Upbeat Update!- June 11, 2009

It has been more than 3-months since surgery and I feel great. A couple of scares since my last post, all of which occured in the Month of March.

The first, a car accident. I did not have any injuries , but as a cautionary measure, I went to the hospital to make sure all was fine.

A couple of weeks later my central line fell out of my chest. If you recall, the Flolan medication is a very potent drug and immediate discontinuation of the drug - for even a few minutes - can result in adverse effects; shortness of breath and chest pain are just a few. I am happy to say, I was off the medication for about 1.5 hours and did not feel any differently. The situation was scary; emergency room visit and surgery to replace the catheter. The upsides; emergency room and surgery were accomplished that same day and this incident did not cause any major set-backs to my recovery; soarness from the surgery and keeping a close eye on the catheter site were the worst of this experience, from a health standpoint. There was a financial set-back, more bills!

I had my 3-month follow-up visit on May 7th. The visit to Dallas included; an MRI, echocardiagram, bloodwork and Dr.'s visit. My pulmonary pressures are back down to the 50's range, which is exciting because last year around this time they were up in the 80's. I have been instructed to discontinue the coumadine and lasix medications, Yeah Again! And the best news of all, I have started decreasing/titrating down on my Flolan. So far, I have succesfully gone down 10 ng in one-month. Yeah! Yeah! Yeah!!!!

Since my May 7th visit, my ALT and AST levels went from doubling the normal levels to 5 times the normal range, which means my liver is "injured". While the most likely cause is the Tracleer medication-which can cause liver dysfunction-the generous amounts of libations I enjoyed during the month of May is also a factor for the dramatic increase. I have been asked to discontinue the Tracleer, while my liver functions are closely monitored with weekly (CMP) bloodwork, but also think it's wise to limit the alcohol.

You all may be wondering why all the indulging in May? Please see my next post....... COMING SOON!!

1-Month After Surgery

Hi All!


First off, THANK YOU! THANK YOU! THANK YOU! .......the love that surrounds me brings tears to my eyes and warms my healing heart.

I was moved by the notes and messages you all included in the scrapbook, co-edited by Autumn and Olivia, thanks Ladies! Also, the Quilt my Mom made using the squares, made by family and friends, gave me great comfort while I was in the hospital.

I want to say a special thanks to; My Family and Chris's Family, especially The Ortiz Clan in Dallas,who for the past year has; opened there home to us, fed us and more. Also, to our friends who traveled to Dallas for the surgery; Autumn, Casandra, Chip and Olivia, the support you provided to not only me and Chris but also our families was appreciated by us all. Lastly, I want to recognize My Love, Chris, who did such an amazing job keeping you all informed of my progress. I too kept up with the blog to better understand the details of the surgery and post-op. That period, for me, was non-existent to foggy to nauseating - all in that order - therefore I required a recap of prior events. And, I have to say, after both reading and hearing about the details, THANK GOODNESS FOR ANESTHESIA AND PAIN MEDICATION!


Because it has been more than two weeks since our last update I have a lot to cover:

Concerning the Pleural Effusion: On Monday 3/2/09 Dr. Torres removed the fluid from my lungs. There was about 400-500 ml of fluid. I couldn't resist, above is a picture of me with the fluid removed minutes before. There was a difference, not so much in my breathing right away, but some pain relief on the right side of my back. The X-ray done on the 4th showed much improvement and therefore Dr. Torres gave me the go ahead to fly home later that day. Eager to get home my mom and I were able to catch an earlier flight.

Overall Progress: As of yesterday, it has been 4 weeks since surgery and I feel I've made a lot of progress. Since last Thursday I have been off the oxygen while doing minimal exercise; light chores around the house, and errands. I am using it only while sleeping and for my walking workouts. Last Saturday, I completed about 1 mile of walking, with the assistance of the O2 and encouragement from Chris. As for the Flolan medication, I am still on it. But, I am hopeful that over the next few months I will no longer need the Flolan.

