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L. KATHLEEN HAGUE, RN, BSN
Pulmonary Hypertension Coordinator
University Hospitals of Cleveland
JUNE 6, 1999 (Updated January 23, 2001, April 4, 2002)
Ms. Hague obtained an Associates of Science in Nursing from Cuyahoga Community College in 1980 and a Bachelor's of Science in Nursing from Medical College of Ohio with honors in 1992. In January, she will start her Masters program. Ms. Hague joined University Hospitals of Cleveland in March of 2002 as a pulmonary hypertension program coordinator. There she has developed a new program with Dr. Robert Schilz and other staff, continuing to participate in patient care, research and development of newer treatment modalities for pulmonary arterial hypertension.
She was previously at the Cleveland Clinic Foundation, where she held the position of Lung and Heart/Lung Transplant Coordinator. The pulmonary hypertension program grew up out of lung transplantation and became it's own job description in 2001. Prior to working as a transplant coordinator, Kathy was a Cardiology Nurse Clinician for heart failure cardiologists that treat heart transplant patients. She also has extensive experience as a Medical, Surgical and Coronary intensive care unit nurse.
Additionally, she has worked many years in Labor & Delivery at a large, inner city hospital in Cleveland, Metro General Hospital.
She frequently lectures both locally and nationally and has authored or co-authored several articles or abstracts on pulmonary hypertension or lung transplantation.
University Hospitals Health System of Cleveland includes a 947-bed medical center and the primary affiliate of Case Western Reserve University (CWRU). Together, University Hospitals and it's partner CWRU form the largest center for biomedical research in Ohio. UHHS is a tertiary medical center with more than 15,000 physicians, nurses and support staff serving more than 45 communities in northern Ohio. It includes several hospitals connected by beautiful hallways and atriums, particularly, Rainbow Babies & Children's Hospital, one of the world's great children's hospitals. |
Edema is defined as an accumulation of an excessive amount of watery fluid in cells, tissues or serous cavities (such as the abdomen). Pulmonary hypertension can lead to right heart failure and edema of the lower and upper extremities and ascites (edema in the abdomen). Edema can be so severe it is called anasarca or edema of the entire body with fluid actually seeping out of the skin.
Edema is a symptom of many different diseases. We will discuss edema related to Pulmonary Arterial Hypertension. Treatment of edema starts with the treating the actual problem. Once the doctors start treating the problem, then we can treat the symptom usually with diet, fluid restrictions and diuretics. It's like treating a cough (symptom) that is due to pneumonia (disease) along with antibiotics to kill the bacteria. You wouldn't just treat the cough!
Physiology
The blood in the body is made up of blood cells (white blood cells, red blood cells and platelets) and serum. The serum is usually about 55-65% of the blood stream. It is a yellow, watery substance. It is usually thicker than water because it has albumin (protein) in it and other electrolytes.
The circulator system includes:
Veins:
Vessels which do not contain muscle that reacts to stimuli and medications. They will dilate or constrict to heat or cold. They carry blue, unoxygenated blood from the cells to the lungs. They have valves to keep the blood from backing up. Veins (drains) bring blood to the heart.
Arteries:
Vessels that react to bodily needs. They have smooth muscle that can open and shut the vessels. They carry the red, oxygenated blood from the lungs to the body. These vessels respond (open and close) to certain drugs and stimuli in the body. Arteries bring blood away from the heart.
Pulmonary Arteries:
These are our "problem children" in pulmonary hypertension. They are similar to veins In that they carry unoxygenated blood, however, the vessels are called arteries because they have smooth muscle, react to stimuli and drugs, and carry blood away from the heart to the lungs.
Pulmonary Veins:
Veins are vessels that carry blood from the lungs to the heart. ThePulmonary Veins carry the oxygenated blood (now physically red) from the lungs to the Left heart. They do not contain muscle that reacts to stimuli and medications.
Lungs:
Although you have two lungs, they act as one. Tiny air sacs (called alveoli) are surrounded by capillaries (one cell vessels - the ends of the Pulmonary Arteries) where oxygen and other gases such as carbon dioxide are exchanged.
Right Heart:
It is a pump that delivers blue unoxygenated blood to the lungs via the Pulmonary Arteries. It is weaker than the left side and can expand with blood and fails easily.
Left Heart:
Pump that delivers red oxygenated blood to the body. Blood comes from the lungs via the Pulmonary Veins to the left heart. It also maintains the blood pressure and is the side of the heart that "dies" during a heart attack because of lack of blood to the muscle of the heart itself.
