Hosting our First Blood Drive for HHT Awareness!

She wanted to give blood by the end, but she didn’t meet the weight requirement 😉

Since marrying into an HHT family, I have felt a need to do more to advocate for my family members, raise awareness, and help in any way I can. I know how precious both money and blood can be to this community, but we are not always in a financial position to give to the foundation (although every little bit really does count!). I decided to reach out to the local Red Cross and my church to coordinate a blood drive. I’d like to talk about this process, how surprisingly easy it can be, and tell you how everything went on the big day!

  • Reaching out to your local American Red Cross representative
    • You can Google this, or look it up on the Red Cross website: redcrossblood.org
    • In my first e-mail, I told my rep, Kristi, about HHT and how I felt like I wanted to do more to help raise awareness. I offered my assistance to volunteer with local blood drives and asked her how I could learn to coordinate one of my own.
    • She was SO NICE! Y’all – seriously, reach out to your local Red Cross folks – even if you don’t want to host a blood drive, just get to know them as your friend. I have never met anyone in this business I haven’t liked. They truly have a heart and soul for service.
  • Bringing awareness through an ‘In Honor of’ blood drive
    • Kristi told me about a campus-wide blood drive happening where I work at Kansas State University. While this drive was already planned and ready to go, they wanted to feature HHT to help bring awareness to the disease and the potential need for blood in the community because of it.
    • If you aren’t ready to coordinate your own blood drive, you can certainly raise awareness about HHT by making an already scheduled blood drive in honor or in memory of someone.
    • I filled out a form talking about my family, the disease, and our experiences with blood donation in the past. My husband and daughter have yet to receive blood transfusions, but my mother-in-law has and her father was reliant on them during the last years of his life.
    • The Red Cross and the campus newspaper did a short interview and press release detailing HHT and the need for blood in the HHT community.
  • Coordinating your own drive
    • If you are ready to host and coordinate your own blood drive, I would suggest getting these things in line:
      • A location
      • A few potential dates
      • A few volunteers to help schedule appointments/make signs/send reminder e-mails and calls/help on the day of the drive
    • Once you have an idea of the above information, tell your Red Cross rep you are ready! They will let you know what dates work best for their schedule and they almost literally do the rest!
    • Once we settled on a day that worked well for the church, Kristi told me what time, how many appointments we would have, and what to do every step of the way.
    • We started with a small drive – 2 Red Cross workers and 21 appointment slots. The drive went from 9am to 3pm this last Sunday.
  • Advertising your blood drive
    • Since we were having our blood drive at church, I asked the pastors if I could speak about HHT and the reason behind this blood drive during church service. They were more than happy to oblige and I just gave a quick 2-3 minute synopsis of HHT and why giving blood is so important, especially to those with diseases like HHT.
    • Kristi had already given me a sign-up sheet and reminder cards for donors. I set up a table in our church’s gathering place to book appointments, and I had almost every appointment booked by the end of that same church day!
    • Kristi had also given me some table toppers with information about the drive, flyers, and contact information sheets. They literally take care of everything!
  • Preparing for the drive
    • A few days before the blood drive, I entered everyone’s information into the Red Cross system. Blood drive coordinators get their own login so you can access appointment times, schedule donors, and send e-mails. All the flyers, advertisements, and resources are also available on the site.
    • Everyone was sent an automatic reminder e-mail about their appointment.
    • I asked my church group to volunteer to make goody bags for the donors. (Since this was a small drive specifically to raise awareness about HHT, I wanted to hand out some HHT information and some treats to donors as a thank you.)
  • Blood drive day
    • I arrived at the church at the same time as the Red Cross staff to help see them into the building.
    • They have everything so organized – the only thing I did was move around some chairs! They had their own tables, music, fans, water, food, and of course all the donation supplies.
    • I stayed at the drive to greet donors as they came in, to guide them to the sign-in sheet, and then I just chatted with them as they waited for their appointment! My job was by far the easiest!
    • At the end of the day, the staff packed everything up and went on their way! They were so nice and made every donor feel special and well taken care of.

