Tuesday, November 29, 2011

November is PH Awareness Month

Tomorrow is the last day of PH Awareness month. I have enjoyed reading all about the disease from fellow PH'ers on Facebook and have learned a lot. I want to talk about pulse oximeters. All it took for Nicole to be referred to a cardiologist was a pulse ox put on her finger. This isn't routinely done (except at the cardiologist and specialist) during a well visit check up or any other time except for in the hospital. The purpose of a pulse ox is to measure the levels of oxygen in the blood. A healthy person should have a reading around 98% or so.

Nicole's pulse ox reading usually falls between 85 - 89%. If she's at rehab and exercising with oxygen her readings have been known to be in the 70's. The respiratory therapist said that since Nicole's oxygen levels have been low for so long her body has compensated for that and she can function very well or at least to the world. If I were to have oxygen saturation levels this low I probably wouldn't be able to function as well.

There should be laws passed in every single state that every baby born has a pulse ox put on their finger at the time of birth. I know that some states have passed this type of law. I can' t help but wonder how our lives would have been different if Nicole had only had a pulse ox put on her finger when she was born.

Sunday, November 27, 2011

Been in a funk...

I've been in a funk this week.  I haven't been sleeping well which is something I usually don't have a problem with but for the last week I either can't fall asleep or can't stay asleep.  I'm not sure why this is. I'm thinking it is because I have altered my evening routine which is watching an hour of TV with Nicole usually until about 9:00 p.m. then I check facebook, email and plan for tomorrow.  After her last dose of medicine I clean the machine and get it ready to for tomorrow.  At this time I usually start water boiling for tea which I have while I read a book for about 20 minutes before I go to bed.  I stopped doing this and believe maybe this is one of the reasons. 

I have been a little depressed/sad because November around Thanksgiving is when my Grandma died and also a very good friend/neighbor of mine died.  I also think about (which I really shouldn't) whether this could be our last holiday together as a family.  I'm not looking forward to Christmas because it will be tight in the gift giving area which isn't the meaning of Christmas but still I feel bad as I won't be able to give like I will be given to.  I don't think that I have looked forward to Christmas since last year which was the year Nicole was diagnosed.

I have been very stressed out trying to switch over insurance coverage for Nicole and coming down to the wire with running out of a couple of her medications because the doctor's office didn't do their job properly.  I am seriously thinking of not returning to their office anymore but I don't want to negatively impact Nicole's treatment. 

I've been  a little upset with myself because I feel that God has put a call on my life and I don't feel that this is unfolding I guess the way I feel that it should.   I have felt pretty much invisible for the majority of this year.  I don't have friends call or come over like they use to and feel as though I am being swallowed up by Nicole's medical issues. 

I know the Bible tells us we are not to worry as it will not add one day to our lives and I really, really do try to do this but with Nicole being so ill, having no insurance for Rick and I, and our finances being so tight along with other issues I can't help but worry for the future for Nicole and Rick and I. 

I think what happens is when I get a lot of issues going on like I have this month with the insurance I get overwhelmed and then I get in a very negative pattern of thinking and I think that is what has happened.  I have to turn around and get the positive back.   I think some of it is the fact that I think more about myself than I do about what I can do for others. 

I look forward to climbing out of the funk. 

Monday, November 14, 2011

PHorum Speech

I was asked to speak at a dinner of PH patients, caregivers and nurses as my role as a caregiver.  This is the speech.  

Hi my name is Jane Northrop and I a PAH caregiver for my daughter Nicole who is in the audience with her dad/my husband Rick.  I am also a Peer Network Mentor.  The PEER Network is a nationwide group of people with PAH that are currently receiving or caring for someone taking Remodulin or Tyvaso and volunteers to share their experiences with others.

PEER Network is a free program sponsored by the same company sponsoring this event which is United Therapeutics.

There's a card in your packet and I would encourage you to visit the web site or call so you can gain real-life perspectives from other Mentor's like me and learn more about what it's really like to take these medicines and how it can fit into your daily routine.

I volunteer as a PEER mentor so I can help others like I have been helped.  To connect with others so they never feel as isolated as I did when Nicole was first diagnosed.  It was several months of trying to find information about PAH and others with the disease before I finally found PHA.  I can't change Nicole's illness so I combat the helplessness by being a PEER mentor  and being a voice for Nicole whether it is on Facebook, writing on my blog, writing on the caregiving site or speaking.

