End Of Year Update

NEXT APPOINTMENT: May 2012, for a sedated echocardiogram and EKG.

In a matter of days 2011 will have passed, and we will look back on another amazing year for Maren Blue. She is a beautiful, smart and playful two and a half year old now, talking, laughing, running, jumping, and mimicking her older brothers as much as she can. Her echocardiogram in November showed that she is holding steady and there is no surgery in her immediate future. Her oxygen levels remain very good (in the low to mid nineties) and her valves are functioning adequately. She is still on track for a new pulmonary valve to be inserted at some point, but potentially not for many years. Further, due to the continuing advances in medicine, it is possible this valve could be inserted via a catheter surgery rather than open chest.

Merry Christmas and Happy New Year!

January 3, 2011: MRI Results

NEXT APPOINTMENT: August 2011! For a sedated echocardiogram and EKG.


On January 3, 2011, we headed down to Rady's Childrens Hospital so that Maren could see Dr. El Said and so we could conference regarding the results of the December 8, 2010 MRI. Although we had been told the results were very positive, we really wanted to talk directly with Dr. El Said about the numbers and details of the MRI, and find out what she envisioned as Maren's treatment plan going forward. As discussed below (skip to conclusion if you like) the bottom line is no surgery this year, and if Maren stays on her current super healthy healing path, she will require only catheter surgery one or two times in the foreseeable future.

MEDICAL DETAILS:

-Oxygen Levels: After getting Maren's height: 31.25 inches, weight: 10.2kg, they took a reading of Maren's oxygen and it was 94/95. This is pretty amazing, as she had been 90 just 6 months ago. (Normal is 97-99).

- Right Ventricle Size: Dr. El Said told us that the MRI showed that the ventricle has grown "significantly". Her determination of its growth is somewhat subjective because this was Maren's first MRI. Thus, there is no baseline MRI result for comparison. (Recall, the MRI is the only test that provides an accurate reading of the volume of the ventricle.) Dr. El Said's determination that the right ventricle has grown significantly is premised in large part on her piecing together all the information she has about Maren's heart including oxygen levels, her generally excellent health, as well as the MRI results. Equally important in assessing Maren's right ventricle is a direct comparison of the volume of the right ventricle with the volume of the left ventricle. Assuming the left ventricle is normal, which Maren's appears to be, the two ventricles should be about equal in size. In Maren's case, her right ventricle appears to be about 2/3 the size of her left ventricle. In sum, this is all excellent news according to the doctors.

- Leakage: Heart valves help to insure blood flows only one direction. A valve that is not working properly will have some leakage/regurgitation where a small amount of the blood flows backwards. The backward leakage in Maren's pulmonary valve and her tricuspid valve is rated +1. This rating is excellent for a girl like Maren. These are issues that could become pronounced over time, but right now, this is very close to normal, and is something the doctors will just continue to keep an eye on. In fact, the leakage at the pulmonary valve helps to increase the right ventricle size, and so has had a side benefit at this point.

-Right Outflow Tract/Pulmonary Flow: In addition to a small right ventricle, another concern has been stenosis of the pulmonary artery where the conduit of blood from the right ventricle through the pulmonary valve is potentially narrow and obstructed so that blood does not flow well. We learned that Maren has only minor issues with this right outflow tract obstruction. The gradient across her pulmonary was around 12mmHg, with normal being 6mmHg, where problems arise in the high 20s. Last October, when Maren had a catheter surgery to reballoon /open her pulmonary valve, this same gradient measurement was in the 30s. Along this line of reasoning and observation, Dr. El Said says Maren's pulmonary artery is not too narrow.

- Tricuspid Valve Z Score: Maren's tricuspid valve Z Score came in as a -0.08 today. This is great news, as tricuspid valve size is a very important indicator of the potential for a PAIVS baby like Maren to see ventricle growth. The tricuspid valve sits at the entrance to the right ventricle. Its performance is critical to getting proper blood flow through the right ventricle. Studies have shown a relationship between tricuspid Z scores and the recovery of PAIVS babies. Thus, many hospitals set up treatment plans based in large part on the tricuspid valve Z score. Most say that a Z score between 0 and -2 is mild, a Z score between -2 and -4 is moderate, and -4 and below is severe. So to see Maren's Z score close to 0 is pretty exciting.

- PDA Stent: According to the tests, the stent they inserted in May 2009 as a "cheat" connection is still open, with some flow. But the doctors seem to think this is not a concern, it will close naturally in time, and that Maren does not actually need the stent at all at this point.

-Atrial Septal Defect: Adding up all of the above information, the outlook for Maren was amazing today. The doctor told us that her immediate focus is on the hole in Maren's heart between the right and left atrium ( known as an atrial septal defect or "ASD"). This extra connection has never been even a blip on the radar in view of our legitimate concerns about right ventricle overhaul, valve replacements, 1.5 heart plans, etc. This ASD is apparently giving a slight right to left shunting of blood and ultimately they will want to close it via a catheter surgery. Our doctor thinks this could be done now, but she is reluctant to change anything, since Maren is doing so well.

