Friday, December 26, 2014

Miscellaneous Ramblings

I hope that everyone had a wonderful Christmas.  Ours was low key this year but I always enjoy the time with family.  I also like presents too but I can live without them :)   The one thing that I want more than anything else is a cure for Pulmonary Hypertension and so far that hasn't come but I believe it will.

Every morning I watch Joyce Meyer and this morning was no exception.  It was a re-run I believe but I always learn something new or if I am struggling with some area a show pertaining to that will air. It's a little freaky sometimes.

This morning's show was about Trusting God When You Don't Understand.  This is something I always have to remind myself.    There was a segment with a couple who had lost their young son (under 5 years old) to a rare genetic disorder which at this time is incurable.  The mother stated when she found out that she was pregnant with him she prayed that he would not only be a blessing to them but to others as well.

I can so see the blessing that Nicole has had in our life.... I have learned so much from her and she is truly my hero.  I also know that she is a blessing to others as well either through her illness and the PH community as well as the way she lives and copes with it.  She is also a blessing through the beauty of her artwork.

As I watched this segment (which I have seen before) with tears rolling down my face I thought about Nicole and how I don't understand why not so much that God allowed her  to be born with congenital heart defects but more so why were her health issues misdiagnosed for 15 years.  I am in awe everyday of the things that could have happened and I am most grateful for God keeping her safe.

Unless you are a parent of a chronically ill child, and it doesn't matter if the child is 2 or 20 they still are your child, it is harder to grasp the struggles that a parent goes through everyday.  It is absolutely heart-wrenching to watch your child suffer. The hopelessness and helplessness can be overwhelming at times.  There is one particular person that was such an influence after I heard her speak about her struggles with her son less than a month prior to Nicole's diagnosis and I just couldn't wrap my brain around the strength and courage that she has everyday. She was and continues to be an inspiration to me.

I truly believe to the depths of my soul that if it wasn't for Nicole's illness she wouldn't be the artist that she is today.  Her artwork is incredible and I am so proud of how she continues to grow and do things that she never would have thought she could do.  The first time she drew a face, the first time she drew a figure etc.   I see the passion in her eyes when she completes  a piece that is she proud of.

My heart goes out to those parents that have lost children and I never, ever want to experience this and I hope that God will spare me this pain but I know if I had to choose to either live my life without Nicole or have her live her life without me I would rather live without her.

As we end 2014 and move into 2015 I would love to see a cure for PH but short of this I would love to see Nicole to continue to grow in her passion of art.  I would also pray that she remains stable and that the medication that she takes even with all of it side effects and chronic pain will continue to do this for her. I pray for continued good health for myself and the rest of my family.  It is very frustrating and stressful dealing with a chronic illness whether it is for yourself or for a child but I pray that I will continue to provide that support and love for Nicole for as long as she requires me to do so.  I would do anything for her and if this is what I need to do I will.

I would love to see others be more compassionate in regards to someone who is disabled either through a chronic illness or through injury especially when their disability may not be "visible" to the naked eye.  I would love to see others think about how they would want someone to hold a door open because they are pushing a person in a wheelchair or are struggling carrying a child etc.  I struggle everyday with being judgmental because something looks different than what it is .... I can tell you that God has really opened my eyes to this  A LOT since 2010 and continues to do so.   As Joyce Meyers says "I'm not where I need to be but thank God I'm not where I use to be."

I wish everyone a happy, healthy, safe and prosperous 2015!!!!!!!!!!!!

Monday, December 22, 2014

Frustration with reordering medications......

Just spent 45 minutes on the phone which included being disconnected once in the midst of transferring me.

I have to get Nicole's medication through a specialty pharmacy and they are shipped via UPS monthly.  We get three medications through this pharmacy two of which are oral medications and the third is her continuous medication which she receives subcutaneously through her skin via an infusion pump.

