Questions to Dr. E. Kotylan at the P.A.H. Support Group at ST. Vincent�s Hospital

1. Should we all be seeking an altitude test before planning air travel?

Yes.

Eugene said that firstly the fact that some of us are considering overseas travel was a great accomplishment.

He said that we should all attend an altitude test before considering air travel.

This is most important for over seas trips, but also important for domestic travel as the plane gets to the same heights and therefore has the same concerns with drop in Oxygen concentration, and the effects that has on our oxygen saturations.

 Long haul flights are most concerning and short haul flights can be assessed on an individual basis. We should expect our oxygen saturations to be between 61 -89% after a 15 min test. If our results fall below 61% then we should have oxygen therapy on the flight.

Oxygen therapy can be organized with Qantas. Flights of 4 hours and less can have an

oxygen bottle under the seat. For flights over 4 hours it can be costly as you need to

purchase an extra seat for the oxygen.

These recommendations are not specific for PAH, as studies have not been done for

patients with PAH, but they have been done for patients with emphysema, who have

similar breathing issues.

Important Key -PLAN AHEAD!

Qantas has forms that need to be completed for them to provided oxygen, and altitude tests must be done well in advance. Tests can be organized through our nurses, and are done in the lung function unit. .

There is also a concern of travel insurance.

{New South Wales: Currently we have an pulmonary arterial hypertension  support group which meets at St. Vincent�s Hospital in Sydney. If you would like to start a support group in your area, its not difficult and we are happy to help! Please contact our message bank at 02 8250 1150, or email us at info@phnsw.org.au}

2. I have developed "small red spots over my face and upper body" - what are they? (Reprinted from the  Raynauds and Scleroderma Newsletter (UK)  Hot News Issue No. 80)

 These small spots are called telangiectasia or spider nevae.  This latter because they have the appearance of a spider with a central body and legs coming out.  Nobody knows why they develop in Scleroderma but they are indeed associated with this disease.  Previously the only remedy was to use thick makeup to cover the small spots.

More recently Laser therapy has been developed.  In this situation a small beam of laser light is directed to the centre of the telangiectasia (i.e. the body of the spider).  The light is very powerful and burns up the blood vessel (coagulates it) so that blood cannot flow through it.  Instead of the rather larger red telangiectasia the patient is left with a small white spot.  It can be very effective and is used predominantly for telangiectasias that are causing cosmetic distress.  A plastic surgeon or a trained member of his team usually carries out the procedure.

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Questions to Dr Chris Denton F.R.C.P. Consultant Rheumatologist, Royal Free hospital (Reprinted From the  Raynaud's and Scleroderma Newsletter (UK) in Scleroderma NSW Newsletter Feb. 2008 p 7)

2. My fingers go white and feel numb when I get cold but why do they change to a bluish colour?

Blanching (whiteness) of the fingers is a result of reduced blood supply. This can be triggered by cold or emotion and is part of the normal stress response. One consequence of a poor blood supply is that the sensory nerves in the fingertips becomes less responsive. This causes numbness. These symptoms can occur in Raynaud's phenomenon. In a classical Raynaud's attack the white phase is followed by a blue appearance due to slowing of the circulation in the veins and finally a red phase as blood flow is increased to the extremities after the blood vessels relax at the end of the Raynaud's attack.

2. I have had Raynaud's for 5 years and it is getting increasingly difficult to cope, especially in the winter months. More recently my fingers have started to swell and look puffy. Is this normal for someone with Raynaud's?

Raynaud's is most often an isolated condition, when it is termed primary Raynaud's phenomenon. Swelling of the fingers is common and may reflect changes in leakiness of blood vessels. it does not necessarily mean that there is an associated inflammatory disease ( as in secondary Raynaud's). However if the symptoms are worsening you should discuss this with your doctor in case further tests or treatments are needed.

This is a rare disease related to localised forms of scleroderma that causes inflammation and scarring (fibrosis) in the layers of tissue under the ski (called facia). It often affects the lower legs and forearms. Sometimes it si triggered by unaccostomed strenuous exercise. It is not associated with internal organ fibrosis or Raynaud's phenomenon. In the early stages it can be mistaken for systemic sclerosis.

4.What are the treatments for calcinosis?

Calcinosis is the medical term for deposition of chalky material under the skin. This occurs in some patients with scleroderma and also other connective tissue diseases such as dermatomyositis - especially when it starts in childhood. The mechanism is unknown but calcinosis causes several severe problems in some cases with skin ulceration, local pain and impaired movement of joints. There is no cure. Local surgery may be effective but the calcinosis often returns. medical treatments that are sometimes used include diltiazen, warfarin, minosycline and colchinine but none of these have been definitely shown to work. More research is needed in this area.