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/

May 6, 2010: Maren Is One!

March 22, 2010 Appointment

Maren saw her cardiologist on March 22. Her SpO2 oxygen reading was 90% and her chest x-rays did not show signs of congestive heart failure. This is fantastic news. Based on her stable condition and stellar physical condition and development, the doctor pushed back the potential MRI scan procedure and has scheduled Maren for another sedated echocardiogram on June 22, 2010. This is great news. Hopefully, it means no surgeries until September.

Every day that Maren can heal on her own, without the risks of surgical intervention, the better. In fact, the very latest studies on babies with conditions similar to Maren's suggest that "catch-up growth" of small right heart structures DOES HAPPEN. Although, this is debated in the medical community.

Incredibly, the entire May 2010 edition of Progress in Pediatric Cardiology, a journal that publishes approximately 4 issues a year, is devoted to PAIVS, and includes research that supports the existence of catch-up growth. In simple terms,this means that if a normal baby has a right ventricle size to left ventricle size ratio of 10:10, while Maren's started out at 5:10, it is possible, in time, for her to close that gap and have a ratio, of say, 8:10. This catch-up growth, is what we are praying for and, we hope, witnessing.

Next Appointment Update

Maren's next appointment was moved from February 22 to March 22, 2010. This appointment will be a check-up type appointment-- they will check oxygen levels via pulse oximetry, take lung x-rays to search for signs of congestive heart failure, and we will talk to the doctor about potentially scheduling an MRI of Maren's heart for a clearer picture of what the latest is. So, we wait...

Maren is 9 months old and is 19 pounds (big girl!). She is close to crawling; up on all fours and scooting around doing the army crawl. She babbles and plays and grabs things. She has been teething for the past couple of weeks and her bottom two teeth have popped through. OUCH!! Her favorite foods are avocado, banana, prunes and zucchini and has started to grab the spoon and try to get it in her mouth to feed herself. Maren's brothers Oliver and Ethan love to "take care of her" by bringing her toys and making her smile and laugh. They call her Mare-Bear.