Our first blood drive was a success and we met our blood donation goal! It really was surprisingly easy to coordinate and everything went really well! The Red Cross did 90% of the work, I really just found a venue and advertised it. I can’t wait to host another this fall! We are hoping to make this happen at our church every two months. This way, regular donors can continue to come back when they are eligible to give (after 56 days for a whole blood donation). If anyone has any questions about this process, let me know or reach out to your local Red Cross people!

 

 

 

HHT Insurance Coverage in Danger!

Health Bill Would Remove Protection for HHT Patients!

Update: The House cancelled the scheduled vote for Friday, March 24. There has been no announcement as to is rescheduling. 

If the American Health Care Act passes it would eliminate insurance coverage for essential health benefits mandated in the Affordable Care Act - including chronic disease management in HHT and other genetic diseases.

Contact Your Representative Now!

If phone lines are jammed, you can send an automatic email using Popvox to your representative.

Here is the link to the AHCA page: https://www.popvox.com/us/federal/bills/115/hr1628

You will need to sign up by entering your name, email address, and zip code. Then click the “Oppose” button under Add Your Voice on the upper right side of the page. You are then directed to a field to add your message and send electronically.

How This Would Affect You

  •  Individual states would be allowed to define “Essential Health Benefits,” meaning many of the basic services covered by the ACA -- such as prescription drugs, maternity care, mental health, emergency services, hospitalization – may no longer be covered.
  • Because annual and lifetime limits apply only to essential health benefits, insurers could put a cap on how much care one receives, but not on how much people owe. Treatment costs for major diseases are incredibly expensive and could easily bankrupt those in need.
  • Because treatments and medications for the HHT community can be very expensive, the elimination of essential health benefits essentially renders meaningless the ACA’s protections against insurance discrimination for those with “pre-existing medical conditions.”
  • Even if you get your health insurance through an employer, the new legislation could dramatically increase your costs while also reducing benefits.

Keep Cure HHT Growing

 

Top 5 Ways Your Donation Matters

 

 

 

HHT and Pregnancy

Most women with HHT can experience normal pregnancy and delivery with the same amount of risk as women in the general population. Though HHT pregnancies are sometimes considered “high-risk," this is not usually the case. While women with more concerning HHT symptoms can benefit from a “high-risk” clinic approach, women who experience minimal symptoms may not need it. The key is to consult with an HHT expert to help make the best decisions for you and your baby.

Remember that HHT affects each person differently, and symptoms are known to change over a lifetime. How HHT affects your body should guide your pregnancy planning and care. For example, if you only experience telangiectasia in the nose and have no abnormal blood vessels in the organs, there's no reason to think your pregnancy or delivery is high-risk. On the other hand, if you have lung AVMs, then several steps and precautions need to be taken to minimize risk, and in some cases this may be considered a high-risk pregnancy.

Here's What You Need to Know

Myth #1:  Women with HHT are at risk of excessive bleeding from the uterus during or after delivery.

There is no evidence of this. In fact, women with HHT are no more likely to bleed excessively at delivery or after delivery than women in the general population.

Here’s why: People with HHT don’t have a clotting disorder. People with HHT bleed from parts of the body where they have abnormal blood vessels, not because of a regular injury, trauma or cut. The uterus is not a typical place for AVMs to occur in HHT, so there is no reason to expect women with HHT to bleed with delivery.

Myth #2: Women with HHT can’t have an epidural.

Rest easy  - this is not the case. Most women with HHT decide to have an epidural, or give themselves the option to have one available in the moment. There is no evidence of increased risk of complications. We'll break it down for you.

Why is there concern? Many anesthetists and other doctors are worried about the risks of epidural anesthesia in women with HHT, specifically about puncturing a spinal AVM when they insert the epidural needle into the women’s lower spine. Approximately 1 percent of HHT patients have AVMs in the spinal canal, but the majority of HHT spinal AVMs are higher on the spinal canal than where the epidural is inserted.

