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Update

I haven’t written anything in a while. November is PH awareness month so I have been focusing on educating people through facebook about the disease so my research and such has been taking up my time.

Last week we went and saw the specialist. I had been banging my head against the wall trying to get his nurse to listen to what I am saying. She makes me feel like I don’t know anything and it isn’t valuable information. Just because I’m not a nurse doesn’t mean that I don’t know anything. I wanted Nicole to start her medicine increases again as she had been on the same dose since Sept. 19th.

The other issue that I was trying to resolve was transplant evaluation. The choices for the evaluation were between two hospitals one is at the University of Florida (UF) which is a 2 1/2 hour drive each way and Florida Hospital (FH) which is roughly a 20 minute drive each way. Florida Hospital has just started doing lungs this year but has been doing kidney, livers, pancreas for many years. The specialist nurse was very adamant that the doctor wanted her to go to UF . I have done some research about transplant at UF. The patient and the caregiver is required to live in the area for six to eight weeks after discharge from the hospital, there is pulmonary rehab five days a week as well as weekly clinic visits which would include bloodwork and routine testing. The same will probably be required at FH also.

I had been waiting for at least two weeks for the nurse to set up a phone conference with the specialist and cardiologist and myself. The day of the appointment this was accomplished. The appointment went very well. The doctor was quite happy with Nicole and how she was doing. He gave me a high compliment about this chart (idea stolen from G-J) that I have to keep track of Nicole’s symptoms. This chart was able to let me see that Nicole’s nausea directly correlates to her taking Tylenol Codeine. This issue was discussed and while the doctor could of switched her pain meds we decided to try alternating the pain med with Advil.

We also had the conference. The doctor and I went into a small little office while Nicole stayed in the exam room as she had to do her six minute walk test to see if she had improvement over last time which was in July I believe.

I learned a lot during the conference. I learned that as opposed as the nurse was to Nicole being evaluated at FH, the doctor wasn’t as opposed and was very open minded about it and actually said that the amount of collaboration that she would receive at FH outweighed what she would receive at UF and that made up for lack of experience with the program as the doctor’s have experience with transplants. I believe at the last cardiologist appointment the doctor said that there are only about 20 lung transplant annually throughout the country. I could be wrong about this number.

One of the issues with Nicole is whether she will need a double lung transplant and a heart transplant or can the heart be repaired and just do lungs. It is the belief that they can do the lung transplant and fix the heart. Nicole has three VSD’s (ventricular septal defects) which are holes in the ventricle which is the bottom part of the heart. She also has one large VSD which is between the right and left ventricle which has allowed for her blue nails and lips (cynosis) because the blue blood which is oxygen poor is going through the hole into the lungs where it isn’t suppose to go. She also has a large ASD (arterial septal defect) which is in the artrial which is the top part of the heart. The plan is to go through a catheter up to the three small VSD’s and correct these. The large VSD should keep enough pressure off her lungs while they take out the catheter and prepare her for open heart surgery to clamp the large VSD. After they have done this she will be put on a heart/lung bypass machine until they get the new lungs in and then preferably on the way out (closing up) they will close the ASD. None of these holes can remain open or she will get PH again with new lungs.

I am a little angry about the fact that the cardiologist has never come out and told us that she has an ASD. Even though in the cath report it says they had to pass through it but it is referred to in some other term. The question is will her heart take this repair. They also have to be absolutely certain that they can do the repair which requires a better anatomy of Nicole’s heart.

A cardiac MRI is the perfect choice but like everything else nothing is easy when it comes to Nicole. Nicole’s infusion pump cannot be in the room during this test which raises the question how to get it out of the room while still attached to her. I don’t think there’s a way to make the tubing longer. The doctor was going to get this set up which I know from experience will take much longer than it should because his office can be slow. It was determined at this conference that both doctors were on the same page and I was to go forward with making an appointment for the evaluation at FH. The cardiologist has had a conference with the surgeon about Nicole’s specific case and he is willing to see her.

Another concern of the cardiologist is that while Nicole is stable right now and the medication is starting to show some benefit for Nicole all it would take is for Nicole to get a virus which could have deadly consequences for her. Anything other than the common cold she would be hospitalized for. Another concern is that she would get a diarrhea virus and not be able to keep down fluids and become dehydrated just as deadly. In this case she would be hospitalized and given IV fluids.

