A Glimpse Into Nicole' s Life

I will start this by giving a brief overview of Nicole’s life to this point.

Nicole was born five weeks premature and weighed 4 lbs. 13 oz. She had some delays due to this in milestones especially in speech.  One of the symptoms of CHD’s is failure to gain weight. Nicole has always been in the 10th percentile on the growth chart until a couple of years ago.  Another symptom is cynosis, which is blueness of the lips, skin, or nails. I don’t know how long Nicole has had purple fingernails but as long as I can remember. I wish that I had known this meant something.

When Nicole was four I switched pediatricians. Her former pediatrician and the County believed that she should be in a day care setting as her social skills as well as others were lacking. I didn’t agree with this and didn’t have peace about it but “they knew best”… right?

I put her in day care three days a week for half a day. She attended for less than two months and during that time had two bouts of strep throat and two bouts of pneumonia. During the second bout of pneumonia I had to take her to the ER with a temperature of 105.6. X-rays showed that she had double lung bacterial pneumonia. We also found out that she had a severe allergy to dust mites as well as some other allergies. She was in the hospital for five days. She never went back to day care.

When she started kindergarten she missed over thirty days during the school year due to illness. Around the age of six she was diagnosed with asthma and put on medication. One of the first slip ups were when she had an endoscopy for some bowel movement issues. She was put to sleep so they had her hooked to an EKG. The reader saw a question on it but the doctor didn’t seem concerned as he told us the EKG was normal. This was impossible.

Out of all the doctors she has seen in her life not one of them have ever questioned her purple fingernails. At 10 years old Nicole started exhibiting some obsessive tendencies with washing her hands. I took her to a counselor and psychiatrist and she was diagnosed with generalized anxiety disorder and obsessive compulsive disorder (OCD). This explained why she had such high anxiety about things and why she worried about things a child shouldn’t. She was put on Zoloft which she still takes. She also sees the same counselor.

On Jan. 23, 2010 when I took Nicole to the pediatrician for a check up the first thing I addressed was her purple fingernails. The doctor put a pulse oximeter on her finger (it clips on you finger to measure the amount of oxygen (O2) in your blood). A healthy person will have a reading of between 98 – 99% Nicole’s read 78%. The doctor thought his machine was broke so got another one and it read 82%.

The next day we were at the cardiologist and had the diagnosis of Ventricular Septal Defect (VSD) and Pulmonary Arterial Hypertension (PAH). I don’t remember much of this appointment. When Nicole had a heart catherization ten days later it was at this time that the cardiologist told us that heart surgery to close the hole wasn’t an option. If the hole was closed her lungs would cease to function. We were in the hospital for 36 hours to monitor Nicole’s blood pressure while they started her on Sildenafil which is Viagra. We came home with oxygen for the ride home and met the respiratory therapist at our house to get an oxygen concentrator set up for use. She would need to use oxygen while she was sleeping. The doctor believed it would be beneficial for her to use it “whenever she was home.”
The follow up appointment is when we realized how bad it really was. PH has no cure. The medicines will only slow the progression and not stop it. Nicole was then put on a second medication one month after starting the first. This drug requires that she have monthly bloodwork for liver testing as the medicine may cause liver damage. We were also told that Nicole will not be able to have children of her own because of her health it would be too much stress on her heart and lungs and because her medication causes serious birth defects. This was a hard pill to swallow. She also has a pregnancy test each month. She will be on these medications for life. The doctor also said “years down the road it is likely she will need a lung/heart transplant.”

Nicole takes three PH medications (in addition to Zoloft, allergy medicines and a handful of vitamins). She takes one pill 3x daily; one pill 1x daily; and an inhaled medication through a nebulizer 4x daily. There are a lot of side effects from the medications including coughing, nasal congestion, sore throat, nausea, dizziness, difficulty sleeping and the list goes on. They can also suppress the bone marrow (white blood cells) and the platelet count (red blood cells). The medications are very expensive costing without insurance approximately $25,000 per month. With our insurance the co-pay is about $200. Fortunately, we have assistance that pays our co-pays for us. Nicole also has to drink a bottle of Gatorade each day to combat dizziness.