The Incision site and sternum: I have mild discomfort and little to no pain. I have increased range of motion of the upper body and can now move a little more comfortable while sleeping. The scar is healing nicely, I'm very pleased with the amount of work that was put in to sewing me back up. I see a bikini in my future!

As of today, I am one more step towards independence. My sister, Tara, arrived from Singapore on the same day I got back from Dallas. She has been helping me these last two weeks and left this morning. Because I don't see my sister very often, it was nice that she came during this time of recovery, the period in which I could remember. Daily exercise and activity included; walking the mall and eating 3 square meals a day, meaning anything we felt like eating. No guilty conscious from either party because we were both on a mission to gain some weight...imagine that. Also, as of yesterday, I am allowed to start driving again. Above, Tara and I are celebrating 1-Month Surgery Anniversary and St. Patricks Day and YES! these are sober smiles!!! Can't say the same for everyone else around us.

I go back to Dallas for a follow-up appointment on March 26th.

The Road to Recovery

Hello everyone. Thought we'd share an update....

Jennifer's exercise levels are low compared to pre-surgery levels. More oxygen is required for rest and activity, 3-6 liters versus 1-3 liters.

Flolan (intravenous medication) remains unchanged. Getting outside at least once we day, we’re able to stave off cabin fever. We are now a great source for obscure movie reviews.

With surgery 12 days ago, Jenn's recovery rate is being monitored closely. On Wednesday the 25th we met the specialty clinic team for a scheduled appointment.

She is believed to have pleural effusion, which is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs.

The fluid is a combination of serum, pus and or blood. The surgeon, Dr. Ring, speculates blood resulting from surgery, which would also explain the fluid’s immobility throughout the cavity.

An excessive amount of such fluid impairs breathing by limiting the expansion of the lungs during inhalation. From x-rays, 500-1,000 ml is estimated in the right lung with less in the left lung.

There are 2 options:

1. Often done as outpatient care, Thoracentesis is an invasive procedure to remove fluid. A cannula, or hollow needle, is introduced into the thorax to draw the unwanted pleural fluid out.
2. Surgery, which also requires a mechanical "scraping" to remove. Surgery would require a hospital stay plus another incision.

If the fluid becomes hardened, it can be difficult for the body to absorb it, and or for it to be removed through use of a syringe.

Jennifer's INR level, a measurement of blood thickness, is 3.5. In order to operate, we must thicken the blood by stopping use of Coumadin and increasing intake of vitamin K.

Coumadin is a blood thinner and a medication in Jennifer's routine to limit the risk of blood clots.

In the meantime, Jennifer is doubling her Lasix. Lasix is an oral medication to aid the body in removing fluid, and also a part of Jennifer's normal routine.

Jennifer is also increasing potassium levels, to contradict the extra fluid loss from doubling Lasix. She is eating more regularly, up throughout most of the day, and still smiling.

Our next appointments are on Monday to check INR levels, evaluate fluid levels, and make decisions.

Jennifer and her Mother, Celina were planning to depart by plane to Albuquerque on Wednesday.

Her father, George, will drive the vehicle back starting Tuesday, after dropping me off at DFW for a trip to Chicago.

If option 1 is chosen, travel plans should be uninterrupted. If option 2 is chosen, there will be a re-evaluation as a hospital stay is accommodated.

There are 2 WINS we're marking in the books. First, Jennifer's x-ray films have shown improvement in fluid levels from Monday's exam. That's before doubling of the Lasix.

Second, Jennifer's incentive spirometer results have increased from a high water mark of 1,000 milliliters to over 1,500 milliliters. Left lung inflation has improved.

I must admit, I did not anticipate this additional challenge. It’s a good reminder of what Yogi Berra told us long ago, "It ain't over 'til it's over."

I continue to admire how Jennifer takes in this experience, including its challenges. It was tough information to take, even as it helped us understand current health difficulties.

But Jennifer is strong and will overcome. We're learning how deep love can run.

