Congenital Heart Defects
Symptoms - bluish skin (cyanosis), breathlesness, rapid breathing, rapid pulse, poor appetite, poor development, exhaustion, difficulty sucking (infants) and heart murmurs.
Description - Congenital heart defects are usually evident before birth although sometimes they do not surface until childhood or later. The defect may be severe enough that the child will not survive outside the womb, or will need immediate surgery, or will need mechanical assistance until surgery is possible. Difficulty breathing, bluish skin, lethargy and an inability to suck (because sucking requires energy) may signal a less dramatic heart defect but one which nonetheless will require medical attention and ultimately surgery. Sometimes symptoms are subtle: a young child easily winded by exercise, easily tired, small for his age, thin for his build, and bluish from extertion may be suffering from congenital heart disease. A heart murmur can also signal a congenital heart defect, even in the absence of other symptoms.
It is not clear what causes most congenital heart defects. Heredity is certainly a factor - children with heart defects tend to come from families with a history of congenital heart disease. Chidren with genetic disorders such as Down Syndrome and Marfan Syndrome are more likely to have congenital heart defects. Children born to mothers who contracted German measles (rubella) during their first trimester, or who drank heavily, or took medicine for epilepsy, also have a higher incidence of heart abnormalities. And mothers who are diabetic or over 40 have a greater chance of giving birth to babies with defects.
About one in every 100 babies is born with a heart defect, although only about eight in 1,000 will have serious malformations. Most of these defects occur in the first eight weeks of fetal development. Partitions between the heart's chambers may have abnormal connections between the two sides of the heart. Or the valves connecting the chambers may be constricted, closed or in the wrong place. Blood vessels may not develop properly, or veins and arteries are transposed. Defects that are not immediately fatal can interfere with the efficiency of the heart. The telltale signs of congenital heart disease are blue skin, labored breathing, marked lack of energy which occurs because the heart is circulating too much blood that has not been oxygenated.
Congenital heart defects require the attention of a medical specialist. Some defects such as a small hole in the wall between the heart's lower chambers (a ventricular septal defect or VSD) may actually correct themselves and need only to be monitored by a doctor. Others such as a slightly narrowed heart valve, may be so minor that no treatment is required. More serious abnormalities such as a large hole, very constricted valve or displacement or major arteries require surgery.
Symptoms - none.
Description - A heart murmur is the presence of noise between the beats of the heart. Heart murmurs are usually detected only by doctors with the aid of a stethoscope. Innocent or functional heart murmurs are extremely common in young children and typically disappear altogether by mid-adolescence. Occasionally, a murmur is something more than just the noisy flow of blood through the heart.
A congenital heart murmur signals abnormality of the heart. A murmur without the presence of far more visible symptoms such as bluish skin, breathlessness, or poor growth is not likely to be life-threatening, eve if it is congenital or present at birth. Children with congenital heart disease do have heart murmurs, but the murmur is not the identifying symptom unless the defect is very minor.
GEM's Heart LinksReal Stories: Stories of other parents experiences and lessons on life with a child who has CHD
Jonah's Page: Chronicles the experiences of Jonah's parents.
Kids With Heart:
Congenital Heart Disease:
Congenital Heart Disease Resource Page - Heart Links: Links to various congenital heart resource webpages
CHASER - Congenital Heart Anomalies Support, Education and Resources: Support group involved in parent-to-parent networking, also distribute a newsletter.
Braveheart Young Adults Group: Braveheart YA is a group to help young adults with congenital heart disease in Scotland.
Hypertropic Cardiomypathy Associaton:
Left Heart Matters: Web page supporting children and parents associated with congenital heart defect Hypoplastic Left Heart Syndrome.
Moms With Heart:
Children's Heart Society:
When Geordi Elizabeth Maybury made her entrance into the world on September 3, 1993, it was nothing short of a miracle.
"Early in the pregnancy an ultrasound revealed a problem with Geordi that the doctors believed would cause her to spontaneously terminate," says Liz. "They gave us little hope and based upon the facts abortion seemed the only logical action."
With doctors telling the couple abortion might be the best decision, Liz tried to work out her feelings in a journal entry dated March 17, 1993.
"Which is worse, terminating her now, aborting a life that appears to be headed for incredible suffering and pain or selfishly choosing to have her just in case her heart can be fixed and so I can hold her - even once. Die briefly in little pain or take a chance and prolong the suffering but still fate is inevitable," Liz wrote. "I can't live with either decision - emotionally I'm depressed regardless - so all that matters is her - what is best for her?"
When Paul and Liz's reluctance to support abortion under any circumstances became known to family members, Paul's maternal grandmother wrote to the couple on April 8, 1993.
