Next?!?

28 03 2014

Here I go, blogging on a stupid iPhone. But I’ll do it, this once. I’m feeling a strong pull to get some things out of my heart and into the world. (Maybe one of you blessed readers will send me a pretty computer in the mail to replace the one that a toddler decided to explore and destroy, thus allowing me to share with you in a manner that doesn’t drive me crazy from typing on a phone screen. A phone, people!)

I’m at a pinnacle of sorts. On an edge. A razor’s sharp, shiny edge. One wrong move and I fall hard. Or slice myself right down the middle, from crotch to head? Maybe that.

I came home from the hospital again seven days ago from yet another week-long stay. I’ve stopped writing about my attacks because I doubt they’re interesting to anyone anymore, yeah? But for those of you keeping track, it was a grand total of something like 7 visits (totaling about 48 days or so?) in 2013, and 4 so far for 2014. Each incident brings a new complication, becomes a little harder to treat, requires more intervention. I lose energy, momentum, will, dignity, modesty each time. My body doesn’t feel like mine anymore. There are no more private parts. After as many catheters, monitors, enemas, scopes, and cameras as I’ve had stuck in and on my body, there is just no room for propriety any longer.

I’ve gone through being unresponsive with only 4-6 breaths per minute, prompting the rapid response team to race to my bedside. I’ve laid perfectly still and remained calm while they told me they were about to administer a drug to stop my heart, in the hopes of resetting it and breaking it from the pattern of supra-ventricular tachycardia it was stubbornly racing through. (Feeling your heart straight-up stop is terrifying and exhilarating and I’m almost inclined to recommend it.) I’ve experienced moments of searing nerve pain so excruciating that all I can do is beg the nurse to rip all the clothes off my body, so I squat on the bed naked, minimizing anything coming into contact with my skin, and cry, because no medications stop nerve pain. I’ve watched blood splatter out of my mouth during moments of violent hurling, from the gashes burned into my esophageal track after such constant illness. I’ve had two ports yanked out, and three put in, because after several months of hemetin and dextrose they gunk up and stop working, essentially threatening my dripped-in promise of life.

And these attacks have, for the most, fallen into a neat little pattern. About four days before my period is to start, I usually feel the first twinges of pain. Usually less than 48 hours from that point, I’m hooked up to my machines and pumps and I’m struggling to post squinty smiley pictures on social media to assure everyone I’m fine.

Seeing as the attacks are following such a tidy calendar, the obvious (to many) conclusion is to eliminate that calendar. Take away my cycle. Treat me with hormones. I started talks with one of my doctors, who happens to be a smart, quiet, funny researcher from NIH for whom I have a great deal of respect and fondness, about just this a couple of months ago. In my mind, we were talking about birth control pills (or a shot or IUD or something,) to stop Lady Flo in her tracks. But In his mind, we were not discussing birth control at all.

We were discussing menopause.

Dr. NIH wants to give me a hormonal therapy that will send me into post-menopausal hormone levels. He dropped a few printed pages of a study done on the treatment for AIP, and said “This is what we’ll do. Find a gynecologist to work with and we’ll get stsrted.”

Just like that.

As though facing menopause at 33 is something I shouldn’t take issue with. Sure! Let’s shut the ovaries down! Dry that silly uterus up! Gain weight! Get hot flashes and mood swings! Kill what libido remains after 5 years of chronic illness! Make sex painful and difficult! Get wrinkles and saggy parts! Turn old, now!

Of course it may not be quite that extreme. And I don’t mean to be offensive to women who are menopausal! (I’m being hyperbolic here because I’m sort of panicking, obvi.) I’m not one to say that I’m dreading menopause, WHENEVER THAT MAY NATURALLY HAPPEN.

But y’all. I’m 33. I’m not 100% sure I’m done with my baby making! I’m feeling (other than sick and exhausted and beat-down,) pretty bangin and confident, actually. I’d like to sit in this third-life space for a while, experience my thirties as a 30-something year old. Not having to worry about significantly increased risks of lady-cancers (I already have a greatly increased risk of liver cancer to work with,) with crazy-times mood swings and even less energy that I have now.

Maybe I’m making too big a deal of it .

Maybe I should do it and shut up .

Maybe I can’t.

This has literally stopped me in my tracks. Reading through the study has not encouraged me, with only about a 25% rate of good results, out of only FOURTEEN WOMEN , one of whom died… But if I don’t, then I am selfishly robbing my family of the opportunity to not lose their mother/wife/teacher/housekeeper/meal-maker/laundry-doer monthly to serious illness and recovery.

I don’t know what to do. Obviously I have more research to sift through, but it’s hard to come by, and no clear answers are going to glare at me from the pages of study summaries . I have a strong feeling that my decision will ultimately be made from my gut. The one that bleeds when it’s upset with me. My heart. My silly heart that beats too fast and too hard and needs round- the- clock monitoring at times.

This is the beginning of the end of living in denial for me.

