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?"