Lazy Days of Summer

We have reached the point in the summer when the vacations are over, our plans are loose, the weather is something like a damp furnace, and fall schedules are just around the corner.  We have been enjoying a hodge-podge of activities...

Julia has been on a breakfast strike lately.  If I left it up to her, she'd have goldfish every morning.  I'm really thinking she might turn into one soon.  We buy the huge carton cube regularly and she is the only one who eats them.  That's a lot of fish.  After several consecutive mornings of debating her options, she said she needed a cup.  She then went out in the backyard, filled it with tomatoes from the garden, and ate those.  I guess that works.  Beats goldfish, right?

Carter was excited to discover we had sweet potatoes.  You can probably tell from the shot over his shoulder that our inaugural year with sweet potatoes was a success.  Our little starter plants quickly took over our little garden.  I had no idea what the outcome would be, but look what we found... 

 Julia and I ate it for lunch and it was really yummy.

We've enjoyed some family rounds of putt-putt.  I think it just makes us feel like we're still at the beach :) Here's Julia giving her Daddy some pointers...

We were invited to a fun backyard barbecue with some friends from church.  The kids loved the pool.

And we even roasted hot dogs and smores over the fire pit.  It's fun to feel like kids again.  A perfect summer night...

We're trying to get to the waterpark as much as we can before the season ends.  It always comes much too fast.

When we're not swimming, we're usually inside escaping the heat.  I'm not sure why the summer weather inspires the kids to break out their snow gear.  Wishful thinking maybe?

We have been getting back to our daily schoolwork routine.  Carter is doing a great job and Julia is coming around.  She still gets emotional at times and has to leave the room to gather herself, but overall she brings a lot of encouragement to the table.  I've learned not to turn my back for too long.  What in the world?!

Last week we had the opportunity for a special field trip.  The Marine Corps League was holding their national conference in town and the first day they had an exhibit open to the public.  I told the kids we would go after lunch if they completed their assignments.  They disappeared while I made lunch and came down dressed and ready to go...

I must say I was impressed.  They both had all their shirts tucked in, belts on, and gear ready.  The exhibit was really impressive.  It included the personal collections of over a dozen Marines.  They had a full display of the past 100 years of service.

The kids were so charming.  They both saluted everyone they met.  In a room full of retirees and their wives , they were a big hit.  I wish I had pictures of some of their greetings, but I was too busy fielding questions and making introductions.  We had the privilege of meeting some amazing people.  The group includes Marines who landed at Guadalcanal.  There were so many stories to take in all at once.

Carter was in awe.  He had me take dozens of pictures and was asking lots of questions.  He couldn't believe he was allowed to touch everything, too.  They were so generous and kind to the kids.  It was a day they will not soon forget.  We are very proud to be a part of the Marine Corps family. 

And on the health side of things we are still plugging along with Julia's GI issues.  We had to stop the Dulcolax suppositories and were told to instead use daily saline enemas.  We started these last week and she handled it all okay, but unfortunately, it did not yield the desired result.  The entire process takes over an hour start to finish and we weren't having success.  So for now we have gone back to using the Phillips oral milk of magnesia.  It is not the most desirable, but at this point it the easiest of some really crummy options.  It is still crazy to me that all of this is just a part of what we have to do everyday.  It definitely impacts everything we do and how Julia feels.  I feel so bad for her having to deal with this and still be in diapers.  I worry about the long term impact this will have on her GI tract and health.  Laxatives were not designed to be used this way, but sadly we have to do this.    

We continue to pray for healing and for the spots on her lungs to disappear.  Her chest x-ray is scheduled for September 2nd.

Little Patients, Losing Patience

Mol Cancer Ther. 2006;5:1905 1908 © 2006 American Association for Cancer Research Editorial

Little patients, losing patience: 
pediatric cancer drug development
The December 2004 Food and Drug Administration (FDA) approval of clofarabine (Clolar, Genzyme) for refractory pediatric acute lymphoblastic leukemia was a milestone in the history of pediatric cancer drug development. A rare occurrence, it represented approval of a new cancer drug for a pediatric cancer indication without prior approval for an adult cancer indication. This is a major exception, as approval of new cancer drugs for pediatric patients is typically an afterthought to their development and approval for treating adult cancers. In fact, of the 120 new cancer therapies for adults approved by the FDA between 1948
and January 2003, only 30 have shown use in children. Of those 30 drugs, only 15 acquired any labeling for pediatric use during that same 55year period (1, 2). This disparity in the number of oncology drugs labeled for use in adults versus children is a manifestation of the many challenges faced in pediatric cancer drug development.

