explained.
“What’s that?” asked Jen.
“It stands for Internationally Normalised Ratio, and is a measurement of how long it takes for your blood to coagulate. Mostly one’s INR level is at around 1.1. But the doctor wants yours at around 2.2, so we need to monitor you regularly. You will see me again tomorrow. Okay, Jenna?”
“Okay.” Jen smiled at her. She didn’t flinch as the needle went in. She wanted to make this poor woman’s job easier. It was the first of many ongoing blood tests.
Turns out it’s quite a thing to keep your INR levels stable. If your INR becomes too high, it greatly increases your risk of internal bleeding. And, in Jenna’s case, if her levels were too low, it greatly increased her risk of clotting and creating more thromboembolisms. What you eat, drink or digest in any way (such as medication, painkillers or supplements) will interfere with your levels. As a blood thinner Warfarin is highly effective, but it has to be managed carefully with consistent diet and blood tests.
“How long will she be on this?” I asked Dr VC.
“I’m hopeful that once we have thinned her blood sufficiently, the clots may start to dissipate,” he said encouragingly. How long would that take, I wondered. A few days, a week, a month? And what did he mean “may”? “For now, I encourage you both to go home so Jen can get some rest. We will talk again tomorrow – I have arranged further testing.”
My eyes snapped open at 5 am. Jen was in the hospital. It was the first day of school – and for those, like Kristi, starting high school at Herschel, it was Orientation Day. What should have been an exciting first day for Kristi now had a different taste to it. She wanted to go via the hospital so that she could say good morning to Jen, so I hurriedly made her lunch-box and we set off. Jen was so loving. She wished Kristi luck and waved her off encouragingly.
As Kristi headed into her first day of high school, I made my way to the headmaster’s office. The office was bustling but he made a plan to see me. He was shocked by Jenna’s news. “Gabi, please stay in touch,” he said. “I will tell Jenna’s teachers and keep an eye out for Kristi. If there is anything you need, absolutely anything, please let me know.”
By day three in hospital Jen was still just as breathless. She was sent for another chest X-ray. I was standing in her hospital room waiting, looking out the window in a daze, when she was wheeled back in by the nurse. She smiled up at me radiantly. It hit me in the chest like a mule kick. My child was in hospital and in a wheelchair, and no one really appeared to know what was going on. I wanted to cry. “Hello, my darling. How was that?” I said instead.
The doctors were more befuddled; it was a confusing medical picture. “I’m thinking of calling in a specialist professor and pulmonologist from UCT Academic Hospital,” Dr VC said to Stuart and me in the passage outside Jen’s room. “He may be the right man to help us towards a diagnosis.”
“Let’s do it,” Stu said. “Why wait?”
Prof. Wilcox from the UCT Academic Hospital was about to become a regular fixture in our lives.
Word got out about Jen fast. Friends began visiting and the phone started ringing off the hook. We didn’t know what to tell people. Kristi took homework to the hospital at night and then Granny Annie or Ali would take her for a meal. Stuart and I stayed with Jen until lights out.
On day four or five, having examined all Jenna’s test results, Prof. Wilcox arrived. He saw Dr VC for a thorough debrief before seeing Jenna. That day was the first time I ever heard the words “pulmonary hypertension”. In my ignorance I believed it sounded better than thromboembolisms.
Prof. Wilcox was a smallish man with light green eyes, glasses, greying hair and scruffy eyebrows – if you were to cast a professor in a movie, he would be awarded the role. He spoke in a quiet and considered manner.
After a long while examining Jen, Prof. Wilcox looked at us intently. “I can’t be sure, but I think this could be pulmonary hypertension,” he said. I was trying so hard to listen, to take it all in, but the words were slippery and foreign. “Or,” he continued, “maybe pulmonary hypertension with an element of chronic thromboembolic disease.” What language was he speaking, I wondered, as he turned his attention to Jen. “We will carry on with your blood thinners, Jenna, but I want to keep your INR at around at least 3 just to be safe. I might also put you on something called Sildenafil, but we will explain that later. Meanwhile, I’m going to move you across to the UCT Academic Hospital for further tests. Is that okay?”
Another hospital? I thought we were about to take her home.
Prof. Wilcox ordered an ambulance the next morning to do the transfer. Wow. It was hard to absorb. “I will see you there tomorrow. But Jenna,” he said in parting, “don’t Google. We don’t know for sure what we are dealing with and there is a lot of misinformation on the internet. Pulmonary hypertension is a complex condition.”
Jen was given a ward on her own in UCT Academic Hospital with the nurses’ station right outside her door. Because of that we were able to be at her side most of the time. Prof. Wilcox had thoughtfully arranged it that way. Friends and family took turns to visit. And delicious meals miraculously arrived at the ward thanks to Andrea, a generous friend and renowned caterer. It was a real gift – there was no time for cooking. I spent barely a minute at home.
Prof. Wilcox did another whole battery of tests, the same and more. Included was a much more targeted echo-cardiogram conducted by a specialist technician who knew exactly what she was looking for. He also did the dreaded “six-minute walk test” with Jen. Her first of many. This is a standard assessment tool for PH patients to clock their exercise tolerance, desaturation and chest pain when walking. Jenna came to dread this test over the years. The hospital’s specialist cardiologist and haematologist also got involved. Jenna’s case was quite a novelty for these academic doctors, but they handled her, and us, with care.
When visitors came Jen would “hold court” from her bed, gracious and dignified, and somehow managing to rock that hospital gown. She often had us in stitches with her dark humour. She was hungry for news of the outside world and for friends. There was no mention of her chest pains, injections, blood tests or nasty side-effects. And no mention of her fear. That she kept to herself. In the hours between visitors and tests she slept while Stu or I sat by her side. In the evenings Kristi balanced her books on the bed to do homework and we sat around chatting as if this was our regular dining room table. I got so used to driving to the hospital that, one day, I drove right to the doorstep of the hospital before realising I was meant to be somewhere else entirely. Some nights, when we got home late, there would be a meal and a note from my friend Mary. If there had been no cars in our driveway all day, she knew we would arrive home hungry and weary. Her thoughtfulness went way beyond the norm.
The echo-cardiogram, among other things, showed that Jenna’s pulmonary pressures were up.
“It’s possible,” Prof. Wilcox said, “that Jenna does have pulmonary hypertension. There is a test, a catheterised angiogram, which is considered the gold standard test for PH, but we will not do that right now.”
“Why not?” I asked.
“It’s a more invasive test,” he said. He wanted to wait for Jen to stabilise before ordering it.
So, for now the diagnosis, as we understood it, was that Jen had chronic pulmonary emboli. There was hope that they would be contained and dissipated with the regular treatment of blood thinners. She was still breathless and fatigued, and had chest pain, but it was
