My Emotional Journey

I started this blog to communicate and document the physical and emotional journey of this experience. Admittedly, I've done a better job communicating the physical aspect of my journey than the emotional side.

Why you ask? I need CONTROLLED JOURNALING! Not because I'd go on and on but rather because I don't know where to begin. This whole experience feels as if I'm living another person's life. Don't be mistaken, I do have my breakdowns. Actually, I decided a long time ago that I would not let my illness define my spirit. Given that, I avoid spending energy on myself as a patient or being sick, and instead, I focus on getting healthy. Also, I'd rather enjoy life being Jenn, the person that I know the best.

In the recent "Pulmonary Hypertension Association" Fall 2008 Newsletter I discovered an article on journalling. A study was done in 1994 "that showed those who wrote about stressful experiences for 20 minutes a day, three days a week, fared better than those who didn't and better than those who wrote on neutral subjects". In addition, the article provided questions to assist with starting the journaling process. THANK GOD!

With the help of this article and getting over a fear of opening a can of worms, I have begin journaling and will be posting these entries frequently.

TO DO Surgery OR NOT TO DO Surgery_UTSW Visit_November 10th, 2008

Great news!

1. Pressures are now in an operable range. Since May, Pulmonary Vascular Resistant (PVR) pressures dropped from 10 to 8.3 PVR. The opererable range is between 6-8 PVR. Therefore, I am in the upper range of that threshold.


2. Signs of shunting from right to left are now non-existant. An indicator that the Pulmonary Pressures have gone down. The Atrial Septal Defect (ASD), which is the hole in the upper chambers of my heart had been providing relief from these high pressures.

After the Right-Heart Catheterization, we meet with Dr.'s; Torres (Pulmonologist), Warner (Cardiologist) and Ring (Cardio Thoracic Surgeon) to get individual opinions on my progress. All are enthusiastic with my progress but collectively they agree that continuting my (medication and oxygen) therapy, for an additional 3-months, is beneficial in determining if PVR and Pulmonary pressures can continue to decrease or if they've plateu . There would be a short -term and long -term benefit from reducing the pressures. Not only would the surgery be less risky but the lower I can reduce my pressures prior to surgery may affect the degree of PH I will be left with from a longterm perspective. The Dr.'s reiterate, "there is no exact science " to conclude whether or not I will be left with PH, after surgery. As a result, I will continue the therapy, trust that pressures go down and maintain the confidence that a life WITHOUT Oxygen, Flolan and (even better) PH are around the corner.

So, in three-months (February 08), we will be headed back to UTSW for yet another Right-Heart Catheterization. With the support of my Dr.'s, I will go ahead and have surgery during that same visit, if pressures continue to decrease. Yeah!!! The Visit is schedule for the week of February 16th, 2009. It'll be a HAPPY NEW YEAR!

Activity: I have been riding my bike 1-2 miles, once or twice a week. I have also been back to yoga, also once or twice a week.

Medications: No change to medications (Flolan, Tracleer, Lasix, Warfarin/Coumadine and Oxygen)

PH Cartoons

These cartoons may be difficult to relate to if you are not a PH patient, but they also you give you a good sense of daily mental and physical obstacles for PH patients.

http://www.sobtoons.com/

Start of Flolan Medication_July 29, 2008

The Goal: 1) Surgery to insert Central Line IV 2). Begin Flolan medication.

Now that surgery is a possibility we have a new goal in sight; get my Pulmonary Vascular Resistance (PVR) pressures down.

As I mentioned, my PVR pressures are at 10 and they need to go down to the 6-8 range for surgery. Flolan is the Dr.'s drug of choice to assist with getting my pressures down. It is not only the oldest PH drug but also the most potent.

The first time Dr. Torres familiarized me with all the PH therapies was in Jan. 2008, prior to the Congenital Heart Defect diagnosis. As a PH patient, being put on Flolan is a good indicator that other therapies are not effective. In addition, Flolan often times is a long term and in even lifetime solution. Therefore, at that time I feared being put on this drug. I don't think I need to explain further, just look at the above pictures of me and the Flolan pump to see what's involved. Because there is an opportunity to fix the defect with surgery, and even cure the PH caused by the defect, I remain hopeful that the Flolan therapy is a short term solution.

