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HAE Documentary Coming Soon

10 May

This a great opportunity for friends of people, like myself, who struggle with Hereditary Angio Edema (HAE) to learn more about the horrors and triumphs of living with this rare blood enzyme deficiency.

Misery Loves Company

1 Jan

Times were tough this past December.

I contracted sepsis on December 11th and was hospitalized until December 27th.

Sunday evening I suddenly experienced:

  • Chills
  • Decreased urine output
  • A fever of 104.3°F
  • Light-headedness due to low blood pressure
  • Rapid heart beat
  • Muscle and joint pain
  • Vomiting

It was a delightful trip to the emergency room, as I’m sure you can imagine.  My step-mother (bless her heart) drove me in the rain at night to the ER 45 minutes away, and then visited and cared for my Yorkshire terriers on her way to and from work while I was hospitalized.  I was in ICU for several days, and fortunately did not experience either kidney or lung failure, or death from septic shock.  They did hit me up with some awful corticosteroids that ballooned my body painfully with 60 lbs. of retained fluid.

Exactly where the infection began is unknown, but two possibilities are (1) in my subcutaneous access port, also called an implantable port or Mediport, which I use every other day to give myself home IV infusions of Cinryze to control my Hereditary Angio Edema, or (2) the passing of the kidney stone I didn’t have the money to have removed before it could cause trouble. My Mediport was removed and a new PowerPort (which allows the port to be used for CT scan contrast infusion) was inserted a few days later.  CT scans and sonograms showed no stones remaining in either kidney.

Living alone never scared me before, and my reluctance to keep my local friends apprised of my health concerns (I just can’t impose on their already busy and complicated lives, and besides, sickness is BORING) narrows the number of people I feel comfortable calling on for help.  My step-mother, Annette, and my retired friend, Bob, have been total gems, and yet I feel guilty accepting their help because I have no way to reciprocate.

So Yuletide came and went without my even knowing, so happily drugged was I on intravenous Dilaudid.  I’m home now on bed rest and IV antibiotics until January 3rd, and am fervently hoping 2012 will not see a repeat of this horrible contagion.

I also wish a Happy New Year to all my readers; may the Goddess hold us in her healing hands and create new opportunities for joy and happiness.

Abbreviated Steps for Portacath Access

6 Jan

This is the list I kept at my side to keep me straight about the sequence of steps needed during the sterile procedures necessary when changing my huber needle until it became an ingrained routine.

  1. Supplies
    • Drape (S)
    • Saline (U)
    • Hep (U)
    • Skin Prep (U)
    • Huber Needle (S) but packaging (U)
    • Clave (U)
    • Cleansers (S)
    • Biopatch (S)
    • Op-Site (S)
    • Tegaderm (S)
  2. Lay out drape
  3. Open saline and hep; don’t put on drape
  4. Open needle; leave in packaging and don’t put on drape
  5. Open clave, put onto needle tubing, fill with saline, recap saline, let clave hang outside needle packaging
  6. Open skin prep; don’t put on drape
  7. Open cleansers, biopatch and tegaderm without touching contents and drop onto drape
  8. Put on gloves (first hand: only touch inside of glove and cuff with other hand; second hand: only touch outside of glove and under cuff with other hand)
  9. Swab port area with cleansers
  10. Insert needle
  11. Place biopatch between skin and needle, blue side out
  12. Flush with saline and then hep; close clamp!
  13. Apply skin prep and cover area with tegaderm

(S)=Sterile, (U)=Unsterile

Detailed steps can be found here.

Leaving Your Portacath Accessed

26 Jun

Going through the sterile procedures to access your portacath can be expensive and a hassle, but if you infuse Cinryze often, you want your port to be quickly and easily accessible.

So, how long can you leave your port accessed; i.e., how long can you leave the Huber needle in the portacath covered by a clear dressing, such as an OpSite?

Well, here are the results of an interesting study that says more than a week is completely safe!

Huber needle in situ inpatients under continuous infusion chemotherapy: results of a study, Phase II

Prof Inferm. 2000 Apr-Jun;53(2):71-4.

[Article in Italian]

Milani A, Vernizzi S, Passoni C, Sociale O, Macciola F, Grimaldi C, Comensoli M, Peruzzotti G, Lunghi L, Colleoni M.

Divisione di Oncologia Medica, Instituto Europeo di Oncologia, Milano, Italia.


