I decided that I wanted a second opinion about Nicole's lung disease pulmonary arterial hypertension as I have had reservations about the care she's receiving from her current ph specialist.
I meant one of the local PH specialist at the local support group meeting and he agreed to see Nicole. His office doesn't take Medicaid but he said he would run it through for her. I talked to children's medical services (CMS) which is the Medicaid plan Nicole has. I was told if her cardiologist sent in some letters stating why it was necessary for Nicole to see him they would take it under advisement and consider covering an out-of-network doctor. I had another nurse after her appointment tell me this wasn't true and they don't pay for any out-of-network doctor's.
Nicole has severe PH caused by a large VSD that wasn't diagnosed until 2 years ago when she was also diagnosed with PH. He said he agreed with the diagnosis. He only had the records from the cardiologist and not the PH specialist. He said that from what he sees at the time of diagnosis she was a borderline Stage 4 (this is the worst stage) and he thinks that she has probably dropped back down to a little above a Stage 3 since her drug therapy has started. He said as well as her other drs. have said that the pressures in her pulmonary arteries will not come down until her heart defect is corrected and her defect can't be corrected because of the PH. He said that the PH was the much more serious of the two. He said he tends to be a little more aggressive and he would have her on Remodulin IV pump with the hopes of her taking part in a study with an implantable pump that is inside the abdomen area. She would be a good candidate for this because of her heart defect. She would need to be on a stable dose (basically this means the dose that they want her on long term as they have to start at a low level and work up) for four months before she could participate. The only issue I have with this is that there are some more complications and it isn't as easy as Tyvaso. It also has to be implanted in the hospital whether it is the IV through a cathedar or the implantable. He did mention something about air getting in the line can be bad for a heart defect. He also said because of her heart defect her PH has progressed very slowly and more than likely she has had it most of her life. He was also concerned that she hasn't had a catherization (this is the most accurate way of obtaining accurate pulmonary pressures but also the most invasive) other than her initial one diagnosing her so there's no information to compare after she's been on therapy. He also said that she would need a lung transplant at some point down the road. I probably would have asked more prognosis related things but Nicole was there and I don't want her to be upset.
She also did a 6MW ( a test that is used to measure the exercise ability it is where you walk for 6 minutes and your distance along with you oxygen saturation levels are measured). She was on 6 LPM and still her O2 stats fell at least twice below 78% (normal is 98% which Nicole rarely hits 90% and this is called cynosis which is blueness) and the nurse made her stop until it went back up. So needless to say she didn't do well. They were also surprised when I said when she had her last one in Oct. it was without O2. He said (contrary to her current PH specialist) that her O2 stats should never be below 85% and she needs to be on whatever O2 that keeps her there. The current PH doctor had told her that she didn't need to be on 8 LPM (liters per minute) at rehab and it was alright if the stats dropped.
Since this appointment she has gone to wearing a mask at rehab which is much better at hi-flow oxygen (anything above 6 LPM) than the cannula's. She is using 12 LPM and it is still hard to keep her stats above 85. Her time and intensity has to be backed down.
I'm not sure what to make of the second opinion. I really like the doctor and he is excellent with Nicole and I feel like even though she is very sick there is still hope I don't feel that most of the time with the other doctor. I don't know if she will be able to switch from Tyvaso to Remodulin because her current PH doctor has almost no chance of going along with this.
I am trying to figure out how to proceed. There are not a lot of ph specialist to begin with and then a lot of adult doctors don't take Medicaid. I know of another group of doctor's here locally (they are pulmonary, not cardiac) but they won't see her until she's 18. The cardiologist would like Nicole to have a transfusion due to her hemoglobin issues and also put her on a hormone that the kidney produces (I can't say it and don't know how to spell it off the top of my head as it is very long) that regulates the amount of red blood cells in the blood in hopes of raising her hemoglobin and well as her oxygen staturation levels. I don't know when or if this will happen.
The ph specialist she saw last week and her cardiologist have been playing telephone tag with each other so I am hoping that they will talk soon so I know what we are looking at.
I feel better with a second opinion but it doesn't really change anything about her diagnosis. The doctor asked Nicole what are the top three wishes she had in regards to getting rid of... these were fatigue, shortness of breath and joint pain (not sure how much of this is related to drug side effects and how much is her BHJS/EDS).
I have to believe that God will provide the right doctor and treatment that Nicole needs. I hope that he will give me the wisdom to know what is best for her with minimal risks and side effects. I have tried to get quiet about this but it is hard when there doesn't seem to be any options but what we already have at this point.