Happy New Year!!!!!!!! I hope 2015 is the best year yet for you.
Nicole saw the hematologist on Dec. 30th. Her last visit prior to that had been Oct. 31st. She had a lot of blood taken at this appointment in Oct. All of this lab work came back normal. I had a feeling going into this appointment on Dec. 30th that there is really no reason to keep seeing him. There is no clear cut reason or answer why she has a chronically low platelet count. We know if she ever needs surgery she will need a platelet transfusion. The doctor did say that her previous diagnosis of idiopathic thromocytopenic purpura (hoping for correct terminology and spelling) was wrong and her immune system isn't attacking her platelets.
The only issue was her IgG was low but not low enough for infusions. He was going to test it again which he did along with some other immunology tests but no follow up appointment was made. I was not surprised. They were suppose to call with the results.
The hematologist believes that her Eisenmenger Syndrome is the cause of her low platelet count. I have never heard this before which I would think a congenital heart defect cardiologist would know and neither one of her CHD doctor's have every mentioned this. The first hematologist Nicole saw said it was her medication and while I know it does contribute it wasn't the reason as her platelet count was low upon diagnosis.
Nicole was having pump issues the week of Christmas and the week of New Year's. The majority of the time, at least for Nicole, these occur while she's sleeping. The first time was Christmas Eve day at 9:00 a.m. but she didn't tell me she obviously unkinked the tubing and the pump was working again. It alarmed again at 1:00 p.m. and wouldn't stop alarming so we had to call Accredo (the specialty pharmacy) and speak with a nurse. The nurse had her unattach the tubing at the site and clean off the tip with alcohol as sometimes the Remodulin builds up and I believe we were told it gets sticky and will gunk up. This seemed to work and got the pump to stop alarming and go back to running.
On Monday, Dec. 29th Nicole's pump malfunctioned with blockage detected twice within a five minute period at 4:20 a.m. and she was able to clear it by running her fingers along the tubing. At 7:35 a.m. it started alarming again and she couldn't get it to stop so we had to call a nurse at Accredo. He had her check for kinks and then had her push some buttons on the screen of the pump and it went back to running. After this Nicole believed she found the kink so we went ahead and changed the tubing. At 10:45 a.m. the pump goes off again for blockage detected and wouldn't stop so called a nurse again. She was able to get the pump to stop by having Nicole check the tubing and hit some buttons but she did say if the pump went off again then she would need to change the pump and if it still alarmed she would have to change her site.
On the 30th Nicole said her pump went off again at 4:00 a.m. and she unattached and reattached the tubing at the site and it stopped.
On the 31st when we went to change the pump the pump that we were switching too wouldn't display properly and was going into setup which is something the patient shouldn't have to do. Had to call a nurse again and she had to walk Nicole through the set up process which she was relunctant to do. I believe she was the same nurse we had spoke with for one of the times we had called with this batch of pump problems. It didn't work the first time because the nurse told Nicole to program in 0.000 and it should have been her pump rate that went there and this is why it didn't work.
Since this time she has had no problems with the pump. However, Nicole started a new vial of Remodulin on Monday and it leaked and I thought that it was because it was the first draw or the syringe was inserted crooked but yesterday it leaked also because it was on my hands as well as Nicole's. I will need to call a nurse today to see what we should do.
Yesterday she went back to pulmonary rehab for the first time since Dec. 18th. She did complete her full routine but did have some chest pain. On the way home we stopped at the hospital where she gets her blood work to pick up the lab results from Oct. 31st (the hematologist) ; 12/4 (her normal monthly blood work) and 12/30 (the hematologist). I knew what her results from 10/31 were all normal. I also knew what her blood work results were for 12/4 and was a little ticked off that her potassium was low and nobody from the doctor's office told me so her potassium could be adjusted. No real surprises. I was suppose to get a call from the hematologist office with the lab results from 10/31 and surprise no call. I did look at and compare the IgG to her last test in Sept. and it is still low but not as low as in Sept. Her iron was tested as I believe the doctor thought she was getting too much iron (which in my opinion was doubtful). Her ferritin level was suppose to be checked but I only saw iron/plasma and I don't think they are the same thing. Her iron saturation percent was low. I'm not sure what this means. Her platelet count had taken a nose dive between 12/4 and 12/30. Hopefully it will go back up this month. Her reticulocytes which is related to red blood cells were all high just like in Sept. which I was told was no big deal and actually were good. Her IgG was still low but went up a bit since Sept. The IgG subclasses were tested and of the four only subclass 1 was low... not sure what this means either but it looks like it could be related to DTP levels which were tested in Oct. and they were normal.
Nicole sees her PH doctor tomorrow so I need to sit down today after calling Accredo, and rescheduling a doctor's appointment she has next week and write down the list of questions we have for him.
I am also waiting on her last echo which she had done in August 2014 at the University of Florida to be faxed to me as none of the doctors ever send copies of any tests to each other. It seems that I am always the one to have to get these and distribute them accordingly.