Process: Arrive in Dallas on Saturday, July 26th. The usual crew; Chris and my parents. We spend Sunday and Monday training with Steven, the Accredo Pharmaceutical Nurse. He familiarizes us with mixing the drug and managing the pump. The processes and responsibilities involved with this drug is overwhelming. Not following the processes can result in; an accidental *bolus or the opposite, due to the drugs *half life of 3-6 minutes.

On Tuesday, July 29th I get the catheter inserted and start on Flolan. I am in the ICU for 2 days being closely monitored. This is protocol for anyone who starts this medication. After the first day Dr. Torres gives me the option for discharge. I think he felt confident in my abilities to manage the Flolan but also felt comfortable releasing me to my mother, the CNP. Either way, I wasn't ready, the thought of managing this drug was a little scary. We agreed that I'd stay another day and continue to increase my dosing.

I recall talking to my mom about the above events, prior to the visit. I mentioned that the stay in the ICU would be "restful" after a central line insertion and starting the Flolan. She responded with "The hospital is not a place to rest". My interpretation, although also true, if a patient is well enough to go home they are discharged.....it is not a hotel. What I did not anticipate, was that I would be interrupted from sleep every couple of hours to check vitals, change ice packs for medication, increase dosing, etc. This is in addition to the leads/wires, oxygen and IV connected and hanging from my body. All I can say is I slept very well in my hotel room that night after being released from the hospital. The entire trip was about 7 days; 2 days traveling; 2days training; and 3 days in the hospital.

Since July 29th, I have been increasing my dose every three days. I am now very comfortable mixing my medication and changing my dressing at the catheter site. As of October 10th, I have reached the goal for my dosing (30 ng/kg/min). I will remain at this dose until November 10th, my next visit to Dallas. During this visit, I will have yet another hearth catheterization to check my pressures. Keep your fingers crossed for me.

Additions to Daily/Weekly Activities Include; 1) Mixing medication on a daily basis 2). Carrying along back-up supplies and pump wherever I go. Not only has it added to my physical activities it has added to my mental activities. I have to remember that I have and IV coming out of my chest with about a 5 pound weight at the end of it. I frequently get up from the couch or bed and start walking away and then will feel a tug, reminding me of the extra luggage. Also, the added accessory of a fanny pack is not one that I favor. What can you do?

Medications: Oxygen (I am now able to be off the oxygen for periods of the day when I am relaxing and not doing a whole lot of activity), Bosantan, Warfarin, Flolan

Flolan side effects; jaw pain, which is similar to putting a sour candy in my mouth. This occurs every time I put something in my mouth; flushing, which looks like I have a sun burn on my face. In August, I developed a bad rash around my catheter sight. It was so uncomfortable that I just wanted to scratch my skin off. I became so obsessed that the rash spread throughout my chest and Chris had to initiate an intervention....a sarcasm intervention. Luckily sarcasm goes a long way in our house. That rash lasted about 3-4 weeks and is now completely gone.

New Phrases: Chris asks me now "How's it Flowing" Meaning, how's the O2 and Flolan flowing.

*Bolus is to administer a "large" dose of IV medication. A bolus of Flolan is highly discouraged within the medical community because it is a potent drug. Because patients mix and manage there own medication there is a window of opportunity for this to happen and also a common occurrence. Loosing consciousness, vomiting are just a couple of negative effects when a bolus occurs.

*The half-life of a drug is how long it takes for half of it to be eliminated from the bloodstream. For Flolan if I am not receiving the medication for 3-6 minutes I would have a similar reaction to a bolus.

Congetive Heart Defect - May 08

The Visit: As part of the Compass III research, I would repeat the following tests; MRI, Echocardiogram and a Right Heart Catheterization. These tests would determine whether Dr. Torres would add the second drug (Sildenefil) to my therapy. I was eager for this visit. Since being diagnosed with PH and having been on Bosantan for 4 months now, I was feeling good and hopeful that I would feel even better if an additional medication was added to my therapy.

The Goal: To add Sildenefil to my therapy.

The Process: After completing the MRI and echocardiogram I still awaited the right heart catheterization. Dr. Warner would perform the catheterization. He would measure my pulmonary pressures as required by the Compass III but also expressed interest in doing further exploring. He performed a right and left catheterization. During the procedure the Dr. Warner indicated that he had new information about my diagnosis but I did not understand the details of the findings. The procedure would be a little longer than intended but that was fine with me as this was very positive news.

