Thursday, January 10, 2013

Decisions in Caregiving: Treatment

Decisions in caregiving can involve placement as Kathy so openly shared with us. 

Caregiving decisions can also be about treatment: What treatment plan to follow?  What medications to use?  Or even: do we do any treatment at all? 

Each answer can leave caregivers wondering if they made the right decision and asking “what if” I had made a different decision?  For quite some time, I questioned my own decision to honor the “I don’t want dialysis” edict from my dad when he was dying of kidney failure in the hospital. 
Jane with daughter, Nicole

Jane Northrop is a caregiver who has to make several decisions about treatment, medications and plans for the future for her 18 year old daughter.  Jane is a stay-at-home, homeschooling mom who was thrust into the role of advocate when her daughter, Nicole, was diagnosed with a rare, life-threatening, incurable, heart-lung disease at 15 years old.  (After doctors – for years – dismissed Jane’s concerns and misread EKGs).

Jane juggles the numerous appointments and medications for Nicole yet has found the time to be a speaker for a Peer Mentor group for one of Nicole’s medications and is actively involved in the Pulmonary Hypertension Association (PHA).  Jane blogs at and she also blogs on her own blog, “A Day in the Life” and has graciously allowed me to reprint a recent post.   

Like many caregivers, Jane has had to educate herself about her daughter’s condition and how the heart and lungs work (and how they aren’t working properly for her daughter) so she, her husband and Nicole can make informed decisions about treatment options.  Sometimes it seems as if caregivers get their own Ph.D. in whatever disease, disorder or condition their loved one has and in record time (because we usually have to get up to speed very quickly)! 

I admire how much Jane knows about Nicole’s condition and appreciate how she shares her knowledge with others.  She has helped more people than she will ever know, even now while moving toward a possible heart/lung transplant for her daughter.

In Jane’s words:

Transplant Evaluation Update
Nicole was recommended for transplant in November of 2012 by her cardiologist and pulmonary hypertension specialist.  On November 20, 2012 she saw the director of the heart/lung transplant department at Florida Hospital Transplant Center.

On Dec. 5th she saw the medical director for the lung transplant department.  There is no question that Nicole needs a double lung transplant but the question is what to do with her heart.  Nicole was born with what they have told us are several congenital heart defects.  She has a large VSD (ventricular septal defect).  A VSD is a hole between the left and right ventricles of the heart which is the lower chambers of the heart.  We were also told she has three other smaller VSD's.  We were told that she also has an ASD (atrial septal defect) which is a hole between the right and left atrium which is the top part of the heart.  Because these defects weren't diagnosed until Nicole was 15 years old it has caused Eisenmengers Syndrome which is a rare heart condition that causes reverse shunting of the heart.  In a normal heart, the blood shunts from left to right but with Nicole it will at some point reverse to right to left which will cause heart failure.  The combination of the heart defects and Eisenmengers has caused her pulmonary arterial hypertension which is high blood pressure within the arteries in the lungs which basically causes the arteries to narrow and cause difficulty with breathing.  The damage done to Nicole's lungs can't be reversed and there is no cure for this disease.   

The lung director had ordered three different tests to get good pictures of Nicole's heart and the way it functions so a team of doctors which include the two at the transplant center, Nicole's cardiologist, Nicole's PH specialist and a pediatric heart surgeon who specializes in congenital heart defects can get together and figure out if the heart can be repaired or if she needs a new heart. 

On Dec. 21st Nicole had her first heart related test called a CCTA scan which stands for cardiac computed tomography angiography.  This is an imaging method that uses a CT scan machine to look at structures and blood vessels of the heart.  Dye is injected into an IV and then pictures are taken of how the dye moves within the heart.  This test seemed to go well and the worse part Nicole said was when they injected the dye as it made her feel real warm and the sensation of peeing her pants.  We were told by the nurse that they got some good pictures.  This was an outpatient procedure.  We ended up driving an hour away to another Florida Hospital because they had a better machine.

