Is there a bleeder in the house?

I've blogged many times about the bleeding disorder in our family which affects our 8-year-old daughter Star the most. Since Star was diagnosed with von Willebrand Disease, we have taken a personal crusade to inform people about this disease and prevent senseless deaths like my mother's. I hope you can help me spread this blog especially to doctors and parents. Who knows, through this you'll be able to save a life. For those with a similar experience, please PM me or email me.

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Is there a bleeder in the house?
By Andrea Trinidad-Echavez

Does your child easily bruise or frequently nosebleed? Do you wonder why he or she always gets bloody while brushing teeth?

Don't take those seemingly harmless signs for granted. They might just be symptoms of something else.

For years, my husband and I had been looking for answers to the condition of our youngest daughter, Star.

Catching colds from her older siblings few days after she was born, we were alarmed when blood started dripping from Star's tiny nostrils barely before she turned a month old.

At first, we thought hers was only a bad case of colds. Nevertheless, alarmed of the sight of blood, we rushed her to the emergency room. The pediatrician ordered blood work-ups on Star. When the results came, there was nothing significant in the findings except that she was anemic. She was given an extra dosage of iron.

As she was growing up though, Star's nosebleedings became more frequent. It came anytime of the day. Initially, we thought it had something to do with the temperature. But even when we confined her inside an air conditioned room on humid days, she would still bleed. It didn't also matter whether she was indoors or outdoors, or playing, sitting down, or sleeping.

Over the years, we got used to her teachers, school nurse or even school service driver calling us to express concern over her frequent nosebleeding. (Star started going to school at 6 months under UP Diliman's Infant Development Program of the Child Development Center.) We could only assure them that we had been seeking medical help on her case. She had undergone so many work-ups, ranging from blood tests to ENT (ear, nose, throat) checks and even neurological tests. Yet, specialists could not see anything wrong with her except that she was perennially anemic.

Last year though, Star's nosebleeding came at an alarming frequency – this time, almost daily. Sometimes, it would come up to four times in a day. After a series of trips to different specialists, we were already becoming frustrated. By then, Star was nosebleeding for almost a month in a row.

Family of bleeders

We had all the reasons to be alarmed. Bleeding runs in our family. My mother literally bled to death while undergoing a minor surgery. This, even after going through routine blood tests prior to the operation. The 10 bags of blood transfused on her didn't help at all. Few hours after she entered the operating room, she expired on the operating table while the doctors were trying to stop her bleeding. She was only 51. An uncle, my mother's older brother, almost died of bleeding while undergoing tonsillectomy. My eldest sister suffered hemorrhage and almost died during her first childbirth. My other older sister had two or three miscarriages.

Every time we bring our daughter to a doctor, I would give a rundown of our family history. Yet, it didn't seem to help pin down where Star's nosebleeding came from. Finally, we were referred to a well-known hematologist who specializes on hemophilia.

Von Willebrand Disease

"I suspect your daughter has von Willebrand Disease," Dr. Mary Chua, founder of Hemophilia Association of the Philippines for Love and Service (HAPLOS), told me after I gave her Star's and our family history.

Von Willebrand Disease, she explained, is a rare clotting disorder similar to hemophilia. While hemophilia is considered a "men's disease" because it occurs primarily among males, von Willebrand Disease can affect both males and females.

Rare genetic disorder

According to kidshealth.org, von Willebrand Disease is the most common bleeding disorder. But bleeding disorders, in itself, is considered rare. Von Willebrand Disease, for instance, affects barely 1 percent of the population. In the Philippines, there are less than 20 known cases.

People born with the disease may either have defective von Willebrand factor, low levels of von Willebrand factor or no von Willebrand factor at all.

Von Willebrand factor is a protein in the blood that is necessary for clotting. Without it or with defective von Willebrand factor, the blood takes longer to clot. This causes abnormal bleeding, and in the case of my mother, could even lead to death from blood loss.

No diagnostic facilities in the country

Unfortunately, there are no facilities in the country that can diagnose von Willebrand Disease. We were told the tests are quite complex that it needed special reagents and machines. The technology is only available either in Hongkong or Singapore in Asia. We were advised to bring our daughter to Queen Mary Hospital in Hongkong.

We flew to Hongkong November last year. The procedure was as simple as regular blood tests, only more blood samples were taken. Since von Willebrand Disease is a hereditary disorder, the doctor also took blood samples from me.

