Transplant Appointment

On November 20,  2012 we had our first transplant appointment after both Nicole's cardiologist and her PH specialist believes the time for this has come.   It was also agreed upon that she would go to the center that is local as opposed to the one 2 1/2 hours one way from our house. 

In December she had a CCTA and TEE to get some pictures of her heart.  The question is can she just have a double lung transplant or does she need a lung/heart transplant.  Nicole has a large 2 1/2 cm size VSD (which is a hole between the right and left ventricle of the heart) as well as two or three other smaller VSD's.  She also has a PFO which is like an ASD (artial septal defect  which is in the top part of the heart) but instead of a hole it is a flap.  She also has Eisenmengers Syndrome which is reverse shunting of the heart.  On Jan. 2nd, 2013 she had a right heart catheterization to round out the cocktail of tests. 

Last week we had our first appointment back to the center since these tests.  The lung director came in and told Nicole and I based on the tests they performed they believe she needs a heart/lung transplant.  Let me tell you,  this was like a kick in the gut... it was like the day of her diagnosis three years ago except for I didn't lose my breath.  We have always known that she would need a lung transplant but as the cardiologist said it would be "years down the road" .  I  didn't realized that was only a mere three years after diagnosis in Jan. 2010.  I tried so hard to keep my composure because I have to be strong for Nicole but I failed and some tears leaked out.  The nurse caught on to this and got me some tissues.  I think the doctor could tell too.  Nicole was sitting in the middle of the room in her wheelchair and I was in the back so she had her back to me.

The doctor went on to explain that the reasoning for this is that while her heart is repairable it would take too much time to do this and they are under a time frame as the lungs have to be in the body within six to seven hours. I record all of Nicole's appointments but haven't been able to listen to this one yet.  It was my understanding that Nicole's PH specialist, her caridologist, the two doctor's at the transplant center and a pediatric heart surgeon (who we haven't met) was going to get together and discuss the options.  I was under the impression that this had happened.  According to the cardiology nurse the meeting that was originally scheduled one of the doctor's at the center had a transplant and only the other transplant doctor and the PH specialist showed up for the meeting.  It was never rescheduled.  It's my understanding that the cardiologist doesn't agree with this recommendation and believes that the heart can be repaired and she would only need lungs.  They have even tossed out that I should go for a second opinion in Gainesville or Miami.

I don't know what to believe.  We also saw the social worker at this appointment.  Nicole was diagnosed with generalized anxiety disorder (GAD) and obsessive complusive disorder (OCD) in 2005 and takes Zoloft for this.  She sees a psychiatrist and a counselor/therapist.  The social worker said that all the things that would be a concern for them we are already taking care of since she doesn't have any drug, alcohol or cigarettes addictions.  The doctor asked for the name of her counselor which I gave to him.  He also said that he would like Nicole to start talking about her feelings toward transplant with her and also he wanted her to do research about it.  They are also going to get a mentor for her which is somebody that has had a lung or a lung/heart transplant.  He also wants a conference with Rick and I and the social worker and it is my understanding that this would be without Nicole present which would allow me to ask questions that I wouldn't ask in front of Nicole.  She also needs to get another 6MW (this is a diagnostic test used to measure how well a medicine is working.  It is basically what its name implies a  6 minute walk. She walks for 6 minutes at her own pace and they will log in the distance to see if she can walk farther than the last time she had one).  I'm not sure if she will be able to get this done at rehab or we will have to go back to the transplant center.  She also needs more bloodwork which we will go to the hospital for on Thursday.  I don't remember what the bloodwork is for though.  I think and hope they will check her potassium as this is like a yo-yo going up and down. 

I got a call that evening from Nicole's counselor asking if I would give her verbal permission to talk with the doctor as he had already called her.  I did give this and I can because I have power of attorney for Nicole and have since the day she turned 18.  She sees her counselor the first full week of March so hopefully she will let us know what the doctor wants her to do. 

This has been very emotional for both Rick and I as well as the rest of the family.  I know it has for Nicole also but she usually has delayed reactions to trauma or stress. It will manifest itself  in  some way. 

I have looked at some statistics for heart/lung transplants and in 2011 the estimated number of heart/lung transplants in the US was 30.  The cost of the procedure was $1,248, 400.   This is the lowest number of all the transplants listed.  According to a 2010 study that assessed the long-term outcomes of people who have had a heart-lung transplant they found that:

• 70% will survive for one year
• 50% will survive for two years
• 39% will survive for five years 

I'm not impressed with these statistics.  I had a hard time coming to terms with a 50% 5 year survival rate for a double lung.  I guess the biggest question for me is does the benefits out weigh the risks and I can't say that I truly believe this.