CONCLUSION:

In sum, Dr. El Said said she did not need to see Maren for another year! Of course, we told her that was ridiculous, and convinced her we should come back in 8 months. Long term, if Maren can stay on her current track, we are looking at catheter surgery in the next year or two to close the hole in her heart (ASD), and at the same time close the PDA stent. After that, she said Maren will at some point need a pulmonary valve replacement, but the timeline for that procedure covers a very very broad spectrum. Thus, some patients may need a new valve as a teenager, some not until their 30s.

At the moment Maren is on the two ventricle heart plan with no open surgical intervention. Based on where she started, from the right ventricle being categorized as moderate to severe with a low Z score of -4 and a prognosis of a possible Fontan surgery, and now looking at her present plan of no surgery at all, well, this is all pretty amazing. I will have to check my stats, but I feel like the probablility of this recovery path was under 10 per cent. Of course it is all guess work. So we will see what comes next, but today sure seems like a good day.

December 8, 2010: Maren's MRI

Next Appointment: Maren's next appointment is January 3, 2011, to discuss the positive MRI results with her cardiologist.

Its been a long, long time since Maren last saw the cardiologists at Rady Children's Hospital. Over six months have passed, and Maren has blossomed from a wobbly little baby to a full-fledged toddler. She talks, she pouts, she plays, she sings, and she has a mind of her own. Every day is a blessing.

On December 8, 2010, Maren visited Rady's for a very important MRI of her heart. Incredibly, this is the first time for Maren to receive an MRI, which provides the most detailed picture of her heart available. In sum, the doctors must determine whether Maren's heart is best suited to a 1, 1.5 or 2 ventricle plan. The MRI is key to this assesment and the timing for any surgery. Recall, her current coronary circulation is dependent, in part, on a tiny stent that connects her red blood with her blue blood. Ultimately, this connection/stent will be closed - either naturally as tissue occludes the stent or by the affirmative actions of the doctors. Further, even if her heart performs well at the time this connection is closed, as she grows, it is likely she will need some type of surgery because, among other potential complications: (1) her right ventricle will still be too small to keep up with her growth; (2) she does not have a functioning pulmonary valve, which is needed to regulate the flow of blue blood from the heart to the lungs; and (3) she has a small hole in her heart that connects her right atrium with her left atrium. The result we hope for is that the only surgeries in her future will be for her pulmonary valve and for the small hole in her heart, and that these will be far down the road.

The MRI required Mare Bear, as we call her, to go under general anesthesia, so that she would stay perfectly still in the MRI tube. We arrived at 7 am. We cannot say enough about the incredible new surgical center at Rady's and the amazing staff. Although there were delays, which we have learned to expect in any hospital setting, the people and the facilities were wonderful. Of course, the actual MRI machine used for Maren is not with Rady, but is with neighboring Sharp Hospital. Through a partnership with Sharp, Rady and Sharp use the same machine. So we had to do some extra traveling, as Maren checked in with Rady, traveled over to Sharp, and once she completed the MRI, she was wheeled back through the hospital to Rady to be extubated and recovery.

So, check in took us until around 10:30 before the anesthesiologist was putting Maren under as we held her hand. She had gotten quite upset around 10:00 a.m. and had balled her little eyes out wondering why we were starving her. It turned out to be worthwhile, as the test came off without a hitch and by 2 pm, we were on our way home.

After the MRI, we did not get to see Maren's doctor. We did get to speak with another cardiologist in her group who said the results were very positive and we should wait to speak with Maren's cardiologist, Dr. El Said. We eventually got an appointment scheduled for January 3, 2011, at which we will talk in more detail about the MRI results. Also, the oxygen level in her blood is now up at least three points from last June, from 90-91, to a range of 93-94. Hopefully, we will find out what this all means in the near term in January.

Maren's June 28, 2010 Echocardiogram

Early Monday morning Maren went to Rady Children's Hospital for a sedated echocardiogram and really strutted her stuff. Doctor El Said was amazed (once again) by Maren and her progress. Incredibly, Maren's next appointment won't be until December 2010, when she will have an MRI (originally going to be scheduled for last December 2009). After we see the results of the MRI, Dr. El Said will conference with all the cardiac surgeons and cardiologists at Rady to determine the best surgery/treatment plan for Maren. This is quite extraordinary.

Now for some of the more detailed results from the echo and Maren's appointment yesterday (for those who are interested):

Maren's oxygen saturations were solid at 89 while she was sedated, and would fluctuate up to 90 and 91 when she was awake. Normal heart function results in an oxygen level of 97-99. Maren's is lower due to her condition, but in particular, she has a small hole in her heart called an atrial septal defect (ASD) which connects the right atrium with the left atrium. Via this hole, blue blood (unoxygenated blood) in the right atrium mixes into the left atrium, where red blood (oxygenated blood) is queuing up to be pumped out to the body. This mixing lowers the oxygen content and results in a lower number in Maren's saturation level.

