Li Fraumeni Syndrome Support Group

Li Fraumeni Syndrome is caused by a mutation to a tumor suppressor gene. In even the best case scenario- a mutation here is not a good thing.  p53 has been called the "guardian of the genome" and plays a HUGE role in making sure cancer doesn't go haywire. It makes sure DNA damage gets noticed and repaired,it makes sure the cell isn't reproducing like a bunny while damaged, and if it can't get a cell repaired- it makes sure the cell dies. It's a pretty cool thing. Except when you don't have it. Then you have p53 envy, you find you have a huge risk of getting cancer- and not just one cancer- many cancers over your lifetime. Kids who have the p53 mutation get more cancers than those who don't and I'm not the first to say- nor will I be the last to say- Kids should never, ever get cancer. It's just not right. That fact, especially when the tumors are brain or adrenal,  is actually one of the BIG flags to doctors that a family might have a mutation like the p53.

I learned about Li Fraumeni Syndrome in the late 80's, early 90's. My brother had just been diagnosed with a brain tumor, months after my cousin had been diagnosed with a brain tumor. An aunt and an uncle were also diagnosed with cancer during these months. Our first thought was- stop drinking the flipping water- but then an amazing article hit the newsstands about these doctors in Boston who were studying family cancers- especially brain tumors. My grandfather died of a brain tumor- so my mom grabbed the phone and started making calls. She talked with a young doctor named Frederick Li who sympathized with our situation, but my brother passed too quickly to be helpful to their studies- there was no biological material. It was mentioned that if my dad were to get a brain tumor- that would be an interesting case study and please let them know. 3 years later, my mother had to make that call. My father was tested, he had the mutation. There were many sleepless nights spent discussing the implications for me and being tested. They let me have the choice. In true 17 year old fashion- I said- um No, thanks, can I go out with my friends? I didn't want to know. I wanted to live, I didn't want to be shackled with the burden of knowing that I was going to die of some rare painful cancer. From what I saw with my dad and brother- knowing vs. not knowing didn't change the outcome one darn bit. That harsh reality came crashing down around me in 2008 when my 3 year old was diagnosed with adrenal cancer. I thought for sure she had a brain tumor. I had gotten a degree in molcular biology, I studied genetics- we talked about Li Fraumeni Syndrome- I never processed that adrenocortical cancers were one of the main syndrome cancers. I had done my research. Before it was Li Fraumeni Syndrome, it was called SBLA- but not in any circles that I was in. It was a wikipedia article that I stumbled on when I was looking up adrenal hyperplasia that brought that to light. It happened to be the day before my son's surgeon called with a referral to an endocrinologist for his pancreas injury-I asked her about my daughter's symptoms and told her our family cancer history and she gave Lily a referral to endocrinology too. This after 2 pediatricians told me not to worry about precocious puberty in a 3 year old- it could happen.

So I called the doctors that studied my family in the 90's and I asked what to do for my other kids, I wanted to know the benefits of testing them. They were supportive and helpful and I also learned about an upcoming conference the following year. It had been decades, but my assumption was this was so rare- there weren't very many other families out there. I wanted to be there. I wanted to meet the others. I wanted to see if they learned to laugh through the tears like my family does- because sometimes laughter is the only medicine.  I wanted to see what progress had been made and where things stood. I wanted hope that my children wouldn't have to watch me die from cancer and I sure as heck don't want to see any of them go through it over and over. At this conference I met others who wanted, like me- to help support each other and work together to find better treatments, screening and ultimately a cure. We are tired of watching our families suffer. We want to help bring together all the different facets of knowledge, research, and experience with this syndrome. We want to be the link the connects the disease to the cure. We are that link. It will take time, and sometimes more time than those with LFS are given. I was so saddened to hear that a lady I met at the conference recently passed. It was agonizing to lose a friend in December who I met because of this disease. But these were two amazing, strong women. 2 women who brought so much love and light to those around them and who both had so much more living to do, but couldn't because of LFS. Since we started in 2009, we have hit lots of bumps and several new cancers in our group. That is the nature of the beast. But through people with Li Fraumeni Syndrome is a better understanding of cancer and through this understanding will be a cure!  If you are interested in donating time to a great cause- check out our support groups at Facebook- we welcome help with open arms. There is one for those with LFS and one for Friends and Family.  But most of all I have to thank my monkeys and my husband who have dealt with a pretty messy house and a scattered mom- but do so gracefully so that I can try to help create something that will do so much good.

https://www.facebook.com/groups/90503981891/ 

Adrenal Insufficiency Initiative

There is an initiative to get the life saving medication Solu-cortef ready available by EMS to treat people like Lily who have an adrenal crisis and are found unresponsive or in an emergency situation. Please support us by signing the petition. This initiative could save Lily's life. The condition may be rare, but it is easy to treat with a medication called Hydrocortisone which can also be used for other conditions. Thank you!

http://www.change.org/petitions/adrenal-insufficiency-united-create-protocols-for-adrenal-insufficiency#

The Sammich Approach

My husband often employs the sandwich approach in parenting. Positive reinforcement, criticism/instruction/discipline, positive encouragement.  On days he is really on his game- the kids don't even know they are being schooled and I often marvel at his technique. Heck sometimes I'm even caught between criticism and postive before I realize what happened. He's gooood. It's one of the many things about him I love and appreciate. Sometimes, when I have time, I try to emulate the finer aspects of his teachings- but time has been in short supply.