No case has ever been reported in medical literature of an epidural causing spinal bleeding in HHT. Complications of spinal AVMs are exceedingly rare in adults (most are discovered during childhood). In fact screening for spinal AVMs isn't routinely recommended in adults with HHT (unlike screening for lung and brain AVMs) given how rare they are in adults. In other words, if there is a risk, it's so low no one has been able to document a case or quantify the risk. However, there is no study “ruling out” risk of epidural in HHT - so we call it a “theoretical” risk.

If a pregnant woman with HHT wants the option of an epidural, she should get the option. Meet with the anesthetist prior to delivery and have a discussion about all the risks,“theoretical” or otherwise. Many anesthetists will agree to proceed if the woman understands the risks.

Myth #3: Women with HHT should have a cesarean section to avoid bleeding and other HHT complications with labor and delivery.

There is no evidence to support the C-Section as a routine approach for HHT patients. We've already discussed the myth of excessive bleeding in #1 so let us continue to other assumed complications.

The second concern is a brain AVM rupturing when pushing at delivery. There is no evidence to support this worry, or that cesarean section is a safer alternative to vaginal delivery in people with brain AVMs. However, this situation needs to be handled on an individual basis.  Screening for brain AVMs can be performed in early third trimester with an unenhanced MRI of the brain. If the MRI is negative, as it is for 90 percent of women with HHT, then there's no reason to be concerned.

If you do have a brain AVM, your case is considered high risk and needs to be discussed with a brain AVM expert and obstetrician. If a brain AVM is diagnosed, the usual recommendation is to treat within two or three months after delivery. As such, since treatment will be delayed anyway, some women opt to postpone the brain MRI until after delivery. This is not unreasonable, but may leave you and your physicians unnecessarily worrying about the possibility of brain AVMs and a false “high-risk” pregnancy.

There is also no evidence that a treated lung AVM is more likely to rupture and bleed during vaginal delivery. However, if you have an untreated lung AVM your pregnancy should be considered high risk. Screening and preventative treatment for lung AVMs should be done prior to pregnancy, when possible. If you are already pregnant, lung AVM treatment during Trimester 2 or 3 with embolization should be considered, but only at an expert HHT center. If you have lung AVMs that have been successfully treated (and recently reassessed), then lung AVMs are not a concern for pregnancy and delivery.

Summary

For most women with HHT, the main problem is nosebleeds, and though bothersome, this is not often a major concern during pregnancy. Some women with HHT have a more extensive issues, with AVMs in the brain or lungs, which may be more concerning. However, with the right screening, treatment and surveillance, most women with HHT can have normal pregnancy and delivery, with no more risk than women without HHT. Any patient with lung AVMs (treated or untreated) should follow pulmonary AVM precautions, including antibiotics before any procedures that can cause bacteria in the blood as well as using an air filter (bubble trap) any time there is an intravenous access.

References

  1. Faughnan, M.E., et al., International guidelines for the diagnosis and management of hereditary haem- orrhagic telangiectasia. J Med Genet. 48(2): p. 73-87.
  2. De Gussem, E.M., et al., Outcomes of Pregnancy in HHT(Abstract). Hematology Meeting Reports, 2009. 3(4): p. 10.
  3. Gershon, A.S., et al., Transcatheter embolotherapy of maternal pulmonary arteriovenous malformations during pregnancy. Chest, 2001. 119(2): p. 470-7.
  4. Shovlin, C.L., et al., Medical complications of pregnancy in hereditary haemorrhagic telangiectasia. QJM, 1995. 88(12): p. 879-87.
  5. Shovlin, C.L., et al., Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services. BJOG, 2008. 115(9): p. 1108-15.
  6. Wain, K., et al., Hereditary hemorrhagic telangiecta- sia and risks for adverse pregnancy outcomes. Am J Med Genet A, 2012.
  7. http://www.nichd.nih.gov/health/topics/high_risk_ pregnancy.cfm

