Nicholas Vermaxidis is a bundle of energy when he greets me and asks if we can go outside to play. His beautiful eyes show the smile that is hidden behind his mask, and he is unaware of the thin hair that sparsely covers his recently bald head. His mother Elena speaks softly to him in Russian, and he runs off to play in the room next door.
Elena Vermaxidis looks right at home in the downstairs kitchen of the St. Pete West House, reserved just for bone marrow transplant families. A Russian beauty with sparkling blue eyes and an excellent grasp of the English language, she recalls the day she was shopping in Walgreens when her husband Angelos called her with the good news, “Sophia is a match for Nicholas!” They cried together as they shared this wonderful news that their little girl Sophia is a perfect bone marrow match for her brother Nicholas, who has been diagnosed with a very rare blood disorder called Fanconi Anemia.
Elena recounts that her first child Nicholas, now four years old, was born from a normal pregnancy. Although he seemed to have a poor appetite, there seemed to be no cause for concern and she was given many tips on breast feeding and formulas to try. However, each consecutive doctor’s visit showed that the little boy was not growing, and by four months had stopped growing altogether. The family patiently waited a long eight months to see a specialist, who did a growth hormone test. The test showed that Nicholas had a deficiency and was prescribed growth hormones.
While on vacation in Russia with Elena’s family, they saw a doctor who performed a sonogram on their baby. The test revealed that one of Nicholas’ kidneys was very small, while one was a normal size. Upon their return to the U.S. they took the baby back to the pediatrician who did another sonogram before referring them to a nephrologist and a urologist. The doctors could see there were issues with the kidneys, but continued with the growth hormones until he was 18 months. Nicholas was finally growing, but the doctors decided to remove the smaller kidney since there was little chance it would grow to a normal size. Prior to surgery, blood work tests showed that his platelet count was much lower than it should be.
The family was referred to a hematology/oncology doctor who they now realize made a huge difference in the treatment of their child. Dr. Iftikhar Hanif, at the Joe DiMaggio Hospital in Hollywood, Florida analyzed all the data from the numerous tests on Nicholas and came up with the resulting diagnosis of Fanconi Anemia (FA). FA prevents the bone marrow from making enough new blood cells for the body to work normally and can cause the bone marrow to make faulty blood cells. This can lead to serious health problems, such as leukemia.
Although FA is a blood disorder, it also can affect many of the body’s organs, tissues, and systems. FA also increases the risk of some cancers and other serious health problems. Children who have Fanconi syndrome pass large amounts of key nutrients and chemicals through their urine.
Armed with a diagnosis, Elena began to search the internet about the disease, becoming more frightened as she read page after page of dire consequences. Finding a bone marrow match for Nicholas was the next step, because the disorder could turn to leukemia. Both parents were tested, along with baby sister Sophia. While waiting for results, Elena and Angelos took Sophia for genetic testing, praying that she had not inherited FA. Both parents are carriers, resulting in a 25% chance that Sophia could also have this rare disease. They also checked their family histories, but given that this disease was not diagnosed even 30 years ago, it was difficult to know if a relative had been affected. Thankfully, Sophia tested negative for FA.
Now that Sophia was a bone marrow match for Nicholas, the next step was finding a hospital to do the transplant. The best hospital for FA patients is in Minnesota, and another in Cincinnati, but neither were a possibility for a family with children on Medicaid. They considered Miami Children’s Hospital, only to learn that the doctors there had no experience with FA. Finally, they found Dr. Aleksandra Petrovic at All Children’s Hospital (ACH) in St. Petersburg. Elena feels very comfortable with Dr. Petrovic, who is from Serbia and speaks a similar dialect. Elena was very worried about where they would stay so far from their home on the East Coast, until she learned about RMH. It was very stressful wondering how they would afford a second apartment and car in order to stay near the hospital. Like many families, the Vermaxidis family did not know what RMH was, and were amazed to find such a wonderful home that would care for them during this nerve-racking time.
A summer date was scheduled for the bone marrow transplant (BMT) which Elena explained is not really a surgery, but more like a blood transfusion. However, like many families with little ones, the kids kept getting sick, and the date was pushed back several times so they could both be healthy. Elena’s mom flew from Russia to help out, and she stayed with Sophia at RMH West to keep her safe from germs, while Elena stayed with Nicholas at ACH. The little boy endured five days of chemotherapy in advance of the BMT to prepare his body for the transplant. Finally the transplant day arrived, and Sophia was anaesthetized to extract the bone marrow, which was immediately sent to Tampa for tests. Later that day, the bone marrow was returned to ACH for transplant into Nicholas.
