It has been a long time since we last updated Gracie’s website. We apologize for the extended hiatus, but as is often the case life seems to fly by at such a rapid pace that before we realize we have even blinked a year has elapsed in an instant. Much has transpired in our lives in the last year and a half. It has been the craziest swell of emotions, heartache, and joy. The love from all of those who surround our precious Grace has helped her reach her 18th month of life as a happy and vibrant spirit. She melts our hearts each and every day. Her vocabulary is astounding for her age and she is already counting to ten. As far as her medical updates are concerned I will break down her overall picture as it stands.

-We are happy to say that as of right now Grace’s kidneys are still functioning at a normal level. Normal kidney function should have a creatinine level of .3 or .4 and Grace is right in there with her last blood draw yielding a .4 reading.

- In July Grace, Nicole, and I went back East to the NIH in Bethesda, Maryland to be part of a study on ARPKD. The research Doctor’s studied Grace from head to toe, performing a variety of tests which included: a brain scan, kidney and liver ultra sounds, blood draws, an eye exam, nutritional analysis, as well as a few other tests that I cannot recall at the moment. It was a whirlwind of tests and consultations. The best news to come out of the testing came from the head kidney researcher, Dr. Genay who has studied ARPKD probably as much as anyone in the country. In her expert, medical opinion, given Grace’s current state of health, she believes Grace is not likely to encounter her kidney’s going into failure until her teenage years or possibly even into early adulthood. If this holds true, which there are no guarantees ever with the human body, but if she does stay on the course predicted by Dr. Genay this is fantastic news. When she was six months old we were simply hoping and praying her kidneys would last long enough to get her to her first birthday and the twenty pound benchmark so her body could handle a transplant and avoid dialysis, and now we are given news that she might not have to endure this battle for many years down the road. Time is an amazing ally in her case. It gives her time to grow, time to become stronger, time for her body to be more prepared, and also time for the medical field to reach new discoveries pertaining to her disease. Ten to twenty

years can make a world of difference. A medicine to control the disease, a cure for ARPKD, the perfection of creating artificial kidneys, or the ongoing stem cell research to regenerate a healthy kidney are all tangible possibilities in the future.

-Grace is now down to three medications. Enalipril for her blood pressure, Atenolol as a beta blocker to assist the blood pressure medication, and Prilosec for reflux. A very small number compared to the ten medications she was sent home on over a year ago.

-The one obstacle that seems to be a constant for Grace is feeding. Though she is in a decent percentile for weight and height she continues to struggle with food. She still gags and chokes often when eating solids and is pretty much only consuming a hyper-allergenic formula, water, oatmeal, and watermelon. She has been diagnosed with allergies to eggs, dairy, and nuts, but there is concern that she might be allergic to many more foods as well. Her GI doctor is going to perform and endoscopy in November to check for a condition called EE. The basics of EE are not being able to break down proteins in various foods, thus causing upset stomach, gagging, and eventual allergy to the foods. We are hoping this is not the case and that she is merely a picky eater, but enough evidence exists to investigate it further.

-Thank you for taking the time to read this and caring for our little miracle. May this note find you well and smiling upon the world.

Nicasio, Nicole, and Grace

We have been so fortunate through a family friend of a friend to be in contact with the head Urologist(kidney surgeon) and head Nephrologist (kidney doctor/pediatrician) at the L.A. Children’s hospital who have a combined nearly eighty years of experience.  I contacted them on Friday and told them of our situation.  After hearing me out they made special arrangements to see Grace and her records(ultra-sounds, radiology) because they felt as we did, that our current doctors could possibly be  acting a bit ahead of pace.  After Grace won their hearts with plenty of smiles to the generous men they proceeded to tell us that they would not touch Grace right now, meaning that surgery would be the last thing on their minds.  They said she is thriving.  She registers in the 50 percentile (perfectly in line) on her growth chart and she has no complications breathing.  These would be the two big alarms if her kidneys were too big, no growth in height and weight, and trouble with her breathing.  Grace shows no sign of either.

Grace’s kidneys have grown, but the L.A. doctors expected as much, she has ARPKD.  They do not seem to think that they will keep growing at such a rate, and even if they do they do not believe there is any sign that this growth will effect her current stat of being.  They even went as far as to say that if the kidneys did eventually get too large and they were compromising Grace’s condition that they would try to remove only one of the kidneys to give her some relief and see how she responded before they removed both and put her on dialysis.   This news gave us such a sense of relief.