The kids are back in school- we are in week 3. I have heard from 2- TWO of the many folks I emailed regarding the Storage and Administration of Medications Policy and it's obvious faults. The email went a little something like this.....

My name is Jennifer Mallory. I have 4 children and have had them in the Hawaii public school system for 6 years.  Last May, a new policy was instituted that affects one of my children significantly based on her medical needs. This policy changes the way the school health aide is allowed to store and administer medication. There are only 3 medications allowed under this policy- Glucagon, Epi pen and Inhalers. There is no space, policy or provision for children who have special medical needs that cannot be treated with these three medications. In 2010-2011 SY, I was able to fill out a form 36 that would allow approval for her medication to be stored an administered at school. This new policy affords no alternative. I requested a 504 plan evaluation to ask for accommodation for her medicine and we had that meeting July 27, 2011.

In 2008, my daughter Lily had 2 types of cancer, adrenocortical carcinoma and a rare sarcoma. She has been through multiple surgeries and chemotherapy. Today she is a healthy, bright 6 year old.  A side effect of the chemotherapy is a condition called adrenal insufficiency, her adrenal glands no longer function. Adrenal glands produce steroid hormones that regulate bodily processes such as blood pressure and stress response. We replace this function with daily medication called Hydrocortisone. I give her this medication daily, outside of school hours. In the case of sickness or trauma, the body releases extra hydrocortisone to help the body maintain life functions. Lily’s body cannot do this, so in the case that she develops a fever, diarrhea, vomiting or is severely injured, extra Hydrocortisone needs to be given.

Adrenal insufficiency is extremely rare. Hydrocortisone was not even on the school formulary last year. That does not mean it is any less important to the safety and survival of my child in an emergency situation. Unlike Glucagon or the Epi pen-you cannot overdose on Hydrocortisone. The injectable form, called Solu-cortef is very similar to Glucagon. Without this medication, in the case of an emergency, Lily could die. She does not fall under the special education protections that I am aware of, as she meets proficiency on almost all of her benchmarks. This policy causes undue stress and work for  families, educators, administrators and the Public Health nursing system.

The solution presently is the recommendation that Lily has to go to the only school in the complex with a nurse who under this policy is licensed to give medication other than the 3 medications of the policy. This policy is discriminatory. The health aides at any public school are allowed to give 2 types of injections in emergency situations, both which could have detrimental side effects- but not allowed to administer another life saving injection?  Her condition requires no more assessment than that of the conditions under the policy. My daughter would then be required by DOE policy to go to an alternate school based on her medical condition, which is in practice discrimination and in  opposition to the Americans with Disabilities Act. The policy is seriously flawed. At a minimum it needs to have a stipulation for medicines other than the epi pen, Glucagon and Inhalers. There are not only 3 conditions that require life saving medications. If a health aide is qualified to give either of those injections, she is overqualified to give a Hydrocortisone injection as it requires no assessment or evaluation.

Please review this Policy. It is not sound practice and does not adequately address the issues implementing it produces. There needs to be a way to get medications approved for storage at schools for all emergency life saving medications. I have contacted EMS and with  a medic alert bracelet and a letter from a physician- they can approve administration of these rare medications they do not have on hand.  There are reasonable solutions to this problem that are neither taxing nor difficult to implement at low or no cost that facilitate the best care of Hawaii’s children.  This policy is a disservice to families who have already faced way too much hardship and carry the burden of caring for  a child with special needs. It is a disservice to the hardworking educators and administrators in the schools who have to try and keep their children safe while the policymakers tie their hands behind their backs. Please contact me to discuss any fine points of this policy that may not be immediately apparent to me.

Thank you for your time,

Jen Mallory

There are many issues with this letter. It goes against many of the finer letter writing suggestions. I think it has about 8 run on sentences. It is far too long, it uses words like diarrhea and vomit and ends on a note of sarcasm. So I daresay it gets skimmed, if read at all. There are a couple of things it does not mention. It doesn't mention storage space being an issue- it is not- the only issue is the policy. It doesn't mention the gender or age of Lily's siblings. It doesn't mention taking any legal action and I assure you I have not the funds to retain legal counsel on this matter. It is funny how one phone call can change the flow of the smoke in front of the mirrors.  Here are the 2 responses I have gotten.

Dear Ms. Mallory:

I was sorry to learn of your daughter’s medical condition, however, it sounds like Lily is a thriving cancer survivor. Your daughter’s need for medication and accommodation at school is a complicated issue impacted by DOE policy and staffing.  It is my understanding that your family has been represented by legal counsel, Eric Seitz.  I have been further informed that a solution was found which meets with your approval. I believe the Leeward Complex Superintendent found a storage space at your child's school so that Lily can remain with her three older siblings.  