So, why has pediatric drug development been the poor stepchild of cancer drug development? What are the major challenges currently confronting pediatric cancer drug development? What are pediatric oncologists and other children's advocates doing to overcome these barriers? Despite these challenges, how have pediatric oncologists managed to make extraordinary strides in improving outcomes for children with cancer?

The Challenges
In general, the major obstacles faced by pediatric oncologists arise from the same underlying issues faced by adult oncologists but are significantly amplified in pediatrics: limited funds, limited time, limited numbers of investigators, limited numbers of patients, and limited political clout. How these factors intermesh to constrain new pediatric cancer drug development is outlined below.

Cancer in Kids Is Not Profitable
Cancer is the most common cause of nonviolent death for children in the United States. One in 300 children will be diagnosed with cancer by the age of 20 years. Yet, the total number of new pediatric cancer diagnoses is miniscule compared with the total number of new adult cancer diagnoses. Whereas 12,000 to 13,000 new cases of pediatric cancer are diagnosed in the United States yearly, a staggering 1,368,030 adults were diagnosed with cancer in the United States in 2004. For additional perspective, there are more cases of breast cancer diagnosed in New York State each year (15,190 in 2004) than there are new pediatric cancer diagnoses nationwide (3).

Once pediatric cancers are broken down by individual diagnoses, their numbers relative to adult cancers become exceedingly small. With the average cost of research and development to bring one drug to market at $802 million and given that 1 in 1,000 new compounds that enter preclinical testing ever make it to human testing and only 1 in 5 agents that enter human trials receive FDA approval (4, 5), it is little wonder that pharmaceutical companies would hesitate to invest in pediatric cancer treatments. Basic economics clearly favors investment in a treatment for 215, 990 adults yearly diagnosed with breast cancer over a treatment for
a mere 425 children yearly diagnosed with rhabdomyosarcoma (3, 6).

What can be done to encourage the pharmaceutical industry to develop new drugs for children? In the 1990s, there were multiple unsuccessful attempts to encourage voluntary pediatric studies. In 1998, the FDA finalized the Pediatric Rule, requiring pharmaceutical companies to conduct pediatric studies under certain circumstances. The first studies were required to be submitted in December 2000. However, that same month, the Association of American Physicians and Surgeons, the Competitive Enterprise Institute, and Consumer Alert filed a lawsuit against the Pediatric Rule, claiming that the FDA had no legal authority to mandate pediatric studies. In October 2002, a Federal District Court invalidated the Pediatric Rule (1, 7, 8).

As neither attempts to mandate pediatric studies nor appeals to altruism have proven successful, the FDA has shifted its strategy toward offering financial incentives for evaluating new agents in children. The result is the Pediatric Exclusivity Provision, initially a component of the FDA Modernization Act of 1997 and later renewed from January 2002 to 2007 as part of the Best Pharmaceuticals for Children Act. This provision extends patent protection on a new agent for an additional 6 months for pharmaceutical companies that do pediatric studies requested by the FDA. As of February 28, 2006, 117 drugs have been granted exclusivity, of which 10 are oncology drugs (9). Although the Pediatric Exclusivity Provision seems to be having a positive effect, there is no guarantee that it will be extended beyond January 1, 2007 (1, 7, 8).

Kids Do Not Vote
A substantial segment of the U.S. population is disenfranchised because of their age. Simply stated, kids cannot vote. Without a voting block to get the attention ofWashington, children must rely on others to make their case for them come budget time. Historically, pediatric cancer has not been a budget priority. For example, the total budget for National Cancer Institute (NCI) for fiscal year 2004 was >$4.7 billion (10). Of that, only $166 million (3 percent) was devoted to pediatric cancers in any form, including funding for prevention, treatment, and longterm followup (11).