The Outcome: Even better outcome than we had anticipated . There was now a cause for the Pulmonary Hypertension. A congenital heart defect. Because I no longer had Primary Pulmonary Arterial Hypertension but rather Secondary Pulmonary Hypertension, there was now hope to cure the Pulmonary Hypertension. It would be (3-4) weeks before we'd find out if surgery, to fix the defect, was a possibility. Dr. Torres would present my case to a team of Dr.'s and collectively they would determine my future. As Chris said at that time (and continues to say) we were "cautiously optimistic" that the Dr.'s would consider performing surgery to correct the defect and hopefully by correcting the defect this would also cure the PH. After the month long wait we received confirmation that surgery was an option.

Because of the change in diagnosis I would no longer be part of the Compass III research. I no longer was considered a PRIMARY Pulmonary Hypertensionon patient.

With surgery on the horizon came other therapies and goals. I would begin Flolan, a continuos IV medication, to reduce my Pulmonary Vascular Resistant (PVR) pressures. Reason being, my PVR's were at 10 and the Surgeon preferred to do surgery when those pressures were in between the 6-8 range. Therefore, it was to dangerous to perform surgery now.


New Diagnosis: Congenital Heart Defect; Pulmonary Vein Anomaly with an Atrial Septal Defect.

First off, if you are like me and are an obsessive GOOGLE R don't expect to find a lot of information about a Pulmonary Vein Anomaly. This is a rare condition in Adults. I have found a little bit of information about the Anomaly but not specific to my condition. My mother has found one article and she's a CNP, who has access to this data.

Anatomy 1

1. External carotid artery and internal jugular vein 2. Dotted line indicates location of anterior lung (not shown) 3. Right pulmonary veins 4. Right pulmonary arteries 5. Right bronchi 6. Internal carotid artery and deep jugular vein 7. Trachea 8. Thyroid gland 9. Left lung, superior lobe 10. Superior vena cava (cut) 11. Ascending aorta (cut) 12. Pulmonary artery (cut) 13. Right lung, inferior lobe 14. Diaphragm 15. Inferior vena cava16. Descending aorta 17. Thymus gland

What is Pulmonary Hypertension

It causes the pressure in the pulmonary artery to rise far above normal levels. At the same time as the pressure rises, the walls of the blood vessels become thicker. This puts intense strain on the heart. Pulmonary hypertension can occur with or without an identifiable or known cause.

Diagnosis of primary pulmonary hypertension (PPH) typically requires several tests. It is often not detected in a routine clinical examination because many of the PPH symptoms are similar to other disease conditions of the heart and lung, such as shortness of breath, dizziness, weakness, swelling of the ankles and extremities, etc.

Pulmonary hypertension is characterized by elevated pressure in the artery connecting the heart and lung (pulmonary artery). The average normal pressure in this artery is 18 mm Hg, while for a patient with PPH, the average pressure is 25 mm Hg. In order to measure the pulmonary artery pressure directly, a heart catheterization is needed.


Primary Versus Secondary Pulmonary Hypertension
When a patient has elevated pulmonary pressure, it does not necessarily indicate a diagnosis of primary pulmonary hypertension. The elevated pressure can also be directly related to heart or lung disease. When pulmonary hypertension is caused by other factors, the disease is called secondary pulmonary hypertension. In order to specifically diagnose PPH, the doctor must rule out other causes of the elevated pulmonary pressure. Once these have been eliminated as causes, and no other direct cause can be determined, then the diagnosis is primary pulmonary hypertension. Other tests that are typically used to rule out other causes of elevated pulmonary hypertension include standard pulmonary tests, X-rays, Doppler Flow studies and ventilation-perfusion testing.

Diet Drugs and PPH
Use of diet drugs, such as Fen Phen, fenfluramine (Pondimin) and dexfenfluramine (Redux) have been known to cause primary pulmonary hypertension. These patients typically present with a defective heart valve. These heart valve defects can be detected with echocardiography, which is a non-invasive ultrasound examination of the heart. Patients with this known heart defect and primary pulmonary hypertension should be screened for previous treatment with these diet drugs. Some studies have suggested that diet drug patients have normal pulmonary artery pressure during rest, but abnormal levels during exercise. An exercise test during catheterization (described above) can be used to diagnose PPH for these patients.

Initial Diagnosis: Pulmonary Arterial Hypertension

In January 2008, at the age of 30 years old I was diagnosed with Pulmonary Arterial Hypertension.