29 days later, Nicholas was discharged from ACH into his mother’s care in the BMT wing of RMH West. This area of the house is reserved just for BMT families due to the risk of infection and special dietary needs. Nicholas was put on a low microbial diet which minimizes the introduction of pathogenic organisms into the gastrointestinal tract by food while maximizing health food options for immunosuppressed patients. This means he can’t have any raw fruit or vegetables in addition to numerous other restrictions. Nicholas is very fond of apples and cucumbers, but has learned after many requests that they are not allowed on his diet. The West House kitchen is perfect for Elena to prepare all of the special foods that Nicholas needs to get better. She feels at home in this special house, which has been her residence since last summer. There are lots of toys for Nicholas, a secure outdoor patio which allows him to let off steam, helpful volunteers and other BMT families. Transportation to and from the hospital was extremely helpful in the early days when even a short walk was not possible for Nicholas. Now that he is further along in recovery, he can ride his bike alongside his mom to and from the hospital. The sight of this beautiful little boy, nicknamed “baby biker” with his mask and sunglasses to protect his sensitive eyes makes everyone smile.
The rule for BMT patients is to stay within one hour of the hospital for the first 100 days after transplant so the patient’s health can be continuously monitored for infections. The greatest risk is called graft-versus-host disease (GVHD), a complication that can occur after a stem cell or bone marrow transplant. With GVHD, the newly transplanted donor cells attack the transplant recipient’s body, with a 30-40% risk of occurrence when the donor and recipient are related.
Because the Vermaxidis home is four hours away, Elena and Nicholas are content to stay at RMH West until it is safe for them to go home. Elena feels like she has two “moms” between Dr. Petrovic and her social worker Julie Miotke, who has been a huge help in doing all the necessary paperwork and coordinating medical visits. Elena wonders what they would have done if not for RMH. She is friends with other FA families on Facebook, including a few in Russia who have to rent a very expensive apartment near the hospital in order to stay near their child. Most people do not believe what RMH has to offer she exclaims, and she cannot believe it either!
Nicholas has a central line for Elena to administer his medications and keep him hydrated with fluids. Her hope is that with proper hydration, his remaining kidney will improve from the damage it retained from taking so many medications. Otherwise he will need a kidney transplant at some future time. Nicholas is a typical child when it comes to taking medications and drinking lots of water, he cares for neither. While he’s only four years old, he understands that he must always wear his mask because he is sick and needs to get better. Living away from home is not easy for a child, but Nicholas has stopped asking when he will go home. Instead he calls this place that his mom has fallen in love with his home. He only asks when his Dad will come to visit. Sophia is staying with her grandparents in Russia until Elena and Nicholas come home and Skypes every night with her mom and brother, along with her father. Nicholas understands that Sophia saved his life, and knows that he must always protect her.
Staying at RMH West has been very helpful in Nicholas’ recovery because he is happy and makes friends easily. His smiling eyes and enthusiasm engage the volunteers and staff at the house. When volunteers learned that he was sad because he didn’t have a costume for Halloween, they went out to buy him one, much to his delight. Elena has also made some good friends at the house, both with volunteers and other long term families. While she has to prepare all of Nicholas’ food, she enjoys eating the meals prepared by the community groups at the house. She was offered the option of staying in nearby apartments run by ACH for BMT families, but is thankful that she chose to stay at RMH because it was a better fit for her and Nicholas.
Now that Nicholas is just past the 100 day point, Elena looks forward to the day they can go home, but is cautious about leaving too soon. She has gained so much perspective in her months at RMH, commenting that at first she was sad to be there until she met families with much greater problems. In the months since she has come to love the people at RMH and ACH. Elena is enrolled in ESL (English as a second language) classes and hopes to attend college one day so she can work in the medical field, which she finds fascinating.
The greatest hope for Elena and Angelos is that advances in the medical field will take place in the coming years so that future health issues for Nicholas can be treated. Once they go home, Elena will have to bring Nicholas back once a week for follow-ups and will likely stay once again at RMH West. According to Elena, “RMH is a place that is the biggest help in our situation and we can’t imagine what we do without it.”