One of my concerns is that Nicole believes that she will be "normal" if she gets a transplant and I don't know if this is realistic.  I heard  Helene Campbell say on the Ellen show after her double lung transplant this it wasn't a cure but a treatment and this is very true.  Nicole will not have the illnesses anymore, nor will she have to be in a wheelchair because she can't walk long distances or be on oxygen 24/7 but  there is a lot of other things that she will have to do for life.  The first two years after transplant is filled with a lot of tests, bloodwork, clinic visits,  rehab etc. but her quality of life will be better so they tell me.

Anger and frustration....

I had really believed that I had worked through my issues of anger at the medical community over the extreme negligence that has been displayed during Nicole's life.  Obviously, I still have a way to go....

After yesterday's, prescription debacle and the anger I have at the doctor who either personally wrote the script for a narcotic for Nicole's site pain or had someone else write it and he just signed it without making sure it was correct and the consequences being that Nicole has to take a narcotic that makes her feel worse with side effects of nausea and dizziness and doesn't seem to manage the pain all that well.  It is just an unacceptable mistake in my eyes.  I realize that we are all human and we all make mistakes and I can accept that and I try very hard not to have unrealistic expectations but when almost every single doctor (and she has seen a lot of them) in Nicole's life has not done something they should not have, not told us something that would have made a difference or made me question things more etc. this gets past the point of just a simple mistake.  

Yesterday, was a day that really tried my patience and forced me to see I still have a lot of anger which I didn't realize until this morning.   I have a goal for 2013 to get my house declutter and to get organized so I can feel more in control of the chaos that is my life.  I have been working every morning for 30 minutes on getting Nicole's medical records in order.  I can almost see the light at the end of the tunnel.   I have been getting the records from her former doctors together and I was working on the allergist.  She saw this doctor from 2001  until 2011.  Nicole has only a few allergies on paper but one of the worst is to dust mites and she was in the office once a week for at least three years for allergy shots.  In 1999 when she was originally tested while in the hospital for double lung bacterial pneumonia her IgE levels were 441 the normal range is between 2 - 25.  Her pediatrician said "she has allergies we don't even know about"  The IgE is one of the five subclasses of antibodies.  IgE antibodies are found in the lungs, skin, and mucous membranes. They are associated mainly with allergic reactions (when the immune system overreacts to environmental antigens such as pollen or pet dander) and parasitic infections.  I was going through her records that I received at some point in the last few years but I believe they stayed in the envelope that they came in because I didn't have a reason (in my eyes to really look at them) I believe these records were through 2007.  This doctor was not the doctor who diagnosed her with asthma he just treated her for it.  If  you have seen an allergist then chances are you have had a pulmonary function test (PFT).   I was looking through the PFT's that she had taken and during 2006 - 2007 she had at least three PFT's that had an interpretation saying this:  "possible EARLY OBSTRUCTIVE PULMONARY IMPRAIRMENT.  This is suggested by the reduced FEF 25 -75 with a normal FVC and FEV1 (I have no clue what that means).  This finding can be due to a mild degree of small airway disease and/or the earliest stages of emphysema.  This may be reversible in nature; therefore, REPEAT TESTING FOLLOWING BRONCHODILATOR ADMINISTRATION IS RECOMMENDED.  This interpretation is only valid upon physician review and signature. "   Now I ask you ....... why wasn't I told this??????? Isn't it a little bit of a HUGE red flag when they are interpreting emphysema in a child of 12 years old.  I don't know of too many of them.  I also don't recall her ever having any bronchodilator administration.  This is just another example of negligence on the doctor's part.  If I had known this I would have asked more questions and had her get more testing etc. 

My concern lately has been the fact that Nicole has had so many GI issues.  What have they missed pertaining to this?  My fear is that she has other issues going on.  She has been seen twice in her life by GI's and the first one I blame for the first real mess up with her diagnosis being missed and this is where her congenital heart defect was missed and she probably also had her PH at this point.  The second GI doctor  who I really, really liked was much more on top of things but all the tests that she did came back normal so the mystery remains and is assumed (which we all know what that means) that it is the Tyvaso (which she was on at the time)  and other PH drugs causing her stomach issues. 

The feelings of helplessness and the lack of support I feel from the medical community is one of the hardest things to accept.  I have to fight and fight for everything.  I know that I am not alone in this fight and there are many others with PH or any other chronic disease that must go through what I do.  The Bible says that God is our vindicator but as of yet I don't feel that there as been any closure or justice for Nicole. 

