Updates

Things have been busy for Ricky. He has had a number of absences from and short days at school due to dizzy spells, migraines, and fatigue. We still don't know what's going on with all of this. Migraines do explain some of the symptoms he has been having, but not all.

Ricky is getting a one-to-one aide at school because he's been having trouble adjusting to high school and its routines. He has been trying very hard in school, though, and his favorite class is 3D design.

Most recently, Ricky had some teeth pulled. He had four (adult) bicuspids that were crowding his mouth and making it so that his adult cuspids could not grow in. He had the right ones pulled several weeks ago, and last week he had the left ones pulled. The extraction on Wednesday of last week were very difficult, involving the dentist having to dig them out of his gums. Ricky got stitches and was in a lot of pain. He also developed a fever and we were scared he had some sort of infection. The dentist and pediatrician conferred and Ricky was started on Septra. By the weekend he was doing a lot better and he was back to school today, I am happy to say!

We've had some close shaves with bowel blockages lately, including an ER visit. He's back on Miralax now though and hopefully it will continue to help.

For Halloween, Ricky was a Ghostbuster. I'm not sure, but this might be the last time he trick-or-treats. I can't believe he's that old already! (He'll be 15 years old this month!)


Guess that's all the news for now. Thank you all for sending Ricky cards and letters. He still loves to get mail!

Long overdue update (again)

I can't believe it's been almost three months since I last blogged here. So here's a thorough update on Ricky.

Last I blogged, I had just taken Ricky to the dentist up at UCSF. He was supposed to get his teeth pulled several weeks after that.

I took him back in July and that day he fell asleep on the way to UCSF. I went to wake him up when we got there, and his nail beds and eyelids were blue! He got a little oxygen in the dental clinic and didn't end up having his teeth pulled. In fact, we ended up spending the afternoon and evening in the UCSF emergency room making sure he was okay.

So Ricky has been rescheduled and is going to get those teeth pulled next month. We are going to try for doing all three, under only local anesthesia. If that doesn't work for any reason he'll go under general anesthesia. Hope it all goes well.

We spent much of the summer doing summery things... Day trips to the beach, a Weird Al concert, a They Might Be Giants concert, trips to Gilroy Gardens. We had a lot of fun!


At the beach with friends.


At the Weird Al concert.

Ricky also spent a lot of time doing this:



I have been back and forth with the neurologist. Ricky has been to see him a couple of times and is going again this week. Ricky sleeps a lot, has had these absence-like seizures, and the dizzy spells combined with nausea and extreme tiredness, throughout the summer and into the fall. The neurologist offered Imitrex to help with the spells. Ricky's supposed to take them when he starts to feel rotten. We have used a pill once. It took away the headache and dizzy spell but knocked him out for hours. More on this farther down...

So school started in late August.



First day of high school!

Yeah, first day of high school. Where did my baby go? The first week was really rough. Ricky became pretty severely dehydrated on a couple of occasions. It was a really hot week. I took him in two flats of bottled water that's kept in his special education classroom for him to use as much as needed. I also bought him some Gatorade more recently, because water may not be enough.

He has also had some neurological issues... Sleeping or passing out and becoming unresponsive. Two times were serious enough that the school personnel called 911. The first occasion, I was able to rouse Ricky and he did not go to the hospital. On the second occasion, I was up at Stanford with Andrew at an appointment and the school nurse called to report that Ricky was experiencing nausea and dizziness. I thought it might be a migraine but I couldn't get there very quickly. As happened on the first occasion, an ambulance was called. The second occasion, I couldn't get there on time and he was transported to the local emergency room. A CT scan and blood tests were done and they couldn't find anything wrong.

We are going back to the neurologist this week and I am hoping we can get to the bottom of things. This is so frustrating. I am also meeting with people from the school this week to talk about a plan.

Ricky started the school year in mainstream 3D design (which was called crafts when I went there -- yes, to this same high school), mainstream Algebra, and mainstream PE. As of this Monday, he will be moving into a math class in his special education class, and adaptive PE. The Algebra class was way too fast paced for him, though he really did try to make it. He was apparently making a great effort. The PE class is made up of way too many students for Ricky to get the help/accommodations that he needs so he is transitioning into adaptive PE with another student in his special ed class. These sound like good solutions.

This month, Ricky saw the gastroenterologist and the pulmonologist. He has grown a bit taller and put on some weight -- he's 5'3 now and around 100-101 pounds. Tall and skinny. He does eat well so hopefully we will see more some improvement in his growth; his growth curve has flattened out and dropped within the past several years.