From a budgetary perspective, it seems that things will only get worse for children with cancer before they get better. For fiscal year 2006, the total NCI budget from the federal government was cut (again) by $39.7 million. This is a major setback for the Children's Oncology Group (COG), the nation's premier pediatric cancer clinical trial organization and a major player in the pediatric oncology drug development world, whose NCI funding has been cut by 10% as a result (Gregory H. Reaman, COG Spring Group Meeting, March 23, 2006). These cuts translate into fewer clinical trials and scientific inquiries for children with cancer at a time when clinical trial enrollment and resulting access to novel drugs have never been more critical. In addition to short term issues of survival, increased capital is likewise essential to address the long term effects faced by childhood cancer survivors from today's toxic treatments (12, 13). As these children now live into their thirties, forties, and beyond, chronic illness and disability are emerging as major challenges. Increased funding is needed both to develop less toxic treatment regimens to minimize late effects and to address those effects when they do occur.

Despite worsening budget constraints, there is a glimmer of hope. One encouraging new development is the Conquer Childhood Cancer Act of 2006. Introduced in March 2006 to the Senate and House of Representatives jointly by Senators Norm Coleman (RMN) and Jack Reed (DRI), Congresswoman Deborah Pryce (ROH),and Congressman Michael McCaul (RTX), the Act would authorize $100 million for over 5 years to support translational research into pediatric cancers (14). It is too soon to tell whether this bill will successfully pass and whether it heralds a change in priority by our government toward curing pediatric cancer.

Kids' Cancers Are Different
Children and adults are affected by different types of cancers. Even the cancers that are "common" to both groups are frequently different on both phenotypic and molecular levels. Molecular profiling of both adult and pediatric cancers will be crucial to understanding these differences as well as uncovering possible overlapping
characteristics and treatment targets between children and adults. As cancer drug development moves progressively toward this molecular targeting and away from nonspecific toxins, it is quite possible that the overlap between effective treatments for adult and pediatric cancers will decrease. The more divisive and targeted treatments become, the fewer total patients there are in each disease subgroup. Smaller numbers not only mean less financial incentive to develop new drugs but pose additional challenges as discussed below.

Kids' Cancers Behave Differently
Cancers in adults and children often act and respond differently. For instance, pediatric cancers are frequently more aggressive and rapidly progressive (which sometimes is an advantage for treatment efficacy) than many of the more indolent adult cancers. This is one of the many reasons it is difficult to accrue pediatric leukemia patients onto singleagent phase I trials. Relapsed acute leukemias often progress too quickly for patients to enroll on study and await response. A recent illustration comes from an adult phase III trial in myelodysplastic syndrome, which showed that the median response time to decitabine, a demethylating agent, was 3.3 months. This is far too long to wait for a rapidly progressive pediatric acute leukemia (15). Pediatric oncologists have tried to address this issue by using newer clinical trial designs, such as administering
novel agents in brief windows followed immediately by administration of combination cytotoxic chemotherapy. They have also moved toward eliminating singleagent phase I leukemia trials altogether, choosing instead to extrapolate toxicity and dosing information from pediatric solid tumor and adult leukemia trials.

Too Few Kids
Besides the financial disincentives to developing new pediatric cancer drugs due to the small patient base, the limited number of pediatric cancer patients presents other challenges. Even with the dearth of pediatric cancer– specific drugs, there are more new oncology drugs in development with potential activity in pediatric cancers than there are pediatric patients in whom to evaluate them. How do we choose which drugs to valuate? How do we make decisions using limited numbers of patients? How can we complete studies more efficiently?

Because of small patient numbers, clinical researchers in pediatrics are often forced to weigh the consequences of conclusions drawn from studies with limited statistical power. By using fewer patients, the increased risk of both type I and type II errors may result in moving forward ineffective drugs or discarding effective ones. Novel approaches to clinical trial design are needed, including designs that require fewer patients. Some adult cancer drug studies have already begun implementing new trial designs. These need to move quickly into the pediatric realm, where the need, based on limited patient numbers, is far greater.
For phase I trials, one approach is to determine dosing based on molecular response in lieu of continuing to enroll patients at escalating doses until a maximum tolerated dose (MTD) is reached. However, which molecular end points have clinical significance? Which end points truly correlate with the anticancer mechanism of the drug? How do we determine statistical significance or draw a "positive/negative" line on a biological continuum? These are just a few of the many questions that require creative answers if we are to move our clinical trial design into the 21st century to match the current age of drug development.