Nicole’s lips will turn blue if she’s without oxygen for long periods of time. We have a portable unit that I take with us if we will be gone for more than a couple of hours in case she needs it. Nicole gets tired very easy just walking and standing for extended periods of time. One of our neighbors has loaned us a wheelchair but he could ask for it back anytime. I would like to be able to purchase her one in the near future. I am trying to save for this. I don’t know if our insurance would cover this or not.

All in all Nicole is blessed. I don’t believe that God would have given Nicole her gift as an artist if she had a healthy body. I am blessed to have such a beautiful, kind, compassionate, courageous and loyal daughter. I have learned much from her.

We are all so blessed as she is currently stable. I hope for more but I am grateful for the stability.

Congenital Heart Defect (CHD)

A congenital heart defect is a defect present at birth. One in every 100 – 150 children are born with a CHD. CHD’s are the most common birth defects in the United States. There are roughly 35 different types of CHD’s. There is no cure for any of them.

Ventricular Septal Defect, referred to as VSD, is the most common of the CHD’s with approximately 1 in 8 of children born with a CHD having this defect. Bascially, it is a hole that is between the right and left ventricles of the heart.

More children die of CHD’s than from all the childhood cancers combined. Only one penny of every dollar donated to the American Heart Association is used for CHD research. The Children’s Heart Foundation is the only organization strictly for CHD research.

Your heart has two sides, separated by an inner wall called the septum. Each side of your heart has an upper and lower chamber. The lower right chamber of your heart, the right ventricle, pumps blood to your pulmonary arteries. The blood than travels to your lungs where it picks up oxygen. The upper left chamber of your heart, the left atrium, receives the oxygen rich blood from your lungs. The blood is then pumped into the lower chamber of your heart, the left ventricle. From the left ventricle the blood is pumped to the rest of your body through an artery called the aorta.

In Nicole’s case she has a VSD. It is large about 2 1/2 cm at its widest point. It is clearly visible on the sonogram. I’ve been told she has two other smaller holes but I’m not sure where they are. In most cases, this heart defect should be detected within the first two years of life. Some holes, if small, will close on their own. For large holes, like Nicole’s, open heart surgery is required to close it. The major sign of this heart defect is a heart murmur which Nicole doesn’t have. I’m told it is because her hole is so large. There are other symptoms which looking back on she had most of them. If her first pediatrician hadn’t been such an idiot or didn’t think I was just being paranoid she would have had it diagnosed. There were many other slip ups and incidents when it should have been diagnosed but wasn’t. Only God knows why.

Eisenmenger’s Syndrome by definition is secondary to an uncorrected CHD that leads to pulmonary hypertension (PH), reversal of flow and cynosis (blueness). The previous left to right shunting is converted to right to left shunting which is the reversal of flow which is what causes the blueness

Pulmonary Hypertension (PH)

Pulmonary hypertension (PH) is increased pressure in the pulmonary arteries. The arteries carry blood from your heart to your lungs to pick up oxygen. PH isn’t a disease by itself, but rather a result of an underlying condition.

Nicole has a rare form of PH called pulmonary arterial hypertension or PAH. PAH affects about 140,000 people worldwide. I believe it is probably more than this. The National Organization of Rare Diseases (NORD) has classified PH as rare. The criteria I believe for this is under 250,000 but I’m not certain of this.

In PAH the pulmonary arteries will become too narrow. The process puts added pressure on the right side of the heart. Over time, this can cause the right side of the heart to become larger and the walls of the heart to thicken. In a normal healthy person your pulmonary pressure should be under 25 when the pressure becomes over 30 you are diagnosed with PH.

With less blood flowing into the lungs and less oxygen making it to the rest of the body symptoms will include dizziness, shortness of breath, persistent cough and fatigue. Other symptoms can include chest pain and swelling of the ankles and legs.