Pancrecarb, the digestive enzyme that Ricky has been taking for many years, was un-approved by the FDA this year -- apparently for no reason except for it being at the request of the Cystic Fibrosis Foundation. This left us with a quandary: What enzymes should he take? We ended up going with Creon, which he took for about five years starting with his diagnosis right after birth. Well, this time, in ways I will not enumerate here to save him some embarrassment (and since I've already Twittered about them), these Creon enzymes are not agreeing with him. He actually missed two days of school. The nutritionist at the CF clinic has offered up several possible solutions and so far they are not working. So we are hanging in there, hoping that at some point the FDA will re-approve Pancrecarb.

The pulmonology (CF clinic) appointment went great. Ricky's lungs are sounding and performing great. His growth, as mentioned, is going well. Hooray Ricky!

We go back to the pulmonologist and gastroenterologist in December.

Ricky's bipolar disorder is currently stable. He is due to go back to the psychiatrist. This summer he again attended camp for bipolar children and teens and he did great. He also made a new friend with whom we've had a couple of visits -- including a trip to the Palo Alto Airport Day last weekend... Ricky, Andrew, and Ricky's friend got to ride in a small plane, and had a blast! Here's a picture.



So I guess that just about catches us up. I promise not to take so long before I update again!

Neuro, dentist and a very important question

Ricky and Andrew on Lombard st.
Originally uploaded by Beckerbuns

I blogged last about what I thought was an absence seizure. The neurology nurse finally called me back after I called and left messages twice. She said it sounded more like a complex partial seizure, because people who have absence seizures usually don't have the other kinds that Ricky has had (complex partial and tonic-clonic). She suggested we up his Trileptal... Going up 1/2 a tablet in the evenings this week and going up another 1/2 tablet in the mornings next week.

I asked AGAIN about the dizzy spells. She told me that there really is nothing else they can do for him. This is not the first time I've been told this but I'm just stunned that this is really what they're telling me. They've done all kinds of tests and he has been tried on one med for this: Topamax. That's all they can do? Really? More about this in a bit.

Today I took Ricky to the dental school at UCSF. His regular dentist referred us there because they were uncomfortable treating him with all of his meds and medical history. (The dentist he's had for years never had any problems with it, but whatever.) We met with a very nice dental resident (and his pushy attending) and went over a thorough history. Ricky had x-rays and a cleaning. No cavities! The dentist was actually shocked that Ricky had no cavities, with his very dry mouth (due to CF or meds, not sure which), poor dental hygiene, and congenital lack of enamel on some of his teeth.

So, the plan is for us to go back there in a few weeks for Ricky to have four of his permanent teeth pulled out to make room for four other permanent teeth that have no room to come in. Once that is done, he can have orthodonture. That's another fun thing to come... Do we pay out of pocket for ortho stuff down here, or do we try to get Medi-Cal to pay for it under medical necessity and do it through UCSF? That would mean going up there every time he needs and adjustment, which would be a pain in the butt. It's an hour away and well, it's in San Francisco, with lots of traffic. Guess we'll see.

As you can see from the picture, the boys and I took the opportunity to do some sightseeing while we were in San Francisco today. That's them on Lombard Street, the crookedest street in the world. I have to say, I was very impressed... We all walked all the way down the crooked block... And then we all walked back up. Ricky hoofed it and made it to the top a good five minutes before Andrew and me. Andrew and I were huffing and puffing and taking breaks all the way up. The kid with lung disease made it up there first! Go Ricky!

Okay, here's the last thing. I think it's time to get a second opinion about Ricky's dizzy spells. It has gotten to the point that he has been knocked out sleeping most of the day 3-4 days per week. He can't stand in lines for long. He has decided to quit soccer because he can't make it through a single game without feeling like he's going to fall over.

So my question is... Aside from Stanford, where are the best/most knowledgeable pediatric neurologists in California? I'm willing to take this to southern California too. I am trying to tap into the knowledge of my friends out there who might have knowledge of these sorts of things. Please put your feelers out and spread the word. Ricky is going to high school this fall. He can't keep dealing with this stuff! We need to find an answer!

