Life by Chloe

Chloe was diagnosed with severe Pulmonary Hypertension four years ago, after having been misdiagnosed for five years.

When I was finally diagnosed, I was in heart failure (the right side of my heart was three times its normal size), I was told that I may not have much time left to live, that I may never leave the hospital again and that if I did, chances were that I would have to be on oxygen for the rest of my life! (Not the best news!)

Her five key elements are:
Nutritional Lifestyle, Exercise, Mindset, Creative Expression, Family & Friends

Lupus and PH

PH or Pulmonary Hypertension is a disease that causes high blood pressure in the lungs due to narrowing in the pulmonary arteries by thickening of the vessel walls. It results in the heart having to work harder to pump the blood and the organ can become enlarged and weakened leading to heart failure. It can be caused by an underlying disease such as Lupus.
Lupus is a less common disease that many people haven’t heard of. It is an autoimmune disease that occurs when the immune system attacks healthy tissues resulting in inflammation, swelling, pain and cell tissue damage. There are different types of Lupus but the most severe is systemic lupus erythematosus (SLE). Symptoms range from mild to severe, and many people will have long periods with few or no symptoms before experiencing a sudden flare-up, where their symptoms are particularly severe. Even mild cases can be distressing and have a considerable impact on a person’s quality of life. Lupus is a complicated condition and common symptoms are fatigue, joint pain and swelling and rashes, however the disease can cause systemic damage and can effect organs like the kidney, heart and lungs.
They are both very separate diseases and PH does not cause Lupus however Lupus can cause PH. Patients suffer from shortness of breath, fatigue and fluid retention; symptoms also seen in patients with PH. The development of lupus-associated PH is not completely understood and “it is not yet known, for example, whether lupus itself can directly cause PH, or whether lupus is simply a trigger for the development of [PH] in susceptible individuals,” the report explains.
It is more common in female patients to develop lupus and PH at a younger age (15-50 years) than those that traditionally develop PH alone. PH can worsen the symptoms of Lupus and it is important to diagnose it early. Individuals should be aware of swelling in the feet, ankles, legs and abdomen, difficulty in breathing, chest pain, light-headedness and fainting.
There is currently no cure for either PH or Lupus but there are treatments that can help to ease the symptoms. Medications to help with inflammation, swelling and to ease blood pressure as well as measures to deal with fluid balance and supplemental oxygen to help with breathlessness and oxygen levels in the blood. Oxygen therapy helps with both diseases simultaneously; by helping to reduce inflammation and increasing blood oxygen levels which helps in tissue repair to reducing breathlessness, easing the hypertension and decreasing blood pressure among many other benefits. If Lupus is in association with the PH then immuno-suppressive medications can sometimes be administered which help with the inflammation and irritation in the body however they can also cause damage in the blood vessels in the lungs which can be counterproductive. Lupus is still very much an unknown disease and with time and understanding of its processes there will hopefully be more effective treatments in the future that could ease Lupus symptoms as well as PH symptoms if the patient suffers with both conditions.
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Pulmonary hypertension (PH) is a condition where the blood pressure in the pulmonary arteries (the blood vessels carrying blood to your lungs) is high. This increased pressure causes progressive damage to the heart and lungs.

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:
•    Oxygen
•    Warfarin
•    Diuretics
•    Targeted therapy
•    Calcium channel blockers
•    Endothelin receptor antagonists
•    Phosphodiesterase 5 inhibitors
•    Prostaglandins
•    Surgery
•    Pulmonary endarterectomy
•    Atrial septostomy
•    Transplant surgery
Most patients will have a regime involving a combination of background and targeted treatment which varies from person to person depending upon the cause of the PH and what stage they are at.
Many patients will need oxygen therapy, although some only need it at night. Oxygen therapy increases the amount of oxygen in the blood and it can also help to relax the arteries in the lungs which leads to reducing the pressure in the pulmonary arteries. Oxygen therapy can reduce tiredness and breathlessness in some people with PH and it can improve concentration and the ability to do everyday tasks.
Having PH can make you tired and lethargic. This may make it more difficult to do ‘normal’, everyday things. Here are some tips from the PHA website from patients to help make life a little easier.
•    Where possible, avoid bending, lifting or over-stretching when doing housework.
•    Use a lighter vacuum cleaner and iron.
•    Do jobs sitting down where possible – a kitchen stool can really help.
In bed
•    If your duvet is heavy, consider buying a lighter one.
•    Use extra pillows to raise your head and make it easier to breathe.
•    If you use an oxygen concentrator, consider putting it outside the bedroom to avoid the noise disturbing you.
•    After a bath, open windows before the house becomes too humid.
•    Have chairs ready for places where you stand (eg, shaving or applying make-up).
•    Slightly larger clothes can be less tiring to put on and take off.
•    Put on a bathrobe straight after a bath or shower to avoid having to towel yourself dry.
•    If bending down to put your shoes on is difficult, use a long-handled shoehorn.
Out and about
•    Plan ahead to avoid having to rush.
•    Consider asking for a wheelchair.
•    If you drive, carry spare medication in the car.
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