The disease can develop slowly so you can have it for years and not know it. This makes it hard for doctors to diagnose. Many people may go through years of testing to rule out other conditions before getting diagnosed. Misdiagnosis is also very common.

There’s two common classification systems for PH. One is to distinguish PAH from other types of PH and the other is to describe the severity of the disease.

The World Health Organization (WHO) divides PH up into five groups: PAH makes up the first group. This is the group that Nicole falls in. PAH which has no known cause is called primary or idiopathic PAH. PAH which has an underlying disease or cause is called associated PAH or APAH. PAH which is inherited is called familial or genetic PAH. Nicole has associated PAH as hers was caused by her congenital heart defect.
 
The New York Heart Association (NYHA) created a system to classify the diseases severity. This is used to monitor the disease progression.

Class I: no symptoms with physical activity.
Class II: some symptoms with ordinary activity and slight limitation of physical activity (example would be shortness of breath caused by two flights of stairs, walking uphill, walking fast, gardening)
Class III: symptoms with less than ordinary activity and increased limitation of physical activity (example would be shortness of breath caused by one flight of stairs, light cleaning, golf, power push lawn mower)
Class IV: symptoms with any activity, possibly even at rest (example would be shortness of breath caused by rest, dressing, showering, walking at home).

I know that they classify these as mild, moderate and severe but I don’t know how they divide them up. Nicole is somewhere between Class III and Class IV and it is considered severe.

When I spoke in January to our monthly homeschool group meeting about Nicole and her illness I had the ladies do an experiment to illustrate what it feels like for Nicole to breathe. Feel free to try this at home.

Get a regular drinking straw and put it in your mouth, plug your nose and breathe through the straw for a minute. This illustrates what it is like for Nicole to breathe normally. Now get a coffee stirrer and do the same thing. This illustrates what it is like for Nicole to breathe after physical activity.

What a Week ....

What a week it has been.... I know it could be worse, always could be worse.  The week started off frustrating.  We have really known for a while that we would have to file bankruptcy after my husband was laid off.  I have asked him and asked him to find an attorney.  He's not home all day and doesn't get the dozen calls everyday.  Finally, I just did it myself.  I told him about the attorney on Monday evening and he hemmed and hawed about it.  I asked when he wanted me to make an appointment.  He said "I don't know".  This and "I don't care" are the two most popular phrases in my house that I get from my husband and my daughter.  It is so frustrating. 

Finally, on Tuesday evening I asked him what time and what day and he told me.  I had done all the leg work all he had to do was show up for the appointment.  My daughter, who I love very much, same with my husband, has no motivation to do anything.  I know that she is very ill with a incurable illness that she's had to try and deal with the past year.  We all have but I feel that I have to be strong and carry everyone.  I just feel that she has so much potential that she's wasting.  All she wants to do is play on the computer and draw.  I'm not saying there's anything wrong with this but everything in moderation. I'm sure some of the issues are typical teenager.

I had to deal with doctor's offices because people don't know how to do their job.  I had to call the hospital because they don't know how to fax over lab results to the issuing doctor.  I wasn't happy with the results as her platelet count continued to drop and is now right above the critically low stage which is 100,000.  I want to know why her count keeps dropping.  I am told that her hemoglobin and red blood count is low also for her.  To look at the numbers she is borderline high but because she is cyanotic (blue) so her numbers should be much higher.  I was suppose to receive a call on Friday after the doctor researched her medications and such to see what to do.  I never received a call. 

I have waited over a week for a response from her ph specialist at Shand's hospital regarding  a couple of questions that her rheumatologist had regarding medicines for her joint pain.  I always seem to wait so long for a response from them.  I did get a response from her local cardiologist. 

Today was no better when I seemed to have lost my debit card.  I completely emptied my purse and all the contents and went through them several times.  I used it at the grocery store and shoved it back in my purse.  I called the grocery store and dollar store (didn't use it at the dollar store but thought maybe it fell out) but nobody had returned it.  I went to cancel it and was on the phone with customer service when my husband starting yelling at me about it. I had to tell the person I would have to call them back.  I told my husband that he didn't help the situation because he made me feel like I was stupid and worthless even though he said he didn't do it but I made myself feel this way.  I told him to do whatever he wanted about it I wasn't handling it.  All he could think/talk about was how HE was going to be inconvenienced. 

