Читать книгу Get me to 21 - Gabi Lowe - Страница 25

One of the curses of PH is that it’s a curiously “invisible” illness. Unless you knew Jen really well, it was impossible to tell how sick she was. She appeared so normal and calm and had become adept at hiding her breathlessness. It was easy to misunderstand why she didn’t hop up when an adult walked into the room, or rush to help stack a dishwasher or clear the table. If you didn’t know any better, you could assume laziness. She hid her struggle and so it was easy to not notice a slight change in her pallor, the leisurely and seemingly casual pace of her walk, or the fact that her lips were darker than normal. Dark lips are the mark of low oxygen saturation, a look that our Western society deems “pretty”.

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 that our worst fears had been confirmed. Jen’s pulmonary pressures were way up. Every awful medical detail I had ever read came at me. I was nauseous. I felt outside of my body. An altered state of time.

“May I curl up now, Doctor?” Jen’s sweet little voice brought me back to the room. “I just want to bend my legs.”

“Yes,” he said. “It should be safe to move now, Jen.”

After the doctor left, Jen needed the toilet. I helped her out of bed and she walked gingerly to the bathroom. I was standing right outside the door when I heard her shout. “Mom!” I could hear panic in her voice. I threw open the door. She stood, wide-eyed with fright, blood literally pumping from her femoral artery. I screamed. A nurse came running, immediately jamming her thumb into Jen’s artery. Together we got her onto the bed and the nurse applied pressure to stop the bleeding. “Get the doctor!” she instructed. I ran down the passage, my heart pounding, but I caught him, and we raced back to the ward. “You’re not going anywhere,” he said to Jen, and to the nurse, “Don’t let up on that pressure.” Jen’s eyes were wide, but she was quiet.

There was blood everywhere. While I talked calmly to Jen, the nurse bore down, applying maximum pressure. It must have been so sore. Jen held my hand and looked out the window, focused on her breath. Eventually the bleeding slowed, and the nurse could reduce the pressure.

At that moment Granny, Grampa and Kristi strolled unsuspectingly around the corner into the ward, smiling and chatting. It was visiting hour. I ran to block their view and hustled them outside into the passage while we stopped the bleed, changed the sheets and tidied Jen up. Jen and I hugged. “Call them in,” she said calmly. She was remarkable, chatting lightly to her grandparents and putting her wide-eyed sister at ease.

Later that night I climbed into bed next to Stu. In the stillness of the night I started to shake. There was no more doubt. No matter which way you looked at it, we could not suspend the truth. Jenna had PH. The full weight of her diagnosis hit me hard.

Two days later Jen came out of hospital. She was limping slightly and had a bruise that spread from above her groin to halfway down her thigh.

There was a school function in the sports hall that evening that she wanted to attend. I remember, with absolute clarity, the moment that the school choir began their opening song. Their emotive voices echoed round the hall with familiar lyrics from “I Hope You Dance” by Lee Ann Womack. It was a singular moment. The music, the words, the sight of all those fresh-faced healthy young girls singing their hearts out. Jen would never dance, she would never take one single breath for granted and she probably didn’t have a fighting chance … and yet no one knew. Seated in the middle of the huge hall, packed with robust children and their hope-filled parents, I felt utterly alone. I held on tightly to Stuart’s hand, scanning the mob of blue school dresses frantically searching for my girls. I needed to see they were okay. I saw Kristi, hair pulled back into a tight high pony, leaning playfully into a friend, chatting animatedly. I scanned the crowd again and found Jen. Her head was lifted high, she was smiling, standing, shoulders back, next to Camilla as if nothing was wrong. She was the ultimate picture of poise, grace and courage. Stu and I held tightly onto each other, our palms sweaty with emotion, battling to contain ourselves.

It was a glimpse of what lay ahead ... the constant struggle to cope in the normal world, while holding the knowledge that Jen was fighting for her life. Nothing for us was normal. It was the ultimate paradox, to hold both fear and hope.

With no more room for denial, Stu and I talked at length about finding ways to consciously create joy. It was tempting to withdraw and batten down the hatches but, having learned the value of staying connected, we made sure our loved ones were always welcome in our home, no matter how sick Jen was. Our friends and family flocked to support her.

Daffy started to visit more regularly – he and Jen had remained friends over the years – and he had become a much-loved part of our family. They grew incredibly close. They played chess, chatted for hours and watched movies. It was a joy to hear them laugh, easily and often. When Daffy visited, Jen was her most natural, relaxed and happy self. She was loved by Daffy for exactly who she was. It didn’t matter how tired or challenged she might be feeling that day. With him she didn’t need to pretend.

There were also regular visits from a gorgeous tall boy called Max, who lived just a few blocks away from us. This friendship was a lot more charged and complicated, as both Jen and Kristi perked up when he arrived … mmm … two young teenage girls with a crush on the same boy. This was a potential recipe for disaster.

On Natalie’s 12th birthday, the 28th of April, we went to the revolving restaurant, the Top of the Ritz, to celebrate a long-held tradition to mark the children’s first “double digit” birthdays. This would have happened for Natalie’s 10th birthday, but she was in surgery at the time. With Natalie and Shirl about to head back to Boston, we knew this was the time to mark the milestone.

Ahead of their departure, Ali and Les kindly arranged to rent the same country house in Greyton where we’d spent Christmas, so that we could insulate from the world and spend a weekend together as a family. The weekend was bittersweet and saying goodbye again was painful.

En route to the airport Shirley stopped in Somerset West to say goodbye to her dad. Peter’s health was deteriorating and she knew it was possible that Natalie would not see her Pop again. He had been diagnosed with multiple myeloma, the same rare bone cancer that had claimed the life of his wife Jean.

We were facing assault from all sides. The harsh reality of Jen’s illness was that even though she had been on a vaso-dilator, Revatio, since the angiogram, we were yet to see any improvement.