Please help.....

I need your help with my current predicament.  

You see, in order for Solstice to have limited pain (she will never be without again.) She needs twice weekly injections of a very expensive drug.   There is an alternative to said drug, which is male hormones.  Yes, the kind that will de-womanize her.  Exactly the type a budding transgenger might buy on the street in order to reverse the effects of puberty. The ones that will change the voice she has worked so long and hard to train. Remove chemicals that make her a woman, this is the insurance company’s suggestion of how I am to treat my 14 year old daughter.

The reason of course, is money.  The cost of the drug that allows her to be the beautiful woman she is costs $300,000 a year.  The hormones, well you don’t need the numbers to do the math. 

Now add on the fact that we don’t have an OFFICAL DIAGNOSES.  The reason the disease is so hard to diagnose is that sometimes it shows up, and sometimes it doesn’t.  It requires a very special test that has to be taken from the arm, spun and frozen within minutes and then sent out.  If she has had the magic medicine within 7 days, the test will show a false negative.  She hasn’t gone three days without the medicine in weeks.

Number of times test taken – 3 Number of times test messed up – 3

Now, every doctor that has had even a smudge of knowledge about this disease knows Solstice has it.  One major fact, is that the only way to reverse these attacks, is a drug called Berinert , hereby known as the magic medicine. This medicine will have to be her constant companion, because even with the maintenance medicine that replaces the proteins she doesn’t make,  she will still have attacks.  Less often, but they still will happen. 

   

 Not every hospital has it, they usually have to go find it and have it couriered to us, which takes precious time.  When she has an attack, she needs to have it a.s.a.p.  Less than four hours is ideal, and every moment it’s not in her body, is longer that the attack goes on and starts to create more issues, all leading to the ultimate throat closing which will could result in death.   

A person who doesn’t have this disease, would not at all be effected positively by the magic medicine.  With Solstice, it’s night and day.  Within minutes you can see the parlor of her skin change color, and the pain contractions lesson slightly.  When she has an attack, the right side of her face swells, her intestines grow, leaving her bloated, looking as though she is eight months pregnant.  She loses her entire inner field of vision.  She starts dry heaving non-stop, but never vomiting, as the pressure pushes everything up.  And she screams.. blood curling screams from the pain.  As it hits, it does so in waves, almost like contractions.  They start at 20 seconds and are as far apart as ten minutes, but if the pain receptors are not blocked, and the magic medicine is not given very quickly they speed up and become longer, until they are basically right on top of one another.  It is the most awful thing I have ever seen.  It’s essentially watching your child be tortured, and it doesn’t end until she gets the magic.  After several days of these attacks, even with the medicine.. the pain doesn’t stop.  It’s constant, until all the swelling goes down, and even then… it will always be in the background of her life. 

Add to that, one of the triggers for this disease is medicine.  I believe it has been set off Tylenol, ibprofren, benedryl (iv), and oxycodone.  I say I think, because these are the thing that she had prior to an attack.  I am in no way qualified to figure this out.  And, avoiding the triggers is perhaps the most important focus of our daily life..  When I arrived at Children’s Hospital I had two goals that I immediately placed on the white board of vital info about my child, under the heading questions.  I then asked the same two questions to every single person that came into our room.

You see, I have found two great doctors that are rockstars and they are working on helping us.  Neither however has seen more than one case of this disease.  Furthermore the first rule of being a patient is knowing that more doctors and more opinions only provides more knowledge and more options.  So this is what I needed, while we were here, to assure my child’s health. 

1)      Access to a doctor that knows about my child’s disease to help me understand, as there is very limited information available, even to a google queen like me.

2)      Pain management, experimenting with non-narcotic options to my child’s care in a safe environment, where the magic drug is accessible. 

By day two I was told several times that they understood my questions and were working toward a solution.

On day three when I broke it down more specifically to Dr. Seltz, when I said “What about Benedryl, it’s supposed to be on the list of things that help.  But when you gave it to her last night, she had an immediate attack.  We stopped the attack with Berinert.  Can we try the Benedryl while we are in the hospital, so I know for sure that it’s safe?”  As he had assured me it couldn’t be the Benedryl, it must be coincidence.  And then he said “No, she doesn’t need it here.  If she needs it at home, try it there.”  To which I replied:  “But I don’t have access to the magic medicine, if I try it at home and she has an attack, I can’t fix her.”  His answer? Ah, but she doesn’t need it right now…. And some shit about none of us liking uncertainty. 

I do have to say, they did send in pain experts that spent serious time with us, and helped Solstice understand the importance of breathing, and educated me on their pain clinics.  They walked her though ideas to distract, discussed acupuncture, good solid material for sure.  She will have a lot of pain in her life now, and narcotics are not the answer.  But in the end, do you know what my discharge instructions say?  Vitamins.  I should give her vitamins.  No magic medicine, no pain alternatives.  Just vitamins.  