I know how much medication I have left when I call so I start with her Letairis which she will run out of first on Dec. 29th.  The customer service representative is suppose to ask certain questions like did she have her monthly pregnancy test etc. and she didn't.  They have their own calculations of when she will run out and most of the time with the orals we are in sync.  She says I can have this delivered on the 29th.... I said "she runs out on the 29th and I don't want to receive it on the day she runs out" and she replies "well there is a holiday" and I reply "on Thursday".  She then says she will have it delivered on Friday the 26th.... this is much better and I will take this.  I realize that I should have called last week to have this set up but they should have called me saying it was time to reorder and to my knowledge they hadn't.

The second medication she was also suppose to ask questions which she didn't.  I asked her how many tablets Nicole had based on their records and it was what I had which was enough for 13 days including today.  She said do you want it delivered on Friday with the Letairis and I said sure if you can but I don't think you will get insurance approval.  She said sure I do this all the time.  She then says I will have it delivered on January 2nd.    I then asked her to reorder the Remodulin and she says she has to transfer me (usually the same c.s. rep will do it all)  while she's transferring me she disconnects me (at least she called me back).  During this "hold" time I count out 13 days and find that she will run out on the 3rd.... I am not waiting until the day before she runs out to get her meds.

I get another c.s rep who is going to do the Remodulin but before she does this I tell her I need her Adcirca for a sooner date than the 1/2 shipment.  She said the rep is entering it now and puts me on hold.  She isn't able to contact the rep so she is going to fix it herself.  She changes the date to the 30th which is still a little to close for comfort but I will live with it.  She then says she wants to confirm that the Letairis will be delivered on 1/2 and I said "no" she runs out on the 29th.  I am so glad that she confirmed this or I would have been speaking to a supervisor on Friday.

She then tells me that it is to early to re-order the Remodulin which means I have to call twice a month to reorder her medications.  I will call back on the 31st to reorder.   I have re-ordered all her medications even when they are a couple of weeks out for the last 4 years.

When I call back I will also find out if I can get a email or something from UPS stating that I will receive a shipment so I know that it was shipped when it was suppose to be.

This is so frustrating and this is where most of it comes from on a monthly basis is from the pharmacies.

Sunday, December 14, 2014


It has been awhile since I have written on this page.  I had created a facebook page called A Day in the Life and was using this page to write about my daughter and our journey with her chronic health issues.

I did like updating the page and did so quite frequently because it was convenient but I have decided that I am going to come back to blogging on here.

I don't know much about how to step up a blog or making it look pretty like some of the blogs that I see but I hope that I will learn as I go.

I will still forward my postings here over onto facebook and will not take down the page.  I will probably share things on there like articles and such that pertain to Pulmonary Hypertension.

I am excited about coming back to this type of forum.  I am not going to put pressure on myself by saying I will blog once a week, twice a week, once a month etc.  I will just do it when I need to update about my daughter.

I am grateful for the followers that I have on my facebook page and hope that they will follow me over here.

Sunday, March 9, 2014

Week of March 3rd 2014

This week started out on Monday with a trip to University of Florida in Gainesville.  This time Nicole was being seen by the pulmonologist in the Lung Transplant Program.  She was also having a chest x-ray, a pulmonary function test (PFT) and a 6MW (minute walk).  Nicole increased her walk distance from 913 ft to 930 ft.  This is good.  The doctor said that her PFT was better than the one she had when she was sixteen.  I was glad to hear this too.

Nothing new happened during the visit with the doctor. He basically said that he was a passenger and the Congenital Heart Center was going to call the shots.  The nurse coordinator did tell Nicole that post transplant she wouldn't be able to have any reptiles, cats, or birds as pets.  This wasn't taken well by Nicole as she does have a turtle that she adores.

It sounds like she will see the pulmonologist at or around the same time as she sees the cardiologist up there so we don't have to make two trips.  She will be seeing the cardiologist there on August 20th and will have another EKG and echo at that time.  She is suppose to have a right heart cath at some point between now and then and this will be up at the University of Florida.

Nicole seemed to like the nurse coordinator and the pulmonologist but Nicole does like everybody for the most part unless they are mean to her.

