A Beautiful Rare

By: Pencil Cut, Ren Masanque - Madrid

So now that things are quieter, I want to take this moment to pen something beautiful. Today, I muster to tell a story of what seemed to be an insurmountable mountain back then. It is a story of God’s faithfulness, and I want to begin with the last two long years of my life. Please ride with my musings.

Like a thief in the night, it came without a warning. On March 1, 2018, my husband was diagnosed with a very rare bone marrow failure disease called PNH. It stands for Paroxysmal Nocturnal Hemoglobinuria. It was benign, to some extent of relief, but still life threatening. With PNH, all of his blood cells were defective. Low red blood cells depleted his energy, decreased white blood cells weakened his immune system, and dangerous levels of platelets put his life at high risk as thrombosis or blood clots was the leading cause of death from PNH. To make it more understandable, I quote our PNH Specialist, think of your bone marrow as a blood factory where your body makes red and white blood cells, and platelets.  PNH shuts down your blood factory.

PNH was something sporadic (paroxysmal) as explained to us. Unfortunately, it randomly chose my husband. It started with a gene mutation of his bone marrow stem cells and then the multiplication of PNH clones or the unhealthy red blood cells as seen through a bone marrow biopsy. And it only took a consistent, noticeable tea-colored or dark or bright red blood (hemoglobinuria) urine at night (nocturnal) or in the morning to validate a bad suspicion. This process was called hemolysis. His other symptoms were muscle cramps, abnormal palpitations, and brain fog and unexplainable tiredness which he dismissed as a jetlag. All these left my husband in desperate need of a treatment, and a permanent cure. He became transfusion dependent. The habit of going to the hospital like it was our home, the call for blood donors, and the administration of blood products which I lost count, became his way of life at the prime age of 36. It was so painful to watch how PNH suddenly turned his life in shambles.

Because PNH was rare with a yearly incidence ratio of 1 is to 1.5 in a million, only few doctors were well aware of it. In the Philippines, we lack PNH specialists unlike in advanced countries like the US, UK, and Canada. It affected a few so the awareness meter was low. Given this limitation, I armed myself with information, attended webinars, and joined PNH support groups where I met my now local and international PNH friends. As I went on with my research, I stumbled upon one of the top PNH Specialists in the world. With the clearance of our attending hematologist, we flew to the US to seek medical care. Notwithstanding a category 5, super typhoon, late of Sep 14 2018, we, together with my father and youngest brother, took that important flight. It was a turbulent ride connecting us to Taiwan because of the direction of the storm. But thank God, we arrived safely. 

Dr. Ilene Weitz, a top PNH Specialist in Los Angeles, California, became our primary doctor overseas who helped us navigate my husband’s condition. She was affiliated to Keck Medical Center of USC. Meeting her was seamless. She was very welcoming and well-loved by her patients. She did a thorough physical check with my husband, patiently answered our questions, and asked about our health insurance. As you know, Eculizumab or Soliris was used to treat PNH or at least put the PNH symptoms at bay. This was not available in our country and the cost of this drug was outrageously expensive. In fact, it was dubbed as one of the most expensive medicines in the world. The price tag was a whopping USD 500,00 per year or an equivalent of P24 million per year for life. Patients in the US, for example, have access to this because of their insurance but for other countries such as ours, it was an unaffordable cost. 

Dr. Weitz wasted no time to help us get Soliris even if we were not US citizens. She recommended our case to a Complement Foundation for the grant of compassionate use, and to the Alexion International team, the maker of the drug, for quick resolution. Back home, our hematologist, Dr. Ivy Escasa, helped trigger all the documentations and sign-offs in our hospital of care, and in a matter of weeks, Soliris found its way to my husband with the first dose administered on Dec 17 2018. But prior to this, my husband and I were already convinced to migrate and submit ourselves in clinical trials. Desperate measures in desperate times. However, God had His own plans. Our thoughts are not His thoughts. He is bigger than our problems. He is a way maker, a God of miracles, and a God who makes the impossible possible.  A sovereign One. We may not feel it but there is a hidden hand helping us even before we asked for it. God has already prepared everything.

We had high expectations that things will get better for my husband. Unfortunately, after 6 months of constant administration of Soliris, his condition did not improve. This led us to make the big decision that I was most afraid of.

Dr. Weitz told us that my husband’s disease has progressed, and the next best course of treatment was the bone marrow transplant (BMT). She had no doubt about it since my husband has an identical twin, Reissite. And this lowers the risk of rejection, and the potential graft versus host disease, a common complication from the procedure. I knew how risky BMT was. But as I also knew that high risk means high reward. At this stage, my husband had gone through a lot – a bout of pneumonia, hearing loss one after the another, weight gain from steroids, bruises, blood blisters, and petechiae all over his skin, refractory platelets, and iron overload from countless blood transfusions which can ultimately damage his vital organs. On top of these, PNH was relatively a high-cost disease. 