"I hear that you are having a bad time with your pregnancy Liz, I am sorry about that! The only thing you can do is listen and consider carefully what the Dr. has to say, and after thinking it over carefully, follow your own convictions! I had a miscarriage at 4 1/2 months, and was devastated until I heard of the baby's condition. His hands and feet were on backwards as well as other deformities. He could have had a lot of pain too! That I wouldn't want, although our baby was certainly wanted! He would have been a laughing stock, and I gradually realized that what happened was for the best - not for us, but for him! All you can do is what you very likely have done - think seriously about what is best for both of you and your baby!"
The couple found inspiration in their decision not to abort from a Star Trek: the Next Generation episode called the Masterpiece Society.
"Something about 'who is to say my quality of life is not worth living'," says Paul of the blind character Geordi LaForge. "Liz decided we had to name her Geordi."
With the pressure to abort gone and depression left, Liz busied herself working on how to give Geordi the best life she could have outside the womb.
"I carefully thought everything though," she says. "I worked on formulating a parenting plan that I could follow after she was born. I tried to eliminate the psychological crutches in my own life so I could meet her needs and not have her meet my own needs."
Determined to get Geordi to 20 weeks of gestation Liz says she was prepared if Geordi had been stillborn "at least she would have been a baby."
"I can't explain how it feels waiting around for your body to betray the life of a human being," she says. "I was waiting for my body to fail."
Despite her fears and pressures from relatives the great love Liz had for her unborn child kept her from making the choice to abort.
"If I had aborted I would have been dead in a year," Liz says. "I knew I couldn't handle it."
Liz had dealt with her father's murder years ago through Bereaved Families but she found she needed a sympathetic ear to listen to her fears and pains from her childhood. Liz found her confident in Paul's mother, a social worker having held jobs in victim crisis counselling and as a child protection worker.
"I was working it all out while she listened," Liz says. "I wanted to be able to create a trusting loving environment so Geordi would have the heathiest environment possible. I decided early on that if I can't give her a perfect heart then I could try my best to give her perfect love."
On October of 1994 reflecting upon her pregnancy while watching Geordi sleeping "I feel confident that I did everything right but I also gasp at the risk I took."
"I loved being pregnant even during all the tests."
With many holding little hope for Geordi's survival, Paul and Liz put off purchasing baby things until the day Liz went into the hospital to have a voluntary C-section performed sparing her child stress of a vaginal delivery. The first item purchased for Geordi was a baby sling which was bought before they went into the hospital.
"A baby doesn't care if it has lots of things," Liz explains. "Geordi would never be associated with things, she was not the essence of her possessions."
Paul's older sister loaned the couple some baby clothes which his mother had washed and folded getting the clothes ready for the new arrival, her long-time boyfriend, a Woolco manager, also bought the couple a baby monitor.
September 10, 1993 Liz would undergo an elective Caesarian section. At 11:45 a.m. Geordi Elizabeth Maybury was born, weighing in at 2800 grams (just over six pounds).
"Since we were told that the heart problems may have a 50 percent chance of recurring, Liz had her tubes tied after Geordi's birth," says Paul.
As Liz wrote to one friend on November 7, 1993, "Paul and I have made the decision not to risk another pregnancy, we had enough excitement this time for four babies."
While fears remained that emergency heart surgery would have been necessary, a physical examination showed that Geordi was within normal limits and she remained stable from a cardiorespiratory view.
A full cardiac evaluation found Geordi did not have any evidence of congestive heart failure or worsening right side obstruction of her right ventricle. Geordi was seen in consultation by the Genetics Division of Sick Children's Hospital Toronto, because of her family history of congenital heart disease, webbed neck and single umbilical artery.
Initally Geordi had some feeding intolerance over the first 48 hours of life but this quickly resolved and over the first five days of life she increased weight daily.
"Geordi was born with multiple heart defects - multiple ventral septal defects and atrial septal defects (holes between the left and right ventricles and atria) plus pulmonary stenosis (a narrowing of the pulmonary artery), complicated by a thickened pulmonary valve," says Paul. "It was determined she was not in need of immediate surgery and we were able to take her home after only ten days."
Liz says she thinks she coped so well with Geordi's ten days in the hospital because she "recognized that birth is not really the beginning of the bonding process but is actually the first separation."
"I needed her to learn to breathe on her own before we could get to know each other on a new level. I think every step of growth in a child requires a new separation from the parent."
Originally worried about how their Husky-Lab cross, Borg (named after his attempts to assimilate pieces of furniture to service his own needs), would take to Geordi, both Paul and Liz were pleased and amazed at how gentle he was with her.
Paul had taken paternity leave for three months and as Liz described in her letter of November 7, 1993 "when he arranged this leave we were expecting Geordi to be in the hospital much longer than she was."