On Thursday,  Nicole had her 3 month visit with the PH specialist.  I had sent  an email to his nurse basically telling her what the cardiologist had suggested in regards to Nicole's medication.  The cardiologist at UF wanted Nicole's Revatio to be increased from 20 mg three times a day to 40 mg three times a day for three to six months and then increase it again to 80 mg three times a day.  The PH doctor didn't have a problem with this, however, he is going to switch her from Revatio (viagra) to Adcirca (cealis).  The reason for this is because Revatio while it is available in 20 / 40 and 80 mg strength it is only approved for 20 mg by the FDA and therefore it is very hard to get the insurance companies to cover one of the other doses.  With Adcirca she can get the equivalent of these doses with no problem and Nicole will only take it one time a day as opposed to three.   I will be refilling her Revatio for the month as it will probably take a couple of weeks for it to go through and get shipped.  I hope that she doesn't have any issues switching over. He said she may have some back pain.

The PH doctor had no problem with increasing her Remodulin but it will be only increased on the same day as she does a site change.  He said she might as well have the pain, nausea, diarrhea all at one time and get it over with.

He didn't want to change her pain management because the stronger the narcotic the more side effects such as drowsiness etc. and he just wants to keep it the way it is.  He said that the ibuprofen did not lower the platelet count but it does affect the way the platelets are formed or something like that.  I have to transcribe the recording of the appointment, hopefully, this week.

He didn't realize that Nicole was changing her site every 30 days because of infection which he said may be because of the very high dose of Remodulin she is on.  He also thought it might be good to try an antibacterial nose spray that she takes for 5 days but I am not clear on when she is suppose to take it.  I will have to contact the nurse tomorrow to ask about this.  He also prescribed Hibiclens in the pump that he wants her to use in the shower like a soap or he wants her to soak in it I'm not clear on this either but I do remember him saying to do it with a site change.  This will be a trial and error thing as he doesn't know how often she will need to use this.  He is hoping that these things will cut down on her infections.

He was upset with me because his nurse said I was rude to her in an email.  I didn't realize I was rude and I am not real sure what email she is referring to but it wasn't my intention.  I did send her an email apologizing to her.   I need to work on being a little nicer (I am nice but if I am having difficulty getting a medication etc. I can get frustrated and not be nice) and really watch what I say.  I am so grateful to the doctor and his nurse for helping Nicole as much as they have with the treatment of her PH.

After the doctor's appointment we went with Jackie, the other support group leader to visit one of our support group members who is in the final stages of PH as well as some other health issues.  The PH specialist (the same one Nicole sees) said there was nothing more he could do for her.  She was so excited to see Nicole and gave her a hug and told her that she loved her.    I don't think Nicole really realizes what that meant to Suzy.  We then went to Costco and Nicole drove a motorized cart for the first time she did an awesome job and didn't run over anybody, knock anything over etc.  

This coming week doesn't have any doctor's appointments on tap only her monthly blood work and a return to rehab.  This will probably be the last full week before she has to change her site again.

I have several things to do this week in regards to Nicole's medical issues.  I have to contact Social Security to find out if she meets the criteria for Medicare.  I also have to transcribe the appointment with the PH specialist. I need to re-order her Revatio.  I need to call Accredo and ask a question regarding some paperwork that they sent me when Nicole got her first shipment of Revatio through them.  I have to call Theralogix which is where I get Nicole's Vitamin D that her rheumatologist has her take because  the bottle of vitamins that they sent me in January expired in June 2013 so I want to be reimbursed for that.

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.

Sunday, January 26, 2014

Week in Review - 1/20/14

I have a goal to write more here this year.  I am going to start with a week in review which I hope to write every Sunday evening just talking about the week we had with Nicole's PH journey.