Thanks everyone. :)

Ricky's week

Ricky went to the pulmonologist this week for a CF checkup. His O2 saturation was down just slightly, he'd grown a tiny bit (but his growth curve is still flat), and his spirometry looked great. He's still got the end of the sinus infection. He's going to start on Periactin again. It's supposed to make him have a bigger appetite. He was on it for a couple of years a while back and it never seemed to do anything, but we'll see what happens this time.

A couple of nights ago, Ricky had what was clear to me to be an absence seizure. He was sitting up watching TV and doing his breathing treatments, and I went over to give him his next nebulizer. He didn't respond to me. Then I noticed that his eyes were open but rolled back in his head. I thought maybe he'd gone to sleep but it didn't make sense because he was sitting straight up. Plus, he had had a 5-6 hour nap that day and by rights, should not have been sleepy even though it was 10 at night. About a minute later, he came out of it and asked Andrew and me what had happened. He was pretty confused and it was clear now that he had *not* been sleeping.

I called the neurologist's nurse yesterday (well, Thursday) to report the seizure. She has yet to call me back -- which seems pretty irresponsible. I want to know what happens next. Change meds? EEG again? Guess we'll wait and see.

The graduate

IMG_5668_cropped
Originally uploaded by Beckerbuns

Thursday of last week, Ricky graduated from middle school! It has been a long, hard road, but he made it. He graduated with 300 classmates. On Saturday we had a graduation party at the local pizza joint.

Last week, Ricky also visited the gastroenterologist. He has grown a little but his weight is basically the same and his growth curve has flattened out. His lungs sounded great, though, even though he has been battling a sinus infection.

Tomorrow it's back to the pulmonologist. Hopefully all is well. Next month he has summer school and bipolar camp.

Sick

Ricky is sick... He started with a cold on Memorial Day and it went to sinus infection and then double ear infection. He is so congested and miserable and he has been out of school all week.

I got him to the pediatrician Friday. I am not crazy about this pediatrician. She usually writes us off, saying "Oh, it's viral." This time she took us seriously and prescribed Bactrim. His O2 sat was down to 95 (low for him) but apparently his lungs sounded fine! If he's not doing better by Monday or seems worse, we need to call again.

Journal prompt for last week -- my relationship with Ricky

I'm participating in a study that involves mothers of children with volatile behaviors journaling on a regular basis. Here is my entry from last week. I needed to write about a relationship. I chose to write about my relationship with Ricky.

My relationship with my son, Ricky, is complicated. He is not a touchy-feely type of kid, but when he is happy to see me he will lean on me and love on me and I know that he loves me. He is often standoffish in social situations, because he doesn't know how he should be acting. But when he leans on me and loves on me, I know he's either not feeling well, or showing me that he loves me.

Recently, a new dynamic has come into play -- Ricky becoming a teenager. I get a bit of attitude from him now. He is hostile and even aggressive toward his brother. I know that, even though he's a wiry, skinny kid, I can probably not restrain him now if he gets to be out of control. And I am on my own, a single mom, so if that happens, it's all on me.

Nowadays he frequently moans and groans at me about his breathing treatments. When he is angry and volatile, sometimes he refuses his meds. That really scares me too. I try to be patient with him but sometimes it's hard to remember that he's different from most kids -- that he doesn't always understand. Most of all, when he's in the middle of a rage or is being unreasonable, it's not under his control. He can't understand reason. When I try to reason with him at one of these times, it's like talking to a brick wall. I have to wait until he has calmed down and only then can we go over what happened and try to figure out a solution.

In the end, and at the end of the day, Ricky is my oldest. My first baby. My initiation into parenthood. And what an initiation it was... A premature baby with multiple health, and later, developmental issues. But from the moment I first held him, when he was a couple of weeks old, I knew I was his mama. My relationship with him has not always been solid or easy, but I am always his mom. I think he knows that, even when he's having a rage. I don't know what I'd do without him. I know I'm going to be facing that eventuality because of his cystic fibrosis... He won't always be around. But for now, I am going to enjoy the relationship I have with this amazing kid.

Long-overdue update!

So, what's been going on with Ricky, you might ask? :)

Lots!!! Where do I start? Well, his cardiology appointment started with an ultrasound of his heart. It still showed a dilation of the ascending aorta, just like a year ago. The cardiologist let us know that it had not changed in size from a year ago. She drew a picture of the heart and its arteries and veins and explained that most likely this would never cause Ricky problems. On the other hand, this sort of defect is almost never found in a child who does not have a syndrome of some type.