This was a shock, as I had danced all of my life and even 6 months prior to my diagnosis I was still teaching ballet classes. Now looking back, over the last 5 years there had been a progressive decline in my ability to teach, due to a lack of stamina, so I thought.

The symptoms that brought me into the emergency room on January 5, 2008 included; shortness of breath, chest pain, back pain and a tremendous amount of anxiety. At one point, I had pain down the left side of my arm and was concerned that I may be having a heart attack.

I was admitted to the hospital and was there for about 4 days. The Pulmonologist (Dr. Dorf)who saw me in the hospital diagnosed me with Pulmonary Arterial Hypertension (PAH), which is high blood pressure of the lungs, the cause, unknown. He insisted that I continue my treatment at a PH Clinic and even scheduled the appointment himself. I am very fortunate that Dr. Dorf initiated this referral. There are many patients who do not get diagnosed or are diagnosed but do not receive proper treatment. It is important that a patient who has symptoms of PH be referred to a specialty clinic. UT Southwest is the Pulmonary Hypertension Clinic where I would be receiving my care. Less than a week after my release, and after an initial diagnosis from Dr. Dorf we (my boyfriend Chris, Mom and Dad) headed to UT Southwest in Dallas, Texas.

The goal of the trip to UT Southwest; 1) Confirm the Pulmonary Hypertension Diagnosis 2) Determine whether my condition was Primary or Secondary and 3) Determine if I was a research candidate. This process took about a week and required multiple tests, blood work,etc.

The outcome at UT SW Medical Center; confirmation that I had Primary Pulmonary Hypertension, with a Patent Foramen Ovale and yes, I would be a candidate for research. As a participant in the "Compass III" study I would be closely examined; with regular blood work, tests and visits to UT to monitor my progress. In addition, I would be receiving the medication at no charge. Overall, this was a great study to participate in. The drugs that were being tested were already approved by the FDA, and dosing regimens would be no different. The research was to test the combination of the two drugs; Bosantan and Sildenafil. Both are oral medications. I would began the Bosantan and then down the road they would decide whether they would add the second medication. This would be decided at my 3rd visit to UT in May.

We were all eager for Friday to come as that would be the day we'd be meeting again with Dr. Torres, who would have all the results from my tests and a plan for my therapy. We had compiled a list of questions over a two week period, but only one (question) that we were all apprehensive about bringing up. The life expectancy question. The answer we got from Torres, I could either live a semi-normal life or experience a rapid decline in health in a couple of years which would result in needing a lung/heart transplant. I was overcome with shock. I felt like I was watching a Lifetime Movie, emotional about the situation but yet did not feel like it was happening to me. Yes, I'm one of those Lifetime Network junkies, who enjoys a good cry every once in a while. But, I was not prepared to be living my own emotional drama. The fact that I am surrounded by a lot of love- a supportive and loving boyfriend, his family, my family and friends - helps with not feeling so alone in this situation as the grief and fear is hard among us all.

Two weeks after my diagnosis I returned to work, a new job. With the help of friends it was possible for me to make a smooth transition into a new job, one that would be less stressful and just as important, a job I truly enjoyed.

Medications: Bosantan, Coumadine, Lasix and Oxygen.

Modifications to Lifestyle: Having recently been diagnosed with a progressive disease, every symptom from being tired to a slight headache was cause for concern. Therefore, with every ache or discomfort was a phone call to my mom, who is a Certified Nurse Practitioner (CNP). Without her expertise, I would have not only spent a lot more time but also had more uncalled-for visits to the emergency room.

The need for oxygen 24/7,is a huge adjustment. I must plan out my day, ensuring I have enough oxygen when leaving the house. In addition, making sure I do not miss my a dose of medications is difficult. I had three medications; 2 of which I took twice of day and 1 once a day.

My energy level overall is good and the level of physical activity is not more than daily with some stretching. I have days when I maintain my energy level and there are days that I am exhausted and must rest for 3-4 hours.

Diet: Because I am on coumadine (blood thinner) I must limit my intake of vitamin k and have a regular diet. At age 11 I would have been very happy to have this as an excuse to turn "the greens" away, unfortunately, at age 30 I have become a fan of the veggies'. Because my pulmonary pressures are high - causing the heart to work harder - limiting the intake of caffeine and alcohol is recommended. This is an adjustment, as I indulge in both, regularly.

All and all, I feel lucky that I was very active growing up. Because of this, my overall health is good, allowing me to live a fairly normal life. Frankly, the mental challenge has been more difficult than the physical challenge.