There were no drugs for the condition available in South Africa at the time, so Wafarin (7-5mg) and oxygen (4L/minute) 18 to 20 hours per day were prescribed for me. My condition deteriorated, and I was put on a disability pension in June of 2002. In April 2003 my details were submitted to Pfizer to enable me to participate in the South Africa leg of a world-wide trial for a long acting version of Sildenafil (Viagra). There were six of us on the trial in Gauteng, South Africa. We became a family and were referred to as “The Viagra Junkies”.

The trial lasted until December of 2006 and was considered to be successful, and so the drug, registered in America under the name “Revatio”, came into being. We may not have a cure for the condition yet, but things are definitely better that they were!

With the increase in knowledge about PH it was found that a certain amount of exercise is beneficial. A physiotherapist, who specializes in pulmonary conditions gave me some exercises to do, and said that it was important to walk short distances, taking care not to over-exert myself.

I needed to find something to do to keep me busy, and was introduced to “puppy raising” for the South African Guide Dog Association. I raised a new puppy every year and through this I was helping other people who I considered less fortunate than me because they cannot see. I was privileged to ‘raise’ 10 puppies, including London, who has become part of Guide Dog Association breeding program, and has had two litters of future Guide Dog puppies so far.

I have completed four 5km 702 ‘Walk the Talk’ events, and four 5km iThemba walks to raise awareness for PH, as well as one ‘Dogs Unite’ walk in aid of the South Africa Guide Dogs Association.

In July of 2017 a second PH drug, Volibris, was approved by our Medical Control Council, and it was prescribed for me in August. Sadly, for the South African PH Community we do not have access to the large number of treatments available in places like the United States, the United Kingdom and Europe.

In October of 2017 my condition deteriorated once again, and I was hospitalized on and off from October to mid-January with multiple Pulmonary Thrombi, a Deep Vein Thrombosis in my leg, as well as swollen, painful legs for which the doctors could find no additional cause. My anti-coagulant was changed from Warfarin to Xarelto, as it was found to be more stable. Although I have recovered, I am not as robust as I was, and I tire very easily. I am now on 8L of continuous oxygen. Although I am no longer as mobile as I used to be, I have found a larger ‘portable oxygen machine’ which almost meets my needs, so I will not be discouraged by this condition.

I consider myself very fortunate as I have met some very interesting and inspiring people, who are in the same situation as me, some sadly no longer with us, and my hope is that we can be a source of strength and comfort to each other in the years to come. I will continue to set and achieve goals and raise awareness for PH through the Pulmonary Hypertension Association of South Africa of which I am a committee member, and advocate for better treatment for patients around South Africa.

Perhaps someday they will find a cure for PH, which in 2000 was a death sentence, and has now become a more manageable condition with better odds!

What seemed to be happening like clockwork every morning at around 1am and 2am turned out to be PPH, something I never even imagined would be a possibility. Each morning Tylor would let out a cry which is still constantly playing in my head. It’s a cry which I cannot explain and one which people don’t tend to understand until heard by their own ears. After a few minutes of this cry he would push his tiny clenched fists against my chest and then go absolutely limp, blue, sweaty and cold. At first they said it might be colic – “we know how babies can sometimes be” they said. At first I accepted this because I guess I just didn’t know better – maybe I was just afraid of knowing.

I’ll never forget the first night he’d stopped breathing – he just lied there like a limp ash grey doll. Luckily I was able to revive him and as soon as he started breathing again we rushed him off to the hospital in a panic. Tylor had by the time we reached the Hospital gotten his colour back and was his chirpy little self again. They admitted us but discharged us without doing any sort of investigation into the “episode”. Somehow whenever we were kept in hospital for “observation” Tylor was fine. I put the word observation in inverted commas because we were mostly left to our own devises and not really observed. We were practically in the emergency ward every morning for about 3 months before I simply refused to leave and refused to listen to the nurses snide comments “that there was absolutely nothing wrong with my child and that I was just worrying about nothing” – eventually Dr Madi from Mulbarton Hospital said they would do a scan on Tylor. Shortly after the scan/x-ray Dr. Madi said that Tylor’s right part of his heart looks slightly bigger and that he would be transferring us to Sunninghill where we would see a paediatric cardiologist immediately. Just before the ambulance arrived to transport us to Sunninghill Tylor suffered another episode - “finally someone other than my mother and me had witnessed what was going on” “finally someone would help my baby”.

