One little boy’s fight against a rare blood disease.
By Bryce Martin
Published August 2, 2013
Dee Dobitz considers her two-and-a-half-year-old grandson, Kellan Solem, to be a daredevil.
Behind his normal little boy appearance, however, wages a serious internal battle.
Solem was diagnosed with Hemophilia B (Factor IX) in 2011 – the second most common type of hemophilia, a blood disorder that prevents blood from clotting correctly resulting in an extended bleeding time.
The disease largely is an inherited disorder, according to the National Hemophilia Foundation (NHF), though Dobitz, a resident of Bowman, said there is no family history of hemophilia. In about 30 percent of cases, there is no family history of the disorder and the condition is the result of a spontaneous gene mutation.
Solem, who lives with his parents Andrew and Miranda in Thief River Falls, Minn., began exhibiting symptoms of the disease shortly after birth. Those symptoms unfortunately went undetected by an inexperienced nurse who mistakingly shrugged off Solem’s excessive blood flow following a Phenylketonuria (PKU) Test. The procedure, in which a baby’s heel is poked with a small needle, is a standard test to determine if the child has phenylalanine, a necessary enzyme needed for proper development.
“When they did the PKU test … there was excessive bleeding,” Dobitz said. “But at the time, it was a new nurse and he told us he had done something wrong and, of course, (the bleeding) stopped. And we just thought … it’s not a big deal.”
In retrospect, it’s possible a more experienced nurse would have spotted the bleeding as a red flag, Dobitz said.
Family practices or general practitioners may not have a deep understanding of hemophilia, said Jana Suder, a nurse practitioner and program coordinator with the Sanford Health Hemophilia Treatment Center in Fargo.
Andrew and Miranda almost lost their precious son when Solem had several spontaneous brain bleeds at 5-1/2 months old.
Strange symptoms occurred when Solem suddenly became lethargic and would not eat or drink. Drs. Duane Browning and Suresh Sreedharen attended to Solem when his father took him to the Sanford Thief River Falls Clinic. Typically absorbed by non-hemophilic brains, a vast amount of blood inside Solem’s brain pooled and resulted in a need for brain surgery. Both identified that it was a critical problem, had him stabilized and transported him via ambulance to Fargo.
“Everything went together really well for us,” Dobitz said with a sigh of relief. “It turned out there was a trauma surgeon working at Sanford that could do the surgery. Otherwise, they’d have to send him to Minneapolis. And even airlifting him, he probably wouldn’t have survived.”
Once in Fargo, the surgeon ran a test and discovered Solem was a hemophiliac. That was the first time Solem’s parents received confirmation of their son’s illness.
Solem can never play contact sports when he gets older. Any kind of fall or head injury needs to be treated immediately. And at such a young age, Solem has a Port-A-Cath, which is a small medical appliance that is installed beneath his skin. A catheter connects the port to a vein. Under the skin, the port has a septum through which medication can be injected and blood samples can be drawn many times. Through the port, Solem undergoes weekly injections of Factor IX concentrates as treatment, unless he has a bleed and has to receive an immediate injection. Factor IX is meant to replace the clotting factor that is absent in hemophiliacs.
Occasionally, Solem will have to sit through up to five or six injections in one week.
If Solem gets a deep cut, or any kind of internal injury, he needs immediate treatment. Bleeds also can happen from repeated use of the body’s joints, by such simple means as constant bending arms or legs. As an infant, his family members had to be extra cautious on how they held Solem in their arms as the incorrect way could cause a bleed. And during his ambulatory learning stage, trying to stand or pull himself up could cause bleeds.
Solem had some developmental delays due to his brain trauma, “but he’s conquered all of them,” Dobitz said.
“He’s ahead of the curve, other than he doesn’t jump like he should,” she said with a chuckle. “And we’re all right with that.”
Two years ago, the family met a semiprofessional baseball player around 30 years old that was a hemophiliac, Dobitz said. He was not allowed treatment by his mother; their plan was to just deal with the disease. Each time he would have a bleed, someone in the family would do a blood donation and transfuse the man.
