Welcome to the Exciting World of Cinryze

What follows are emails I composed for the Hereditary Angio Edema (HAE) Digest in response to requests for information, posted here for those who may not be on the Digest’s mailing list (to join the mailing list, which is open to HAE patients and their families, click here).

PERMANENT IV ACCESS

I live in the Orlando area and have had my mediport for 2 years now. I live alone and a home care nurse trained me to access the port myself. I was a tad nervous at first, but soon I could do it in about 5 minutes. I received my first shipment of Cinryze two days before Christmas 2008 and self-infuse three times a week. I leave my port accessed from Monday through Friday and go needle-free for the weekend. I place a clear OpSite IV patch over my port and needle during the week to keep the site sterile and have not had any problems with showering or bathing. I also place a biopatch (an antimicrobial dressing with chlorhexidrine gluconate) between the needle housing and my skin to further decrease my chances of infecting my port. I only needle-up once a week to prolong the life of my port.

When my port was implanted during outpatient surgery at the Florida Radiology Lab, I was completely awake and watched the insertion on radial TV; it took about 2 hours and someone had to drive me home (technically). My port is slightly visible because I don’t have much muscle or fat below my collarbone, where the port is placed; all that is visible is a small bump and I’m able to use a very short needle (1/2″). I can feel with finger pressure the internal catheter line that travels up to my jugular vein, where the catheter then bends down towards the heart. I use a Huber needle to access the port; my Huber needle has a short length of IV line attached and a plastic housing that slides over the needle after removal for needle safety (it must still be disposed of properly; my local fire station provides free needle containers, as does my Cinryze specialty pharmacy).

When I administer a Cinryze infusion, I flush my port with 10cc of saline to remove heparin from the line, then I administer 10cc of Cinryze over a ten minute period so the proteins are evenly distributed throughout the bloodstream. I then administer another 10cc of saline to flush the Cinryze remaining in the line into the bloodstream, and then flush 5cc of heparin into the line to remove the saline and keep blood from entering the catheter line and forming clots. Note: If the port is not accessed within a 30 day period, the line must be flushed with saline and filled with new heparin.

My port has given me back my independence and I highly recommend it. I created a “cheat sheet” for myself of the sterile steps involved in accessing my port and will happily share it with anyone interested; just leave me a comment.

ANABOLIC STEROIDS AND CINRYZE:  PLUS OR VS?

Here’s my take on the anabolic steroids plus or versus Cinryze issue (it’s basically a rewrite of what I wrote my brother when he asked me if Cinryze was a replacement for his stanazol):

This is an issue between your and your doctor. The goal is to have MANY more options for HAE patients, and Cinryze is just another (albeit new) one. For me, Cinryze is currently the ONLY option, as danocrine, stanazol and oxandrine do not work for me. Cinryze is not a replacement unless your doctor determines that it should be. Some reasons that it might be are: (1) pregnancy (a woman should NOT take danocrine during pregnancy because of the effects on the embryo), (2) unacceptable side effects (high cholesterol and diabetes are sometimes side effects of anabolic steroids), and (3) unacceptable number of breakthrough attacks (i.e., anabolic steroid treatment is only minimally effective) (I’m sure the HAE community can think of many others, this is not intended to be a comprehensive list as indicated by the use of the word “some”). Cinryze can be used both prophylactically and for acute treatment, although acute treatment is currently off-label (but FDA approval is being sought).

In light of the HAE-related deaths in the United States last year that could have been provided if Cinryze had been available, it is my considered opinion that EVERY HAE patient in the US should have, at the very least, 2 doses of Cinryze on their premises (if they don’t live alone and there is someone who can be trained to administer it) or at their local fire station/emergency rescue service (if they live alone) for emergency administration in the event of a laryngeal attack.

The answer might be to stay on your anabolic steroid and have Cinryze as an emergency reserve for acute, breakthrough attacks. The answer could be to switch completely, but it may be to do nothing. Each HAE patient’s case varies by frequency, duration and complications and one size has never fit all.

CARING FOR YOUR PORT

On the premise that no information shared is ever wasted and no one can ever have too much knowledge, here’s some great info on how to take care of your port.

First, let’s define “port:”

In medicine, a port (or portacath) is a small medical appliance that is installed beneath the skin. A catheter connects the port to a vein. Under the skin, the port has a septum through which drugs can be injected and blood samples can be drawn many times, usually with less discomfort for the patient than a more typical “needle stick.” The term portacath is a portmanteau of “portal” and “catheter”. Port-a-Cath is a brand name; others include Microport, Bardport, PowerPort (power injectable), Passport, Infuse-a-Port, Medi-Port, and Lifesite (for hemodialysis patients). The term Totally Implantable Venous Access System (TIVAS) is also used. Additionally, portacaths are a form of a “central venous access device” and are frequently referred to as such in the medical field.