On arrival at Sunninghill Dr. Dansky was waiting for us in the Neo Natal ICU because DR. Madi had called ahead and explained what happened. And so we did a Sonar – once again nothing, Dr. Dansky had said there is absolutely nothing wrong with Tylor, that there are no heart defects – we were discharged again.

As a last hope we visited a paediatrician in Garden City Hospital – Dr. Miles Bartlet. After consulting with us he agreed to admit us into hospital and run every test until he could tell us what the problem was. Tylor spent about 2 weeks in hospital undergoing every test imaginable (MRI’s, EEG’s, Stomach and swallowing tests) – everything came up “clean”. Our last test would be a visit to the ENT – after the ENT examination the ENT sat me down and told me in no uncertain terms that Tylor was not sick and that I was making him sick, that I needed to take my baby home and learn to cope with motherhood.

After our ENT visit all I wanted to do was take my baby home and not see another doctor ever again. That same evening Dr. Bartlet was running late on his rounds and only came by the ward at around 19h00. He came to confirm that they had found “nothing” and that we would be discharged in the morning. It’s like Tylor heard him – because he started fitting, foaming, vomiting and then went into cardiac arrest. Dr. Bartlet rushed off to the “procedure room” and disappeared with Tylor for what seemed like a life time. Eventually when he emerged from the operating room he said that he had to revive Tylor 4 times already and that he had never ever seen anything like Tylor’s episode. Once Tylor was stabilised and placed on oxygen and nitro’s oxide machines he was transferred to the NEO Natal ICU downstairs. He just lied there on that cold hard bed not moving with pipes comimg out everywhere while being doped up with morphine. The next day Dr. Dansky was called back in to look at Tylor’s heart. Dr. Dansky discovered that Tylor had PPH – PPH is not easily diagnosed he said – “it’s a very rare, one in a million condition”. I think at that stage I was angrier than anything else and it felt like I wanted to strangle the man. What they couldn’t figure out was why Tylor was fitting – why the epileptic attack – was it the epilepsy that aggravated the PPH or was it the PPH that caused the fits – and to this day they still do not know.

After his diagnoses in late September 2004 Tylor was only discharged from hospital in mid November 2004. During Tylor’s stay in hospital we almost lost him more times than I wish to remember. We called the priest in so many times fear still runs through my bones every time I talk to or think of one.

After Tylor’s diagnoses Dr. Bartlet prescribed Viagra for Tylor on an four hourly basis together with eight hourly epilym. Tylor eventually came off the machines and recovered nicely– my little soldier. Upon discharge Dr. Bartlet stated that we should stay with Dr. Dansky as he was the best in his field – and so we kept Dr. Dansky as Tylor’s treating doctor. We were also told that we cannot keep Tylor on Viagra indefinitely and that we should systematically wean Tylor off the Viagra – and after a period passed without a episode we increased the period of time between Tylor’s dosages – from 4 hourly then to six hourly then to 8 hourly. And every time we increased the time period between Tylor's dosages he suffered an attack and every time the doctors would say increase the medication back to 4 hourly but indicated that we would need to wean him off slowly to see what the correct dosage is and “see what happens”. I could never understand how a doctor could say “let’s see what happens” to a mother. We saw so many doctors in the first two years and every doctor we saw seemed to know less and less. Eventually I just stopped searching the internet for answers, I stopped going to other doctors and focused on taking Tylor only to Dr. Bartlet and Dr.Dansky in order to create some sort of normality and stability for Tylor – all these doctor visits we taking a toll on Tylor as eventually it seemed like he could smell the doctor coming from a mile away and he would start screaming his head off.