Our celebration of this news is not in any way to disparage the San Diego division or to do finger pointing of any kind, we only want what is best for Grace, but it is mind boggling to listen to the opposite opinions from both places.  We went from requests from doctors to schedule surgeries in about three weeks to doctors who do not even feel they need to see us until late August for an ultra-sound on the kidneys to to see how they are progressing.  It is not a matter of like or loyalty, because everyone at San Diego has been fantastic, sometimes it simply comes down to experience and a comfort level in cases like Grace’s.  There was no rush, no panic, no worry at all really, to push anything forward.  They want, as we do, to keep Grace with her native kidneys for as long as we can, because even her diseased kidneys are a world better than going on dialysis.

The fact remains that there still is no cure for ARPKD.  This did not change and Grace will still need new kidneys at some point in her life, but the doctors in L.A. brought us back to our original hope that this still might be a ways off.  We are not blinding ourselves and we know things can alter as time goes on, but every month of natural life is another month closer to getting her to an appropriate size and age to accept her transplant without having to be on extended dialysis.  We love you all and keep you with us always.  Thanks for listening and hope we can see or hear from you soon.

Love,
Grace, Nicole, Nicasio  

Our results have come in a mixed bag.  We met with our cardiologist about an hour after her heart ultrasound and he came in with good news.  Grace’s heart is almost all the way back to normal.  As numbers go she is just about where she should be, but the echo still showed slight enlargement so she will remain on her heart medications for a while longer.  Our cardiologist did say that by our next visit with him we should be able to take her off her heart medications completely.

We got to leave Thursday night with this great news and had every reason to believe the kidney ultrasound would yield steady results, as her kidney function is still at a normal level, but as it goes with this disease, things have not been very predictable.  Grace’s kidneys have grown from 8 to 11 centimeters since being discharged form the hospital.  According to the nephrologists (kidney doctors) this is a much quicker growth rate than they expected or hoped for Grace.  Three centimeters in growth might not sound like a lot, but her 3 month old body is starting to run out of room.  Originally all signs pointed to her kidneys remaining at a tolerable level in size for Grace to grow along with them, but according to the latest results this is probably no longer the case.

What this means.  The large kidneys mean a few things.  First off, their rapid growth continues to interfere with her blood pressure, which is on a constant roller coaster ride.  Without a well controlled blood pressure Grace is at risk for many problems.  Another issue is that she is simply running out of room.  Her kidneys can get too big and can then start pressing in on her stomach which will make it hard for her to eat and grow.

The pattern forming with her kidneys leads to a difficult road.  Our hope was that this would not happen for a long time and she would be big enough to accept a transplant and then get on with her life, but if this pattern continues the doctors will have to act much sooner.  This action would consist of removing her large kidneys that are poisoning her body.  After the surgery it will be necessary to go to dialysis four times a week for 3 to 4 hours at a time.  She will need to continue this process until she is large enough to accept a full kidney (around one year of age and at least 20 pounds) because she will not have her kidneys to do the work for her.  After 2 to 3 months of dialysis at the hospital there is an option to insert a catheter into Grace and then we can run a machine out of home and put it on while she sleeps at night instead of driving to San Diego 4 times a week.

From a great high to a gut wrenching low.  We are doing our best to remain positive and trying to focus our energy into believing that Grace will defy the odds, that she will somehow make her kidneys stop growing at such a pace, that she will keep steadily gaining weight, and astound all of the doctors.  Right now it is a pattern, a concerning pattern to the doctors for sure, but not yet set in stone of staying on this same course.  She has such spirit, she is so full of life, she is so happy and full of love, and when she smiles at us it seems as though she can defy any odds or walk on top of the ocean.  She is so loved and thought of by all of you and that radiates to her entire being, we know it, and we know she feels it.

Much will come to light with the next ultrasound.  If it continues and we have to go down that road, then that is our fight and we will battle through.  She is so tough, way stronger than Nicole and I, and will plow right through it, while the two of us try to keep each other standing.  We know and are so thankful that all of you will be there to keep us upright and positive.

Grace’s doctors did ask us to start gathering data for potential donors.  Her blood type is O positive, but I believe she can have either form of O, negative or positive.  Blood type is a place to start, but after blood comes extensive testing for a compatible kidney.  I wish I could rip both of mine out right now and give them to her, but I cannot, our types do not match.  It kills me that I cannot do this for her.  Nicole is a possible match and she would love nothing more than to give her one of hers, but the testing yields very fickle results and we have no way of knowing right now.  I know some of you have mentioned you would love to give her one of yours if you could, but please, really consider all of the scenarios before saying you would like to be tested.  This is a huge decision, one I wish I never had to ask, one I wish I could take care of myself, but it is a reality Grace now faces.

Feel free to contact us at anytime, on the site, email, facebook, and I will do my best to get back to you promptly and also to keep the website posted as soon as we hear anything new.  Thank you all for being who you are.  Keep Grace in your hearts, we love you all.

