What’s the Next Step?
Oct 11 2012 in Jane's Blog by Jane
Nicole started a new continuous drug that is supplied via an infusion pump that is delivered subcutaneously (through the skin) on July 25th. The doctor had high hopes that this would significantly improve Nicole’s symptoms and make her feel better.
Between July 25th and August 27th Nicole was slowly being weaned off of the inhaled medicine, Tyvaso, that she had been on since Dec. 2010. After she stopped the Tyvaso I saw a big difference in her symptoms and how she was feeling. It wasn’t an improvement. On Sept. 7th she went in to see the PH specialist and he saw how bad she looked and we discussed how she had been feeling. He immediately had her get an echo and then gave us a script for a chest x-ray to rule out any pneumonia or anything since she had been congested. The echo came back with no change since the last one which was three months prior to that. At this time, the doctor didn’t have an answer for why she wasn’t feeling well and her symptoms had increased. He said to increase her Remodulin every other day with pump changes until the vial we were using was gone and then when we started a higher strength vial we would go back to increases every three days.
We had been told by one of the nurses that if her blood pressure got below 85 (the top number) we were suppose to contact the PH doctor. On Sept. 16th after Nicole finished with rehab they took her blood pressure and it was 80/60 so I had to call the doctor’s office. I talked to one of the trauma nurses who told me to have Nicole drink some juice and a bottle of water and re-take her blood pressure in thirty minutes we were not to leave the hospital. If her blood pressure didn’t come up we would have to leave Florida Hospital and go to ORMC as this is where her doctor has privileges. Nicole was showing no adverse effects of the low blood pressure and said she felt no different than usual. Thankfully, after thirty minutes her blood pressure did go back up and we were able to come home. The nurse had asked me about side effects and her current rate of Remodulin. She said that she would give all the information to the doctor. Later that afternoon the doctor called me and said that he was disappointed that he hasn’t seen more progress with the Remodulin. Nicole was having some major side effects with the dose increases that wasn’t helping. At this time he stopped the increases and told me that he thought it was time to talk about the next step which is transplant. I remember that I started to tear up and get emotional. We have known since Nicole’s diagnosis that this would be the end result but I didn’t think it would be so soon. Since this conversation with the doctor Nicole has been on a steady dose of the Remodulin and she is doing well compared to August and the beginning to middle of September. I haven’t heard from the doctor since this conversation but I know Nicole’s cardiologist has spoken to him.
Nicole saw her cardiologist on Tuesday for her three month check-up. He had called around to some major hospitals around the country inquiring about transplants and what there procedures were etc. He also contacted two of the transplant hospitals in this area and told me where he thought would be the best place for us to go. He also agrees with the PH specialist that it is time to be evaluated. It should be sooner rather than wait for later. The transplant process is long.
I have mixed feelings about this. I don’t think that the Remodulin has been at a high enough dose nor used long enough to see if in fact it will significantly improve her symptoms. I have noticed with Nicole especially in July when she started taking the hormone to regulate her periods that her exercise tolerance at rehab suffered. I think that anytime she starts/stops a medication her body rebels and she doesn’t feel well for a period of time. She is just now starting to get back her exercise tolerance at rehab.
I remember on Tuesday sitting in the cardiologist listening to him talking and remembering that January day in 2010 in that same office being told that Nicole had this deadly disease and a heart defect that she had been born with. I have been able to since this time for the most part stick my head in the sand and forget just how sick she is (even though she doesn’t look it). When the cardiologist recommended the evaluation also it all hit me in the face. I can deal with the life that we have now with this illness but I don’t know if I can watch Nicole go through what she will go through during major heart surgery and a double lung transplant. I was reading a little about lung transplant results and found that the survival rate for lung transplants is 82% but in my mind I think not of the 82% percent who survive but the 18% who don’t.
A part of me believes that she needs to be on a higher dose and longer time on the Remodulin before we can say it was a failure but something that the cardiologist said really hit be like a brick in the face. He said ” if she had one bout of diarreha where she could keep no fluids down it would be lethal.” I can talk myself into believing that even though Nicole is ill she isn’t that ill and she does all right for herself even though I know in the back of my mind if she got the flu she would be in the hospital and it could be potentially deadly for her. I understand the doctors don’t want to wait to long because of the length of time this process takes but I am just not ready to deal with this and I don’t think that I have been able to deal with this since her diagnosis but I just fake it.
In 2009 I believe God put in my heart to “get my house in order”. I have quite a bit of clutter and not enough house. I, of course, have put this off thinking that God didn’t really speak to me etc. I have been trying to declutter at least 15 minutes a day if it is busy and if I am home for the day I will go for 30 minutes if not longer. I struggle with this because I don’t want to do this (just like I don’t want Nicole to be sick). I have to make decisions every day about Nicole’s medical issues, finances etc. and don’t seem to have a hard time with this most of the time but make me look at a piece of clutter and decide what to do with it and I can’t. I was already overwhelmed with my duties the past couple of week as Nicole’s teacher and getting out her progress reports to the school we use for that along with a ton of paperwork that I have to fill out for Nicole’s disability review for SSI as well as some other commitments that I have and my mind has turned into a tornado swirling around.