Eventually I just didn’t listen to the instructions to wean Tylor off – I just kept him on four hourly medication. After a while his condition stabilised and I simply said I would wean him off to the doctor but never in did. Don’t get me wrong both Dr. Bartlet and Dr. Dansky are brilliant doctors and I am so grateful to both of them for saving my son on so many occasions – I guess it’s just the circumstances of it all that creates great anger within me.

From 9 November 2008 to 6 January 2010 Tylor suffered no “episodes” apart from the occasional tiredness after a busy day. The medical aid required a update from Dr Bartlet as well as Doctor Dansky in November 2009. Dr. Dansky “suggested” that 4 hourly was to stressful for both Tylor and myself and so we decided that we would increase the amount of Viagra and increase the period between dosages. I was far too afraid to do this until I could be at home with Tylor for a while to make sure nothing happens to him while he is at crèche. So during my leave in December 2009 just before Christmas I implemented the change in the dosage of the medication. On 6 January 2010 on the second day after my return to work Tylor suffered a really bad episode and stopped breathing. His back on his 4 hourly medication now and doing well.

Through everything Tylor has been such a strong happy little boy – looking at him gives me strength. I just hate looking into his eyes after his had an episode – he always looks up at me as in to say “why is this happening to me, why do I feel so ill, why can’t I run with my friends” – I cannot look at his eyes after his been sick because I cannot answer the questions that are in his eyes, in my heart and constantly on my mind. I think it’s the not knowing what’s going to happen next, how long we still have and the not knowing what to say to Tylor that is the scariest – I sometimes wonder if I’ll be less fearful of tomorrow if I knew those answers but something tells me that I might fear tomorrow more after knowing what tomorrow might bring.

I wanted so much more than this for Tylor – I hate thinking of him as developmentally slow, I hate thinking that he will never run like the other kids – live a life like other kids. I hate this decease and I would do anything to take away my sons pain and give him a full and certain future.

After multiple tests, x-rays and scans Dr Sareli discovered that the right side of my heart was double the normal size and I would have three months to live if I didn’t get treatment.

They say things happen for a reason and I am a firm believer of that. We met the right doctors at the right time which led us to getting the right treatment for me.

After many fundraisers and many tears. We travelled to the US, where I met my 3rd guardian angel- Dr Sager. (A South African that is now practicing in Santa Barbara). We tried multiple treatments, from oral to oxygen therapies etc. Lucky for me, the oral treatment worked beautifully and that was the treatment that I lived on for 6 years. (Tracleer (Bosentan), Adalat 240XL & Warfarin) Thankfully, my symptoms improved over time and I am currently only taking Adalat XL60 twice a day. It’s been 13 years and I’m not giving up yet!

PH completely changed my life, but it gives a person perspective. Your world can turn upside down in a second but don’t give up hope. I count my blessings every day and I know I am, still here for a greater purpose. Don’t give up hope and keep fighting the PHight!

In 1998 I was sent to a physician in Pretoria who could hardly believe the story I told him and told me I had been grossly abused by the medical profession and confirmed I had Lupus. I did not think that anyone could have been as happy as I was to be given such bad news, but finally I had a name for what was making my life so hard and I could finally try deal with it. Somehow my health issues having a real and recognized name, made it a whole lot easier to deal with!

Fast forward to 2009 or thereabouts, I was finding it very hard to get around and found myself very short of breath with almost any exertion, even eating would leave me exhausted. Eventually at the end of November 2010 I went to see a new Rheumatologist who immediately admitted me to hospital where a number of different specialists were called in to assess what was going on with me. I will never forget the Cardiologist who arrived at my bed with his portable echo machine ~ he announced that he knew what was wrong with me, he then told me I had Pulmonary Hypertension & needed a heart catheterization to confirm this. Unfortunately, neither he, nor anyone else explained to me, exactly what this was or how it should be dealt with. I was put on Revatio which almost immediately helped with my breathing. I also had a sleep study and was found to have rather bad sleep apnea and had to start using a CPap machine.

For almost a year, things were going along very well, then I started to notice that once again I was getting very short of breath. The doctor wanted to increase the dosage of my Revatio but the Medical Insurance refused, saying there was nothing to suggest that larger doses would be of any benefit, so my doctor at the time, in his wisdom, decided that I should then stop taking it, which I did.