My fabulous allergist, Dr. Andrews at Denver Allergy and Asthma associates attempted to contact my doctor throughout the weekend.  He never called back.  She believed that we should not be let out of the hospital until I was trained on how to inject Solstice with the magic medicine, and give it upon release, as Solstice’s case is so serious.  She has been working diligently towards approval with the insurance company.  She has also been in contact directly with the manufacturer to obtain this for a little girl who needs it very badly. 

You see, if my doctor had taken the time to actually understand the disease, he would understand how serious this is.  We were sent on flight for life to the main hospital because her throat was in process of closing.  And he just wanted me to try the benedryl at home, you know….  

He also made two separate references of an HAE attack being the same thing as a pain receptor attack.  I don’t think he even took the time to read a Wikipedia article.

    

So, right now this is where we are at:

1)       We cannot get her the twice weekly injections that control the attacks, unless we want a son.

2)      The only way to reverse an attack is with the magic medicine. 

   

3)      We have no idea what specifically the triggers are, thus no way to avoid them.

4)      We have no way to control pain, except vitamins. 

Can you perhaps sense my frustration?  I was so scared for so long.  We have been going to emergency rooms, getting the drug during an attack, getting discharged, and then causing attacks with the drugs they give us or tell us to use to manage the pain.

We’ve known she needs to be hospitalized and watched for weeks, and finally they do… and then they don’t lift a finger towards my goals to keep my child safe.   Am I out of line to have these expectations? 

That is my child.  The one I birthed and raised mostly on my own her whole life.  My one real purpose in this world is to keep her safe and when I beg for help, no one hears me.  I’m just one crazy mama.  

Well, that’s where you come in.  Just come here and read sometimes, and tell people about this blog.  If you know anything about this disease, please contact me.  The only way I can get anyone to listen is if you first listen to me, show that you care about Solstice, and prove that one crazy mama has a voice.  

Just imagine the moment after we were told to go home with vitamins when Solstice looked at me through tear streaked eyes and said:  "I thought the hospital was going to help us, that we'd finally found someone to help me?  why won't anyone help me?"

How to survive an illness of someone you love

This is an example of my instinct preparing me for the future.  I wrote this a few months ago to a friend, but it turned out to be exactly what I needed to get through this time.  Funny how things work that way.

  Dr.'s and nurses are not your friends. Never get too comfortable with them, but treat them with respect..always greet them with the dr in their name, and even through in a few sirs or maams, and bring them greens from your garden, or a fresh baked bread (even if you just buy it) Make them like you, make them care, but never let your "like" for a physician stop you for a minute from trusting your instincts. Always, always speak up if you don't feel something is right, no matter how small it may seem. Change doctors, ask for new nurse....do whatever needs to be done, but do it with kindness and class. You know your child, you know her body, you know, and she knows what is right for you two.

 Don't let anyone else tell you how to feel, or what to do. If people give you advice, take it if you want and leave it if you don't. Bad life sitations bring out really good in some of your friends and relatives, and really bad in others. Start now learning how to laugh about how stupid people can be, so when they eventually make hurtful comments, you can laugh about it, instead of cry. It's a long road just with the doctors and the decisions, don't let people watching the situation get you down.

Ask for help when you need it. Just do it. They only reason I am successful is I ask people to do things all the time. I create things because I'm not afraid to ask for anything, it's my most powerful skill, and it's not hard, it doesn't make you weak.. it makes you stronger. If your house is a mess and you can't get it done. Ask someone to do it for you. You don't need to be cleaning when you could be sitting in bed with your kiddo doing crosswords. If you need someone to bring over meals, ask... people want to help. They want to give their time, they just don't know what to do, they have all this emotional energy and it can be used for good or evil, use it in your favor. It's a gift to the giver sometimes to ask for help. Just remember that some people will offer, and will not come through. Don't let it get you down, just file it away and don't ask that person again. If people make you feel bad, just don't talk to them for a while. Like I said, some people are really good with the bad, and others really good with the good. It's only us of higher focus that actually try to be good all the time. They are still your friends, it is just something they are incapable of... it's not that they don't love you.

 Laugh, be silly... ..make a fool of yourself, if it puts a smile on your child's face, its the perfect thing to do. Once you get inventive, its fun to see what you can find in the drawers of a hospital room. I've created sculptures, figuring out what else you could use that impliment for is a good one... Do extra things, like post a note on reddit/r/cards and ask people from all around the country to write a note about their first kiss (make sure you have a po box), or when you see someone performing in the hospital in the kids wing, ask them to come to her room and do her favorite song. Play people watching games, as you will be spending a lot of time waiting. You can play things like "mistress or daughter" or "mail order bride" or first person you see with glasses on their face and their head, or whole family's wearing crocs... or count the number of times that a teenager flicks her hair with her hand... strange things to keep your mind off things... it can be a lot of fun. Find a card game you really like and play for silly bets... like who has to go to the nurse station and ask for strange items or speak only gibberish to the volunteer at the front... whatever you can do to laugh.. laugh.. laugh...