For us it can be a challenge some days to fit in the four treatments of Tyvaso.  I have to plan her treatments around whatever activities we have for the day which determines if we have to take it with us or not.  So far we have managed very well and rarely do we not get in the four treatments.

We live in Longwood which is between Orlando and Daytona Beach.  Rick works with his brother at an auto-repair shop.  I homeschool Nicole who is 17 and in 11th grade.  I am enrolled it the pharmacy technician program through Penn-Foster which is a distance learning school.  I enjoy cross-stitching, crocheting, reading and writing.  Nicole is a very talented artist and photographer.

I have noticed as far back as 2 years old that Nicole would struggle to breathe after exertion.  Her doctor told me everything was fine.  He would also imply that I was paranoid and over-protective.

Her fingernails have been purple ever since I could remember.  At 6 years old she was diagnosed with exercise induced asthma.  She also has severe allergies to dust mites which she took weekly shots for two-three years.

In 2009,  I started noticing that she was struggling more with exercise.  She didn't have the stamina to ride her bike and would stop and rest.  She would also put her hand on her heart.  Honestly, and I feel so bad for feeling this way, I thought she was being lazy and didn't want to exercise and/or being over dramatic. I wish that I had taken her more seriously.

On Jan. 21st, 2011 I took her for an annual check up.  I was going to get answers for her purple fingernails and her shortness of breath.  The first thing the doctor did was put a pulse ox on her finger which read 79.  He thought his machine was broke so he  got another one and it read 82.  I will never forget when he said "this isn't good."  He said he would set up an appointment for me with a cardiologist.  That afternoon I  got a call from the cardiologist.

On Jan. 22nd, 2011 we saw the cardiologist for an EKG and echo.  It was at this time that she was diagnosed with a large VSD (a hole between the right and left ventricle), Eisenmenger's and PAH.  I don't remember much of what the doctor said.  I remember thinking, okay, I wasn't crazy all these years.  I also thought we could "fix" everything.  I felt like I was kicked in the chest that day and that our whole world collapsed.  I felt this way again a few days later when I received the report from the doctor which said things in it that he didn't tell us at the appointment.  I knew in this moment that this wasn't going to be "fixable".  A word about pulse ox's.  All it took was a pulse ox reading to get her to a cardiologist but these aren't routinely used at a doctors appointment.

On Feb. 2nd, 2011 we had the right heart catherization which confirmed that she had severe PAH with pressures around 90-100.  She was kept overnight to monitor her blood pressure and started on Revatio.  We left St. Joseph's  in Tampa with O2 and an appointment with a respiratory therapist when we got home.

In August she was referred to the Heart and Lung Transplant Center at Shand's Hospital at the University of Florida in Gainesville.  The doctor decided to change her meds in hope of a better "quality of life".  I really dislike this statement because to me it is void of hope.  We would switch from Revatio to Adcirca for convenience, Tracleer to Letairis as he thought this would be more beneficial and Tyvaso as an alternative to the IV meds.

I think the first and most dramatic improvement I saw upon starting Tyvaso was the change to her breathing.  She didn't seem to breathe so hard and struggle so much.  Her stamina has improved also.  Overall, I am very please with the results of Tyvaso.

When I called the Adcirca program from their co-pay card to ask about Patient Assistance for cost of the medication I was told it wasn't offered for minor children because the drug wasn't approved for minors by the FDA and they didn't want the liability.

I had previously spoken with another Peer mentor, Colleen, who I was also facebook friends with as well as on an online support group through PHA.  I was so upset that I sent her an email venting my frustrations at both Adcirca and United Therapeutics.  She asked is she could forward it to her contact at UT.  I said "sure" and forgot about it.  A couple of weeks later I  received an email from a senior VP within UT.  My email to Colleen had landed on his desk.  He wanted to know what he could do to resolve the problem.  I had already decided that Nicole would stay on Revatio as I wasn't going to go through all the stress for a drug of convenience.  A phone conference was set up with the VP.  A week or so after my phone conference I got a call from Adcirca telling me that they had changed their policy and they would now offer assistance for minor children.  I asked if this applied to everyone or just to Nicole and she said everyone.

In January I received an email from another VP in UT asking if he could have a phone conference with me about my interaction with the other VP.  UT has an annual Global Sales Meeting where they invite a patient that has been helped in some way by UT.  They asked Nicole to be that patient.  They wanted us to surprise this VP by appearing at this meeting which was in Honolulu.  It was a wonderful experience and one my family with always remember.