I will be meeting with Superintendent Kathryn Matayoshi to discuss a range of school health issues before the end of the month. I will share with her your concerns.  Thank you for bringing the issue of medication storage and administration to our attention.

Again, I am pleased to learn that Lily and your other daughters are doing well. 

I immediately recognized the sammich approach and respect the skills- but unsatisfactory.

and this one is my favorite..from someone I was told by 3 different and unrelated sources is the person who made the policy in question.

Dear Ms. Mallory,

My apologies as have been out of office and tardy in responding. I hear your concerns but I am not involved in the policy and its implementation. Please continue to work with your school team and the Public Health Nurse to address your daughter's needs.

Thank you and take care.

I would like to, but the policy doesn't seem to allow for the implementation of that.

Paradise Lost

I never dreamed that 4 years ago, when we were fighting to keep Lily alive and healthy- that today I would be fighting to keep her in school. Part of me says keep her home, put her in the K12 internet academy program and just have fun with her because if we have learned anything in this journey:life is too short and too fraught with sickness to take it for granted. But Lily loves people and school. She cried when I told her she might have to start school later than her brother and sisters. Not because they might get to go, and she might not- but because she was supposed to see Mrs. Saba at recess and say hi to her and learn all kinds of new things in her new class. She has been practicing addition all summer because she heard they were going to be learning that in the 1st grade.

The Economy is Failing. The School systems are failing. Our overall Health as a country, failing. Yet one of the reasons we choose to stay in this great country is for the freedoms. We are guaranteed a free public education. We are guaranteed in theory, Life, Liberty and the Pursuit of Happiness. When you face cancer- you learn that life is not in fact Guaranteed. All health institutions are not created equal.  You are at Liberty to choose which institution you go to, especially if you raise your personal debt ceiling as far as creditors will allow you. Then you will owe creditors everything for the rest of your life. But you get a parent with a child whose life is threatened and you have an individual who will do anything so their child may pursue a lifetime of happiness.

The school system in Hawaii is inferior. I can not precisely tell you inferior to what. My children have attended school in this system for a collective 7 years. With 2 exceptions, they have had very good teachers. 2/7- less than 30% is not bad odds. All 3 of the kids teachers last year were not just good, they were great. Lily's teacher helped us address her quirks and gave her responsibilities in the class that suited her maturity level. Kiera's teacher took her from being below profiency in most subject areas- to being at or above all but one section!  Phillip's teacher gave them a well rounded curriculum, over and above what the standards required and gave them the skills and confidence to face a new challenge , middle school. She kept in constant contact with parents, which is truly appreciated because most 6th graders are seeking independence and report their days as "fine".  I tried to convince myself that poor administrators were not as important of an issue- what really matters is the teachers. I found out this past week how wrong I was.

This past week I got a lesson in politics and the legal aspects surrounding the education system. Everyone is afraid of liability. Laws are in place to protect all those under the department of education employ- Hawaii has Good Samaritan laws that protect anyone from litigation if they are trying to help save someone's life. Last year, school health aides became Department of Education(DOE) employees and not Department of Health(DOH or public health) . Homer Simpson lives permanently perched in the forefront of my consciousness, oh the irony.   There was a very good reason that school health rooms were under the DOH- that is where sick kids go. DOH has nurses who have licenses to train aides and oversee their facilities. In an effort to save money, the DOE took over this position, because health aids do not have licenses- they could be paid less if their delegated responsibilities were less. So in order to do that, not only did the position have to change, the INTERPRETATION of legislation had to change. We call this policy. So the DOE changed POLICY last May that does not allow health aides to make any assessments, give any medications, store any medications or perform any life saving functions- EXCEPT 3.  Health aides are allowed to give an asthmatic their inhaler and call their parents. Health aides can give a diabetic child a Glucagon shot and call 911. Health aides can give a child with an allergic reaction, the epi pen and call 911.

I talked to the DOE, I talked to DOH. DOE says public health nursing says they cannot allow health aides to do any assessment. But wait- they can assess on the 3 aforementioned conditions? DOH says that DOE makes the policy, they just recommend what is safest for the children and medically sound and their policy does not allow assessment. But you just said the aides COULD assess in 3 situtations?  DOE wants to save money. DOH wants their nurses paid and their control was just taking out of the health room. I'm pretty sure I just dealt with this situation. Yes, yes I did. Bella wanted to use Lily's DS. Lily did not want Bella to use it because she doesn't know how and she will break it. Bella swears she knows how and won't break it that Lily just won't let her use it. SO if Lily lets her use it and Bella breaks it- she's out a DS- if Bella uses it and breaks it- it's chalked up to she didn't know better so she's not responsible. So I stepped in and made sure Bella knew she was responsible for her actions and her actions alone and if she broke it, she was responsible.  Lily was under no obligation to share, but it was the right thing to do. Sometimes the right thing to do is risky. Far too many people choose feigned security- because if Lily doesn't share- she sets up a precedent of not sharing. The next time Lily wants to borrow something of Bella's guess what will happen? And it won't be about this item- it will be about the DS. So I say 1) always do the right thing. 2) Look at every situation in the relevant context.