It’s easier to take care of something if you know how it works:

A port consists of a reservoir compartment (the portal) that has a silicone bubble for needle insertion (the septum), with an attached plastic tube (the catheter). The device is surgically inserted under the skin in the upper chest or in the arm and appears as a bump under the skin. It requires no special maintenance and is completely internal, so swimming and bathing are not a problem. The catheter runs from the portal and is surgically inserted into a vein (usually the jugular vein, subclavian vein, or superior vena cava). Ideally, the other end of the catheter terminates just upstream of the right atrium. This position allows infused agents to be spread throughout the body quickly and efficiently .

The septum is made of a special self-sealing silicone rubber; it can be punctured hundreds of times before it weakens significantly. To administer treatment or to withdraw blood, a health professional will first locate the port and disinfect the area. Then he or she will access the port by puncturing the overlying skin with a 90° Huber point needle. Due to its design, there is a very low infection risk, as the breach of skin integrity is never larger than the caliber of the needle. This gives it an advantage over indwelling lines such as the Hickman line. Negative pressure is created to withdraw blood into the vacuumized needle, to check for blood return and see if the port is functioning normally. Next, the port will be flushed with a saline solution. Then, treatment will begin. Also, after each use, a heparin lock is made by injecting a small amount of heparin (an anticoagulant) into the catheter. This prevents development of clots in the system. The port can be left accessed for as long as required. The port is covered in a dressing to protect the site from infection and to secure the needle in position.

Check out this site to see what a port looks like before implantation and to see a quick video of a needle insertion: http://www.gynoncology.com/Movies/Portacath_Insertion.htm

This link is to a photo-essay by a gentleman using a port to get chemotherapy: http://diehlmartin.com/infusion/

Source: Wikipedia

Care Tips:

• After the port is put implanted and the skin over the port is healed, you may return to normal activities.
• Live as normally as possible. The port is just a part of your life.
• The port needs to be kept safe. Ask your doctor before you play a contact sport.
• After the skin over the port is healed, you can get the skin wet.
• When there is no needle in the port, can take a bath, shower, or swim. You may wash the skin as usual. A dressing is only put over the port when a needle is in it.
• You should not swim or take a shower when the needle is in. You can take a tub bath while the needle is in only if you keep the dressing dry.
• You must change the needle at least once a week if the dressing is transparent (OpSite, Tegaderm, and Sorbaderm are some examples of transparent dressings).
• If using a gauze dressing, change the needle every 48 hours.
• Call your doctor right away if the site around the port shows bruising, swelling, redness, bleeding or pain, or if you have a fever over 101є F, have chills, or the port seems to have moved.
• A port puts you at higher risk for an infection when getting dental work. Before and after dental work, you should be given antibiotics, so see your doctor first.
• Look at the skin over the port every day. If the port stops working, or causes discomfort, return to the doctor’s office or hospital to have it checked as the port may be infected.
• If you cannot flush the port, or cannot get a blood return:
  • Do not use force to flush the port. The pressure could loosen a plug so it is free to block a blood vessel. Forceful pressure could even break the port.
  • Make sure the tubing is not clamped.
  • Make sure the port needle is pushed in so it touches the back or the port.
  • Raise your arms over your head, or lay on your side, and try again.
  • If you still cannot flush the port or get a blood return, call your health care team.
• Signs of infection can be drainage or oozing, such as pus, swelling, tenderness, soreness, warmth, pain, redness at the exit site, redness along the catheter path beneath the skin, a temperature above 101F orally (check with your health care provider), or odor from the exit site. If you see any of these signs, call your health care team right away.

Source: Phoenix Children’s Hospital Procedure Brochure #810

COST OF CINRYZE AND COSTS ASSOCIATED WITH USING CINRYZE

Keeping in mind that I use Cinryze three times a week and one shipment contains 12 doses (i.e., 24 bottles)…

I received my first shipment of Cinryze two days before Christmas and co-pay was maxed out at $2,700, only because that was the amount it took for me to reach my catastrophic maximum for the year. Apparently it would have been more, but I won’t know by exactly how much more until I receive the Explanation of Benefits from my insurance company (frankly, I was afraid to ask my specialty pharmacy case manager the total cost because I didn’t want to have nightmares!). I have a catastrophic maximum each year (the maximum for 2009 is $5,000). When I receive my next shipment of Cinryze next week (NLT Thursday 1/15), my co-pay will not exceed $5,000.