Today I am using Oxygen at night with my CPap, pills to reduce my blood pressure and racing pulse, along with diuretics to help with water overload and Celebrex for my RA. I do my best to live as normal a life as possible and not dwell on all the things I can no longer do, but do still try to get out and about with my friends and craft buddies!

I am accustomed to extreme fatigue and no energy and always getting infections. You see, in 1989, I was diagnosed, when I was only 15, with the auto-immune disease, Systemic Lupus Erythematosus. Instead of the immune system fighting infection, it turns on itself, in my case attacking my skin, joints, tissue and kidneys. Cortisone was used to suppress the immune system and when suppressed, I was prone to infection! A vicious circle!

In the years that followed I learned to accept what came my way. I overcame swallowing handfuls of pills without gagging and also my fear of needles! I watched my friends as they married and had babies. It was a bitter pill for me to swallow knowing, but finally accepting, that I would never have children of my own.

I was fortunate enough to have a very caring and understanding family who stood by me supporting me financially and emotionally.

I had a remarkable relationship with my rheumatologist. I would often tell him I felt I needed oxygen and also shared that I would become breathless whenever I walked up a slope. He told me it was probably due to the fact that I was unfit and he suggested that I should get more exercise. At this time I was under a pulmonologist as well. I would struggle through my lung function tests. She was treating me for asthma. After many, many months of continually struggling with shortness of breath, early in 2011, I took myself off to my G.P. who examined me thoroughly and called my mom on the phone to say that I needed to see a cardiologist without delay. He set up an appointment for me at the Union Hospital, not too far from my home. As it was a long weekend and he was going away, the cardiologist admitted me into the CCU (Coronary Care Unit) and I was under the care of a specialist physician. It was then that I was diagnosed with Pulmonary Arterial Hypertension, which is secondary to my S.L.E.

After the weekend, I was referred back to my pulmonologist who was at a hospital some distance away. I was admitted and seen by a cardiologist at that hospital. Numerous tests were done, confirming the diagnosis. I was put onto Revatio 20mg 3 times a day. I was sent for a sleep study and declared to have sleep apnea. I began using a CPAP machine.

I felt the benefit of the Revatio almost immediately although obtaining it from the medical aid has never been an easy feat! On one of my visits to my rheumatologist, he asked me how long I would be on the revatio, thus indicating that he wasn’t all that familiar with PAH. At that point, I suggested a ‘second’ opinion with a cardiologist to which he agreed and referred me to a very handsome cardiologist who could have made any lady’s heart skip a beat! Once more Pulmonary Arterial Hypertension was confirmed and it was he who steered me in the direction of Milpark Hospital telling me about Dr P.G. Williams and Professor M.R. Essop who had a keen interest in PAH......... and so began my journey under these two specialists. It’s been over 7 years now since diagnosis and almost 6 years under their care. About 2 years ago his patients were put onto dual therapy when medical aid finally agreed to sponsor Bosentas (generic of Tracleer.) It was brought into the country from India and fell under a section 21 drug. Every 6 months we had to get authorisation to continue it’s use and pay a fee over to the Medical Control Council. I cannot thank my father enough for having me as a dependent on his medical aid and for the hours and hours he has had to spend on the phone to Discovery sorting out all the issues and my mom for walking the long road with me and being my best friend. Dr P. Williams increased my Revatio to 40mg 3x a day and Prof Essop has performed 2 angiograms which revealed that the disease is irreversible and the last one showed the pressure in my lungs to be 90.

The PH patients are all being changed over to cialus (in place of Revatio) and Volibris (in place of Bosentas, as Volibris has now been registered in South Africa.) Some patients are being treated with Iloprost which has to be administered through a port. I have made and sadly lost many good friends with pulmonary arterial hypertension. Some have undergone double lung transplants, others have passed away suddenly. It makes you think how fragile our lives are and how we need to live each moment to the fullest. I have decided that I do not want to go the route of Iloprost nor transplant but want to live out my days with as much quality of life as, possible.

