Explaining PAH to others
From Pahwiki
Contents |
[edit] Local physicians, nurses and EMS personnel
[edit] Teachers and school administrators
[edit] Siblings
[edit] A child with PAH
One of the most difficult discussions that I have struggled with is discussing PAH with a child who suffers from the disease. How much information do you share about the disease, particularly the risks and potential outcomes? At what age or maturity level do you have those discussions? How do you approach the discussion? Clearly all children are different, and their individual situations require unique and tailored approaches.
Several good articles are included:
- Helping Children Cope with Chronic Illness - Very good article, particularly the section "Coping with a life-threatening illness." Difficult for parents of young patients to read.
- HOW PARENTS CAN HELP THEIR CHILD COPE WITH A CHRONIC ILLNESS - Various pointers an explaining to children the illness, preparing children for their medical procedures, leading a normal life, discipline and family life as well as other helpful tips.
[edit] Other family members and friends
Pulmonary Hypertension (PH) is in many ways an invisible condition. Those who suffer from it may appear to be perfectly normal; there is no visible sign of limitation. It's only if you watch them try to climb a set of stairs, or walk any distance, or up a mild incline, that you begin to notice symptoms. And, in fact, it is often their own difficulty doing those everyday activities that first alerts the patient or their doctors to the presence of a problem.
To understand what PH is and why it's of concern, it may be helpful to just back up a little bit and remind ourselves of what is happening when the heart and lungs are working normally in a healthy person. At the simplest level, what the heart does is pump blood around the body, carrying oxygen to the muscles and cells of the body to enable all of those parts of the body to keep doing their work. The lungs, for their part, are where the oxygen is put into the blood, and, to complete this very high level picture, where the carbon dioxide (the waste material returned from the muscles and cells) is removed. When any person does work, physical activity, more oxygen is required; the heart beats faster to get more blood out there to deliver more oxygen. The greater the exertion, the more oxygen is needed, and the harder the heart has to work.
In PH, the heart has to work extra hard to pump blood through the lungs; there is more resistance than there should be. The lungs themselves are normally very soft and non-resistant; if you think of active and passive participants in an activity, the heart is the active agent in circulation, while the lungs are passive. Everybody is familiar with the measuring of blood pressure when you visit a doctor’s office. A cuff is put on your arm, air is pumped in and slowly released, and the blood pressure -- in this case, what is known as systemic blood pressure -- is read in terms of two numbers, first the systolic pressure, showing the pressure when the heart contracts, second the diastolic, the pressure when the heart muscle is relaxed. A normal systemic BP reading is considered to be between 110/70 and 120/80. A person is said to have systemic hypertension when the heart has to work harder, and that shows itself with elevated readings such as 140/90 or 160/110.
It’s far more difficult to detect pulmonary hypertension. All of the blood vessels involved are deep within the chest cavity. The condition is usually first detected, as noted above, based on difficulties experienced during physical exertions, even relatively minor ones, such as walking short distances or climbing a flight of stairs. Then diagnostic procedures such as echocardiograms are used to observe the heart in operation and and to use these observations to determine the pressure in the pulmonary artery. The body is designed such that the heart normally should have an easy job pumping blood into the lungs: the normal systolic pressure in the artery to the lungs is 20. Pulmonary hypertension is said to exist if the readings are over 30 or 40 (depending on whether the person is at rest or exercising). It is not unusual for a PH patient to show systolic readings approaching or exceeding 80, indicative of “moderate to severe pulmonary hypertension.”
When pulmonary hypertension is present — when the lungs are that resistant to the normal and necessary flow of blood — then the heart has to work extra hard to do its work. Hence, to return to the start of this little article, when the heart has to work extra hard, it can quickly fall behind in its work. It just won't be getting the job done. And that is why it is very common for the person to experience shortness of breath. And that's why shortness of breath (often abbreviated "SOB") is typically one of the first signs of PH, an otherwise invisible condition.
This extra work causes the muscle that is the heart to begin to expand, to get larger in ways that it shouldn’t. This enlargement of the right chamber of the heart can encroach on, and impede the normal functioning of, the left chambers of the heart, the part of the heart that pumps the oxygenated blood to the rest of the body. And this process can continue to compound if not treated, with the potential for serious cardiac complications. Thus, the importance of having some kind of medication --- typically a vasodilator, to help the blood vessels in the lungs relax and open up, so the blood flows more readily --- to minimize damage to the heart.