I don't care about the politics of whose job is whose and how the DOE and DOH departments dys-function. I am very sorry that someone did the right thing and got sued. I am even more sorry that our "justice" system allows such careless litigation to proceed. Americans are known internationally for being litigiously careless and overzealous all at the same time. We are the butts of many jokes. I got fat- sue McDonald's, I am unhappy, sue my employer, I was lazy -sue for discrimination, I had an idea- sue the person who worked to realize it, she took my boyfriend- sue her for looking like me. It has to stop. People are responsible for their actions. Legislation cannot absolve anyone from that responsibilty, yet we continue to interpret it as such. Unfortunately Legislators and policymakers set the precedent in all the wrong ways. Until common sense starts prevailing, we are lost. Until the people of this great country stop accepting injustice as part of the policy and assuming it can't be changed- it will not change. I would love to spend my time pursuing personal happiness, but I am going to pursue change. The future starts with our children and the children of this country are being set up to fail by the laws and the policymakers who refuse to be held accountable. Write your Legislators. Write your Board of Education. Let the Governor know. Next time you pay a tax- think of all the people who use that money to provide a disservice to you and hold them accountable. When someone does the right thing, excels at their job and accepts responsibility- let's give them the praise and accolade they deserve. You will find there are so many out there doing the right thing and fighting too. There can be change. It can always get better if we make it so.

Dealing with Discrimination In the Hawaii Public School System

Two days ago, a quick search on the KeoneUla school website for a copy of the school supply list turned into a veritable shitstorm. I could not find the list. What I did find was an announcement tucked away in the bottom of the page about school rezoning that will take place. As of next year, Lily and Bella would not be allowed to continue at the school they are at, despite a grandfather clause issued 2 years ago allowing them to start and complete through 6th grade there. When the initial anger subsided, I realized despite the fact the school has new facilities- they are not taking advantage of them. The administration is noncommunicative, narrowminded and at times rude. It is not a school that I wish to continue to fight for. I volunteered over 20 hours last year, often with Bella at my side assembling binder folders. Not helping kids read, do math, science or social studies, or teachers with menial tasks to save them time- but squirreled away in a room gluing and taping folders together to put in binders- despite the 8 administrative assistants that "greet" you in the office. And by greet I mean look at you when you walk in. I was done. I was ready to pull my kids. I went into the office and was greeted and asked admin assistant #1 if she knew anything about the rezoning. She did not. Of course not- the employees of the school have no worries about their kids or neighbors kids who they carpool in with being excluded. I spoke with the Vice principal who did not have details but said they were not really giving geographic exemptions out to stay in the school. So I have to figure out how to get 4 kids to 3 different schools within a 10 minute period next year- nice. Not gonna happen. I take 3 GE forms. Not to stay in that school, but to apply to a school we went to years ago before this school was built.

I call the Principal at the new school- leave a message and send an email. I let her know Lily needed medication and our situation and inquired about availability. Within hours she had called me back and sent an email- there were exactly 3 positions open in the grades I needed.  I sighed relief.  But it was only the calm before the storm. I got a call from our Public health nurse that afternoon regarding the new policy for Lily's medications at school. They wanted to schedule a meeting for 9am the next day- could I make it on short notice? It looks like I don't have a choice if I would like to get Lily in school for Monday and once again Phil can't be there because he is TDY.

Last May- the very last week of school I receive a letter that explains the change in Department of Education Policy for storage and administration of medications at school. I went into the office to see how I could proactively get this settled before the new school year. I was told to talk with my child's counselor- I did and I left a message- but technically no one could do anything until they went back to work at the end of July. So I was on hold.

The new policy only allows for epi pens, glucagon and inhalers to be administered at school. Yes diabetes, allergies, and asthma are the most common medical conditions in the public school population, but they are far from the only medical needs. Health Aides are now DOE employees, not Department of Health employees. They take a course, they can administer the 3 meds listed, they cannot assess any child's condition(which is utterly ridiculous- because in order to give any of the 3 listed meds- a precise assessment and evaluation needs to be performed). Only Licensed nurses can give meds, but the policy doesn't allow other meds to be kept at school. Each Complex(similar to mainland school districts) has 1 school with a school nurse. Therefore the DOE is saying that any child who may need medication other than the 3 listed, IF they approve the medication- which now can ONLY be done by procuring a 504 plan- that child with special medical needs can ONLY go to the ONE school in the complex that has a nurse. So on the very day I learn that my kids will only be allowed to go to the school they are at this year, I learn that Lily may only be allowed to go to one school in the district and believe me not all schools are created equal. I start formulating my remarks for Lily's 504 needs evaluation the next morning- knowing full well that the school adminstrator who neither respects me or my children has the power to fight for us and she will not. My only hope is that the principal of the new school was was welcoming and accomodating and willing to work with us on Lily's special needs, will.

