No Parent Should Ever Have to Say "Good-Bye"!!!!
by Laurie Leveille
(St. Catharines, Ontario, Canada)
After the doctors at Juravinski were able to consult with one another, they agreed that Clayton was most likely suffering from a form of HLH. This disease is normally found in children under the age of three but on rare occasions, individuals in their early twenties can also acquire it. This disease results in the destruction of one’s own blood. His body sees it as a foreign substance and destroys itself. There are two forms of HLH, genetic and EBV induced. With the genetic form, females are carriers whereas males inherit it. Since we have three other boys, none of whom had shown any symptoms, the doctors believed that Clayton had developed the EBV induced version of HLH. Previous tests revealed that his body was saturated with the virus so this was completely plausible. Before they could start treatment, the EBV needed to be eliminated. If not, when Clayton’s immune system was eradicated by the treatment, he would succumb to the virus itself. A week later, it was confirmed that the EBV in Clayton’s blood had been greatly reduced. Finally, we believed that treatment would begin shortly. Once again, however, we waited. They needed two consecutive blood tests showing decrease in EBV before they would begin treatment.
Heading into the third week at Juravinski, Clayton continued to wait while nothing was being done. With all this waiting, he developed thrush. Eating, drinking and talking became extremely difficult. For some reason, the doctors now needed to prove conclusively that Clayton was suffering from HLH. Treatment was not a high priority. Both my husband and Clayton agreed to sign off saying that they’d take full responsibility for HLH treatment. The doctors refused. They needed 100% proof. Clayton’s hope for a treatment was being ripped right out from under him. He was losing his will to fight. We all knew that without it, Clayton couldn’t possibly survive. Why would the medical profession totally disregard a patient’s request? After seven months, Clayton needed some sort of treatment to be started. He needed to know that there was some hope. Because the doctors were only 85% certain that he was suffering from HLH, Clayton did not have any say with respect to starting treatment. Basically, his life was completely left in the hands of the medical profession. As time went on, Clayton was merely being kept alive in order to obtain an indisputable HLH diagnosis. In attempts to gain the other 15% proof, Clayton’s body was beginning to weaken quickly. It was highly unlikely that he would be able to handle the treatment even if HLH was conclusively proven. Still, the doctors wanted their proof. They decided that a liver biopsy might give them what they wanted. Although Clayton’s platelet count was now at 2,000 and internal bleeding was a great risk, the need for indisputable proof outweighed any negative effects of the biopsy. In Florida, however, the doctors refused to do a liver biopsy when his platelet count was at 40,000. Why were the doctors at Juravinski playing Russian roulette with our son’s life? If only they would have begun treatment when he arrived, Clayton could still be here. As parents, we just wanted something done to give him a chance. Because of the extremely low platelet count, they couldn’t put Clayton out under anesthetic. In order to perform the biopsy, a tube was to be inserted into a vein in his neck which led directly to the liver. Through this tube, a probe was used to obtain the liver samples. They did assure us that he wouldn’t feel a thing and he would not know what was happening during the half hour procedure. The biopsy took over two and a half hours. The liver is an organ which apparently bleeds easily. With as few platelets as Clayton had, the doctor had difficulty controlling the bleeding. When our son finally came out of the procedure, he was exhausted. When questioned, he did indicate that he saw and felt everything. The fear in his eyes was heart breaking. He no longer even tried to speak and slept for most of the time. Mid-week, the doctors found one of the liver samples to contain abnormal cells. They believed that these were most likely HLH. Treatment was finally started. The next day, however, his breathing had become laboured so he was put on 50% oxygen. It seemed to be helping but by evening, he relapsed and oxygen was increased to 100%. A catheter was inserted to try to remove excess fluid which was accumulating in his body. He had been swelling for quite some time. Clayton only woke when his pain became unbearable. During the night, he struggled to breathe. One or more family members always remained with Clayton. No one wanted him to go through this alone. By early morning, it was evident that Clayton was having a hard time clinging to life. He was transferred to ICU where they could monitor his care more closely. He now had to be ventilated since he was struggling to breathe on his own.