You see, I truly have been in denial since I was diagnosed. You may not think I have, but I have. Each time I experience an attack , I think it’s the last (you’d really think I’d have caught on after the 12th or so hospitalization…) And I have a deeply buried belief that I am responsible for being ill, that my symptoms are not some disease that I innocently inherited through no fault of my own, but that are manifestations of my own poor decisions and sins. And that porphyria wouldn’t rule my life if only I didn’t let it.

It’s time to face my life head-on now. Either find a way to live peacefully with the disease , or find the fortitude (and energy and money) to fight the disease. I think there are merits in both. Validity in wanting rest and surrender and passivity, and validity in not wanting to roll over and settle for this as a way of life.

Which way will I go?

It’s time to make some choices.

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14 responses

29 03 2014
Ruth D. Bruno

Sabrina, I read your post holding my breath and crying. i was your age with 2 little boys when I felt the same anxiety and pain and bleeding you described. I had a “a total HYSTERECTOMY”. GOT BETTER AND NEVER REGRETTED THAT DECISION. I NEVER subjected my self to HEMATIN,or PANHEMATIN and I am 74 now. As a nurse I knew it was to contoversial. I still have attacks and no they are never nice but I am still a beautiful FEMALE, libido and all. Life will always keep happening we all age and it is OK. The hormones occur naturally, but any HRT, BIRTH CONTROL ETC. are steriods and bring on attacks. That is common sense. Hysterectomy just eliminates the on going use of those drugs. write me, call me please lets talk. I wish I could write the way you do, clearly you are a talanted young woman. If you do not mind I am going to a meeting here in Florida April 5 and will have a chance to talk to 2 of the “porphyria experts”. I really want to show them your post. Sincerely, Ruth D. Bruno.

29 03 2014
myswelldisease

Thank you Ruth. I so value hearing your perspective. I know I’m not the first woman with AIP to have this internal battle, and I want (and hope) to hear from others! I consider posts on this blog a complete open book, there for anyone in the world to read, so please feel free to share! Thank you!

29 03 2014
Ruth D. Bruno

Lets stay in touch. AIP is a mystery but maybe sharing and talking can help. You are such a talanted writer and I feel your frustration to my very soul. Ruth

4 04 2014
Leslie

Hi Sabrina,
I’m so sorry, I know words don’t really help but my heart hurts for you. I have Porphyria Variegate a Sth African strain of this disease and also an acute one. I have been suffering attacks and health complications since I was 18 and was put onto the pill, boy wasn’t that the wrong thing to do. I ended up being the 1st person diagnosed with this in Australia back then and I instantly became a Guinea pig. I tend to treat myself at home mostly because every time I’ve gone to a hospital no one has really understood it or known what to do and it is a constant battle explaining what my treatment should be when they want to start poking and testing. Anyway, I also went through a tough time with periods, I had an ablation done which helped immensely but it meant no luck as far as being able to have children. I don’t regret not having periods that would last 6 months and that was at age 35. I am now 48. I have just come home from having a bilateral knee replacement done, I went into an attack at hospital the 1st night and stayed in ICU for 2 days, I am doing ok though and healing well. I also agree with Ruth and have also never used Hematin or Panhematin and agree they are not something I would subject myself to either. I personally found the best way to deal with this is to make sure I have Vit C powder I have a teaspoon in juice in the mornings, I always have lots of glucose powder on hand and as soon as I feel my pain starting I start eating glucose by the spoonful till it subsides then mix a lot into a drink bottle to sip on the whole day. A new product called Colostrum MAX has been a huge huge help and one I would definitely recommend to you all. http://www.colostrummax.com/ it works on our immune system and also repairs so many things. I truly think the more intervention we have the worse we are, if we can self treat and also manage then the complications are far less. Your vitamins, sleep, food, mind, rest, all come into play, we are our own worst enemies! I too am still in denial, I never think of myself as having a disease, I have been diagnosed with fibromyalgia and also osteoporosis, I get skin lesions and blisters easily etc. But we are incredibly strong people, we cope with a huge amount of pain and still keep going, do what you feel is best for you, if you want another child do it and then look at having the hysterectomy as Ruth talked about, they might be able to do it at the time of birth to make it easier in a way. What ever we want in life is worth fighting for, I have lost my family to tragic deaths, been unable to have any children of my own and now I have to fake knees lol But we can do it all my darling, Please look into some of the things I’ve mentioned. I really feel it might help and get you off this horrible roundabout. Sending much love and light xoxo

20 05 2014
Allison

Dear Sabrina,
I am so sorry to hear what you are going through. I have HCP and am age 20. I’m exactly the same way as far as the cycle thing. For me it starts about 4 days before my period, as well as when I ovulate. That one is more of a smoldering, mini-attack and it only last like 3 days. I recently had my gynecologist talk about a hysterectomy (at my age!) because of what my Porph specialist told her. I so badly want to have biological children…..I refuse to have a hysterectomy now. I guess I’m in denial too, 😛 I know it’s coming every month but to me its worth it….even though I can feel more and more nerve damage after every attack. But for me in my life right now, it’s worth it. I sincerely wish you the very best and I encourage you to go with your gut 🙂

20 05 2014
wardwatch

You have to email me. I have just been diagnosed less than a month and came to your blog having googled ‘Porphyria’ and ‘smell’. I’ve only read some of your blog so far as today I don’t have the energy. But I will tell you one thing, reading it felt like I was reading my story and in a voice so similar to mine it spooked me. You have to look to alternatives to heam. I have already found people who can help. Don’t lose heart.

