Mornings were tough and getting to school on time impossible. I would take Kristi, then go back to wake Jen and help her get dressed. She was dizzy and oxygen deprived in the mornings, but wanted no pity. She arrived at school all smiles. It was hard for those who were not in the know to understand what was going on. To some it appeared as if Jen had special privileges, sometimes attending class and sometimes not, sometimes attending social events and sometimes not. I understand it was confusing, but it is also true that teenage girls can be cruel and judgemental. There was some petty politics and doubt as to the “authenticity” of her illness. It was hard to manage, knowing what we did, but luckily Jen’s close inner circle knew just how much she was battling to function and were incredibly supportive. And, of course, she had Kristi at her side, her fierce protector.
On days when Jen had enough energy (her days were highly variable) we encouraged her to see her friends, go to Debating and find ways to remain connected with her social groups and her passions. If we’d based our daily decisions on medical data only, I would probably never have let her leave the house. Thank heavens neither Stu nor Jen would allow that. Regardless of the massive adjustments she had to make, at no stage did Jen play the role of victim or display one iota of self-pity. Where others might have asked “Why me?”, she would ask “Why not me?”
Teenage socialising moved to our home, where it was safer. Kristi spent a lot of time with Jen and her friends and became integrated into her group. We also spent an inordinate amount of time with family, which was comforting. We came to know every inch and corner of the winding road to Shirl’s house, where we all spent many evenings together with Ali, Les and Matt, all supporting each other. Natalie was at this stage being tutored at home; she was able to attend school for a few hours on some days, but recovery from surgery was hard and slow.
Between the heaviness of our medical challenges and the mundanity of endless blood tests, logistics and doctors’ appointments, it would lift my spirits so much to walk down the passage at home and hear Jen and her friend Cami giggling loudly as they played a ridiculous game they called “marry one, date one, kill one”. Between school projects and maths homework they wrote silly lists of boys’ names on pieces of paper, which they then drew out of a container. They would then double over guffawing at the results, sometimes inviting me to join in the silliness as I brought tea and shortbread biscuits. It was so good to see Jen having some adolescent, carefree moments.
But mostly, while friends played hockey, danced or swam, Jen sat quietly and watched, with a graciousness that made me both proud and sad. Sometimes, cuddled up in her bed together, she would tell me how isolating it felt to be physically incapable of joining in. Sometimes I’d notice her watching Kristi in a way that made me wonder if she had moments of fleeting envy, just as there were times, I think, Kristi felt guilty for her health and vitality. It was a complex dynamic to balance. Roles and rules easily confused.
There was a lot going on for our family and we had learned and developed black humour, which, though shocking to outsiders, became an important and powerful coping mechanism for all of us. As Freud said in his essay Humour: “Humour is not resigned; it is rebellious.” Natalie and Jenna were particularly observant, quick-witted, wry and funny – they truly led the way when it came to dark humour – and I think this is exactly the point: black humour has the ability to “assert itself against the unkindness of real circumstances”.
It helped us deal with the terrible uncertainty. Jen was clearly very sick, although her diagnosis was not yet confirmed, and Natalie’s recovery was uncertain. Just as we were forced, in a very real way, to stay in the moment and squeeze out all the joy that we could, it also felt as though the Sword of Damocles was hanging over the heads of two of our much-loved children.
On the 12th of April we were dealt a heavy blow. A follow-up PET scan revealed a recurrence of Natalie’s tumours. It was devastating. Shirley knew all too well what this meant and moved quickly to get Natalie back to her doctors in Boston.
By mid-April Jen had been on Warfarin for a while. There were some hopeful moments of improvement, but they didn’t last. She came down with a chest infection and within days her severe chest pains returned. Her breathlessness was extreme. We went directly to see Prof.
A chest X-ray showed significant deterioration since January and “blunting” on either side of her lungs (mosaic perfusion and bilateral peripheral pruning in the small vessels). Prof. arranged a Doppler – a non-invasive ultrasound – of Jen’s legs, pelvis, abdomen, neck and upper limbs. A Doppler is done by bouncing high-frequency sound waves (ultrasound) off circulating red blood cells to estimate the blood flow through one’s blood vessels. A regular ultrasound uses sound waves to produce images but can’t show blood flow. A Doppler can. The idea – and, weirdly, our great hope – was that we would find a thrombosis somewhere that could explain the cause of the emboli. If so, then we would insert an IVC, an interior vena-cava filter. The Doppler checked Jenna’s entire body. Nothing. There was no thrombosis and the CTPA had shown no evidence of a proximal clot amenable to surgery. Small- vessel pulmonary emboli in children or adolescents normally have a predisposing cause or evidence of thrombosis. Not so in Jenna’s case. It was a confusing picture.
Jenna’s bilateral chest pains were becoming increasingly frequent, despite anti-coagulation, and Prof. felt it was time to do the catheterised angiogram. On physical examination there was still evidence of a loud P2 and clinical pulmonary hypertension, but a right and left heart catheterisation needed to be done to be sure. A catheterised angiogram is a dye test used to check the pressures in the lungs and heart. A long tube, a catheter, with a camera on the end is inserted into the femoral artery located in the groin and guided all the way up into the heart to X-ray the veins and arteries measuring the pressures.
Jen was re-admitted to UCT Academic Hospital on the 23rd of April 2012. The procedure, in and of itself not dangerous, would be invasive but quick, but the fact that she was on high doses of blood thinners made things a little more complex. Despite her risk of clotting, she needed to come off the blood thinners to decrease the risk of a bleed-out in surgery.
Jen was prepped and ready, smiling at me as she was wheeled in. She was awake for the procedure, having elected to skip sedation because of the effect it had on her breathing. It was only a 30- to 45-minute procedure, but I paced outside the theatre door. An hour went by. I was still pacing. This was taking much longer than expected.
It was another half an hour before Jenna came out. She was strained, blue lipped, cold and miserable. She was shivering and her blood pressure was low. We got her to the ward quickly.
“Mom, I could feel a warm flush as the dye went in,” she said breathlessly. “It wasn’t easy lying still for so long. The stainless-steel table was so cold, and I needed to pant. I tried very hard to stay totally still, Mommy.”
She was hungry. I calmed her and fed her small bits of crumbed chicken schnitzel from home. We piled her high with blankets, found hot water bottles and brought warm tea. Her blood pressure stabilised but her back ached and all she wanted was to curl up and pull her knees into her tummy to relieve the pain. But it was high risk, the catheter had been inserted into her femoral artery in her groin and she needed to stay straight-legged. For six hours. I read to her as she dozed in and out of sleep.
Later that evening the cardiologist checked in on us and Jenna asked him if he had got what he needed. “Yes,” he said. “We could see that your pressures are way up.” My heart skipped a beat. This was not good. “We took extra time to check if your pressures responded to nitrates, but they didn’t. Prof. Wilcox will explain.” I went cold. Skin crawling. I knew this meant