December 11, 2007
by Brian and Melissa Brewer
This article was also submitted to the Davie County Enterprise-Record, which published a shortened version on Dec. 6, 2007.
November is Pulmonary Hypertension Month.
Today, looking at Bailey at three years old, you would never guess she has a potentially life-threatening disease. She has what is considered a silent disease. It is extremely rare, it has no cure, and there are few treatment options available.
Two and a half years ago, our family’s lives changed dramatically. Our youngest daughter, Bailey, was diagnosed with Pulmonary Hypertension.
About Pulmonary Hypertension
Pulmonary hypertension (PH) is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life threatening. It is a disease that affects people of all ages and ethnic backgrounds although some people are more prone to developing PH.
PH is often not recognized until the disease is quite advanced. Symptoms of pulmonary hypertension include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting. These symptoms may seem common and simple, but they severely impair a PH patient's ability to lead a normal life. Simple activities like walking, dressing oneself, and cleaning can become difficult to perform. Thus, a normal, healthy-looking individual might be incapable of some basic functions due to PH.
When pulmonary hypertension occurs in the absence of a known cause, it is referred to as primary pulmonary hypertension (PPH). This term should not be construed to mean that because it has a single name it is a single disease. There are likely many unknown causes of PPH. PPH is extremely rare, occurring in about two persons per million population per year.
Secondary pulmonary hypertension (SPH) means the cause is known. Common causes of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus (SLE). Congenital heart diseases that cause shunting of extra blood through the lungs like ventricular and atrial septal defects, chronic pulmonary thromboembolism (old blood clots in the pulmonary artery), HIV infection, liver disease and diet drugs like fenfluramine and dexfenfluramine are also causes of pulmonary hypertension.
Although there are over 100,000 people known to suffer from PH, there may be thousands of others whose symptoms have yet to be diagnosed. Many people may have PH without knowing it or are misdiagnosed with illnesses such as asthma or bronchitis, delaying proper treatment.
Until 1990, there were no accepted treatments for PH, but today patients have options to discuss with their doctors although there is still no known cure. The goals of treatment for patients with PH, which includes medications, oxygen and transplantation, are to treat the underlying cause (if possible), reduce symptoms and improve their quality of life, slow the development of blood clots and to increase the blood and oxygen to the heart, reducing its workload.
Bailey’s Story
Bailey began showing symptoms when she was about 15 months old. She learned to walk at one year. When she mastered walking and gaining speed, her symptoms became apparent. We first noticed problems in June of 2005 when we were outside playing in the heat and humidity on a Friday afternoon. She would loose her breath easily and have a blue tinge around her lips and mouth. And, if you felt her chest, her heart would be pounding. At one point, she even fell to the ground, exhausted and limp. It was then decided that we would be seeing a doctor the following morning.
At the pediatrician’s office, we explained the symptoms and the events of the previous afternoon. We were told that Bailey was most likely having breath holding spells and it was her way of having a temper tantrum. We knew that was not the case. As a comfort to us and after talking with the pediatrician for a lengthy amount of time, an EKG was ordered at the hospital for that afternoon to evaluate her condition. Then, as we have learned over and over since, the waiting game began.
The EKG came back abnormal, Bailey’s heart was enlarged. An echocardiogram was ordered for the following Monday. Again, the wait continued. The echocardiogram indicated that there was definitely a problem but because Bailey was not very cooperative, the test was unclear. We were referred to a pediatric cardiologist. The appointment was for three weeks later. The wait continued.
No one could tell us what was wrong with Bailey. No one knew. The symptoms were clear but a diagnosis was not.
Bailey’s cardiologist appointment was on a Thursday in mid-July. At the beginning of the week of her appointment, she caught a virus. Fevers, congestion, the works. She got to the point that she could no longer walk. She had no energy, had a gray-blue tinge constantly and slept for most of the day and night. We visited the pediatrician on Monday and again on Wednesday. We were scared. Wednesday night, Bailey would doze off for a few minutes and then her chest would arch and she would cry out in pain. She was having chest pains because her heart was in such stress. That was the worst night of our lives.