There are some benefits to Maren having the ASD. The hole actually prevents strain to the small right ventricle by providing a small release valve. In time, however, the ASD could be closed in a catheter surgery or as part of whatever open heart surgery Maren may have in the future. The bottom line is that based on the progress Maren has been making, the benefits of closing the hole at this time do not justify the burdens imposed by a surgical procedure.

Maren's tricuspid valve Z score is between -.13 and -.85. This is great news, as the tricuspid valve, which is the valve at the entrance to the right atrium, is one part of the right heart structures that can be measured with reasonable accuracy via echocardiogram. This measurement is one thing that is used to determine whether a PAIVS baby will be able to achieve a 2 ventricle, 1.5 ventricle, or 1 ventricle repair, with the 2 ventricle repair being the best of these options. Basically, although different hospitals/doctors around the country take different approaches, in all hospitals, a Z score of 0 through -2, along with a reasonable ventricle size (next paragraph), suggest 2 ventricle repair.

Maren's right ventricle is rated by her doctor as mild to moderately hypertrophic. This is an eyeball rating from the echocardiogram, and is among the most subjective of the assessments. The MRI will ultimately give a much better picture of the ventricle. This is a very important assessment as far as prospects for 2 ventricle repair. With her tricuspid size and her right ventricle healing, Maren has pretty clearly put herself into 2 ventricle repair territory. This was NOT the case just nine months ago, when she was in the category of babies where there is significant controversy as to what is the best treatment (1.5 or 2 ventricle repair).

Maren's pulmonary artery was not narrowing too much.

Maren's stent looks to have narrowed a little bit, but is basically still open and providing additional blood to her lungs. She also had an EKG which did not show anything unusual.

So, we are optimistic about this latest news. But mostly, we are just excited by the chance of six more months without surgery! In the interim, we have to keep an eye out for a drop in Maren's oxygen levels, which, literally, we have been advised we can detect by, among other things, her turning blue! This never ceases to amaze us, as we have never seen this happen and cannot imagine how scary it would be. Still, without fail, the doctors and nurses ask if Maren has blue lips sometimes, and a blue appearance, as if it is the most normal thing in the world. This is something we hope never to see...

The hope is to wait years until any surgery, and for there to be only one open surgery-- when Maren is a small girl, to replace Maren's pulmonary valve. But that is looking way down the road. For now, we look forward to having the best six months imaginable with our Maren Blue.

June 22, 2010 Appointment Delayed

NEXT APPOINTMENT: June 28, 2010 7am, Sedated Echocardiogram

Due to a scheduling error by the hospital, today's appointment was moved to June 28, 2010. This was a little disappointing as it is hard not to be a little anxious to find out what Maren's oxygen saturation levels are, and for her to have the sedated echocardiogram. This procedure will, among other things, give the doctor a picture of the size of her right ventricle (from which to guesstimate); give a measurement of her tricuspid valve; tell us if she is hypertensive (a narrowing) in her pulmonary artery; take a look at her "PDA Stent" to see if it is partially occluded; and look at her pulmonary valve to see if it is obstructed (again); and finally, in general, give some idea on how the blood is flowing through her heart. It has been since December that we had such critical data-- But of course, Maren LOOKS amazing and IS quite amazing!!

June 8, 2010: 1 Year Check-Up

Maren visited her pediatrician for her 1 year check-up on June 8, 2010, and they confirmed what we see: Maren is making her milestones.

30 inches long, 65th percentile in length
40th percentile in weight (not as fat as some have suggested apparently!)
75th percentile in head size (big ol’ brain)
She passed her hearing test.

No signs of heart failure, (note, they don’t test for oxygen levels or take chest xrays, but the doctor does feel her organs and feet primarily.)

while every day is another real time, real test, we go back to see Dr. El Said at Rady's on June 22 for a sedated echocardiogram which will be our first picture of Maren's heart for some time.

June 5, 2010: Miracle Makers Gala

Jessica and I were lucky to attend a black tie benefit for the Rady's Children's Hospital at a table purchased by my law firm. The event was sold out, and was attended by nearly 1,000 people. The goal of the gala was to raise money for the new Acute Care Pavilion, which will be home to the new Peckham Center for Cancer and Blood Disorders, the state-of-the-art Warren Family Surgical Center, and a Neonatal Intensive Care Unit that is truly family-centered. This is the surgical center and NICU which housed us after Maren's birth and was the site of Maren's surgeries, and the will be again in the future. Recall, the catheter lab at Rady's, which is outfitted to enable catheter procedures on babies like Maren draws patients from Hawaii, Riverside and San Diego. And yet, with rapid advances in medicine, it still needs updates, as components of the technology (Xray arms and machines used during surgery, monitors,...) are outdated.

You can learn more about Rady's Foundation: http://www.helpsdkids.org/