My shipments also contain 24 bottles of sterile water (to reconstitute the Cinryze), saline and heparin flushes with matching micro-clave (they were worried they might not match what I had, so they sent their own), double-ended transfer needles (to transfer the sterile water to Cinryze bottle), and empty syringes and needles to extract the reconstituted Cinryze.

Someone emailed for the following information and so I will share it also. Since I infuse at home, the following supplies are required and I currently receive them from a medical supply company by prescription by my immunologist (when these supplies run out, they will probably be supplied by the specialty pharmacy since it doesn’t make sense to have delivery of the supplies divided into two sources) (Note: The exact supplies you may need/use could differ depending upon your needs/supplier or availability):

• Pre-Filled Saline Flushes
• Pre-Filled Heparin Flushes
• Central Line Dressing Change Kits that contain: Sterile Drape, Sterile Gloves, OpSite Dressing, Chloraprep Sepp Applicator (to clean/sanitize skin over/around port; most kits contain betadine, but I’m allergic to betadine), Mask, Alcohol Pad, Tape, Gauze, etc.
• Micro-Clave (this fits on the end of the IV line so you can attach flushes/syringes by twisting onto end instead of using a needle)
• Biopatch
• Huber Needle with attached IV line
• Protective Skin Prep (I’m really sensitive to adhesive and this gives me some protection from the OpSite)

My co-pay for these supplies was running $45/month.

I must also note here that I applied and was approved for assistance through PSI, who paid my first co-pay of $2,700 and will pay my full co-pay for Cinryze (but not supplies) through 2009. Their assistance will be capped at $5,000 because my insurance company will cover ALL my medical expenses thereafter and their assistance will no longer be required. Your Cinryze Solutions case manager is responsible for recommending you to PSI for assistance. Once they recommend you, PSI will contact you, provide you with the application form, advise you of the documentation required (it’s very minimal), and advise you of their determination.

Your Cinryze Solutions case manager is also there to help you determine if your insurance (1) covers Cinryze, and (2) how much of the cost they cover. If you cannot determine if your plan has a catastrophic maximum limit, give them a call and find out. If you do not have any insurance, your Cinryze Solutions case manager will recommend you to PSI for financial assistance.

I am very blessed to have such a wonderful insurance plan and to have worked very closely with Cinryze Solutions from their inception so that I could quickly get access to this new HAE treatment. In return, if you have any questions about the process or the use of Cinryze, I will do my best to answer.

COST OF CINRYZE – DETAILED ANSWER

I just received my first Explanation of Benefits (EOB) for my first delivery of Cinryze in December 2008 and can finally give a full and complete answer to the subject question.

First, the shipment was billed as a prescription drug, not as a medical service (as I was told it would be during the HAE Association’s 2008 National Patient Conference and International Leadership Forum), which is very interesting. How your insurance will be billed may or may not be different (you all know who this game works).

Second, TheraCom/CVS/Caremark, my specialty pharmacy (and I believe the specialty pharmacy that covers the East Coast) is shown as a “Preferred Provider” under my plan (Blue Cross/Blue Shield Federal Employee Program for anyone else who has this same plan). This means that my co-pay was 25% in 2008 and will be 30% in 2009.

Third, the submitted charges were $55,575.00 for 12 doses (24 bottles) of Cinryze. This equates to an average of $4,630.00 per dose ($2,316.00 per 500IU bottle).

This means my co-pay for December should have been $13,893.75!! And my co-pay for January will be $16,672.50!!!

I’m so glad I didn’t know this 2 days before Christmas last year.

BUT NOT TO PANIC…

Two things saved me from utter financial ruin…

My catastrophic maximum and PSI.

My catastrophic maximum in 2008 was $4,500, against which I had already amassed costs of $1,800, leaving a gap of $2,700. This reduced my December co-pay from $13,894 to $2,700 (whew!), the full amount of which was covered by PSI. Note: PSI transfers their funds directly to the specialty pharmacy, you do not have to fiddle around with reimbursement (wayyy whew!) and so far PSI and TheraCom are working well together.

My catastrophic maximum in 2009 will be $5,000, against which I have not amassed any costs as of yet. Thus, my January co-pay will be reduced from $16,672 to $5,000 (another whew!), the full amount of which will be covered by PSI, and “as a result, no further calendar year deductible, prescription drug, deductible, coinsurance or copayment will apply for this patient for this calendar year” as stated on my EOB.