@ our Dallas Home

Jennifer is ecstatic to be out of the hospital. Despite appreciation for the special attention, she's happy to be away from the poking, prodding and sleepless nights. Jennifer received in-patient care until late into the afternoon on Saturday, February 21st.

We were told to expect a 3-7 day stay in the hospital after surgery. Jennifer was in for 5. We’ve been in Dallas for a little over a week, since Sunday February 15th. The remainder of our stay in Dallas will be in a rental house we found on www.homeaway.com.

As with many caring for Jennifer, the anesthesiologist took a special interest, having felt an immediate connection. Young and female, she made sure extra care and attention was given to the suture.

A comparatively small scar will result, ensured by the entire surgical team from incision to stitch. Jennifer’s scar will be a badge of honor, and serve to remind us of her strength throughout the process.

We are focused day-by-day on rehabilitation. Jennifer’s days are filled with rest, light eating, and a variety of light exercises. Nights are filled with intermittent sleep, early hour pain medication, and the occasional bloody nose.

Shortness of breath persists, even more so than before surgery. The culprit, at least partially, is atelectasis. A common postoperative complication, atelectasis is a collapse of lung tissue. It is a condition where the alveoli are deflated. Alveoli are "spherical outcroppings of the respiratory bronchioles and are the primary sites of gas exchange with the blood."

Jennifer’s oxygen saturation levels are below pre-surgery levels at rest and with exertion. More oxygen is required to maintain minimum desired level of saturation, 90%. What took 2-3 liters at elevation in Albuquerque is now taking 4-6 liters in Dallas.

At least for the near future, Jennifer is expected to maintain use of assisted oxygen and the intravenous medication Flolan. As we move along, levels of each will be lowered and Jennifer's reaction gauged. We are unsure what outcome can be achieved. While some Pulmonary Hypertension will always exist, we are hopeful we can eliminate the use of assisted oxygen and replace the use of Flolan with an oral medication.

Focusing on the present, Jennifer’s immediate goal is to complete breathing exercises every 2 hours, and to take increasingly longer walks every 4 hours. The respiratory exercises are done using an incentive spirometer. Anyone who’s suffered from a lung or heart ailment will remember this device, likely with disdain. But combined with very light physical exertion, its use is critically important. Walking consists of a few laps around the living room.

It is difficult to interpret what is attributable to surgical recovery and what our new “baseline” is. We continue to monitor Jennifer’s health closely, with pending follow-up appointments scheduled over the next week.

A good resource to plan for life after open heart surgery is named the AHA / ACC 2008 Guidelines of Adults with Congenital Heart Disease http://circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.108.190690.

As health care has advanced, a larger community of adults with congenital heart disease exists. Despite operative success, the condition should be monitored throughout life. With the importance often under-estimated, these guidelines are a great place to start.

Each day brings greater regularity. Small but important steps are being made towards realizing the benefits of surgery.

Big News from Dallas: Open Heart Surgery

Open heart surgery was a major success! Jennifer's heart defects have been fully repaired.

The pulmonary vein anomaly, defined as a "congenital heart defect," existed since birth. The atrial septal defect, the hole between the right and left atrium chambers in Jennifer's heart, resulted from high pressures caused by the pulmonary hypertension.

The current belief is the pulmonary hypertension has been "neutralized." Meaning, what exists will remain but we're hopeful it will get no worse moving forward. We will learn more as we move along over the next few weeks.

The pulmonary vein anomaly, once thought to include only 1 of 3 veins on the right side of Jennifer's heart, was more extensive. All 3 veins needed to be "baffled" so oxygenated, "blue" blood could flow properly to the left side of the heart for distribution throughout the vascular system.

The surgeon, Dr. Ring, was prepared to address this additional complexity. Dr. Torres recommended Dr. Ring for this very purpose, knowing he would be poised and ready to handle complications in stride.

Improvements have been recorded for most of the vital pressure measurements, post-operation. Jennifer has achieved her goal, set so many months ago, of a successful surgical outcome.

Jennifer initially recovered in the ICU until Thursday night, at which point she was transferred around 10 pm CST to a "step down" unit in the Telemetry section of the hospital.