The blood flow in everyone's body is directed in a clockwise fashion. Pulmonary Hypertension is defined as a condition where the pressure in the Pulmonary Arteries (mean or average) is greater than 25 mmHg at rest or 30 mmHg with exercise (40 mmHg is considered severe). Any condition which causes blood to slow its forward progress can cause this condition. The problem is believed to start with an inflammation in the tiny capillaries (the ends of the pulmonary arteries). The inflammation leads to small sores or lesions. The lesions will develop small blood clots and will fibrose (scare) and thicken. The vessels no longer work correctly (they do not open when they need to) and eventually close off completely, which blocks the circulation. It causes two things to happen: you can't get enough oxygen making you short of breath and all of the blue, unoxygenated blood can't get through the lungs (like a log jam in a river) backing up the blood in the right heart, liver and legs. This is called right heart failure.
If there is a problem that exists that possibly can cause pulmonary hypertension, it is called Secondary Pulmonary Hypertension. When a cause cannot be found, it is called Primary, Sporatic or Idiopathic Pulmonary Hypertension. Any condition that existed prior to the pulmonary hypertension, is blamed for the cause, despite the fact that the doctor may not agree. No matter what caused the problem, the capillaries that circle the air sacs of the lungs usually appear the same under the microscope and react to the same drugs.
Conditions that can cause Pulmonary Arterial Hypertension include:
Sarcoidosis (connective tissue disorder)
Scleroderma, CREST or MCTD (connective tissue disorder)
Rheumatoid Arthritis
Vasculitis (inflammation of the vessels in the lungs)
Portal Hypertension (cirrhosis or disease of the liver)
Lupus (connective tissue disorder)
Eisenmenger's Syndrome (a congenital hole in the heart)
HIV
IV drug abuse
Using amphetamines or diet pills
Familial (a family member has had the disease)
Other causes which may contribute to pulmonary arterial hypertension:
Tumors of the heart or lungs
Emphysema or COPD (smoking)
Chronic Bronchitis (inflammation of the tubules of the lungs)
Idiopathic Pulmonary Fibrosis (scarring of the lungs)
Radiation (for cancer, etc.)
Schistosomiasis (parasite)
Pulmonary emboli (Large blood clots than can come from the legs and lodge in the lungs)
Coronary Artery Disease (blockages in the arteries that feed the heart)
Cardiomyopathy (enlarged left heart)
Valvular heart disease (such as mitral valve disease)
Obesity (more than 130% of ideal body weight)
Sleep Apnea (stop breathing while snoring)
Whatever the disease process, when the blood can't move forward, it begins to back up. Blood vessels are similar to garden hoses, some are larger and some are as small as one cell. If you can imagine kinking the hose, behind the kink, the hose would be large and pressure would be high, with the water at the other end trickling out. Physiologically, that is just what happens in the body.
Since Primary Pulmonary Hypertension and most secondary forms starts with the vessels in the Pulmonary Arteries, the back up begins in the right heart. The right heart begins to enlarge and swell with blood. The blood then backs up in the liver where ascites can occur and in the legs where the serum is pushed into the cells and your feet, ankles and legs distend with water.
The blood trickling into the left heart is a significant problem. As you can imagine, if there is a trickle in, then the left heart can only pump out a trickle. We call this the cardiac output. The cardiac index is the cardiac output divided by your height and weight. A low cardiac index will cause your blood pressure to be very low, after awhile, you could pass out and your kidneys could fail. This is why the right heart catheterization is so important in monitoring your health.
University Hospitals of Cleveland tries every method of actually treating the problem first. Many tests are run to make sure we are treating the right problem. After we have begun treatment, we can start reducing edema by dietary means prior to using medications.
Ways in which you can change your life style to help the disease:
- Eliminating salt from your diet is the first step.Using fluids wisely is another alternative, and you should discuss this with your doctor.Maintain a good body weight.Stop all smoking, no second hand smoke.Limit alcohol to very rarely.Take your medications exactly as directed, checking blood work frequently.See your doctor regularly.Avoid heavy lifting, pushing or pulling (20 pounds). Avoid aerobics. Ask your doctor about pulmonary rehabilitation.Get plenty of rest.Avoid crossing your legs and elevate them when sitting.Go from a squatting or lying position to stand very slowly.Slow your pace, especially with stairs or a hill.
- Wear your oxygen if the doctor has prescribed it.
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