Monday started out with me going to Walmart, Michaels and the Dollar Tree.  I love the dollar tree because everything is a buck.... period.  I have certain things I like to get there.  I then went to Michaels as it is in the same plaza with the dollar store.  I was looking for some stamping supplies as well as a couple of items for Nicole's drawing class for the new semester.  I had no luck with finding what I wasn't able to find through Blink.  The university only puts out the supply list two weeks before classes start.  As of tonight, I still haven't found one pencil and have to order the other online and the spring semester starts tomorrow.  I then went to Walmart because Nicole needed 1 1/2 yards of black, white and gray fabric for her drawing class... curious to see what this is for.  I also picked up a couple other things.  I had a Publix coupon for Yoplait yogurt buy one and get one free and it was a specific kind.  The cashier said they don't take those coupons which according to their coupon policy that take all competitor coupons.  A lady came over, I'm not sure what she was, but she took one look and said it's a Publix coupon we don't take it.  I was like fine... I will give them my business instead of you which would have worked better if I had told him I didn't want the yogurt but Nicole was out of it and so I got it.  The guy behind me in line was shooting me the evil eye.   Had lunch at Wendy's which was nice as I love their asiago bacon chicken sandwich.

When Nicole changed her pump about 4:30 p.m. the silhouette which has the catheter attached was starting to lift off her skin and she had some sores underneath.  She use to get these all the time for several months and we tweaked a bunch of stuff like changing the barrier wipe brand and letting the area dry more etc.  She would need to change the site soon as she was on day 28.

On Tuesday, Nicole had an appointment with her psychiatrist.  We were in and out of their quite fast which I was happy about and I am happy that it is literally five minutes from our house as opposed to the 20 - 30 minutes when we have to go into Orlando.

Nicole had decided that she would change her site today.  I know she would have probably pushed it to Wednesday but she wanted to go to lunch with me on Friday so she opted to do it today.  About two hours after she changed her site she started having pain and before the evening was over she was taking her pain medication.

Nicole also sent an email to the pre-transplant coordinator asking for another letter from their center stating why they were not listing her for transplant.  I had done a rough draft of it and she made it her own.  She also said that letters sent to other transplant centers with her records had not been included in the records that we had picked up and she wanted them emailed or faxed to her.  At the time of writing this we haven't heard from anybody at the center.

On Wednesday, Nicole was in a lot of pain and I always try to help her as much as possible by getting her ice packs, getting her water, making her lunch etc.  I always make brownie's for her also which is one of her favorite treats.  It is painful for her to get up and walk as her infusion site is in her thigh.  We spent the day at home.  Monday, Wednesday and Fridays are the days that we are usually home and I can get a lot accomplished around the house if I don't let the computer etc. distract me.

This day marked the four year anniversary of Nicole's diagnosis.  Nicole didn't mention it at all but I know she didn't forget.  It is a very difficult day for all of us.  It is sad to say but it is getting harder and harder to remember "our normal life" prior to that.

Normally on Thursday Nicole would go to rehab at the hospital but after a site change she can't go for about a week to ten days.  I was spending a lot of time this week working on my speech I would be giving tonight at our PH support group meeting on  how I keep Nicole's medical records/issues organized.   I also had to get all the stuff together for the meeting.  I was taking a whole bunch of 1 " binders and spiral notebooks that I had accumulated, hopefully, for people to take home.  I was so grateful that a person who comes with one our members works at a school and took what was left behind to give out to the students that didn't have binders.  I am going to give her some more also as I have a ton of them. Nicole wasn't able to go to the meeting as she was in too much pain and was  more comfortable at home.

Friday started with me calling  Accredo, the speciality pharmacy to re-order two of Nicole's PH medication (her Remodulin is one of them)  this call always takes about 30 minutes.  I always call when I open the last vial of Remodulin and I was going to be doing that on this day.

I was getting Nicole up when she takes her morning meds at 10:15 for our lunch date with Kathy who was coming by at 11:00 a.m.  Nicole was going to tough it out and go because she wanted to see Kathy and she wanted to go to Olive Garden which is her favorite restaurant.

We had a wonderful lunch.  My father-in-law had given Nicole this cane called the hurry cane which has like a claw on the bottom to keep it from falling over and giving the person more stability.  Since Nicole's infusion site is in her thigh she will limp pretty good (until her pain is gone) and putting her weight on her leg makes it hurt.  She used the cane for the first time and did a wonderful job with it.

It breaks my heart because I know how much she hates it when she knows people are staring at her.  She is walking with a cane in her left hand and pulling her oxygen concentrator with her right.  I know she sees the looks of people.  I don't understand  why it is no big deal to see an older person walking with a cane on oxygen.  I guess because it isn't normal to see a young person or child like this people have to stare.   I have been very close a couple of times of asking somebody if they had something they wanted to ask about my daughter because they kept staring at her but I refrained myself so I wouldn't embarrass Nicole.