It saddens me that I can no longer fly overseas but I am thankful for the trips I made prior to diagnosis. I now travel from the comfort of my couch, on my oxygen, visiting places all over the world through the television screen! I sleep between12 to 14 hours each night, waking up towards lunch time the following day. I rest when tired and try not to exert too much energy as even climbing the stairs in my house is a challenge.

I constantly remind myself that there are others worse off than myself. I don’t like to part on bad terms with family or friends as life is uncertain. I am friendly and like to bring sunshine, smiles and laughter into people’s lives when I initiate conversations in the shopping centre right from customers, to shop assistants or even the cleaning lady! I am so thankful for the support I receive, for the dedication of my doctors, for the new drugs that are slowly, but surely, being used in South Africa and mostly for my God who loves me and has carried me through all the tough times.

At the time I was in a relationship with a captain in the South African Police Force. I was relying more and more on him for help and yet I still refused to believe that there was anything wrong. This continued into June of the same year. I woke up one morning to find my feet and legs very badly swollen from water retention, something that I had never experienced before. My condition had deteriorated to the point where a good friend, whose father was a General Practitioner in Sea Point, insisted that I make an appointment with him to find out what was wrong.

During the course of his examination he ascertained that my heart was struggling and recommended that rather than wait to see a doctor at a government hospital I make an appointment to see a private cardiologist as a matter of urgency. I managed to make an appointment to see a cardiologist at the City Park Hospital the very next day.

The Cardiologist, on his examination, suspected that I had a leaking heart valve. I was prescribed medication for my heart and sent home with the instruction that I should stay off my feet for the next three months and stop smoking immediately if I wanted to live. Needless to say, that very same day I stopped smoking. However, the medication I was prescribed had horrific side effects. When I asked for the medication to be changed because of the side effects the cardiologist insisted it was ‘all in my head’ and refused to change the medication. I decided to seek a second opinion.

I was referred to a second cardiologist who after examining me and carrying out further tests confirmed that I had a leaking heart valve. He also diagnosed Pulmonary Hypertension a disease I had never heard of before. I was prescribed the correct medication Revatio (active ingredient Sildenifil), the only drug registered for Pulmonary Hypertension available in this country at the time. Despite the medication my condition continued to deteriorate. I was referred to another cardiologist whose special interest was Pulmonary Hypertension by a fellow PH patient, Denneys Niemandt. This cardiologist, after examining me and acknowledging that I had had all the necessary tests for PH suggested that I have an HIV test.

I had only had two long term relationships in my life and was sure that neither of the partners could have infected me, so I agreed to have the test. I was shocked two days later when the test came back positive. I was sure they had made a mistake and insisted they do the test again. Sadly, that also came back positive. I was shattered, not only did I have Pulmonary Hypertension but HIV as well. I subsequently I found out that the policeman with whom I had been in a relationship for a full five years had been having gay relationships throughout our time together. It was an exceedingly difficult time for me especially as the prognosis I was given was 6 months.

I am a positive person though and would not allow all these things defeat me. I was prescribed a second medication which was not registered in this country at the time but was brought in from India for me courtesy of my brother who lives in Israel. The medication, which is awfully expensive, improved my condition but sadly the supply was erratic, and my brother, having retired can no longer assist me. The drug is registered in this country now under the name of Volibris (Ambrisentan), but not having a medical aid the cost is more than I can afford.

I joined the PH Clinic at Groote Schuur, a large Government hospital in Cape town, in 2015. The Clinic was started in early 2014. The Jenna Lowe Trust assisted the Clinic with a registered nurse in late 2015.

Besides having access to some of the best PH Specialists in the country, the Clinic at Groote Schuur runs a monthly Support Group for PH Patients and their Caregivers. We have had some wonderful meetings with a couple of interesting guest speakers.

Sadly, my condition continues to deteriorate, and I have been advised to consider a lung transplant. The team at Groote Schuur have agreed to supply me with the Ambrisentan that I require to stabilise my condition until such time as my quality of life is such that a transplant is the only option.

I spend time creating awareness or Pulmonary Hypertension and am a Rare Disease Voice for Pulmonary Hypertension.

I live in the hope that they will find a cure for this life-threatening debilitating condition.