The following is a personal post about Tamara Grand’s family’s experience with heart disease and as such, is rather long. Tamara debated editing more, but found it oddly therapeutic to write. Why every day is heart health month at my house.
Read more from Tamara Grand on her blog, Fitnitchick.
In my house, we don’t wait until February to think about heart health; every day is heart health month for us. Every eight hours we are reminded of the effects of heart disease.
My 12-year old daughter, Clara, has chronic pulmonary arterial hypertension (PAH). Three times each and every day, we must stop what we’re doing and administer her medication.
PAH is characterized by having abnormally high blood pressure in the arteries of the lungs. It forces the right side of the heart to work harder than normal and typically leads to enlargement of the muscle and damage to the small blood vessels of the lungs over time.
There is no cure for PAH. It is is a life long and life-limiting condition. It affects women more frequently than men.
Causes include autoimmune diseases that damage the lungs (e.g., rheumatoid arthritis and scleroderma), blood clots in the lung (e.g., pulmonary embolism), heart valve disease, HIV infection and lung diseases including COPD and pulmonary fibrosis. It can also be caused by congenital birth defects of the heart.
Clara was born 4 weeks premature. A teeny, tiny thing weighing in at just 5 lbs, 3 oz. However, she passed her Apgar with flying colours and we left the hospital the day after her birth.
Always a sleepy baby, Clara would frequently doze while breastfeeding and needed to nurse hourly around the clock. We attributed it to her small size and thought nothing of it. At her one month well-baby check up, the doctor suggested that we start supplementing breast milk with formula. Clara was not growing well (I believe ‘failure to thrive’ was the term he used) and had slipped below the 5th percentile for both height and weight.
We were referred to a pediatrician for assessment and told to increase the concentration of her formula and alternate nursing bouts with bottle feeding (breast feeding is much harder work for babies than feeding from a bottle; Clara needed both the extra calories from the formula and the easier option so as not to exhaust herself while feeding).
At her 4-month check up, the pediatrician detected a ‘heart murmur’, a little extra heart sound that was most likely ‘innocent’, but worth investigating further. We were referred to a paediatric cardiologist and after two months of waiting, finally arrived at the hospital for a series of tests (echocardiogram, electrocardiogram and blood work).
Immediately upon examining the echo, the cardiologist confirmed that the murmur ‘was not innocent’ and in fact was caused by a congenital heart defect. Clara had both a ventral septal defect (VSD, more commonly known as a ‘hole in the heart’) and a patent ductus arteriosus (PDA; the artery that allows blood to bypass the lungs in utero and automatically closes with baby’s first breath of air remained open). She would need surgery immediately.
After a quick consultation with her team, our cardiologist called to tell us that they would not be able to close the hole until Clara was significantly bigger. (It was a fairly large hole, in a difficult to reach spot). Instead they would perform a ‘bandaid’ procedure; a surgery to reduce the volume and pressure of blood travelling from the heart to the lungs, in an attempt to prevent damage to the pulmonary artery until the definitive surgery could be performed. We had two weeks to prepare ourselves.
I recall very little about that hospitalization, other than the relief I felt when, after 3 days in ICU, they passed me my baby girl to nurse for the first time post surgery. I openly wept as I put her to my breast. (I took photos the whole time she was in hospital, much to my husband’s horror, to remind myself and to later, show Clara what had caused the scars on her back and chest. Out of respect for her privacy, I’ve chosen not to share them here).
The next year passed without incident. Clara put on weight and met all of her developmental milestones. Despite her healthy outward appearance, we knew that it was only a matter of time before we’d be back, once again at the hospital, this time for a longer and more invasive, open-heart procedure.
Clara’s second surgery was scheduled for mid-July of 2004; exactly 6 weeks after her younger brother was born. Unlike before the first surgery, we met with the hospital social worker and psychologist to prepare us for what was to come; a bigger incision, a higher likelihood of complications and a potentially longer time to recovery and healing.
What they didn’t prepare us for was the possibility that the surgery wouldn’t be the end of Clara’s health concerns.
I could write an entire post about the 11 days we spent in hospital (5-6 more than is normal for this surgery). About the difficulty the ICU staff had in getting her off the pain meds and breathing comfortably on her own. About the lack of sleep and the constant driving to and from the hospital. About 16 hour days at the hospital with an infant. About O2 sats and PA pressures and drainage tubes. About the moment we almost lost her. About not seeing her smile or laugh for nearly a month after she came home from the hospital. But I won’t. Dredging up those memories still makes me cry.
In the end, my daughter’s surgery was only a partial success. Sometime between the first and second surgeries, the PA band slipped out of position, resulting in permanent damage to her pulmonary artery. She takes medication daily to reduce her PA pressure, which is still significantly higher than yours or mine. There is no expectation that it will ever be normal and we’re ecstatic every time we see the cardiologist (now only every 6 months…) and hear the words ‘no change’. No change means that the disease is not progressing. Not progressing is good.
Post-surgery tests revealed that the hole wasn’t entirely closed. There is a small ‘jet’ remaining, which puts her at risk for bacterial endocarditis (a life-threatening infection of the heart muscle). She must take prophylactic antibiotics before she visits the dentist and chest colds cannot be left to linger, lest she end up with pneumonia (been there, done that). Flu shots are mandatory for our entire household. Bi-annual blood work is required to ensure that the medications aren’t damaging her liver.
And as I write this post, we are waiting for a call from the hospital to schedule yet another visit to the cardia catheterization lab. A routine procedure for the doctors, but not without stress and anxiety for my husband and I.
She’ll never be an endurance athlete. Running for more than a few minutes leaves her out of breath.
She may never have children. Women with PAH are discouraged from becoming pregnant.
She can’t take birth control pills. They interfere with the medications that keep her hypertension in check.
She may someday require a heart-lung transplant. We never talk about this possibility.
Upon meeting Clara, you would never suspect that she lives with a chronic heart condition. She is smart, funny, energetic, loving, tenacious, caring and empathic.
Despite the hole, she has the biggest heart of anyone I’ve ever known. I love her with all of my being and cherish every moment we have together (um, except for maybe the ones in which she’s fighting with her brothers…).
Do you know any one with pulmonary arterial hypertension?
How are YOU taking care of your own heart health?
Jody - Fit at 57
Saturday 28th of February 2015
Thx for sharing Tamara!