Hina’s Bone Marrow Journey
As Hina’s mother, I know that it is difficult to watch your child suffer through frequent pain crises. It is a helpless feeling, and naturally, as a mother, you want to do all that you can for them. The reason I want to share Hina’s story is to give other parents going through a similar situation a clear view of what receiving a bone marrow transplant may entail. There are numerous complications to be aware of and the procedure is extremely risky. Still, there is always hope that the transplant may be a success.
Hina was diagnosed with Sickle Cell Beta Thalassemia 3 weeks after birth. Every time she had a severe pain crises we wished there was a cure; the pain was unbearable for us to watch and more so for her to suffer. The multiple complications of Sickle Cell Disease clouded the minds of my husband and I and led us to look for different alternatives. My husband came across the option of a bone marrow transplant. Since our other child was not an HLA match, he looked for an unrelated donor. An HLA-typed match was found (9 out of 10): a 42 year-old male donor. We sought the opinions from two expert transplant hospitals: Children’s Hospital of Los Angeles and Lucille Packard Children’s of Stanford and were told there could be a success rate of approximately 80-85%. Complications such as GVHD and infections were also discussed and we were also informed that the procedure could possibly be life threatening. We spoke to two patients with Sickle Cell Disease who had undergone bone marrow transplants and survived with very few complications, which made us hopeful. Prior to the transplant, Hina had a complete check up of all her organs and was deemed healthy enough to undergo the procedure. Hina underwent the bone marrow transplant on May 31, 2008 at CHLA. She had 10 days of conditioning with chemotherapy to ablate her existing marrow cells. Hina tolerated the chemotherapy regimen without any problems and the new donor marrow was infused. Two weeks later, her white blood cells, including the neutrophils, red blood cells, and platelets counts were on the rise and she was completely engrafted with 100% donor cells. She had no more Sickle Cell Disease, the news we had hoped for. After 45 days of dealing with headaches and hypertension, she developed rashes on her arms and upper back. This was her first sign of skin GVHD and she was placed on treatment with Prednisone. After 60 days of staying in an isolation room, Hina was sent home. We were very happy, but soon more complications developed and we were making visits to CHLA one or two times every week. Over the next two years after the transplant, Hina was in and out of the hospital for a cumulative duration of 9 months in isolation. We lived in constant fear, not knowing what problem we would have to face in the next minute. We traveled to the Seattle Children’s Hospital on December 27, 2010 in hopes that they would be able to cure our daughter. They made many adjustments to her GVHD medications, which further complicated the problem. Since Seattle was too far to travel for medical attention needed on daily basis, we sought medical care at Mattel Children’s Hospital at UCLA. She was treated by the Pediatric Blood and Marrow Transplant team for 4 months, but the complications of her liver GVHD became so severe that nothing more could be done and she passed away on May 5, 2010 at UCLA.
Chemotherapy Medications used for conditioning
Busulfan, Cyclophsophamide, Fludarabine, and Alemtuzumab
Adenovirus in blood and stool, GVHD of skin (rash & loss of hair), GVHD of gut (8-10 large volume diarrhea EVERY day for months, which eventually resulted in bloody diarrhea), GVHD of the liver (yellow eyes & skin), Clostridium Difficile colitis (7-10 diarrhea with blood daily), Hypertension (high blood pressure), Hyperglycemia (high blood sugar), Hypothyroid, Pancreatitis, Fevers (but no detectable bacterial infection), Pancytopenia (shortage in the amount of WBC, RBC, and Platelets), Thrombocytopenia (low platelet counts), Edema (swelling due to water retention), Hematoma (collection of blood outside of a blood vessel), leg cramps, headaches, anorexia, Weight gain due to prednisone, Osteoporosis, Hypercholeserolemia (high cholesterol), electrolyte imbalance, abdominal pain, gas, and countless others.
List of medication over 2 year period of BMT
Cidofovir weekly with Probenecid and IV hydration, Methylprednisolone, Prednisone, Azathioprine, Tacrolimus, Sirolimus, Cyclosporine, Mycophenolate, Budesonide, Daclizumab, Pentostatin, Basiliximab, Rituximab, Beclomethasone oil, Fluocinonide Cream, Tacrolimus ointment, Metronidazole, Vancomycin, Clonidine patch, Nifedipine, Enalapril, Chlorothiazide, Furosemide, Spironolactone, Humulin Insulin, Lantus Insulin, Levothyroxine, Norethindrone, Yaz, Ursodiol, Octreotide, Filgrastim, Epoetin Alfa, IVIG, Meropenum, Cefepime, Piperacillin, Ciprofloxacin, Levofloxacin, Cephalexin, Amoxicillin, Penicillin, Bactrim, Pentamidine, Atovaquone, Acyclovir, Voriconazole, Micafungin, Caspofungin, Oseatamivir, Gabapentin, Suclarafate, Lansoprazole, Omeprazole, Pantoprazole, Famotidine, Ranitidine, Diphenhydramine, Ondansetron, Promethazine, Vitamin K, Magnesium, Potassium Chloride, Sodium Chloride, Calcium, Vitamin D, Folic Acid, Multivitamin, and TPN.
Hickman Catheter, Picc Line, Endoscopy/Colonoscopy 4 times, Bone Marrow Biopsy 4 times, Skin Biopsy, Liver Biopsy, Intubation for Angiogram, Cauterization on the cecum area of colon.
Lab tests 3-4 times per week, going to home health care 3 times per week for IV meds, daily infusing of IV meds & TPN at home, going 3 to 4 times per week for platelet and blood transfusions, changing picc line dressing weekly, taking 60 to 90 pills per day, many trips to the emergency room.
Total Platelet Transfusions over 2 years: 129 times
Total Blood Transfusions over 2 years: 44 times
Our brave daughter went through an indescribable amount of suffering, but never once did she complain about any of it. She kept a positive attitude, smiled continuously, and studied in the isolation room to maintain her 4.1 GPA and high AP exam scores. She graduated high school and was determined to become a pharmacist. She applied and was accepted to the University of the Pacific Pharmacy Program, which sadly, she was never able to attend.
If I, as a mother, had a second chance, I would not have decided to put my daughter through the transplant process. While there might be a chance of a cure, there is also the possibility that the child will be worse off than they were to begin with. Bone Marrow Transplant is a hope for a cure but not one that should be considered lightly. No one, including physicians, has a crystal ball (the ability to see the future) to predict the outcome for any given individual. I implore every family considering a Bone Marrow Transplant to research and explore every option and seek out multiple medical opinions before making such a life changing decision. For more information please feel free to contact me.