Gastroschisis, the what.....
Gastroschisis is a birth defect that causes the intestines to protrude from a hole in the abdomen, typically to the right of the umbilical cord. Occasionally, other organs are involved as well. This must be repaired surgically after birth, although surgery is not always possible immediately and depends on many factors including the amount of bowel outside of the body. While approximately 90% of affected infants survive in industrialized nations with proper medical care, there is still a 10% mortality rate due to complications or related defects and infections – even with the best medical care. [In the USA, published survival rates range from 83%-97%1]In nations without access to proper medical care, the mortality rate is often 100%.
In the United States, Gastroschisis affects 1 out of every 2229 babies according to the CDC2, although the actual rate varies by state. California, for example, published a 2005 report which showed the incidence to be 1 in 1727 births3and Texas rates show the incidence to be 1 in 1683 in 20094. In Canada, the current rate is 1 in 2272 with an exception of the area of Nunavut, which for unknown reasons is as high as 1 in 510 births5. Worldwide, rates of gastroschisis in industrial nations vary between 1 in 2000 and 1 in 33336, although some regions have higher rates, such as Wales which is reported at 1 in 16127 births and an area in Australia with an incidence as high as 1 in 8928. According to the International Clearinghouse for Birth Defects Surveillance and Research, a 2009 report from 16 separate registries shows that South America as a whole has the highest reported incidences of gastroschisis in the world, at 1 in 1063 births4. It is worth noting that there is little to no data collected on incidences in Africa.
Rates of gastroschisis are increasing worldwide, and this trend is being called a “pandemic” by many researchers9. The medical community still does not know what causes gastroschisis, but researchers have found many correlations and risk factors including aspirin and other medication use10, exposure to certain chemicals, low maternal body mass index, and genitourinary infections11. Studies from Utah are demonstrating a familial link to gastroschisis, with one study showing that 23% of families studied had more than one relative with gastroschisis12. However, the single largest risk factor for having a child with gastroschisis continues to be young maternal age. The CDC continues to monitor these trends in the US13. Additionally, a 2006 study from the UK showed that mothers under 20 years old have an incidence of gastroschisis ranging from 1 in 1123 to 1 in 40914.
Although the overall prognosis is good for most infants born with this birth defect provided they have access to medical care, complications do arise. Studies show that gastroschisis is the leading cause of intestinal transplantation15, and that 40% of gastroschisis survivors will need to be readmitted to the hospital at some point due to complications, the most common of which are bowel obstruction and abdominal pain16. Furthermore, having a child with a birth defect can put many stressors on a family that others simply do not experience, and it is recommended that families have a strong support network17.
The Why of Avery's Angels:
Avery’s Angels Gastroschisis Foundation is the only nonprofit in the United States to assist and support those affected by gastroschisis. When we first formed, Avery’s Angels was the only nonprofit in the world with this cause; now we are joined by the Luey Jacob Sharp Foundation18 in the United Kingdom.
In 2008, Avery's Angels founder, Meghan Hall and her partner Jared Rauen found out that their little boy would be born with gastroschisis. During Avery’s life, he not only provided them with hope and joy, there was an incredible rise in their community to support the little family through the process. From meals, to gas cards, prayers and thoughts to folks donating blood in solidarity for Avery’s fight. Even Avery’s team at UNC Chapel Hill NCCC was also heavily involved in educating and supporting Meghan and Jared during Avery’s time. There were angels around them the entire time. Avery stayed 107 days in the NICU- from April 14th 2009 and passed on July 30th 2009 unexpectedly from unforeseen complications. During this time the little family had many happy memories and a well-supported experience because of the tremendous efforts of their entire community.
At the time there was not a centralized organization for support or information. Inspired by the “angels” that had been present in their own experience and fueled by the hope that was Avery’s life, Meghan began blogging about forming a foundation to promote that community of support, education and awareness for others affected- providing the same angels they had, for others. With the encouragement of “Avery’s Angels” the team at UNC Chapel Hill, their family and community, Meghan launched Avery’s Angels September of 2009.
Between Meghan’s first blog post and Avery’s ngels acceptance as a registered 501c3 nonprofit on April 29, 2011, some amazing individuals came together and worked collaboratively to make the dream of a gastroschisis nonprofit possible. The initial core team was Meghan Hall, Herb Richter, June McDowall, Sheila Palus, and Kayte Thomas. The core team worked hard on planning, advocacy, legal work, and logistics to make the foundation possible. Added in soon after were Lea Krutel, Megan Turcotte, and Autumn Hernandez who further contributed to the founding efforts. These passionate pioneers were all impacted by having a child in their family born with gastroschisis – a combination of parents, grandparents, and great grandparents who wanted to make the world a better place for others who were dealing with the same experiences. Each of them brought a unique perspective to the budding organization because of their specific outcome with gastroschisis; they brought with them the knowledge of life, death, short bowel syndrome, divorce, advocacy, heart break, and love beyond measure, amongst other things. It is to this committed group of people that the world owes gratitude for the existence of the first nonprofit in the world dedicated to supporting gastroschisis families.
