In November 2013 Nicole's old doctor at Shands Transplant Center at the University of Florida received a referral from the local transplant center requesting Nicole have a second opinion. Nicole's former doctor at UF called and recommended that she see a doctor in the Congenital Heart Clinic. I sat on this information for about a month because of some issues going on at the local transplant center. In December I called and got an appointment for Feb. 21st. In January of this year we received a letter from the local center stating that they would not be listing Nicole. So our appointment to UF was now more of a first appointment and a fresh start rather than a consult/second opinion. It is funny how you see things in hind sight. I couldn't understand back in 2010-2011 when she was being seen there why the doctor was so interested in Nicole's artwork and her personal life. Now I do.
We left on the 21st at 6:00 a.m. for Nicole's 9:00 a.m. echo and EKG followed by a 10:00 a.m. appointment with the doctor. Nicole laid in the back seat and tried to get some more sleep. We always stop at a certain rest area which we did on the way up as usually by this time the coffee as gone through me and I need a restroom.
We arrived in Gainseville about 8:20 a.m. or so but I was glad for the wiggle room as Rick missed the turn and we had to backtrack. Fortunately, we were able to find a handicap spot in the parking lot and not have to park in the garage.
To the second floor we went. I checked Nicole in. We were called for vitals (height, weight, bp, pulse ox). Her blood pressure was a little higher than it normally is and her pulse ox was a little lower than normal but her O2 was set on 3 when she's normally on 5 as we had to conserve the battery as much as possible. Also went over Nicole's list of medication which is long and they didn't have most of them in there system because we left there 2 1/2 years ago.
We were taken to a room. Rick went down to the car to get Nicole some more water and while he was gone the doctor came into the room. He spend several minutes calculating Nicole's Remodulin dosage. Nicole has been on 200 ng since July 2013. He thinks she should be increased and can tolerate that. I'm sure Nicole wasn't happy to hear this as increases are a bitch with all the side effects that accompany that. Also, talked about her other PH medications.
The doctor talked about survival rates for transplant and said that while it is a wonderful option for some patients they don't want to pursue this avenue if the quality of life of the patient is good and the medical therapies are keeping the disease process at bay. He did say that patients with Eisenmenger's can have a very good quality of life without transplant into their 40's and 50's. With both transplant centers they recommend pushing out transplant as long as possible because for one transplant has its own problems and issues and they are coming out with new medical advancements every day.
He said that Nicole would get an echo and then he would see her again to let her know what he thought about it and what he thought in regards to heart repair vs. heart transplant. He did say that Nicole's pressure's in the lung (normal is 25 .... Nicole's are in the mid/high 80's) wouldn't go down but with the Remodulin and other medical therapies her PVR (pulmonary vascular resistance) could drop. After he read Nicole's echo he came back. He was very pleased with her echo and her heart function and how the ventricle's were working. He said that she has an ASD (atrium septal defect) but other doctor's have told us it was a PFO (patent formanen ovale) which is a flap instead of a hole. She has a very large VSD which is the reason she has Eisenmenger's and PH and then two other smaller holes in the muscular portion of the VSD. I guess doctors learn in medical school how to write so patients can't read the writing. I can't read his writing well enough to interpret what he wrote on the picture of the heart drawing he gave us. It has always been a question as to whether she has two or three small holes in the ventricle in addition to the large hole.
He said that a repair was doable but without consulting the lung transplant doctor he didn't know if it was the best choice even if it was doable. Sometimes it is easier to do in block (double lung with heart). He also said it was his opinion that she isn't ready for transplant yet.
His recommendations were:
Nicole's Revatio be increased from 20 mg three times a day to 40 mg three times a day and then park at this dose for 3-6 months and then increase it again to 80 mg three times a day.
That she starts increases of her Remodulin because she has room to go up on this.
That she come back to the congenital heart center in six months
That she has another heart catheterization within the next three months.
The doctor did say more than once that her PH specialist has done a great job in controlling her PH. I totally agree. The doctor also said that they never put one of their patients on IV Remodulin because of the serious risk involved with a heart patient. He was glad that she was on subcutaneous.
If her PH specialist agrees to this we don't have a problem complying with this. I am uncomfortable with Nicole having a heart cath up at Shands though as the last two of her heart caths have been done by the same CHD pediatric cardiologist and the same anesthesiologist at Arnold Palmer but we will discuss it with her PH specialist on March 6th when Nicole see him for her three month check up.
The nurse that Nicole had with her old doctor at UF came over and saw us as well as played the cardiologist's nurse. She went over Nicole's medication list and made some suggestions and then consulted the doctor. The first thing that she wanted to know was why Nicole was taking aspirin. I said her former cardiologist had put her on it in hopes of keeping her off of Coumadin (anti-coagulant thins the blood). She also takes hydrocodone/acetaminophen and prescription ibuprofen for her site pain. The nurse didn't like this because the aspirin and the ibuprofen will lower the platelet count and hers is already low. She said there was other alternatives to controlling her pain. The doctor didn't want to switch up her medicines on the first visit nor did he want to step on another doctor's toes. He did say that while he wasn't thrilled with Nicole being on aspirin because of her platelet count unless it got down to 60 K he would be okay with her taking it. The nurse also said that Nicole needs to take her Omperazole (PPO) one hour before she takes her other meds to coat her stomach. Nicole has a lot of stomach issues and most of them are from the medications that she takes and what it does to the lining of her stomach. The nurse was surprised she doesn't have an ulcer. So we will try this. The nurse also said (I have never been told this before) that the ibuprofen will diminish the effectiveness of the Remodulin and if she stopped taking it her Remodulin would work even better.
Nicole has another appointment with the lung transplant program on March 3rd. This will be a very long day. Friday was a cake walk compared to this. We will leave our home at 6:30 a.m. Nicole has a chest x-ray scheduled for 9:30 a.m.; followed by a pulmonary function test (PFT) at 10:30 a.m. (she hasn't had one of these tests since she was 12 I think); followed by a 6MW test at 12:30 p.m. followed by her appointment with the doctor in clinic at 1:00 p.m. and I believe they are going to order bloodwork which will be done down in the lab after the appointment. I don't expect to leave Gainesville until at the earliest 4:00 p.m. We will eat dinner before we leave and travel home. As soon as we get home we will have to change Nicole's pump. It will be an extremely long, stressful and emotional day.
Overall, I was pleased with the appointment. I really liked the doctor and it was good to see the nurse and she was very happy to see Nicole. She loves Nicole's artwork and told the doctor what a wonderful artist she was.
I hope that her next appointment goes just as well. The doctor did say that he would talk to the lung department so the visits could be coordinated so she could be seen at both clinics at the same time so we didn't have to make two trips.
When we left the medical plaza building it was thundering out with torrential rain. We drove through town and on I-75 through this weather for about 15 - 20 minutes before we made it through it. It was quite nerve-racking.
I try to find something that I can improve on with every appointment / experience and I need to re-do my medication list to reflect what date she started her medications (the nurse asked me when she started taking aspirin and I couldn't remember) and also I have a sheet with all of Nicole's Remodulin increases listed and I need to make headings so the doctor knows what each column is as there is the date, the pump rate, the strength of medication and how much medicine goes into the syringe. He was unclear what each column was. I think there was something else also but I can't remember what it was that I wanted to tweek.