ABCs of AVMs: Vascular Malformations of the Lungs and Brain in HHT

Presenters

Conrad, Miles UCSFDr. Miles Conrad is Associate Clinical Professor in the Department of Radiology at UCSF and Co-Director of the UCSF HHT Center of Excellence.  Dr. Conrad received his MD from Dartmouth Medical School and his MPH from Harvard School of Public Health. He completed a Diagnostic Radiology residency and a fellowship in Interventional Radiology at University of Arizona. He specializes in the treatment of PAVMs.

 

Hetts, Steven UCSFDr. Steven Hetts is Associate Professor of Radiology, Co-Director of the UCSF HHT Center of Excellence and Chief of Interventional Neuroradiology at USCF Mission Bay Hospitals. Dr. Hetts graduated from Harvard Medical School, and completed a Diagnostic Radiology residency and fellowship in Diagnostic and Interventional Neuroradiology at UCSF

 

 

Webinar

Join Doctors Miles Conrad and Steven Hetts for a discussion on the diagnosis and treatment of brain arteriovenous malformations (BAVM) and an update on the latest lung AVM (PAVM) treatment followed by a Q&A period.

Webinar Highlights

 

ACT NOW

Cure HHT members will have access to unlimited webinars, discounted conference registration and much more. Please consider joining or renewing your Cure HHT membership so that you can participate in all activities within the HHT community.

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Cure HHT is the cornerstone of the HHT community, advancing one common hope - to give those affected by HHT a chance for a normal life. This webinar series is just one of many ways we accomplish this mission. Beginning in May, Cure HHT members will have access to unlimited webinars, discounted conference registration and much more.

Ask the Doctor: Jason Hamilton

Q: Can excessive treatment for nosebleeds permanently harm my nose?

Dr. Jason Hamilton, Director of Plastic and Reconstructive Surgery at Osborne Head and Neck Institute: It's possible to develop a septal perforation, which occurs when cartilage separating the two nostrils (known as the septum) develops a hole or fissure. This can cause a variety of symptoms, most notably nose bleeding. In HHT patients, septal perforation is usually attributed to nasal trauma from aggressive laser or electric coagulation sometimes used in treating epistaxis.

A septal perforation alters the anatomy of the nose, affecting its function. The normal humidity in the nose is decreased by constant airflow across the weak or torn edges of the perforation, causing the site of the perforation to dry out, leading to a crusted or scabbed area likely to bleed. When left untreated, it causes further deterioration of the condition.

The surgical procedure to repair a septal perforation should be performed soon after a diagnosis, and not as a last resort. The goal is to restore normal nasal anatomy and humidification and to reestablish the structural integrity of the nose – it is not a cure for HHT-related epistaxis.

Ask the Doctor: Dr. Felix Ratjen

Q: Can kids with HHT play sports? What are your recommendations?

Dr. Felix Ratjen, Toronto Pediatric HHT Center: Regular exercise is important for everybody, and this certainly includes children who have HHT. Most children with HHT can participate in sports at all levels including competitive sports, but there are a few exceptions. Contact sports are not recommended for children with HHT who have brain AVMs unless they are completely treated; you should talk to your doctor about that, if you are unsure what the status is. For children with untreated pulmonary AVMs, if their oxygen saturation is not normal, strenuous exercise may put them at extra risk and I usually recommend against it until treatment of the AVM has improved the situation. Even if the oxygen saturation is fine at rest, this may change during exercise. In cases where this is not clear, I perform a formal exercise test in the clinic. This also often provides comfort to parents as the test can determine the "safe" level of exercise that their child can participate in.

Ask The Doctor: Dr. Brian Graham

Q: What are the risks and precautions an HHT patient should be aware of when getting a tattoo?