“Consequently, of all the kids he was growing up with, he’s the only one who is still living,” she said.
“So it’s not that long ago that it was pretty much a death sentence.”
As the only hemophilia treatment center in North Dakota, the Sanford Health Hemophilia Treatment Center currently sees around 100 people with bleeding disorders, hemophilia being one of them. Suder said that is an accurate indication of how many cases are present in North Dakota.
Hemophilia affects 1 in 10,000 people, with about 18,000 cases nationwide, according to Jim Paist, executive director of the Hemophilia Foundation of Minnesota/Dakotas (HFMD), which serves Minnesota, North and South Dakota.
“It’s pretty consistent in North Dakota, to the best of our knowledge, with percentages in the rest of the country,” Paist said. “It’s a very rare condition.”
The disease also almost exclusively affects males – 99 percent of those affected by hemophilia are male. Women are carriers of the genetic mutation that causes hemophilia. If Solem were to have children as an adult, his sons would not have hemophilia, but his daughters would be carriers, according to Suder. Carriers don’t exhibit symptoms, but do show red flags without the excessive bleeding.
In addition to the severe issue of Solem’s health, other indirect problems also occurred for the family, including suspicions of the parents being responsible for child abuse.
During one hospital visit, emergency personnel became concerned about Solem’s symptoms. According to Dobitz, hospital workers intended to contact the authorities because they suspected Solem had shaken baby syndrome, the intentional shaking of a baby that causes subdural hematomas, retinal hemorrhages and cerebral edemas.
“Even when people are familiar with bleeding disorders, every time a child is brought in with excessive bruising, an alert gets triggered and there’s a big suspicion of abuse,” Suder said. “And hemophilic children bruise in places where you wouldn’t find bruises on normal kids.”
There was another incident where Solem was taken into the hospital with bruising on his ribs. They kept Andrew at the hospital, asking him questions for nearly six hours on false suspicions of child abuse.
“I have heard that scenario happen before because the child bruises so easily that when they bring the child in to see a doctor that certainly the bruise is of concern,” Paist said.
Suder agreed, adding that it does happen quite often.
While there is no cure, only treatment, a pediatrician said Solem would grow out of the disease by the time he’s a teenager, which is false according to the National Hemophilia Foundation.
“Try and find someone who is knowledgeable and find out what it is,” Dobitz advised. “If anything seems suspicious or out of the ordinary, don’t be afraid to get it checked.”
Suder also said that if a person has excessive bruising or prolonged bleeding, they should contact their physician.
When he was diagnosed, Solem needed his medication three times a week. Two years later and he’s only needing them once a week as long as there’s no bleed shows that medical researchers and doctors are discovering new things about hemophilia, Dobitz said. “Research is critical,” she said. “They’ve come huge degrees since the last 15 years.”
According to Paist, children with hemophilia can live long lives because of the advancement in medical technology and the health of a child born with hemophilia today compared to 30 years ago is amazing.
“It’s really remarkable to see the advancement,” he said.
Science is leading the way in gene therapy, Suder said, as they look at, down the road, being able to cure hemophilia through gene alterations and gene therapy.
Dobtiz also advocates for blood donation, which Solem only had a couple of but could need at any time if having a serious bleed.
To promote awareness of hemophilia, a disease with which not many people are very familiar, the HFMD is holding a Step Out for Bleeding Disorders fundraising walk Sunday at Lindenwood Park in Fargo. It is the first kind for North Dakota, but walks previously were held in the Twin Cities. Donations will be accepted at that time for the Solem family.
“There’s currently no cure, but the treatment is outstanding,” Paist said. “If the child gets proper care, the chances are he can lead a very active, healthy life.”
Despite his illness, Solem is said to be able to have a relatively normal childhood and he’s looking forward to turning three in November.
For more information on Sunday’s walk, visit www.firstgiving.com/hfmd/NorthDakotaWalk. For more information about hemophilia, visit the National Hemophilia Foundation’s website at www.hemophilia.org. Contact Bryce Martin at firstname.lastname@example.org.