Kids Get the HandMeDowns
As discussed previously, it is rare that a drug is brought to evaluation in children before completion of at least one adult trial. Phase I pediatric trials are initiated an average of 2 years after the adult phase I trials are published (2). This traditional pattern of evaluation, which effectively relegates children to second class
status, further delays newer treatments from reaching children.

Kids Are Not Little Adults
Pharmacokinetics, efficacy, and toxicity can be different between children and adults, which limits the utility of extrapolating data from adult trials.

Limited Preclinical Models for Kids' Cancers
There are limited preclinical models available for pediatric malignancies. Traditionally, pediatric tumors have not been included in the NCI panel of cell types used to screen potential anticancer compounds. The Cancer Therapy Evaluation Program of the NCI recently addressed this gap by initiating a Pediatric Preclinical Testing Program, which makes use of cell lines and animal models for pediatric tumors to test new compounds (2). The program is currently evaluating 11 to 12 new agents yearly (Malcolm A. Smith, COG Spring Group Meeting, March 23, 2006). These pediatric specific preclinical models enable more efficient evaluation of new drugs and prioritization of which drugs to move forward to pediatric trials. Additionally, this program includes testing of pediatric tumor tissues and cell lines for gene and protein expression, thus
facilitating the development of molecularly targeted drugs for pediatric tumors (2).

Too Few Formulations for Kids
The average age a child can begin swallowing pills is 7 years. For younger children, inability to swallow oral agents poses a significant treatment obstacle. If an oral agent has no liquid formulation and the pill form cannot be crushed, patients may have no other treatment options available. There is currently little financial incentive for pharmaceutical companies to invest the time, effort, and capital needed to develop pediatric liquid formulations. Consequently, a pediatric oncologist may be left to tell parents that no treatment is available for their 5yr old child because he cannot swallow a pill.

Ethical Considerations with Kids
Institutional Review Boards are much more cautious when it comes to "experimenting" on children. A parent's ability to "consent" on behalf of a child may be more limited than that parent's ability to consent to treatment for himself or herself. For instance, in an effort to evaluate a molecularly targeted drug, it would be ideal to obtain a sample of tumor tissue after a drug is administered to evaluate for effect on the desired target (this is often done in the adult oncology world). But, is it ethically appropriate to subject a child on a phase I study to the pain involved with a repeat tumor biopsy to assess molecular response? Can we ethically ask a parent to consent to that? These ethical considerations often lead to use of surrogates, such as normal peripheral blood mononuclear cells, to evaluate target modulation. Effects on these surrogates, however, may not accurately reflect the activity of the drug in the malignant cell.

Another controversial issue plaguing Institutional Review Boards nationwide is whether a child can or should consent on his or her own behalf to participate in a study (called "assent"). Questions abound. At what age, if any, is assent appropriate? Is true informed "assent" even realistic? How should differences in children's developmental levels be taken into account? Should the type of study (e.g., phase III for a newly diagnosed patient versus phase I for a relapsed patient) affect how much say a child has in the decision to participate? To address these and other issues, the COG Bioethics Committee convened a multidisciplinary task force in 2003 to address issues of assent and to formulate guidelines for pediatric oncology (16).

Despite all of the challenges in developing new pediatric cancer drugs, outcomes for children with cancer have improved steadily to a current overall event free survival of 75% (17). Clearly, pediatric oncologists have confronted daunting obstacles yet have somehow managed to work around them, often outpacing their adult oncology counterparts. The many successes in pediatric oncology can be attributed to a variety of factors.

Kids' Well-Oiled Clinical Trial Machines
Pediatric oncologists have integrated clinical trials into a culture of standard practice. Whereas only 2% of adult cancer patients enroll in NCI-sponsored clinical trials, 50% of children with cancer do (18). COG is a well-established, well-organized clinical trial machine. As with any large organization, efficiency could be improved, but the overall success of COG and its predecessor organizations has been extraordinary. Other complementary pediatric cooperative groups have also emerged, enabling even more drugs to be tested and giving patients greater access to new agents.