My stomach is in knots. We fought and continue to fight to keep her alive. The life expectancy for a kiddo with her tumor is 25 months to 5 years. I ignore that. I plan life like she is a normal kid and has a normal life expectancy, otherwise you go crazy. She is on medication she will be on for the rest of her life, that is her normal and we treat it as such. In the case of an emergency, she needs extra medication or she will die. I am trying to give all my children the best oppotunities I can with the resources at my disposal. Making people aware of her condition is for her safety, I do not want her treated differerent on a daily basis. I give her Hydrocortisone daily at home. She does not require daily meds to be given at school. She is bright, meets all but one proficiency level and that one is the ability to show numbers in a variety of ways. I laugh because for Lily- it's her way or the highway, so that totally makes sense. But IF she gets a fever, starts vomiting, has diarrhea or a traumatic injury- her body will not produce the extra hydrocortisone it needs to sustain life function. That medication has to be given in that situation. Reference last May's hospitalization due to the flu.

My neighbor coerces her husband to prevent our collective 6 children from harming themselves or their worldly possessions and she comes along to the meeting as Lily's emergency contact and my transcriptionist. We join the meeting- the principal, counselor, student services coordinator and public health nurse I have worked with before. There are 3 other representatives from the district there as well. We begin discussion about Lily's condition and her needs. They are simple in my eyes, she needs medication at school for the case of emergency. She needs someone to give her the medication in the case of an emergency. And that is where the simplicity ends. It is repeatedly reiterated by the principal that the new policy will not allow anyone to administer this medication. Um yeah- that's why we are in this meeting to evaluate if a 504 plan can help and if Lily is eligible for it. Fortunately I wasn't the only one in the room who was under that impression as the District ladies suggest doing the formal eligibility paperwork before we discuss anything further.  Since Lily had cancer and her current medical need for emergency medication is life threatening, she qualifies for a 504 plan. I knew this because after she was denied eligibility last year, I did my research and found out that they should not have denied her. I was much better prepared this go around- chalk another one up to experience being a great teacher. I did not appeal that decision last year because the plan was worked out and the school agreed to keep her meds and the health aide agreed to administer them.

Now comes the tricky part- deciding which accomodations we are asking for under the 504 plan. We can ask for whatever, but the final decision lies in the hands of the DOE and they can refuse it or accomodate in a way they see fit. To me it is clear cut- I want her meds kept at school and I want to be reasonably assured that someone would give them to her if the emergency occurred. I know that under the current policy the health aide cannot be required to. But the good samaritan law protects any volunteer who would rise to the occasion and save my child's life in the unplannable event that there is an emergency. I would hope that at a minimum someone would be able to call 911 and let them know Lily's condition and even though they cannot dispense her medication- at least have it on hand to know what she needs. I have 3 doctors letters explaining the condition and the necessity of the medication in different ways so that everyone could understand it. I show them the emergency kit I have prepared, with the shot, the pills and the index card with step by step pictures how to administer it provided by the NIH. I show them the Emergency care plan that I prepared for all Lily's teachers, the health aide and staff last year- it has her picture, her condition, all of our emergency contact numbers and a 6 box table that states: if you see this(fever, diarrhea, vomiting, etc) Lily needs this(pills, injection, 911). And I plead with them. All I want to know is that some concerned staff member would take resonable steps in an emergency to help my child live. I have worked too hard to keep her alive for her to get sick or die when it can be avoided. Accidents happen, kids pass out and sometime preexisting conditions are found after it's too late. Everyone always says- if we had only known. Well in Lily's case we know and it can be avoided. I find it hard to believe that in a school with 80 some employees- not one person would be willing to volunteer to save her, that they would stand idly by with hands bound by policy or administration and watch a child die when life saving medication is at their disposal. If that is truly the case- this is not an institution any child is safe in. The principal points out that an accomodation should be made to allow Lily to make up school work due to absences. The DOE employees are incredulous-that isn't the policy at your school already? I get a wink from panel member. Right on, I am not crazy in thinking that was a stupid policy. But then again, I'm beginning to think I have yet to find a policy I like.

The meeting takes the ugly turn. Debate over how the policy is written ensues. I point out it does not state that no one can volunteer to give meds. The principal says she would not allow anyone to volunteer. You would not allow someone to volunteer to save a child's life when you know what they need and have it on hand? I would not allow it, she says. Let- me -be- clear- on -this. If the DOE ALLOWS her medication to be kept at school and she is laying passed out at your feet and someone volunteers to give her life saving medication- you would NOT allow them? I would not allow them, she said. In the collective silence of  the room you could hear both my heart break and the angry lava start to spill out as my neighbor shook her head and mouthed- it's over.  Nope- It is just beginning.

The principal has the audacity to continue about how at her school it is always safety first and that since they cannot meet Lily's safety needs, perhaps we need to have her go to Ewa Beach where they have a nurse. I hope to God no child ever passes out or is severely injured at her school because she would not allow them to be helped. Unless of course she would just not help Lily because of her condition, but that wouldn't be possible because that would be discrimination and that is not allowed. There are policies against that.  And if she truly would only act in that way because of policy then the policy itself is discriminatory by nature and also wrong. The panel would like to discuss what we do about Lily. She cannot start school at Keoneula Monday because a plan is not in place and the next 2 "work" days are furloughs. Someone points out that I should discuss it with the principal in private. I agree, that would be a better opportunity to have her sign the GE because even IF they met Lily's needs- I would not allow my children to stay at that school under her watch. That is no longer an option.