On Dec. 28th she had her second heart related test called a TEE which stands for transesophageal echocardiogram which uses sound waves to create high quality moving pictures of the heart and its blood vessels.  A camera was stuck down Nicole's throat and pictures were taken from Nicole's stomach of her heart and captured on a sonogram machine.  The worst part of this test was the gargling of the numbing solution.  Nicole had to gargle three times for a minute or two each time and then swallow the stuff.  The first time her gag reflex took over and she spit it back out so the next two times I had to hold her mouth shut. When Nicole had to start the gargling the nurses told me that I had to go into the waiting room but Nicole got very nervous and anxious which her doctor saw and said that mom should stay for this part.  Right before I left they gave her the medicine for the sedation part.  The doctor wanted to make sure that she didn't remember any of the procedure – which she didn't.  She seemed to take a long time to come out of the sedation.  The doctor showed me all the pictures and explained everything to me.  During the procedure they inject bubbles and they can determine where the holes are by watching them.  The doctor could not see any evidence of an ASD as the bubbles didn't travel across from the ventricles to the atrium (there's a flap type thing that separates the two chambers).  He also didn't see three smaller VSD's but what he believed was a channel that gives off the illusion of three smaller holes but he thinks it is only one.  He said that her right ventricle looked very good.  With PH the disease will weaken the right ventricle and so far this hasn't happened in Nicole's case.  He said her bicuspid valve looked very good with no leakage and he thought maybe the pressures have come down in her lungs.  The doctor and I were very encouraged by this but an echo isn't always an accurate indicator so this is why the gold standard testing is the right heart catheterization. 

On Jan. 2nd Nicole had a right heart catheterization. Anytime she has this procedure it requires an overnight stay in one of the lovely suites in the cardiac ICU floor of the hospital. Nicole was going to be sedated again for the procedure since putting a patient "to sleep" is very dangerous for someone with PH.  Once again she woke up during the procedure because the anesthesiologist I think is a little over cautious which isn't necessarily a bad thing but it can create a bad experience for Nicole.  The procedure seems to take longer than I always think.  We were in the prep room where they start the IV get all the history meet the doctor's etc. and my friend and Nicole's old respiratory therapist at rehab happened to look on Facebook and see that Nicole was at the hospital and came by to see her.  She stayed in the waiting room with us until I was called and told that the procedure was over.  She then went home.  I told her she could stay and go see her but she didn't want to intrude on "family time."  I absolutely love Kathy and everything she has done for Nicole and me.  It was very nice of her to spend her first day off from work in a long time to sit at the hospital with us. 
One of the doctor's came in and explained how things went.  He said her pressures didn't go down but the number he used reflected a lower number than last time.  He said essentially everything was the same as her last cath in June 2012.  He did say that she doesn't have an ASD but a PFO which is a patent foramen ovale which is a hole between the left/right atrium but technically isn't a "true" hole but more like a flap that intermediately will open and allow blood to cross over.  I think that this is right but I could have it reversed but the left side of the heart has the red blood and the right side has the blue blood and with Nicole's VSD they are mixing so her lungs and other parts of her body are getting blue blood when she should be receiving red blood. 
If you see Nicole without O2 (oxygen) her lips may be blue and her fingernails and toenails are always blue and this is called cynosis which is caused by her VSD. The two doctor's present for the cath both agreed with Nicole's PH specialist that there was a channel along the ventricle that gave off the illusion of three smaller holes but in actuality he believed there was only one. 
During this procedure they also checked the pressures in her liver because increased pressures in the lungs can travel and impact the liver; thankfully her liver is good.  After the procedure Nicole had to lay flat without moving for four to six hours.  When they took the balloon thing off her groin she was almost in tears from the pain... it breaks my heart.  She is so brave and is such a trooper. 
It is my assumption that I will get a call from the lung department at the transplant center having us come in to explain all the conclusions that the testing came up with and what is the plan in regards to Nicole's heart: transplant vs. repair. 

I know that there are more tests that need to be done but I hope that these were the most strenuous for Nicole.  It is very stressful and difficult watching Nicole get all these tests done and the anxiety and stress this causes all of us.

Many thanks to Jane for sharing a most difficult few months. Please share your own caregiving decisions about treatment in the comment section or email me at to share your caregiving story. 

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