Kids Have Parents
Medical staff and families are especially aggressive about seeking cures for children with cancer. Consequently, parents, with the support of an enthusiastic and encouraging medical staff, are highly motivated to enroll their children on clinical trials.

Response Assessment Easier in Kids
As noted previously, pediatric tumors are often more rapidly progressive than many of the more indolent tumors found in adults (e.g., sarcomas versus carcinomas). This is one reason pediatric cancers are frequently more responsive to cytotoxic therapy, which targets rapidly dividing cells. This in turn impacts the approach to clinical trial design. Rapid response allows efficacy assessments to be carried out sooner, supporting the use of early response as a surrogate marker and thereby expediting drug development.

Kids Are (Otherwise) Healthy
Children typically have good underlying organ function. They can usually tolerate more aggressive, more toxic treatment.

Kids Make HandMeDown Fit
Although the progress of pediatric drug development is delayed while awaiting initial adult clinical trial results, this carries some advantages. By the time pediatricians are conducting a phase I trial, adult data on toxicity is available. Dose escalation can then be based on the adult MTD. Because children tolerate toxicity better than adults, the pediatric MTD is rarely less than the adult MTD. Typically, the initial dose level in a pediatric phase I will be 80% of the adult MTD and the dose will escalate from there. This helps limit both the total number of patients needed to complete a pediatric phase I trial as well as the number of patients treated at lower, often subtherapeutic doses. Additionally, adult trials provide preliminary efficacy data. This can help in prioritizing which drugs to bring into pediatric trials. While care must be taken when extrapolating efficacy data from adult studies to pediatrics, doing so can provide useful information beyond preclinical models.

The Future
Despite our success in markedly increasing survival in pediatric cancer patients, there is much work to do. We need better science to unravel the molecular underpinnings that drive childhood cancer. We need better agents for children who do not respond to currently available treatments. We need better clinical trial designs to increase efficiency of the drug development process and to optimize how we treat children with the new generation of anticancer drugs. We need better approaches to the short and long term toxicities of treatments, from major organ toxicities to secondary malignancies, which will hopefully decrease with the rational use of molecularly targeted agents.

Although many obstacles remain for pediatric cancer drug development, none are insurmountable. No longer able to wait patiently, our little patients require the ingenuity, creativity, cooperation, and perseverance of individuals and organizations dedicated to putting themselves out of business.

Jessica Boklan
Center for Cancer and Blood Disorders, Phoenix Children's Hospital, Phoenix, Arizona
E mail: jboklan{at}phoenixchildrens.com

Matters of the Heart

It has been an interesting week around here.  I called GI when we got back to come up with a new plan for Julia.  The nurse was very understanding and agreed we needed to find something better than the oral laxatives, but knew the dulcolax was not going to be a long term option.  She talked with the doctor and it was decided to put her on daily saline enemas.  The dulcolax is effective and convenient but long term use of the stimulant drug will damage the muscles in her GI tract.  So the safe alternative in her case is daily saline enemas.  We have been given our instructions and are gathering all the needed supplies.  We cannot use the OTC Fleet version because the phosphate will mess up her electrolyte balance.  I am dreading having to do this to her.  Unfortunately we have no choice.  This is not something that can be ignored.  It has to be addressed daily.

After a lot of prayer, discussion, and emails with fellow Wilms' parents; we decided to get a chest x-ray for Julia in September.  I called the oncology clinic to leave a message for Dr. McLean with our request.  When I told Ann our concerns, she scheduled it on the spot.  It means so much to me that they trust parents' instincts and honor our requests without a second thought.  So now Julia will have a chest x-ray on September 2nd.  We are praying that the 3 spots on her lungs have healed and disappeared, but if there is something we need to see, we pray that God makes that very clear.

Tuesday, I returned to the cardiologist to receive my Holter monitor.  I was hooked up and strapped in. 

 They failed to mention to me in advance that you cannot shower or remove your bra while wearing one of these.  Would have been nice to know in advance.  Thank goodness I took a shower at the last minute! It is going to be a long few days, especially with the 100+ temps.  I had to document all my activity and any symptoms I felt during the 3 days.  I find it all strangely ironic that I am wearing this contraption that is looking into my heart. 