As of Friday morning, coinciding with the writing of this post, the plan and goal of medical staff is to discharge Jennifer later today or Saturday.

We are working through a few complications, which are not expected to persist. Jennifer is feeling nauseous, tired, and is in some pain, but all things considered we could not have scripted a better outcome.

There's a long road to recovery, which we can now focus on. The total rehabilitation period can last up to 3 months, with the first phase 6-8 weeks.

http://www.webmd.com/heart-disease/guide/heart-disease-recovering-after-heart-surgery gives some sense of what recovery will entail, as it talks about standard rehabilitation for overcoming heart surgery.

We have a new goal, which we're calling "back to basics." This means establishing a routine, to include things such as normal and deep breathing, eating a regularly, healthy diet, walking and other exercise, copious rest, etc.

This morning Jennifer had a bagel with cream cheese, banana, and cranberry juice. She's also taking 3 walks a day. In fact, Jennifer purportedly set a new speed record for walking the perimeter of the ICU. So we're on track.

A testament to the power of the human will, this experience has reinforced how blessed we feel to be surrounded by such a powerful support network.

Friends, family, colleagues and others have come to our aid, helping in any way possible to ease the burden Jennifer and her caregivers bear. When the load's been greatest, the support's been strongest.

The surgical team, physicians, nurses and others have all taken a special interest in Jennifer. It's clear her attitude and youth have won over their hearts, giving us a special level of care and oversight.

We hardly have a minute where someone's not coming by and checking on her health, making sure she's comfortable and disciplined about her recovery.

While some of you have heard this, it's worth repeating. Not all battles are won, and this war is far from over. But in the immortal words of Mark Twain "it's not the size of the dog in the fight, it's the size of the fight in the dog." Or in this case, the beautiful young woman.

NEWS FROM DALLAS: Will there be OPEN HEART SURGERY?

A right heart catheterization was completed at 12 pm CST Monday, 16 Feb 2009 by Dr. John Warner http://www.utsouthwestern.edu/findfac/professional/0,,17742,00.html.

Good news! Decision rendered by Dr. Ring, surgeon http://www.utsouthwestern.edu/findfac/professional/0,,16089,00.html , is that open heart surgery is a go for Tuesday 17 Feb 2009.

3 primary indicators are all positive from the right heart catheterization, which is an invasive procedure to closely evaluate the pressures in the heart. A catheter was routed through Jennifer's femoral vein (in her right leg) into her right atrium, right ventrical, and pulmonary artery.

1). The first, pulmonary vascular resistance (PVR), has improved from 8.1 three months ago to 6.7. Measured in dynes, PVR is a term used to define the resistance to flow that must be overcome from the vasculature of the lungs.

2). Secondly, pulmonary artery pressure, a measurement of blood pressure in the pulmonary artery, has improved from 64 initially, to the 50s, and now resides in the 30s.

3). Third, the degree of "left-to-right" shunting. Jennifer has a hole in her heart, defined specifically as an atrial septal defect (ASD). The shunting can be defined by the volume of blood that flows through it for a period of time. In Jennifer's case, the volume has decreased from 4 liters to 2.5, representative of half the total that should be pumped from her heart to vascular system.

While the shunting is not ideal, it has both gone down and is an indicator of what could result during the recovery period. Meaning, significant progress quickly is possible after the surgery, given how much the shunt is being utilized.

Surgery is scheduled for 7 am. Jennifer will be wheeled down to the Operating Room (OR) at 6 am for preparations.

During the 3-4 hours, Jennifer's heart will be momentarily stopped. She will remain stable through the use of a heart-lung machine. During that time, a simultaneous repair will be made to close the hole in her heart and "baffle" her pulmonary vein.

One of Jennifer's pulmonary veins, typically architected to deliver oxygenated blood from the lungs to the left side of the heart, is currently "recycling" blood back into the right side of the heart. This creates a "loop within a loop."