20 05 2014
myswelldisease

Just out of curiosity- why do you feel it necessary to find alternatives to hemetin? It’s not the best FOR ME prophylactically, but it does help a bit I believe, and it CERTAINLY stops an attack for me. Without fail. I know it works different in different bodies though, so what are you experiencing and who are you talking to to find hemetin alternatives? Always looking for resources!
I’m sorry about your diagnosis. It’s overwhelming. It’s probably, in part, a relief to you to know you have an answer to a lot of things you’ve dealt with in your life, but it can be scary and feel huge. Sounds like you’re already taking the reins though and staying in charge of your healthcare, which is really the most important step of all!

20 05 2014
wardwatch

Have you checked out Prof Steve Rochlitz http://www.wellatlast.com
Sorry, too tired to write a full response. Will write again soon. I need sleep. B

22 05 2014
myswelldisease

Ok. I’ll look into him. I think he may have been recommended by another reader as well.

1 10 2014
Sarah

Sabrina- I am clinically undiagnosed…we’ve moved several times during my sick years, so every time I start to form a relationship with a new dr. And they believe the porphyria NOS diagnosis, we end up moving again…which then leads to the next set of doctors wanting to run all if the old tests again and start at square one. Sigh. We are in the DC/Baltimore area, starting over again with doctors…
Anyhow, like clockwork, I can count on being sick and smoldering during ovulation and my cycle…before we even knew the word porphyria, my obgyn decided to start me on stacked birth control (I didn’t have a period for 1.5 years)…he took ultra sounds of my uterine lining and it took three kinds (stronger each time) to get it just right…but, for the first (& last) time ever, I felt good…thennn I had an episode and the word porphyria was thrown out and I had the testing done and we read that birth control could cause a false negative test result and I went off of stacked birth control bc I was certain that I could get a positive test result. I regret going off of birth control. I know all of the literature waves a giant red flag at birth control, but since mine were out of balance, this really really helped (we were basically blocking progesterone).

1 10 2014
myswelldisease

I’ve been with a couple of practices in the Northern VA area and can rec a couple of good hematologists. Remember- the trick is a 24 hour dark urine catch (meaning the urine is never exposed to light) and preferably while you’re symptomatic. If you do have it, regular hemetin therapy may help. I’m still playing with hormones. Mine are clearly out of whack. I’ve recently had a abnormal pap and am in the process of exploring that. Unfortunately, there hasn’t been adequate research on hormone therapy preventing porph attacks, so it’s uncharted territory. We just have to play around and see what works, and hope it doesn’t make it worse. If you’d like the names of docs I’ve worked with in the northern VA area (not too far from you!) let me know, and I’ll send you a private email.

1 10 2014
Sarah

Oh gosh I would love that!!! Are you still in this area?! My email addy is ringo loves me at gmail- all one word.
I think I saved some wise advice from a woman in a porphyria group regarding birth control and hormone control. I will see if I still have it. I actually started birth control bc when my symptoms first showed up I suddenly started getting this strange acne…my obgyn listened to my observations correlating my cycle and my episodes and decided To stack the pills to see what happened. I have to wonder if your pap results weren’t a result of out of whack hormones? Perhaps this is what will get the ball rolling on testing to see if hormone therapy helps you.
I see an oncologist here next week, and I just started seeing a dr. At Hopkins who has a handful of porphyria patients…but, again, it feels as if the diagnostic process is starting all over again in order to get help.
In the last state we lived In, I seized when I went in to have a cavity filled…then, in the doctors office while hooked up to glucose I reacted to two more anesthetics (that were injected for testing) and they pulled me out of my uncontrollable shaking with a bag and a half of glucose…anyhow, my tooth with the cavity that needed filled then, has now crumbled (serious) bc no dentist would treat me…so here I am in a new place trying to get a cavity filled, not helping my cavity bc of carb loading, and having a difficult time trying to come up with a game plan bc no one wants to take the risk since I react so strongly to local anesthetics.
Anyhow, all that to say, thank you so much, I would love any and all advice/recommendations you have!! Thanks so much for reaching out!!! Also, I’m so glad you found your music again…I’m an artist…we need that creative expression to breathe!!

25 10 2014
myswelldisease

I’m just reading this; sorry, been away from the blog for a bit. I’ll send you an email right now, so check your box soon!

8 02 2015
sofia

I just found your blog and have to thank you for sharing your story. I was diagnosed in April 2014 and have been on the Depo-Provera shot since then. It has saved my life! So far I have only had one attack due to something I consumed. i’m also in the DC area and see a doctor at Hopkins, probably the same one Sarah was talking about 🙂 I would love to know what doctor you see in Northern Virginia as it would be much easier to get to if/when I have an attack.

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