 Can it really see what's in there?

• Does it know what it feels like to fear your child's cancer may relapse?
• Does it know what the scars of cancer treatment look like on a mother's heart?
• Does it know how it feels to give your daughter an enema just to function every day?
• Does it know my heart is swinging through fire and clinging tightly to faith?
• Does it know the pain of seeing other children relapse and die from this beast called cancer?
• Does it see and feel all the unknowns the same way I do?
• Does it know how much heart surgery was not in my plans this fall?

I guess we'll have to wait and see.  I get the results from the monitor recordings in two weeks when I visit the cardiac electrophysiologist.  I have been doing some research into possible surgeons in the event I do have to proceed with the ablation.  One of the toughest things lately has been the time devoted to medical research.  It is hard enough to have to deal with the medical situations we've faced, but as a cancer parent and cardiac patient, I also have to be an advocate for both of us which involves research.  It has been both consuming and empowering.  I feel like I have some good leads in both areas and have made some good progress this week. 

When the week began I was still struggling with the emotional weight of it all.  I have felt the burden ease a little day by day.  I attribute much of that to my precious friends.  I am blessed to have the kind of friends that call even when they know there is nothing they can say, that cry along with me when we need to, that aren't afraid of what may come, that send letters and emails at the leading of the Spirit, that drop in to do daily life because they just sense the need, and that never cease to pray for our family.  I have the friends that hold up my arms when I can't gather the strength and I am blessed beyond belief. 
 

Wilms' Warriors

I have begun to add tabs to my blog.  I love this new feature of Blogger.  You will see under the title picture a row of tabs.  I will be adding more soon.

The first one is a compilation of Wilms' Kids.

Beach Week

You may have been wondering where we've been.  I'm happy to say we were on vacation.  Our annual family trip to Emerald Isle on the Crystal Coast of NC.

Absolute beach perfection...

The beach never disappoints.  We had sun, clouds, rain, winds, calm, and choppy- a little bit of everything- and we found ways to enjoy it all.

Carter was eager to hit the waves and didn't waste any time...

He was fearless in the water.  He has learned to swim well this summer and it has given him the extra confidence boost to boogie board and body surf with ease. 

It still makes us all laugh because two years in a row he wouldn't even get his feet wet!

Now he's getting lessons and learning fast.

The frequent wipeouts didn't deter him at all.  He'd hop right back up and head out again...

Julia loves the ocean, too.  She prefers to be held, but has no fear in going in.  She cries when she gets saltwater in her eyes, but begs you not to take her back to shore :) She's one tough cookie!

Anderson is a water rat himself and jumped at any chance to go join in the fun.

It still surprises me to see something new at the beach.  This year it was jumping fish! They would jump about 2 feet out of the water right in front of us! In my 30+ years of beach-going, I have to say I have never seen anything like it.  Anderson kept saying, "Here fishy, fishy! Come out, come out 'whereber' you are!"

Julia is still a big time sand lover.  She can sit and play for hours and did so everyday.  She was happy to have Daddy to bury...

and made many sandcastle masterpieces...

Mimi and her kiddos...

The many tide pools were a great place to rinse off and splash... or scuba dive...

Little Mr. Blue Eyes, such a charmer!

When we weren't at the beach, you could usually find us in the pool.  Water fights were a big hit...

and the giggles abound....

Made even better by late afternoon treats of popsicles and ice cream sandwiches.  It is vacation after all!

"Hey, are you going to finish that?"

And the best part about having a pool on the deck is the amount of energy burned.  We have decided it's like a treadmill for kids.  They swam until they couldn't move.

And Carter still adamantly refuses to nap.  He will go to great lengths to keep himself up, even holding his eyes open! He closed his eyes for about 2 min one afternoon and I caught him in the act... 

Many morning started with a round of putt putt.  All the kids are big fans.  Grandpa took the trio one morning and they were a site.  We had to laugh at the bodies moving everywhere.  Think golf meets Frogger :)

Julia was so proud of her putting and sticks with it for all 18 holes.  She even had a few hole in ones...

Followed by some sweet treats- salt water taffy...