 As a caregiver we have a different set of issues that the patient does.  We have to watch our loved one be sick and suffer side effects from medicines etc.  The loss of expectations for that person and their life.  Being on call 24/7.   Also the feelings of responsibility for their care and well-being and for me the feelings of guilt.

There's the loss of social activities.  I can't make the commitments that I use to because I don't know what Nicole's day will be like.  I have to tweek a lot of things every day just to work around how Nicole feels, I also don't have the time to do the things that I enjoy as much.

With my experience with UT and our medication issues I learned that one person can make a difference.  We must keep fighting for our caree.  We must keep hope and faith in a cure for PH and for the treatments that we have to fight the disease.  We must never give up.  Sometimes I feel like a meany or a pest when I call the doctor's so much that they know my voice and I don't even have to tell them who this is.  I will continue to fight for and be a voice for Nicole for as long as I am here to do so.  Do I get tired and want to give up?  Absolutely, some days are lousy and I feel that I just can't continue but each day is a new day and we can start all over again.

For caregivers we carry a lot of stress and the weight of the world is on my shoulders some days but for me I have to have faith and hope in God.

Earlier this year I entered an essay contest on facebook for a page called PHAmerica Honors that was created by Brenda Reynolds whose niece lost her battle with PH at 22 years old.  She remains a voice in the PH community in honor of her.  She with PHA had this contest to choose ambassadors to spread awareness for PH. There were six categories or so stemming from children, newly diagnosed, caregivers etc.  I was encouraged by a couple of facebook friends to enter so I did and my essay won in the caregiver category.  They also made Nicole honorary ambassador for her age group because they had no entries.  The judges felt a mother/daughter team would be cool.

 I will close with a quote from this essay " I must remain positive for no other reason that for Nicole. I will always be her voice and advocate no matter how old she is.  I will be with her every step of the way on this journey holding her hand.  Every day cherish the moments that you have and look for the good in them.  I wish more people (including myself) would  realize this before they or a loved one are faced with a serious illness."

Thursday, November 3, 2011

The story of Nicole's life

For Pulmonary Hypertension Awareness Month I thought that I would write the story of Nicole's life to raise awareness and maybe help someone else.  So here goes... warning.... this is long.

When I found out that I was pregnant with Nicole it was one of the happiest days of my life.  We had tried for seven long years with many tears on my part before I gave it over to God.

I had an uneventful pregnancy except for throwing up every day of it.  I had a sonogram at 12 weeks.  They couldn't tell the sex at that time but I always knew that it was a girl.   I wanted another sonogram but the doctor said that unless it was medically necessary the insurance company wouldn't pay for it.  I really, really felt I needed a sonogram and didn't have peace about it but squashed that.  I should have paid for it myself.

I woke up early on Sept. 23rd and was all wet. I thought I had peed my pants because I was too lazy to get out of bed.  I got cleaned up and went and laid down on the couch a little longer before I had to go to work.  I kept feeling wet though. When Rick woke up he made me call the doctor who told me to go to the hospital.  My water had started to break but I wasn't having any contractions.  When the doctor got there to do an exam is when my water "officially" broke.  They had to induce labor.  Nicole was born 5 weeks premature and while she only weighed 4 lbs. 13 oz., there was no indication that anything was wrong.

In my opinion, it seemed like Nicole was sick a lot and I was always at the doctor's office.  Nicole would always cry and scream when she saw the doctor.  Rick didn't like him either but I was okay with him.  As time went on, I started noticing that he didn't take my concerns seriously  as he thought I was overprotective and paranoid.

Nicole didn't start talking until she was 2 years old.  The doctor was concerned about this and her lack of social skills etc.  When she was three or so he had me take her to the county school district for evaluation.  They determined that she had a speech delay as well as some motor/social skill delays.  It was recommended that I put her in daycare.

Around the time of her fourth birthday I took her to the doctor because she was running a fever again.  When he came in the room Nicole, as always, started crying.  He said to her "what is your problem?  What are you crying for there are a lot of other kids much sicker than you are."  I left his office that day never to return again.