Because of the intricate procedures in the tests, the diagnosis came out only in March this year. It confirmed Dr. Chua's suspicion. My daughter has von Willebrand Disease Type 2M. And I have it too.

Types of von Willebrand Disease

According to the World Hemophilia Federation, there are three main types of von Willebrand Disease – Type 1, Type 2 and Type 3.

Type 1 is the most common form of the disease, accounting for approximately 80% of all cases. It involves a deficiency of the von Willebrand Factor.

Type 2, of which there are four subtypes – Type 2A, B, M and N, involves a qualitative defect of the factor wherein it does not function properly.

Type 3, on the other hand, involves a near or complete absence of the factor, and is the most serious type.

Diagnosis

Diagnosing von Willebrand Disease is not easy. That is why a lot of patients live with it for many years before finding out that they have it, if at all. Many doctors may not even be familiar with it.

In our case, we have sought out at least 10 specialists over the past seven years, ranging from hematologists, ENT, neurologist to nephrologists, if only to find out what was causing our daughter's nosebleeding.

Family history

Unlike hemophilia, von Willebrand Disease cannot be spotted in just a few blood tests. Oftentimes, it is misdiagnosed because many doctors are not familiar with it. For instance, a person with a low platelet count because of Type 2 von Willebrand Disease could be diagnosed with leukemia. Or a woman with heavy, prolonged menstrual bleeding because of von Willebrand Disease, who has not responded to hormone therapy, could be advised to have a hysterectomy.

In some cases, nosebleeding could be construed as deviated septum (physical abnormality in the nose) or that it could simply be brought about by either extreme or sudden change in temperatures.

Dr. Chua said this is where clinical history plays a crucial role. Most documented von Willebrand Diseases cases in the Philippines were identified through their clinical history, such as the symptoms shown by the patient. These may include frequent or prolonged nosebleeding, easy bruising, gum bleeding, prolonged or heavy menstruation, and prolonged bleeding during surgery, accident, medical or dental procedures.

Family history could also be very helpful in identifying possible von Willebrand Disease patients. In our case, my side of the family history helped Dr. Chua identify my daughter as a probable patient.

Laboratory tests are necessary to know what type of von Willebrand Disease the patient has so that appropriate treatment could be administered.

Not fatal but…

Fortunately, von Willebrand Disease is not as fatal as cancer, leukemia or other blood diseases. People affected by the disease may still live a normal life for as long as they receive regular treatment.

Treatment can vary from nasal spray, oral medication or blood transfusion depending on the type of von Willebrand deficiency.

But patients are normally advised against engaging in high impact activities that may cause injury. Because of the clotting factor deficiency, deep cuts may take long to stop and worse, may lead to death by blood loss like my mother. Von Willebrand Disease patients are also prone to internal bleeding.

In von Willebrand Disease, according to Dr. Chua, it's the patient's quality of life that suffers. "They have to contend with constant bleeding. They may feel weak or they may acquire other diseases as a result of the low immunity," she said.

Prior to any medical or dental procedure, patients are also advised to see their hematologist for proper treatment to regulate clotting and prevent blood loss.

So, if you suspect someone in your family has a clotting problem like von Willebrand Disease, don't waste time. See a hematologist. It's always better safe than sorry. (ate)

My Hero

Your miracle begins when you place whatever is in your hands into Jesus' hands.

It's been a roller-coaster ride of sorts the past month or so with all of us in the family, save for the hubby, getting sick one after the other. I had been practically bedridden for almost a week and managed to get back on my feet only late last week. Up to now, Star is still recovering and thankfully, she no longer has fever this evening.

I was tempted not to attend this morning's service because Star still had fever and she begged me to stay. But thanks to my husband's kindness, he volunteered to stay instead. I'm so thankful to the Lord for finally being able to attend service after missing it for three Sundays (first Sunday I was in Malaysia and the next two, we got sick).

Too bad I didn't catch the first parts of the Hero series. But the preaching on "The Kid" today was as powerful I'm sure. It talks about the little boy who gave up his five barley loaves and two fishes. We all know how the Lord used the little boy's unselfishness act to feed thousands. The little deed of a nameless child became one of Jesus' greatest miracles recorded in the Bible. Who would have thought that five little loaves and two tiny fishes would feed thousands of hungry followers, and with leftovers at that! In the hands of the little boy, the loaves and the fishes could have been more than enough to sustain him throughout the day. But he chose to give it to Jesus. And in Jesus' hands, the miracle happened.