I step into her office, tell her I called Iroquois Point and they have spots available and could she please sign the GEs for my children. She bumbles around not knowing where to sign saying oh, IP is a good school, they are IB too. She tells me that she is sorry. I already knew that.

Taking Shots

When Lily was diagnosed with adrenal insufficiency, we were given the emergency kit. A vial, a syringe and instructions. Phil got the initial instruction and relayed them to me in the style o Pulp Fiction. To this day he doesn't understand why I don't keep a sharpie in the kit. Because you don't give the shot in her heart, dear. That isn't to say giving the shot won't get you in the heart. Because when you have to give the shot- you are in dire straits.

So we have had the stomach flu. The good old fashioned, getcha every which way with a vengeance virus that makes you question your mortality and leaves you praying to the porcelein god. It was like clockwork. Phillip was the first hit and I questioned whether or not it was pancreatic insufficiency or just a tummy bug.   A call to the doctor earned Phillip an extra emergency shot and me a lesson in advanced diabetic treatment. Usually ketones are high when blood sugar is high-you solve the problem with an insulin shot. SO what happens when a diabetic is sick and ketones are high and blood sugar is low? Problem #1 You get a lesson in advanced diabetes management. So you have to mix up this special glucagon shot which tells the liver to release all the sugars it's got stored. But since he was sicka nd not unconscious- we only needed part of the shot. Yet since he uses an insulin pen- we were never issued syringes- problem #2. A Dr. Pepper later and a trip to the drug store by my neighbor- procures us the correct equipment to treat the problem. Lesson learned. Then 2 days later- Lily gets it. Narrowing down the field of suspicion of causes- yet her immunity is questionable as well. I worry that she is having insufficiency issues. I don't get the luxury of dismissing a virus anymore as just that. For both kids, regular sicknesses can rapidly become medical emergencies.

SO Bella starts puking a couple days later. I have now confirmed it is not a bit of bad luck for the insufficient children- and by insufficient I mean Organ-izationally challenged- not that they aren't good enough. I get her on the mend and we have a whole day with no one puking. The next morning Lily wakes up with a tummy ache. Lily frequently has tummy aches- when she's hungry, when she has to go to the bathroom- when the wind blows. I take them seriously- but I also know they are fleeting at times. She tells me she threw up. I am about to question the authenticity of this when she dodges into the bathroom again. Lovely. I think this technically makes it an epidemic.

One of the problems with adrenal insufficiency is illness. The adrenals boost cortisol production to help keep all the systems in check- blood pressure, heart rate, fluid balance, temperature. Mess with the system and you get one sick kiddo. I know the importance of getting Lily to take her meds and keep them down. We try two more times. No luck. I break out the stores of her chemo anti nausea meds. I wait 15 minutes and try her hydrocortisone again. I even mash the pills up really fine and dissolve it in ginger ale. This time it stays down a half an hour which is on the cusp of counting. We are approaching lunchtime. Since she is sick, I need to triple her dose and I am not even sure she's had one. I call the doctor's office. No answer. I get distracted by repeated pukings. Next thing I know- it's almost 2. We have reached emergent status- I page her endocrinologist. We are into needing the shot territory. As I call, Lily starts to get sleepy. Her endo says get the shot ready. 2 months ago I would have been unsure of this process- but due to the presence of a certain diabetic in the household- shots are kind of a regular thing. A sardonic thought crosses my mind- everything happens for a reason.  I call my sitter over to help hold Lily, it isn't too necessary as the shot is quick. I know she needed it because she hardly cries. Her endo calls back immediately and wants us to head into the ER. 

We go. I hate the ER. I hate the necessity that brings us there. I hate feeling like I am faced with an indomitable challenge-parking at Tripler, or just day to day life anymore. There is one of me, 4 kids and my husband is TDY. The night before he apologized for leaving, I promised we'd be ok after all we needed the healthcare. Way to jinx it.  It's not a good feeling. I would stop to cry or maybe even scream, but there is no time. My phone rings- it's the husband. I am too tired to pretend we are OK. He promises to be on the first flight home after training the following day. We'll be fine, at least that's what I keep telling myself.  We get triaged and her heart rate is very high- 137. She is in crisis. They give her more Hydrocortisone. Lesson learned- I waited too long on the shot. This run of the mill virus just bought us a stay at Hotel Tripler via the better safe than sorry passage. Another night of no sleep. But that's nothing new- the accomodations are just less comfy to toss and turn in.

The nurse in the ward is new, but has been told we know our way around. She covers her bases while not going into overkill on the welcome. I laugh at having to fill out the admission questionaires- the qc for the floor to see if we can be trusted. She apologizes, but it's protocol. I fill it out joking along the way- when it asks for social support system- I put Facebook. I put a line through it with a smiley face and put the obligatory family and friends. Last time Phil was there the last question asked if the hospitalization was normal. He wrote truthfully- despite that we have done this dozens of times- it is not nor ever will be normal. I was looking forward to writing my own quippy response, but the question had changed- it was now- Will this hospitalization affect your lifestyle Yes or No?  Seriously. Yes it will flipping AFFECT my lifestyle. What part of hanging out in a small cell with beeping equipment, iv lines, routine vital checks, invasive procedures and barrage of parading residents says lifestyle of choice? That disregards the reason for being here- the serious illness of my child. Something I have little or no control over here. If something beeps at my house- I turn it off. If someone knocks on the door and I don't want to visit- I don't answer it.  I don't catalogue every ounce of urine my child excretes generally speaking- because it's gross. When people talk about living the glamourous life- none of those things are present.