I did put her in daycare against my own gut.  The first faciility I put her in had a few incidents involving Nicole and I didn't like the way they handled it so I took her out.  She was only going a half a day three times a week.  Three days after she started daycare she got strep throat. A couple of weeks later she starting running a fever of close to 105.  She had pneumonia based on a chest x-ray.  She had to get shots in both legs and 10 days later had a follow-up x-ray which showed that the pneumonia was gone.  I told the doctor at this time she had been stuffed up for two days and he said it was probably a cold.  On Aug. 22nd in the middle of the night her fever spiked to 105.6.  I could only get it down to 103.  I called the doctor who told me to go to the ER and have them do a chest x-ray and he would meet us there.  I carried her into the ER.  They took her oxygen saturations level which were 80% which was attributed to her fever and whatever was causing it.  She had double lung bacterial pneumonia.  She was admitted to the hospital where she spent five days on oxygen and antibiotics.  It was at this time that the doctor suggested that I keep her out of daycare.  At this time they also found out that she had a severe allergy to dust mites.  This was something I tried to tell the first doctor but he kept blowing me off.  At six years old she was diagnosed with asthma because of the inflammation they kept seeing in her lungs via chest x-ray.

At seven years old we were seeing a gastroenterologist for constipation issues.  He ordered an endoscopy.  Prior to the procedure they did a CBC (complete blood count) which I don't recall them telling us they  did but I'm sure they told me.  During the procedure itself they did an EKG.  On the report of the endoscopy the technician questioned something.  On the actual EKG print out the cardiologist read it as normal.

At ten years old during the summer between 4th and 5th grade Nicole was attending a day camp at the local high school.  It was during this time that I noticed that she became obsessive with germs and using hand sanitizer.  I took her to a mental health counselor and psychiatrist.  She was diagnosed with generalized anxiety disorder and OCD (obsessive compulsive disorder).  Finally, a reason for the "melt-downs" that she had had over her life for sometimes little things.  She was put on Zoloft.

After she finished 5th grade I started homeschooling her.  She wasn't too happy about this at the beginning but before the year was up she loved it.  I continue to homeschool her to this day and she is now in 11th grade.

I guess it was in 2009 that I really started noticing her purple fingernails even though I believe she has had them for years (even though not one of her many doctors ever mentioned it).  I also noticed her getting colder faster and more often especially in the pool when her lips would turn blue I thought it was from coldness.    I also noticed that she was having a harder time doing physical activity then she usually did.

On January 22, 2010 she was diagnosed with a large VSD which is a congenital heart defect and is basically a hole between the right and left ventricle's of the heart.  She was also diagnosed with Pulmonary Arterial Hypertension which is a rare form of Pulmonary Hypertension.  This was all diagnosed by a cardiologist after the doctor took a pulse ox reading the day before and it read 80% (normal reading should be between 95 - 98%).  Imagine my shock and outrage to find out that she had a heart defect at birth and she was now 15 years old.

She immediately had a heart catherization to confirm the diagnosis.  She had to spend the night in the hospital to monitor vitals after being put on Revatio for her PH.  We were sent home with an overwhelming amount of information as well as oxygen which she would be on at night while she slept and whenever she was home.  We have had so many changes between the medications (she was put on two more PH drugs), oxygen and specialists and just the knowledge of what life will be like for us  as Nicole's parents and for Nicole as a 15 year old.

In Feb. 2011 she was diagnosed with Benign Hypermobility Joint Syndrome which may also be Ehler's - Danlos Syndrome which from the little research I have done is a rare genetic connective tissue disorder.  The only way to get a true EDS diagnosis is with genetic testing.  Unfortunately, this is something else she was born with.  All her life she has suffered from joint pain and I was always told it was "growing pains" but once I got her diagnosis I took her to a rheumatologist as I just don't believe doctor's very much these days.

Sometime after this the cardiologist requested Nicole's records from her former gastroenterologist as she would be seeing another one for continued stomach pain.    It was at this time we "officially" found out that the EKG at seven years old was in fact not normal nor would she ever have a normal EKG.  We also found out about the bloodwork being all over the place and that she had been anemic at that time.  None of this we were told.

Today, our life's are filled with doctor appointments, labwork, medications, oxygen, battles with insurance companies, social security, doctor offices for information etc.  All of these things stem from a diagnosis that we should have known about from birth if not in utero.

Even with the pressures and complications of our life's I love my daughter with all of my being and it doesn't matter whether she is healthy or ill.  I will go through all the tears and the heartache that I must go through just to keep her alive with us.