"We are totally smitten with our little bundle," Liz wrote thanking a friend for the baby bath given to the couple. "We are lucky because Gem appears to like baths. In fact she is a quiet, but alert and responsive baby. We are so spoiled!"
Always grateful for the many prayers and gifts they had received from friends and their church, Liz expressed her gratitute in one letter dated January 20, 1994 to the Carleton University Chaplaincy Office.
"We were thrilled to hear from you," Liz wrote. "Our precious little Gem came home from the hospital exactly four months ago today. Yesterday Geordi took her second set of immunizations like a pro and weighed exactly 12 pounds ten ounces. She is continuing to amaze the doctors with her health and spirits. We are very grateful for the many prayers for Geordi. We know they have been answered because the doctors have no other explanation for her continuing to thrive despite what was at first considered a terminal genetic heart defect. Her doctors gave permission to start solids next month (already) so the gift certificates will be used to buy her first dishes.. Thank you so much for remembering us."
Paul also expressed his gratitute in a letter to a friend dated one day earlier.
"We are very thankful to everyone who helped out with gifts and prayers over the last year. It would have been much harder without all our friends in Ottawa. I know Liz found it easier to handle the situation with the doctors knowing she could talk freely about her fears," Paul wrote. "Now Geordi is growing so well, it's hard to understand sometimes how the specialists could be so mistaken about her chances. Although there are still some problems, we don't expect her heart to limit her very much. It is enough that she is here, and we are thankful she is doing so well."
The year of 1994 would prove highly eventful for Geordi, besides celebrating several firsts with her parents and trips to Kincardine, Toronto, London, Cambridge, Brantford, Ottawa and Dunnville to visit friends and relatives, Geordi became an honorary flower girl at Paul's older brother David's wedding and went to the Toronto Metro Zoo and Paramount Canada's Wonderland.
"She slept through the whole wedding," Liz says adding she was originally worried that Geordi might start fussing during the service. "She behaved perfectly...better than some of the guests."
As a gift for being in their wedding party, David and his soon-to-be wife Teresa gave Geordi a giant stuffed Lion King Simba doll which could also doubled as a large hand puppet. The lion quickly became Geordi's favorite toy, following her around on her travels to meet friends and relatives.
Unfortunately despite their family bliss, Paul and Liz found they had a few problems. After repeated comments from his mother about Geordi's legs looking under-developed, Paul and Liz brought the subject up to Geordi's doctor on one of her monthly appointments.
"We were told Geordi's legs were fine," Paul says. "When I had told Mother what the doctor said she kept insisting the doctor was wrong, so I checked with Geordi's paeditrician and again got told Geordi's legs were fine...but Mother kept dismissing everything I told her."
Paul says with the criticism he was recieving from family members, it made the couple more paranoid about Geordi's development than they should have.
"When Geordi was nine months old, she stopped gaining weight," Paul says. "The paediatrician we had been seeing regularly contacted Sick Children's Hospital who had done a full heart check-up at six months but was told it could not be the heart."
Paul says after trying everything possible to get Geordi to gain weight "we were really getting desperate."
Looking back on the situation now and having watched food battles other parents have had with their children, Paul says he doesn't believe that the situation was as bad as he and Liz thought at the time.
"It would have been worse if she had started losing weight," he says. "She didn't, she just didn't gain any more."
Still breast feeding on demand, Geordi ate small amounts of baby food, enjoying Cheerios and baby cookies.
"She learned to feed Borg," Liz says. "She would take a spoonful for herself, then give a spoonful to him, then feed herself again."
Experimenting with new tastes, Geordi decided she liked the taste of raspberry ice tea after stealing a sip of Liz's drink in September of 1994. Later the same day she went out with her parents and newly married Uncle David and Aunt Teresa to a restaurant and would have her attempts to grab Paul's ice tea thwarted after three attempts. A quick problem solver, Geordi waited patiently until Paul was distracted. Seizing the opportunity Geordi quickly grabbed the glass to pull it to her mouth knocking ice tea all over Paul and the table, causing a great deal of laughter from an unsoaked Liz, David and Teresa.
For her new year resolutions Liz decided she would begin to read Geordi every single day and to "go to church every time I am able."
In February of 1995 Geordi would take her first steps towards crawling, bum sliding across the floor.
"Borg couldn't understand what was happening," says Paul. "One day he could sit a couple feet away from Geordi and not be bothered, the next day no matter where he went she was right beside him."
Very proud of her daughter Liz was amazed at how affectionate Geordi was, even to Borg.
"I would say hug Borg and she would flop on him and give him a huge hug," Liz says. "It was very sweet."
GEM's Surgery: February 1995 would forever change Paul, Liz and Geordi's life.