Patient story

Nicolene Muller

"My GP did an ECG, but could not find anything wrong. He also sent me for an X-ray of my lungs and heart to see if I possibly had asthma. He said I was overweight and needed to do more exercise."

My story began in July 2007 when I was advised by a dietician to use a diet pill to lose some weight. I was trying to get pregnant and already stopped using the contraceptive pill 18 months before that. I did not want to gain any more weight with another pregnancy, so I used the diet pill for only one month. Suddenly I had shortness of breath, was easily getting tired. I went to my general practitioner (GP) to do an electrocardiogram (ECG) as my cousin died three year prior of heart failure after giving birth to her son. I knew that I had a wonderful pregnancy with Janke, but was afraid that something could go wrong as on my mother’s side of the family, they had heart problems. My great-grandmother, grandfather and two of his brothers died of heart attacks.

Nicolene and Nico MullerMy GP did an ECG, but could not find anything wrong. He also sent me for an X-ray of my lungs and heart to see if I possibly had asthma. He said I was overweight and needed to do more exercise. On the X-ray he did not pick up anything. After a month my symptoms got worse and my GP referred me to a physician and I told her my story. She also looked at the X-ray and could not see anything of concern. She then told me that she just want to do a check-up and listen to my heart. She told me that she heard a second sound on my heart and that she wants me to go for an ultrasound. They did an ECG again and also an ultrasound. The physician told me that the ultrasound showed that the right side of my heart was enlarged and that I must not get pregnant before they determine what caused the enlargement. They then did a CT scan and I was diagnosed with primary pulmonary hypertension on 17 September 2007.

The physician first put me on high blood pressure pills, but I could not tolerate it. She then called a cardiologist and he suggested that I must be given Cialis. I had low blood pressure so I only used a half of a tablet of the Cialis. We went for a second opinion at a Cape Town Pulmonologist, which confirmed the diagnosis. I also started using Warfarin.

In August 2008, we got an appointment with Dr. Paul Williams, a Critical Care Specialist at Milpark Hospital, to be worked up for a possible lung transplant. When we arrived at the hospital, Dr. Williams told us that the South Australia Medical Board approved a trail drug for our disease and that he wanted to put me on the trail drug. We did not know if I was on the placebo, 3mg or 10mg of the trail drug. Back home, my life changed for the worse as my health deteriorated rapidly. I had to stop working; I was 24 hours in bed and on oxygen.

In November 2008 my dad and I visited Dr. Williams for my first 3 month appointment. As we got to Johannesburg, I passed out. The same happened that evening and the next morning. Dr. Williams was not available and I saw a colleague of him, namely Dr. Pahad. He told us that it looks like that I maybe on the placebo and that he was going to speak to Dr. Williams when he returns, so that they could ask Actelion (the trail drug company) to put me onto the open label of the trial. Then they would know that I get the active drug. At that stage I could only walk 120 meters (~130 yards) during my six minute walk.

"I just want to say that when you can diagnose PH earlier, you can live a fairly normal life."

We returned to Milpark two weeks later and I was placed on the open label of the trial drug. After one day of use, Drs. Williams and Pahad could already see a difference as my lips were not blue as it used to be. As I started the active drug, I got better and better. My current 6 minute walk is 456 meters (499 yards). I don’t use oxygen anymore. I only use my wheelchair when we have to walk long distances and when we use the airplane. I went to Europe last year and did walking without any wheelchair assistance. In June we flew to the US and I did not use any oxygen. When we visited New York I had to climb all the stairs at the subways. I did more exercise in nine days than all my exercise since diagnosis in 2007. I just want to say that when you can diagnose PH earlier, you can live a fairly normal life.

Firstly I would like to thank God for this miracle as I received a second chance on life. I like to thank my wonderful husband Nico and beautiful daughter Janke for their support and love. It is wonderful to link up with others and to realize that you are not alone in the fight against primary pulmonary hypertension.



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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.