When I am convinced Lily will live, I start pestering docs to order her CT scans inpatient style. No sense in me coming back in 2 days, making her not eat - when she currently has nothing in her stomach and is right there. Apparently this is something akin to an act of congress. But we make it happen.  It takes me, my mother in law, my neighbor and finally Phil all putting in requests each time a doc walked in the room.  These tests that a week ago, I agonized over. Tests that I worry will damage her already fragile system. Yet through all of this- I find a stasis. No news is good news. The CT doesn't reveal anything urgent. We will wait and see in 3 months. It is the best answer I can hope for. It is our new normal. Waiting and seeing. I don't know what it means- I don't know what 3 months time will do. I know that a lot can happen in just 2 days and we'll take it a day at a time. We take some shots along the way- but I found out I can give them too.

worried sick

Sick kids should not be in school. Sick kids need to stay home and get better and more importantly not infect me and my little petri dishes with their vomitude. A runny nose , does not make one "sick". It may make one "allergic" or "runny" but that too unless accompanied by another factor does not count as an exclusive reason to be kept home from school. SO what of a runny nose AND a vomiting episode, you might ask? Well experience tells me that in a child with a diminuitive gag reflex- such factors are so intrinsically linked that they should really be counted as one then therefore NOT and exclusive reason to be kept home from school. The only reason I would keep them home from school under these circumstances was to prevent them from having to lounge in the healthroom, picking up even more vile illnesses, which has happened now- twice.

The problem with routine illnesses is not that they are present- it's just that all of the sympoms mimic those of acute conditions or induce acute conditions in special endocrine patients whom reside in my home. Which is to say- life becomes and hour by hour mess until crises can be ruled out and virus ruled in. Lily has been coughing for a week or so. One of those allergic, postnasal drip annoying, gets worse when she lays down kinda coughs. Non productive, no real runny nose, no fevers- until Monday night. The night after her sedation for her scans. The night after her doc called to say there's a blip mid femur we would like to take a better look at with another CT and xrays.

So now the worry sets in again. Like it ever leaves, it just burrows in the recesses of my mind - a cancer in it's own right. What if the nagging cough is a symptom of something more- the first sign of a bigger issue? I am so tired. I haven't slept a full night in months. I try to push the worries back and duct tape them to the corner with the knowledge that we will take it as it comes. Nothing is permanent. The worry is as damaging as the radioactive rays they want to bombard her with. I need to find the positive and harness it. Many would turn to prayer. Yet their God is on my shit list once again. Part of me feels that by the destruction of one of the major sources of evil in the world has again tipped the delicate balance of good and bad. I don't see why a 6 year old has to take on any bit of evil when there are puppeteers available with too much good. The balance seems entirely out of whack.

I don’t want her to have unnecessary radiation exposure, but environmentally we are bombarded with it anyhow. Yet the manmade versions frighten me so much more. I can’t explain it- I just know it to be the case. I guess it’s written in my DNA. Or mutated into it anyhow. I would really like to not deal with any of it. But it won’t go away if I bury my head in the sand- it might- but the fear of it getting worse chains me to this progression of events. I’m not ready to throw up my hands and let cancer win- nor am I willing to give it ammunition.

Lily's 6th Birthday!!

We took a trip to the Big Island and celebrated Lily's 6th Birthday there. We got to see the sights, bowl a little and most of all wish Monkey 3 a very happy birthday.

Cupcakes for Class

It was so much fun to make and take cupcakes to Lily's class to celebrate her 6th birthday. Since her birthday falls on Monday next week and the kids are on spring break, I didn't want her to miss out. The kids were so excited, it really is contagious. Bella seamlessly blended herself into the environment and even got to take part in a rousing game of Head's Up 7-Up. What was even funnier was watching Bella systematically put down a whole table's worth of thumbs and no one guessing it was her- despite that she was wearing her rainboots and clopping around like a Clydesdale. I love Kindergarteners and that the effect of sugar on their little systems is akin to crack. Much better to sugar them up and send them home!

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Long Term Effects Of Cancer

A friend just posted a quick note about seeing a mom with a lil cancer fighter on her lap at a school event.  I am not surprised by my reactions, yet they always surprise me. It was equal parts saddness, nausea and relief. I am sad that any child, any family has to go through it- it is not fair. Sad because I know how serious it is and if the disease doesn't kill them, the treatment might. I was nauseous because it's still fresh enough that I remember the stress, the day to day ups and downs and the gut wrenching emotions that you shove to the side just so you can get through.  I feel a bit guilty to admit that I was relieved. Relieved that the memories that sting now are just that- memories. I actually don't think about the what if's too much. Just enough to formulate a basic survival plan- but the thoughts themselves are toxic. 