Many evenings we headed to the beach for some kite flying.  This is something every kid wants to do often, but the wind doesn't usually cooperate.  The beach is a different story.  You pretty much release the string and it's airborne...

and some sand chipping for the boys...

 

We had some fun family time.  Daddy was able to stay for four days.  Everyone loves their Daddy-time!

My hands are full, but so is my heart...

It was such a gift to see Julia so strong and energetic this year.  Last year she was bald, pale, neutropenic, and exhausted.  The difference is incredible.  We praise God for this amazing gift!

She has been feeling well overall.  The bowel incontinence remains and I tweaked her medications and schedule to give us the best possible success and experience at the beach.  We are not following the GI doctor's orders right now, but it worked.  We decided quality of life was more important this week.  I was finally able to find some other Wilms' parents with the same symptoms.  They have been told much the same thing.  The surgery, organ removal, and radiation have all impacted the bowels.  The scar tissue, trauma, radiation damage, and organ resettling creates a host of issues.  They ensure it usually returns by puberty.  Everyone I spoke with is treating the issue a little differently, so we will continue to work to find the best plan for her that will help move toward one day healing.

We were all sad to see the week end.  The kids kept asking why we had to go home.  "Can't we just live here all summer?" I am SO with them on that one.  The beach is, by far, the best way to spend summers in the hot and humid southeast.  

We spent the last day enjoying the beach for as long as possible.  Grandpa and his little ducks...

After many hours of swimming and playing, Julia had sand everywhere- ears, eyelids, hair, skin.  She was 'breaded' from head to toe.  We are a bunch of beach bums for sure.  I am so thankful my kids love it as much as the rest of us do.  You have to drag us away.

We were on the beach into the early evening.  Julia and Anderson were dancing in circles and making up songs.  It was the epitome of simple childhood joy...

It was such a gift to be surrounded by God's beautiful creation and have the opportunity to have fun with our family. This was the first time I noticed all the flowers growing in the dunes.  The beach truly has it all...

I must confess that emotionally, we are still riding a roller coaster.  I spent much of the week wrestling with fear and faith.  They are both such strong, consuming forces.  My faith muscles were overwhelmed and abiding in peace was fleeting.

At this point it feels like we're walking along a tightrope of faith, while the fiery fear laps at our feet from below.  Just when we feel steady, the fire laps up and burns again.  Sadly, Skye Getter earned her angel wings August 1st.  She began her battle with the most favorable form of Wilms' and endured four relapses, surgeries, dialysis, and eventually death.  Unfortunately she is not the only one.  Statistics have always been haunting to us.  They are often used to bring hope, but in the end usually the opposite is true.  We know of so many Wilms' kids who are relapsing and having complications right now and it is something you can't ignore.  This is life in the cancer world.

I said to someone recently, "I don't fear the big things in life, it's the microscopic ones."  There is no way to know if your child's body still has anaplasia/cancer cells and where those cells may be lurking.  I have been talking with other families whose children had lung spots and they live with the same uncertainties.  Lung biopsies seem to be done when spots reach 5mm.  In the meantime it is watching and waiting to see if cancer grows.  Some have lung spots, some liver, and some kidney.  It is a part of the world we live in now and we have to find a way to make peace with it.  I'm not there yet.  Sometimes the fear and the unknown just swallows me whole- when I hear her laugh, when she says something sweet, when I see the kids playing together, and sometimes in the middle of a deep sleep.

I hate that I have thoughts of "will this be her last beach trip feeling this good?", "what will next summer look like?", "what is the future of our family?" I am trying desperately to live out the words of one of our first oncology nurses, "Keep your head where your feet are." It is the best we can do.
 
We are all changed from all of this and what we have and continue to witness.  Our priorities have changed. Our tolerance of selfishness and ignorance have changed. Our outlook on the future has changed.  Our sense of security and predictability has changed.  And our normal continues to change, more times than I can even count.

So for now we are savoring every moment, enjoying every opportunity, and hanging on to the ones we love in simple gratitude... 

        

Thank you so much for your continued prayers for each of us. It is a gift we cherish and a power unmatched. Join us as we continue to pray that the lung spots disappear and Julia's body heals. We give God all the glory for what He has done and continues to do! And we ask Him to carry us through these unknown waters.  We need sustaining faith.