My advice: Research the catastrophic maximum for your insurance plan. Is there one? Is it yearly? Is it a lifetime cap? How much is it? Are there any restrictions? And don’t hesitate to ask your Cinryze Solutions case manager about applying for PSI assistance (remember that you must be nominated/sponsored/recommended for assistance through Cinryze Solutions). To read about Patient Services Incorporated (PSI is a non-profit premium and co-payment foundation) and their good works, check out their website at http://www.uneedpsi.org.

The purpose of a fore-runner is to break through the worst barriers and pave a path for those who follow. I pray I am doing that and, as difficult as your journey seems now, please know I started mine almost a year ago as this process geared up and started development. Talk about fits and starts, backtracking and confusion, reversals and changeovers! I now have twelve case managers just for Cinryze, they’re all on speed dial, and we’re all on a very chatty, first name basis. Persevere, dear friends, because if Cinryze is right for you, it’s worth even the tiniest of frustrations!

No-swell blessings to all,

Kat

DISCLAIMER: The above comments are based on the personal experiences and opinions of the writer, and any errors are unintentional oversights (or possibly just plain ignorance). Readers should always consult their own physician for current and official medical facts, advice and opinions. ALWAYS take everything anyone (including me) tells you with (preferably) two grains of salt (especially if aspirin upsets your stomach as this could result in an abdominal attack [grin!]) and please don’t call me in the morning if you don’t like what I say. Bright blessings for a happy and swell-free day.

Typical Portacath: Portal, Septum and Catheter

Pre-Filled Flushes: Saline Usually Have White Caps, Heparin Have Yellow

Shows Threads So Syringe Screws onto Clave

Example of a MicroClave

MicroClave Attached to IV Tubing

Biopatch -- A Little Bigger Than a Quarter

These are the types of Huber needles that I know about:

Huber Plus from Now Medical

The Huber Plus Safety Huber Needle was the first self-contained, passive, safety non-coring huber needle to receive FDA pre-market notification as a safety device. It requires minimal change in user technique and can be activated using a one-handed operation.  The safety feature does not interfere with the normal use of the product. The HUBER PLUS safety wings are color-coded to identify needle gauge size and is offered in several needle lengths ranging from ½” to 1 ½”.  Straight sets are available as well as sets containing a needle free y-site featuring a positive pressure valve.

The Huber Plus is activated by simply grasping the wings with thumb and forefinger and squeezing.  The needle is encapsulated within the locked wings as the needle is removed from the port.  The safety mechanism provides both visual and audible (the safety “snap”) confirmation that the exposed needle is now covered and the clinician is protected from accidental needlestick injuries.  Additional information can be obtained from Now Medical by clicking here or call 610-455-0258.

Gripper Plus from Deltec

The design of the GRIPPER PLUS® Safety Needle allows the user to de-access the needle in one safe, easy, natural motion. Its unique feature is a safety arm that is lifted to lock the needle into a protected position when removing it from an implanted port.  The fingers are placed on each side of the needle base. With the other hand, place a finger on the tip of the safety arm. The safety arm is lifted straight back until it CLICKS into its locked position.  The product can then be dropped into a sharps container.

The Gripper Plus has a cushioned needle platform for greater patient comfort, needle stabilization and protection of the access site.  It has a removable contoured grip for controlled needle placement. The clamps are color-coded for needle gauge size identification. For more information on the Gripper Plus click here or call 800 426 2448.

LifeGuard™ Safety Huber needle from Horizon Medical Products (HMP)

This is what I use.

LifeGuard’s needle trap fully encapsulates the needle upon removal.  Compared to traditional Huber needles, Lifeguard is designed for maximum control and safety with minor changes to technique. It provides both visual and audible confirmation of safety.  It has colored safety handle for needle gauge confirmation.  To remove the needle from the port septum simply raise the needle trap upward to 90°.

Using non-dominant hand, grasp the safety hinge with thumb and middle finger. Gently push the safety hinge down against the patient’s skin and port.

Flip the safety needle towards the needle trap into the safe position. For more information contact Horizon Medical Products at 800-472-5221 or visit their website here.

Surecan® Safety Huber Needle by B. Braun

Whether you’re delivering chemotherapy, antibiotic therapy, or parenteral nutrition, the Surecan® Safety Huber Needle’s patented safety clip will automatically engage as you withdraw the needle from the base plate.

It is color–coded by size with 19, 20, and 22 gauge needles available. The Surecan® is latex-free to avoid the risk of allergic reaction and DEHP-free for compatibility with chemo drugs and lipids. Enables you to inject medication or withdraw blood from the Y–site with a simple luer connection. The base of the port is stabilized by firmly holding down with the non-dominate hand. The needle is withdrawn by pulling straight out by the hub grip. The safety clip automatically activates covering the needle.  For more information click here or call 800-523-9676.