PURPOSE: Chemotherapy administered as a continuous infusion is a widely used treatment in oncology. Huber needle deserves close attention during chemotherapy, but no data are reported on how long it can be left in situ without change. We therefore evaluated the feasibility of leaving in situ the needle for a prolonged time. METHODS: Patients candidated to continuous infusion chemotherapy were considered eligible for the study. The needle was changed at the end of the 21-day period when the patient started a new cycle of chemotherapy. On that occasion the site of injection was evaluated while replacing the needle. RESULTS: On 129 evaluable patients submitted to continuous infusion chemotherapy, 124 patients did not demonstrate any adverse cutaneous reaction. Five patients (3.8%) presented sores but we were able to continue the treatment leaving in situ the needle. CONCLUSION: Our results demonstrated that the needle can be left in situ for the entire time the patient is at home between cycles of chemotherapy. This procedure avoids patient stress and anxiety due to unjustified substitutions of the needle.

So, the above study supports leaving the ports accessed for as long as 3 weeks.  This following study supports an even longer timeframe, a whole month:

Port needles: do they need to be removed as frequently in infusional chemotherapy?

J Infus Nurs. 2003 Jul-Aug;26(4):239-42.

Karamanoglu A, Yumuk PF, Gumus M, Ekenel M, Aliustaoglu M, Selimen D, Sengoz M, Turhal NS.

Outpatient Chemotherapy Unit, Marmara University Hospital, Tophanelioglu C, 13/15 Altunizade, Uskudar, 81190 Istanbul, Turkey.


Protracted chemotherapy regimens are new treatment modalities used to treat patients with cancer. These treatments are preferred because of the ease of administration and limited side effects in the outpatient setting. Sixty patients were treated with continuous infusion chemotherapy via implanted infusion ports at Marmara University Hospital Outpatient Chemotherapy Unit in Istanbul, Turkey, from January 2000 to December 2001. Although usage of Huber needles for central venous catheters was limited to between 48 and 72 hours, needles were not removed unless there were signs of inflammatory reaction. The needles remained in place for 28 days (1-49 days) on average. No catheter infections, signs of local irritation, or thrombus formation were observed despite prolonged stay of the Huber needles. Huber needles can be left in place up to several weeks without any untoward effects as long as proper aseptic technique is used.

For the past month, I have been leaving my port accessed for two weeks and everything is going well.  If all continues to be well, I may extend another week with a final goal of changing my Huber out monthly.  This will decrease expense (i.e., supplies) and wear on the port.

Panic and Paranoia

28 Mar

That is what I have lived on for the past six days.  Not very tasty and certainly not very filling.  Also, not even remotely good for my Hereditary Angio Edema (HAE).

Apparently, the powers that be decided to “consolidate” distribution of Cinryze, the blood product I use to control my HAE and keep it from killing me, without telling any of us patients.  By the time I found out the reason no one was returning my phone calls the week of March 15th (which was when my Specialty Pharmacy should have contacted me to set up my monthly refill) by virtue of their voice mail boxes disappearing on March 22nd, I was down to only 3 doses, enough to last me until March 26th.  Need I say this was not good?

When I was put through to the new distribution team on March 22nd, they hadn’t even finished inputting patients into their computer or verifying insurance information.  Enter Madame Panic and Monsieur Paranoia, who all by themselves were capable of making those 3 doses only last through March 24th.  Lots of hot soaks in the tub and meditation ensued between bouts of phone calls to every resource I could think of to bounce me to the top of the priority list and keep me there.

I kept all 8 fingers and both thumbs on the carotid artery of the operation, making sure that everyone was doing what they needed to do to make IT HAPPEN, and a good thing, too, because on March 24th they were going to shift me to the California distribution center because, wowzer!, my co-pay was going to be $5,000 and it would be billed differently by the California dudes.  I’d have laughed if I could.  My co-pay is a lot more than $5,000!  My co-pay is closer to $9,400 a month.  It’s still coming up as $5,000 now because my insurance carrier still hasn’t paid my January or February Cinryze claims (but that’s another fricken story).  The first claim to get paid will put me at my catastrophic maximum (what President Obama and that hateful new health law wants EVERYBODY to have) of $5,000 – so my co-pay can’t be more than my catastrophic max.  “Oh, then let’s keep you on the East coast!”  Duh!  Plus, we’re trying to do in 3 days what normally takes a few weeks and on March 24th you want to send to California where I have to start everything all over again?  I swear, I thought I felt an aneurism developing that day.

The next day, Thursday, they FedEx everything over night for Friday delivery, but you know, don’t you, that the shipment wasn’t right, ‘cuz that’s Murphy’s Law.  So they FedEx over night for Saturday delivery the last bits and pieces, and Madame Panic and Monsieur Paranoia finally left the building.

