crossed. :) He'll be getting extra treatments still. If he gets worse we need to go back in, but at this point I'm thinking that he'll start to improve. The Prednisone is going to go on longer this time, and that should help... It was after he finished the Prednisone before that he started to feel worse.
Thursday is when they'll be doing the prizes for the magazine fundraising drive that Ricky was so excited about, so at the very least it would be good if he were back by then.
So, yay. :) Go Ricky!
Wednesday, February 21, 2001
Icky news (post from 2001)
I finally talked to Ricky's NP at the CF clinic and he cultured two bugs last
week.
(1) Serratia which I guess we've figured out isn't dangerous at all.
(2) Stenotrophamonas Maltophilia (sp?) which really knocked the wind out of
me.
Just when we were starting to do so well, now this. I guess we can't ever do
WELL with this disease, we just have to keep getting knocked for a loop.
week.
(1) Serratia which I guess we've figured out isn't dangerous at all.
(2) Stenotrophamonas Maltophilia (sp?) which really knocked the wind out of
me.
Just when we were starting to do so well, now this. I guess we can't ever do
WELL with this disease, we just have to keep getting knocked for a loop.
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