Nicole enjoyed her lunch even though she was in pain and had to take a pain pill while we were at the restaurant.  She didn't eat much of her lunch because she filled up on soup and breadsticks.  That's okay we came home with another meal of left overs :).

Weekends are not very structured.  I go shopping at Target and Publix and sometimes I will hit Staples as they are all together in the same plaza.  I always enjoy spending this time with my mother-in-law.  We have gone shopping on Saturday mornings since 1995 so she could spend some time with Nicole who came with me until she was about 12 years old or so.  I really don't do much housework on weekends either.

On Saturday nights I go out to Denny's (usually) with my bff, Margaret, and hang out for a couple hours.  We usually go to Aldi or the dollar store across the street from Denny's.  This is always something that I look forward to so much and is a great stress reliever.

Today is Sunday and I spend time planning out my week of what I want to accomplish as far as organizing, decluttering and personal accomplishments as well as what appointments and other commitments do we have this week.   Rick and I went to Home Depot to get some potting soil and I found a peppermint plant .... woohoo... I also went into Staples looking for the annual monthly/weekly calendar refill for the Arc planner but they didn't have it.  I did get a couple of the uni-ball pink signa gel pens... I love them and they are only 50 cents.

I made a great dinner of white chili and cornbread.  I filled Nicole's medications for the week in her daily pill boxes and I also filled her vitamins in her weekly pill box.  I was mad at myself that I didn't have enough Zyrtec for the week and I had thought I had another bottle.  I usually make a point to buy another bottle of something when I open the last one but it slipped through the cracks.  I will have to stop by Walgreens on Tuesday after Nicole's counseling appointment and get some Zyrtec.  I also am low on some of her vitamins which I will have to get either at Publix (if on sale) or Target.  I really would like to get a better system for keeping track of  refilling her medications and her vitamins.

I asked Nicole tonight at dinner if she was excited to be starting school tomorrow and she said yes.  I will have to call the local art store tomorrow and see if they have the items that I am looking for and if so, have Rick pick them up on his way home from work as he goes right past it.

I am hoping and praying for a good week that is sickness free for all of us but especially for Nicole.  Last night some kid was standing up in the booth and started coughing right behind me.  Obviously, his mother hasn't taught him to cover his mouth.   I worry so much and am so paranoid especially this time of year.

Wednesday, January 22, 2014

January 22, 2010

This post is part of the #Blog4Care blog carnival being hosted by Caring Across Generations. We're hoping that by sharing our caregiving stories, we can begin to come up with solutions to the care crisis that is affecting millions of Americans.

Four years ago today we took Nicole to a cardiologist due to low oxygen saturation levels.  I never, ever thought that we would hear that she had been born with a major heart defect.  We were told that she had a large (2.8 cm) hole (along with several other smaller ones) in the lower chamber of the heart this is called a ventricular septal defect (VSD).  She also had a patent foramen ovale (PFO) that is like a intermittent flap in the atrium (top part of the heart).  Sometimes it is also called an atrial septal defect (ASD).  Due to the lack of diagnosis of these two heart defects she developed Eisenmenger's syndrome (reverse shunting of the heart) and severe pulmonary hypertension.

I remember sitting in the office like it was yesterday after being told all of this.  It is like being kicked in the chest and you can't get any air.   We had believed that Nicole was healthy up to this point even though she was always sick, didn't gain weight, had anxiety issues, etc. etc.  It was also a blow to the future... everything you want for your child evaporated that day.  I don't think it was this initial visit but it was shortly after anyway that we were told she would never be able to get pregnant and have a child of her own.  She would also need at least a lung transplant with her heart issues being up in the air as to repair/transplant.

I remember coming home and googling pulmonary hypertension.... bad mistake... what I found literally scared the shit out of me (excuse my language) and I literally had nightmares.  It was six months before I would find PHA (Pulmonary Hypertension Association).

