Sunday, February 23, 2014

Visit to Gainesville....

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.

Monday, February 17, 2014


I'm not sure that this is the proper title for this blog but this is all that is coming to mind.  It has been a difficult week with fighting to get a medication that Nicole needs which I will write about in another blog as soon as I have it all straightened out.

This blog today is about my frustration over people and how they will say and assume things about Nicole and her illness but they do not live her life nor are they qualified to even make assumptions or give there opinion without knowing what Nicole goes through.

I am tired of people saying that Nicole's illness/health issues are over emphasized and that it is no harder for her to travel, go on a day trip to Disney etc. than it is for anybody else.  This makes my blood boil.  I would say to anybody with this attitude to come spend 24/7 for  a week or two at our house and observe what Nicole goes through before you make assumptions that are not correct.

I have been very focal on facebook about the journey that Nicole and our family goes through with Nicole's illness.  I will say that even though you can look at Nicole without her oxygen on and she looks perfectly healthy SHE IS NOT..... she has a rare, life-threatening, progressive, incurable disease.

I agree that Nicole should be treated as normal as the next person and she shouldn't be looked at any different because of her health issues/disability than anybody else and as much as I hope and pray that she was normal and healthy she is not and at this point in time it is safe to say she never will be.  Every day Nicole fights to have a decent quality of life.  Nicole can't just be spontaneous and go out somewhere with no thought or planning this just can't happen.

Travel is a real big issue for Nicole.  On Friday of this week we will travel to Gainesville which is a 2 1/2 hour drive each way from our home so Nicole can have a consult at the cardiac clinic at the transplant center at the University of Florida.  I have already starting planning for this trip.  I have a master checklist of all the things that need to be done or make sure I have for this trip.

The biggest obstacle that Nicole faces with any type of travel is oxygen.  Her portable concentrator will only last about 3 1/2 hours because the battery needs to be recharged.  We have a pack that plugs into the wall and this charges her battery.  There is also a charger for the car but the problem with this is it doesn't fit in every lighter outlet in every car so I have to make sure it does in  ours.  We will have to find a spot in the waiting room close to an outlet once we get to Gainesville and then also use an outlet in the exam room.  The ultimate would be that they would put her on a tank so she can get continuous as opposed to the pulse (by the breath) of her concentrator.   She is very fortunate that she has two concentrators so we could and probably will take both with us.

We also have to take in account her pump changes which are daily.  We have about 27 hours between pump changes before the medicine runs out.  This means that everyday about the same time I have this event scheduled in my calendar in red because this is like one of the most important things that Nicole needs to do on a daily basis.  We will be gone from 6:00 a.m. and probably not returning home before at  least 4:00 p.m.  Now I have to decide how we are going to make sure that Nicole doesn't run out of medication because changing a pump in the car or restroom isn't an ideal situation and should be avoided.  Does the schedule need to be changed leading up to this day etc?  More than likely yes.... just to be safe rather than sorry.   All the supplies for a pump change and/or a site change needs to be taken with us along with the medication and the pump.

We will also have to take all her other daily medication so basically it will be her pill box for the day.  She will need to take a pillow and blanket so she may rest during the drive.  We also have to load in the wheelchair because the walking is too much for her so Rick and I will take turns pushing her.  She will have her concentrator in the chair with her. We will also have a big jug of water in the car so she stays hydrated.  She will also have things to do with her whatever this may be.  I also like to make sure that she takes a change of clothes (just in case of vomiting or diarrhea).  Also needs some snacks to take along just in case.  Nicole will get nauseated if she is hungry.

It is much more difficult if the trip is longer than a day trip and involves flying.  There are so many rules and restrictions when it comes to oxygen and those who travel with it must know exactly what the airline requires.  When we went to Hawaii in 2011 the airlines were still supplying O2 on the flights.  I believe now they do not anymore.  You need to know how long the flight is because most of the time the concentrator isn't go to last long enough for a flight.  It is something that really needs to be planned for down to the smallest detail.   In 2011 you also needed a script for the O2 from the doctor.  Nicole can not walk through the x-ray machines with her pump nor could her inhaler (Tyvaso) go through the x-ray machine when we went to Hawaii.  Nicole also needs letters from the pharmacy stating all of this as well.

I would challenge anybody who feels that Nicole or any other person who has a chronic "invisible" illness to get the facts before they judge somebody and say that they are faking it or over stating it.  I would challenge everybody to take the straw test.  Take a regular drinking straw and put it in your mouth and plug your nose.  Then set a timer for 1 minute and breath through that straw. This is what it is like for Nicole and other PH patients to breath on a daily,regular basis.  Then take a coffee stirrer and set the timer for one minute and breath through that and this is what it feels like for Nicole to breath after walking down the hall, up a flight of stairs etc.  Another option would be to climb a mountain and see how the air thins and affects the breathing the higher up you go.

Then next time somebody wants to judge Nicole I say come stay with us for a week or two and spend 24/7 with her and then if  you still feel her illness is over emphasized than that is fine.  Just because somebody looks fine to the eye you never know what that person's life is like.

Sunday, February 2, 2014

Week in Review - 1/21/14

Here's my second week in review... so far so good.

Nicole started her second semester of college on Monday.  She is taking Analysis of Form and Composition for the Artist (I believe this is the name or it could be of the artist).  I think she's had a little bit of a problem getting back into it especially if it is an assignment she doesn't want to do.  She has to do an artist biography of herself which she's not looking forward to.  Her assignments are due on Sundays at 12:00 midnight pacific time.  I wish she wouldn't wait until the last minute but it is her decision and she has to live with it.

Nicole saw her counselor  on Tuesday and all three of us talked about the issues with the transplant center and how Nicole was feeling about that.   She was still in some pain so she didn't go to rehab and we went back home and she went back to sleep.

On Wednesday, Nicole received a certified letter from the transplant she's been seen at.  Nicole had sent an email to the pre-transplant coordinator regarding a letter she received that was dated Dec. 19th but was not postmarked until Jan. 6th and we got it on Jan 7th.  The letter basically stated that the center did not have to give a reason why they would not list a patient according to OPTN which I thing is wrong, wrong, wrong.    It was also stated that the delay of the letter was due to the holidays. I'm sorry but that is a poor excuse considering it was a 19 day gap between the date of the letter and when we received it.  The date of the certified letter was January 24th but it was postmarked until January 28th with us receiving it on the 29th.   To say that I am angry about the letter is an understatement.    I am not going to say anything more about it and we move on to another center and hope that things go better.  Nicole has two appointments schedule for Feb. 21st and March 3rd.   This same day we received a packet from the new center with paperwork to fill out.

Thursday Nicole had to get her monthly blood work done.  We are still waiting on the results of that especially for her platelet count, potassium and magnesium levels.  We waited in the lab for 40 minutes before she was called and there was only two people in front of her.  We were able to walk down the hall to radiology and request a CD of her last chest x-ray for the transplant center and they had it ready before the lab work was complete. Nicole then went  back to rehab for the first time since before her site change on January 21st.  She didn't do well and her monitor kept beeping because of low oxygen saturation levels and how know how much she gets frustrated by this because she wants to be able to do more.  I hope that it was just because of the cold weather.  It is suppose to be warmer this week so I am hoping this will help.

Overall, it was a good week other than the stress of  having to start over with the transplant process.  It breaks my heart that Nicole has to go through this.  It also infuriates me that a medical professional will give an opinion about a patient and base it on the few minutes (20 - 30 minutes) they see this person every three months especially when they have no reasoning for it.