Grace, Nicole, Nicasio

Her blood pressure is still a bit of a challenge to control because of her growth. She is now over eleven
pounds and doing much better at keeping her food down. This growth does make make it necessary to
change the dosage on her blood pressure meds as she continues to get bigger. Her kidneys remain at normal
function and as posted before, we hope they will remain that way for a long time before she has to deal with
a transplant or dialysis.

On to the fun stuff. She is an amazing little baby. Her alertness is astounding. She follows Nicole and I
with her eyes and head as we move about the room. Grace loves to smile and wants to talk so badly.
Music of all kinds tends to put her at ease and also the sound of the vacuum cleaner, go figure. Grace also
does very well in public settings and enjoys loading up in the car and going somewhere new. Just in the last
two weeks she has been to the beach, we have visited family and friends at the Ponto Campgrounds, been
to a couple of restaurants, taken walks, and has soaked up the sun in our backyard. She is so happy and has absolutely stolen our hearts. We know she is still receiving so many good thoughts and prayers from all of you and we thank you for keeping her in your hearts.

If you have any questions or comments please feel free to email me at cosmo875@hotmail.com or leave a comment on the website.

I also added some updated photos along with this post. Thank you all again. We love you so much.

We are home.  We were discharged Monday evening from Children’s after another step forward
on Grace’s heart ultrasound.  Her list of medications goes on for way too long, but she is home,
with us, and we can tell that she is happy to be here.  As for her long term diagnosis, there is still
much unknown, but this is what I can update you on:

Her blood pressure is now under control (with the help of the meds.).  A  few of the kidney
doctors still believe the blood pressure is what caused her heart  condition, but the cardiologists
believe her heart problem was a separate issue.  We are not sure the  exact cause, but either
way, her heart is improving.

Grace’s heart issue has to do with her left ventricle, which was found inflamed and enlarged. The
left ventricle sends our blood back to all the parts of our body after it has been oxygenated by the
lungs.  The measure of how this works is called the ejection fraction.  A perfectly healthy heart will
have an ejection fraction of 60 percent, and according to the cardiologists Grace’s ejection fraction was down
to 9 percent when we were admitted into the hospital.  Their belief was that Grace’s enlarged kidney’s were
causing the high blood pressure, which then caused the inflamed heart, and after controlling the blood pressure
the heart would shrink back to normal size.  Grace did not respond quickly to their medications which led them
to believe her heart issue was not related to her kidney disease.  But, after time her ejection fraction rose with
each ultrasound. It went from 9, to the high teens, to 26, to 34, and on Monday it was at 42 percent.  Whatever
the cause, her heart gets stronger by the day, and there is great hope to believe that eventually it will be strong
enough to eliminate the heart medications she now has to take.

Her kidneys are functioning perfectly at the moment.  Our kidneys have a creatanine level which lets doctors know how well our kidneys our functioning.  In newborns and infants the level should be at .3 and that is the exact level
Grace is at right now so she is far ahead of the game as compared to many children born with ARPKD.  Her lungs
and liver, which often get damaged by the disease, are perfectly healthy.  The spectrum is massive for how long
her kidneys can remain this way.  There are cases where children need a transplant or dialysis right after birth, to
cases where they do not need a transplant until they reach college.  There is no way to predict the future of when
the time will come for Grace, but all signs point (if she continues to eat well and grow) that she might not have to
deal with this for a long time.  That is our hope so she will be strong, and able to accept a new kidney when the time comes.  A baby only needs to be 15-20 pounds to be able to accept a full adult kidney, but we hope it will be much further on down the road.

We are home, but it will take some time to get used to Grace’s schedule of medications. Currently she has to take
10 different medications. Many of the 10 are supplements (sodium, magnesium etc.) to counter balance her loss of
these elements caused by her blood pressure medications. There are at least 2 of the 10 that we should be off of in
a months time. After that, if she continues to grow and get stronger, we hope the medicines will be scaled back, and a few of them eliminated completely.

We have no words.  Nicole and I are at a complete loss for them.  All of your love and support has been so incredible, so overly amazing, that we have been brought to tears over and over again from all of your generosity and caring. Thank you does no justice.  We are simply awed.  What an amazing community of love, caring, family, and friendship. Grace is so loved, and because of this wealth of love and energy from everyone she is healing herself and will continue to do so.  She will be able to move mountains.  She already has in many ways.  She has taught us so much about courage about life, and above all, grace.  What an appropriate name for you little one, we love you so much.

So with all of our hearts, energy, love and soul, we thank everyone for every well wish, every prayer, every contribution, in any way shape or form.  We love you all so much and know that are the most fortunate beings to blessed with all of you in our lives.

With all the love we have,

Grace, Nicole, Nicasio

We’ll keep you updated with any news.