Once again, PNH brought me to my knees. I was afraid. I consulted our spiritual advisers, Fr. Greg and Ate Jenny, my husband’s eldest cousin, who told me to not give up to the Lord. She said to fill myself with the power of God. We have limitations but God is unlimited. Continue believing His promises.  Thy will be done on earth as it is in heaven should be the prayer. With this comes full trust to the One who holds our life, who knows everything, and who journeys with us. God is rich in mercy. He is the Divine Healer. He will heal according to His Will. 

We prayed incessantly for guidance. We said our novenas to Our Lady of Lourdes, Health of the Sick, to whom God has entrusted us, to St. Pio, who is the Patron Saint of Pain and Suffering and whose relics we were able to touch and venerate when they arrived in the country, and to all our favorite saints, old and newly introduced to us. We visited churches even if my husband just stayed inside the car so he wouldn’t be exposed, and dropped letters of petitions in Mother of Perpetual Help in Baclaran, Black Nazarene Church in Quaipo with the help of my sister, and as far as to Our Lady of Lourdes in France and San Giovanni Rotondo, Italy during the trips of our relatives and friends.  I tried my best to cling to my faith. Then one day, Mama Mary’s lines resonated to me as she said, “My child, Be at peace. We will be with you. Are you not in the hollow of my mantle, in the crossing of my arms? Let nothing else worry you nor disturb you.  I am here. I am your Mother. Do not fear. I am near.” This ultimately gave us confidence and assurance that everything will be well with our decision.

On Nov 14 2019, one week after my husband’s 38th birthday, we began the allogeneic BMT process. He received the conditioning regimen prior to day zero which included daily chemotherapy and ATG, which was short for Anti Thymocyte Globulin, an infusion of horse antibodies against human T cells, but this sent rashes all over his body, and chills as a side effect. Then, in the morning of Nov 22, 2019, as led by our topnotch BMT doctor, Dr. Francisco Lopez, his twin’s donated stem cells were finally implanted. On the other hand, Soliris was still given 3-4 times as the marrow recovers. While we waited patiently for the new stem cells to travel to my husband’s bone marrow and start producing new, and healthy cells, I saw how things were still extra challenging. The transplant itself was long and arduous. It was not painful but accurately difficult. 

Along the way, my husband battled two infections, one was bacterial and the other one was a fungal infection (a.k.a. Galactomannan) as a result of the procedure and this made his suffering even worse. He coughed 24/7 which kept him awake night and day and the high-grade fevers never went away. We needed extra blood donors as well.  At this juncture, he was already very exhausted and so am I. For the first time, I saw my husband broke down and cried inconsolably. God seemed so silent and distant. But then I remembered the words of St. Pio of Pietrelcina, “Stay in the boat in which our Lord has placed you, and let the storm come. You will not perish. It appears to you that Jesus is sleeping, but let it be so. Don’t you know that if he sleeps, his heart vigilantly watches over you?” This maybe our rock bottom. But when we all go at this point, it is there that the Lord is nearest, almost that it can’t be felt like cloth near to the skin. When we are at our lowest, God leans down and lifts us up, just as I read in one social media post. That night of Dec 13, 2019, I let go and I trusted God more. In the following days, a miracle happened. I saw an image of the Divine Mercy while resting. The rays of Mercy I can still vividly recall.  Maybe I was just imagining but then I took it as a sign. I was reminded that God has not abandoned us. He was carrying us all this time of trials and hardships and even though I walk through the valley of the shadow of death, I will fear no evil, for you are with me, your rod and your staff, they comfort me.  The medical team managed to treat the infection and my husband became okay. And just 6 days before Christmas and after that 35 enduring days in the hospital, we were discharged.

PNH was a faith testing experience, a purification, and our special assignment on earth. PNH was our invisible cross but also our sweet share to God’s suffering. While I didn’t like the path of PNH, still it made me reflect on the brevity of life, the importance of the ones we love, and what matters most. Of course, I was guilty since I chalked PNH up as a simple anemia at the beginning. Again, I was busy with my career. But PNH was written in our destiny, not to destroy us but to serve its purpose of experiencing God who is real. Remember, winds of testing do not last forever. Just persevere until the end like the woman who bled for 12 years. Isaiah 58:8 said, “Then your light will break forth like the dawn, and your healing will quickly appear; then your righteousness will go before you, and the glory of the Lord will be your rear guard.” God is always faithful even to the faithless. He is with us. He answers our prayers. I will always be grateful to God for my husband’s second chance in life. It only means that his mission on earth has just started and together we are called to evangelize and spread the goodness of God. Isaiah 42:9, “Behold, the former things have come to pass, and new things I now declare; before they spring forth I tell you of them.”

Fast forward to 2020, and a year later, my husband was cured of PNH. I am extravagantly happy to share that he is doing well. And this is our silver lining during this time of pandemic. I pray that this story of us and our Lord, Jesus Christ, carry you this 2021 and the years to come.

P.S. To our parents, our families here and abroad, and friends, thank you for everything. You know who you are. To our medical team, nurses, and staff thank you for taking good care of us. To our blood donors, and GoGetFunding supporters, thank you for the generosity. God bless you a hundredfold. Finally, to our Lord, and Redeemer, thank you so much. You are the Source of all graces that overflows.  - Ren & Ren