It would be a lie to say that this has been an easy journey.  For  the first two years at least I would wake up every morning thinking this was all a nightmare until I realized it wasn't a nightmare with the humming of the oxygen concentrator.  Now I just wake up and ask what day is it today and what do we have to do.

If you are a caregiver, you know the stress that comes with this.  If you are a caregiver and a parent to a chronically ill child that is also incurable  I think that adds a little bit more stress.  I have constant worry about Nicole.  If I go shopping etc. and leave her home alone I worry about her and pray that she is okay.  I worry about her future and that she will mentally, physically, and emotionally be able to come to terms with this as best as she can.  Every morning as soon as I get up I peak into her room and watch her to make sure she is still breathing.  I have tried very hard the past few months to not do this but even as I sit here writing this the urge to go check on her is so strong and it probably would cause me a lot less anxiety if I just went and checked on her.  The worry never, ever stops with this disease that can take a turn for the worse at any moment.  This time of year is worst because of flu season.  I was out having coffee with my friend the other night and one of the waiters went past our table and said hi and grabbed my hand and I immediately used hand sanitizer.  Even though Nicole and I both have had our flu shots if Nicole was to get the flu she would have an immediate pass to the hospital and quite frankly any type of respiratory infection could be deadly for her.  I pray everyday that she won't be  running a fever  etc.

I'm not sure if I will ever fully come to terms with this.  This isn't where I saw my life or Nicole's life four years ago.  I have come to terms with the fact that she was born with a congenital heart defect  as it is the number one  birth defect which, unfortunately, I didn't know in 1994.  I blame myself in a lot of ways because I didn't listen to my gut and my instinct and just "trusted" the doctors who all said she was perfectly healthy.  How do you come to terms with your daughter's whole life being a lie so to speak?

I have come a long way with the anger and negative emotions I have toward the pediatrician that Nicole had until she was four years old and the doctor who read her EKG wrong when she was six and even the pulmonologist that misdiagnosed her with asthma at seven years old.  Will I ever truly get passed it... I don't think so... I do the best that I can though because no matter how much I fight it and try to rationalize it the facts will not change.  Nicole fights everyday just to breathe and I find everyday for awareness of a disease that has taken so many lives that people have never heard of just like I had never heard of it.

Even though the last four years have been hard and this is just from my perspective and I know it is much, much harder for Nicole to actually have to live it.  There have been many, many blessings along the way.  We have met some awesome people and have made some awesome friends that we would never have had the chance to meet if it wasn't for Nicole's illness.  I truly believe that God gave Nicole her gift as an artist to compensate for her not being able to run and play and jump like other children. We have also had to learn along the way who are true friends really are because when you have a crisis of any kind people tend to scatter.

I have learned that I can hopefully use my big mouth for good and to help others with this disease or to raise awareness for pulmonary hypertension, for congenital heart defects and having newborns tested for them, and for the importance of organ donation  and to be an advocate for Nicole.

I will always speak out to raise awareness for PH as well as other rare diseases that don't get any recognition.  I will always, always be here for Nicole as long as there is breath in my body and I will always speak on her behalf and be an advocate for her.   I will always (or at least do my very, very best) to keep the faith and to have hope that one day there will be a cure for this disease and Nicole will be healthy.  I ultimately know that even though the doctor's hold her life in their hands in a lot of ways her fate is up to God and nobody else.  God has a great future planned for Nicole whether she struggles everyday with a debilitating lung disease or whether she is healthy.

I am so proud of the young lady Nicole has become and how she has dealt with these issues for the last four years.  She is an inspiration to me everyday and I have learned so many things from her.  She is the greatest love of my life.

My deepest wish (other than a cure) is for Nicole to realize just how special she is ... just how much courage she has... just how strong she is.  I wish that she wouldn't allow other people to tell her what they feel is  wrong with her and what she doesn't do right through their eyes.  She will never truly be happy until she starts living for herself and not through what others think of her.  I know as I struggle with this too... that this is a very hard lesson to learn.

Wednesday, January 1, 2014

My Three (Well, Maybe Five) Goals for 2014

Happy New Year!