I have been very good about my online classes but won't be able to study until at least Thursday of this week which I can't go more than a day without studying or I can't  expect to get it done by the due date. 

I really do feel so much better when I write.  My computer is giving me so many problems because the track pad (laptop) which I don't use because I like a mouse is so sensitive it does weird things and I don't know enough about Window 7 to adjust the sensitivity. 

I feel invisible.  I feel that nobody offers to help me with anything whether it be my husband, my daughter, the rest of my family or friends.  I feel like I have to make every and all decision because my husband/daughter either can't or won't make any.  I feel so alone trapped in my own little box.  It is very difficult when you have financial burdens and also have a daughter who is chronically ill.

I will try and stay positive and hope next week is better. 

Week in Review

Well this is my first attempt of writing at least weekly.  We are out of school now so I must combat laziness and Nicole from being on the computer all day long !!!! 

Nicole had a rheumatologist appointment on Tuesday.  Her first appointment was in Feb. I really like the doctor but had problems with the nurse who I guess has been let go so lets see if I have better luck with getting paperwork and calls returned.  She addressed Nicole's  vitamin D deficiency which isn't at the critical state but still low.  She has her taking 4000 IU's of vitamin D daily for the next three months and then her vitamin D will be re-tested and see where we are at.  I guess vitamin D deficiency can cause joint pain so we aren't sure at this point if her pain is related to the Ehler's - Danlos syndrome or from vitamin D.  She also said she wanted Nicole to start walking on most days.  We start out just walking to the end of the street and then work our way up.  She would like to see her participate in cardiac or pulmonary rehab but she can't order this her cardiologist and/or pulmonary hypertension specialist must. She had blood taken but I'm not sure what for... should have asked.  I should have asked for a script because they charge $10 for it and she had to get her monthly blood work which we decided to wait a couple of days so she didn't get stuck twice in one day and she wasn't feeling well.    We will go back in three months. 

Rick had a cyst removed from the back of his neck so I have had too change the bandage and clean it etc.  yuck..... I would rather clean up puke :o) It looks like it is doing well and I don't see any redness or sign of infection.

I made a post yesterday that I had posted on Facebook on Wednesday I believe about me becoming a peer mentor for Tyvaso.  I had an interview which was 1 1/2 hours long on Friday evening.  I think it went pretty well.  They said I will be an official peer mentor when I complete the training and take and pass a quiz.  The training is being revised and they have to have approval of it before they can send me the link so I won't be able to get started for at least a few weeks.  They will also send me a welcome pack when the revisions to this are made also.  I'm really excited. I will have the chance to talk to people via email/phone about the medication and answer any questions they may have.  I will also have the opportunity to travel and speak at events at their expense.

On Friday, I took Nicole to the hospital for her bloodwork which is done for another month.  We will see if they took her CBC and her platelet count is low still or most importantly gotten any lower.

I believe that this is about all of my news from the week.  I know I live a very exciting life but it's mine and I love it.

Hugs:o)
Jane

A New Chapter in My Life!

 This is a post that I wrote on facebook on Wednesday of this week: 

My life and the life of my family changed forever on January 22, 2010 with Nicole's diagnosis.  Prior to this, I didn't know what my purpose was or what I was called to do.  I believe that my purpose is to be an advocate and voice for Nicole and for pulmonary hypertension and congenital heart defects.

One thing that I have learned in the last year is to wait to hear from God.  I am so proud of myself that I did this when I thought He spoke to my heart about training to be a pharmacy technician and He did provide for that. I know that this is His calling.