Continuing the transplant process

We started the transplant evaluation process in November 2012 after both Nicole's PH specialist and her cardiologist agreed that it was time for this.  She saw the heart/lung surgeon and than she saw the lung director at the transplant center.  The doctor ordered several tests which included an echocardiogram, a CCTA scan in which they inject dye into the patient and watch how it flows through the heart, a TEE which is where a tube was stuck down her throat with a camera which shows images being seen on the sonogram machine, and lastly a right heart catheterization.  After the doctor had gotten the results of all these tests the doctors at the transplant center, another heart surgeon that specializes in congenital heart defects, her cardiologist, and her PH specialist were all going to sit down to discuss a plan of action, namely can they repair Nicole's heart or does she need a new one in addition to two lungs.  I know there was a meeting schedule for the second week in January but one of the doctor's at the transplant center had a heart transplant and couldn't attend.  The PH specialist told me that only himself and the lung director showed up for that meeting.  To my knowledge another meeting has not be scheduled.  During the week of January 21st when Nicole was in the hospital the transplant center center had called because they had received an email about the PH support group meeting which also stated that Nicole was in the hospital.  The doctor wanted his coordinator to call and find out how Nicole was doing.  She also wanted to schedule an appointment to come in and continue with the transplant process and told me to call either the following week or week after.  It has been two full weeks since Nicole was released and I have been procrastinating about calling.  They did call on Friday but my cell phone was in Nicole's room as I have her medicine alarms on it. I had a call so Nicole had to take her medicine without me waking her up.  I didn't return the call on Friday like I should have either.  I MUST DO this on Monday regardless of my fears. 

My friend, Denise, founder of caregiving.com,  had asked me to blog about this and write out my feelings.  I am hoping that this will let me come to terms with my feelings.  I am procrastinating because I am afraid.  I am afraid because I don't want to hear the results of the testing and what they will determine, I am afraid she will die during the transplant itself or after the transplant because of complications (infection, rejection).  The 5 year survival rate for lung transplants is not as good as I would like and while I don't have the exact number in front of me I believe it is right around 50%.  I am afraid I will not be strong enough to endure this as it will mean a long hospital stay and require Nicole being hooked up to many machines and breathing devices.  I can't in good conscience leave Nicole by herself in the hospital.  I am afraid that they will deny her (either the transplant center or Medicaid) or we will not be able to raise the money needed.  The estimated cost of a double lung transplant in 2011 was $797,300 and the cost of a heart/lung is $1,248,400.

I'm afraid that Nicole's perception of being "normal" after a transplant is unrealistic.  I don't have the specific information in front of me but I know that while she will be "cured" of PH and she will no longer have heart issues she will not be able to go out into any public place like malls, Disney etc. for the first six months after transplant.  She will have to wear a mask to protect from germs in public for the rest of her life.  She will have to visit the center on a weekly basis for either the first six months or year.  She will also get regular testing.  She will still take a lot of medication (maybe not as much as she does now).  She will have to keep a daily log of symptoms and all her vitals including blood pressure, oxygen saturation levels, temperature twice a day for the rest of her life.  She will be normal in the respect that she will be able to breath normal but I don't think she really grasp what after transplant life will be like... the question is will it better than it is now or not??????  

Follow up with Infectious Disease Doctor

Nicole took her last doses of antibiotics yesterday.  I know she was so thankful as the Flagyl caused a very bad taste in her mouth where nothing tasted good and also the nausea was brutal.  She also vomited last night. 

We had a follow up appointment that the doctor requested upon her discharge from the hospital.  He answered all our questions today.   My biggest question was/is how did she get colitis?  He doesn't know.  Every blood culture and every test for bacterias that cause colitis came back negative.  He is also curious as to why they couldn't see her appendix on the catscan.  He said he wasn't totally convinced that she had infectious colitis as this is usually widespread through the colon and hers was focal in one spot where the colon joins the appendix that they couldn't see.  The antibiotics took the fever away so as he said to Nicole she is just a mystery to them.  I agree with this.  Nicole her whole life has had issues that the doctors can't tell us why she has them.   He couldn't answer with certainty if this would happen again.  I explained to him about the Remodulin causes diarrhea and he said that her having diarrhea on a regular would not cause colitis. 

He did recommend that she be evaluated again with a gastroenterologist.  She has seen two different ones in her lifetime.  She has had a sonogram, an endoscopy, been tested for lactose intolerance, celiac disease and I believe that is all.  He believes that she should be tested for crohn's disease and/or colitis that is caused by ulcers which both fall into irritable bowel disease.  I didn't find anything that said irritable bowel syndrome will cause colitis and he didn't mention being tested for that.  He said that they did not have to put her to sleep for a colonoscopy they could just sedate her. 

I will just wait for a report from his office of the visit and then talk to her ph specialist and her cardiologist and get their opinion on how this should be handled.  I definitely don't want to see her in the hospital again and I know she doesn't either.