Caitlin-update

Loooonnnnngggg day at the hospital today! From 7:50 until 2:45! First we started off with the rheumatologist. The appointment went well, but the dr does thing Caitlin has something "brewing" that hasn't developed yet so she has diagnosed her with undifferentiated connective tissue disease. She wants Caitlin to start Plaquenil asap, but has to get the green light from the hematologist because this drug can lower platelets. They also discovered a lump in her wrist, that is quite large so they want her to see a surgeon about getting it removed. They think it is just a cyst. We've known about it, but we always thought it was a bone sticking out and it just kept getting a little larger. We will tackle that after we get back from teh cruise.

While we were at the rheumatologist, we were sent down for labs and then the fire alarms started flashing and they kept saying "code red" throughout the clinic. At first, the nurse who draws blood looked like she was going to ignore it, but then several people came in saying we needed to leave. They evacuated the entire building and we stood outside for about 25 minutes. Found out later that there was a fire somewhere in the building but they were able to put it out quickly and let people back in. (I think it was in radiology.) We got her blood drawn (6 vials instead of 12 like last time). Thankfully they weren't in the middle of her blood draw when we had to evacuate!

After that, we went to hematology and waited a long time for anyone to come out. Finally we were told that her platelet count was at 147 and we still had to get the drug. I was a little resistant and asked if we could wait until after we got back from the cruise, and the study coordinator said we could, but then we'd get kicked off the trial. Then I asked about the reduced dose and they said they still had to follow protocol. (The hematologist still hadn't heard back from the physician at Amgen.) Grrr...so Caitlin still got 8 ml/kg and we were sent home with three vials. We have to go back on Friday for a platelet count to see if it is low enough for her to receive the injection while we are on the cruise. Then we go back the following week too. We were really wanting a break from having to go so often, but it doesn't look like it is going to happen.

Another bit of important information...the rheumatologist is still very adament that Caitlin not get a splenectomy. Said that if she were to develop Lupus, her body would have an even harder time fighting infection if her spleen were out than someone who just has only ITP. We just need Caitlin's body to go into remission on it's own, I guess!

Thanks for the update. I like any info you provide because our daughters are very similar. Nice platelet count - I hope that Caitlin is feeling well. And how exciting to be going on a cruise! What tests did Caitlin have that made the Rheumatologist think it was heading towards Lupus? I still don't know what to think about Emmy because she had a virus prior to ITP she had vaccinations and she tested positive for h-pylori twice Right now she is on 20 mg of Dexametheson and she went up 100k in 4 days, but we'lll see how long it lasts.
After her soccer tournament in New Mexico the DR wants to add Rituxan to her treatment.
Shauna

Thanks for the update. I like any info you provide because our daughters are very similar. Nice platelet count - I hope that Caitlin is feeling well. And how exciting to be going on a cruise! What tests did Caitlin have that made the Rheumatologist think it was heading towards Lupus? I still don't know what to think about Emmy because she had a virus prior to ITP she had vaccinations and she tested positive for h-pylori twice Right now she is on 20 mg of Dexametheson and she went up 100k in 4 days, but we'lll see how long it lasts.
After her soccer tournament in New Mexico the DR wants to add Rituxan to her treatment.
Shauna

Pauline - I really like that Rheumatologist. She thinks outside the box and looks at the big picture. I'm not a doctor, but I agree with her 100%.

Did she mention the pre-Plaquenil eye exam? The baseline is important.

There is also a chance that Plaquenil will cause her platelet counts to go up. Since I've been on Plaquenil, I have been in ITP remission. Could be coincidence, but Plaquenil is known to hinder antibody production. Could go either way. If it causes a drop, you may never know it anyway because Caitlin bottoms out without N-Plate.

What a shame that she had to get the injection with counts of 147! There are times when protocol is ridiculous.

Shauna-Caitlin's initial ANA titer was really high at 1:1020 (almost 3 years ago)and she also has neutropenia (low white blood cell count). After not responding to some of the first line treatments, the hematologist ordered more tests to evaluate further. The results of these was what made her refer Caitlin to a rheumatologist and then she did all the tests, which revealed some things that meet the criteria for Lupus.