The story reminded me of so many incidents where I saw God's miracles up close and personal. I dare not imagine what could have happened if Jesus didn't come to the rescue. Just last year, we were preparing to bring Star to Hongkong for her diagnosis. By our initial pencil pushing, we would easily spend tens of thousands of dollars in hospital bills alone. Already, we were a bit concerned. Then God pulled out a surprise -- Ben was diagnosed with Primary Koch Infection. Weeks later, just right before we were supposed to leave for Hongkong, he got confined. Worse, the water in his lungs increased so quickly and the doctor told us to brace for an OR appointment.

By this time, we were a bit shaken. We didn't see it coming. But then we realized, surely, God wouldn't give us something that He knows we couldn't take. We knew He would heal Ben. He had awaken Ben from coma when he was a tiny baby. How much more now that Ben was bigger and stronger. Yet, the mounting hospital bills and Star's overdue diagnosis stared at us in the face.

In my heart though I knew God would walk us through it. I didn't know how but I knew He would. He had plucked us out of the valley of darkness too many times for us to doubt. And so like the other times, I would surrender all concerns to the Lord and say, "Lord, problema mo na yan. Bahala ka na."

So with confidence, Star and I left for Hongkong not knowing how in the world would we be able to pay the bills both in Hongkong for Star and in Manila for Ben. But God is our Jehovah Jireh! Because we passed on the "ball" in His hands, He took over. Suddenly, blessings after blessings poured in. An overdue payment came -- more than enough to settle Ben's hospital bills. And more unexpectedly, a church in Hongkong gave us a love gift that enabled us to settle Star's hospital bills. What more, one of their pastors chaperoned us all the time and wouldn't let us spend a cent. So, after Star's diagnostic tests, we even had more than enought to visit Ocean Park and Disneyland!

Everytime we go through difficult times, I look back at the birth of Ben (how God woke him up from coma and now he's almost as big as I am), the incident last year and the many little triumphs God gave us along the way. My faith may not be intact all the time. Sometimes I falter. But one thing is sure, God is a mighty God. He is a miracle-working God who is the same yesterday, today and forever. The miracles He performed in the olden times, He is still able to perform today if we only allow Him to. But the miracle will only begin if we put all our cares in His hands.

Ben is in the hospital again

After getting recharged from my trip in Kuala Lumpur, life has become a roller coaster ride of sorts again. I had to work double time on some unfinished businesses. Then Ben complained of dizziness. When I got home, I found out he missed school because he wasn't feeling well. I brought him to his pedia the day after I arrived and she ordered a new x-ray on Ben. We found out his Primary Koch has not yet completely healed. It means he has been having asymptomatic pneumonia for the past two years of his life. His pedia, a pulmonologist, said she found it quite strange. Even though Ben was born premature and had pneumonia at birth, he should have already been well enough by this time considering all the medicines he had taken. Dra. Mendoza, who heads St. Luke's pulmonary department, said she might have to do bronchoscopy on Ben soon to find out what's inside his lungs and why his pneumonia just wouldn't go. Aside from pneumonia, his allergic rhinitis is getting worse, so he was given two weeks worth of anti-biotics. We've been convincing him to take wheatgrass to boost his immune system. But sometimes it's just so difficult to convince kids to try something new. =(

After more than a week of taking antibiotics, Ben felt a little better only to get fever last Sunday. Like last year prior to his hospitalization, he wouldn't respond to paracetamol. When the fever persisted and even went up to 40.5C despite round-the-clock medication, we decided to bring him to St. Lukes. We stayed at the ER from midnight to 7am and went home when his fever subsided. But by mid-morning, he had high fever again so we went back to see his pedia. Upon seeing him red and all, Dra. Mendoza told us not to go home anymore.

Thankfully, his CBC shows high platelet count. But Dra. Mendoza said we should pray he doesn't have dengue although she highly suspects because of his physical appearance (red).
Today is Ben's second day in the hospital. Thank God, he is better. At least, there was a little window today when his fever was gone. Tomorrow, they will get his blood sample for CBC again.

Well, everything happens for a reason. Maybe, God wants to slow me down a bit to remind me where priorities should be. I'm sure He's telling Ben and each one of us other things as well.

I thank God that He is faithful in all of His ways. Times like these makes me appreciate Him more. Not that I like being in difficult situations. But truly when you're under fire, it makes you appreciate more the things that God has given but you sometimes take for granted -- like relationships with family and friends.

I praise God for the support of all our families (blood and spiritual) as well as friends. They are indeed God's great channels of blessings.

Please continue to pray for Ben's fast and complete recovery.