On the 22nd of March I realized that, for the past year, I have lived with a false sense of security and safety when it comes to my HAE.  Oh, I never stopped being conscientious about or conscious of my condition, but I had felt a huge weight lift when I finally had Cinryze in my home and could do infusions myself in December of 2008.  Now I recall with vivid clarity just how much I am at the mercy of the supplier and distribution system and the weight is back.  Any of us who MUST have a medical treatment to survive that we cannot make ourselves is in thrall.  At any time the manufacturer can decide to stop making the product, and there are as many ways to disrupt distribution as there are to die.

Having a life-threatening, chronic illness doesn’t make for a carefree life, but I hope I can fake it really well until I make it feel that way again.

Second C1 Inhibitor Treatment Approved for HAE

11 Oct

The FDA released this statement on October 9th:

The U.S. Food and Drug Administration today approved Berinert, the first treatment for acute abdominal attacks and facial swelling associated with a rare and potentially life-threatening genetic disease called hereditary angioedema (HAE).

Berinert is approved for adults and adolescents with HAE, which can occur spontaneously or during stress, surgery, or infection in patients diagnosed with HAE. The symptoms during abdominal attacks include severe abdominal pain, nausea, vomiting, cramps, and diarrhea.

“Berinert will enhance the treatment options for individuals who experience acute abdominal attacks and facial swelling associated with hereditary angioedema,” said Karen Midthun, M.D., acting director of FDA’s Center for Biologics Evaluation and Research.

Berinert is a protein product derived from human plasma. It regulates clotting and inflammatory reactions that, when impaired, can lead to local tissue swelling. In a clinical trial of 124 adults and adolescents with C1 esterase, inhibitor deficiency, Berinert was shown to be effective at treating the symptoms of acute moderate to severe abdominal attacks and facial swelling in patients with HAE.

Berinert is contraindicated in patients with a history of life-threatening hypersensitivity reaction to C1 esterase inhibitor preparations.  The most serious adverse reaction reported in clinical studies was an increase in the severity of pain associated with HAE. The most common adverse reactions include subsequent HAE attack, headache, abdominal pain, nausea, muscle spasms, pain, diarrhea and vomiting.

Berinert is manufactured by CSL Behring, Inc., Marburg, Germany.

This announcement has two implications:

First, there are now two C1 inhibitor products derived from human blood available in the US.  Both are intravenous infusions:  Cinryze and Berinert.  Both can/could be used preventatively (Berinert off-label) and acutely (Cinryze off-label).

The second implication is that because Berinert received FDA approval for acute attacks, i.e., attacks already in progress, CSL Behring has the sole right to market their product for that purpose for the next seven years under the Orphan Drug Act.  Even though Cinryze works perfectly well for acute attacks, ViroPharma is not permitted to state this use in its prescription literature (but doctors can still use Cinryze off-label for acute attacks).  ViroPharma has and did submit data supporting the acute use of Cinryze, but I think it was in the US patient community’s best interest for the FDA to approve Berinert for acute use instead because such a move now makes two products available (and hence competition, eventually) in the US. This is especially important for patients who have not responded or are experiencing a decreased response to Cinryze.

Conversely, Berinert could be used preventatively, off-label, but I believe it has a shorter half-life than Cinryze, which makes its use as a prophylactic impractical — for instance, if I were to switch from Cinryze to Berinert for prophylactic treatment, I’d be going from infusions every other day to (possibly) twice a day.  However, I am not 100% certain about the exact effects of the half-life, so don’t take this as gospel.  I’m smart, but I’m no scientist and (thank the Lady) and I ain’t no doctor.

Thus far, I’d have to say it’s been a wonderful new century for sufferers of HAE – hooray!!


Cat Owners! Take Heed!

25 Aug

My friend, Maddwitch, found an interesting article about the affects of clumping litter on the digestive systems of cats.  Since clumping litter is made with materials designed to absorb moisture and can expand to 15 times its original volume, just licking the dust of clumping litter off their fur can create enough mass in a cat’s bowels to create a life-threatening blockage.  I wonder if this is the “mass” that killed my beloved Missy.

Dogs living in homes that use clumping litter are also at risk if they “snack” out of the cat’s litter box.

What is really maddening is that the companies know this is happening, but say its our responsibility to ensure our animals don’t eat the litter.  Kind of hard to keep a cat from licking itself clean, though.

The only way you can be sure this won’t happen to your cat is to start using non-clumping litter.  If I still had a cat, I certainly would.

Missy was the only one out of four cats that I could bring with me when I moved from RI to FL; she always wanted to be an only child.  Here she is up North showing off her mid-air catching skillz:


And her retrieving skillz; I think she may have been a dog in her previous life lol:


Or maybe she was a nurse, because she was always near me when I was having an HAE swelling (fortunately, this attack was limited to my right hand and forearm):


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