On January 1st a lot of people make resolutions each year and very few actually follow through with them.  I stopped making resolutions years ago.

I do make goals though and do try to follow through on them.  My major goal for 2013 was to get my house decluttered (most specifically my living room which is the dump room as it isn't really used) decluttered.  I did not accomplish this goal :(  .  I am disappointed in myself and don't know how I couldn't get this achieved within twelve months.  I did make major strides toward this but I wasn't consistent enough.  I didn't realize how much stuff I have accumulated through procrastination and overspending.  I learned a lot through many different organizational experts.   I have to learn to be brutal when it comes to what I should keep and what I should let go off.

My goals for 2014 are only through March 1st at this point.  I will continue to make goals from March 1st - May 1st in the meantime.  I will only choose three things (or try) to focus on.  I think this was a problem last year because I had too many goals and they were not specific enough.

My first goal is to lose 10 lbs. before March 1st.  I did weigh myself this morning but I still need to do measurements (yuck....) .  The way I will achieve this is:

*  start exercising three days per week and try to be more active through housework  and not spend so much time at my desk unless I have a specific purpose other than reading emails and/or facebook.

*  watch my chocolate and sweet intake.  This I will need to accomplish by not bringing it into the house and leaving it at the store.  For instance, I have a love for Reese's peanut butter cups so I will buy a king size one at the checkout when I grocery shop and eat two on Saturday and two on Sunday.  This I am okay with I just can't bring in bags of candy.  I can still bring in sweets for Nicole and Rick but it will be those things that I have self-control over and I know what this stuff is.

*   I will also watch my portion control and only go by the serving size.  I have cut back a lot on bread (or I had until the holiday season) and want to get back into only having bread once or twice a week at the most.

* I also want to focus on drinking more water.

My second goal is getting my living room totally done by March 1st.  I broke this down into zones and what their  purpose is.  For instance, we have two wall bookcases and one is for books the other is for photo boxes, binders, cook books, journals etc.  (this is the purpose for it).

I also have the purpose for the two trunks I have in there and the craft area as well as the foyer off the living room.  The only area I didn't do was the fireplace mantel which I will need to find a purpose for but not sure what that is yet.   I guess it could just be clean except for some fake flowers or a plant.

The third goal is something I heard from Ellen Rogin about not making all your goals things to do (lose weight, declutter, jump out of an airplane etc.) but also on emotions so the first word that came to me when I heard her say this was peace so my goal to March 1st is to read at least one book on peace which I have already picked out by Joyce Meyer and write the scriptures on peace in the concordances of several different Bibles (amplied, NIV, KJV etc.).

The fourth goal ( I know I only said three) is to save enough money prior to March 1st to get a new iPhone.  I don't want a contract for my cell phone and Straight Talk is great and much cheaper.  Even though I only got the phone last year (2012) for Christmas it is a 3GS and came out in 2009 so I really do need to upgrade.   I also would like to save $200 in savings by March 1st.  I will also have a consistent habit of keeping track of my spending both for  our family and for Nicole (since I am her representative  payee of her SSI and am accountable for what she spends).  This will include consistently sticking to a budget....

My fifth goal stems from a free scholarship that I won for a Nutritional Healing Class along with a couple of other classes regarding naturopathic healing.  I want to complete this first class by consistently spending at least 30 minutes five days a week working on it.  I will need to schedule this into my calendar just like exercising and decluttering.

I know I should have only listed three goals but these all weigh heavy on my heart.  I would like to think that each one is realistic and I will be able to achieve them.

I know by recording my goals in a specific notebook (which also has a specific home) and writing this blog I will have these goals in front of me and will not forget them or misplace them.

I would also like to work on being more diligent with keeping up with Nicole's medical notes and paperwork and not let it pile up.  I will be happy to make a habit of filing all medical related paperwork at least one day every two weeks (which I will put in my tickler system so I don't forget and it can be planned).   I also want to consistently keep updated in Evernote with daily happenings with Nicole's medical issues such as phone calls, appointments etc.

I have so many things that I want to work on and improve and I want to do them all at once when I know I can't do this and it is unrealistic.