Congenital Heart Defects Theme for 2012 Birth Defects Prevention Month!

For a heart mom like me, this is an exciting month as the National Birth Defects Prevention Network (NBDPN) promotes January 2012 as National Birth Defects Prevention Month!  This 2012 theme focuses on congenital heart defects, "And the Beat Goes On… Looking to the Future for Healthy Hearts." The NDBPN Education and Outreach Committee has developed prevention materials, fact sheets and preconception checklists to assist state program staff in promoting birth defects prevention during 'January is Birth Defects Prevention Month'.  A complete 2012 packet is available at the NBDPN website.  There are two great resources available for families to who would like to learn more about congenital heart defects, "Maternal Steps to Help Prevent Congenital Heart Defects" and "5 Things You Need to Know about Congenital Heart Defects." Please share this information with your families and friends to increase awareness of congenital heart defects. 

To see what programs are available in your state please visit the NBDPN State Program Website.  To view your state's specific birth defects profile visit the NBDPN State Specific Profile Website.

Are you interested in promoting CHD awareness? The National Health Observances Toolkit provides sample announcements, tweets, web badges and e-cards to assist you on your mission! 

The Centers for Disease Control and Prevention offers FREE brochures, fact sheets, and other educational materials to promote CHD awareness in addition to links for activities and prevention. 

The March of Dimes promotes CHD awareness in healthy pregnancy and pregnancy planning in their News Moms Need - what moms and moms-to-be need to know Blog.

If you have questions about the NBDPN January 2012 Birth Defects Prevention Month, please contact Mary Knapp at Mary.Knapp@doh.state.nj.us or Cara Mai at cmai@cdc.gov. 

My Wish For You

As the holidays approach, I am reminded of our blessings and how our amazing journey began, and continues as our child born with a “half a heart” has more heart than I could have ever imagined. I came across this fitting poem to share with other heart families who have also faced this journey.  I wish you all the very best this holiday; love, peace, strength and happiness. See in the next year.  Julie


CONGENITAL HEART DEFECT

Somewhere....someplace...today....
A family is waiting to hear.....
Is something wrong with their baby?
The answers aren't quite clear...
This family has entered an unwanted world...
And they just don't know what to expect...
Somewhere...someplace...today
They first heard the words: heart defect.
And how they hoped this was not true...
And thought...this cannot be...
I too...know just how this feels..
This happened to ME...


Somewhere...someplace...today...
A man and a woman embrace...
Their baby is in surgery...
They long to see her face...
They haven't got to hold her yet...
Without...a cord or line...
They pace the room awaiting news...
And hope she'll be just fine.
Prayers fill this busy waiting room..
And mom and dad are scared...


Somewhere...someplace..today...
The tiniest hearts are repaired.


Somewhere...someplace...today...
A child's growing fast...
Smiling, laughing, thriving...
Her mom thinks...can this last?
It's almost easy...to forget
That anything is wrong...


Somewhere...someplace...today...
Her child seems so strong.


Somewhere...someplace...today...
A little girl fights...just to live...
A father holds her tiny hand...
His love...all he can give...
The doctor's are all baffled...
They fear that she might die...


Somewhere...someplace...today...
A family says goodbye...


Somewhere...someplace...each year...
More than 40,000 families will see...
What it means...when something's wrong...
They'll face a CHD.
Today...for just a moment...
Stop...remember...reflect...
My life has been forever changed by a heart defect.

-Author Unknown

An Overview of Children’s Hospital of Wisconsin, Herma Heart Center

 The Hospital with the Best Published Survival Rates for

Hypoplastic Left Heart Syndrome Worldwide

       The following information is available from the Children’s Hospital of Wisconsin Website 

Hypoplastic left heart syndrome is one of the most complex congenital heart defects. Less than 20 years ago, most infants born with this defect died. Today, repairing this defect and other complex single ventricle lesions accounts for a large part of the work we do. At the Herma Heart Center, each era had significant advancements in preoperative, surgical and postoperative management techniques that are reflected in the improving outcomes, with survival rates over 90 percent since 2000. The Herma Heart Center has the best published survival rates worldwide for treating hypoplastic left heart syndrome (see Tweddell JS. Hoffman GM. Mussatto KA. Fedderly RT. Berger S. Jaquiss RD. Ghanayem NS. Frisbee SJ. Litwin SB. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. [Journal Article. Research Support, Non-U.S. Gov't] Circulation. 106(12 Suppl 1):I82-9, 2002 Sep 24). The center has set national benchmarks for surgical outcomes of the Norwood procedure, the first of three operations to treat this defect, and has been a pioneer in development of monitoring tools that have become standard practice throughout the world. What we're doing to provide the best care: We have the only cardiothoracic surgeons in the state of Wisconsin with American Board of Thoracic Surgery Certificates in Congenital Heart Surgery. We continue to investigate and develop better perioperative techniques to improve safe recovery for children with hypoplastic left heart syndrome, including drugs that minimize the effects of heart-lung bypass and novel non-invasive tools to measure how blood is flowing to the body as a gauge of heart recovery. Through the Fetal Concerns Center of Wisconsin, we can diagnose many birth defects and other health concerns in unborn babies and offer counseling and coordinated medical care for mother and baby. This allows families and staff time to anticipate needs and plan care, which improves the care provided at birth. The Froedtert & Medical College Birth Center is located inside Children's Hospital to reduce the time it takes to get a fragile newborn into surgery. Research shows outcomes improve when the delivery room and operating room are close together. This also offers families the added convenience of having mother and baby hospitalized near one another. We established an access and feedback work team to make sure patients and referring physicians always have easy access to our facilities and specialists. Our goal is to communicate with and transfer care back to the patient's community physician for seamless follow-up and long-term care. We have a highly specialized Children's Transport Team available 24 hours a day to stabilize and transport seriously sick and injured infants and children to our center. More than 1,500 transports are provided each year.  We continue to investigate new techniques to improve our ability to stabilize and recover infants with hypoplastic left heart syndrome, including the use of novel perioperative monitoring techniques and special medical therapies. We initiated a home monitoring program in 2000 that dramatically improved infant survival rates after patients were discharged home following hypoplastic left heart surgery. We train parents to measure fluid intake, weight gain or loss and oxygen levels daily. Clear guidelines allow parents to provide objective data to clinical staff, which lead to earlier interventions if problems arise. We offer a special developmental follow-up clinic for children with serious heart conditions to identify any problems as early as possible and provide support as necessary. Patients/Families - If your child is being treated for a heart defect: Be an advocate for your child. Participate in daily bedside clinical discussions, provide any information about your child that may be helpful to staff. Follow medical instructions fully and carefully before and after surgery. Ask questions if you don't understand the plan of care or if you are not sure how to care for your child at home. Attend any and all follow-up appointments.  Referring physicians: Fill out and fax a referral form prior to sending the patient to Children's Hospital for outpatient ancillary services or a specialty clinic visit.  Forward any pertinent patient documentation, including test results, to the specialty physician's office prior to the patient's visit.  If you have questions about this data or information, e-mail us or call (414) 266-6556.