We went into the cardiologist’s office not knowing what would happen. The cardiologist started going over so many different scenarios. She had concluded from the reports from her EKG and echocardiogram that Bailey had a hole in her heart and it could be fixed with a simple surgery. We were in shock. Our daughter would need heart surgery. Then, the cardiologist listened to Bailey’s heart. Everything changed immediately. She told us the Bailey did not have a hole in her heart, she had pulmonary hypertension. We were so relieved that surgery was no longer needed. If we only knew what pulmonary hypertension meant for Bailey.
Bailey was admitted to hospital for a series of tests including x-rays, sedated echocardiograms, heart catheterization, and blood work. A heart catheterization is the only way to get true pulmonary artery readings. And, during the heart catheterization, the different types of treatments were introduced to Bailey to see what she responded to. Only 25% of people with PH respond to treatment. Fortunately, she had a response to all of the treatments. After the official diagnosis of Primary Pulmonary Hypertension (PPH) on July 11, 2005, she was started on bedtime oxygen and new medicines. Bailey was released after a weeklong stay in the hospital.
After some trials and changes, Bailey has a great network of doctors that care for her. She has a local pediatrician, Dr. Christopher Magryta of Salisbury Pediatrics, and nurses, Brenda and Gretchen. Dr Magryta cares for her typical pediatric visits. Bailey’s local cardiologist is Dr. Herbert Stern of the Sanger Clinic in Charlotte. He sees Bailey every six months. And, her Pulmonary Hypertension Specialist is Dr. Ericka Berman-Rosenzweig of New York Presbyterian Hospital in New York City, New York. We travel to New York every four to six months for check-ups and testing. These doctors and nurses have not only saved our little girl’s life but provide us comfort in knowing that she is being well taken care of.
Currently, Bailey takes three medications each day: a baby aspirin; Revatio, phosphodiesterase type 5 (PDE5) inhibitor; and Procardia XL, a calcium channel blocker. She is also on oxygen at bedtime.
Bailey’s diagnosis changed our lives in so many different ways. We now have a new “normal”. Our “normal” includes bedtime oxygen, “medicine times”, doctor appointments, trips to New York to see her specialist and hospital stays. We have a new outlook on life. We appreciate the small things. We cherish moments that some may let slip by unnoticed.
This year has brought on new challenges. Bailey has started her first year of preschool. This is the first time she has been allowed into a classroom setting with other children. Not because her disease is contagious but because of the germs associated with all children. Colds and viruses affect PHers differently. Something as simple as a fever puts extra stress on their already stressed hearts. We are happy to say that Bailey is doing very well and is excited to see Ms. Gena and Ms. Donna every chance she gets. And, with the new allowance of school settings, she has also started going to Sunday School at church. On special occasions, she gets to visit her older sister Brianna’s school, something she hasn’t been able to do in the past.
Unfortunately, there is no known cure for Bailey’s disease. However, recent data indicates that the length of survival is continuing to improve, with some patients able to manage the disorder for 15 to 20 years or longer.
Research is the key to finding a cure. If you are interested in making a donation to research, please contact us. In early spring, a family cookbook, Bailey’s Treasures, will be published. It will be filled with family recipes that have been handed down over the years from all sides of Bailey’s family. The money raised from this fundraiser will be donated in honor of Bailey to the Pulmonary Hypertension Association (PHA) for research. (For information on the PHA, visit their website www.phassociation.org.)
We would also like to take the time to thank each and every one of you that continues to love, support and pray for our family during Bailey’s fight and our ups and downs along the way. Having a daughter, sister, granddaughter, niece, and cousin with PH is not an easy road but knowing that we are not alone makes it bearable.
As much as this disease has changed our lives, we try to make every day as normal as possible for Bailey. We think that’s the goal for all families with a child with a life-threatening disease. You make things as normal as possible and you enjoy the life that God has given you.
November is Pulmonary Hypertension Awareness Month. Please visit the PHA website, www.phassociation.org, for information on this disease, symptoms, treatment, etc. If you are interested in ordering a copy of Bailey’s Treasures or want to ask us questions, feel free to email us at mom2brewers@aol.com. For updates on our journey with Bailey fighting this disease, please visit her webpage at www.carepages.com. Bailey’s carepage name is baileybrewer.
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