LiftLoc™ Safety Infusion Set manufactured by Specialized Health Products(R) International, Inc. and marketed by Bard Access Systems

LiftLoc Safety Infusion Set features a robust shield that is deployed as the needle is withdrawn from the port, effectively reducing the risk of accidental needlesticks, including rebound injuries. A Patient Comfort Pad™ is included with each LiftLoc set, and is designed for patient comfort. The use of the Patient Comfort Pad is optional.  LiftLoc’s safety mechanism is easy-to-use and conforms to common user technique. To access simply grasp the wings and lift them.  As the wings are lifted up, the clinician pulls the LiftLoc from the port while covering the needle.

The LiftLoc Safety Infusion Set is distributed under the SHPI label by Cardinal Health, Medline Industries, Inc., and Physician Sales and Service, Inc. (“PSS”), leading distributors with a strong presence in the oncology, chronic hematology and long-term intravenous nutritional markets.  The product line is distributed in the hospital market on a private label basis by Bard Access Systems, Inc., a subsidiary of C.R. Bard, Inc.  For more information about the product contact Bard Access Systems, Inc. at 800/545-0890 or go to their website.

Luther Safety Huber Needle Protector by Luther Research Partners

The Luther Safety Huber Needle Set is a device intended to administer medications to a patient through a subcutaneously implanted port.  This safety device is for use on all Huber needles manufactured from ½ to 1 ½ inch.

The Huber Safety Needle cover is manually activated.  It uses a permanent locking tip that renders the sharp portion of the needle inaccessible upon removal of the needle from the implanted port.   It has a foam pad for patient comfort. The sure-grip handle is available for easy insertion and removal from even the most difficult ports. Simply grab the sure-grip handle and lift the needle from the implanted port septum.

The safety system cannot be defeated or reset.   For more information call (714) 434-1564 ext 4 or visit their website here.

Kawasumi Laboratories America, Inc. – K-Shield

Kawasumi America is marketing a new needle safety device designed to protect oncology clinicians from Huber needle stick injuries incurred while removing needles from subcutaneous ports. The device also provides a convenient means for disposing the Huber needle after removal from patients, but does not discomfort the patient at any time, nor does it present any needle movement during the time of infusion. The K-shield ensures safety for the clinician while maintaining maximum patient comfort. When deaccessing the Kawasumi Huber needle from the implanted port, simply lift up and fold the wings down on top of the needle. [kshield.jpg]   For more information on this product call 800-KAWASUMI (529-2786) or visit their website here.

TriState – Centurion® HuberGuard™ Safety Device ( Manufactured by Harmac and distributed by TriState Hospital Supply Corporation)

Centurion® HuberGuard(tm) Safety Device provides needlestick prevention when used in conjunction with the Centurion® huber infusion sets for removal and disposal. The low-profile needle is comfortable for the patient and reduces bumping and dislodgement from the port.  To deaccess from the port septum the HuberGuard is aligned over top of the Centurion huber needle.  The needle is pulled up by the right wing so that the needle is held within the HuberGuard. The needle clicks securely into place providing both tactile and audible safety lock position indicators. The needle is then disposed of in a sharps container. The Centurion HuberGuard is available in sterile or non-sterile configurations.  For more information contact Tri-State Hospital Supply Corporation at 517.546.5400 or visit their website here.

Huberloc (Manufactured by MedCare Medical Group, Inc. and Distributed and Marketed through Baxter Healthcare)

The Huberloc is a safety product that removes the needle from the port and contains it thus eliminating rebound needlestick.  It allows the clinician to use the huber needle of their choice.  The Huberloc Device also offers a positive pressure flush and lock technique.  Because the Huberloc Device enables you to remove a huber needle with one hand, you can perform a positive pressure flush during decannulation with the other hand.

This simultaneous flush and lock technique helps maintain catheter patency. The Huberloc is designed for compatibility with 19 GA x 1 ½” long and smaller 90° winged huber needles.  Simply align the HuberLoc with the needle hub that is opposite of the tubing.  The forks of the HuberLoc are slid forward until the needle shaft is all the way to the back of the forks.  While pressing down on the top of the HuberLoc with your thumb, pull up on the finger grips with your first two fingers (like syringe motion). This extracts the Huber needle. Continue this motion until the slide “snaps” into its locked position. For more information please phone 888-229-0001 or visit their website here.