This terrifies me so much…. and I really don’t know which terrifies me more … the chance she gets a virus or the transplant itself. I can talk myself into the fact that Nicole is doing just fine on the medication and we can continue on this path but at some point her heart issues will have to be addressed. Her heart can’t be repaired without new lungs.

I try so hard not to have fears and to dwell on them but my worst fear even before Nicole’s diagnosis was losing her. The statistics aren’t the best for a lung transplant and especially one as complicated as Nicole’s.

I know this probably sounds so selfish but if I had to choose between losing Nicole and Nicole losing me through death I would choose losing Nicole as I don’t want her to go through the pain when I can through instead. That doesn’t do anything to comfort me. I can’t understand (and I know I’m not suppose to) why Nicole has to suffer the way she does and go through what she does every day. She didn’t do anything to be born with a bad heart. I see all the time on facebook “PH sucks” and “I hate PH” while I agree it does suck, however, I can’t hate the disease maybe because she just doesn’t have it for no reason… it is caused by her heart defects that she was born with and through the negligence of doctors and the lack of listening and follow through it went undetected for so long.

I finally called yesterday to set up the evaluation, which doesn’t mean she will qualify for transplant it is just evaluating her to see if she meets the criteria. The appointment is Tuesday the 20th. I wanted to go to the rehab’s support group meeting that day and it seems for the last three months something has come up on this day that has kept me for really taking part in the group. If I didn’t take this appointment than it would have had to wait until December. Fortunately, the transplant center is right across from rehab.

About Jane

Avatar of Jane
My name is Jane and I care for my 19 year old daughter, Nicole, who has several congenital heart defects, Eisenmenger's syndrome and pulmonary hypertension which is a rare, life-threatening, incurable and progressive lung disease that causes blood pressure within the pulmonary arteries to become higher than normal. She also has several other health issues that are not as severe. I have two blogs one is "A Day in the Life" which chronicles Nicole's diagnosis and our everyday life. My other blog is "Let's Get Organized" which chronicles my struggle to get the clutter out of my house and get organized.

5 comments

  1. Avatar of Denise

    Hi Jane–It’s just so much. I’m grateful you given us an update.

    How does Nicole feel about the evaluation and moving forward with the transplant?

    I also wonder if they’ve explained what will happen on Tuesday?

  2. Avatar of Jane

    Hi Denise:

    I love the picture of us on the home page with my blog and the PH awareness picture above :) Nicole is ready for the transplant as she said she will be “normal” . I keep forgetting to have the conversation with her about what her definition of “normal” is. I will have to do a blog on the transplant process before and after.

    I did ask when I was speaking with Lisa who handles all new patients and she said it would take about one hour. She sent me the application via email which I have to have filled out prior to the appointment.. They would have her medical records from the cardiologist and specialist. I need to bring her list of medications (which I always have just have to update because one med has been added and her Remodulin has been increased). They will probably do a EKG as well as go over her history and a plan of care. She said there wouldn’t be any evasive testing done at this appointment.

    I guess we will see.

    Hugs;o)
    Jane ~ mom to Nicole, 18 yo, VSD/ASD, PAH, Eisenmengers (dx 1/22/10) BHJS (dx 2/4/11)
    “You’re braver than you believe, and stronger than you seem, and smarter than you think.”

  3. Avatar of ejourneys

    Jane, I will be thinking of you and Nicole on Tuesday. You are doing extraordinary work with all this, especially given the bureaucratic and personality hurdles like the specialist’s nurse! And thank you for all your PH posts on Facebook.

    Thank goodness the doctors are coordinated and communicating. I don’t see you as selfish or dwelling overly much on your fears at all. You are facing them and fighting your way through them. You are actively looking for the best route through a very, very hard place. Courage does not mean absence of fear — it means facing and moving through fear. I think you have tremendous courage. *Hugs*

  4. Avatar of Jo

    Jane,

    Indeed will be thinking of you and Nicole this Tues. Agree with ejourneys, you’re not selfish, you love your daughter and her suffering hurts you. Still your attitude is remarkable… as is your strength. You may not feel it, but its obvious to the rest of us, and especially to any Dr who crosses you :-)

  5. Avatar of

    Hi Jane,
    Thinking and praying for you both today. I’m glad for the questions that you have thought to ask. It’s important you feel as though you can ask them, you know Nicole and her needs more than anyone. I hope the explanations that come today, bring comfort to you both.

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