When you exercise the heart beats more quickly to get more oxygen to the muscles. At the same time the pulmonary arteries expand to allow more blood through so that more oxygen can be carried to the muscles. They expand by stretching outwards slightly to create a larger inner area. In a person with PH, the walls of the pulmonary arteries are thicker, so are less able to stretch.
Because of this the heart has to work harder to pump blood to the lungs, especially during exercise. If the heart has to work harder than usual over a long period of time then it begins to work less effectively and damage occurs. PH affects many different types of people. In most people with PH, it is associated with another medical condition:
• portal hypertension
• connective tissue disease, eg systemic sclerosis
• HIV infection
• congenital heart disease
• sickle cell anaemia.
Some people develop PH with no known cause which is referred to as idiopathic PH and in some rare cases it can be inherited. Your stage of PH is classified as shown in the table below, depending upon when your symptoms occur.
WHO classification of PH: Class Description
1 No symptoms of any kind. Physical activity does not cause any symptoms
2 Comfortable at rest, but symptoms occur with ordinary physical activity
3 Comfortable at rest, but symptoms occur with less-than-ordinary effort (eg lifting the arms)
4 Symptoms while resting Treatment for PH can be split into three categories:
• Conventional therapy (often called background therapy), which can include the following
• Targeted therapy
• Calcium channel blockers
• Endothelin receptor antagonists
• Phosphodiesterase 5 inhibitors
• Pulmonary endarterectomy
• Atrial septostomy
• Transplant surgery
For those people who suffer with any type of lung condition they may have difficulties when travelling by air. This is due to the reduced air pressure in the aircraft cabins as well as the lack of mobility for long periods of time. Air pressure in an aircraft cabin is lower than air pressure at ground level and feels like being at 6000 to 8000 feet on a mountain. At high altitudes blood oxygen levels fall in everyone, and some people may feel a little breathless.
In most people this has no health effect, but if you already have low blood oxygen levels because of your lung condition, then the extra dip that happens while you are in the plane can cause breathlessness and discomfort for you. As long as you meet a minimal fitness criteria agreed with your doctor then it is still possible to fly, even if you require constant oxygen therapy. Before departure : If you are able to walk 100 metres on ground level without needing oxygen, at a steady pace without feeling breathless or needing to stop, then you are not likely to be troubled by the reduced pressure in aircraft cabins. If you cannot do this then you will need to talk to your doctor about whether you should travel by air. You may need to have some breathing tests to show if a fall in your blood oxygen level is likely to be a problem to you while travelling.
You should also check your travel insurance policy to make sure you are fully covered for any medical costs that may arise in connection with your lung condition. It is important that your travel medical insurance includes the cost of return by air ambulance if you were to become too ill to return on a commercial flight. Many policies will not cover you for costs from your lung condition unless you have a written note from your doctor that he or she feels you are fit to fly.
Oxygen and air travel :
If tests show that your usual blood oxygen levels are so low that air travel may be a problem for you, you may still be able to travel by air, if oxygen is provided for you. Airlines can arrange extra oxygen, but remember that most will charge for providing oxygen. Different airlines have different charges; check with each one before you arrange your flight. Don’t trust to luck that planes will have oxygen on board. They carry emergency supplies but not enough for several hours. You will also need permission from the airline to take on board and use any electrical equipment you need for your treatment. Equipment must be battery driven, and you will not be allowed to use it during take off or landing. Using an inhaler with a spacer is just as effective as using a nebuliser.
You may find it easier to organise your oxygen needs via a company that can arrange oxygen for both the plane and the entirety of the holiday, just in case you need oxygen throughout your trip or if you just want to arrange to have a ‘back-up’ supply just in case. It’s easier to have dealings with just one company rather than multiple, especially if you’re travelling to more than one place during your trip.
Essential Tips from the NHS website to remember before flying:
1. Ask your doctor well in advance for a letter to take in your hand luggage with details of your condition and medication.
2. Be sure to take your inhalers in your carry-on bags. One of the most common problems is that people pack their inhalers in the luggage that goes into the hold.
3. If you get breathless when walking, make sure you have help at airports. Distances to departure gates can be long. Disabled assistance at airports can be arranged before you travel.
4. When you are in the aeroplane try to move about every hour or so and exercise your legs. Sitting for too long can lead to blood clots in the legs.
5. Drink plenty of water and non-alcoholic drinks during the flight.
6. Remember the golden rule: If in doubt about travelling, check with your doctor.