Sometimes I just feel like I want to stop the world just for a day and not have any problems so I can get my head in order, unfortunately, I know this isn’t likely to happen. I always think of myself and what this is like for me but I don’t consider that it is Nicole that has to live this life. I feel that life is the hardest for those that are left behind. We are the ones who grieve the loss not the person who passed. I don’t know if this is just my warped feelings or if it has some merit. I have been reading this book called “When Bad Things Happen to Good People” . The author was talking about how people respond to tragedy and that the majority (I’m included) assume that God is the cause of the suffering because we deserve punishment or He doesn’t care. We are left hating ourselves for deserving the fate or hating God for sending this to us. He asks could it be that God doesn’t cause our suffering but He stands ready to help cope with the tragedies? I have been thinking a lot about this lately. I’m still not clear on my feelings about it though.
Kare said on October 11, 2012
Jane~ No wonder you wish you could stop the world for a day and not have any problems to deal with. You have so many serious things going on. Have you shared with the cardiologist your feeling that he hadn’t gone up far enough on the Remodulin? What you feel is important. Of course,you want to be sure he’s tried all avenues before a transplant is considered. We all deal with periods of denial. It’s just a way to temporarily be “free” from whatever bad thing is going on. In the absence of major symptoms,I can easily understand that your mind refuses to accept what your heart knows…that Nicole is very ill. When she has a very good day,you might even doubt her diagnosis. I think that’s perfectly normal. As you said,though,the transplant process is a long one. I’m sure she will have to meet certain criteria,before she can even be put on the list. I think it’s truly remarkable that you find the time to actually do due clutter work! Now,mind you,this is coming from someone who just THINKS about doing the same ,becomes overwhelmed,and gets NOTHING done! I’m a great one for writing ” to do” lists that always seem to remain ” to do”. I need to do the timer thing and stick to it. I don’t believe God is the cause of all our suffering. I admit, I often question why bad things happen to people. God never promised that they wouldn’t…just that He’d be there to help us through them. I know not everyone believes that. But I know I have had many challenges and difficulties,and have felt God there every single time. Not always in big ways. I remember when the doctor found a lump in my breast and I had to go in for a biopsy. I decided not to tell anyone,even my friends,because I just didn’t want to take the chance it would,somehow,get back to my mother. I mean,why have her worry before she needed to,if you know what I mean. So there I was in the doctor’s office,with my body about to be punctured,when I told God that now I wished I had brought a friend to hold my hand through this. At that VERY moment,the nurse who was in the room took my hand in hers and held it for the rest of the procedure. I don’t believe that was just a coincidence. By the way…it was benign. Anyway,I believe in God and the power of prayer. In my bedroom,I have a dry erase board with the names of people who have asked for prayers or who I just want to pray for. I added Nicole’s name. I hope that’s okay. I’m sending a big hug and hoping you can ,maybe,take a little time for yourself.
EllysGdaughter said on October 11, 2012
Dear Jane,
You have been so strong in tackling the change in medical procedures for Nicole! I see you being able to tackle this next “adventure with Nicole” one baby step at a time. Your love for her shines through. I think you keep moving forward even when you are dealing with the clutter! I don’t know what it would be like to be in your shoes but I will pray that you will be able to give God some of the “heavy suitcases” you are carrying right now. Looking at the transplant issue is certainly a huge step, but starting the process doesn’t mean that Nicole has to have the transplant if it becomes unnecessary? I hear you say that you may regret not having these steps in place when you want the option later? What does Nicole say about the transplant conversation? Meeting with young folks who have gone thru the transplant may be helpful for both of you. Is there another ABEKA home-schooling mom to help you? Thanks for sharing these hard steps, I’ll be praying.
ejourneys said on October 11, 2012
Hi, Jane — I’ll be praying, too. I understand the denial — in my own case I was afraid that I would be overreacting otherwise. Sometimes it can be so hard to trust our own judgment, especially when there seem to be no guideposts or an instruction manual in the “regular world.”
Since the transplant process is a long one, I wonder if printing out this entry, circling your thoughts about the Remodulin and about Nicole’s reactions when starting/stopping medications, and sharing it with the doctors might help. It’s not arguing with them. It’s asking them for more information and sharing what you know and have experienced. At least that way, you know that you have covered all the bases. (((Hugs))) to you and Nicole.
Jan said on October 12, 2012
Hi Jane,
I can feel your anxiety and worry. You’re not just a caregiver, you’re a mom, too. Worrying comes with the territory.
I know Nicole has a competent team of doctors on her case. In your journey down this road, have you considered getting a second opinion about her care plan?
My 12-year-old niece just recently had open heart surgery for a congenital valve defect. She is doing great and will return to school soon. It was absolutely wrenching to watch her and her family go through this process. I cannot begin to describe the emotions, but praise God, now she is recovering very well.
I know your mind must be reeling, but I wanted to let you know that there is hope. In the weeks that I was agonizing over my niece’s surgery, a friend from church told me about her son, who had open heart surgery when he was just 4 years old. He’s a grown man now, and no that there had ever been anything wrong.
In the hospital waiting room, we met a young woman who was there waiting for her baby to undergo some tests. She seemed to be very savvy about the hospital (All Children’s Hospital in St. Pete — excellent!), and the doctors and procedures. Turns out that her baby had already had three heart surgeries (and she’s just two years old) and they were evaluating her for a fourth. She told us her baby had been born with only half a heart. But, she says, the little girl runs around and plays and sings and goes about her business as any healthy child would. She just gets tired more easily. Hearing from my friend, and from this young woman helped me to know that we were not alone.
I hope you know that you are not alone, too. You have a lot of people in your corner. You will make the right decisions. And, like the book says, God doesn’t “cause” bad things to happen. But He does promise to be with us through them all.
I’ll remember you in my prayers. Here’s a hug!