In general once you have gone through the process of being diagnosed with pulmonary hypertension and become the patient of an accredited PH Centre for Excellence (see list) they will want to monitor you on an ongoing basis. (PH Medical professionals not listed are welcome to contact PHWA).
This could be quite frequently initially especially if you are having increases in your medication doses as they will want to monitor your reactions to this and any side effects you may experience. Sometimes you may need to be admitted into hospital for treatment or tests depending on your type of PH and the medications/treatments that have been prescribed for you as part of your therapy. You may have ongoing questions about anything from side effects, to contraindications or needing extra support or information. Most PH Clinics and doctors/nurses are happy to refer you to the local support network so you can decide if this is the direction you would like to take.
You will generally be required to have monthly blood tests for which a form will be provided to you either by post or in hand at an appointment. If you are too unwell to go into a blood collection centre Pathwest can organise for a bulk billed visit to your home by the phlebotomist to perform this test for you, either on a continuous basis or as a one off. You just need to give them a call and tell them you are a patient with PH who requires their home service. They will take it from there! They are based at SCGH (formally Osborne Park) the telephone number for Domiciliary bookings is 9346 3281 or 13 72 84, select option 3.
Depending on which clinic you attend and what your individual needs are, the Federal Government PBS requires that patients living with pulmonary hypertension are tested every six (6) months to be re-scripted. This means you have a 6 minute walk test with the physio or PH nurse, a cardioechogram, for which the referral will be posted out to you, and a review at your clinic/doctor to discuss how you are progressing and your test results as well as answer any questions you may have.
As a rule unless significant changes occur your new script will be issued, but don't panic your doctor and PH nurse are there to walk you through this process if there are any changes. A RHC (right heart catheter) which is a test that is used initially to reveal your pulmonary hypertension is generally only performed once and only again if there are changes that cannot be accounted for such as low iron, need for infusion either iron or blood, shift in fluid, and other various things that may impact on you. You do NOT have to have a RHC every 6 months to be re-scripted.
Once you have your scripts, test referrals and blood forms keep them safe in your diary or your PH Patient Pack (see below) for easy access. It is important to be as prepared as possible for your appointment. Try to write down any questions you may have, script requests or a list of problems you are having if any. In general the clinic nurses will handle the initial part of your visit and they will require an up to date medications list. These are all in the 3-P-Pack for your convenience. They will weigh you, take your blood pressure, heart rate and oxygen saturation, and temperature. Go through your medications, check your Hickman line site (if you are on continuous intravenous medication) and ask you about your overall health since they last saw you.
If you have any problems with attending these very important appointments you need to contact your clinic/nurse/doctor in advance if possible to re-organise as this is your re-scripting period and it falls within a certain time frame that they must legally adhere to. Wherever possible try to be an informed patient that self advocate so you feel you have some control in your care plan. Discuss your individual needs with your PH Nurse and Doctor/s so that you feel you are part of the process rather than a passive participant. It will give you a feeling of empowerment that may help with any emotional or anger issues you are experiencing due to your circumstances. Try to take responsibility for your role in the health care plan by being as prepared as possible, and having a plan in place for all scenarios, emergencies, travel, family planning etc so you know and understand what is expected of you as early as possible. Make sure you know about the risks or contraindications involved in taking over the counter medications or things that you should avoid due to your PH and medications (click for some info).
Try to remember the health care professionals that take care of us are only human and all have different personalities and lives with things impacting on them too. Some people who handle you may be more approachable than others but do not take it personally. Always speak and behave with respect and good manners. You can get your point across without being rude or becoming frustrated. There are also many alternatives to advocacy if you find this is the path you need to go down, but always try to communicate effectively with your existing team before moving to a new doctor. You are of course always entitled to a second opinion, this is your right. Remember though that different PH doctors have different medications available to them or treatment options and most importantly do they have 24 hour 7 days a week medical back up for emergencies? We are a team and must try to work together.
Once your PH has stabilised your visits will be less frequent, probably monthly, eight weekly and even three monthly depending on your individual care plan and needs. This is the time for you to ensure you have a solid emergency plan in place for any unexpected issues that may arise. Make sure you know the protocols for your individual clinic/doctor for emergencies out of hours. What constitutes as an emergency? Can it be handled over the phone or by paging the medical team? If you can afford it we urge you to have Ambulance cover so that you can be transported by professionals to the ED Click Here you can ring St John Ambulance and organise to email them your details as a priority to the hospital where your PH team is situated or your PH Doctor prefers you to go. Most trips to hospital do not usually result in emergency services being needed but it is very expensive if you do need an ambulance and it gives you peace of mind. Click here for your "I Have PH" emergency pamphlet.
One of the best things you can do for yourself is to make sure you have a good relationship with your GP. Initially there may be a feeling of needing to ring the PH clinic doctors/nurses every time you think there is something wrong. This is normal and will get better as your knowledge grows, so will your confidence. Establish when the clinic should be contacted or notified but always try to start with your GP depending on the reason and symptoms. For example if you think you are getting a cold you can see your GP and if you are at risk of infection this is when you notify the clinic. If you have a need for scripts that is your GP, if you have chest pain that is your clinic or the ED.
Some of the side effects of the various PH medications can sometimes cause other issues to arise such as rashes, blood noses (epistaxis), nasal congestion, headaches, nausea to name a few. You need a plan in place as to how to treat these and including your GP in at this stage is a good way to self manage as well as asking your clinic for answers or suggestions. You need to know and understand the difference and once you do you will make these decisions with ease. Remember that every time you have a test, whatever it is, bloods or echo, you and your GP are entitled to a copy of the results so always ask for the results to be sent to you and the GP so you can stay abreast of your results. It's fun to try to learn the abbreviations of the blood tests, and learn more about the echo's, but a word of warning, do not over react if you become alarmed by certain wording, because you are not a health care professional and you may not fully understand the results. Always ask your health care professional to explain your results to you! It's all part of being responsible for your own health care.
PHWA are fortunate enough to have a flagship Medical Centre, Central City as our first GP Aware centre in WA. Dr Chris Denz is also our PH Ambassador. He and his team are well trained and advanced in many areas of treatment. If you do not have a GP or need a second opinion, you may like to visit the centre and meet Dr Denz so he can assist you in your PH journey (click here for more info). They have a multitude of services available for their patients.
If you are struggling and feel that some counselling would help ask your PH team if there is a service connected to the clinic. Your GP can refer you to a list of good psychologists after doing a mental health plan and Medicare will cover a certain amount depending on the fee you may not even be out of pocket. There are also some excellent anti anxiety and depression medications you can take to help manage the side effects of the disease and the medications. If you need help ASK FOR IT, being brave isn't meant to be easy and nobody expects you to be a hero!
Because of the amazing advancements in the field of pulmonary hypertension in the last decade and with the multiple medications now available patients living with pulmonary hypertension are living longer and more fulfilling lives.
The medical & health care professionals that care for us are becoming more and more knowledgeable about the disease process and how it affects us. Due to the global awareness raising by organisations such as PHWA and the USA based PHA and phaware.global, PHA Canada and many more throughout Europe and Asia we have raised the profile to a level where the question is finally being asked, "What if it's PH"? This had led to many pharmaceutical companies leading the way in advancement of new PH medications and medical trials, which are all going to benefit the patients living with PH today and into the future.
CLICK HERE to order your PH Patient Pack. Available to anyone living with pulmonary hypertension. Healthcare/medical professionals who wish to share this resource with their patients are welcome to order as well.