Sandi-we met another rheumatologist as well this morning (both came in) and I loved them both. They just seem so thorough with her. The reason they want her on the Plaquenil asap now is because she's been having such good counts. When we went before, her count was between 9-13 so they didn't even want to consider it with the idea that it could possibly lower her count. I hope Planquenil is what is keeping you in remission. That would be awesome if the same happens for Caitlin! I just wish this whole "protocol" thing with romiplostim would go away, but I do understand that they have to do what they have to do. If we go off the trial, we risk not being able to have the drug at all for her since it still isn't approved for pediatric patients.

I know, but considering that her counts got so high, you'd think the safety of the patient would outweigh the need for protocol. How frustrating!

I hope her counts improve with Plaquenil also. Just so you know, I've never had any side effects from it. Ha - one of the few meds I take that I can say THAT about.

Pauline,
Is there a reason she has to be on the study? Timothy is not on a study he just gets what he needs or doesn't need. Why can't Caitlin get the meds according to what she needs instead of by what the study says they want?

Donna...you are lucky that you were able to get Timothy on when the drug hasn't been approved for pediatric patients. Part of me wonders if it is because Tim is getting close to adulthood and he may be at an adult weight where even as a pediatric patient, they would follow the same dosing as they would an adult. But I could totally be wrong. Our doctor told us there is no guarantee that they'd be able to get her on the drug without the trial. (We looked into this when Phase 2 of the trial ended last December.) The other reason is...financial reasons. We have a TON of bills that we are paying for from all of her stuff from Nov. 2009 through now because of the ER visits, CT Scans, and everything else. We would like to at least keep her on the trial until we can pay off some of these medical bills then we will feel better about taking on the costs for Romiplostim. I know that is kind of selfish putting financial reasons before Caitlin's health, but while we have that option, I'd like to use it. However, if I see that Caitlin really suffers from these high doses (like she did two weeks ago), I will drop her from the study in a heartbeat and find a way to make this work. But then again, we run the risk of not being able to get the drug at all for her.

I am curious now how many kids are actually on this drug that are not on the trial. I was under the assumption that the kids on here who are currently on the drug are on some sort of trial. It would also be nice to have a real good idea of how much this would cost us if we were to get off the trial because I've heard things from a $50 copay per month to $1K-$3K per mcg.

In all honesty, I think we are just going to be in a cycle (get the injection, have a high count the next week, skip the dose for a week or two, back on with a reduced dose) for maybe another month or two and then she will stabilize at a lower dose. (I think around .03 or .04 mcg.) After that, I am sure she will slowly start working her way back up again. Who knows, I may just be kidding myself to make myself feel better about listening to what everyone else tells me to do (drs).

Pauline,
That's complicated. I hope you don't think I am EVER challenging your decisions. I know you are making the best decisions for Caitlin that you can make and are doing all the best you can for her

If you want I can ask why Tim isn't on a study just for your information. Also, for us it is easier because Timothy is on SSI and so he gets medicaid as well and does not have any out of pocket medical expenses. Its difficult having to be low enough income to qualify for SSI/Medicaid, but its really great that we don't have to pay any of the huge medical bills that now all three of my boys incur (Two are on SSI/medicaid, and on just medicaid. We would be bankrupt! I'm glad I don't have to add them to my private medical insurance because it would really cost to have to pay their copays and premiums!

I hope things get easier for you and Caitlin and I hope that they allow more room to breathe for her with the meds. You are doing a great job managing everything, and now with a new diagnosis on top of it, I hope things can get a little better with the ITP (remission please!).

Dont worry Donna...im not thinking that way at all! . I was just explaining why i keep her on the trial. I know how medical bills can bankrupt you but i am thankful for payment plans! Just hate having ao much to owe and its amazing how many people are in the same boat as we are.

Thanks for your compliment on how i do the best i can. I do try and even though i have a husband who loves and supports me, i do feel all the decision making relies on me and sometimes it can be hard. (Not complaining here though!)