The surgery will redesign this vein to deliver oxygenated blood, alongside the other pulmonary veins, to the left side of the heart.

Friends and family remain positive, highly optimistic, and confident. Despite the seriousness of the situation, we are all managing to share a few laughs.

Surgery Update_February 2009

As you know, we will be headed back to UTSW in a few weeks. Here's the plan; I will have a catheterization on February 16th, which will determine if pressures have improved, stabilized or increased. If pressures have either improved or stabalized then it is my understanding surgery will be a go ahead the following day (February 17th). If they have increased, we will have to re-evaluate plan.

I have spent the last three months preparing for surgery, as I am thinking and acting optimistically. Family and close friends will be meeting us in Dallas, for support. If surgery is not a go ahead I imagine I may need more emotional support than if surgery does happen, therefore, I feel very fortunate to have such gracious family and friends.

In the event I do have surgery, we will be staying at a rental home for about 2 1/2 weeks and then return to New Mexico. During this time of recovery I will have somebody keep my blog up to date so you all can keep up with my progress.

I have received such support this year; the prayers, thoughts and emails have given me such strength. Please continue praying, thinking of me and I'm not opposed to the good ole' keeping your fingers crossed for me, and my family, over the next few weeks.

Activity Update:

I'm happy to report that I am working out 2- 3 times per week. Workout includes a combination of the following; yoga 2 times per week, Cardio 2-3 times per weeks, and light weights. Unfortunetly, I recently discovered that I was doing too much and needed to be better about watching my saturation (O2) levels, as they were dropping below the 90 O2 level. So, I've cutback on the intensity of the work-out and increased my liter flow while working out. As I've mentioned to a few of you, I'm probably the happiest person in the gym. Go figure, imagine this, the girl with the O2 and other accessories hanging from her body is ALL SMILES!!! I'm so happy to be working out again.

Medications:

No changes to medications. BUMMER!

Some Personal Thoughts_Part I

When do I think my illness first took up occupancy inside me?

Given I have a congenital heart defect, it has always been with me.
As for, when do I think the PH started to occupy me, well, I think it was hiding for a while as far back as college (14 years ago), that I can remember. As a dancer who trained 3-5 hours (per day) I lacked stamina, which I always didn't understand. I really noticed a change when I moved back to NM in 2000. From that time on shortness of breath and anxiety worsened.

Who else is involved?
Those I am in physical contact with on a day to day or regularly basis; Chris, Co-workers, Parents, Friends, Relatives and of course my (2) kitty cats.

How does my health effect these relationships?
Not able to take a brisk walk to grab coffee with co-workers. Although, happy to say brisk walks are more feasible now that I am on Flolan.

Planning or rather having to change or modify social functions so that they are within my physical capabilities. Activities that I enjoy but are unable to do (Right now!); hiking, hot tubing, skiing, and dancing with my Girlfriends.

Other people whom are affected by my health:

Acquintances. I'd like to think I have remained positive throughout this experience. Therefore, I hope I have touched people in a positive way.

What are the worst things about it?
Calling it, IT. No really! PH is not something you can refer to as a pronoun or ignore. It has a name, Pulmonary Hypertension. PH takes on a life of its own, even though it lives within you. Speaking from only my personal experience ONLY, PH is mostly physically pain free. Instead, it is what I’ll compare to as a “Prison”. A prison because although I have the physical components; the legs and the emotional drive; desire, your body dominates and takes over those two motivating factors. PH has a weird way of discouraging me, because I WAN’T to walk up that hill but my body is communicating that I CAN’T walk up that hill. For me that is the hardest part. Hikes or even short walks became something I dreaded. I found it difficult even before starting a walk I felt anxious, nervous and even discouraged. I thought it was my lack of motivation, desire and drive for activity. Keep in mind I was still teaching ballet up until 6 months prior to my diagnosis. I had always been very active dancing 5-6 days a week so I couldn’t understand why I could be in such bad shape and lacked desire to do anything other than dancing. Even while teaching I could not demonstrate like I had.

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)