Healthy Living

Couple Raises Twins with Cystic Fibrosis

Couple Raises Twins with Cystic Fibrosis

There’s a lot for parents to process and prepare for when they have a baby on the way. To find out that twins are coming monumentally adds to the planning necessary for their arrival. For parents to know they’ll be caring for children with a debilitating genetic disease is even more pressure than most people could fathom.

But, that is the reality for the Hively family of Atlanta, Georgia. Amanda Hively and her husband were still processing that they were having twins when they found out at 20 weeks that their twins likely had cystic fibrosis. Though a daunting diagnosis, the Hively family have done a tremendous job of balancing raising two young boys and managing their disease and treatments.

More about CF

Cystic fibrosis (CF) is a genetic disorder that causes abnormal function of the secretory glands, which control body fluids like mucus and sweat. The dysfunction of these glands leads to a buildup of a thick mucus in the organs. This buildup can severely affect lung and pancreas function. In the short term, CF makes someone very susceptible to infections and interferes with digestion. Long term, the mucus buildup can cause severe lung damage, and block breathing to the point of respiratory failure.

Each year, 1,000 people are diagnosed with CF in the United States, and 30,000 people are currently living with the disease. CF is something that a patient is born with; it does not develop over time, nor is there anything you can do to cause or prevent CF from developing. So, more than 75% of people with CF are diagnosed before age two. In the case of CF, being diagnosed as a young child is actually a good thing. The longer that physicians are aware of the diagnosis, the better the symptoms and mucus can be managed and ultimately the long-term effects may be less severe, and a high quality of life may be maintained for a longer period of time.

The twins’ diagnosis

As stated, the majority of CF patients are diagnosed as young children. In the case of the Hively twins, physicians recognized the likelihood of CF while still in utero due to digestive problems apparent on the ultrasound. Amanda Hively and her husband were both carriers for CF, so their children each had a 25% chance of being born with the disease.

The first step to diagnosing CF is a blood test or genetic test. If these tests suggest CF, doctors will then conduct a sweat test. This test measures the amount of salt present in sweat. Salty skin is a major symptom of CF. High salt levels, in conjunction with positive blood or genetic test results, indicates CF.

A blood or genetic test is always performed on newborns as part of the newborn screening program to identify chronic diseases. Early detection of CF is extremely important in order to prevent more serious problems later in life.

CF in children

Currently, there is no cure for CF. However, there are many treatments and therapies to reduce the severity of symptoms of CF and prolong a healthy life for patients. Early detection and starting treatment at a young age can improve the quality of life for years to come.

Cystic fibrosis can affect children in many ways. Most notably, perhaps, is its impact on growth. CF can hinder the release of digestive enzymes in the stomach, thus inhibiting the absorption of valuable nutrients. This can negatively impact the growth and development of children, who typically grow at a rapid-fire pace. Research shows that children who are diagnosed and begin treatment early in life have better nutrition than those whose treatment is delayed. Mucus secretions in the lungs also can cause shortness of breath and other asthma-like symptoms. This means they may need to refrain from participating in sports and other activities common to children due to the risks these activities can pose to their health.

This, along with demands of CF treatment, can take an emotional toll on children and adolescents living with the condition. The inability to participate in typical childhood activities can lead to feelings of social isolation. In addition, the impact CF has on digestion can lead to stunted growth, and children will likely be smaller than their peers. Additionally, puberty may be delayed by a few years. Given all of these factors, it’s completely normal that children with CF may feel some emotional distress. It’s important for the child’s family and caregivers to look out for signs of depression and anxiety. It may be a good idea for the child to talk to a therapist, or even better join a support group of other children with cystic fibrosis. Finding others who are experiencing the same things will give the child a sense of community, make them feel like less of an outsider, and most importantly remind them that they are not alone.

Another symptom of CF is increased susceptibility to lung infections such as bronchitis and pneumonia. The large amount of mucus in the body traps the pathogens responsible for these infections, making them a recurrent issue. This is especially problematic in children, who are already more likely to get infections than adults. In children with CF, extra caution needs to be taken to avoid the spread of germs that could lead to infection.

Treatment for children with CF

There’s other challenges specific to children with CF. While there is an ever-growing number of treatments for CF, some of them can be harder to implement when dealing with young children rather than adults, or even older adolescents. One of these treatment methods involves using an inflatable vest to help clear out mucus that has built up in the chest. But, this specific treatment requires patients to stay relatively still for a period of time, which is no easy feat for children. As Amanda Hively pointed out, her sons have now even learned how to press buttons and turn off the machine controlling their vests. And this is just one example of the extra difficulties that comes with dealing with young children with a chronic disease.

While most kids live a carefree, relatively schedule-free lifestyle, CF treatments are timely and require sticking to a pretty strict schedule. The exact treatment plan will vary from patient to patient, but typically involves both pulmonary treatments and nutritional therapy to ensure children are getting the nutrients necessary for development.

Additionally, children will need to see their doctors for checkups every 1-3 months. Amanda Hively and her family make the 2-hour trek to Atlanta every few months so that her twins can be seen by a team of specialists at Children’s Healthcare of Atlanta.

There are 120 Cystic Fibrosis Foundation certified care centers all over the country. These care centers are located within hospitals, and both provide patient care and participate in clinical research. A specialized CF care team can ensure that children are getting the best possible treatment options to postpone and possibly even prevent CF associated negative health outcomes later in life. These specialized care centers also are more likely to be able to guide you to appropriate mental health resources and support networks for families dealing with cystic fibrosis.

Amanda Hively makes a great point that she and her husband are simply trying to balance raising two young sons with raising children struggling with a chronic illness. As anyone who has cared for a family member with CF or other chronic illnesses know, this is no easy feat. But, a great care team and knowing where to find support can help make this very difficult task just a little bit easier.