My commitments that I have had with NHE (homeschool group) are coming to a close and my involvement with the group will probably be minimal.  I have made some wonderful friends who have taught me so much.  I don't think that I would have been able to speak in Hawaii, do the video for Hawaii, speak at a NHE monthly meeting about Nicole's illness to the last speaking engagement I had on the radio a couple of weeks ago if it wasn't for the four years I was on the board at NHE.  I truly believe this has prepared me for the next chapter in my life.

Last year I met Colleen Brunelli through Generation Hope and she took the time out of her busy schedule to call me and give me encouragement and tell me about Tyvaso, which is one of the medications that Nicole takes for her PH.  It turns out that she was a peer mentor for Tyvaso and answers questions etc. for people starting the medication.  I was so impressed by this that I felt that this is something I would like to do.  One of the requirements was you had to be on the medication for six months.  Well, today it has been six months that Nicole has been on Tyvaso.  I contacted the peer mentor group on Tuesday to find out the procedure I had to go through and training etc. and talked to them yesterday.  They told me that I was in and I have an interview with them Friday evening.  I am SO excited.  If I can help others like Colleen helped me I am blessed.  When you have a child that has a chronic illness with no cure you feel helpless.  You have lost all control and you can't make them better or kiss them and make it go away.  I believe in my heart and with all my heart that my studying for a pharmacy technician degree and becoming a peer mentor will help Nicole first and for most and a lot of other people.  I hope to become more involved in PHAssociation also and I believe that this will happen but only if it's God's will.

To say that I'm not scared on this new path because it is scary for me, for Nicole and for my family but it is a road that I must take whether I feel like it or not.

A Recap

Wow... I didn't realize it's been so long since I have written on here.  I'm sorry especially if anyone actually comes here to read my ramblings. 

I enrolled at Penn Foster on May 9th in the pharmacy technician program.  I have one year to complete the program and then I will hopefully go on and become certified.  If I was ten years younger and was better in Math I may consider going on to become a pharmacist as it only takes 5 -6 years of school and lots of money :o)  I have been working on this mostly every day.  I have done my first two exams and have a 98 average. I was making tabs tonight to put into a binder and really looked at the study units and I really hope that I can maintain an "A" average.  I know that God is the one that put this into my heart.  I have no other explanation for it. I believe it was sometime in March around the beginning of the month I was sitting at my desk one day grading Nicole's quizzes and I had a feeling or something tell me that I should go back to school to become a pharmacy technician.  I thought that's a joke because I can't afford it no matter how much I wanted to do it.  I told God if this was His will then he would provide.  I asked for him to give me a sign and it was very specific and He did.  I checked out a pharmacology book out of the library, researched some online schools and found some you tube video's of pharmacology classes which I started watching.   I had joined a caregiving website back on March 17th  and I made a few posts and wrote a few blogs.  Around April 12th I received an email that I would no longer be required to do a job within the homeschool group which we belong to.  I would be lying if I said this didn't upset me as I really wanted to do it one more year anyway.  I knew that I would be coming off the board and I was happy about that because I have been on it for four years so I was just relieved of two commitments.  On the 15th I was contacted by the woman who owns the caregiving website and she asked me if I would be interested in being awarded their caregiver of the month.  This was such a huge surprise especially when I found out that I would receive $500.  One of the online schools was having offering a discount if you paid the full amount of tuition which was $498.  I don't think I would have chosen this school if I had my choice but I believe God did.  I am surprised that I have been finding the time to actually study.  Back in Dec. 2009 I really wanted to take a herbalism class in which I would get a certificate of completion.  My husband let me have the money and I purchased it. I had a problem with the shipment and it took longer and then within three weeks Nicole was diagnosed with her medical issues.  I did complete two of the lessons there are ten but I did notice that something would always come up every time I wanted to study.  I realize it wasn't God's will.  I hope to still do it but I just don't know when. 

Nicole has been doing well.  We finished school last week.  I can't believe that she is officially done with 10th grade.  We have just been hanging out at home a lot which I'm sure is good for her but she also should be getting out and doing things.

I am going to try and make sure that I at least write once a week and also I will make more of an effort to transfer things over from facebook.

Hope everyone has a great Memorial Day.

Hugs:o)
Jane