The Thymus: to Remove or Not to Remove? That is the Question.

Why do I ask?

I believe it's very relevant to a parent of a child with Hypoplastic Left Heart Syndrome who has had surgical repair, because in order to access the heart, a surgeon must remove the thymus located above it.

What is the thymus?

The thymus gland is an organ of the immune system. The thymus gland is located in the upper part of the chest cavity, directly behind the sternum. It is largest in size in childhood, about the size of an apple, continues to grow throughout puberty, and slowly shrinks in size into adulthood, to the size of a pea.

                                

What does the thymus do?

The role of the thymus gland is to process lymphocytes, which are white blood cells that travel the body through the bloodstream. These lymphocytes, also called T-lymphocytes or T-cells, protect the immune system by blocking the invasion of viruses and bacteria. They also help to prevent abnormal cell growth that occurs with cancer.

What we don't know.

If the thymus gland is removed in infancy, the immune system may never fully develop. Does this increase the chance of infection in children who have no thymus gland or whose thymus never developed properly, because the majority of t-cell production occurs early in life?

What we do know.

Ancient Greeks believed the thymus to be the seat of the soul, but for centuries its significance remained unremarkable. It wasn't until 1961 when Dr. Jacques Miller, a French-Australian immunologist, solved an ancient medical mystery and proved an unbelievable result…the thymus was of crucial importance to the immune system.

A 1982 article in The New York Times discusses aging and the gradual decline in immune function, demonstrating the importance of the thymus gland in function and protection of the immune system. Many studies since the initial finding of Miller in 1961 have supported the importance of thymus related to asthma, allergy, dermatitis, rheumatoid arthritis, autoimmune disease and cancer.

What does this mean to a child with HLHS?

Are children who have had their thymus removed potentially becoming immunodeficient?  Many doctors do not believe there is an increased risk of infection in children who have had their thymus removed. Does the medical community underestimate the important immunity function of the thymus gland? Research experiments in the 1960's proved it was a necessary organ, and current research studies are being conducted to understand the relationship of thymus gland function and immunity.

Examples of current research.

In 2004, researchers at Children's Hospital Iceland, Reykjavik, published "The influence of partial or total thymectomy during open heart surgery ininfants on the immune function later in life," which describes their findings from a 10-year study conducted on nineteen children who underwent open heart surgery compared to nineteen healthy controls of the same age and gender. Each subject's blood was tested at age ten.  The study group had lower counts for lymphocytes (part of the white blood cell that recognizes a foreign substance) and higher counts for neutrophils (part of the white blood cells that respond to infection) compared to the control group confirming lower t-cell production, but did not demonstrate clinical significance early in life.

Similar studies with similar findings.

Immunodeficiency following neonatal thymectomy in man

Levelsof Recent Thymic Emigrant Cells Decrease in Children Undergoing Partial Thymectomy during Cardiac Surgery

Thymic Function and Impaired Maintenance of Peripheral T Cell Populations in Children with Congenital Heart Disease and Surgical Thymectomy

Complete DiGeorge syndrome: Development of rash, lymphadenopathy, and oligoclonal T cells in 5 cases

Longitudinal analysis of immune function in the first 3 years of life in thymectomizedneonates during cardiac surgery

For neonates undergoing cardiac surgery does thymectomy as opposed to thymic preservation have any adverse immunological consequences?

Where do we go from here?

In 2002, a Monash University researcher presented a very interesting finding in the study, "Regrowing the Thymus Gland," in which he describes the successful regrowth of the thymus back to full size in mice, to begin producing t-cells again to strengthen the immune system. Barring the regrowth of the thymus gland, strengthening the immune system today can be approached in several ways. Proper nutrition in the form of a well balanced diet and antioxidants is one approach. Sweetbreads, made from the thymus glands of young calves, like many organ meats, are a nutritional powerhouse packed with vitamins, minerals, amino acids and other substances vital for nutrition. Although not well studied in humans, supplementing a diet with the thymus gland extract is said to boost the immune system. Thymic hormone replacement therapy, although unapproved and unregulated by the FDA, has demonstrated some potential benefits clinically, with the regular use of thymus therapy.

We simply need more research. Thymus re-implantation in the future? Seems like an inevitable course. 

Author's Note:  My son was diagnosed at 18 months of age with Common Variable Immune Deficiency, a primary immune deficiency.  For more information about primary immune deficiencies, please visit The Immune Deficiency Foundation.