When people we haven't seen in awhile run across us- the first thing they ask is - How's Lily? Lily is fine- it's the rest of us you should worry about.  I see friends with their out of control children- hopped up on psychotropic meds for behavior problems.  They are searching for answers in all the wrong places.  I think back to a time when I had to choose the lesser of two evils- taking the chance on cancer killing her or the chemo meds and their long term effects. One drug causes deafness, the other heart deterioration, the other can cause secondary cancers, the alternative- death. Not much of a choice. Yet I know parents who have their kids on cocktails of meds because they won't sit still or listen to them. We won't mention how the protocol for treatment requires CT scans every three months and by nature of our genetic mutation- the radiation from the CT should be avoided if at all possible.  Catch 22 much? 

I asked the doc once about long term affects(effects?) potAto pOtato. His response in a nutshell included the pearlly wisdomous bite- not many kids with this tumor type survive long enough to gather data on the long term effects- there will probably be some.  SO when you look at it all big picture-style- you come away being grateful for the imperfect package you have been given- because some people don't get a package at all and you know darn well far too many get awesome deals and don't even appreciate it.

 I sit here watching Lily play Barbies- Currently Barbie is getting ready to invite Ken to her Birthday party. They will fawn over each other, they will dance and then they will kiss.  Her Barbies seem to do that a lot. I started to worry. Is it hormones, should I get her checked- is she having changes?  And then I thought about it- when I was 5. And 6, and 7- Barbie and Ken(which often times was substituted with a GI Joe doll or a more appropriately sized A-team type doll) I re enacted their wedding scene over and over and over. I saved for months to get Crystal Barbie. Her dress looked just like an iridescent shimmery wedding gown. They would wed- go to sleep and in the morning Barbie would have a baby. For awhile it was a mini Baby doll and then finally I procured a miniature baby out of a gumball machine. Really- how important is scale in make-believe land? The point is- she's playing normal and despite my first instinct to over react- I take a minute and realize we are on an appropriate course.

Sure she can be rambunctious. And she can be manic. And she tends toward roid rage at times. But I remember Lily was a less than happy baby with rage tendencies.  Everyone else thought  she was so darn cute and happy, I consider the fact that maybe it's just her personality.  Yep- we called her Bi-polar baby. I admit it.  But there is no way of knowing the cause. I don't even know if it's that important- if I spend less time worrying about a cause I had no control over and spend more time teaching, mentoring, and behavior modifying- the result should be a functional bipolar child. We spent all this time worrying about her being normal, acting appropriately, and fitting in when it occurs to me- all 5 year olds are Bipolar. They are ADD. They are ADHD and oppositional defiant. They talk to people no one else can see, or hear. The have intense moments of rage and/or depression over trivial items.  And they totally sing off key. Lily's missing the higher frequencies of hearing- what are the other kids' excuses? By all counts- not only would they get booted from American Idol in the first round- according to the DSM IV they should all be locked up in maximum security psychiatic facilities for observation. I don't think the kids are getting crazier- I think we are.

The daily reminders of our year long vacation at cancer camp have become routine. It is more like brushing teeth now. They're about as painful too. Lily- did you put your hearing aids in? Lily ,did you hear me? PUT YOUR HEARING AIDS IN! Even the kids chime in- what are you, deaf? Everyone laughs. Especially Lily.  Just like we laugh at  Kiera's weird thumbs and Bella's ginormous butt and Phillip's abnormally long torso. None of these things can be helped, they are who they are and at some point in their life someone will point it out. Now they can laugh at it and move on. It's not so much about making your kids perfect- it's about helping them love their imperfections. Do I love my big butt- well not always- but I found me a man who does....and that my friends is how you deal the cards you are given.

Everyday Lily takes her meds and I have to prepare her school and our friends for emergencies. I didn't think about her playing soccer and the can of worms it opened regarding potential injury. Not only can she not wear her medic alert bracelet at the game- she might actually get hurt. Her coach works 3 jobs and coaches 2 teams- so practice has been less than consistent. Before I know it, we are to our first game and I never told anyone that she is adrenal insufficient. No one knows what it means and it's a bit of a pain to explain and when you start mentioning unconsciousness and shots- people get all wonky. I tackle the conversation with the team mom and she is very supportive. I never plan on sending Lily to a practice or game without my being there, so it's not a problem. Until her first game. Which fell on our anniversary.  We are going to be on the other side of the island. I have all the ducks in a row, everything is in place. I nervously text our sitter every 5 minutes during the game. All is well- she did great- she scored 3 goals and had fun. When I get home, we watch the 13 seconds of video from the game. It's not a lot- but I rank care and keeping of the magical creatures over photographic evidence- and all were cared for very well. I notice that she skips up and down the field. I laugh- because that's Lily. La de da. I show Phil. Yeah-he says- I noticed that at practice too. I wondered if she would have any long term effects from the leg surgery- but she adapts well. 

It hits me suddenly, I hadn't even thought of that. I am overcome by the familiar feeling of saddness, nausea, and relief.  I hadn't attributed the skipping to her leg surgery. I just figured it was her. Maybe it is.