And in that department, a couple of weeks back I got word from the genetic counselor that the first genetic test, which was a long shot anyhow, had come back negative. This week, I got a call from her about the other test we were waiting for, a genetic array. This time, an abnormality was found. Ricky has extra genetic material (known as a duplication) on one part of chromosome 11. We are not sure yet what this means -- in truth, the field of genetics is really still in its infancy. The next step is for them to test my DNA (they already took blood when they took Ricky's 2 months ago) to see if I have this too, and if not, they'll want to test the boys' dad. He has offered to be cooperative with medical stuff, so we shall see (if it comes up). On the other hand, from what I have read online, many times these sorts of defects happen spontaneously during or just before conception, and have nothing to do with inheritance. So mysterious!

Ricky has had one ER visit lately; it was due to some pretty severe abdominal pain. It turned out to be nothing, and his lungs look(ed) great. He has been on Dulcolax on a regular basis since then and it seems to help.

He is still having pretty bad dizzy spells. I am so frustrated with the neurologist that I haven't called there in a while. I was supposed to call the nurse to tell her how we had decided to proceed. I upped Ricky's morning dose of Topamax, which hasn't done a lot, but may have improved things.

Had a recheck with the psychiatrist, mostly to check in, and things are looking good.

I took both boys to their new dental center, which was amazing! Disney Channel, video games, all kinds of fun stuff. Wow. Unfortunately they were unable to work on Ricky's complicated teeth... He needs to have his bicuspids (all four -- adult teeth) removed so his cuspids can come in (I may have that backwards). He needs this stuff done before he can have orthodonture. This dental center felt uncomfortable doing this work (or even cleaning his teeth) given his medical history and all of the meds he is on. They gave me a referral to take him to the dental school at UCSF, where the procedure would be supervised by a physician as well. I am glad they're being careful, but jeeeeez.

School is going okay. He has good and bad days. He has days where he refuses to do work at all. We had his IEP meeting last month and decided on his placement for high school -- it happens to be the same place I went to high school. I need to make an appointment for Ricky and me to go visit the classroom. I met the teacher at the IEP meeting and the program sounds really good.

There have been sibling... Issues. Ricky and Andrew both have limited understanding of what Misty is capable of "getting". She wrecks their stuff, that sort of thing, and they can't figure her out. They think she is being malicious but I honestly believe that at her age (almost 4) she is mostly being... 4. Curious.

Ricky's digestive enzymes, Pancrecarb, have not been approved by the FDA by its digestive enzyme approval deadline (April 28), so the CF clinic staff has been scrambling to get people set up with new enzymes. We have quite a few bottles of Pancrecarb left, but after he runs out he'll be on Creon. We are not sure of the future availability of Pancrecarb, which appears to have a grim outlook. Creon was what Ricky was on from when he was diagnosed to when he was put onto Pancrecarb, so it will probably be okay. It just sucks to have to switch to something else after what he's been on all this time has done so well for him.

His labs also came back with his vitamin D being significantly low, so he's going to have to go onto an extra supplement (besides what is in his multivitamin) for a little while. Don't want him to get rickets! Yow!

So, now we wait to see how things go with the genetic tests. CF-wise, Ricky has been out of the hospital since November and things are looking good. His lung function is great. :)

That's all for now!

update - doctor visits

Week before last, Ricky saw the neurologist about his increased fatigue and dizziness on the increased Topamax dose he's been on for a while. The decision was made to reduce the dose back down to what it was before and see what happens.

Ricky had an eventful week last week. On Monday, in addition to a bunch of other labs that he gets done yearly, he had his three hour glucose tolerance test, which ended up being normal, yay. That day he also saw his gastroenterologist and that went well, too.

On Tuesday, we had an eventful day. Ricky saw the endocrinologist, who looked over his other blood tests from the day before. His thyroid level was, as always, borderline low. Most of the other stuff looked normal. He has grown two inches in the past year, and the endocrinologist decided to put off any intervention with regard to his delayed puberty. They want us to come back in a year.

Next he had his yearly full pulmonary function tests. Those went well, and his numbers looked great.

Then Ricky saw the pulmonologist. His weight has flatlined and his growth curve is actually dropping. We're supposed to push lots of weight gain shakes. His lungs sounded great and the doc was pleased with the results of the PFTs. His iron was a bit low too, so we're temporarily decreasing his iron supplement frequency.