To this day, we are founded on that initial cause of providing support to families as being our primary purpose, funding the needs of the cause above all else, and encouraging research to discover the causes and promote prevention.
What do we do? And why do we do it?
Avery’s Angels Gastroschisis Foundation’s mission is to promote awareness, research, and support for gastroschisis worldwide. We accomplish this by:
– Creating a national and international network of families dealing with gastroschisis and related complications.
– Providing emotional and financial support to families who have or are expecting babies born with gastroschisis.
– Supporting friends and families of survivors of gastroschisis and those who have lost a loved one to this disease.
– Promoting national awareness annually via Gastroschisis Awareness Day events every year on July 30th.
– Collaborating with clinicians to facilitate research to find the cause.
– Assisting in other ways as indicated by the circumstances.
AAGF is an all-volunteer foundation. There are no paid employees. We depend on your donations and volunteers to accomplish this vital mission. Please encourage family and friends to “Get Involved.” To learn more about how you can help visit the Volunteer page on our website!
It is tremendously important that our parents and patients have lifetime support, which is what Avery's Angels is founded on providing. The initial NICU stay can range anywhere from 14 days (our personal record!) to over a year. One of our parents, Laura Violet Marie writes on the most important advice for an expecting or NICU gastroschisis parent is:
"Patience! It's probably the hardest thing to do but you cant rush those little tummy's.. We all want to go home right away and as each day adds to your total you get more and more anxious and envious of each mom that got to take their baby home the next day.. But your time will come and your baby will heal in the time that he is she is supposed to! Tell people to stop asking you when he/she will be coming home! I was frustrated when people would ask me because i myself didnt know and i follow gastro mom's stories now and see countless people comment on their pictures "when is she coming home? How much longer?" And it aggravates me for them!"
This is so true! Gastroschisis is variant, based on many physiological features, how large the defect is, how much intestine was out, how extensive the damage was/is, what gestational age the baby was born at, how the bowel is recovering or functioning. This is why it is crucial that our parents have proper support from those who have been through this and most importantly set up a good line of open communication with their physicians and practitioners. Gastroschisis parent Erin Herrman says:
"Most importantly, ask questions. It's scary to leave your new born there alone ( alone because YOU are not there 24/7). The worst feeling is to be alone and confused. Do not worry that your question(s) might sound stupid. There is no such thing as a stupid question. Knowing and understanding everything your newbie is going through will make the time apart more bearable and make you feel more comfortable knowing the best health care professionals are there to treat your baby in a way you may not be incapable of."
This is crucial. While it is easy to get caught up with how disenfranchising being a NICU can feel for a parent, it is important to take a breath and find the right way to communicate with those around you. Remember, the physicians and practitioners are there with the same goals as you are- healing, health and home. It is extraordinarily difficult to build a relationship in the NICU especially with so much going on, but speaking up, speaking often and seeking those opportunities will not only benefit your child, it will also help a parent heal from the NICU experience too.
Recovering from your NICU experience is important. Especially for our gastroschisis parents. Having confidence in both yourself as a NICU parent and the medical relationships you are forming is crucial. Building those healthy relationships with the team will help families feel more confident at home. Most importantly if and when signs may pop up at home that warrant seeking medical involvement, help families will not have as many reservations for seeking timely professional help. Odd temperature spikes, tummy pains, changes in stools, excessive vomiting, distention in the bowel area can all indicate concerning signs that timely medical intervention can improve outcomes for.
Support is everything, so asking for help is the name of the game, Gastroschisis mother Belinda Smithson says:
"We didn't receive any help from anyone during our stay or when we arrived home. It was awful and I wasn't going to ask for it. Before you go into hospital write a list, or get others on here to write a list of things that could be of help. We lived on takeaway food for the whole time and it was awful frown emoticon a nice home cooked meal would have been lovely. This goes for when you get home too."
But make sure you have a good balance of NICU visitors (remember! more people bedside ups the risk of infection!) once baby gets home it is OK to take your time with visitors if you feel you need alone time (finally!). Gastroschisis mom Sarah Boyer says:
"Be sure to make time for you, your husband and child to get things back to normal before inviting any other family members over. Whether this takes a week or a month...they can wait. Of course, if you need help, ask them! But don't feel obligated to have everyone over right away."
Support is what our families need, from their practitioners to their families and also in the resources that are available to them. That's where Avery's Angels has found its purpose. We not only provide all the information accessible to our families, we also have over 10 programs to help support our families across the entire NICU experience. We are here to be your angels!
Learn more about Avery's Angels at www.averysangels.org