 Spread kindness, like wildfire. If you see someone who needs a hand, give it (so long as it doesn't overburden you). Don't hesitate, don't get lost in your own issues. Help others, it will make you feel fantastic, better than anything else does. And it will make asking for help when you need it, so much easier in your head.

The Blanket Party

Special thanks to the Children's Hospital Association of Volunteers for this beautiful blanket they made for the Solstice.  She was too sick to make it downstairs today, but they were nice enough to send one up.

These brief moments of smiles and smart ass remarks between the bad moments of pain are the best parts of our day. Thanks for the blanket, but even more so for the smile you inspired.  Tear ridden smiles... but a smile all the same.

The pain team

We were just visited by the Acute pain team at Children's hospital a few moments ago. We were lucky enough to get a place with a physician who will help integrate narcotic pain therapy with meditation and tools to help Solstice survive this very painful disease.

 You see, what happens with HAE is the proteins don't make the right stuff, so the liquid that is suppose to be in her veins, get stuck in certain tissues. One main area for Solstice is her tummy. Her stomach intestines and tissues are so filled with fluid that at times she looks 9 months pregnant. This is extremely painful, and we don't want to treat everything with narcotics.. obviously the goal is to manage the pain without creating a fourteen year old junkie.

As a mother, watching her writh in pain is too much to bare.... far worse than having it yourself.

Invisible

Meet Solstice.  She's 14 years old.  She loves to ski, sing, and write songs.

A soulful child, I often explain to her the only reason I can think that she ended up with this horrible disease instead of that asshole down the street is:

"You were given too many gifts, somebody had to even the score."

She's a particularly thoughful child.  The one who always helps out the little guys.  She has an intense moral structure and feels things deeply.  A talented song writer and singer, well.. please see for yourself.

Children's Hospital Day Three

I can't start at the begining of our journey, as we are already a little way in.  Day three is where our story begins.   My 14 year old daughter Solstice was diagnosed with hereditary angioedema three weeks ago after what seems like a lifetime of unexplained pain.

Just in the last three months we have seen over twenty doctors and every single time the tests have come back normal.  She's had cat scans, x rays and a MRI on her brain.  Every time we saw a negative result it was a sigh of relief paired with frustration..  The doctors said nothing was wrong, yet she hurt so badly. Waking up nearly everyday with stomach pains that would leave tears in her eyes.  She had a period of a week where she couldn't move her arm without intense pain.  Nearly a month and a half of crippling pain in her knee.  Then her face started to swell.  Just a little, at random times.

The emergency room said it was an allergy, the allergist just shrugged her shoulders.  I've seen three or more doctors gathered around my child, studying her, just perplexed, rubbing their chins in confusion.  Then I changed family doctors to Dr. Shannon Hill at Broomfield Family Practice.  She was a DO, which stands for Doctor of Osteopathic Medicine.   I didn't pick her for her qualifications.  I chose her because of her reactions.

You see, each doctor, after their tests showed negative... every single one of them mentioned "the strength of the human brain" and that perhaps my next stop should be a therapist instead of another MD.  But when Dr. Hill gently said this to me, I looked her in the eye and said  "I know my child is manipulative, she after all was raised by me, and I've had to depend on my people skills for survival more often than I'd like to admit.  But this pain, it's real.  Please help me.  And instead of shaking her head as everyone before her, she looked me in the eye and said "Okay, lets go".

We worked together for close to two months, both researching tying to find an answer to the Solstice puzzle.  She got all the tests approved, then got them approved again to double check.  I started testing for radon and mold at home.  We did heavy metal tests, lyme disease, mri's, anything that would explain.

Then one day we had an appointment, and Solstice's face was particularly swollen.  She walked in the room and said  "Oh my gosh, I know what it is."

That was three weeks ago, since we have been in the emergency room 4 times and have been here at the hospital for 3 days.  Even now, in what totes itself as one of the top ten Children's Hospital in the country I'm pretty sure the doctor just called my daughter's momentary pain an HAE episode.  Which means that he doesn't even know what an HAE episode consists of.

That's the reason for this blog.  Because this disease is so rare, I want to find a way to connect with other mothers in my position, people who know something about this disease, doctors who know what is happening.  I feel like I'm at the wheel driving, and I don't have the tools I need to even start the ignition.

Until next time....