Ricky's dizzy spells, meanwhile, have been debilitating now that the Topamax dose has been decreased. Monday he spent much of his school day sleeping off another dizzy spell. So I called the neurologist's nurse to ask what we should do. She called me back to tell me she'd ask the doctor and get back to me. She called back and left a voicemail... I couldn't believe her response. Either we increase the dose back up... Which will make him sleep all day again... Or he just "gets used to the dizziness sensation." Seriously? What kind of a choice is that, exactly? I was so livid I couldn't even call her back to tell her what our decision was (for now, I have not increased the dose). I don't know what to do. Second opinion? Angry voicemail to tell her just what I think of her message?

I've also been playing phone tag with the cardiology clinic. Ricky is supposed to go there for another heart ultrasound with regard to the enlarged aortic valve that the geneticist mentioned (the pulmonologist explained it to us, so now I know a little bit more about what we're looking at).

The aortic valve issue and the dizziness (in this case referred to as dysautonomia) could also be symptoms of Ehlers-Danlos. Of course, he's not going to be tested for that unless the genetic tests we're waiting on come back as normal. And we won't have those results for weeks.

Also made another psychiatrist appointment... It's been a while since Ricky's been to see his psychiatrist.

Things just never get dull around here, eh?

Another diagnosis? And an update.

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!

We have a plan.

I called the CF nurse this morning after Ricky woke me up at 5:30am unable to breathe well, and having an upset stomach. I left a message that we needed to come in.

He had a neuro visit scheduled for 1 and the CF nurse called back later and said to come in at 11. So Ricky and I dropped Misty off at day care and headed up to Stanford.

Ricky has lost a little weight and is down to 97 pounds but he's up to 62 inches (5'2"). His oxygen saturation was 98 -- excellent -- and his spirometry was right where it has been for quite some time. His lungs sounded great as well. The doctor asked a lot of questions and, combined with the physical examination, concluded that whatever is going on with him, which is definitely significant, is not pulmonary.

So we had lunch at Taco Bell and then came back for his neurology appointment. First we saw a resident, because Ricky's doctor had been called to the OR. He came later. Both of them did thorough neuro exams on Ricky and apparently he was okay there. The ultimate decision was to back off on the Topamax so that he'd be on his old dose, and see if all of this stuff stops.

Our last appointment of the day was his bone density scan. That was fine... He was very good for it. :)

So I backed off on the Topamax tonight and by tomorrow he'll be on his old dose (25mg two times per day). Hopefully he starts to feel better.

Ricky's sick

For about a week now, Ricky's dizziness has been far more severe (along with periodic fever and chills). He's been sleeping a lot more (like 8+ hours in the middle of the day one day last week!). He's had headaches and stomach aches, and just hasn't been feeling well.

The CF doc prescribed Septra, which I couldn't understand. This was clearly a virus as far as I could tell and the CF nurse could tell.

Well, then, today, Ricky slept all day yet again (he has had at least 4 hours of nap(s) every day since this started) and this evening, even after his breathing treatments, his chest was tight. He said that last night he'd had trouble getting to sleep because his breathing was bad.

Tomorrow he has an appointment with the neurologist and an appointment for his yearly bone density scan. Those are both up at Stanford and I'm going to try to get us there early so we can maybe see someone in the CF clinic. I do not like how quickly this has gone downhill!

status quo for now

Wow! It has been a long time since I posted... Over two months!

Just wanted you all to know that Ricky is doing very well. We have had a couple of emergency room visits for CF issues that turned out to be not as serious as we feared, thank goodness.

He still hasn't entered puberty, so we're going to the endocrinologist soon, and we will also be visiting the geneticist again, in March. We are supposed to go there periodically for a chat and further blood tests, since it's suspected he's got something else going on (yeah, like we need something else!) and there are new genetic tests available all the time. Also have a pulmonology appointment coming up, the same day as the endo visit, and he'll also have full PFTs that day. Going to be a very exhausting day.

Unfortunately, Ricky's dizzy spells are back in nearly full force. The neurology nurse gave me the go-ahead to up his dose of Topamax to see if that helps, so I've started the titration process. I hate that these spells are back because they totally incapacitate him and make him miserable.

Things are going well in school. He continues to be a teacher's aide in the office and they all love him there. We are gearing up for the transition to high school (!!!) this fall. I'm not sure yet where he's going to be going, but one of the options is my alma mater! Now that would be weird! Anyhow, we're having the transition IEP meeting in March and I should get more details then.

Keep your fingers crossed that Ricky stays healthy through the winter!