Dr. Brian Graham, Co-Director of University of Colorado HHT Center: I have come to the consensus that it should generally be safe. I'd recommend you not have a tattoo applied to a visible telangiectasia, as that may bleed quite a bit. Be sure that the skin was carefully cleaned at the site, such as with rubbing alcohol, to avoid any infection, and probably avoid areas that are less sterile such as in the pelvis.

If you have known pulmonary arteriovenous malformations (PAVMs), consider being extra careful by taking a single dose of an antibiotic at the time of the procedure, to avoid any bacteria released from the skin passing through a lung AVM and going to the brain or other places in the body and causing an infection.

As a more general precaution, make sure they use clean needles (to avoid getting infected with HIV or hepatitis) and stick to reputable places, rather than doing it “homemade."

Let’s Get Personal – HHT in Women

Presenter

Dr. Els Maria de Gussem is an HHT clinician in the Respirology Division at Grace Hospital, University of Manitoba in Canada. She has dedicated her career to developing an expertise in HHT, both in clinical care and research. Dr. de Gussem has performed a study on the influence of pregnancy in HHT along with studies on quality of life and life expectancy in HHT. She received the Robert I. White Young Clinician Award in 2009 for her clinical compassion and dedication to improving HHT care.

Webinar

Join HHT Physician Dr. de Gussem for an in-depth look at the effects of HHT on the changing female body. She will discuss the impact of birth control, pregnancy and menopause as well as recommended management or treatment options for women with HHT.

Webinar Highlights

  • Hormonal changes in women through puberty, pregnancy and menopause.
  • Pregnancy complications, prevention of complications, and recommended management during pregnancy in women with HHT
  • Birth control in women with HHT
  • Post-menopausal management of HHT-related symptoms and complications
  • HHT and Pregnancy handout and Website links provided in the webinar

ACT NOW

  • SHARE this information and webinar link with your family members affected by HHT

Cure HHT

Cure HHT is the cornerstone of the HHT community, advancing one common hope - to give those affected by HHT a chance for a normal life. This webinar series is just one of many ways we accomplish this mission. If you found value in this information, please consider making a donation NOW to keep this initiative moving forward. It is through the generosity of our donors that we can continue to provide this valuable education to the HHT community.

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Legislative Toolkit

Here is all the information, pre-written letters, materials, instructions and training you need to make an effective advocacy effort. There are no excuses - it's time to ACT NOW .

Email Your Legislators

Support bills and message your legislators  and ask them to support amendments that will make a positive impact on the lives of those who have HHT.

 

Ask Family, Friends, Co-Workers, and Neighbors to Contact Their Legislators

Send your family, friends, co-workers, neighbors, etc. an email with information about legislation and ask them to support you by contacting their legislators.  Every entry is directly emailed or delivered to your representative. Each office keeps records on how many emails they receive.

You don’t have to have HHT to be an advocate!

 

Follow-Up with Your Legislators

Following-up with your legislator is very important! Check our sponsorship list regularly to see if your representative has joined the Cure HHT mission. If not, get on the phone with them or send them a letter.

> Find Your Senator    >Find Your House Representative

Don't forget to send your Senator or Representative a thank you letter following the meeting. Here are some templates for you to work from!

> Senate Thank You   > House Thank You

District Visits

Meeting with your legislators at home in their district offices is one of the most effective methods in gaining their support and co-sponsorship. Legislators are usually in their district office when Congress is in recess.

Additional Legislative Documents

HR 1849 HHT DATA Act Summary 2015

HR 1849 HHT DATA Act Dear Colleague Letter

Sample - Dear Congressman Letter

Senate - Sample Appointment Request

House - Sample Appointment Request

CDC Final Report - HHT June 2009

Advocacy 101

These resources will help you successfully and locate your Senators and House Representatives as well as guide you through the communication and appointment process.

How a Bill Becomes a Law - written explanation

How a Bill Becomes a Law - chart diagram

Glossary of Congressional and Legislative Terms